总结了1例丙酸血症合并癫痫持续状态患儿的护理体会。护理要点：针对本案例起病急、进展快的特点,及时采集标本,加强对患儿病情的动态观察。采取癫痫发作时的急救护理;做好营养筛查和评估,正确摄入蛋白质,维持水、电解质、酸碱平衡;高氨血症脑病的早期观察和预防;低血糖的观察和预防;指导家庭紧急处理。经过8 d的积极治疗和精心护理,患者病情得到控制,予以出院。电话随访2年,家长了解疾病相关知识,患儿病情稳定。
The nursing experience of a child with propionic acidemia complicated with status epilepticus was summarized. According to the characteristics of the urgent onset and rapid progress of this case,specimens were collected in time to strengthen the dynamic observation of the child's condition. Key points of nursing also included emergency care for epileptic seizures;nutrition screening and assessment,proper protein consume and maintenance of water,electrolyte and acid-base balance;early observation and prevention of hyperammonemia encephalopathy;observation and prevention of hypoglycemia;guide of family emergency management. After 8 days of active treatment and careful nursing,the patient's condition was controlled and discharged. The parents were followed up by telephone for 2 years,and the child's condition was stable.
[1] 陈占玲,温鹏强,王国兵,等. 丙酸血症患儿PCCA和PCCB基因突变分析[J]. 中华医学遗传学杂志,2015,32(1):26-30.
Chen ZL,Wen PQ,Wang GB,et al.Analysis of PCCA and PCCB gene mutations in patients with propionic acidemia[J]. Chin J Med Genet,2015,32(1):26-30.
[2] 中华医学会神经病学分会神经重症协作组. 惊厥性癫痫持续状态监护与治疗(成人)中国专家共识[J]. 中国现代神经疾病杂志,2015,15(11):844-851.
Cooperative Group of Severe Neurological Diseases of Neurology Branch of Chinese Medical Association. Expert consensus of care and treatment for convulsive status epilepticus in China(adults)[J]. Chin J Contemp Neurol Neurosurg,2015,15(11):844-851.
[3] 刘兆娥,韩波,孙正芸,等. 伴代谢危象的有机酸血症患儿53例临床分析[J]. 中国小儿急救医学,2015,22(12):857-860.
Liu ZE,Han B,Sun ZY,et al.Clinical features of infantile organic acidemia accompanied with acute metabolic crisis[J]. Chin Pediatr Emerg Med,2015,22(12):857-860.
[4] 曾妃,沈婷芳,陈晨. 丙酸血症患儿行活体肝移植术后的护理经验[J]. 护理与康复,2021,20(8):63-65.
Zeng F,Shen TF,Chen C.Nursing experience of children with propionic acidemia after living donor liver transplantation[J]. J Nurs Rehabil,2021,20(8):63-65.
[5] 陈晓飞,陈朔辉,李美,等. 1例先天性簇绒肠病患儿的营养管理[J]. 中华护理杂志,2022,57(12):1512-1516.
Chen XF,Chen SH,Li M,et al.Nutritional management of a child with congenital tufted bowel disease[J]. Chin J Nurs,2022,57(12):1512-1516.
[6] 李东燕,陈晓春,戴小英. 婴幼儿丙酸血症5例的护理[J]. 护理与康复,2014,13(2):124-125.
Li DY,Chen XC,Dai XY.Nursing of 5 cases of infant propionic acidemia[J]. J Nurs Rehabil,2014,13(2):124-125.
