How geneticists think about Differences/Disorders of Sexual Development (DSD): A conversation.

J Pediatr Urol

Division of Pediatric Urology, Hasbro Children's Hospital, Providence, RI, USA. Electronic address:

Published: December 2020


Category Ranking

98%

Total Visits

921

Avg Visit Duration

2 minutes

Citations

20

Article Abstract

A multidisciplinary DSD clinic offers the opportunity for different specialties to learn from each other, as each provides their own perspective and expertise to the management of these complex patients, leading to collaborative care. For the patient, a multi-disciplinary clinic can improve access to care and decrease stress, as patients see all of the specialists on one day. For urologists seeing patients with DSD within a multi-disciplinary DSD clinic as well as independently, understanding what other specialists provide can help facilitate care and referral. Medical genetics is part of a multi-disciplinary DSD clinic. Given the recent advances in genetic diagnostics, many of the offered tests may be less familiar to the pediatric urologist. Therefore, this conversation reviews the clinical presentations and genetic testing options including chromosomal microarray, genetic testing panel, whole exome sequencing, and whole genome sequencing and how these can be helpful in the diagnosis and management of patients with DSD conditions.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpurol.2020.08.015DOI Listing

Publication Analysis

Top Keywords

dsd clinic
12
patients dsd
8
multi-disciplinary dsd
8
genetic testing
8
dsd
6
geneticists differences/disorders
4
differences/disorders sexual
4
sexual development
4
development dsd
4
dsd conversation
4

Similar Publications

This case report elucidates the diagnostic trajectory of a female newborn, presenting with apparent clitoromegaly, ultimately diagnosed with congenital adrenal hyperplasia (CAH). The patient was born in a prominent obstetrics and gynecology center in Tbilisi, Georgia, where the anomaly was promptly identified following a physiologically normal pregnancy and labor. Despite the relative infrequency of such cases in our center, particularly among term infants, the handling of this case was swift and successful.

View Article and Find Full Text PDF

Disorders of sex development (DSDs) encompass a spectrum of congenital conditions characterized by discordance among chromosomal, gonadal, and anatomical sex. Advances in genetic and molecular technologies have elucidated a complex landscape of underlying etiologies, including mutations in genes regulating sex determination and differentiation, copy number variations, and epigenetic alterations. These discoveries have not only enhanced diagnostic accuracy but also deepened our understanding of the molecular mechanisms driving DSDs.

View Article and Find Full Text PDF

Strategies for improving the knowledge of patients and carers.

Best Pract Res Clin Endocrinol Metab

August 2025

Clinic for Paediatric Endocrinology and Diabetology, Charité - Universitätsmedizin Berlin, Campus Virchow-Klinikum, Augustenburger Platz 1, Berlin 13353, Germany. Electronic address:

Treating people with a difference of sex development requires a multidisciplinary team. In recent years, knowledge of diagnoses and approaches to support and treatment have both evolved. To enable people with DSD to make informed decisions about medical treatments related to their diagnosis, they and their legal guardians must be provided with comprehensive information.

View Article and Find Full Text PDF

Reconciling oncologic outcomes with renal preservation in Denys-Drash syndrome and recurrent Wilms Tumor. A case-based discussion.

Urology

September 2025

Atrium Health Carolinas Medical Center, Department of Urology, 1225 Harding Place Charlotte Unit 3100, NC 28204; Atrium Health Levine Children's Hospital, 1001 Blythe Blvd Suite 4100a Charlotte, NC 28204.

Denys Drash syndrome (DDS) results from a mutation in the WT1 tumor suppressor gene manifesting early in childhood. The classic presentation is characterized by early end stage renal disease (ESRD), differences of sexual differentiation (DSD), and high risk of Wilms tumor (WT). Management varies based on individual patient presentations.

View Article and Find Full Text PDF

Introduction: Aesthetic outcomes are increasingly vital in dental care, especially in achieving harmony between facial and dental features. Traditional smile design methods rely heavily on clinician judgment, which introduces subjectivity and variability. Digital Smile Design (DSD) offers an eco-friendly and standardized alternative, promoting improved aesthetic outcomes with reduced environmental impact.

View Article and Find Full Text PDF