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Screening for comorbidities lacking in young girls with Turner’s syndrome
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2010 Pediatric Academic Societies Annual Meeting
Despite changes that have occurred in medical practice after the implementation of international guidelines for Turner’s syndrome, screening for associated comorbidities was deficient in more than 50% of young girls with the disease.
Zeina M. Nabhan, MD, and Erica A. Eugster, MD, both of the department of pediatric endocrinology and diabetology at Indiana University School of Medicine, reviewed the medical records of 124 girls with Turner’s syndrome (mean age, 13.2 years). The girls were followed for a mean of six years in a pediatric endocrinology clinic at a tertiary care center with a focus on changes in management since 2007. Average age at diagnosis was 4.1 years.
The researchers presented the data at a Lawson Wilkins Pediatric Endocrine Society session at the 2010 Pediatric Academic Societies Annual Meeting.
The review revealed that hearing tests were performed in 51% of the girls, thyroid screening in 95% and blood pressure measurement in 100%.
Before 2007, no girl was screened for celiac disease, liver disease, lipids, routine electrocardiography or cardiac MRI. However, after implementation of the guidelines in 2007, 62% of girls were tested for celiac disease, 50% had liver screening and 10% had lipid levels measured. ECG was performed in 19% of girls and a cardiac MRI was obtained in 32% of girls aged 10 years and older. Bovine aortic arch, mild to moderate aortic root/ascending aortic dilatation and elongation of transverse aortic arch were noted on the MRI; routine ECG did not detect these findings. Primary ovarian failure was present in 87% of the girls.
The average age of estrogen replacement was 14 years. Conjugated equine oral estrogen was the main mode of estrogen replacement in 65% of girls. A significant increase was found in the use of transdermal estrogen after 2007 — 78% of girls were treated with transdermal estrogen vs. 10% of girls before 2007. Age at initiation of estrogen replacement therapy remain unchanged before and after 2007.
“Special emphasis should be directed at surveillance for hyperlipidemia, liver abnormalities and cardiac health — particularly in older girls,” the researchers said.
This study highlights the importance of ongoing education for physicians caring for girls with Turner's syndrome, as screening for comorbidities was lacking in more than 50% of this cohort. Screening for cardiovascular comorbidities is especially important, as up to 1% of girls with Turner's syndrome are estimated to develop aortic dissection. Although all girls had blood pressure screening, only 32% received cardiac imaging with MRI. In addition, conjugated equine oral estrogen was the mode of estrogen therapy in the majority of patients despite potential advantages of transdermal estradiol, including positive effects on linear growth, bone accrual, uterine growth and development of secondary sexual characteristics. Since the implementation of guidelines in 2007, there have been significant improvements in screening practices and the use of transdermal estradiol. Prospective studies on the prevention of associated comorbidities are needed.
– Becky Fudge, MD
Division of Pediatric Endocrinology, University of North Carolina
For more information:
- Nabhan ZM. #1455.47. Presented at: 2010 Pediatric Academic Societies Annual Meeting; May 1-4, 2010; Vancouver, British Columbia.
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