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]

## Contents

* 1 Signs and symptoms
* 2 Pathophysiology
* 3 Diagnosis
* 4 Management
* 5 History
* 6 References
* 7 External links

## Signs and symptoms[edit | edit source]

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[edit | edit source]

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[edit | edit source]

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[edit | edit source]

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[edit | edit source]

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[edit | edit source]

1. ↑ 1.0 1.1 "Waldmann disease". Archived from the original on 2009-05-13. Retrieved 2009-06-11.
2. ↑ 2.0 2.1 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. ↑ 3.0 3.1 3.2 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. ↑ 4.0 4.1 4.2 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.

## External links[edit | edit source]

Classification|

* ICD-10: I89.0
* ICD-9-CM: 457.1
* DiseasesDB: 12214

|

* v
* t
* e

Lymphatic disease: organ and vessel diseases

Thymus|

* Abscess
* Hyperplasia
* Hypoplasia
* DiGeorge syndrome
* Ectopic thymus
* Thymoma
* Thymic carcinoma

Spleen|

* Asplenia
* Asplenia with cardiovascular anomalies
* Accessory spleen
* Polysplenia
* Wandering spleen
* Splenomegaly
* Banti's syndrome
* Splenic infarction
* Splenic tumor

Lymph node|

* Lymphadenopathy
* Generalized lymphadenopathy
* Castleman's disease
* Intranodal palisaded myofibroblastoma
* Kikuchi disease
* Tonsils
* see Template:Respiratory pathology

Lymphatic vessels|

* Lymphangitis
* Lymphangiectasia
* Lymphedema
* Primary lymphedema
* Congenital lymphedema
* Lymphedema praecox
* Lymphedema tarda
* Lymphedema–distichiasis syndrome
* Milroy's disease
* Secondary lymphedema
* Bullous lymphedema
* Factitial lymphedema
* Postinflammatory lymphedema
* Postmastectomy lymphangiosarcoma
* Waldmann disease

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*[t]: Discuss this template
*[e]: Edit this template