Written Flashcards

Question

List causes of acute paralysis 5

Answer 1

1. Cerebral strokes e.g. infective endocarditis 2. Spinal cord a. Transverse myelitis b. Spinal cord trauma as in traffic road accidents. 3. Anterior horn cells: Poliomyelitis: asymmetric ascending paralysis. 4. Peripheral nerves a. Guillain Barre syndrome (the commonest cause): Symmetric ascending paralysis b. Post diphtheritic paralysis: Symmetric descending 5. Neuromuscular: Botulism: (symmetric descending paralysis), myasthenia gravis

Answer 2

Acute viral infection of the anterior horn cells Transmission: is oral and droplet with man being the only reservoir The passive transplacental immunity remains for 6 months and after active immunity with vaccine it is life long (basically eradicated in Egypt)

Answer 3

Unapparent infection (95%) Abortive poliomyelitis (5-10%) Non paralytic Paralytic Encephalitic poliomyelitis

Answer 4

Acute paralysis of the lower motor neuron lesions; which is purely motor and no sensory affection as site of destruction is AHC or cranial nuclei Distribution is unilateral and if bilateral; it is asymmetrical. Mainly lower limbs and my extend to abdominal muscles, trunk and neck In some patients---> BULBAR AND RESPIRATORY AFFECTION Gradual incomplete recovery: Paralysis remains stationary for several weeks after which gradual incomplete recovery

Answer 5

Acute stage (first 2 wks): isolation and treatment of bulbar and respiratory affection Restoration stage (2 wks to 6 mo): physiotherapy Residual stage (after 6 mo): orthopedic correction of contractures

Answer 6

a. Cross reactivity: - There is molecular mimicry between streptococci and tissue antigens (myosin, vimentin) - Antibodies formed against streptococcal antigens react with human tissue antigens b. Genetic susceptibility: - Several genes responsible for the autoimmune reaction have been described recently Pathology - Proliferative lesions: Aschoff nodules that heal by fibrosis - Exudative lesion: Effusion that resolve without residual damage

Answer 7

1. Polyarthritis: (70 % of cases ) * Polyarticular * Migratory or fleeting and asymmetrical * Leaves the joint completely free * Dramatic response to salicylates 2. Carditis (50% of cases) a. Endocarditis (valvulitis) (most consistent) b. Myocarditis c. Pericarditis (rare) 3. Chorea (15% of cases) *Darting tongue *Speech abnormalities (dysarthria) *Milk-maid grip *Boat-shaped hands *Pendular knee reflex c. Emotional lability 4. Erythema marginatum (5%) 5. Subcutaneous nodules (2-10%)

Answer 8

Monoarthalgia Fever greater or equal to 38C Prolonged PR interval unless carditis is present as a major criterion Acute phase reactants: elevated ESR and CRP

Answer 9

Recent scarlet fever +ve throat culture Rapid antigen test Antistreptococcal antibodies: high titre of: ASOT Antistreptokinase Antihyaluronidase Anti-DNase

Answer 10

o Two major or One major and Two minor criteria AND o Evidence of recent streptococcal infection * Diagnosis of recurrent rheumatic fever depends on: As before or 3 minor criteria * Diagnosis based on 2 major criteria is stronger than that based on 1 major and 2 minors * Elevated ESR, elevated CRP and Leukocytosis are all considered as one minor criterion * Subclinical carditis: significant cardiac affection detected by echocardiography with absence of any murmur. Recently considered as a major criterion * Evidence of recent streptococcal infection is required for all cases except rheumatic chorea * In Egypt, an ASOT level >= 250-400 IU/ml is taken as positive

Answer 11

1. Primary prevention * Prevention of Streptococcal infection: Good housing and adequate ventilation * Tonsillectomy for frequent recurrence * Proper treatment of Streptococcal throat infection o IM Benzathine penicillin 1.200.000 IU once (Sensitivity skin test is essential) is the best treatment (Erythromycin) * Primary prevention is difficult because :- o 30% of streptococcal pharyngitis are subclinical o 30% of patients pass to RHD after subclinical carditis.

Answer 12

2. Secondary prevention (Prevention of recurrence of rheumatic fever) Indicted in all patients with documented history of rheumatic fever or isolated chorea Drug used a. Benzathine penicillin * IM: every 2-3 weeks (sensitivity skin test...) * Dose: 1.200,000 IU for weight 20 Kg and 600.000 IU for weight less than 20 Kg * Duration: 10 years after the last attack or till the age of 21 years whichever longer then reassess: a No RHD: stop prophylaxis b RHD: continue till the of 40 years or longer b. Oral penicillin 250 mg twice daily c. Penicillin sensitive patients: erythromycin 250 mg twice daily d. Oral sulphadiazine 0.5 gram once daily.

Answer 13

A) Antibiotics - IM Benzathine penicillin: 600.000-1.200.000 IU (Sensitivity skin test is essential) - Given to eradicate streptococci and serves as the 1st dose of penicillin prophylaxis B) Supportive Management a. Diet: Salt restriction in cases of heart failure and Fluid restriction in cases of severe heart failure b. Rest: For patients with arthritis, carditis or heart failure C) Specific Management a. Arthritis * Salicylates 100 mg/Kg/day , 4 times / day for 2 weeks followed by 75 mg/Kg/day, 3 times/ day for 2-3 weeks b. Carditis * Prednisone: 2 mg/Kg/day 4 times daily for 2 weeks with gradual tapering (Over 4 weeks) * Salicylates: 75 mg/Kg/day 3 times daily started with steroid tapering and continued for 6 weeks c. Chorea: * Phenobarbitone and Haloperidol

Answer 14

a. Heart failure: Fluid and restriction, Diuretic therapy, Digoxin therapy, ACE inhibitors b. Rheumatic heart disease: * Medical: rheumatic activity and infective endocarditis * Surgical: Valve repair or replacement

Answer 15

Streptococcus viridians (most common) Staphylococcus aureus Gram -ve bacteria HACEK High risk: -RHD: Lt sided valves, Rt sided valves, Regurge, Stenosis -CHD: Fallot, TGA, VSD, PDA, Coarctation -IV drug use -Prosthetic valve, surgical shunts -Previous IE -Cardiac transplantation

Answer 16

Dental procedures Adenotonsillectomy Non-sterile instrument of GIT Central venous catheters IV drug use

Answer 17

Turbulence of blood flow across stenotic or incompetent valves damages the endothelium--------> Platelets and fibrin adhesion form thrombi------>Circulating bacteria adhere and grow in these thrombi forming vegetations------>Valve destruction--------> Embolization of emboli to any organ in the body------->Septic emboli phenomena: osteomyelitis, meningitis, and glomerulonephritis---------> Immunologic response with deposition of circulating immune complex on various endothelial surfaces leading to vasculitis.

Answer 18

1. Fever, headache, anorexia, malaise 2. Pallor and loss of weight 3. Eye: Subconjunctival hemorrhage, retinal infarction 4. CNS: Embolic hemiplegia, ICH 5. Hands * Clubbing (Pale or toxic clubbing) * Osier's nodules: Pulps of fingers * Splinter hemorrhages: Under the nails * Janeway lesions: painless hemorrhagic lesion with necrotic center on the palms and soles * Pulse: may be absent (Embolization) 6. Splenomegaly (70% of cases) 7. Hematuria 8. Arthritis/arthralgia

Answer 19

1. Feature of the underlying cardiac disease 2. Appearance of a new murmur (Sea-gull murmur = rupture chorda tendinae) 3. Change in the character of an already present murmur 4. Heart failure due to valve damage

Answer 20

A) Laboratory * Blood culture (Repeated 3 times after proper skin decontamination) * CBC, ESR, CRP B) Imaging * CXR, ECG * ECHO (Vegetations) * Transesophageal echocardiography may be required in case of prosthetic valves

Answer 21

Positive blood culture: -Typical organism including, strept, staph, or HACEK -2 +ve cultures of 3 cultures drawn > 12 hours apart for other organism consistent with IE Evidence of endocarditis on ECHO: -New vegetations or abscess -New valve regurge or variation in the degree of regurge

Answer 22

Predisposing factors Single +ve blood culture Fever > 38 C +ve blood culture for atypical organism Vascular embolic manifestations: Splenic infarctions, cerebral infarctions or hemorrhage, renal infarctions, necrotic skin or gangrene, retinal infarction, conjunctival hemorrhage, Janeway lesions Immune complex deposition: -Glomerulonephritis -Roth spots (retinal hemorrhage) -Splinter hemorrhage -Osler nodules -Petechiae

Answer 23

Diagnosis is established with: * TWO major or * ONE major+ THREE minor or * FIVE minor

Answer 24

1. Proper oral hygiene 2. Oral, respiratory or dental procedures: * Oral or IV Amoxicillin single dose 50 mg/Kg; 1 hour before procedure * In allergic patients: Azithromycin 3. GIT (except esophageal) or genitourinary procedures: * IV ampicillin (50 mg/Kg) and IV gentamycin: 30 minutes before procedure * In allergic patients: azithromycin * 6 hours after procedure: oral amoxicillin (25 mg/Kg)

Answer 25

1. Prolonged parenteral therapy according to culture and sensitivity to kill all bacteria in the vegetations A. Empirical therapy till culture results 1. Anti-staphylococcal penicillin (methicillin) + Aminoglycoside (gentamycin) 2. Suspected methicillin resistant S.aureus: Vancomycin + gentamycin 3. Treat according to culture result * Amphotericin B for fungal infection. * Patients with prosthetic valves should be treated for 6 weeks according to culture 4. Surgical care: It carries a high mortality, indicated in certain situations as * Progressive cardiac failure * Worsening valve obstruction or regurge * Periventricular abscess * Fungal infections and failed response to medical treatment as with large vegetations

Answer 26

Hypertension: Systolic and /or diastolic BP >= 95th % for age, gender and height on at least 3 occasions -Renal HTN: renal parenchymal disease, renovascular (renal artery stenosis) -Coarctation of the aorta -Endocrinal causes: Cushing syndrome, Aldosterone excess -Catecholamine excess: Pheochromocytoma, neuroblastoma -Iatrogenic: Corticosteroids -Increased intracranial tension -Essential HTN -Pain

Answer 27

- Picture of the cause - Headache, vomiting, difficulty initiating sleep, daytime tiredness, - HTN encephalopathy: coma, convulsions - Failure to thrive and cardiac failure are the most common features in infants

Answer 28

1. Kidney function tests (Creatinine, BUN) 2. Uric acid, urine analysis and culture 3. Electrolytes (Na, K), 4. Fasting glucose 5. Fasting lipids profile 6. Abdominal sonar, echo, renal Doppler

Answer 29

- Treatment of the cause: coarctectomy - Life-style modification and Anti-hypertensive drugs: * Indications: Severe symptomatic HTN, secondary HTN, insufficient response to life style modification. * Preferred medications: Diuretics, B blockers, ACE-I and calcium channel blockers

Answer 30

1. The illness starts acutely, few days after the onset of nasopharyngitis 2. Fever and earache: Any febrile patient with unexplained irritability or excessive crying should be examined for otitis media. 3. Ear discharge (otorrhea): May occur with drum perforation. 4. Ear examination with otoscope reveals a congested bulging eardrum.

Answer 31

Acute cough (duration less than 2 weeks) 1. Without respiratory distress: Acute bronchitis, laryngitis, and sinusitis. 2. With respiratory distress: Acute bronchiolitis, pneumonia, and acute asthmatic attack. Prolonged cough (duration is 2 weeks to 4 weeks) 1. Complicated bronchitis (bacterial bronchitis, segmental collapse, pneumonia). 2. Sinusitis (due to postnasal discharge). 3. Pertussis (whooping cough) and pertussis-like illness. Chronic cough (duration more than 4 weeks) 1. Chronic infections (pulmonary tuberculosis, bronchiectasis). 2. Chronic or persistent asthma. 3. Recurrent aspiration.

Answer 32

1. Prodromal stage: Mild URI with nasal discharge and sneezing for few days. 2. Respiratory distress and expiratory wheezing: Manifestations of respiratory distress (rapid respiration and retractions) with paroxysmal wheezy cough become evident. Chest auscultation reveals expiratory wheezing. This stage usually lasts for few days. 3. Rapid recovery usually occurs within few days. The case fatality rate is less than 1%. Investigations: 1. Chest X-ray: hyperinflation of the lungs with focal atelectasis. 2. Blood gas analysis: hypoxia- CO2 retention. 3. RSV antigen detection from nasopharyngeal secretions.

Answer 33

1. Infants with minimal or mild respiratory distress (at home) a) Close observation: Increasing distress is an indication for hospitalization. b) Drugs as cough medicines and bronchodilators are generally not helpful. c) Careful feeding to avoid aspiration. 2. Infants with moderate to severe respiratory distress (at hospital) a. Oxygen therapy to correct hypoxemia. b. I.V maintenance fluid therapy to prevent dehydration. c. Nebulized salbutamol may be used and some infants may benefit. d. Corticosteroids are not beneficial and antiviral agents (as ribavirin) are not necessary.

