急性腹症に潜むAHP

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class="top-level-title-block__title">急性腹症に潜むAHP</h1> </div> </div> </div> <div id="block-porphyriajp-content" class="block block-system block-system-main-block"> <article role="article" about="/acute-abdomen-in-AHP" typeof="schema:WebPage" class="node node--type-page node--view-mode-full"> <span property="schema:name" content="急性腹症に潜むAHP" 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>急性腹症に潜むAHPの診断</h1> <p>フィンランドの大学病院に入院した15歳以上の急性腹症患者を前向きに調査した研究によると、急性腹症の33.0％(211/639例)は、診断のつかない非特異的腹痛(non-specific abdominal pain：NSAP)であることが報告されています<sup>1</sup>。近年、検査技術の進歩により確定診断に至る割合は高くなりつつありますが、原因疾患の鑑別が困難な場合も存在します。</p> <div class="image-box"> <figure class="scalable-figure acute-abdomen-in-ahp__image"> <figcaption class="scalable-figure__caption">急性腹症の疾患別割合（海外データ）</figcaption> <div class="js-scalable-container"> <div class="js-scalable-content scalable-content"><picture>  <img alt="急性腹症の疾患別割合" src="/themes/custom/porphyriajp/images/acute-abdomen-in-AHP/percentage-of-acute-abdomen-by-disease.png" /> </picture></div> </div> <p class="scalable-figure__number_of_samples">（n=639）</p> </figure> </div> <p>腹痛は多くの急性肝性ポルフィリン症（acute hepatic porphyria：AHP）患者に共通してみられる症状ですが、本邦におけるポルフィリン症の調査(1920～2010年)によるとAHPの初期診断として急性腹症が最も多く<sup>2</sup>、「急性腹症診療ガイドライン2015」の中でも鑑別すべき疾患の1つとして挙げられています<sup>3</sup>。NSAPの中にAHPが潜在している可能性も考えられます。</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初期診断</figcaption> <div class="js-scalable-container"> <div class="js-scalable-content scalable-content"> <table class="basic-table basic-table--with-summary-column misdiagnosis-example-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> <th>急性腹症</th> <td>50</td> <td>13</td> <td>12</td> <td>19</td> <td>94</td> </tr> <tr> <th>イレウス</th> <td>28</td> <td>3</td> <td>3</td> <td>5</td> <td>39</td> </tr> <tr> <th>虫垂炎</th> <td>15</td> <td>0</td> <td>1</td> <td>3</td> <td>19</td> </tr> <tr> <th>ヒステリー、心因性反応</th> <td>15</td> <td>0</td> <td>0</td> <td>0</td> <td>15</td> </tr> <tr> <th>妊娠悪阻</th> <td>6</td> <td>1</td> <td>1</td> <td>2</td> <td>10</td> </tr> <tr> <th>急性膵炎</th> <td>9</td> <td>2</td> <td>1</td> <td>1</td> <td>13</td> </tr> <tr> <th>てんかん</th> <td>2</td> <td>0</td> <td>7</td> <td>3</td> <td>12</td> </tr> <tr> <th>急性胃炎、胃・十二指腸潰瘍</th> <td>4</td> <td>0</td> <td>2</td> <td>2</td> <td>8</td> </tr> <tr> <th>肝障害</th> <td>4</td> <td>1</td> <td>1</td> <td>0</td> <td>6</td> </tr> <tr> <th>ギラン・バレー症候群</th> <td>2</td> <td>2</td> <td>0</td> <td>0</td> <td>4</td> </tr> <tr> <th>軸捻転（卵巣）</th> <td>2</td> <td>0</td> <td>0</td> <td>1</td> <td>3</td> </tr> <tr> <th>胆石</th> <td>1</td> <td>1</td> <td>0</td> <td>0</td> <td>2</td> </tr> <tr> <th>異所性妊娠</th> <td>2</td> <td>0</td> <td>0</td> <td>0</td> <td>2</td> </tr> <tr> <th>スモン</th> <td>2</td> <td>0</td> <td>0</td> <td>0</td> <td>2</td> </tr> <tr> <th>日光皮膚炎</th> <td>0</td> <td>1</td> <td>1</td> <td>0</td> <td>2</td> </tr> <tr> <th>腎・尿路結石</th> <td>1</td> <td>0</td> <td>0</td> <td>0</td> <td>1</td> </tr> <tr> <th>ミエロパチー</th> <td>1</td> <td>0</td> <td>0</td> <td>0</td> <td>1</td> </tr> <tr> <th>その他</th> <td>1</td> <td>0</td> <td>2</td> <td>0</td> <td>3</td> </tr> <tr> <th>総計</th> <td>145</td> <td>24</td> <td>31</td> <td>36</td> <td>236</td> </tr> </tbody> </table> </div> </div> </figure> </div> <h2>AHP患者Aさんの声（50代女性）</h2> <p class="patient-voice">学生の頃から激しい腰痛や腹痛がたびたびあらわれるようになり、長年その原因がわからないままでした。