The disease congenital aganglionic megacolon is commonly known as:

Appeared in: DSSSB 13 August 2024

Explanation

Hirschsprung's disease is the correct medical term for congenital aganglionic megacolon.
It is a birth defect where nerve cells (ganglion cells) are missing from the muscles of the baby's colon, most commonly the rectum and sigmoid colon.
This absence of nerve cells prevents the colon from relaxing and causes a functional blockage.
As a result, stool cannot pass through the affected segment, causing the part of the colon before the blockage to swell, a condition known as megacolon.

Option A: Cardiochalasia, also known as achalasia, involves the esophagus and the lower esophageal sphincter, not the colon. It is characterized by impaired relaxation of the sphincter, leading to difficulty swallowing.
Option B: Celiac disease is an autoimmune disorder affecting the small intestine, triggered by gluten. It is not a congenital structural defect and does not involve aganglionosis of the colon.
Option C: Pyloric stenosis is a condition where the muscle of the pylorus (the outlet of the stomach) thickens, causing a blockage that prevents food from entering the small intestine. It presents with projectile vomiting and is unrelated to the colon.

Clinical Relevance

Nursing practice connection: Knowing Identification of the common name for congenital aganglionic megacolon helps nurses interpret findings accurately and avoid errors in routine assessment, medication administration, and patient teaching.
A key nursing assessment for newborns is monitoring for the passage of the first stool (meconium). Failure to pass meconium within 24-48 hours is a classic sign of Hirschsprung's disease.
Diagnosis is confirmed by a rectal biopsy, which shows the absence of ganglion cells. Nursing care involves preparing the infant and family for this procedure and providing post-procedure care.
Treatment is surgical removal of the aganglionic bowel segment. Post-operative nursing care is crucial and includes ostomy care (if applicable), monitoring for complications like enterocolitis, and parent education.

How to Approach the Question

This is a factual recall question that tests your knowledge of medical terminology and synonyms for diseases.
First, break down the term 'congenital aganglionic megacolon'.
'Congenital' means present at birth.
'Aganglionic' means the absence of ganglion (nerve) cells.
'Megacolon' means an enlarged colon.
Review the options to identify the disease that matches this description: a birth defect involving missing nerve cells that causes an enlarged colon.

Concept Tested & Keywords

Concept Tested: Identification of the common name for congenital aganglionic megacolon.
Stem keywords: congenital aganglionic megacolon
Lead-in keywords: commonly known as

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E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed p. 757-759

E6 Nelson Textbook of Pediatrics(2024) — Volume 2 p. 76-78

Practise the full DSSSB 13 August 2024

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