Medicine General 01 Flashcards

Question

Asthma Severity Markers Moderate - severe - Life threatening

Answer 1

Moderate asthma: = PEF >50-75% of best or predicted = No features of severe asthma = increasing symptoms ------------------------ Severe Asthma: = Inability to complete sentences in one breath = PEF 33-50% of best or predicted (normal 450-550 L/min in adult males and 320-470 L/min in adult females) = Resp Rate > 25 = Heart rate > 110 ----------------------------- Life threatening Asthma: = Silent chest = PEF < 33% = Cyanosis = Feeble respiratory effort = Arrhythmia, Hypotension = Exhaustion, altered sensorium = sPo2 < 92% = PaO2 < 8 kpa = Normal PaCO2 (4.6-6.0 kpa) should be low, Respiratory acidosis , needs NIV

Answer 2

Treatment for severe asthma: = Oxygen supplementation = Salbutamol/Terbutaline puff: 4 puff stat and 2 puffs every 2 min - total 10 = Nebulization: Ventolin/Terbut 5/10 mg = Nebulization: Ipratropium 500-4 hrly = Prednisolone 50 mg OD x 5 days = MgSO4 2 Grams iv over 20 min = Aminophylline 5 mg/kg loading dose 20 min and infusion@ 0.5 mg/kg/Hr

Answer 3

FEV1 = Forced expiratory volume in 1second FVC = Forced vital Capacity = the amount of air that can be forcibly exhaled from your lungs after taking the deepest breath possible. FEV1/FVC ratio is 0.8 - normally ----------------------- Obstructive respiratory = Ratio < 0.7 Restrictive Respiratory = Ratio > 0.8

Answer 4

The FEV1/FVC ratio (Tiffeneau-Pinelli index) is used in the diagnosis of obstructive & restrictive lung disease = proportion of a person's vital capacity that they are able to expire in the first second of forced expiration to the full, forced vital capacity. ERV: Expiratory reserve volume: the maximal volume of air that can be exhaled after normal end-expiratory position FEFmax: The maximum instantaneous flow achieved during a FVC maneuver FEFx: Forced expiratory flow related to some portion of the FVC curve; modifiers refer to amount of FVC already exhaled IC: Inspiratory capacity: the sum of IRV and TV PEF: Peak expiratory flow: The highest forced expiratory flow measured with a peak flow meter TLC: Total lung capacity: the volume in the lungs at maximal inflation, the sum of VC and RV VC: Vital capacity: the volume of air breathed out after the deepest inhalation

Answer 5

NIV Inclusion criteria: = Alert, co-operative patient = able to protect airway = Recovery to quality of life acceptable = Patient wishes = Endotracheal intubation considered inappropriate for some reason

Answer 6

NIV Exclusion Criteria = Life threatening hypoxemia = Severe comorbids = confusion-agitation-cognitive impair = Facial burns-trauma-recent surgery = Fixed upper airway obstruction = Undrained pneumothorax = Upper GIT surgery - gastrectomy = Inability to protect airway = copious respiratory secretions = Patient moribund = Bowel Obstruction = Hemodynamically unstable, inotropes

Answer 7

CPAP Indications = Hypoxemic respiratory failure = Acute hypoxemic pulmonary edema ------------------ BiPAP indications: = Hypercapneic resp failure: COPD = Acute Hypercapneic resp failure in cases of: Chest wall deformity, NMD, decompensated OSA, = Cardiogenic pulmonary edema refractory to CPAP = Pts in whom intubation inappropriate = Type I RF resistant to Oxygen alone = Weaning from mechanical ventilation

Answer 8

BiPAP - when to start in COPD = PH between 7.25 to 7.35 = Pco2 > 6 KPA = Maximal medical therapy failure (Nebs, Oxygen, Prednisolone, Atrovent, Antibiotics etc)

Answer 9

NIV initial settings: CPAP - start 5 cmH2O - max up-to 15 Oxygen - to maintain sPo2 > 88-92% PH Target > 7.35 BiPAP: = IPAP of 10, increased by 2 up to 20 = EPAP of 3-5 cm H2O

Answer 10

Well's score for PE probability = Clinical DVT likely = 03 pts = Alternate diagnosis less likely = 03 = Heart Rate > 100 =1.5 pts = Immobilization/Surgery <4 wks, 1.5 = History of DVT or PE = 1.5 = Haemoptysis = 1 = Malignancy: ActiveRx/Within last 6 months/ on Palliative care = 1 pt ------------------------ Score > 6 = High probability Score < 2 = Low PE probability, 1.6%

Answer 11

Massive PE points - suspect clinically alone if collapse, tachycardic, unexplained Hypoxia, Dilated RA on bedside ECHO, Neck veins distended, Hypotension, ECG - Thrombolysis immediate if unstable on clinical grounds alone - Alteplase: 100mg if stable, 50 if not - Give UFH after 3 Hrs of thrombolysis ECG changes - RAD, RBBB, S1Q3T3, Sinus Tachycardia alone, Inferior MI, etc

Answer 12

CURB-65 Score 0-1: 1/2/3 1. Amoxycillin 500 mg TID or 2. Doxycycline 200 mg stat -100 BD or 3. Clarithrymycin 500 mg BD (no dual antibiotics here) ------------------------------------------ CURB-65 Score > 2 = Dual antibiotics Amoxycillin + Clarithromycin/ Doxycycline/ Levofloxacin 500 mg OD/ Moxifloxacin 400 mg OD -------------------------------------------- CURB-65 Score > 3 = Dual antibiotics 1. Augmentin 1.2 Gram TID + Klacid iv or 2. Benzyl Penicillin 1.2 Grams + either of those = Levoflox/ Ciprofloxacin/ Cefotaxim/ Ceftriaxone/ Klacid

Answer 13

HAS-BLED is an acronym for Hypertension, Abnormal liver/renal function, Stroke history, Bleeding predisposition, Labile INR, Elderly, Drug/alcohol usage.

Answer 14

small = << 2 cm at Hilum Large = > 2 cm at hilum -------------------------------- If secondary (smoker, > 50yr old) = ICD if primary (young with no co-mobids) = Aspirate observe, discharge ------------------------ Aspirate up to 2500 ml air with a syringe connected to 14/16 Gz cannula in 2nd ICS just above third rib with a 3 way cock other side connected to IV set dipped into saline bottle. Aspirate and push into under water side tube. ------------------------- **Repeat CXR in 2-4 weeks** no AIR-TRAVEL until after 1 week of full resolution in CXR and clinically no DIVING forever

Answer 15

= Tracheobronchitis = Chronic bronchitis = Pneumonia = Tuberculosis = Malignancy, Ca Lung, Ca bronchus = Pulmonary AV malformations = Pulmonary sequestration = Pulmonary infarction = Aspergilloma = Traumatic

Answer 16

= Tracheobronchitis = Chronic bronchitis = Pneumonia = Tuberculosis = Malignancy, Ca Lung, Ca bronchus = Pulmonary AV malfromations = Pulmonary sequestration = Pulmonary infarction = Aspergilloma = Traumatic

Answer 17

= Pulmonary embolism = LV failure = Mitral stenosis = Pulmonary arterial Hypertension = Aorto-bronchial fistula -------------------------------------------- Vasculitic causes: = Wegners granulomatosis = Goodpasture syndrome = SLE

Answer 18

Symptoms of acute prostatitis include severe pain in or around the penis, testicles, or anus; lower abdomen or lower back pain; dysuria; urinary frequency (particularly at night); urgency; problems related to "stop-start" urinating, and the presence of blood in the urine. Patients also report feeling unwell (aches, pains, and possibly fever) and experience lower back pain and pain on ejaculation. Symptoms requiring urgent medical attention include being unable to urinate, which leads to a build-up of urine in the bladder (acute urinary retention) Fosfomycin is only effective for treating lower UTIs, not upper UTIs.

Answer 19

This indicates the amount of solute dissolved in the urine. **Causes of low specific gravity** include conditions that result in the production of dilute urine (e.g., diabetes insipidus). **Causes of raised specific gravity** may include dehydration, glycosuria, and proteinuria (e.g., nephrotic syndrome). ------------------------- pH level indicates the acidity of urine. **The normal pH range is 4.5–8**. **Low urinary pH** may be caused by starvation, diabetic ketoacidosis, and other conditions that cause metabolic acidosis (e.g., sepsis). **Causes of raised urinary pH** may include a urinary tract infection, conditions that cause metabolic alkalosis (e.g., vomiting) and medications (e.g., diuretics). ----------------- Nitrites are a breakdown product by gram-negative organisms such as E. coli. The presence of nitrites may suggest a urinary tract infection.

Answer 20

Antibiotic treatment for asymptomatic bacteriuria is **not recommended** for the following population groups: = Women older than 65 years (ASB occurs in 15–20% of women between 65–70 years old) = Pre-menopausal & non-pregnants = Diabetic women = Older persons in the community (no correlation can be found between treatment and an improved outcome in older persons) = Elderly institutionalized patients = Persons with a spinal cord injury = Catheterized patients while the catheter remains intact

Answer 21

Lower UTI Rx: Non-pregnant females (16 years and older): = Acute and uncomplicated lower UTIs may resolve without antibiotic treatment. A backup prescription can be given in the event that the symptoms worsen or do not improve within 48 hours. --------------------------------------- Males (16 years and older), pregnant females (12 years and older), and children (3 months–16 years old): = An immediate prescription is recommended. An MSU should be obtained and sent to the lab for culture and susceptibility before treatment. In children under 16, consider the appropriate guidelines for diagnosis and management.

Answer 22

Children at high risk for serious illness, e.g., cancer: Seek specialist advice Non-pregnant females: If behavioral and personal hygiene measures were ineffective, the following could be considered: = a single dose of antibacterial treatment = a trial of daily antibacterial treatment if a single dose treatment was inadequate. Males (16 years and older), pregnant females, and children under 16 years: = If behavioral and personal hygiene measures were ineffective, a trial of daily antibacterial prophylaxis could be considered.

Answer 23

These options include vaginal estrogen, immunotherapy, D-mannose (a monosaccharide in many plants and fungi, including cranberries), and methenamine.

Answer 24

Spleenomegaly + Rheumatoid arthritis + Neutropenia Other features: = Lymphadenopathy = Skin pigmentation = Vasculitic Leg ulceration = Keratoconjunctivitis sicca = thrombocytopenia = ANA and RA factor both positive

Answer 25

Causes of nephrotic syndrome are: (MMM-FF) 1. Minimal change disease in children 2. Focal, segmental Glomeruloscleritis 3. Membranous Glomerulonephritis 4. Mesangio-capillary glomerulonephritis 5. Fibrillary glomerulonephritis

Answer 26

1. Primary (Idiopathic) MCD: with atopy(HLA B-12) or without atopy 2. Secondary MCD: = Lymphoma = cancers (Renal, Lungs, Pancreas) = IgA nephropathy = Diabetes mellitus = AIDS **IgG to Transferrin clearance** ratio is < 0.15 in minimal change disease

Answer 27

1. Primary, idiopathic, superimposed on MCD 2. Secondary: = HIV infection = Drugs: Heroin abuse, NSAIDs, analgesic abuse = Reduced renal mass: cortical necrosis, renal dysplasia = Normal renal mass: DM, HTN, Alport syndrome, Sickling, Cystinosis, Sarcoidosis

Answer 28

1. Primary, idiopathic 2. Secondary: = Infections: Hepatitis B/C, Malaria, Schistosomiasis = Multisystem: SLE, Mixed CTD, Dermatomyositis, Sjogren’s = cancers: Lung, Colon, Stomach, Breast, Lymphoma = Drugs: Gold, Mercury, Penicillamine = Familial: sickle cell disease = Miscellaneous: de novo in renal allografts, Bullous pemphigoid, Franconi syndrome

Answer 29

1. Primary, idiopathic = Type 1. Subendothelial deposits = Type 2. Dense deposit disease 2. Secondary: = SLE = Cryoglobulinemia = Scleroderma = Light and heavy chain disease

Answer 30

Fibrillary Gn can be due to: = Amyloidosis = Multiple myeloma = Cryoglobulinemia = Lupus nephritis

Answer 31

Excess serotonin(5HT), bradykinin, histamine, tachykinin, prostaglandins from tumors of enterochromaffin cells ————- Tumor sites: appendix, stomach, duodenum, bile ducts, ileum, gonads, pancreas, lungs etc High urine HIAA (5-Hydroxyindoleacetic Acid)for diagnosis Do CT, MRI, PET scan ————- Face, upper chest flushing Headache after flushing Bronchospasm wheeze Diarrhoea: 2-30 episodes per day Tachycardia with Hypotension Weight loss, cachexia, Tummy cramp ——————————- Triggers for flushing: alcohol, food, stress, Liver palpation, or after administering pentagastrin, catecholamines or reserpine.

Answer 32

Cushing’s syndrome - ectopic ACTH from bronchial carcinoid ———— Dilutional Hyponatremia due to ADH from bronchial carcinoid —————- Hypoglycemia due to insulin from pancreatic carcinoid ————————- Acromegaly due to ectopic Growth Hormone releasing hormone, GHRH from foregut carcinoid ————————- Gynecomastia due to excess HCG from gastric carcinoid

Answer 33

Tumor debulking surgery Somatostatin analogues Cyproheptidine Leukocyte interferon **Chemotherapy** with Cisplatin, Etopocide, Doxorubicin, Streptozocin

Answer 34

MEN 1 is an **autosomal dominant** disorder characterized by the occurrence of multiple tumors, particularly in the pancreatic islets, parathyroid and pituitary glands, and neuroendocrine tumors. The **MEN 1 gene** is located on band **11q13** and likely functions as a tumor-suppressor gene. In a study of 46 sporadically occurring tumors, 78% had LOH at this site, with almost the entire allele missing in 5 patients. In the remaining cases, genetic heterozygosity had a discontinuous pattern. Some have postulated that sporadically occurring **carcinoid tumors** evolve after inactivation of a tumor-suppressor gene on chromosome 11 as well as genetic mutations that affect DNA-mismatch repair.

Answer 35

Urinary 5-HIAA levels increased but may not help in detecting tumors at an early stage of development when they are potentially curable with resection. measurement of other peptides (eg, SP, neuropeptide K, chromogranin) may be necessary for diagnosis and follow-up. Fasting plasma 5-HIAA assay is more stable than whole-blood serotonin assay and is more convenient than 24-hour urine collection. In one study, CDX2 was highly indicative of GI carcinoid tumor, whereas TTF-1 had high specificity for pulmonary tumors. One (17%) of 6 gastric carcinoids stained with CDX2, whereas 8 (53%) of 15 pulmonary carcinoids stained with TTF-1. None of the GI tumors stained with TTF-1

Answer 36

= Pruritus with scratches, excoriations = Hepatomegaly, may be massive = Icterus, Jaundice = Usually middle aged female = Xanthelasma, around eyes = Spleenomegaly may be = Xanthomata over joints, skin folds, trauma lines = Malabsorption, Osteomalacia, = Coagulopathy

Answer 37

Clinical: Icterus, Pruritus, middle age female, Xanthelasma, Hepatomegaly, ? Spleen palpable Signs of Cirrhosis if late - ascites, asterixis, Encephalopathy Labs: Antimitochondrial antibody (>95%) RA Factors, SMA +, Anti-Thyroid + Liver Enzymes: Raised ALP, Bilirubin Liver Biopsy Rx - Oral Urso-Deoxy-cholic acid Low Fat Diet + Statins Fat Soluble vitamins(ADEK) to give Rx of Osteomalacia Pruritus Rx: UDC, Cholestyramine, Barbiturates, Rifampicin, Naloxone, Propofol, or = Norethandrolone in resistant cases but increased jaundice (sold under the brand names Nilevar and Pronabol among others, is an androgen and anabolic steroid medication which has been used to promote muscle growth and to treat severe burns, physical trauma, and aplastic anemia but has mostly been discontinued. )

Answer 38

= Sjogren's syndrome = Systemic sclerosis = CREST syndrome = Rheumatoid arthritis = Hashimoto thyroiditis = Renal tubular acidosis = Coeliac disease = Dermatomyositis = Skin diseases- Discoid LE, Pemphigoid, Lichen planus

Answer 39

Xanthelasma are yellow growths on or near the eyelids. They can be flat or slightly raised. They form when deposits of cholesterol (lipid or fat) build up under the skin. While xanthelasma themselves are not harmful, they can be a sign of heart disease, PBC. Xanthelasma palpebrum is the most common cutaneous xanthoma, characterized by yellowish plaques over eyelids – most commonly, over the inner canthus of the upper lid. It is triggered by hyperlipidemia, thyroid dysfunction, and diabetes mellitus.

Answer 40

Autosomal recessive, HLA-A3 Chromosome 6, short-arm, C282Y Cysteine is replaced by Tyrosine@282 ------------------- Iron Deposition in body, male any age, female usually after menopause - Slate Grey skin - Arthropathy, PsuedoGout, 2/3 MTP - Hepatomegaly, spleenomegaly - Decreased Hypothalamic-Pituitary axis causing Testicular atrophy, Addisons disease, Weight loss, Postural Hypotension, - Diabetes mellitus with thin body and normal insulin levels - Cardiomyopathy, CHF - Cirrhosis: Palmar erythema, Jaundice, Ascites, Spider naevi, Pruritus, Coagulopathy, Varices with bleeding - Hypothyroidism, Hypo-PTH

Answer 41

- Weekly phlebotomy untill Hb < 11, Ferritin in normal levels (Takes about 2-3 years - then maintainance phlebotomy every 3 months Progression to cirrhosis can be halted if this started in pre-cirrhotic state hence FAMILY screening is important. - Desferrioxamine if phlebotomy not possible regularly as in anemia - Ascorbic acid increases Iron excretion -

Answer 42

Alcoholic Liver disease might have high stainable iron levels and more iron on Liver Biopsy. Here it is important to check HFE mutation as venesection will stop progression to cirrhosis.

Answer 43

1. Hereditary Hemolytics: = Sickle cell anemia = Thalassemia = Hereditary spherocytosis = Hereditary elliptocytosis 2. Acquired Hemolytics: = Acquired Autoimmune HA = Primary Hemolytic anemia 3. Secondary Hemolytics: = Lymphoproliferative disorders = SLE = Non-Lymphoid cancers: Ovarian = Drugs: - MethylDopa, Mefenamic Acid = Infections: Brucellosis, Infectious mononucleosis = Chronic inflammations: UC

Answer 44

= Hemolytic anemias: Hereditary, Acquired, Secondary = Infective endocarditis(look for peripheral stigmata) = SLE (multi-organ signs, malar rash) = Pernicious anemia (B12 deficiency, SACD, neuroligical signs) = Infectious mononucleosis (URTI, Bull neck)

Answer 45

Autosomal dominant, family history Jaundice, Anemia, Splenomegaly Gallstones, colics Leg ulcers, skin ulcers **Low MCV, High MCHC** Growth delays, Pseudogout **Osmotic fragility test** Spherical RBCs on smear Low spectrin content in RBC **negative direct antiglobulin test** (DAT) and an elevated reticulocyte count.

Answer 46

Autosomal means the gene is located on any chromosome except the X or Y chromosomes (sex chromosomes). Genes, like chromosomes, usually come in pairs. Dominant means that only one copy of the responsible gene (causal gene) must have a disease-causing change (pathogenic variant) in order for a person to have the disease. Mutation is an older term that is still sometimes used to mean pathogenic variant. In some cases, a person inherits the pathogenic variant from a parent who has the genetic disease. In other cases, the disease occurs because of a new pathogenic variant (de novo) in the causal gene and there is no family history of the disease. Each child of an individual with an autosomal dominant disease has a 50% (1 in 2) chance of inheriting the variant and the disease. Typically, children who inherit a dominant variant will have the disease, but they may be more or less severely impacted than their parent. Sometimes a person may have a pathogenic variant for an autosomal dominant disease and show no signs or symptoms of the disease.

Answer 47

commonest causes: NHL and Hodgkins Other Causes: = Infectious mononucleosis, (URTI) = Tuberculosis, contact history = Brucellosis ? farm worker = Toxoplasmosis, (Glandular fever) = Malignancies = Sarcoidosis = Thyrotoxicosis (signs, symptoms) = Secondary syphillis = HIV related progressive generalised

Answer 48

Bimanually ballotable, can get above, resonant percussion on it, = Signs of CKD, ANEMIA, AVF, = ADPKD with single palpable = Renal cell carcinoma = Hydronephrosis = Hyeprtrophy of single functioning one

Answer 49

2% of adult malignancies Smoking is risk Spreads to aortic, para-aortic nodes Mets to : Lungs, Liver Skin, bone, Brain Hematuria + Pain + Mass in abdomen Malignancy Signs: Wt Loss, Anemia, Lymphadenopathy, Poor appetite, Fever, Polycythemia as tumor produces E-poetin.

Answer 50

= Facial plethora = Cyanosis of mucous membranes = Cyanosis of Hands, feet, Face = Bruises, spontaneous = Pruritus related scratch marks = Fundus: grossly dilated retinal veins = congested conjunctival vessels = Splenomegaly = High blood pressure ------------------------------ Labs: *Raised = RBC, Hb, Platelets, Hct *Low MCV with iron deficiency as exhausted stores (Low MCV + High Hb) *

Answer 51

= Habitual smoking, COPD = High altitude living = E-poetin producing RCC, Adrenal, HCC, Ovarian, cerebellar Hemangioblastoma, Phaeochromocytoma = Chronic Rt to Lt shunt = Chronic Hypoxemia, Pickwickian syn = Congenital low 2, 3-DPG = Cobalt poisoning = Chronic CO poisoning =E-poetin production by renal cysts, Hydronephrotic kidneys

Answer 52

= Peri anal fissures, fistulae, abscesses = chronic lip cracks and swelling due to granulomatous inflammation. = Chronic diarrhoea = Multiple laparotomy scars due to recurrent need for surgeries = RIF mass = Oral aphthous ulcers = Fever, Clubbing, Arthritis (including sacro-ileitis) = Erythema nodosum = Pyoderma gangrenosum = Iritis, Red eyes = Malabsorptions, anemia = Ankle dema, Hypoproteinemia = Angular stomatitis, recurrent --------------------------------- Rx Options: = Immunosuppression, Azathioprine = Amionsalicylates, Sulfasalazine, Mesalazine = Corticosteroids = Anti TNF ALFA antibodies as Infliximab, Adalimumab,

Answer 53

= Crohns disease, UC (IBD's) = Simple fistula after abscess = Tuberculosis = Ca Rectum = Trauma = Radiation therapy = Lymphogranuloma venerum

Answer 54

In Crohn's disease, there are healthy parts of the intestine mixed in between inflamed areas. Ulcerative colitis, on the other hand, is continuous inflammation of the colon. Ulcerative colitis only affects the inner most lining of the colon while Crohn's disease can occur in all the layers of the bowel walls. ---------------------------------- CD can affect any part of the GI tract, but most commonly involves both the large and small intestines. Although CD is more severe than UC, the global prevalence is much lower, -------------------------------

Answer 55

Microscopic colitis is a chronic inflammatory bowel disease (IBD) in which abnormal reactions of the immune system cause inflammation of the inner lining of your colon. chronic diarrhea. ----------------------

Answer 56

- Crohn's colitis - Caecal carcinoma - Amoebic abscess (travel abroad) - Lymphoma - Appendicular abscess - Ovarian mass - Ileal Carcinoid - Transplanted kidney - Faecal matter in Caecum

Answer 57

Portal hypertension is a term used to describe elevated pressures in the portal venous system (a major vein that leads to the liver). Portal hypertension may be caused by intrinsic liver disease, obstruction, or structural changes that result in increased portal venous flow or increased hepatic resistance. Cirrhosis of liver is the most common cause.

Answer 58

Effects of portal hypertension: = Ascites = icterus = Spider naevi = Dilated veins over abdomen (caput medusae) = Altered sensorium = Leuconychia, white nails = peripheral edema = Virchows node(also called Troisier’s sign) left supraclavicular in malignancy causing ascites

Answer 59

Cirrhosis of liver Congestive cardiac failure TB peritonitis Malabsorption low albumin Nephrotic syndrome Constrictive pericarditis Budd-Chairi syndrome Myxaedema Meig’s syndrome Pancreatic ascites (pancreatitis) Chylous ascites Malignancy: Gastric, Ovarian, Liver, Peritoneal mesothelioma or mets SAAG> 25 Exudative ascites SAAG < 25 - Transudate

Answer 60

Budd-Chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow. The obstruction may be thrombotic or non-thrombotic anywhere along the venous course from the hepatic venules to junction of the inferior vena cava (IVC) to the right atrium. = tender hepatomegaly + ascites + Icterus without other CLD signs Can be due to: = Polycythemia rubra Vera = Ulcerative colitis = Tumor infiltration = Severe dehydration, DKA, HHS

Answer 61

Huge spleen (more than 4 cm) = Myeloproliferative disorders such as CML, PCVera, myelofibrosis, essential thrombocythemia = Chronic Malaria = Kala Azar ———————- Moderate spleenomegaly, 2-4 cm = Myeloproliferative disorders = Lymphoproliferative disorders such as CLL, Lymphoma, Myelomatosis, Multiple myeloma, Hairy cell leukaemia, ALL, Waldenstroms macroglobulinemia also may have lymphadenopathy +Hepatomegaly) =Cirrhosis of Liver —————————— Mild spleenomegaly, upto 2cm = Myeloproliferative disorders = Lymphoproliferative disorders = All causes of Portal Hypertension =Infections as: Glandular fever, viral Hepatisis, SABE, Malaria

Answer 62

Brucellosis, Typhoid, Trypanosomiasis, Echinocochosis, Disseminated TB, Sarcoidosis Gaucher’s disease (Lipid issue) Haemolytic anemias Idiopathic thrombocytopenic purpura Paroxysmal nocturnal Hemoglobinuria Fealty’s syndrome in RA Bunty’s syndrome Amyloidosis SLE, chronic IDA Thyrotoxicosis

Answer 63

Chronic congestive spleenomegaly with pancytopenia, portal Hypertension and GI bleeding.

Answer 64

Common causes of Hepatomegaly = Cirrhosis, usually alcoholic = Congestive cardiac failure = secondaries in Liver ———————————- Other causes: = Infections: Weil’s disease(Leptospirosis), Glandular fever, viral Hepatitis A,B,E = Hepatocellular malignancy = Hepatic adenoma with OC pills = Lymphoproliferative disorders = Primary Biliary Cirrhosis = Macro-nodular cirrhosis = Haemochromatosis = Hepatic steatosis = Sarcoidosis = Amyloidosis = Hydatid cysts = Amoebic Liver abscess = Budd Chairi syndrome = Emphysema

Answer 65

Malignancy, primary or secondary Poly cystic liver with ? ADPKD Macro-nodular cirrhosis Hydatid cysts Syphillic gummas

Answer 66

With signs of CLD: = Cirrhosis with portal HTN = Chronic Autoimmune ———————————- With lymphadenopathy: = Lymphoma (Hodgkin, NHL) = Chronic lymphocytic leukaemia ———————————- Without both above: anemic = Myeloproliferative disorders = Lymphoproliferative disorders = Cirrhosis with portal HTN sometimes —————— Other causes: = Malaria, kala azar, TB, Schistosomiasis, Brucellosis, Leptospirosis, Toxoplasmosis, CMV, = viral hepatitis = pernicious & megaloblastic anemia = Storage disorders: Gaucher’s = Amyloidosis due to chronic diseases = Budd chairi syndrome = Infantile polycystic disease

Answer 67

Primary, Myeloma associated Secondary: to chronic illnesses = rheumatoid arthritis = TB, Leprosy = chronic sepsis = Crohn’s, UC = Ankylosing spondylitis = Paraplegia +bedsores +Recurrent UTI = Malignant lymphoma Amyloidosis Can cause: - nephrotic syndrome(proteinuria) - Hypoadrenalism (postural Hypotension) - GI tract issues (do rectal biopsy)

Answer 68

Hand signs of CLD: Leuconychia, Dupuytren’s contracture, Flapping hand tremors, wasting of hands, Pallor, Icterus, Clubbing Spider naevi on dorsum of hand Koilonychia, Paronychia, tendon xanthomata —————————- Other signs of CLD: Body spider naevi Icterus and pallor at eyes Caput medusae with blood flow away from umbilicus Hepatomegaly, tense distended abdomen (ascites), everted umbilicus Wasted extremities Signs of coagulopathy - bruises, ecchymosis Scratch marks over body due to pruritus Splenomegaly Gynecomastia, poor body hairs Small atrophic testicles Ankle edema due to low albumin

Answer 69

= Alcoholic liver disease = Viral hepatitis = Autoimmune hepatitis, SMA+ = Primary Biliary cirrhosis = Haemochromatosis = non alcoholic fatty liver disease = cryptogenic = cardiac failure, constrictive pericarditis = Budd Chairi syndrome = Biliary cholestasis = Wilson’s disease: KF rings, párkinsonian features, = alfa 1 antitrypsin deficiency ( also has COPD) = Toxic causes: Methotrexate, Carbon tetrachloride, Methyl dopa, INH, Amiodarone, Aspirin, Phenytoin, Propyl thiouracil, Sulfonamides = Metabolic: Galactosemia, Tyrosinemia, Type 4 Glycogenolysis

Answer 70

= AD PKD = Bilateral Hydronephrosis = Amyloidosis = Tuberous sclerosis = Von Hippel Lindau disease

Answer 71

Presentations: flank pain, hematuria, recurrent UTI, renal stones, obstructive uropathy, obstructive pressure symptoms = pkd1 on chromosome 16, pkd2 on chromosome 4, pkd3 = bilateral enlarged ballotable kidneys = uremic frost, AV fistula = liver cysts also present = Berry aneurysm of cerebral vessels, risk of SAH = Hypertension = Mitral valve prolapse due to collagen defects (25%) = USS, CT, MRI, genetics recommended after age 20 yrs

Answer 72

Autosomal dominant, defective tumor suppressor gene Presents with - renal angiomata tumors - brain and spinal cord haemangiomas - Renal cell carcinoma - Endolymphatic sac tumors - Phaeochromocytomas with resistant hypertension - Papillary cyst adenomas of epididymis - angiomata of Liver and kidneys - Cysts of pancreas, kidneys, Liver, Epididymis

Answer 73

- Diabetes mellitus - Hypertension - Autosomal Dominant Polycystic kidney disease - Glomerulonephritis - NSAID overuse CKD - Myeloma ——————————- - Systemic Sclerosis - Sequelae of Hemolytic Uremic syndrome - Sickle cell disease - Focal glomerulosclerosis - Oxalosis, Cystinosis, Fabry’s disease

Answer 74

Vitamin D is converted to its active form 1,25-dihydroxycholecalciferol (Calcitriol) in the kidney. In Renal failure, there is decreased excretion of phosphate which leads to hyperphosphatemia and low calcitriol leads to hypocalcemia (High phosphate & Low calcium). They cause secondary hyper-parathyroidism and renal osteodystrophy. Due to this arterial calcifications occur increasing cardiovascular morbidity and mortality. Tertiary hyper-parathyroidism due to prolonged stimulation makes it independent of low calcium status @ leading to autonomous continuous secretion of parathormone leading to Hypercalcemia. Treatment: Aim is to reduce bone resorption, arterial calcification & development of tertiary hyperparathyroidism. = Low phosphate diet = Vitamin D supplementation (alfa-calcidol) = Cincalcet, a calcimimetic which tricks parathyroid into thinking that extra-cellular calcium levels are higher and gland reduces hormone Secretion. = Sevelamer, Phosphate binder in diet (or Lanthanum carbonate)

Answer 75

1. Primary pulmonary TB - initial inhalation of Mycobacterium bacillus leading to draining lymph node infection called as Ghon focus - Miliary TB is dissemination of TB as primary infection - Tuberculous meningitis as primary infection directly 2. Post-Primary Pulmonary TB : reactivation when immunity low 3. Bone and Joint TB 4. Urinary TB 5. Genital TB 6. Cutaneous TB: primary papeles, sinus, Miliary, verrucous, Scrofuloderma, Lupus vulgaris 7. Tuberculous lymphadenitis 8. Tuberculous peritonitis with ascites Diagnosis: = sputum ZN staining = Lowenstein Jenson medium TB culture = Interferon gamma release assay = Gold quantiferon test Treatment: 2 weeks isolation-initially RIPE drugs for 2 months then Rifampin + INH for 4 months

Answer 76

Ankylosing spondylitis Tuberculosis pulmonary infection Ca bronchus Atypical pneumonia as with Klebsiella pneumoniae Pulmonary infarction Fungal pulmonary infection

Answer 77

QuantiFERON-TB Gold (QFT) is a simple blood test that aids in the detection of Mycobacterium tuberculosis, the bacteria which causes tuberculosis (TB). QFT is an interferon-gamma (IFN-γ) release assay, commonly known as an IGRA, and is a modern alternative to the tuberculin skin test (TST, PPD or Mantoux). Unlike the TST, QFT is a controlled laboratory test that requires only one patient visit and is unaffected by previous Bacille Calmette-Guerin (BCG) vaccination. QFT is highly specific and sensitive: a positive result is strongly predictive of true infection with M. tuberculosis. However, like the TST and other IGRAs, QFT cannot distinguish between active tuberculosis disease and latent tuberculosis infection, and is intended for use with risk assessment, radiography, and other medical and diagnostic evaluations. Like any diagnostic aid, QFT cannot replace clinical judgment. The US Centers for Disease Control (CDC) Guidelines recommend the use of IGRAs in all situations in which the TST was historically used, with IGRAs being the preferred test for persons who have been BCG vaccinated or are unlikely to return for TST reading (1).

