1.Comparative study on analgesic effect of bupivacaine liposome and ropivacaine on quadratus lumborum block in patients undergoing laparoscopic total hysterectomy
Huan-xi XU ; Xiong-xiong PAN ; Rong MA ; Xu XU ; Xian-rui LI ; Hao WU
Journal of Regional Anatomy and Operative Surgery 2025;34(10):892-896
Objective To clarify the differences in the effects of bupivacaine liposome and ropivacaine on quadratus lumborum block in patients undergoing laparoscopic total hysterectomy.Methods A total of 70 patients who underwent laparoscopic total hysterectomy at Suqian First People's Hospital Affiliated to Nanjing Medical University from January 2023 to October 2024 were selected as the research subjects.The patients were randomly divided into the control group and the observation group,with 35 cases in each group.Before general anesthesia,patients of the control group were given ropivacaine quadratus lumborum block,while patients of the observation group were given bupivacaine liposome quadratus lumborum block.The general conditions before and after operation,visual analogue scale(VAS)scores at different time points after operation,rescue analgesia,mini-mental state examination(MMSE)scores at different time points after operation,postoperative rehabilitation and analgesic adverse reactions were compared between the two groups.Results There was no statistically significant difference in the general conditions of patients in the two groups before and after the operation(P>0.05).At 24 hours,48 hours,and 72 hours after surgery,the abdominal resting VAS scores of patients in the observation group were significantly lower than those in the control group(P<0.05).The time of the first self-controlled use of the intravenous analgesic pump after surgery in the observation group was significantly later than that in the control group(P<0.05).The number of analgesic pump compressions and the rate of oxycodone rescue analgesia in the observation group were significantly fewer/lower than those in the control group within 48 hours after surgery(P<0.05).At 24 hours,48 hours,and 72 hours after surgery,the MMSE scores of patients in the observation group were significantly higher than those in the control group(P<0.05).The time for patients to get out of bed and move around after surgery and the recovery time of anal exhaust in the observation group were significantly earlier than those in the control group(P<0.05).There was no statistically significant difference in the incidence of postoperative analgesic adverse reactions between the two groups of patients(P>0.05).Conclusion Compared with the use of ropivacaine for quadratus lumborum block in patients undergoing laparoscopic total hysterectomy,bupivacaine liposome can provide a longer-lasting analgesic effect,reduce patients'dependence on intravenous analgesia,be conducive to protecting patients'cognitive function,accelerate the recovery of patients without increasing the incidence of adverse reactions.
2.Comparative study on analgesic effect of bupivacaine liposome and ropivacaine on quadratus lumborum block in patients undergoing laparoscopic total hysterectomy
Huan-xi XU ; Xiong-xiong PAN ; Rong MA ; Xu XU ; Xian-rui LI ; Hao WU
Journal of Regional Anatomy and Operative Surgery 2025;34(10):892-896
Objective To clarify the differences in the effects of bupivacaine liposome and ropivacaine on quadratus lumborum block in patients undergoing laparoscopic total hysterectomy.Methods A total of 70 patients who underwent laparoscopic total hysterectomy at Suqian First People's Hospital Affiliated to Nanjing Medical University from January 2023 to October 2024 were selected as the research subjects.The patients were randomly divided into the control group and the observation group,with 35 cases in each group.Before general anesthesia,patients of the control group were given ropivacaine quadratus lumborum block,while patients of the observation group were given bupivacaine liposome quadratus lumborum block.The general conditions before and after operation,visual analogue scale(VAS)scores at different time points after operation,rescue analgesia,mini-mental state examination(MMSE)scores at different time points after operation,postoperative rehabilitation and analgesic adverse reactions were compared between the two groups.Results There was no statistically significant difference in the general conditions of patients in the two groups before and after the operation(P>0.05).At 24 hours,48 hours,and 72 hours after surgery,the abdominal resting VAS scores of patients in the observation group were significantly lower than those in the control group(P<0.05).The time of the first self-controlled use of the intravenous analgesic pump after surgery in the observation group was significantly later than that in the control group(P<0.05).The number of analgesic pump compressions and the rate of oxycodone rescue analgesia in the observation group were significantly fewer/lower than those in the control group within 48 hours after surgery(P<0.05).At 24 hours,48 hours,and 72 hours after surgery,the MMSE scores of patients in the observation group were significantly higher than those in the control group(P<0.05).The time for patients to get out of bed and move around after surgery and the recovery time of anal exhaust in the observation group were significantly earlier than those in the control group(P<0.05).There was no statistically significant difference in the incidence of postoperative analgesic adverse reactions between the two groups of patients(P>0.05).Conclusion Compared with the use of ropivacaine for quadratus lumborum block in patients undergoing laparoscopic total hysterectomy,bupivacaine liposome can provide a longer-lasting analgesic effect,reduce patients'dependence on intravenous analgesia,be conducive to protecting patients'cognitive function,accelerate the recovery of patients without increasing the incidence of adverse reactions.
