An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar
Protein-losing enteropathy (PLE) is a rare condition characterized by protein loss through the gastrointestinal tract, leading to hypo-proteinemia. Patients may be asymptomatic or present with variety of complications of hypoproteinemia (e.g., oedema, ascites, pleural, and cardial effusions). We des...
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doaj-fa6d10f817e9407e9da2cf709ed393882020-11-25T02:29:31ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602020-03-01810.3389/fped.2020.00082520479An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind TrichobezoarMariangela Stinco0Alessandra Montemaggi1Bruno Noccioli2Massimo Resti3Salvatore Grosso4Sandra Trapani5Pediatric Section, Department of Health Sciences, Meyer Children's University Hospital, University of Florence, Florence, ItalyPediatric Section, Department of Health Sciences, Meyer Children's University Hospital, University of Florence, Florence, ItalyPediatric Surgery Unit, Meyer Children's University Hospital, University of Florence, Florence, ItalyPediatric Section, Department of Health Sciences, Meyer Children's University Hospital, University of Florence, Florence, ItalyPediatric Neurology-Immunology and Endocrinology Unit, Le Scotte Hospital, University of Siena, Siena, ItalyPediatric Section, Department of Health Sciences, Meyer Children's University Hospital, University of Florence, Florence, ItalyProtein-losing enteropathy (PLE) is a rare condition characterized by protein loss through the gastrointestinal tract, leading to hypo-proteinemia. Patients may be asymptomatic or present with variety of complications of hypoproteinemia (e.g., oedema, ascites, pleural, and cardial effusions). We describe a case report of a young girl suffering from behavioral disorder since childhood who presented with generalized oedema, hypoproteinaemia, and microcytic hypochromic anemia. In addition, the girl had an intervention for jejunal atresia and intestinal malrotation in her past medical history. Upper gastrointestinal endoscopy revealed a trichobezoar extending from stomach into the small bowel, thus classified as Rapunzel Syndrome (RS), causing mechanical obstruction of intestinal lumen and intestinal lymphatic drainage resulting in a protein-losing enteropathy (PLE). Trichobezoar was successfully removed by a surgical laparotomy resulting in resolution of symptoms and normalization of biochemical parameters. Possibly, previous surgery might have had an influence on intestinal dysmotility and trichobezoar formation. PLE is a very rare presenting symptom of RS, developing as result of intestinal obstruction caused by large trichobezoars. RS has to be considered in patients, especially adolescents, suffering from behavior disorder as trichotillomania and trichophagia. Surgical removal and nutritional supplementation are the gold treatment of large trichobezoar.https://www.frontiersin.org/article/10.3389/fped.2020.00082/fullRapunzel Syndrometrichobezoarhypoproteinemiaprotein-losing enteropathyoedema |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Mariangela Stinco Alessandra Montemaggi Bruno Noccioli Massimo Resti Salvatore Grosso Sandra Trapani |
spellingShingle |
Mariangela Stinco Alessandra Montemaggi Bruno Noccioli Massimo Resti Salvatore Grosso Sandra Trapani An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar Frontiers in Pediatrics Rapunzel Syndrome trichobezoar hypoproteinemia protein-losing enteropathy oedema |
author_facet |
Mariangela Stinco Alessandra Montemaggi Bruno Noccioli Massimo Resti Salvatore Grosso Sandra Trapani |
author_sort |
Mariangela Stinco |
title |
An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar |
title_short |
An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar |
title_full |
An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar |
title_fullStr |
An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar |
title_full_unstemmed |
An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar |
title_sort |
unusual case of hypoproteinemia in childhood: keep in mind trichobezoar |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Pediatrics |
issn |
2296-2360 |
publishDate |
2020-03-01 |
description |
Protein-losing enteropathy (PLE) is a rare condition characterized by protein loss through the gastrointestinal tract, leading to hypo-proteinemia. Patients may be asymptomatic or present with variety of complications of hypoproteinemia (e.g., oedema, ascites, pleural, and cardial effusions). We describe a case report of a young girl suffering from behavioral disorder since childhood who presented with generalized oedema, hypoproteinaemia, and microcytic hypochromic anemia. In addition, the girl had an intervention for jejunal atresia and intestinal malrotation in her past medical history. Upper gastrointestinal endoscopy revealed a trichobezoar extending from stomach into the small bowel, thus classified as Rapunzel Syndrome (RS), causing mechanical obstruction of intestinal lumen and intestinal lymphatic drainage resulting in a protein-losing enteropathy (PLE). Trichobezoar was successfully removed by a surgical laparotomy resulting in resolution of symptoms and normalization of biochemical parameters. Possibly, previous surgery might have had an influence on intestinal dysmotility and trichobezoar formation. PLE is a very rare presenting symptom of RS, developing as result of intestinal obstruction caused by large trichobezoars. RS has to be considered in patients, especially adolescents, suffering from behavior disorder as trichotillomania and trichophagia. Surgical removal and nutritional supplementation are the gold treatment of large trichobezoar. |
topic |
Rapunzel Syndrome trichobezoar hypoproteinemia protein-losing enteropathy oedema |
url |
https://www.frontiersin.org/article/10.3389/fped.2020.00082/full |
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