Objective: To describe the later health status of newborn infants who received extracorporeal membrane oxygenation (ECMO)-for acute respiratory failure in th e era after the UK ECMO trial. Design: Prospective follow u...Objective: To describe the later health status of newborn infants who received extracorporeal membrane oxygenation (ECMO)-for acute respiratory failure in th e era after the UK ECMO trial. Design: Prospective follow up study of newborn in fants who received ECMO at a single centre between January 1997 and January 2001 . Setting: Departments of ECMO and Paediatric Intensive Care, University Hospita ls of Leicester. Patients: All babies who received ECMO within 14 days of birth. Interventions: Neurodevelopment screening using the schedule for growing skills -II (SGS-II) assessment tool. Main outcome measures: Survival at 12 months of age by disease and functional development at follow up. Results: A total of 145 neonates received ECMO for treatment of respiratory failure. Of these, 108 (75% ) were alive at 1 year of age. There were no deaths in children treated for resp iratory failure secondary to meconium aspiration syndrome (73/145). Ninety three (86%of survivors) infants attended a follow up visit at 11-19 months postnata l age. Eighty two were classed as normal, seven as having “impairment”, and fo ur as having “severe disability”. Conclusions: Most newborn infants with acute respiratory failure treated with ECMO will have a normal neurodevelopment scree ning assessment at 11-19 months of postnatal age. There is no evidence to sugge st that changes in neonatal practice since the UK ECMO trial have led to changes in outcome of infants undergoing ECMO therapy.展开更多
Objectives: To compare neurodevelopmental outcomes in severe and moderate congenital hypothyroidism (CH) among 3 different initial L-thyroxine doses and to examine the effect of the time to thyroid function normalizat...Objectives: To compare neurodevelopmental outcomes in severe and moderate congenital hypothyroidism (CH) among 3 different initial L-thyroxine doses and to examine the effect of the time to thyroid function normalization on neurodevelopmental outcomes. Study design: Neurodevelopmental assessments of 31 subjects included the Mullen Scales of Early Learning, Wechsler Preschool and Primary Scale of Intelligence-Revised, Wechsler Intelligence Scale for Children, Wide-Range Achievement Test, and Child Behavioral Checklist. Results: Subjects started on higher initial L-thyroxine doses (50 μg) had full-scale IQ scores 11 points higher than those started on lower (37.5 μg) initial doses. However, verbal IQ, performance IQ, and achievement scores did not differ among the 3 treatment cohorts. Subjects with moderate CH had higher full-scale IQ scores than subjects with severe CH, regardless of the initial treatment dose. Subjects who took longer than 2 weeks to normalize thyroid function had significantly lower cognitive, attention, and achievement scores than those who achieved normal thyroid function at 1 or 2 weeks of therapy. Conclusions: Initial L-thyroxine dose and faster time to normalization of thyroid function are important to optimal neurodevelopmental outcome. In severe CH, it is important to choose an initial dose at the higher end of the recommended range to achieve these goals.展开更多
