http://120.24.161.253:5617/index.php?action=history&feed=atom&title=%E8%BF%9B%E8%A1%8C%E6%80%A7%E5%AE%B6%E6%97%8F%E6%80%A7%E8%82%9D%E5%86%85%E8%83%86%E6%B1%81%E6%B7%A4%E7%A7%AF%E7%97%87-%E5%90%8C%E4%B9%89%E8%AF%8D-PFIC 2026-04-24T15:58:44Z 本wiki的该页面的版本历史 MediaWiki 1.30.0 http://120.24.161.253:5617/index.php?title=%E8%BF%9B%E8%A1%8C%E6%80%A7%E5%AE%B6%E6%97%8F%E6%80%A7%E8%82%9D%E5%86%85%E8%83%86%E6%B1%81%E6%B7%A4%E7%A7%AF%E7%97%87-%E5%90%8C%E4%B9%89%E8%AF%8D-PFIC&diff=2028276&oldid=prev 2026-04-16T04:38:02Z

<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) 04:38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=%E8%BF%9B%E8%A1%8C%E6%80%A7%E5%AE%B6%E6%97%8F%E6%80%A7%E8%82%9D%E5%86%85%E8%83%86%E6%B1%81%E6%B7%A4%E7%A7%AF%E7%97%87-%E5%90%8C%E4%B9%89%E8%AF%8D-PFIC&diff=2000344&oldid=prev 2026-04-15T13:12:46Z

<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; 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">遗传代谢性疾病与肝移植</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">进行性家族性肝内胆汁淤积症的研究现状</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">3．</del>[[进行性家族性肝内胆汁淤积症]](<del class="diffchange diffchange-inline">progressivefamilialintrahepaticcholestasis，</del>[[PFIC]])<del class="diffchange diffchange-inline">：常见</del>[[PFIC]]<del class="diffchange diffchange-inline">有ATP881缺陷病</del>(<del class="diffchange diffchange-inline">PIFC1型</del>)<del class="diffchange diffchange-inline">、ABCBll缺陷病</del>(<del class="diffchange diffchange-inline">PIFC2型</del>)<del class="diffchange diffchange-inline">及ABCB4缺陷病</del>(<del class="diffchange diffchange-inline">PIFC3型</del>)<del class="diffchange diffchange-inline">。</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>[[进行性家族性肝内胆汁淤积症]]([[PFIC]])<ins class="diffchange diffchange-inline">是一组罕见的异质性常染色体隐性遗传性疾病,发病率约为1/50 000～1/100 000.依据编码肝细胞膜转运蛋白基因的不同,可将</ins>[[PFIC]]<ins class="diffchange diffchange-inline">分为三型.</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">===进行性家族性肝内胆汁淤积症的最新研究进展===</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">[[进行性家族性肝内胆汁淤积症]]</ins>(<ins class="diffchange diffchange-inline">[[PFIC]]</ins>)<ins class="diffchange diffchange-inline">是一组异质性的常染色体隐性遗传病.</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">===肝移植治疗进行性家族性肝内胆汁淤积症的疗效分析===</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><ins class="diffchange diffchange-inline">目的探讨肝移植术对[[进行性家族性肝内胆汁淤积症]] </ins>(<ins class="diffchange diffchange-inline">processive familial intrahepatic cholestasis, [[PFIC]]</ins>) <ins class="diffchange diffchange-inline">的临床疗效.</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">===进行性家族性肝内胆汁淤积症1型报告及讨论===</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">[[进行性家族性肝内胆汁淤积症]]</ins>(<ins class="diffchange diffchange-inline">[[PFIC]]</ins>)<ins class="diffchange diffchange-inline">是一组表现在新生儿时期或者1岁内发病,以严重肝内胆汁淤积及严重的皮肤瘙痒为主要特征,在儿童期或者青春期可因肝衰竭而导致死亡的罕见的异质性常染色体隐性遗传性疾病.</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">===先天性胆汁酸合成障碍（2019年版诊疗指南）===</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">1.[[进行性家族性肝内胆汁淤积症]]（progressive familial intrahepatic cholestasis，[[PFIC]]）是一组常染色体隐性遗传性疾病。</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">===进行性家族性肝内胆汁淤积症（2019年版诊疗指南）===</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">[[进行性家族性肝内胆汁淤积症]]（progressive familial intrahepatic cholestasis，[[PFIC]]）是一组常染色体隐性遗传性疾病。</ins></div></td></tr> </table>

