What is the role of hypochlorous acid (HOCL) in chronic granulomatous disease?

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From the Research

Hypochlorous acid (HOCl) production is impaired in chronic granulomatous disease (CGD) due to genetic defects in the NADPH oxidase complex, leading to recurrent bacterial and fungal infections. The role of HOCl in CGD is crucial, as it is a potent antimicrobial agent that helps kill ingested pathogens in healthy individuals. However, in CGD patients, the absence of HOCl production due to the impaired NADPH oxidase system and myeloperoxidase enzyme results in an inability to effectively kill pathogens, despite normal neutrophil counts. Some key points to consider in the management of CGD include:

  • The use of prophylactic antimicrobials, such as trimethoprim-sulfamethoxazole, to prevent infections 1
  • The management of acute infections, which may require hospitalization and aggressive treatment 2
  • The potential for hematopoietic stem cell transplantation as a curative treatment option for selected patients 1
  • The importance of understanding the genetic and molecular aspects of CGD, as well as its impact on clinical expression and infectious complications 3 It is essential to note that while current therapies can help manage the symptoms and prevent infections, they do not directly correct the underlying HOCl production defect. Therefore, hematopoietic stem cell transplantation remains the only proven curative treatment for CGD 1.

References

2
Research

Modern management of chronic granulomatous disease.

British journal of haematology, 2008

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