Core Conditions Continued 2 Flashcards
1
Q
What does aldosterone do?
A
Regulates salt and water balance by increasing the retention of sodium and water, and increasing the excretion of potassium.
2
Q
How is Addison’s diagnosed?
A
Short synacthen test: measure baseline cortisol, give synthetic ACTH, measure cortisol again after 30 and 60 minutes.
Failure of cortisol to rise by at least double = primary adrenal insufficiency
3
Q
Addison’s disease can cause which of the following:
- Metabolic alkalosis with normal anion gap
- Metabolic alkalosis with high anion gap
- Metabolic acidosis with normal anion gap
- Metabolic acidosis with high anion gap
A
Metabolic acidosis with normal anion gap
4
Q
Tx for Addison’s:
A
Hydrocortisone to replace cortisol
Fludrocortisone to replace aldosterone
Crisis: IV steroids, IV fluids, correct hypoglycaemia, careful monitoring
Carry a medical ID tag, follow sick day rules (double steroids if temp >38 or having D&V)
5
Q
What is cushing’s syndrome?
Give 3 causes:
A
Syndrome of excessive cortisol
Primary: adrenal tumour secreting cortisol
Secondary: pituitary tumour secreting ACTH (Cushing’s Disease!)
Iatrogenic: corticosteroids
6
Q
4 functions of cortisol and the symptoms these cause in cushing’s syndrome:
A
- Anti-insulin → hyperglycaemia
- Lipolysis and fat mobilisation → buffalo hump, central adiposity
- Muscle protein degradation → Muscle wasting
- Anti-inflammatory → immunosuppression, easy bruising, poor wound healing
7
Q
Cushing’s Syndrome Presentation: (10)
A
“CUSHINGOID”
C - cataracts
U - ulcers
S - skin striae/bruising/thinning
H - hypertension and hirtuism
I - infection
N - necrosis of femoral head
G - glycosuria
O - obesity/osteoporosis
I - irritability/depression/lethargy/psychosis
D - diabetes
8
Q
How do you diagnose Cushing’s syndrome?
A
24 hour urinary cortisol test
Morning/midnight cortisol (if diurnal variation is preserved → unlikely to be cushing’s)
Dexamethasone suppression test
9
Q
What is Conn’s syndrome?
3 signs?
A
A unilateral adrenal adenoma that secretes excessive aldosterone.
Hypernatraemia, hypertension, hypokalaemia.
10
Q
What is a phaeochromocytoma?
A
A rare adrenal tumour that secretes cathecholamines.
Results in: episodic headaches, paroxysmal hypertension, excessive sweating, tachycardia
11
Q
What is acromegaly?
A
Excessive growth hormone, most commonly caused by a pituitary adenoma.
12
Q
3 symptoms of tissue overgrowth in acromegaly:
A
- Prominent forehead and brow (frontal bossing)
- Macroglossia
- Large hands and feet (shoes stop fitting, ring too small)
13
Q
What visual defect might acromegaly cause? Why?
A
Bitemporal hemianopia
Acromegaly is most commonly caused by a pituitary adenoma. If the adenoma is large, it’ll press on the optic chiasm.
14
Q
Ix for acromegaly:
A
Initial screening: insulin-like growth factor (raised)
OGGT whilst measuring growth hormone (high glucose SHOULD suppress growth hormone)
MRI pituitary
Visual fields testing
15
Q
Other than surgical removal of the pituitary tumour, how might you treat acromegaly?
A
Block growth hormone with:
- Somatostatin
- Dopamine agonists
- Pegvisomant
16
Q
What is carcinoid syndrome?
A
Occurs due to neuroendocrine tumours secrete hormones that result in diarrhoea, SOB and flushing.
17
Q
What metabolic abnormality does renal failure most commonly cause?
A
Raised anion gap metabolic acidosis
18
Q
What is anti-glomerular basement membrane disease? (AKA goodpasture’s syndrome)
A
A rare small vessel vasculitis associated with pulmonary haemorrhage and rapidly progressive glomerulonephritis.
Renal biopsy will show linear IgG deposits along the GBM. Tx is plasma exchange and steroids.
19
Q
What is haemolytic uraemic syndrome (HUS)?
A
A triad of: AKI, haemolytic anaemia, thrombocytopenia
Most commonly secondary to E.coli infection in children. Toxins (shiga toxin) released by E.coli trigger thrombosis in small blood vessels.
Typically onset 5 days after diarrhoea.
