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Glucose-6-phosphate dehydrogenase deficiency

Последний просмотренный: 18 Aug 2025
Last updated: 05 Dec 2024

Резюме

Определение

Анамнез и осмотр

Ключевые диагностические факторы

  • at-risk demographic
  • positive family history
  • history of infection or exposure to oxidative drugs or broad (fava) beans
  • jaundice
  • pallor
  • dark urine
Полная информация

Другие диагностические факторы

  • nausea
  • cataract
  • splenomegaly
Полная информация

Факторы риска

  • male sex
  • neonate
  • ethnic origin in the Mediterranean, sub-Saharan Africa, Middle East, or Southeast Asia
  • family history
  • recent exposure to oxidative drugs
  • infection
  • recent exposure to broad beans (favism)
Полная информация

Диагностические исследования

Исследования, которые показаны в первую очередь

  • CBC
  • reticulocyte count
  • urinalysis
  • unconjugated (indirect) bilirubin
  • LDH
  • haptoglobin
  • peripheral blood smear
Полная информация

Исследования, проведение которых нужно рассмотреть

  • G6PD fluorescent spot test
  • G6PD spectrophotometry
  • molecular analysis
Полная информация

Неотложные исследования

  • point of care testing

Алгоритм лечения

Острый

acute hemolysis

neonates with prolonged indirect hyperbilirubinemia

ПРОДОЛЖЕНИЕ

chronic nonspherocytic hemolytic anemia

Составители

Авторы

Atul Bhanu Mehta, MA, MD, FRCP, FRCPath

Professor of Haematology and Consultant Haematologist

Royal Free Hospital and University College London School of Medicine

London

UK

Раскрытие информации

ABM declares that he has no competing interests.

Рецензенты

Lucio Luzzatto, MD

Professor of Hematology

University of Firenze

Florence

Italy

Раскрытие информации

LL declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Список литературы

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Основные статьи

WHO Working Group. Glucose-6-phosphate dehydrogenase deficiency. Bull World Health Organ. 1989;67(6):601-11. Аннотация

Cappellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet. 2008;371:64-74. Аннотация

Roper D, Layton M, Rees D, et al. Laboratory diagnosis of G6PD deficiency. A British Society for Haematology Guideline. Br J Haematol. 2020 Apr;189(1):24-38.Полный текст  Аннотация

Luzzatto L, Nannelli C, Notaro R. Glucose-6-phosphate dehydrogenase deficiency. Hematol Oncol Clin North Am. 2016 Apr;30(2):373-93. Аннотация

World Health Organization. Guide to G6PD deficiency rapid diagnostic testing to support P. vivax radical cure. Jul 2018 [internet publication].Полный текст

Статьи, указанные как источники

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
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