Clinical features

The respiratory burst of phagocytes is required for the optimal killing of a wide variety of bacteria and fungi. In its absence the microbes are engulfed but killing is impaired. This manifests in the patients in an increased frequency of infection, predominantly in the reticulo endothelial system. The most common sites include cervical lymph nodes, lungs, bone marrow (leading to osteomyelitis), liver and skin. Staphylococcus aureus is by far the most common infecting pathogen, although a wide variety of otherwise relatively avirulent organisms, for example Ser-ratia marcescens can cause serious infections in these subjects. CGD must be excluded in children presenting with cervical or inguinal adenitis, liver abscesses, or aspergillus or nocardia pneumonia.

The defective function of phagocytes from these patients is not limited to their failure to kill microbes. Digestion by these cells is also grossly defective. This results in the diffuse development in the tissues of the granulomas that have lent their name to this syndrome. Granulomas in biopsies from these patients often result in an incorrect diagnosis of tuberculosis being made. Abnormal digestion and the granulomatous response result in poor healing, with the breakdown of wounds and chronic sinus formation. Exuberant attempts at healing can result in narrowing and obstruction of hollow muscular organs like the duodenum and urinary system. Chronic inflammation of the gut may mimic inflammatory bowel disease, particularly Crohn's disease, where bowel disease is associated with the presence of granulomas in histological sections.

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