Background: Better language outcomes are reported for preschool children with hearing impairment (HI) diagnosed very early, irrespective of severity.However, population studies of older children are required to substa...Background: Better language outcomes are reported for preschool children with hearing impairment (HI) diagnosed very early, irrespective of severity.However, population studies of older children are required to substantiate longer term benefits of early detection.Aims: To study impact of age of diagnosis and severity of HI in a population cohort of 7-8 year old children.Methods: Eighty eight 7-8 year old children born in Victoria, who were (a) fitted with hearing aids for congenital HI by 4.5 years and (b) did not have intellectual or major physical disability were studied.Main outcome measures were Clinical Evaluation of Language Fundamentals (CELF) and Peabody Picture Vocabulary Test (PPVT).Predictors were pure tone average (0.5, 1, 2 kHz) in better ear at diagnosis and age at diagnosis.Marginal (adjusted) means were estimated with general linear models.Results: Response rate was 67%(n = 89; 53 boys).Mean age at diagnosis was 21.6 months (SD 14.4); 21%had mild, 34%moderate, 21%severe, and 24%profound HI; mean non-verbal IQ was 104.6 (SD 16.7).Mean total CELF score was 76.7 (SD 21.4) and mean PPVT score 78.1 (SD 18.1).Age of diagnosis, adjusted for severity and IQ, did not contribute to language scores.In contrast, adjusted mean CELF and PPVT language scores fell sequentially with increasing severity of HI.Conclusions: More severe HI, but not later diagnosis, was strongly related to poorer language outcomes at 7-8 years.Further systematic study is needed to understand why children with hearing impairment have good or poor outcomes.展开更多
Abstract Abstract Background: Intraventricular haemorrhage and periventricular leukomalacia are associated with poor outcome of very preterm infants, while the role of more subtle cerebral alterations, as detected by ...Abstract Abstract Background: Intraventricular haemorrhage and periventricular leukomalacia are associated with poor outcome of very preterm infants, while the role of more subtle cerebral alterations, as detected by cranial ultrasound, is less clear. Aim: In this study, we related periventricular echodensities and signs of brain atrophy to neurodevelopmental outcome at 3 y of age. Patients and methods: All preterm infants born in 1997 in our institution with a gestational age < 32 wk or birthweight < 1500 g were subjected to repeated standardized cranial ultrasound examinations until discharge. Survivors were examined at 3 y of age employing the Bayley Scales of Infant Development II. Results: Eighty-seven infants were enrolled (birthweight 430-2500 g (median 1200 g), gestational age 24-34 wk (median 29 wk)). Periventricular echodensities were detected in 42 infants (48%); in 12 cases persisting < 7 d, in 30 cases > 7 d. At discharge, 18 infants (22%) had signs of brain atrophy. Neurodevelopmental outcome was assessed in 64 infants. Infants with signs of brain atrophy scored significantly lower on MDI (atrophy 91.8, no atrophy 101.9; P = 0.02), PDI (atrophy 91.4, no atrophy 106.5; P = 0.001) and Behaviour Rating Scale (atrophy 41.1, no atrophy 66.4; P = 0.01) than infants without atrophy. Periventricular echodensities were not related to outcome. Conclusion: Our data show that infants with sonographic signs of brain atrophy at discharge achieve lower scores in neurodevelopmental testing at 3 y.展开更多
