Favism: Clinical Features at Different Ages

Beretta, Alice; Manuelli, M.; Cena, Hellas

doi:10.3390/nu15020343

Cited by 5 publications

(2 citation statements)

References 37 publications

(32 reference statements)

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“…G6PD deficiency results in a decreased level of NADPH, which is crucial for reducing oxidized glutathione, particularly in red blood cells. The oxidized glutathione acts as a potent oxidizing agent, ultimately causing the onset of a hemolytic crisis 6 . Hemolytic crisis caused by fava beans is rare in children with G6PD deficiency.…”

Section: Discussionmentioning

confidence: 99%

Severe Hemolysis Triggered by Favism in a Female Patient with G6PD Deficiency: A Case Report

Rameli,

Juan,

Chi

et al. 2023

Biomed. Res. Ther.

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Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a hereditary enzymatic disorder that can result in acute hemolytic anemia under certain conditions. Favism, induced by ingesting fava beans, is a well-known trigger of hemolysis in individuals with G6PD deficiency. We report the case of a five-year-old girl who presented to our hospital with signs and symptoms of hemolysis. At admission, we were unaware that she had an underlying G6PD deficiency and detected it while performing laboratory investigations to determine the etiology.

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Section: Discussionmentioning

confidence: 99%

Severe Hemolysis Triggered by Favism in a Female Patient with G6PD Deficiency: A Case Report

Rameli,

Juan,

Chi

et al. 2023

Biomed. Res. Ther.

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“…It is not surprising that diabetic rats compensate for ROS stress through increasing the phosphate pentose pathway to increase the NADPH level [73]. On the other hand, note that the key enzyme of NADPH formation is glucose-6-phosphate dehydrogenase, and the importance of this route in the maintenance of the redox state is illustrated in erythrocytes where the inactivation of this enzyme leads to hemolytic anemia due to the deficit in NADPH [128,129].…”

Section: Nadph Oxidase (Nox)mentioning

confidence: 99%

Hyperglycemia and Oxidative Stress: An Integral, Updated and Critical Overview of Their Metabolic Interconnections

González

Lozano

Ros‐Berruezo

et al. 2023

IJMS

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This review focuses on the multiple and reciprocal relationships that exist between oxidative stress, hyperglycemia and diabetes and related metabolic disorders. Human metabolism uses most of the consumed glucose under aerobic conditions. Oxygen is needed in the mitochondria to obtain energy, as well as for the action of microsomal oxidases and cytosolic pro-oxidant enzymes. This relentlessly generates a certain amount of reactive oxygen species (ROS). Although ROS are intracellular signals necessary for some physiological processes, their accumulation leads to oxidative stress, hyperglycemia, and progressive resistance to insulin. A cellular pro-oxidant versus an antioxidant equilibrium would regulate ROS levels, but oxidative stress, hyperglycemia, and pro-inflammatory conditions feed back to each other and the relevance of the interconnections tends to increase those conditions. Hyperglycemia promotes collateral glucose metabolism through protein kinase C, polyols and hexosamine routes. In addition, it also facilitates spontaneous glucose auto-oxidation and the formation of advanced glycation end products (AGEs), which in turn interact with their receptors (RAGE). The mentioned processes undermine cellular structures, finally giving place to a progressively greater degree of oxidative stress with further hyperglycemia, metabolic alterations, and diabetes complications. NFκB is the major transcription factor involved in the expression of most of the pro-oxidant mediators, while Nrf2 is the major transcription factor regulating the antioxidant response. FoxO is also involved in the equilibrium, but its role is controversial. This review summarizes the key factors linking the diverse glucose metabolic routes enhanced in hyperglycemia with ROS formation and vice versa, emphasizing the role of the major transcription factors involved in the desirable balance between pro-oxidant and antioxidant proteins.

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Medications and Acute Hemolysis in G6PD‐Deficient Patients – A Real‐World Study

Gronich,

Rosh,

Stein

et al. 2024

Clin Pharma and Therapeutics

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Many drug labels contain precautions of use in G6PD‐deficient patients due to hemolytic concerns, but much of this is based on scarce clinical, epidemiological, or structural data. In this real‐world study, we aimed to examine if the administration of presumably risky medications for G6PD‐deficient patients was followed by hemolysis. The study is based on data from Clalit Health Services database that provides inclusive health care for more than half of the Israeli population (~ 4.7 million). Within the database, we identified all G6PD‐deficient patients by G6PD <6 U/g Hb. Within the G6PD‐deficient cohort, we identified all hospitalizations with a discharge diagnosis of hemolysis (January 1, 2010 to December 31, 2022), validated the cases, and identified the culprit event. For the rest of the G6PD‐deficient patients with no‐hemolysis, we recorded filled prescriptions of medications listed as presumably risky. We identified 31,962 G6PD‐deficient patients. Within the cohort, there were 71 cases of major hemolysis requiring hospitalization (0.2% of the cohort), of whom 51 (71.8%) had been caused by ingestion of fava beans, six (8.5%) were associated with an infection, and three (4.2%) suggested to be associated with medications (nitrofurantoin, phenazopyridine, and a “pain killer”). Within the 31,875 patients with no major hemolysis, nitrofurantoin has been prescribed safely to 1,366 G6PD‐deficient males and females; hundreds/thousands of G6PD‐deficient patients had been prescribed safely ciprofloxacin, glibenclamide, ofloxacin, phenazopyridine, sulfamethoxazole/cotrimoxazole, sulfasalazine, hydroxychloroquine, glimepiride, mesalazine, and sulfacetamide. In this real‐world study, we are showing that a list of medications, suspected previously as carrying risks for hemolysis in G6PD‐deficient patients, have been prescribed safely to G6PD‐deficient patients, providing reassurance to patients, prescribers, and regulators.

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Favism: Clinical Features at Different Ages

Cited by 5 publications

References 37 publications

Severe Hemolysis Triggered by Favism in a Female Patient with G6PD Deficiency: A Case Report

Severe Hemolysis Triggered by Favism in a Female Patient with G6PD Deficiency: A Case Report

Hyperglycemia and Oxidative Stress: An Integral, Updated and Critical Overview of Their Metabolic Interconnections

Medications and Acute Hemolysis in G6PD‐Deficient Patients – A Real‐World Study

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