20
Q
2 causes of acute tubular necrosis:
A
- Ischaemia due to hypoperfusion (shock, sepsis, dehydration)
- Direct damage from toxins (radiology contrast dye, gentamicin, NSAIDs)
21
Q
Ix in acute tubular necrosis: (1)
A
Muddy brown casts on urinalysis
22
Q
What metabolic abnormality might salicylate poisoning cause?
A
Raised anion gap metabolic acidosis
23
Q
What is Alport’s syndrome?
A
An X-linked inherited condition.
A defect in the gene that codes for type IV collagen results in an abnormal glomerular basement membrane.
24
Q
How does Alport’s syndrome present?
A
Usually presents in childhood with:
- progressive renal failure
- microscopic haematuria
- bilateral sensorineural deafness
25
Why might a renal transplant fail in a patient with Alport's syndrome?
May have anti-GBM antibodies.
26
What is the emperical treatment for epididmo-orchitis caused by an STI?
IM ceftriaxone stat dose + doxycycline 10-14 days
27
What is the most common cause of acute epididmo-orchitis in sexually active younger adults?
Chlamydia
28
What is Paget's disease?
A disorder of excessive bone turnover, leading to patchy areas of high density (sclerosis) and low density (lysis).
Results in enlarged misshapen bones with structural problems and increased risk of pathological fractures.
Mainly affects the axial skeleton.
29
4 XR findings in Paget's disease:
1. Bone enlargement and deformity
2. Osteoporosis circumscripta = defined osteolytic lesions
3. Cotton wool appearance of the skull
4. V shaped osteolytic defects in long bones
30
How does Paget's disease affect blood results?
High ALP (all other LFTs normal)
Normal calcium
Normal phosphate
31
Why is septic arthritis an emergency?
Can cause irreversible joint damage within 24 hours
32
What is the acronym used to remember Kocher's criteria?
NEWT
Non-weight bearing
ESR >40 in first hour
WBC >12,000 cells/mm3
Temp >38.5
33
How do you treat septic arthritis?
IV flucloxacillin ± clindamycin for 4 weeks
Joint washout
Debridement
Pain relief
Splinting
Physio
34
What is SLE?
An autoimmune condition in which anti-nuclear antibodies attack proteins in cell nuclei triggering systemic inflammation.
35
Which antibody is the most specific for SLE?
Anti-dsDNA
36
Complications of SLE: (6)
CVS disease - HTN and coronary artery disease (leading cause of death)
Infection
Pulmonary fibrosis
Anaemia of chronic disease
Recurrent miscarriage
Lupus nephritis → end stage renal failure
37
Symptoms of SLE:
1. Photosensitive malar rash (butterfly shape on face)
2. Fever
3. Fatigue
4. Weight loss
5. Raynauds
6. Arthritis & joint pain
38
Tx of SLE: (4)
NSAIDs (pain relief)
Hydroxychloroquine (first line for mild SLE)
Steroids (prednisolone)
Suncream and sun avoidance
39
What is Sjogren's?
An autoimmune condition that affect exocrine glands → dry mucous membranes
40
Which antibodies are present in Sjogren's?
Anti Ro
Anti La
ANA
41
What is Marfan's syndrome?
An autosomal dominant condition affecting the gene responsible for creating fibrillin, an important component of connective tissue
42
Px of Marfan's syndrome: (7)
Tall stature
Long neck
Long limbs
Long finger (arachnodactyl)
High arch palate
Hypermobility
Pectus carinatum or excavatum
43
5 complications of Marfan's:
Aortic or mitral valve prolapse → regurgitation
Aortic aneurysms
Joint dislocations
Scoliosis of the spine
44
What type of anaemia does iron deficiency cause?
Microcytic anaemia
45
Give five causes of normocytic anaemia:
Anaemia of chronic disease
CKD
Aplastic anaemia
Haemolytic anaemia
Acute blood loss
46
What is acute lymphoblastic leukaemia?
Malignant proliferation of lymphoid progenitor cells
47
What kind of anaemia would beta thalassaemia cause?
Microcyctic
48
Give 6 blood transfusion reactions:
Haemolytic febrile reaction
Minor allergic reaction
Anaphylaxis
Acute haemolytic reaction
Transfusion-associated circulatory overload (TACO)
Tranfusion related acute lung injury
49
What is transfusion associated circulatory overload?
Fluid overload and pulmonary oedema due to rapid infusion of blood
50
Lead poisoning can cause what type of anaemia?