3.Different methods in predicting mortality of pediatric intensive care units sepsis in Southwest China
Rong LIU ; Zhicai YU ; Changxue XIAO ; Shufang XIAO ; Juan HE ; Yan SHI ; Yuanyuan HUA ; Jimin ZHOU ; Guoying ZHANG ; Tao WANG ; Jianyu JIANG ; Daoxue XIONG ; Yan CHEN ; Hongbo XU ; Hong YUN ; Hui SUN ; Tingting PAN ; Rui WANG ; Shuangmei ZHU ; Dong HUANG ; Yujiang LIU ; Yuhang HU ; Xinrui REN ; Mingfang SHI ; Sizun SONG ; Jumei LUO ; Juan LIU ; Juan ZHANG ; Feng XU
Chinese Journal of Pediatrics 2024;62(3):204-210
Objective:To investigate the value of systemic inflammatory response syndrome (SIRS), pediatric sequential organ failure assessment (pSOFA) and pediatric critical illness score (PCIS) in predicting mortality of pediatric sepsis in pediatric intensive care units (PICU) from Southwest China.Methods:This was a prospective multicenter observational study. A total of 447 children with sepsis admitted to 12 PICU in Southwest China from April 2022 to March 2023 were enrolled. Based on the prognosis, the patients were divided into survival group and non-survival group. The physiological parameters of SIRS, pSOFA and PCIS were recorded and scored within 24 h after PICU admission. The general clinical data and some laboratory results were recorded. The area under the curve (AUC) of the receiver operating characteristic curve was used to compare the predictive value of SIRS, pSOFA and PCIS in mortality of pediatric sepsis.Results:Amongst 447 children with sepsis, 260 patients were male and 187 patients were female, aged 2.5 (0.8, 7.0) years, 405 patients were in the survival group and 42 patients were in the non-survival group. 418 patients (93.5%) met the criteria of SIRS, and 440 patients (98.4%) met the criteria of pSOFA≥2. There was no significant difference in the number of items meeting the SIRS criteria between the survival group and the non-survival group (3(2, 4) vs. 3(3, 4) points, Z=1.30, P=0.192). The pSOFA score of the non-survival group was significantly higher than that of the survival group (9(6, 12) vs. 4(3, 7) points, Z=6.56, P<0.001), and the PCIS score was significantly lower than that of the survival group (72(68, 81) vs. 82(76, 88) points, Z=5.90, P<0.001). The predictive value of pSOFA (AUC=0.82) and PCIS (AUC=0.78) for sepsis mortality was significantly higher than that of SIRS (AUC=0.56) ( Z=6.59, 4.23, both P<0.001). There was no significant difference between pSOFA and PCIS ( Z=1.35, P=0.176). Platelet count, procalcitonin, lactic acid, albumin, creatinine, total bilirubin, activated partial thromboplastin time, prothrombin time and international normalized ratio were all able to predict mortality of sepsis to a certain degree (AUC=0.64, 0.68, 0.80, 0.64, 0.68, 0.60, 0.77, 0.75, 0.76, all P<0.05). Conclusion:Compared with SIRS, both pSOFA and PCIS had better predictive value in the mortality of pediatric sepsis in PICU.
