Lymphangiectasia
Sign in to saveAlso known as lymphangiectasis, Lymphangiectases (disorder), Lymphangiectasis (morphologic abnormality), obsolete lymphangiectasis
Lymphangiectasia, also known as "lymphangiectasis", is a pathologic dilation of lymph vessels. When it occurs in the intestines it is known as intestinal lymphangiectasia, colloquially recognized as Waldmann's disease in cases where there is no secondary cause. The primary defect lies in the inability of the lymphatic system to adequately drain lymph, resulting in its subsequent accumulation and leakage into the intestinal lumen. This condition, first described by Waldmann in 1961, is typically diagnosed in infancy or early childhood. However, it can also manifest in adults, exhibiting a broad
Research
2,310 papers- Congenital pulmonary lymphangiectasia.Journal of perinatal medicine · 2017
- Primary intestinal lymphangiectasia in children: A review.Journal of paediatrics and child health · 2020
- Vulval lymphangiectasia.Genitourinary medicine · 1989
- Renal lymphangiectasia in pediatric population: case series and review of literature.Annals of Saudi medicine · 2022
- [Primary intestinal lymphangiectasia (Waldmann's disease)].La Revue de medecine interne · 2018
via PubMed
Clinical Trials
5 registered- RECRUITINGA Prospective Natural History Study of Lymphatic AnomaliesEunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) · NCT05731141
- TERMINATEDCharacterization of the Pathogenesis of Primary and Secondary Lymphatic DisordersNational Heart, Lung, and Blood Institute (NHLBI) · NCT02156115
- PHASE2COMPLETEDSafety and Efficacy Study of Sirolimus in Complicated Vascular AnomaliesDenise Martin Adams · NCT00975819
- UNKNOWNInternational Lymphatic Disease and Lymphedema RegistryStanford University · NCT01336790
- UNKNOWNTranscriptome and Metabolic Analyses of CHAPLE DiseaseMarmara University · NCT03950804
~4 min read
Encyclopedic overview
7 sectionsContents
- Signs and symptoms
- Cause
- Diagnosis
- Treatment
- In animals
- References
- External links
Lymphangiectasia, also known as "lymphangiectasis", is a pathologic dilation of lymph vessels. When it occurs in the intestines it is known as intestinal lymphangiectasia, colloquially recognized as Waldmann's disease in cases where there is no secondary cause. The primary defect lies in the inability of the lymphatic system to adequately drain lymph, resulting in its subsequent accumulation and leakage into the intestinal lumen. This condition, first described by Waldmann in 1961, is typically diagnosed in infancy or early childhood. However, it can also manifest in adults, exhibiting a broad spectrum of clinical symptoms.
==Signs and symptoms== The pathophysiology of intestinal lymphangiectasia is centered around the dilation of the lymphatic vessels in the intestinal mucosa, submucosa, and sometimes the mesentery. This dilation impedes the normal flow of lymph from the intestines back to the circulatory system. The overflow of lymphatic fluid into the intestines leads to the loss of lymphocytes, immunoglobulins, and proteins, causing lymphopenia, hypogammaglobulinemia, and hypoalbuminemia, respectively. The loss of proteins contributes to the development of protein-losing enteropathy, a major clinical manifestation of this disease.
Excerpted from Wikipedia’s “Lymphangiectasia” article, available under the CC BY-SA 4.0 licence.