Klinefelter syndrome

References

Key articles

Butler G, Srirangalingam U, Faithfull J, et al. Klinefelter syndrome: going beyond the diagnosis. Arch Dis Child. 2023 Mar;108(3):166-71. Abstract

Zitzmann M, Aksglaede L, Corona G, et al. European Academy of Andrology guidelines on Klinefelter syndrome. Endorsing organization: European Society of Endocrinology. Andrology. 2021 Jan;9(1):145-67.Full text  Abstract

Gravholt CH, Chang S, Wallentin M, et al. Klinefelter syndrome: integrating genetics, neuropsychology, and endocrinology. Endocr Rev. 2018 Aug 1;39(4):389-423.Full text  Abstract

Jayasena CN, Anderson RA, Llahana S, et al. Society for Endocrinology guidelines for testosterone replacement therapy in male hypogonadism. Clin Endocrinol (Oxf). 2022 Feb;96(2):200-19.Full text  Abstract

Bhasin S, Brito JP, Cunningham GR, et al. Testosterone therapy in men with hypogonadism: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2018 May 1;103(5):1715-44.Full text  Abstract

Reference articles

1. Groth KA, Skakkebæk A, Høst C, et al. Clinical review: Klinefelter syndrome - a clinical update. J Clin Endocrinol Metab. 2013 Jan;98(1):20-30.Full text  Abstract

2. Butler G, Srirangalingam U, Faithfull J, et al. Klinefelter syndrome: going beyond the diagnosis. Arch Dis Child. 2023 Mar;108(3):166-71. Abstract

3. Zitzmann M, Aksglaede L, Corona G, et al. European Academy of Andrology guidelines on Klinefelter syndrome. Endorsing organization: European Society of Endocrinology. Andrology. 2021 Jan;9(1):145-67.Full text  Abstract

4. Gravholt CH, Chang S, Wallentin M, et al. Klinefelter syndrome: integrating genetics, neuropsychology, and endocrinology. Endocr Rev. 2018 Aug 1;39(4):389-423.Full text  Abstract

5. Gravholt CH, Ferlin A, Gromoll J, et al. New developments and future trajectories in supernumerary sex chromosome abnormalities: a summary of the 2022 3rd International Workshop on Klinefelter Syndrome, Trisomy X, and XYY. Endocr Connect. 2023 Mar 1;12(3):e220500.Full text  Abstract

6. Zhao Y, Gardner EJ, Tuke MA, et al. Detection and characterization of male sex chromosome abnormalities in the UK Biobank study. Genet Med. 2022 Sep;24(9):1909-19.Full text  Abstract

7. Ridder LO, Berglund A, Stochholm K, et al. Morbidity, mortality, and socioeconomics in Klinefelter syndrome and 47,XYY syndrome: a comparative review. Endocr Connect. 2023 May 1;12(5):e230024.Full text  Abstract

8. American College of Obstetricians and Gynecologists. Screening for fetal chromosomal abnormalities: ACOG practice bulletin, number 226. Obstet Gynecol. 2020 Oct;136(4):e48-e69Full text  Abstract

9. Dungan JS, Klugman S, Darilek S, et al. Noninvasive prenatal screening (NIPS) for fetal chromosome abnormalities in a general-risk population: an evidence-based clinical guideline of the American College of Medical Genetics and Genomics (ACMG). Genet Med. 2023 Feb;25(2):100336.Full text  Abstract

10. Johnston M, Warton C, Pertile MD, et al. Ethical issues associated with prenatal screening using non-invasive prenatal testing for sex chromosome aneuploidy. Prenat Diagn. 2023 Feb;43(2):226-34.Full text  Abstract

11. Viuff M, Skakkebæk A, Johannsen EB, et al. X chromosome dosage and the genetic impact across human tissues. Genome Med. 2023 Mar 28;15(1):21.Full text  Abstract

12. van Rijn S, Swaab H, Aleman A, et al. X chromosomal effects on social cognitive processing and emotion regulation: a study with Klinefelter men (47,XXY). Schizophr Res. 2006 Jun;84(2-3):194-203. Abstract

13. van Rijn S. A review of neurocognitive functioning and risk for psychopathology in sex chromosome trisomy (47,XXY, 47,XXX, 47, XYY). Curr Opin Psychiatry. 2019 Mar;32(2):79-84.Full text  Abstract

14. Rogol AD. Human sex chromosome aneuploidies: the hypothalamic-pituitary-gonadal axis. Am J Med Genet C Semin Med Genet. 2020 Jun;184(2):313-9. Abstract

15. Juul A, Gravholt CH, De Vos M, et al. Individuals with numerical and structural variations of sex chromosomes: interdisciplinary management with focus on fertility potential. Front Endocrinol (Lausanne). 2023;14:1160884.Full text  Abstract

16. Thirumalai A, Anawalt BD. Epidemiology of male hypogonadism. Endocrinol Metab Clin North Am. 2022 Mar;51(1):1-27. Abstract

17. Bouw N, Swaab H, Tartaglia N, et al. Early social behavior in young children with sex chromosome trisomies (XXX, XXY, XYY): profiles of observed social interactions and social impairments associated with autism spectrum disorder (ASD). J Autism Dev Disord. 2023 Aug;53(8):3194-207.Full text  Abstract

18. Davis S, Howell S, Wilson R, et al. Advances in the interdisciplinary care of children with Klinefelter syndrome. Adv Pediatr. 2016 Aug;63(1):15-46. Abstract

