High Yield Topics for cracking PGMEE

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

Topic 1

Pseudohypoaldosteronism type 2, also known as Gordon syndrome

1. Defect in WNK KINASES 1, 4 (AUTO. DOMINANT)
2. GoF mutation leading to increased activity of distal sodium-chloride transporter.
3. This leads to high S. Sodium and chloride levels; Hence Renin and aldosterone levels are normal to low.
4. Secondarily, S. Potassium is also raised due to impaired secretion; this also leads to acidosis.
5. This is a scenario of Hyperkalemic Hypertension with Hyperchloremic acidosis (correlate from above)
6. Treatment would require reduction of the activity of this transporter, and hence we use thiazides.

P.S. Hypercalciuria is a feature of gordon syndrome, and hence nephrolithiasis and bone demineralization may be seen.

Topic 2

Pulmonary Alveolar Proteinosis

1. >90% cases acquired, occurring due to IgG antibodies against GM-CSF; Congenital cases due to mutations in SP-B gene; Secondary cases due to acute silicosis and hematologic malignancies.
2. Males more commonly involved, usually present with non-productive cough. (Gelatinous expectoration may also be seen)
3. Pathogenesis is due to the impaired function of the alveolar macrophages. Inflammation is characteristicaly absent/very low.
4. Investigations: Polycythemia, Increased LDH, Hypergammaglobulinemia, High titres of anti-GM CSF igG, especially in BAL fluid.
5. CXR- Bat wing opacities.
6. HRCT - Ground glass appearance with thickened interlobular septa and intralobular structures.
7. Treatment: Whole lung lavage.

     

Topic 3

Pulmonary Alveolar Proteinosis

Alopecia Areata

First we have to know that AA is a CHRONIC, auto-immune disease which leads to destruction of hair (via CD8+ cells), and frequently involves nails in the form of pitting.
The resulting alopecia is a NON-SCARRING one and hence reversible.

1. Alopecia is patchy(leading to smooth bald patches), may involve complete scalp (A.A.totalis) or complete scalp and body hair (A.A.Universalis) {Totalis/Universalis indicate bad prognosis}
2. Due to its auto-immune etiology, it can be associated with thyroiditis, vitiligo and pernicious anaemia.
3. Classical exclamation hair is seen which has a thin proximal shaft and a normal distal shaft.
4. Majority of cases have spontaneous improvement. Those requiring treatment are given intralesional corticosteroid injections.

     

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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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