Microcytic
51
3 causes of macrocytic megaloblastic aneamia:
folate deficiency
b12 deficiency
cytotoxic drugs
52
5 causes of normoblastic macrocytic anaemia:
Liver disease
Hypothyroidism
Alcohol excess
Reticulocytosis
Pregnancy
53
Define the following types of miscarriage: missed, threatened, inevitable, incomplete, complete
Missed: fetus dead, no symptoms, cervix closed
Threatened: bleeding, cervix closed, fetus alive
Inevitable: bleeding, cervix open
Incomplete: retained products, cervix open
Complete: no retained products
54
After ovulation, how does pregnancy occur? (days 0 to 8)
Day 0 = ovulation - the corpus luteum starts producing oestrogen and progesterone
Day 1 = fertilisation (within 12 to 24 hours of ovulation)
Cells begin to divide, forming a blastocyst by day 4
Day 5 = implantation - the blastocyst implants, helped by a low oestrogen:progesterone ratio
Day 6 = the trophoblast (outer cells of the blastocyst) burrows into the endometrium
Day 8 = the trophoblast cells start producing hCG, which tells the corpus luteum a successful fertilisation and implantation has occurred so to carry on making oestrogen and progesterone (suppressing the development of any other new follicles)
55
How does the levels of oestrogen, progesterone and hCG change throughout pregnancy?
Where are these hormones produced?
In weeks 0 to 9 the corpus luteum is producing oestrogen and progesterone - levels rise continuously
Meanwhile, the trophoblast is producing hCG, this peaks at week 9 and then begins to fall...
This triggers the corpus luteum to degenerate and instead, the placenta now takes over...
Weeks 9 to 40 the placenta produces oestrogen, progesterone and a small amount of hCG
56
What is the hormone hPL? Why is it produced in pregnancy?
Human placental lactogen - made by the placenta to counteract the mother's insulin in order to provide the baby with enough glucose
57
Changes to the cardiovascular system in pregnancy: (6)
1. 30 to 50% increase in blood volume → decreased haematocrit and physiological anaemia of pregnancy
2. Increased HR by ~20 bpm to increase cardiac output (and push the extra blood around)
3. Slight fall in BP due to vasodilation (caused by progesterone)
4. Heart nudged slightly upwards by uterus, point of maximum intensity moves slightly to the left
5. Uterus presses on pelvic veins → varicose veins and swelling in legs and ankles
6. Uterus presses on inferior vena cava when laying down → hypotension (avoided by lying on your side)
58
How are the kidneys affected by pregnancy?
During pregnancy the blood volume increases by 30-50%.
This increases the blood flow to the kidneys, increasing the GFR and therefore increasing urinary output. This explains why pregnant women experience urinary frequency.
To compensate, the kidneys and ureters enlarge causing physiological hydropherosis and hydroureter.
High progesterone levels also cause hypermotility of the ureters.
The enlarged kidneys are at greater risk of urinary stasis and therefore increased risk of an upper UTI.
59
How does pregnancy affect the lungs?
The uterus presses on the lungs → SOB.
Progesterone causes ligaments in the thorax to relax → increased diameter of the ribcage
Increased diameter of the ribcage → increased tidal volume
Increased tidal volume → drop in blood CO2 levels
Lower CO2 → mild alkalosis
Mild alkalosis is advantageous for gasesous exchange across the placenta
60
How does oestrogen affect the upper respiratory tract in pregnancy?
Oestrogen → increased vascularisation & capillary engorgement
→ nasal stuffiness, nose bleeds, sinus congestion
61
What causes the 'waddling gait' seen in pregnancy?
Progesterone and relaxin (produced by the placenta) cause ligaments in the pelvis (sacroiliac joints and symphsis pubis) to relax in preparation for fetal passage through the birth canal in labour.
62
How does pregnancy affect the GI system?
Hormonal changes lead to smooth muscle relaxation and decreased peristalsis → constipation and bloating
Hormonal changes can also lead to relaxation of the lower oesophageal sphincter → reflux
You can also get morning sickness
63
How does pregnancy affect breast tissue?
Oestrogen and progesterone trigger breast development which may result in symptoms of tingling, fullness and tenderness.
Oestrogen also stimulates prolactin secretion from the anterior pituitary. High levels of progesterone inhibits the affects of prolactin until after the baby is born. Once born, prolactin will stimulate milk letdown from the breasts.
64
What is the linea nigra seen in pregnancy?
The linea alba is a stripe of fibrous tissue that runs down the centre of the abdomen.
In pregnancy, the anterior pituitary secretes increased melanocyte-stimulating hormone which darkens the linea alba → linea nigra
It also darkens the nipples.
65
How is the thyroid affected by pregnancy?
