急性肝性ポルフィリン症（AHP）の症状

<!DOCTYPE html> <html lang="ja" dir="ltr" prefix="content: http://purl.org/rss/1.0/modules/content/ dc: http://purl.org/dc/terms/ foaf: http://xmlns.com/foaf/0.1/ og: http://ogp.me/ns# rdfs: http://www.w3.org/2000/01/rdf-schema# schema: http://schema.org/ sioc: http://rdfs.org/sioc/ns# sioct: http://rdfs.org/sioc/types# skos: http://www.w3.org/2004/02/skos/core# xsd: http://www.w3.org/2001/XMLSchema# "> <head> <meta charset="utf-8" /> <link rel="canonical" href="https://porphyria.jp/symptom/AHP-symptoms" /> <meta name="description" content="急性肝性ポルフィリン症では腹痛に加えて、末梢神経系、中枢神経系、自律神経系などに1つ以上の症状がみられます。" /> <meta name="keywords" content="AHP,急性肝性ポルフィリン症,神経障害,腹痛,暗褐色尿" /> <meta name="Generator" content="Drupal 8 (https://www.drupal.org)" /> <meta name="MobileOptimized" content="width" /> <meta name="HandheldFriendly" content="true" /> <meta name="viewport" content="width=device-width, initial-scale=1.0" /> <link rel="shortcut icon" href="/themes/custom/porphyriajp/favicon.ico" type="image/vnd.microsoft.icon" /> <link rel="revision" href="https://porphyria.jp/symptom/AHP-symptoms" /> <script src="/sites/default/files/google_tag/default/google_tag.script.js?sldymw" defer></script> <title>急性肝性ポルフィリン症（AHP）の症状 | Porphyria.jp</title> <link rel="stylesheet" media="all" href="/core/assets/vendor/normalize-css/normalize.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/misc/normalize-fixes.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/ajax-progress.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/align.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/autocomplete-loading.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/fieldgroup.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/container-inline.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/clearfix.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/details.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/hidden.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/item-list.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/js.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/nowrap.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/position-container.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/progress.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/reset-appearance.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/resize.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/sticky-header.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/system-status-counter.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/system-status-report-counters.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/system-status-report-general-info.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/tabledrag.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/tablesort.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/stable/css/system/components/tree-child.module.css?sldymw" /> <link rel="stylesheet" media="all" href="/themes/custom/porphyriajp/css/base.css?sldymw" /> <link rel="stylesheet" media="all" href="/themes/custom/porphyriajp/css/layout.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/action-links.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/breadcrumb.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/button.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/collapse-processed.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/container-inline.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/details.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/exposed-filters.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/field.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/form.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/icons.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/inline-form.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/item-list.