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Wilson disease: What is still unclear in pediatric patients?

机译:威尔逊病:小儿患者尚不清楚什么?

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Since Wilson disease (WD) may not be present with evident clinical symptoms of liver injury and neurological presentation is rare in children, establishing a diagnosis is often challenging, especially in childhood. Increased transaminases can be the only abnormality found in early course of WD. In clinical practice, high suspicion is crucial for early diagnosis and timely treatment to ensure better outcomes. Conventional diagnostic criteria established for adults are commonly agreed for children but may not always be appropriate in very young age. Currently, the best therapeutic approach for each specific presentation of the disease remains controversial and there are no clear indications about how to treat pediatric WD patients with a mild liver disease.
机译:由于可能不会出现威尔逊病(WD)并伴有明显的肝损伤临床症状,并且在儿童中很少出现神经系统表现,因此尤其是在儿童时期,确定诊断通常具有挑战性。转氨酶升高可能是WD早期发现的唯一异常。在临床实践中,高度怀疑对于早期诊断和及时治疗以确保更好的结局至关重要。为成人建立的常规诊断标准通常适用于儿童,但在很小的年龄可能并不总是合适的。目前,针对该疾病的每种具体表现的最佳治疗方法仍存在争议,并且没有明确的迹象表明如何治疗患有轻度肝病的小儿WD患者。

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