摘要
总结1例单核细胞减少与分枝杆菌感染综合征患儿的护理体会。针对患儿疾病罕见、移植高风险、预后未知等特点,采取降低有创诊疗风险等级,协助诊断、明确移植需求;居家肺部治疗联合远程康复,寻找最佳移植时机;双学科协同长程随访,优化患者预后等措施。患儿多次住院经反复穿刺活检及病理会诊,提示鸟分枝杆菌感染,PAS(+),DPAS(+),GATA2基因2号外显子插入突变,确诊为单核细胞减少与分枝杆菌感染综合征合并肺泡蛋白沉积症。于2022年10月行造血干细胞移植术,术后继续抗感染治疗。随访至今,间质性病变较移植前明显改善,继续抗排异治疗,现居家学习中。
To summarize the nursing experience of a child with monocytopenia and mycobacterial infection(MonoMAC) syndrome. In response to characteristics such as the rare disease,high-risk of transplantation,and unknown prognosis,measures were taken to reduce the risk level of invasive diagnosis and treatment,to assist in diagnosis and clarify the needs for transplantation. And we implemented home lung therapy combined with remote rehabilitation to find the best time for transplantation,multidisciplinary collaboration for long-term follow-up to optimize the prognosis of patients. After repeated biopsy and pathological consultation,the patient was diagnosed with Mycobacterium avium infection,PAS(+),DPAS(+),GATA2 gene exon 2 insertion mutation,and was diagnosed with MonoMAC syndrome with pulmonary alveolar proteinosis. In October 2022,a hematopoietic stem cell transplantation was performed. Treatment for MAC lung disease continued after the surgery. Follow-up to the present showed that the interstitial lesions have significantly improved compared to that before the transplantation.The patient continued anti-rejection treatment and is currently studying at home.
作者
徐小燕
吕张红
孙赛君
XU Xiaoyan;LüZhanghong;SUN Saijun
出处
《中华急危重症护理杂志》
CSCD
2024年第6期548-551,共4页
Chinese Journal of Emergency and Critical Care Nursing
基金
浙江省医药卫生科技计划项目(2021KY688)。
