ABSTRACT
Objective To evaluate the health-related quality of life (HRQoL) in children with β-thalasse-mia major and their parents,and to analyze its affecting factors.Methods PedsQLTM 4.0 generic core scale and a demographic questionnaire were used to assess HRQoL in 45 children with β-thalassemia major and their parents,which were between 5 and 12 years old,had received blood transfusion in Chengdu Women and Children's Central Hospital during 2016.Results The total summary score of patients' HRQoL was (74.58 ± 7.29) score,in which,the social functioning subscale score was the highest,and school functioning subscale score was the lowest.The total summary score of parents' HRQoL was (64.43 ± 11.54) score,and the difference was statistically significant (P ＜ 0.05).Parents' s core of the psychosocial health [(69.03 ± 12.24) score],the emotional functioning [(67.78 ± 12.67)score]and the School functioning [(57.92 ± 11.61) score]were significantly lower than those of children[(78.19 ±13.42) score,(83.75 ±9.05) score,(69.58 ± 10.30) score],and the differences were statistically significant(all P ＜0.05).The HRQoL of children was positively correlated with onset age of anemia and hemoglobin (Hb) level before transfusion (r =0.771,0.824,all P ＜ 0.01),but which was negatively correlated with iron chelation therapy (r =-0.573,P ＜ 0.01).In contrast,gender,frequency of blood transfusions during the previous 3 months,the type of blood transfusion and iron chelation treatment were not significantly related to HRQoL among these patients.All of these factors were not related to the HRQoL of their parents(P ＞ 0.05).A multiple regression analysis revealed that the HRQoL of children with β-thalassemia major was significantly correlated with onset age of anemia(P =0.005),Hb level before transfusion (P =0.026) and iron chelation therapy (P =0.000).Conclusions The HRQoL of children with β-thalassemia major and their parents were remarkably low.Comprehensive programs including social support,medical service and psychological care should be provided for these children and their parents.
ABSTRACT
Objective To explore the clinical characteristics and prognosis factors of child hemophagocytic syndrome(HPS)in different age groups.Methods Eighty-five children cases of HPS were divided into 3 age groups,0-2 years old (group A),＞2-8 years old (group B) and ＞8-14 years old (group C).The etiology,pathology,clinical symptoms,laboratory detection and prognosis were retrospectively analyzed.Results In the etiology,the etiology in the group A,B and C was dominated by EB virus infection,the infection positive rate in the group C was significantly higher than that in the group B and C;in the bone marrow examination:the occurrence rate of granulocytes to erythrocytes ratio ＜1.0 in the group A was higher than that in the group C;in the clinical symptoms,cervical lymph node enlargement mostly occurred in the group B,hepatosplenomegaly was most rare in the group C,and the serous cavity effusion commonly occurred in the group B;in the laboratory detection,the LDH level in the group B was higher than that in the group A,the above differences were statistically significant(P＜0.05).There were no statistically significant differences in other clinical symptoms and laboratory detection results among the three groups (P＞0.05);In the comparison between the survival group and death group,the serous cavity effusion,triglyceride(TG),CD4/CD8 ratio and granulocytes to erythrocytes ratio in bone marrow had statistical difference(P＜0.05).The above factors were performed the Logistic analysis,the results showed that TG＞3.5 mmol/L,CD4/CD8 ratio ＜1.24 and bone marrow granulocytes to erythrocytes ratio ＜1.02 were the unfavorable risk factors affecting the prognosis of child HPS (P＜0.05).Conclusion Partial clinical characteristics of child HPS are related with the onset age,TG level,CD4/CD8 ratio and bone marrow granulocytes to erythrocytes ratio are the risk factors affecting prognosis.