Reply

Abstract

To the Editor:We acknowledge Dr. Rosenbloom's concerns regarding the possible consequences regarding delayed diagnostic testing of patients with severe hypospadias. We do not agree completely with all of his comments, however.Dr. Rosenbloom suggests that our editorial1Smith EP Wacksman J Evaluation of severe hypospadias.J Pediatr. 1997; 131: 344-346Abstract Full Text PDF PubMed Scopus (10) Google Scholar conveys the conclusion that bilateral descent of the testes is “strong evidence of normal androgen action.” More precisely, our editorial indicates “bilateral descent of the testes into a well differentiated scrotum is strong evidence that androgen action is largely intact and the hypospadias is very likely to have been caused by a non-hormonal idiopathic process.” Our primary point was that on the basis of the review of Albers et al.2Albers N Ulfichs C Gliier S Hiort O Sinnecker GHG Mildenberger H et al.Etiologic classification of severe hypospadias: implications for prognosis and management.J Pediatr. 1997; 131: 386-392Abstract Full Text PDF PubMed Google Scholar and other similar recent studies,3Sinnecker GHG Hiort O Dibbelt L Dorr HG Haub H Hemrich U et al.Phenotypic classification of male pseudohermaphroditism due to steroid 5-α reductase deficiency.Am J Med Genet. 1996; 63: 223-230Crossref PubMed Scopus (156) Google Scholar a patient with well-differentiated scrotum, bilaterally descended testes, and adequate phallic size is “very unlikely” to have a definable cause given the existing diagnostic tests available. In these individuals, after a normal 46 XY karyotype and ultrasound, there is no uncertainty about sex assignment, and therefore the urgency for further testing is abated and a judgment can be made whether further testing is warranted given the resources available. To the Editor:We acknowledge Dr. Rosenbloom's concerns regarding the possible consequences regarding delayed diagnostic testing of patients with severe hypospadias. We do not agree completely with all of his comments, however.Dr. Rosenbloom suggests that our editorial1Smith EP Wacksman J Evaluation of severe hypospadias.J Pediatr. 1997; 131: 344-346Abstract Full Text PDF PubMed Scopus (10) Google Scholar conveys the conclusion that bilateral descent of the testes is “strong evidence of normal androgen action.” More precisely, our editorial indicates “bilateral descent of the testes into a well differentiated scrotum is strong evidence that androgen action is largely intact and the hypospadias is very likely to have been caused by a non-hormonal idiopathic process.” Our primary point was that on the basis of the review of Albers et al.2Albers N Ulfichs C Gliier S Hiort O Sinnecker GHG Mildenberger H et al.Etiologic classification of severe hypospadias: implications for prognosis and management.J Pediatr. 1997; 131: 386-392Abstract Full Text PDF PubMed Google Scholar and other similar recent studies,3Sinnecker GHG Hiort O Dibbelt L Dorr HG Haub H Hemrich U et al.Phenotypic classification of male pseudohermaphroditism due to steroid 5-α reductase deficiency.Am J Med Genet. 1996; 63: 223-230Crossref PubMed Scopus (156) Google Scholar a patient with well-differentiated scrotum, bilaterally descended testes, and adequate phallic size is “very unlikely” to have a definable cause given the existing diagnostic tests available. In these individuals, after a normal 46 XY karyotype and ultrasound, there is no uncertainty about sex assignment, and therefore the urgency for further testing is abated and a judgment can be made whether further testing is warranted given the resources available. We acknowledge Dr. Rosenbloom's concerns regarding the possible consequences regarding delayed diagnostic testing of patients with severe hypospadias. We do not agree completely with all of his comments, however. Dr. Rosenbloom suggests that our editorial1Smith EP Wacksman J Evaluation of severe hypospadias.J Pediatr. 1997; 131: 344-346Abstract Full Text PDF PubMed Scopus (10) Google Scholar conveys the conclusion that bilateral descent of the testes is “strong evidence of normal androgen action.” More precisely, our editorial indicates “bilateral descent of the testes into a well differentiated scrotum is strong evidence that androgen action is largely intact and the hypospadias is very likely to have been caused by a non-hormonal idiopathic process.” Our primary point was that on the basis of the review of Albers et al.2Albers N Ulfichs C Gliier S Hiort O Sinnecker GHG Mildenberger H et al.Etiologic classification of severe hypospadias: implications for prognosis and management.J Pediatr. 1997; 131: 386-392Abstract Full Text PDF PubMed Google Scholar and other similar recent studies,3Sinnecker GHG Hiort O Dibbelt L Dorr HG Haub H Hemrich U et al.Phenotypic classification of male pseudohermaphroditism due to steroid 5-α reductase deficiency.Am J Med Genet. 1996; 63: 223-230Crossref PubMed Scopus (156) Google Scholar a patient with well-differentiated scrotum, bilaterally descended testes, and adequate phallic size is “very unlikely” to have a definable cause given the existing diagnostic tests available. In these individuals, after a normal 46 XY karyotype and ultrasound, there is no uncertainty about sex assignment, and therefore the urgency for further testing is abated and a judgment can be made whether further testing is warranted given the resources available.