The features of patients with favism in Turkey

Abstract

Glucose-6-phosphate dehydrogenase deficiency (G6PD) in south of Turkey is a very important health problem; the incidence of G6PD was reported 5, 4-20% by the surveillance studies. Patients with favism are admitted to urgent hospital service after ingestion of vicia faba beans especially at the spring season. Aim of this study was to investigate patients with favism who had a history of consumed fava as clinical, haematological, biochemical and mutations. Fifty patients, aged 1-16 years (mean±SD: 5.94±4.54 years), 40 males (80%) and 10 females (20%) were included in this study. The complaints of them were pale (100%), icterus (84%), haemoglobinuria (72%), abdominal pain (60%) and fever (4%). In their history they had neonatal hyperbilurubinaemia (40%) repeated acute haemolysis (10%) and chronic non-spherocytic haemolytic anaemia (6%). G6PD activities were ranging from a complete deficiency in 10 (20%), moderate in 12 (24%), mild in 5 (10%) to normal levels in 23 patients (46%). Molecular studies of the patients were detected in 35 subjects (70%); only Mediterranean mutations were detected (563T). In conclusion, our patients with favism had the same complaints and clinical findings with other G6PD patients but their biochemical enzyme levels were heterogeneous and the incidence of Mediterranean mutation was high. Copyright © Hellenic Society of Haematology.