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Urinary System

Congenital Kidney Disorders Practice Questions

20 free Congenital Kidney Disorders practice questions for the NCLEX Exam. Tap an option to answer — you get instant feedback, the correct answer, and a detailed explanation for every question.

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Question 1 of 20 Medium

Which statement explains the association between Autosomal Dominant Polycystic Kidney Disease (ADPKD) and berry aneurysms?

  1. A Cysts directly compress intracranial arteries causing ballooning
  2. B ADPKD connective-tissue abnormalities raise intracranial aneurysm risk
  3. C Aneurysms arise from the same gene mutation that causes liver cysts
  4. D ADPKD-related hypertension protects against aneurysm formation

Correct answer: ADPKD connective-tissue abnormalities raise intracranial aneurysm risk

ADPKD carries increased risk of intracranial (berry) aneurysms due to vascular and connective-tissue abnormalities.

Question 2 of 20 Medium

Which inheritance pattern and typical age of presentation correctly match Autosomal Recessive Polycystic Kidney Disease (ARPKD)?

  1. A Autosomal dominant; symptoms begin childhood (ages 5-10)
  2. B X-linked recessive; symptoms begin adulthood (30-40)
  3. C Autosomal recessive; often presents in infancy or early childhood
  4. D Mitochondrial inheritance; presents in late adulthood

Correct answer: Autosomal recessive; often presents in infancy or early childhood

ARPKD is autosomal recessive and frequently presents in infancy or early childhood.

Question 3 of 20 Medium

Which diagnostic hallmark supports a diagnosis of Polycystic Kidney Disease (PKD)?

  1. A Unilateral kidney enlargement with cortical thinning
  2. B Bilateral enlarged kidneys with multiple fluid-filled cysts
  3. C Solitary large cyst in one kidney with normal contralateral kidney
  4. D Fine medullary cysts only seen on microscopic kidney histology

Correct answer: Bilateral enlarged kidneys with multiple fluid-filled cysts

PKD is characterized by bilateral kidney enlargement with numerous fluid-filled cysts disrupting normal parenchyma.

Question 4 of 20 Medium

Which extra-renal organ involvement is most characteristic and clinically important in ARPKD?

  1. A Pancreatic cysts causing pancreatitis
  2. B Congenital hepatic fibrosis and biliary tract abnormalities
  3. C Pulmonary cysts causing early respiratory failure
  4. D Cardiac septal defects causing heart failure

Correct answer: Congenital hepatic fibrosis and biliary tract abnormalities

ARPKD often involves the liver with congenital hepatic fibrosis and biliary duct ectasia, a hallmark of the disease.

Question 5 of 20 Medium

For the disorder Nephronophthisis, which clinical presentation is most typical in children?

  1. A Early childhood hypertension and gross hematuria
  2. B Polyuria, polydipsia, and progression to ESRD in adolescence
  3. C Massive proteinuria with hyperlipidaemia and edema
  4. D Nephrolithiasis with recurrent urinary tract infections

Correct answer: Polyuria, polydipsia, and progression to ESRD in adolescence

Nephronophthisis tends to present with polyuria and polydipsia, and progresses to End-Stage Renal Disease (ESRD) often in adolescence.

Question 6 of 20 Medium

A fetus is found on ultrasound to have massively enlarged echogenic kidneys and oligohydramnios. The most likely diagnosis is:

  1. A ADPKD (autosomal dominant polycystic kidney disease)
  2. B ARPKD (autosomal recessive polycystic kidney disease)
  3. C Unilateral renal agenesis
  4. D Horseshoe kidney

Correct answer: ARPKD (autosomal recessive polycystic kidney disease)

ARPKD can present prenatally with enlarged echogenic kidneys and oligohydramnios.

Question 7 of 20 Medium

Which genetic mutation is a distinguishing feature for ADPKD type 1?

  1. A PKHD1 gene mutation
  2. B PKD1 gene mutation
  3. C NPHS1 gene mutation
  4. D WT1 gene mutation

Correct answer: PKD1 gene mutation

ADPKD type 1 is caused by PKD1 gene mutation; PKHD1 causes ARPKD.

Question 8 of 20 Medium

A patient with ADPKD is treated with a vasopressin V2-receptor antagonist (e.g., tolvaptan). What is the rationale of this therapy?

  1. A It reduces fluid intake, thereby shrinking established kidney cysts
  2. B It lowers blood pressure, which directly halts further cyst growth
  3. C It lowers cyclic AMP in tubular cells, slowing cyst expansion
  4. D It raises glomerular filtration rate to flush cysts from the tubules

Correct answer: It lowers cyclic AMP in tubular cells, slowing cyst expansion

In ADPKD, increased cyclic AMP drives cyst growth; V2-receptor antagonists reduce cAMP and slow cyst expansion.

Question 9 of 20 Medium

Which congenital renal anomaly describes fusion of the kidneys at the lower poles, forming a U-shaped structure that often lies lower in the abdomen?

  1. A Horseshoe kidney
  2. B Crossed renal ectopia
  3. C Multicystic dysplastic kidney
  4. D Duplex collecting system

Correct answer: Horseshoe kidney

In horseshoe kidney, the lower poles fuse forming a “horseshoe” shape, and the kidneys often are positioned lower and anteriorly.

