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How to treat an extensive form of primary intestinal lymphangiectasia?

Identifieur interne : 000E08 ( PubMed/Curation ); précédent : 000E07; suivant : 000E09

How to treat an extensive form of primary intestinal lymphangiectasia?

Auteurs : Rosana Troskot [Croatie] ; Dragan Jur I [Croatie] ; Ante Bili [Croatie] ; Marija Gomer I Pal I [Croatie] ; Stanko Težak [Croatie] ; Ivana Brajkovi [Croatie]

Source :

World journal of gastroenterology [ 2219-2840 ] ; 2015.

RBID : pubmed:26109821

Descripteurs français

KwdFr :
- Adulte, Agents gastro-intestinaux (usage thérapeutique), Agrégat d'albumine marquée au technétium (99mTc), Biopsie, Entéroscopie double ballon, Grand mal épileptique (étiologie), Humains, Intestins (), Intestins (anatomopathologie), Intestins (imagerie diagnostique), Lymphangiectasie intestinale (), Lymphangiectasie intestinale (diagnostic), Lymphangiectasie intestinale (traitement médicamenteux), Lymphoedème (), Lymphoedème (diagnostic), Lymphoedème (traitement médicamenteux), Mâle, Octréotide (usage thérapeutique), Radiopharmaceutiques, Résultat thérapeutique, Scintigraphie, Valeur prédictive des tests.
MESH :
- anatomopathologie : Intestins.
- diagnostic : Lymphangiectasie intestinale, Lymphoedème.
- imagerie diagnostique : Intestins.
- traitement médicamenteux : Lymphangiectasie intestinale, Lymphoedème.
- usage thérapeutique : Agents gastro-intestinaux, Octréotide.
- étiologie : Grand mal épileptique.
- Adulte, Agrégat d'albumine marquée au technétium (99mTc), Biopsie, Entéroscopie double ballon, Humains, Intestins, Lymphangiectasie intestinale, Lymphoedème, Mâle, Radiopharmaceutiques, Résultat thérapeutique, Scintigraphie, Valeur prédictive des tests.

English descriptors

KwdEn :
- Adult, Biopsy, Double-Balloon Enteroscopy, Epilepsy, Tonic-Clonic (etiology), Gastrointestinal Agents (therapeutic use), Humans, Intestines (diagnostic imaging), Intestines (drug effects), Intestines (pathology), Lymphangiectasis, Intestinal (complications), Lymphangiectasis, Intestinal (diagnosis), Lymphangiectasis, Intestinal (drug therapy), Lymphedema (complications), Lymphedema (diagnosis), Lymphedema (drug therapy), Male, Octreotide (therapeutic use), Predictive Value of Tests, Radionuclide Imaging, Radiopharmaceuticals, Technetium Tc 99m Aggregated Albumin, Treatment Outcome.
MESH :
- chemical , therapeutic use : Gastrointestinal Agents, Octreotide.
- complications : Lymphangiectasis, Intestinal, Lymphedema.
- diagnosis : Lymphangiectasis, Intestinal, Lymphedema.
- diagnostic imaging : Intestines.
- drug effects : Intestines.
- drug therapy : Lymphangiectasis, Intestinal, Lymphedema.
- etiology : Epilepsy, Tonic-Clonic.
- pathology : Intestines.
- Adult, Biopsy, Double-Balloon Enteroscopy, Humans, Male, Predictive Value of Tests, Radionuclide Imaging, Radiopharmaceuticals, Technetium Tc 99m Aggregated Albumin, Treatment Outcome.

Abstract

We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia, which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa. Although numerous therapeutic strategies are available, only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.

DOI: 10.3748/wjg.v21.i23.7320
PubMed: 26109821

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<front><div type="abstract" xml:lang="en">We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia, which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa. Although numerous therapeutic strategies are available, only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.</div>
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<Title>World journal of gastroenterology</Title>
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<ArticleTitle>How to treat an extensive form of primary intestinal lymphangiectasia?</ArticleTitle>
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<Abstract><AbstractText>We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia, which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa. Although numerous therapeutic strategies are available, only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.</AbstractText>
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<CommentsCorrectionsList><CommentsCorrections RefType="Cites"><RefSource>J Pediatr Gastroenterol Nutr. 2010 Oct;51(4):454-7</RefSource>
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<CommentsCorrections RefType="Cites"><RefSource>Gastroenterology. 1961 Sep;41:197-207</RefSource>
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<CommentsCorrections RefType="Cites"><RefSource>J Korean Med Sci. 2004 Jun;19(3):466-9</RefSource>
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<CommentsCorrections RefType="Cites"><RefSource>Dig Dis Sci. 2005 Aug;50(8):1467-70</RefSource>
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<CommentsCorrections RefType="Cites"><RefSource>Ann Vasc Dis. 2012;5(1):96-9</RefSource>
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<CommentsCorrections RefType="Cites"><RefSource>Z Gastroenterol. 2013 Jun;51(6):576-9</RefSource>
<PMID Version="1">23229460</PMID>
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<CommentsCorrections RefType="Cites"><RefSource>Nihon Naika Gakkai Zasshi. 1988 Jul;77(7):1037-40</RefSource>
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<CommentsCorrections RefType="Cites"><RefSource>Pediatrics. 2002 Jun;109(6):1177-80</RefSource>
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<CommentsCorrections RefType="Cites"><RefSource>J Gastroenterol. 2001 Feb;36(2):129-32</RefSource>
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<CommentsCorrections RefType="Cites"><RefSource>J Nucl Med. 1996 Apr;37(4):639-41</RefSource>
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How to treat an extensive form of primary intestinal lymphangiectasia?

How to treat an extensive form of primary intestinal lymphangiectasia?

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