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A case of the yellow nail syndrome associated with massive chylous ascites, pleural and pericardial effusions.

Identifieur interne : 00B887 ( Ncbi/Curation ); précédent : 00B886; suivant : 00B888

A case of the yellow nail syndrome associated with massive chylous ascites, pleural and pericardial effusions.

Auteurs : N P Malek [Allemagne] ; K. Ocran ; U J Tietge ; H. Maschek ; K F Gratz ; C. Trautwein ; S. Wagner ; M P Manns

Source :

RBID : pubmed:8956479

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English descriptors

Abstract

A 26-year-old male patient with a history of chronic peripheral lymphedema, yellowish coloured slow growing nails and pleural effusions since early childhood is described. After 23 years he developed a chylous ascites and scintigraphy with technetium-99m labeled albumin clearly demonstrated a diffuse protein loss involving the whole jejunum and ileum. Subsequent jejunal and duodenal biopsies showed the typical histological findings of intestinal lymphangiectasia thereby confirming a diffuse intestinal lymphatic damage. In addition to the gastrointestional symptoms the patient developed a pericardial effusion diagnosed by echocardiographic imaging. Dietary treatment with middle chained triglycerides and intravenous human albumin supplementation was followed by the reduction of the ascites and improvement of the peripheral lymphedema. To our knowledge this is the first description of the yellow nail syndrome associated with a diffuse lymphangiectasia involving the whole small bowel.

PubMed: 8956479

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<nlm:affiliation>Dept. of Gastroenterology and Hepatology, Medizinische Hochschule Hannover, Germany.</nlm:affiliation>
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<name sortKey="Ocran, K" sort="Ocran, K" uniqKey="Ocran K" first="K" last="Ocran">K. Ocran</name>
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<name sortKey="Tietge, U J" sort="Tietge, U J" uniqKey="Tietge U" first="U J" last="Tietge">U J Tietge</name>
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<name sortKey="Maschek, H" sort="Maschek, H" uniqKey="Maschek H" first="H" last="Maschek">H. Maschek</name>
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<name sortKey="Gratz, K F" sort="Gratz, K F" uniqKey="Gratz K" first="K F" last="Gratz">K F Gratz</name>
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<name sortKey="Trautwein, C" sort="Trautwein, C" uniqKey="Trautwein C" first="C" last="Trautwein">C. Trautwein</name>
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<name sortKey="Wagner, S" sort="Wagner, S" uniqKey="Wagner S" first="S" last="Wagner">S. Wagner</name>
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<term>Adult</term>
<term>Biopsy</term>
<term>Chylous Ascites (diet therapy)</term>
<term>Chylous Ascites (etiology)</term>
<term>Chylous Ascites (pathology)</term>
<term>Combined Modality Therapy</term>
<term>Duodenum (pathology)</term>
<term>Humans</term>
<term>Jejunum (pathology)</term>
<term>Lymphangiectasis, Intestinal (complications)</term>
<term>Lymphangiectasis, Intestinal (diet therapy)</term>
<term>Lymphangiectasis, Intestinal (pathology)</term>
<term>Male</term>
<term>Nail Diseases (diet therapy)</term>
<term>Nail Diseases (etiology)</term>
<term>Nail Diseases (pathology)</term>
<term>Pericardial Effusion (diet therapy)</term>
<term>Pericardial Effusion (etiology)</term>
<term>Pericardial Effusion (pathology)</term>
<term>Pigmentation Disorders (diet therapy)</term>
<term>Pigmentation Disorders (etiology)</term>
<term>Pigmentation Disorders (pathology)</term>
<term>Pleural Effusion (diet therapy)</term>
<term>Pleural Effusion (etiology)</term>
<term>Pleural Effusion (pathology)</term>
<term>Serum Albumin (administration & dosage)</term>
<term>Triglycerides (administration & dosage)</term>
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<term>Adulte</term>
<term>Ascite chyleuse (anatomopathologie)</term>
<term>Ascite chyleuse (diétothérapie)</term>
<term>Ascite chyleuse (étiologie)</term>
<term>Association thérapeutique</term>
<term>Biopsie</term>
<term>Duodénum (anatomopathologie)</term>
<term>Humains</term>
<term>Jéjunum (anatomopathologie)</term>
<term>Lymphangiectasie intestinale ()</term>
<term>Lymphangiectasie intestinale (anatomopathologie)</term>
<term>Lymphangiectasie intestinale (diétothérapie)</term>
<term>Mâle</term>
<term>Onychopathies (anatomopathologie)</term>
<term>Onychopathies (diétothérapie)</term>
<term>Onychopathies (étiologie)</term>
<term>Sérumalbumine (administration et posologie)</term>
<term>Triglycéride (administration et posologie)</term>
<term>Troubles de la pigmentation (anatomopathologie)</term>
<term>Troubles de la pigmentation (diétothérapie)</term>
<term>Troubles de la pigmentation (étiologie)</term>
<term>Épanchement pleural (anatomopathologie)</term>
<term>Épanchement pleural (diétothérapie)</term>
<term>Épanchement pleural (étiologie)</term>
<term>Épanchement péricardique (anatomopathologie)</term>
<term>Épanchement péricardique (diétothérapie)</term>
<term>Épanchement péricardique (étiologie)</term>
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<term>Serum Albumin</term>
<term>Triglycerides</term>
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<term>Duodénum</term>
<term>Jéjunum</term>
<term>Lymphangiectasie intestinale</term>
<term>Onychopathies</term>
<term>Troubles de la pigmentation</term>
<term>Épanchement pleural</term>
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<term>Chylous Ascites</term>
<term>Lymphangiectasis, Intestinal</term>
<term>Nail Diseases</term>
<term>Pericardial Effusion</term>
<term>Pigmentation Disorders</term>
<term>Pleural Effusion</term>
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<term>Ascite chyleuse</term>
<term>Lymphangiectasie intestinale</term>
<term>Onychopathies</term>
<term>Troubles de la pigmentation</term>
<term>Épanchement pleural</term>
<term>Épanchement péricardique</term>
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<term>Chylous Ascites</term>
<term>Nail Diseases</term>
<term>Pericardial Effusion</term>
<term>Pigmentation Disorders</term>
<term>Pleural Effusion</term>
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<term>Chylous Ascites</term>
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<term>Jejunum</term>
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<term>Pericardial Effusion</term>
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<term>Biopsy</term>
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<front>
<div type="abstract" xml:lang="en">A 26-year-old male patient with a history of chronic peripheral lymphedema, yellowish coloured slow growing nails and pleural effusions since early childhood is described. After 23 years he developed a chylous ascites and scintigraphy with technetium-99m labeled albumin clearly demonstrated a diffuse protein loss involving the whole jejunum and ileum. Subsequent jejunal and duodenal biopsies showed the typical histological findings of intestinal lymphangiectasia thereby confirming a diffuse intestinal lymphatic damage. In addition to the gastrointestional symptoms the patient developed a pericardial effusion diagnosed by echocardiographic imaging. Dietary treatment with middle chained triglycerides and intravenous human albumin supplementation was followed by the reduction of the ascites and improvement of the peripheral lymphedema. To our knowledge this is the first description of the yellow nail syndrome associated with a diffuse lymphangiectasia involving the whole small bowel.</div>
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