4.Clinical characteristics and pregnancy outcomes of multifetal pregnancy reduction: a 12-year cohort study
Li LI ; Yanyi LI ; Rui YANG ; Mengjie FAN ; Shuo YANG ; Jia LI ; Ying SONG ; Ningning PAN ; Tian TIAN ; Caihong MA ; Rong LI ; Ping LIU ; Jie QIAO
Chinese Journal of Reproduction and Contraception 2024;44(8):784-790
Objective:To analyze the clinical characteristics, pregnancy outcomes and factors affecting live birth of patients undergoing multifetal pregnancy reduction (MFPR), in order to provide reference for clinical strategies.Methods:A retrospective cohort study was conducted on all patients who underwent multifetal pregnancy reduction among polychorionic multifetal pregnancy patients at the Center for Reproductive Medicine, Department of Obstetrics and Gynecology of Peking University Third Hospital during a period of 12 years from January 1, 2009 to December 31, 2020. The overall and annual clinical characteristics were analyzed, pregnancy outcomes were followed up. Patients were divided into live birth group ( n=1 555) and not live birth group ( n=205), and factors affecting live birth were analyzed by multivariate logistic. Through further subgroup analysis, multiple pregnancies were divided into three subgroups: dichorionic diamniotic twin, triplet pregnancy, and four or more high sequence multiple pregnancy. Results:A total of 1 925 patients who underwent MFPR were included, and 1 760 pregnancy outcomes were followed up. In the past 12 years, there had been an increase in dizygotic twins, and the proportion of transabdominal fetal reduction had significantly increased, from 3% in 2009 to 77% in 2020. The annual live birth rate of reduction patients fluctuated between 83% and 94%. The live birth rate of patients with MFPR was related with the type of multiple pregnancies, the method of reducing pregnancies, and the number of retained embryos. The live birth rate of four or more high sequence multiple pregnancies [75.8% (72/95)] was lower than that of dichorionic diamniotic twins [90.0% (796/884), P<0.001], the dichorionic diamniotic twins [89.9% (241/268), P<0.001], the trichorionic triamniotic triplet pregnancy [86.9% (446/513), P=0.005]. The live birth rate of transabdominal fetal reduction [91.4% (655/717)] was higher than that of transvaginal fetal reduction with fetal cardiac activity area injection of KCl [84.9% (304/358), P=0.001], and vaginal embryo aspiration [87.0% (596/685), P=0.009]. There was no statistically significant difference in the live birth rate between vaginal KCl injection and vaginal aspiration ( P=0.351). The survival rate of patients with retained singletons [89.7% (1 062/1 184)] was higher than that of patients with retained twins [85.6% (493/576), P=0.012]. After adjusting for confounding factors such as age, assisted pregnancy method, type of multiple pregnancies, and number of retained embryos, transabdominal fetal reduction was an independent protective factor for live birth rate ( P=0.040, OR=1.604, 95% CI: 1.021-2.519). Conclusion:With the change of transplantation strategy, the proportion of dichorionic diamniotic twins increased, and the proportion of transabdominal fetal reduction increased, which pregnancy outcomes might be better. There was no difference in pregnancy outcomes between those who underwent vaginal aspiration and transvaginal fetal reduction with fetal cardiac activity area injection of KCl. The outcomes of four or more high sequence multiple pregnancies were poor, and it was necessary to strictly control the number of embryo transfers and optimize ovulation promotion plans in clinical practice.
