1例Ⅲ型戈谢病伴重度脊柱后凸畸形患儿的围手术期护理

doi:10.3761/j.issn.0254-1769.2022.06.014

摘要/Abstract

摘要：

总结1例Ⅲ型戈谢病伴重度脊柱后凸畸形患儿的围手术期护理。护理要点包括：术前多学科协作诊治、进行充分的术前准备、预防神经功能损伤;术后实施个体化的快速康复方案、麻痹性肠梗阻的护理、感染的预防、安全管理、患儿及其家长的心理护理、出院宣教及延续性护理等。经过积极的诊治和精心的护理,患儿18 d后出院,随访1年状况良好,已恢复正常的学习和生活。

关键词: 戈谢病, 脊柱后凸, 围手术期护理, 儿科护理学

Abstract:

The perioperative nursing care of a child with type Ⅲ gaucher disease with severe kyphosis is summarized. Nursing points include preoperative multidisciplinary collaborative diagnosis and treatment,adequate preoperative preparation,prevention of neurological damage,implementation of individualized rapid plans,postoperative care for paralytic intestinal obstruction,infection prevention,safety management,and psychological health of children and their families. After active treatment and meticulous care by the medical team,the child was discharged 18 days later. After 1 year of following up,he is in good condition and has returned to the normal school and family life.

Key words: Gaucher Disease, Kyphosis, Perioperative Care, Pediatric Nursing

余群飞, 张玉萍, 任英, 许国萍, 马姚静, 赵腾飞. 1例Ⅲ型戈谢病伴重度脊柱后凸畸形患儿的围手术期护理[J]. 中华护理杂志, 2022, 57(6): 736-739.

YU Qunfei, ZHANG Yuping, REN Ying, XU Guoping, MA Yaojing, ZHAO Tengfei. Perioperative nursing care of a child with type Ⅲ gaucher disease with severe kyphosis[J]. Chinese Journal of Nursing, 2022, 57(6): 736-739.

