Intestinal Lymphangiectasia

Intestinal Lymphangiectasia

Synonyms

Dysproteinemia, Familial
Enteropathy, Hypercatabolic Protein-Losing
Familial Hypoproteinemia with Lymphangietatic Enteropathy
Hypoproteinemia, Idiopathic
Intestinal Lymphangiectasia
Intestinal Lymphangiectasis
Lymphangiectasia, Primary Intestinal
Lymphangiectasis, Primary Intestinal
Lymphangiectatic Protein-Losing Enteropathy
Lymphedema, Neonatal due to Exudative Enteropathy
Protein-Losing Enteropathy Secondary to Congestive Heart Failure

Lymphangiectasia is a condition wherein the lymphatics are dilated. The result is that lymph channels may become blocked and there is a "leakage" of fluid into the affected body area.

Signs and symptoms

Symptoms include diarrhea, nausea, vomiting, fatty stools, and abdominal pain may be present. Other indicators may include edema, low protein levels (caused from the protein loss from the condition), and low albumin levels.

Histology

Malformation or dilation of lymphatic channels resulting in lymph blockages and accumulation of fluids in the affected body areas.

Aetiology

This condition can either be primary (hereditary), primary (congenital) or can be secondary as a result of cancers, cardiac conditions, chronic inflammations, tuberculosis, scleroderma, lupus, fibrosis, endometriosis as well as other factors.

Treatment

There is no cure for lymphangiectasia. Treatment is focused on control of complications, control through dietary habits and possible drug therapy for various symptoms. In the case of secondary lymphangiectasia treatment also focuses on the underlying cause.

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Synonyms and related keywords: primary intestinal lymphangiectasia, congenital intestinal lymphangiectasia, Milroy disease, protein-losing gastroenteropathies, hypoproteinemia, lymphocytopenia, hypogammaglobulinemia, anergy, impaired allograft rejection, diarrhea, peripheral edema

Author: Raoul Joubran, MD Chief, Fellow, Department of Internal Medicine, Division of Gastroenterology/Hepatology, University of Louisville School of MedicineBackground: Traditionally, protein-losing gastroenteropathies have been classified into 3 groups (depending on the mechanism of their etiology), which include (1) those causing mucosal damage leading to increased permeability to protein (usually not involving mucosal ulcerations), (2) those with mucosal erosions and/or ulcerations, and (3) those in which protein loss is secondary to mechanical lymphatic obstruction. While a more detailed discussion on Protein-Losing Enteropathy

eMedicine

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Pathophysiology: Intestinal lymphangiectasia is a disease characterized by hypoproteinemia and lymphocytopenia, resulting from blocked intestinal lymphatics and loss of lymph fluid into the gastrointestinal (GI) tract. This leads to immunologic abnormalities, including hypogammaglobulinemia, anergy, and impaired allograft rejection. In addition to the loss of other serum components (eg, lipids), iron and certain trace metals may also be affected.

** For complete article - see link below**

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INTESTINAL LYMPHANGIECTASIA

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Hypoproteinemia in intestinal lymphangiectasia; Contribution of albumin "trapping" in the lymphedematous extremityT Herskovic, SJ Winawer, R Goldsmith, R Klein and N Zamcheck

Department of Medicine, Yale University School of Medicine, New Haven, Connecticut

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All About Lymphangiectasia Yahoo Support Group

Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.

Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com

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Primary intestinal lymphangiectasia causing severe weight loss: CT, MR and histologic findings


Ianaccone R, Pietropaolo A, Marin D, Celestre M, Pasqualini V

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Successful resection of localized intestinal lymphangiectasia post-Fontan: role of [sup.99m]technetium-dextran scintigraphy

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A Case of Protein-Losing Enteropathy Caused by Intestinal Lymphangiectasia in a Preterm Infant

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Hennekam's Syndrome

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Hennekam's Syndrome - Intesetinal Lymphangiectasia

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