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Endocrine, Metabolic and Immune Disorders - Drug Targets 2020-Aug

Clinical Manifestations and Therapeutic Findings of the Children with Glucose-6-Phosphate Dehydrogenase Deficiency Presenting Favism

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Fariba Tarhani
Alireza Nezami
Ghobad Heidari
Babak Abdolkarimi

Cuvinte cheie

Abstract

Aim: Favism is characterized as acute anemia, due to Glucose-6-phosphate dehydrogenase (G6PD) deficiency as a result of fava beans intake. It is associated with paleness, jaundice, and hemoglobinuria. In this study, signs, symptoms and therapeutic findings of the patients with hemolysis due to G6PD deficiency were investigated in Shahid Madani Hospital of Khorramabad, Lorestan.

Methods: This is a single-center cross-sectional descriptive study that was conducted on all children with G6PD deficiencyinduced hemolysis.

Results: 308 children (64.3% male and 35.7% female) were included in this study. The most common complaint was jaundice (82.5%) and the most common cause of hemolysis was the intake of fava bean (85.7%). 68% of the children were treated with hydration/fluid therapy. Blood transfusion was conducted in 36.36% of the cases and the mean of blood administered was 18.9 cc/kg.

Conclusion: In this study, hydration therapy was performed in most of the children presenting favism. Also, the incorrect calculation of the amount of blood needed for transfusion has increased the frequency of blood transfusions and prolonged hospitalization time.

Keywords: G6PD deficiency; blood transfusions; favism; hematology; hemoglobinuria.; pediatrics.

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