Detailed Information on Microscopic Polyangiitis

Microvillous inclusion disease is a rare inherited disorder of the small intestine that is inherited in an autosomal recessive pattern. It is caused by a congenital lack of apical microvilli in the epithelial cells of the small intestine. Microscopic polyangiitis results in metabolic acidosis and severe dehydration. Pregnancy and birth are generally normal. Signs of microvillus inclusion disease usually show up within hours or days after birth; however, sometimes the symptoms show up later (around two months after birth) and are less severe. A baby might have severe, watery diarrhea that doesn't go away, and not be able to absorb nutrients.

Microscopic polyangiitis is affected both boys and girls. It does seem to appear in girls more often. Because this particular gene is recessive, both parents must carry it in order to pass the disease on to their child. In some families, more than one child is affected. People who have a family history of microvillus inclusion disease can benefit from genetic counseling when they begin to plan for children, but there is currently no way to predict or prevent microvillus inclusion disease. Microvillus inclusion bodies in Microvillus inclusion disease originate from autophagocytosis of the apical membrane of enterocytes with engulfing of microvilli.

Microvillous inclusion disease is thought to be extremely rare. Dissimilar medicines have been tried to prevent or counteract the severe diarrhea, but none of them has proven effective. Babies with microvillus inclusion disease rely on nutritionally balanced formulas given directly into the veins (intravenously) known as total parenteral nutrition (TPN). TPN can help stabilize a baby's health, but often it is not a good long-term solution. Over time, TPN may cause liver damage and increase the risk of infections. When TPN produces these kinds of complications, intestinal transplantation is often the best option.

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