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6 out of 14

Which one of the following statements is wrong regarding APKD(AIIMS May 04)

A Kidneys are enlarged in size

B The presentation is unilateral

C Intracranial aneurysms may be associated

D Typically manifests in the 3rd decade

Ans. B The presentation is unilateral

1. Autosomal dominant polycystic kidney disease (ADPKD)

Prevalence: 1:1000, Genes on chromosomes 16 (PKD1) and 4 (PKD2).

Common Clinical Features

1. Flank pain and vague abdominal discomfort

2. Acute loin pain or renal colic due to hemorrhage into the cysts.

3. Hypertension may appear after the age of 20 years.

4. Nocturia, hematuria and urinary infection appear in the third or fourth decade.

5. CRF can occurs but with enlarge kidney size.

6. Nephrolithiasis commonly due to calcium oxalate may be there.

7. Cysts may be there in liver, pancreas, and spleen. Cysts are not present in lungs.(AIIMS May 07)

8. Intracranial (berry) aneurysmsalso may be present which can cause SAH.

9. Azotemia is usually progressive.

10. Commonest extra renal manifestation is hepatic cyst. (AIPG 06)

11. Colonic diverticulae are common.

12. Diagnosis is by ultrasound.


1. Treating BP is important to prevent cardiovascular complication and SAH.

2. Treat infections, dialysis or transplantation for ESRF, genetic counseling.

3. Pain may be helped by laparoscopic cyst removal or nephrectomy.

Controversy about most common extra renal feature in ADPKD

i. In 12th edition HarrisonPg 1196, There is no mention of occurrence of colonic diverticulosis in ADPKD.

ii. In 14th edition Harrison, (Pg 1563) it is clearly mentioned that most common extra renal feature is colonic diverticulosis.

iii. But in 17th& 18th edition above said line is not there and both these edition don’t mention clearly that which is the most common extra renal feature. However both mention that hepatic cyst is very common in ADPKD.

iv. Hence liver cyst should be the best answer to the question about most common extra renal feature of ADPKD!!!.

Medullary cystic disease

1. It manifests with polyuria, acidosis, and salt wasting, which precedes slowly progressive renal failure.

2. Cyst are in medulla

3. Autosomal dominant

4. Most patients with MCKD2 and the genetically related familial juvenile hyperuricemic nephropathy also have severe hyperuricemia and precocious onset of gout.(Ref. Hari- 18th ed., Pg. 2359)

Diagnosis : a. Ultrasound, b. CT (more sensitive)

Treatment: Supportive, Transplant

Kidney Flashcard List

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