What does G6PD
Glucose-6-phosphate dehydrogenase deficiency (favism)
Glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency) is an inherited metabolic defect in the red blood cells (erythrocytes).
Author: Prof. Dr. med. Arnulf Pekrun, Julia Dobke, created on: February 13th, 2019, editing: Ingrid Grüneberg, approval: PD Dr. med. Gesche Tallen, Prof. Dr. med. Ursula Creutzig, last changed: 07/17/2020
The glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency) is an inherited metabolic defect of the red blood cells (erythrocytes) It is the world's most common enzyme deficiency disease. The defect means that the erythrocytes necessary for the transport of oxygen in the blood, under certain conditions, break down very quickly in the bloodstream (hemolysis) and are therefore no longer functional. As a result, life-threatening anemia, a so-called haemolytic crisis, with the need for a blood transfer (blood transfusion) can occur within hours to a few days. A hemolytic crisis can be triggered by certain foods or drugs and occasionally by febrile infections. Hemolytic crises can occur at any age. Fortunately, the great majority of those affected by glucose-6-phosphate dehydrogenase deficiency rarely or never experience a hemolytic crisis.
The following information text is aimed in particular at patients with G6PD deficiency and their relatives, friends, teachers and other caregivers as well as the interested public. It should help to better understand this disease, the possibilities of its treatment and the problems and special needs of the patients. Our information is not a substitute for the necessary clarifying discussions with the treating doctors; however, they can help to prepare these conversations and to understand them better.
Notes on the text
The information contained in this patient text was created by the authors named above. These authors are specialists in blood disorders in children and adolescents, especially G6PD deficiency. The information text is based on the experience of the specialists and on the literature given below. The text was last edited by the above-mentioned editors for this information portal in January 2019 and released for posting on the Internet for a period of five years. It should be checked again and updated no later than the end of this period.
Please note that the following are general information and recommendations that - given the complex situation - do not necessarily apply to every patient in their entirety.
- Kunz J, Kulozik A: Hemolytic anemia. In: Niemeyer C, Eggert A. ed. Pediatric Hematology and Oncology Springer; 2018, 55 [DOI: 10.1007 / 978-3-662-43686-8_2] KUN2018a
- Luzzatto L, Arese P: Favism and Glucose-6-Phosphate Dehydrogenase Deficiency. The New England journal of medicine 2018, 378: 60 [PMID: 29298156] LUZ2018
- Youngster I, Arcavi L, Schechmaster R, Akayzen Y, Popliski H, Shimonov J, Beig S, Berkovitch M: Medications and glucose-6-phosphate dehydrogenase deficiency: an evidence-based review. Drug safety 2010, 33: 713 [PMID: 20701405] YOU2010
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Recommended reading review
Medication administration / avoidance
In this review, drugs are divided into three groups: I. drugs to be avoided by G6PD patients, II. Possible drugs with evidence limitations, III. Drugs, no evidence against use in G6PD patientsReview Youngster et al 2010
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