High Yield Topics for cracking PG Medical Entrance Exams

MyPGMEE presents High Yield Topics for fast revision that will help you prepare for PG entrance exams like AIPGMEE, DNB CET, AIIMS PG, JIPMER, UPSC.

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MyPGMEE Authors: Dr. Mudit Khanna, Dr. Sushant Bhanja, Dr. Punit Bhojani and Dr. Ashwin Singh Parihar.

Topic 1

Liddle Syndrome

1.      Occurs due to gain of function mutation of ENaC (epithelial Sodium channels)
Autosomal Dominant inheritance.

2.      Since these ENaC persist independent of Mineralocorticoid stimulus, Sodium is constantly reabsorbed with loss of Potassium and Protons.

3.      Hence, Serum Sodium is high, with a low potassium and alkalosis. This is also referred to as Hypokalemic Hypertension

4.      In response to the dyselectrolemic changes, renin is suppressed, thereby lowering levels of aldosterone.

5.      Now, this state is a low aldosterone state, but looking at the dyselectrolemias, it looks as if the aldosterone levels are high, hence the name PSEUDO-hyperaldosteronism.

6.      For treatment, all we need is to inhibit these ENaC, for which we use Potassium sparing diuretics like Amiloride/Triamterene. 

Topic 2

MEN syndromes          

MEN1 (WERMER SYNDROME)

1.      Mutation at Chromosome 11q of MENIN (tumor suppressor gene)

2.      Inheritance - AD / sporadic.

3.      Primary glands involved - Pituitary, Parathyroid, Pancreas.

4.      Parathyroid(most common inv) - hypercalcemia due to adenoma.

5.      Pituitary- Ant pituitary adenoma - most commonly producing prolactin.

6.      Entropancreatic tumors- gastrinoma(most common), insulinoma, vipoma, ppoma, glucagonoma.

7.      Other associated tumors- adrenocortical tumors, foregut carcinoids, lipoma, angiofibroma,  collagenoma, meningioma, pheochronocytoma(rare)

MEN2 (SIPPLE SYNDROME)

1.      Mutation of RET protooncogene at Chr 10.

2.      MEN2A- Medullary Ca Thyroid(most common), pheochromocytoma(maybe B/L), parathyroid adenoma(milder than in MEN1). Associated with hirschsprung disease, cutaneous lichen amyloidosis.

3.      MEN2B - MTC, Pheochromocytoma( both more aggressive than in MEN2A). Associated with marfanoid habitus, ganglioneuromas and megacolon.

4.      FMTC - only medullary ca thyroid.

MEN4

1.      Chr12 mutation.

2.      Parathyroid adenoma + Pituitary adenoma + Reproductive tumors
Asso with renal, adrenal tumors.

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