BackgroundPrimary systemic vasculitis presenting in childhood is an uncommon but serious condition. As these patients transfer to adult clinics for continuing care, defining long term outcomes with emphasis on disease and treatment- related morbidity and mortality is important. The aim of this study is to describe the long- term clinical course of paediatric patients with ANCA vasculitis.MethodsThe adult patients in our vasculitis clinics who had presented in childhood, with a follow up time of greater than 10 years were included. We also reviewed the literature for articles describing the clinical outcome of paediatric patients with ANCA vasculitis.ResultsWe describe the clinical course of 8 adults who presented in childhood with ANCA vasculitis. 7 patients had Wegener's granulomatosis and 1 had microscopic polyangiitis. The median age at presentation was 11.5 years, and follow up time ranged form 11 to 30 years. Induction therapy for all patients was steroids and/or cyclophosphamide. Maintenance therapy was with azathioprine or mycophenolate mofetil. Biological agents were used in 3 patients for relapsed disease in adulthood only.Seven patients achieved complete remission. All patients experienced disease relapse, with a median of 4 episodes. Kidney function was generally well preserved, with median eGFR 76 ml/min. Only one patient developed end-stage renal failure and one patient died after 25 years of disease. Treatment-related morbidity rates were high; 7 suffered from infections, 4 were infertile, 2 had skeletal complications, and 1 developed malignancy.ConclusionClose long- term follow up of paediatric patients with ANCA vasculitis is imperative, as this patient cohort is likely to live long enough to develop significant treatment and disease- related morbidities. Prospective cohort studies with novel therapies including paediatric patients are crucial to help us determine the best approach to managing this complex group of patients. In addition, although not yet observed in our series, late cardiovascular morbidity remains a major longer-term potential concern for adult survivors of paediatric vasculitis.
Introduction Whilst excision biopsy is traditionally preferred, advances in radiological and histological techniques warrant a re‐look at core biopsy as a viable primary diagnostic method. Method Over a 3‐year period, all patients who underwent core biopsy to investigate lymphoma at our centre were included. Results 554 consecutive patients were included (40.1% prior lymphoma and 59.4% new presentations). Three or more cores were taken in 420 (75.8%) cases. Median time from request to biopsy and biopsy to histology report was 2 (0‐40) days and 7 (1‐24) days, respectively. 510/544 (93.8%) biopsies were diagnostic. There was no difference in whether the biopsy was diagnostic based on indication (new vs. relapsed lymphoma) (P = .445), whether biopsy was PET‐directed (P = .507), for T‐cell lymphoma (P = .468) or nodal vs. extra‐nodal (P = .693). Thirty‐eight patients (6.9%) required a second biopsy due to inadequate tissue. In a patient experience survey, only 13.9% reported any complications (1 self‐limiting minor bleeding, 4 bruising) whilst 16.7% reported any discomfort beyond 12 hours. Conclusion Core biopsy performed by experienced radiologists and analysed by expert haemato‐pathologists is a reliable, well‐tolerated method for diagnosing lymphoma and confirming relapse. Multiple cores can be obtained under local anaesthetic yielding sufficient material in the majority of cases.
Objective: To report on the use of RFA for the treatment of symptomatic benign and autonomously functioning thyroid nodules (AFTNs) in the first reported UK cohort. Methods: Patients treated over a 19-month period were retrospectively reviewed. Nodules were assessed pre-treatment and at 1 and 6 months post-treatment. Nodule volume was calculated and cosmetic assessment and thyroid-related quality of life (QoL) scores were recorded at each time point. Thyroid function tests (TFTs) were recorded at all three time points for patients with ATFNs. Results: 46 patients with 50 nodules were treated with no complications. The mean volume reduction 1-month post-treatment was 53 +- 14.9 % ( p < 0.0001). Six month data was available for 31 nodules and showed a mean 67 +- 17.6% vol reduction ( p < 0.0001). Five of the six patients with ATFNs were euthyroid at 1-month post-procedure. 6-month data was available on three of these patients, and all remained euthyroid. The thyroid-related QoL and cosmetic scores also improved. Data from 23 patients was available pre-treatment and at 6 months post-treatment and there was a significant ( p < 0.0001) reduction in QoL score. Pre-treatment, 82 % of nodules were readily visible at rest, decreasing to 12.5 % 6 months after treatment ( p < 0.0001). Conclusions: Results align with published data suggesting that RFA is effective at reducing nodule volume and at treating ATFNs and leads to improvement in thyroid-related QoL and cosmetic scores. Advances in knowledge: This early UK experience demonstrates that day-case radiofrequency ablation can provide safe and effective treatment of benign symptomatic thyroid nodules.
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