Fava Bean- Versus Non-Fava Bean-Induced Acute Hemolytic Crisis in Children With Glucose-6-Phosphate Dehydrogenase Deficiency: A Prospective Comparative Study

IF 2.4 3区医学 Q2 HEMATOLOGY

Pediatric Blood & Cancer Pub Date : 2025-02-16 DOI:10.1002/pbc.31609

Gowda Parameshwara Prashanth, Mohammed Al-Shafey, Anita Tandon, Salim Ismail

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引用次数: 0

Abstract

Background

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a red cell enzymopathy in which exposure to oxidative stressors, such as drugs or fava bean ingestion, can trigger acute hemolytic episodes (AHEs). This study aimed to compare the clinical characteristics of fava bean-induced hemolysis (FBIH) with non-fava bean-induced hemolysis (NFBIH) in children with G6PD deficiency in a high-prevalence setting.

Methods

A prospective cohort study was conducted at a region referral hospital in Oman. Hospital records of children hospitalized for AHE due to G6PD deficiency over a 3-year period were analyzed. Participants were categorized into FBIH and NFBIH groups based on the documented precipitating factor.

Results

Among the 236 recruited cases, 51.6% AHEs were attributed to FBIH. Children with FIBH were younger, more likely to present with abdominal pain, and had greater severity of hemolysis upon admission (hemoglobin: 4.8 vs. 6.7 g/dL; p < 0.001). Lab markers such as serum ferritin, blood urea, lactate dehydrogenase, and alkaline phosphatase were significantly elevated in FBIH. The least squares regression model demonstrated a strong link between various predictor variables and hemoglobin levels, explaining about 76.6% of the variance in the study cohort.

Conclusion

Children with FBIH experience more severe hemolytic episodes compared to those with NFBIH. Our statistical model identified clinical and laboratory parameters potentially useful in early risk stratification during AHEs. Culturally sensitive dietary education of patients and caregivers is necessary, particularly in regions where fava beans are a dietary staple. The potential influence of specific G6PD genotypes within the NFBIH group merits future investigation.

查看原文本刊更多论文

蚕豆与非蚕豆诱导的葡萄糖-6-磷酸脱氢酶缺乏儿童急性溶血性危象：一项前瞻性比较研究。

背景：葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症是一种红细胞酶病，暴露于氧化应激源，如药物或蚕豆摄入，可引发急性溶血发作（AHEs）。本研究旨在比较高患病率G6PD缺乏症儿童蚕豆诱导溶血（FBIH）和非蚕豆诱导溶血（NFBIH）的临床特征。方法：在阿曼一家地区转诊医院进行前瞻性队列研究。分析了3年来因G6PD缺乏而住院的AHE患儿的住院记录。根据记录的诱发因素将参与者分为FBIH组和NFBIH组。结果：236例入选病例中，51.6%的ahs归因于FBIH。FIBH患儿年龄更小，更容易出现腹痛，入院时溶血严重程度更高(血红蛋白：4.8 vs 6.7 g/dL；结论：与NFBIH患儿相比，FBIH患儿经历更严重的溶血发作。我们的统计模型确定了临床和实验室参数在ahs的早期风险分层中可能有用。对患者和护理人员进行文化敏感的饮食教育是必要的，特别是在以蚕豆为主食的地区。特异性G6PD基因型对NFBIH组的潜在影响值得进一步研究。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Blood & Cancer 医学-小儿科

CiteScore

4.90

自引率

9.40%

发文量

546

审稿时长

1.5 months

期刊介绍： Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.