University Hospital for Children and Adolescents, University Hospital, University of Leipzig, Liebigstrasse 20 a, 04103 Leipzig, Germany.
J Inherit Metab Dis. 2011 Jun;34(3):701-9. doi: 10.1007/s10545-011-9284-x. Epub 2011 Feb 9.
Transition from pediatric to adult health care is a particularly vulnerable period for patients with inborn metabolic diseases. Aim of the present study was to evaluate the current transition situation of patients with phenylketonuria (PKU) in Leipzig, Germany, by analysis of the medical care, metabolic control, patients' satisfaction, socio-economic and psychosocial status, in order to identify areas of weakness and potential improvement.
Patients who had been transferred from pediatric to adult medical care between 2005 and 2008 were identified. An interview was performed using a questionnaire. Pediatric case notes and the present physician's case notes were analyzed retrospectively. Socio-demographic data were compared to data derived from the annual statistics of the city of Leipzig, Germany in 2008.
seventy two transferred patients were identified and included in the study, 48 patients responded to the questionnaire, the data of 24 non-responders were analysed retrospectively. About 90% of the responding patients with PKU were satisfied with the current transition situation. However, they agreed to several suggestions of improvement. Most specifically an interdisciplinary appointment before the definite transfer to the adult clinics was asked for. At the time of transition, most of the patients were in good metabolic control according to current treatment guidelines (median dried blood phenylalanine concentration 853 μmol/l before versus 690 μmol/l after transition). Of the interviewed patients 92% were still on a low phenylalanine diet in combination with the intake of a phenylalanine free amino acid mixture. Of the interviewees 77% carried a secondary school certificate or a secondary modern school qualification, but only 19% had achieved senior high school diploma (controls 38.2%). Marital status was comparable with the population of Leipzig. However, fewer patients with PKU had children (15% versus 37%).
Transition of patients with PKU from pediatric to adult care seems to be successful in Leipzig. Patients were mostly satisfied with the transition situation. Still, some suggestions for improvements appeared to be desirable. During transition medical care and metabolic control were stable. However, with regard to psychosocial and socioeconomic data differences to the control population were detected.
从儿科到成人保健的过渡时期对于患有先天性代谢疾病的患者来说是一个特别脆弱的时期。本研究的目的是通过分析医疗保健、代谢控制、患者满意度、社会经济和心理社会状况，来评估德国莱比锡苯丙酮尿症 (PKU) 患者的当前过渡情况，以确定薄弱环节和潜在的改进领域。
确定了 2005 年至 2008 年间从儿科转入成人医疗保健的患者。使用问卷进行了访谈。回顾性分析儿科病历和现任医生的病历。社会人口统计学数据与德国莱比锡市 2008 年的年度统计数据进行了比较。
确定了 72 名转移患者并纳入研究，48 名患者对问卷做出了回应，对 24 名未回应者的资料进行了回顾性分析。约 90%的有 PKU 的回应患者对当前的过渡情况感到满意。然而，他们同意了一些改进的建议。特别是要求在确定转入成人诊所之前进行跨学科预约。在过渡时期，根据当前的治疗指南，大多数患者的代谢控制良好（过渡前干血苯丙氨酸浓度中位数为 853 μmol/l，过渡后为 690 μmol/l）。接受采访的患者中有 92%仍在接受低苯丙氨酸饮食，同时摄入无苯丙氨酸氨基酸混合物。受访者中有 77%持有中学证书或中学现代学校资格，但只有 19%获得了高中毕业证书（对照组为 38.2%）。婚姻状况与莱比锡市的人口相当。然而，患有 PKU 的患者中只有 15%有孩子（37%）。
莱比锡 PKU 患者从儿科到成人保健的过渡似乎是成功的。患者对过渡情况大多感到满意。然而，一些改进的建议似乎是可取的。在过渡期间，医疗保健和代谢控制是稳定的。然而，在心理社会和社会经济数据方面，与对照组存在差异。
