Importance:Emicizumab(EMI)is efficacious and safe for hemophilia A(HA)prophylaxis.However,its high cost poses a challenge in China.Objective:To explore the possibility of using reduced-dosage EMI in Chinese HA childre...Importance:Emicizumab(EMI)is efficacious and safe for hemophilia A(HA)prophylaxis.However,its high cost poses a challenge in China.Objective:To explore the possibility of using reduced-dosage EMI in Chinese HA children.Methods:We conducted a retrospective study for HA children in our Comprehensive Care Center.Data were collected pre-and post-EMI treatment to evaluate bleeding rates.Laboratory analyses included factor VIII(FVIII)-like activity and EMI concentration measurements.Results:Thirty-four HA children receiving EMI prophylaxis for a median(range)24.5(2.5–47.9)months by June 2023.Of these,25(73.5%)were under 3 years of age,26(76.5%)had severe hemophilia and 12(35.3%)were minimally treated or previously untreated patients.Thirty-one(91.2%)of the 34 patients received reduced-dosage EMI for economic reasons.EMI concentration and FVIII-like activity measured showed a strong correlation.Overall,while on EMI,their annual treated bleeding rate(ATBR)and annual bleeding rate(ABR)decreased significantly(2–0)while their zero-bleeding rate(ZBR)increased significantly(11.5%–65.4%).After 6 months of EMI,there was no significant difference in ATBR and ABR among various maintenance dosages.However,ZBR was significantly lower in dosages under 4 mg/kg(P=0.0156).Receiver operator characteristic curves suggested the following cutoff values for zero bleeding:EMI 4-weekly maintenance dosage 3.8 mg/kg,EMI concentration 48.1µg/mL,and FVIII-like activity 15.4 IU/dL.Interpretation:We showed EMI effectively prevented bleeding even at reduced dosages.However,the bleeding risk may be higher with EMI 4-weekly maintenance dosage<3.8 mg/kg,EMI concentration<48.1µg/mL,and FVIII-like activity<15.4 IU/dL for zero bleeding.It is important that dosage reduction be done rationally.Dosage tailoring is possible.展开更多
Background and Objective Patients with glioma experience a high symptom burden and have diverse palliative care needs.However,the assessment scales used in palliative care remain non-standardized and highly heterogene...Background and Objective Patients with glioma experience a high symptom burden and have diverse palliative care needs.However,the assessment scales used in palliative care remain non-standardized and highly heterogeneous.To evaluate the application patterns of the current scales used in palliative care for glioma,we aim to identify gaps and assess the need for disease-specific scales in glioma palliative care.Methods We conducted a systematic search of five databases including PubMed,Web of Science,Medline,EMBASE,and CINAHL for quantitative studies that reported scale-based assessments in glioma palliative care.We extracted data on scale characteristics,domains,frequency,and psychometric properties.Quality assessments were performed using the Cochrane ROB 2.0 and ROBINS-I tools.Results Of the 3,405 records initially identified,72 studies were included.These studies contained 75 distinct scales that were used 193 times.Mood(21.7%),quality of life(24.4%),and supportive care needs(5.2%)assessments were the most frequently assessed items,exceeding half of all scale applications.Among the various assessment dimensions,the Distress Thermometer(DT)was the most frequently used tool for assessing mood,while the Short Form-36 Health Survey Questionnaire(SF-36)was the most frequently used tool for assessing quality of life.The Mini Mental Status Examination(MMSE)was the most common tool for cognitive assessment.Performance status(5.2%)and social support(6.8%)were underrepresented.Only three brain tumor-specific scales were identified.Caregiver-focused scales were limited and predominantly burden-oriented.Conclusions There are significant heterogeneity,domain imbalances,and validation gaps in the current use of assessment scales for patients with glioma receiving palliative care.The scale selected for use should be comprehensive and user-friendly.展开更多
