Waldmann disease | |
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Other names: Waldmann's disease, Primary intestinal lymphangiectasia | |
Waldmann disease is a rare disease[1] characterized by enlargement of the lymph vessels supplying the lamina propria of the small intestine.[2] Although its prevalence is unknown, it being classified as a "rare disease" means that less than 200,000 of the population of the United States are affected by this condition and its subtypes.[1][3]
Signs and symptoms of the disease include diarrhea, nausea, swelling of the legs, protein-losing enteropathy, immunodeficiency and loss of lymphatic fluid into the intestines.[2][4] It is usually diagnosed before the patient is 3 years old, but it is sometimes diagnosed in adults.[3]
The illness is usually caused by lymphatic vessels that were misshaped at birth, causing obstruction and subsequent enlargement. The condition can also be a result of other illnesses such as constrictive pericarditis and pancreatitis.[citation needed]
The disease is diagnosed by doing a biopsy of the affected area. Severity of the disease is then determined by measuring alpha1-antitrypsin proteins in a stool sample.[4]
Once the main cause of the disease is treated, a diet of low-fat and high-protein aliments, supplemental calcium and certain vitamins has been shown to reduce symptom effects.[4] This diet, however, is not a cure. If the diet is stopped, the symptoms will eventually reappear.[3]
The disease was first reported in 1961 by T.A. Waldmann. He described 18 cases of patients having a low level 131I-albumin.[5] [6] Biopsies of the small intestine were examined under the microscope and found various levels of dilatation of the lymph vessels.[7]
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