Abstract
It has been 50 years since chronic granulomatous disease was first reported as a disease which fatally affected the ability of children to survive infections. Various milestone discoveries from the insufficient ability of patients' leucocytes to destroy microbial particles to the underlying genetic predispositions through which the disease is inherited have had important consequences. Longterm antibiotic prophylaxis has helped to fight infections associated with chronic granulomatous disease while the steady progress in bone marrow transplantation and the prospect of gene therapy are hailed as long awaited permanent treatment options. This review unearths the important findings by scientists that have led to our current understanding of the disease.
Highlights
In 1954, at an annual meeting of the Society for Pediatric Research, Janeway and colleagues [1] first reported five cases of children with elevated serum gamma globulin levels that suffered from recurrent infections
Quie and colleagues further went on to demonstrate the defective bactericidal capacity of the polymorphonuclear leukocytes from affected males, meaning that these patients were unable to ward off infections [4]
Reports of the disease in females began to emerge, thereby revealing an autosomal recessive inheritance of the same phenotype [7,8]. It was that Nathan and Baehner [9] showed that leukocytes from chronic granulomatous disease (CGD) patients, unlike normal human leukocytes, did not reduce nitroblue tetrazolium (NBT), a compound that converts to insoluble blue formazan product upon reduction by the superoxide anion (O2-.)
Summary
Absence of both cytochrome b-245 subunits from neutrophils in X-linked chronic granulomatous disease. Umei T, Takeshige K, Minakami S: NADPH-binding component of the superoxide-generating oxidase in unstimulated neutrophils and the neutrophils from the patients with chronic granulomatous disease. Curnutte JT, Kuver R, Scott PJ: Activation of neutrophil NADPH oxidase in a cell-free system. Partial purification of components and characterization of the activation process.
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