Answer 34

Infectious pneumonia 1. Bacterial: - Gram +ve: pneumococci, streptococci, staphylococci. - Gram -ve: H. influenza, klebsiella, pseudomonas - TB 2. Parasitic: Loffler's pneumonia 3. Viral: RSV (most common) Adenovirus 4. Mycotic: - Aspergillosis - Candidiasis 5. Other microorganisms:- Actinomyces - Pneumocystis carnii- - Chlamydia and Mycoplasma Non infectious pneumonia 1. Aspiration and chemical 2. Hypostatic pneumonia

Answer 35

under 1 m: Group B streptococcus 1m to 3m: Respiratory syncytial virus, Streptococcus pneumoniae 4m to 4y: Respiratory syncytial virus, Streptococcus pneumoniae = >5 years: Mycoplasma pneumoniae

Answer 36

1. Lobar ■ Unilateral affection of one or more lobes. ■ Etiology: It is mostly bacterial ■ Chest x-ray shows lobar consolidation 2. Bronchopneumonia ■ Bilateral affection of both lungs with small foci. ■ Etiology: bacterial or viral. ■ Chest x-ray shows fine nodular or patchy infiltration. 3. Interstitial ■ Bilateral affection of interstitial lung tissues. ■ Etiology: mostly viral. ■ Chest x-ray shows parahilar shadow with radiating streaks

Answer 37

a. Symptoms -Pneumonia should be suspected in every case of respiratory distress, fever and cough -Difficult feeding and referred pain (neck or abdomen) b. Signs - Manifestations of respiratory distress: Bacterial comes with more severe fever and highly elevated ESR, severe course and mainly lobar. Viral is mild, less liable to complicate and is mainly interstital - According to pathological types a. Lobar pneumonia: Bronchial breathing over the involved lobe is the main finding. Some dullness to percussion over the involved lobe may be present. (Bacterial) b. Bronchopneumonia: Fine bilateral consonating crepitations are the main finding. c. Interstitial pneumonia: Severe spasmodic cough and tendency to expiratory wheezing are the main findings. (viral)

Answer 38

1. Respiratory failure: the most serious complication and the main cause of death. 2. Pleural effusion with bacterial pneumonias especially pneumococcal,staphylococcal pneumonia. 3. Lung abscess and pneumatoceles with bacterial pneumonia especially staphylococcal pneumonia. 4. Myocarditis and acute heart failure especially in infants with severe bacterial pneumonia.

Answer 39

A. Hospital management (7-10 days): 1. Indications - Severe pneumonia (severe RD) or complicated pneumonia - Small infants (Less than 6 months) 2. Supportive measures: Humidified oxygen - IV fluid (NPO) - suction 3. Specific treatment: Broad spectrum combined parenteral antibiotics to cover G+/( Ampicillin 50- 100 mg/kg/day+ gentamycin 4-6 mg/kg/day). 4. Treatment of complication: Drainage of empyema. Mechanical ventilation (in respiratory failure) B. Home management for most cases: 1. In Older children with mild pneumonia without distress. 2. Oral or better intramuscular antibiotics. 3. Amoxicillin 50 mg/kg/day for 7-10 days.

Answer 40

1. General symptoms: Loss of appetite, loss of weight, night sweat and night fever. 2. Chronic cough: It is the main symptom. Sputum may be mucoid, purulent or blood stained. The patient may complain of localized wheezing, recurrent colds or pneumonia for a number of months before diagnosis, with no response to routine treatment. 3. Chest signs: They vary according to the pathological lesion: a. In pneumonic lesions, there may be signs of consolidation. b. In pleural effusion, physical signs of effusion may be detected. c. In fibrosis, deviation of the trachea and mediastinum to the same side occurs. d. In compression of the trachea and bronchi by tuberculous lymph nodes, wheezes may be noticed and the condition should be differentiated from bronchial asthma.

Answer 41

1. CBC: Lymphocytosis 2. ESR: Very high ESR usually above 100. 3. Tuberculin test: Mantoux test is the most important immunological diagnostic tool. less than 5 mm is -ve. 5-9 is suspicious. 10 or more is +ve (in infection or vaccination) 4. Isolation and culture of organism: □ Get sputum or morning gastric aspirate □ Direct smear with ZN stain □ Culture on a Lowenstein Jensen medium (4 weeks) * Quantiferon TB test * Radiological studies: Chest x-ray and CT scan * Recent methods for diagnosis: Usage of ELISA and PCR (polymerase chain reaction).

Answer 42

1. Good nutrition, good housing and better aeration 2. Elimination of TB in cattle and pasteurization of milk. 3. Mass radiography centers for early diagnosis and treatment 4. Repeated examination and radiography of employees who deal with children in hospitals, schools and nurseries to detect diseased persons. 5. BCG Vaccination: - This is the cheapest and most effective way for prevention of TB. - It should be administered as early as possible to infants 6. Chemoprophylaxis: When an individual has to live near a tuberculous contact for variable periods, isoniazid is used

Answer 43

- Drugs are classified into first and second lines or alternative drugs. Antituberculous regimen o In the first 2 months: isoniazid + rifampicin + pyrazinamide + Ethambutol o In the following 4 months: isoniazid + rifampicin only. Alternative drugs Streptomycin (STM), I.M. Ethionamide (ETH)

Answer 44

1. Allergy to antituberculous drugs. 2. TB serositis 3. In miliary TB affecting the suprarenal glands. 4. In endobronchial TB to avoid lung collapse. 5. After surgical removal of cervical TB lymph nodes

Answer 45

1. TB lymphadenitis 2. Skeletal TB * TB of the spine (Pott's disease) * TB arthritis 3. Abdominal TB * TB enteritis (primary or secondary) * TB peritonitis * Tuberculous mesenteric lymphadenitis (Tabes mesentrica) 4. TB of the CNS * TB meningitis 5. Other types * TB pericarditis * TB of the skin {lupus vulgaris) * TB of the eve and ear

Answer 46

Asthma should not be confused with other causes of wheezing and should never be diagnosed on the basis of single wheezing episode common causes of persistent or recurrent wheezing: 1. Bronchial asthma (the commonest cause of wheezing in children). 2. Recurrent aspiration syndromes 3. Foreign body inhalation 4. Chronic or recurrent infections 5. Congenital anomalies or compression of the airways 6. Bronchiectasis 7. Laryngotracheobronchomalacia 8. Primary ciliary dyskinesia 9. Congenital heart disease 10. Vocal cord dysfunction

Answer 47

* Airway obstruction in asthma is due to: (1) Bronchoconstriction (2) Hyper-secretion of mucus (3) Mucosal edema (4) Cellular infiltration with inflammatory cells and desquamation of bronchial epithelial cells * Obstruction is most severe during expiration (airways normally are narrower in expiration) * Airway obstruction leads to: (1) Areas of hyperinflation (2) Areas of segmental atelectasis (3) Ventilation-perfusion mismatch (4) Alveolar hypoventilation (leading to hypoxia, hypercarbia and acidosis)

Answer 48

1. More than one symptom: wheeze, shortness of breath, cough, chest tightness 2. Symptoms are paroxysmal with symptom free intervals 3. Symptoms often worse at night or in the early morning 4. Symptoms vary over time and in intensity 5. Symptoms are triggered by viral infections (colds), exercise, allergen exposure, changes in weather, laughter, or irritants such as car exhaust fumes, smoke or strong smell 6. Wheeze and distress precipitated by a trigger and relieved with bronchodilators. 7. History of other atopic manifestations in the child e.g. allergic rhinitis, skin, eye allergy or family history of atopy. Physical Examination 1. No findings in symptom free intervals. 2. Prolonged expiration ± expiratory rhonchi in symptomatic periods according to severity

Answer 49

Objective measurement of airflow obstruction (using peak expiratory flow meter (PEFM) or — spirometry pre and post bronchodilation) Assessment of atopy (skin prick tests, serum total or specific IgE) Others: chest X-ray, peripheral blood eosinophilia.

Answer 50

INITIAL ASSESSMENT:A: airway B: breathing C: circulation if: Fatal: ICU, Oxygen, SABA and prepare for intubation Mild- Moderate: Oxygen, SABA, oral corticosteroids, ipratropium bromide Severe: Oxygen, SABA, high dose corticosteroids, ipratropium bromide

Answer 51

a. Hospitalization in ICU b. Oxygen therapy c. I.V. fluid therapy d. Drug therapy o Nebulized salbutamol o I.V salbutamol o I.V methyl prednisolone o I.V Magnesium sulphate o I.V. aminophylline e. Mechanical ventilation: With marked CO2 retention, severe hypoxemia, severe acidosis or disturbed consciousness.

Answer 52

Inhaled corticosteroids and oral montelukast are the most effective. Long term control medications for asthma 1. Inhaled glucocorticosteroids. 2. Leukotriene receptor antagonists - LTRA ( montelukast). 3. Long-acting inhaled B2-agonists - LABA (Salmeterol- Formeterol). 4. Systemic glucocorticosteroids- (OCS). 5. Anti-lgE , anti-interleukin 5 (anti -IL5). 6. Long acting anticholenergic agents e.g. Tiotropium . 7. mast cell stabilizers 8. Immunotherapy is indicated in certain atopic patients IgE-sensitization towards clinically relevant one (not more than three) relevant inhalant allergen associated with consistent symptoms of allergic rhinitis/conjunctivitis and/or allergic asthma. Immunotherapy should not be given to children less than 5 years of age and should be given under the supervision of an allergist.

Answer 53

1. Icteric hepatitis: it is the commonest form(3 stages) a. Pre-icteric stage: 4-6 days * Fever, anorexia, headache and malaise. * Abdominal pain and vomiting. * Urine usually becomes dark(bilirubinemia) during the last 1-3 days b. Icteric stage: 2-4 weeks * Jaundice, dark urine * Liver is enlarged and tender. * The early manifestations as fever, vomiting disappear but anorexia may continue c. Convalescence stage gradual decline of jaundice and decrease in liver size 2. Cholestatic hepatitis * Marked obstruction of biliary flow—>Pruritus (bile acids) and marked clay colored stool. 3. Anicteric hepatitis * Commoner in infancy. * Jaundice is absent, presentations include: anorexia, vomiting, diarrhea and colic 4. Fulminant hepatitis: Acute liver failure (the least common but the most serious: 70% mortality)

Answer 54

To diagnose liver cell injury 1. Bilirubin: direct or mixed hyperbilirubinemia. 2. AST and ALT are elevated (10 folds). 3. Urine analysis: bilirubin is present. To diagnose acute hepatic failure 1. Raising Bilirubin level 2. Liver transaminases (ALT, AST) are increased (10 -100 times normal). 3. Low serum albumin 4. INR (international normalized ratio) >= 2, 5. High blood ammonia level 6. Metabolic Acidosis. 7. Hypoglycemia, Hypokalemia and Hyponatremia.

Answer 55

1. Hepatitis A * IgM antibodies to hepatitis A (anti-HAV IgM): recent infection(acute disease). * IgG anti HAV antibodies persist after recovery (immunity). 2. Hepatitis B * HBsAg (surface antigen) followed by anti-HBc (core antibodies) IgM: indicate recent infection. * Anti-HBs (surface antibodies) appear after recovery and indicate immunity. * Persistence of HBsAg (surface antigen) and anti-HBc IgG indicate chronicity. 3. Hepatitis C * Anti-HCV antibodies: these antibodies denote exposure to infection but do not mean recovery or development of immunity. * HCV- RNA antigen detected by PCR denotes viremia * Viral load can be assessed by quantitative PCR for treatment purposes 4. Hepatitis D, and E * Anti - HDV and anti HEV antibodies (IgM). * Hepatitis D virus antigen

Answer 56

Pre-hepatic: -Portal vein thrombosis due to thrombosis: via umbilical sepsis or umbilical vein catheterization and congenital portal vein obstruction. Hepatic: -Presinusoidal: congenital hepatic fibrosis and bilharziasis -Sinusoidal: causes of liver cirrhosis and cholestasis -Post sinusoidal: central vein thrombosis (veno-occlusive disease) Post hepatic: -Budd-chiari syndrome -Constrictive pericarditis

Answer 57

1. Upper GIT endoscopy: detect esophageal varices 2. Abdominal ultrasonography and Doppler: * Direction of flow within the portal system * Patency of the portal vein and * Presence of portosystemic collaterals. 3. CT angiography and MR venography (demonstrate vessel patency) 4. Liver function test 5. Investigation for the cause: * Hepatitis markers * autoimmune screening * sweet chloride test * liver biopsy

Answer 58

1. Emergency therapy for bleeding varices:- o Hospitalization: Anti-shock measures: blood transfusion, intravenous fluids. o Correction of coagulopathy: vitamin k, fresh plasma, platelets transfusion, o Nasogastric tube placement. o H2 receptor blocker (ranitidine) IV to decrease risk of bleeding from gastric erosion o Third generation cephalosporins. o Vasopressin infusion if bleeding persists. 2. Emergency endoscopy (if hemodynamically stable): and either injection sclerotherapy or band ligation. 3. Emergency shunt: o Trans jugular intrahepatic Porto-systemic shunt (TIPSSJ o Surgical Porto-systemic shunts Prevention of bleeding from varices 1. Prevention of the first attack of bleeding o Avoid aspirin and non-steroidal anti-inflammatory drugs, o B adrenergic blockers (Propranolol) to lower the pressure in portal area, o Prophylactic sclerotherapy or band ligation. 2. Prevention of re-bleeding; in addition to the above, the following may needed: o Beta adrenergic blockers (propranolol) o Endoscopic sclerotherapy or band ligation o Surgical porto-systemic shunt o Liver transplantation

