http://120.24.161.253:5617/index.php?action=history&feed=atom&title=%E7%B3%96%E5%8E%9F%E7%B4%AF%E7%A7%AF%E7%97%85-%E7%97%85%E5%9B%A0-%E5%85%88%E5%A4%A9%E6%80%A7%E9%85%B6%E7%BC%BA%E9%99%B7 2026-05-13T04:42:24Z 本wiki的该页面的版本历史 MediaWiki 1.30.0 http://120.24.161.253:5617/index.php?title=%E7%B3%96%E5%8E%9F%E7%B4%AF%E7%A7%AF%E7%97%85-%E7%97%85%E5%9B%A0-%E5%85%88%E5%A4%A9%E6%80%A7%E9%85%B6%E7%BC%BA%E9%99%B7&diff=2026794&oldid=prev 2026-04-16T04:37:19Z

<p>导入1个版本</p> <table class="diff diff-contentalign-left" data-mw="interface"> <tr style="vertical-align: top;" lang="zh-CN"> <td colspan="1" style="background-color: white; color:black; text-align: center;">←上一版本</td> <td colspan="1" style="background-color: white; color:black; text-align: center;">2026年4月16日 (四) 04:37的版本</td> </tr><tr><td colspan="2" style="text-align: center;" lang="zh-CN"><div class="mw-diff-empty">（没有差异）</div> </td></tr></table>

KGadmin http://120.24.161.253:5617/index.php?title=%E7%B3%96%E5%8E%9F%E7%B4%AF%E7%A7%AF%E7%97%85-%E7%97%85%E5%9B%A0-%E5%85%88%E5%A4%A9%E6%80%A7%E9%85%B6%E7%BC%BA%E9%99%B7&diff=2000454&oldid=prev 2026-04-15T13:12:47Z

<p>导入1个版本</p> <table class="diff diff-contentalign-left" data-mw="interface"> <col class="diff-marker" /> <col class="diff-content" /> <col class="diff-marker" /> <col class="diff-content" /> <tr style="vertical-align: top;" lang="zh-CN"> <td colspan="2" style="background-color: white; color:black; text-align: center;">←上一版本</td> <td colspan="2" style="background-color: white; color:black; text-align: center;">2026年4月15日 (三) 13:12的版本</td> </tr><tr><td colspan="2" class="diff-lineno" id="mw-diff-left-l5" >第5行：</td> <td colspan="2" class="diff-lineno">第5行：</td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; 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border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div>===<ins class="diffchange diffchange-inline">肝糖原累积病研究进展</ins>===</div></td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del class="diffchange diffchange-inline">[[糖原累积病]]是一组由于[[先天性酶缺陷]]所致的糖代谢障碍疾病。</del></div></td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div>[[糖原累积病]](<ins class="diffchange diffchange-inline">glycogen storage disease,GSD</ins>)<ins class="diffchange diffchange-inline">是一种由</ins>[[先天性酶缺陷]]<ins class="diffchange diffchange-inline">所导致的糖代谢障碍疾病,主要临床表现为低血糖、肝脾大及生长发育迟缓.</ins></div></td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div>&#160;</div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del class="diffchange diffchange-inline">===23例成人肝糖原累积病的临床和病理分析</del>===</div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div>[[糖原累积病]](<del class="diffchange diffchange-inline">glycogenstoragedisease，GSD</del>)<del class="diffchange diffchange-inline">是一组由于[[先天性酶缺陷]]所致的糖代谢障碍，欧洲发病率为4／10万一5／10万。</del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div>&#160;</div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del class="diffchange diffchange-inline">===青少年痛风的罕见原因——糖原累积病Ⅰa型临床研究===</del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del class="diffchange diffchange-inline">[一j[太纠I线片J6乏腹部CTIA双手x线片娃示双侧掌指关节周嗣软组织肘，胀、关节间隙狭窄1B双足x线片显示双侧第一跖趾关节周围软组织肿胀、父节间隙狭窄，第·跖趾火节骨边缘有骨缺损1C腹部CT硅示肝大，肝左叶异常密度灶[[糖原累积病]]是一组由于</del>[[先天性酶缺陷]]<del class="diffchange diffchange-inline">所致的糖代谢障碍疾病，根据酶缺陷不同至少有12种类型，其中I型最常见，发病率约1／100000～1／400000，为常染色体隐性遗传，I型又分为Ia和Ib亚型，Ia型约占80％，主要源于G6PC基因突变导致的葡萄糖一6一磷酸酶活性异常，导致糖原在肝脏、肾脏、肠道等组织中大量沉积而引起一系列症状，包括肝大、低血糖、生长发育迟缓、反复鼻衄等，可出现高脂血症、高乳酸血症、高尿酸血症等代谢紊乱，持续的高尿酸血症患者易在青春期并发痛风，长期并发症有肝腺瘤、肝腺瘤恶变、肾脏损害甚至肾衰竭等。</del></div></td><td colspan="2">&#160;</td></tr> </table>

