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G6PD Deficiency Treatment & Management

  • Author: Lawrence C Wolfe, MD; Chief Editor: George T Griffing, MD  more...
 
Updated: Jul 28, 2015
 

Approach Considerations

Most individuals with G6PD deficiency do not require any treatment.

The acute hemolytic anemia in G6PD-deficient patients is largely preventable by avoiding exposure to fava beans, drugs, and chemicals that can cause oxidant stress. Identification and discontinuation of the precipitating agent is critical in management of hemolysis in patients with G6PD deficiency.

Anemia secondary to mild-to-moderate hemolysis in G6PD deficient patients is usually self-limited and often resolves in 8-14 days. Transfusion is rarely needed in cases of severe anemia.

 

 

 

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Medical Care

Infants with prolonged neonatal jaundice as a result of G6PD deficiency should receive phototherapy. Exchange transfusion may be necessary in cases of severe neonatal jaundice or hemolytic anemia caused by favism.

Systematic assessment for the risk of severe hyperbilirubinemia should be performed before discharge in neonates in whom G6PD deficiency is suspected to provide early and focused follow-up to prevent bilirubin encephalopathy.[11, 13, 5]

Persons with chronic hemolysis or nonspherocytic anemia should be placed on daily folic acid supplements. Consultations with a hematologist are ideal for long-term follow up.

Vaccination against hepatitis A and B is recommended in communities with high prevalence of G6PD deficiency.[33]

Transcriptional upregulation of G6PD enzyme in response to HDACi (histone deacetylase inhibitors) in in-vitro experiments on human B cells and erythroid precursor cells has been reported by Makarona K et al, which opens new areas of potential treatment in future.[18]

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Surgical Care

There is no evidence of selective red cell destruction in the spleen; hence splenectomy is usually ineffective and not recommended.

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Consultations

Consultations with a hematologist are ideal for long-term follow up of those with chronic hemolysis or nonspherocytic anemia.

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Contributor Information and Disclosures
Author

Lawrence C Wolfe, MD Associate Chief for Hematology and Safety, Division of Pediatric Hematology-Oncology, Cohen Children's Medical Center

Lawrence C Wolfe, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association of Blood Banks, American Society of Hematology, Children's Oncology Group, Eastern Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Shilpa Shukla, MBBS Fellow in Pediatric Hematology/Oncology, North Shore-LIJ Cohen Children’s Medical Center

Shilpa Shukla, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society of Pediatric Hematology/Oncology, Medical Council of India, Hemostasis and Thrombosis Research Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

George T Griffing, MD Professor Emeritus of Medicine, St Louis University School of Medicine

George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, International Society for Clinical Densitometry, Southern Society for Clinical Investigation, American College of Medical Practice Executives, American Association for Physician Leadership, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical and Translational Research, Endocrine Society

Disclosure: Nothing to disclose.

Additional Contributors

Frederick H Ziel, MD Associate Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Physician-In-Charge, Endocrinology/Diabetes Center, Director of Medical Education, Kaiser Permanente Woodland Hills; Chair of Endocrinology, Co-Chair of Diabetes Complete Care Program, Southern California Permanente Medical Group

Frederick H Ziel, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society for Bone and Mineral Research, California Medical Association, Endocrine Society, International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Acknowledgements

Bernard Corenblum, MD, FRCP(C) Professor of Medicine, Director, Endocrine-Metabolic Testing and Treatment Unit, Ovulation Induction Program, Department of Internal Medicine, Division of Endocrinology, University of Calgary, Canada

Disclosure: Nothing to disclose. Gregory A Kline, MD Associate Professor, Department of Medicine, Division of Endocrinology, Richmond Road Diagnostic Centre, University of Calgary Faculty of Medicine, Canada

Gregory A Kline, MD is a member of the following medical societies: Canadian Medical Association and Christian Medical & Dental Society

Disclosure: Nothing to disclose.

Vasudevan A Raghavan, MBBS, MD, MRCP(UK) Director, Cardiometabolic and Lipid (CAMEL) Clinic Services, Division of Endocrinology, Scott and White Hospital, Texas A&M Health Science Center College of Medicine

Vasudevan A Raghavan, MBBS, MD, MRCP(UK) is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Heart Association, National Lipid Association, Royal College of Physicians, and The Endocrine Society

Disclosure: Nothing to disclose.

References
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