Objective: To compare neurodevelopmental outcome of survivors of the multicent re trial of etamsylate (the iRNN for ethamsylate) for prevention of periventricu lar haemorrhage in very low birthweight infants. Design: ...Objective: To compare neurodevelopmental outcome of survivors of the multicent re trial of etamsylate (the iRNN for ethamsylate) for prevention of periventricu lar haemorrhage in very low birthweight infants. Design: Double blind, single ob server, prospective follow up of placebo controlled study. Setting: Six neonatal intensive care units in the United Kingdom. Neurodevelopmental outcome was asse ssed in health premises or children’s homes. Subjects: 268 of 276 survivors of the original study were seen between 3.5 and 4.2 years of age. All were inborn and weighed 1500 g or less at birth. Intervention: Etamsylate 12.5 mg/kg or pl acebo six hourly from within one hour of delivery for four days. Main outcome me asures: McCarthy scales of children’s abilities, standardised neurological exam ination, full physical examination, functional assessment, seven letter Stycar v ision test, and audiometry. Results: There was no difference between the groups in neuromotor outcome (cerebral palsy) or in the general cognitive index (GCI) o f the McCarthy scales (mean GCI was 93.3 for the etamsylate group (n = 133) and 89.7 for the placebo group (n = 131); p = 0.10). There were more children wit h GCI < 70 (9 v 19; p = 0.047) or ≤50 (3 v 11; p = 0.03) in the placebo group . Fewer children in the etamsylate group had squints (17 v 30; p = 0.042) or re quired surgery for patent ductus arteriosus (1 v 8; p = 0.036). Conclusions: Et amsylate was not associated with a reduction in cerebral palsy. Severe cognitive impairment was reduced, but more children died and the improvement may be becau se fewer survived with low GCI.展开更多
Aim: Homozygous α -thalassaemia, also called haemoglo-bin (Hb) Bart’ s hydrops fetalis, has been thought to be a lethal condition. Due to prenatal diagnosis and intrauterine blood transfusions, a few patients with H...Aim: Homozygous α -thalassaemia, also called haemoglo-bin (Hb) Bart’ s hydrops fetalis, has been thought to be a lethal condition. Due to prenatal diagnosis and intrauterine blood transfusions, a few patients with Hb Bart’ s hydrops fetalis have survived. This fact raises the urgent questions of clinical management and appropriate follow-up of these patients, both of which are addressed in this article. Methods: We report on a 6.5-y-old patient with homozygous α -thalassaemia and review the literature of 13 other survivors published to date. Transfusion requirements were evaluated and the rate of liver iron accumulation was assessed by biomagnetic liver susceptometry before and after institution of iron-chelating therapy. Psychometric evaluation was carried out using Munich’ s Functional Development Test, the Columbia Mental Maturity Scale, the Kaufman Assessment Battery for Children, and the Peabody Picture Vocabulary Test. Results: Our patient had significant delay of psychomotor development. Psychometric evaluation at the age of 5 y revealed an IQ of 85 and an intellectual level of a 4-y-old child. Early tissue iron overload was seen, but a negative iron balance was achieved after institution of desferrioxamine treatment at dosages used for β -thalassaemia. Conclusion: Homozygous α -thalassaemia should no longer be regarded as a lethal condition. Early intervention during pregnancy and careful haematological as well as neuropsychological follow-up was able to provide long-term survival and good life quality in our patient.展开更多
