Successful transition for adolescent survivors of CHD: the Cambridge cardiac transition pathway.

PURPOSE: National standards to ensure effective transition and smooth transfer of adolescents from paediatric to adult services are available but data on successful transition in CHD are limited. The aim of this study is to assess the effectiveness of our transition pathway. METHODS: Adolescents with CHD, aged 15-19 years, who attended the joint cardiac transition clinic between 2009 and 2018 were identified from the Patient Administration Systems. Patient attendance at their first adult CHD service appointment at Royal Papworth Hospital was recorded. RESULTS: 179 adolescents were seen in the joint cardiac transition clinic in the 9-year study period. The median age of the patients when seen was 16 (range 15-19) years. 145 patients were initially planned for transfer to the Royal Papworth Hospital adult CHD service. Three patients were subsequently excluded and the success of the transfer of care in 142 patients were analysed. 112 (78%) attended their first follow-up in the adult CHD clinic as planned, 28 (20%) attended after reminders were sent out with 5/28 requiring multiple reminders, and only 2 (1.4%) failed to attend. Overall, transfer of care was achieved in 140 (98.6%) patients. CONCLUSION: A dedicated joint cardiac transition clinic involving multi-professional medical and nursing teams from paediatric and adult cardiology services appears to achieve high engagement rates with the adult services. This approach allows a 'face' to be put on a named clinician delivering the adult service and should be encouraged.

Potpourri of retinopathies in rare eye disease - A case series.

This case series describes the ocular and retinal manifestations of rare eye diseases in systemic syndromes. This observational case series consists of five patients with varied ophthalmic manifestations and documentation of imaging in rare pediatric and adult retinopathies. Two patients had Kearns Sayre syndrome (KSS) based on the classical triad of external ophthalmoplegia, pigmentary retinopathy, and onset before 20 years of age. In one patient of KSS, the mitochondrial retinopathy was seen in an asymmetric pattern, and the second patient presented with KSS after being mis-diagnosed as myasthenia gravis elsewhere. A case of Senior Loken syndrome in pediatric age is described in this series with varied ophthalmic manifestations ranging from retinitis pigmentosa to orbital abscess. This series also enlightens features of Hallervorden Spatz syndrome presenting with bull's eye maculopathy and a case of spino-cerebellar ataxia type 7 presenting with pigmentary retinopathy.

A positive BAL galactomannan in non-haemato-oncology patients risks harmful overtreatment.

Introduction. Evidence for the clinical utility of bronchoalveolar lavage (BAL) galactomannan in the management of fungal disease outside of haemato-oncology patients is limited.Aim. To determine how the introduction of BAL galactomannan testing impacted on the diagnosis and management of invasive aspergillosis and other fungal diseases in non-haemato-oncology patients.Methodology. Retrospective review of all adult patients (age ≥16 years) without a diagnosis of haematological malignancy who had a positive BAL galactomannan from 1 November 2014 to 30 April 2018. Using electronic patient records we obtained demographic data, clinical details, laboratory investigations, relevant radiology and antimicrobial history for each case.Results. In total, 121 episodes with a galactomannan OD index of ≥0.500 were included in the study; 29 cases (24 %) were felt to reflect fungal disease. Antifungal therapy was commenced as a direct consequence of a positive BAL galactomannan result in 13 patients where the ultimate diagnosis was subsequently considered to be non-mycological: associated medication-related side-effects in this group included deranged liver function tests (n=3), rash (n=1) and fever (n=1), related to amphotericin B (n=1) and voriconazole (n=4).Conclusion. We show that vigilance is required when interpreting galactomannan results in non-haematology patients to avoid potentially harmful overtreatment.