Immune deficiency in glycogen storage disease type 1B.
Patients with glycogen storage disease type 1B present the clinical features of glycogen storage disease type 1A. In addition, they are susceptible to recurrent bacterial infection and have recurrent oral ulcers and occasionally Crohn's-like disease. There features are due to immune dysfunction caused by a combination of neutropenia and impaired phagocytic functions.
Garty
B Z
BZ
Department of Pediatrics, Schneider Children's Medical Center of Israel, Petah Tikva.
Douglas
S D
SD
Danon
Y L
YL
D001424:Bacterial Infections / Q000209:etiology; D003424:Crohn Disease / Q000209:etiology; D005953:Glycogen Storage Disease Type I / Q000145:classification / Q000150:complications* / Q000201:enzymology / Q000276:immunology*; D006801:Humans; D016867:Immunocompromised Host / Q000276:immunology*; D009000:Monocytes / Q000276:immunology*; D009503:Neutropenia / Q000209:etiology*; D019226:Oral Ulcer / Q000209:etiology; D010585:Phagocyte Bactericidal Dysfunction / Q000209:etiology*; D012008:Recurrence