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/link.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/links.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/more-link.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/pager.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/tabledrag.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/tableselect.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/tablesort.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/tabs.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/textarea.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/ui-dialog.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/messages.css?sldymw" /> <link rel="stylesheet" media="all" href="/core/themes/classy/css/components/node.css?sldymw" /> <link rel="stylesheet" media="all" href="/themes/custom/porphyriajp/css/components.css?sldymw" /> <link rel="stylesheet" media="all" href="/themes/custom/porphyriajp/css/scroll-hint.css?sldymw" /> <link rel="stylesheet" media="all" href="/themes/custom/porphyriajp/css/state.css?sldymw" /> <link rel="stylesheet" media="all" href="https://fonts.googleapis.com/icon?family=Material+Icons" />  </head> <body class="page-node-6 page-node-type-page path-node"> <a href="#main-content" class="visually-hidden focusable skip-link"> Skip to main content </a> <noscript aria-hidden="true"><iframe src="https://www.googletagmanager.com/ns.html?id=GTM-W5BZGH9" height="0" width="0" style="display:none;visibility:hidden"></iframe></noscript> <div class="dialog-off-canvas-main-canvas" data-off-canvas-main-canvas> <div class="layout-container"> <div class="layout-menu-cover js-menu-cover"></div> <header role="banner" class="layout-header layout-container-header"> <div class="layout-header-inner"> <a class="layout-header-logo" title="Home" href="/"> <picture> <source media="(max-width: 991px)" srcset="/themes/custom/porphyriajp/images/logo.png"> <img src="/themes/custom/porphyriajp/images/logo-pc.png" alt="Porphyria.jp AHP医療関係者向けサイト" width="619" height="157"> </picture> </a> <div class="layout-header-menu js-header-menu"> <div class="region region-header"> <nav role="navigation" aria-labelledby="block-porphyriajp-main-menu-menu" id="block-porphyriajp-main-menu" class="block block-menu navigation menu--main"> <h2 class="visually-hidden" id="block-porphyriajp-main-menu-menu">Main navigation</h2> <ul class="menu"> <li class="menu-item menu-item--expanded"> <a href="/about-AHP/porphyria-subtypes" data-drupal-link-system-path="node/2">AHPについて</a> <ul class="menu"> <li class="menu-item"> <a href="/about-AHP/porphyria-subtypes" data-drupal-link-system-path="node/2">ポルフィリン症の分類</a> </li> <li class="menu-item"> <a href="/about-AHP/genetic-defect-in-heme-synthesis" data-drupal-link-system-path="node/3">ヘム生合成過程の異常とAHP</a> </li> <li class="menu-item"> <a href="/about-AHP/onset-factor" data-drupal-link-system-path="node/4">AHPの発症要因</a> </li> <li class="menu-item"> <a href="/about-AHP/epidemiology" data-drupal-link-system-path="node/5">疫学</a> </li> </ul> </li> <li class="menu-item menu-item--expanded menu-item--active-trail"> <a href="/symptom/AHP-symptoms" data-drupal-link-system-path="node/6" class="is-active">症状</a> <ul class="menu"> <li class="menu-item menu-item--active-trail"> <a href="/symptom/AHP-symptoms" data-drupal-link-system-path="node/6" class="is-active">AHPの症状</a> </li> <li class="menu-item"> <a href="/symptom/importance-of-differential-diagnosis" data-drupal-link-system-path="node/7">AHPにおける鑑別診断の重要性</a> </li> <li class="menu-item"> <a href="/symptom/triggers-for-acute-attacks" data-drupal-link-system-path="node/8">急性発作の主な誘因</a> </li> <li class="menu-item"> <a href="/symptom/complication-risk" data-drupal-link-system-path="node/9">長期罹患に伴う合併症リスク</a> </li> </ul> </li> <li class="menu-item menu-item--expanded"> <a href="/examination/urinalysis" data-drupal-link-system-path="node/10">検査</a> <ul