Literature DB >> 8393406

Ten years experience with masculinizing genitoplasty in male pseudohermaphroditism due to 17 beta-hydroxysteroid dehydrogenase deficiency.

A Farkas1, A Rosler.   

Abstract

An extensive survey in a highly inbred Arab community documented over 60 cases of male pseudohermaphroditism due to 17 beta hydroxysteroid dehydrogenase deficiency. During a period of 10 years (1981-1991), 16 such patients underwent masculinating genitoplasty. Two children have had a one stage corrective genitoplasty while all others (2 adults, 3 adolescents and 9 children) had staged operations. Cosmetic and functional results are very encouraging in children and adolescents but very poor among adults. Based on our experience and follow-up, we advocate early sex assignment and early masculinizing genitoplasty with preoperative hormonal therapy to male pseudohermaphrodites due to 17 beta hydroxysteroid dehydrogenase deficiency.

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Year:  1993        PMID： 8393406     DOI： 10.1007/bf02125448

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN： 0340-6199            Impact factor:   3.183


  6 in total

1.  Crawford modification of Denis Browne hypospadias procedure.

Authors:  W J Yarbrough; J H Johnston
Journal:  J Urol       Date:  1977-06       Impact factor: 7.450

2.  The nature of the defect in familial male pseudohermaphroditism in Arabs of Gaza.

Authors:  B Eckstein; S Cohen; A Farkas; A Rösler
Journal:  J Clin Endocrinol Metab       Date:  1989-02       Impact factor: 5.958

3.  Familial male pseudohermaphroditism with gynecomastia due to a testicular 17-ketosteroid reductase defect. I. Studies in vivo.

Authors:  J M Saez; E De Peretti; A M Morera; M David; J Bertrand
Journal:  J Clin Endocrinol Metab       Date:  1971-05       Impact factor: 5.958

4.  Male pseudohermaphroditism due to 17 beta-hydroxysteroid dehydrogenase deficiency: gender reassignment in early infancy.

Authors:  D J Gross; H Landau; G Kohn; A Farkas; E Elrayyes; R el-Shawwa; E E Lasch; A Rösler
Journal:  Acta Endocrinol (Copenh)       Date:  1986-06

5.  Male pseudohermaphroditism due to 17 beta-hydroxysteroid dehydrogenase deficiency: studies on the natural history of the defect and effect of androgens on gender role.

Authors:  A Rösler; G Kohn
Journal:  J Steroid Biochem       Date:  1983-07       Impact factor: 4.292

6.  Male pseudohermaphroditism secondary to 17 beta-hydroxysteroid dehydrogenase deficiency: gender role change with puberty.

Authors:  J Imperato-McGinley; R E Peterson; R Stoller; W E Goodwin
Journal:  J Clin Endocrinol Metab       Date:  1979-09       Impact factor: 5.958
  6 in total
  6 in total

Review 1.  Clinical, endocrine, and molecular findings in 17beta-hydroxysteroid dehydrogenase type 3 deficiency.

Authors:  M F Faienza; L Giordani; M Delvecchio; L Cavallo
Journal:  J Endocrinol Invest       Date:  2008-01       Impact factor: 4.256

2.  The use of laparoscopy in intersex patients.

Authors:  Boris Chertin; Dmitry Koulikov; Josef Alberton; Irit Hadas-Halpern; Pethachia Reissman; Amicur Farkas
Journal:  Pediatr Surg Int       Date:  2006-03-07       Impact factor: 1.827

3.  Consensus in Guidelines for Evaluation of DSD by the Texas Children's Hospital Multidisciplinary Gender Medicine Team.

Authors:  Ganka Douglas; Marni E Axelrad; Mary L Brandt; Elizabeth Crabtree; Jennifer E Dietrich; Shannon French; Sheila Gunn; Lefkothea Karaviti; Monica E Lopez; Charles G Macias; Laurence B McCullough; Deepa Suresh; V Reid Sutton
Journal:  Int J Pediatr Endocrinol       Date:  2010-10-17

4.  Complexities of gender assignment in 17β-hydroxysteroid dehydrogenase type 3 deficiency: is there a role for early orchiectomy?

Authors:  Janet Chuang; Amy Vallerie; Lesley Breech; Howard M Saal; Shumyle Alam; Peggy Crawford; Meilan M Rutter
Journal:  Int J Pediatr Endocrinol       Date:  2013-09-12

5.  46,XY Sex Development Defect due to a Novel Homozygous (Splice Site) c.673_1G>C Variation in the HSD17B3 Gene: Case Report

Authors:  Nurdan Çiftci; Leman Kayaş; Emine Çamtosun; Ayşehan Akıncı
Journal:  J Clin Res Pediatr Endocrinol       Date:  2021-01-04

6.  Severe Undervirilisation in a 46,XY Case Due to a Novel Mutation in HSD17B3 Gene.

Authors:  Ayfer Alikaşifoğlu; Doğuş Vurallı; Olaf Hiort; Nazlı Gönç; Alev Özön; Nurgün Kandemir
Journal:  J Clin Res Pediatr Endocrinol       Date:  2015-09
  6 in total

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