KGadmin http://120.24.161.253:5617/index.php?title=%E8%BF%9B%E8%A1%8C%E6%80%A7%E5%AE%B6%E6%97%8F%E6%80%A7%E8%82%9D%E5%86%85%E8%83%86%E6%B1%81%E6%B7%A4%E7%A7%AF%E7%97%87-%E5%90%8C%E4%B9%89%E8%AF%8D-PFIC&diff=2028275&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; 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">进行性家族性肝内胆汁淤积症的研究现状</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">遗传代谢性疾病与肝移植</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>[[进行性家族性肝内胆汁淤积症]]([[PFIC]])<del class="diffchange diffchange-inline">是一组罕见的异质性常染色体隐性遗传性疾病,发病率约为1/50 000～1/100 000.依据编码肝细胞膜转运蛋白基因的不同,可将</del>[[PFIC]]<del class="diffchange diffchange-inline">分为三型.</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">3．</ins>[[进行性家族性肝内胆汁淤积症]](<ins class="diffchange diffchange-inline">progressivefamilialintrahepaticcholestasis，</ins>[[PFIC]])<ins class="diffchange diffchange-inline">：常见</ins>[[PFIC]]<ins class="diffchange diffchange-inline">有ATP881缺陷病</ins>(<ins class="diffchange diffchange-inline">PIFC1型</ins>)<ins class="diffchange diffchange-inline">、ABCBll缺陷病</ins>(<ins class="diffchange diffchange-inline">PIFC2型</ins>)<ins class="diffchange diffchange-inline">及ABCB4缺陷病</ins>(<ins class="diffchange diffchange-inline">PIFC3型</ins>)<ins class="diffchange diffchange-inline">。</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">===进行性家族性肝内胆汁淤积症的最新研究进展===</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">[[进行性家族性肝内胆汁淤积症]]</del>(<del class="diffchange diffchange-inline">[[PFIC]]</del>)<del class="diffchange diffchange-inline">是一组异质性的常染色体隐性遗传病.</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>&#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">===肝移植治疗进行性家族性肝内胆汁淤积症的疗效分析===</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><del class="diffchange diffchange-inline">目的探讨肝移植术对[[进行性家族性肝内胆汁淤积症]] </del>(<del class="diffchange diffchange-inline">processive familial intrahepatic cholestasis, [[PFIC]]</del>) <del class="diffchange diffchange-inline">的临床疗效.</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">===进行性家族性肝内胆汁淤积症1型报告及讨论===</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">[[进行性家族性肝内胆汁淤积症]]</del>(<del class="diffchange diffchange-inline">[[PFIC]]</del>)<del class="diffchange diffchange-inline">是一组表现在新生儿时期或者1岁内发病,以严重肝内胆汁淤积及严重的皮肤瘙痒为主要特征,在儿童期或者青春期可因肝衰竭而导致死亡的罕见的异质性常染色体隐性遗传性疾病.</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>&#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">===先天性胆汁酸合成障碍（2019年版诊疗指南）===</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">1.[[进行性家族性肝内胆汁淤积症]]（progressive familial intrahepatic cholestasis，[[PFIC]]）是一组常染色体隐性遗传性疾病。</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>&#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">===进行性家族性肝内胆汁淤积症（2019年版诊疗指南）===</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">[[进行性家族性肝内胆汁淤积症]]（progressive familial intrahepatic cholestasis，[[PFIC]]）是一组常染色体隐性遗传性疾病。</del></div></td><td colspan="2">&#160;</td></tr> </table>

Admin http://120.24.161.253:5617/index.php?title=%E8%BF%9B%E8%A1%8C%E6%80%A7%E5%AE%B6%E6%97%8F%E6%80%A7%E8%82%9D%E5%86%85%E8%83%86%E6%B1%81%E6%B7%A4%E7%A7%AF%E7%97%87-%E5%90%8C%E4%B9%89%E8%AF%8D-PFIC&diff=2000343&oldid=prev 2026-04-13T13:56:59Z

<p>修改1</p> <p><b>新页面</b></p><div>主实体：[[进行性家族性肝内胆汁淤积症]]，关系：[[同义词]] ，尾实体：[[PFIC]]<br /> <br /> ==关系介绍==<br /> 待补充<br /> <br /> ==来源==<br /> ===进行性家族性肝内胆汁淤积症的研究现状===<br /> [[进行性家族性肝内胆汁淤积症]]([[PFIC]])是一组罕见的异质性常染色体隐性遗传性疾病,发病率约为1/50 000～1/100 000.依据编码肝细胞膜转运蛋白基因的不同,可将[[PFIC]]分为三型.<br /> <br /> ===进行性家族性肝内胆汁淤积症的最新研究进展===<br /> [[进行性家族性肝内胆汁淤积症]]([[PFIC]])是一组异质性的常染色体隐性遗传病.<br /> <br /> ===肝移植治疗进行性家族性肝内胆汁淤积症的疗效分析===<br /> 目的探讨肝移植术对[[进行性家族性肝内胆汁淤积症]] (processive familial intrahepatic cholestasis, [[PFIC]]) 的临床疗效.<br /> <br /> ===进行性家族性肝内胆汁淤积症1型报告及讨论===<br /> [[进行性家族性肝内胆汁淤积症]]([[PFIC]])是一组表现在新生儿时期或者1岁内发病,以严重肝内胆汁淤积及严重的皮肤瘙痒为主要特征,在儿童期或者青春期可因肝衰竭而导致死亡的罕见的异质性常染色体隐性遗传性疾病.<br /> <br /> ===先天性胆汁酸合成障碍（2019年版诊疗指南）===<br /> 1.[[进行性家族性肝内胆汁淤积症]]（progressive familial intrahepatic cholestasis，[[PFIC]]）是一组常染色体隐性遗传性疾病。<br /> <br /> ===进行性家族性肝内胆汁淤积症（2019年版诊疗指南）===<br /> [[进行性家族性肝内胆汁淤积症]]（progressive familial intrahepatic cholestasis，[[PFIC]]）是一组常染色体隐性遗传性疾病。</div>

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