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Chronic granulomatous disease: Treatment and prognosis

Authors
Sergio D Rosenzweig, MD
Steven M Holland, MD
Section Editor
E Richard Stiehm, MD
Deputy Editor
Elizabeth TePas, MD, MS

INTRODUCTION

Chronic granulomatous disease (CGD) is a genetically heterogeneous condition characterized by recurrent, life-threatening bacterial and fungal infections and granuloma formation. CGD is caused by defects in phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (phox). The cornerstones of CGD management are antimicrobial and immunomodulatory prophylaxis, early diagnosis of infections, and aggressive management of infectious complications.

CGD was initially termed "fatal granulomatous disease of childhood" because patients rarely survived past their first decade in the time before routine use of prophylactic antimicrobial agents. The average patient now survives at least 40 years. Overall, survival rates are better for females than males, reflecting the greater severity of X-linked CGD.

The treatment and prognosis of CGD are reviewed here. The pathogenesis, clinical manifestations, and diagnosis of CGD, as well as an overview of primary disorders of phagocytic function, are discussed separately. (See "Chronic granulomatous disease: Pathogenesis, clinical manifestations, and diagnosis" and "Primary disorders of phagocytic function: An overview".)

MANAGEMENT

The management of chronic granulomatous disease (CGD) focuses on aggressive diagnosis and treatment of infections. The reduction in mortality and morbidity seen over the past few decades is largely attributable to antimicrobial prophylaxis and rapid recognition and treatment of infections in these patients [1-4].

The cornerstones of CGD management are [5]:

              

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Literature review current through: Nov 2016. | This topic last updated: Tue Jul 14 00:00:00 GMT+00:00 2015.
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