Objective To explore and quantify the association of hot night exposure during the sperm development period(0–90 lag days) with semen quality.Methods A total of 6,640 male sperm donors from 6 human sperm banks in Chi...Objective To explore and quantify the association of hot night exposure during the sperm development period(0–90 lag days) with semen quality.Methods A total of 6,640 male sperm donors from 6 human sperm banks in China during 2014–2020were recruited in this multicenter study.Two indices(i.e.,hot night excess [HNE] and hot night duration[HND]) were used to estimate the heat intensity and duration during nighttime.Linear mixed models were used to examine the association between hot nights and semen quality parameters.Results The exposure-response relationship revealed that HNE and HND during 0–90 days before semen collection had a significantly inverse association with sperm motility.Specifically,a 1 ℃ increase in HNE was associated with decreased sperm progressive motility of 0.0090(95% confidence interval[CI]:–0.0147,–0.0033) and decreased total motility of 0.0094(95% CI:–0.0160,–0.0029).HND was significantly associated with reduced sperm progressive motility and total motility of 0.0021(95% CI:–0.0040,–0.0003) and 0.0023(95% CI:–0.0043,–0.0002),respectively.Consistent results were observed at different temperature thresholds on hot nights.Conclusion Our findings highlight the need to mitigate nocturnal heat exposure during spermatogenesis to maintain optimal semen quality.展开更多
Objectives:To investigate the parenting stress,care burden,and coping styles in mothers of autistic children during the pandemic of COVID-19.The outbreak of COVID-19 and the closure of education and care centers for t...Objectives:To investigate the parenting stress,care burden,and coping styles in mothers of autistic children during the pandemic of COVID-19.The outbreak of COVID-19 and the closure of education and care centers for these children may affect stress,care burden,and adaptation of these mothers.Methods:This study was a cross-sectional research.A total of 110 mothers completed questionnaires.Data were analyzed by descriptive statistics(frequency,percentage,mean,and standard deviation),independent t-test,ANOVA,and multiple linear regressions.Results:Findings showed that parenting stress has a strong and direct correlation with caring burden(P<0.001,r=0.95),and a strong and indirect correlation with coping styles(P<0.001,r=−0.91).Variables of caring burden,coping strategies,mother’s age,mother’s job,mother’s education,number of autistic children,economic status,children’s age,and functional level of autism in children can predict 72.21%of the variance in parenting stress in these mothers.Conclusions:In the present study,parenting stress of mothers of autistic children was reported to be high during the COVID-19 pandemic.Based on the findings of this study,it can be concluded that pediatric nurses and health policymakers should provide a suitable educational and supportive environment for mothers of autistic children to enhance the coping level of these mothers and consequently reduce their parenting stress and care burden.展开更多
Glioblastoma multiforme(GBM)is the most common malignant primary brain tumor with a poor prognosis and limited survival.Patients with GBM have a high demand for palliative care.In our present case,a 21-year-old female...Glioblastoma multiforme(GBM)is the most common malignant primary brain tumor with a poor prognosis and limited survival.Patients with GBM have a high demand for palliative care.In our present case,a 21-year-old female GBM patient received inpatient palliative care services including symptom management,mental and psychological support for the patient,psychosocial and clinical decision support for her family members,and pre-and post-death bereavement management for the family.Furthermore,we provided the family members with comprehensive psychological preparation for the patient's demise and assisted the patient's family throughout the mourning period.The aim of this study is to provide a reference and insights for the clinical implementation of palliative care for patients with malignant brain tumors.展开更多
目的:探讨俯卧位通气对体外膜氧合(ECMO)支持患者的应用效果。方法:系统检索中国知网、万方数据库、中国生物医学文献数据库、PubMed、Embase、Cochrane图书馆、Web of Science等数据库自建库至2024年10月关于俯卧位通气治疗ECMO支持患...目的:探讨俯卧位通气对体外膜氧合(ECMO)支持患者的应用效果。方法:系统检索中国知网、万方数据库、中国生物医学文献数据库、PubMed、Embase、Cochrane图书馆、Web of Science等数据库自建库至2024年10月关于俯卧位通气治疗ECMO支持患者的所有研究,由2名研究者独立进行文献筛选、资料提取和文献质量评价,采用RevMan 5.4软件对数据进行Meta分析。结果:共纳入10篇文献,包括2项随机对照研究和8项队列研究;包含1513例患者,其中俯卧位通气组674例、仰卧位通气组839例。对6项研究的分析结果显示,与仰卧位通气相比,俯卧位通气可提高ECMO成功撤机率(OR=1.47,95%CI:1.07~2.01,P=0.02)。对8项研究的分析结果显示,与仰卧位通气相比,俯卧位通气可延长ECMO治疗时长[均数差(MD)=4.86 d,95%CI:0.95~8.77,P=0.01]。对6项研究的分析结果显示,俯卧位通气组的重症监护病房(ICU)住院时长较仰卧位通气组显著延长(MD=5.16 d,95%CI:1.08~9.25,P=0.01)。对5项研究的分析结果显示,俯卧位通气组的总住院时长明显长于仰卧位通气组(MD=7.72 d,95%CI:2.10~13.34,P<0.01)。对6项研究的分析结果显示,与仰卧位通气相比,俯卧位通气可延长机械通气时长(MD=6.06 d,95%CI:0.63~11.49,P=0.03)。俯卧位通气在提高患者生存率方面并无明显优势。结论:俯卧位通气有助于提高ECMO成功撤机率,延长ECMO治疗时长及机械通气时长,但对患者的生存率无明显影响。由于本Meta分析纳入研究的样本量普遍较小,需更大样本量的研究来确认俯卧位通气对ECMO支持患者的实际疗效。展开更多
