PEDBASE.org - The Pediatric Database - Detailed information of JUVENILE POLYPOSIS OF INFANCY

JUVENILE POLYPOSIS OF INFANCY

DEFINITION:

A disorder involving the colon characterized by masses of benign inflammatory polyps rarely mixed with potenitally malignant adenomatous polyps.

EPIDEMIOLOGY:

incidence: rare
age of onset:
- less than 2 years of age
risk factors:
- familial - autosomal recessive
  - chrom.#: ?
  - gene: ?

PATHOGENESIS:

1. Background

Juvenile Polyposis Syndrome is classified into three disorders each associated with inflammatory polyps:
1. Juvenile Polyposis of Infancy

2. JUVENILE POLYPOSIS OF INFANCY

3. Gastrointestinal Polyposis

CLINICAL FEATURES:

1. Gastrointestinal Manifestations

1. Painless Rectal Bleeding

+/- bloody diarrhea

2. Complications

anemia
failure to thrive
protein-loosing enteropathy
malignancy potential: very rare
intussusception
malnutrition

2. Other Manifestations

increased susceptibility to infection
associated anomalies:
- renal, gastrointestinal, and central nervous system abnormalities

INVESTIGATIONS:

1. Colonoscopy

procedure of choice

2. Imaging Studies

barium enema with air contrast (Double Contrast)

3. Biopsy

inflammatory polyps +/- adenomatous polyps
areas affected: colon, small intestine

4. Serum

microcytic hypochromic anemia
reduced serum iron and ferritin with elevated transferrin

MANAGEMENT:

1. Supportive

iron supplementation for anemia
nutritional support

2. Surgical

if significant complications

Pediatric Database - JUVENILE POLYPOSIS OF INFANCY

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