Survival rates are improving following cancer therapy for childhood brain tumours. There is therefore a growing cohort of survivors at risk of late effects of cancer therapy. Endocrine problems are very common in these patients. The recognition and prompt management of these are essential to prevent further morbidity and impairment of quality of life.Cranial radiation can damage hypothalamic-pituitary function, most frequently affecting GH status; however, higher radiation doses may cause more widespread hypothalamic-pituitary damage. Early puberty secondary to cranial irradiation is now being managed with gonadotrophin-releasing hormone analogues to improve final height. Prompt diagnosis and management of GH deficiency may improve final height outcome; continued GH therapy beyond final height aids the achievement of adult body composition (lean body mass and bone mass) and GH therapy in adulthood improves quality of life. Both cranial irradiation alone and with spinal irradiation can result in radiation damage to the thyroid resulting in hypothyroidism and thyroid nodules, a high proportion of which are malignant. Gonadal damage secondary to spinal irradiation and adjuvant chemotherapy may have long-term consequences including infertility.
Since 1988, when a retrospective study of patients attending this unit was published, we have advocated the use of the short synacthen test (SST) as the primary screening investigation to detect ACTH deficiency. However, others have published comparisons of SST and insulin tolerance tests that suggest a significant false negative rate with SST, leading to concern that some patients who pass the SST are in danger from the clinical consequences of ACTH deficiency. To address this, we audited biochemical results and clinical outcome in 63 patients who did not have ACTH deficiency detected (i.e. who passed the test) by SST after pituitary surgery. Twelve of the 63 patients who passed a SST after pituitary surgery became ACTH-deficient later as diagnosed by SST: 4 within the first year, 2 of whom had received postoperative radiotherapy (3 had symptoms of tiredness and 1 was admitted to the hospital with a viral infection); 8 in yr 3-5, 7 of whom had received postoperative radiotherapy (all had either no symptoms or symptoms of tiredness alone). Thus, the predictive value of the SST in excluding ACTH deficiency is approximately 97% (2 of 63 patients who initially passed the SST were found to be ACTH-deficient within 12 months without having received postoperative radiotherapy). Only 1 patient was ill enough to require hospital admission. Setting the risk of false negatives with SST against the morbidity and manpower implications associated with insulin tolerance tests, SST remains the primary screening test for ACTH deficiency in our practice. However, a high index of clinical suspicion to detect false negative results must be maintained.
The lack of focus on young people as a group with particular healthcare needs in medical training and the health service underpins the difficulty that we have experienced as a profession in improving transition in the UK. This article discusses current progress towards improving training in young people's health in the UK, the evidence base for transitional care in young people with chronic conditions with interventions that focus on staffing, service delivery and young people, a practice based approach for transitional care in young people with learning difficulties and complex needs, the need for monitoring and evaluation of transitional care, and the challenge of funding.
Half of all young people with CAH referred to specialist adult services are no longer attending. Introducing the adult endocrinologist prior to transfer had no positive effect on engagement with adult services. Attendance at the first two appointments in the adult services should be seen as an indicator of 'reasonable' engagement.
Background During transition from children's to adults' healthcare, young adults with long-term conditions may show delays in psychosocial development compared to their peers without long-term conditions, and deterioration of their conditions' medical control. Methods This paper integrates the findings, already published in 10 separate papers, of a 5-year transition research programme. Implications There is an important role for funders (commissioners) of adults' services to fund transitional healthcare, in addition to funders of children's services who currently take responsibility.
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