Clinical Analysis In 355 Childhood G6PD Deficiency

Objective:By collecting a total clinical data of 5 years in our hospital from 2 009 to 2013, found 355 cases of G6PD deficiency with hemolysis that were diagnosed and treated In clinical, and to reviwe and summarize 3 55 cases of children with G6PD deficiency in Chong Qing, and the c auses, the clinical manifestation, the treatment and prognosis were ana lyzed,and to understand the present situation of G6PD deficiency, to gi ve some guiding significance to the clinical prevention and control work, At the same time, to indicate the direction of the problem for the futur e clinical and research findings.Methods:1.To reviwe and summarize 355 cases of children with G-6-PD deficiency in the clinical data,and the causes,the pathogenesis,the clinical manifestation,the laboratory examination, the treatment and prognosis were analyzed.2.The main detection methods of G6PD deficiency in laborator y:(1) High-speed Rail hemoglobin reduction test; (2) The method to m easure the activity of G6PD; (3) G6PD gene detection.3.The statistical methods:by using SPSS 17 software to analyse t he data; statistical methods and statistical indicators:four table, chi squar e test, Fisher exact probability, mean, median, maximum value,minimum value, percentage, frequency.Results:1.The peak age of the disease incidence was 1 to 3 years old, the sex ratio was 17.7:1, and more in spring.2.Vicia faba,drugs,infection and other factors were the disease caus es,vicia faba have the highest morbidity(78.3%).3.Anemia,jaundice,hepatomegaly and the urine color change were t he m-ain clinical manifestations of the disease.4.Blood routine,urine routine,bilirubin,CK-MB, G-6-PD enzyme acti vity,methemoglobin reduction test,serum haptoglobin were abnormal.5.The disease had the 3 most common pathogenic mutations in the G-6-PD gene,the-y were G1388A(38.6%),G1376T(31.2%),A95G(10.8 %).6.All patients with removing incentives,blood transfution, alkalized urine and other supportive treatment could be cured,the disease had a good prognosis,with certain selflimiting.Conclusion:1.Favism was the most common clinical manifestation of G-6-PD deficiency in Chongqing, South West China.2.The three most common pathogenic genetic variants in this area were also the three most common genetic variants in China.3.Between each of these variants we found no significant difference for the clinical manifestation and laboratory results.4.Bying comparing and analysing the G-6-PD activity in each geno type, indicated that the reducing rate of G-6-PD activity in the three g enotypes was roughly the same, and the activity of G-6-PD decrease d with the genotype was not related.