背景:我们知道综合症或严重的慢性疾病可能与可能干扰睡眠的症状有关,显著影响儿童和照顾者的生活质量。治疗失眠的常用药物,比如褪黑激素,苯二氮卓类药物,尼拉嗪,和抗组胺药,通常无效或与不良反应有关,需要新的治疗观点。右美托咪定是一种选择性α-2激动剂,具有催眠和抗焦虑作用,which,通过刺激蓝斑中的α-2肾上腺素能受体,诱导与非REM阶段的第2-3阶段相当的睡眠,而在镇静期间基本上不影响呼吸驱动。在儿科重症监护或程序镇静文献中已经广泛描述了其使用。2018年,意大利药品管理局(AgenziaItalianaDelFarmacoAIFA)授权在接受姑息治疗的儿童的重症监护之外使用右美托咪定,以控制与病理和难治性睡眠障碍相关的痛苦症状,文献报道了儿童在家服用右美托咪定的病例。
目的:我们的研究旨在描述右美托咪定在失眠或顽固性肌张力障碍儿童中的家庭使用。
方法:我们通过对12个意大利儿科姑息治疗中心的问卷调查,对家庭使用右美托咪定治疗睡眠障碍和顽固性肌张力障碍进行了回顾性分析。
结果:我们收集了一组9名在家接受右美托咪定治疗的儿童,8通过鼻内和1通过静脉途径。所有儿童在专门入院期间在医院或临终关怀医院接受了第一次药物治疗,密切监测生命体征参数72小时(3天,范围2-7天)。放电后,该药物的潜在副作用已向患者家属解释,and,一旦获得知情同意,右美托咪定的家庭给药继续进行,由姑息治疗小组跟进。在家里,右美托咪定给药3000天(至少1个月,最长36个月)。第一个病人治疗了1095天,从2019年到2021年(因潜在疾病相关死亡而停产)。所有患者都观察到治疗对症状的持续益处,他们没有因药物相关的不良反应或认为缺乏治疗效果而停止右美托咪定给药.
结论:因此,在家庭中使用它可能是治疗儿科姑息治疗儿童顽固性睡眠障碍或肌张力障碍的一种有希望的方法。需要进一步的研究来证实我们的结果。
We know that syndromic conditions and severe chronic diseases can be associated with symptoms that may interfere with sleep, significantly impacting the life quality of children and caregivers. Drugs commonly used in treating insomnia, such as melatonin, benzodiazepines, niaprazine, and antihistamines, are often either ineffective or associated with adverse effects, requiring new therapeutic perspectives. Dexmedetomidine is a selective alpha-2 agonist with hypnotic and anxiolytic effects, which, by stimulating alpha-2 adrenergic receptors in the locus coeruleus, induces sleep comparable to stages 2-3 of the non-REM phase without substantially affecting the respiratory drive during sedation. Its use has already been extensively described in pediatric intensive care or procedural sedation literature. In 2018, the Italian Medicines Agency (Agenzia Italiana Del Farmaco AIFA) authorized the off-label use of dexmedetomidine outside of intensive care in Children undergoing palliative treatment to control distressing symptoms related to pathology and refractory sleep disorders, and the literature reported cases of children who received dexmedetomidine at home.
Our study aims to describe the home use of dexmedetomidine in children with insomnia or intractable dystonic states.
We conducted a retrospective analysis through a questionnaire addressed to 12 Italian pediatric palliative care centers regarding the home use of dexmedetomidine in sleep disorders and intractable dystonic states.
We collected a case series of 9 children treated with dexmedetomidine at home, 8 via intranasal and 1 via intravenous route. All children received the first drug administration in the hospital or hospice during a dedicated admission, under close monitoring of vital signs parameters for 72 hours (3 days, range 2-7 days). After discharge, the potential side effects of the drug were explained to the patient\'s families, and, once informed consent was obtained, the home administration of dexmedetomidine continued, with follow-up by the palliative care team. At home, dexmedetomidine was administered for 3000 days (minimum 1 month, maximum 36 months). The first patient was treated for 1095 days, from 2019 to 2021 (discontinued due to underlying condition-related death).
All patients observed a persistent benefit from the treatment on symptoms, and none of them discontinued dexmedetomidine administration due to drug-related adverse effects or perceived lack of therapeutic efficacy.
Therefore, its use at home may represent a promising therapeutic approach for intractable sleep disorders or dystonic states in pediatric palliative care children. Further studies are needed to confirm our results.