5.Clinical characteristics and pregnancy outcomes of multifetal pregnancy reduction: a 12-year cohort study
Li LI ; Yanyi LI ; Rui YANG ; Mengjie FAN ; Shuo YANG ; Jia LI ; Ying SONG ; Ningning PAN ; Tian TIAN ; Caihong MA ; Rong LI ; Ping LIU ; Jie QIAO
Chinese Journal of Reproduction and Contraception 2024;44(8):784-790
Objective:To analyze the clinical characteristics, pregnancy outcomes and factors affecting live birth of patients undergoing multifetal pregnancy reduction (MFPR), in order to provide reference for clinical strategies.Methods:A retrospective cohort study was conducted on all patients who underwent multifetal pregnancy reduction among polychorionic multifetal pregnancy patients at the Center for Reproductive Medicine, Department of Obstetrics and Gynecology of Peking University Third Hospital during a period of 12 years from January 1, 2009 to December 31, 2020. The overall and annual clinical characteristics were analyzed, pregnancy outcomes were followed up. Patients were divided into live birth group ( n=1 555) and not live birth group ( n=205), and factors affecting live birth were analyzed by multivariate logistic. Through further subgroup analysis, multiple pregnancies were divided into three subgroups: dichorionic diamniotic twin, triplet pregnancy, and four or more high sequence multiple pregnancy. Results:A total of 1 925 patients who underwent MFPR were included, and 1 760 pregnancy outcomes were followed up. In the past 12 years, there had been an increase in dizygotic twins, and the proportion of transabdominal fetal reduction had significantly increased, from 3% in 2009 to 77% in 2020. The annual live birth rate of reduction patients fluctuated between 83% and 94%. The live birth rate of patients with MFPR was related with the type of multiple pregnancies, the method of reducing pregnancies, and the number of retained embryos. The live birth rate of four or more high sequence multiple pregnancies [75.8% (72/95)] was lower than that of dichorionic diamniotic twins [90.0% (796/884), P<0.001], the dichorionic diamniotic twins [89.9% (241/268), P<0.001], the trichorionic triamniotic triplet pregnancy [86.9% (446/513), P=0.005]. The live birth rate of transabdominal fetal reduction [91.4% (655/717)] was higher than that of transvaginal fetal reduction with fetal cardiac activity area injection of KCl [84.9% (304/358), P=0.001], and vaginal embryo aspiration [87.0% (596/685), P=0.009]. There was no statistically significant difference in the live birth rate between vaginal KCl injection and vaginal aspiration ( P=0.351). The survival rate of patients with retained singletons [89.7% (1 062/1 184)] was higher than that of patients with retained twins [85.6% (493/576), P=0.012]. After adjusting for confounding factors such as age, assisted pregnancy method, type of multiple pregnancies, and number of retained embryos, transabdominal fetal reduction was an independent protective factor for live birth rate ( P=0.040, OR=1.604, 95% CI: 1.021-2.519). Conclusion:With the change of transplantation strategy, the proportion of dichorionic diamniotic twins increased, and the proportion of transabdominal fetal reduction increased, which pregnancy outcomes might be better. There was no difference in pregnancy outcomes between those who underwent vaginal aspiration and transvaginal fetal reduction with fetal cardiac activity area injection of KCl. The outcomes of four or more high sequence multiple pregnancies were poor, and it was necessary to strictly control the number of embryo transfers and optimize ovulation promotion plans in clinical practice.