Answer 59

1- Idiopathic neonatal hepatitis (giant cell hepatitis): The most common cause (with IUGR) 2- Infections: a. Bacterial: neonatal sepsis, urinary tract infection b. Viral: CMV, Rubella c. Protozoal: toxoplasma 3. Metabolic: a. CHO metabolism: Galactosemia b. Amino acid metabolism: Tyrosinemia (early liver cell failure) c. Lipid storage disease: Niemann-Pick disease (cherry red spots in fundus examination) d. Alpha-1- antitrypsin deficiency e. Bile acid biosynthetic defects 4- Familial cholestatic syndromes: a. Paucity of intrahepatic bile ducts b. Alagille syndrome: Paucity of intrahepatic bile ducts c. Progressive familial intrahepatic cholestasis (PFIC) d. Benign recurrent cholestasis 5- Extrahepatic biliary atresia: The 2nd most common cause (with normal birth weight) 6- Choledochal cyst

Answer 60

1. Chronic viral hepatitis(B,C,D) 2. Autoimmune chronic hepatitis. 3. Drug induced (INH, Rifampicin). 4. Metabolic disease: Wilson disease , alpha 1 antitrypsin 5. Chronic inflammatory bowel disease e.g. ulcerative colitis. 6. Nonalcoholic fatty liver disease

Answer 61

1. Primary hypothyroidism (congenital and acquired) -Congenital hypothyroidism a) Maldevelopment and mal-descent of thyroid gland (nongoitrous) * Agenesis (aplasia) is the most common, Dysgenesis (hypoplasia) and ectopic. b) Dysmorphogenesis(goitrous): Autosomal recessive inborn error of hormonal synthesis c) Iodine deficiency (endemic goiter): in Sinai and Oasis d) Antenatal maternal intake of goitrogens: antithyroid drugs -Acquired hypothyroidism a) Hashimoto thyroiditis: autoimmune disease 2. Secondary hypothyroidism * Isolated TSH deficiency is rare (less than 1% of cases) and is usually associated with pituitary dysfunction. * It usually manifests by Growth hormone, gonadotrophin and adrenocorticotrophic hormone (ACTH) deficiencies leading to hypoglycemia or micropenis and undescended testes

Answer 62

* At birth: no symptoms due to Trans placental passage of maternal T4 (thyroxin). * Later on: early weeks of life: History 1. Prolonged gestation period may be noticed 2. Prolonged jaundice. 3. Poor feeding 4. Crying is little with much sleep (decreased activity) 5. Constipation Examination 1. Fontanels: Widely opened anterior and posterior fontanels 2. Failure to thrive 3. Hypothermia (cold, mottled skin) and slow pulse 4. Hypotonia: abdominal distention and umbilical hernia In older kids: motor, mental and physical retardation. Opened anterior fontanelle, delayed teething, coarse and brittle hair, wide opened mouth with large protruded tongue

Answer 63

Laboratory: Thyroid profile: T3, T4 and T.S.H. 1. Low T4 2. High TSH. It is the most sensitive test for primary hypothyroidism. Imaging 1. Plain X ray: delayed bone age. it is characteristic for congenital hypothyroidism 2. Thyroid scanning (radioactive iodine : Iodine 123) it is essential for diagnosis of the cause (it can differentiate between aplasia, ectopic dysplasia, and malfunction of the thyroid gland) 3. Thyroid Ultrasonography: Neonatal thyroid screening It is implemented in Egypt for all newborns to prevent mental retardation. Between 3nd and 7th day of life.

Answer 64

1. Known diabetic child who appears sick and/or has any of the precipitating factors but DKA can be the 1st presentation of diabetes 2. Preceding symptoms: Weight loss - polyuria - polydipsia * Early manifestations ■ Vomiting and abdominal pain (excretion of ketone bodies in the stomach) * Late manifestations (complications) ■ Dehydration and may be shock: due to vomiting and polyuria. Deep rapid breathing due to metabolic acidosis + acetone odor of breath ■ Drowsiness due to cerebral depression and may be coma ■ Depression and arrhythmias of the heart from acidosis and electrolytes disturbances

Answer 65

1. Blood glucose: hyperglycemia (blood sugar above 200 mg/dl) 2. Blood gas analysis: metabolic acidosis (pH<7.3, bicarbonate <15 mEq/L) 3. Urine analysis: glucosuria and ketonuria 4. Urea, creatinine and electrolytes (especially potassium) 5. Evidence of a precipitating cause e.g. infection (blood picture, blood and urine cultures)

Answer 66

1. Initial measures A: Open the airway. B: Oxygen therapy. C: IV line. 2- Fluid therapy: a) Resuscitation (Shock therapy): 10 ml/Kg normal saline in 30-60 min. b) Deficit therapy: according to the degree of dehydration c) Maintenance: According to body weight or surface area ( if glucose < 300 then add glucose) 3- Insulin therapy (O.lunit/kg/h slow infusion) to avoid brain edema. No boluses 4- Potassium: 30-40 mEq/L after the resuscitation, after the first hour and after the patient passes urine provided K is not elevated. 5- Monitoring: ■ Hourly capillary blood glucose (strips) ■ Plasma glucose, Na, K, blood gases and urinary ketones ■ Renal functions, CBC and sepsis screen if suspected 6- Acidosis: Don't use Bicarbonate This is because: a) Most cases will be self-corrected by fluids and insulin b) Bicarbonate can exacerbate hyperosmolarity and electrolyte disturbance c) to avoid the risk of alkalosis 7- Treat Complications: Shock (if septic then Antibiotics), brain edema (by mannitol), pulmonary edema (by mannitol), and arrythmia (K monitoring).

Answer 67

a. According to the duration: * Transient: Fever, exercise, seizures. Common on random urinalysis * Persistent: Orthostatic (Postural) Proteinuria b. According to the origin: * Glomerular: Nephrotic syndrome, glomerulonephritis * Tubular: Acute tubular necrosis, tubular disorders as cystinosis

Answer 68

1. Generalized edema: * Site of onset: Periorbital (more apparent in morning and decreases by the end of the day) * March: Then becomes generalized (LL, ascites, pleural effusion, scrotal/ vulval edema) * Character: Pitting 2. No persistent hypertension, No hematuria 3. Nephrotic syndrome has a relapsing nature; often triggered by upper respiratory tract infection

Answer 69

1. Infection (The major complication) Predisposing Factors: * Edema, ascites "culture medium" * Urinary loss of Immunoglobulins * Steroid and immunosuppressive therapy Organisms: Pneumococci and Staphylococci are the most common Site of infection: * Peritonitis—>acute abdominal pain * Sepsis, pneumonia, cellulitis, UTI 2. Thromboembolic events: Predisposing Factors: * Hemoconcentration * Hyperlipidemia * Urinary loss of natural anticoagulants (Antithrombin III, Protein C & S) Site: * Deep venous thrombosis (DVT) * Pulmonary embolism 3. Hypovolemia: Precipitated by aggressive diuresis or diarrhea 4. Other complications: Respiratory distress, abdominal pain & dyslipidemia

Answer 70

A) Laboratory a. Urine * Urinalysis: Proteinuria (3+ or 4+) * Urine protein/Creatinine ratio> 2 * 24 hour urine proteins: > 40 mg/m2 / hr (needs timed urine collection which is difficult) * Proteinuria is selective (mainly albumin ,no high molecular weight proteins) b. Blood * Low Serum albumin * High Serum cholesterol * Kidney functions: Normal * Complement (C3 &C4): Normal (No consumption) B) Imaging: Renal U/S C) Invasive: Renal biopsy (not routine);

Answer 71

a. Pre-treatment (when Minimal change nephrotic is unlikely): * Age at onset < 1 yr or > 10 yrs * Gross hematuria * Persistent hypertension * Renal impairment * Hypocomplementemia (decreased C3 and/or C4) * Family history of NS b. Steroid resistant NS (SRNS) = Failure to achieve remission after 4-6 weeks of steroid therapy

Answer 72

a. Diet: * Salt restriction * Fluid restriction in cases of severe hyponatremia * Lipid restriction * Protein intake: Normal intake or mildly increased b. Edema * Mild: Salt restriction * Moderate: Diuretics; Furosemide (1-2 mg/Kg/day) * Severe: Salt-free albumin+ Furosemide (1-2 mg/Kg/day) c. Infection: Antibiotics (3rd generation cephalosporin) 3. Specific Management Induction: Prednisone (2 mg/Kg/day); using dry not edematous weight * If the child is steroid-responsive (Maintenance): o Shift to alternate-day therapy (2 mg/Kg/day Single morning dose) o With gradual tapering over 3 months * If the child is steroid resistant: non minimal lesion o Do renal biopsy o Shift prednisone to alternate-day therapy with gradual tapering o Add angiotensin-converting enzyme inhibitors: adjuvant to reduce proteinuria o Add other immunosuppressive drugs: Cyclosporine and Tacrolimus

Answer 73

Immune Complex Deposition or Autoimmunity: -Often triggered by infections (e.g., post-streptococcal glomerulonephritis), autoimmune diseases (like lupus), or vasculitis (e.g., ANCA-associated). -Immune complexes deposit in the glomerular basement membrane (GBM) o, activating complement and recruiting inflammatory cells (neutrophils, monocytes). Inflammation of the Glomeruli: -The immune response causes swelling, proliferation of glomerular cells, and infiltration of inflammatory cells. -Capillary lumens become narrowed or blocked, reducing glomerular filtration rate (GFR): Capillary Wall Damage Disruption of the GBM and endothelium allows: -Red blood cells to leak into the urine → hematuria -Mild to moderate protein loss → subnephrotic-range proteinuria -Retention of sodium and water → hypertension and edema Tubular Effects: -RBCs in the tubules form RBC casts, a hallmark of glomerular hematuria. -Tubules may also be affected secondarily by protein overload or ischemia.

Answer 74

Clinical picture (Peak incidence= 5-12 years) A) Acute nephritic syndrome * History of preceding streptococcal infection (Pharyngitis or skin) 1-6 weeks ago * Hematuria: gross painless, brown, smoky, tea-like or cola-like * Oliguria: due to decreased GFR * Hypertension (60% of cases) * Edema: usually mild (may be generalized if HF or renal failure) B) Heart failure: Secondary to HTN or hypervolemia Tachycardia, Tachypnea, Tender liver C) Hypertensive encephalopathy Blurred vision, severe headache, altered mental status, coma & convulsions

Answer 75

1. Laboratory a. Urine (Urinalysis): Proteinuria (mild to moderate) Color: -Brown, tea or cola-like or smoky -(RBCs cast and deformed RBCs under microscpe) b. Blood * KFT (Urea & creatinine): may be impaired * Evidence of recent Streptococcal infection (elevated ASOT, throat or skin contact) * Decreased C3 (returns to normal within 8 weeks) * Normal C4 2. Imaging: Renal U/S 3. Invasive: Renal biopsy is rarely indicated

Answer 76

a. Severe renal impairment (Rapidly progressive GN = RPGN) b. Persistent hematuria or proteinuria > 6-12 months c. Normal C3 d. Persistent hypocomplementemia > 3 months

Answer 77

2. General measures a. Diet: * Salt restriction * Fluid balance: Intake = insensible + urine output (Insensible water loss = 400cc/m2/day) b. Rest: During the oliguric phase 3. Supportive treatment a. Edema: Salt restriction, fluid balance, diuretics (Furosemide) b. Hypertension: * Salt restriction, fluid balance, diuretics (Furosemide) * Ca channel blockers (Amlodipine) 4. Treatment of complications a. Renal failure: Fluid balance, diuretics, dialysis (in severe cases) b. Heart failure: * Preload reduction: Diuretics * Afterload reduction: ACE inhibitors * Inotropes: Dopamine (digitalis should be avoided) c. Hypertensive encephalopathy: Antihypertensive (IV Hydralazine)

Answer 78

Pre-renal: -Hypovolemia (hemorrhage, dehydration, diarrhea) -Hypotension (shock all types) Intrinsic Renal: -Acute Tubular Necrosis ■ Uncorrected prerenal or postrenal ARF ■ Drugs: aminoglycosides -Acute Glomerulonephritis -Vascular: Renal thrombosis, hemolytic uremic syndrome -Intrinsic nephritis: by nephrotoxic drugs Post-renal (obstruction uropathy) -Congenital obstruction: PUV and stricture -Acquired obstruction: stones and tumor -Functional obstruction: Vesicoureteric reflux

