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Abstract：
Background: Girls born with congenital adrenal hyperplasia have virilized external genitalia. There is considerable debate regarding both the outcomes of feminizing genitoplasty and timing of the surgery in this population. Objective: To investigate outcomes of females 46,XX individuals with CAH in Malaysia, the surgical outcomes of feminizing genitoplasty (FG) and their attitudes toward surgery. Study Design: This is a cross-sectional study involving the two main tertiary centers in Malaysia. All 46,XX patients with CAH and raised female, who had undergone FG were identified and invited to participate. Data on socio-demographic, medical profiles, and attitudes toward surgery were collected. A standardized evaluation of the external genitalia was undertaken including the anatomic and cosmetic evaluation by independent gynecologists. Results: Of 61 individuals identified, 59 participated-consisting of children (n = 12), adolescents (n = 29) and adults (n = 18). All but one had classical CAH (98.3%) and had undergone FG (n = 55, 93.2%) with surgery mostly undertaken by pediatric surgeons trained in DSD work (n = 44, 74.6%). Complications overall were low (20.3%), with repeat surgery rate of 9.1%. External genital examination was performed in 38 participants. Overall 36.8% had absent clitoral glands and 39.5% had a persistent urogenital sinus and in 10.5%, no vaginal orifices were seen. Poor cosmetic outcomes were present in 42.1% with 55.3% recommended for further assessment under general anesthetic. Almost half participants did not venture an opinion on FG, those who did varied from having a positive attitude toward it (18 participants) to 3 opining that it should not be done, or avoided or delayed. From the participants, 35.5% preferred FG to be done early in life compared to 44.0% of the parents. Conclusions: The reoperation rates of the feminizing genitoplasty surgeries were low however due to the anatomic and cosmetic outcomes, reassessment of the external genitalia of these CAH patients may be required once they consider becoming sexually active as they may require further treatment. Many factors such as cultural sensitivities and access to medical treatment and late diagnoses have an impact on attitudes toward FG.
摘要：
背景：先天性肾上腺增生的女孩具有外生殖器的毒性。在该人群中，关于女性化生殖器成形术的结果和手术时机都存在相当大的争议。目的：调查马来西亚女性46,XX例CAH患者的结局，女性化生殖器成形术（FG）的手术结果及其对手术的态度。研究设计：这是一项涉及马来西亚两个主要高等教育中心的横断面研究。所有46例，XX例CAH患者，女性，已接受FG的人被确认并被邀请参加。社会人口数据，医学资料,收集了对手术的态度。对外生殖器进行了标准化评估，包括由独立妇科医生进行的解剖和美容评估。结果：在确定的61个人中，59名参与者-由儿童组成（n=12），青少年（n=29）和成人（n=18）。除一名患者外，所有患者均患有经典CAH（98.3％），并接受了FG（n=55，93.2％），手术主要由接受DSD工作培训的儿科外科医生进行（n=44，74.6％）。并发症总体较低（20.3%），重复手术率为9.1%。38名参与者进行了外生殖器检查。总体上有36.8％的阴蒂腺体缺失，39.5％的泌尿生殖窦持续存在，10.5％，没有看到阴道口。不良的美容结果为42.1％，建议在全身麻醉下进行进一步评估的55.3％。几乎一半的参与者没有冒险对FG发表意见，那些对它持积极态度的人（18名参与者）到3名认为不应该这样做，或避免或延迟。从参与者那里，35.5%的父母倾向于在生命早期完成FG，而44.0%的父母则倾向于在生命早期完成FG。结论：由于解剖和美容结果，女性化生殖器成形术的再手术率较低，一旦这些CAH患者考虑性活跃,可能需要对其外生殖器进行重新评估,因为他们可能需要进一步治疗.许多因素，例如文化敏感性，获得医疗和晚期诊断都会影响对FG的态度。