The thyroid secretes more thyroid hormones to increase the cellular basal rate and meet the increased demands of the pregnancy
66
Why are pregnant women at a greater risk of VTE? Explain the physiology of this:
Oestrogen promotes blood clotting in two ways:
- Increases plasma fibrinogen and the activity of coagulation factors
- Decreases the activity of antithrombin III
This makes pregnancy a hypercoagulable state → increased risk of VTE
67
What is the difference between placenta accreta, placenta increta and placenta percreta?
Placenta accreta: placenta attaches to the myometrium
Placenta increta: placenta grows into the myometrium (inner muscular layer)
Placenta percreta: placenta invades through the perimetrium (outermost layer of the uterus)
68
Criteria for surgical management of an ectopic pregnancy: (6)
1. Foetal heartbeat
2. Size > 35mm
3. Ruputred
4. Pain
5. hCG >5,000 IU/L
6. Co-existing intrauterine pregnancy
69
How are perineal tears managed?
1st degree: no repair required
2nd: suture on the ward by suitably experienced clinician
3rd: repair in theatre
4th: repair in theatre
70
Which antibiotic should you NOT give to a breastfeeding woman?
Ciprofloxacin, tetracycline, chlroamphenicol, sulphonamides
71
Two psychiatric drugs that are contraindicated in breastfeeding:
Lithium
Benzodiazepines
72
When should you advise a higher dose of folic acid prior to and during pregnancy?(8)
If any of the following apply:
- Patient's partner has NTD
- Prev pregnancy affected by NTD
- Family hx of NTD
- Patient taking antiepileptics
- Patient has coeliac disease
- Patient has diabetes
- Patient has thatlassaemia trait
- Patient has a BMI >30
73
What kind of anaemia does myeloma cause?
Macrocytic
74
How do you treat hypoglycaemia?
Conscious and able to swallow → oral glucose e.g. 40% glucose gel, repeat after 15 mins up to a maximum of 3 times
Decreased consciousness → IM glucagon in the community, IV glucose 10% or 20% in secondary care/whenever IV access is possible
NB: IM glucagon can cause flushing and nausea
75
Which diabetes type II medication is approved for use in CKD?
Sitagliptin (DDP4i)
76
Contraindications for pioglitazone: (4)
DKA
Hx of heart failure
Previous or active bladder cancer
Uninvestigated macroscopic haematuria
77
What are the two most common causes of hypercalcaemia?
1. Primary hyperparathyroidism
2. Malignancy
78
What is the first line emergency treatment for hypercalcaemia?
1. Increase circulating volume with 0.9% saline to help increase urinary output of calcium
79
If saline infusion doesn't help, what's the next step in treating hypercalcaemia?
IV zolendronic acid
80
What is a quinsy?
A peritonsillar abscess - this is a complication of acute tonsillitis, where pus is trapped between the tonsillar capsule and the lateral pharyngeal wall
81
What are the most common causative organisms of a quinsy? (2)
Strep. pyogenes
Staph. aureus
H. influenzae
82
How does a quinsy present? (4)
Trismus - difficulty opening mouth
Altered voice quality - "hot potato voice"
Painful swallow
Earache on affected side
General systemic signs of infection
83
How do you treat a quinsy? (4)
IV fluids
Analgesia
IV antibiotics: penicillin with metronidazole
Needle aspiration, incision and drainage
84
What advice about the progression of recovery from pneumonia can you give patients?
1 week = fever resolved
4 weeks = chest pain and sputum reduced
6 weeks = cough and breathlessness significantly reduced
3 months = most symptoms resolved, some fatigue present
6 months = most people feel back to normal
85
Mx of community acquired pneumonia:
Low severity (CURB65 = 0-1)
5 days oral amoxicillin
Moderate severity (CURB65 = 2):
Amoxicillin PO or IV
+ clarithromycin or erythromycine (safe in pregnancy) if atypical pathogen suspected
Or doxycycline if pencillin allergic
High severity (CURB65 = 3+):
5 days PO or IV co-amoxiclav
+ clarithromycin or erythromycin (safe in pregnancy) if atypical pathogen suspected
Or levofloxacin if penicillin allergic
86
Describe the CURB-65 scoring system and what the results mean:
Confusion
Urea >7mmol/L
Resp rate > 30
Blood pressure low - systolic < 90, diastolic <60
65 years or older
0-1 = low risk, <3% mortality
2 = intermediate risk, 3-15% mortality, consider hospital based care
3-5= high risk, >15% mortality, consider ITU care
87
Indications for possible ICU admission with pneumonia: (7)
Severe pneumonia (CURB65 >3) with serious co-mordbities
Respiratory or metabolic acidosis
Hypotension
PaO2 <8 despite inspired oxygen >60%
Progressive hypercapnia on serial ABGs
Exhaustion/drowsiness/LOC
Evidence of septic shock
88
What kind of antibiotic are penicillins?
