New Pathogenic Mutations Associated with Diacylglycerol O-Acyltransferase 1 Deficiency.

Eldredge, Jessica A; Couper, Michael R; Barnett, Christopher P; Rawlings, Lesley; Couper, Richard T L · J Pediatr · 2021

case_report · Level V

Where this comes from

Abstract

Diacylglycerol O-acyltransferase 1 deficiency is a recently discovered, rare congenital diarrheal disorder. We report 2 patients with newly described pathogenic mutations in diacylglycerol O-acyltransferase 1 with compound heterozygous inheritance and unusual phenotypes. This included a macrophage activation syndrome-like response seen in one patient, ameliorated with low dietary fat.

Medical subject headings