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1.
World J Pediatr Surg ; 6(4): e000599, 2023.
Article in English | MEDLINE | ID: mdl-37860275

ABSTRACT

Background: Complications are frequently reported after hypospadias repair and there is a need to understand the factors that influence their occurrence. Methods: Data from boys with hypospadias born between 2000 and 2020 were obtained from the International Disorders of Sex Development (I-DSD) Registry. Logistic regressions, fisher's exact tests and spearman's correlation tests were performed on the data to assess associations between clinical factors and complication rates. Results: Of the 551 eligible boys, data were available on 160 (29%). Within the cohort, the median (range) External Masculinization Score (EMS) was 6 (2, 9). All presented with one or more additional genital malformation and 61 (38%) presented with additional extragenital malformations. Disorders of androgen action, androgen synthesis and gonadal development were diagnosed in 28 (18%), 22 (14%) and 9 (6%) boys, respectively. The remaining 101 (62%) patients were diagnosed as having non-specific 46,XY Disorders of Sex Development. Eighty (50%) boys had evidence of abnormal biochemistry, and gene variants were identified in 42 (26%). Median age at first hypospadias surgery was 2 years (0, 9), and median length of follow-up was 5 years (0, 17). Postsurgical complications were noted in 102 (64%) boys. There were no significant associations with postsurgical complications. Conclusions: Boys with proximal hypospadias in the I-DSD Registry have high rates of additional comorbidities and a high risk of postoperative complications. No clinical factors were significantly associated with complication rates. High complication rates with no observable cause suggest the involvement of other factors which need investigation.

2.
Expert Rev Endocrinol Metab ; 17(3): 225-234, 2022 05.
Article in English | MEDLINE | ID: mdl-35535380

ABSTRACT

INTRODUCTION: Differences and disorders of sex development (DSD) are a diverse group of conditions, which often present in early childhood and may require input from a group of experts in a wide range of clinical fields. Clinical guidance in this field recommends that these experts function as a multidisciplinary team (MDT) within which each expert has a defined role, which ensures an integrated and streamlined approach to the care of affected individuals. AREAS COVERED: This review will focus on the benefits of multidisciplinary care for people with DSD, as well as the challenges that may be faced. EXPERT OPINION: Core members of the MDT for people with DSD include endocrinologists, surgeons, psychologists, geneticists, specialist nurses, radiologists, and gynecologists, although many other health-care professionals may also be pertinent, at different stages of the patient's life. With greater acceptance of remote and digital health-care technology, there is a need to review the traditional concepts of the clinical MDT so that new care models can be explored for effective and efficient delivery of complex care.


Subject(s)
Disorders of Sex Development , Child, Preschool , Disorders of Sex Development/therapy , Humans
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