Helsmoortel-Van der Aa综合征(HVDAS)是一种罕见的神经发育障碍性疾病,主要由活性依赖性神经保护蛋白(ADNP)基因突变引起,是最常见的孤独症谱系障碍(ASD)单基因病因之一。本文分析2024年就诊于海口市妇幼保健院的1例患儿,男,3岁10个月...Helsmoortel-Van der Aa综合征(HVDAS)是一种罕见的神经发育障碍性疾病,主要由活性依赖性神经保护蛋白(ADNP)基因突变引起,是最常见的孤独症谱系障碍(ASD)单基因病因之一。本文分析2024年就诊于海口市妇幼保健院的1例患儿,男,3岁10个月,因“运动、语言发育迟缓2年余,社交障碍1年余”入院,检查发现患儿存在孤独症谱系障碍、全面发育迟缓、特殊面容、矮小、脑积水等临床表现,基因检测发现患儿携带ADNP基因杂合突变{NM_001282531.3(ADNP):c.2189(exon6)delG[p.(Arg730Glnfs3)]},其父母及妹妹均未携带该突变。总结临床资料并结合文献复习,ADNP基因在染色质重塑和神经发育障碍中发挥重要作用,ADNP基因突变可累及多系统。此例HVDAS具有典型临床表现,并拓宽了脑积水的表型谱,为此类基因变异的遗传咨询以及临床医师的早期识别提供了参考依据。展开更多
基金National Natural Science Foundation of China,Grant/Award Number:82270133
文摘Importance:Emicizumab(EMI)is efficacious and safe for hemophilia A(HA)prophylaxis.However,its high cost poses a challenge in China.Objective:To explore the possibility of using reduced-dosage EMI in Chinese HA children.Methods:We conducted a retrospective study for HA children in our Comprehensive Care Center.Data were collected pre-and post-EMI treatment to evaluate bleeding rates.Laboratory analyses included factor VIII(FVIII)-like activity and EMI concentration measurements.Results:Thirty-four HA children receiving EMI prophylaxis for a median(range)24.5(2.5–47.9)months by June 2023.Of these,25(73.5%)were under 3 years of age,26(76.5%)had severe hemophilia and 12(35.3%)were minimally treated or previously untreated patients.Thirty-one(91.2%)of the 34 patients received reduced-dosage EMI for economic reasons.EMI concentration and FVIII-like activity measured showed a strong correlation.Overall,while on EMI,their annual treated bleeding rate(ATBR)and annual bleeding rate(ABR)decreased significantly(2–0)while their zero-bleeding rate(ZBR)increased significantly(11.5%–65.4%).After 6 months of EMI,there was no significant difference in ATBR and ABR among various maintenance dosages.However,ZBR was significantly lower in dosages under 4 mg/kg(P=0.0156).Receiver operator characteristic curves suggested the following cutoff values for zero bleeding:EMI 4-weekly maintenance dosage 3.8 mg/kg,EMI concentration 48.1µg/mL,and FVIII-like activity 15.4 IU/dL.Interpretation:We showed EMI effectively prevented bleeding even at reduced dosages.However,the bleeding risk may be higher with EMI 4-weekly maintenance dosage<3.8 mg/kg,EMI concentration<48.1µg/mL,and FVIII-like activity<15.4 IU/dL for zero bleeding.It is important that dosage reduction be done rationally.Dosage tailoring is possible.
文摘Background and Objective Patients with glioma experience a high symptom burden and have diverse palliative care needs.However,the assessment scales used in palliative care remain non-standardized and highly heterogeneous.To evaluate the application patterns of the current scales used in palliative care for glioma,we aim to identify gaps and assess the need for disease-specific scales in glioma palliative care.Methods We conducted a systematic search of five databases including PubMed,Web of Science,Medline,EMBASE,and CINAHL for quantitative studies that reported scale-based assessments in glioma palliative care.We extracted data on scale characteristics,domains,frequency,and psychometric properties.Quality assessments were performed using the Cochrane ROB 2.0 and ROBINS-I tools.Results Of the 3,405 records initially identified,72 studies were included.These studies contained 75 distinct scales that were used 193 times.Mood(21.7%),quality of life(24.4%),and supportive care needs(5.2%)assessments were the most frequently assessed items,exceeding half of all scale applications.Among the various assessment dimensions,the Distress Thermometer(DT)was the most frequently used tool for assessing mood,while the Short Form-36 Health Survey Questionnaire(SF-36)was the most frequently used tool for assessing quality of life.The Mini Mental Status Examination(MMSE)was the most common tool for cognitive assessment.Performance status(5.2%)and social support(6.8%)were underrepresented.Only three brain tumor-specific scales were identified.Caregiver-focused scales were limited and predominantly burden-oriented.Conclusions There are significant heterogeneity,domain imbalances,and validation gaps in the current use of assessment scales for patients with glioma receiving palliative care.The scale selected for use should be comprehensive and user-friendly.
