MEH Flashcards
What enzyme is missing in fructose intolerance, and so how would you treat it?
Aldolase missing. Remove fructose form diet.
How would you treat marasmus and kwashiorkor?
Correct any hypoglycaemia, hypothermia and dehydration. Replace electrolytes and micronutrients then start cautious feeding, of small amounts at regular intervals (5-10 kcal/kg/day increasing gradually over 1 week).
What drug is used to treat chronic alcohol dependence?
Disulfiram - an inhibitor of aldehyde dehydrogenase.
What drug is used to treat a paracetamol overdose?
Acetylcysteine - replenished glutathione levels.
How would you manage a patient with a genetic defect leading to deficiency of one of the enzymes in the urea cycle and defect in amino acid metabolism?
Low protein diet and replace amino acids in diet with keto acids.
How would you manage a patient with homocystinuria?
Low-methionine diet, avoiding milk, meat, fish, cheese eggs, nuts and peanut butter. Take cysteine, vitamins B6, betaine B12 and folate supplement.
How would you manage a patient with phenylketonuria?
Strictly controlled low phenylalanine diet, avoid artificial sweeteners, and avoid high protein foods eg meat, milk and eggs.
How would you manage a patient with hyperlipoproteinaemia?
Reduce cholesterol and saturated lipids in diet, increase fibre intake. Increase exercise and stop smoking. If no response to this then commence statins (inhibit HMG-CoA reductase), and bile salt sequestrants (force liver to produce more bile salts using more cholesterol).
How would you manage a patient with iron deficiency?
Dietary advice, oral iron supplements, intramuscular iron injections, intravenous iron, transfusion (not unless there is severe anaemia with imminent cardiac compromise).
How would you manage a patient with dyserythropoiesis?
Treat the underlying cause.
How would you manage a patient with thalassaemia?
Regular blood transfusions, iron chelation, folic acid, immunisation, holistic care (cardiology, endocrine and psychological. Stem cell transplantation in some patients.
What drug is used to treat severe neutropenia and sepsis after chemotherapy?
Recombinant GCSF?[.
What drug can be used to treat Hashimoto’s disease?
Oral thyroxine (T4).
What drug can be used to treat Grave’s disease and thyrotoxicosis?
Carbimazole - antithyroid drug. Also surgical excision and ablative dose of radioactive iodine.
What drug can be used to treat growth hormone deficiency (pituitary dwarfism)?
Growth hormone therapy.
What drugs can be used to treat a prolactinoma?
Bromocriptine and cabergoline - dopamine agonists.
What drugs can be used to treat acromegaly?
Surgically removed.
If in cavernous sinus then surgically remove and then:
Dopamine agonist eg cabergoline and somatostatin analogues eg octreotide.
GH receptor blocker eg pegvisomant.
What drugs can be used to treat hyperaldosteronism?
Spironolactone - mineralocorticoid receptor antagonist, and aldosterone-producing adenomas are removed surgically.
What drugs can be used to treat inflammatory disorders eg inflammatory bowel disease and rheumatoid arthritis and suppress immune reaction to organ transplantation?
Steroid drugs eg prednisolone.
What drugs can be used to treat an addisonian crisis?
Fluid replacement and cortisol.
What drugs can be used to treat Addison’s disease?
Glucocorticoid eg hydrocortisone and mineralcorticoid eg fludrocortisone.
What drugs can be used to treat bilateral hyperplasia of the adrenal glands?
Aldosterone antagonist.
How would you manage a baby with congenital adrenal hyperplasia?
Treat adrenal crisis, determine sex, long term glucocorticoid and mineralcorticoid. Corrective surgery.
How would you manage a patient with a pheochromocytoma and paraganglioma?
Alpha blockade eg phenoxybenzamine and beta blockade eg bisoprolol. Surgical excision.
