Chronic granulomatous disease.

ABSTRACT:

Introduction

Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by recurrent, life-threatening bacterial and fungal infections of the skin, the airways, the lymph nodes, the liver, the brain and the bones. Frequently found pathogens are Staphylococcus aureus, Aspergillus species, Klebsiella species, Burkholderia cepacia, Serratia marcescens and Salmonella species.

Sources of data

CGD is a rare (∼1:250 000 individuals) disease caused by mutations in any one of the five components of the NADPH oxidase in phagocytic leucocytes. This enzyme generates superoxide and is essential for intracellular killing of pathogens by phagocytes.

Areas of agreement

CGD patients suffer not only from life-threatening infections, but also from excessive inflamma

SUBMITTER: Roos D 

PROVIDER: S-EPMC5127417 | biostudies-literature | 2016 Jun

REPOSITORIES: biostudies-literature