-
Postgraduate Medical Journal Dec 1994
Review
Topics: Anemia, Hemolytic; Favism; Female; Glucosephosphate Dehydrogenase Deficiency; Humans; Infant, Newborn; Jaundice, Neonatal; Male
PubMed: 7870632
DOI: 10.1136/pgmj.70.830.871 -
Frontiers in Pharmacology 2021Restrictions on the cultivation and ingestion of fava beans were first reported as early as the fifth century BC. Not until the late 19th century were clinical... (Review)
Review
Restrictions on the cultivation and ingestion of fava beans were first reported as early as the fifth century BC. Not until the late 19th century were clinical descriptions of fava-induced disease reported and soon after characterised as "favism" in the early 20th century. It is now well known that favism as well as drug-induced haemolysis is caused by a deficiency of the glucose-6-phosphate dehydrogenase (G6PD) enzyme, one of the most common enzyme deficiency in humans. Interest about the interaction between G6PD deficiency and therapeutics has increased recently because mass treatment with oxidative 8-aminoquinolines is necessary for malaria elimination. Historically, assessments of haemolytic risk have focused on the clinical outcomes (e.g., haemolysis) associated with either a simplified phenotypic G6PD characterisation (deficient or normal) or an ill-fitting classification of G6PD genetic variants. It is increasingly apparent that detailed knowledge of both aspects is required for a complete understanding of haemolytic risk. While more attention has been devoted recently to better phenotypic characterisation of G6PD activity (including the development of new point-of care tests), the classification of G6PD variants should be revised to be clinically useful in malaria eliminating countries and in populations with prevalent G6PD deficiency. The scope of this work is to summarize available literature on drug-induced haemolysis among individuals with different G6PD variants and to highlight knowledge gaps that could be filled with further clinical and laboratory research.
PubMed: 33790795
DOI: 10.3389/fphar.2021.638885 -
ACS Omega Jan 2023Red blood cells (RBCs) are exposed to both external and internal sources of oxidants that challenge their integrity and compromise their physiological function and... (Review)
Review
Red blood cells (RBCs) are exposed to both external and internal sources of oxidants that challenge their integrity and compromise their physiological function and supply of oxygen to tissues. Autoxidation of oxyhemoglobin is the main source of endogenous RBC oxidant production, yielding superoxide radical and then hydrogen peroxide. In addition, potent oxidants from other blood cells and the surrounding endothelium can reach the RBCs. Abundant and efficient enzymatic systems and low molecular weight antioxidants prevent most of the damage to the RBCs and also position the RBCs as a sink of vascular oxidants that allow the body to maintain a healthy circulatory system. Among the antioxidant enzymes, the thiol-dependent peroxidase peroxiredoxin 2, highly abundant in RBCs, is essential to keep the redox balance. A great part of the RBC antioxidant activity is supported by an active glucose metabolism that provides reducing power in the form of NADPH via the pentose phosphate pathway. There are several RBC defects and situations that generate oxidative stress conditions where the defense mechanisms are overwhelmed, and these include glucose-6-phosphate dehydrogenase deficiencies (favism), hemoglobinopathies like sickle cell disease and thalassemia, as well as packed RBCs for transfusion that suffer from storage lesions. These oxidative stress-associated pathologies of the RBCs underline the relevance of redox balance in these anucleated cells that lack a mechanism of DNA-inducible antioxidant response and rely on a complex and robust network of antioxidant systems.
PubMed: 36643550
DOI: 10.1021/acsomega.2c06768 -
Canadian Medical Association Journal Sep 1960
PubMed: 20326465
DOI: No ID Found -
British Journal of Haematology Feb 1970
Review
Topics: Black People; Chemical Phenomena; Chemistry; Dihydroxyphenylalanine; Favism; Female; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Humans; Isoenzymes; Male; Mutation; Neoplasms; Sex Chromosomes; White People
PubMed: 4909233
DOI: 10.1111/j.1365-2141.1970.tb01426.x -
Nutrition Bulletin Sep 2019Pulse crops have been known for a long time to have beneficial nutritional profiles for human diets but have been neglected in terms of cultivation, consumption and... (Review)
Review
Pulse crops have been known for a long time to have beneficial nutritional profiles for human diets but have been neglected in terms of cultivation, consumption and scientific research in many parts of the world. Broad dietary shifts will be required if anthropogenic climate change is to be mitigated in the future, and pulse crops should be an important component of this change by providing an environmentally sustainable source of protein, resistant starch and micronutrients. Further enhancement of the nutritional composition of pulse crops could benefit human health, helping to alleviate micronutrient deficiencies and reduce risk of chronic diseases such as type 2 diabetes. This paper reviews current knowledge regarding the nutritional content of pea ( L.) and faba bean ( L.), two major UK pulse crops, and discusses the potential for their genetic improvement.