KGadmin http://120.24.161.253:5617/index.php?title=%E7%B3%96%E5%8E%9F%E7%B4%AF%E7%A7%AF%E7%97%85-%E7%97%85%E5%9B%A0-%E5%85%88%E5%A4%A9%E6%80%A7%E9%85%B6%E7%BC%BA%E9%99%B7&diff=2026793&oldid=prev 2026-04-13T13:56:59Z

<p>修改1</p> <table class="diff diff-contentalign-left" data-mw="interface"> <col class="diff-marker" /> <col class="diff-content" /> <col class="diff-marker" /> <col class="diff-content" /> <tr style="vertical-align: top;" lang="zh-CN"> <td colspan="2" style="background-color: white; color:black; text-align: center;">←上一版本</td> <td colspan="2" style="background-color: white; color:black; text-align: center;">2026年4月13日 (一) 13:56的版本</td> </tr><tr><td colspan="2" class="diff-lineno" id="mw-diff-left-l5" >第5行：</td> <td colspan="2" class="diff-lineno">第5行：</td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; 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border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div>===<ins class="diffchange diffchange-inline">儿童肝糖原累积病的临床特点分析===</ins></div></td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div>[[糖原累积病]](<del class="diffchange diffchange-inline">glycogen storage disease,GSD</del>)<del class="diffchange diffchange-inline">是一种由</del>[[先天性酶缺陷]]<del class="diffchange diffchange-inline">所导致的糖代谢障碍疾病,主要临床表现为低血糖、肝脾大及生长发育迟缓.</del></div></td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div><ins class="diffchange diffchange-inline">[[糖原累积病]]是一组由于[[先天性酶缺陷]]所致的糖代谢障碍疾病。</ins></div></td></tr> <tr><td colspan="2">&#160;</td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div>&#160;</div></td></tr> <tr><td colspan="2">&#160;</td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div><ins class="diffchange diffchange-inline">===23例成人肝糖原累积病的临床和病理分析</ins>===</div></td></tr> <tr><td colspan="2">&#160;</td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div>[[糖原累积病]](<ins class="diffchange diffchange-inline">glycogenstoragedisease，GSD</ins>)<ins class="diffchange diffchange-inline">是一组由于[[先天性酶缺陷]]所致的糖代谢障碍，欧洲发病率为4／10万一5／10万。</ins></div></td></tr> <tr><td colspan="2">&#160;</td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div>&#160;</div></td></tr> <tr><td colspan="2">&#160;</td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div><ins class="diffchange diffchange-inline">===青少年痛风的罕见原因——糖原累积病Ⅰa型临床研究===</ins></div></td></tr> <tr><td colspan="2">&#160;</td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div><ins class="diffchange diffchange-inline">[一j[太纠I线片J6乏腹部CTIA双手x线片娃示双侧掌指关节周嗣软组织肘，胀、关节间隙狭窄1B双足x线片显示双侧第一跖趾关节周围软组织肿胀、父节间隙狭窄，第·跖趾火节骨边缘有骨缺损1C腹部CT硅示肝大，肝左叶异常密度灶[[糖原累积病]]是一组由于</ins>[[先天性酶缺陷]]<ins class="diffchange diffchange-inline">所致的糖代谢障碍疾病，根据酶缺陷不同至少有12种类型，其中I型最常见，发病率约1／100000～1／400000，为常染色体隐性遗传，I型又分为Ia和Ib亚型，Ia型约占80％，主要源于G6PC基因突变导致的葡萄糖一6一磷酸酶活性异常，导致糖原在肝脏、肾脏、肠道等组织中大量沉积而引起一系列症状，包括肝大、低血糖、生长发育迟缓、反复鼻衄等，可出现高脂血症、高乳酸血症、高尿酸血症等代谢紊乱，持续的高尿酸血症患者易在青春期并发痛风，长期并发症有肝腺瘤、肝腺瘤恶变、肾脏损害甚至肾衰竭等。</ins></div></td></tr> </table>

