Shmueli Moshe, Ling Galina, Elamour Siham, Weisel Yaron, Ben-Shimol Shalom
Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva 84101, Israel.
The Pediatric Day-Care Unit, Soroka University Medical Center, Beer-Sheva 84101, Israel.
J Clin Med. 2025 Jul 24;14(15):5258. doi: 10.3390/jcm14155258.
: Congenital insensitivity to pain with anhidrosis (CIPA) is a rare genetic disorder, often leading to injuries and serious infections. In 2018, we established a multidisciplinary clinic (MDC) to provide structured, proactive care. We assessed the MDC's impact on hospitalizations, surgeries, and infection rates. : A retrospective study of genetically confirmed CIPA patients, treated from 2014 to 2024. Data from electronic medical records were compared between the pre-MDC (2014-2017) and post-MDC (2018-2024) periods. The core MDC team includes an infectious disease specialist, orthopedic surgeon, and nurses. The patients are stratified according to their carriage of resistant organisms and are managed using strict infection control measures. Follow-ups are scheduled routinely or as needed. Treatment is guided by clinical findings and culture results. : A total of 59 patients were included in the study. The baseline age did not differ significantly between the two periods. Hospitalization rates declined by 30.7% (from 57.7 to 40.0 per 1000 days), and clinic visits decreased by 42.9% (25.5 to 14.6). Overall surgical rates remained stable (2.8 to 2.7), with a 61.9% decrease in eye surgeries and a 130.5% increase in elective tooth extractions. Infection rates increased by 52% (from 6.6 to 10.1 per 1000 days). : The implementation of the MDC bundle led to reduced hospitalizations, clinic visits, and eye surgeries, alongside the increased use of elective tooth extractions and culture testing. Closer monitoring and early infection management contributed to fewer severe complications. These findings support the value of structured, proactive multidisciplinary care in improving outcomes for children with CIPA.
先天性无痛觉伴无汗症（CIPA）是一种罕见的遗传性疾病，常导致受伤和严重感染。2018年，我们设立了一个多学科诊所（MDC），以提供系统化的主动护理。我们评估了该多学科诊所对住院率、手术率和感染率的影响。：对2014年至2024年接受治疗的基因确诊CIPA患者进行回顾性研究。比较了多学科诊所成立前（2014 - 2017年）和成立后（2018 - 2024年）期间电子病历中的数据。多学科诊所的核心团队包括一名传染病专家、一名骨科医生和护士。患者根据其携带耐药菌的情况进行分层，并采用严格的感染控制措施进行管理。定期或根据需要安排随访。治疗以临床检查结果和培养结果为指导。：共有59名患者纳入研究。两个时期的基线年龄无显著差异。住院率下降了30.7%（从每1000天57.7次降至40.0次），门诊就诊次数减少了42.9%（从25.5次降至14.6次）。总体手术率保持稳定（从2.8次降至2.7次），眼部手术减少了61.9%，择期拔牙增加了130.5%。感染率增加了52%（从每1000天6.6次升至10.1次）。：实施多学科诊所综合措施导致住院率、门诊就诊次数和眼部手术减少，同时择期拔牙和培养检测的使用增加。更密切的监测和早期感染管理减少了严重并发症的发生。这些发现支持了系统化、主动的多学科护理对改善CIPA患儿治疗效果的价值。
