Infantile polycystic kidney disease is otherwise called:

Appeared in: PGICH Noida NO-2025

Explanation

Infantile polycystic kidney disease is the former name for Autosomal Recessive Polycystic Kidney Disease (ARPKD).
It is a genetic disorder inherited in an autosomal recessive pattern, meaning a child must inherit a non-working gene from both parents.
This condition is rare and typically presents at birth (perinatal) or in early infancy with severe renal dysfunction.
The genetic defect is linked to the PKHD1 gene on chromosome 6.

Option B: This is the complete failure of one or both kidneys to develop. It is an absence of kidney tissue, not a cystic transformation of existing tissue.
Option C: This refers to the underdevelopment of the kidneys, resulting in smaller organs with fewer nephrons. The kidney structure is otherwise relatively normal, unlike the cyst-filled kidneys in PKD.
Option D: This is the adult form of PKD (ADPKD). It is much more common, has an autosomal dominant inheritance pattern, and symptoms typically appear between the ages of 30 and 50.

Clinical Relevance

Nursing practice connection: Knowing Classification and genetics of polycystic kidney disease helps nurses interpret findings accurately and avoid errors in routine assessment, medication administration, and patient teaching.
Nurses caring for newborns with ARPKD must perform meticulous monitoring of renal function (urine output, BUN, creatinine) and respiratory status, as the massively enlarged kidneys can impair lung development (pulmonary hypoplasia) and function.
Genetic counseling is crucial for families with an affected child. Since it is an autosomal recessive condition, parents are carriers and have a 25% chance of having another affected child with each subsequent pregnancy.
What if? If the question described a 40-year-old man with a family history of kidney cysts presenting with hypertension and flank pain, the most likely diagnosis would then be Autosomal Dominant Polycystic Disease (ADPKD), not the infantile form.

How to Approach the Question

This is a factual recall question that tests your knowledge of medical terminology and disease classification.
First, identify the key term in the question stem: 'Infantile polycystic kidney disease'.
Recall the two main types of polycystic kidney disease and their key associations: 'infantile' is linked to 'autosomal recessive', and 'adult' is linked to 'autosomal dominant'.
Scan the options to find the one that matches the 'infantile' form.
Select 'Autosomal recessive polycystic disease' as the correct synonym.
Eliminate the other options by recalling their definitions: renal agenesis (absent kidney), renal hypoplasia (underdeveloped kidney), and autosomal dominant PKD (adult form).

Concept Tested & Keywords

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E6 PATHOLOGY QUICK REVIEWBased on Harsh Mohan Textbook of PATHOLOGY p. 217-219

Practise the full PGICH Noida NO-2025

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