Spontaneous abdominal esophageal perforation in a patient with mitochondrial neurogastrointestinal encephalomyopathy
Yükleniyor...
Tarih
2015
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Maney Publishing
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive multisystem disorder caused by thymidine phosphorylase deficiency. Severe denutrition is almost constant during the course of the disease which leads to severe malnutrition and requires long-term parenteral nutrition in most cases. Patients with MNGIE syndrome and chronic intestinal pseudo-obstruction have a particularly poor prognosis and they usually die around 40 years of age. Gastrointestinal perforation associated with MNGIE is extremely rare. Herein we present our unique case with MNGIE associated abdominal esophageal perforation.
Açıklama
Onder, Fatih Oguz/0000-0003-2845-201X
Anahtar Kelimeler
Esophagus, MNGIE, CIPO, Perforation
Kaynak
Acta Clinica Belgica
WoS Q Değeri
Q3
Scopus Q Değeri
Q1
Cilt
70
Sayı
1
Künye
closedAccess
