Retrospective Study of Glucose-6-Phosphate-Dehydrogenase Deficiency

来源 :重庆医科大学 | 被引量 : 0次 | 上传用户:yanmu1984
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To investigate the pattern of G6PD deficiency patientswhom had been hospitalized in Children Hospital of Chongqing MedicalUniversity from January 2002 to May 2009.Methods:All G6PD deficiency patients that admitted at ChildrenHospital of Chongqing Medical University from January 2002 to May 2009included in the study. The result categorized by the gender, age, clinicalsymptoms, laboratory examination result, genetic variants and treatments.The results were analyzed with SPSS 17 statistical software by usingPearson Chi-Square test, and multivariate statistic. Finding was consideredstatistically significant if the P value<0.05.Results:A total of 308 patients whom had been admitted in ChildrenHospital of Chongqing Medical University from January 2002 to May 2009,8 years study, were include in this study. The male to female ratio was 6.89:1.The age peak incidence of the hospitalized patients is in the group of 1~3 years old children (58.8%). There are 200 patients(64.9%) with favismin this study, which is the most common form of G6PD deficiency inChongqing area. Followed by fava bean and infection induced hemolysisoccurred in 30 patients (9.7%), fava bean, infection and drugs inducedhemolysis occurred in 21 patients(3.6%), fava beans and drug inducedhemolysis occurred in 20 patients (6.5%), infection and drugs inducedhemolysis occurred in 11 patients (3.6%), Drugs induced hemolysisoccurred in 7 patients (2.3%), infection induced hemolysis occurred in 3patients (1.3%), CNSHA occurred in 3 patients(0.9%),and unclear reasoninduced in 13 patients(4.9%). The clinical symptoms of the hospitalizedpatients including pale in 283 patients (91.9%), the change of urine colorsin 279 patients (90.6%), jaundice in 277 patients (89.9%), fever in 142patients (46.1%), nausea and or vomiting in 114 patients(37%), centralnervous system symptoms(including delirium, spoor, coma, headache, andseizure) in 94 patients (30.5%), and abdominal pain in 30 patients(9.7%).The genetic variants study revealed as G1388A (G6PD Kaiping)in 147patients (47.7%), G1376T (G6PD Canton) in 91 patients (29.5%), andA95G in(G6PD Gaohe)23 patients (7.5%). The other 47 patients definedas unknown variants (15.3%). Statistical analysis of these three variantsshowed no significant difference among the variants concerning on theoccurring of clinical manifestations, except for the nausea and or vomitingand the change in urine colors. Of these three variants, the laboratory dataanalysis showed no significant statistic difference. From all of thehospitalized patients, 1 patient died from severe anemia caused mufti organ failure.Conclusions:This study showed that in Chongqing favism is the mostcommon clinical type of G6PD deficiency. The three most common geneticvariants of G6PD deficiency in Chongqing are G1388A (G6PD Kaiping),G1376T (G6PD Canton),and A95G (G6PD Gaohe),it covered 84.74%patients in this study. Statistical analysis showed there were no significantdifference of the G6PD genetic variants for the occurring of the mostclinical manifestation and laboratory results.
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