Waldmann disease: Difference between revisions
→Management: updated treatment section to include medications |
Added Citation, Corrected Citation |
||
| Line 34: | Line 34: | ||
==Management== |
==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.<ref name="merck"/> This diet, however, is not a cure. If the diet is stopped, the symptoms will eventually reappear.<ref name="pubmed"/> Medication is also used to treat this disease, including Octreotide, Sirolimus, Anti-plasmin and, at least in one case, Trametinib.<ref>{{Cite web |last= |
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"/> Medication is also used to treat this disease, including Octreotide, Sirolimus, Anti-plasmin and, at least in one case, Trametinib.<ref>{{Cite web |last=Primary Intestinal Lymphangiectasia |first=Unraveling Adult-Onset |title=Unraveling Adult-Onset PIL |url=http://lymphangiectasia.com/ |access-date=2023-11-26 |website=lymphangiectasia.com |language=en}}</ref><ref>{{Cite journal |last=Kwon |first=Yiyoung |last2=Kim |first2=Mi Jin |date=2021-09-01 |title=The Update of Treatment for Primary Intestinal Lymphangiectasia |url=https://doi.org/10.5223/pghn.2021.24.5.413 |journal=Pediatric Gastroenterology, Hepatology & Nutrition |language=en |volume=24 |issue=5 |pages=413–422 |doi=10.5223/pghn.2021.24.5.413 |issn=2234-8646 |pmc=PMC8443852 |pmid=34557394}}</ref> |
||
Revision as of 00:52, 27 November 2023
| Waldmann disease | |
|---|---|
| Other names | Waldmann's disease, Primary intestinal lymphangiectasia |
Waldmann disease 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.[citation needed]
Diagnosis
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] Medication is also used to treat this disease, including Octreotide, Sirolimus, Anti-plasmin and, at least in one case, Trametinib.[5][6]
History
The disease was first reported in 1961 by T.A. Waldmann. He described 18 cases of patients having a low level 131I-albumin.[7] [8] Biopsies of the small intestine were examined under the microscope and found various levels of dilatation of the lymph vessels.[9]
References
- ^ a b "Waldmann disease". Archived from the original on 2009-05-13. 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)". Orphanet Journal of Rare Diseases (Free full text). 3: 5. doi:10.1186/1750-1172-3-5. PMC 2288596. PMID 18294365.
{{cite journal}}: CS1 maint: unflagged free DOI (link) - ^ a b c Ruiz, Atenodoro R. "Intestinal Lymphangiectasia". Merck. Retrieved 2009-06-11.
- ^ Primary Intestinal Lymphangiectasia, Unraveling Adult-Onset. "Unraveling Adult-Onset PIL". lymphangiectasia.com. Retrieved 2023-11-26.
- ^ Kwon, Yiyoung; Kim, Mi Jin (2021-09-01). "The Update of Treatment for Primary Intestinal Lymphangiectasia". Pediatric Gastroenterology, Hepatology & Nutrition. 24 (5): 413–422. doi:10.5223/pghn.2021.24.5.413. ISSN 2234-8646. PMC 8443852. PMID 34557394.
{{cite journal}}: CS1 maint: PMC format (link) - ^ Waldmann, T. (1961). "Gastrointestinal protein loss demonstrated by 51Cr-labelled albumin". Lancet. 2 (7194): 121–3. doi:10.1016/s0140-6736(61)92646-0. PMID 13782655.
- ^ Waldmann, T.; Steinfeld, J.; Dutcher, T; Levin, E; Berlin, N. (1961). "The role of the gastrointestinal system in "idiopathic hypoproteinemia"". Gastroenterology. 41 (3): 197–2–7. doi:10.1016/S0016-5085(19)35130-3. PMID 13782654.
- ^ 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 Médecine Interne (in French). 28 (3): 173–175. doi:10.1016/j.revmed.2006.11.019. ISSN 0248-8663. PMID 17229491.