Congenital adrenal hyperplasia patient perception of ‘disorders of sex development’ nomenclature

International Journal of Pediatric Endocrinology

Maria G Vogiatzi¹  Dix P Poppas²  Oksana Lekarev¹  Karen Lin-Su¹ 

[1] Department of Pediatric Endocrinology, Weill Cornell Medical Center, 505 East 70th Street, 3rd floor, New York 10021, NY, USA;Department of Pediatric Urology, Weill Cornell Medical Center, 525 East 68th Street, Suite F-943, New York 10065, NY, USA

关键词: Nomenclature;    Disorders of sex development;    Congenital adrenal hyperplasia;   

Others  :  1145446
DOI  :  10.1186/s13633-015-0004-4

 received in 2014-10-28, accepted in 2015-01-25,  发布年份 2015

【 摘 要 】

Background

As the benefits of patient-centered care have become more widely recognized, it is important to understand patients’ sentiments regarding aspects affecting their care. In an effort to display more sensitivity to patient concerns, the term “disorders of sex development” (DSD) was proposed in 2006 as new nomenclature to replace older terms that were considered to have negative connotations.

Methods

The objective of the study was to examine the views of congenital adrenal hyperplasia (CAH) patients and their caregivers regarding the new nomenclature. The study was observational to evaluate the views of the CAH community, and the primary endpoint was perception of the term DSD. The study was conducted as a survey about views regarding DSD nomenclature. The survey was sent via email to eligible subjects. Along with a short introduction explaining the term DSD, the survey was sent to eligible CAH patients and their caregivers. 589 CAH patients or family members participated in the survey.

Results

A total of 589 responses were received (255 classical females, 104 non-classical females, 174 males, 56 not specified) (547 U.S., 42 international) (128 CAH patients, 408 parents or other family members). 70.6% had never heard the term DSD. 71.0% disliked or strongly disliked the term DSD. 83.6% stated they did not identify with the term DSD. 76.0% felt that the term DSD has a negative effect on the CAH community. There was no significant difference in opinion of DSD between classical females and other CAH patients, between US and international, between surgical and non-surgical patients, or between patients and parents. There was no correlation with patient age.

Conclusions

Our results indicate that the majority of parents and patients with CAH are dissatisfied with the term DSD. Our results highlight the challenges within the field of DSD to reach a consensus regarding a sensitive topic and to bridge the gap between current medical practice and patient satisfaction. It is the authors’ belief that reconsideration of the current nomenclature and ongoing dialogue between the medical community and patients will eventually lead to removal of stigmatization, better management protocols, and improved outcomes.

【 授权许可】

   
2015 Lin-Su et al.; licensee Biomed Central.

【 图 表 】

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【 参考文献 】

• [1]Houk CP, Hughes IA, Ahmed SF, Lee PA: Writing Committee for the International Intersex Consensus Conference P: Summary of consensus statement on intersex disorders and their management. International Intersex Consensus Conference. Pediatrics. 2006, 118(2):753-7.
• [2]Hughes IA, Houk C, Ahmed SF, Lee PA, Group LC, Group EC: Consensus statement on management of intersex disorders. Arch Dis Child 2006, 91(7):554-63.
• [3]Lee PA, Houk CP, Ahmed SF, Hughes IA: International Consensus Conference on Intersex organized by the Lawson Wilkins Pediatric Endocrine S, the European Society for Paediatric E: Consensus statement on management of intersex disorders. International Consensus Conference on Intersex. Pediatrics. 2006, 118(2):e488-500.
• [4]Hughes IA: Disorders of sex development: a new definition and classification. Best Pract Res Clin Endocrinol Metab 2008, 22(1):119-34.
• [5]Pasterski V, Prentice P, Hughes IA: Consequences of the Chicago consensus on disorders of sex development (DSD): current practices in Europe. Arch Dis Child 2010, 95(8):618-23.
• [6]Nimkarn S, Lin-Su K, New MI: Steroid 21 hydroxylase deficiency congenital adrenal hyperplasia. Pediatr Clin North Am 2011, 58(5):1281-300. xii
• [7]Davies JH, Knight EJ, Savage A, Brown J, Malone PS: Evaluation of terminology used to describe disorders of sex development. J Pediatr Urol 2011, 7(4):412-5.
• [8]Mohammed K, Nolan MB, Rajjo T, Shah ND, Prokop LJ, Varkey P, Marad MJ. Creating a Patient-Centered Health Care Delivery System: A Systematic Review of Health Care Quality From the Patient Perspective. Am J Med Qual. E-published ahead of print on July 14, 2014
• [9]Rosenthal TC: The medical home: growing evidence to support a new approach to primary care. J Am Board Fam Med 2008, 21(5):427-40.
• [10]Strickland B, McPherson M, Weissman G, van Dyck P, Huang ZJ, Newacheck P: Access to the medical home: results of the National Survey of Children with Special Health Care Needs. Pediatrics 2004, 113(5 Suppl):1485-92.