Available online 19 August 2022

Turner syndrome is associated with multiple multiorgan comorbidities

Adherence to screening guidelines is vital to prevent undue morbidity and mortality

Comorbidity screening in Turner syndrome remains suboptimal in pediatrics

Multidisciplinary clinic is recommended and may facilitate ease of screening

To evaluate the 2016 Cincinnati International Turner syndrome (TS) consensus guideline adherence within our pediatric tertiary referral center and to determine if patients managed in our single day, coordinated multidisciplinary clinic (MDC) format showed superior adherence rates compared to those managed outside our MDC format.

We retrospectively reviewed the charts of TS patients followed at our center between January 1, 2018, and April 30, 2020. The individual and overall adherence rates of nine age-appropriate screening recommendations were evaluated. Rates of TS comorbidities within our cohort were also evaluated.

A total of 111 girls met study criteria. Sixty-eight were managed in the MDC and 43 were managed outside the MDC. Only 42% of all the girls met all nine age-appropriate screening recommendations evaluated, 33/47 (70%) of whom were managed in MDC compared to 14/47 (30%) in the non-MDC. Girls managed in the MDC had higher screening adherence rates vs non-MDC girls for 7 of the 9 screens evaluated, with especially large differences noted for TSH (95% vs 78%, p-value 0.034), auditory evaluation (97% vs 65%, p-value <0.001), and HgA1c levels (82% vs 54%, p-value 0.014).

Girls managed in the MDC format showed higher rates of screening guideline adherence, both overall and with multiple specific screening tests, compared to those managed outside the MDC format. Overall guideline adherence remained low (42%), highlighting the need for continued optimization and improvement in guideline adherence in this unique population.

Turner syndrome

pediatrics

Multidisciplinary clinic

Fluorescence in situ hybridization

Thyroid stimulating hormone

Aspartate transaminase

Gamma-glutamyl transferase

Tissue transglutaminase

© 2022 Published by Elsevier Inc. on behalf of the AACE.