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性发育障碍的外科治疗:单中心经验

Managing disorder of sexual development surgically: A single center experience.

作者信息

Kumar Jatinder, Kumar Vikas, Bhatia Vijaylakshmi, Dabadghao Preeti, Chaturvedi Samit, Kapoor Rakesh, Ansari M S

机构信息

Department of Urology and Renal Transplantation and Pediatric Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh, India.

出版信息

Indian J Urol. 2012 Jul;28(3):286-91. doi: 10.4103/0970-1591.102703.

DOI:10.4103/0970-1591.102703
PMID:23204656
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3507397/
Abstract

INTRODUCTION

Ambiguous genitalia are a major cause of parental anxiety and create psychological and social problems to patient, if not managed properly. Here we present our experience in managing patients with ambiguous genitalia.

MATERIAL AND METHODS

We retrospectively reviewed clinical records of all patients with ambiguous genitalia managed surgically at out institute between December 1989 and January 2011. Relevant history, clinical examination, investigations and surgical procedures performed were analyzed and results were evaluated in terms of anatomical, functional and psychosexual outcomes.

RESULTS

Female pseudohermaphroditism was the most common cause of genital ambiguity in our patients. Male and female genitoplasty was done according to gender of rearing, genital anatomy and parental choice. Twenty six patients (86.6%) reported satisfactory cosmetic outcome and 22 (73.3%) satisfactory functional outcome on long term follow-up. Among the 24 patients diagnosed as male pseudohermaphroditism 14 (82.3%) patient have reported satisfactory cosmetic outcome and 13 (76.4%) have reported satisfactory functional outcome. In patients with mixed gonadal dysgenesis and true hermaphroditism satisfactory cosmetic and functional outcome was seen in 70% patient.

CONCLUSION

Managing patients of genital ambiguity according to gender of rearing, genital anatomy and parental choice carries good prognosis in terms of anatomical, functional and psychosexual outcome.

摘要

引言

两性畸形是导致父母焦虑的主要原因,若处理不当会给患者带来心理和社会问题。在此,我们介绍我们在处理两性畸形患者方面的经验。

材料与方法

我们回顾性分析了1989年12月至2011年1月期间在我院接受手术治疗的所有两性畸形患者的临床记录。分析相关病史、临床检查、检查结果及所实施的外科手术,并从解剖学、功能和心理性结果方面评估结果。

结果

女性假两性畸形是我们患者中两性畸形最常见的原因。根据抚养性别、生殖器解剖结构和父母的选择进行男性和女性生殖器成形术。26例患者(86.6%)在长期随访中报告了满意的美容效果,22例(73.3%)报告了满意的功能效果。在24例被诊断为男性假两性畸形的患者中,14例(82.3%)报告了满意的美容效果,13例(76.4%)报告了满意的功能效果。在混合性性腺发育不全和真两性畸形患者中,70%的患者有满意的美容和功能效果。

结论

根据抚养性别、生殖器解剖结构和父母的选择来处理两性畸形患者,在解剖学、功能和心理性结果方面预后良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fcb/3507397/aa98255f3a95/IJU-28-286-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fcb/3507397/39f2cd095c1a/IJU-28-286-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fcb/3507397/aa98255f3a95/IJU-28-286-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fcb/3507397/39f2cd095c1a/IJU-28-286-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fcb/3507397/aa98255f3a95/IJU-28-286-g002.jpg

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Early management and gender assignment in disorders of sexual differentiation.性发育异常的早期管理与性别指定
Endocr Dev. 2007;11:47-57. doi: 10.1159/000111057.
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The role of the pediatrician in the management of children with genital ambiguities.儿科医生在处理生殖器模糊儿童方面的作用。
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Consensus statement on management of intersex disorders. International Consensus Conference on Intersex.关于两性畸形疾病管理的共识声明。国际两性畸形共识会议。
Pediatrics. 2006 Aug;118(2):e488-500. doi: 10.1542/peds.2006-0738.
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Feminizing reconstructive surgery for ambiguous genitalia: the Leipzig experience.两性畸形生殖器的女性化重建手术:莱比锡经验
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J Urol. 2005 Jul;174(1):284-8; discussion 288. doi: 10.1097/01.ju.0000161211.40944.87.
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Behavioral and physical masculinization are related to genotype in girls with congenital adrenal hyperplasia.先天性肾上腺皮质增生症女孩的行为和身体男性化与基因型有关。
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