Chronic granulomatous disease CGD

• Facts:
  Defect of phagocytic cells
  Dysfunction of the NADPH oxidase enzyme complex
  Recurrent infections with catalase positive organisms
• History / PE:
  Lymphadenopathy
  Hepatosplenomegaly
  Supporative adenitis
  Hypergammaglobulinemia
  ACD
  Chronic diarrhea
  Short stature
• Diagnosis:
  Neutrophils filled with bacteria on Gram stain
  Abnormal nitroblue tetrazolium (NBT) slide test
  Flow cytometry
  Positive superoxide production testing
• Notes:
  Catalase positive organisms (S. aureus, Aspergillus, Serratia marcescens, Klebsiella, Pseudomonas)