class="menu"> <li class="menu-item"> <a href="/examination/urinalysis" data-drupal-link-system-path="node/10">尿検査による急性発作時の診断</a> </li> <li class="menu-item"> <a href="/examination/biochemical-abnormalities" data-drupal-link-system-path="node/11">AHPで確認される生化学異常</a> </li> <li class="menu-item"> <a href="/examination/screening-kit" data-drupal-link-system-path="node/27">尿PBG迅速スクリーニングキットのご案内</a> </li> </ul> </li> <li class="menu-item"> <a href="/treatment" data-drupal-link-system-path="node/15">治療</a> </li> <li class="menu-item"> <a href="/acute-abdomen-in-AHP" data-drupal-link-system-path="node/17">急性腹症に潜むAHP</a> </li> </ul> </nav> </div> </div> <button class="layout-header-toggle-menu js-toggle-header-menu"><span>menu</span></button> </div> </header> <main role="main" class="layout-main layout-container-main"> <a id="main-content" tabindex="-1"></a> <div class="layout-content"> <div class="region region-content"> <div data-drupal-messages-fallback class="hidden"></div> <div id="block-top-level-title" class="block block-porphyriajp-top-level-title-block"> <div class="top-level-title-block"> <div class="top-level-title-block__inner"> <h1 class="top-level-title-block__title">症状</h1> </div> </div> </div> <div id="block-link-to-supervisor-page" class="block block-fixed-block-content block-fixed-block-contentlink-to-supervisor-page"> <div class="clearfix text-formatted field field--name-body field--type-text-with-summary field--label-hidden field__item"><p class="link-to-supervisor-page"><span class="link-to-supervisor-page__label">監修:</span><a href="/supervision">堀江裕先生<span class="link-to-supervisor-page__position">（島根県済生会江津総合病院名誉院長、芝浦スリーワンクリニック・ポルフィリン症外来）</span></a></p> </div> </div> <div id="block-porphyriajp-content" class="block block-system block-system-main-block"> <article role="article" about="/symptom/AHP-symptoms" typeof="schema:WebPage" class="node node--type-page node--view-mode-full"> <span property="schema:name" content="AHPの症状" class="rdf-meta hidden"></span> <div class="node__content"> <div property="schema:text" class="clearfix text-formatted field field--name-body field--type-text-with-summary field--label-hidden field__item"><h1>AHPの症状</h1> <p>急性肝性ポルフィリン症（acute hepatic porphyria：AHP）では、ヘム前駆体であるアミノレブリン酸（ALA）やポルフォビリノーゲン（PBG）などが蓄積することにより、神経障害が生じると考えられています。 <span class="br">腹痛を訴えて受診することが多く、9割以上の患者が腹痛を主訴に受診したとのデータもあります<sup>1</sup>。加えて、末梢神経系、中枢神経系、自律神経系などに1つ以上の症状がみられます。</span> <span class="br">また、AHP患者の25-35%に、低ナトリウム血症がみられることも明らかになっています<sup>1</sup>。</span></p> <div class="full-width-image-area"> <figure class="full-width-image-area__inner"> <figcaption class="full-width-image-area__caption">急性肝性ポルフィリン症<sup>∗</sup>の症状<sup>2-5</sup></figcaption> <picture> <source media="(max-width: 767px)" srcset="/themes/custom/porphyriajp/images/symptom/ahp-symptoms-sp.webp" type="image/webp"></source> <source media="(max-width: 767px)" srcset="/themes/custom/porphyriajp/images/symptom/ahp-symptoms-sp.png"></source> <source srcset="/themes/custom/porphyriajp/images/symptom/ahp-symptoms-pc.webp" type="image/webp"></source> <img alt="急性肝性ポルフィリン症の症状" height="990" src="/themes/custom/porphyriajp/images/symptom/ahp-symptoms-pc.png" width="1960" /> </picture></figure> </div> <p>日本でAHP患者を対象に行われた調査では、自覚的初発症状として腹痛が最も多く78.8％（278/353例）、次いで嘔吐が44.5%（157/353例）、便秘が22.9％（81/353例）にみられました<sup>5</sup>。 <span class="br">自覚的初発症状に、病型による大きな差異は認められませんでした。