Biliary Atresia in Adolescence and Adult Life: Medical, Surgical and Psychological Aspects.
Biliary Atresia in Adolescence and Adult Life: Medical, Surgical and Psychological Aspects.
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DOI：
10.3390/jcm12041594
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发表时间：
2023-02-17
影响因子：
3.9
通讯作者：
中科院分区：
文献类型：
--
作者：
在1955年之前，当Morio加塞首次进行肝门肠吻合术（现在以他的名字命名）时，胆道闭锁（BA）是一种一致的致命疾病。加塞手术和肝移植都明显改善了患有这种疾病的婴儿的前景。虽然用自体肝长期存活的情况很少，但肝移植后的存活率很高。大多数出生时患有BA的年轻人现在将存活到成年，但他们对医疗保健的持续需求将需要他们从以家庭为中心的儿科服务过渡到以患者为中心的成人服务。尽管近年来过渡服务迅速增长，过渡护理也取得了进展，但从儿科服务过渡到成人服务仍然存在临床和心理社会结果不佳以及医疗保健费用增加的风险。成人肝病学家应了解胆道闭锁的临床处理和并发症，以及儿童肝移植的长期后果。儿童疾病的幸存者需要一个不同的方法，为18岁后的年轻人提出了仔细考虑他们的情绪，社会和性健康。他们需要了解不依从的风险，无论是诊所预约还是药物治疗，以及对移植物丢失的影响。为这些年轻人提供适当的过渡期护理是基于儿童-成人界面的有效合作，是21世纪世纪儿童和成人提供者面临的重大挑战。这需要对患者和成年医生进行教育，以使他们熟悉长期并发症，特别是对于那些用自体肝脏存活的人，以及在需要时考虑肝移植的时机。这篇文章的重点是儿童胆道闭锁谁生存到青春期和成人生活的结果，考虑到他们目前的管理和预后。
Prior to 1955, when Morio Kasai first performed the hepatic portoenterostomy procedure which now bears his name, Biliary atresia (BA) was a uniformly fatal disease. Both the Kasai procedure and liver transplantation have markedly improved the outlook for infants with this condition. Although long-term survival with native liver occurs in the minority, survival rates post liver transplantation are high. Most young people born with BA will now survive into adulthood but their ongoing requirements for health care will necessitate their transition from a family-centred paediatric service to a patient-centred adult service. Despite a rapid growth in transition services over recent years and progress in transitional care, transition from paediatric to adult services is still a risk for poor clinical and psychosocial outcomes and increased health care costs. Adult hepatologists should be aware of the clinical management and complications of biliary atresia and the long-term consequences of liver transplantation in childhood. Survivors of childhood illness require a different approach to that for young adults presenting after 18 years of age with careful consideration of their emotional, social, and sexual health. They need to understand the risks of non-adherence, both for clinic appointments and medication, as well as the implications for graft loss. Developing adequate transitional care for these young people is based on effective collaboration at the paediatric–adult interface and is a major challenge for paediatric and adult providers alike in the 21st century. This entails education for patients and adult physicians in order to familiarise them with the long-term complications, in particular for those surviving with their native liver and the timing of consideration of liver transplantation if required. This article focusses on the outcome for children with biliary atresia who survive into adolescence and adult life with considerations on their current management and prognosis.
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影响因子：
4.6
作者：
Chung PHY;Chan EKW;Yeung F;Chan ACY;Mou JWC;Lee KH;Hung JWS;Leung MWY;Tam PKH;Wong KKY
通讯作者：
Wong KKY
影响因子：
1.3
作者：
Lurie, S;Shemesh, E;Shneider, B L
通讯作者：
Shneider, B L
DOI：
10.1097/mpg.0000000000003116
发表时间：
2021-07-01
影响因子：
2.9
作者：
Jain, Vandana;Burford, Charlotte;Samyn, Marianne
通讯作者：
Samyn, Marianne
影响因子：
5.1
作者：
Annunziato, Rachel A.;Bucuvalas, John C.;Shemesh, Eyal
通讯作者：
Shemesh, Eyal
影响因子：
11.1
作者：
Johansson, H.;Svensson, J. F.;Ellis, E.
通讯作者：
Ellis, E.