Admin http://120.24.161.253:5617/index.php?title=%E7%B3%96%E5%8E%9F%E7%B4%AF%E7%A7%AF%E7%97%85-%E7%97%85%E5%9B%A0-%E5%85%88%E5%A4%A9%E6%80%A7%E9%85%B6%E7%BC%BA%E9%99%B7&diff=2000453&oldid=prev 2026-04-13T13:56:59Z

<p>修改1</p> <table class="diff diff-contentalign-left" data-mw="interface"> <col class="diff-marker" /> <col class="diff-content" /> <col class="diff-marker" /> <col class="diff-content" /> <tr style="vertical-align: top;" lang="zh-CN"> <td colspan="2" style="background-color: white; color:black; text-align: center;">←上一版本</td> <td colspan="2" style="background-color: white; color:black; text-align: center;">2026年4月13日 (一) 13:56的版本</td> </tr><tr><td colspan="2" class="diff-lineno" id="mw-diff-left-l1" >第1行：</td> <td colspan="2" class="diff-lineno">第1行：</td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>主实体：[[糖原累积病]]，关系：[[病因]] ，尾实体：[[先天性酶缺陷]]</div></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>主实体：[[糖原累积病]]，关系：[[病因]] ，尾实体：[[先天性酶缺陷]]</div></td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"></td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;">实体ID:''10931898''</del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>==关系介绍==</div></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>==关系介绍==</div></td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>待补充</div></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>待补充</div></td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"></td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>==来源==</div></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>==来源==</div></td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div>===<del class="diffchange diffchange-inline">肝糖原累积病研究进展（中华医学会文献）</del>===</div></td><td class='diff-marker'>+</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div>===<ins class="diffchange diffchange-inline">肝糖原累积病研究进展</ins>===</div></td></tr> <tr><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>[[糖原累积病]](glycogen storage disease,GSD)是一种由[[先天性酶缺陷]]所导致的糖代谢障碍疾病,主要临床表现为低血糖、肝脾大及生长发育迟缓.</div></td><td class='diff-marker'>&#160;</td><td style="background-color: #f9f9f9; color: #333333; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #e6e6e6; vertical-align: top; white-space: pre-wrap;"><div>[[糖原累积病]](glycogen storage disease,GSD)是一种由[[先天性酶缺陷]]所导致的糖代谢障碍疾病,主要临床表现为低血糖、肝脾大及生长发育迟缓.</div></td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;"></del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;">===儿童肝糖原累积病的临床特点分析（中华医学会文献）===</del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;">[[糖原累积病]]是一组由于[[先天性酶缺陷]]所致的糖代谢障碍疾病。</del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;"></del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;">===23例成人肝糖原累积病的临床和病理分析（中华医学会文献）===</del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;">[[糖原累积病]](glycogenstoragedisease，GSD)是一组由于[[先天性酶缺陷]]所致的糖代谢障碍，欧洲发病率为4／10万一5／10万。</del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;"></del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;">===青少年痛风的罕见原因——糖原累积病Ⅰa型临床研究（中华医学会文献）===</del></div></td><td colspan="2">&#160;</td></tr> <tr><td class='diff-marker'>−</td><td style="color:black; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div><del style="font-weight: bold; text-decoration: none;">[一j[太纠I线片J6乏腹部CTIA双手x线片娃示双侧掌指关节周嗣软组织肘，胀、关节间隙狭窄1B双足x线片显示双侧第一跖趾关节周围软组织肿胀、父节间隙狭窄，第·跖趾火节骨边缘有骨缺损1C腹部CT硅示肝大，肝左叶异常密度灶[[糖原累积病]]是一组由于[[先天性酶缺陷]]所致的糖代谢障碍疾病，根据酶缺陷不同至少有12种类型，其中I型最常见，发病率约1／100000～1／400000，为常染色体隐性遗传，I型又分为Ia和Ib亚型，Ia型约占80％，主要源于G6PC基因突变导致的葡萄糖一6一磷酸酶活性异常，导致糖原在肝脏、肾脏、肠道等组织中大量沉积而引起一系列症状，包括肝大、低血糖、生长发育迟缓、反复鼻衄等，可出现高脂血症、高乳酸血症、高尿酸血症等代谢紊乱，持续的高尿酸血症患者易在青春期并发痛风，长期并发症有肝腺瘤、肝腺瘤恶变、肾脏损害甚至肾衰竭等。</del></div></td><td colspan="2">&#160;</td></tr> </table>

