The Relationship Between Age, Infection And Treatment, Prognosis In Children With Glucose-6-Phosphate Dehydrogenase Deficiency In Chong Qing City

Objective:Heredity glucose-6-phosphate dehydrogenase(G6PD) deficiency in red blood cells is the most common enzymopathy of humans, afecting approximate 400 million people worldwide. The clinical manifestation of G6PD deficiency is diverse. From Symptomless to neonatal jaundice, acute haemolysis caused by drug or infection, chronic nonspherocytic hemolytic anemia(CNSHA). Between age, infection and treatment, prognosis in many diseases is relative. But the relationship between age, infection and treatment, prognosis in children with G6PD deficiency is unclear. The relative report is few. This article analysis clinical cases of patients with G6PD deficiency from April 2008 to April 2009 in children's hospital of Chong Qing medical university. Dividing into groups according to age and infection, comparing the difference of clinical manifestation, treatment and prognosis of every group, comprehending the relationship between age, infection and treatment, prognosis, in order to offer help to prevention, diagnosis, treatment. Methods:Retrospective analysis 124 patients with G6PD deficiency in hospital from April 2008 to April 2009 in children's hospital of Chong Qing medical university. All of them were final diagnosed with G6PD deficiency testified by quantitative analysis of nitroblne tetrazolium. Diagnosis all accords with Zhang Zhinan'criterion of blood illness diagnosis and effect. According to age they were divided into neonate group (72 cases) and non-neonate group (52 cases). Neonate group include 64 male and 8 female, with a median age of 8 days 10 hours. Non-neonate group include 45 male and 7 female, with a median age of 3 years 10 days. The two groups were divided into neonate+infestation group(62 cases), neonate+non-infestation group(10 cases), non-neonate+infestation group(38 cases), non-neonate+non-infestation group(14 cases) according to whether or not complicating infectious diseases. Comparing causative factor, clinical manifestation, laboratory examination, treatment and prognosis between neonate group and non-neonate group, comparing clinical manifestation, laboratory examination, treatment and prognosis between neonate+infestation group, neonate+non-infestation group,non-neonate+infestation group and non-neonate+non-infestation group.Results: 1.Causative factor: Infection is an important cause in neonate group and non-neonate group. The percentage of perinatal agent in neonate group is higher than non-neonate group(P<0.05); the percentage of eating broad bean in neonate group is lower than non-neonate group.2.Clinical manifestation: The main clinical manifestation of neonate group is jaundice; non-neonate group is anaemia, which have statistical significance(P<0.05). The percentages of jaundice, anaemia in neonate+infection group are higher than neonate+non-infection group, the percentages of fever, dark brown urina in non-neonate+infection group are higher than non-neonate+non-infection group.3.Laboratory examination: The hemoglobin(Hb) of neonate group is higher than non-neonate group(P<0.05); The total bilirubin(TBIL) of neonate+infection group is higher than neonate+non-infection group(P<0.05); the TBIL of non-neonate+infection group is higher than non-neonate+non-infection group(P<0.05). G6PD activity has no significant differences(P>0.05).4.Management: All the patients were given combined therapy. In neonate group 94.4% cases were given photoradiation treatment; In non-neonate group 69.2% cases were given blood transfusion, which have statistical significance(P<0.05). Blood transfusion of non-neonate+infection group is higher than non-neonate+non-infection group(P<0.05). 5.Prognosis: Length of stay and cure rate of neonate group and non-neonate group has no statistical significance(P>0.05); Length of stay of neonate+infection group is longer than neonate+non-infection group(P<0.05); Length of stay of non-neonate+infection group is longer than non-neonate+non-infection group (P<0.05).Conclusion:Infection is main causative factor to different ages children with G6PD deficiency. Therapic method of different ages children with G6PD deficiency is slightly different, but the relationship between age and prognosis is little. Infection can influence treatment and prognosis in children with G6PD deficiency. If patient with G6PD deficiency combines infection, patient's condition is worse, the length of stay is longer.