Answer 79

1. Congenital anomalies * Aplasia & hypoplasia * Obstructive uropathy (Stricture, posterior urethral valve ... ) 2. Urolithiasis (renal stones) 3. Genetic nephrotic syndrome: (e.g. congenital nephrotic syndrome) 4. Cystic kidney diseases: Autosomal recessive & dominant polycystic kidney disease (ARPKD& ADPKD), nephronophthisis ... 5. Focal segmental glomerulo sclerosis 6. Chronic glomerulonephritis: Lupus nephritis ... 7. Chronic pyelonephritis (e.g., 2ry to VUR): Reflux nephropathy "Scarred kidneys" 8. Metabolic disorders

Answer 80

1. Growth failure & short stature 2. Delayed puberty 3. Rickets (Non-nutritional, renal rickets} 4. Anorexia & vomiting 5. Chronic unexplained anemia 6. Chronic unexplained hypertension 7. Polyuria, polydipsia and 2ry enuresis

Answer 81

1. Laboratory a. Urine analysis b. Blood * KFTs (Urea & Creatinine): Impaired in stages 2-5 * Hyperphosphamemia & hypocalcemia * Blood gases: Metabolic acidosis * CBC: anemia 2. Imaging * Renal US: Atrophic kidneys, renal cysts, stones ... * X ray: Long bones (Rickets), Left wrist (Delayed bone age) 3. Invasive: Renal biopsy may show picture of the cause only early in the disease

Answer 82

A. DYSHEMATOPOIETIC ANEMIA * Iron deficiency anemia * Folic acid &Vitamin B12 (megaloblastic anemia). B. BONE MARROW FAILURE 1. Pure red cell aplasia * Inherited: Diamond- Blackfan anemia * Acquired: with Parvovirus B19 infection 2. Aplastic anemia (pancytopenia) * Congenital: e.g. Fanconi anemia * Acquired: idiopathic or secondary to infections: (hepatitis) -Toxins (insecticide) - irradiation 3. Infiltration of bone marrow: Malignant cell e.g. Leukemia

Answer 83

A. CORPUSCULAR DEFECTS [HEREDITARY] 1. Membrane defect: * Spherocytosis * Elliptocytosis 2. Enzyme defect: * G6PD deficiency(A) * Pyruvate kinase def. 3. Hemoglobin defect: * Quantitative: Thalassemia * Qualitative: Sickle cell anemia. B. EXTRA CORPUSCULAR (EXTRINSIC) [ACQUIRED] 1. Immunologic disorders -Rh &AB0 incompatibility Autoimmune hemolytic anemia: -Idiopathic -Infections (e.g. EBV, CMV) -Drug-induced (e.g.methyldopa, penicillin) -Collagen vascular diseases (SLE) Non-immunologic disorders -Hemolytic uremic syndrome -Hypersplenism (leads to pancytopenia) -Sepsis -Malaria -Wilson disease -Artificial valve -DIC

Answer 84

1. Diminished stores - Anemic mother with deficient iron supplementation - Premature and twins 2. Deficient dietary iron intake - Prolonged breast feeding (low iron content but 50% absorbed) - Excessive Cow milk (higher iron content but 10% absorbed - Iron poor diet - Protein energy malnutrition 3. Diminished absorption: -Chronic diarrhea -Malabsorption 4. Blood loss: -Chronic hemorrhage -Ankylostoma -Schistosomiasis -Cow milk allergy 5. Increased requirements: In (adolescents especially girls- acute hemorrhage)

Answer 85

Blood picture: - Low Hb. - Microcytic hypochromic anemia - Color index= Hb% divided by (RBC X2). It will be below 1 - Reticulocytic count is normal. It shows mild increase with therapy. Blood chemistry: - Low serum iron < 50mcg % (normal: 90 -150 pg/dl) - Low serum ferritin < 10 ng (normal: 30 -150 pg/ml) - Increased iron binding capacity (normal: 250 -350 pg/dl). Detect the cause - Adequate clinical history to discover dietary problems. - Focused and systemic clinical examination to rule out other causes of anemia - Stool analysis: to detect Ankylostoma - blood in stool - bilhariziasis - Endoscopy might be indicated: to exclude peptic ulcer or chronic H-Pylori infection

Answer 86

1. Adequate supply of iron to pregnant female. 2. Making powdered formula well-fortified with iron 3. Avoid cow milk introduction in the first year of life 4. Prophylactic iron therapy to premature. 5. Proper weaning by supplying iron containing foods 6. Provision of appropriate amount of iron rich food for infants and children according to their age and economic resources

Answer 87

Treatment of the cause first * Oral iron therapy: - Ferrous sulfate or gluconate 3-6 mg/kg elemental iron - Iron supplementation should be continued until the Hb is normal and then for a minimum of a further 3 months to replenish the iron stores. * Parenteral iron preparations: Indications: poor compliance or malabsorption. Oral therapy is otherwise as fast, as effective, much less expensive and less toxic. * Diet: Rich in iron (Meat, liver, green vegetables) and vitamin C. Packed RBCs transfusion should never be necessary for dietary iron deficiency. Even children with an Hb as low as 6 g/dl to 7 g/dl due to iron deficiency can tolerate it.

Answer 88

BY 1st DAY: Reduced irritability, improved appetite By 2nd day: Erythroid hyperplasia in Bone marrow, show erythroid hyperplasia By 3rd day: Reticulocytosis peaking at 5-7 days (good therapeutic test) By 1st month: elevated hemoglobin 0.4- lgm/dl 4-6 weeks: Increased Stores.

Answer 89

Beta thalassemia major (Cooley's anemia): Early development of hypersplenism CBC: low Hb, microcytosis, anisocytosis, target cells, poikilocytosis Hb electrophoresis: affected child: Hb F is markedly elevated (10-90%) with reduced Hb A. Sickle cell anemia: Blood film: Sickling Characteristic sickle RBCs in blood film under low 02 tensions. Hb electrophoresis: Hb S is present(> 50%) no Hb A may lead to Vaso-occlusive crisis and Autosplenectomy Hereditary Spherocytosis: defect in the red cell membrane protein, mainly Spectrin, Ankyrin or band 3. Abnormal osmotic fragility test Abnormal acidified glycerol lysis test

Answer 90

G6PD deficiency: Specific clinical picture: History of first intake of beans Specific Investigations: Heinz bodies ,G6PD assay Autoimmune Hemolytic Anemia: Specific clinical picture: History of Drug intake - infection within 2 wks. May be Associated arthritis -skin rash Specific Investigations: Positive direct antiglobulin test (Coomb's test) Hemolytic uremic syndrome (HUS): Specific clinical picture: History of severe gastroenteritis,Hemolytic anemia,Purpura may develop due to thrombocytopenia Specific Investigations: Thrombocytopenia, Elevated renal function Infection (Malaria): Specific clinical picture: Traveling to endemic area, Pattern of fever Specific Investigations: Blood film is diagnostic Sepsis: Specific clinical picture: Toxic patient (septicemia), Purpuric eruption. Shift to Lt, increased ESR, CRP

Answer 91

Heme causes: Hemolytic anemias, Leukemias , Lymphomas (Hodgkin and non hodgkin), Hypersplenism Infection: Malaria, CMV, EBV, AIDS, bacterial endocarditis. Congestive: Portal HTN and Rt sided HF Inflammatory: SLE and juvenile idiopathic arthritis Metabolic storage disorder: Neiman-peick disease

Answer 92

(1) Congenital (inherited): * Fanconi anemia: most common * Dyskeratosis congenital: with dysgenesis in skin & nails (2) Acquired: * Idiopathic: the most common (70%) * Secondary to: - Viral Infection (EBV - hepatitis viruses) - Exposure to radiation - Exposure to toxins as benzene - Drugs: Chemotherapy, chloramphenicol, sulphonamides

Answer 93

* Anemia * Purpura * Fever: persistent fever resistant to treatment - persistent oral fungal infection * Specific picture of the cause: a. Fanconi anemia: - Autosomal recessive - bone marrow failure which usually appear around the age of 5 years - Congenital anomalies: microcephaly, microphthalmia, short stature, absent thumb, absent radius, renal malformations, pigmented skin lesions b. Acquired aplastic anemia: - Spontaneous onset of pallor, purpura and recurrent infections

Answer 94

a. Fanconi anemia: * CBC will show pancytopenia * Bone marrow aspirate and biopsy will show hypocellular bone marrow * Chromosomal breakage study (spontaneous and induced): increased chromosomal breaks of peripheral blood lymphocytes. * Skeletal survey might show absent thumb or radius * abdominal U/S might show renal anomalies Acquired aplastic anemia: * CBC will show pancytopenia * Bone marrow aspirate and biopsy will show hypocellular bone marrow * Chromosomal breakage study will be normal

Answer 95

a. Fanconi anemia: * Supportive therapy: (controlling anemia - infection - bleeding) * Bone marrow transplantation from normal, HLA- matched donor is the only curative treatment b. Acquired aplastic anemia: Supportive therapy: (controlling anemia - infection - bleeding) * Mild: Immunosuppressive therapy (Anti-thymocyte globulin (ATG) and/ or cyclosporine) * Moderate and Severe cases: Bone marrow transplantation (BMT) is the treatment of choice from HLA matched sib

Answer 96

1- Non-Thrombocytopenic Purpura: a. Small B.Vs. defects - Infections as meningococcemia - Vitamin C deficiency (scurvy) - Inherited: Ehlar Danlos syndrome - Marfan syndrome - Immune vasculitis (Henoch Schonlein puprpura) b. Defective platelet function (Qualitative) * Inherited: - Giant platelet syndrome * Acquired: - Uremia - NSAIDs 2- Thrombocytopenic Purpura a. increased Destruction: * Immune: - ITP (the commonest) - Systemic lupus erythematosus * Non-immune: - DIC - Hypersplenism b. decreased Production * Inherited: - Fanconi anemia * Acquired: - As a part of acquired aplastic A. - B.M. infiltration (e.g. leukemia) - Drugs (aplasia of megakaryocytes) - Nutritional (decreased B12 & folic acid)

Answer 97

* Onset: 1-2 weeks after viral illness (age 2- 10 years) * SC bleeding in the skin: - Non-blanching not raised purple in color then change within days to green then brown then fade - Variable size: petechiae (spots <3 mm), purpura (3-10mm) or ecchymosis (>1 cm) - Generalized over limbs and trunk and face * Bleeding from the mucous membranes: bleeding gums, epistaxis or hematuria * Anemia with severe bleeding * No other features as hepatosplenomegaly or congenital anomalies ITP is a diagnosis of exclusion Careful attention must be paid to the history and clinical features

Answer 98

* CBC: - Thrombocytopenia, usually< 20,000 I mm3 (Normal: 150,000 - 400.000mm) - May be low Hb due to blood loss. - Normal WBCs count with relative lymphocytosis. * Bone marrow examination: Megakaryocytes are normal or increased in number with defective budding * Anti-platelet antibodies: are found in 60 % of cases.

Answer 99

- 1. In mild cases: - Cutaneous hemorrhage only: conservative management and close follow up with direct platelet count to ensure that the count is safely above 10,000. - Avoid trauma and salicylate and NSAIDs. - Advice the parents to attend to clinical care immediately if the child has active bleeding other than cutaneous hemorrhage like gum bleeding or epistaxis 2. In moderate cases:_Persistent muco-cutaneous hemorrhage - Prednisone: Dose 2 mg/kg/d. * Action: It inhibits antibody synthesis & phagocytic activity . * Duration: 2 weeks followed by gradual withdrawal - Intravenous immunoglobulin (IVIG): dose 0.8-1 gm/kg/day, Duration for 2 days . * Action: It causes rapid rise of platelet count (block the phagocytic activity) 3. In severe cases: (severe muco-cutaneous hemorrhage or intra-cranial hemorrhage) - I.V. methyl prednisolone 20 mg /kg/ day for 5 days - Platelet transfusion after starting steroid+/- Fresh whole blood if needed. - IVIG. - Emergency splenectomy: final solution. 4. In chronic cases: (>12 months): - Careful evaluation for associated disorders (E.g. SLE: frequent screening of autoantibodies). - Prednisone & IVIG. - OR Splenectomy (75% curative). - OR Immunosuppressive therapy

Answer 100

X linked disease with reduced factor VIII conc, (therefore more in male) 1. Bleeding in the neonatal period [circumcision bleeding- prolonged bleeding from heel prick or venipuncture from umbilical stump] 2. Extensive bruising, hematoma and bleeding with minor trauma 3. Hemarthrosis * The hallmark of hemophilia * With trauma or spontaneous * If repeated: degenerative joint changes and fibrosis (fixed ANKYLOSIS} 4. Spontaneous Bleeding from orifices: epistaxis or hematuria in severe cases 5. Internal organs: intracranial hemorrhage. Intramuscular hemorrhage (e.g. psoas hemorrhage)

Answer 101

1. Phase I coagulation defect (prolonged PTT) 2. Specific factor VIII assay (reduced below normal) Normal> 60% & Carrier 30 -60% (female). - mild hemophilia 5-30%: (bleeding with trauma or surgery) - moderate hemophilia 1-5%: (bleeding with minor trauma) - severe hemophilia< 1%: (spontaneous joint bleeding)

