电邮这篇文章 SURGICAL CORRECTION OF NONHORMONAL CLITOROMEGALY IN NEUROFIBROMATOSIS TYPE 1
- 作者: Morozov D.A.1, Raigorodskaya N.Y.2, Pimenova E.S.1, Bolotova N.V.2, Airyan E.K.1
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隶属关系:
- I.M. Sechenov First Moscow State University
- V. I. Razumovsky Saratov State Medical University
- 期: 卷 6, 编号 4 (2016)
- 页面: 92-96
- 栏目: Case reports
- ##submission.dateSubmitted##: 11.06.2017
- ##submission.dateAccepted##: 11.06.2017
- ##submission.datePublished##: 10.12.2016
- URL: https://rps-journal.ru/jour/article/view/298
- DOI: https://doi.org/10.17816/psaic298
- ID: 298
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详细
Neurofibromatosis 1 type (NF1, Recklinghausen disease) is caused by mutations in the NF1 gene (localized to chromosome 17q) and is an autosomal dominant genetic syndrome. The disease occurs in 1 of every 2500–3000 births. In neurobibromatosis the urogenital tract is affected rarely. The article presents the clinical case of examination, diagnosis verification and surgery of a 12‑year-old girl complaining of an increased clitoris with the aggravated genealogical anamnesis (her uncle suffers from neurofibromatosis type 1). The child examined by an endocrinologist, removed diagnosis violation forming the floor. Spend plastic feminized external genitalia with the removal of nodules neurofibromatosis imitating virilization of the external genitalia, resection of the corpora cavernosa, labiaplasty of mucocutaneous grafts. Cosmetic outcome immediately after the operation is regarded as satisfactory.
作者简介
D. Morozov
I.M. Sechenov First Moscow State University
编辑信件的主要联系方式.
Email: damorozov@list.ru
Доктор медицинских наук, профессор, заведующий кафедрой детской хирургии и урологии-андрологии 俄罗斯联邦
N. Raigorodskaya
V. I. Razumovsky Saratov State Medical University
Email: nraygorodskaya@gmail.com
Доктор медицинских наук, доцент кафедры пропедевтики детских болезней, детской эндокринологии и диабетологии 俄罗斯联邦
E. Pimenova
I.M. Sechenov First Moscow State University
Email: fake@neicon.ru
Кандидат медицинских наук, доцент кафедры детской хирургии и урологии- андрологии 俄罗斯联邦
N. Bolotova
V. I. Razumovsky Saratov State Medical University
Email: fake@neicon.ru
Доктор медицинских наук, профессор, заведующая кафедрой пропедевтики детских болезней, детской эндокринологии и диабетологии 俄罗斯联邦
E. Airyan
I.M. Sechenov First Moscow State University
Email: fake@neicon.ru
детский хирург, аспирант кафедры детской хирургии и урологии-андрологии 俄罗斯联邦
参考
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- Sutphen R., Galán-Goméz E., Kousseff B. G. Clitoromegaly in neurofibromatosis //Am J Med Genet. 1995.55 (3):325–30.
- Pascual-Castroviejo I., Lopez-Pereira P., Savasta S. Neurofibromatosis type 1 with external genitalia involvement: Presentation of 4 patients // J of Pediatric Surgery. 2008.43:1998–2003.
- Болотова Н. В., Морозов Д. А., Райгородская Н. Ю., Жарков Д. А., Григорьева М. М., Цмокалюк Е. Н. Критерии кли- нической диагностики и выбор лечебной тактики у пациентов с неопределенностью пола // Саратовский научно- медицинский журнал. 2010. Т.6. №1. С. 178–182.
- Bolotov N.V., Morozov D.A., Raigorodskaya N.Y., Zharkov D.A., Grigoriev M.M., Tsmokalyuk E.N. Criterias for clinical diagnosis and choice of treatment strategy in patients with gender uncertainty // Saratov Journal of Medical Science. 2010 V.6. №1. Pp 178–182.
- McDonnell C. Neurofibromatosis of bladder and prostate // Am J Surg 1936; 34: 90–3.
- Thomas W. J., Bevan H. E., Hooper D. G., Downey E. J. Malignant schwannoma of the clitoris in a 1-year-old child // Cancer. 1989 Jun 1;63 (11):2216–9.
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