How do they work?
Give 3 examples:
Beta-lactams
Inhibit cell wall synthesis
Benzylpenicillin, flucloxacillin, amoxicillin
89
What kind of antibiotics are cephalosporins?
How do they work?
Give 2 examples:
Beta-lactams
Inhibit cell wall synthesis
Cephalexin, cefuroxime
90
What kind of antibiotic are carbapenems?
How do they work?
Give 2 examples:
Beta-lactam
Inhibit cell wall synthesis
Meropenem, ertapenem
91
Give 3 groups of antibiotics are that beta-lactams:
1. Penicillins
2. Cephalosporins
3. Carbapenems
92
How do glycopeptide antibiotics work?
Give 2 examples;
Inhibit cell wall synthesis
Vancomycin, teicoplanin
93
How do fluroquinolone antibiotics work?
Give 2 examples:
Inhibit nucleic acid synthesis
Ciprofloxacin, levofloxacin
94
Give three groups of antibiotics which work by inhibiting protein synthesis:
Macrolides
Tetracyclines
Aminoglycosides
95
What kind of antibiotic is gentamycin and streptomycin?
Aminoclycoside
96
What kind of antibiotic is doxycycline?
Tetracycline
97
What kind of antibiotic are clarithromycine and erythromycin?
Macrolides
98
If a bacteria is resistant to all beta-lactam antibiotics (not just penicillins), what kind of organism is this?
An extended spectrum beta-lactamase producer
99
What do you call a pleural effusion that is high in protein?
Give 3 possible causes:
Exudative
Cancer, TB, PE, pneumonia, autoimmune
100
What do you call a pleural effusion that is low in protein?
What causes this?
Transudative
High hydrostatic pressure or low plasma oncotic pressure, usually caused by: CHF, cirrhosis, nephrotic syndrome, PE or hypoalbuminaemia
101
What qualifies are recurrent miscarriage?
Three or more consecutive misscarriages
102
5 risk factors for miscarriage:
Increasing age (>30)
Smoking
Excess alcohol
Low pre-pregnancy BMI
Paternal age >45
Illicit drug use
Stress/traumatic events
Uncontrolled diabetes
103
What is the formula for calculating serum osmolarity?
(2 x Na+) + glucose + urea
104
Why does high serum glucose cause dehydration? (e.g. in diabetes)
High serum glucose drives water out of cells and into the blood - leaving cells dehydrated
High volumes of water leads to polyuria -> dehydration
105
Why does insulin affect potassium levels?
Insulin shifts potassium into cells by promoting the entry of sodium into cells, which potassium follows
106
Symptoms of hypokalaemia:
Mild: asymptomatic
Severe (<3): generalised weakness, muscle pain, constipation
Extremely severe (<2.5): respiratory failure, paraesthesia, severe muscle weakness and paralysis, ileus
107
ECG findings for hypokalaemia:
Typically evident at K+<3
Flat T waves
ST depression
Prominent U waves
108
Examination findings for severe hypokalaemia: (4)
Hypotension
Irregular pulse
Decreased muscle power
Diminished tendon reflexes
109
Reed-sternberg cells are found in which cancer?
Hodgkins lymphoma
110
What is lymphoma?
Malignant proliferation of lymphocytes, accumulating in the lymph nodes causing lymphadenopathy
111
What are reed-sternberg cells?
cells with mirror image nuclei
found in hodgkins lymphoma
112
What staging system is used for lymphoma?
Ann Arbor Staging
I = single lymph node region
II = two or more on the same side of the diagphragm
III = both sides of the diaphragm
IV = spread beyond the lymph nodes e.g. in the liver or bone marrow
113
How does lymphoma present? (7)
Large, rubbery, non-tender superficial lymph nodes
Pruritus
Mediastinal mass
Alcohol induced lymph node pain
'B' Symptoms:
- unexplained fever >38
- weight loss (>10% in 6 months)
- drenching night sweats
114
3 conditions that increase the risk of lymphoma:
Coeliac → increased risk of enteropathy associated T cell lymphoma
Chronic H.pylori → MALT lymphoma
Hashimoto's → MALT lymphoma
(MALT = mucosa-associated lymphoid tissue, a slow growing type of non-hodgkins lymphoma)
115
10 mg of oral morphine is equivalent to what dose of oral codeine?
