Congenital Kidney Conditions Overview

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Questions and Answers

What is the primary characteristic of horseshoe kidney?

  • Kidney is caught on the inferior mesenteric artery during ascension. (correct)
  • Kidney connection occurs at the upper pole.
  • Kidneys are located in the upper abdomen.
  • Kidneys are always absent on one side.

Which condition is incompatible with life due to its associated features?

  • Unilateral renal agenesis.
  • Bilateral renal agenesis. (correct)
  • Polycystic kidney disease (autosomal dominant form).
  • Dysplastic kidney.

Which factor is characteristic of autosomal recessive polycystic kidney disease?

  • It often presents with Potter sequence in newborns. (correct)
  • It is commonly associated with medullary collecting duct cysts.
  • It presents in adults with hematuria.
  • It leads to hypertrophy of the existing kidney.

What is the primary cause of hypertension in patients with autosomal dominant polycystic kidney disease?

<p>Increased renin production. (B)</p> Signup and view all the answers

Which feature distinguishes medullary cystic kidney disease from other renal conditions?

<p>Presence of cysts in the medullary collecting ducts. (C)</p> Signup and view all the answers

Flashcards

Horseshoe Kidney

Two kidneys fused together at the lower end, typically located low in the abdomen. It's the most common kidney malformation. The kidney can get stuck on the inferior mesenteric artery as it ascends, preventing it from reaching its normal position.

Renal Agenesis

Complete absence of one or both kidneys during fetal development. If only one kidney is absent, the remaining kidney usually grows larger.

Dysplastic Kidney

A kidney with many cysts (fluid-filled sacs) and abnormal tissue, like cartilage. It usually affects only one kidney.

Polycystic Kidney Disease (PKD)

A genetic disease where cysts form in the kidneys, leading to enlarged organs and eventual failure. The most common type is autosomal dominant, affecting adults.

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Medullary Cystic Kidney Disease

An inherited disorder where cysts develop specifically in the collecting ducts of the kidney. This leads to scar tissue and kidney shrinkage.

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Study Notes

Congenital Kidney Conditions

  • Horseshoe Kidney: Two kidneys fused together, typically at the lower pole. Common congenital anomaly. Kidney positioned lower in the abdomen.
  • Renal Agenesis: Absence of one or both kidneys. Unilateral agenesis leads to the other kidney enlarging. Bilateral absence is incompatible with life, causing developmental problems in the extremities and lungs.
  • Dysplastic Kidney: Malformed kidneys. Often unilateral (one kidney affected). Characterized by cysts and abnormal tissue. If bilateral, must be differentiated from inherited polycystic kidney disease.
  • Polycystic Kidney Disease (PKD): Inherited condition causing bilateral kidney enlargement with cysts. Autosomal recessive PKD affects infants, leading to worsening renal failure, hypertension. Also associated with congenital hepatic fibrosis and hepatic cysts. Autosomal dominant PKD affects young adults, involving hypertension, hematuria, and worsening renal failure; associated with cysts developing over time and connected to other conditions like berry aneurysms, hepatic cysts, and mitral valve prolapse.

Medullary Cystic Kidney Disease

  • Medullary Cystic Kidney Disease: Inherited condition, autosomal dominant. Cysts in the medullary collecting ducts. Kidney tissue damage (fibrosis) leads to shrunken kidneys and worse kidney function.

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