Background: Turner syndrome (TS) is associated with significant morbidity and a reduction in life expectancy. National guidelines have been developed to support the management of adult patients with TS but little is known about their implementation. We therefore sought to compare the management of patients with TS seen within a single tertiary referral centre by endocrinology, either general-purpose or Turner-specific clinics (TSC), or by other specialties.
Methods: Authorisation was provided by University Hospitals Birmingham (UHB) Clinical Governance Department. All patients coded with TS and seen on an outpatient basis within the last two calendar years were identified via informatics or through a manual search of clinic letters. Data relating to specific management standards covering annual and 5-yearly investigations were extracted from electronic records, including routine observations and laboratory investigations, use of imaging modalities and autoimmune screening.
Results: Data was extracted for 85 patients, 38 from TSC, five from general endocrine clinics, 11 from cardiology, and one from ENT. All patients within the TSC had BMI and BP measured, compared to 97% in general clinics and 58% cardiology/ENT. Excluding unsuitable patients, 92% of TSC patients were on HRT vs 82 and 66% in general endocrinology and other specialities respectively. 97.4% of patients in the TSC had thyroid function checked in the last year (85.7% in general and 8.3% in other), with 68% of patients also having their 5-yearly TPO antibodies. Echocardiograms were used extensively by all clinics, but 92% of TSC patients had received a cardiac MRI in the past 5 years, vs 63.6% seen by cardiology.
Conclusion: Patients attending specialist TS clinics were more likely to receive management in line with national guidance than those seen outside these clinics. Hormone profiling and cardiovascular imaging more frequently met guidance in Turner-specific clinics than cardiology clinics.