Post-operative complications following feminizing genitoplasty in moderate to severe genital atypia: Results from a multicenter, observational prospective cohort study.
Post-operative complications following feminizing genitoplasty in moderate to severe genital atypia: Results from a multicenter, observational prospective cohort study.
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DOI：
10.1016/j.jpurol.2020.05.166
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发表时间：
2020-10
影响因子：
2
通讯作者：
Baskin L
中科院分区：
文献类型：
--
作者：
Baskin A;Wisniewski AB;Aston CE;Austin P;Chan YM;Cheng EY;Diamond DA;Fried A;Kolon T;Lakshmanan Y;Williot P;Meyer S;Meyer T;Kropp B;Nokoff N;Palmer B;Paradis A;Poppas D;VanderBrink B;Scott Reyes KJ;Tishelman A;Wolfe-Christensen C;Yerkes E;Mullins LL;Baskin L
性发育障碍/差异(DSD)是一种先天性疾病，其中存在不典型的染色体、性腺和/或表型性行为。虽然围绕传统的二元性概念仍然存在争议，但大多数DSD患者的养育方式取决于他们的遗传性别、性腺性别、生殖器表型和他们内部生殖道的状况。这项研究使用了来自全美12家专门从事DSD护理的机构的前瞻性数据。我们把重点放在女性养育的患者身上。符合条件的患者有中到重度生殖器非典型性(定义为Prader Score>；2)，入院时≤为2岁，以前没有生殖器成形术。这项研究的目的是描述年轻患者接受女性化生殖器成形术的现代方法的术后早期并发症。在队列中的91名参与者中，57名(62%)是雌性的。其中52例为先天性肾上腺增生症(CAH)，1例为卵巢睾丸综合征，2例为混合性性腺发育不良，2例为部分雄激素不敏感综合征(PAIS)。在接受早期生殖器成形术的50名参与者中，43名(86%)在术后6-12个月进行了随访。32例(%)接受了阴蒂成形术，31例(62%)接受了部分泌尿生殖器松动术，4例(8%)接受了完全泌尿生殖器松动术。18%(9/50)经历了手术后并发症，其中7(14%)被评为Clavien-Dindo III级。父母和外科医生都报告说，与基线相比，参与者手术后对生殖器外观的满意度有所提高。这些关于手术后并发症的信息将有助于指导家庭在决定是否继续为患有中度至重度生殖器异型性的女性患者进行手术时，与当代对幼儿进行女性化生殖器成形术的方法有关。
Disorders/differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. While there remains controversy around the traditionally binary concept of sex, most patients with DSD are reared either male or female depending on their genetic sex, gonadal sex, genital phenotype and status of their internal genital tract. This study uses prospective data from 12 institutions across the United States that specialize in DSD care. We focused on patients raised female. Eligible patients had moderate to severe genital atypia (defined as Prader score >2), were ≤2 years of age at entry, and had no prior genitoplasty. The aim of this study is to describe early post operative complications for young patients undergoing modern approaches to feminizing genitoplasty. Of the 91 participants in the cohort, 57 (62%) were reared female. The majority had congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (n = 52), 1 had ovotesticular syndrome, 2 had mixed gonadal dysgenesis and 2 had partial androgen insensitivity syndrome (PAIS). Of the 50 participants who received early genitoplasty, 43 (86%) had follow-up at 6–12 months post-surgery. Thirty-two participants (64%) received a clitoroplasty, 31 (62%) partial urogenital mobilization and 4 (8%) total urogenital sinus mobilization. Eighteen percent (9/50) experienced post-surgical complications with 7 (14%) being rated as Clavien-Dindo grade III. Both parents and surgeons reported improved satisfaction with genital appearance of participants following surgery compared to baseline. This information on post-operative complications associated with contemporary approaches to feminizing genitoplasty performed in young children will help guide families when making decisions about whether or not to proceed with surgery for female patients with moderate to severe genital atypia.
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影响因子：
2.9
作者：
Baskin, Laurence;Shen, Joel;Cunha, Gerald R.
通讯作者：
Cunha, Gerald R.
影响因子：
--
作者：
Thyen, Ute;Lanz, Kathrin;Hiort, Olaf
通讯作者：
Hiort, Olaf
影响因子：
6.6
作者：
Baskin, LS;Erol, A;Cunha, GR
通讯作者：
Cunha, GR
影响因子：
4.1
作者：
Finlayson, Courtney;Rosoklija, Ilina;Cheng, Earl Y.
通讯作者：
Cheng, Earl Y.
影响因子：
2
作者：
Palmer, Blake W.;Wisniewski, Amy B.;Kropp, Bradley P.
通讯作者：
Kropp, Bradley P.