100 mg
116
10 mg of oral morphine is equivalent to what dose of IM/IV/SC morphine?
5 mg
117
10 mg of oral morphine is equivalent to what dose of IM/IV/SC diamorphine?
3 mg
118
10 mg of oral morphine is equivalent to what dose of oral oxycodone?
6.6 mg
119
10 mg of oral morphine is equivalent to what dose of oral tramadol?
100 mg
120
What is the principle of double effect?
Sometime is it permissable to cause harm as a side effect of bringing about a good result provided that the side effect was not an intended outcome
121
Causes of iron deficiency anaemia: (4)
1. Insufficient dietary intake
2. Increased iron requirements (pregnancy)
3. Loss of iron (bleeding)
4. Inadequate absorption of iron (coeliac, Crohn's)
122
What would a blood film of iron deficiency anaemia show?
Microcytic hypochromic anaemia with target cells and pencil cells
123
What is sideroblastic anaemia?
Failure of haem production caused by a congenital defect or aquired (lead poisoning, anti-TB drugs).
Blood film will show ringed sideroblasts.
124
4 hereditary conditions that cause haemolytic anaemia:
Hereditary spherocytosis
G6PD deficiency
Sickle-cell
Thalassaemia
125
4 acquired conditions that cause haemolytic anaemia:
Haemolytic newborn disease
Drug induced (penicillin, methyldopa)
Prosthetic heart valves
Infections (malaria)
126
What is hereditary spherocytosis? What is its pattern of inheritance?
Autosomal dominant condition affecting red blood cells.
Membrane disorder causing abnormal sphere-shaped red blood cells which are broken down by the spleen → anaemia, jaundice, splenomegaly
127
What is G6PD deficiency? What is the pattern of inheritance?
An X-linked inherited condition causing deficiency of the G6PD enzyme needed to prevent oxidative damage to cells.
128
What might trigger a crisis in G6PD deficiency?
Infection, broad beans, medications → acute haemolysis, fever, jaundice, malaise, dark urine, sudden Hb drop
129
What is the inheritance pattern of sickle cell anaemia?
Autosomal recessive
130
What is the inheritance pattern for thalassemia?
Autosomal recessive
131
Diagnostic criteria for aplastic anaemia:
Hypocellular bone marrow with at least two of:
- Hb <10
- Neutrophils <1.5 x10⁹/L
- Platelets <50x10⁹/L
132
3 causes of megaloblastic macrocytic anaemia:
Folate deficiency
B12 deficiency
Cytotoxic drugs
133
3 causes of non-megaloblastic macrocytic anaemia:
Alcohol excess
Liver disease
Hypothyroidism
Pregnancy
134
Why does B12 deficiency cause anaemia?
B12 is required in the thymine production pathway to make DNA, therefore it can affect all cells and develop into pancytopenia
135
How is B12 absorbed?
Gastric parietal cells release intrinsic factor that binds to B12 and protects it until it reaches the terminal ileum where it is absorbed via cubulin receptors
136
Pathophysiology of pernicious anaemia:
Autoantibodies against intrinsic factor prevent the absorption of B12
B12 deficiency leads to anaemia
137
How do you treat B12 deficiency?
1 mg cyanocobalamin PO OD if there are no signs of pernicious anaemia or malabsorption
or
1mg hydroxycobalamin IM 3 times/week for 2 weeks, then once every 3 months
Also need folic acid and to monitor K+ (starts to drop 5 days after starting replacement)
138
What kind of anaemia does B12 deficiency cause?
Megaloblastic macrocytic anaemia
139
Down's syndrome is associated with an increased risk in which type of leukaemia?
ALL
140
Explain the pathophysiology of ALL:
ALL is the malignant proliferation of lymphoid progenitor cells.
Lymphoid precursors proliferate in their immature state and replace normal cells of the bone marrow.
Blast cells (immature cells) also spill into peripheral circulation.
This proliferation and infiltration in the bone marrow leads to anaemia, neutropenia and thrombocytopenia.
The majority of ALL cases are of a B lymphocyte origin but some are from T lymphocyte precursors.
141
Explain the pathophysiology of acute myeloid leukaemia:
AML is the malignant proliferation of myeloid progenitor cells.
Myeloid precursor cells proliferate in their immature state and replace the normal cells of the bone marrow.
Blast cells (immature cells) also spill into the peripheral circulation.
Leads to anaemia, neutropenia, thrombocytopenia.
142
The presence of auer rods indicates which type of leukaemia?
AML
143
Which is the most common type of leukaemia overall?