</span></p> <div class="image-box"> <figure class="scalable-figure"> <figcaption class="scalable-figure__caption">急性肝性ポルフィリン症の自覚的初発症状</figcaption> <div class="js-scalable-container"> <div class="scalable-content js-scalable-content"> <table class="basic-table basic-table--with-summary-column"> <caption class="basic-table__note"><span>AIP: 急性間欠性ポルフィリン症、</span><span>VP: 異型ポルフィリン症、</span><span>HCP: 遺伝性コプロポルフィリン症</span></caption> <thead> <tr> <th> </th> <th>AIP<br /> (198例中)</th> <th>VP<br /> (56例中)</th> <th>HCP<br /> (41例中)</th> <th>分類不明<br /> (58例中)</th> <th>計<br /> (353例中)</th> </tr> </thead> <tbody> <tr class="basic-table__colored-row"> <th>消化器症状</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>腹痛</th> <td>165</td> <td>37</td> <td>31</td> <td>45</td> <td>278</td> </tr> <tr class="basic-table__second-level-row"> <th>嘔吐</th> <td>76</td> <td>29</td> <td>26</td> <td>26</td> <td>157</td> </tr> <tr class="basic-table__second-level-row"> <th>便秘</th> <td>38</td> <td>14</td> <td>10</td> <td>19</td> <td>81</td> </tr> <tr class="basic-table__second-level-row"> <th>下痢</th> <td>8</td> <td>1</td> <td>4</td> <td>0</td> <td>13</td> </tr> <tr class="basic-table__colored-row"> <th>神経症状</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>脱力および運動麻痺</th> <td>25</td> <td>18</td> <td>13</td> <td>6</td> <td>62</td> </tr> <tr class="basic-table__second-level-row"> <th>四肢知覚障害</th> <td>23</td> <td>11</td> <td>10</td> <td>1</td> <td>45</td> </tr> <tr class="basic-table__second-level-row"> <th>言語障害</th> <td>1</td> <td>4</td> <td>1</td> <td>1</td> <td>7</td> </tr> <tr class="basic-table__second-level-row"> <th>嚥下障害</th> <td>1</td> <td>3</td> <td>1</td> <td>0</td> <td>5</td> </tr> <tr class="basic-table__second-level-row"> <th>痙攣</th> <td>5</td> <td>1</td> <td>4</td> <td>5</td> <td>15</td> </tr> <tr class="basic-table__second-level-row"> <th>意識障害</th> <td>1</td> <td>0</td> <td>1</td> <td>0</td> <td>2</td> </tr> <tr class="basic-table__colored-row"> <th>皮膚症状</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>日光紅斑</th> <td>0</td> <td>14</td> <td>3</td> <td>1</td> <td>18</td> </tr> <tr class="basic-table__second-level-row"> <th>色素沈着</th> <td>0</td> <td>11</td> <td>4</td> <td>0</td> <td>15</td> </tr> <tr class="basic-table__colored-row"> <th>暗褐色尿</th> <td>35</td> <td>16</td> <td>12</td> <td>11</td> <td>74</td> </tr> </tbody> </table> </div> </div> </figure> </div> <p>他覚的症状としては、末梢性運動麻痺、高血圧、頻脈、四肢知覚障害（表在性）などが確認されています。他覚的症状にも、病型による大きな差異は認められませんでした。 <span class="br">ポルフィリン症では、急性発作時に、骨髄性プロトポルフィリン症（EPP）以外の病型でみられるポートワイン尿ともいわれる尿の着色が特徴的とされています。しかしながら、暗褐色（赤色）尿がみられる頻度は半数以下で、353例中132例（37.4％）に過ぎないことが、国内のデータから明らかになっています<sup>5</sup>。</span> <span class="br">そのため、尿の着色の有無のみでの判断は難しく、検査結果の解釈には注意が必要です。</span></p> <p><a class="link-button" href="/examination/urinalysis"><span>「尿検査による急性発作時の診断」のページへ</span></a></p> <div class="image-box"> <figure class="scalable-figure"> <figcaption class="scalable-figure__caption">急性肝性ポルフィリン症の他覚的病状<span class="small-text">（全経過中にみられたもの）</span></figcaption> <div class="js-scalable-container"> <div class="scalable-content js-scalable-content"> <table class="basic-table basic-table--with-summary-column objective-symptoms-table"> <caption class="basic-table__note"><span>AIP: 急性間欠性ポルフィリン症、</span><span>VP: 異型ポルフィリン症、</span><span>HCP: 遺伝性コプロポルフィリン症</span></caption> <thead> <tr> <th> </th> <th>AIP<br /> (198例中)</th> <th>VP<br /> (56例中)</th> <th>HCP<br /> (41例中)</th> <th>分類不明<br /> (58例中)</th> <th>計<br /> (353例中)</th> </tr> </thead> <tbody> <tr class="basic-table__colored-row"> <th>腹部症状</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>圧痛</th> <td>52</td> <td>7</td> <td>9</td> <td>10</td> <td>78</td> </tr> <tr class="basic-table__second-level-row"> <th>イレウス</th> <td>42</td> <td>2</td> <td>4</td> <td>4</td> <td>52</td> </tr> <tr class="basic-table__colored-row"> <th>神経症状</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>末梢性運動麻痺</th> <td>102</td> <td>25</td> <td>15</td> <td>25</td> <td>167</td> </tr> <tr class="basic-table__second-level-row"> <th>四肢知覚障害（表在性）</th> <td>72</td> <td>15</td> <td>13</td> <td>18</td> <td>118</td> </tr> <tr class="basic-table__third-level-row"> <th>異常知覚</th> <td>23</td> <td>4</td> <td>2</td> <td>1</td> <td>30</td> </tr> <tr class="basic-table__second-level-row"> <th>深部反射亢進</th> <td>12</td> <td>1</td> <td>3</td> <td>1</td> <td>17</td> </tr> <tr class="basic-table__third-level-row"> <th>減弱消失</th> <td>54</td> <td>10</td> <td>8</td> <td>18</td> <td>90</td> </tr> <tr