Answer 102

Prevention: Avoid trauma-aspirin - non-steroidal anti-inflammatory- make sure the child is vaccinated against Hepatitis B virus - Physiotherapy prevents joint contracture. Treatment: 1. Cold compresses minimize bleeding in mild cases 2. Replacement: * I.V. infusion of cryoprecipitate (Plasma concentrate of factor VIII). This dose applies to hemarthrosis. * I.V. infusion of Purified plasma derived factor VIII concentrate * IV infusion of Recombinant factor 8. * Prophylactic F VIII in severe hemophilia 3. Desmopressin: In mild hemophilia A it increases endogenous release of FVIII (ineffective in hemophilia B). 4. Physiotherapy specially after immobilization to prevent muscle wasting and joint contracture

Answer 103

Diagnosis: On clinical basis (no specific lab finding). Clinical manifestations: 1. Skin: palpable purpura (in absence of coagulopathy and thrombocytopenia) is the hallmark of the disease. It occurs in lower limbs and buttocks. Subcutaneous edema localized to the dorsal of hands and feet is also common. 2. Arthritis: usually self-limiting and oligoarticular. 3. GIT: including abdominal pain, vomiting, diarrhea, paralytic ileus and melena. Intussusception is uncommon. 4. Renal: nephritis, hypertension or proteinuria. 5. Neurologic manifestations can occur due to hypertension or CNS vasculitis. laboratory 1. Complete blood picture: normal platelet count 2. ESR, CRP: elevated (inflammation) 3. Urine analysis (screen for hematuria) 4. Stool analysis (screen for occult blood in stool) 5. Blood Urea, creatinine and C3: screen for nephritis

Answer 104

Supportive treatment with maintenance of good hydration and electrolytes Control of pain with simple analgesics Prednisone for severe gastrointestinal diseases or hemorrhage If HSP nephritis; IV methyl prednisolone and cyclophosphamide

Answer 105

1- Fever persisting at least 5 days (38 degrees of more, unremitting). 2- Presence of at least 4 of 5 features: a. Changes in extremities: * Acute: Erythema or edema of hands and feet. * Subacute: Periungual peeling of fingers and toes (weeks 2 and 3). b. Polymorphous skin rash. c. Bilateral bulbar conjunctival injection without exudate. d. Changes in lips and oral cavity: erythema, lip cracking, strawberry tongue e. Cervical lymphadenopathy (>1.5 cm diameter), usually unilateral. 3- Exclusion of other diseases with similar findings (DD) 4- Other clinical features a. Cardiovascular affection: coronary artery aneurysms, myocarditis. b. Hepatic dysfunction. c. Arthritis d. CNS: extreme irritability e. Perineal desquamation * in atypical or incomplete KD, patients have persistent fever but fewer than 4 of the characteristics. In these patients, laboratory and echocardiographic data can assist in the diagnosis.

Answer 106

1. Leukocytosis with neutrophilia, elevated ESR and CRP, hypoalbuminemia, thrombocytosis after week 1 2. Sterile pyuria 3. Elevated serum transaminases and plasma lipid Echocardiography should be performed at diagnosis and again after 2-3 weeks of illness. If the results are normal, a repeat study should be performed 6-8 weeks after onset of illness.

Answer 107

1. Viral infections e.g. Measles, EBV, CMV, Enteroviruses. 2. Bacterial infections 3. Rheumatological disease: Systemic-onset JIA 4. Other: Drug-hypersensitivity reaction

Answer 108

Aspirin: as anti inflammatory and anti thrombotic -30-50 mg/kg/6hrs until patient is afebrile (anti-inflammatory) -3-5 mg/kg/day until normal ESR and platelets (anti-thrombotic) Aspirin continued for 2 yrs if coronary abnormalities have resolved If coronary abnormalities persist, it is continued for life If large coronary artery aneurysm; anticoagulants like warfarin IV immunoglobulin: administered 10 days within the onset of fever, reduces risk of coronary artery disease.

Answer 109

1. Cranial causes * Familial large head. * Chronic hemolytic anemia. * Rickets. * Achondroplasia and mucopolysaccharidosis 2. Intracranial (increased intracranial tension) * Hydrocephalus. * Subdural hematoma and Subdural effusion. * Brain tumors. * Neurofibromatosis * Cerebral gigantism (Sotos syndrome) * CNS storage disease e.g. mucopolysaccharidosis (Hurler's syndrome)

Answer 110

A. True microcephaly * Primary (genetic): 1. Familial ( autosomal recessive) 2. Autosomal dominant 3. Syndromes (Down: trisomy 21) - (Edwards: trisomy 18) * Secondary (non- genetic or acquired) Destructive processes affecting the brain during fetal and early infantile life: 1. Prenatal causes: . Congenital infections . Irradiation . Drugs 2. Perinatal causes: . Hypoxic ischemic injury. . Intracranial hemorrhage. 3. Post- natal causes: . Kernicterus . CNS infections: meningitis -encephalitis. . Intracranial hemorrhage. B. Craniostenosis Premature closure of skull sutures.

Answer 111

Short parents Delayed puberty 1. Genetic and chromosomal disorders * Down syndrome. * Turner syndrome * Skeletal dysplasia: Achondroplasia 2. Endocrinal causes: (bone age is markedly * GH deficiency o Panhypopituitarism e.g. craniopharngioma o Isolated growth hormone deficiency. * Hypothyroidism : congenital /autoimmune thyroiditis * Hypoparathyroidism * Hypogonadism * Adrenal hormones: Cushing syndrome- Adrenal insufficiency- Corticosteroid therapy 3. Severe systemic diseases in infancy and childhood * Chronic diseases * Chronic infections: Tuberculosis-bilharziasis. 4. Nutritional starvation (malnutrition) 5. Social short stature :Psychological deprivation: disturbed child-mother or family relation reduces GH release

Answer 112

1. Bone age assessment: x-ray of the left hand and wrist will diagnose the normal variant (familial/constitutional) and save unnecessary investigations. 2. Karyotyping in short females to exclude Turner syndrome (45 XO). 3. Anti-tissue transglutaminase (positive in case of Celiac disease). 4. Skeletal survey if skeletal dysplasia is suspected (e.g. Achondroplasia). Rickets also should not be missed as a cause. 5. If bone age is markedly delayed: Endocrinal assessment is warranted including: Thyroid profile - Growth hormone stimulation tests - Steroids and ACTH if clinically suspected. 6. If pituitary hormonal deficiency is diagnosed (Panhypopituitarism): MRI to detect craniopharyngioma or brain anomalies

Answer 113

Onset of puberty before 8 years in girls or before 9 years in boys

Answer 114

Failure to develop secondary sexual characters by 13 years in girls or 14 years in boys.

Answer 115

-Bifidus factor:favors the growth of lactobacillus bifidus responsible for acid production that inhibits growth of bacteria e.g. E-coli -Intestinal growth factor: repair of damaged intestinal cells -Lactofererrin: Fe binding protein reducing its availability for bacteria. -Immunoglobulins: especially secretory IgA that provide mucosal protection -Lymphocytes: macrophages and polymorphs: role in phagocytosis and cytokine production -Interferon: antiviral -Lysozymes and peroxides: attack bacterial cell wall -Low incidence of allergic disorders and necrotizing enterocolitis (NEC)

Answer 116

1. Mechanical factors ■ Suckling and complete emptying of the breast are the most important factors 2. Nutritional factors ■ Adequate diet ■ High: protein, vitamins and fluids. Avoid: coffee, smoking, onions, spices and chocolates 3. Psychological factors ■ Relaxation, confidence and happiness are very important factors

Answer 117

1. Early skin to skin contact immediately after delivery 2. Early breast feeding, if possible within the first hour after birth 3. Avoid giving the newborn baby glucose after delivery, unless medically indicated e.g. hypoglycemia 4. Avoid use of pacifiers as it can slow down the weight gain 5. Avoid bottle feeding as it leads to nipple confusion 6. Rooming in: the baby stays on mother's room or better co-bedding 7. On demand feeding with no restriction to time, frequency or duration 8. Exclusive breast feeding without supplements during the first 6 months of life 9. Ensure perfect positioning of the baby and attachment on the breast 10. Emptying the breast before shifting to the other

Answer 118

1. Weight gain: the most reliable: 200 gram/week or 250 gram /10 days (in the first 4 months) 2. Feeding: Baby feeds at least 8 times I day. Baby makes swallowing noises throughout the feeding. Baby is satisfied and sleeps after feeds for 2- 4 hours. 3. Urine: baby wets 6 or more diapers/day. 4. Stool: 4 or more motions /day (soft yellow stools commonly with every feed). Early week of life loose stool passed with each feed (gastro colic reflex)

Answer 119

1. Nipple soreness * Management - Continue breast feeding considering proper positioning and attachment. - Begin nursing on less affected side - Nursing for shorter periods. - Use of nipple shield - Temporary pumping may be needed 2. Breast engorgement * Management - Continue breast feeding - Analgesics and fomentation (warm before feed and cold after feeds) - Alternate the side on which feeding begins - Proper breast evacuation - Temporary pumping may be needed (Manually or by a pump) 3. Insufficient breast milk * Diet: adequate diet and fluid to the mother * Breast measures - Massage breast before and during feeding - Hot towels to the breast 3-5 minutes before feeding - Frequent feeding Infant problems: Nasal obstruction, oral fungal inf, choanal atresia, cleft palate, regurgitation, RDS and HF

Answer 120

A. Maternal contraindications: 1. Maternal diseases: Open pulmonary TB (Infectious) 2. Neurologic diseases and substance abuse 3. Chronic debilitating diseases: Heart failure, CRF, liver cell failure 4. Maternal medications (very few e.g.Chloramphenicol,Tetracycline) Infants: -Galactosemia -Phenylketonuria -Lactose intolerance

Answer 121

1. Complementary feeding (breast feeds are completed by bottle feeds) Indications: Insufficient weight gain after full trial of breast feeding for a month, LBW infants to avoid further loss of weight 2. Supplementary feeding (some breast feeds are replaced by bottle feeds) Indications: 1. Working mother: one or more feeds are given during the period of mother absence (Absent part of the day) 2. Twins: if poor weight gain is established breast & bottle should be given to each baby alternatively 3. Substitutive feeding (all breast feeds are replaced by bottle feeds) * Indications when breast feeding is contraindicated

Answer 122

1. Humanized Formulas (Standard) - First choice for feeding healthy infants during the first 6 months of life 2. Follow on milk formulas - Healthy infants> 6 months of life 3. Lactose free formulas: - Lactose intolerance(congenltal or acquired 2ry to persistent diarrhea) - Galactosemia 4. Soya based formulas: - Cow's milk allergy - persistent diarrhea 5. Elemental formulas: - Allergy to both cow milk and soy protein - Chronic diarrhea - Malabsorption e.g. cystic fibrosis 6. Preterm infant formulas: - Weight gain achievement in preterm infants before discharge

Answer 123

Introduction of solid foods other than milk in infants diet to complement the nutritional gap 1. Start with small amount of one type & assess the infant's tolerance and atopy 2. Each new food should be tried separately (3-5 days interval) to identify adverse reaction 3. New foods should be mashed and given by spoon 4. New foods should be given in an attractive way (plates, spoons ... ) 5. New foods should be given before breast feeding at fixed times 6. If disliked, omit & retry again few weeks, exposure to a food type for 4-15 times in positive environment allows brain to recognize and accept the new taste. 7. Establish routines for meals & snacks in a "schedule" (e.g, 3 meals, 2-3 snacks) 8. Limit meals to 15 to 20 minutes 9. Responsive feeding method: responding to infants hunger and satiety prevents obesity and picky eater habits 10. Encourage finger food when age appropriate

Answer 124

* 4-6 months: start with vegetables and fruits, then introduce Iron fortified cereals {rice and wheat). * 6 months: red meat, chicken, fish, eggs yolk and white are introduced separately then together, legumes except for fava beans. * 8-9 months: Can start to share ordinary foods (all food groups) * 9-12 months: Most table foods can be given (provided soft & in small pieces) * > 12 months: 3 main meals and 2 snacks with only two breast or formula feeds (in cup)

Answer 125

* Do not leave the baby alone during feeding (Choking) * Avoid salt, sugar & honey * Avoid cow's milk in the 1st year of life * Fresh and processed juices are contraindicated in first year of life and only 180 ml/day of fresh juice is allowed after first year. * Avoid breast addiction * Avoid high risk choking foods (nuts, grapes) * Avoid distractions during meals (no screens allowed) * If microwave is used to heat food it should be properly stirred before feeding

Answer 126

Marasmus: severe form of acute undernutrition with wasting Kwashiorkor: Severe for of acute PROTEIN deficiency with wasting that is masked by edema Marasmic-KWO: mix of both Nutritional dwarfism: Linear growth affection due to chronic undernutrition (short stature) Failure to thrive (FTT): Under-nutrition due to inadequate caloric intake

