34例肝糖原累积病的临床病理观察

刘琳; 管小琴; 王立娟; 赵瑞秋; 许红梅; 廖晓岗

doi:10.13406/j.cnki.cyxb.2010.04.008

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34例肝糖原累积病的临床病理观察

临床研究 | 更新时间：2024-01-17

- 34例肝糖原累积病的临床病理观察
- The clinical and pathological manifestations of 34 cases hepatic glycogen storage disease
- 重庆医科大学学报 2010年35卷第4期页码：605-607
- 作者机构：
  
  1. 重庆医科大学基础医学院病理教研室
  2. 重庆医科大学附属儿童医院感染消化科
  3. 重庆医科大学基础医学院电镜室
- 作者简介：
- 基金信息：
- DOI：10.13406/j.cnki.cyxb.2010.04.008
  中图分类号： R473.72
- 纸质出版日期：2010，
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[1]刘琳,管小琴,王立娟,赵瑞秋,许红梅,廖晓岗.34例肝糖原累积病的临床病理观察[J].重庆医科大学学报,2010,35(04):605-607.

LIU Lin, et al. The clinical and pathological manifestations of 34 cases hepatic glycogen storage disease[J]. Journal of Chongqing Medical University, 2010,35(4):605-607.
[1]刘琳,管小琴,王立娟,赵瑞秋,许红梅,廖晓岗.34例肝糖原累积病的临床病理观察[J].重庆医科大学学报,2010,35(04):605-607. DOI： 10.13406/j.cnki.cyxb.2010.04.008.

LIU Lin, et al. The clinical and pathological manifestations of 34 cases hepatic glycogen storage disease[J]. Journal of Chongqing Medical University, 2010,35(4):605-607. DOI： 10.13406/j.cnki.cyxb.2010.04.008.

摘要

目的:分析肝糖原累积病的临床病理表现

以提高对该病的认识。方法:34例确诊为肝糖原累积病患儿的临床资料

用光镜及电镜观察其肝活检组织的病理形态。结果:肝糖原累积病患儿临床主要表现为肝大

生长发育滞后

部分有低血糖表现;实验室检查可见低血糖

高乳酸血症

肝功能异常;肝穿刺活检光镜观察见肝细胞肿胀

呈植物细胞样镶嵌状排列;胞质内有大量红色粉尘样物

过碘酸希夫染色（Periodicacid-schiff

PAS）阳性证实其为糖原;电镜示肝细胞胞质内大量糖原沉积及大小不等脂滴形成。结论:肝糖原累积病发病隐匿

对临床不明原因的生长发育迟缓、肝脏肿大、有低血糖及酸中毒表现的患儿应疑诊本病。肝穿刺活检PAS染色及电镜观察对本病的确诊有重要意义。

Abstract

Objective:To investigate the clinical features

pathological changes of 34 cases that had suffered infantile hepatic glycogen storage disease in order to improve the recognition.Methods:The clinical manifestations of 34 cases of infantile hepatic glycogen storage disease during May 2005 to May 2009 were collected.The hepatic tissues from liver biopsy were observed under microscope and electron microscope.Results:Hepatomegaly and growth retardation were the main clinical manifestations of hepatic glycogen storage disease

partial cases were complicated with the manifestations of hypoglycemia.Hypoglycemia

hyperlactacidemia and abnormality of liver function were detected.And the pathology features were observed under microscope and electron microscope.Conclusion:The clinical manifestations of infantile hepatic glycogen storage disease were common

and the final diagnosis can depende on liver biopsy.

关键词

糖原累积病肝脏婴幼儿

Keywords

Glycogen storage diseaseliverinfant

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