6. Effect of total flavonoids of Rosa rugosa on PI3K/AKT pathway and endoplasmic reticulum stress apoptosis in rats with cerebral ischemia-reperfusion injury
Jing-Rong ZHANG ; Xiu-Shi YU ; Rui-Juan GAO ; Liang-Jing-Yuan KONG ; Pan-Xi SUN ; Chong-Yang ZHANG ; Li-Li WEI ; Jun-Qiang SI ; Li LI
Chinese Pharmacological Bulletin 2023;39(5):896-902
Aim To investigate the effects of total flavonoids from Rosa rugosa (TFR) on cerebral ischemia reperfusion injury (CIRI) in rats, and to investigate whether TFR inhibited neuronal apoptosis by regulating phosphoinositide 3-kinase/protein kinase B (PI3K/AKT) signaling pathway and endoplasmic reticulum stress (ERS) pathways. Methods SD rats were randomly divided into sham operation group, model group, low-dose group (50 mg · kg
7.Analysis of clinical outcome of whole frozen-thawed embryo transfer in patients with high ovarian response using GnRH-agonist trigger or combined with low-dose hCG trigger in GnRH-antagonist protocol
Binbin TU ; Ningning PAN ; Lixue CHEN ; Jin HUANG ; Min LI ; Rui YANG ; Ping LIU ; Rong LI ; Jie QIAO
Chinese Journal of Reproduction and Contraception 2023;43(7):683-689
Objective:To compare the clinical outcome of whole frozen-thawed embryo transfer in patients with high ovarian response using gonadotropin-releasing hormone agonist (GnRH-a) single trigger or combined with low-dose human chorionic gonadotropin (hCG) dual trigger in the gonadotropin-releasing hormone antagonist (GnRH-A) protocol.Methods:Retrospective cohort study was conducted in Center for Reproductive Medicine, Department of Obstetrics and Gynecology of Peking University Third Hospital between January 2018 to December 2020, and the patients with high ovarian response undergoing in vitro fertilization/intracytoplasmic sperm injection (IVF/ICSI) were divided into GnRH-a single trigger group (group A, n=251) and GnRH-a combined with low-dose hCG dual trigger group (group B, n=741) according to the trigger protocol. The clinical outcome was compared between the two groups. Results:There were no significant differences in clinical characteristics between group A and group B. Estrogen level on the day of trigger, number of embryos formed, number of two pronuclei (2PN) embryos formed, number of high-quality embryos formed and rate of embryo formation in group A were significantly higher than those in group B [(22 905.84±9 513.28) pmol/L vs. (17 200.82±6 811.64) pmol/L, P<0.001; 18.84±9.50 vs. 17.34±8.04, P=0.025; 16.06±8.24 vs. 14.87±7.07, P=0.042; 11.25±6.92 vs. 10.25±5.97, P=0.027; 68.6% (4 730/6 899) vs. 66.5% (12 852/19 326), P=0.002]. However, there were no significant differences in number of oocytes retrieved, mature oocyte rate and high-quality embryo rate between group A and group B (all P>0.05). There were also no significant differences in hCG positive rate, clinical pregnancy rate, abortion rate, ectopic pregnancy rate, persistent pregnancy rate and live birth rate per frozen-thawed embryo transfer between the two groups (all P>0.05). There were no significant differences in cumulative persistent pregnancy rate, cumulative live birth rate, proportion of low birth weight infants and birth defects per ovarian stimulation cycle between the two groups (all P>0.05). The incidence of early-onset severe ovarian hyperstimulation syndrome (OHSS) in group A was lower than that in group B, but there was no significant difference [0% vs. 1.5% (11/741), P=0.075]. Conclusion:The pregnancy outcome of whole frozen-thawed embryo transfer in patients with high ovarian response using GnRH-a single trigger was comparable to dual trigger combined with low-dose hCG in the GnRH-A protocol, and the risk of early onset severe OHSS was low. Therefore, in patients with high ovarian response in IVF/ICSI cycle using GnRH-A protocol, the use of GnRH-a single trigger followed by whole frozen-thawed embryo transfer could be considered as an optimal choice to minimize the risk of early onset OHSS without loss of pregnancy outcome.
