2024/12/31 by BenRabehTrabelsi, R., Mazigh S, Atitallah S +5
#Congenitaltuftingenteropathy #Dysplasieépithélialeintestinale #congenitalenteropathy #entéropathiecongénitale #nutritionparentérale #parenteralnutrition
paper · doi:10.71566/pist-rmp-194752
Congenitaltuftingenteropathy(CTE)isacongenitalabnormalityofintestinalmucosadevelopmentpresenting withearly-onsetsevereintractablediarrheacausingsevereintestinalfailureandrequiringlong-termparenteral nutritionandinsomecasessmallboweltransplantation. WereportacaseofCTEdiagnosedat35daysoflife to illustratethe diagnostic and management difficulties ofthis rare disease,to reportthevarious complications relatedtothetreatmentandtodescribetheclinical,histologicalandgeneticheterogeneityofCTE.