National service evaluation of the quality of care for children and young people with congenital adrenal hyperplasia in the United Kingdom: survey responses from patients and clinicians.

INTRODUCTION: Quantifying differences in service provision for children and young people (CYP) living with Congenital Adrenal Hyperplasia (CAH) across the United Kingdom. METHODS: A national service evaluation using online questionnaires circulated to patients and clinicians from secondary and tertiary UK centres managing CYP with CAH, and via the "Living with CAH" support group mailing list. RESULTS: Total of 195 responses relating to patients aged 0-20 years attending 33 clinics (43 patients, 152 carers), as well as 34 clinicians from 18 trusts working across the 33 clinics. Only 12% of clinicians were 'completely satisfied' with the service provided, compared to 68% of carers and 76% of patients. Whilst 94% of clinicians reported providing formal training to families with CAH, over 80% of both patients and carers reported not attending what they considered formal training. Appetite for further training was higher in carers (86%) than patients (55%), although further 'unsure' responses suggested formal training sessions would likely be well attended. Access to psychological services was difficult for 44% of clinicians. Biochemical monitoring of treatment was broadly in keeping with international guidelines, with 67% of clinicians reporting regular use of dried blood spots, and 12% regular urinary steroid metabolites. CONCLUSION: While there is overall good satisfaction with care provision among patients and carers with CAH in the UK, extra resources addressing the psychological and educational needs about the disease and its management would benefit patients and carers. Improved access to allied health professionals and psychologists will help support families and improve patient outcomes.

Blood Pressure in Children with Congenital Adrenal Hyperplasia Due To 21-Hydroxylase Deficiency.

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) leads to impaired glucocorticoid and mineralocorticoid synthesis with excess production of androgens. Replication of the normal circadian cortisol secretion is challenging and supraphysiological doses of glucocorticoids are often required. Most patients experience transient episodes of hyper- and hypocortisolaemia during the day leading to adverse metabolic outcomes such as insulin resistance, visceral adiposity and cardiovascular morbidity, including hypertension. These health problems are commonly diagnosed in adolescence and adulthood. Herein, we review the published data on the variation in blood pressure in CAH due to 21OHD and the interrelation with disease and treatment factors.

Does current provision of undergraduate education prepare UK medical students in ENT? A systematic literature review.

OBJECTIVE: To systematically identify and analyse all published literature relating to the provision of undergraduate education for preparedness in ear, nose and throat (ENT) surgery, as perceived by medical students and clinicians in the UK. DESIGN: Systematic literature review. DATA SOURCES: 5 major databases were searched: MEDLINE, EMBASE, ERIC, Cochrane and Web of Science. The literature search was conducted from February to April 2015. ELIGIBILITY CRITERIA FOR SELECTING STUDIES: Primary research or studies that report on the provision of undergraduate education for preparedness in ENT, from the perspective of medical students and clinicians in the UK. The timescale of searches was limited from 1999 onwards (ie, the past 15 years). DATA EXTRACTION: The literature search was conducted by 2 independent reviewers. Search terms used involved the combination and variation of 5 key concepts, namely: medical student, clinician, ENT, undergraduate medical education and UK. A data extraction form was designed for and used in this study, based on guidelines provided by the UK National Health Service (NHS) Centre for Reviews and Dissemination. Textual narrative synthesis was used for data analysis. RESULTS: A total of 7 studies were included in the final review. 4 main themes were identified: confidence in managing patients, teaching delivery, student assessment and duration of rotations. A consistent finding in this review was that the majority of final year medical students and junior doctors did not feel adequately prepared to practise ENT. Important factors influencing preparedness in ENT included the duration of clinical rotations, the opportunity for hands-on learning and formal assessment. CONCLUSIONS: The findings of this review suggest the need for further development of the ENT undergraduate curricula across the UK. However, there is insufficient evidence from which to draw strong conclusions; this in itself is beneficial as it highlights a gap in the existing literature and supports the need for primary research.