Kearney S, Sharathkumar A, Rodriguez V, Chitlur M, Valentino L, Boggio L, Gill J
Children's Hospitals and Clinics of Minnesota, Pediatric Hematology Oncology, Minneapolis, MN, USA.
Haemophilia. 2015 Jan;21(1):52-7. doi: 10.1111/hae.12528. Epub 2014 Dec 4.
Neonatal circumcision in patients with severe haemophilia has not been well studied. We performed a survey of paediatric haematologists from Hemophilia Treatment Centers (HTC) across the United States to better understand the attitudes toward and management of neonatal circumcision in haemophilia patients. Response rate to our survey was 40% (n = 64/159). Thirty-eight percent of respondents (n = 24) said that they would allow this procedure in the newborn period but in many cases this was against medical advice. The most reported concern regarding neonatal circumcision in haemophilia patients was the risk of development of an inhibitor (n = 25; 39%) followed by the concern for bleeding (n = 22; 34%) and issues related to vascular access in the neonate (n = 11; 17%). All respondents recommended at least one preprocedure dose of factor replacement. Twenty-two percent (n = 14) of respondents did not use more than one dose of factor replacement but 32% (n = 21) used 1-2 postoperative doses. The remainder of paediatric haematologists surveyed recommended between 3-5 (16%; n = 10) and 6-10 (3%, n = 2) additional days postoperatively. There was wide variation in both techniques of circumcision as well as adjuvant haemostatic agents used. Only 22% of respondents said that they had an established protocol for management of circumcision in the newborn haemophilia patient. These survey results highlight the need for evidence-based guidelines regarding the optimal management of circumcision in neonates with severe haemophilia.
对于患有严重血友病的新生儿进行包皮环切术尚未得到充分研究。我们对美国各地血友病治疗中心(HTC)的儿科血液学家进行了一项调查,以更好地了解对血友病患者新生儿包皮环切术的态度和管理。我们调查的回复率为40%(n = 64/159)。38%的受访者(n = 24)表示他们会在新生儿期允许进行该手术,但在许多情况下这与医疗建议相悖。关于血友病患者新生儿包皮环切术最常报告的担忧是产生抑制剂的风险(n = 25;39%),其次是出血担忧(n = 22;34%)以及与新生儿血管通路相关的问题(n = 11;17%)。所有受访者都建议至少术前给予一剂凝血因子替代治疗。22%的受访者(n = 14)使用不超过一剂凝血因子替代治疗,但32%(n = 21)使用1 - 2剂术后治疗。其余接受调查的儿科血液学家建议术后额外使用3 - 5天(16%;n = 10)和6 - 10天(3%;n = 2)。包皮环切术技术以及使用的辅助止血剂都存在很大差异。只有22%的受访者表示他们有针对新生儿血友病患者包皮环切术管理的既定方案。这些调查结果凸显了针对患有严重血友病的新生儿包皮环切术最佳管理制定循证指南的必要性。