Pathology definition - Chronic Granulomatous Disease Of Childhood
Chronic Granulomatous Disease Of The Childhood
Children with chronic granulomatous disease may present with susceptibility to opportunistic bacterial infection such as Aspergillus, Staphylococcus aureus, E. Coli and Serratia.
Chronic granulomatous disease of the childhood is an inherited autosomal recessive disorder or X linked inherited disorder. This may result in defect in the gene which coded for the component of the NADPH oxidase. NADPH oxidase is an enzyme which produce superoxide. The treatment will focus on the TMP - SMX prophylaxis and gamma interferon.
Chronic granulomatous disease of the childhood may present with lacking of the superoxide.Superoxide may be useful to fight bacteria. Superoxide may be required by neutrophils in myeloperoxidase halide system to combat bacteria. Superoxide may also be produced by bacterial metabolism.
Certain type of organism such as catalase positive organism may destroy the superoxide. If NADPH oxidase is not present in case of chronic granulomatous disease of childhood there will be not enough superoxide and the myeloperoxidase halide system to kill bacteria mostly catalase positive bacteria ( staphylococcus aureus, e. coli, aspergillus and serratia. Laboratory investigation may reveal negative nitroblue tetrazolium dye reduction test.
Children with chronic granulomatous disease may present with susceptibility to opportunistic bacterial infection such as Aspergillus, Staphylococcus aureus, E. Coli and Serratia.
Chronic granulomatous disease of the childhood is an inherited autosomal recessive disorder or X linked inherited disorder. This may result in defect in the gene which coded for the component of the NADPH oxidase. NADPH oxidase is an enzyme which produce superoxide. The treatment will focus on the TMP - SMX prophylaxis and gamma interferon.
Chronic granulomatous disease of the childhood may present with lacking of the superoxide.Superoxide may be useful to fight bacteria. Superoxide may be required by neutrophils in myeloperoxidase halide system to combat bacteria. Superoxide may also be produced by bacterial metabolism.
Certain type of organism such as catalase positive organism may destroy the superoxide. If NADPH oxidase is not present in case of chronic granulomatous disease of childhood there will be not enough superoxide and the myeloperoxidase halide system to kill bacteria mostly catalase positive bacteria ( staphylococcus aureus, e. coli, aspergillus and serratia. Laboratory investigation may reveal negative nitroblue tetrazolium dye reduction test.