PubMed: 31598097
DOI: 10.1111/nbu.12399 -
Anesthesia Progress 2009Glucose-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... (Review)
Review
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.
Topics: Anesthesia, Dental; Dental Care for Chronically Ill; Glucosephosphate Dehydrogenase Deficiency; Hemolysis; Humans; Mass Screening; Oxidative Stress; Perioperative Care; Risk Factors
PubMed: 19769422
DOI: 10.2344/0003-3006-56.3.86 -
Journal of Clinical Laboratory Analysis Jul 2019Leukocytosis is a condition marked by abnormal increase in leukocyte count due to an inflammatory response as a defense against most of the infections, or bone tumors;... (Clinical Trial)
Clinical Trial
INTRODUCTION
Leukocytosis is a condition marked by abnormal increase in leukocyte count due to an inflammatory response as a defense against most of the infections, or bone tumors; including leukemia. The aim of this study is to analyze the effect of blood transfusion in leukocytosis patients with favism as compared to patients treated with antibiotics or combination of both.
METHODS
A total of 97 patients with favism who were referred to the University hospital in 2016-2017 were studied.
KEY FINDINGS
These patients experienced acute hemolysis following beans meal. These patients were either treated with blood transfusion, antibiotics or combination of both.
CONCLUSIONS
This study shows that blood transfusion is an effective therapeutic option for the treatment of leukocytosis. Antibiotics are not deemed necessary for the treatment and blood transfusion alone, can decrease leukocytes to the normal level.
Topics: Anti-Bacterial Agents; Blood Transfusion; Favism; Female; Humans; Leukocyte Count; Leukocytosis; Male; Treatment Outcome
PubMed: 31074073
DOI: 10.1002/jcla.22906 -
The American Journal of Clinical... Sep 2009This review explores the relation between evolution, ecology, and culture in determining human food preferences. The basic physiology and morphology of Homo sapiens sets... (Review)
Review
This review explores the relation between evolution, ecology, and culture in determining human food preferences. The basic physiology and morphology of Homo sapiens sets boundaries to our eating habits, but within these boundaries human food preferences are remarkably varied, both within and between populations. This does not mean that variation is entirely cultural or learned, because genes and culture may coevolve to determine variation in dietary habits. This coevolution has been well elucidated in some cases, such as lactose tolerance (lactase persistence) in adults, but is less well understood in others, such as in favism in the Mediterranean and other regions. Genetic variation in bitter taste sensitivity has been well documented, and it affects food preferences (eg, avoidance of cruciferous vegetables). The selective advantage of this variation is not clear. In African populations, there is an association between insensitivity to bitter taste and the prevalence of malaria, which suggests that insensitivity may have been selected for in regions in which eating bitter plants would confer some protection against malaria. Another, more general, hypothesis is that variation in bitter taste sensitivity has coevolved with the use of spices in cooking, which, in turn, is thought to be a cultural tradition that reduces the dangers of microbial contamination of food. Our evolutionary heritage of food preferences and eating habits leaves us mismatched with the food environments we have created, which leads to problems such as obesity and type 2 diabetes.
Topics: Biological Evolution; Culture; Diet; Environment; Food Preferences; Genes; Humans; Taste Perception
PubMed: 19656837
DOI: 10.3945/ajcn.2009.27462B -
Bulletin of the World Health... 1973Favism is a potential obstacle to the use of the fava bean in the development of a locally produced, inexpensive weaning food for the Middle East and North Africa. The...
Favism is a potential obstacle to the use of the fava bean in the development of a locally produced, inexpensive weaning food for the Middle East and North Africa. The purposes of this study were to define the epidemiology of favism, to evaluate the advisability of using the fava bean in a weaning food, and to suggest ways of avoiding or eliminating the toxic factor in the bean. Field observations, locally acquired data, and a literature review suggested that the use of the fava bean in a weaning food would be hazardous, but that the hazard might be overcome by using certain strains of the bean or, more particularly, by using old dried beans. The disease is usually directly related in time to the harvesting and availability of fresh beans, but it is also associated with fresh dried beans. On the basis of the age distribution of the disease, patterns of bean consumption, and local food taboos it appears that the toxic factor is concentrated in the skin of the bean, that it is heat-stable, that in dried beans it decreases with age, and that it crosses into the breast milk of lactating mothers. It also appears that disease expression may be a result of the interaction of several host factors, such as nutritional status and the consumption of other foods. These observations are consistent with the results of laboratory studies, which incriminate vicine, divicine, and DOPA in the etiology of favism.
Topics: Adolescent; Africa, Northern; Aged; Asia, Western; Child; Child, Preschool; Europe; Favism; Female; Glucosephosphate Dehydrogenase Deficiency; Humans; Infant; Male; Vegetables
PubMed: 4541143
DOI: No ID Found