Path Key Points 6

Question 1

Transfusion associated acute lung injury (TRALI)

Answer 1

During/<6 hrs after transplant
Fever, SOB, low O2, high HR, low BP
CXR - bilateral pulmonary infiltrates

Question 2

Delayed haemolytic transfusion reaction

Answer 2

Jaundice, rash several days later
High bilirubin, low haemoglobin, high reticulocytes
Ix - U&E

Question 3

Transfusion associated graft versus host disease

Answer 3

Immunosuppressed patients
Diarrhoea, liver failure, skin desquamaiton, bone marrow failure
Death in weeks/month post transfusion

Question 4

Post transfusion purpura

Answer 4

Purpura 7-10 days after transfusion

Mx - IVIG (resolves in 1-4 weeks)

Question 5

Transfusion associated haemosiderosis

Answer 5

Bronze diabetes, liver/heart failure, malaise, erectile dysfunction
Mx - iron chelation (desferoxamine) with transfusion after ferritin >1000

Question 6

Causes of hypernatraemia

Answer 6

Hypervolaemic - hypertonic saline, Conn’s syndrome
Euvolaemic - tachypnoea, sweating, DI
Hypovolaemic - diarrhoea and vomiting, burns, loop diuretics

Question 7

Causes of hypokalaemia

Answer 7

Renal - Conn’s syndrome, Cushing’s disease
Redistribution - insulin, beta agonist, metabolic alkalosis
Rare - renal tubular acidosis 1/2/4, hypomagnesaemia

Question 8

Causes of hyperkalaemia

Answer 8

Intake - parenteral nutrition
Transcellular movement - acidosis, DKA, rhabdomyolysis
Decreased excretion - AKI, CKD, spironolactone, Addison’s disease, NSAIDs, ACEI/ARB

Question 9

Pt is hyponatraemic - what do you do?

Answer 9

Assess serum osmolality

• high - glucose infusion
• normal - hyperlipidaemia/paraproteinaemia
• low - true hyponatraemia - now assess fluid status

Question 10

Causes of hypovolaemic hyponatraemia

Answer 10

Urinary sodium >20 –> renal - diuretics, Addison’s disease, salt losing nephropathy
Urinary sodium <20 –> non-renal - diarrhoea and vomiting, burns

Question 11

Causes of euvolaemic hyponatraemia

Answer 11

Hypothyroidism, glucocorticoid deficiency, SIADH

Question 12

Causes of hypervolaemic hyponatraemia

Answer 12

Urinary sodium >20 –> renal - AKI/CKD

Urinary sodium <20 –> non-renal - HF, liver failure

Question 13

Urine specific gravity

Answer 13

Normal - 1.000-1.030
High - hyponatraemia - dehydration, diarrhoea and vomiting, DM, SIADH, HF
Low - hypernatraemia - DI, ATN, primary polydipsia

Question 14

8hr fluid deprivation test

Answer 14

Urine osmolality is <300 after deprivation and >800 after desmopressin - cranial diabetes insipidus
Urine osmolality is <300 after deprivation and <300 after desmopressin - nephrogenic diabetes insipidus
Urine osmolality is >500 after deprivation and >500 after desmopressin - primary polydipsia

Question 15

Frederickson’s classification of primary hyperlipidaemia

Answer 15

Type 1 - familial hyperchylomicronaemia - LPL deficiency –> accumulation of triglycerides and chylomicrons
Type 2a - familial hypercholesterolaemia - LDL receptor mutation –> excess LDL –> tendon xanthoma
Type 3 - familial dysbetalipopproteinaemia - apoE deficiency –> accumulation of chylomicron remnants –> palmar xanthoma
Type 4 - familial hyperlipidaemia - excess VLDL –> pancreatitis

Question 16

Galactosaemia

Answer 16

Diarrhoea and vomiting, bilateral cataracts, high conjugated bilirubin
E. coli sepsis is common
Ix - urine reducing agents, red cell gal-1-put
Mx - no milk

Question 17

MCADD

Answer 17

Can cause cot death
Hypoketotic hypoglycaemia, hepato/cardiomegaly
Ix - acyl carnitine profile
Mx - cornstarch for hypoglycaemia

Question 18

Pharyngitis/tonsilitis treatment

Answer 18

Mild - 500mg penicillin V PO qds
Moderate - 1.2g benzylpenicillin IV qds + 500mg metronidazole IV tds
Oral step down if no positive cultures - 500mg penicillin V PO qds + 400mg metronidazole PO tds
Therapy is for 10 days

Question 19

Severe combined immunodeficiency disease

Answer 19

IL-2 receptor defect
Low T cells, no B cells or immunoglobulins
Fatal in first few months of life

Question 20

Leukocyte adhesion deficiency

Answer 20

Neutrophilia but absent pus in ulcers