Answer 78

QFT uses unique blood collection tubes that enable immediate exposure of viable blood lymphocytes to highly specific TB antigens (ESAT-6/CFP-10/TB-7.7(p4)) and test controls coated on the inner surface of the tubes. Antigen exposure produces a quantifiable immune response to aid in the diagnosis of TB infection. Nil tube Negative control to adjust for background IFN-γ TB Antigen tube To detect the CD4+ T cell responses to TB antigens Mitogen tube Low response may indicate inability to generate IFN-γ

Answer 79

Symptoms of SVC obstruction: = Headache, more on cough = breathlessness = swallowing difficulty = Dizziness or blackouts Signs of SVC obstruction: = facial plethora = distended neck veins = venous angiomata sublingual = dilated chest, arm veins = radiation therapy marks = suffused eyes Pemberton Maneuver: on raising both arms for a minute will make face red

Answer 80

Causes: CA bronchus Lymphoma Multimodal Goitre Aortic aneurysm Mediastinal fibrosis Rx by stent, radiation, Dexamethasone, surgery

Answer 81

= Tracheal deviation if Upper lobe, Rt = Reduced chest expansion same side = Cardiac apex shifted to same side = Dull percussion note & bronchial breath sounds below = Vocal resonance increased anteriorly with Upper Lobe and below scapula in Lower lobe collapse = Sputum production increased if chronic collapse as infection sets in.

Answer 82

Acute causes of Lung collapse: = Mucus plugging of bronchus = Inhaled foreign body blocks bronchus = Chest wall injury/Rib # casuing reduced local movement and collapse -------------------- Sub-Acute causes of Lung Collapse: = Proximal obstructing lesion, ex. tumor = Post Op, Prolonged Bed rest with atlectasis in a chronic Lung disease -------------------------------- Chronic Causes of Lung collapse: = Post Pneumonia focal fibrosis = Bronchiectasis = Chronic aspiration with excess secretions or recurrent infections = Diaphragmatic palsy = Pulmonary fibrosis = Trapped Lung due to pleural thickening

Answer 83

it is cor pulmonale secondary to extreme obesity = High BMI > 40 = Rapid shallow breathing, Ankle edema, Cyanosis = JVP elevated = Chronic Hypoxemia with Hypercapnia = Night Obstructive sleep apnea (10 second or more of no breathing), day time sleepy, = usually can only sleep sitting = PAH develops - Polycythemia -Cor pulmonale = Morning headaches = Impotence ----------------------------------- Waist:Hip > 0.85 in women, > 1.o in men is abnormal

Answer 84

OSA: - upper pharyngeal cavity collapses with negative intra-thoracic pressure during inspiration aided by thick neck, fat accumulation, big tonsils and micrognathia - all this leads to hypoventilation - hypoxia and some how apnea spells. ------------------------ Central sleep apnea: cessation of sleep drive from brain which lasts for 10-30 seconds, CO2 builds up, hypoxia kicks in breathing re starts. There are no diaphragmatic movements, no thoracoadbomninal movements during this apnea. Obese are prone but cause unknown.

Answer 85

= Obstructive sleep apnea-PAH-Cor pulmonale = Insulin resistance with DM type 2 = Hypertension ? due to Hyperinsulinemia+Na retention = LVH, more if Hypertensive with risk of SCD = Dyslipidemia, Low HDL, High LDL, high TG = Venous thrombosis-embolism = Lower limb early osteoarthritis due to weight load = Cancers: high risk of: women - endometrial/ Bile/ Postmenopausal Ca-breast Men - Colon, rectum, prostate = cholesterol GB stones = Intertrigo along skin folds = High mortality due to all of above

Answer 86

Hypothyroidism: - Hoarse voice, rough skin, Menorrhagia, Hard stools, thin hairs, Low mood, non pitting tissue swelling, slow pulse, slow relaxing DTR Cushing's disease: truncal obesity, moon face, purple striae, proximal muscle weakness, Polycystic ovaries: Hirsutism, Oligomenorrhea, Excess androgen from ovaries, USS suggestive Hypothalamic damage - Hyperphagia - Stroke, trauma, craniopharyngioma, inflammation, tumors

Answer 87

#Prader willi syndrome #Laurence-Moon-Bardet-Bidl syndrome #Alstrom syndrome #Cohen's syndrome- microcephaly, MR, ShortStature, Facial abnormalities, Obesity #Carpenter syndrome - sagital craniosynostosis, Acrocephaly, Turricephaly (tower shaped skull) Syndactyly@fingers (fused), Polysyndactyly@toes, undescended testicles, AutoRec(chr6) #Blount's disease - bowed legs, tibial torsion, obesity

Answer 88

= Excessive mucus with plugging, recurrent respiratory infections and bronchiectasis, EXCESS SPUTUM = Underdeveloped, thin, short, pale = Pancreatic insufficiency+ malnutrition = Recurrent pain abdomen, history of fecal impactions = Steatorrhea with foul smell stools, difficult to flush down = GBstones, Biliary cirrhosis, Portal HTN = iNFERTILITY ASPERMIA = iNTUSUSSEPTION = COR PULMONALE (LONG STANDING)

Answer 89

Vascular: PAH - primary or secondary, Eisenmenger syndrome --------------------- Restrictive: IPF, 2ry Pulmonary fibrosis as in sarcoidosis, drug induced, CTD, chronic allergic alveolitis ---------------------------- Obstructive: Emphysema with or without A1AT deficiency -------------------------- Suppurative: Cystic fibrosis, Bronchiectasis

Answer 90

perioperative complications = dehiscence --------------------------- infections: CMV, EBV, HSV, Bacterial, Aspergillosis, Candida, Pneumocystis ----------------------------- Rejection: Hyperacute- within Hours Acute- within days, dry cough/ dyspnea, malaise Bronchiolitis obliterans Drug side effects: Cushings syndrome due to steroids Azathioprine, cyclosporine,

Answer 91

autosomal recessive, 1 in 30,000 Male patients-invariably infertile because of immotile spermatozoa. The immotility is due to variety of ultrastructural defects in respiratory cilia and sperm tail. -------------------------------------- Dextrocardia Bronchiectasis/COPD/Pneumonia young, underweight, pale male Situs inversus infertility Sinusitis Dysplasia of frontal sinus Otitis media, Deafness Retinitis pigmentosa may be present

Answer 92

Medicines for lung problems include: 1. Antibiotics to prevent and treat chest infections. 2. Combination of 3 medicines (Kaftrio) to treat the root cause of cystic fibrosis in people age 12 and over 3. Medicines to make the mucus in the lungs thinner & easier to cough up – for example, dornase alfa, hypertonic saline and mannitol dry powder 4. Medicine to help reduce the levels of mucus in the body – for example, ivacaftor taken on its own (Kalydeco) or in combination with lumacaftor (Orkambi, but this is only available on compassionate grounds if people fulfil several criteria set by the manufacturer) 5. Bronchodilators to widen the airways and make breathing easier 6. Steroid medicine to treat small growths inside the nose (nasal polyps) 7. Routine vaccinations and have the flu jab each year once they're old enough.

Answer 93

Conditions linked to bronchiectasis: Acquired obstruction- foreign body, tumour, lymphadenopathy, COPD, mucoid impaction and connective tissue diseases. ------------------------------------- Congenital obstruction - bronchomalacia, tracheobronchomegaly, ectopic bronchus, pulmonary sequestration, pulmonary artery aneurysm and yellow nail syndrome. --------------------------------------- Immunodeficiency states with recurrent infections - IgG, IgA deficiencies, abnormalities of leukocyte function, conditions affecting primary antibody production. ------------------------------ Abnormal secretion clearance - immotile cilia syndrome, cystic fibrosis, Young's syndrome. ---------------------------- Miscellaneous disorders - alpha-1 antitrypsin deficiency, recurrent aspiration pneumonia, inhalation of poisonous dust and fumes and chronic rejection following organ transplantation.

Answer 94

CXR, CT scans Transmission EM of cilia Semen analysis Sachharin Test Nitric oxide inhalation/ Exhalation Pulmonary Function Testing Tracheal/ Nasal mucosal biopsy

Answer 95

**Inspiratory Stridor:** = Upper airway foreignbody = Retropharyngeal abscess = Acute Epiglottitis, Croup = Smoke inhalation with airway edema = Neoplastic, Tracheal stenosis ------------------------- **Expiratory Stridor**: lower thoracic = Foreign body = Intra-luminal neoplasm = Lower tracheal stenosis = Bronchial stenosis = Severe bronchial asthma

Answer 96

= Congenital web or tracheal stenosis = Acquired post tracheaostomy = Acquired Post intubation = Post airway/ thoracic trauma = Post infections as TB = Neoplasms related

Answer 97

Kyphosis is forward spine curve Scoliosis is lateral spine curve 1.Idiopathic - 65% 2. Congenital Kypho-Scoliosis: = Fused vertebre (klippel-feil) = Fused ribs = Hemi vertebrae 3. Paralytic Kypho-Scoliosis - secondary to supportive action of the trunk and spinal muscles, anterior poliomyelitis 4. Neuropathic Kypho-Scoliosis: as a complication of: = Neurofibromatosis = Spina bifida = Cerebral palsy = Syringomyelia = Friedrich's ataxia 5. Myopathic: DMD, BMD, 6. Metabolic: Marfan's syndrome, Cystine Storage diseases

Answer 98

Arthrogryposis, also called arthrogryposis multiplex congenita (AMC), involves a variety of non-progressive conditions that are characterized by multiple joint contractures (stiffness) and involves muscle weakness found throughout the body at birth. thought to be related to inadequate room in utero and low amniotic fluid. The patient may have an underlying neurological condition or connective tissue disorder.

Answer 99

Postural Degenerative spine, Osteoporosis Scheurmann's disease Congenital kyphosis Nutritional, Vit D deficiency Post TB of spine, Gibbus Post traumatic, vertebral fractures ---------------------------- Kypho-Scoliosis leads to restrictive lung disease with reduced FEV1 and FVC but ratio remains normal as both reduced. FVC of under 1 Lit will increase the risk of hypercapneic respiratory failure.

Answer 100

Cobb angle is a measurement of the degree of side-to-side spinal curvature, which is a deformity you may know as scoliosis. A Cobb angle describes the maximum distance from straight a scoliotic curve may be. Generally, it takes at least 10 degrees of deviation from straight before scoliosis is defined. It is an angle between a line drawn parallel to superior endplate of one vertebra above deformity AND a line drawn parallel to inferior endplate of the vertebra one level below the deformity.

Answer 101

= Rheumatoid Hands/nodules/deform = basal fine inspiratory crackles = Clubbing may be present ----------------------------- pleural disease: rub/ effusion with dullness, pleural fluid high in protein+LDH+RAfactor and Low in Sugar+C3+C4, wbc<5000 in fluid/ulit ----------------- Intra-Pulmonary nodules: small, may cavitate, resembles TB, tumor, may bleed. May rupture causing pneumo-thorax. Caplan syndrome is big cannon ball rheumatoid nodules + pneumoconiosis. ------------------------- Obliterative bronchilitis: dyspnea, Hyperinflation, expiratory wheeze. ---------------------- Gold or penicillamine treatment associated fibrosing alveolitis. ------------------- Pulmonary arteritis Apical fibro-bullous disease

Answer 102

= Bronchial carcinoma = Secondary malignancy = Pulmonary embolism + infarction = Pneumonia = Tuberculosis = Mesothelioma = Rheumatoid arthritis pleural disease = SLE associated = Lymphoma associated

Answer 103

- Dullness at base on auscultation - Stony dull percussion note as bases - Reduced tactile fremitus - Reduced vocal resonance - Bronchial breathing above dullness - Trachea usually central unless large

Answer 104

Cardiac failure Liver cirrhosis Nephrotic syndrome --------------------------------- Other causes of Pleural Effusion: = Meig's syndrome (ovarian fibroma) = Subphrenic abscess = Para pneumonic = Peritoneal dialysis = Hypothyroidism = Pancreatitis = Dressler syndrome = Asbestos exposure = Traumatic Hemothorax = Yellow Nail syndrome = Chylothorax

Answer 105

Pleural effusion/fluid/bLood = RAISED HEMIDIAPHRAGM - hepatomegaly or phrenic palsy = Basal Lung collapse = Collapse consolidation = Pleural thickening

Answer 106

Bronchiectasis is **irrversible dilatation of the bronchioles** with mucus plugging, recurrent infections & obstruction. = crackles over bronchiectactic area = polyphonic wheeze, changes to cough = Sputum pot by the bed = thin, malnourished, underweight = clubbing if long term -------------------------------------- **Other differentials**: = Fibrosing alveolitis = Post TB = Lung abscess = Sarcoidosis = Carcinoma lung

Answer 107

= Post Pertusis/ Measles/ TB = Cystic fibrosis = Yellow nail syndrome = Kartagener syndrome (AutoRece) = Bronchial obstruction (TB, tumor, FB) = Fibrosis = Hypo-gamma-globulinemia = Allergic BPA = Marfan's syndrome = Smoker COPD = Congenital causes: Sequestred lung segment, Bronchial atresia, KS

Answer 108

Chronic Bronchitis: A lung condition that develops over time in which the bronchi (large air passages that lead to the lungs) become inflamed and scarred. Cough with sputum on most days for at least 3 months for 2 yrs. ----------------------------------- Emphysema: a Histological/CT diagnosis: disorder affecting the alveoli (tiny air sacs) of the lungs. The transfer of oxygen and carbon dioxide in the lungs takes place in the walls of the alveoli. In emphysema, the alveoli become abnormally inflated, damaging their walls and making it harder to breathe. ---------------------- COPD - comprises both, chronic bronchitis and emphysema and labeled after PFT done. Patients with COPD have airflow obstruction that is caused either by destruction of the air sacs that exchange gas in the lungs (emphysema) and/or inflammation of the airways (chronic bronchitis).

Answer 109

= Hyperexpansion of lungs = Hyper-resonant percussion notes = Pursed Lips breathing = thin stature, rib indrawing = pink puffer or blue bloater = Suprasternal to cricoid distance <3cm = Long forced expiratory time >5 = prolonged expiration with wheeze = breathlessness at rest = Flapping hand tremors, check fundi = Liver and cardiac percussion lost = cor pulmonale features may be

Answer 110

A1 anti-trypsin deficiency Smoking Coal Dust **McLeod-Swyer-James syndrome**, unilateral emphysema after childhood bronchitis/ bronchiolitis episodes ---------------------------- work up: FEV1 < 80% PREDICTED, DIAGNOSTIC FEV1/FVC Ratio < 0.7 TLC, RV elevated, TLCO reduced CXR - > 7 posterior ribs elevated means hyper expanded Flattened diaphragn Irregular Lung vasculature Bullae in lung fields ABG to see hypercapnea HRCT Chest Alfa 1 anti-trypsin levels **6 minute walk test**, spo2 drops during it

Answer 111

A1 anti-trypsin deficiency Smoking Coal Dust **McLeod-Swyer-James syndrome**, unilateral emphysema after childhood bronchitis/ bronchiolitis episodes ---------------------------- **work up:** FEV1 < 80% PREDICTED, DIAGNOSTIC FEV1/FVC Ratio < 0.7 TLC, RV elevated, TLCO reduced CXR - > 7 posterior ribs elevated means hyper expanded Flattened diaphragn Irregular Lung vasculature Bullae in lung fields ABG to see hypercapnea HRCT Chest Alfa 1 anti-trypsin levels **6 minute walk test**, spo2 drops during it

Answer 112

Acute ILD: = Vasculitic, Goodpastures = Infection related, Immunosupression = Eosinophillic - Parasitic, ABPA, Drugs = ARDS

Answer 113

**Chronic ILD:** = Cryptogenic fibrosing alveolitis = Rheumatoid Lung = Systemic sclerosis = SLE = Polymyositis-Dermatomyositis = **Sjogren's syndrome** = Mixed CTD = Ankylosing spondylitis = Sarcoidosis = Extrinsic allergic alveolitis = Asbetosis = Silicosis = Radiation fibrosis = Chemical inhaled: **Beryllium, Mercury** = Poison ingested - **Paraquat** = ARDS - also called Acute interstitial pneumonia = Drugs causing ILD: **Bleomycin, Busulfan, Nitrofurantoin, Amiodarone**

Answer 114

Giant wave before carotid pulse is “a” wave and indicates PAH, Pulmonary stenosis. Giant wave after carotid pulse is “v” wave and indicates Tricuspid regurgitation or CCF.

Answer 115

**Diffuse Alopecia:** = male pattern baldness = Cytotoxic drugs = Hypothyroidism = Hyperthyroidism = Iron deficiency **Patchy alopecia:** = Alopecia areata = Ring worm infection = Discoid lupus erythematosus = Lichen planus

Answer 116

A rare, inherited disorder in which blisters form on the skin and the moist inner lining of some organs and body cavities. The disease appears at birth or during the first few years of life, and lasts a lifetime. Prognosis is variable, but tends to be serious. Life expectancy is 50 years, and the disease brings with it complications related to infections, nutrition and neoplastic complications. Supportive care — There is no specific therapy for most forms of epidermolysis bullosa (EB). Treatment is largely supportive and includes wound care, control of infection, nutritional support, and prevention and treatment of complications.

Answer 117

Dermatitis herpetiformis (DH) is a chronic autoimmune blistering skin condition,[3] characterised by intensely itchy blisters filled with a watery fluid.[4] DH is a cutaneous manifestation of coeliac disease,[5] although the exact causal mechanism is not known. DH is neither related to nor caused by herpes virus; the name means that it is a skin inflammation having an appearance (Latin: -formis) similar to herpes. The age of onset is usually about 15 to 40, but DH also may affect children and the elderly. Men are slightly more affected than women.[6] Estimates of DH prevalence vary from 1 in 400 to 1 in 10,000. It is most common in patients of northern European and northern Indian ancestry, and is associated with the human leukocyte antigen (HLA) haplotype HLA-DQ2 or HLA-DQ8 along with coeliac disease and gluten sensitivity.[7][8][9][10][11] Dermatitis herpetiformis was first described by Louis Adolphus Duhring in 1884.[12] A connection between DH and coeliac disease was recognized in 1967. TREATMENT - Gluten free diet +Dapsone

Answer 118

Pemphigus: is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. The name is derived from the Greek root pemphix, meaning "pustule". In pemphigus, autoantibodies form against desmoglein, which forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes detached, a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a large area of the skin.

Answer 119

1. Pemphigus vulgaris: most common form, occurs when antibodies attack desmoglein3. Sores often originate in the mouth, making eating difficult and uncomfortable. Although PV may occur at any age, it is most common among people between 40 and 60. Rarely, it is associated with myasthenia gravis. Nail disease may be the only finding and has prognostic value in management. 2. Pemphigus foliaceus (PF) is the least severe of the three varieties. Desmoglein 1 is targeted by the autoantibodies, is enriched in the upper skin layers. PF is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. Mouth sores do not occur. It is not as painful as PV, and is often misdiagnosed as dermatitis or eczema. PF is endemic in Brazil [9] and Tunisia. 3. Intraepidermal neutrophilic IgA dermatosis is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis. 4. Paraneoplastic pemphigus (PNP): LEAST COMMON, most severe, with usually lymphoma or Castleman's disease. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the esophagus. In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). Complete removal of and/or cure of the tumor may improve the skin disease, but lung damage is generally irreversible. 5. Endemic pemphigus foliaceus includes fogo selvagem, the new variant of endemic pemphigus foliaceus in El Bagre, Colombia, and the Tunisian endemic pemphigus in North Africa.

Answer 120

Hailey-Hailey disease, also called familial benign pemphigus, is an inherited skin disease, not an autoimmune disease, so it is not considered part of the pemphigus group of diseases. characterized by outbreaks of rashes and blisters on the skin. Affected areas of skin undergo repeated blistering and inflammation, and may be painful to the touch. Areas where the skin folds, as well as the armpits, groin, neck, buttocks and under the breasts are most commonly affected. In addition to blistering, other symptoms which accompany HHD include acantholysis, erythema and hyperkeratosis. The cause of the disease is a haploinsufficiency of the enzyme ATP2C1;[6] the ATP2C1 gene is located on chromosome 3, which encodes the protein hSPCA1. A mutation on one copy of the gene causes only half of this necessary protein to be made and the cells of the skin do not adhere together properly due to malformation of intercellular desmosomes, causing acantholysis, blisters and rashes. There is no known cure.

Answer 121

Mikulicz syndrome is a chronic condition characterized by the abnormal enlargement of glands in the head and neck, including those near the ears (parotids) and those around the eyes (lacrimal) and mouth (salivary). The tonsils and other glands in the soft tissue of the face and neck may also be involved. According to the criteria of Schaffer and Jacobsen, 1 Mikulicz's disease is of unknown etiology and follows a benign course, whereas Mikulicz's syndrome is associated with some other disorders such as leukemia, lymphosarcoma, tuberculosis, and sarcoidosis. Benign lymphoepithelial lesion or Mikulicz' disease is a type of benign enlargement of the parotid and/or lacrimal glands. This pathologic state is sometimes, but not always, associated with Sjögren's syndrome.

Answer 122

Separately felt in: = Reactive hyperplasia = infectious mononucleosis = Lymphoma Matted feel in: = Neoplastic = Tuberculous Soft, rubbery, Fleshy = Hodgkins Hard, fixed to skin = neoplastic If found without Goiter = also examine axillae, Groin and spleen.

Answer 123

Hypothyroidism: = Immobile, uninterested, slow wits = Slow speech, Slow movements = Slow pulse, Bradycardiac < 60 = Slow relaxing DTR, specially ankle = constipation, Hard stools = frequent bloating of abdomen = overweight = Hair loss, thinning = Menorrhagia = soft tissue swelling@ ankles Hyperthyroidism: = fine tremors hand -paper on dorsum = anxious, fidgety = sweaty palms = Rapid pulse, may be AFib = Exophthalmous: sclera visible above lower eye lid (normally not) = Lid retraction - sclera visible above cornea (normally not) = Test for Lid Lag = thin over active stature, good appetite Goitre may be present No both states Euthyroid will have goiter but no other signs of over or hypo thyroidism

Answer 124

1. ask to take a sip of water - hold it in mouth - swallow while u see at neck 2. palpate center isthmus with index+middle fingers and move laterally to lobes 3. Ask to swallow while palpating lobes 4. feel of Lymph nodes in neck 5. Listen over thyroid lobes both sides for bruit - sign of overactivity - occlude IJV while listening to it and rotate head to other side as well. 6. Percuss over sternum for extension of thyroid gland, should be dull note 7. Assess thyroid status 8. Feel the pulse for Afib 9. Comment on stature - thin or obese 10. check slow relaxing ankle reflex by asking to stand with knee on chair and foot handing at edge - tap it with hammer

Answer 125

are skin lesions commonly found on the palms and soles but which may spread to the scrotum, scalp and trunk. The lesions may resemble psoriasis. Keratoderma blennorrhagicum is commonly seen as an additional feature of reactive arthritis in almost 15% of male patients. The appearance is usually of a vesico-pustular waxy lesion with a yellow brown colour. These lesions may join to form larger crusty plaques with desquamating edges. Can be due to Reiter's syndrome, (rash mimics syphilitic infection), sometimes referred to as “seronegative spondyloarthropathy” or “reactive arthritis”, consists of predominately three symptoms: arthritis, conjunctivitis, and urinary tract infections, or urethritis. More recently, doctors have recognized a fourth major feature, ulcerations of the skin and mouth.

Answer 126

Differential for this is is vast: = Malaria = Dengue fever = Traveller's diarrhea: Shigella, Campylobacter, amoebic, Typhoid = Enquine encephalitis, Meningitis = Lyme disease, Rocky mountain spotted fever = Viral Hepatitis = Avian influenza, COVID-19, = HIV = Viral haemorrhagic fevers

Answer 127

= impaired consciousness of seizures = Hypoglycemia = Parasite counts more than 2% = Haemoglobin at or below 8 Gms% = Spontaneous bleeding or DIC = Haemoglobinuria with normal G6PD = Renal impairment, PH < 7.30 = Pulmonary edema or ARDS = Shock status with BP < 90/60

Answer 128

complication of malaria - massive intra-vascular hemolysis leads to anemia, hemoglobinuria where urine turn coca cola like on standing. a feature of severe falciparum malaria.

Answer 129

Suspect DVT: = Malignancy: wt loss, anemia, family history, = IBD: bleeding diarrhea, oral ulcers, pain abdomen, abd surgeries = Thrombophilia: H/o recurrent abortions = Long travels (air, car) = OC pill-HRT-Tamoxifen use, = Smoking or IVDU = Recent surgery, POP cast = about swollen Leg itself: trauma/redness/rash/injuries/bites/temperature/

Answer 130

= Leg Deep vein thrombosis = Ruptured Baker's cyst, popliteal = Stasis edema due to long immobility = Cellulitis of the Leg = Injury with compartment syndrome = Muscle injury, Haematoma Note - if DVT US scan is negative but clinical probability is high (Well's score > 2) = repeat scan after 1 week and continue LMWH till then.

Answer 131

Score 1 point for each: = Active malignancy = Paralysis or recent POP casting = Recently bedridden > 3 days = Major surgery in past 12 weeks = Localized tenderness along veins = Entire Leg swollen = Calf swelling > 3 cm (10 cm below tibial tuberocity) = Pitting edema confined to same leg = Collateral superficial non varicose veins = Previous documented DVT Score minus 2 for each of: = Alternative diagnosis likely 2 Points or more = DVT likely

Answer 132

= Hand wasting of lumbricals (assist in metacarpophalangeal joint flexion) and interossei (4 palmar, 4 dorsal, adduction and abduction of fingers) = Foot wasting of Extensor digitorum brevis which extends 4 toes and only proximal phalanx of great toe = Pes cavus, Claw Hands (indicates long standing neuropathy) = fasciculations (MND) = Foot ulcerations (neuropathic) = Etiological clues: finger prick marks, insulin marks, vasculitic purpura, Bruises of CLD, ALD, Leuconychia (poor nutrition), AVFistula/HD cath. = Other neurological examination..

Answer 133

Causes of nerve thickening: (the mnemonic HAGALO) 1. Hypertrophic neuropathy - a rare autosomal dominant condition. 2. Acromegaly. 3. Guillain Barre - relapsing & remitting 4. Amyloid. 5. Leprosy. 6. Others - e.g. Neurofibromatosis, Diabetes, Sarcoidosis, Charcot-Marie-Tooth disease.

Answer 134

1. Metabolic: Pyridoxine, Niacin, Thiamine, B12 deficiency 2. Para-neoplastic: Small cell Lung Ca (Anti Hu AB+), MULTIPLE MYELOMA 3. Infection: - HIV, Lyme, Diphtheria, HSV, HTLV, CMV, 4. Hereditary: HSMN (CMT) 5. Systemic: Uremia, Myxedema, Thyrotoxicosis, Diabetes mellitus 6. Inflammatory: CIDP, AIDP, Sarcoidosis, SLE, Vasculitis (ANCA) 7. Drugs- Platinum/Vinca alkaloids, Metronidazole, Phenytoin, OP Poison, Chloramphenicol, INH, Alcohol, Heavy Metals, Nitrofurantoin, Amiodarone

Answer 135

= Onset, duration, persistent or not = Past similar episodes? = is it one eye or both? = Is it painful or not? (optic neuritis) = Is it all directions or specific? = Does it disappear on covering one eye = Visual acuity - blurring, scotoma, total =Any headache? Autoimmune/ inflammatory/ vascular causes = Any other neurological symptoms- dysarthria/ focal weakness/ Sensory/ Autonomic/ cognitive symtoms?

