Postoperative nursing care of 6 children with classic maple syrup urine disease undergoing liver transplantation

doi:10.3761/j.issn.0254-1769.2022.01.012

Abstract

Abstract:

This article summarized the postoperative nursing experience of 6 children with classic maple syrup urine disease undergoing liver transplantation. Key points of nursing care included：precisely take immunosuppressants to prevent acute rejection;early rehabilitation exercises to promote the development of the nervous system;closely observe changes in the condition and do emergency care for gastrointestinal bleeding;structured skin care to reduce diaper dermatitis;provide multiple forms of support to relieve family pressure;implementation of the "Internet +" follow-up model,continuous family rehabilitation care. After careful treatment and care,the 6 children recovered and were discharged after surgery. They were followed up for 15 days to 4 months and recovered well up to now.

Key words: Maple Syrup Urine Disease, Liver Transplantation, Pediatric Nursing, Perioperative Nursing

摘要：

总结6例经典型枫糖尿病患儿行肝移植的术后护理经验。护理要点包括：精确服用免疫抑制剂,预防急性排异反应;早期康复功能锻炼,促进神经系统发育;密切观察病情变化,做好消化道出血的急救护理;实施结构化皮肤护理,减轻尿布性皮炎症状;提供多形式支持,缓解家属压力;实施"互联网+"随访模式,持续家庭康复护理。经过精心的治疗与护理,6例患儿术后康复出院,随访15 d~4个月,恢复效果良好。

关键词: 枫糖尿病, 肝移植, 儿科护理学, 围手术期护理

WANG Fang, LU Fangyan, WANG Yan. Postoperative nursing care of 6 children with classic maple syrup urine disease undergoing liver transplantation[J]. Chinese Journal of Nursing, 2022, 57(1): 79-82.

王芳, 卢芳燕, 王燕. 6例经典型枫糖尿病患儿行肝移植的术后护理[J]. 中华护理杂志, 2022, 57(1): 79-82.

References 17

[1]	Rodan LH, Aldubayan SH, Berry GT, et al. Acute illness protocol for maple syrup urine disease[J]. Pediatr Emerg Care, 2018, 34(1):64-67. DOI URL
[2]	Abiri M, Karamzadeh R, Mojbafan M, et al. In silico analysis of novel mutations in maple syrup urine disease patients from Iran[J]. Metab Brain Dis, 2017, 32(1):105-113. DOI URL
[3]	田礼, 贺颖, 焦春雷, 等. 胆总管囊肿术后并发晚发枫糖尿症一例并文献复习[J]. 中华小儿外科杂志, 2021, 42(1):34-38.
	Tian L, He Y, Jiao CL, et al. Diagnosis and treatment of late-onset maple syrup urine disease after choledochal cyst excision[J]. Chin J Pediatr Surg, 2021, 42(1):34-38.
[4]	宋东坡, 李文杰. 遗传代谢病枫糖尿症研究进展[J]. 中国儿童保健杂志, 2017, 25(2):143-146.
	Song DP, Li WJ. Research on metabolic disorder of maple syrup urine disease[J]. Chin J Child Heal Care, 2017, 25(2):143-146.
[5]	杨彩飞, 陈涛, 雷小光, 等. 枫糖尿病的研究进展[J]. 中华医学遗传学杂志, 2019(7):737-741.
	Yang CF, Chen T, Lei XG, et al. Progress of research on maple syrup disease[J]. Chin J Med Genet, 2019(7):737-741.
[6]	Uaariyapanichkul J, Saengpanit P, Damrongphol P, et al. Skin le-sions associated with nutritional management of maple syrup urine disease[J]. Case Rep Dermatol Med, 2017, 2017:3905658.
[7]	Handoom B, Megdad E, Al-Qasabi D, et al. The effects of low protein products availability on growth parameters and meta-bolic control in selected amino acid metabolism disorders patients[J]. Int J Pediatr Adolesc Med, 2018, 5(2):60-68. DOI URL
[8]	Herden U, Grabhorn E, Santer R, et al. Surgical aspects of liver transplantation and domino liver transplantation in maple syrup urine disease:analysis of 15 donor-recipient pairs[J]. Liver Tra-nspl, 2019, 25(6):889-900.
[9]	Roda KMO, Vincenzi R, Fonseca EA, et al. Domino liver transplant in maple syrup urine disease:technical details of cases in which the first surgery involved a living donor[J]. Transplan-tation, 2019, 103(3):536-543.
[10]	Rodríguez-Perálvarez M, Guerrero-Misas M, Thorburn D, et al. Maintenance immunosuppression for adults undergoing liver transplantation:a network meta-analysis[J]. Cochrane Database Syst Rev, 2017, 3:CD011639.
[11]	中华医学会器官移植学分会. 中国肝移植术后随访技术规范(2019版)[J/CD]. 中华移植杂志(电子版), 2019, 13(4):278-280.
	Branch of Organ Transplantation of Chinese Medical Association. Technical specification for postoperative follow-up after liver transplantation in China(2019 edition)[J/CD]. Chin J Transplant (Electronic Version), 2019, 13(4):278-280.
[12]	刘经飘, 曾秋月, 梁腊梅. 重力喂养与常规鼻饲在早产儿喂养中的应用[J]. 护理实践与研究, 2014, 11(6):3-4.
	Liu JP, Zeng QY, Liang LM. Comparison analysis of clinical outcome of gravity feeding and routine nasogastric feeding for premature infant’s feeding condition[J]. Nurs Pract Res, 2014, 11(6):3-4.
[13]	Carecchio M, Schneider SA, Chan HD, et al. Movement disorders in adult surviving patients with maple syrup urine disease[J]. Mov Disord, 2011, 26(7):1324-1328. DOI URL
[14]	顾赛霞. 1例新生儿枫糖尿病的护理[J]. 全科护理, 2017, 15(31):3961-3963.
	Gu SX. Nursing care of a neonate with maple syrup urine disease[J]. Chin Gen Pract Nurs, 2017, 15(31):3961-3963.
[15]	Kristensen IH, Kronborg H. What are the effects of supporting early parenting by enhancing parents’ understanding of the infant? Study protocol for a cluster-randomized community-based trial of the newborn behavioral observation(NBO) me-thod[J]. BMC Public Health, 2018, 18(1):832. DOI PMID
[16]	Hanouneh IA, Confer BD, Zein NN, et al. Recurrent upper gas-trointestinal bleeding masquerading as gastric variceal bleeding on endoscopy after liver transplantation[J]. Liver Transpl, 2013, 19(8):926-928. DOI URL
[17]	张琳琪, 李杨, 宋楠, 等. 婴幼儿尿布性皮炎护理实践专家共识[J]. 中华护理杂志, 2020, 55(8):1169.
	Zhang LQ, Li Y, Song N, et al. Expert consensus on diaper dermatitis nursing care for infants and toddlers[J]. Chin J Nurs, 2020, 55(8):1169.