背景:发育性髋关节发育不良常易导致患儿肢体畸形,其诊疗的相关研究已逐渐明确;近来有限元法因其优势在发育性髋关节发育不良的相关研究中受到学者重视。目的:通过文献检索综述有限元法在儿童发育性髋关节发育不良及治疗中的研究进展,...背景:发育性髋关节发育不良常易导致患儿肢体畸形,其诊疗的相关研究已逐渐明确;近来有限元法因其优势在发育性髋关节发育不良的相关研究中受到学者重视。目的:通过文献检索综述有限元法在儿童发育性髋关节发育不良及治疗中的研究进展,分析总结其优势与不足,并探讨未来进一步研究的方向及应用前景。方法:应用计算机在Pub Med、SCI、CBM和中国知网数据库中检索2014年1月至2023年11月发表的相关文献,以“developmental dysplasia(dislocation) of the hip,dysplasia of the hip,finite element analysis(method),pavlik harness,fixation in herringbone position,biomechanics,pelvic osteotomies,pemberton,salter,dega,periacetabular osteotomy,children”为英文检索词,以“发育性髋关节发育不良,发育性髋关节脱位,髋关节发育不良,儿童,有限元,Pavlik吊带,人字型固定,生物力学,骨盆截骨术,髋臼周围截骨术”为中文检索词,同时纳入少量远期文献,通过筛选最终纳入62篇文献进行分析。结果与结论:(1)儿童发育性髋关节发育不良髋关节力学环境异常,髋臼内部压力不均匀、应力增大并集中,关节接触面积减小,股骨颈局部应力集中;(2)在Pavlik吊带及人字型固定位患髋力学环境改善,集中的高应力区域消失,关节接触面积增加,但外展角度过大会导致髋臼及股骨头外侧应力增加;(3)骨盆截骨术治疗后髋关节及骶髂关节应力环境得到改善,3种截骨术没有单一的铰链,其应力负载部位因患儿年龄而存在差异;(4)髋臼周围截骨术治疗后关节接触压力接近正常,而非球形股骨头者恢复困难;(5)术后X射线片表现不能说明关节接触力学达到最佳;(6)提示利用有限元法可以获得体内无法测量的信息,其在虚拟环境中操作不受时间和伦理的限制;能直观地看到正常与发育性髋关节发育不良患者髋关节应力变化的区域,从力学角度说明治疗的有效性,为需要截骨手术治疗的患者建立特定的有限元模型、量身定做手术计划;发育性髋关节发育不良的有限元建模及儿童髋关节材料特性参数尚无规范、统一的标准,由于有限元固有的局限性,目前还不能分析同时包含骨骼、软骨、韧带、肌肉等元素的模型;有限元分析操作难度较大,虽有优势但不具普适性,且目前的研究样本量较少,还需进一步扩大及验证。展开更多
文摘Objective: To describe the later health status of newborn infants who received extracorporeal membrane oxygenation (ECMO)-for acute respiratory failure in th e era after the UK ECMO trial. Design: Prospective follow up study of newborn in fants who received ECMO at a single centre between January 1997 and January 2001 . Setting: Departments of ECMO and Paediatric Intensive Care, University Hospita ls of Leicester. Patients: All babies who received ECMO within 14 days of birth. Interventions: Neurodevelopment screening using the schedule for growing skills -II (SGS-II) assessment tool. Main outcome measures: Survival at 12 months of age by disease and functional development at follow up. Results: A total of 145 neonates received ECMO for treatment of respiratory failure. Of these, 108 (75% ) were alive at 1 year of age. There were no deaths in children treated for resp iratory failure secondary to meconium aspiration syndrome (73/145). Ninety three (86%of survivors) infants attended a follow up visit at 11-19 months postnata l age. Eighty two were classed as normal, seven as having “impairment”, and fo ur as having “severe disability”. Conclusions: Most newborn infants with acute respiratory failure treated with ECMO will have a normal neurodevelopment scree ning assessment at 11-19 months of postnatal age. There is no evidence to sugge st that changes in neonatal practice since the UK ECMO trial have led to changes in outcome of infants undergoing ECMO therapy.
文摘Objectives: To compare neurodevelopmental outcomes in severe and moderate congenital hypothyroidism (CH) among 3 different initial L-thyroxine doses and to examine the effect of the time to thyroid function normalization on neurodevelopmental outcomes. Study design: Neurodevelopmental assessments of 31 subjects included the Mullen Scales of Early Learning, Wechsler Preschool and Primary Scale of Intelligence-Revised, Wechsler Intelligence Scale for Children, Wide-Range Achievement Test, and Child Behavioral Checklist. Results: Subjects started on higher initial L-thyroxine doses (50 μg) had full-scale IQ scores 11 points higher than those started on lower (37.5 μg) initial doses. However, verbal IQ, performance IQ, and achievement scores did not differ among the 3 treatment cohorts. Subjects with moderate CH had higher full-scale IQ scores than subjects with severe CH, regardless of the initial treatment dose. Subjects who took longer than 2 weeks to normalize thyroid function had significantly lower cognitive, attention, and achievement scores than those who achieved normal thyroid function at 1 or 2 weeks of therapy. Conclusions: Initial L-thyroxine dose and faster time to normalization of thyroid function are important to optimal neurodevelopmental outcome. In severe CH, it is important to choose an initial dose at the higher end of the recommended range to achieve these goals.
