Contributions
Abstract: PB1845
Type: Publication Only
Background
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common inherited enzyme deficiency, that affects more than 400 million people around the world with more than 300 variants. According to data by the World Health Organization which was published in 1989; 7.5% of people in the world have at least one gene G6PD deficiency and this ratio is the highest in sub-Saharan Africa and Southeast Asia (15-26%). This ratio is in the range of 0.5-2.9% in Turkey, as United States and the neighboring countries to Mediterranean Sea. The epidemiological studies about G6PD deficiency in Turkey were mostly regional or limited to a city.
Aims
We aimed to evaluate in terms of regional distribution and clinical features of G6PD deficiency by screening the patients who applied for soldier recruitment.
Methods
The patients who applied for soldier recruitment between January 2011-March 2016, were analyzed retrospectively. Patients, who were diagnosed G6PD deficiency were scanned by using hospital patient information system. The patients' ages, the cities they lived, complaints and the stories of them were questioned. Complete blood count, serum AST, LDH, total and direct bilirubin levels of all the cases in the study were recorded. G6PD levels were measured by quantitative spectrophotometric methods in biochemistry laboratory. The World Health Organization (WHO) is divided G6PD enzyme deficiency into five classes based on enzyme activity levels and clinical findings.
Results
The distribution of the cities where the cases were living, was given on the map in figure 1. Patients’ average age, hemoglobin, and G6PD levels were 26.42±4.62, 14.68 ± 1.51, and 0.86 ± 0.81 respectively. According to clinical history of patients prior to diagnosis, 29 patients (20.7%) were diagnosed after acute hemolytic episodes. Of these patients 23, 4, 2, had hemolytic episodes due to drug, infection, chemical respectively. Subsequently, 78 (54.5%) and 27 (18.9%) of the remaining patients were diagnosed G6PD deficiency after the examinations due to hemolysis after favism and prolonged neonatal jaundice respectively. 6 patients (4.3%) were diagnosed of G6PD deficiency by screening because of family history, but they didn't have any hemolytic episodes before. After the patients evaluated with their clinical history and hemolysis findings; 6 patients (4.3%), who had chronic hemolysis, was considered compatible with Class I variant. 128 cases were considered as Class II variants.
Conclusion
G6PD enzyme deficiency in Turkey is seen most frequently in the Mediterranean region and the prevalence of G6PD deficiency in Central Anatolia and Aegean regions was seem to be over the Turkey average (2.9%). Nearly half of the patients had hemolytic anemia due to favism. It is followed by hemolysis due to neonatal hyperbilirubinemia and drugs. 128 (91.4%) patients who had severe G6PD deficiency with intermittent hemolysis, were considered as Class II variants.
Session topic: 27. Enzymopathies, membranopathies and other anemias
Abstract: PB1845
Type: Publication Only
Background
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common inherited enzyme deficiency, that affects more than 400 million people around the world with more than 300 variants. According to data by the World Health Organization which was published in 1989; 7.5% of people in the world have at least one gene G6PD deficiency and this ratio is the highest in sub-Saharan Africa and Southeast Asia (15-26%). This ratio is in the range of 0.5-2.9% in Turkey, as United States and the neighboring countries to Mediterranean Sea. The epidemiological studies about G6PD deficiency in Turkey were mostly regional or limited to a city.
Aims
We aimed to evaluate in terms of regional distribution and clinical features of G6PD deficiency by screening the patients who applied for soldier recruitment.
Methods
The patients who applied for soldier recruitment between January 2011-March 2016, were analyzed retrospectively. Patients, who were diagnosed G6PD deficiency were scanned by using hospital patient information system. The patients' ages, the cities they lived, complaints and the stories of them were questioned. Complete blood count, serum AST, LDH, total and direct bilirubin levels of all the cases in the study were recorded. G6PD levels were measured by quantitative spectrophotometric methods in biochemistry laboratory. The World Health Organization (WHO) is divided G6PD enzyme deficiency into five classes based on enzyme activity levels and clinical findings.
Results
The distribution of the cities where the cases were living, was given on the map in figure 1. Patients’ average age, hemoglobin, and G6PD levels were 26.42±4.62, 14.68 ± 1.51, and 0.86 ± 0.81 respectively. According to clinical history of patients prior to diagnosis, 29 patients (20.7%) were diagnosed after acute hemolytic episodes. Of these patients 23, 4, 2, had hemolytic episodes due to drug, infection, chemical respectively. Subsequently, 78 (54.5%) and 27 (18.9%) of the remaining patients were diagnosed G6PD deficiency after the examinations due to hemolysis after favism and prolonged neonatal jaundice respectively. 6 patients (4.3%) were diagnosed of G6PD deficiency by screening because of family history, but they didn't have any hemolytic episodes before. After the patients evaluated with their clinical history and hemolysis findings; 6 patients (4.3%), who had chronic hemolysis, was considered compatible with Class I variant. 128 cases were considered as Class II variants.
Conclusion
G6PD enzyme deficiency in Turkey is seen most frequently in the Mediterranean region and the prevalence of G6PD deficiency in Central Anatolia and Aegean regions was seem to be over the Turkey average (2.9%). Nearly half of the patients had hemolytic anemia due to favism. It is followed by hemolysis due to neonatal hyperbilirubinemia and drugs. 128 (91.4%) patients who had severe G6PD deficiency with intermittent hemolysis, were considered as Class II variants.
Session topic: 27. Enzymopathies, membranopathies and other anemias