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| __NOTOC__
| | #REDIRECT [[Primary Intestinal Lymphangiectasia]] |
| {{SI}}
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| {{CMG}}
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| ==Overview==
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| '''Waldmann disease''', also known as '''Waldmann's disease''' and '''Primary intestinal lymphangiectasia''', is a rare [[disease]]<ref name='nih'>{{cite web|url=http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&DiseaseID=7873&expand=SupportGroups |title=Waldmann disease |accessdate=2009-06-11 }}</ref> characterized by enlargement of the [[lymph vessels]] supplying the [[lamina propria]] of the [[small intestine]].<ref name='wdo'>{{Cite journal | pmid = 15229406 | last1 = Boursier | date=May 2004 | first1 = V. | last2 = Vignes | first2 = S. | title = Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease) | volume = 29 | issue = 2 | pages = 103–106 | issn = 0398-0499 | journal = Journal des maladies vasculaires | doi = 10.1016/S0398-0499(04)96722-4}}</ref>
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| 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.<ref name='nih'/><ref name='pubmed'>{{Cite journal| doi = 10.1186/1750-1172-3-5| last1 = Vignes| date = Feb 2008 | first1 = S. | last2 = Bellanger | first2 = J. | title = Primary intestinal lymphangiectasia (Waldmann's disease) | volume = 3| pages = 5| pmid = 18294365| pmc = 2288596| journal = Orphanet Journal of Rare Diseases| type = Free full text}}</ref>
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| ==Signs and symptoms==
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| Signs and symptoms of the disease include [[diarrhea]], [[nausea]], [[Swelling (medical)|swelling]] of the legs, [[protein-losing enteropathy]], [[immunodeficiency]] and loss of lymphatic fluid into the intestines.<ref name='wdo'/><ref name='merck'>{{cite web|url=http://www.merckmanuals.com/home/digestive_disorders/malabsorption/intestinal_lymphangiectasia.html |title= Intestinal Lymphangiectasia |accessdate=2009-06-11 |last=Ruiz |first=Atenodoro R. |publisher=[[Merck & Co.|Merck]] }}</ref> It is usually diagnosed before the patient is 3 years old, but it is sometimes diagnosed in adults.<ref name='pubmed'/>
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| == Pathophysiology ==
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| 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 alpha<sub>1</sub>-antitrypsin proteins in a stool sample.<ref name='merck'/>
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| ==Management==
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| 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.<ref name='merck'/> This diet, however, is not a cure. If the diet is stopped, the symptoms will eventually reappear.<ref name='pubmed'/>
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| ==History==
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| The disease was first reported in 1961 by T.A. Waldmann. He described 18 cases of patients having a low level <sup>131</sup>I-albumin. Biopsies of the small intestine were examined under the microscope and found various levels of dilatation of the lymph vessels.<ref name='biop'>{{Cite journal | first2 = J.| last2 = Bellanger| title = Intérêt de l'entéroscopie par vidéocapsule dans le diagnostic des lymphangiectasies intestinales primitives| trans_title = Videocapsule endoscopy as a useful tool to diagnose primary intestinal lymphangiectasia| language = French| last1 = Vignes|first1=S.| journal = La Revue de medecine interne / fondee ... par la Societe nationale francaise de medecine interne | volume = 28 | issue = 3 | pages = 173–175 | date=Mar 2007 | issn = 0248-8663 | pmid = 17229491 | doi = 10.1016/j.revmed.2006.11.019}}</ref>
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| ==References==
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| {{reflist|2}}
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| {{Lymphatic disease}}
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| [[Category:Rare diseases]]
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