1.Assessment of cardiac involvement related to iron overload inβ-thalassemia major
Journal of Clinical Pediatrics 2015;33(5):490-493
Cardiac involvement related to iron overload caused by long-term blood transfusion is a major cause of death for patients with thalassemia major. Patients' survival will be improved greatly if cardiomyopathy resulted from transfusional iron overload could be detected timely and effective iron chelation be initiated promptly. In the present article, various evaluation Methods currently used in clinical practice are reviewed, with particular focus on cardiac MRI for the monitoring of myocardial involvement related to iron overload.
2.Relationship between red blood cell distribution width and prognosis in children with sepsis
Ling JIN ; Xingjiang LONG ; Tong YANG ; Xianwen HUANG
Journal of Clinical Pediatrics 2013;(10):946-948
Objective To explore the relationship of red blood cell distribution width (RDW) with severity and prognosis in children with sepsis. Methods The RDW, clinical features and prognosis of 494 sepsis children were retrospectively ana-lyzed. Results The RDW was increased in 305 sepsis children (61.74%) among whom 49.30%in sepsis group, 60.27%in sever sepsis group and 83.46%in septic shock group. The difference was signiifcant (P<0.001). The mortality of RDW increased children among three groups was 1.89%, 20.45%, 67.57%, and it was signiifcantly different (P<0.001). The rates of increased RDW was 56.15%in 374 survived cases and 79.17%in 120 died cases, and the difference was signiifcant (P<0.001). Pediatric critical illness score (PCIS) was negatively correlated with RDW (P<0.001), while mortality was positively correlated with RDW (P<0.001). Conclusions The rate of increased RDW is high in children with severe sepsis and septic shock and the level of RDW is closely related to the prognosis of patients.
3.Value of serum albumin combined with red blood cell distribution width on evaluation of severity of heart failure in children
Ling JIN ; Xingjiang LONG ; Tong YANG ; Xianwen HUANG
Journal of Clinical Pediatrics 2018;36(5):360-363
Objective To explore the relationship between serum albumin (ALB) combined with red blood cell distribution width (RDW) in progression and prognosis of children with heart failure. Method A total of 144 children with heart failure were divided into light, medium, and heavy group according to the modified ROSS score; another aspect, they also were divided into survival group and death group. ALB, RDW, and modified ROSS score were measured to assess the relationship between the factors and the death by logistic regression analysis. Results There are 73 boys and 71 girls. The cases of mild, moderate, and severe group were 78, 39, and 27, respectively. Age and gender difference in three groups were not statistically significant (P>0.05). The ratio of high RDW was 54.86% (79/144), the ratio of low serum ALB was 33.33% (48/144). Chi-square analysis indicated that a severer status of the disease correlated with a lower ALB level (P <0.05) and a higher RDW. The analysis of rank correlation indicating the degree of heart failure was negatively correlated with the related trends of ALB and RDW (P<0.05). Logistic regression analysis indicating a higher risk of death of the disease correlated with a lower ALB level (P<0.05, OR=0.839)and a higher modified ROSS score(P<0.05,OR=1.324).Conclusions The ALB,RDW,and the modified ROSS score are closely related to the severity in heart failure children. The ALB and the modified ROSS score have important clinical prognostic significance in evaluating the prognosis.
