过多的与Cree和Ojibwa-Cree儿童的严重渐进性胆固醇症有关
M J Phillips1, C A Ackerley, R A Superina
1Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.
Lancet (London, England)
|March 30, 1996
概括
六名患有慢性胆固醇性肝病的加拿大土著儿童表现出过多的肝铜和. 这表明金属新陈代谢或环境因素的潜在遗传障碍有助于他们的严重肝脏状况.
科学领域:
- 儿科肝病学 儿科肝病学
- 医学遗传学 医学遗传学
- 微量元素的新陈代谢
背景情况:
- 高肝铜与各种肝脏疾病有关.
- 六名加拿大土著儿童出现了严重的慢性胆固醇性肝病.
- 这些儿童表现出过多的肝铜和水平.
研究的目的:
- 为了研究患有严重慢性胆固醇性肝病的儿童肝组织的元素组成.
- 为了比较受影响儿童和对照儿童的肝脏铜和度.
主要方法:
- 对六名儿童 (22个月至8岁) 的肝脏样本或活检材料进行分析.
- 使用扫描传输电子显微镜 (STEM) 的X射线元素微分析.
- 采用原子吸收光谱光度计用于元素量化.
- 结果与四个对照样本进行了比较.
主要成果:
- 切开的肝脏表现出先进的胆管缩,在溶酶体和细胞质中存在密集的沉积物.
- 与对照组相比,患者的肝铜度显著增加 (47.6-56.9μg/g干重) (2.3-2.9μg/g).
- 肝脏的度在患者 (104-128μg/g干重) 和对照组 (1.9-3.2μg/g) 之间也明显较高.
结论:
- 过多的肝铜可能是慢性胆固醇症的结果.
- 过多的肝的原因仍然无法解释.
- 由于家族联系,被认为是金属代谢的遗传性疾病,但环境因素并没有被排除在外.
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