Answer 136

EXPLAIN (mnemonic) E = Exogenous drugs, eg insulin, oral hypoglycaemics, alcohol, eg a binge with no food; aspirin poisoning; ACE-i; B-blockers; pentamidine; quinine sulfate; aminoglutethamide; insulin-like growth factor. P = Pituitary insufficiency. L = Liver failure A = Addison’s disease I = Islet cell tumours (insulinoma) and immune hypoglycaemia (eg anti-insulin receptor antibodies in Hodgkin’s disease). N = Non-pancreatic neoplasms, eg fibrosarcomas and haemangiopericytomas.

Answer 137

= Cataracts = Refractive error = Corneal distortion or scarring = Vitreous abnormalities = Functional diplopia

Answer 138

NMJ = Myasthenia gravis, fatiguable EOM cause = Orbital inflammation/ fracture or tumor causing restriction of EOM muscle movements Horizontal Dplopia: Abducens palsy due to SOL, MS, Stroke, DM, Aneurysm etc Horizontal and/or vertical: 3

Answer 139

NMJ = Myasthenia gravis, fatiguable EOM cause = Orbital inflammation/ fracture or tumor causing restriction of EOM muscle movements Horizontal Dplopia: Abducens palsy due to SOL, MS, Stroke, DM, Aneurysm etc Horizontal and/or vertical: 3rd nerve palsy: DM, Stroke, MS,trauma 4th nerve palsy: Stroke, DM, Aneurysm, trauma, SDH Graves ophthalmopathy

Answer 140

3 or more of following: = onset at 50 yrs of age or older = New type of headache = Clinically abnormal Temporal artery = ESR > 50 = Arterial Biopsy: granulomatous inflammation with giant nucleated cells, skip lesions possible - do biopsy within 3 days and take 2cm sample. Also Ask about Jaw claudication and Proximal myopathy symptoms. Steroid Treatment in GCA: Prednisolone: Start 60-80 mg OD if visual symptoms Start 40 mg OD if no visual, for 1-4 wks Taper to 30 mg week 5-6 Taper to 20 mg week 7-8 then slowly over 2 years. + PPI + Bone protection calcium/VitD Inform DVLA if vision involved

Answer 141

Ursodeoxycholic acid or ursodiol is a naturally occurring bile acid that is used dissolve cholesterol gall stones and to treat cholestatic forms of liver diseases including primary biliary cirrhosis. UDCA can be obtained from cholic acid (CA), which is the most abundant and least expensive bile acid available. It is synthesized in the liver in some species, and was first identified in bile of bears of genus Ursus, from which its name derived. Cholic (koe' lik) acid is a naturally occurring, primary bile acid that represents a major component of the total bile acid pool in humans. Cholic acid is synthesized from cholesterol in the liver and is conjugated to either glycine (glycocholic acid) or taurine (taurocholic acid) before secretion in the bile.

Answer 142

Thiamine deficiency can result in clinical syndromes other than WE. These syndromes fall under the rubric of beriberi. There are two major types of beriberi: wet beriberi which affects the cardiovascular system and dry beriberi which affects the nervous system. Wet beriberi is characterized by high-output, predominantly right-sided heart failure, orthopnea, and pulmonary or peripheral edema.6 Dry beriberi is a painful, distal sensorimotor axonal polyneuropathy that affects the lower limbs more than the upper.47 Patients may experience limb weakness (ie foot drop), decreased proprioception, and reduced or absent peripheral reflexes.50 Gastrointestinal beriberi is a lesser known form which manifests as abdominal pain, nausea, vomiting, and lactic acidosis.7 Infants between two and twelve months of age can develop beriberi if either breastmilk or baby formula is thiamine-deficient.6 Infantile beriberi is characterized by failure to thrive, cardiomyopathy, dyspnea, restlessness, nystagmus, and aphonia. An example of infantile beriberi took place in Israel in 2003. An imported soy-based infant formula was deficient in thiamine, resulting in infantile beriberi in a cluster of newborns. Protracted vomiting of any cause increases the risk of WE. Pyloric stenosis, gastritis, Crohn’s disease, bowel obstruction, pancreatitis, migraine attacks with vomiting, hyperemesis gravidarum, and gastrointestinal or orofacial cancers comprise many of the abdominal conditions that predispose to WE.12 Gastrointestinal surgery is another major risk factor. WE usually develops two to eight months postoperatively in patients with >7 kg of weight loss per month.

Answer 143

Korsakoff Syndrome Early in the disease course, patients with WE may develop memory disturbance, indifference, and disorientation. If left untreated, this impairment can become profound and progress into an amnestic disorder referred to as KS. Rarely, KS occurs in isolation without the preceding acute features of WE. Lesions of the dorsomedial thalami have been linked to memory loss. An animal study using young chicks found that alcohol neurotoxicity may be a contributing factor to memory loss. KS is associated with both anterograde and retrograde amnesia. The former is a defect in acquiring new memories and may be as severe as not recalling events occurring within the past thirty minutes. The latter affects the ability to recall events of the recent past, although long-term memory is retained. Working memory rather than reference memory is preferentially impaired. Patients may be rendered capable of only performing the most habitual tasks. Memory loss leads to confabulation, which becomes less prominent over time. The invented memories fill gaps left by memory loss, and patients believe in the authenticity of the memories. It is thought that these effects on memory are due to irreversible damage to the diencephalic-hippocampal circuits.

Answer 144

Thiamine is mainly derived from foods such as brown rice, cereals, and pork.47 Rice is a predominant part of the diet in about two-thirds of the world’s population. A prime example of unbalanced nutrition is a diet consisting primarily of polished white rice. Because of removal of the husk through milling, polished white rice lacks thiamine.13 The incidence of beriberi in Asian countries has decreased since the initiation of thiamine fortification in their food. Thiamine is absorbed in the small intestine, via an active, carrier-mediated, and rate-limited mechanism, with highest absorption capacity in the duodenum.30,57 In healthy subjects, the maximal amount of thiamine absorption from a single oral administration is approximately 4.5 mg.56 After uptake by the brush border, thiamine is exported out of the enterocyte via the basolateral membrane.55 Most thiamine is protein-bound within the plasma for transport to the tissues. Humans are incapable of de novo thiamine synthesis and, therefore, require dietary supplementation. The human body can store 30–50 mg of thiamine,7 mainly in the skeletal and cardiac muscle, liver, kidneys, and brain.40 The dietary thiamine requirement is 0.5 mg for every 1000 kcal consumed or about 1.4 mg per day in a person with a typical diet.6 Thiamine deficiency leads to brain lesions within two to three weeks, as body stores of thiamine are only sufficient for 18 days.

Answer 145

Blackouts, Epilepsy: - Inform DVLA and stop driving - Insurance lost if driving before DVLA rules what to do - Groups 1 license: No driving 12 months from first seizure if no recurrence - Group 2, HGV license: No driving for 5 yrs - for 10 yrs without medications/ seizures. Post MI: 1 wk - if PCI done successfully 1 month - if no PCI done 6 month for - group 2 licence

Answer 146

1 unit of Alcohol is: the amount of alcohol a person can clear from body in 1 Hour. = 10 ml or 8 Gram of pure alcohol = 25 ml of spirits (single shot) = Half Pint of 4% Beer (284ml) = 125 ml of 12% wines is 1..5 units = Calories in alcohol: 7 Kcal/Gram 175 ml of 12% wine = 133 Kcal 50 ml of 40% Gin = 95 Kcal Pint of 5% Beer = 239 Kcal 1 Pint is = 568.261 ml 1 Pint is = 19.215 Ounce 1 US Gallon - 3.78 Liter 1 US Tea Spoon = 4.92 ml

Answer 147

Lager >5.2% Cider > 5.2% Alcopop > 5% Bitter > 5 % Wine, White > 13% Wine, Red, Rose >12.5% PIMMS >25% Cognanc > 40% Double Whisky > 40% Gin and Tonic > 40% Sambuca Shot > 40% Cosmopolitan Cocktail > 40%

Answer 148

A= Abortions, inform CMO B= Birth to register C= Court or police request D= Drug addictions P= Public interest, Partner Harm O= Organ Transplant I= IVF Treatment N= Notifiable diseases T= Terrorist act or Related suspect Refusing to STOP driving - tell first, try to convince then meet with consultant then inform in writing then inform DVLA finally Refusing to inform GP - do not inform his GP - STI clinic can release if pt consented - encourage the patient to tell GP himself -

Answer 149

2W-2S-CVA - Written - Witnessed - Specific wish - Signed - Continuously valid till changed/cancel - Voluntary (without any corecion) - Aged > 18 yrs when made

Answer 150

1. Explain procedure itself = Before hot o prepare: fasting = During, How-Long, WhoDoIt = After-Recovery-Where-How long stay 2. Explain benefits of this procedure 3. Explain Risks - common, uncommon = Bleeding = Pain = Injury = Infection 4. Explain about the consent, Legal issue

Answer 151

= It is a congenital disorder of muscle weakness and myotonia, induced by cold and aggravated with continued activity. The patients develop prolonged myotonia or weakness in a localized group of muscles, which is not associated with the change in serum potassium level. = It mainly affects eyelids, neck, and upper limb muscles. Patients characteristically present in their childhood complaining of inability to open their eyes following rapid, forceful successive closures. Weakness and myotonia last for minutes to hours. Even after the immediate rewarding of the muscles, cold-induced weakness usually persists for several hours. = The disease is non-progressive, does not cause muscle wasting or hypertrophy. It is caused by mutations in the sodium channel gene SCN4A that codes for the alpha-subunit of the skeletal muscle sodium channels, i.e., voltage sensor domain. = Patients usually live a normal life and do not affect longevity. The serum potassium level is moderately elevated. = EMG taken during cooling of a muscle shows profuse myotonic discharges and reduced CAMP amplitudes. The mainstay of therapy is an avoidance of cold exposure and physical overactivity.

Answer 152

Diuretics use Type IV renal tubular acidosis Hyperaldosteronism Hyperglucocorticoidism Gitelman syndrome Bartter syndrome Liddle syndrome

Answer 153

The distal myopathies are caused by deficiency or lack of specific proteins that play an essential role in the proper function and health of muscle cells. Laing distal myopathy is caused by mutations in the beta cardiac myosin (MYH7) gene located on the long arm (q) of chromosome 14 (14q12). Welander distal myopathy Tibial muscular dystrophy Markesberry Griggs distal myopathy Laing distal myopathy Nonaka distal myopathy Miyashi distal myopathy Myofibrillar distal myopathy

Answer 154

Hereditary, Autosomal dominant form and recessive form both types Axial muscular weakness proximal weakness(Gower sign) Cardiomyopathy, conduction blocks EMG - myopathic NCV - normal Rise in CK, 4-25 times Calf hypertrophy seen Tight heel cords Cataracts early Rigid spine may be noted

Answer 155

Lower limb girdle weakness unlike MG Malignancy association - Small cell Ca Anti-Presynaptic CaChannel antibodies EMG shows 2nd wind phenomenon on repetitive stimulation (improves) Poor DTRS becomes brisk after some exercise

Answer 156

mechanism: Defective oxidation of branched chain FA(phytanic acid) which rises in serum and urine Onset - Late childhood Progression - slow progressive sensory motor hypertrophic neuropathy (demyelinating, remyelinating and hyertrophy) thickened nerves = Anosmia = Cerebellar ataxia = Sensory neural deafness = Cataracts early = Retinitis pigmentosa association = Night blindness occurs, Tunnel vision = Skin icthyosis = Short 4th finger both hands = Syndactyly (fused fingers) Rx = Diet free of phytanic acid Plasma exchange, Dialysis, Phytanic acid is found in-Dairy, Beef, Lamb, Fish etc

Answer 157

Arginin-Vasopressin Test: Antidiuretic hormone (ADH), also called arginine vasopressin (AVP), is a hormone that helps regulate water balance in the body by controlling the amount of water the kidneys reabsorb while they are filtering wastes out of the blood. This test measures the amount of ADH in the blood.

Answer 158

non Neurogenic causes: = Cardiac failure, DCM = Dehydration, reduced volume = Adrenal insufficiency, reduced tone = Hypoaldosteronism = Hypokalemia = Venous Pooling: Alcohol, Post Meal, During exercise, Heat, Sepsis = Drugs: Nitrates, Diuretics, Anti BP, Alfa blockers (Omnic), Beta blockers, TCA, sedatives, Phenothiazines

Answer 159

Kearns Sayre syndrome: - mutation in mitochondrial DNA - onset less than 20 yrs of age - progressive external ophthalmoplegia - Retinitis pigmentosa, tunnel vision, Night blindness - Diabetes mellitus some times - Complete Heart Block - CSF proteins > 1 Grams - Cerebellar ataxia - Delayed puberty, short stature, - Infertility Other MM's: = Chronic Progressive Ext. ophthalmoplegia CPEO = MERRF (Myoclonic epilepsy, ragged red fibers) = MELAS (mitochondrial encephalopathy, Lactic acidosis, Stroke like episodes) = ARCO (Autosomal Recessive Cardiomyopathy + Ophthalmoplegia) = ARCO

Answer 160

1. Duchenne muscular Dystrophy: XR, dystrophin gene, starts < 5 yrs of age, Proximal myopathy, Cardiomyopathy, mental retardation (High CK) 2. Becker MD: XR, dystrophin, appears > 15 yrs, Girdle weakness, Cardiomyopathy, (High CK) 3. Limb Girdle MD: AD/ AR, early childhood, shoulder+Hip myopathy, Cardiomyopathy, 4. Emery-Dreifuss: XR/ AD, Emenin or Lamin gene, childhood to adult onset, Elbow contractures, Humeral weakness, peroneal weakness, cardiomyopathy 5. Congenital: AR, at birth or early, Hypotonia 6. Myoclonic dystrophy, Type 1&2, CTG repeat, Myotonia, AD, anticipation, cataracts, Arrhythmias, foot drop 7. Fascio-Scapulo-Humeral MD: AD 8. Oculo-pharyngeal dystrophy

Answer 161

combines Estrogen & progestogen To be taken - 21 days then off 7 days Side effects: mood swings, nausea, breast tenderness and headaches –usually settle down in a few months. There is no evidence that the pill will make you gain weight. very low risk blood clots & cervical cancer. = The pill is not suitable if over 35 & smoke, or if you have certain medical conditions. = does not protect against STI, so use a condom as well. = There may be a link between the pill and depression. = The pill prevents the ovaries from releasing an egg each month (ovulation) and thickens the mucus in endometrium. Monophasic 21-day pills = most common type. Microgynon, Marvelon and Yasmin Phasic 21-day pills = contain 2 or 3 sections of different coloured pills in a pack. Each section contains a different amount of hormones. 21/7 off pattern. Phasic pills need to be taken in the right order. Logynon is an example. Every day (ED) pills = There are 21 active pills and 7 inactive (dummy) pills in a pack. The two types of pill look different. Every day pills in the right order. Microgynon ED is an example

Answer 162

Starting on the 1st day of your period If you start the combined pill on the 1st day of your period (day 1 of your menstrual cycle) you will be protected from pregnancy straight away. You will not need additional contraception. Starting on the 5th day of your cycle or before If you start the pill on the 5th day of your period or before, you will still be protected from pregnancy straight away. tarting after the 5th day of your cycle You will not be protected from pregnancy straight away and will need additional contraception until you have taken the pill for 7 days. f you vomit within 3 hours of taking the combined pill, it may not have been fully absorbed into your bloodstream. Take another pill straight away and the next pill at your usual time. If you continue to be sick, keep using another form of contraception until you've taken the pill again for 7 days without vomiting. Very severe diarrhoea (6 to 8 watery poos in 24 hours) may also mean that the pill does not work properly. Keep taking your pill as normal, but use additional contraception, such as condoms, while you have diarrhoea and for 2 days after recovering.

Answer 163

The pill may not be right for you if you: = are pregnant = smoke and are 35 or older = stopped smoking less than a year ago and are > 35 = are very overweight = take certain medicines Past H/o: = blood clots in a vein, for example in your leg or lungs = stroke or any other disease that narrows the arteries = close family having a blood clot under the age of 45 = a heart disease, including high blood pressure = severe migraines, especially with aura = breast cancer = disease of the gallbladder or liver = Diabetes with complications or > for the past 20 years

Answer 164

Antibiotics: = Rifampicin and rifabutin can reduce the effectiveness of the combined pill. Other antibiotics do not have this effect. Epilepsy and HIV medicines, and St John's wort: = Enzyme inducers speed up the breakdown of hormones by your liver, reducing the effectiveness of the pill. Examples of enzyme inducers are: = Carbamazepine, = Oxcarbazepine, phenytoin, phenobarbital, = Primidone and Topiramate St John's wort (a herbal remedy) HAART

Answer 165

= Bronchial Carcinoma = Lung cancer- Small cell, Adeno, Squamose = TB infection, Pneumonia, Fungal = Vasculitis - Wegner's, Goodpasture syndrome = Pulmonary embolism, LVF = Sarcoidosis = AV malformations

Answer 166

= pleural effusion = Pleural nodules = Interstitial Lung disease = Pneumonitis = Pulmonary bleeding = Caplan syndrome = Bronchiectasis = Bronchiolitis obliterans = Pulmonary Hypertension = Pulmonary vasculitis = Drug induced ILD as with Gold and Methotrxate

Answer 167

Rheumatological diseases Connective tissue disorders Drugs - Gold, Methotrexate, etc Extrinsic Allergic alveolitis Granulomatous diseases: TB, Sarcoid Industrial dust disease - asbestosis, Cola pneumoconio Post radiation therapy Cryptogenic fibrosing alveolitis

Answer 168

Gold injections Methotrexate Amiodarone Belomycin Nitrofurantoin Sulfasalazine

Answer 169

DM - fingerprick marks, Lipodystrophy at insulin site, retinopathy (poor vision, fundus angiogenesis), foot ulcers, amputations HTN - Blood pressure, Medication list Vasculitis: Skin lesions, Purpuric rach, Rheumatological findings Polycystic Kidneys: Ballotable kidneys, transplant kidney, AV fistula,, Nephrectomy scars, Hearing aid with Alport's syndrome Tuberous sclerosis: Adenoma sebaceum, Sub-ungual fibroba, shagreen patches, Ash Leaf macules, Epileptic Membrano-proliferative Glomerulonephritis: Lipodystrophy of face/ trunk/ arms

Answer 170

common: nausea, vomiting, fatigue, anorexia, weight loss, muscle cramps, = Altered mental status = Uremic frost: skin hue = Pruritus with excoriations = Pericarditis and pericardial rub = Asterixis, (metabolic encephalopathy) Blood urea levels can be lowered by consuming less protein and avoiding products that contain creatinine. Carrots and potatoes, for example, help to alkalize urine and lessen the effects of high blood urea levels. Cinnamon, lemon, red bell pepper, turmeric, and other healthy foods can also be used to lower the BUN. = Petechiae, bruise due to low platelets = Tachypnea due to Acidosis,

Answer 171

Phenothiazines Penicillins, Augmentin Rifampicin Macrolides as Klacid Sulfonamides Cabamazepine Synthetic androgens: Menabol Diclofenac

Answer 172

conditions requiring multiple transfusions such as: = Thalassemia major = Sickle cell disease = Refractory anemias Porphyria cutanea tarda Chronic lymphocytis leukemia HCV Iron transfusions african hemosiderosis Acquired Hemolytic anemia

Answer 173

Can be functional with Sicklers or after removal of spleen Sickle cell disease with chronic spleen damage Vasculitic syndromes Splenic artery thrombosis Infiltratinve: Sarcoidosis, Amyloidosis Coeliac disease Auto-immune diseases

Answer 174

Stage 01: creatinine increases by 1.5 to 2 times of baseline or by 0.3 mg/dl Urine output drop less than 0.5 ml/kg/Hr for at least last 6 Hrs. Stage 02: Increased creatinine by 2-3 times above baseline. Urine < 0.5 ml/kg/Hr for last 24 hrs. Stage 03: Increased creatinine > 3 times above baseline or > 4 mg/dl with an acute rise of 0.5 mg/dl at least from baseline. Urine Output less than 0.3 ml/Kg/Hr for last 24 Hrs

Answer 175

Propofol therapy Amitryptiline Indomethacine Promethazine

Answer 176

Neurologica Dysphagia: = Bulbar Palsy = Lateral medullary/PICA syndrome = Myasthenia syndrome = Syringomyelia = Oculo-pharyngeal dystrophy Malignancy: = Ca esophagus = Pharyngeal carcinoma = Gastric carcinoma = Extrinsic pressure by thyroid/ thymus = Lung carcinoma = Lymph node pressing on it Other Dysphagia: = Benign strictures = Pharyngeal Pouch = Achalasia = Systemic Sclerosis/ CREST = Esophagitis = Iron deficiency (Plummer Vinson Esophageal Web)

Answer 177

cause of 1% colorectal cancer (other causes are: Sporadic 95%, HNPCC) Autosomal dominant 100's of polyp[s by age 30-40 yrs APC gene mutation, loss of function of Tumor suppressor gene Located on chromosome 5 Needs WBC DNA analysis Rx - Total colectomy with Ileoanal pouch Risk of duodenal tumors Variations: Gardner syndrome, Turcot's syndrome.

Answer 178

a variation of familial adenomatous polyposis Autosomal Dominant Polyps in colon and extra-intestinal features as: = Desmoid tumors = Sebaceous cysts = Lipomas = Osteoma's = Supermammary teeth Risk of colon cancers Total colectomy required

Answer 179

a variation of familial adenomatous polyposis with young age Ca colon risk Needs early total colectomy Autosomal dominant other features: = Medulloblastoma may occur

Answer 180

Hereditary Non-Polyposis ColoRectal cancer (HNPCC), nonpolyps Autosomal dominant High penetration, 1-3% Ca colon chance Highly aggressive cancer Other associated tumors possible: = Ovarian tumor = Stomach cancers = Liver - GB tumors = Urogenital cancers = Small colon cancers Amsterdam Criteria for Diagnosis: > 3 relatives with Ca colorectum, 1 first degree At least 2 generations of ca colon 1 or more cancers before age of 50 Genes: MSH2 (60%), MSH 1 (30%)

Answer 181

Autosomal dominant its a anti-neutrophil elastase produced normally by hepatocytes Levels > 11 mmols (protective) < 9 deficient Phenotypes: M (medium) S (slow) Z (very slow), Defect alters the molecules which is not released from liver cells Effects: 1. Lung tissue destruction by neutrophil elastase as it is not neutralized, COPD 2. Chronic lung disease occurs as cirrhosis smoking accelerated disease by 10 yrs Rx: Stop Smoking, Manage COPD/ CLD Avoid toxic drugs for Liver AT replacement Lung/Liver transplant

Answer 182

Chronic Liver disease IBD, UC, Crohns disease Coeliac disease + malnutrition GI Lymphoma Tropical sprue Whipple disease

Answer 183

used to assess severity of alcoholic hepatitis MDF = 4.6 x ( Patient PT - Control PT) + Total Bilirubin/ 17 if > 32 = 50% mortality at 30 days PT = prothrombin time

Answer 184

Bloody stools > 6 in 24 Hrs and Hb < 10.5 gm% ESR > 30 mm at 1 Hr Pulse > 90/min Temperature > 37.5 c

Answer 185

Unrelated: = Thrombophillia - Budd chairi syn. = Fulminant Hepatitis E Related: = Obstetric cholestasis: in 1/2 trimester with pruritus, USG Liver normal, start Urso-deoxy-cholic acid = Acute Fatty Liver of Pregnancy: Life threatening in 3rd trimester, High Liver enzymes, Rx delivery + supportive = HELLP syndrome: Hemolysis + Elevated Liver enzymes + Low Platelets - similar to above AFLP but mild. Nausea, RUQ pain, Jaundice, Emesis Rx by delivery and manage - pre-eclamsia, convulsions, DIC,

Answer 186

- chronic Liver disease, Cirrhosis - Dyspepsia, peptic ulcers - Pancreatitis - HTN, Cardiomyopathy - Withdrawal Seizures (delirium tremens) - Psychiatric disease - Wernicke's or Korsakoff's - Social difficulties - Increased risk of malignancies: Oropharyngeal, Laryngeal, Esophageal, Liver, Breast

Answer 187

Education and counseling Multidisciplinary team management Acute presentation: = Plasma pheresis to remove antibodies = IV IG injections = Supportive Between attacks: = Corticosteroids except in Psoriasis or Myasthenia as exacerbates CST sparing agents: Azathioprine (check MPTP activity first), Methotrexate, HCQ etc Biologicals: Infliximab (anti TNF), Rituximab (Anti CD20)

Answer 188

Pituitary adenoma will cause: Cavernous sinus compression - Hypothalamic comopression - Hydrocephalus as blocked foramen of monroe - Hormonal deficiencies - visual field defects (Bitemporal or binasal hemoanopia) = Low TSH: 2ry Hypothyroidism, wt gain, Hoarse voice, Hard stools, Menorrhagis, Hot preference, slowness of thought = Low ACTH: pale white skin, smooth contour, wt loss, Low BP = Low GH: Cretinism in kids, Poor bone or muscle mass in adults = Low FSH, LH: in Men: Loss of libido, Loss of facial, axillary, pubic hairs, Erectile dysfunction, infertility in women: amenorrhea, infertility, Hot flushes = Low Pralactin: no lactation after delivery

Answer 189

Risks to explain: Cardiovascular, OSA, GERD, GB stones, Diabetes mellitus, risk of GI/Uterine/ Breast cancers Enroll in weight loss program Dietician consultation Physiotherapy-Exercise regime Medications: Orlistat - lipase inhibitor Sibutramine: if wt loss at least 5% at 3 months - give for 2 yrs Bariatic surgery, Gastrectomy if BMI > 40 - might cure DM/ HTN/ OSA

Answer 190

= Vitamin D deficiency = Hypo-parathyroidism (Sx, Autoimm) = Pancreatitis, acute or chronic = Renal failure = Acute Hyper-phosphatemia = Hyper-calciuric hypocalcemia + kidney stones (Autosomal dominant) = Pseudo-Hypo-parathyroidism, 1a, 1b = Hypomagnesemia = Drugs: Cisplatin, Foscarnet, Biphosphonates

Answer 191

Type 1a = clinical features + skeletal abnormalities Type 1b = end organ PTH resistance without skeletal abnormalities Pseudo-Pseudo-HypoPTH: = in first degree relatives: only skeletal abnormalities but NO PTH resistance Clinically: Short stature, Round facies, Short 4th metacarpal as knuckle absent of both hands, Short or absent distal thumb phalanx absent - short stubby thumb with cutaneous calcinosis called - Murderer's thumb Obesity if common (high BMI) cutaneous calcifications as in SS Associtations: = Signs of Hypocalcemia: Chvostek or Trousseu signs, tetany, spasms, QTc = Hypothyroidism features

Answer 192

Acute severe asthma: - PEFR 33-50% of best/predicted - Respiratory rate > 25/min - Pulse > 110/min - cannot complete sentence in one breath Life threatening asthma: - PEFR < 33% of best/ predicted - SpO2< 92% - PaO2 < 8 kPa - normal PaCO2 (should be low) - altered sensorium, Fatigue, Exhaustion, Arrhythmias, Hypotension, Cyanosis, Silent chest, poor effort

Answer 193

1. No breathlessness except on strenuous exercise 2. Short of breath when walking up a short hill in a hurry 3. Walks slower than contemporaries on level ground because of breathlessness or has to stop for breath if walking at his own pace 4. Stops for breath after walking about 100 meters or after few minutes on level ground. 5. To breathless to leave the house

Answer 194

- severe outflow obstruction, FEV1<30% - Cyanosis - Polycythemia - Peripheral edema - Raised JVP - SpO2 < 92% at room air

Answer 195

Mass: Adenoma, Craniopharyngioma, Cysts, Metastasis Surgery or Radiotherapy Infiltrative: Sarcoid, Haemochromatosis, Histiocytosis X Lymphocytic Hypophysitis: in Late pregnancy, Postpartum Infarction - Sheehan syndrome, Postpartum Haemorrhage + Hypovomemia Apoplexy: Sudden haemorrhage in pituitary gland Empty Sella syndrome Trauma, Sub Arachnoid Haemorrhage

Answer 196

pituitary means phlegm like from early belief that it caused phlegm to produce Sella turcica means turkish saddle it looks like. = Primary empty sella syndrome is not with hypo-pituitarism. = As a general rule, in progressive pituitary disease, secretion of GH (growth hormone) & gonadotropin is affected before that of ACTH, TSH - hence loss of bone & muscle mass, hair loss occurs first. = Pituitary ACTH deficiency doesnot cause salt wasting , (low Na, High K and hypovolemia) because mineralo-corticoids are maintained through RAA system but glucocorticoids deficiency adds to hypotension.

Answer 197

Pre-Op: Baseline check visual fields, Baseline Labs: TFT, Prolactin, Estradiol, FSH, LH, Morning cortisol Day of Surgery: Start IV Hydrocortisone 200 mg Post Op: Oral steroid 72 Hrs after surgery Watch for DI (High urine volume, High Sr Osmolality, Low Urine Osmolality means dilute. Rx - Desmopressin Nasal On Discharge: Medic alert bracelet to give, says on CST Steroid Card to give, mentions doses After 3-4 weeks: Do Pituitary Stimulation Tests Free T4 replacement if low as TSH may be Normal.

Answer 198

1. Testicular tumors: = Germ Cell Origin: secrets HCG, 5% = Leydig cell tumor: cause precocious puberty 20% = Sertoli Cell tumors: with Peutz-Jegher syndrome or alone - excess adrogen production + conversion to Estrogen 2. Adreno-cortical Tumors: High adrogens secretions converted to estrogens with effects 3. Ectopic HCG Tumors: Ca Lung,/ Stomach/ Kidneys/ HCC 4. Post Chemo or radiotherapy for tumors of gonads.

Answer 199

Anti-androgens: Cyproteron, Finasteride, Dutasteride GI drugs: Ranitidine Cancer chemodrugs: Vinca alkaloids, Imatinib, Ciclosporin CVS Drugs: Digoxin, Spironolactone - dissociated estrogen from Sex-Hormone binding globulin. Amiodarone, Methyl DOPA. Anti-Microbial Drugs: INH, Ketoconazole, Metronidazole, HAART therapy Neuro Drugs: TCA, Phenothiazines, Metoclopramide, Opiates

Answer 200

Secrateogogues: stimulates insulin release 1. Sulfonyl Ureas: Glipizine, Gliclazide, Glimipiride, Chlorpropamide, Tolbutamide 2. Megalitinide analoguea: Natilidine, Repaglinide Insulin Sensitizers: 1. Biguanindes: Metformin 2. Thiazolidinones: Rosiglitazone, Pioglitazone Slowing Digestion: alfa Glucosidase inhibitors: Acarbose, Miglitol GLP-1 analogues: Exenatidine DPP-4 inhibitors: Sitagliptin, Vildagliptin Sodium-Glucose transporter-2 (SGLT-2) inhibitors: canagliflozin, dapagliflozin, and empagliflozin Dopamine agonists: Bromocriptine (cycloset) but can cause lung fibrosis.

