Waldmann disease
| Waldmann disease | |
|---|---|
| Classification and external resources | |
| ICD-10 | I89.0 |
| ICD-9-CM | 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]
Signs and symptoms
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]
Pathophysiology
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. 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]
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.[4] This diet, however, is not a cure. If the diet is stopped, the symptoms will eventually reappear.[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.[5]
References
- 1 2 "Waldmann disease". Retrieved 2009-06-11.
- 1 2 Boursier, V.; Vignes, S. (May 2004). "Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)". Journal des maladies vasculaires. 29 (2): 103–106. doi:10.1016/S0398-0499(04)96722-4. ISSN 0398-0499. PMID 15229406.
- 1 2 3 Vignes, S.; Bellanger, J. (Feb 2008). "Primary intestinal lymphangiectasia (Waldmann's disease)". Orphanet Journal of Rare Diseases (Free full text). 3: 5. doi:10.1186/1750-1172-3-5. PMC 2288596
. PMID 18294365. - 1 2 3 Ruiz, Atenodoro R. "Intestinal Lymphangiectasia". Merck. Retrieved 2009-06-11.
- ↑ Vignes, S.; Bellanger, J. (Mar 2007). "Intérêt de l'entéroscopie par vidéocapsule dans le diagnostic des lymphangiectasies intestinales primitives" [Videocapsule endoscopy as a useful tool to diagnose primary intestinal lymphangiectasia]. La Revue de medecine interne / fondee ... par la Societe nationale francaise de medecine interne (in French). 28 (3): 173–175. doi:10.1016/j.revmed.2006.11.019. ISSN 0248-8663. PMID 17229491.