CLL
144
Epidemiology of ALL: (4)
Single most common childhood cancer
Presentation peaks at 2-5 years
Affects boys > girls
Down's syndrome increases the risk by 10 to 20 fold
145
The philadelphia chromosome is present in which types of leukaemia?
>80% of cases of CML
30% of adult cases of ALL, 3-5% of childhood cases of ALL
146
Which age groups are affects by ALL, CLL, AML and CML?
ALL = children under 5
CLL = adults over 55
CML = adults over 65
AML = adults over 75
147
What is the philadelphia chromosome?
Translocation of chromosome 9 and 22
148
What is it called when CML transforms into high-grade lymphoma?
Richter's transformation
149
Which type of leukaemia is associated with warm haemolytic anaemia?
CLL
150
Which type of leukaemia is associated with smudge/smear cells?
CLL
151
What is myeloma?
Cancer of the plasma cells resulting in large quantities of antibodies being produced (most commonly excess IgG)
152
What is MGUS?
Monoclonal gammopathy of undetermined significance: excess of a single type of antibody/antibody components without other features of myeloma or cancer
Needs to be monitored in case of progression
153
Symptoms of myeloma (4):
Calcium elevated
Renal failure (immunoglobulins block renal tubules)
Anaemia
Bone lesions
154
Ix of myeloma: (6)
Bence jones proteins detected using urine electrophoresis
Serum free light chain assay
serum protein electrophoresis
bone marrow biopsy
assess for bone lesions with whole body MRI (punched out lesions, lytic lesions, raindrop skull)
155
How do you treat malaria?
IV artesunate or quinine dihydrochloride (for severe or complicated malaria)
Or
oral doxycycline (for uncomplicated malaria)
156
What might cause an anaphylactic reaction to a blood transfusion?
An IgA deficieny patient with anti-IgA antibodies will have an anaphylactic reaction in response to IgA antibodies from the donor blood
157
Symptoms of the following transfusion reactions:
ABO haemolytic reaction
Transfusion associated circulatory overload
Transfusion related acute lung injury
ABO haemolytic reaction: fever, abdo pain, hypotension
Transfusion associated circulatory overload: pulmonary oedema, hypertension
Transfusion related acute lung injury: non-cardiogenic pulmonary oedema, hypoxia, hypotension, fever, pulmonary infiltrates on CXR
158
What metabolic abnormalities might you see in tumour lysis syndrome?
High potassium
High phosphate
Low calcium
May also see: increased creatinine, cardiac arrhythmia or sudden death, seizure
159
What can be given as prophylaxis against tumour lysis syndrome in a patient starting chemotherapy?
Allopurinol IV or oral
160
What is methaemoglobinaemia?
Give one congenital and one acquired cause:
Hb has been oxidised from Fe2+ to Fe3+ resulting in tissue hypoxia has Fe3+ cannot bind to oxygen.
Congential: Hb chain variants e.g. HbM, HbH
Acquired: drugs e.g. poppers (nitrates), sulphonamides, aniline dyes
161
What is the treatment for methaemoglobinaemia?
Acquired: IV methylene blue
Congenital NADH methoglobinaemia reductase deficiency: ascorbic acid
162
What anticoagulant should a pregnant woman with anti-phospholipid syndrome be on?
LMWH (and aspirin to reduce risk of pre-eclampsia)
163
Mx of polycythaemia vera:
Aspirin: to reduce risk of thrombotic events
Venesection: to keep Hb in normal range
Chemotherapy: hydroxyurea - only used second line, risk of secondary leukaemia
164
What are the main components of managing a vaso-occulisve crisis in sickle-cell disease?
IV analgesia, IV fluids, oxygen
165
What is Paget's disease?
A disorder of bone turnover, where there is excessive osteoblast and osteoclast activity. Leads to areas of high density (sclerosis) and low density (lysis).
Cause is unknown (multifacotrial/autoimmune)
166
Presentation of Paget's disease: (4)
Bone pain
Bone deformity
Fractures
Hearing loss if bones of the ear are affected
167
X-ray findings in paget's disease: (4)
Bone enlargement and deformity
Osteoporosis circumscripta - osteolytic lesions that appear less dense than normal bone
Cotton wool skull - patchy areas of increased and decreased density
V shaped defect - long bones with v shaped lytic lesions
168
What is mesenteric adenitis?
Inflamed abdominal lymph nodes, typically occurring in children associated with tonsillitis or an URTI. No treatment required.
169
What is spondylothesis?
Anterior or posterior displacement of a vertebra out of line with the one below.
Common pathology, often resulting in lumbar canal stenosis.
