Diagnosis and Management of MNGIE Syndrome in Children: Case Report and Review of the Literature

ABSTRACT Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) syndrome is a rare disorder that presents in childhood; however, marked delay in diagnosis is common. We report a case and review the literature describing the typical features that should alert pediatricians to the diagnosis. We...

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Veröffentlicht in:	Journal of pediatric gastroenterology and nutrition 2002-09, Vol.35 (3), p.377-383
Hauptverfasser:	Teitelbaum, J. E., Berde, C. B., Nurko, S., Buonomo, C., Perez‐Atayde, A. R., Fox, V. L.
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Biological and medical sciences Celiac plexus neurolysis Child Duodenum - diagnostic imaging Female Gastroenterology. Liver. Pancreas. Abdomen Gastrointestinal Diseases - diagnostic imaging Gastrointestinal Diseases - physiopathology Gastrointestinal Diseases - therapy Humans Malformations Medical sciences Mitochondria Mitochondrial Encephalomyopathies - diagnostic imaging Mitochondrial Encephalomyopathies - physiopathology Mitochondrial Encephalomyopathies - therapy Parenteral Nutrition, Total Pseudoobstruction Stomach - diagnostic imaging Stomach. Duodenum. Small intestine. Colon. Rectum. Anus Tomography, X-Ray Computed Ubiquinone - therapeutic use Vitamins - therapeutic use
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Beschreibung
Zusammenfassung:	ABSTRACT Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) syndrome is a rare disorder that presents in childhood; however, marked delay in diagnosis is common. We report a case and review the literature describing the typical features that should alert pediatricians to the diagnosis. We also describe a novel management strategy for providing symptomatic relief.
ISSN:	0277-2116 1536-4801
DOI:	10.1002/j.1536-4801.2002.tb07841.x