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<p>修改1</p> <p><b>新页面</b></p><div>主实体：[[糖原累积病]]，关系：[[病因]] ，尾实体：[[先天性酶缺陷]]<br /> <br /> 实体ID:''10931898''<br /> ==关系介绍==<br /> 待补充<br /> <br /> ==来源==<br /> ===肝糖原累积病研究进展（中华医学会文献）===<br /> [[糖原累积病]](glycogen storage disease,GSD)是一种由[[先天性酶缺陷]]所导致的糖代谢障碍疾病,主要临床表现为低血糖、肝脾大及生长发育迟缓.<br /> <br /> ===儿童肝糖原累积病的临床特点分析（中华医学会文献）===<br /> [[糖原累积病]]是一组由于[[先天性酶缺陷]]所致的糖代谢障碍疾病。<br /> <br /> ===23例成人肝糖原累积病的临床和病理分析（中华医学会文献）===<br /> [[糖原累积病]](glycogenstoragedisease，GSD)是一组由于[[先天性酶缺陷]]所致的糖代谢障碍，欧洲发病率为4／10万一5／10万。<br /> <br /> ===青少年痛风的罕见原因——糖原累积病Ⅰa型临床研究（中华医学会文献）===<br /> [一j[太纠I线片J6乏腹部CTIA双手x线片娃示双侧掌指关节周嗣软组织肘，胀、关节间隙狭窄1B双足x线片显示双侧第一跖趾关节周围软组织肿胀、父节间隙狭窄，第·跖趾火节骨边缘有骨缺损1C腹部CT硅示肝大，肝左叶异常密度灶[[糖原累积病]]是一组由于[[先天性酶缺陷]]所致的糖代谢障碍疾病，根据酶缺陷不同至少有12种类型，其中I型最常见，发病率约1／100000～1／400000，为常染色体隐性遗传，I型又分为Ia和Ib亚型，Ia型约占80％，主要源于G6PC基因突变导致的葡萄糖一6一磷酸酶活性异常，导致糖原在肝脏、肾脏、肠道等组织中大量沉积而引起一系列症状，包括肝大、低血糖、生长发育迟缓、反复鼻衄等，可出现高脂血症、高乳酸血症、高尿酸血症等代谢紊乱，持续的高尿酸血症患者易在青春期并发痛风，长期并发症有肝腺瘤、肝腺瘤恶变、肾脏损害甚至肾衰竭等。</div>

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