Question 10 of 20 Medium

In ARPKD, the histologic origin of cysts is predominantly:

  1. A Proximal convoluted tubule
  2. B Loop of Henle segments
  3. C Distal tubule, collecting duct
  4. D Glomerular Bowman's capsule

Correct answer: Distal tubule, collecting duct

ARPKD cysts primarily arise in distal tubules and collecting ducts.

Question 11 of 20 Medium

Which clinical sign in ADPKD signals a complication requiring urgent evaluation?

  1. A Mild flank discomfort after exercise
  2. B Sudden severe headache and vomiting
  3. C Occasional nocturia without other symptoms
  4. D Mild haematuria after a strenuous workout

Correct answer: Sudden severe headache and vomiting

In ADPKD, intracranial berry aneurysm rupture can cause sudden severe headache and vomiting.

Question 12 of 20 Medium

A child with ARPKD develops portal hypertension and splenomegaly. This is most likely due to:

  1. A Compression of the portal vein by renal cysts
  2. B Congenital hepatic fibrosis linked to the disease
  3. C Recurrent bacterial infections of the liver
  4. D Fatty liver unrelated to the kidney disease

Correct answer: Congenital hepatic fibrosis linked to the disease

ARPKD is associated with congenital hepatic fibrosis, leading to portal hypertension, splenomegaly, and liver complications.

Question 13 of 20 Medium

Which feature is more characteristic of ADPKD rather than ARPKD?

  1. A Presentation in infancy with renal failure
  2. B Liver cysts in adulthood
  3. C Autosomal recessive inheritance
  4. D Microcystic kidneys detected in utero

Correct answer: Liver cysts in adulthood

ADPKD may present in adulthood and often involves extra-renal cysts such as in the liver; ARPKD presents early and is autosomal recessive.

Question 14 of 20 Medium

In a patient with a congenital duplex collecting system, the major risk the nurse should monitor for is:

  1. A Rapid decline into end-stage renal disease by infancy
  2. B Recurrent urinary tract infections and reflux
  3. C Massive proteinuria progressing to nephrotic syndrome
  4. D Development of intracranial berry aneurysms

Correct answer: Recurrent urinary tract infections and reflux

Duplex collecting systems increase risk of urinary stasis, reflux, and UTIs rather than immediate renal failure or vascular aneurysms.

Question 15 of 20 Medium

What finding on ultrasound is most consistent with early ADPKD in an adult with a positive family history?

  1. A Unilateral small kidney with cortical scarring and atrophy
  2. B Bilateral numerous cysts with increased kidney volume
  3. C Solitary simple cyst in each kidney, parenchyma normal
  4. D Horseshoe configuration with fused lower renal poles

Correct answer: Bilateral numerous cysts with increased kidney volume

Early ADPKD shows bilateral cysts and increased kidney size (total kidney volume) even before function declines.

Question 16 of 20 Medium

In an infant with suspected ARPKD, which antenatal ultrasound finding would raise concern?

  1. A Normal amniotic fluid volume and small kidneys
  2. B Oligohydramnios and enlarged echogenic kidneys
  3. C Polyhydramnios with normal kidneys
  4. D Bilateral renal agenesis on ultrasound

Correct answer: Oligohydramnios and enlarged echogenic kidneys

Oligohydramnios and enlarged echogenic kidneys are classic prenatal clues for ARPKD.

Question 17 of 20 Medium

Which statement about the genetic transmission of ADPKD is correct?

  1. A Both parents must carry a mutation for children to be affected
  2. B If one parent has the mutation, each child has a 50% chance
  3. C It is only passed to male children
  4. D It is an X-linked condition

Correct answer: If one parent has the mutation, each child has a 50% chance

ADPKD is autosomal dominant; one mutated allele from one parent gives a 50% chance of passing to each child.

Question 18 of 20 Medium

Which of these is not typically a complication of PKD?

  1. A Systemic arterial hypertension
  2. B Hepatic (liver) cysts
  3. C Intracranial berry aneurysm
  4. D Massive proteinuria with nephrotic syndrome

Correct answer: Massive proteinuria with nephrotic syndrome

Massive proteinuria/nephrotic syndrome is not a typical early complication of PKD; PKD more commonly causes hypertension and cystic involvement in liver and brain.

Question 19 of 20 Medium

A child with a congenital kidney disorder has salt-wasting (polyuria, polydipsia) rather than early hypertension. Which diagnosis is more likely?

  1. A ADPKD (adult polycystic disease)
  2. B Nephronophthisis
  3. C ARPKD (recessive polycystic disease)
  4. D Horseshoe kidney

Correct answer: Nephronophthisis

Nephronophthisis often presents with a concentrating defect (polyuria/polydipsia) rather than hypertension; PKD typically leads to hypertension.

Question 20 of 20 Medium

Which management strategy is most important in early-stage ADPKD before significant renal failure develops?

  1. A Immediate kidney transplant regardless of current function
  2. B Aggressive blood pressure control with lifestyle changes
  3. C High-protein diet to maintain total kidney volume
  4. D Prophylactic surgical removal of all hepatic cysts

Correct answer: Aggressive blood pressure control with lifestyle changes

Early ADPKD management focuses on controlling hypertension and modifying lifestyle to slow cyst growth and preserve renal function.

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