Spontaneous Restoration of Nutrition Autonomy in a Case of Intestinal Failure Secondary to a Gastrointestinal Neuromuscular Disease

Spontaneous Restoration of Nutrition Autonomy in a Case of Intestinal Failure Secondary to a Gastrointestinal Neuromuscular Disease

Polyglucosan inclusion body myopathy (PIBM) is a recently described gastrointestinal neuromuscular disease. Given its rarity, very little is known of its natural history and prognosis. In this case report, we present the first case of PIBM and resultant intestinal failure in which, following 7 years...

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Veröffentlicht in:Nutrition in clinical practice 2019-12, Vol.34 (6), p.935-939
Hauptverfasser: McCulloch, Adam, Malhi, Hardip, Parkinson, Sharmalie, Martin, Joanne E., Cooper, Sheldon C.
Format: Artikel
Sprache:eng
Schlagworte:
enteric dysmotility
Gastrointestinal Motility
gastrointestinal neuromuscular disease
Glucans - metabolism
Glycogen Storage Disease - therapy
Humans
Inclusion Bodies - pathology
Intestinal Diseases - etiology
Intestinal Diseases - pathology
Intestine, Small - pathology
Male
Middle Aged
Nervous System Diseases - therapy
Nursing
Nutritional Status
parenteral nutrition
Parenteral Nutrition, Home - methods
polyglucosan body myopathy
Prognosis
Recovery of Function
Treatment Outcome
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Zusammenfassung:Polyglucosan inclusion body myopathy (PIBM) is a recently described gastrointestinal neuromuscular disease. Given its rarity, very little is known of its natural history and prognosis. In this case report, we present the first case of PIBM and resultant intestinal failure in which, following 7 years of home parenteral nutrition, gastrointestinal symptoms subsided and nutrition autonomy was restored.
ISSN:0884-5336
1941-2452
DOI:10.1002/ncp.10312