Common cause is degenerative changes with age.
170
What is spondylolysis?
A bony defect in the pars interarticularlis of the vertebral arch, separating the dorsum of the vertebra from the centrum.
Most common affects the fifth lumbar vertebra.
Bony defect can be congenital or due to a stress fracture)
171
What is a vault prolapse?
Following a hysterectomy, the vault (top) of the vagina descends into the vagina.
172
What is a retrocele?
A prolapse of the rectum into the vagina throgh the posterior vaginal wall.
Px: constipation, urinary retention, palpable lump in the vagina.
173
What is a cystocele?
A prolapse of the bladder into the vagina through the anterior vaginal wall.
174
Mx of a pelvic organ prolapse: (2)
1. Conservative:
- physio (pelvic floor exercises)
- weight loss
- lifestyle changes
- vaginal oestrogen
2. Vaginal pessary (ring, shelf, cube, donut, hodge)
175
How many words should a child express by 18 months old?
5-10 words
176
How many words should a child express by 2 years old?
50+ words and will combine 2 words
177
At what age should a child be able to attempt feeding itself with a spoon?
14-18 months
178
Number needed to treat:
1/absolute risk reduction
absolute risk reduction: control event rate - experimental event rate
Example:
A control group has 240 participants and 48 deaths in two years.
The experimental group has 120 participants and 12 deaths in 2 years.
Control event rate: 48/240 = 0.2
Experimental event rate: 12/120 = 0.1
Absolute risk reduction: 0.2 - 0.1 = 0.1
NNT: 1/0.1 = 10
179
What is the usualy eGFR cut off for stopping metformin in an AKI?
eGFR<45 = stop metformin
180
When should you give IV calcium gluconate for hyperkalaemia?
Severe hyperK e.g. >5.5
181
When should you refer a patient with CKD to a specialist? (4)
eGFR <30
ACR ≥ 70 mg/mmol
Accelerated progression defined as a decrease in eGFR if 15 or 25% or 15 ml/min in 1 year
Uncontrolled hypertension despite ≥ 4 antihypertensives
182
When should you offer an ACEi in possible CKD?
Diabetes + ACR >3
HTN + ACR>30
All patients with ACR>70
NB: don't forget to monitor serum potassium as both CKD and ACEi cause hyperkalaemia!
183
4 aims of CKD management:
Slow progression → optimise diabetic and HTN control
Reduce CVS risk → lifestyle modification, statin, dietary advice
Reduce risk of complications → lifestyle modification, statin, dietary advice
Treat complications → oral bisphosphonates, vit D, iron, EPO
184
Give 3 causes of cranial diabetes insipidus:
Haemochromatosis
Post head injury
Sarcoidosis
Idiopathic
Pituitary surgery
185
Give three causes of nephrogenic diabetes insipidus:
Genetic defect in ADH receptor
Lithium
Tubulo-interstitial disease e.g. sickle-cell
186
4 causes of avascular necrosis of the hip in adults:
Long term steroid use
Chemotherapy
Alcohol excess
Trauma
187
What adverse reaction is caused co-prescribing azathioprine and allopurinol? Why?
Bone marrow suppression
Both are xanthine oxidase inhibitors
188
Is azathioprine safe to use in pregnancy? Name a rheumatological condition it might be used to treat
Yes
SLE
189
In cauda equina, what is a late sign that indicates possible irreversible damage?
Incontinence
190
What imaging do you need for suspected cauda equina?
MRI spine within 6 hours
191
What are the salter harris fracture types?
1 = fracture through the physis (growth plate) only
2 = fracture through the physis and metaphysis
3 = fracutre through physis and epiphysis, to include the join
4= fracture involving physis, metaphysis and epiphysis
5 = crush injury involving the physis
192
What is Felty's syndrome?
An extra-articular manifestation of rheumatoid arthritis. Has a classic triad of: neutropenia, hepatosplenomegaly, low white cell count
193
What is Felty's syndrome?
An extra-articular manifestation of rheumatoid arthritis. Has a classic triad of: neutropenia, hepatosplenomegaly, low white cell count
194
What causes subacute degeneration of the spinal cord? How does it present?
B12 deficiency resulting in impairment of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts.
195
Features of subacute degeneration of the spinal cord:
Dorsal column involvement:
- impaired proprioception and vibration
- distal tingling/burning/sensory loss symmetrically but affecting legs>arms
Lateral corticospinal tract involvement:
- muscle weakness, hyperreflexia, spasticity
Spinocerebellar tract involvement:
- sensory ataxia
- positive rhombergs
