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目的 分析小儿肝豆状核变性临床误诊的原因以及因误诊而对治疗效果的影响。方法 回顾分析初次住院的患儿 ,分为临床误诊、诊断不明和近期确诊三组 ,并对各组误诊误治有关资料进行统计学分析。结果 36 3例中 2 17例入院前被长期误诊 (5 9 8% ) ,5 2例长期诊断不明 (14 3% ) ,仅 94例 (2 5 9% )能在首发症状出现 3个月内获得正确诊断。被长期误诊的 2 17例中误诊病名多达 5 0余种 ,最常见的依次为各种类型肝炎、肝硬化和(或 )腹水、脾肿大、脾功能亢进、关节炎、肾炎及脑炎 (或脑病 )等 ,共 34 7例次。治疗前后三组间的严重程度及治疗效果对比有显著差异 (P <0 0 1)。结论 本病易被长期误诊或诊断不明 ,早期治疗对预后至关重要。建议对具有上述易被误诊疾病症状的患儿常规行角膜K -F环及铜代谢检查 ,以免误诊误治
Objective To analyze the causes of clinical misdiagnosis of pediatric hepatolenticular degeneration and its influence on the therapeutic effect due to misdiagnosis. Methods The first-time hospitalized children were retrospectively analyzed and divided into three groups: clinical misdiagnosis, unknown diagnosis and recent diagnosis. The data of misdiagnosis and mistreatment in each group were statistically analyzed. Results Of the 363 patients, 217 were long-term misdiagnosed (59.8%), 52 were long-term (14.3%) unknown, and 94 (25.9%) were diagnosed within 3 months of onset of symptoms Get the correct diagnosis. Of the 17 cases misdiagnosed for a long time, there were as many as 50 misdiagnosis names. The most common were hepatitis, cirrhosis and / or ascites, splenomegaly, hypersplenism, arthritis, nephritis and encephalitis (Or encephalopathy), a total of 347 cases. There was significant difference between the severity of the three groups before and after treatment and the treatment effect (P <0.01). Conclusion The disease is easily misdiagnosed or diagnosed long-term, early treatment is crucial for prognosis. Proposed to have the above symptoms are easily misdiagnosed children with routine corneal K-F ring and copper metabolism check to avoid misdiagnosis