首页> 外文期刊>Hepatitis Monthly >LIVER STORAGE DISEASE IN IRAN: A TEN YEAR STUDY OF LIVER BIOPSIES IN CHILDREN MEDICAL CENTER HOSPITAL IN TEHRAN-IRAN
【24h】

LIVER STORAGE DISEASE IN IRAN: A TEN YEAR STUDY OF LIVER BIOPSIES IN CHILDREN MEDICAL CENTER HOSPITAL IN TEHRAN-IRAN

机译:伊朗肝存储疾病:德黑兰儿童医学中心医院肝活检十年研究

获取原文
       

摘要

Background: Liver storage diseases are rare biochemical and inherited diseases that affect multiorgan systems.Objectives: This study was performed to determine the rate of storage diseases and their types in liver pathology specimens of subjects who were referred to a tertiary pediatric center.Patients and Methods: Two pathologists evaluated 2216 pathology specimens (stained with hematoxylin and eosin and periodic acid-Schiff) from subjects who were referred to the largest pediatric tertiary referral center in Iran between 1996 and 2007. Baseline data and clinical and laboratory manifestations were retrieved from the patients’ files.Results: We identified 117 patients who had storage diseases. A combination of clinical and laboratory findings was used to assess the final diagnosis. Glycogen storage disease (GSD) was observed in 85 of cases, compared with lysosomal storage diseases (LSD) in 31 patients and mucopolysaccharidoses in 1 case. LSD was more prevalent in those aged between 1 month and 1 year, whereas GSD was more frequent in those aged between 1 and 6 years. Most of the patients aged between 1 and 6 years. Most patients with LSD and GSD had unknown types of the disease. The most common known types in the LSD and GSD groups were Niemann-Pick disease and GSD type I respectively. The most common clinical and laboratory manifestation was hepatomegaly and abnormal liver enzymes, respectively.Conclusions: Most of our patients with storage diseases had Gaucher disease. Hepatomegaly and elevated transaminase levels were the most striking finding. However, with regard to the limitations of our methodology, further studies that collect more accurate data are warranted.
机译:背景:肝存储疾病是罕见的生化遗传疾病,会影响多器官系统。目的:本研究旨在确定被转诊至三级儿科中心的肝脏病理学标本中的存储疾病发生率及其类型。 :两名病理学家评估了1996年至2007年间被转诊到伊朗最大的儿科三级转诊中心的受试者的2216个病理标本(用苏木精和曙红染色以及高碘酸席夫氏染色)。从患者中检索了基线数据以及临床和实验室表现结果:我们确定了117名患有贮藏病的患者。临床和实验室检查结果的组合用于评估最终诊断。糖原贮积病(GSD)85例,相比溶酶体贮积病(LSD)31例,粘多糖贮积酶1例。 LSD在1个月至1岁之间的人群中更为普遍,而GSD在1至6岁之间的人群中更为频繁。大多数患者年龄在1至6岁之间。 LSD和GSD的大多数患者患有未知的疾病类型。 LSD和GSD组中最常见的已知类型分别是Niemann-Pick病和I型GSD。最常见的临床和实验室表现分别是肝肿大和肝酶异常。结论:我们大多数患有贮藏病的患者患有高雪氏病。肝肿大和转氨酶水平升高是最惊人的发现。但是,关于我们方法的局限性,有必要进行进一步的研究以收集更准确的数据。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱:kefu@zhangqiaokeyan.com

京公网安备:11010802029741号 ICP备案号:京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有
  • 客服微信

  • 服务号