Answer 127

1. Edema (Diagnosis cannot be made in absence of edema) Cause: Hypoalbuminemia (decreaased Oncotic pressure) increasd ADH and aldosterone (2ry to decreased effective plasma volume) 2. Mental changes Cause: * decreased Amino acids (Neurotransmitters) * decreased Niacin (and NAO, NADP) * Maternal deprivation 3. Disturbed muscle/fat ratio * Muscle: Wasting (decreased Proteins) * Fat: Excess SC fat (increased CHO) * Detected by Skin fold thickness 4. Growth failure May be masked by edema and excess SC fat

Answer 128

1. Hair changes: Sparse, soft and easily detached +flag sign 2. Skin changes: Lesions: Cracking, fissuring, ulceration,hypo or hyperpigmentation ± infection 3. Hepatomegaly 4. GIT * Anorexia * Vomiting * Diarrhea 5. vitamin deficiency (complications) * Vitamin A: blindness * Vitamin B: angular Stomatitis * Vitamin C: Scurvy * Vitamin D: Rickets does not occur (Rickets is a disease of growing bones) * Vitamin K Bleeding (Hypoprothrombinemia) 6. Infections: Gastroenteritis (GE), pneumonia 7. Anemia

Answer 129

1. Recurrent infections: Pneumonia, gastroenteritis (GE) ... 2. Hypothermia and hypoglycemia (Due to disturbed glucose metabolism) 4. Electrolyte disturbances and dehydration 5. Shock: Hypovolemic or septic 6. Mineral deficiency: Iron deficiency anemia 7. vitamin deficiency

Answer 130

1. Indications - Moderate or severe Kwashiorkor - Marasmic kwashiorkor - 3rd degree marasmus - Complicated marasmus 2. Management plan (Stabilization and management of complications) - Infection {GE, pneumonia ... ): Proper antibiotics - Shock: Shock therapy (Immediate IV fluid: Lactated ringer's 20 ml/Kg) - Dehydration: IV fluid therapy (Deficit therapy) - Electrolyte disturbances: should be corrected - Anemia: Packed RBCs - Hypoglycemia (IV glucose), hypothermia: adequate clothing or radiant warmer

Answer 131

k. Non organic: environmental and psychological factors : 95% 1. Inadequate availability of foods 2. Psychological deprivation 3. Neglect or child abuse B. Organic: 5% 1. Inadequate intake Impaired suckling or swallowing Chronic illness lead to anorexia 2. Inadequate retention: vomiting and severe GERD reflux 3. Inadequate absorption (malabsorption): e.g. cystic fibrosis 4. Inadequate metabolism: failure to utilize nutrients amino acid and storage disorders insulin dependent diabetes - congenital hypothyroidism 5. Increase requirements * Malignancy. * Chronic infections * Thyrotoxicosis

Answer 132

1. Bone pathology in rickets: Proliferation without ossification 2. Epiphysis: failure of Ca- Ph crystal deposition in the cartilage cells leading to excess cartilage cell proliferation that invade the metaphysis (broadening and fraying) 3. Diaphysis: bone rarefaction and fracture

Answer 133

A) Vitamin D deficiency rickets with 2ry increased PTH - Vitamin D deficiency - Malabsorption of vitamin D - Hepatic disease - Renal osteodystrophy B) Primary P04 deficiency (No 2ry increased PTH) - XL-D Hypophosphatemic rickets - Fanconi syndrome: PCT dysfunction C) End-organ resistance to 1,25 (OH) D) Cases resembling rickets: Hypophosphatasia Another classification Nutritional: Lack of vitamin D a. Inadequate intake of vitamin D * Prolonged exclusive breast milk * Cow milk (Non-optimum Ca/Ph ratio) b. Inadequate sun exposure * Wrapping * Windows * Winter-time (Scandinavia) * Dark skinned people Non nutritional A. Renal rickets B. Malabsorption: * Cystic fibrosis * Celiac disease C. Hepatic rickets

Answer 134

Labs: -serum Ca (usually normal but my decrease due to depletion, parathyroid exhaustion) -Serum phosphorus: decreased -Serum alkaline phosphatase: decreased -Vit D: Low -Parathyroid: high Imaging:

Answer 135

1. Nutritional education Value of breastfeeding, proper weaning ... * Diet rich in vitamin D: Oily fish (salmon, sardines), egg yolk, liver, butter, fortified milk * Avoid rachitogenic diet 2. Vitamin D supplementation [40 IU = 1 pg] * Full-term: 400 IU/day (since birth) * Preterm: 600 IU/day (as early as the 1st month) 3. Sun exposure (UVR) 4. Regular assessment of nutritional state: Early manifestations of rickets

Answer 136

A) Vitamin D therapy a. Oral therapy * Dose - Vitamin D3: 3000-5000 IU/day * Duration: 2-4 weeks b. Parenteral therapy * Dose: Vitamin D3: 600.000 IU (Shock therapy) * Duration: Single IM dose * Advantages: * More rapid * No need for parents compliance * Diagnosis of non-vitamin D deficiency rickets * Disadvantages (Side effects): * Tetany * Hypervitaminosis D

Answer 137

a. Tetany: IV Ca gluconate 10% "1 ml/g"(Slowly while monitoring heart rate) b. Deformities and Fractures Orthopedic care (After complete bone healing) c. Infections: proper antibiotics d. Iron deficiency anemia: Iron (6 mgg/day)

Answer 138

Etiology * Prolonged oral vitamin D therapy * Parenteral vitamin D therapy (Shock therapy) Clinical picture * GIT: Anorexia, nausea, vomiting, constipation * Renal: Polyuria, polydipsia, renal stones,nephrocalcinosis (Dysuria, colics, UTI . .. ) Investigations * Laboratory: increased serum Ca, increased Urinary Ca * Imaging: X-rays, US (Nephrocalcinosis, stones) Prevention * Careful vitamin D therapy Treatment * Stop vitamin D and Ca therapy, IV fluids, Steroids

Answer 139

a) Autosomal abnormalities: Trisomy: one chromosome is represented by 3 copies "extra-chromosome" e.g. trisomy 21: (Down syndrome), trisomy 18: (Edward's syndrome) Trisomy 13 (Patau syndrome). Monosomies: one chromosome is missing: (monosomy: 21-22). b) Sex chromosomal abnormalities: klinefilter syndrome (47,XXY male). Turner syndrome (45,XO female

Answer 140

a) Translocation: transfer of material from one chromosome to another Example: translocation Down syndrome b) Deletion: loss of a portion of chromosome mostly through breakage. Example: loss of the tip of the short arm of chromosome 5 e.g. Cri du chat syndrome. c) Duplication: The presence of 2 copies of a segment of a chromosome It results from unequal crossing-over during meiosis d) Ring chromosome: deletion in which both ends of the chromosome have been lost and the two broken ends have reunited to form a ring.e) e) Inversion: fragmentation of chromosome followed by reconstruction but with segment inverted.

Answer 141

Genotype: 45 XO Etiology: Non-disjunction (The X chromosome is usually of maternal origin) Clinical picture female * Neonatal period 1. Webbing of the neck. 2. Edema (lymphedema) of the hands and feet. 3. Low posterior hair line 4. Widely spaced nipples 5. Cardiac anomalies: coarctation of the aorta in 20% of cases and bicuspid aortic valve * Childhood period 1. Short stature is the most common presenting feature. (Mean= 143 cm) 2. Limbs: cubitus valgus (wide carrying angle).Short 4th metacarpal * Adolescence 1. Failure of development of secondary sexual characters. 2. Primary amenorrhea {the ovary is a streak of connective tissue). 3. Normal mentality {MR in 18%)

Answer 142

1. Karyotyping: 45 XO 2. Hormonal study {gonadal failure); increased FSH and LH 3. Thyroid profile {more prone to hypothyroidism). * Imaging 1. X ray to determine bone age 2. Echocardiography: may be aortic coarctation 3. Abdominopelvic ultrasound: may be renal anomalies {horse shoe, ectopic kidney : 40% of cases), uterine anomalies, ovaries {streaks of connective tissues)

Answer 143

1. Recombinant Growth hormone to reach at least 5 feet height 2. Estrogens: To induce the development of 2ry sex characters {Start at 11-12 years) 3. Follow up thyroid function 4. Management of any associated conditions e.g. aortic coarctation

Answer 144

Genotype: 47 XXY ( extra X) Etiology: Non-disjunction Clinical picture male 1. Disproportionate tall stature: * Span > height, * Lower segment > Upper segment 2. Gynecomastia 3. Small testes - (azoospermia) 4. Absence of Secondary sexual characters. 5. Infertility: the most common presentation 6. Intelligence usually in the normal range but may have educational and psychological problems.

Answer 145

Most common cause of inherited intellectual disability and less severe in females due to X inactivation Mode of inheritance * XL-R (FMRI gene) * All mothers of affected males are carriers * Subsequent generations with more repeats condition gets worse Clinical Picture 1. Male * Mental retardation * Macro-orchidism after puberty * Facies: Long face, large everted ears, broad forehead, prominent mandible * Behavioral: Autistic and/ or hyperactive behavior * Cardiac: Mitral valve prolapsed 2. Female o Variable degrees of MR (usually mild to moderate) and learning disability

Answer 146

* Both syndromes are genetic disorder due to the same genes deletion on chromosome 15, that is genomic imprinting 1. Prader-willi syndrome results from a paternal mutation 2. Angel man syndrome result from maternal mutation Clinical Picture Prader-willi syndrome * Very floppy neonate with failure to thrive and feeding difficulties, then hyperphagia and rapid gain of weight in the second year of life * Cognitive delay * Short stature * Small hand and feet Angelman syndrome * Severe intellectual disability * Paroxysms of laughter * Seizures and ataxic gait * Feeding difficulties * Protruding jaw * Small head and short stature

Answer 147

a) Face: mongoloid features d) Eyes: mongoloid or antimongoloid slant. c) Ears: malformed or low set ears. d) Mouth and mandible: cleft lip and palate, micrognathia e) Hands and feet: simian crease (single palmer crease), polydactyly (extra finger), clinodactyly (incurved little finger), syndactyly (fused fingers), gap between first and second toe. 2. Mental retardation 3. Ambiguous genitalia 4. Delayed puberty: klinefelter (males) and Turner 5. Spontaneous abortion 6. Amenorrhea

Answer 148

Indications: * Old maternal age> 35 years * Previous baby with Down syndrome Methods: 1. Triple test: done in maternal serum at 15-16 weeks of gestation * decreased a-Fetoprotein * decreased Unconjugated estriol * increased B-hCG 2. Fetal karyotyping: * Amniocentesis: 14-16 weeks of gestation * Chorionic villus sample: 9-12 weeks of gestation 3. Fetal US: * Nuchal Translucency thickening: thickening of the nuchal fold at the back of the neck * Short femur

Answer 149

Autosomal dominant=not affected with consanguinity Polycystic kidney disease Neurofibromatosis Hereditary Spherocytosis Osteogenesis imperfecta

Answer 150

Thalassemia Sickle cell anemia Galactosemia Phenylketonuria

Answer 151

Hemophilia A Hemophilia B G6PD deficiency Duchenne muscular dystrophy Color blindness

Answer 152

Acute laryngitis and acute laryngeotracheobronchitis Bacterial tracheitis Acute epiglottitis (life threatening) Laryngospasm Laryngomalacia Laryngeal foreign body Laryngeal compression Laryngeal diphtheria

Answer 153

1. Grade I: exertional stridor: during crying or exercise. 2. Grade II: stridor at rest, become worse with crying. 3. Grade III stridor with retractions (suprasternal and supraclavicular). 4. Grade IV: stridor with respiratory failure: cyanosis and disturbed consciousness.

Answer 154

Grade I: home management; warm steam inhalation, oral steroids, close observation Treatment plane (hospital) 1. Oxygen therapy (mask, head box) follow up by pulse oximetry. 2. Cold steam inhalation: ultrasonic nebulizer. 3. Parenteral steroids to decrease laryngeal edema. 4. Antibiotics in suspected bacterial disease. 5. Close observation: worsening endotracheal intubation. Treatment of acute epiglottitis: ICU 1. Endotracheal intubations (very skilled personnel) 2. Parenteral antibiotics: 3rd gen cephalosporin. 3. With proper treatment most children recover within 2-3 days. 4. Prophylaxis with rifampicin for close contacts

Answer 155

it may precipitate complete airway obstruction

Answer 156

Pulmonary edema Respiratory distress syndrome Aspiration syndrome Pneumonia Acute bronchiolitis

Answer 157

Heart failure Metabolic acidosis Acute severe anemia Central hyperventilation

Answer 158

Grade I: tachypnea and working alae nasi Tachypnea: RR above 60/ minute, in neonates and infants < 2 months 50/ minute, in infants 2-12 months 40/ minute, in children 1-5 years 30/ minute, in older children 20/minute. Grade II: intercostal and subcostal retractions Grade II grunting Grade IV: respiratory failure (central cyanosis, disturbed consciousness, shock and arrhythmias)

Answer 159

Primary brain lesion: May be focal or lateralizing 1. Traumatic brain injury (concussion, contusion or laceration) 2. Intracranial hemorrhage (traumatic or non-traumatic) 3. Intracranial infections : meningitis, encephalitis and brain abscess 4. Intracranial neoplasm 5. Others: brain infarction - post status epilepticus Secondary brain lesions :( encephalopathy) usually diffuse: diagnosed mainly by labs 1. Hypoxic encephalopathy: hypoxia - shock- severe anemia. 2. Endogenous encephalopathy (metabolic comas): diabetic ketoacidosis- acute renal failure liver cell failure- severe dehydration- metabolic acidosis, severe electrolytes disturbances 3. Exogenous encephalopathy: poisoning and drug intoxication

Answer 160

1. Hypovolemic shock: the most common in children * Severe dehydration * Severe hemorrhage 2. Septic shock * Primary septicemia due to fulminant sepsis. * Secondary septicemia due to serious focal infection. 3. Obstructive shock * Cardiac tamponade due to pericardial effusion. * Tension pneumothorax. * Massive pulmonary embolism 4. Cardiogenic shock * Severe acute heart failure. * Myocardial depression in septic shock * Late septic shock or any advanced shock (Part of multiple organ system failure) 5. Kinetic (distributive)shock * Neurogenic shock (spinal cord trauma). * Anaphylactic shock (due to drugs, foods, serum).

