Paeds Vivas · genetics-dysmorphology-and-metabolism
Klinefelter syndrome and sex chromosome aneuploidy — branching viva
Branching viva on Klinefelter syndrome and sex chromosome aneuploidy: recognising the clinical pattern across the lifespan, the extra-X gene-dosage model and X-inactivation escape genes, the endocrine trajectory and testosterone replacement, the neurodevelopmental and psychosocial phenotype, the prenatal counselling cascade, and the lifespan surveillance framework.
On this page
Study tools
Target exams
Opening question
A tall, shy fourteen-year-old boy is referred because he has not started puberty. On examination his testicular volume is 4 mL bilaterally — small and firm — and he has mild gynaecomastia and a history of language delay and learning difficulties. How do you confirm the diagnosis, and why is the karyotype essential rather than a microarray alone? [1] [2]
References4ShowHide
- [1]Groth KA, Skakkebæk A, Høst C, Gravholt CH, Bojesen A. Clinical review: Klinefelter syndrome--a clinical update. J Clin Endocrinol Metab, 2013.PMID 23118429
- [2]Lanfranco F, Kamischke A, Zitzmann M, Nieschlag E. Klinefelter's syndrome. Lancet, 2004.PMID 15262106
- [3]Kanakis GA, Nieschlag E. Klinefelter syndrome: more than hypogonadism. Metabolism, 2018.PMID 29382506
- [4]Gies I, Unuane D, Velkeniers B, De Schepper J. Management of Klinefelter syndrome during transition. Eur J Endocrinol, 2014.PMID 24801585