8.Analysis of clinical outcome of whole frozen-thawed embryo transfer in patients with high ovarian response using GnRH-agonist trigger or combined with low-dose hCG trigger in GnRH-antagonist protocol
Binbin TU ; Ningning PAN ; Lixue CHEN ; Jin HUANG ; Min LI ; Rui YANG ; Ping LIU ; Rong LI ; Jie QIAO
Chinese Journal of Reproduction and Contraception 2023;43(7):683-689
Objective:To compare the clinical outcome of whole frozen-thawed embryo transfer in patients with high ovarian response using gonadotropin-releasing hormone agonist (GnRH-a) single trigger or combined with low-dose human chorionic gonadotropin (hCG) dual trigger in the gonadotropin-releasing hormone antagonist (GnRH-A) protocol.Methods:Retrospective cohort study was conducted in Center for Reproductive Medicine, Department of Obstetrics and Gynecology of Peking University Third Hospital between January 2018 to December 2020, and the patients with high ovarian response undergoing in vitro fertilization/intracytoplasmic sperm injection (IVF/ICSI) were divided into GnRH-a single trigger group (group A, n=251) and GnRH-a combined with low-dose hCG dual trigger group (group B, n=741) according to the trigger protocol. The clinical outcome was compared between the two groups. Results:There were no significant differences in clinical characteristics between group A and group B. Estrogen level on the day of trigger, number of embryos formed, number of two pronuclei (2PN) embryos formed, number of high-quality embryos formed and rate of embryo formation in group A were significantly higher than those in group B [(22 905.84±9 513.28) pmol/L vs. (17 200.82±6 811.64) pmol/L, P<0.001; 18.84±9.50 vs. 17.34±8.04, P=0.025; 16.06±8.24 vs. 14.87±7.07, P=0.042; 11.25±6.92 vs. 10.25±5.97, P=0.027; 68.6% (4 730/6 899) vs. 66.5% (12 852/19 326), P=0.002]. However, there were no significant differences in number of oocytes retrieved, mature oocyte rate and high-quality embryo rate between group A and group B (all P>0.05). There were also no significant differences in hCG positive rate, clinical pregnancy rate, abortion rate, ectopic pregnancy rate, persistent pregnancy rate and live birth rate per frozen-thawed embryo transfer between the two groups (all P>0.05). There were no significant differences in cumulative persistent pregnancy rate, cumulative live birth rate, proportion of low birth weight infants and birth defects per ovarian stimulation cycle between the two groups (all P>0.05). The incidence of early-onset severe ovarian hyperstimulation syndrome (OHSS) in group A was lower than that in group B, but there was no significant difference [0% vs. 1.5% (11/741), P=0.075]. Conclusion:The pregnancy outcome of whole frozen-thawed embryo transfer in patients with high ovarian response using GnRH-a single trigger was comparable to dual trigger combined with low-dose hCG in the GnRH-A protocol, and the risk of early onset severe OHSS was low. Therefore, in patients with high ovarian response in IVF/ICSI cycle using GnRH-A protocol, the use of GnRH-a single trigger followed by whole frozen-thawed embryo transfer could be considered as an optimal choice to minimize the risk of early onset OHSS without loss of pregnancy outcome.
9.Clinical effect of fetal reduction by intracranial and intrathoracic KCl injection.
Hong Xia ZHANG ; Rui YANG ; Shuo YANG ; Ning Ning PAN ; Lin Lin WANG ; Rong LI
Journal of Peking University(Health Sciences) 2022;54(5):943-947
OBJECTIVE:
To evaluate the feasibility and effectiveness of fetal reduction by transabdominal intracranial KCl injection for multifetal pregnancies in the early second trimester.
METHODS:
The data of 363 patients who underwent transabdominal fetal reduction in the Reproductive Medical Center of Peking University Third Hospital from January 2006 to December 2019 were analyzed retrospectively. According to the location of fetal reduction, they were divided into two groups: Intracranial injection group (n=196) and intrathoracic injection group (n=167). The process of fetal reduction and pregnancy outcome of the two groups were compared.
RESULTS:
There was no significant difference between the two groups in the average age and the proportion of type of infertility before assisted reproductive technology, conception method, indication for fetal reduction, starting number of fetuses, reduced number of fetuses, and finishing number of fetuses (P>0.05). There was no significant difference between the two groups in the proportion of the number of puncture ≥ 2 times (12.1% vs. 8.6%, P=0.249) and the incidence of replacing puncture site (10.7% vs. 6.4%, P=0.161). The next day after fetal reduction, color Doppler ultrasound was rechecked. In the intracranial injection group and intrathoracic injection group, the incidence of fetal heartbeat recovery [3.6% (8/224) vs. 1.1% (2/187), P=0.188], the volumes of KCl used [(2.6±1.0) mL vs. (2.8±1.1) mL, P=0.079], and the abortion rate within 4 weeks after fetal reduction (1.0% vs. 0.6%, P=0.654) were of no significant difference. In addition, there was no significant difference in the total abortion rate after fetal reduction, premature delivery rate, cesarean section rate, delivery gestational week and neonatal birth weight between the two groups (P>0.05).
