Waldmann disease
| Waldmann disease | |
|---|---|
| Classification and external resources | |
| ICD-10 | I89.0 |
| ICD-9 | 457.1 |
| DiseasesDB | 12214 |
Waldmann disease, also known as Waldmann's disease and Primary intestinal lymphangiectasia, 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]
History
The disease was first reported in 1961 by T.A. Waldmann. He described 18 cases of patients having a low level 131I-albumin. Biopsies of the small intestine were examined under the microscope and found various levels of dilatation of the lymph vessels.[4]
Pathophysiology
The illness is usually caused by lymphatic vessels that were misshaped at birth, causing them to get obstructed and later on enlarged. The condition can also be a result of other illnesses such as constrictive pericarditis and pancreatitis. 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.[5]
Symptoms
Symptoms of the disease include diarrhea, nausea, swelling of the legs, protein-losing enteropathy, immunodeficiency and loss of lymphatic fluid into the intestines.[2][5] It is usually diagnosed before the patient is 3 years old, but it is sometimes diagnosed in adults.[3]
Management
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.[5] This diet, however, is not a cure. If the diet is stopped, the symptoms will eventually reappear.[3]
References
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- ↑ 5.0 5.1 5.2 Ruiz, Atenodoro R. "Intestinal Lymphangiectasia". Merck. Retrieved 2009-06-11.