「AHP」と診断されるまでに要した期間は30年以上。学生時代から、結婚、子育て、就職を経て現在に至るまで、つらい症状を抱えながら大変な日々を過ごされてきました。病名が明らかになっても、治らない病気であることを知り、簡単にポジティブな気持ちにはなれませんでした。それでも最近では、ゆっくり、マイペースでやれることをやっていこうと、少しでも自分らしく前向きな気持ちで日常生活を送られています。</p> <div class="patient-voice__journey-container"> <div class="patient-voice__journey scrollable-wrapper"><img alt="Aさんのペイシェントジャーニー" class="patient-voice__journey-image scrollable" src="/themes/custom/porphyriajp/images/patient_a_journey.png" /></div> </div> <p class="patient-voice__journey-note">患者インタビューをもとに作成</p> <p>AHPでは確定診断されるまで長い年月を要するケースがあり、患者は原因不明のまま複数の医療機関又は診療科を受診している現状があります。<br /> 腹痛のほかにも、四肢の痛みや筋力低下などの末梢神経症状、不安や幻想・妄想などの中枢神経症状、便秘や嘔吐などの自律神経症状等、<a href="/symptom/AHP-symptoms">様々な症状</a>が認められ、他の疾患との鑑別が必要になります。<br /> 病院総合診療医は、特定の症状にかかわらずどのような疾患にも対応し診療を行います。家庭医と連携を取り支援し、専門医とも連携を取り専門的治療も実施します。原因不明の腹痛の中にはAHPに限らず診断に難渋する稀な疾患が少なからず潜んでいる可能性があり、未診断患者に対する速やかな診断も病院総合診療医の役割の1つといえます。</p> <h2 id="gp_case_anchor">病院総合診療医の取り組み事例</h2> <div class="gp_case_container__wrapper"> <div class="gp_case_container"><a class="gp_case" href="/acute-abdomen-in-AHP/case-of-fukuoka-u"><span class="gp_case__institution">福岡大学病院<span class="gp_case__department">総合診療部</span></span></a> <a class="gp_case" href="/acute-abdomen-in-AHP/case-of-nms"><span class="gp_case__institution">日本医科大学付属病院<span class="gp_case__department">救急・総合診療センター</span></span></a> <a class="gp_case" href="/acute-abdomen-in-AHP/case-of-saga-u"><span class="gp_case__institution">佐賀大学医学部附属病院<span class="gp_case__department">総合診療部</span></span></a><a class="gp_case" href="/acute-abdomen-in-AHP/case-of-asahikawa-med"><span class="gp_case__institution">旭川医科大学病院<span class="gp_case__department">総合診療部</span></span></a><a class="gp_case" href="/acute-abdomen-in-AHP/case-of-gifu-u"><span class="gp_case__institution">岐阜大学医学部附属病院<span class="gp_case__department">総合内科</span></span></a><a class="gp_case" href="/acute-abdomen-in-AHP/case-of-okayama-u"><span class="gp_case__institution">岡山大学病院<span class="gp_case__department">総合内科・総合診療科</span></span></a><a class="gp_case" href="/acute-abdomen-in-AHP/case-of-nagasaki-u"><span class="gp_case__institution">長崎大学病院<span class="gp_case__department">総合診療科</span></span></a></div> </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>Miettinen P, Pasanen P, Lahtinen J, et al. <i>Ann Chir Gynaecol</i>. 1996;85(1):5-9.</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> <li class="references__item"><span>急性腹症診療ガイドライン出版委員会編. 急性腹症診療ガイドライン2015, 第1版, 医学書院, 2015. </span></li> </ol> </dd> </dl> </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 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