Endocrine Abstracts (2008) 15 S56

The management of Turner Syndrome from paediatric to adult care

Gerard Conway


University College London, London, UK.


Until recently it was usual to discharge teenagers with TS to primary care once final height had been attained in the paediatric setting. It is now clear that adults with TS benefit from dedicated TS clinics in order to optimise health care for this commonly neglected group. In adult life there is a great deal of preventable morbidity in TS which lies within the endocrine remit: osteoporosis, hypothyroidism, obesity, diabetes, hyperlipidaemia and hypertension. Other disorders such as congenital cardiovascular disease, sensorineural deafness and the need for assisted reproduction and psychological support may require the input of a multidisciplinary team. Co-ordination between paediatric and adult physicians is essential for the smooth hand-over of care allowing lessons learned in each field to be shared. For instance, we are beginning to learn that the seeds of optimal quality of life and fertility outcome with ovum donation might be set in paediatric timing of oestrogen replacement.

The rapidly expanding knowledge of late pathology raises new insecurities. The relationship between raised liver enzymes in young life and cirrhosis in older women is yet to be clarified. The optimal scheme for monitoring aortic root dilatation and preventing aortic dissection is unknown. Our ability to fulfil expectations of fertility through egg donation may not be as successful as first thought. For all these reasons an organised programme of health care, similar to that which exists in diabetes, is essential for young adults with TS.

Reference

1. Bondy CA. Care of girls and women with Turner syndrome: A guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab 2007 92 10–25.

2. Sybert VP & McCauley E. Turner’s syndrome. NEJM 2004 351 1227–1238.

2. Ostberg JE, Storry C, Donald AE, Attar MJ, Halcox JP & Conway GS. A dose-response study of hormone replacement in young hypogonadal women: effects on intima media thickness and metabolism. Clin Endocrinol 2007 66 557–564.

4. Ostberg JE, Donald AE, Halcox JP, Storry C, McCarthy C & Conway GS. Vasculopathy in Turner syndrome: arterial dilatation and intimal thickening without endothelial dysfunction. J Clin Endocrinol Metab 2005 90 5161–5166.

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