Intestinal Lymphangiectasia (Idiopathic Hypoproteinemia)

Intestinal lymphangiectasia is a malabsorptive disorder caused by abnormalities such as enlargement and obstruction of the lymph vessels which line the wall of the small intestine. It is also referred to as idiopathic hypoproteinemia.

  • Abnormalities in the lymphatic vessels cause this condition.
  • The most common symptom is chronic diarrhea.
  • Biopsy of a part of the intestine showing enlarged lymphatic vessels helps in diagnosing the condition.
  • Treatment involves resolving the cause of the condition, as well as managing the symptoms by following a low-fat diet high in proteins, in addition to taking vitamin and mineral supplements

 After the food is digested, the small intestine absorbs the nutrients. Some of the nutrients are assimilated directly into the blood. The function of the lymph vessels in the lining of the small intestine is to absorb the fats and carry them to the blood vessels. But when there are abnormalities in the lymph vessels, it affects the assimilation of proteins and fats into the blood. Congenital abnormalities in the lymph vessels are the most common cause which results in their enlargement. Pancreatitis, in which pancreas is inflamed, and constrictive pericarditis, in which pericardium enclosing the heart becomes stiff, are two other conditions resulting in the enlargement of lymph vessels. The enlargement of vessels results in leakage of the lymphatic fluid back into the small intestine inhibiting the absorption of proteins and fats into the blood.

Symptoms and Diagnosis

Nausea and vomiting, pain in the abdomen, and chronic diarrhea with fatty stools are the most common symptoms of intestinal lymphangiectasia. Blockage of lymph vessels in other parts of the body may cause edema. Tissue swelling may occur due to the lower than normal protein levels in the blood caused by this condition. Lower levels of cholesterol and reduction in the lymphocyte count are other symptoms resulting from intestinal lymphangiectasia.

Intestinal lymphangiectasia is diagnosed if the biopsy of a portion of the small intestine shows enlarged lymphatic vessels. The quantity of a protein called alpha1-antitrypsin in the stool is measured to assess how much protein is lost back into the intestines.

Treatment

When the cause of the intestinal lymphangiectasia is determined, the cause is treated appropriately. A low-fat diet rich in proteins is followed to get relief from symptoms. There are some fats called medium-chain triglycerides which can be absorbed by the blood directly without the aid of the lymphatic system. The diet can be supplemented by medium chain triglycerides, and also other minerals and vitamins to prevent deficiency diseases.

Intestinal Lymphangiectasia (Idiopathic Hypoproteinemia) Video Summary

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Yasser Elnahas

MD, PHD, Professor Of CardioVascular Surgery
Dr. Yasser Elnahas, Is an associate Professor of Cardiovascular Surgery. Dr. Elnahas was trained as a fellow At Texas Heart Institute And Mayo Clinic Foundation.Dr. Elnahas is dedicated to educating the general public about different disease conditions and simplifying the medical knowledge in an easy to understand terminology.

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