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]
Contents |
[edit] 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]
[edit] 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.[5]
[edit] 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]
[edit] 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]
[edit] References
- ^ a b "Waldmann disease". http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&DiseaseID=7873&expand=SupportGroups. Retrieved 2009-06-11.
- ^ a b 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.
- ^ a b c Vignes, S.; Bellanger, J. (Feb 2008). "Primary intestinal lymphangiectasia (Waldmann's disease)" (Free full text). Orphanet Journal of Rare Diseases 3: 5. doi:10.1186/1750-1172-3-5. PMC 2288596. PMID 18294365. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2288596.
- ^ Vignes S, B. J.; Bellanger, J. (Mar 2007). "Videocapsule endoscopy as a useful tool to diagnose primary intestinal lymphangiectasia [Videocapsule endoscopy as a useful tool to diagnose primary intestinal lymphangiectasia]" (in French). La Revue de medecine interne / fondee ... par la Societe nationale francaise de medecine interne 28 (3): 173–175. doi:10.1016/j.revmed.2006.11.019. ISSN 0248-8663. PMID 17229491.
- ^ a b c Ruiz, Atenodoro R.. "Intestinal Lymphangiectasia". Merck. http://www.merckmanuals.com/home/digestive_disorders/malabsorption/intestinal_lymphangiectasia.html. Retrieved 2009-06-11.
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