Waldmann disease

From Wikipedia, the free encyclopedia
Jump to: navigation, search
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[edit]

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[edit]

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]

Symptoms[edit]

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[edit]

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[edit]

  1. ^ a b "Waldmann disease". Retrieved 2009-06-11. 
  2. ^ 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.  edit
  3. ^ a b c 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.  edit
  4. ^ 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.  edit
  5. ^ a b c Ruiz, Atenodoro R. "Intestinal Lymphangiectasia". Merck. Retrieved 2009-06-11.