4.Multicenter evaluation of minimal residual disease monitoring in early induction therapy for treatment of childhood acute lymphoblastic leukemia
Xiaojun WU ; Ning LIAO ; Huirong MAI ; Xinyu LI ; Wuqing WAN ; Lihua YANG ; Libin HUANG ; Xiangqin LUO ; Chuan TIAN ; Qiwen CHEN ; Xingjiang LONG ; Yunyan HE ; Ying WANG ; Chi-Kong LI ; Honggui XU
Chinese Journal of Pediatrics 2024;62(4):337-344
Objective:To evaluate the role of minimal residual disease (MRD) monitoring during early induction therapy for the treatment of childhood acute lymphoblastic leukemia (ALL).Methods:This was a multicenter retrospective cohort study. Clinical data of 1 164 ALL patients first diagnosed between October 2016 and June 2019 was collected from 16 hospitals in South China Children′s Leukemia Group. According to MRD assay on day 15 of early induction therapy, they were divided into MRD<0.10% group, MRD 0.10%-<10.00% group and MRD≥10.00% group. According to MRD assay on day 33, they were divided into MRD<0.01% group, MRD 0.01%-<1.00% group and MRD≥1.00% group. Age, onset white blood cell count, central nervous system leukemia (CNSL), molecular genetic characteristics and other data were compared between groups. Kaplan-Meier method was used for survival analysis. Cox regression model was used to analyze prognostic factors.Results:Of the 1 164 enrolled patients, there were 692 males and 472 females. The age of diagnosis was 4.7 (0.5, 17.4) years. The white blood cell count at initial diagnosis was 10.7 (0.4, 1 409.0) ×10 9/L. Among all patients, 53 cases (4.6%) had CNSL. The follow-up time was 47.6 (0.5, 68.8) months. The 5-year overall survival (OS) and 5-year relapse-free survival (RFS) rates were (93.1±0.8) % and (90.3±1.1) %. On day 15 of early induction therapy, there were 466 cases in the MRD<0.10% group, 523 cases in the MRD 0.10%-<10.00% group and 175 cases in the MRD≥10.00% group. The 5-year OS rates of the MRD<0.10% group, MRD 0.10%-<10.00% group and MRD≥10.00% group were (95.4±1.0) %, (93.3±1.1) %, (85.4±2.9) %, respectively, while the RFS rates were (93.2±1.6) %, (90.8±1.4) %, (78.9±4.3) %, respectively ( χ2=16.47, 21.06, both P<0.05). On day 33 of early induction therapy, there were 925 cases in the MRD <0.01% group, 164 cases in the MRD 0.01%-<1.00% group and 59 cases in the MRD≥1.00% group. The 5-year RFS rates in the MRD 0.01%-<1.00% group was lowest among three groups ((91.4±1.2) % vs. (84.5±3.2) % vs. (87.9±5.1) %). The difference between three groups is statistically significant ( χ2=9.11, P=0.010). Among ALL patients with MRD≥10.00% on day 15 of induction therapy, there were 80 cases in the MRD <0.01% group on day 33, 45 cases in the MRD 0.01%-<1.00% group on day 33 and 45 cases in the MRD≥1.00% group on day 33. The 5-year RFS rates of three groups were (83.9±6.0)%, (67.1±8.2)%, (83.3±6.9)% respectively ( χ2=6.90, P=0.032). Univariate analysis was performed in the MRD≥10.00% group on day 15 and the MRD 0.01%-<1.00% group on day 33.The 5-year RFS rate of children with CNSL was significantly lower than that without CNSL in the MRD≥10.00% group on day 15 ((50.0±20.4)% vs. (80.3±4.4)%, χ2=4.13, P=0.042). Patients with CNSL or MLL gene rearrangement in the MRD 0.01%-<1.00% group on day 33 had significant lower 5-year RFS rate compared to those without CNSL or MLL gene rearrangement ((50.0±25.0)% vs. (85.5±3.1)%, χ2=4.06, P=0.044;(58.3±18.6)% vs. (85.7±3.2)%, χ2=9.44, P=0.002). Multivariate analysis showed that age ( OR=0.58, 95% CI 0.35-0.97) and white blood cell count at first diagnosis ( OR=0.43, 95% CI 0.27-0.70) were independent risk factors for OS. The MRD level on day 15 ( OR=0.55,95% CI 0.31-0.97), ETV6-RUNX1 fusion gene ( OR=0.13,95% CI 0.03-0.54), MLL gene rearrangement ( OR=2.55,95% CI 1.18-5.53) and white blood cell count at initial diagnosis ( OR=0.52,95% CI 0.33-0.81) were independent prognostic factors for RFS. Conclusions:The higher the level of MRD in early induction therapy, the worse the OS. The MRD levels on day 15 is an independent prognostic factor for RFS.The MRD in early induction therapy guided accurate risk stratification and individualized treatment can improve the survival rate of pediatric ALL.