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Paediatric

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Gastro Intestinal System

Question
38 out of 56
 

The histological features of coeliac disease include all of the following Except - (AIIMS May 2013)

A. Crypt hyperplasra

B. Increase in thickness of the mucosa

C. Increase in intraepithelial lymphocytes

D. Increase in inflammatory cells in lamina propyria

38a. Which of the following shows the most specific finding in pediatric age with nutritional/ malabsorption state: (LQ)



A Malnutrition
B Giardiasis

C Abetalipoproteinemia
D Tropical sprue

Ans. B

Increase in thickness of mucosa

a. In celiac disease proximal intestine is commonly involved while in tropical sprue whole of the intestine is involved-

b. Biopsy of intestine in celiac sprue demonstrates.

i. Villous atrophy, loss of microvilli brush border

ii. The crypts are hyperplastic and become elongated.

iii. Presence of following cells on lamina propia --> Plasma cells, Lymphocyte4 Macrophages, Eosinophils, Mast cells.

iv. Remember that overall mucosal thickness remains same in celiac sorue

c. Celiac ds. is associated with dermatitis herpetiformis.

d. Pathological features

i. Celiac disease is caused by action of acidic peptcontaining the gliadin fraction of wheat,

ii. The basic abnormality is thought to be an increased rate of loss of epithelial cells. Crypt cells show increased activity. This causes hypertrophy of crypt. But even this increased activity, cannot keep pace with loss of cells, resulting in progressdecrease in height of villi causing villous atrophy

iii. The epithelial cells show decreased cytoplasm and mucus

The intestinal biopsy shows decresses in the villus: crypt ratio. Normally the villi are 3-4 tthe height of the crypts (ratio of 3-4: 1); in celi.ac disease, the ratio decreases progressively (partial, subtotal and total villous atrophy). Total villous atrophy is seen in severe celiac ds and is quite characteristic of celiac ds (partial villous atrophy may be seen in mainly other conditions).

38a. Ans. C.

Abetalipoproteinemia

1. Abetalipoproteinemia Acanthocytosis is a rare, autosomal recessive disorder in which the microsomal triglyceride transfer protein is absent because of mutations in the MTP gene.

2. Patients have total cholesterol levels <50 mg/dL and virtually no VLDL, IDL, LDL, or chylomicrons. Because dietary fat as well as vitamins A and E are transported from the intestine in chylomicrons, these patients have malabsorption of fat and fat soluble vitamins.

3. Vitamin E deficiency results in neurologic problems.

4. If vitamin replacement is adequate patients can lead normal life.

5. Lipid droplets can be seen in intestinal mucosal cells obtaby peroral biopsy which is pathognomic for this disease.

Gastro Intestinal System Flashcard List

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