Intestinal Lymphangiectasia
Intestinal lymphangiectasia (idiopathic hypoproteinemia) is a disorder in which the lymph vessels supplying the lining of the small intestine become enlarged and obstructed.
The lymph vessels from the digestive tract, which carry white blood cells called lymphocytes, may be enlarged at birth. Less commonly, these lymph vessels may enlarge later in life as a result of such conditions as inflammation of the pancreas (pancreatitis) or stiffening of the sac that envelops the heart (constrictive pericarditis). Lymphatic fluid leaks from the swollen lymph vessels in the wall of the intestine, preventing fat and proteins from being absorbed into the bloodstream.
Symptoms and Diagnosis
A person with intestinal lymphangiectasia has diarrhea. Nausea, vomiting, fatty stools, and abdominal pain may also develop. The person may also have swelling (edema) if lymph vessels elsewhere in the body are blocked.
Levels of protein in the blood are low. The low protein levels result in tissue swelling. The number of lymphocytes in the blood is decreased, and cholesterol levels in the blood may be normal or low.
The diagnosis is established by a biopsy of the small intestine showing enlargement of the lymph vessels. Measurement of a certain protein, called alpha1-antitrypsin, in the stool can indicate the severity of protein loss into the intestines.
Treatment
When intestinal lymphangiectasia is caused by a specific condition, the underlying condition is treated. Symptoms can be relieved by eating a low-fat diet and taking supplements of certain triglycerides, which are absorbed directly into the blood and not through the lymph vessels.
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