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. 2017 Jun;175(2):268-278.
doi: 10.1002/ajmg.c.31558. Epub 2017 May 30.

Disorders of sex development (DSD): Clinical service delivery in the United States

Aimee M Rolston  1   2 , Melissa Gardner  1 , Kathleen van Leeuwen  3 , Lauren Mohnach  2   4 , Catherine Keegan  5   6 , Emmanuèle Délot  7 , Eric Vilain  7 , David E Sandberg  1 ; members of the DSD-TRN Advocacy; Advisory Network Accord Alliance
Affiliations

Disorders of sex development (DSD): Clinical service delivery in the United States

Aimee M Rolston et al. Am J Med Genet C Semin Med Genet. 2017 Jun.

Abstract

Following the principles of care recommended in the 2006 Consensus Statement on Disorders of Sex Development (DSD), along with input from representatives of peer support and advocacy groups, this study surveyed DSD clinical management practices at healthcare facilities in the United States. DSD are congenital conditions in which development of chromosomal, gonadal, or anatomic sex is atypical. Facilities providing care for patients with DSD were targeted for participation. Specialty providers completed a survey with questions in six broad categories: Institution Information, Nomenclature and Care Guidelines, Interdisciplinary Services, Staff and Community Education, DSD Management, and Research. Twenty-two of 36 targeted sites (61%) participated. Differences were observed between sites with regard to what conditions were considered to be DSD. All sites reported some degree of involvement of pediatric urology and/or surgery and pediatric endocrinology in the care of DSD patients. Gynecology and neonatology were most frequently not represented. Wide variation was observed across sites in continuing education standards, obtaining informed consent for clinical procedures, and in specific clinical management practices. This survey is the first to assess DSD clinical management practices in the United States. The findings establish a baseline of current practices against which providers delivering care to these patients and their families can benchmark their efforts. Such surveys also provide a practical framework for collaboration in identifying opportunities for change that enhance health and quality of life outcomes for patients and families affected by DSD.

Keywords: disorders of sex development; intersex; quality improvement; survey.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Mean score of institutions on elements of clinical management practices. Composite score of 1 = ideal score. Composite scores are calculated for institutions completing at least 80% of items comprising the scale.
Figure 2
Figure 2
Pediatric specialist availability. †1 pt = “always involved”; 0.5 pt = “referral/consult involvement only”
Figure 3
Figure 3
Elements of informed consent: discussion and documentation
Figure 4
Figure 4
Continuing education for providers. Composite score of 1 = ideal score
Figure 5
Figure 5
Comparison of institutions participating in the DSD-TRN (n=7) with others (n=15) Composite score of 1 = ideal score. For details regarding items comprising each composite scale, see Tables II–IV.
See this image and copyright information in PMC

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