Primary intestinal lymphangiectasia

What is the life expectancy for primary intestinal lymphangiectasia diagnosed within the first few months of life?

Generally, the earlier the onset of primary intestinal lymphangiectasia, the more severe the disease. If the disease is apparent in the newborn period or the first few months of life, symptoms may include massive edema, diarrhea, malabsorption, and infection. Still, infants with this disease can (and have) survived into adulthood. Because the severity of symptoms in infants can vary, the infants healthcare providers are best able to provide specific information regarding a child's prognosis.

Last updated on 05-01-20

How might primary intestinal lymphangiectasia be treated?

Unfortunately, there is no cure for primary intestinal lymphangiectasia (PIL). It is typically managed through dietary restrictions, including a low-fat diet and supplementation of a specific type of fat more easily absorbed by individuals with this condition (medium chain triglycerides). These restrictions usually continue throughout life. Periodic intravenous (IV) infusion of a protein called albumin may be useful in treating swelling of the lower limbs and/or buildup of excess bodily fluids. Use of certain hormones, such as octreotide, may be useful in treating individuals not responding to dietary changes. Surgery is usually not needed unless the disease is limited to one area of the intestine.

Last updated on 05-01-20

Name: Lymphatic Education and Research Network 261 Madison Avenue
New York, NY, 10016, United States
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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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