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Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 2
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Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 1| Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 2
1.Carpopedal spasm, cramps & tetany are clinical features of ?
A. Bartter’s syndrome
B. Gitelman’s syndrome
C. Liddle’s syndrome
D. Von Hippel-Lindau Disease
2. Which of the following best relate to Liddle’s syndrome ?
A. Unregulated sodium reabsorption
B. Overactive ENaC in cortical collecting duct
C. Chloride-independent sodium reabsorption
D. All of the above
3. Which of the following is false for Liddle’s syndrome ?
A. Hypertension
B. Hypokalemia
C. High aldosterone levels
D. Metabolic alkalosis
4. Which of the following drug is ineffective in treatment of Liddle’s syndrome ?
A. Spironolactone
B. Amiloride
C. Triamterene
D. All of the above
5. Polyuria is a prominent feature of all except ?
A. ADPKD
B. Medullary cystic kidney disease (MCKD)
C. Bartter’s Syndrome
D. Gitelman’s syndrome
6. During antenatal period, fetal polyuria causes maternal polyhydramnios and premature labor in ?
A. ADPKD
B. Medullary cystic kidney disease (MCKD)
C. Bartter’s syndrome
D. Gitelman’s syndrome
7. ENaC best relates to ?
A. Amiloride-sensitive epithelial sodium channel
B. Thiazide-sensitive Na-Cl co-transporter
C. Na-K-2Cl co-transporter
D. Sodium-bicarbonate co-transporte
8. Which of the following is false about hereditary nephrogenic diabetes insipidus (NDI) ?
A. High plasma levels of vasopressin
B. Hyponatremia
C. Polyuria
D. Seizures & mental retardation
9. Which of the following renal tubular acidosis type is rare ?
A. 1
B. 2
C. 3
D. 4
10. Serum anion gap is normal in which of the following renal tubular acidosis (RTA) type ?
A. 1
B. 2
C. 4
D. All of the above
11. Hereditary diseases that cause type 1 RTA include ?
A. Ehler-Danlos syndrome
B. Fabry’s disease
C. Wilson’s disease
D. All of the above
12. Systemic disorder that cause type 1 RTA include ?
A. Sjogren’s syndrome
B. Chronic active hepatitis
C. Lupus
D. All of the above
13. Which of the following inherited disorders produce Type 2 (Proximal) RTA ?
A. Wilson’s disease
B. Cystinosis
C. Glycogen storage disease type I
D. All of the above
14. Serum potassium is high in which of the following RTA ?
A. 1
B. 2
C. 4
D. All of the above
15. Drug-induced type 4 RTA is due to which of the following drugs ?
A. NSAIDs
B. ACE inhibitors
C. Heparin
D. All of the above
16. Which of the following statements is false ?
A. Type 1 RTA is due to impaired H+ ion secretion or HCO3-reabsorption in distal nephron
B. Type 2 RTA is due to impaired HCO3- reabsorption in proximal
tubule
C. Type 1 RTA may present as “marble-brain disease”
D. None of the above
17. Marble-brain disease consists of ?
A. Osteopetrosis
B. Short stature
C. Mental retardation
D. All of the above
18. Marble-brain disease is due to mutations in ?
A. CA2
B. SLC3A1
C. SLC6A19
D. CLCN5
19. Which of the following is not a feature of Type 1 (Distal) RTA ?
A. Hypokalemia
B. Hypocitraturia
C. Hypocalciuria
D. Rickets or osteomalacia
20. In Type 1 (Distal) RTA, patients fail to acidify urine to pH ?
A. < 5.5
B. < 6.5
C. < 7.0
D. < 7.5
21. Which of the following stone is formed in Type 1 (Distal) RTA ?
A. Uric acid
B. Cystine
C. Calcium oxalate
D. Calcium phosphate
22. Bicarbonate replacement dose in Type 1 (Distal) RTA is ?
A. 0.2 – 1 mmol/kg/day
B. 1 – 3 mmol/kg/day
C. 3 – 5 mmol/kg/day
D. 5 – 10 mmol/kg/day
23. Renal glucosuria is due to mutations in which gene ?
A. SLC6A19
B. SLC5A2
C. SLC3A1
D. SLC7A9
24. Low-molecular-weight proteinuria is a feature of ?
A. Renal glucosuria
B. Hartnup Disease
C. Cystinuria
D. Dent’s disease
25. Cerebellar ataxia & pellagra-like skin lesions are a feature of ?
A. Renal glucosuria
B. Hartnup Disease
C. Cystinuria
D. Dent’s disease
24. Inherited systemic disorders associated with Fanconi syndrome include ?
A. Wilson’s disease
B. Galactosemia
C. Tyrosinemia
D. All of the above
25. Acquired disorders associated with Fanconi syndrome include ?
A. Multiple myeloma
B. Heavy metal toxicity
C. Chemotherapeutic drugs
D. All of the above