Browsing by Subject "congenital adrenal hyperplasia"
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Item Characteristics of Female Genital Restoration Surgery for Congenital Adrenal Hyperplasia Using a Large-scale Administrative Database(Elsevier, 2018) Roth, Joshua D.; Casey, Jessica T.; Whittam, Benjamin M.; Bennett, William E., Jr.; Szymanski, Konrad M.; Cain, Mark P.; Rink, Richard C.; Urology, School of MedicineObjective To analyze nationwide information on the timing of surgical procedures, cost of surgery, hospital length of stay following surgery, and surgical complications of female genital restoration surgery (FGRS) in females with congenital adrenal hyperplasia (CAH). Materials and Methods We used the Pediatric Health Information System database to identify patients with CAH who underwent their initial FGRS in 2004-2014. These patients were identified by an International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for adrenogenital disorders (255.2) in addition to a vaginal ICD-9 procedure code (70.x, excluding vaginoscopy only) or perineal ICD-9 procedure code (71.x), which includes clitoral operations (71.4). Results A total of 544 (11.8%) females underwent FGRS between 2004 and 2014. Median age at initial surgery was 9.9 months (interquartile range 6.8-19.1 months). Ninety-two percent underwent a vaginal procedure, 48% underwent a clitoral procedure, and 85% underwent a perineal procedure (non-clitoral). The mean length of stay was 2.5 days (standard deviation 2.5 days). The mean cost of care was $12,258 (median $9,558). Thirty-day readmission rate was 13.8%. Two percent underwent reoperation before discharge, and 1 (0.2%) was readmitted for a reoperation within 30 days. Four percent had a perioperative surgical complication. Conclusion Overall, 12% of girls with CAH underwent FGRS at one of a national collaborative of freestanding children's hospitals. The majority underwent a vaginoplasty as a part of their initial FGRS for CAH. Clitoroplasty was performed on less than half the patients. Overall, FGRS for CAH is performed at a median age of 10 months and has low 30-day complication and immediate reoperation rates.Item Infants With Congenital Adrenal Hyperplasia Are at Risk for Hypercalcemia, Hypercalciuria, and Nephrocalcinosis(Endocrine Society, 2017-08-01) Schoelwer, Melissa J.; Viswanathan, Vidhya; Wilson, Amy; Nailescu, Corina; Imel, Erik A.; Pediatrics, School of MedicineIn a retrospective study, most young children with CAH had at least one episode of hypercalcemia, whereas a smaller percentage was found to have hypercalciuria and/or nephrocalcinosis.Item Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex(Elsevier, 2021-04) Szymanski, Konrad M.; Rink, Richard C.; Whittam, Benjamin; Hensel, Devon J.; Life with Congenital Adrenal Hyperplasia Study Group; Urology, School of MedicineIntroduction To assess opinions of females with CAH, and parents of females with CAH, about designating this population “intersex,” particularly in legislation about genital surgery during childhood. Methods We conducted a mixed-methods (quantitative and qualitative) anonymous cross-sectional online survey of females with CAH (46XX, 16+years old) and independently recruited parents of girls with CAH (2019–2020) diagnosed in first year of life from the United States. A multidisciplinary CAH team drafted the survey in collaboration with women with CAH and parents. Fisher's exact test was used to compare female and parent responses. A qualitative thematic approach was used to analyze open-ended answers for emergent categories of reasons why CAH females should or should not be considered as intersex. Results Of 57 females with CAH participating (median age: 39 years, 75.5% of ≥25year olds had post-secondary degree), all had classical CAH and 93.0% underwent genital surgery at median 1–2 years old. While 89.5% did not endorse the intersex designation for CAH, the remaining 5.3% did (5.3% provided no answer, Summary Figure). Most CAH females (63.2%) believed CAH females should be considered separately in “any laws banning or allowing surgery of children's genitals” (19.3% disagreed, 17.5% neutral, 0.0% no answer). Most common themes identified by females with CAH not endorsing an intersex designation were: normal female internal organs, sex chromosomes, personal identity, genital appearance, issues with language, hormones, and those endorsing it: genital appearance, community/group experiences, topic complexity. Overall, 132 parents of females with CAH participated (parent/child median ages: 40/11 years, 81.7% of ≥25year olds had post-secondary degree). All children had classical CAH and 78.8% underwent surgery at median <1 year old. While 95.5% of parents did not endorse the intersex designation for CAH, 2.3% did (2.3% no answer), similar to females (p = 0.29). Most parents (81.1%) believed CAH females should be considered separately in legislation (9.1% disagreed, 6.1% neutral, 3.8% no answer), a slightly higher percentage than females (p = 0.01). Discussion Echoing previously published disagreement with clinically designating CAH females as intersex, majority of CAH females and parents oppose a legal intersex designation. Differing opinions among females and parents strengthen concern about a one-size-fits-all approach to legislation about childhood genital surgery. Differences in opinions between female and parent responses, while statistically significant, were relatively small. Conclusion: Majority of females with CAH and their parents believe CAH should be excluded from the intersex designation and be considered separately in legislation pertaining to childhood genital surgery in childhood.Item Peripheral precocious puberty including congenital adrenal hyperplasia: causes, consequences, management and outcomes(Elsevier, 2019) Haddad, Nadine G.; Eugster, Erica A.; Pediatrics, School of MedicinePeripheral precocious puberty results from peripheral production of sex steroids independent of activation of the hypothalamic-pituitary gonadal axis. It is much less common than central precocious puberty. Causes are variable and can be congenital or acquired. In this review, we will discuss the diagnosis and management of the most common etiologies including congenital adrenal hyperplasia, McCune Albright syndrome, familial male-limited precocious puberty, and adrenal and gonadal tumors.Item Response to commentary re ‘Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex'(Elsevier, 2021-04) Szymanski, Konrad M.; Whittam, Benjamin; Rink, Richard C.; Pediatrics, School of MedicineItem Treatment of the Enlarged Clitoris(Frontiers, 2017-08-28) Kaefer, Martin; Rink, Richard C.; Urology, School of MedicineManagement of the enlarged clitoris, because of its import for sexual function, has been and remains one of the most controversial topics in pediatric urology. Early controversy surrounding clitoroplasty resulted from many factors including an incomplete understanding of clitoral anatomy and incorrect assumptions of the role of the clitoris in sexual function. With a better understanding of anatomy and function, procedures have evolved to preserve clitoral tissue, especially with respect to the neurovascular bundles. These changes have been made in an effort to preserve clitoral sensation and preserve orgasmic potential. It is the goal of this manuscript to describe the different procedures that have been developed for the surgical management of clitoromegally, with emphasis on the risks and benefits of each. Equally important to any discussion of such a sensitive topic is an understanding of long-term patient outcomes. As we will see, despite its importance, there has been a dearth of data in this regard. Future work in the arena of patient satisfaction will undoubtedly play a major role in directing our surgical approach.