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

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 deficiency-induced 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 increased the frequency of blood transfusions and prolonged hospitalization time.

Leukocytosis in patients with favism and association with blood transfusion.

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.

[Favism after ingestion of fava beans in a three-year-old child with glucose-6-phosphate dehydrogenase deficiency].

A three-year-old Syrian boy was hospitalized with symptoms of acute haemolytic anaemia after ingestion of fava beans. He was stabilized by blood transfusion, and genetic examination revealed glucose-6-phosphate dehydrogenase (G6PD) deficiency. Oxidative stress, e.g. ingestion of fava beans, can induce acute haemolytic anaemia in affected individuals. Approximately 400 million people worldwide suffer from G6PD deficiency. The prevalence is high in African, Mediterranean and Middle East countries. Due to increased immigration, we might expect the condition to occur more often in Danish healthcare.

Favismo: una entidad a tener en cuenta en la zona mediterránea / Favism: a disease to keep in mind in the Mediterranean area

Se denomina favismo a la hemólisis aguda que se desarrolla tras la ingestión de habas o el polen de estas1. Los síntomas se desarrollan horas después de la ingestión, siendo comunes las náuseas, vómitos, malestar y vértigo. A estos síntomas le sigue una hemólisis. El favismo en el área mediterránea se debe a una variante de la glucosa-6-fosfato deshidrogenasa (G6PDH)1. Para su diagnóstico es fundamental una completa anamnesis, exploración física y la determinación de la actividad enzimática para la G6PDH. Los pacientes deben ser educados con unos consejos dietéticos y deben conocer la posibilidad de crisis hemolíticas agudas ante determinadas infecciones y exposición a determinados fármacos o tóxicos

Favism is called acute hemolysis that has developed after intake of fava beans or their pollen1. The symptoms develop hours after intake. The common symptoms are nausea, vomits, malaise and vertigo. Hemolysis follows these symptoms. Favism in the Mediterranean area is due to a variant of glucose-6-phosphate-dehydrogenase (G6PDH)1. A complete anamnesis, physical examination and measurement of enzyme activity for G6PDH are essential for its diagnosis. The patients should be educated with dietary advise and should know the possibility of acute hemolytic episodes when there are certain infections and exposure to certain drugs or toxics

[Favism. Acute hemolysis after intake of fava beans]. / Favisme. Akutt hemolyse etter inntak av favabønner.

Acute haemolysis due to Glucose-6-Phosphate-Dehydrogenase deficiency is a common disorder in American and African Blacks, in Mediterranean people and among Orientals. The erythrocytes in affected individuals have insufficient reducing power against toxic peroxydes and free radicals generated during metabolism. Normally, affected individuals are without signs of disease, but under the influence of oxydants severe intravascular haemolysis may occur. One of the most important oxydants is the fava bean which, when ingested, may cause acute favism, a condition which has a 10% mortality if not treated properly. We describe a 35 year-old man from Iraq who developed serious haemolytic anemia with a fall in haemoglobin to 6.5 g/100 ml three days after ingestion of fava beans. He was treated with intravenous fluids and blood transfusions. He recovered and was discharged from hospital after nine days. This is the first described case of favism in Norway.

Favism in a female newborn infant whose mother ingested fava beans before delivery.

We describe a case of favism in a female newborn infant with glucose-6-phosphate dehydrogenase (G6PD) deficiency whose mother had ingested fava beans 5 days before delivery. At birth there were clinical and hematologic signs of hemolytic anemia, hemoglobinuria, and no blood group immunization. Study of the G6PD activity and 2-deoxy-glucose-6-phosphate utilization rate revealed that the infant and the mother were heterozygous for G6PD deficiency.

Haptoglobin therapy for acute favism: a Japanese boy with glucose-6-phosphate dehydrogenase Guadalajara.

We report the case of a 2-year-old Japanese boy with acute favism who was treated with human haptoglobin products. He had been exhibiting chronic nonspherocytic haemolytic anaemia until the diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency when 14 months old. He suffered a favic crisis at 24 months of age, when the administration of haptoglobin was effective for relieving bilirubinaemia and haemoglobinuria. Serum-free Hb rapidly decreased to normal levels despite the sustained level of serum lactate dehydrogenase. His G6PD gene was G6PD Guadalajara. This is the first application of haptoglobin therapy for acute favism and the first reported case of Japanese G6PD deficiency with typical favic crisis. Haptoglobin treatment might be helpful for managing the haemolytic crisis in the disease.