| Manejo perioperatorio de los pacientes con deficiencia de glucose-6-fosfato deshidrogenasa: revisión de la literatura. |
Perioperative management of the glucose-6-phosphate dehydrogenase deficient patient: a review of literature.
Elyassi AR, Rowshan HH.
Tripler Army Medical Center, Honolulu, Hawaii, USA.Ali.Elyassi@US.Army.Mil
Anesth Prog. 2009 Autumn;56(3):86-91.
Abstract
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzymatic disorder of red blood cells in humans. It is estimated that about 400 million people are affected by this deficiency. The G6PD enzyme catalyzes the first step in the pentose phosphate pathway, leading to antioxidants that protect cells against oxidative damage. A G6PD-deficient patient, therefore, lacks the ability to protect red blood cells against oxidative stresses from certain drugs, metabolic conditions, infections, and ingestion of fava beans. The following is a literature review, including disease background, pathophysiology, and clinical implications, to help guide the clinician in management of the G6PD-deficient patient. A literature search was conducted in the following databases: PubMed, The Cochrane Library, Web of Science, OMIM, and Google; this was supplemented by a search for selected authors. Keywords used were glucose-6-phosphate dehydrogenase (G6PD) deficiency, anesthesia, analgesia, anxiolysis, management, favism, hemolytic anemia, benzodiazepines, codeine, codeine derivatives, ketamine, barbiturates, propofol, opioids, fentanyl, and inhalation anesthetics. Based on titles and abstracts, 23 papers and 1 website were identified. The highest prevalence of G6PD is reported in Africa, southern Europe, the Middle East, Southeast Asia, and the central and southern Pacific islands; however, G6PD deficiency has now migrated to become a worldwide disease. Numerous drugs, infections, and metabolic conditions have been shown to cause acute hemolysis of red blood cells in the G6PD-deficient patient, with the rare need for blood transfusion. Benzodiazepines, codeine/codeine derivatives, propofol, fentanyl, and ketamine were not found to cause hemolytic crises in the G6PD-deficient patient. The most effective management strategy is to prevent hemolysis by avoiding oxidative stressors. Thus, management for pain and anxiety should include medications that are safe and have not been shown to cause hemolytic crises, such as benzodiazepines, codeine/codeine derviatives, propofol, fentanyl, and ketamine. The authors of this article make 5 particular recommendations: (1) Anyone suspected of G6PD deficiency should be screened; (2) exposure to oxidative stressors in these individuals should be avoided; (3) these patients should be informed of risks along with signs and symptoms of an acute hemolytic crisis; (4) the clinician should be able to identify both laboratory and clinical signs of hemolysis; and finally, (5) if an acute hemolytic crisis is identified, the patient should be admitted for close observation and care.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2749581/pdf/i0003-3006-56-3-86.pdf |
| Manejo anestésico en pacientes con deficiencia de glucose-6-fosfato deshidrogenasa sometidos a neurocirugía. |
Anaesthetic management in patients with glucose-6-phosphate dehydrogenase deficiency undergoing neurosurgical procedures.
Valiaveedan S, Mahajan C, Rath GP, Bindra A, Marda MK.
Department of Neuroanaesthesiology, All India Institute of Medical Sciences, New Delhi, India.
Indian J Anaesth. 2011 Jan;55(1):68-70.
Abstract
Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is an X-linked recessive enzymopathy responsible for acute haemolysis following exposure to oxidative stress. Drugs which induce haemolysis in these patients are often used in anaesthesia and perioperative pain management. Neurosurgery and few drugs routinely used during these procedures are known to cause stress situations. Associated infection and certain foodstuffs are also responsible for oxidative stress. Here, we present two patients with G-6-PD deficiency who underwent uneventful neurosurgical procedures. The anaesthetic management in such patients should focus on avoiding the drugs implicated in haemolysis, reducing the surgical stress with adequate analgesia, and monitoring for and treating the haemolysis, should it occur.
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| Deficiencia de glucose-6-fosfato deshidrogenasa: perspectiva histórica |
Glucose-6-phosphate dehydrogenase deficiency: a historical perspective.
Beutler E.
Department of Molecular and Experimental Medicine, Scripps Research Institute, La Jolla, CA, USA.
Blood. 2008 Jan 1;111(1):16-24.
Abstract
Glucose-6-phosphate dehydrogenase deficiency serves as a prototype of the many human enzyme deficiencies that are now known. Since its discovery more than 50 years ago, the high prevalence of the defect and the easy accessibility of the cells that manifest it have made it a favorite tool of biochemists, epidemiologists, geneticists, and molecular biologists as well as clinicians. In this brief historical review, we trace the discovery of this defect, its clinical manifestations, detection, population genetics, and molecular biology
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