A female patient is having diarrhea and abdominal distension. Small intestinal biopsy shows villous atrophy and crypt hyperplasia. Diagnosis is -
Ans. is 'a' i.e., Celiac sprue o Villous atrophy with crypt hyperplasia is characteristic of celiac disease.Celiac disease (celiac sprue / gluten - sensitive enteropathy)o Celiac disease is a chronic disease, in which there is a characteristic mucosal lesion of the small intestine and impaired nutrient absorption, which improves on withdrawal of wheat gliadins and related grain proteins from the diet.o The fundamental disorder in celiac sprue is sensitivity to gluten which is alcohol soluble, water-insoluble protein component. It is found in wheat and closely related grains such as oat, barley, and rye.o Celiac disease presents with features of malabsorption and the characteristic is that withdrawal of gluten-containing substances from the diet i.e. (wheat, oat, barley, and rye) produces a dramatic improvement in the disease,o Almost all individuals with celiac disease share the major histocompatibility complex HLA-DQ 2 or HLA - DQ8.o Gliadin is deamidated by the enzyme transglutaminase and deamidated gliadin peptides bind to DQ2 and DQ8.o Recognition of these peptides by CD4+T cells leads to secretion of IFN-y, which damages the intestinal wall.Pathophysiologyo Celiac disease is caused by the action of acidic peptides containing the gliadin fraction of wheat,o 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 the loss of cells, resulting in a progressive decrease in height of villi causing villous atrophyo The epithelial cells show decreased cytoplasm and mucuso The intestinal biopsy shows a decrease in the villus: crypt ratio. Normally the villi are 3-4 times the height of the crypts (ratio of 3-4: 1); in celiac 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 many other conditions).
Celiac sprue
Tropical sprue
Whipples disease
Hirchsprung's disease