Answer 201

General: Baseline sugar level, Cortisol, ACTH, Bone density scan, K levels Specific: = Adrenal, Pituitary MRI = 24 Hr urinary cortisol = Midnight serum cortisol = Overnight Dexa suppression Test: 1 mg dexa given at 11pm and cortisol checked next day at 8 am. = Low Dose Dexa suppression: 0.5 mg dexa every 6 Hrly for 48 Hrs and then cortisol levels checked = High Dose Dexa Suppression: 2 mg dexa every 6 Hrly for 48 Hrs then check cortisol levels = CRH (corticotropin releasing Hormone) Test: corticotropine is given after low dose dexa suppression = Inferior petrosal sinus ACTH sample versus peripheral ACTH level.

Answer 202

Cushingoid features due to excess cortisol, obesity, moon face, central obesity BUT = No proximal myopathy features. = No skin manifestations like purpura, bruising, striae etc seen with: = Depression = Alcoholism = Morbid obesity, Pickwickian = Chronic infections

Answer 203

ACTH dependant: with bilateral adrenal hyperplasia 1. cushing's disease: Pituitary tumors, visual field defects 2. Ectopic ACTH source: Ca Lung, Ca pancreas, Carcinoid : Wt loss, Hyperpigmentation, Pallor, other cancer signs ACTH independant: Adrenal adenomas Adrenal carcinomas Exogenous Steroid Intake

Answer 204

If cushings syndrome (2ry to CST therapy) suspected, look for: 1. complications such as : DM: finger prick, insulin marks, lipoatrophy at insulins sites, retinal changes HTN: BP, Retinopathy Osteoporosis: Vertebral collapse, Kyphosis Cataracts: secondary to steroids given 2. Steroid responsive conditions: Asthmatic - COPD features ILD patient Rheumatoid arthritis patient SLE patient 3. Ectopic ACTH tumor: Ca lung or pancreases Carcinoids - flushing attacks, wheeze

Answer 205

1.Various Skin problems: Atrophoc thin, striae, purpura, acnes 2. Proximal myopathy: difficulty risin from chair or toilet seat, difficulty combing hairs 3. Bilateral adrenalectomy scars- usually on back due to retro-peritoneal approach 4. Visual fields: Bitemporal Hemoanopia with headache 5. Glucose intolerance or DM 6. Uncontrolled HTN due to ectopic ACTH despite multiple drugs 7. Hyperpigmentation: Nelson syndrome due to High ACTH after adrenal removal due to negative feedback 8. Hypokalemia due to High ACTH, High mineralocorticoids - DO ECG, check for myopathic weakness

Answer 206

Skin atrophy, thinning of stratum corneum Poor wound healing Purpura, due to loss of subcut tissue Violaceous striae-skin atrophy Fungal infections: Nails, Interdigital Acena, Hirsutism (androgen excess) Hyperpigmentation (Nelson syn) Acanthosis nigricans - velvety Hyperpigmented skin at neck, axilla Telangiectasisa - topical steroids Other non skin: HTN, Proximal myopathy, Osteoporosis, DM

Answer 207

Head: Headache, confusion, Depression Chest: Heartburn, ECG short QTc Abdomen: STONE PAIN, Constipation, Hematuria, Polyuria, Polydipsia Low back pain (in myeloma)

Answer 208

Primary Hypothyroidism: - Autoimmune, Hashimoto's - Idiopathic - Post radio-iodine therapy - Post thyroidectomy - Iodine deficiency - Anti-thyroid medication use - Transient: Subacute/ Post partum painless Infiltrative: Systemic sclerosis, Sarcoidosis, Reidel's thyroiditis Other Drugs: Amiodarone, Lithium, Interferon alfa, Sunitinib, Phenytoin, Rifampicin, Carbamazepine Secondary causes: Pituitary disorder with Low TSH Hypothalamic

Answer 209

Wolf-Chaikoff Effect: Iodine excess can cause hypothyroidism by preventing organification of iodide & inhibiting thyroxine synthesis Jod-Basedow Phenomenon: iodine excess also can induce hyperthyroidism in people with baseline iodine deficiency

Answer 210

Infective: Peri orbital cellulitis Allergic: conjunctivitis Local: blepharitis Systemic: dermatomyositis Nephrotic syndrome Cardiac failure Liver failure Hypothyroidism: due to deposition of glycosaminoglycans

Answer 211

- Chemosis: periorbital and lid edema - Exophthalmous: eye pushed anteriorly, sclera visible above lower lid (Look from side and then from above) - Ophthalmoplegia: Restricted eye movements due to fibrosis and edema of eye muscles. Most early & prominant with upgaze to early Inf rectum involvement. complex ophthalmoplegia if multi directional. - Exposure keratitis & corneal ulceration - Optic nerve compression: Look for visual acuity, color vision, RAPD - Glaucoma: due to low episcleral venous flow. - Pre-tibial myxedema Thyroid acropachy: thickening of fingers, acropachy Goiter: diffuse and nodular with Bruits

Answer 212

digital clubbing, skin tightness with or without digital clubbing and usually with small-joint pain (in severe cases), soft tissue edema, and reactional periosteum, and skin alterations in fingers and nails may also be present

Answer 213

check TFT 4 wkly on treatment Propranolol Thionamides: = Carbimazole: can cause agranulocytosis, ANCA vasculitis = Propyl Thiouracil: preferred in pregnancy with Graves Na 131-Iodine: = ablates thyroid gland in 6-18 weeks + Steroids to reduce worsening ophthalmoplegia. = Avoid smoking as exacerbates it = Avoid contact with children, pregnants for 2 wks if radio-iodine given = Avoid pregnancy upto 4 months post iodine therapy Risk of hypothyroidism development 2-3% per year after radio-iodine Rx

Answer 214

- sleep upright with eyes taped shut to reduced lid edema - lubricating eye drops (Systane ultra, Reflesh, Celluvisc) - Prism glasses to reduce diplopia - Lateral tarsorrhaphy, helps with edema - Stop smoking, wear sun goggles - Inform DVLA and stop driving

Answer 215

Stellwag's sign is a sign of infrequent or incomplete blinking associated with exophthalmos or Graves orbitopathy. It is accompanied by Dalrymple's sign, which is a retraction of the upper eyelids resulting in an apparent widening of the palpebral opening. Kocher's sign: fixed staring look Gottron's sign: absent forehead creases on upward gae Mobius sign: poor convergence Von Graeffe sign: Lid Lag Ballet Sign: restriction of > 1 EOM Grave Sign: resistance to pulling down the retracted eyelid.

Answer 216

for adrenal insufficiency, done if morning cortisol if < 500 (low) How: = short dose of synthetic ACTH (synacthen) is given (250 mcg) and cortisol is measured at 30 min and 60 minutes. Should rise normally. Results interpretation: Normal Cortisol > 550 mmols/Lit Secondary Adrenal failure:= if ACTH < 10 ng/lit ie pituitary causing low cortisol release from adrenal Primary Adrenal failure: impaired cortisol response and ACTH level > 200 ng/Lit

Answer 217

adrenal autoimmune failure with low cortisol: - 21-hydroxylase antibodies are markers of autoimmune Addison disease, in Females: Loss of axillary, pubic hairs as adrenals produce androgens and lost in Males: no loss of axillary, pubic hairs as testicles produce androgens Other features: - Pigmentation along: oral mucosa, skin creases, pressure areas due to ACTH - Widespread pigmentation in Nelson syn (rule out Bitemporal Hemianopia) - Postural Hypotension - attacks of Hypoglycemia, hungerpangs - Evidence of Past TB or Past adrenalcectomy scars on back - calcification of auricular cartilage if 9 am cortisol > 500 - not addison

Answer 218

Vitiligo Type I IDDM Autoimmune thyroiditis, Graves disease Pernicious anemia: pallor, splenomegaly, polyneuropathy, SACD Hypoparathyroidism: chvostek sign, Trousseu sign, Cataracts, Tetany Alopecia areata Rheumatoid arthritis, Hand signs SLE - SOAP BRAIN MD Sjogren's syndrome: dry eyes, arthritis, raynaud's, vasculitis Non immune causes: TB, Meningococcemia

Answer 219

Autosomal recessive Cystathione B synthetase Homocysteine accumulation and excess urinary excretion - marfanoid features, tall stature, spidery fingers, high arch palate - Downward lens dislocation (upward in marfans syndrome) - Genu vulgum, knock knees - Pes cavus, Pectum excavatum/carinatum - Flush across cheeks - Intellectual disability - Seizures, Glaucoma, Myopia, Cataracts, Optic Atrophy - Intravascular atheroma: thrombosis tendencies Also can occur with deficiency of B12, B6, folate Rx - High dose of B6 and correct other deficiencies

Answer 220

overnight fasting to do and measure baseline Weight, Osmolalities During Test: Check Wt + Urine+Plasma Osmolalities every 2 Hrly for 8 Hrs = STOP if > 5% weight loss = Give Desmopressin if 3% Wt loss, Sr Osmolality > 300 mosm/kg and Urine Osmolality < 600 at 8 hrs Normal response: Uosm > 600 Cranial DI: Uosm > 600 after desmopressin subcutaneous Nephrogenic DI: Uosm < 300 even after Desmopressin subcute Primary Polydipsia: Uosm rises after desmo but doesnt reach normal ie > 600

Answer 221

Cranial DI: due to head injury/ infections post pituitary surgery for adenomas Radiation therapy to head craniopharyngiomas, Meningioma Pituitary mets or adenomas Infections: TM, Meningococci, abscess Inflammatory: Sarcoid, Langherhans histiocytosis Vascular: Apoplexy, aneurysms, Autoimmunes Nephrogenic DI: resistance to ADH action: Lithium induced Ofloxacin Orlistat Amphotericin Demeclocycline Foscarnet Chronic kidney disease Familial type Hypokalemia, Hyperkalemia

Answer 222

these tests are done to check pituitary function 3, 4 weeks after surgery 1. Metyrapone Test: 2. Insulin Tolerance Test: both should cause Hypoglycemia. Stop steroid replacement night before test. 3. Short synacthen Test: the test can distinguish whether the cause is adrenal (low cortisol and aldosterone production) or pituitary (low ACTH production). - artificial ACTH is given, 250 mcg of Synacthen, Baseline cortisol+ACTH levels measured before test and cortisol measured at 30 min, 60 min after. Cortisol should rise > 550 ng at 30 min. In Addison's disease, both the cortisol and aldosterone levels are low, and the cortisol will not rise during the cosyntropin stimulation test = In secondary adrenal insufficiency, due to exogenous steroid administration suppressing pituitary production of ACTH or due to primary pituitary disorder causing insufficient ACTH production, the adrenal glands will atrophy over time and cortisol production will fall and patients will fail stimulation testing. Early in the development of secondary adrenal insufficiency, the adrenals may not have atrophied and can still stimulate, resulting in a normal cosyntropin stimulation test. If secondary adrenal insufficiency is diagnosed, the insulin tolerance test (ITT) or the CRH (corticotropin-releasing hormone) stimulation test can be used to distinguish between a hypothalamic (tertiary) and pituitary (secondary) cause but is rarely used in clinical practice. Chest again at 2 Yrs.

Answer 223

ACTH is a hormone produced in the anterior pituitary gland that stimulates the adrenal glands to release cortisol, dehydroepiandrosterone (DHEA), dehydroepiandrosterone sulfate (DHEA-S), and aldosterone.

Answer 224

female gender as only x present =Short stature but fertility normal = Square chest, widely spaced nipples, short sternum. webbed & short neck due to hypoplasia of Cx vertebrae. = Nail dysplasia: small, more convex = Cubitus vulgus = Short 4th Metacarpal due to absent knuckle (Archibald Sign) , +? 5th also = Multiple body naevi = High arched palate (As in Marfans) Systemic: VSD/ Eyes/ Thyroid/ DM/ Legs/ Skeletal possible

Answer 225

two x chromosomes, male phenotype = Tall, long lower limbs = Gynecomastia upto 50% due to more estradiol -Ca breast risk high = Broad Hips = Mental Retardation: poorly in school = High pitched voice, Low Cognition = Hypospadius = Radio-ulnar synostosis (fused) = Reduced facial/ pubic/ axillary hairs = Small Low volume testicles, = Reduced Bone, Muscle mass (low testo) = High incidence of: MVP, Varicosities, = Auto-immune associations: SLE, RA, Sjogrens, DM, Hashimoto's Other Karyotypes possible: 46XX, 47 XXY Mosaic, 48 XXYY, 48 XXXY

Answer 226

Primary Ovarian failure with amenorrhea - most need HRT Cardiac: Bicuspid AV, Co-arc, HTN, Heartblocks Eyes: Ptosis, Strabismus (squint), Cataracts, Nystagmus Endocrine: Hypothyroidism, Goiter, Diabetes mellitus Legs: Lymphedema, MSK: Osteoporotic fractures, Scoliosis, Synostosis at Radio-Ulnar Jts Clinical: Short stature, webbed neck, widely spaced nipples, absent 4th knuckle & hence short 4th finger, Short convex nails, Cubitus vulgus

Answer 227

Cubitus valgus is a medical deformity in which the forearm is angled away from the body to a greater degree than normal when fully extended. A small degree of cubitus valgus (known as the carrying angle) is acceptable and occurs in the general population. Non-union of lateral condyle humerus fractures can cause progressive cubitus valgus deformity and has been reported to be the most common cause of TUNP. The cubitus varus is a complication of malunited supracondylar fracture. The deformity is triplanar consisting of varus, hyperextension, and internal rotation component. The prime indication for surgery is a cosmetic concern, while other indications include symptoms of instability and ulnar nerve involvement.

Answer 228

Turner syndrome, 45 XO Noonan syndrome: Male turner type Watson syndrome: AutoDom with PulmStenosis, CafeAuLait, Mental reta Klippel Feil syndrome: fused multiple neck vertebrae +cleft palate + Spina bifida +Scoliosis + Cardiac anomalies Diamond Blackfan anemia: Erythroid aplasia, low rbc+ cleft palate +abnormal thumbs+Urogenital defects +Cardiac anomalies

Answer 229

Male turner, xx/xy KARYOTYPE PTPN gene mutation Triangular facies, Prosis, Squint, Low Set Ears, Down like slanting eyes, Von willebrand disease Small genitalia, undescended Mental retardation Cardiac anomalies: PS, PVS, HCM, ASD, VSD, Branch PA stenosis MSK anomalies: webbed neck, short, wide nipples, cubitus vulgus, pectum excavatus/ carinatum

Answer 230

Elastin gene deletion, 7q11.23 Multiple anomalies Cardiac: subvalvular Aortic stenosis, PulmStenosis, MitralRegurg Sensory neural hearing deafness Hypercalcemia Elfine facies Chronic ear infections and/or deafness Dental abnormalities, such as poor enamel and small or missing teeth. Endocrine: hypothyroidism, early puberty and diabetes in adulthood. Farsightedness. Feeding difficulties in infancy.

Answer 231

C1esterase inhibitor (INH) deficiency, AutoDominant bradykinin induced airway edema No typical Urticaria/anaphylaxis/allergic triggers Can present as severe tummy pain also C4 is low during attacks Treatment: During attack: iCantibant, FFP Prophylaxis: Danazol, Lanadelumab iCatibant, (brand-Firazyr) is for acute attacks of hereditary angioedema in adults with C1-esterase-inhibitor deficiency. It is not effective in angioedema caused by medication from the ACE inhibitors. It inhibits bradykinin B2 receptors.

Answer 232

Allergic Angioedema: - IgE mediated mast cell degranulattion when exposed to triggers and histamine release. Urticaria present. Test IgE levels and skin allergy test. Drugs allergies as an example. Rx is with anti histamines - avoid triggers. Idiopathic Angioedema: non IgE mediated. Heat/cold/exercise induced mast degranulation & Histamine release + Urticaria. Dermagraphism noted. IgE levels normal + Skin tests negative. Exclude NSAID-Opiod-Contrast dye-Hypothyropidism to exclude. Rx antihistamines. Hereditary Angioedema: C1 esterase inhibitor deficiency, hereditary - bradykinin induced - AutoDom - No triggers - Sudden angioedema or airways+ pain abdomen. Low C4 levels, Low C1INH levels. Rx - Icantibant which is bradykinin B2 receptor antagonist. Danazol. ACE inhibitor induced Angioedema: Can happen anytime during treatment, even after years. Because of bradykinin accumulation. No urticaria, No anaphylaxis. Airway edema ++. Rx- Stop ACEi, ARBS also.

Answer 233

Inappropriate response of carotid sinus at bifurcation - to simple stimuli as neck movements, shaving, carotid massage, neck trauma - hypersensitive baroreceptors leads to vagus stimulation - sudden bradycardia, vasodilatation, hypotension and syncope. Testing: Do Carotid massage under close ECG monitor = ventilation pause > 3 seconds = Systolic BP fall > 50 mm Hg = Cardiac Pacing as Rx if symptomatic bradycardia results.

Answer 234

= Orthostatic - neuroCardiogenic = Supine: Cardiac arrhythmias = Vasovagal: fasting, stress, long standing, Heavy meals, Hot day = Situational - micturition/ cough/ defecation/ sneeze (vagal induced) = Seizures - flashy lights, sleep lack, Hunger, drugs, alcohol = Exertional-cardiac until proven otherwise (AS and HOCM) Carotid Sinus Hypersensitivity: shaving, neck massage, trauma, Tight shirt collar = Hypoglycemia - pre meal, fasting, insulin induced, OHA induced = Other Emotional: anxiety, Fear

Answer 235

Infection: mumps, arthritis Testicular torsion and loss, Sx CryptOrchidism: undescended testicles with risk of cancer and Hypogonadism Klinefelter syndrome, 47 XXY, primary hypogonadism Noonan syndrome: autoDominant elastin gene deficiency Varicocel with Left renal cancer Myotonic dystrophy, atrophic testicles Radiotherapy - atrophy as growing germinal cells sensitive to radiation induced damage Chemo-Drugs: Vinca, Cyclophosphamide, Cisplatin, HAART

Answer 236

Primary adrenal failure: #Autoimmune - addison's (Antibodies: Anti-21-Hydroxylase, Anti-17-Hydroxylase, Anti-cytochrome 450) #Tuberculosis of adrenals #Bilateral adrenalectomy for CAH #Amyloidosis #Adrenal metastasis #Adrenal bleeding with waterhouse friedrichson meningococcemia #HIV infection #Haemochromatosis #Disseminated Fungal infection - Histoplasmosis, Cryptococcosis #Congenital Adrenal Hyperplasia, Sx

Answer 237

Increased Platelet destruction: = ITP, usually after viral illness = TTP = Drugs - Heparin = SLE = DIC = Lymphoma = Hypersplenism Other mechanisms: = Platelet clumping = Bone marrow failure

Answer 238

CLO test for “Campylobacter-Like Organism”, is a rapid urease test (RUT) that accurately detects the urease enzyme of Helicobacter pylori in gastric mucosal biopsies. The test consists of a well of urease indicator gel sealed inside a plastic slide. The basis of the test is the ability of H. pylori to secrete the urease enzyme, which catalyzes the conversion of urea to ammonia and carbon dioxide. The urease test is involved with urea formation. The substrate in the urease test is urea. The products are carbon dioxide and ammonia. Since ammonia production is there, which is basic, it brings out a change of pH in the medium and eventually the colour of the medium. Procedure of urease test: Prepare an Urea broth by dissolving 2.95g of urea powder in 150ml of distilled water. Add urea after autoclaving the media to prevent urea from initial breakdown. Inoculate the given sample of organism aseptically using wire loop. Incubate the tubes at 37°C for 24 hours. Observe the result.

Answer 239

= Neoplastic intrathoracic disease associated with clubbing includes bronchogenic carcinoma, malignant and benign pleural tumors, metastatic cancers, Hodgkin lymphoma, thymoma, pulmonary artery sarcoma, nasopharyngeal carcinoma, rhabdomyosarcoma, primary lymphosarcoma of the lung, and esophageal cancer. = Suppurative: lung abscess, bronchiectasis, cystic fibrosis, empyema, and chronic lung cavitary mycobacterial or fungal infection. = Diffuse pulmonary disease associated with clubbing includes idiopathic pulmonary fibrosis, asbestosis, Langerhans cell histiocytosis, lipoid pneumonia, and pulmonary arteriovenous malformations. = Cardiovascular disease associated with clubbing includes cyanotic congenital heart disease, infective endocarditis, arterial graft sepsis, brachial arteriovenous fistula, aortic aneurysm, atrial myxoma, and hemiplegic stroke. = Gastrointestinal disease associated with clubbing includes inflammatory bowel disease, celiac disease, amoebiasis, ascariasis, and lymphoma of the gastrointestinal tract. = Hepatobiliary disease including biliary cirrhosis and juvenile cirrhosis also correlates with nail clubbing. = Metabolic diseases such as thyroid acropathy and severe secondary hyperparathyroidism too may present with the finding of clubbing. = Medication-induced cases also exist, including laxative overuse, interferon alfa-2A, and prostaglandin infusion.

Answer 240

High dysphagia: = Oropharyngeal cancers = Acute URTI, Abscesses = Pharyngeal pouch (chronic symptoms but no other red flags) Low Dysphagia: = Esophageal cancer = Esophageal candidiasis (+ DM, chr steroid use, Immunocompromised) = H/o Barrett's esophagus = GERD, chronic esophagitis = Eosinophillic eosophagitis = Esophageal dysmotility Disorders: Spasm, Nutcracker esophagus: High pressure spasms > 180mmHg. = Webs, Achalasia = Past Caustic injury = Sjogren's syndrome, SS, = Benign/ Peptic strictures: tell Avoid alcohol, Loose Wt, Stop smoking: Repeat OGD and dilatation after a month once histology proves bening

Answer 241

= an abnormal (metaplastic) change in the mucosal cells lining the lower portion of the esophagus, from stratified squamous epithelium to simple columnar epithelium with interspersed goblet cells that are normally present only in the small intestine and large intestine. This change is considered to be a premalignant condition because it is associated with a high incidence of further transition to esophageal adenocarcinoma, an often-deadly cancer. The condition is found in 5–15% of patients who seek medical care for heartburn (gastroesophageal reflux disease, or GERD), although a large subgroup of patients with Barrett's esophagus are asymptomatic. The condition is named after surgeon Norman Barrett (1903–1979) even though the condition was originally described by Philip Rowland Allison in 1946. frequent and longstanding heartburn trouble swallowing (dysphagia) vomiting blood (hematemesis) pain under the sternum where the esophagus meets the stomach pain when swallowing (odynophagia), which can lead to unintentional weight loss The risk of developing Barrett's esophagus is increased by central obesity (vs. peripheral obesity).[8] The exact mechanism is unclear. The difference in distribution of fat among men (more central) and women (more peripheral) may explain the increased risk in males.

Answer 242

= symmetrical deforming arthropathy of both hands with PIP & MCP joints swollen, spindling (DIP spared) = Palmar subluxation at MCP with Ulnar deviation gradually = Deformities: Swan Neck, Bouttinnier/ Z thumb = Rheumatoid nodules on extensor surfaces forearms = Palmar erythema = Wasting of small muscles of Hand = Flexor tendon nodules of trigger finger

Answer 243

Confirm if in pain, take permission to examine and put both hands on pillow. Look for nail changes, Heberden or Bouchard nodes @ DIP, PIP joints. Look for piano key signs and pseudo benedection sign. Movements: 1. Squeeze my fingers 2. Now spread your fingers 3. Do prayer sign & reverse prayer 4. Put hand on shoulder across chest 5. Nodules on olecranon bursae & psoriatic plaques over extensor elbows 6. Check shoulder Mobility: Put hands behind your head 7. Palpate ear lobes for gouty tophi, and hairline psoriasis

Answer 244

Bouchard nodes- swelling of PIP joints and Heberdene nodes are swelling @ DIP joints. Swan Neck: PIP hyperextension and MCP + DIP flexion deformity Boutonnier Deformity: Flexion@ PIP and Hyperextension & contracture @MCP, DIP Z deformity: Flexion @ IP and hyperextension at MCP joint Piano key sign: radio-ulnar Jt subluxation on pressure which springs back when released Psuedo-Benediction sign: Inability to extend 4th, 5th fingers due to flexor tendon ruptures by styloid process

Answer 245

# Eyes: Episcleritis, Scleritis, Cataracts, Sjogren's, Scleromalacia perforens, pallor due to anemia Cervical Spine: Atlanto-axial subluxations, Myelopathy # CNS: polyneuropathy, mononeuritis multiplex # Lungs: effusions, fibrosis, Caplans, Bronchiectasis # Heart: Pericarditis # Abdomen: Splenomegaly + neutropenia (Felty's), Hepato-splenomegaly # Kidneys: Nephrotic syndrome: anasarca, proteinuria, hypercholesterolemia, Low immunity as IgG lost also # MSK: Shoulder, Elbow, knee, Feet problems

Answer 246

= GFR drops below 30 ml/min = Uremic pericarditis or pleuritis = Uremic bleeds: Epistaxis, Hematuria = Volume overload: refractory diuretics = Severe metabolic acidosis = Severe Resistant Hyperkalemia > 5.5 = Uremic encephalopathy = Refractory Hypertension = Toxins: Lithium, Salicylates

Answer 247

PLASMA-TV - Phenobarbitol - Lithium - Acidosis - Salicylates - Metformin - Alcohols - Theophylline - Valproic Acid

Answer 248

Osteitis fibrosa: increased bone turnover due to secondary hyperparathyroidism causing bone cysts, bone pain & tenderness Osteomalacia: aluminium deposition in the bone causing decreased proper mineralization Adynamic Bone Disease: Most common with HD/PD and low PTH levels due to phosphate binders - without any defect in mineralization. How to treat: - Reduce phosphate intake: Give Calcium carbonate - Dexa Scan for bone density, is low: give Vitamin D/ Calcium - Biphosphonates

Answer 249

Clinical syndrome characterized by heavy proteinuria > 3 Grams per day, Hypoalbuminemia (< 30 Gms%), hyperlipidemia and thrombotic tendencies. 30% due to DM, HTN, Amyloidosis, SLE Rest due to intrinsic renal diseases as: 1. Minimal change disease - due to DM, NSAID, Hodgkins, HIV, IgA deficiency 2. Focal Segmental glomerulosclerosis: with HIV, Morbid obesity, NSAID, Idiopathic 3. Membranous GN: HepC, Malaria, Autoimmune, Paraneoplastic, Drugs 4. Mesangiocapillary GN with HepB/C, Leprosy, Cryoglobulinemia, Autoimmunes

Answer 250

micro-albuminuria - Protein uria - Nephrotic range proteinuria Urine Albumin: 30-300/24 Hrs Urine albumin/ Creatinine ratio >2.5 Proteinuria: Urine Albumin>300, AC ratio >30 Nephrotic range proteinuria: Urine albumin > 300, AC Ratio > 300, + edema + hypoalbuminemia Other Tests: 1. DM profile: OGTT, HBA1C 2. Autoimmune profile: ANA, dsDNA, ANCA, C3C4 3. Viral Serology: Hepatitis B, C 4. Urine and Plasma Electrophoresis 5. ASO titres 6. Renal Biopsy

Answer 251

Alcoholic Liver disease Viral Hepatitis B, C Autoimmune: PBS, PSC Metabolic: NASH, A1AT deficiency, Wilsons, Cystic fibrosis Drugs: Methotrexate, INH, Amiodarone, Phenytoin Effects of Portal Hypertension: varices and bleeding, Splenomegaly, Ascitis, SBP, Effects of Liver Dysfunction: Low albumin, Encephalopathy, Jaundice, Coagulopathy, HCC

Answer 252

Sedative use GI bleeds, Peptic ulcers, Melaena Sepsis: SBP, UTI Catabolic stress: Sx, Trauma TIPS High protein intake Electrolyte disturbance, Hypokalemia Large volume paracentesis-fluid loss Constipation - Hyperammonemia

Answer 253

reduced synthesis of coagulation factors 2, 7, 9, 10 Increased Prothrombin time Low platelet counts Portal vessels bleed due to high pressure as from varices Peptic ulcer bleeding - alcohol CLD

Answer 254

Grade 1: insomnia, Day:Night reversal Grade 2: Lethargy, disorientation Grade 3: Confusion, Somnolence Grade 4: Coma Causes of decompensation of CLD and precipitation of encephalopathy - any infection, SBP, Pneumonia - Sedative medications - Alcohol intake - GI Bleeding with digestion - Hypokalemia - HCC

Answer 255

Normal Portal flow - 1-1.5 Lit/min Normal Portal pressures-5-10 mmHg Pre-Hepatic Causes: - Portal vein thrombosis - Extensive compression ? tumor Hepatic Causes: - Cirrhosis, acute hepatitis - Congenital hepatic fibrosis Post Hepatic causes: - constrictive pericarditis - Budd chairi syndrome: hepatic vein thrombosis, ascites, hepato-splenomegaly, Portal HTN

Answer 256

1. Bilirubin - rising 2. Ascitis - present/absent 3. Encephalopathy - grades 4. Prothrombin time - prolonged 5. Albumin - low, how much? > 10 Score - 33% survival @ 1 yr

Answer 257

9.57 × loge (creatinine) + 3.78 × loge (total bilirubin) + 11.2 × loge (INR) + 6.43 if more than 10 - refer to Hepatology Rx strategies for CLD: *No Alcohol *Antivirals if Viral hepatitis *Immunosuppression *6 monthly USG to rule out HCC *6 monthly AFP to rule out HCC *OGD for variceal screening and Rx

Answer 258

Infections: Viral Hepatitis Alcoholic Hepatitis Malignancy Hepatic congestion due to CHF Vascular Liver disease: Budd Chairy, Sickle cell disease

Answer 259

Acute Hepatic VOC:sinusoidal congestion Hepatic sequestration/ Reverse sequestration Sickle cell intra-Hepatic Cholestasis: Acute-Benign-Chronic Miscellaneous: Budd chairy syndrome (Hepatic Vein thrombosis) Hepatic abscess-Bilioma-Infarction Zinc deficiency + Hyperammonemia Autoimmune hepatitis (AMA+SMA+) Associated C recurrent transfusions Iron overload, GB stones, Hepatitis B/C

Answer 260

ADPKD Multiple simple renal cysts Tuberous sclerosis VHL syndrome Meckel Gruber syndrome Laurence Moon Bardet Biedl syndrome Trisomy-13, 18, 21 (Patau-Edward-Down) Non-cystic Enlarged kidneys: - = Amyloidosis, = Carcinoma = Hydronephrosis