Answer 161

1. Grade 1: early shock: tachycardia and poor peripheral perfusion 2. Grade II: established shock: tachycardia+ poor peripheral perfusion+ hypotension 3. Grade III: advanced shock: vital organ hypoperfusion: + multiple organ system failure. 4. Grade IV: irreversible shock: Fatal shock. MOSF and refractory metabolic acidosis, not responding to maximal supportive management

Answer 162

1. Clinical monitoring: heart rate, respiratory rate, blood pressure, peripheral perfusion, temperature and urine output. 2. CVP and other hemodynamic parameters as CO & SVR (advanced or refractory cases in the ICU) 3. Laboratory: blood gases, electrolytes, blood sugar and cultures. 4. Imaging : Chest x-ray, echocardiography Cardiovascular support: 1. Oxygen therapy then according to the type of shock: 2. Preload augmentation to correct hypovolemia and also indicated in distributive and septic shock. Avoid when there is systemic or pulmonary congestion (crystalloid) 3. Contractility augmentation (inotropic drug support): IV infusion of dopamine, dobutamine or adrenaline IV drip 4. Vasopressors e.g. noradrenaline or afterload reduction e.g. sodium nitroprusside infusion in some cases 5. Treatment of arrhythmia. a. Treatment of precipitating factors as hypoxia and acidosis b. Anti-arrhythmic drugs

Answer 163

a. GIT losses e.g. diarrhea b. Urinary losses e.g. diuretics, steroids & renal tubular defects c. Intracellular shift of potassium e.g. insulin and B2 agonists d. Deficient intake

Answer 164

1. Vomiting, 2. Muscle weakness 3. Reduced intestinal motility, paralytic ileus, 4. ECG changes, arrhythmias and cardiac arrest

Answer 165

1. Eye: Allergic conjunctivitis. 2. Nose: Allergic rhinitis 3. Mouth: Oral itching and edema of lips, tongue or palate. 4. Respiratory: Dysphagia, hoarseness, stridor, dyspnea, repetitive cough, wheezing. 5. Cardiovascular: chest pain, palpitation, hypotension 6. Gastrointestinal: Nausea, colic, cramps, vomiting and diarrhea 7. Skin: hot, flushing (wheels), urticaria & edema 8. Finally shock (Discuss)

Answer 166

1. ABCs: a. Establish airway, assess breathing: 100% 02 b. Assess circulation and establish IV access. 2. Drugs: a. Epinephrine: 0.01 ml/kg (IM) is the most important b. Antihistaminic: Histamine 1 receptor antagonist (diphenhydramine) orally c. Anti-inflammatory: Corticosteroids: I.V. hydrocortisone. d. Nebulized salbutamol for bronchospasm 3. Treatment of shock will also require a. Trendelenburg position b. 20 ml/kg ringer or saline

Answer 167

1. Simple focal infections * Respiratory: [URTI is the most common]:Nasopharyngitis, otitis media, sinusitis, bronchitis. * Digestive: Stomatitis, gastroenteritis. * Urinary: Urinary tract infections (cystitis). * Cutaneous: Cellulitis, abscess. 2. Serious focal infection (you should exclude them depending on these criteria) * Meningitis: disturbed consciousness, convulsions, meningeal irritation, * Pneumonia: respiratory distress, focal chest signs (rales, bronchial breathing). * Pyelonephritis: loin tenderness or a swelling. * Peritonitis: abdominal distension and generalized tenderness * Osteomyelitis or arthritis: tenderness, swelling and limitation of movements B. Fever without focus : Simple fever "Nonspecific febrile illness * Viremia * Bacteremia * Septicemia

Answer 168

* Systemic bacterial infections: >Tuberculosis * Hidden bacterial focal infections: > Abdominal abscess and brain abscess > Endocarditis, pyelonephritis and osteomyelitis * Viral: > Infectious mononucleosis -CMV- HIV > Cytomegalovirus infection - Hepatitis viruses. * Parasitic: Malaria-Toxoplasmosis * Fungal: systemic candidiasis 2. Rheumatic diseases * Rheumatic fever * Juvenile Idiopathic arthritis * SLE and Kawasaki disease 3. Malignancy * Leukemia, Lymphoma and neuroblastoma

Answer 169

1. Gram - ve bacteria * Meningococcal infection (the commonest cause of septic shock) * Other gram -Ve bacteria :Klebsiella, H.influenza and E coli 2. Gram +ve bacteria: * Staphylococcus aureus, * Non-pyogenic streptococci and Streptococcus pneumonia (pneumococcus).

Answer 170

high fever, poor feeding with persistent vomiting, history of focal inf, history of predisposing factors Exam: Very ill, toxic, pale, lethargic, cold extremities, purpuric rash in meningococcal septicemia, In toxic encephalopathy; disturbed consciousness and seizures In heme causes: acute hemolysis, DIC and thrombocytopenia

Answer 171

1. Serious bacterial infections: (20%) * Meningococcal septicemia is the most common. * Other causes of sepsis (gonococcal, pneumococcal infection) * Hemophilus influenzae type b Listeria and Staphylococci. Infective endocarditis 2. Viral infections: (80%) * Enterovirus infection (especially echovirus type 9) is the most common Adenovirus * Hemorrhagic fevers: Black measles, Dengue fever and Cytomegalovirus * Congenital rubella and congenital cytomegalovirus 3. Other vasculitis: * Thrombocytopenia with fever * Henoch-Schonlein purpura * Malaria

Answer 172

Document fever well Detailed history and exam CBC, ESR, CPR Urine and blood culture Serology tests Tuberculin test Chest xray Abdominal US ECG

Answer 173

1. Child is younger than 3 months of age 2. A temperature of more than 40C or a fever lasting more than 48 hours 3. Color: pale mottled or cyanosed 4. Level of consciousness is reduced, neck stiffness 5. Significant respiratory distress 6. Persistent vomiting or child not eating or drinking normally 7. Signs of shock or dehydration 8. Swelling of a limb or joint 9. Skin rash

Answer 174

1. Infections a. Infections in which the rash is essential for diagnosis. * Measles * German measles * Roseola infantum * Scarlet fever b. Other infections (rash may be present) * Typhoid fever * Infectious mononucleosis * Enteroviral infections * Parvovirus B 19 {slapped cheek): usually in school age 2. Rheumatic diseases * Juvenile idiopathic arthritis * Systemic lupus erythematosus * Kawasaki disease 3. Skin disorders * Sweat rash: fine papules on the neck and trunk and napkin area * Urticarial rash: wheals with itching

Answer 175

1. Infection * Chickenpox * Herpes zoster ( shingles) * Herpes simplex virus * Coxachie virus infection (hand and foot syndrome) * Impetigo 2. Skin disorders * Papular urticaria * Erythema multiforme (Steven Johnson syndrome)

Answer 176

Causative organism: group A beta hemolytic streptococcus Mode of transmission: droplet Incubation period: 2-4 days Complications 1. Spread of the infection: * Local: Cervical adenitis, sinusitis, otitis media, mastoiditis and lateral sinus thrombosis, bronchitis, bronchopneumonia * Distant: Osteomyelitis, arthritis and meningitis 2. Late complications * Post-streptococcal rheumatic fever * Post streptococcal glomerulonephritis. * Erythema nodosum (nodules on shin of tibia)

Answer 177

1. CBC: Leukocytosis(PNL 10,000 - 20,000 /mm3 ) and anemia 2. Elevated ESR and CRP 3. Serologic tests: A significant rise of Antistreptolysin O titer (ASO titer). 4. Throat culture: group a beta hemolytic streptococcus.

Answer 178

1. Antibiotic therapy: * Oral penicillin V: 400. 000 IU/dose 4 times/day /10 days * Procaine penicillin: injection for 10 days * Erythromycin (50 mg/kg/day)in penicillin sensitive patient for 10 days 2. Antipyretics for the high fever. Prognosis: excellent with treatment- late complication don't depend on the severity of the disease

Answer 179

Fever and sore throat Strawberry tongue Abdominal pain Enlarge tender cervical lymph nodes At rash: Blanching erythema and sandpaper rash more intense around skin folds Flushed cheeks and circumoral pallor

Answer 180

Caused by varicella-zoster virus (prevented by live attenuated vaccine) and varicella zoster immunoglobulin give in early incubation 1. Secondary bacterial infection and sepsis 2. CNS complications * Encephalitis. * Cerebellitis: occurs within a week of the illness presents with ataxia. * Aseptic meningitis. 3. Neonatal varicella syndrome

Answer 181

1. Antipruritic agents (local and systemic) 2. Antipyretics. Aspirin should not be used as it increases the risk of Reye syndrome (acute encephalitis with fatty infiltration of the liver) 3. Antiviral drugs: acyclovir I.V in ( immunocompromised - encephalitis -pneumonia) 4. Antibiotics are indicated only if secondary bacterial infections occur.

Answer 182

Caused by mumps virus 1. Prodroma: fever, anorexia, ,headache and malaise 2. Diagnostic stage a. Pain of one or both parotids: pain around the ear aggravated by chewing movements of the mandible. b. Swelling: The parotid enlargement is characteristic: * Behind the angle of the mandible and elevates the ear lobule. * Painful and tender * Peak in 3 days and subsides slowly over 3- 7 days. * Bilateral or less common unilateral * Hyperemia of the parotid duct opening * Subclinical in 30% of cases

Answer 183

1. Encephalitis * Incidence: more common in males\ 2. Guillain barre syndrome 3. Orchitis * Extremely rare in pre-pubertal children 4. Pancreatitis * Clinical picture fever,epigastric pain, vomiting and tenderness * Investigations: elevated serum amylase 4. Oophoritis: severe pain. If on the right side may be confused with appendicitis 5. Deafness: Auditory neuritis 6. Ocular: Optic neuritis, scleritis,retinal vein thrombosis

Answer 184

* Contraindications (conditions in a recipient that increases the risk for a serious adverse reaction) and precautions to vaccination are conditions under which vaccines should not be administered. * A precaution is a condition in a recipient that might increase the risk for a serious adverse reaction, might cause diagnostic confusion, or might compromise the ability of the vaccine to produce immunity. * The presence of a moderate or severe acute illness with or without a fever is a precaution to administration of all vaccines.

