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Hormonal replacement therapy and its potential influence on working memory and competency/adaptive functioning in 47,XXY (Klinefelter syndrome).
Am J Med Genet A 2019; 179(12):2374-2381AJ

Abstract

This cross-sectional, retrospective analysis investigated the possible effect of hormonal replacement therapy (HRT) on working memory (WM) and competency/adaptive functioning (CAF) in boys with 47,XXY; the effect of timing of 47,XXY diagnosis on these variables; and the relationship between WM and CAF, if any. A total of 111 boys with 47,XXY, ranging from 6 to 16 years of age (M = 9 years 4 months; SD = 2 years 1 month), were evaluated using the Wechsler Intelligence Scale for Children, and Child Behavior Checklist. Participants were grouped by HRT status and timing of diagnosis. Analysis of variance testing performed on the prenatally diagnosed boys revealed a statistically significant difference in WM for the HRT groups (F[3,84] = 7.467, p = .000174), where WM of the no-HRT group (M = 92.37, SD = 17.83) was lower than that of the early hormonal therapy group (M = 106.39, SD = 12.01; p = .0092). Additionally, there was a positive correlation between low WM capabilities and poor school performance (r = .5106, p = .0027) in the prenatally diagnosed, untreated boys. Our results highlight the potentially positive effects of HRT on WM and CAF in boys with 47,XXY. Further research is required to better determine the underlying relationship among the biological mechanisms of HRT, WM, and CAF outcomes, and timing of diagnosis in boys with 47,XXY.

Authors+Show Affiliations

The Focus Foundation, Davidsonville, Maryland.The Focus Foundation, Davidsonville, Maryland. Department of Human and Molecular Genetics, Florida International University, Miami, Florida. Department of Pediatrics, George Washington University, Washington, District of Columbia.The Focus Foundation, Davidsonville, Maryland.Department of Economics, George Washington University, Washington, District of Columbia.Department of Neurology, George Washington University, Washington, District of Columbia. Division of Neurogenetics and Developmental Pediatrics, Children's National Health System, Washington, District of Columbia.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31577063

Citation

Tran, Selena L., et al. "Hormonal Replacement Therapy and Its Potential Influence On Working Memory and Competency/adaptive Functioning in 47,XXY (Klinefelter Syndrome)." American Journal of Medical Genetics. Part A, vol. 179, no. 12, 2019, pp. 2374-2381.
Tran SL, Samango-Sprouse CA, Sadeghin T, et al. Hormonal replacement therapy and its potential influence on working memory and competency/adaptive functioning in 47,XXY (Klinefelter syndrome). Am J Med Genet A. 2019;179(12):2374-2381.
Tran, S. L., Samango-Sprouse, C. A., Sadeghin, T., Powell, S., & Gropman, A. L. (2019). Hormonal replacement therapy and its potential influence on working memory and competency/adaptive functioning in 47,XXY (Klinefelter syndrome). American Journal of Medical Genetics. Part A, 179(12), pp. 2374-2381. doi:10.1002/ajmg.a.61360.
Tran SL, et al. Hormonal Replacement Therapy and Its Potential Influence On Working Memory and Competency/adaptive Functioning in 47,XXY (Klinefelter Syndrome). Am J Med Genet A. 2019;179(12):2374-2381. PubMed PMID: 31577063.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hormonal replacement therapy and its potential influence on working memory and competency/adaptive functioning in 47,XXY (Klinefelter syndrome). AU - Tran,Selena L, AU - Samango-Sprouse,Carole A, AU - Sadeghin,Teresa, AU - Powell,Sherida, AU - Gropman,Andrea L, Y1 - 2019/10/02/ PY - 2019/05/15/received PY - 2019/08/12/revised PY - 2019/09/05/accepted PY - 2019/10/3/pubmed PY - 2019/10/3/medline PY - 2019/10/3/entrez KW - 47,XXY KW - adaptive functioning KW - competency KW - early hormonal therapy KW - working memory SP - 2374 EP - 2381 JF - American journal of medical genetics. Part A JO - Am. J. Med. Genet. A VL - 179 IS - 12 N2 - This cross-sectional, retrospective analysis investigated the possible effect of hormonal replacement therapy (HRT) on working memory (WM) and competency/adaptive functioning (CAF) in boys with 47,XXY; the effect of timing of 47,XXY diagnosis on these variables; and the relationship between WM and CAF, if any. A total of 111 boys with 47,XXY, ranging from 6 to 16 years of age (M = 9 years 4 months; SD = 2 years 1 month), were evaluated using the Wechsler Intelligence Scale for Children, and Child Behavior Checklist. Participants were grouped by HRT status and timing of diagnosis. Analysis of variance testing performed on the prenatally diagnosed boys revealed a statistically significant difference in WM for the HRT groups (F[3,84] = 7.467, p = .000174), where WM of the no-HRT group (M = 92.37, SD = 17.83) was lower than that of the early hormonal therapy group (M = 106.39, SD = 12.01; p = .0092). Additionally, there was a positive correlation between low WM capabilities and poor school performance (r = .5106, p = .0027) in the prenatally diagnosed, untreated boys. Our results highlight the potentially positive effects of HRT on WM and CAF in boys with 47,XXY. Further research is required to better determine the underlying relationship among the biological mechanisms of HRT, WM, and CAF outcomes, and timing of diagnosis in boys with 47,XXY. SN - 1552-4833 UR - https://www.unboundmedicine.com/medline/citation/31577063/Hormonal_replacement_therapy_and_its_potential_influence_on_working_memory_and_competency/adaptive_functioning_in_47,XXY_(Klinefelter_syndrome) L2 - https://doi.org/10.1002/ajmg.a.61360 DB - PRIME DP - Unbound Medicine ER -