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首页> 外文期刊>Journal of paediatrics and child health. >Outcomes of paediatric patients with chronic liver disease in early adulthood: A heterogeneous, but representative, regional cohort study
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Outcomes of paediatric patients with chronic liver disease in early adulthood: A heterogeneous, but representative, regional cohort study

机译:Outcomes of paediatric patients with chronic liver disease in early adulthood: A heterogeneous, but representative, regional cohort study

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摘要

Aim Advances in paediatric hepatology have led to the increasing survival of patients with paediatric‐onset chronic hepatobiliary disease into adulthood. Data are lacking with regard to the outcomes of this heterogeneous group of patients and current transition models may be insufficient. This retrospective regional cohort study examined the outcomes of these patients cared for in a paediatric gastroenterology centre following transfer to adult services. Methods A prospective database of paediatric patients with liver disease identified those already transferred to adult services. Following exclusions, medical notes were examined and health parameters recorded including initial diagnoses, transplant status, fertility and mortality. Descriptive statistics were used to calculate follow‐up data and transplant‐free survival (TFS). Results Overall, 63 patients (52 male) entered the final analyses with a median follow‐up of 27.5?years. The most common diagnosis was biliary atresia (19); 27 different diagnoses were apparent within the cohort highlighting the heterogeneity within a single centre. Transplant prevalence at adult transfer was 41; 14 of patients were lost to follow‐up including 10 of transplant patients. TFS for biliary atresia was 17 after 37.4?years follow‐up and was 54 for the total cohort. There were seven documented pregnancies and the prevalence of any psychological or psychiatric input was 44. Transplant complications occurred in 38 of patients; there were two cancer diagnoses and two deaths following transfer. Conclusions Although overall mortality was low, the health‐care burden of patients with paediatric‐onset chronic liver disease is high. This group is also very heterogeneous, making structured transition to adult services difficult.

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