Answer 185

Obstetric: ■ Uterine anomaly and cervical incompetence - multiple pregnancy ■ Placental separation- preeclampsia Premature rupture of membranes. General: chronic diseases 2. Fetal : Hydrops fetalis

Answer 186

1. Respiratory a. Respiratory distress syndrome. b. Apnea of prematurity c. Recurrent aspiration 2. Neurological a. Intracranial hemorrhage. b. Periventricular leukomalacia c. Kernicterus 3. GIT a. Necrotizing enterocolitis with intestinal perforation b. Poor sucking and swallowing c. Gastro esophageal reflux disease and vomiting 3. Hematological anemia of prematurity 4. Sepsis: due to immature immune system 5. Metabolic problems a. Hypoglycemia, Hypocalcemia, hypomagnesemia b. Hypothermia

Answer 187

1. Maternal * Placental: placental insufficiency e.g. pre-eclampsia, vascular malformation and placental infarction * General: chronic disease e.g. hypertension, heart or renal diseases and collagen vascular disease. 2. Fetal causes: Congenital infections, Congenital anomalies , Chromosomal disorders

Answer 188

1. Respiratory * Perinatal depression * Meconium aspiration. * Pulmonary hemorrhage. 2. Metabolic * Hypoglycemia due to poor glycogen stores. * Hypothermia due to loss of subcutaneous fat. 3. Congenital infections are common association 4. Congenital anomalies are common

Answer 189

Malformations: VSD and sacral agenesis Macrosomia Fetal hyperglycemia and hyperinsulinism Intrauterine growth retardation RDS Hypoglycemia due to hyperinsulinism Hypertrophic cardiomyopathy Hypocalcemia and hypomagnesaemia

Answer 190

1. Laboratory: Hematocrit, blood glucose, calcium and magnesium. 2. Imaging: Chest X ray, Echocardiography, abdominal ultrasound. Management 1. Delivery should be in a hospital where the newborn can be carefully monitored 2. After birth: monitoring for metabolic disturbances especially hypoglycemia a. Blood glucose should be done within the first hour of delivery, b. Asymptomatic infants if clinically well should start early feeding. c. Breastfeeding should be started as soon as the infant is stable. d. If the infant is unable to tolerate oral feeding , glucose should be given by peripheral IV infusion at rate of 6-8 mg/kg/minute 3. Careful monitoring of complications —>NICU admission for, unstable baby

Answer 191

Differentiate between physiological and pathological jaundice Differentiate between unconjugated from conjugated jaundice Clinical evaluation: history and examination INV for unconjugated and conjugated jaundice

Answer 192

1. Production: overproduction of bilirubin increased Rate of RBCs destruction: Isoimmune Hemolytic disease of the newborn: Rh and ABO incompatibility. Hemolytic anemia: spherocytosis (autosomal dominant), G6PD (X linked recessive) Extravasated blood: large cephalhematoma. Septicemia. increased Mass of RBCs: polycythemia. 2. Uptake defect: Y and Z protein * Gilbert disease: familial deficiency of Y and Z proteins. 3. Conjugation defect: Glucuronyl transferase enzyme Crigler Najjar syndrome * Suppression of enzyme activity: breast milk jaundice and cretinism 4. Increased enterohepatic circulation * Pyloric stenosis, intestinal obstruction e.g. ileal atresia * Breast feeding jaundice

Answer 193

1- Idiopathic neonatal hepatitis (giant cell hepatitis): The most common cause - associated with IUGR 2- Infections: a. Bacterial: neonatal sepsis, urinary tract infection b. Viral: CMV, Rubella c. Protozoal: toxoplasma 3. Metabolic: a. CHO metabolism: Galactosemia( sepsis like picture and early liver cell failure) b. Amino acid metabolism: Tyrosinemia(early liver cell failure) c. Lipid storage disease: Niemann-Pick disease d. Alpha-1- antitrypsin deficiency e. Bile acid biosynthetic defects 4- Familial cholestatic syndromes: a. Paucity of intrahepatic bile ducts b. Alagille syndrome: * Paucity of intrahepatic bile ducts d. Benign recurrent cholestasis 5- Extrahepatic biliary atresia (Recently biliary atresia}2nd most common cause (Associated with normal birth weight) 6- Choledochal cyst

Answer 194

1. Measurement: Head circumference: microcephaly in TORCH infection. 2. Head * Cephalhematoma, forceps marks. * Jaundice. * Pallor (suggest hemolytic cause or hematoma). * Polycythemia (in infant of diabetic or small for gestational age). 3. Trunk Microcephaly Cephalhematoma * Umbilicus: omphalitis (umbilical sepsis). * Skin color: lemon yellow (unconjugated) - greenish (conjugated). 4. Signs of Omphalitis * Sepsis Lethargy and poor feeding in sepsis * Hypothyroidism e.g. umbilical hernia or opened posterior fontanel. * Kernicterus e.g. convulsions, deep jaundice * Cholestasis and in utero infections: hepatosplenomegaly.

Answer 195

Serum (total and direct) bilirubin to determine type 1. Blood picture HB % and reticulocytosis ——hemolysis. WBC count and I/T ratio —septicemia. 2. To exclude hemolytic diseases Blood grouping (ABO and Rh) for baby and mother. Coombs'test 3. To exclude hemolytic anemia Enzyme assay: G6PD deficiency. RBCs morphology and osmotic fragility test: spherocytosis. 4. If septicemia is suspected C reactive protein- Erythrocyte sedimentation rate- cultures. 5. Thyroid profile if not done in neonatal screening program

Answer 196

Investigations to diagnose cholestasis 1. Increased total and direct bilirubin ( direct bilirubin 20% of total bilirubin) 2. Increased AST and ALT 3. Increased alkaline phosphatase and Gamma Glutamyl Transpeptidase Investigations to determine the cause (5 steps):- 1. Search for treatable cause * Galactosemia Measuring the enzyme galactose-l-phosphate-uridyl transferase in red cells. * Septicemia and other bacterial infections: CBC, CRP, ESR, cultures. 2. Search for TORCH infections 3. Search for metabolic conditions * a1 antitrypsin assay * Tyrosinemia: succinyl acetone in urine 4. Exclude choledochal cyst: By abdominal ultrasonography 5. Differentiation between neonatal hepatitis and extrahepatic biliary atresia * Persistent clay-colored stools, inspected by physician, suggestive of biliary atresia Liver biopsy: the most important * In neonatal hepatitis: giant cell transformation and degeneration of hepatocyte. * In atresia: expansion of portal areas with fibrosis and bile duct proliferation. HIDA scan: Hepatobiliary iminodiacetic acid (radioisotopic scan): appearance of the dye in the intestine excludes biliary atresia

Answer 197

Phototherapy @ 450-460 mn for infants with bilirubin levels higher than 15 mg or according to hours specific monogram, before after or in between transfusions and prophylactic in LBW infants or infants with hemolytic diseases. Exchange transfusion: in hemolytic disease of newborn, when phototherapy is not effective and baby has risk of kernicterus Pharmacological: IVIG

Answer 198

l. Respiratory distress syndrome RDS: the most common in preterm 2. Transient tachypnea of newborn TTN: the most common in full term. 3. Meconium aspiration syndrome. 4. Pneumonia (intrauterine-aspiration of milk or secretion) 5. Pulmonary air leak e.g. pneumothorax 6. Pulmonary hemorrhage 7. Bronchopulmonary dysplasia Lung malformations 1. Congenital lobar emphysema. 2. Congenital diaphragmatic hernia. 3. Lung hypoplasia

Answer 199

1. Airway: rare * Choanal atresia (bilateral). * Laryngomalacia. * Trachea-esophageal fistula. 2. Chest wall: rare * Neonatal myasthenia. * Thoracic dystrophy 3. Cardiac * Congenital heart disease with heart failure. 4. Central * Cerebral Hypoxia (encephalopathy). * Intracranial Hemorrhage (birth trauma). Meningitis 5. Metabolic * Hypothermia * Hyperthermia * Hypoglycemia. * Acidosis.

Answer 200

1. Basic life support measures a. Thermoregulation b. Correction of acidosis, fluid and electrolyte balance. 2. Prophylactic antibiotics until cultures appear (as RDS is clinically indistinguishable from early-onset severe group B streptococcal disease) 3. Monitoring a. Heart rate, respiratory rate, arterial blood pressure and temperature. b. Oxygen saturation by transcutaneous 02 pulse oximeter. c. Arterial blood gases d. Hemoglobin, electrolytes, calcium, glucose and albumin. 4. Correction of hypoxemia a. In the delivery room : prophylactic Nasal continuous positive air way pressure (CPAP) 5. Surfactant replacement therapy a. Natural or synthetic surfactant can be given via an endotracheal tube or other routes

Answer 201

Sepsis is a potentially life-threatening complication of an infection, where the host response releases inflammatory cytokines and activates endothelial cells that can damage multiple organ systems.

Answer 202

1. Complete blood count with differential WBCs and absolute neutrophilic count 2. CRP: serial measures 3. Cultures o Blood culture and other cultures 4. Others o Chest X ray and arterial blood gas for suspected pneumonia o Lumber puncture for suspected meningitis: CSF analysis & CSF culture o Hyperglycemia, metabolic acidosis may be present o Thrombocytopenia, increased PT, PTT, INR in severely ill neonates

Answer 203

1. Antibiotics o Type: Empirically (first line): Ampicillin + Aminoglycoside (as gentamycin), then according to results of the culture. o Duration: 14 days in proven sepsis, 3 weeks in meningitis and 6 weeks in septic arthritis 2. Supportive o Respiratory support including mechanical ventilation for pneumonia o Anticonvulsants for seizures o Volume and pressor support for hypotension and poor perfusion

Answer 204

1. Severe burning pain, drooling, vomiting, dysphagia 2. Respiratory: Dyspnea, strider, cyanosis 3. Oral lesions may be absent 4. Odynophagia is prominent at the onset 5. Dysphagia and vomiting

Answer 205

Esophageal perforation and mediastinitis related death Malnutrition and weight loss because of dysphagia Higher incidence of aspiration and recurrent chest infections Esophageal strictures have a negative impact on the child's life

Answer 206

a. Vomiting in doing well baby * Amniotic gastritis * Hemorrhagic disease of the newborn * Swallowed maternal blood * Feeding problems (overfeeding) * Gastroesophageal reflux b. Vomiting in sick baby 1. Medical conditions * Infections (septicemia, meningitis) * Increased intracranial pressure * Metabolic: Galactosemia 2. Surgical conditions * Esophageal atresia with trachea-esophageal fistula * Congenital hypertrophic pyloric stenosis {vomiting after 2-3 weeks) * Intestinal obstruction {bile stained vomitus) Vomiting in infancy and childhood: Diet related, Infections, DKA, chronic kidney disease

Answer 207

A. Acute diarrhea 1. Infective diarrhea(= Gastroenteritis) a. Bacterial agents: * Salmonella * E. coli, b. Viral agents: Rotavirus, adenovirus, norovirus c. Parasitic agents: Entameba histolytica, Giardia lamblia. Non-infective diarrhea: -Dietetic diarrhea: overfeeding, irregular feeding -Drug induced diarrhea: ampicillin

Answer 208

1. Lactose Intolerance 2. Cow milk protein allergy 3. Bacterial Overgrowth

Answer 209

a. Chronic Infections e.g. TB enteritis, Giardiasis b. Malabsorption 1. Celiac disease: sensitivity to gluten-containing cereals 2. Cystic fibrosis: characterized by recurrent chest infection and malabsorption 3. Cholestasis: all causes

Answer 210

Gastroenteritis should be the first possibility in every case of acute diarrhea a. Severity of the diarrhea Diarrhea can be mild, moderate or severe according to the number of motions: - Mild: 4-6 motions per day - Moderate: 6-10 motions per day - Severe: More than 10 motions per day b. Associated symptoms - High fever should suggest bacterial infections - Vomiting may be absent, mild or severe and persistent c. Possible complications d. Possible causative organism

Answer 211

-Dehydration: loss of ECF -Shock: hypovolemic, septic shock -ARF: decrease renal perfusion -Metabolic acidosis -Hypokalemia: ++Intestinal loss -Hypocalcemia: +++ intestinal loss -Bleeding: --Vit. K and DIC -Convulsions: toxic, metabolic, febrile -Malnutrition: marasmus and kwashiorkor

Answer 212

Composed of NaCl, Na citrate, KCl, Glucose - Diarrhea with No dehydration: 50-100 ml after each loose stools - Mild dehydration: 50-80 ml/Kg - Moderate dehydration: 80-100 ml/Kg - Thirst mechanism is effective in regulating the received amount -If child vomits, wait 10 min then continue more slowly Feeding: Enteral nutrition important for intestinal mucosa repair. Breast milk, or solid foods in older kids Symptomatic treatment: antiemetics, antipyretics, metronidazole for giardia or entamoeba Antibiotics: usually not used as it can ruin gut flora only in: -Cholera: tetracycline -Shigella: 3rd gen cephalosporin -Salmonella: 3rd gen cephalosporin

Answer 213

1. Shock therapy:(over 20-30 minutes) Lactated ringer (or normal saline) solution 10 -20 ml/kg . 2. Deficit therapy (over 6- 8 hours) * Glucose 5 % and saline in a ratio {1: 1) * Containing 20 mEq/L K when patient passes urine and serum K not elevated * Amount depends on the degree of dehydration a. 50 ml/kg in mild dehydration b. 80 ml/kg in moderate dehydration c. 100 ml/kg in severe dehydration 3. Maintenance therapy: (over the remaining 16-18 hours if needed) * Glucose 5 % and saline in the ratio (4:1 ) * Amount a. 100 ml/Kg for the first 10 kg b. 50 ml/kg for each kg from ll-20kg c. 20 ml/kg for each kg above 20kg * Given IV over the remaining 16-18 hrs if needed * If dehydration has been corrected, oral intake tolerated and no ongoing severe losses—> resume oral intake

Answer 214

GE Hepatitis Appendicitis Cholecystitis Pyelonephritis Peritonitis Pancreatitis HSP Lower lobe pneumonia DKA Sickle cell anemia (vaso-occlusive crisis) Drug intoxication Surgical causes

Answer 215

1) Dysfunctional (Psychogenic recurrent) abdominal pain: -Family troubles, loss of parents, jealousy of new sibling -School phobia -To gain attention -Imitation of adults 2) Organic abdominal pain -parasitic inf -Chronic constipation -bad diet -lactose intolerance -Peptic ulcer -Ulcerative colitis -Sickle cell anemia -Renal stones

Answer 216

* Incidence >90% of chronic abdominal pain cases * Site Peiumbilical (central) location is characteristic * Nature Vague (Dull aching} * Severity Not severe * Duration Subside in < 20 min * Appearance Healthy * Association No * Abdominal Examination Normal * Simple investigations Normal (CBC, urinalysis, stool, Abd. Xrays) * Investigations Normal * Treatment Reassurance

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