参考文献 17

[1]	Nguyen Y, Stirnemann J, Belmatoug N. La maladie de Gaucher:quand y penser?[J]. La Revue De Médecine Interne, 2019, 40(5):313-322. DOI URL
[2]	唐湘凤, 卢伟, 井远方, 等. 非血缘脐血或单倍体来源的造血干细胞移植治疗戈谢病的临床研究[J]. 中国小儿血液与肿瘤杂志, 2020, 25(4):195-199.
	Tang XF, Lu W, Jing YF, et al. Clinical study of allogeneic hematopoietic cell transplantation from unrelated cord blood or unmanipulated haploidentical donors for Gaucher disease[J]. J China Pediatr Blood Cancer, 2020, 25(4):195-199.
[3]	国家卫生健康委员会,科学技术部,工业和信息化部, 等. 关于公布第一批罕见病目录的通知[J]. 卫生健康委员会公报, 2018(5):15-19.
	National Health Commission,Ministry of Science and Technology,Ministry of Industry and Information Technology, et al. Circular of publishing the first list of rare diseases[J]. Gazette Natl Heal Comm People’s Repubulic China, 2018(5):15-19.
[4]	韩冰, 陈苗, 杨辰. 戈谢病多学科诊疗专家共识(2020)[J]. 协和医学杂志, 2020, 11(6):682-697.
	Han B, Chen M, Yang C. Consensus of gaucher disease by experts from multidisciplinary team(2020)[J]. Med J Peking Union Med Coll Hosp, 2020, 11(6):682-697.
[5]	Mehta A, Kuter DJ, Salek SS, et al. Presenting signs and patient co-variables in Gaucher disease:outcome of the Gaucher earlier diagnosis consensus(GED-C) Delphi initiative[J]. Intern Med J, 2019, 49(5):578-591. DOI PMID
[6]	Ito K, Kawakami N, Tsuji T, et al. Corrective surgery for kyphosis in a case of Gaucher’s disease without history of vertebral compression fractures[J]. Spine Surg Relat Res, 2017, 1(4):222-224. DOI URL
[7]	朱红梅, 诸纪华, 郑智慧, 等. 加速康复外科理念在围手术期患儿中的应用进展[J]. 中华护理杂志, 2021, 56(4):628-634.
	Zhu HM, Zhu JH, Zheng ZH, et al. New progress in the application of the enhanced recovery after surgery during the perioperative period of pediatric surgery[J]. Chin J Nurs, 2021, 56(4):628-634.
[8]	吴孟娇, 张燕迪, 杨改清, 等. 术中神经电生理监测技术的应用进展[J]. 现代电生理学杂志, 2020, 27(1):36-44.
	Wu MJ, Zhang YD, Yang GQ, et al. Progress of intraoperative neuroelectrophysiological monitoring[J]. J Mod Electrophysiol, 2020, 27(1):36-44.
[9]	刘永胜, 王树杰, 赵宇. 经颅电刺激运动诱发电位在胸椎管狭窄症手术中的应用价值[J]. 中华骨与关节外科杂志, 2018, 11(1):35-39.
	Liu YS, Wang SJ, Zhao Y. Application of intraoperative motor evoked potentials during the surgery for thoracic spinal stenosis[J]. Chin J Bone Jo Surg, 2018, 11(1):35-39.
[10]	王文轩, 程鹏哲, 王媛媛. MRI图像结合JOA评分对椎间孔镜髓核摘除术治疗腰椎间盘突出症的术后评估[J]. 中华养生保健, 2020, 38(2):84-85.
	Wang WX, Cheng PZ, Wang YY. Postoperative evaluation of MRI images combined with JOA score for the treatment of lumbar disc herniation with foraminal endoscopic nucleus pulposus[J]. Chin Heal Care, 2020, 38(2):84-85.
[11]	蔡立柏, 刘延锦, 李英, 等. 多学科协作干预方案在全膝关节置换术后恐动症患者中的应用研究[J]. 中华护理杂志, 2020, 55(4):494-499.
	Cai LB, Liu YJ, Li Y, et al. The effects of multidisciplinary teamwork intervention for total knee arthroplasty patients with kinesiophobia[J]. Chin J Nurs, 2020, 55(4):494-499.
[12]	张燕, 杨旭, 陈亚萍, 等. 脊柱侧凸矫形术患儿术后恶心呕吐发生现状及其影响因素分析[J]. 中华现代护理杂志, 2019, 25(36):4734-4739.
	Zhang Y, Yang X, Chen YP, et al. Postoperative nausea and vomiting in scoliosis children and its influencing factors[J]. Chin J Mod Nurs, 2019, 25(36):4734-4739.
[13]	Fleshner PR, Aufses AH, Grabowski GA, et al. A 27-year experience with splenectomy for Gaucher’s disease[J]. Am J Surg, 1991, 161(1):69-75. PMID
[14]	陈丽丽. 微波多功能治疗仪在外科术后伤口护理中的应用效果[J]. 医疗装备, 2020, 33(12):167-168.
	Chen LL. The application effect of microwave multifunctional therapeutic instrument in postoperative wound care[J]. Med Equip, 2020, 33(12):167-168.
[15]	Hughes D, Mikosch P, Belmatoug N, et al. Gaucher disease in bone:from pathophysiology to practice[J]. J Bone Miner Res, 2019, 34(6):996-1013. DOI URL
[16]	朱海英, 刘楠, 曾辉. Humpty Dumpty儿童跌落风险评估量表敏感度和特异度的评价[J]. 中国实用护理杂志, 2019(12):924-928.
	Zhu HY, Liu N, Zeng H. The analysis of sensitivity and specificity of Humpty Dumpty Pediatric Fall Assessment Tool for Hospitalized Children[J]. Chin J Practi Nurs, 2019(12):924-928.
[17]	莫志强, 王大勇, 李小松, 等. 脾切除及保留副脾治疗儿童戈谢病的疗效观察[J]. 中华小儿外科杂志, 2016, 37(7):537-540.
	Mo ZQ, Wang DY, Li XS, et al. Curative effect of splenectomy and preserving accessory spleen for children with Gaucher’s disease[J]. J Chin Ped Surg, 2016, 37(7):537-540.