class="basic-table__second-level-row"> <th>病的反射</th> <td>5</td> <td>0</td> <td>2</td> <td>2</td> <td>9</td> </tr> <tr class="basic-table__second-level-row"> <th>意識障害</th> <td>53</td> <td>9</td> <td>11</td> <td>12</td> <td>85</td> </tr> <tr class="basic-table__second-level-row"> <th>痙攣、てんかん発作</th> <td>33</td> <td>5</td> <td>14</td> <td>11</td> <td>63</td> </tr> <tr class="basic-table__second-level-row"> <th>脳神経麻痺</th> <td>28</td> <td>5</td> <td>5</td> <td>5</td> <td>43</td> </tr> <tr class="basic-table__second-level-row"> <th>球麻痺</th> <td>40</td> <td>8</td> <td>1</td> <td>13</td> <td>62</td> </tr> <tr class="basic-table__second-level-row"> <th>膀胱直腸障害</th> <td>39</td> <td>2</td> <td>1</td> <td>1</td> <td>43</td> </tr> <tr class="basic-table__second-level-row"> <th>筋萎縮</th> <td>31</td> <td>5</td> <td>2</td> <td>1</td> <td>39</td> </tr> <tr class="basic-table__second-level-row"> <th>自律神経症状</th> <td>30</td> <td>8</td> <td>4</td> <td>5</td> <td>47</td> </tr> <tr class="basic-table__colored-row"> <th>精神障害</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>幻覚、妄想</th> <td>51</td> <td>4</td> <td>3</td> <td>4</td> <td>62</td> </tr> <tr class="basic-table__second-level-row"> <th>ヒステリー</th> <td>16</td> <td>0</td> <td>1</td> <td>0</td> <td>17</td> </tr> <tr class="basic-table__colored-row"> <th>循環器障害</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>高血圧</th> <td>87</td> <td>20</td> <td>11</td> <td>10</td> <td>128</td> </tr> <tr class="basic-table__second-level-row"> <th>頻脈</th> <td>85</td> <td>17</td> <td>10</td> <td>7</td> <td>119</td> </tr> <tr class="basic-table__colored-row"> <th>内分泌代謝異常</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>電解質異常（SIADH含む）</th> <td>45</td> <td>2</td> <td>2</td> <td>4</td> <td>53</td> </tr> <tr class="basic-table__second-level-row"> <th>糖代謝異常</th> <td>19</td> <td>2</td> <td>0</td> <td>2</td> <td>23</td> </tr> <tr class="basic-table__second-level-row"> <th>甲状腺機能異常</th> <td>6</td> <td>3</td> <td>1</td> <td>2</td> <td>12</td> </tr> <tr class="basic-table__colored-row"> <th>皮膚症状</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>日光紅斑</th> <td>0</td> <td>10</td> <td>3</td> <td>0</td> <td>13</td> </tr> <tr class="basic-table__second-level-row"> <th>色素沈着</th> <td>1</td> <td>19</td> <td>3</td> <td>1</td> <td>24</td> </tr> <tr class="basic-table__second-level-row"> <th>爪・指などの変形・欠損</th> <td>0</td> <td>0</td> <td>0</td> <td>0</td> <td>0</td> </tr> <tr class="basic-table__colored-row"> <th>その他</th> <td> </td> <td> </td> <td> </td> <td> </td> <td> </td> </tr> <tr class="basic-table__second-level-row"> <th>肝障害</th> <td>45</td> <td>13</td> <td>6</td> <td>7</td> <td>71</td> </tr> <tr class="basic-table__second-level-row"> <th>暗褐色尿</th> <td>90</td> <td>19</td> <td>11</td> <td>12</td> <td>132</td> </tr> </tbody> </table> </div> </div> </figure> </div> <dl class="references"> <dt class="references__label">参考文献</dt> <dd class="references__content"> <ol class="references__list"> <li class="references__item"><span>Ventura P, Cappellini MD, Biolcati G, et al. <i>Eur J Intern Med</i>. 2014;25(6):497-505.</span></li> <li class="references__item"><span>Anderson KE, Bloomer JR, Bonkovsky HL, et al. <i>Ann Intern Med</i>. 2005;142(6):439-450.</span></li> <li class="references__item"><span>Puy H, Gouya L, Deybach JC. <i>Lancet</i>. 2010;375(9718):924-937.</span></li> <li class="references__item"><span>Balwani M. <i>Clin Adv Hematol Oncol</i>. 2016;14(11):858-861.</span></li> <li class="references__item"><span>近藤雅雄, 矢野雄三, 浦田郡平. <i>ALA-Porphyrin Science</i>. 2012;1(2):73-82.