Application of the New Classification on Patients with a Disorder of Sex Development in Indonesia
Disorder of sex development (DSD) patients in Indonesia most often do not receive a proper diagnostic evaluation and treatment. This study intended to categorize 88 Indonesian patients in accordance with the new consensus DSD algorithm. Diagnostic evaluation including clinical, hormonal, genetic, im...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | International Journal of Endocrinology |
| Online Access: | http://dx.doi.org/10.1155/2012/237084 |
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| author | A. Zulfa Juniarto Yvonne G. van der Zwan Ardy Santosa Remko Hersmus Frank H. de Jong Renske Olmer Hennie T. Bruggenwirth Axel P. N. Themmen Katja P. Wolffenbuttel Leendert H. J. Looijenga Sultana M. H. Faradz Stenvert L. S. Drop |
| author_facet | A. Zulfa Juniarto Yvonne G. van der Zwan Ardy Santosa Remko Hersmus Frank H. de Jong Renske Olmer Hennie T. Bruggenwirth Axel P. N. Themmen Katja P. Wolffenbuttel Leendert H. J. Looijenga Sultana M. H. Faradz Stenvert L. S. Drop |
| author_sort | A. Zulfa Juniarto |
| collection | DOAJ |
| description | Disorder of sex development (DSD) patients in Indonesia most often do not receive a proper diagnostic evaluation and treatment. This study intended to categorize 88 Indonesian patients in accordance with the new consensus DSD algorithm. Diagnostic evaluation including clinical, hormonal, genetic, imaging, surgical, and histological parameters was performed. Fifty-three patients were raised as males, and 34 as females. Of 22 patients with 46, XX DSD, 15 had congenital adrenal hyperplasia, while in one patient, an ovarian Leydig cell tumor was found. In all 58 46, XY DSD patients, 29 were suspected of a disorder of androgen action (12 with an androgen receptor mutation), and in 9, gonadal dysgenesis was found and, in 20, severe hypospadias e.c.i. Implementation of the current consensus statement in a resource-poor environment is very difficult. The aim of the diagnostic workup in developing countries should be to end up with an evidence-based diagnosis. This is essential to improve treatment and thereby to improve the patients' quality of life. |
| format | Article |
| id | doaj-art-79bd77faaed645a2b25e83e41cac5474 |
| institution | Kabale University |
| issn | 1687-8337 1687-8345 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Endocrinology |
| spelling | doaj-art-79bd77faaed645a2b25e83e41cac54742025-02-03T01:11:13ZengWileyInternational Journal of Endocrinology1687-83371687-83452012-01-01201210.1155/2012/237084237084Application of the New Classification on Patients with a Disorder of Sex Development in IndonesiaA. Zulfa Juniarto0Yvonne G. van der Zwan1Ardy Santosa2Remko Hersmus3Frank H. de Jong4Renske Olmer5Hennie T. Bruggenwirth6Axel P. N. Themmen7Katja P. Wolffenbuttel8Leendert H. J. Looijenga9Sultana M. H. Faradz10Stenvert L. S. Drop11Department of Human Genetics, Center for Biomedical Research, Faculty of Medicine Diponegoro University (FMDU), Semarang 50321, IndonesiaDivision of Pediatric Endocrinology, Department of Pediatrics, Sophia Children's Hospital, Erasmus MC, P.O. Box 2060, 3000 CD Rotterdam, The NetherlandsDepartment of Urology, Dr. Kariadi Hospital, Semarang 50321, IndonesiaDepartment of Pathology, Josephine Nefkens Institute, Erasmus University Medical Center, 3015 CE Rotterdam, The NetherlandsSection of Endocrinology, Department of Internal Medicine, Erasmus University Medical Center, 3015 CE Rotterdam, The NetherlandsDepartment of Clinical Genetics, Erasmus University Medical Centre, 3015 CE Rotterdam, The NetherlandsDepartment of Clinical Genetics, Erasmus University Medical Centre, 3015 CE Rotterdam, The NetherlandsSection of Endocrinology, Department of Internal Medicine, Erasmus University Medical Center, 3015 CE Rotterdam, The NetherlandsDepartment of Paediatric Urology, Erasmus University Medical Centre, 3015 CE Rotterdam, The NetherlandsDepartment of Pathology, Josephine Nefkens Institute, Erasmus University Medical Center, 3015 CE Rotterdam, The NetherlandsDepartment of Human Genetics, Center for Biomedical Research, Faculty of Medicine Diponegoro University (FMDU), Semarang 50321, IndonesiaDivision of Pediatric Endocrinology, Department of Pediatrics, Sophia Children's Hospital, Erasmus MC, P.O. Box 2060, 3000 CD Rotterdam, The NetherlandsDisorder of sex development (DSD) patients in Indonesia most often do not receive a proper diagnostic evaluation and treatment. This study intended to categorize 88 Indonesian patients in accordance with the new consensus DSD algorithm. Diagnostic evaluation including clinical, hormonal, genetic, imaging, surgical, and histological parameters was performed. Fifty-three patients were raised as males, and 34 as females. Of 22 patients with 46, XX DSD, 15 had congenital adrenal hyperplasia, while in one patient, an ovarian Leydig cell tumor was found. In all 58 46, XY DSD patients, 29 were suspected of a disorder of androgen action (12 with an androgen receptor mutation), and in 9, gonadal dysgenesis was found and, in 20, severe hypospadias e.c.i. Implementation of the current consensus statement in a resource-poor environment is very difficult. The aim of the diagnostic workup in developing countries should be to end up with an evidence-based diagnosis. This is essential to improve treatment and thereby to improve the patients' quality of life.http://dx.doi.org/10.1155/2012/237084 |
| spellingShingle | A. Zulfa Juniarto Yvonne G. van der Zwan Ardy Santosa Remko Hersmus Frank H. de Jong Renske Olmer Hennie T. Bruggenwirth Axel P. N. Themmen Katja P. Wolffenbuttel Leendert H. J. Looijenga Sultana M. H. Faradz Stenvert L. S. Drop Application of the New Classification on Patients with a Disorder of Sex Development in Indonesia International Journal of Endocrinology |
| title | Application of the New Classification on Patients with a Disorder of Sex Development in Indonesia |
| title_full | Application of the New Classification on Patients with a Disorder of Sex Development in Indonesia |
| title_fullStr | Application of the New Classification on Patients with a Disorder of Sex Development in Indonesia |
| title_full_unstemmed | Application of the New Classification on Patients with a Disorder of Sex Development in Indonesia |
| title_short | Application of the New Classification on Patients with a Disorder of Sex Development in Indonesia |
| title_sort | application of the new classification on patients with a disorder of sex development in indonesia |
| url | http://dx.doi.org/10.1155/2012/237084 |
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