Waldmann disease

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.

Diagnosis

Left Endoscopy shows D1 covered with white smear Right D2 appears with edematous intestinal mucosa

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.[5] [6] Biopsies of the small intestine were examined under the microscope and found various levels of dilatation of the lymph vessels.[7]

References

  1. 1 2 "Waldmann disease". Archived from the original on 2009-05-13. Retrieved 2009-06-11.
  2. 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.
  3. 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.
  4. 1 2 3 Ruiz, Atenodoro R. "Intestinal Lymphangiectasia". Merck. Archived from the original on 2011-11-10. Retrieved 2009-06-11.
  5. ↑ Waldmann, T. (1961). "Gastrointestinal protein loss demonstrated by 51Cr-labelled albumin". Lancet. 2 (7194): 121–3. PMID 13782655.
  6. ↑ Waldmann, T.; Steinfeld, J.; Dutcher, T; Levin, E; Berlin, N. (1961). "The role of the gastrointestinal system in "idiopathic hypoproteinemia"". Gastroenterology. 41: 197-2-7. PMID 13782654.
  7. ↑ 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 français). 28 (3): 173–175. doi:10.1016/j.revmed.2006.11.019. ISSN 0248-8663. PMID 17229491.
Classification
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