</span></li> </ol> </dd> </dl> <ul class="node-pc-pager"> <li class="node-pc-pager__item node-pc-pager__item--next"><a href="/symptom/importance-of-differential-diagnosis"><span class="node-pc-pager__title">AHPにおける鑑別診断の重要性</span></a></li> </ul> <ol class="node-sp-pager"> <li class="node-sp-pager__item is-active"><span>AHPの症状</span></li> <li class="node-sp-pager__item"><a href="/symptom/importance-of-differential-diagnosis">AHPにおける鑑別診断の重要性</a></li> <li class="node-sp-pager__item"><a href="/symptom/triggers-for-acute-attacks">急性発作の主な誘因</a></li> <li class="node-sp-pager__item"><a href="/symptom/complication-risk">長期罹患に伴う合併症リスク</a></li> </ol> </div> </div> </article> </div> </div> </div> </main> <div class="layout-modal"> <div class="region region-modal"> <div id="block-modal-confirm" class="block block-fixed-block-content block-fixed-block-contentmodal-confirm"> <div class="clearfix text-formatted field field--name-body field--type-text-with-summary field--label-hidden field__item"><div class="modal-confirm js-modal-confirm"> <div class="modal-confirm__inner"> <p class="modal-confirm__body">このサイトの情報は日本国内の医療関係者を対象とするもので、一般の方および国外の医療関係者に対する情報提供を目的としたものではありません。<br /> あなたは日本国内の医療関係者ですか？</p> <div class="modal-confirm__actions"><button class="modal-confirm__button js-ok-button" type="button">はい</button><a class="modal-confirm__button" href="https://www.alnylam.jp/">いいえ</a></div> </div> </div> </div> </div> <div id="block-waibusaitohenoqianyiquerenmotaru" class="block block-block-content block-block-contentb6a2a460-4152-4afe-999e-efdb2b3a1641"> <div class="clearfix text-formatted field field--name-body field--type-text-with-summary field--label-hidden field__item"><div class="modal-confirm js-modal-external-link"> <div class="modal-confirm__inner"> <p class="modal-confirm__head">外部サイトを<span class="nobreak">開こうとしています</span></p> <p class="modal-confirm__body">Alnylam Japanのウェブサイトをご覧いただきありがとうございます。<br /> これより先は外部サイトになります。<br /> 外部サイトの内容について、当社ではいかなる責任も負いません。<br /> 各サイトの利用条件に従ってご利用ください。</p> <p class="modal-confirm__confirm">上記の内容に留意して<span class="nobreak">外部サイトを開きますか？</span></p> <div class="modal-confirm__actions"><button class="modal-confirm__button js-ok-button" type="button">はい</button><a class="modal-confirm__button js-cancel-button" href="https://www.alnylam.jp/">いいえ</a></div> </div> </div> </div> </div> </div> </div> <a href="#" class="layout-scroll-top js-scroll-top">ページトップ</a> <footer role="contentinfo" class="layout-footer layout-container-footer"> <div class="layout-footer-inner"> <a href="https://www.alnylam.jp/" class="layout-footer-logo" title="" target="_blank"> <img src="/themes/custom/porphyriajp/images/footer-logo.png" alt="Alnylam PHARMACEUTICALS" width="193" height="51"> </a> <div class="layout-footer-content"> <div class="layout-footer-menu"> <div class="region region-footer"> <nav role="navigation" aria-labelledby="block-porphyriajp-footer-menu" id="block-porphyriajp-footer" class="block block-menu navigation menu--footer"> <h2 class="visually-hidden" id="block-porphyriajp-footer-menu">Footer menu</h2> <ul class="menu"> <li class="menu-item"> <a href="https://alnylampolicies.com/ja/privacy" target="_blank">プライバシーポリシー</a> </li> <li class="menu-item"> <a href="https://alnylampolicies.com/ja/legal" target="_blank">ご利用に関して</a> </li> <li class="menu-item"> <a href="https://alnylampolicies.com/contact#japan" target="_blank">お問い合わせ</a> </li> <li class="menu-item"> <a href="/sitemap" data-drupal-link-system-path="node/12">サイトマップ</a> </li> </ul> </nav> </div> </div> <p class="layout-footer-copyright">© 2020-2024 Alnylam Japan K.K. <span>All rights reserved. NP-JPN-00059</span></p> </div> </div> </footer> </div> </div> <script type="application/json" data-drupal-selector="drupal-settings-json">{"path":{"baseUrl":"\/","scriptPath":null,"pathPrefix":"","currentPath":"node\/6","currentPathIsAdmin":false,"isFront":false,"currentLanguage":"en"},"pluralDelimiter":"\u0003","suppressDeprecationErrors":true,"user":{"uid":0,"permissionsHash":"251d185dd7d7cbc9fda2747534d59d514ecd3a8330a57e8811009afcb6b97256"}}</script> <script src="/sites/default/files/js/js_ae_-I5PVAIqb6vW_3MqFI-j7q1PvW6IZYJ86tvB56-A.js"></script> </body> </html>

CINXE.COM

急性肝性ポルフィリン症（AHP）の症状 | Porphyria.jp