Answer 261

patients must have recurrent abdominal pain for at least 3 days per month during past 3 month associated with 2/ more: 1. Relieved by defecation 2. Onset with change in stool frequency 3. Onset in change in stool appearance Supportive symptoms: - Altered stool frequency - Altered stool form - Altered stool passage-straining/urge - Mucus with stool - Abdominal bloating

Answer 262

3-2-1 Rule: 3 affected family members over 2 generation and 1 is < 50 yr old 3 or more relatives with Lynch related cancers: Ca colon, Ca Stomach, Uterine cancer, Ovarian cancer, Renal cancer = one in first degree relatives Lynch associated cancers in 2 genera One or more cancers before age 50 IF Amsterdam criteria is negative: 50% possibility of Colo-Rectal-Cancer Refer for routine genetic test counselling

Answer 263

why do it? to prevent death and delay complications. Age 18-20 yrs: = Screen with frequent BP monitoring = screen if going to donate kidney Age > 20 yrs: = Screen with USG scan every 3-5 yrs

Answer 264

Proctitis: Topical 5-ASA suppositories once daily without or without steroid foams Left Colitis: (mild to moderate) Top Down: Bottom up regimen Oral ASA - 2 gram OD orally Rectal ASA - 1 Gram enema OD Add Oral steroids if > 10 days tried Extensive Colitis: TrueLove Witt criteria to apply here Admit and start: = IV Steroids = S/q LMWH start as thrombotic state = Daily AXR: megacolon> 7cm - Sx = while on steroids: CRP > 45@72 Hrs and > 3 stools/Day = Colectomy need in 85% cases. Alternative Rx: IV Ciclosporin, OR single dose Infliximab in UC

Answer 265

rounded clots - UB origin, elongated clots - ureter fever, flank pain - Stone, Pyelonephritis, Retention of clot Hematuria@ start of Urine: urethral bleed or lower bladder urine Hematuria throughout urine: Ureter, Kidney, UB source Hematuria terminal: bladder base or prostate source Recent URTI then Hematuria: IgA nephropathy Cough, Wheeze, Epistaxis, Hematuria: Wegner's granulomatosis, Goodpastures Pleuritic Chest pain & Hematuria: SLE Non blanching purpura + hematuria: SLE, Wegner's, Fever, Rigors, Hematuria: Pyelonephritis, IE, Vasculitis

Answer 266

= Addison's disease, High ACTH = Nelson syn., post adrenalectomy = Ectopic ACTH production-Ca lung = Haemochromatosis = Jaundice of any cause = Uremic frost = Pregnancy with pallor = Solar purpura = Cafe au lait spots: Neurofibromatosis = Venous eczema = Porphyria cutanea tarda Drug eruptions: Tetracyclines, Barbiturates, Sulfonamides, NSAIDs

Answer 267

Pseudohypoparathyroidism (Albright) Gouty tophi Calcified nodules of scleroderma Ehler Danlos syndrome Pseudoxanthoma elasticum Dermatomyositis Cutaneous tumors: Sarcoma's

Answer 268

Asymmentrical, DIP joints, onycholysis, nail pitting, transverse nail ridging, scaly silvery erythematous plaques over extensor surfaces Patterns: 1. Asymentrical 2. Symentrical Oligo Polyarthritis 3. DIP Arthritis 4. Arthritis mutilans 5. Spondylitis with sacro-ileitis Sites: extensors, scalp, navel, post ear Kobner phenomenon: sites of trauma Inverse Psoriasis: on flexor surfaces and intertriginous Associations: - Hyperuricemia and Gout - Aortitis, Aortic regurgitation - Apical Lung fibrosis - Conjunctivitis, Uveitis

Answer 269

sausage shaped swelling of finger or toes due to inflammation of the tendon sheath and soft tissues Occurs in: - Spondyloarthropathies - Sickle cell disease - Tuberculosis - Syphilis - Sarcoidosis

Answer 270

symptomatic sacro-ileitis with persistent pain and stiffness more than 3 months associated with morning stiffness & improvement on exercise. Reduced Chest expansion. Protuberant abdomen Severe kyphosis Question mark posture Reduced cervical movements Reduced spinal movements Increased Occiput: wall distance Modified Shrober Test Aortic regurgitation Apical Lung fibrosis Heel Pain due to enthesitis Uveitis AV conduction blocks HLA B 27 association (95% caucasian) RA Factor negative (seronegative) Rx: - Multidisciplinary approach - General: Education, Physiotherapy, Occupation, - Treatment: Pulses steroids, DMARD's, Biologicals (Adalimumab)

Answer 271

de novo or late Polycythemia rubra vera, essential thrombocythemia Fibrosis by stimulated fibroblasts as growth factors secreted by diseases platelets in thrombocytosis develops around 50-60 years of age Marrow fails: anemia, Leucopenia, Low platelets Splenomegaly as extra morrow erythropoesis Blood film: Leuco-Erythroblastic film with Tear drop cells Exclude: MPD, 2ry myelofibrosis Can progress to AML

Answer 272

Platelet counts > 600k Risk: thrombosis, bleeding, myelofibrosis (10% chance by 10 years) Progression: AML, 1-2% @10 yrs JAK2 mutation in 50% cases Reactive thrombocytosis need to be excluded - look for IDA, Bleeding tendencies, inflammation, Solid malignancies Rx: Aspirin, Hydroxyuria, Anagrelide

Answer 273

Venesection: initially weekly then 2 weekly and 3 monthly until rbc-Hb stable, takes around 2 yrs Cytoreductive therapies: HU Aspirin if Platelets < 1500k Target PCV < 0.45, Platelets < 400k Other Options: Interferon alfa Anagrelide

Answer 274

mostly due to hypercoagulation Strokes, STEMI, Priapism Erythromelalgia: red fingers/ hand/ feet with burning sensations Hepato-splenomegaly Peptic ulceration Gout attacks Leukemic transformation Lymphopenia Myelofibrosis Hypo-splenism with infection risk Extra medullary hematopoesis with bone deformities, Hepatomegaly, splenomegaly

Answer 275

1. Polycythemia rubra vera: increased red cell mass, High Hb>17, normal EPO levels, High PCV 2. Essential thrombocythemia 3. Idiopathic myelofibrosis 4. Chronic myeloid Leukemia 5. Chronic Neutrophillic Leukemia 6. Chronic Eosinophillic Leukemia

Answer 276

1. High PCV > 45% High RBC mass Absolute polycythemia EPO normal: P. rubra vera EPO high: 2ry Polycythemia = EPO appropriately High: CHD, Hypoxia, CO poisoning, Chronic lung disease, OSA, Smoking, COPD = EPO inappropriately high: Renal cell cancers, Uterine liomyoma, Cerebellar Haemangioblastoma, HCC 2. High PCV but RBC mass normal suspect reduced plasma volume with Apparent Polycythemia: due to dehydration, diuretics, Obesity, Alcohol, Smoking, Stress

Answer 277

PCV > 0.51 in men, > 0.48 women Hb > 17 in men or > 15 in females Rbc mass > 60 in men, > 56 women - headache - Lethargy - Breathlessness, Dizziness - Weight Loss - Night sweats - Pruritus: increased after hot baths - LUQ pain due to splenomegaly - Flushed face - conjunctival suffusion - Engorged retinas

Answer 278

- Anemia of chronic disease - NSAID induced GI loss - Folate deficiency with methotrexate - Pernicious anemia of Autoimmune+ - Felty's syndrome, with splenomegaly - Autoimmune Hemolytic anemia - Anemia of CKD, low EPO - Bone Marrow Suppression: due to therapies: Gold, Methotrexate, Sulfasalazine, Penicillamine

Answer 279

Episcleritis: acute red eye but no pain Scleritis: deep eye pain Kerato-conjunctivitis: Sicca, Sjogren's EOM Palsy or tenosynovitis due to mononeuritis multiplex Scleromalacia perforans Penicillamine induced MG, ptosis Steroid induced cataracts Chloroquin induced retinopathy, OA

Answer 280

Men-1: PPP, Chromo 11q13 1. Parathyroid adenoma, High calcium (95%) 2. Pituitary Tumors: prolactinoma: GH producing adenoma: 3. Pancreatic Neuro-Endocrine tumors (40%) like: Insulinoma: Hypoglycemia, Wt gain VIPoma: watery diarrhea, Low K Somatostatinoma: Glucagonoma: Glucose intolerance Gastrinoma: Peptic ulcers May be adrenal(cortisol) or carcinoid tumors, Lipomas

Answer 281

MEN-2: two types 2a: TAP *Thyroid medullary cancer *Adrenal phaeochromocytoma, 50% benign and bilateral adenomas *Parathyroid hyperplasia, 80% 2b: TAN *Thyroid medullary cancer *Adrenal phaechromocytoma *Neuromas of Lips, Cheek, Tongue, Eyelids, Visible corneal nerves Alone MEN-2 - Thyroid medullary Ca Phenotype: Marfanoid habitus, Mucosal abnormalities, Megacolon, Medullated corneal nerve fibers

Answer 282

MEN-3 or 4 MEN-3: Neurofibromatosis MEN-4: Carney complex: Spotty skin pigmentation Atrial myxomas Pituitary adenomas Adrenal tumors Testicular tumors

Answer 283

Barter Syndrome: similar to frusemide action defect in Calcium transport in loop (Na/K/Cl) exchanger channel: = Metabolic alkalosis (HCO3 gain) = Hyponatremia (Na loss) = Hypomagnesemia (Mg loss) = Hypokalemia (K loss) = Hypocalcemia (Ca Loss) = Hypercalciuria (causes stones) Gitelman Syndrome: Thiazide like effect due to defect in distal Na-Cl co-transporter in distal tubules (which is blocked by thiazides with same effect as this syndrome) = Hyponatremia (Na loss) = Hypercalcemia (Ca gain) = Hypocalciuria (Ca less secreted)

Answer 284

K-Ketoacidosis, DKA U-Uremia, CKD, AKI S-Salicylates overdose, Starvation M-Methanol, Ethanol A-Alcohol L-Lactic Acidosis > 4 mmols Ethylyne Glycol (AntiFreeze) High Lactate Levels: = Ischemia = Infarction = Sepsis = Metformin

Answer 285

Low HCO3 but normal AG: Diarrhea, Renal tubular acidosis G- Glycols: Ethylene, Propylene O- Oxoproline L- L Lactate D- D Lactate M- Methanol A- Aspirin overdose R- Renal failure, Uremia K- Ketoacidosis

Answer 286

Suspect PNH: Any change in color of urine, does it turn darker in morning Suspect Behcets: painful Orogenital ulcers, suspect APLS: recurrent miscarriages, blood clot history (DVT, PE) Risks of DVT: Long travels, POP cast, Recent major surgery, Immobilization Cancer screen: Wt Loss, unexplained anemia, Lumps-bumps in body, family history Suspect Polycythemia: Smoking, Hyper-viscocity symptoms: Dizziness, Flushed face, Pruritus after hot baths, headaches

Answer 287

if one autoimmune present - also screen for presence of others suspect Addisons: = Postural Hypotension = frequent hunger pangs as hypoglycemia = Skin hyperpigmentation Suspect Thyroid problem: hot or cold preference Hard stools Staring looks, racing heart Weight loss despite good appetite Menorrhagia or poor menses PCOD- Hirsutism, Wt Gain, Suspect RA: hand small Jt arthropathy rheumatoid nodules Rheumatoid Lung Suspect Coeliac disease: Pale, frequent bloating with certain foods, malnutrition, clubbing, dermatitis herpetiformis, Hard to flush stools Premature Ovarian Failure: female history, infertility, Wt loss, Hot flushes, vaginal dryness, Night sweats, dry eyes, poor libido Pernicious Anemia: B12 deficiency, limb paraesthesias, Looks pale, Poor appetite, diarrhea or constipation, Postural Dizziness, Swollen red tongue, Bleeding gums, Exertional dyspnea

Answer 288

Addison's disease Melanoma Peutz Jegher syndrome Leukoplakia Lichen planus Amalgam tattoo : Mercury, Silver stains

Answer 289

Male Urethral Discharge: infective: Gonorrhea Chlamydiae Trichomoniasis Non Specific urethritis Ureaplasma urealyticum Herpes simplex Non infectious: Cystitis, Prostatitis, Reiter's syndrome Female Vaginal Discharge: Physiological Pathological Infective: STI - Chlamydia, Gonorrhea Non STI - Candida, Trichomoniasis Non-infective: Ectropion, Polyp, Cancer, atrophic vaginitis

Answer 290

when patient information can be shared = Legal requirement = Patient agrees = Divulgence is in patients best interest = Divulgence is in public's best interest Consent: Capacitous + Informed + voluntary without coercion + continuous until withdrawn

Answer 291

BANJC Beneficence - do good Autonomy - right to decision, Rx Non-maleficence -do no harm Justice - same for all Duty of candor

Answer 292

urine > 3 Lit/ Day 1. Inability to concentrate urine: - Diabetes insipidus: Cranial (< 4 wks onset), Nephrogenic 2. Osmotic diuretics: - Diabetes mellitus - Diuretics - AKI recovery phase 3. Primary Polydipsia: - Psychogenic - Habitual water drinking

Answer 293

CAM Tool 1. Acute onset - Fluctuating: Is this a dramatic change from baseline? Does abnormal behavior come & go? 2. Inattention: Does he/she have difficulty focussing or easily destractable? 3. Disorganized thinking: Do you have difficulty following his/her conversation? 4. Altered LOC: does he/she have episodes of drowsiness or agitation? Confusion present: 1 + 2 and/or 3/ 4 ie Acute, Fluctuating + Inattention

Answer 294

Metabolic causes: - Cushing's syndrome - Acromegaly - Hypothyroidism - PCOD - Insulinoma - Hypothalamic lesions + Hyperphagia Fluid Retention: - Chronic Lung, Heart, Kidney disease - Protein loss with anasarca Drug induced: - Insulin, OHAs - OC Pills - Depot Hormones - Steroid therapy - Olanzapine - Clozapine - Risperidone - Tricyclics antidepressants - Valproate, Carbamazepine Other Causes: - Abdominal masses - Obesity - Sedentary lifestyles - Pregnancy wt gain - Nicotine withdrawal - Leptin deficiency - Prader willi syndrome

Answer 295

CD4: 200-350/cumm = Pulmonary TB = Pneumococcal pneumonia = Oral-Vaginal candidiasis = Oral Hairy Leukoplakia = Kaposi's sarcoma = Cervical intra-epithelial neoplasia CD4: < 200/cumm = Esophageal candidiasis = Muco-cutaneous HSV = Cryptosporidium diarrhea = Miliary TB = Pneumocystis jeroveci pneumonia = Disseminated TB CD4 < 100/cumm = Atypical mycobacterial infections = Brain toxoplasmosis = Non Hodgkins lymphoma = Cryptococcal meningitis = CMV retinitis, colitis = HIV Dementia

Answer 296

Bacterial rashes: = Staphylococcal impetigo, Folliculitis = Gonorrhea: maculopapular = Meningococcal: maculopapular = Secondary syphilis: macular Viral Rashes: - HIV seroconversion - Varicella zoster - Coxsackie Hand foot Mouth disease - Hepatitis B associated - Parvoviral infection Fungal: Cryptococcal: maculopapular/ violaceous nodular Intertrigo

Answer 297

reduced hair loss in males as low testosterone and menstrual change in female, irregular, scanty or menopause Primary Hypogonadism: Testicular failure with high LH, FSH levels - Klinefelter syndrome Increased peripheral aromatization of testosterone to estrogens with Gynecomastia Secondary Hypogonadism: 2ry to pituitary causes or Hypothalamic causes High Prolactin, low LH levels Low androgens Low LH, Low FSH 1. Adolscents patient: Do GnRH test- give it - Rise of LH, FSH: Delayed puberty - No Rise of LH, FSH - True Hypogonadism: #1. Check Prolactin = High, Do MRI pituitary # 2. Check Transferrin = High - suspect Haemochromatosis and check Serum Iron, Genetics 2. Adult patient: repeat LH, FSH, Testosterone after 6 weeks: consistenly low - suspect systemic illness and work up for ? Cushings Adrenal cortex continues to produce estrogen precursors which are converted to estrogen peripherally with effects as gynecomastia, los hair loss, low libido etc

Answer 298

Low Androgens, Low LH/FSH - Hypogonadism - Kallman syndrome: anosmia, cleft palate, Osteoporosis, mutations in the ANOS1, CHD7, FGF8, FGFR1, PROK2, or PROKR2 gene, both sexes affected, no GnRH produced - Prader Willi syndrome: Chr 15deleted (Paternal), both sexes, Obesity, DM - Dandy walker malformation: congenital with cerebellar ataxia, late spasticity, MR, Hypotonia, Hypocephalus - Isolated LH deficiency - Hyperprolactinemia, low androgens - Haemochromatosis - Morbid obesity - Malnutrition

Answer 299

Hypogonadism: primary (testicular) or secondary (pituitary or other) Diabetes mellitus Autonomic Neuropathy chronic Liver disease Drug induced

Answer 300

Symptoms of uncontrolled HTN: - Headaches, Blurred vision - Palpitations - Chest pain, ACS - Ankle edema, proteinuria Reversible: Obesity, Alcohol #Macrovascular complications: MI, Strokes, PVD, Eye problems #Microvascular complications: retinopathy, proteinuria, coronary, Encephalopathy Secondary Hypertension: -Hyperthyroidism - Acromegaly - Cushing's syndrome/disease - Conn's syndrome - High aldosterone - Phaemochromocytoma ? MEN - White coat Hypertension - Renal disease HTN - Co-arctation of aorta - Obstructive sleep apnea - Drugs: NSAIDs, Pseudoephedrine, Anabolic steroids, Cocaine, MDMA, OC pills, Corticosteroids, Ciclosporin

Answer 301

M - medication effects E - Emotional, Dementia, depression A - Anorexia, Alcohol, Apathy L - Late life paranoia S - Swallowing disorders O - Oral health: dentures, thrush N - No money W - wandering dementia behavior H - Hyper/Hypothyroidism, Hypercalcemia, Hypoadenalism E - Enteric problems, Malabsorption E - Eating problems, unable to feed themselves as in stroke etc LS - Low salt, Low cholesterol diet, Social problems

Answer 302

Crazy - hyperthyroid Lazy - Hypothyroid Hypercalemic conditions Hyperparathyroidism Hypomagnesemia Hypophosphatemia Hypokalemia Wilson's disease Acromegaly Haemochromatosis Rheumatoid arthritis

Answer 303

It is caused by the deficiency of the uroporphyrinogen III decarboxylase (UROD) enzyme. inherited 20% AutoDominant Acquired 80% cases - very itchy bullous skin lesions on sun exposed areas, bullous and scars on rupture with excess hairs around and dark reddish urine. acute attacks of pain abdomen. - Triggers: Sun exposure, OC pills, Alcohol, Iron supplements, Many medications can cause pseudoporphyria, including propionic acid–derivative NSAIDs (e.g., naproxen, diflunisal, ketoprofen, nabumetone, oxaprozin, and mefenamic acid), furosemide, tetracycline, fluoroquinolones, amiodarone, cyclosporine, dapsone, etretinate, and flutamide. HCV association due to frequent needling-transfusions-surgeries. Diagnostics: combination of blood, urine or stool testing to measure porphyrin levels. Take social history, offer help Offer MDT approach Pain killers Offer List of precipitating factors Rx - blood transfusions

Answer 304

The specific names of the eight types of porphyrias are: 1. Delta-aminolevulinate-dehydratase deficiency porphyria. 2. Acute intermittent porphyria. 3. Hereditary coproporphyria. 4. Variegate porphyria. 5. Congenital erythropoietic porphyria. 6. Porphyria cutanea tarda. 7. Hepatoerythropoietic porphyria. 8. Erythropoietic protoporphyria.

Answer 305

Repetitive use- Tennis elbow, Housemaids knee Other causes: Gout, Pseudogout Rheumatoid arthritis Uremia Hypertrophic pulmonary osteoarthropathy Infections: TB, Bacterial infections

Answer 306

Common causes: Hepatitis B, C Rubella arthralgia Rubella vaccine Parvo-virus B19 infection Rare causes: EBV infection HIV infection Mumps Caxsackie Chickengunya

Answer 307

can be due to enteritis, urethritis asymmetrical, mostly lower limbs, circinate balanitis Enteritis due to - Salmonella, Shigella, Yersinia, Campylobacter, Claustridium, Urethritis associated: Gonococcal Septic Joint Chlamydiae trachomatis

Answer 308

Psoriasis SLE Rheumatic fever Lyme disease Viral exanthems Drugs: Gold, Penicillamine, NSAIDs, Sulfasalazine Vasculitis: including rheumatoid vasculitis Raynaud's phenomenon Behcet's disease, orogenital ulcers Reactive Reiters syndrome: arthritis + Urethritis + uveitis

Answer 309

inflammatory arthritis: worse in the morning as in AS degenerative arthritis as in OA worsens throughout day Mono-OligoArthritis: Trauma Infection - Gonococcal/ Non gonococcal Crystal arthropathy: Gour, Pseudogout Seronegative arthritis Lyme disease Malignancy Polyarthritis causes: = Rheumatoid arthritis = Osteoarthritis = Viral infections = SLE, Vasculitis = Malignancy, Sarcoidosis = Seronegatives: AS, Reiter's, Psoriatic, Enteropathic

Answer 310

Past factures Maternal H/o Hip fracture Maternal H/o Osteoporosis Steroids Early menopause Oophorectomy Thyrotoxicosis Cigarrette smoking

Answer 311

Type 1 Polyglandular Autoimmune Rare, Auto-recessive, AIRE, chr21 = DM, asplenism = HypoPTH: Low Ca, cataracts, = Muco-cutaneous candidiasis + = Addison's disease + = Primary Hypogonadism in adolescence Type 2 Polyglandular Autoimmune: common, familial = Addison's +/- = Autoimmune thyroiditis = Type I DM (no hypo PTH, no immunodeficiency) Type 3: X linked PolyEndocrinopathy - neonatal - Autoimmunity over whelming - Neonatal Diabetes + - Loss of regulatory T cells - Immunodeficiencies - malabsorption

Answer 312

granuloma formation in multisystem cause unknown Clinical: Lupus pernia over face Uveitis attacks Lymphadenopathy, mainly mediastin Lungs: inspiratory velcro crackles chronic dry cough with wheeze Facial plethora Latgrens Triad: Bilateral Hilar Lymphadenopathy + Arthritis + erythema nodosum (shins) Hepatosplenomegaly Hypercalcemia High ACE enzyme levels TNF induced high fever with E nodosum Cardiomyopathy, Heart Blocks Renal damage: nephritis, Hypercalcemia related stones, Hypopituitarism Hypothyroidism Myositis, Polyarthritis, dactylitis Bone sclerosis with fractures risk Facial palsies infiltrative damage Rx: CST, Immunosuppression

Answer 313

Autosomal dominant TSC1/2 gene mutations, Chr 9/16 gene is next to ADPKD gene hence TS with ADPKD possible Clinical: Epilepsy- convulsions Renal cysts Shagreen patch over abdomen Periungual fibromas at nails Sebaceous adenoma over cheeks Ash leaf macules Lung cysts Renal failure needing HD, Transplants, Retinal phacoma's Rail track skull in xrays Tuberous masses may in brain MRI Hamartomas in heart, kidneys EPILOIA - Epilepsy + Low intelligence +adenoma sebaceous

Answer 314

activation of donor lymphocytes (allogenic) in hematopoetic stem cell transplant where these donor lymphocytes acts against recipient and damages gut, skin, Liver etc sometimes they can eliminate recipient tumor cells as well (useful)

Answer 315

reciprocal translocation between chromosome 9 and 22 to form shortened chromosome 22 called Philadelphia chromosome t(9,22) contains chimeric gene bcr/abl which is an oncogene. abl encodes tyrosine kinase. Mutation can be spontaneous or radiotherapy related. Chronic phase : > 5% blasts Accelerated Phase: rising blasts, 3-5 yrs Blast crisis: sudden rise of WBC's, blasts to 20-400k, High Vitamin B12 Diagnosis: Marrow study, bcl/abl mutation identification by FISH or RT-PCR Clinical: Wt loss, Fever, Night sweats (B symptoms). fatigue Splenomegaly Petechial rash, easy bruising pancytopenias, Herpes Zoster due to low immunity, recurrent other infections possible

Answer 316

> 60 yrs mostly Anemia, Bleeding bruising, Oral ulcers, Recurrent infections as sore throat, Hepato-splenomegaly, Monocytosis: GUm hypertrophy, PLeural effusions, Pericardial effusions, Arthritis, Skin lesions High vitamin B2 levels Blood Film: macrocytes, anisopoikilocytes, dimorphic pictures, Polychromasia, Basophillia, Normoblasts, Reticulocytopenia, Hypogranular neutrophils, Hypersegmented neutrophils, Mono or Bilobed neutrophils, Monocytosis, Promonocytes, Agranular platelets, Giant platelets Marrow Features: Erythroid hyperplasia Multi-nuclear rbc precursors Ring sideroblasts Hypogranularity of myeloid precursors increased Promonocytes Blast cells increased More abnormal megakaryocytes

Answer 317

causes: parvo-virus B19, many drugs, PNH associated, Non severe: = Neutro 0.5 to 1.5 = Platelets 20-100 = Retics: 20-60 Severe: Neutro 0.2 to 0.5 Retics < 20 Platelets < 20 Very Severe: Neutro < 0.2 Retics < 20 Platelets < 20 Hypocellular marrow Transfusion dependant Rx: Supportive as transfusions Anti-Thymosite Globulin + Ciclosporin Allogenic Stem cell transplants

Answer 318

Tumors of enterochromaffin cells sites: Stomach, pancreas, appendix, Duodenum etc Association: MEN 1 Secrets excess serotonin and other hormones Diagnosis: Clinical and tests Urinary High Hydroxy-indol-acetate levels High Vasoactive peptide (VIP) Somatostatin Receptor scintigrapy Clinical: = Flushed skin in attacks = Diarrhea episodes = Hepatomegaly = Wheezing chest: bronchospasm = Endocardial fibrosis, TR = Pulmonary valve stenosis = Pellagra as excess serotonin reduces tryptophan absoprtion False + diagnsis due to banana, avocado, pineapple, Plum, Walnut, Vanilla, Aubergine, Caffeine, Naproxen, Rx: Anti histamines, Loperamide, Niacin supplements, Octeotride, Tumor debulking surgery

Answer 319

Behcets disease: painful ulcers Crohn's disease, or UC HSV infections Lichen planus Syphilis Goncoccal infections HIV infection Pemophigus Stevens Johnson syndrome

Answer 320

no longer as childhood routine offered specifically to: = Healthcare workers, (Montoux -ve) = TB contacts < 35 yr(Montoux -ve) = New UK immigrants from endemics = Neonates from High incidence zones of UK or family members with active TB = Children, 1-16 yrs, Mountoux - ve, High risk setting or with chronic illness, immunocompromised

Answer 321

Constipation causes: = IBD, Ca colon = Hypothyroidism = Spinal cord issues = Diverticulitis = Hypercalcemia of malignancy/ sarcoidosis/ HyperPTH Diarrhoea causes: = Infective: Amoebiasis, Shigella, CD = Chronic pancreatitis = DM, Autonomic neuropathy = Hyperthyroidism = Systemic sclerosis: overgrowth = Autoimmunes: Coeliac, Grave's, Vitiligo Carcinoid associated, VIP secretion Diarrhea and Drugs: Laxatives Digoxin ACEi Antibiotics Thyroxine SSRI sTATINS Metformin long term use Magnesium Proton pump inhibitors

Answer 322

result of chimeric fusion protein due to fusion of PML gene on chr 15 and RARA gene on Chr 17 abnormal lymphocytes, low platelets APML cells contain pro-coagulant material and there lysis leads to hypercoagulable states and DIC Rx - All trans retinoic acid - to induce remission without other chemo and then post induction chemo is required to maintain remission - Arsenic trioxide, Idarubicin, Gemtuzumab, Hydroxyurea, Dexamethasone Keep: INR < 1.5, Platelets > 30k, fibrinogen > 100 mg/dl, avoid invasive procedures like CVP, LP etc

Answer 323

generalized painless lymphadenopathy, painful after taking alcohol, Histology: REED STEINBERG cells in lymphocytes (Owl eye appearance) Types: 1. Nodular sclerosis 2. Mixed cellularity 3. Lymphocyte depletes 4. Lymphocyte rich 5. Nodular Lymphocyte predominant B symptoms - Night sweats, Wt loss, Fevers Cotsworld staging: I - Lymph node involved II - 2 Lymph node areas on same side of diaphragm (axillary + Cervical) III - Lymph node areas involved on opposite sides of diaphragm IV - Extra nodal metastasis - infiltration as bone, liver etc Stage IA, IIa - short chemo, Radiation Stage IB, IIB,M IIIA - chemotherapy with ABVD or COPP OR BEACOPP

Answer 324

Demargnitation: a process where neutrophils adherant to endothelium slip off, commonly with steroid therapy and doubles the count in Lab tests (falsly high) Imatinib: oral inhibitor of bcl/abl tyrosine kinase inhibitor - for new diagnosis of CML Interferon alfa Allogenic bone marrow transplant from HLA identical donor Dasatinib, Nilotinib, new TK inhibitors Hydroxyurea Alkylating agents: chemo

Answer 325

Acute Leukemias: AML, ALL undifferentiated blasts in blood Marrow failure signs: pancytopenias Organ infiltrations signs: Lymphadenopathy, Splenomegaly, Hepatomegaly, Meningitis, Testicular infiltration, Skin infiltration, Gum hypertrophy Chronic Leukemia: due to mature cell origin, CLL, CML same signs as above can turn to blast crisis

Answer 326

B cell Lymphoma: > 85% of NHL, large cell, follicular B Lymphoblastic Lymphoma: Precursor cell involvement ie blast Peripheral Mature: CLL, B pro-lymphoblastic leukemia: Lymphoplasmacytic/ Waldenstroms macroglobulinemia Splenic marginal cell Lymphoma Hairy cell leukemia Plasma cell: Myeloma, Plasmacytoma, Heavy chain disease Mucosa associated Lymphoid tissue (MALT) - gastric MALT lymphoma Follicular: Mantle cell - diffuse large B cell Mediastinal large B cell Intra-vascular large B cell Burkitt's lymphoma