文摘Background: Better language outcomes are reported for preschool children with hearing impairment (HI) diagnosed very early, irrespective of severity.However, population studies of older children are required to substantiate longer term benefits of early detection.Aims: To study impact of age of diagnosis and severity of HI in a population cohort of 7-8 year old children.Methods: Eighty eight 7-8 year old children born in Victoria, who were (a) fitted with hearing aids for congenital HI by 4.5 years and (b) did not have intellectual or major physical disability were studied.Main outcome measures were Clinical Evaluation of Language Fundamentals (CELF) and Peabody Picture Vocabulary Test (PPVT).Predictors were pure tone average (0.5, 1, 2 kHz) in better ear at diagnosis and age at diagnosis.Marginal (adjusted) means were estimated with general linear models.Results: Response rate was 67%(n = 89; 53 boys).Mean age at diagnosis was 21.6 months (SD 14.4); 21%had mild, 34%moderate, 21%severe, and 24%profound HI; mean non-verbal IQ was 104.6 (SD 16.7).Mean total CELF score was 76.7 (SD 21.4) and mean PPVT score 78.1 (SD 18.1).Age of diagnosis, adjusted for severity and IQ, did not contribute to language scores.In contrast, adjusted mean CELF and PPVT language scores fell sequentially with increasing severity of HI.Conclusions: More severe HI, but not later diagnosis, was strongly related to poorer language outcomes at 7-8 years.Further systematic study is needed to understand why children with hearing impairment have good or poor outcomes.
文摘Abstract Abstract Background: Intraventricular haemorrhage and periventricular leukomalacia are associated with poor outcome of very preterm infants, while the role of more subtle cerebral alterations, as detected by cranial ultrasound, is less clear. Aim: In this study, we related periventricular echodensities and signs of brain atrophy to neurodevelopmental outcome at 3 y of age. Patients and methods: All preterm infants born in 1997 in our institution with a gestational age < 32 wk or birthweight < 1500 g were subjected to repeated standardized cranial ultrasound examinations until discharge. Survivors were examined at 3 y of age employing the Bayley Scales of Infant Development II. Results: Eighty-seven infants were enrolled (birthweight 430-2500 g (median 1200 g), gestational age 24-34 wk (median 29 wk)). Periventricular echodensities were detected in 42 infants (48%); in 12 cases persisting < 7 d, in 30 cases > 7 d. At discharge, 18 infants (22%) had signs of brain atrophy. Neurodevelopmental outcome was assessed in 64 infants. Infants with signs of brain atrophy scored significantly lower on MDI (atrophy 91.8, no atrophy 101.9; P = 0.02), PDI (atrophy 91.4, no atrophy 106.5; P = 0.001) and Behaviour Rating Scale (atrophy 41.1, no atrophy 66.4; P = 0.01) than infants without atrophy. Periventricular echodensities were not related to outcome. Conclusion: Our data show that infants with sonographic signs of brain atrophy at discharge achieve lower scores in neurodevelopmental testing at 3 y.
文摘Objective: To compare neurodevelopmental outcome of survivors of the multicent re trial of etamsylate (the iRNN for ethamsylate) for prevention of periventricu lar haemorrhage in very low birthweight infants. Design: Double blind, single ob server, prospective follow up of placebo controlled study. Setting: Six neonatal intensive care units in the United Kingdom. Neurodevelopmental outcome was asse ssed in health premises or children’s homes. Subjects: 268 of 276 survivors of the original study were seen between 3.5 and 4.2 years of age. All were inborn and weighed 1500 g or less at birth. Intervention: Etamsylate 12.5 mg/kg or pl acebo six hourly from within one hour of delivery for four days. Main outcome me asures: McCarthy scales of children’s abilities, standardised neurological exam ination, full physical examination, functional assessment, seven letter Stycar v ision test, and audiometry. Results: There was no difference between the groups in neuromotor outcome (cerebral palsy) or in the general cognitive index (GCI) o f the McCarthy scales (mean GCI was 93.3 for the etamsylate group (n = 133) and 89.7 for the placebo group (n = 131); p = 0.10). There were more children wit h GCI < 70 (9 v 19; p = 0.047) or ≤50 (3 v 11; p = 0.03) in the placebo group . Fewer children in the etamsylate group had squints (17 v 30; p = 0.042) or re quired surgery for patent ductus arteriosus (1 v 8; p = 0.036). Conclusions: Et amsylate was not associated with a reduction in cerebral palsy. Severe cognitive impairment was reduced, but more children died and the improvement may be becau se fewer survived with low GCI.
