Zhao Wang1, Si-Han Liu1, Li-Jin Bo1, Yi-Zhou Zheng1, Jun Shi1, Yan Xu1, Xue Sun1, Yu-Ping Zhao2. 1. Institute of Hematology and Blood Disease Hospital，Chinese Academy of Medical Sciences & Peking Union Medical College，Tianjin，300020，China. 2. Institute of Hematology and Blood Disease Hospital，Chinese Academy of Medical Sciences & Peking Union Medical College，Tianjin，300020，China.E-mail：zhaguinv@163.com.
Abstract
OBJECTIVE: To explore clinical features and laboratory data of glucose-6-phosphate dehydrogenase（G6PD）deficiency and to investigate the relationship between them. METHODS: Clinical data of 43 patients with G6PD deficiency was analyzed, the statistical method was applied to investigate the relationship between clinical features and laboratory data. RESULTS: Among 43 patients，neonatal jaundice occurred as the first symptom in 10 cases，while acute hemolytic anemia occurred as the first symptom in 23 cases. The major clinical symptoms of G6PD deficiency included icteric skin and/or sclera，dark urine，fever，gastrointestinal symptoms，fatigue and lethargy. Symptoms of 26 patients were caused by obvious inducement，including fava beans（61.5%），infection（34.6%）and miocardial infarction（3.8%）. All of 43 patients showed decreased G6PD activity，while the level of their indirect serum bilirubin（IBIL）was positively correlated with reticulocyte percentage（Ret%，r=0.5881，P=0.013） and mean corpuscular volume（MCV，r=0.6854，P=0.0024）. Patients with neonatal jaundice as the first symptom，showed higher level of Ret%（P<0.01）and MCV（P<0.001）and low RBC count（P<0.01）and low Hb level（P<0.01）. as compard with patients with acute hemolytic anemia as first symptome. CONCLUSION: Neonatal jaundice and acute hemolytic anemia are common clinical features of G6PD deficiency. Laboratory results of IBIL，Ret% and MCV have auxiliary value to evaluate the severity of hemolysis induced by G6PD deficiency. Patients with neonatal jaundice as their first symptom show more severe hemolysis than those only suffered from acute hemolytic anemia.
OBJECTIVE: To explore clinical features and laboratory data of glucose-6-phosphate dehydrogenase（G6PD）deficiency and to investigate the relationship between them. METHODS: Clinical data of 43 patients with G6PD deficiency was analyzed, the statistical method was applied to investigate the relationship between clinical features and laboratory data. RESULTS: Among 43 patients，neonatal jaundice occurred as the first symptom in 10 cases，while acute hemolytic anemia occurred as the first symptom in 23 cases. The major clinical symptoms of G6PD deficiency included icteric skin and/or sclera，dark urine，fever，gastrointestinal symptoms，fatigue and lethargy. Symptoms of 26 patients were caused by obvious inducement，including fava beans（61.5%），infection（34.6%）and miocardial infarction（3.8%）. All of 43 patients showed decreased G6PD activity，while the level of their indirect serum bilirubin（IBIL）was positively correlated with reticulocyte percentage（Ret%，r=0.5881，P=0.013） and mean corpuscular volume（MCV，r=0.6854，P=0.0024）. Patients with neonatal jaundice as the first symptom，showed higher level of Ret%（P<0.01）and MCV（P<0.001）and low RBC count（P<0.01）and low Hb level（P<0.01）. as compard with patients with acute hemolytic anemia as first symptome. CONCLUSION:Neonatal jaundice and acute hemolytic anemia are common clinical features of G6PD deficiency. Laboratory results of IBIL，Ret% and MCV have auxiliary value to evaluate the severity of hemolysis induced by G6PD deficiency. Patients with neonatal jaundice as their first symptom show more severe hemolysis than those only suffered from acute hemolytic anemia.