What is the morbidity and mortality associated with pediatric chronic granulomatous disease (CGD)?

Updated: Aug 07, 2019
  • Author: Lawrence C Wolfe, MD; Chief Editor: Cameron K Tebbi, MD  more...
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Answer

A detailed study of the natural history of CGD is unavailable. The registry data suggest that both morbidity and mortality rates are highest in patients with the X-linked form of the disease. A substantial number of patients in the registry died during the second and third decades of life, though some survived beyond the fourth decade. Approximately 80% of patients were alive at 5 years after they were entered in the registry. Even in the modern age of care for this disease, sporadic data suggest a potential excess in mortality in individuals aged 10-30 years. In a European study of 429 patients, based on 2000-2003 data, mean survival time for patients with X-linked CGD (gp91phox deficient) was 37.8 years, and for those with autosomal recessive CGD, 49.6 years. [7]


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