19. van Rijn S, de Sonneville L, Swaab H. The nature of social cognitive deficits in children and adults with Klinefelter syndrome (47,XXY). Genes Brain Behav. 2018 Jul;17(6):e12465.Full text  Abstract

20. Tartaglia N, Ayari N, Howell S, et al. 48,XXYY, 48,XXXY and 49,XXXXY syndromes: not just variants of Klinefelter syndrome. Acta Paediatr. 2011 Jun;100(6):851-60. Abstract

21. St John M, Ponchard C, van Reyk O, et al. Speech and language in children with Klinefelter syndrome. J Commun Disord. 2019 Mar-Apr;78:84-96. Abstract

22. Butler G. Incidence of gynaecomastia in Klinefelter syndrome adolescents and outcome of testosterone treatment. Eur J Pediatr. 2021 Oct;180(10):3201-7. Abstract

23. Jayasena CN, Anderson RA, Llahana S, et al. Society for Endocrinology guidelines for testosterone replacement therapy in male hypogonadism. Clin Endocrinol (Oxf). 2022 Feb;96(2):200-19.Full text  Abstract

24. Sánchez XC, Montalbano S, Vaez M, et al. Associations of psychiatric disorders with sex chromosome aneuploidies in the Danish iPSYCH2015 dataset: a case-cohort study. Lancet Psychiatry. 2023 Feb;10(2):129-38. Abstract

25. Kreukels BPC, Köhler B, Nordenström A, et al. Gender dysphoria and gender change in disorders of sex development/intersex conditions: results from the dsd-LIFE dtudy. J Sex Med. 2018 May;15(5):777-85. Abstract

26. Aksglaede L, Skakkebaek NE, Almstrup K, et al. Clinical and biological parameters in 166 boys, adolescents and adults with nonmosaic Klinefelter syndrome: a Copenhagen experience. Acta Paediatr. 2011 Jun;100(6):793-806. Abstract

27. Rose NC, Barrie ES, Malinowski J, et al. Systematic evidence-based review: the application of noninvasive prenatal screening using cell-free DNA in general-risk pregnancies. Genet Med. 2022 Jul;24(7):1379-91.Full text  Abstract

28. Badeau M, Lindsay C, Blais J, et al. Genomics-based non-invasive prenatal testing for detection of fetal chromosomal aneuploidy in pregnant women. Cochrane Database Syst Rev. 2017 Nov 10;11(11):CD011767.Full text  Abstract

29. Ramasamy R, Chiba K, Butler P, et al. Male biological clock: a critical analysis of advanced paternal age. Fertil Steril. 2015 Jun;103(6):1402-6.Full text  Abstract

30. Jacobs PA, Hassold TJ, Whittington E, et al. Klinefelter's syndrome: an analysis of the origin of the additional sex chromosome using molecular probes. Ann Hum Genet. 1988 May;52(2):93-109. Abstract

31. De Souza E, Morris JK, EUROCAT Working Group. Case-control analysis of paternal age and trisomic anomalies. Arch Dis Child. 2010 Nov;95(11):893-7. Abstract

32. Bhasin S, Brito JP, Cunningham GR, et al. Testosterone therapy in men with hypogonadism: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2018 May 1;103(5):1715-44.Full text  Abstract

33. Linden MG, Bender BG, Robinson A. Sex chromosome tetrasomy and pentasomy. Pediatrics. 1995 Oct;96(4 pt 1):672-82. Abstract

34. Beighton P, de Paepe A, Danks D, et al. International nosology of heritable disorders of connective tissue, Berlin, 1986. Am J Med Genet. 1988 Mar;29(3):581-94. Abstract

35. De Paepe A, Devereux RB, Dietz HC, et al. Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet. 1996 Apr 24;62(4):417-26. Abstract

36. Hannema SE, Sävendahl L. The evaluation and management of tall stature. Horm Res Paediatr. 2016;85(5):347-52.Full text  Abstract

37. British Society for Paediatric Endocrinology and Diabetes. BSPED guideline: testosterone therapy in infancy and adolescence. April 2019​ [internet publication].Full text

38. Stancampiano MR, Lucas-Herald AK, Russo G, et al. Testosterone therapy in adolescent boys: the need for a structured approach. Horm Res Paediatr. 2019;92(4):215-28.Full text  Abstract

39. Vogiatzi M, Tursi JP, Jaffe JS, et al. Testosterone use in adolescent males: current practice and unmet needs. J Endocr Soc. 2021 Jan 1;5(1):bvaa161.Full text  Abstract

40. Corona G, Pizzocaro A, Lanfranco F, et al. Sperm recovery and ICSI outcomes in Klinefelter syndrome: a systematic review and meta-analysis. Hum Reprod Update. 2017 May 1;23(3):265-75.Full text  Abstract

41. Deebel NA, Galdon G, Zarandi NP, et al. Age-related presence of spermatogonia in patients with Klinefelter syndrome: a systematic review and meta-analysis. Hum Reprod Update. 2020 Jan 1;26(1):58-72.Full text  Abstract

42. Swerdlow AJ, Higgins CD, Schoemaker MJ, et al. Mortality in patients with Klinefelter syndrome in Britain: a cohort study. J Clin Endocrinol Metab. 2005 Dec;90(12):6516-22.Full text  Abstract

43. Bojesen A, Juul S, Birkebaek NH, et al. Morbidity in Klinefelter syndrome: a Danish register study based on hospital discharge diagnoses. J Clin Endocrinol Metab. 2006 Apr;91(4):1254-60.Full text  Abstract

Use of this content is subject to our disclaimer