Answer 327

1. Mild UC: < 4 liquid stools/Day, little or no blood, no systemic symptoms 2. Moderate UC: 4-6 liquid stools/Day, moderate blood + systemic symptoms 3. Acute Severe UC: Profuse bloody stools> 6 day, rectal blood and mucus, urgency-cramps before motions, Suspect Complications if: Anorexia, Weight loss, Fever, Tachycardia, anemia, high WBC, High ESR Anatomical classification: Large Bowel UC Rectal Pancolitis Colitis to spleenic flexure

Answer 328

Topical Rx for distal UC/ proctitis: - Mesalazine suppositories - Budesonide enema, foams Systemic involvement: - Prednisolone 40 mg OD x 1 wk then reduce to 30 mg/wk, then reduce by 10 mg every 2 wks to 5 mg maintenance. - Oral Pentasa ie mesalazine 4 Gms PO OD + Night 1 gram per rectal for 1-2 months Maintenance: Mesalazine, Olsalazine or Sulfasalazine regular daily

Answer 329

Duration, control, Last HBA1C Any admissions for DKA/HHS Any admission for Hypoglycemia About insulin- types, regimens, dosing, who gives it, Side effects, Skin changes, recent changes DAFNE program: Dose Adjustment For Normal Eating About OHA - Current, Past, Side effects, Dosage Diet: consultation, Current diet, appetite Complications: 1. Macrovascular - MI, Stroke, CAD, PND, Ankle edema 2. Microvascular - Vision, Frothy urine, paraesthesia, Foot disease, BP, Lipid levels

Answer 330

Serum Prolactin ? raised TSH and T3 T4 LH and FSH Short Synacthen Diet Growth Hormone + Insulin like growth Factor + Glucose Tolerance Test

Answer 331

Activated Protein C resistance Protein C and S levels Factor V leiden mutation Antithrombin levels Anti cardiolipin antibody levels Lupus anticoagulant

Answer 332

- any tummy striae - any acne, hirsutism - Mood low - Easy bruising (thinning of skin) - difficulty getting up from chairs: Proximal weakness - Oligo-amenorrhoea - Hallucinations, delusions (Pshychosis) - Cataracts, Infections, Osteoporosis - Hypothalamic Disease: Headache, Vomiting, Polyuria, Polydipsia, Nocturia

Answer 333

? ask about Oligomenorrhea Low fertility (ask any children) Acne - may on Rx Hirsutism - may be treated Male pattern baldness in women

Answer 334

Fatigue more end of day, Droopy eye lids Triggers: Meds as steroids, pregnancy, infection Basic Exam: Hands, Pulse, BP, Eyes for ptosis, normal pupils, diplopia in all direction, Neck goiter, tenderness as association with autoimmune thyroid, any vitiligo, any alopecia areata Targeted: #Vision: acuity, count fingers #Eyes - horizontal and sustained upgaze, cant do it #Overshooting of upper eye lids- drops fast from upgaze #H shape movement check - diplopia in most directions #Blow cheeks - counting back from 20, cannot hold, voices changes to nasal #Neck sideways movements against resistance while 20 counted Shoulder shrug - count 20 backwards

Answer 335

Symmetrical hand joints deformities not correctable (as opposed to correctable in Jaccouds arthropathy of SLE) - swan neck, Z thumbs, Boutonniere, tender MCP, Bouchard nodes - Color changes in fingers on cold - Raynaud's (primary/secondary) Eyes -dry, redness, (Sjogrens) Skin - Malar rash, Sun exposure rash (SLE, APLS) Mouth - oral ulcers in Behcets Neck swelling - Goiter - ask weather preference, weight gain or loss, palpitations, diarrhea, staring look, Dysphagia - CREST Chest: dyspnea, basal ILD Heart - any chest pain, pericarditis etc Gut: tummy pain, bloody diarrhea, seronegatives with enteropathy or UC/Crohn's Kidneys: frothy urine, High BP Back: back pain, stiffness > 1Hr, improves with exercise in Ankylosis Posterior neck pain and stiffness, ask about any trauma also Lupus: Lumps,Blood clots, miscarriages, new drugs started

Answer 336

A - ask about smoking status A - assess readiness to quit A - advice to quit A - Assis in quit efforts: counselling, drugs, CBT A - arrange follow up

Answer 337

Flashing lights Zigzag lines Scintillating scotoma's : = Migraine, Retinal dettachments, Posterior vitreous dettachment, Occipital lobe SOL, Occipital epilepsy Halo's around light: = Cataracts, Glaucoma

Answer 338

SAJID-2W-2H-6P's-STD SAJID: Smoking-Alcohol-Job-Impact of illness on life-Driving 2W = who lives with, who is carer 2H = Housing, Hobbies 6P = Pregnancy=Pill OC=Periods=Past abortions=Past children with ages=Past Psychiatric history STD: = S = Sex: Any troubles, intimate relations T = Travel: any recent travels? -when where, How, stay, activities, street food, water type, sick contacts, injections, illicit drugs, swimming in lakes, camping etc D = Diet details: probe if suggestive for DM, Gout, Coeliac, Special meal plans, allergies

Answer 339

Clinical hyperuricemia is Gout = Deficiency of Hypoxanthine:guanine phosphoribosyltransferase can be+ 1. Podagra: 1st MTP Joint affected 2. Asymmetrical deforming swelling of small joints of Hand 3. Tophi - collection of urate, nodules @ forearms on extensor side, fingers, feet, ear helix, achilles tendon 4. Olecranon bursa inflammation Uncommon sites for tophi: Carpal tunnel, Cardiac Conduction system causing blocks, Valves causing VHD Pre-disposing conditions/ Factors: CKD, Recent chemo-radiation, Steroids, Thiazides, Aspirin use, PseudoGout: Hypothyroid, HyperPTH, Haemochromatosis

Answer 340

Painful Thyroiditis: = Subacute granulomatous thyroiditis: initial hyperthyroid phase as pre formed hormone released for 2-6 weeks followed by hypothyroidism (DeQurvains thyroiditis) = Infectious Thyroidis: can be due to Gram +/- bacteria, fungal, viral or mycobacterial = Radiation thyroiditis Painless Thyroiditis: = Autoimmune Hashimotos thyroiditis - initially transient hyperthyroid and then permanent hypothyroid, myeloperoxidase + = PostPartum thyroiditis: usually 3 months after delivery, initial Hyper then hypothyroid, Start Thyroxine at 6 months and then STOP at 12 months and monitor

Answer 341

PFAM: papillary-follicular-anaplastic-medullary 1. Papillary: most common, may be part of FAP or Gardner syndrome, slow growth, origin from follicular cells-papillae or finger like projections in histopath 2. Follicular: 2nd most common, capsular or vascular invasion seperates it from adenoma-treat with surgery or radioiodine 3. Anaplastic: aggressive, undifferentiated, anaplasia, to form backward to become undifferentiated 4. Medullary: neuroendocrine tumors of parafollicular or C cell which produce calcitonin, part of MEN2 syndrome

Answer 342

Dyspnea Dysphagia Thoracic outlet syndrome (pemberton test) Horner's syndrome - sympathetic chain destruction Recurrent Laryngeal nerve palsy, change in voice Jugular vein compression-thrombosis Cerebro-vascular Steal syndrome

Answer 343

Increased Hormone production: Graves disease Toxic adenoma nodule Toxic Multinodular Goitre Iodine induced effect: contrast media, Amiodarone TSH secreting pituitary tumor Trophoblastic tumor -beta HCG mimics TSH action Germ cell Tumor - high Beta HCG mimics TSH action Increased Hormone release so temporary Hyper: DeQuervains thyroiditis Infections affecting thyroid Radiation affecting thyroid Post partum thyroiditis (after 3-upto 12 months) Drugs: Lithium, Amiodarone, IFN-gamma

Answer 344

Primary Thyroid Failure: - Autoimmune Hashimoto's thyroditis - Idiopathic atrophy of gland - Post radio-iodine therapy - Thyroidectomy total - Anti thyroid drugs: PTU, Carbimazole - Iodine deficiency - Infiltrations: Sarcoid, Sclerosis, Reidel's thyroiditis - Post Partum/Subacute/Painless - Infections Secondary Thyroid Failure: Pituitary or Hypothalamic diseases

Answer 345

serous effusions- pleural, pericardial Bradycardia Cerebellar disease with signs Heart failure High cholesterol, High LDL levels Depression, Psychosis Anemia normocytic Normochromic Low Vitamin B12, pernicious type effect Other autoimmunes present: Addisons Treat: Levothyroxine, if IHD present: start low dose, 25 mcg/Day if IHD not present: start 50-100 mcg/Day and then titrate upto 150 mcg max

Answer 346

Thyroid Binding Globulin (TBG) alteration affects T3, T4 and total Levels. High TBG = estrogen, OC pills, Pregnancy, Liver diseas Low TBG = Protein loss causes If TSH low - T4 normal - check Free T3 Free T3 toxicosis with normal FT4 occurs in early hyperthyroidism of any cause Isolated T4 toxicosis: iodine excess of any cause Sick Euthyroid: means altered TFT due to non thyroid illness but gland is normal. Normal T4, Low T3, normal TSH. TSH receptor antibodies can cross placenta & affect fetus.

Answer 347

Clinical effects of acute Hyperthyroidism: - Tachycardia, possible rapid atrial fibrillation - Restlessness, febrile > 40c - Hypertension, resistant - Altered sensorium - diarrhea, vomiting, MODS - Very low TSH and very High T3T4 Treatment of Thyroid storm: ICU Propyl thiouracil 600 mg stat then 200 mg 6 hrly Lugol's iodine - after 1 Hour, 8 mg Iodine per drop - give 10 drops every 8 Hrly ( 80 mg TID) Propranolol 60 mg, 4 Hrly Dexamethasone IV IV Fluids and temperature control Apply Burch-Wartofsky Point scale Labs: low TSH, High T3T4, Hyperglycemia, Deranged LFT, High LDH, Low calcium, High WBC count

Answer 348

Titration Regimen: Carbimazole, 30-60 mg daily TID in divided - reduce over 4-8 weeks, check Free TH normalization. Block and Replace Regimen: Carbimazole 40 mg daily, no tapering at 4 weeks - check Free T4: if low or normal - add Levothyroxine to regimen (both to give)

Answer 349

Multi-nodular Goitre: irodine deficiency Physiological Graves disease Hashimoto's thyroiditis DeQuervains thyroiditis Goitrogens Dyshormonogenesis Solitary Goitre: Thyroid adenoma Toxic adenoma (Plummer's) Cysts in thyroid Single nodule within Goitre

Answer 350

Retro sternal Goiter Thymoma Lymphoma Germ cell tumors -Teratoma, Seminoma Parathyroid masses Mediastinal cysts: Pericardial, Bronchogenic

Answer 351

Thyroglossal cyst (a branchial anomaly) Lymphadenopathy (bacterial, Viral, TB, granuloma) Vascular: Carotid aneurysm Carotid body tumor Jugular venous thrombosis Jugular haemangioma Sialoadenitis Salivary tumors Neurogenic: Schwannoma's, Neurofibroma

Answer 352

Central obesity ie waist circumference > 102 cm for men, > 88 cm for women ++ Hypertension new or on treatment > 130/85 ++ Raised Triglycerides > 150 or on treatment, ++ Low HDL cholecsterol < 1 mmols ++ Raised fasting glucose > 5.6 mmols or DM on Rx

Answer 353

Two types of NAFLD are nonalcoholic fatty liver (NAFL) and nonalcoholic steatohepatitis (NASH). People typically develop one type of NAFLD or the other, although sometimes people with one form are later diagnosed with the other form of NAFLD. Accordingly, in the NASH CRN system, Liver fibrosis stage 0 = no fibrosis; stage 1 = centrilobular pericellular fibrosis (or periportal fibrosis in children); stage 2 = centrilobular and periportal fibrosis; stage 3 = bridging fibrosis; and stage 4 = cirrhosis The first line of management in NASH involves lifestyle modifications, mainly sustained weight loss (through a calorie-restricted diet) and increased physical activity (exercise). Liver biopsy is the only test that can prove a diagnosis of NASH and show clearly how severe the disease is. Liver biopsy can show fibrosis at earlier stages than elastography can.

Answer 354

# Age < 60 = 0, Age 60-79 = 01, Age > 80 = 02 predicts mortality in patients with upper GI bleeding - calculated as Pre and Post Endoscopy score, both added for final score. Pre endoscopy score of 0 identifies very low risk of death 0.2% and risk of rebleeding 0.2% Pre Endoscopy Score: # Evidence of shock: none = 0 Pulse >100, SBP > 100 = 01 SBP < 100 = 02 # Co-morbidities: none = 01 CCF, IHD, Other major = 02 Renal/ Liver failure, metastatic Ca - 03 Post Endoscopy Score: # Endoscopic stigmata: none = 0 Blood in upper GI tract /clot/ visible bleeding = 02 # Endoscopic Diagnosis: Mallory weis Tear = 0 no stigmata of recent bleed no lesions seen All Other diagnosis = 01 Malignancy of UGI tract = 02

Answer 355

predicts need for intervention parameters considered are as: Blood Urea Haemoglobin Levels - Men, Women Systolic Blood pressure Other Markers: = Pulse > 100 = Present with melaena = Presents with Syncope = Hepatic disease = Cardiac disease

Answer 356

F = Feedback to give regarding risk & level of alcohol intake R = Responsibility, she has to change A = Advice, clear advice when requested M = Menu - explain Options E = Empathy approach S = Self efficacy: optimism about behavior change

Answer 357

Clinical - mild iron deficiency anemia, frequent bloating, diarrhea, lethargy, mouth ulcers, dermatitis herpetiformis, Osteoporotic fractures, anemia symptoms, angular cheilosis, Steatorrhea Labs- Tissue tranglutaminase antibodies, IgA difeiciency in 10% coeliacs, FBC, UE, LFT, CRP, ESR, Iron profile, B12, Folate levels HLA DQ2 association in 95% OGD and duodenal biopsy while on Gluten diet for 6 weeks even if TTG negative

Answer 358

Coeliac disease Giardiasis Bacterial overgrowth NSAID overuse Tropical sprue Whipples disease Lymphoma, MALT Hypogammaglobulinemia

Answer 359

Dermatitis herpetiformis Diabete mellitus Autoimmune - thyroid/adrenals Irritable bowel syndrome SLE Psoriasis LFT elevations Complications: Anemia, Vitamin deficiencies Osteoporosis due to malabsorbtion Splenic atrophy GI Lymphomas Ulcerative jejunitis Esophageal cancer Small bowel adenocarcinoma

Answer 360

= Diabetic mauclopathy = Vitreous haemorrhage = Branch or Retinal artery occlusion = Branch or Retinal vein occlusion = Stroke/ Hemianopia = Rubeosis ie neovascularization = Cataracts = Anterior ischemic optic neuropathy = Ocular ischaemic syndrome

Answer 361

1. Background Diabetic Retinopathy: 2. Pre-prolieferative: 3. Proliferative - needs panretinal photocoagulation 4. Diabetic Maculopathy- central vision loss: Rx with Intravitrela lasers or Anti VEGF agents as Lusentis, Eylea 5. Rubeosis without neovascular glaucoma: Pan retinal photocoagulation (PRP) and close monitoring, Need IOP monitoring and treatment

Answer 362

A group of inherited metabolic diseases caused by the absence or malfunctioning of certain enzymes needed to break down glycosaminoglycans, which are long chains of sugars. Clinical: Short stature, Intelectual disability, Coarse facial features, Hepato-splenomegaly, Corneal clouding, Joint contractures, Leucocyte inclusions, Dysostosis multiplex, multi system effects Types: Hurler syndrome (MPS I-H), Hurler-Scheie syndrome (MPS I-H/S), and Scheie syndrome (MPS I-S), listed from most to least severe. Diagnosis: = Excess excretion of mucopolysaccharides in the urine = Enzyme assays

Answer 363

Abetalipoproteinemia (ABL) is a rare autosomal recessive disorder marked by low or absent levels of plasma cholesterol, low-density lipoproteins (LDLs), and very-low-density lipoproteins (VLDLs). It should not be confused with a deficiency in beta-lipoproteins. = caused by mutations in the MTTP gene, which provides instructions for making a protein called microsomal triglyceride transfer protein. This protein is essential for creating molecules called beta-lipoproteins in the liver and intestine . = pale, bulky foul-smelling stools (steatorrhea), diarrhea, vomiting and swelling (distension) of the abdomen. Affected infants often fail to gain weight and grow at the expected rate (failure to thrive). These symptoms result from poor absorption of fat from the diet. = Night blindness develops, with retinal degeneration and later onset of pigmentary changes secondary to vitamin A deficiency. White dots are present throughout the fundus and are most numerous in the periphery. A retinitis pigmentosa-like picture eventually ensues = high doses of fat-soluble vitamins A, E, K. Vitamin E, and Vitamin A can be used to treat retinal and neurological complications. Bone growth problems are treated using Vitamin D medications. Fat-soluble vitamins blood levels are measured regularly.

Answer 364

Diffuse thyroid enlargement causes: 1. Smooth and Euthyroid Goiter: Iodine deficiency Puberty - physiological Viral Thyroiditis Postpartum thyroiditis Goitrogens: Kelp, Seaweed, Amiodarone Hashimoto's thyroiditis (autoimm.) 2. Smooth & Hyperthyroid/Toxic: Grave's disease - palpitations, may be Afib - sweaty palms - Weight loss with good appetite - Anxious, irritable, - Lid lag, thyroid bruit

Answer 365

SOB, wheeze if trachea < 8mm Cough, choking sensations Hoarse voice if RCN compressed Horner's syndrome SVC obstruction - do pemberton Subclavian steal symptoms Dysphagia less common as esophagus is posterior near spine

Answer 366

due to long standing neuropathy - Diabetes mellitus - Syringomyelia - tabes dorsalis + ARPupil - Paraplegia - Leprosy - Myelomeningocele - Hereditary sensory neuropathies - Congenital insensitivity to pain Work up: FBC, UEG, CRP, ESR, LFT, TFT, B12, Folate, HBA1C, ANA screen, ANCA, Protein electrophoresis, Joint Xrays, NCV studies, VDRL/TPHA, Urine proteins, A:C ratio,

Answer 367

Clubfoot most often presents at birth. Clubfoot is caused by a shortened Achilles tendon, which causes the foot to turn in and under. Clubfoot is twice as common in boys. Treatment is necessary to correct clubfoot and is usually done in two phases — casting and bracing. During an examination of the foot, there are distinctive deformities associated with clubfoot. These include midfoot cavus, forefoot adductus, hindfoot varus as well as hindfoot equinus. These deformities may vary relative to the extent of deformity and their stiffness. The most common congenital deformity of feet is TEV; however, there are other forms of clubfoot, specifically talipes calcaneovalgus (in which the ankle joint is dorsiflexed and the forefoot deviated outwards), and talipes calcaneovarus (in which the ankle joint is dorsiflexed and the forefoot deviated inwards)

Answer 368

Headaches Visual defects - bitemporal anopia Increased - Hand, shoe, ring size Coarse facial features Glucose intolerance, DM develops Other associated pituitary hormone features: amenorrhea, Libido down, sweating palms, Proximal myopathy Fatigue, mood changes, CTS Clinical Signs: Big spade like Hands, Sugar pricks Bounding pulses, Prognathism - low jaw protrudes Interdental separation - Gaps Coarse thick upper eye ridges Enlarged Lips, tongue, Nose Bitemporal Hemianpopia Carpal Tunnel Signs - Tinels+ Hearing Aids Hirsutism in females Acanthosis nigricans skin tags, increased Proximal weakness features

Answer 369

Growth Hormone random level Insulin Like Growth factor-1 levels Post Oral Glucose TT, 75 Gms - 0, 30, 60, 90, 120, 150 min checks for GH levels - normally drops < 1 ng/ml - paradoxically rises in Acromegaly MRI pituitary Pituitary screen: TSH, TFT, LH, FSH, Prolactin, ACTH, Morning Cortisol etc ECHO, ECG, HBA1C, Other Basic Labs Rx: Cabergoline, Octreotide, Trans-sphenoidal resection of pituitary adenoma, Post Sx OGGT Treat DM, Cardiomyopathy, Treat Arrhythmias Stop Driving If no Pituitary tumor found at MRI - look for ectopic GH producing tumors of Pancreas, Adrenal, Lungs, Ovaries Post Surgical - CSF leak, SIADH, Strokes, Hypopituitarism

Answer 370

Associated conditions include type 2 diabetes, obesity, obstructive sleep apnea, heart disease, mood disorders, and endometrial cancer. Signs and symptoms of PCOS include irregular or no menstrual periods, heavy periods, excess body and facial hair, acne, pelvic pain, difficulty getting pregnant, and patches of thick, darker, velvety skin, ovarian cysts, enlarged ovaries, excess androgen, weight gain and hirsutism

Answer 371

1. Hypothyroidism: Cold intolerance, hoarse voice, hirsutism, menorrhagia, weight gain, proximal myopathy 2. Hypercalcemia: stone colics, depression, nausea, vomiting, thirst 3. Diabetes: polyuria, polydipsia, wt loss, polyphagia, autonomics 4. Parkinson's: bradykineia, rigitidty, tremor.. 5. Spinal cord compression or demyelination - urinary incontinence, Sensory/Motor leg neuropathy, Sphincter disturbances, peri anal or perineal numbness 6. Medications causing GI upsets: TCA's, Anticholinergics, Anticonvulsants, CCBs, Diuretics, Iron supplements, Immodium, NSAIDs, Opiods

Answer 372

Moderate Risk cases: < 50 yr old = no screening > 50 yr olds: = Annual fecal occult blood = 5 yrly flexible sigmoidoscopy = 10 yrly colonoscopy For High Risk cases: family H/o first or second degree relative with CRC or adenomatous polyposis in a relative < 60 yr old: = annual fecal OB = 5 yrly flexible sigmoidoscopy = 10 yrly colonoscopy starting at 40yr Familial adenomatous polyposis: = genetic mutation check@ 12 yr age = Yrly flexible sigmoidoscopy = Polyps noted - full colonoscopy = Colectomy = OGD surveillance Hereditary non-Polyposis CRC: = Colonoscopy at 20-25 yr old = Test 1st degrees Peutz-Jegher Syndrome: = 2 yrly OGD+Colonoscopy + barium follow through = watch for breast, Gonadal, Testicular cancers Past H/o and surveillance: Polyps: 3 yrs scopy CRC - 3-6 months after surgery then 1 yrly and then 3-5 yrly scopies Acromegaly: 3 yrly scopy from age 40 IBD:

Answer 373

Peripheral arthropathy Erythema nodosum Episcleritis, Anterior Uveitis Mouth Ulcers Pyoderma gangrenosum other unrelated to colitis but may be associated with IBD: - Ankylosing spondylitis - Sacro-ileitis - Primary sclerosing cholangitis

Answer 374

Smoking Alcohol Job Impact on Job and Life Driving or not Housing - Hobbies Who is carer, Lives with Who 6P: Pill use, Pregnancy, Post partum, Past abortions, Periods, Psychiatric Mood & history Sexual details Travel history Diet details

Answer 375

History to elicit: End of day fatigue, droopy eye lids, diplopia in all directions Triggers: Stress, Medications, Steroid use, Pregnancy, Infections (ask) Basic Examination:Hands, Pulse, BP, Eyes, Oral cavity, Neck swelling Targetted Examination: 1.Visual acuity, single eye finger counting or snellen chart 2. Eye Movements - ask diplopia - all directions = then check pupils (normal) = then sustained upgaze while counting 20 (cant hold, eyelids starts drooping) = overshooting of upper eye lid 3. Blow cheeks, hold while counting back from 20- cant hold and voice changes to nasal quality 4. Sideways neck movements against resistance - while counting 20 5. Shoulder shrugg - count back from 20

Answer 376

if > 6 bloody stools/day, crp > 45 check TMPT Level for azathioprine Rx Rectal & IV Hydrocortisone IV fluids and K monitoring Thromboprophylaxis, LMWH Rescue therapy: Ciclosporin, Infliximab, Azathioprine 2 Grams Avoid NSAIDs Avoid Loperamide, Opiods Avoid Buscopan use Colectomy if toxic megacolon>24 hrs

Answer 377

Triad of: 1. chronic watery diarrhea + 2. Normal colonoscopy + 3. Microscopic inflammation of lamina also includes: Collagenous colitis, Lymphangitis colitis Rx: Budesonide, Azathioprine

Answer 378

starts above 40 yrs Chromosome 6p, C282Y mutation family H/o DM, CLD, Arthropathy multisystem effects 1. Diabetes mellitus symptoms 2. Liver cirrhosis, CLD develops 3. HCC can develop as well 4. Pituitary failure signs - loss of libido, smooth skin 5. Arthopathy, PseudoGout of wrists, Hips, Knees 6. Cardiomyopathy develops - heart failure, arrhythmias Chest Ferritin, Iron levels

Answer 379

inflammatory bowel disease Ischemic colitis Microscopic colitis Radiation colitis Drug induced colitis-NSAIDs, Antibiotics Rectal mucosal prolapse, Ca colon

Answer 380

-Renal glycosuria, genetic -Aminoaciduria: Hartnup disease, Cystinuria, Homocystinuria -Cystinosis -Barter syndrome: Loop diuretic like -Gitelman syndrome: Thiazide like -Nephrogenic Diabete insipedus -Proximal RTA - Liddle syndrome - Glucocorticoid remediable Hyperaldosteronism - Pseudo Hypo Aldosteronism - HypoPhosphotemic rickets - X linked Hypocalciuric Nephrolithiasis

Answer 381

Focal, segmental glomerulosclerosis Diabete mellitus CKD IgA nephropathy Hemolytic Uremic syndrome-TTP Anti GBM disease Oxalosis

Answer 382

appears 4-5th decade with genetic anticipation phenomenon, PKD1 with chr 16p & PKD2. PKD1 has association with tuberous sclerosis, Marfans syndrome and familial intracranial aneurysms as genes lie close to each other. Renal features: gradually appearing large hundreds of renal cysts with loss of function, hematuria, and CKD effects, HTN, anemia, UTI, stones, masses Extra Renal features: ICH with bleeding risk Liver, Pancreatic, Ovarian cysts Renal, Extra renal cysts can bleed Mitral valve prolapse Anemia of CKD Polycythemia due to High EPO levels rare

Answer 383

PreHepatic: Hemolysis - Anemic, High retics, High LDH (in Sicklers, G6PD deficiency, Malaria) Hepato-cellular: Gilbert's syndrome or Criggler Najjar Chronic Liver disease Acute Hepatitis Post-Hepatic: Obstructive causes - GB stone, Ca pancreas, Lymphadenopathy Drug induced cholestasis

Answer 384

Gilbert syndrome: UDPG transferase low levels causes defective conjugation leading to indirect hyperbilirubinemia, AutoDom Criggler-Najjar syndrome: absent UDPGT - Autosomal recessive, present as kernicterus in new born Dubin Johnson syndrome: defect in transporter out of liver cell - bilirubin is conjugated but remains in liver cells. Green black liver at Biopsy, AutoRecessive.

Answer 385

Major criteria: - Hypergamma globulinemia, IgG - ANA , Anti SMA + - Soluble Liver antigen antibody + - Liver-Kidney antigen antibody + - absent Viral Hepatitis markers - Portal Hepatitis on Biopsy Minor Criteria: H/o other autoimmunes H/o family members with autoimmun Arthralgia HLA DR3, DR4 Spontaneous fluctuating course

Answer 386

if HBeAG ++ 1.interferon alfa or 2. Nucleoside analogue-Lamivudine or 3. Nucleotide analogue - Adepovir diporoxil if HBeAG -- HBV DNA > 10 x4 = INF alfa OR Nuceloside analogues Nucleotide analogues

Answer 387

RNA virus, 6 genotypes Spread: Sex, needles, transfusions, sharing toothbrush/ razors/ tattoos/ acupuncture/ needle stick injuries 1:30 chance Anti HCV antibody ++ after 6 months Treatment criteria: # Age > 18 yrs, # persistently elevated ALT, AST, # compensated CLD ie normal Bilirubin/Albumin/PT, aPTT/Platelets/ no encephalopathy, no varices, no ascites # Liver Biopsy shows: chronic inflammation with portal fibrosis # No malignancy or other chronic issues

Answer 388

Pegylated IFN for 24 weeks + Ribavirin/ Entecavir

Answer 389

Primary prevention of bleeding: - Elective banding + PPI + BB's - B1 block reduced cardiac output - B2 block causes splanchnic vasoconstriction Acute Variceal Bleed Rx: - Teripressin 1 mg iv - Fluid, PRBC, FFP's - Vitamin K 10 mg iv - Balloon tamponade within 12 Hrs - OGD ligation or sclerotherapy - IV Ceftriaxone cover - Stop Alcohol intake = TIPS therapy

Answer 390

1. Secretory Diarrhea: - Large volume, doesnt improve with fasting - VIPoma 2. Osmotic Diarrhea: - Moderate volume, improves with fasting 3. Inflammatory diarrhea: - bloody, Painful 4. Steatorrhea: - Hard to flush, pale, malabsorption, Coeliac disease, Sprue, Pancreatitis 5. Proctitis: - rectal inflammation, urgency, might be blood stained 6. Nocturnal Diarrhea: waking up to pass stools - IBD or Diabetic autonomic neuropathy 7. Overfloe diarrhea: - Fecal impaction and overflow diarrhea (initial constipation) 8. Other: Bile acid malabsorption causes diarrhea as after cholecystectomy, ileus, post vagotomy etc

Answer 391

Hematuria in < 45 yrs: 1. Pulmonary Renal syndromes: Goodpasture's, Wegners, Microscopic polyangitis 2. Glomerulonephritis: IgA nephropathy, Post infectious GN, Alport syndrome, Sickle cell nephropathy 3. Vasculitis: PAN, HSP 4. Connective Tissue disorders: Lupus nephritis, Systemic Sclerosis Hematuria in > 45 yrs: 1. Malignancies: RCC, TCC bladder, Ca prostate 2. Benign: BPH 3. Infectious: Prostatitis Hematuria in all age groups: 1. Renal stones 2. Infections: pyelonephritis, IE, Cystitis, TB 3. Drugs: Anticoagulation, Cyclophosphamide, 4. Other causes: Traumatic, Coagulopathy, Chemo-radiation

Answer 392

- death within 2 weeks if no Rx - any GN but mostly due to SLE, Vasculitis or Goodpasture syndrome - Hematuria+Proteinuria+Rapid ESRD - Histologically: Glomerular sclerosis, Crescent rbc casts, IG deposits Treatment: High Dose steroids, + Cyclophosphamide or MMF

Answer 393

Renal tubular acidosis (RTA) occurs when the kidneys do not remove acids from the blood into the urine as they should as in CKD Type 1 RTA: distal tubular defect, causes HYPOKALEMIA, Autosomal recessive or dominant, Bone loss, Metabolic acidosis, Bone pain, renal stones Causes: Autoimmunes, SLE, Hypergammaglobulinemia, Nephrocalcinosis, NSAID induced Type II RTA: proximal tubular defect Loss of HCO3, alkaline Urine Causes: Amyloidosis, Cystinosis, Wilson's disease Fanconi Syndrome: cannot absorb PO4 + Glucose + Amino acid - cystin accumulates. Type IV RTA: caused by either decreased aldosterone production or resistance. Primary adrenal insufficiency results in decreased aldosterone whereas spironolactone can cause aldosterone resistance. Hyperkalemia as K not excreted - addisons disease - Aldactone treatment - ACEi - SLE, DM nephropathy

Answer 394

- unilateral headche + aura + fortification spectra ie zigzag flashing lines in field of vision arranged as a fort wall - Scintillating scotoma: area of scotoma with bright edges - Positive sensory symptoms - Triggers: Red wine, cheese, chocolate - Can have transient hemiplegic weakness in Hemiplegic type - lasts 12-24 Hrs, maximum upto 72 - Photophobia, Phonophobia, Osmophobia Preventive Rx: 1. Propranolol, Atenolol 2. Amitryptiline/ Topiramate/ Valproate if > 3 attacks per month Acute Attack Rx: Paracetamol, NSAIDs, + Anti emetics + Sumatriptan SQ or Ergot derivatives

Answer 395

all should be inquired if 1 present 1. Hypo-adrenalism: - Hunger pangs due to sugar low - Postural dizziness - Nausea, vomiting - Hyperpigementation 2. Hypo-gonadism: - impotence, infertility or low fertility - reduced lipido - smooth skin, smooth voice, less bald 3. Hypo-thyroidism: - hot preference - Hoarse voice - hard stools - menorrhagia - low mood, slowness of thought

Answer 396

1.Addisons disease: hypoglycemia, hyperpigmentation, postural dizziness, nausea, vomiting 2. Acute intermittent porphyria: Tummy pain, nausea, Seizures, Psychosis, AutoDom, females 3. Angioedema, C1 INH deficiency 4. Colics: Renal, Biliary, Colitis, GE 5. Pan vasculitis: Hep B association, as or as part of Systemic vasculitis 6. Familial Mediterranean fever: Autosomal recessive, family H/o negative Laparotomies, as: Fever + Serositis (Pleuritis, Peritonitis, Pericarditis) + Pain abdomen

Answer 397

Duration of diagnosis Controlled or not Last HBA1C or blood pressure Medication List, compliance, SE, any recent change, Disease complications: - Vision - Urine: frothy, facial or leg swelling - Sensations: burning, pins needles, - Any stroe, STEMI, TIA, Caludications - Any active chest pain

Answer 398

Onset Duration Intensity, score 0-10 Progression - better, worse over time Aggrevating factors Relieving factors Anything else

Answer 399

I am sorry to tell you that the condition you have been suffering is part of XXX or called XXX in medical language. Please do not bother with the term. We will involve a group of specialists comprising of my consultant, specialist nurse, nerve doctor, woman dr, gland dr etc. I will examine you now if you agree, draw some blood and probably will arrange for imaging. Also I will discuss it with my consultant. We will again meet next week (give specific day, time etc) if it suits you to discuss results of those tests and further plan. How does that sound? Do you have any concern or questions for me now?