CONCLUSION
Intracranial KCl injection can be an effective alternative to intrathoracic KCl injection for multifetal pregancy reduction.
Cesarean Section
;
Female
;
Humans
;
Infant, Newborn
;
Pregnancy
;
Pregnancy Outcome
;
Pregnancy Reduction, Multifetal/methods*
;
Pregnancy Trimester, Second
;
Retrospective Studies
10.Clinical characteristics of Wilson's disease with onset of acute liver failure in 19 children.
Pan LIU ; Chang SHU ; Li TANG ; Xi LIU ; Xiao Kang PENG ; Rui Na LI ; Ya Rong LI ; Xiao Guai LIU
Chinese Journal of Pediatrics 2022;60(5):452-456
Objective: To analyze the clinical characteristics of Wilson's disease (WD) with onset of acute liver failure (ALF) in children. Methods: Clinical data of 19 children diagnosed with WD presented with ALF in Xi'an Children's Hospital from January 2016 to April 2021 were retrospectively analyzed, including general condition, clinical manifestation, laboratory examination, and gene detection. The children were divided into the death group and survival group according to the clinical outcome. The children who had hepatic WD with non-ALF onset during the same period were selected as the control. The general conditions and laboratory indexes were compared between death group and survival group, ALF group and non-ALF group. T-test, Mann Whitney U test or χ2 test were used to compare the differences between the two groups. Results: Of the 19 WD children with ALF onset, 10 were females and 9 were males. The age of admission was (10.1±2.6) years and time to onset of first visit was 9 (4, 15) days. Among the WD children with ALF onset, 4 children were lost to follow-up, 5 cases death (death group) and 10 cases survived (survival group). The ceruloplasmin in the death group was higher than that in the survival group (0.078 (0.055, 0.105) vs. 0.033 (0.027, 0.058) g/L, Z=-2.33, P=0.020). There were 95 children who had hepatic WD with non-ALF onset. The WD patients with ALF onset were older at admission (9.9 (8.0, 11.1) vs. 5.4 (3.7, 6.9) years, Z=-5.25, P<0.001), had higher ceruloplasmin (0.060 (0.030, 0.078) vs. 0.024 (0.006, 0.060) g/L, Z=-3.11, P=0.002), 24 h urinary copper (674 (205, 1 803) vs. 149 (108, 206) μg, Z=-4.25, P<0.001), and positive rate of K-F ring [17/19 vs. 7%(7/95), χ2=50.17, P<0.001] while shorter onset time at initial visit (0.3 (0.1, 0.5) vs. 1.0 (0.7, 6.0) months, Z=-4.28, P<0.001). There was no gender difference between the two groups [9/19 vs. 61%(58/95), χ2=1.22, P=0.269]. Of the 19 WD children with ALF onset, 13 had the ATP7B gene tested, and 15 reported variants were detected. The main variations were c.2333G>T (p. Arg778Leu), c.2621C>T (p. Ala874Val) and c.2975C>T (p. Pro992Leu). The allele frequencies were 6/26(23%), 4/26(15%) and 3/26(12%), respectively. Conclusions: Children of WD onset with ALF are school-aged and above. They have an acute onset, a short course of the disease, and poor prognosis. The positive rate of K-F ring, ceruloplasmin and urinary copper are higher than those of the hepatic WD children with non-ALF onset.
Ceruloplasmin/metabolism*
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Child
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Copper/metabolism*
;
Female
;
Hepatolenticular Degeneration/genetics*
;
Humans
;
Liver Failure, Acute/therapy*
;
Male
;
Retrospective Studies

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