[MCQ] Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 2

I. Start the exam by click the “Start” button

Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 2

Please wait while the activity loads.
If this activity does not load, try refreshing your browser. Also, this page requires javascript. Please visit using a browser with javascript enabled.

If loading fails, click here to try again

Start

Congratulations - you have completed Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 2. You scored %%SCORE%% out of %%TOTAL%%. Your performance has been rated as %%RATING%%

Your answers are highlighted below.

Question 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

Question 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

Question 3

Which of the following is false for Liddle’s syndrome ?

A	Hypertension
B	Hypokalemia
C	High aldosterone levels
D	Metabolic alkalosis

Question 3 Explanation:

Liddle’s syndrome mimics a state of aldosterone excess. Features include early and severe hypertension, hypokalemia & metabolic alkalosis. Plasma aldosterone & renin levels are low

Question 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

Question 4 Explanation:

Amiloride or triamterene block ENaC and, combined with salt restriction, provide effective therapy for hypertension and hypokalemia

Question 5

Polyuria is a prominent feature of all except ?

A	ADPKD
B	Medullary cystic kidney disease (MCKD)
C	Medullary cystic kidney disease (MCKD)
D	Gitelman’s syndrome

Question 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

Question 6 Explanation:

In antenatal period, fetal polyuria may cause maternal polyhydramnios & premature labor in Bartter’s syndrome.

Question 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-transporter

Question 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

Question 8 Explanation:

Hereditary nephrogenic diabetes insipidus (NDI) presents in infancy with severe vasopressinresistant polyuria, dehydration, failure to thrive, and dilute urine despite the presence of hypernatremia.

Question 9

Which of the following renal tubular acidosis type is rare ?

A	1
B	2
C	3
D	4

Question 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

Question 10 Explanation:

Inherited renal tubular acidosis presents as nonanion gap (hyperchloremic) metabolic acidosis from proximal tubular bicarbonate wasting or impaired distal net acid excretion.

Question 11

Inherited renal tubular acidosis presents as nonanion gap (hyperchloremic) metabolic acidosis from proximal tubular bicarbonate wasting or impaired distal net acid excretion.

A	Ehler-Danlos syndrome
B	Fabry’s disease
C	Wilson’s disease
D	All of the above

Question 12

Inherited renal tubular acidosis presents as nonanion gap (hyperchloremic) metabolic acidosis from proximal tubular bicarbonate wasting or impaired distal net acid excretion.

A	Ehler-Danlos syndrome
B	Fabry’s disease
C	Wilson’s disease
D	All of the above

Question 13

Systemic disorder that cause type 1 RTA include ?

A	Sjogren’s syndrome
B	Chronic active hepatitis
C	Lupus
D	All of the above

Question 14

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

Question 14 Explanation:

Inherited disorders that produce Type 2 (Proximal) RTA are Wilson’s disease, cystinosis, tyrosinemia, galactosemia, hereditary fructose intolerance, glycogen storage disease type I, and Lowe’s syndrome.

Question 15

Serum potassium is high in which of the following RTA ?

A	1
B	2
C	4
D	All of the above

Question 15 Explanation:

Type 4 RTA is acquired in association with moderate renal dysfunction & is characterized by hyperkalemia.

Question 16

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

Question 17

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 1 RTA is due to impaired H+ ion secretion or HCO3- reabsorption in distal nephron
C	Type 1 RTA may present as “marble-brain disease”
D	None of the above

Question 18

Marble-brain disease consists of ?

A	Osteopetrosis
B	Short stature
C	Mental retardation
D	All of the above

Question 19

Marble-brain disease is due to mutations in ?

A	CA2
B	SLC3A1
C	SLC6A19
D	CLCN5

Question 19 Explanation:

Marble-brain disease consists of osteopetrosis, short stature, and mental retardation with dRTA. It is due to mutations in CA2 (carbonic anhydrase II).

Question 20

Which of the following is not a feature of Type 1 (Distal) RTA ?

A	Hypokalemia
B	Hypocitraturia
C	Hypocalciuria
D	Rickets or osteomalacia

Question 20 Explanation:

Type 1 (Distal) RTA presents as hypokalemia, hypocitraturia, hypercalciuria, nephrocalcinosis, nephrolithiasis. Chronic untreated acidosis may lead to rickets or osteomalacia

Question 21

In Type 1 (Distal) RTA, patients fail to acidify urine to pH ?

A	< 5.5
B	< 6.5
C	< 7.0
D	< 7.5

Question 21 Explanation:

In Type 1 (Distal) RTA, patients fail to acidify urine to pH <5.5 even after acid loading with oral ammonium chloride or calcium chloride

Question 22

Which of the following stone is formed in Type 1 (Distal) RTA ?

A	Uric acid
B	Cystine
C	Calcium oxalate
D	Calcium phosphate

Question 22 Explanation:

Increased proximal citrate absorption leads to hypocitraturia and together with hypercalciuria predisposes to nephrocalcinosis & calcium phosphate stone formation

Question 23

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

Question 23 Explanation:

In Type 1 (Distal) RTA, bicarbonate replacement dose is 1 - 3 mmol/kg/day in divided doses. While, in Type 2 (proximal) RTA the dose is 5 - 15 mmol/kg/day along with supplemental potassium & vitamin D.

Question 24

Renal glucosuria is due to mutations in which gene ?

A	SLC6A19
B	SLC6A19
C	SLC3A1
D	SLC7A9

Question 24 Explanation:

Renal isolated glucosuria with normal blood glucose concentration is due to mutations in SLC5A2 gene that encodes sodium-glucose co-transporter SGLT2 in the proximal renal tubule.

Question 25

Low-molecular-weight proteinuria is a feature of ?

A	Renal glucosuria
B	Hartnup Disease
C	Cystinuria
D	Dent’s disease

Question 25 Explanation:

Low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis and nephrolithiasis are features of Dent’s disease.

Question 26

Cerebellar ataxia & pellagra-like skin lesions are a feature of ?

A	Renal glucosuria
B	Hartnup Disease
C	Cystinuria
D	Dent’s disease

Question 26 Explanation:

In Hartnup disease, tryptophan is retained in intestinal lumen & converted to indole compounds that are toxic to CNS. Consequent niacin deficiency leads to skin manifestations.

Question 27

Inherited systemic disorders associated with Fanconi syndrome include ?

A	Wilson’s disease
B	Galactosemia
C	Tyrosinemia
D	Tyrosinemia

Question 28

Acquired disorders associated with Fanconi syndrome include ?

A	Multiple myeloma
B	Heavy metal toxicity
C	Chemotherapeutic drugs
D	All of the above

Once you are finished, click the button below. Any items you have not completed will be marked incorrect. Get Results

There are 28 questions to complete.

←

List

→

Return

Shaded items are complete.

1	2	3	4	5
6	7	8	9	10
11	12	13	14	15
16	17	18	19	20
21	22	23	24	25
26	27	28	End

Return

See all quizzes of Polycystic Kidney Disease and Other Inherited Tubular Disorders at here:

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

Polycystic Kidney Disease and Other Inherited Tubular Disorders

[MCQ] Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 2

I. Start the exam by click the “Start” button

Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 2

Share this:

Related

[MCQ] Polycystic Kidney Disease and Other Inherited Tubular Disorders- Part 1

[MCQ] Vascular Injury to the Kidney

You may also like

Leave a Comment Cancel Reply