Clinical–Liver, Pancreas, and Biliary TractHepatic Involvement and Portal Hypertension Predict Mortality in Chronic Granulomatous Disease
Section snippets
Materials and Methods
A large cohort of patients with CGD has been followed up at the Clinical Center of the National Institutes of Health as part of a natural history protocol. All patients had the diagnosis of CGD confirmed by either nitroblue tetrazolium reduction or dihydrorhodamine oxidation.14 The specific gene defect was determined by immunoblotting and/or sequencing.
Results
A total of 194 patients with CGD were evaluated. Thirty-one patients were excluded because of inadequate clinical follow-up evaluation (<4 mo), including 2 patients who died. Characteristics of the remaining 163 patients are shown in Table 1.
Discussion
Major advances in the management of patients with CGD have led to marked improvements in survival; however, premature mortality caused by repeated infectious complications is still the reality for patients with this disease. In an attempt to understand why after surviving repeated infections patients die from a given infectious episode, we examined predictors of mortality in a large cohort of well-characterized patients with CGD. Although all deaths were caused by infection, regression analysis
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This research was supported by the Intramural Research Programs of the National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Allergy and Infectious Diseases, Clinical Center, and National Cancer Institute, National Institutes of Health.