Paeds Cases · genetics-dysmorphology-and-metabolism
Counsel a family on a new Prader-Willi diagnosis and the trajectory ahead — OSCE
OSCE communication and shared decision-making station: explaining to parents what a new Prader-Willi syndrome diagnosis means for their hypotonic two-week-old neonate, why a normal karyotype did not detect it, what methylation testing involves, what the syndrome-specific management looks like including growth hormone therapy, why the molecular subtype matters for family recurrence risk, and what the trajectory from neonatal feeding failure to hyperphagia and obesity will look like — while addressing grief, guilt, fear about the future, and the demand for a cure.
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Counsel the parents. You have five minutes. Demonstrate an organised, empathic, and accurate explanation that addresses the five questions a fellowship communication station rewards: what the diagnosis means and why the earlier test missed it, what the trajectory looks like (neonatal feeding failure to hyperphagia), what the plan is for their son (growth hormone, dietary control, behavioural support), what the molecular subtype means for recurrence risk, and the honest truth about cure. The management framework follows the GeneReviews clinical guideline and the PWS growth hormone consensus. [1]
References4ShowHide
- [1]Driscoll DJ, Miller JL, Cassidy SB Prader-Willi Syndrome. GeneReviews, 1993.PMID 20301505
- [3]Cassidy SB. Prader-Willi and Angelman syndromes. Disorders of genomic imprinting. Medicine, 1998.PMID 9556704
- [4]Beygo J, et al. EMQN/ACGS best practice guidelines for molecular analysis of PWS and AS. Eur J Hum Genet, 2019.PMID 31235867
- [7]Koch L. Consensus guidelines for GH therapy in Prader-Willi syndrome. Nat Rev Endocrinol, 2013.PMID 23609333