Answer 400

Clinical: any Weight loss if yes: How much and over how long duration? intentional or not? How is your appetite? Any pallor, any one said that.. Any GI Bleeding, black stools Any lumps or bumps in body Any yellowishness of eyes Family History to ask: Any diseases running in family? Any family history of similar conditions? Any other family history of note?

Answer 401

in MEN (with Low LH+FSH): reduced body hairs, reduced libido, impotence in WOMEN (low LH+FSH): oligomenorrhea, reduced libido, infertility, Hot flushes Secondary Hypothyroidism: menorrhagia, Hard stools, Hoarse voice, dry sin, Weight gain ACTH deficiency: Nausea, emesis, Weight loss, postural dizziness, no hyperpigementation as in addisons Hypothalamic signs: galactorrhoea, weight gain or weight loss depending on center affected as satiety affected Pituitary infiltration due to mets from lung, breast or due to sarcoidosis, tuberculosis, langherhans cell histiocytosis should be kept in mind

Answer 402

Sarcoidosis is an inflammatory disease in which the immune system overreacts, causing clusters of inflamed tissue called "granulomas" to form in different organs of the body. Sarcoidosis most commonly affects the lungs and lymph nodes, but it can also affect the eyes, skin, heart and nervous system. A well-developed sarcoid granuloma consists of a tightly formed conglomerate of epithelioid- and multinucleated-giant cells (MGCs) encircled by lymphocytes, especially CD4+ T helper (Th) cells, but also rare CD8+ T cells and B cells

Answer 403

rule out RED FLAGS ie obstructive symptoms first: as swallowing difficulty, speech difficulty or change in voice, breathlessness, noisy breathing ie stridor Is it thyroid? ask to swallow water and watch, moves with swallowing. if it is - check Hypo or Hyper thyroid: - weather preference - hard stools or diarrhea - weight gain or loss - eye changes, staring look - fatigue slow or hyperactive restless - menstruation: Hyper/ hypo/ normal - wrist, finger thickening think of MEN syndrome association think of thyroglossal cyst if not thyroid - elevated when tongue protruded

Answer 404

SOCRATES: Site Onset Character Radiation Association Timing Exacerbating Factors Severity: 0-10 scale

Answer 405

OPQRST-AAA Onset Provoked by Quality of pain (intensity) Region, Radiation Severity, 0-10 scale Time, any specific, duration Aggrevating & alleviating factors Associations Adapatations

Answer 406

How to deal with person with pain Sit forward Open questions: tell me about it Lean slightly Eye contact to maintain throughout Reach out - offer help (analgesia)

Answer 407

= Exposure: date, place of diagnosis, = Route of exposure: sex, needles, blood transfusion, surgery etc = Lowest CD4 count if known = Highest Viral load if known = Any drug resistance documented? = Any associated illnesses requiring admissions? = Current & previous HAART regimens? = Any previous AIDS defining illnesses? = Any adverse reactions to drugs? Current HIV Physician or unit?

Answer 408

Site of rash and distribution onset and duration itch or tenderness bleeding or discharge mucosal involvement - oral ulcers, diarrhea Associated: - Lip or tongue swelling - Urticaria - SOB, Wheeze

Answer 409

Intentional or not Exercise: intensity How much weight loss? How long over weight loss? How is the appetite? Level of exercise, moderate, intense? Pattern of diet, High calorie? Distribution of weight-central, peripheral, overall. Clothe size change? Any ring side, shoe side change? Any change in facial appearance

Answer 410

- Show me your medication List - Any OTC medications use, names, duration, doses, SEs - Any HERBAL medication use, names, duration, doses, SE, from where - Any recreational drug use, name, frequency, routes, sharing needles, admissions due to SE, rehabilitation attempts etc

Answer 411

Last contact Gender of partner Casual or long term Any other contacts in last 3 months? Country of partners Any known diseases with partner? Type of sex: Anal, oral, vaginal? Use of barriers, any breach? Ever paid of sex or got paid? Any past tests for HIV, Hepatitis etc Hepatitis B Vaccination status, boosters? Other questions: Tattoos, Travels and any sex there with protection or not? Any transfusions in the past and when, where, why? Any blood donations? any drug abuse? financial support?

Answer 412

Acromegaly: Headaches, change in appearance to coarse, Visual field defects, H/o bumping into things, parking issues ring + shoe + clothes getting tighter Cushings Syndrome: steroid use history, sin striae, purpura, central obesity, Moon face, Buffalo hump, cant get up from chair ie proximal weakness, infections, ulcers, Conn's syndrome: Hyperaldosteronism, weakness, Lethargy, Palpitations, Muscle cramps, constipation due to hypokalemia, High BP with headache Phaechromocytoma: high epinephrine and metaboilites, palpitations, sweating, flushing, wt loss

Answer 413

Morning - OSA, day time sleepy Evening fatigue - MG, diplopia Throughout Fatigue: with weight loss/ weight gain. with Wt Loss - appetite poor or good with Wt gain - distribution, intentional, diet etc

Answer 414

Are you in a long term relationship? Do you practice safe sex, barrier? Do you have single or multiple partners, male or female? Protected safe sex with each or not? Country of partner, any diseases known? Have you ever paid for sex or got paid? any travels and sexual contacts there? Past diseases?

Answer 415

- Familial tall stature - Klinefelter syndrome - Marfan's syndrome - Homocysteineuria - Precocious puberty - MEN 2b syndrome - Growth hormone Gigantism - Hyperthyroidism

Answer 416

47XXY, male with gynecomastia, small testicles, learning difficulties

Answer 417

Autosomal dominant (fibrillin gene) arm span>Legs, pectus excavatum, high arch palate, spidery long fingers, blue lens, upward Lens dislocation, Aortic dissection or aneurysm, Joint dislocations, Flat feet, crowded teeth

Answer 418

Cystathione betasynthetase deficiency, Autosomal Recessive High homocysteine levels in blood/urine AutoRec, tall , marfanoid habitus, Long limbs, pes cavus, Genu vulgum, pectus excavatus or carinatum, downward Lens dislocation, nearsightedness, Osteoporosis, Glaucoma, VOC pain attacks, Rx - Vit B6 supplementation

Answer 419

85% - Cx lymphadenitis Thyroid swelling: - 8% = Goiter: Grave's, Multinodular, Hashimoto's, Subacute = Solitary nodule = Adenoma = Ca thyroid, Medullary ca as in MEN2 Other causes 7% = congenital: Brachial cyst, Dermoid cyst, Thyroglossal cyst = HNF: Cancers, Carotid aneurysm, Chemoductoma, Cervical Rib = Salivary: Parotid Tumors, Duct stones, other neoplasms

Answer 420

Achondroplasia Hypochondroplasia Cretinism, congenital Hypothyroidism Familial short stature - Back pain is common in all due to spinal canal stenosis, OA or spinal deformities - Frontal bossing, Low set ears

Answer 421

Achondroplasia- Early onset in childhood, Severe effects (Dwarf) defective fibroblast growth factor receptor, t makes your arms and legs short in comparison to your head and trunk. You may also have a larger head and weak muscle tone. Hypochondroplasia: a form of short-limbed dwarfism. This condition affects the conversion of cartilage into bone (a process called ossification), particularly in the long bones of the arms and legs very short stature, large head, accentuated lordosis, short arms and legs, and broad, short hands and feet. Other features include a limited range of motion in the elbows, lordosis, and bowed legs. Uncommon symptoms may include learning difficulties and convulsions. (FGF receptor 3 gene mutation)

Answer 422

Cretinism: untreated congenital hypothyroidism, with or without a goiter. Stunted growth and mental retardation. Short stature (dwarfism). Mild neurological impairment with reduced muscle tone and coordination. Hearing and speech defects.

Answer 423

Localized causes: Infections: = URTI, Otitis media, Tonsillitis, Dental abscess, EBV Infectious mononucelosis, CMV infection, Hepatitis, Brucella, Borelliosis, Toxoplasmosis Malignancies: Lymphoma, leukemias, Metastasis from GIm, breast, Lungs, Melanoma, HNF cancers Generalized causes: Hematological malignancies Infections as TB, HIV, EBV, Toxoplasmosis, Cat scratch disease Inflammatory: Sarcoidosis, SLE Drugs: DAAC, Phenytoin, Allopurinol, Atenolol, Cephalosporins

Answer 424

Hypogonadism Testicular tumors Chronic Liver disease Chronic Kidney disease Klinefelter syndrome 47XXY Thyrotoxicosis Ca Lung Hepatocellular cancers Drugs: Digoxin, Aldactone In liver disease there is an increased production of androstenedione by the adrenal glands, increased aromatisation of androstenedione to oestrogen, loss of clearance of adrenal androgens by the liver and a rise in SHBG, resulting in gynaecomastia. Malnourishment and re-feeding syndrome.

Answer 425

5A + STC Asthma Allergic rhinitis Acid reflux ACE inhibitors Alveolitis (Extrinsic allergic alveolitis) Sarcoidosis TB, Tumor Churg Straus syndrome, Carcinoids

Answer 426

R = Rheumatological diseases R = Renal:- Lupus, vasculitis, systemic sclerosis, RA stenosis E = Endocrine: high-Cortisol, Acromegaly D = Drugs - NSAIDs, phenyl ephrine, Psuedoephedrine Ca = Co-arctation of Aorta P = Psychiatric, Pregnancy CHAPS: Cushings: high cortisol Hyperaldosteronism: Conn's Aortic Co-arcation Pheochromocytoma, Pregnancy Stenosis of renal artery

Answer 427

in inflammatory diarrhea - calprotectin will be more than 150 mg/ Kg Functional diarrhea due to stress, food Diarrhea persists with Fasting, > 1 Lit = Neuroendocrine tumors: VIPoma, Carcinoids, Gastrinoma, Medullar thyroid cancer = Laxatives: see Osmotic Gap, MgPO4, Melanosis coli = Other causes: Mastocytosis, Villous adenoma with Hypokalemic alkalosis --------------- Diarrhea resolves with Fasting, < 1 Lit = Coeliac disease, diet association = CHO: Hydrogen breath test = Bacterial overgrowth in intestine: Breath test helps to diagnose = Biliary diarrhea (post GB Sx) = Diabetes related = Microscopic colitis

Answer 428

1. IBD- UC, Crohns, oral ulcers, clots 2. Cancer to suspect, RED FLAGS 3. Infectious: Shigella, Amoebiasis, E coli, Campylobacter 4. Bleeding tendencies: Warfarin 5. Diverticulosis with infection 8. Intussusception

Answer 429

Endocrine causes: = Acromegaly = Insulinoma = Cushing's disease, syndrome = Hypothyroidism ------------------- Non Endocrine Causes: = Chronic Liver, Heart, Kidney diseases = Protein loosing enteropathy Anasarca

Answer 430

With Airflow obstruction in Spiro - Asthma, COPD - Bronchiectasis, Cystic fibrosis - ABPA - Ca lung - Sarcoidosis - Anaphylaxis - Foreign body in airway - Laryngo-tracheal stenosis - Churg Strauss syndrome - Carcinoid syndrome, - Drugs: Aspirin, NSAIDs, beta blocker --------------- without Airflow obstruction in Spiro = chronic cough syndromes = Hyperventilation syndrome = Vocal cord dysfunction = Rhinitis, = GERD associated = LVF, Cardiac asthma = Pulmonary fibrosis

Answer 431

Age > 65 with new tummy pain, Left unintentional weight loss, tummy pain altered bowel habit iron deficiency anemia Fatigue, Heart failure with anemia Rectal bleeding Frequent nocturnal diarrhea/ pain Family H/o colon cancers

Answer 432

Haemorrhagic causes: = ADPKD = HHT telangiectasis = Traumatic = Bleeding tendencies = Illicit drugs Ischaemic causes: = HTN, Hyperglycemia, Hyperlipidemia, Heart disease, Smoking, Polycythemia Thrombophillic causes: = PNH: urine darkens after some time in air (Hep B associated) = Behcet's disease = Antiphospholipid antibody syndrome = Screen cancers/ DVT risk/ Family history

Answer 433

Acute Jaundice: = GB stones, cholangitis = Acute Hepatitis = Hemolysis = Budd Chairi syndrome: Hepatic vein thrombosis -------------------- Sub Acute Jaundice: Weeks to months = Pancreatic malignancy = Hepatobiliary malignancy = Right sided Heart Failure = Intra hepatic cholestasis: Drugs, Autoimmune, infiltrative ----------------------- Recurrent Jaundice: = GB disease = Bile transport disorders: Gilberts

Answer 434

Short incubation < 10 days - Dengue, Influenza - Typhoid fever - Legionnair's disease - Acute HIV - Meningococcal - Rickettsiel infections - Malaria ------------------ Medium incubation < 30 days - Malaria - Amoebic Liver abscess - CMV - Hepatitis A - Schistosomiasis - Toxoplasmosis ------------------- Long Incubation, 3 months Brucellosis Malaria Bartonellosis Histoplasmosis Lyme disease Tuberculosis Syphillis

Answer 435

Penile discharge Dysuria Scrotal swelling, pain Pelvic or abdomen pain Anal symptoms: bleed, discharge, tenesmus, pruritus, fissure, piles Genital ulcers or mouth, fingers Rashes Warts, peri anal

Answer 436

Painful Genital Ulcers: = Chancroid (Haemophillus) = Herpes simplex = Behcet's disease = Balanitis, candida, Trichomonos = Lichen planus ---------------------- Painless Genital Ulcers: = Primary Syphyllitic chancre = Lymphogranuloma venerum = Donovanosis, Klebsiella = Cancerous ulcer = Lichen sclerosus = Secondary syphilis (multiple)

Answer 437

Vaginal discharge - White candidiasis - Yellow trichomoniasis - Gray vaginosis Vaginal, Vulval soreness Dysuria, urethral or vulval pain Pruritus, usually candidiasis Ulcers: Genital, Oral, Fingers, butt Rashes, Warts Ask: Menstrual history, Gynecological history and about contraception

Answer 438

with Hypercalcemia: - Malignancy - Hyperparathyroidism - Ask: hard stools, renal stones, ECG QTc shortening, Psychosis, depression, bone pain without Hypercalcemia: - DM - Diabetes insipidus - Diuretics - Psychogenic Polydipsia

Answer 439

Denial then Isolation then Anger then Bargaining then Depression then Acceptance Ask- How you have been coping with death of your XXX. (Don't move forward with other history questions) Say = Since your XXX's illness was such a shock, I can see that you must be worried about your own health. Give a moment and inquire about cause, coping, how feels now, offer any help.

Answer 440

SPIKE Set the scene Perceive prior knowledge from Pt Invite discussion (seek-permission) Knowledge (explain it) Empathetic approach Summarize ICE Ideas - what do you think? Concerns: any concerns you have? Expectations: what are ur expectations from me?

Answer 441

it is essentially outcome of judgement in Bolam versus Frier management case Essence of this principle is to examine whether or not a practitioner's actions fall short of or differ from body of those of reasonable practitioners ie certain medical action matches most other physicians in same condition.

Answer 442

Immediate Advice: 1. Squeeze finger 2. Run copious cold water on area 3. Wash with soap or gel 4. Fill up incident report form 5. Referral to occupational health To Ask: When incident occurred Exact mode of injury Gloves wore or not Wound washed or not Immunization status (HepB) Details of patient: Dead/Unknown/Physician as source - consent from other party will be sought by another member of team for viral marker tests: 1. If capacitous 2. If lacks capacity temporarily - then later 3. If lacks capacity permanently, proxy by team or POA

Answer 443

Hepatobiliary: Fatty Liver, GB stones, Pancreatitis, Primary sclerosing cholangitis Genitourinary: Renal stones, amyloidosis, cystitis, CKD Musculoskeletal: Clubbing, Erosive arthritis, Hypertrophic osteoarthropathy, AVN of Hip, Muscle abscess Other: Erythema nodosum, Uveitis, Sweets syndrome (febrile neutrophillic dermatosis) Growth retardation

Answer 444

Say. -i appreciate your concern I acknowledge that this medications has SE But priority is to treat your condition and this is the recommended medication. Controlled doses will be used which will control your disease with minimal or no Side effects. We will regularly follow you up for any side effect and complications. If refused - ask if any specific reason you can sort out. If keeps refusing - offer meeting with consultant.

Answer 445

Why refusing -appreciate concern but ask why Solution: Explain benefits - May i explain to you that this is recommended procedure for your condition. Adverse Complications - can happen if you choose to refuse. Consultant meeting to offer finally

Answer 446

1. Introduce yourself 2. Confirm pt idendity 3. Confirm agenda of meeting - we are here to discuss your health issue, is that ok with you 4. Ask if would like any one else to attend this meeting if not BBN 5. Assess knowledge - can you tell me how much you known about your condition. 6. Do you have any expectations from this meeting.

Answer 447

1997, bolitho vs Hackney Health authority House of Lorde held that court was not bound to accept that a clinician was not negligent merely because an expert could be found to affirm that defendants actions were reasonable.

Answer 448

Who will do it? a professional who has done hundreds of such procedure and will also talk to you again. Before procedure: need to fast, check in time, travelling required, attendant required, approximate stay in hospital to explain During procedure: Short or long sedation Local or general anaesthesia Skin numbness or not possible duration After procedure: Recovery stay and possible duration After effects How long fasting Medication effects Procedure effects What can go wrong?

Answer 449

Acute Liver failure: Viral hepatitis, PCM toxicity Alcoholic CLD (reqd abstinence) Hepatitis B/C with cirrhosis Primary Biliary cirrhosis Genetic Haemochromatosis Wilson's disease Hepato-cellular carcinoma Cholangiocarcinoma

Answer 450

chronic fibrosis of intrahepatic and extra hepatic ducts and is associated with other autoimmunes Most common with UC 80% Can affect any age group MRI of bile ducts diagnostic High risk of cholangiocarcinoma, High risk of Ca colon Symptoms: Fatigue, Pruritus in middle age female, abdomen pain, cholangitis flare ups

Answer 451

Type I Autoimmune hepatitis: - classic, 80% - acute or chronic - Signs of CLD, palm redness, spider naevi, - ANA + SMA + LP antibodies + Soluble liver antigen antibodies - Polyclonal Hyper IgG Rx - Steroids, Azathioprine, MMF ------------ Type 2 Autoimmune Hepatitis: 10% cases, Liver-kidney-microsomal antibody + Antibody to cytochrome P450-11D6 Might be Hepatitis C carrier Rx - steroids Type 3 Autoimmune Hepatitis: Less than 10% acute or chronic kind Soluble Liver Antigen antibodies Antibodies to UGA suppressor Antibodies to tRNA Mild IgG raised Rx - steroids

Answer 452

= ATP7B gene mutations, chr 13, = Auto somal Recessive = manifests 5-45 year old = reduced Caeruloplasmin = Serum copper high, low or normal = 24 hr urinary copper - HIGH (Defect - copper cannot be incorporated into ceruloplamin and accumulates) = Clinical Effects: Liver cirrhosis, Hepatomegaly, Kayser-Fleischer rings in Iris, Sunflower cataracts, Tremors, Chorea, Rigid dystonia, Cramped writing Depression, Neurosis, Anti-social behavior, Emotional labile, Poor memory, Difficult abstract thinking, Fanconi Syndrome: due to Cu infiltration causing disruption of renal tubule exchange Arthritis Cardiomyopathy, arrhythmias, Hypo-PTH

Answer 453

General: Patient Education, Physiotherapy, Occupational therapy, Bone protection, Calcium + Vitamin D supplementations, Vaccinations Specific: Immunosuppression to reduce autoimmunity.. 1. Steroids: Oral, IM depots, into joints, useful in acute attacks flares 2. DMARDS: (Steroid sparing) Methotrexate, Sulfasalazine, Leflunomide, Azathioprine, Sodium Aurothiomalate, Hydroxychloroquin, Ciclosporin 3. Biologicals: Infliximab, Adalimumab, Etanercept, Anakinra, Rituximab - Cause induce TB, CTL

Answer 454

Rheumatoid factor: an IgM antibody Anti Citrullinated Peptide (CCP) antibody ----------------------- Antinuclear antibodies (ANA) Anti ds-DNA antibody Anti smooth muscle antibody Anti Ribo-nuclear (RNP) antibody Anti Jo, Ro, La antibodies Anti Neutrophil cytoplasmic antibody (ANCA-Perinuclear or ANCA-cytoplasmic)

Answer 455

= Sun exposure = Stress induced = Smoking = Drugs (beta-blockers, lithium, antimalarial-chloroquine, interferons, imiquimod, and terbinafine) = Trauma to skin = Steroids = HIV infection = Flu, recent infections = Alcohol

Answer 456

S- Supressibility of GH: Glucose Load P- Pituitary Hormones: TSH, ACTH, LH, FSH, Prolactin I- Insulin like Growth Factor levels C- Calcium, Mg levels T- target Glands: TFT, Cortisol, Sex hormones R-Radiology, CT/MRI

Answer 457

Acromegaly case can appear as something else: 1. Back pain - due to canal stenosis, OA, deformities 2. MEN-1 syndromes: association as pituitary tumor so look for other 3. OSA, same as Cushings, weight gain 4. Colorectal Cancer 5. DM, Resistant HTN 6. Hypogonadism (Hypopituitarism) symptoms (low libido, low fertility, poor muscles, more hairs on head)

Answer 458

Moans - Depression effects, complaining nature Bones - Bone pain Groans - tummy pain, dyspepsia Stones - Ureteric stones CHIMPANZEES: causes Calcium supplementation, excess Hyper PTH, Hyperthyroidism Iatrogenic, Immobilization Multiple myeloma, milk Alkali Syn Parathyroid adenoma, Pagets disease Alcohol addiction Neoplasms of breast, Lung Zollinger-Ellison syndrome (Gastric) Excess Vitamin D Excessive Vitamin A Sarcoidosis, Also - Familial Hypocalciuric Hypercalcemia

Answer 459

if > 20% blast cells- its Leukemia and not MDS MDS refers to group of clonal disorders of Haematopoetic stem cell which retain ability to differentiate into mature cells BUT do so in disordered and ineffective manner leading to hypercellular bone marrow and peripheral cytopathies MDS Types as per WHO classification: 1. Refractory anemia with Ring sideroblast 2. Refractory anemia without ring sideroblasts 3. Refractory cytopenia with multilineage dysplasia 4. Refractory anemia with excess blasts, Type 1 (5-9% blasts) Type 2 (10-195 blasts) 5. 5q syndrome 6. Unclassifiable MDS

Answer 460

Myelodysplastic/Myeloproliferative Overlap Syndromes: 1. Atypical Chronic Myeloid Leukemia (CML) 2. Juvenile Myelo-Monocytic Leukemia 3. Chronic Myelo-monocytic Leukemia 4. Unclassifiable

Answer 461

without Reed-Steinberg cells Ann Arbor Staging WHO classification, B cell, T cell types Clinical: painless generalized lymphadenopathy at axillary, cervical, inguinal with hepatosplenomegaly B symptoms: Fever, Night sweats, Weight loss, Anorexia Unusual Sites: CNS, Tonsils, Adenoids, Salivary glands, Jaw Burkitts, Lung, Skin, GI tracts, Testicles Treatments: Combination chemo + HSCT (Fludarabine, Chlorambucil, Cyclophosphamide) Splenectomy Rituximab (Anti CD20 receptors) H pylori eradication for MALT Field radiotherapy Intra thecal methotrexate

Answer 462

Myeloid Series: WBCs (Granulocytes, Monocytes), RBCs, Platelets Lymphoid Series: B & T lymphocytes F.A.B classification: M0 - acute myeloblastic with minimal differentiation M1 - acute myeloblastic without maturation M2 - acute myeloblastic with maturation M3 - Acute Promyelocytic Leukemia (APML) M4 - Acute myelomonocytic leukemia (AMML) M5 - acute monocytic monoblastic leukemia M6 - Erythro Leukemia M7 - Megakaryocytic Leukemia Auer rods are granule clumps seen in M1M2M3 types in leukemic cells.

