Waldmann disease

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