基金funded by the Guangdong Provincial Natural Science Foundation of China(No.2022A1515011517).
文摘Objective To explore and quantify the association of hot night exposure during the sperm development period(0–90 lag days) with semen quality.Methods A total of 6,640 male sperm donors from 6 human sperm banks in China during 2014–2020were recruited in this multicenter study.Two indices(i.e.,hot night excess [HNE] and hot night duration[HND]) were used to estimate the heat intensity and duration during nighttime.Linear mixed models were used to examine the association between hot nights and semen quality parameters.Results The exposure-response relationship revealed that HNE and HND during 0–90 days before semen collection had a significantly inverse association with sperm motility.Specifically,a 1 ℃ increase in HNE was associated with decreased sperm progressive motility of 0.0090(95% confidence interval[CI]:–0.0147,–0.0033) and decreased total motility of 0.0094(95% CI:–0.0160,–0.0029).HND was significantly associated with reduced sperm progressive motility and total motility of 0.0021(95% CI:–0.0040,–0.0003) and 0.0023(95% CI:–0.0043,–0.0002),respectively.Consistent results were observed at different temperature thresholds on hot nights.Conclusion Our findings highlight the need to mitigate nocturnal heat exposure during spermatogenesis to maintain optimal semen quality.
基金supported by Hamadan University of Medical Sciences supported financially this research(No.140005123994).
文摘Objectives:To investigate the parenting stress,care burden,and coping styles in mothers of autistic children during the pandemic of COVID-19.The outbreak of COVID-19 and the closure of education and care centers for these children may affect stress,care burden,and adaptation of these mothers.Methods:This study was a cross-sectional research.A total of 110 mothers completed questionnaires.Data were analyzed by descriptive statistics(frequency,percentage,mean,and standard deviation),independent t-test,ANOVA,and multiple linear regressions.Results:Findings showed that parenting stress has a strong and direct correlation with caring burden(P<0.001,r=0.95),and a strong and indirect correlation with coping styles(P<0.001,r=−0.91).Variables of caring burden,coping strategies,mother’s age,mother’s job,mother’s education,number of autistic children,economic status,children’s age,and functional level of autism in children can predict 72.21%of the variance in parenting stress in these mothers.Conclusions:In the present study,parenting stress of mothers of autistic children was reported to be high during the COVID-19 pandemic.Based on the findings of this study,it can be concluded that pediatric nurses and health policymakers should provide a suitable educational and supportive environment for mothers of autistic children to enhance the coping level of these mothers and consequently reduce their parenting stress and care burden.
基金National High Level Hospital Clinical Research Funding(2022-PUMCH-B-113).
文摘Glioblastoma multiforme(GBM)is the most common malignant primary brain tumor with a poor prognosis and limited survival.Patients with GBM have a high demand for palliative care.In our present case,a 21-year-old female GBM patient received inpatient palliative care services including symptom management,mental and psychological support for the patient,psychosocial and clinical decision support for her family members,and pre-and post-death bereavement management for the family.Furthermore,we provided the family members with comprehensive psychological preparation for the patient's demise and assisted the patient's family throughout the mourning period.The aim of this study is to provide a reference and insights for the clinical implementation of palliative care for patients with malignant brain tumors.
文摘Helsmoortel-Van der Aa综合征(HVDAS)是一种罕见的神经发育障碍性疾病,主要由活性依赖性神经保护蛋白(ADNP)基因突变引起,是最常见的孤独症谱系障碍(ASD)单基因病因之一。本文分析2024年就诊于海口市妇幼保健院的1例患儿,男,3岁10个月,因“运动、语言发育迟缓2年余,社交障碍1年余”入院,检查发现患儿存在孤独症谱系障碍、全面发育迟缓、特殊面容、矮小、脑积水等临床表现,基因检测发现患儿携带ADNP基因杂合突变{NM_001282531.3(ADNP):c.2189(exon6)delG[p.(Arg730Glnfs3)]},其父母及妹妹均未携带该突变。总结临床资料并结合文献复习,ADNP基因在染色质重塑和神经发育障碍中发挥重要作用,ADNP基因突变可累及多系统。此例HVDAS具有典型临床表现,并拓宽了脑积水的表型谱,为此类基因变异的遗传咨询以及临床医师的早期识别提供了参考依据。