Answer 463

Reed steinberg cell (Owl's eye appearance) in histology Cervical and mediastinal lymphadenopathy typically Types: 1. Lymphocyte predominant 2. Mixed cellularity 3. Nodular Sclerosing 4. Lymphocyte depleted bimodal distribution, age groups Curable with chemo

Answer 464

1. B cell lymphomas > 85% of all NHL 2. T & NK cell lymphomas, rare 3. B lymphoblastic leukemia/Lymphoma, CD79, 19, 109 4. T lymphoblastic Leukemia/Lymphoma, CD2, 3 5. Peripheral mature B cell : CLL, B cell Prolymphocytic or Other types as: - Waldenstroms macroglubulinemia - Splenic margincal zone lymphoma - Mucosa associated Lymhoid Tissue - Plasma cell MYELOMA, Plasmacytoma, - Heavy chain disease 6. Peripheral mature T cell lymphoma 7. Hodgkins Lymphoma: 30% of all lymphomas, Reed steinberg cell +

Answer 465

Signs of marrow failure: anemia, hepatosplenomegaly, Lymph nodes + weight loss, petechial rash, Herpes zoster, Can be: B or T cell type Transformation to Richter Large cell lymphoma EBV related transformation to Lymphoma/ CML Rai Classification: 0 - Lymphocytosis 1 - Lymphocytosis + L.adenopathy 2 - Lymphocytosis + splenomegaly + ? Hepatomegaly also 3 - Lymphocytosis + anemia, Hb < 11 4 - Lymphocytosis + Low platelets < 100k Binet Classification: A - 0-2 organs involved, hb >10, Platelets > 100k B - 3-5 organs involved, Hb > 10, Platelets > 100k C - 3-5 organs involved, Hb < 10, Platelets < 100k Diagnosis: CD23 +, CD5+, Bone marroes aspiration or biopsy, Blood films, CT Scans, Immunophenotyping Rx: chlorampbucil + Fludarabine + Prednisolone Campath + Rituximab

Answer 466

Immunophenotyping is a technique used to study the protein expressed by cells. This technique is commonly used in basic science research and laboratory diagnostic purpose. This can be done on tissue section, cell suspension, etc. An example is the detection of tumor markers, such as in the diagnosis of leukemia

Answer 467

Treatment not until: - Weight loss > 10% baseline - Progressive anemia - Fever > 2 weeks continuous - Night sweats > 1 month - Progressive marrow failure signs - Symptomatic splenomegaly, pain - Massive Lymphadenopathy Chemo Regimens: 1. Fludarabine based: Fl + Cyclophos + Rituximab Fl + Rituximab every 28 days ( (++Infection prophylaxis) 2. Pentostatin based: Pentostatin + Cyclophos + Rituximab 3. Bendomustine based: Bendomustine + Rituximab every 28D 4. Alemtuzumab(CAMPATH) 5. Infection prophylaxis : Septran + Valacyclovir

Answer 468

Major criteria: A1- Red cell mass high, PCV > 0.6 males, > 0.56 females A2 - No secondary cause A3 - Palpable spleen A4 - clonal markers positive other than Philadelphia chromosome or bcl/abl gene Minor Criteria: B1 - thrombocytosis B2 - neutrophillic leucocytosis B3 - splenomegaly by scan B4 - growth of burst forming units, erythroids or reduced EPO

Answer 469

Suspect when raised red cell mass and normal PaO2 with either splenomegaly or two of: - WCC > 12k - Platelets > 400k - Raised B12 binding protein - Low neutrophil ALP

Answer 470

1. Anterior uveitis, 40%, Red eye 2. Aortic regurgitation, MR also 3. AV Conduction defects, 10% cases 4. Apical lung fibrosis + costovertebral rigidity 5. Atlanto-Axial subluxation: Neuro sequelae, vertebral #, Cauda equina 6. Achilles tendonitis 7. Amyloidosis in long term

Answer 471

Basic work up: CBC, CRP, ESR, RFT, LFT, Hand Xrays (for erosive arthritis) Immune work up: RA factor, Anti CCP antibody, ANA screen Poor prognostic factors for RA: = RA positive status = Anti CCP positive status = Early Xray erosions = Impaired functional status = Persistent active state

Answer 472

American Rheumatology Association 4 out of following: = Morning stiffness > 1 hr = Symmetrical joint involvement = Arthritis affecting > 3 joints = Small Hand joints involved = Positive RA Factor = Rheumatoid nodules present = Radiolgical erosive arthritis

Answer 473

has features of cavernous syndrome and ipsilateral optic nerve involvement as well. Cavernous Sinus features: = Diplopia = Painful ophthalmoplegia = Visual loss = Facial numbness (V1 Trigeminal) = Corneal reflex loss (V1) = Proptosis = Pulsating exophthalmous = Ipsilateral 3, 4, 6 cranial palsies Optic Nerve involvement: = Reduced visual acuity = Reduced color vision = RAPD

Answer 474

C - Cystic fibrosis, CHD L - Lung cancer or abscess U - Ulcerative colitis B - Benign mesothelioma B - Bronchiectasis I - Interstitial Lung disease N - Neurogenic tumors G - GI diseases as IBD

Answer 475

immunodeficiency syndrome X linked Genetic defects in FoxP3 gene hence defective Treq (a regulatory gene) - Immune dysregulation - Polyendocrinopathy - IDDM - Enteropathy

Answer 476

AIRE (autoimmune regulator gene) mutations Type 1: Monogenic syndrome candidiasis + hypoparathyroidism + addisons (AIRE on chr 21) Type 2: Schmidt syndrome Adrenal insufficiency + Autoimmune thyroid +_ DM +_ pleuritis/pericarditis (HLA D3, D4 association) Type 3: Autoimmune thyroid + other autoimmunes but not addisons Type 4: Autoimmune combinations other than above Vitiligo, Alopecia areata, Pernicious anemia, Coeliac disease, Dermatitis herpetiformis, chronic atrophic gastritis

Answer 477

Rare, due to mutation of glucocorticoid receptor but no features of Cushings High ACTH levels and High cortisol levels Virilization in women due to excess andogens HTN due to salt retention by ACTH action No proximal myopathy or thin skin as in Cushing

Answer 478

maternally inherited from mitochondrial DNA defects which only present in ovum and not in sperms, no introns 1. MELAS: Mitochondrial Encephalopathy + Lactic acidosis + Stroke like episodes 2. MERRF: Myoclonic epilepsy + Ragged Red fibers 3. Mitochondrial inherited DM and Deafness 4. Leber's Hereditary Optic Atrophy

Answer 479

C3 deficiency: leads to recurrent bacterial infections C5 Deficiency: predisposes to Leiner disease ie infantile seborrheic dermatitis, recurrent diarrhea, CNS issues, failure to thrive, recurrent local and systemic infections C5 TO C9 deficiency: predisposes to meningococcal dissemination C1q, C1rs, C2 C4 deficiencies: predisposes to immune complex disease, SLE, HSP etc

Answer 480

1. Neutrophil disorders: - chronic granulomatous disease - Chediak Higashi syndrome - Leucocyte adhesion deficiency 2. B cell disorders: - common variable immunodeficiency - Bruttons agammaglobulinemia - selective IgA deficiency 3. T cell disorders: - DiGeorge syndrome 4. Combined B and T cell disorders: - severe combined immunodeficiency - Ataxia telangiectasis - Wiskott Aldrich syndrome

Answer 481

Gels & Coombs classification: Type 1 Hypersensitivity: Anaphylactic, IgE on mast cells reacxts with antigens, common with asthma, eczema, Hay fever Type 2 Hypersensitivity: Cell bound, IgG or IgM binds to antigen on cell seen in ITP, Goodpastures syndrome, Autoimmune hemolysis, Transfusion reactions, Rheumatic fever, Bullous pemphigoid, Pemphigus vulgaris Type 3 Hypersensitivity : Immune complex mediated, antigen + antibody, IgA, IgG, seen in Serum sickness, SLE, Post strepococcal GN, Acute allergic alveolitis Type 4 Hypersensitivity: Delayed type, T cell mediated seen with TB, Tuberculin reaction, GVHD, Allergic contact dermatitis, scabies, GBS, MS Type 5 Hypersensitivity: antibodies recognizes and binds to cell surface receptors, seen with Graves disease, myesthenia gravis

Answer 482

Autosomal dominant, C1 Inhibitor (a serine protease inhibitor) deficient, leads to excess bradykinin levels. C2 AND C4 complement levels are low. May get painful macular rash and later painless tissue edema of upper airways, skin, abdominal organs. (NO URTICARIA) Diagnosis: C4 levels, C1INH levels Rx: C-INH concentrates as iCantibant, FFP, Danazol

Answer 483

0 - normal CXR I - HIlar nodes IIa - Hilar nodes with diffuse pulmonary IIb - diffuse pulmonary disease without L nodes III - pulmonary fibrosis Starts Prednisolone 0.5 mg/Kg/Day for 4 weeks then tapen and maintain 5-15 mg/Day, start at stage IIa

Answer 484

Stein-Leventhal syndrome Cushing's syndrome 21 hydroxylase deficiency PCOD: LH:FSH> 2, Hirsutism, Wt gain, menstrual irregularities, ovarian cysts, Testosterone >80ng DHEAS levels > 8000 suggests adrenal tumor DHEAS, 4000 to 8000 - Congenital adrenal hyperplasia Testosterone levels of 150-200 ng/dl in females are suggestive of ovarian cancer

Answer 485

shifting eosinophilic pulmonary infiltrates, usually associated with parasitic infestation.

Answer 486

Lipoprotein lipase - Hyper TG Aryl Sulfatase A - Metachromatic Leucodystrophy: progressive myelin destruction due to sulfatide accumulation causing CNS, PNS damage Beta Glucosidase - Gaucher's disease: lipid accumulates in spleen, Liver Hexosaminidase A - Tay Sachs disease: a type of Lysosomal storage disorder, ganglioside accumulation causing brain damage Sphingomyelinase deficiency: Nieman Pick disease: abnormal amounts of lipids (fatty materials such as waxes, oils, and cholesterol) build up in the brain, spleen, liver, lungs, and bone marrow causing dysfunction Idurudinase deficiency - Hurler syndrome: Mucopolysacchridosis: failed newborn hearing screen, respiratory symptoms, difficulty latching, and otitis media. Other symptoms such as kyphosis, corneal clouding, cardiac disease, joint restrictions, and enlarged head circumference typically appeared slightly later (median age, 8–10 months)

Answer 487

Excessive anterior curvate of spine - Osteoporosis - Spina bifida - Calves and Scheurmann's osteochondritis - Cancer with vertebral mets - Wedge fractures - Tuberculosis of spine - Polio neuropathy - Paget's disease - Ankylosing spondylosis

Answer 488

Synovial fold above medial meniscus of knee is inflammed with superomedial pain. There may be brief locking of knee. Rx: RICE protocol, Surgical division of synovial fold.

Answer 489

happens usually in Runners Thought to be due to overuse and associated with movement of iliotibial tract ie thickened lateral edge of tensor fascia lata and lateral femoral epicondyle. Present with lateral knee pain and tenderness over lateral femoral epicondyle. Rx - RICE protocol and Hips adductor strengthening exercises.

Answer 490

Common in young adults, children - immune mediated complement, perivascular damage without or without myositis. Features: Heliotropic violaceous rash around eyes but also possible on chest, upper back, Gottron's papules over knuckles, fingers, Nail fold erythema Complications: Swallowing difficulty, Wt loss, Malnutrition Aspiration pneumonia , SOB, with Chest myopathy, Calcinosis cutis Also can have - Raynaud's phenomenon, SLE, CTD, Rheumatoid arthritis, Cardiomyopathy, ILD, Malignancy as Ca lung association (Look for it) Work up: CK, LDH, LFT, CXR, EMG, MRI, FBC, ESR, CRP, Skin or muscle bIopsy, Malignancy work up. Treatment: Prednisolone, Azathioprine, MMF, Rituximab, Antimalarials, Sunscreens, IV-IG, remove Calcinosis surgically.

Answer 491

Acquire Inflammatory Myopathies = Polymyositis = Dermatomyositis = Inclusion body myositis = Drug induced in all tender muscles, rash, marked distal weakness and atrophy Metabolic/ Endocrine: Cushing's associated Thyroid myopathy with Hypo/Hyper Hypoadrenalism Osteomalacia Malignancy, Paraneoplastic Alcohol associated Hypophosphatemia can cause weakness

Answer 492

Muscular Dystrophies: = prominent Limb Girdle weakness = Non Limb Girdle MD with cranial palsies Congenital Myopathies: = Many example - Centro-nuclear myopathy with Long thin Face, High Arch Palate, Distal weakness Muscle Ion Channelopathy: = Myotonic Dystrophy (DM1) = Proximal Myotonic Myopathy (DM2) = Non Dystrophic Myotonias = Periodic paralysis Genetically determined Metabolic Myopathies: = McArdle disease + Glycogen Storage disorders (myalgia on exercise) = Mitochondrial disorders (MELAS, MERRF) = Malignant Hyperthermia

Answer 493

initial muscle fiber necrosis replaced later by fat fibrosis = Duchenne Muscular dystrophy, = Becker Muscular Dystrophy, = Limb-Girdle Muscle dystrophy, = Oculo-pharyngeal muscle dystrophy, = Fascio-scapulo-Humeral Muscle dystrophy

Answer 494

X linked, dystrophin production is reduced begins age 2-3 yr old Severe proximal weakness in childhood, cant stand and shows Gower Sign ie tries to climb his own body to stand Calf pseudohypertrophy is a feature Waddling gait initially then chair bound Pneumonias are common due to myopathy ECG shows tall R waves & can develop cardiomyopathy, High CK like 80k

Answer 495

Milder form compared toDMD, start 5-15 yrs dystrophin is produced but low levels Slow progression over years, chair bound Proximal thigh and arm weakness Knees bend back to take weight walking Atrophy or thigh muccles, waddling gait, Gower Sign + Calf Hypertrophy ++ Myalgia ++, high CK levels

Answer 496

Autosomal recessive over 30 different forms Starts late childhood or adulthood Proximal weakness of Limb, girdle muscles High CK, myalgia etc

Answer 497

Ophthalmoplegia with Ptosis Bulbar weakness Autosomal dominant appears 40-60 yr old, Early dementia Can have proximal weakness also Can have Chest, Cardiac signs

Answer 498

Auto somal dominant Facial weakness, difficulty smiling, whistling, eye closure etc Weak lips, difficulty opening mouth Dysarthria as weak buccals Winging of scapula Weak shoulder movements

Answer 499

Autoimmune Inflammatory myositis, appears > 50 yrs age usually Symmetrical proximal muscle weakness, 50% Painful, tender muscles No -Ocular involvement or Skin rash when lone Malignancy risk, Ca Lung, Ca breast Enzymes: CK 5-50 times, LDH, Transaminase raised Extra Muscle features: = No skin rash but fatigue, fever, Wt Loss = Calcinosis: Skin, Face, Intra-muscular = Symmetrical Large Joint pain = Dysphagia = Diffuse parenchymal Lung disease ++ = Raynaud's phenomenon = Myocarditis, Pericarditis = Proteinuria, Nephrotic syndrome = Mesangial proliferative Glomerulonephritis ANA > 80%, ENA (Ro, E1, tRNA synthetase)+ Anti SRP, Anti-M2 antibodies Monitor: Muscle power, CK levels, SE of medications as Dyspepsia, Osteoporosis, Depression Rx: Good response to steroids, Azathioprine, Methotrexate, Ciclosporin

Answer 500

1. Early onset MG with thymoma: occurs < 40 yr old, HLS B8-DR3 association, female>Male 4:1 2. Late onset MG with thymus atrophy HLA B7, DR3, Female:Male=2:1 3. MG with Thymoma without HLA association 4. Pure motor MG in 20% 5. Musk Antibody + MG, Late onset Rx of MG: = Pyridostigmine 60 mg TID = Propantheline low dose (Anticholinergic) = Prednisone, Azathioprine (check TMPT levels) = Thymectomy = Avoid triggers

Answer 501

paraneoplastic Autoimmune MG, > 50-60 yr old Look for associated malignancy ie Ca lung May precede cancer by 5 yrs Fatigability without ocular, bulbar weakness Initial areflexia returns after some exercise No ptosis or diplopia Antibodies to Calcium gated channels at NMJ EMG shows improvement on repetitive testing, amplitude improves

Answer 502

Small vessel vasculitis, affects children 4-15 yrs 1. GI symptoms: Pain, diarrhea, rectal bleed 2. Arthralgia: Large Joints 3. Purpura: Lower Limbs, buttocks 4. Nephritis Complications: Perforation, Intusussesption Acute renal failure Diagnosis: Clinical + Skin biopsy + Renal Biopsy Rx: Supportive, Methyl prednisolone

Answer 503

Deficiency of homogentisic acid oxidase, affects breakdown of Tyrosine to fumarate Autosomal Recessive Dark connective tissue, Brown sclera Urine turns black on long air exposure May cause severe arthralgias as homogentisic acid is toxic to cartilages.

Answer 504

X Linked recessive, more in boys B cell does not mature due to defect in BTK gene Recurrent bacterial infection after 6 months of life as maternal IgG used up Less immunoglobulins of all types

Answer 505

Due to deficiency of Galactose 1-phosphate arityl transferase which leads to accumulation of galactilol (Galactose converted instead to Galactilol by aldose reductase) which accumulates in Lens of eye. Infantile cataract Failure to thrive, Mental retardation Jaundice, Hepatomegaly Autosomal recessive Rx: Galactose & Lactose free diet

Answer 506

Hereditary deficiency of aldolase B Lead to fructose accumulation causing decreased availability of phosphate as Fructose 1 phosphate is not broken done by aldolase and leads to inhibition of glycogenolysis and gluconeogenesis. Hypoglycemia attacks Cirrhosis of Liver Liver cirrhosis, Jaundice, vomiting, Autosomal recessive Rx- Fructose less diet

Answer 507

Deficiency of alfa ketoacid dehydrogenase blocks degradation of branched chain amino acids (Isoleucine, Valine, leucine) leading to accumulation of alfa keto acids in blood causing CNS defects, mental retardation, death. Urine smells of maple syrup Autosomal recessive Mnemonic: I Love Vermont Maple Syrup (I-Isoluecine, L-Leucine, V-Valine)

Answer 508

X linked defect in WAS gene on X chromosome T cells are unable to reorganize actin cytoskeleton TIE triad: Thrombocytopenic purpura + Infections + Eczema High IgE, High IgA, Low IgM Low platelets Presents from Birth to 25 yrs but usually around 21 months with bruises, ecchymosis

Answer 509

1:10000 to 20,000 births, Auto recessive Phenyl alanine is an essential amino acid which is not broken down due to deficiency of Phenyl alanine hydroxylase (and Tetra hydrobiopeterin) and leads to accumulation. It breaks down into phenyl ketones instead which causes: - Prominent Maxilla - Microcephaly - Wide spaced teeth - Mental Retardation - Growth retardation - Convulsions - Fair skin with eczema - musty body odor, detectable in babies after 2/3 days as maternal normal PK hydroxylase circulated till then in babies blood. Rx: High Tyrosine diet

Answer 510

inherited transmission of Prion proteins causing this GSS syndrome in newborns with: = ataxia = Spongiform encephalopathy When acquired - called kuru When Sporadic =Creutzfeldt Jacob disease (rapid ly progressive dementia)

Answer 511

Loss of function mutation in WNK kinases in distal renal tubule causes familial HTN with Hyperkalemia. There is over activity of Na/K co-transporter in distal tubule leading to over absorption of Na leading to volume expansion and HTN. Hyperkalemia is due to decreased activity of K channels, a primary route or K secretion.

Answer 512

in neonates, chloramphenicol causes gray discoloration due to lack of lover enzyme UDP glucoronyl transferase. Drugs is not metabolized properly due to this and toxic levels develops. Baby looks ashen grey with cyanosis and Hypotension.

Answer 513

condition characterized by very low HDL lipid levels due to = Mutations in ABC A1 gene (ATP binding cassette transporters) which transfers cholesterol from cells to nascent HDL particles and is lacking in Tangier disease. Severe deficiency or absence of high-density lipoprotein (HDL) in the circulation resulting in tissue accumulation of cholesteryl esters throughout the body, particularly in the reticuloendothelial system. = Enlarged orange yellow tonsils = Fatty deposits can also form in other organs causing enlargement of the throat, liver, spleen, or lymph nodes. A low-fat diet helps in reducing liver enlargement and preventing atherosclerosis. LDL-lowering drugs are required in patients with cardiovascular risk factors or overt signs of cardiovascular disease, especially when hepato-splenomegaly is absent.

Answer 514

Mutations in SDHS, SDHC amd SDHD gene causes disease when inherited from father only. Maternal gene is imprinted ie inactivated. Autosomal dominant Examples: Phaeochromocytoma Glomus jugulare

Answer 515

a genetic imprinting syndrome wherein one copy of any chromosome from a parents is inactivated - here abnormal imprinting of IGF2/H19/P57kip/KVLgQT1 gene on Chr 11p15 Features: Large birthweight Neonatal Hyperinsulinemia with low sugars Omphalocele, Exomphalous Hemihypertrophy Facial Naevus flammeus Increased risk of childhood tumors as Wilm's tumor, Hepatoblastoma

Answer 516

Familial HOCM Carney complex Type 1 Usher syndrome Non syndromic deafness

Answer 517

Carney complex is a hereditary condition associated with spotty skin pigmentation; myxomas, which are benign (noncancerous) connective tissue tumors; and a number of other tumors of the endocrine (hormone-producing) glands. Tumors can be benign or cancerous. A benign tumor means the tumor can grow but will not spread. Symptoms syndrome can include obesity above the waist (but with thin arms), a round face, skin changes, weak bones and muscles, and slow growth rate in children. Carney complex tumors on the adrenal glands, called primary pigmented nodular adrenocortical disease (PPNAD), can also lead to Cushing syndrome. Carney triad is a rare condition that describes the occurrence of three kinds of endocrine tumors in the same child. The tumors comprising the triad are tumors in the gastrointestinal tract (known as gastrointestinal stromal tumors, or GIST), pulmonary chondromas, and paragangliomas.

Answer 518

Genetic Causes: - Cornelia de Lange syndrome - Coffin Sins syndrome - Lepre chagnism - Hurler syndrome - Trisomy 18 Drugs induced Syndromes: - Fetal Hydantoin syndrome - Fetal alcohol syndrome - Diazoxide - Post natal steroids

Answer 519

Congenital micro deletion of long arm of chromosome 7, includes elastin gene deletion, elfin facies, intellectual disability, Hypercalcemia: increased sensitivity to Vit-D Well developed verbal skills CVS problems Extreme friendliness to strangers

Answer 520

due to micro deletion of 22q11 CATCH-22 C - Cleft palate A - abnormal facies T - thymic aplasia, T cell deficiency, infections C - cardiac defects as ASD, VSD H - Hypocalcemia due to parathyroid aplasia 22 - chromosome affected Examples: DiGeorge syndrome VeloCardioFacial syndrome

Answer 521

only one sex chromosome ie single X affected girls have classif features - webbed neck - short stature - primary ovarian failure, amenorrhea Risk of: - Heart defects - Recurrent otitis media - Renal malformations - Deafness - Early age osteoporosis - Lymphedema development - Aortic dissection - Hypothyroidism, Obesity

Answer 522

Genetic imprinting ie one of autosome inactivated from either of parent origin Maternal uniparental disomy here, Chr 7 Small stature Triangular facies Poor feeding in infancy Relative macrocephaly Asymmetry of features 5th finger clinodactyly (curved)

Answer 523

Autosomal dominant PA - pyogenic sterile arthritis P - Pyoderma gangrenosum A -Acne

Answer 524

21 chromosome triple (1 extra) Most common cause of Mental retardation USG shows increased nuchal translucency in 1st trimester - Flat facies - epicanthal folds - Simian crease (single) - Gap between 1/2 toes - Duodenal atresia - Cardiac defects: ASD, VSD - Risk of early Alzeimer's, - Risk of acute lymphoblastic Leukemia 1:25 chance if mother > 45 yr old

Answer 525

Trisomy 18 ie 3 autosomes at 18 Severe mental retardation Rocker bottom feet Micrognathia Low Set ears Clenched hands Prominent occiput Cardiac defects as ASD, VSD etc Death within 1 yr of birth usually

Answer 526

X linked defect affecting methylation and expression of FMR1 gene Second most common genetic cause of mental retardation after Down's Macro-orchidism (Large testicles) Large face with large jaw, large everted ears Autism Mitral valve prolapse by birth Trinucleotide repeat disorder (CGG)

Answer 527

3 D's of pellagra: Diarrhea + Dermatitis + Dementia Glossitis, angular cheilosis Niacin (B3) required pyridoxine (B6) FOR SYNTHESIS from tryptophan - various problems with Tryptophan and B6 can cause B3 problems as well causing pellagra. Examples: 1. Hartnup disease: poor tryptophan absoprtion 2. Malignant Carcinoid syndrome: causing increased rapid tryptophan metabolism 3. Drug induced: INH causes reduced B6 levels

Answer 528

vitamin C (Ascorbic acid) deficiency causes defect in collagen synthesis ie impaired hydroxylation of proline and lysine - swollen gums, bleeds - Easy skin bruising - Haemarthrosis with minor trauma - Anemia - Poor wound healing - Weak immune response to infections

Answer 529

Lofgrens syndrome: presents with hilar lymphadenopathy, erythema nodosum, arthralgias and fever Heerfordt's syndrome: acutely presents with fever, uveitis, parotid swelling and other salivary/lacrimal gland swelling. Facial nerve palsy may be a presenting feature. May have lupus pernio, skin granulomas, lung lesions. Meningism, Ophthalmoplegia, Pupil reflex abnormal might be seen as well due to neurosarcoidosis.

Answer 530

unknown cause Hyper turnover of bones with abnormal healing Defective remodelling leads of thickened bones Typically presents in elderlies Elevated ALP Normal serum calcitonin Almost normal ESR Hyperdynamic circulation with CHF possible Deafness due to entrapment neuropathy Bone pain Sabre tibia Protruding skull Heart Failure features Nerve compression palsies - cranials Pathological fractures with warmth Osteosarcoma development RISK Rx - Biphosphonates

Answer 531

High levels of Macroglobulin ie IgM > 2.5 - High viscosity of blood - Pancytopenia as marrow infiltrated - Raynaud's phenomenon + Cyroglobulinemia type 2 Coagulation abnormalities Polyneuropathies Cold agglutinin disease Anemia Primary amyloidosis IgM DEPOSITION in skin, Gut, Kidneys (all effects of high IgM levels)

Answer 532

Form of Hemolytic anemia Can be idiopathic or acquired due to lymphoproliferative diseases as Leukemia or with Mycoplasma infection form of autoimmune hemolytic anemia (AIHA) in which cold agglutinins (IgM autoantibodies against red blood cell [RBC] antigens that bind at cold temperatures) can cause clinical symptoms related to RBC agglutination in cooler parts of the body and hemolytic anemia. symptoms of cold agglutinin disease? Fatigue. Weakness. Dizziness. Sweating. Shortness of breath (dyspnea). Fast heartbeat (tachycardia). Skin that appears pale or drained of blood (pallor). Bluish fingers, toes or ears after spending time in the cold (Raynaud's phenomenon). Detection: The Coomb's test can detect cold agglutinin antibodies in the blood. The test comes in two types, direct and indirect. The direct Coomb's test detects antibodies and other immune system-related proteins that are attached to red blood cells. Rx - recommends rituximab, with or without bendamustine, for first-line treatment of patients with cold agglutinin disease who require therapy

Answer 533

rare type of autoimmune hemolytic anemia Sudden onset Hemoglobinuria in response to exposure to cold for even few minutes Auto antibodies causes intra vascular hemolysis PNH is not caused by an autoantibody to red cells in contrast to PCH, which is caused by an autoantibody (detectable by the Donath-Landsteiner test) More in children Patients present with a combination of the following: sudden onset of back and abdominal pain, headache, leg cramps, fever, rigors, chills, nausea, vomiting, diarrhea, and esophageal spasms. Management of PCH has been primarily supportive with maintaining warm ambient temperatures to prevent further autoantibody binding, and transfusions using a blood warmer as needed.

Answer 534

Type III immune complex disease antibodies to foreign proteins are produced, takes about 5 days - and deposited in membranes where they fix complements leading to tissue damage Most commonly due to drugs Presents: fever, urticaria, proteinuria, Lymphadenopathy (happens 5-10 days after drug exposure)

Answer 535

Autosomal dominant Hypokalemia, Low Renin, Low Aldosterone Mutations in epithelial Na channel genes leading to more Na absorption causing HTN, Hypokalemia as K lost, Metabolic alkalosis Symptoms are of hypertension, fluid retention, and metabolic alkalosis. Aldosterone levels are high in hyperaldosteronism, whereas they are low to normal in Liddle syndrome. Rx - Amiloride

Answer 536

Mutations in genes encoding for the alfa 3/4/5 chains of Type 4 collagen, produces split glomerular basement membrane and glomerulosclerosis. Renal dysfunction with its effects (Hematuria, protein uria) - nephritic Deafness Ocular disturbance Rx - ACE inhibitors reduced CKD progression

Answer 537

Autosomal recessive Granulomatous colitis + Interstitial Lung disease, ILD + Oculo-cutaneous albinism Bleeding tendencies due to platelet dysfunction Accumulation of chromolipid, Lipofuscin material in the cells of RE system

Answer 538

Autoantibodies to alfha 3NC1 domain of type 4 collagen causes pulmonary haemorrhages and glomerulonephritis Presents after sore throat followed by hematuria and haemoptysis Needs immunosuppression and steroids

Answer 539

due to enterohaemorrhagic E coli strain infection O157:H7 Presents with triad of: = Acute renal failure = Microscopic Hemolytic anemia = Thrombocytopenia with normal clotting after episode of bloody diarrheal episodes Can have stroke, seizures, coma, pancreatitis, pleural and pericardial effusions Blood smears shows MAHA, helmet RBCs Hemolysis markers: Low Hb, High LDH, High bilirubin, Low haptoglobin Deranged renal function with uremia, hematuria Petechial rash possible with low platelets

Answer 540

Autosomal recessive deficiency of 11-beta-OH-steroid dehydrogenase which results in a defect of the peripheral metabolism of cortisol to cortisone (inactive). Cortisol half-life in the plasma is prolonged from approximately 80 minutes to 120 to 190 minutes. The resultant activation of the mineralocorticoid receptor causes hypertension (hypokalemia and alkalosis), resulting in a syndrome of apparent mineralocorticoid excess (AME). This enzyme is inhibited by glycyrrhetinic acid in licorice. AME is usually diagnosed within the first years of life and is characterized by polyuria and polydipsia, failure to thrive, severe hypertension with low renin and aldosterone levels, profound hypokalemia with metabolic alkalosis, and most often nephrocalcinosis. Mineralocorticoid receptor antagonists normalize blood pressure, correct hypokalemia and reduce hypertensive end-organ damage in patients with AME. Low dose dexamethasone can be considered, though the response may be variable. Future directions of therapy include selective mineralocorticoid antagonists.

Answer 541

Pregnancy Hypothyroidism, high TRH stimulates prolactin release Non Functional pituitary adenoma causing stalk compression and prolactin release PCOD Drugs: Dopamine antagonist as Chlorpromazine, Haloperidol, Domperidone, SSRI etc

Answer 542

Alpha-gal syndrome is a type of food allergy to mammalian (red) meat products. Alpha-gal syndrome is associated with tick bites, most commonly from the Lone Star tick in the US, but also from different types of ticks in other areas of the world. During a bite, a tick injects a type of sugar molecule (galactose-alpha-1,3-galactose), also called alpha-gal, which leads to formation of an antibody. Because alpha-gal is present on cells of most mammals, including cows, pigs, lambs, and goats, when affected individuals eat these animal products, an allergic reaction may occur.

Answer 543

Alpha-gal syndrome is a type of food allergy to mammalian (red) meat products. Alpha-gal syndrome is associated with tick bites, most commonly from the Lone Star tick in the US, but also from different types of ticks in other areas of the world. During a bite, a tick injects a type of sugar molecule (galactose-alpha-1,3-galactose), also called alpha-gal, which leads to formation of an antibody. Because alpha-gal is present on cells of most mammals, including cows, pigs, lambs, and goats, when affected individuals eat these animal products, an allergic reaction may occur.

Answer 544

Hb < 13 in men Hb < 12 in women, non pregnant Hb < 11 in first trimester Hb < 10.5 in 2+3rd trimester Hb < 10 post partum

Answer 545

1. Ask - identify those who can benefit. 2. Assess - current activity level 3. Advise - individualize plan 4. Assis - provide a written plan & support material 5. Arrange - appropriate follow up and referral

Answer 546

1. Post exertion malaise 2. Unrefreshing sleep 3. Substantial reduction in ability to engage in Pre illness levels of various activities that persists for more than 6 months & accompanied by new profound fatigue (not due to ongoing exertion & not relieved by rest) And one of: 1. Cognitive impairment 2. Orthostatic dizziness

Answer 547

1. Unprovoked bruising on trunk > 5cm 2. Frequent unprovoked epistaxis or gingival bleeding 3. Menorrhagia with iron deficiency 4. Haemarthrosis with mild trauma 5. H/o excessive surgical blood loss 6. H/o reoperation for blood loss 7. Family history 8. Severe liver or kidney disease 9. Medications suggestive

Answer 548

**Delirium** **Infection** - urethritis, cystitis etc **Lower Atrophic** urethritis/vaginitis **Pharmaceuticals** - diuretics, anticholinergics, Psychotropics, Opiod, Alfa blockers in women, Alfa agonists in men, CCB **Psychological** **Excess urinary** output **Restricted mobility** **Stool impacting**

Answer 549

**Stage 01** - Non blanchable erythema of intact skin **Stage 02** - partial thickness skin loss with exposed dermis **Stage 03** - Full thickness skin loss ** Stage 04** - Full thickness skin & tissue loss **Unstageable** - obscured full thickness skin and tissue loss **Deep tissue ** - persistent non blanchable deep red, maroon or purple discolouration

Medicine General 01 Flashcards

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