Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s Disease

Tricho-hepato-enteric syndrome (syndromic, phenotypic diarrhea, SD/THES) is a rare inborn disease, which affects bowels. It is caused by the mutation of genes SKIV2L or TTC37. Manifestations include intrauterine hypotrophy, severe chronic diarrhea, which starts in infancy, characteristic facial feat...

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Main Authors: Е. А. Roslavtseva, Т. E. Borovik, Е. G. Tsimbalova, А. О. Anushenko, А. S. Potapov, М. М. Lokhmatov, I. L. Chashchina, М. D. Bakradze
Format: Article
Language:English
Published: "Paediatrician" Publishers LLC 2015-08-01
Series:Вопросы современной педиатрии
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Online Access:https://vsp.spr-journal.ru/jour/article/view/709
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author Е. А. Roslavtseva
Т. E. Borovik
Е. G. Tsimbalova
А. О. Anushenko
А. S. Potapov
М. М. Lokhmatov
I. L. Chashchina
М. D. Bakradze
author_facet Е. А. Roslavtseva
Т. E. Borovik
Е. G. Tsimbalova
А. О. Anushenko
А. S. Potapov
М. М. Lokhmatov
I. L. Chashchina
М. D. Bakradze
author_sort Е. А. Roslavtseva
collection DOAJ
description Tricho-hepato-enteric syndrome (syndromic, phenotypic diarrhea, SD/THES) is a rare inborn disease, which affects bowels. It is caused by the mutation of genes SKIV2L or TTC37. Manifestations include intrauterine hypotrophy, severe chronic diarrhea, which starts in infancy, characteristic facial features and hair growth abnormalities, immune disorders. There are data on two patients dealing with tricho-hepato-enteric syndrome with underlying Crohn’s disease. This is the first description of cases of aggravated tricho-hepatoenteric syndrome ever found in Russian medical literature.
format Article
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institution Kabale University
issn 1682-5527
1682-5535
language English
publishDate 2015-08-01
publisher "Paediatrician" Publishers LLC
record_format Article
series Вопросы современной педиатрии
spelling doaj-art-64338c96cf0546b997e50b28f9330d762025-08-20T03:35:16Zeng"Paediatrician" Publishers LLCВопросы современной педиатрии1682-55271682-55352015-08-0114341642110.15690/vsp.v14i3.1381709Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s DiseaseЕ. А. Roslavtseva0Т. E. Borovik1Е. G. Tsimbalova2А. О. Anushenko3А. S. Potapov4М. М. Lokhmatov5I. L. Chashchina6М. D. Bakradze7Scientific Centre of Children Health, Moscow, Russian FederationScientific Centre of Children Health, Moscow, Russian Federation I.M. Sechenov First Moscow State Medical University, Russian FederationScientific Centre of Children Health, Moscow, Russian FederationScientific Centre of Children Health, Moscow, Russian FederationScientific Centre of Children Health, Moscow, Russian Federation I.M. Sechenov First Moscow State Medical University, Russian FederationScientific Centre of Children Health, Moscow, Russian Federation I.M. Sechenov First Moscow State Medical University, Russian FederationScientific Centre of Children Health, Moscow, Russian FederationScientific Centre of Children Health, Moscow, Russian Federation I.M. Sechenov First Moscow State Medical University, Russian FederationTricho-hepato-enteric syndrome (syndromic, phenotypic diarrhea, SD/THES) is a rare inborn disease, which affects bowels. It is caused by the mutation of genes SKIV2L or TTC37. Manifestations include intrauterine hypotrophy, severe chronic diarrhea, which starts in infancy, characteristic facial features and hair growth abnormalities, immune disorders. There are data on two patients dealing with tricho-hepato-enteric syndrome with underlying Crohn’s disease. This is the first description of cases of aggravated tricho-hepatoenteric syndrome ever found in Russian medical literature.https://vsp.spr-journal.ru/jour/article/view/709malabsorption syndromeintestinal failureparenteralenteral nutritiontricho-hepato-enteric syndromesyndromic (phenotypic) diarrhea
spellingShingle Е. А. Roslavtseva
Т. E. Borovik
Е. G. Tsimbalova
А. О. Anushenko
А. S. Potapov
М. М. Lokhmatov
I. L. Chashchina
М. D. Bakradze
Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s Disease
Вопросы современной педиатрии
malabsorption syndrome
intestinal failure
parenteral
enteral nutrition
tricho-hepato-enteric syndrome
syndromic (phenotypic) diarrhea
title Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s Disease
title_full Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s Disease
title_fullStr Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s Disease
title_full_unstemmed Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s Disease
title_short Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea) with Underlying Crohn’s Disease
title_sort cases of trichohepatoenteric syndrome syndromic diarrhea with underlying crohn s disease
topic malabsorption syndrome
intestinal failure
parenteral
enteral nutrition
tricho-hepato-enteric syndrome
syndromic (phenotypic) diarrhea
url https://vsp.spr-journal.ru/jour/article/view/709
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