文摘Aim: Homozygous α -thalassaemia, also called haemoglo-bin (Hb) Bart’ s hydrops fetalis, has been thought to be a lethal condition. Due to prenatal diagnosis and intrauterine blood transfusions, a few patients with Hb Bart’ s hydrops fetalis have survived. This fact raises the urgent questions of clinical management and appropriate follow-up of these patients, both of which are addressed in this article. Methods: We report on a 6.5-y-old patient with homozygous α -thalassaemia and review the literature of 13 other survivors published to date. Transfusion requirements were evaluated and the rate of liver iron accumulation was assessed by biomagnetic liver susceptometry before and after institution of iron-chelating therapy. Psychometric evaluation was carried out using Munich’ s Functional Development Test, the Columbia Mental Maturity Scale, the Kaufman Assessment Battery for Children, and the Peabody Picture Vocabulary Test. Results: Our patient had significant delay of psychomotor development. Psychometric evaluation at the age of 5 y revealed an IQ of 85 and an intellectual level of a 4-y-old child. Early tissue iron overload was seen, but a negative iron balance was achieved after institution of desferrioxamine treatment at dosages used for β -thalassaemia. Conclusion: Homozygous α -thalassaemia should no longer be regarded as a lethal condition. Early intervention during pregnancy and careful haematological as well as neuropsychological follow-up was able to provide long-term survival and good life quality in our patient.
文摘背景:发育性髋关节发育不良常易导致患儿肢体畸形,其诊疗的相关研究已逐渐明确;近来有限元法因其优势在发育性髋关节发育不良的相关研究中受到学者重视。目的:通过文献检索综述有限元法在儿童发育性髋关节发育不良及治疗中的研究进展,分析总结其优势与不足,并探讨未来进一步研究的方向及应用前景。方法:应用计算机在Pub Med、SCI、CBM和中国知网数据库中检索2014年1月至2023年11月发表的相关文献,以“developmental dysplasia(dislocation) of the hip,dysplasia of the hip,finite element analysis(method),pavlik harness,fixation in herringbone position,biomechanics,pelvic osteotomies,pemberton,salter,dega,periacetabular osteotomy,children”为英文检索词,以“发育性髋关节发育不良,发育性髋关节脱位,髋关节发育不良,儿童,有限元,Pavlik吊带,人字型固定,生物力学,骨盆截骨术,髋臼周围截骨术”为中文检索词,同时纳入少量远期文献,通过筛选最终纳入62篇文献进行分析。结果与结论:(1)儿童发育性髋关节发育不良髋关节力学环境异常,髋臼内部压力不均匀、应力增大并集中,关节接触面积减小,股骨颈局部应力集中;(2)在Pavlik吊带及人字型固定位患髋力学环境改善,集中的高应力区域消失,关节接触面积增加,但外展角度过大会导致髋臼及股骨头外侧应力增加;(3)骨盆截骨术治疗后髋关节及骶髂关节应力环境得到改善,3种截骨术没有单一的铰链,其应力负载部位因患儿年龄而存在差异;(4)髋臼周围截骨术治疗后关节接触压力接近正常,而非球形股骨头者恢复困难;(5)术后X射线片表现不能说明关节接触力学达到最佳;(6)提示利用有限元法可以获得体内无法测量的信息,其在虚拟环境中操作不受时间和伦理的限制;能直观地看到正常与发育性髋关节发育不良患者髋关节应力变化的区域,从力学角度说明治疗的有效性,为需要截骨手术治疗的患者建立特定的有限元模型、量身定做手术计划;发育性髋关节发育不良的有限元建模及儿童髋关节材料特性参数尚无规范、统一的标准,由于有限元固有的局限性,目前还不能分析同时包含骨骼、软骨、韧带、肌肉等元素的模型;有限元分析操作难度较大,虽有优势但不具普适性,且目前的研究样本量较少,还需进一步扩大及验证。
