neurocognitive disability

  • 文章类型: Journal Article
    背景:父母经常报告说,由于担心未经治疗的术后疼痛,在孩子手术后保留未使用的阿片类药物。评估患有神经认知障碍的青少年的疼痛具有挑战性。我们假设患有神经认知障碍的青少年的父母可能报告阿片类药物的使用较少,阿片类药物的保留率较高。
    方法:青少年(13-20岁)接受择期手术(后路脊柱融合术,髋关节重建,关节镜,扁桃体切除术)于2019年至2020年在三级儿童医院进行了前瞻性登记。仅包括出院时使用阿片类药物的青少年。父母在30天和90天完成了收集社会人口统计学特征的术前调查和两次术后调查。神经认知障碍在入学时由护理人员报告确定,包括患有脑瘫的青少年,严重的自闭症谱系障碍,和伴有严重神经认知障碍的离散综合征。
    结果:在125名父母青少年中,14人患有神经认知障碍。出院时处方阿片类药物的中位数量因神经认知障碍而无差异(29,四分位距{IQR}:20.0-33.3对30,IQR:25.0-40.0,P=0.180)。两组家长报告阿片类药物使用累积天数相似(7.0,IQR:3.0-21.0vs.6.0,IQR:3.0-10.0,P=0.515),阿片类药物使用数量相似(4,IQR:2.0-4.5vs.12,IQR:3.5-22.5,P=0.083)。两组的父母报告的未使用阿片类药物数量相似(17,IQR:12.5-22.5对19,IQR:8.0-29.0,P=0.905)和未使用阿片类药物的保留率(15.4%对23.8%,P=0.730)。
    结论:处方阿片类药物的数量因神经认知障碍而没有差异,父母报告的阿片类药物使用和未使用的阿片类药物保留相似。需要更大规模的研究来确定改善神经认知障碍儿童术后疼痛控制的机会。
    Parents frequently report retaining unused opioid pills following their child\'s surgery due to fear of untreated postoperative pain. Assessment of pain in adolescents with neurocognitive disability is challenging. We hypothesized that parents of adolescents with neurocognitive disability may report less opioid use and higher opioid pill retention.
    Adolescents (13-20 y) undergoing elective surgery (posterior spinal fusion, hip reconstruction, arthroscopy, tonsillectomy) were prospectively enrolled from a tertiary children\'s hospital from 2019 to 2020. Only adolescents prescribed opioids at discharge were included. Parents completed a preoperative survey collecting sociodemographic characteristics and two postoperative surveys at 30- and 90-d. Neurocognitive disability was determined at time of enrollment by caregiver report, and included adolescents with cerebral palsy, severe autism spectrum disorder, and discrete syndromes with severe neurocognitive disability.
    Of 125 parent-adolescent dyads enrolled, 14 had neurocognitive disability. The median number of opioid pills prescribed at discharge did not differ by neurocognitive disability (29, interquartile range {IQR}: 20.0-33.3 versus 30, IQR: 25.0-40.0, P = 0.180). Parents of both groups reported similar cumulative days of opioid use (7.0, IQR: 3.0-21.0 versus 6.0, IQR:3.0-10.0, P = 0.515) and similar number of opioid pills used (4, IQR: 2.0-4.5 versus 12, IQR: 3.5-22.5, P = 0.083). Parents of both groups reported similar numbers of unused opioid pills (17, IQR: 12.5-22.5 versus 19, IQR: 8.0-29.0, P = 0.905) and rates of retention of unused opioids (15.4% versus 23.8%, P = 0.730).
    The number of opioid pills prescribed did not differ by neurocognitive disability and parents reported similar opioid use and retention of unused opioid pills. Larger studies are needed to identify opportunities to improve postoperative pain control for children with neurocognitive disability.
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  • 文章类型: Case Reports
    Schaaf-Yang综合征(SYS)是由MAGEL2基因的致病变异引起的一种罕见的神经发育障碍。通常是产后诊断为肌张力减退和喂养困难的婴儿。没有产前诊断的病例。在怀孕期间,据报道,最常见的发现是羊水过多和胎动减少,这是相对常见和不具体的。我们在出生后诊断为SYS的胎儿中介绍了一例胎儿马蹄内翻足和临床畸形,以及与该综合征相关的产前发现的简要回顾。
    Schaaf-Yang syndrome (SYS) is a rare neurodevelopmental disorder caused by pathogenic variants in the MAGEL2 gene. It is usually a postnatal diagnosis in infants with muscular hypotonia and feeding difficulties. There are no cases diagnosed antenatally. During pregnancy, the most common findings reported are polyhydramnios and decreased fetal movements, which are relatively common and unspecific.We present one case of fetal clubfoot and clinodactyly in a fetus postnatally diagnosed with SYS, as well as a brief review of the prenatal findings associated with this syndrome.
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