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<article role="article" about="/home" typeof="schema:WebPage" class="node node--type-page node--view-mode-full"> <span property="schema:name" content="急性肝性ポルフィリン症（AHP）とは？" 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"><div class="hero"> <div class="hero__image-area"> <div class="hero__image-area-inner"><picture> <source media="(max-width: 767px)" srcset="/themes/custom/porphyriajp/images/frontpage/hero-sp.webp" type="image/webp"></source> <source media="(max-width: 767px)" srcset="/themes/custom/porphyriajp/images/frontpage/hero-sp.png"></source> <source media="(max-width: 1199px)" srcset="/themes/custom/porphyriajp/images/frontpage/hero-pc.webp" type="image/webp"></source> <source media="(max-width: 1199px)" srcset="/themes/custom/porphyriajp/images/frontpage/hero-pc.png"></source> <source srcset="/themes/custom/porphyriajp/images/frontpage/hero-xl.webp 1x,
 /themes/custom/porphyriajp/images/frontpage/hero-xl@2x.webp 2x" type="image/webp"></source> <img alt="" height="448" src="/themes/custom/porphyriajp/images/frontpage/hero-xl.png" srcset="/themes/custom/porphyriajp/images/frontpage/hero-xl.png 1x,
 /themes/custom/porphyriajp/images/frontpage/hero-xl@2x.png 2x" width="845" /> </picture> <div class="hero__content"> <div class="hero__content-inner"> <p class="hero__catchcopy">繰り返す激しい腹痛などの<br /> 症状がみられたとき、<br /> 疑ってほしい疾患があります。</p> <p class="hero__text">急性肝性ポルフィリン症は、 <span class="hero__text-oneline">QOLに影響を及ぼす遺伝性の疾患です。</span><br /> 早期に診断し、<span class="hero__text-oneline">早期から適切な治療を行うことが重要です。</span></p> </div> </div> </div> </div> <div class="hero__movie-area-wrapper"> <div class="patient_site_kit-link-wrapper"><a href="/examination/screening-kit"><img alt="AHP-Info" src="/themes/custom/porphyriajp/images/examination/urinalysis-kit.webp" /><span class="patient_site_link-text">尿 PBG 迅速スクリーニングキット提供中</span></a></div> <div class="hero__movie-area"> <div class="hero__movie-area-inner"><iframe allowfullscreen="" class="embed-responsive-item" frameborder="0" height="360" mozallowfullscreen="" src="https://player.vimeo.com/video/1015725533?h=0f711ce0ba" style="border: 0;" webkitallowfullscreen="" width="640"></iframe></div> </div> </div> </div> <h1>急性肝性ポルフィリン症とは？</h1> <p>急性肝性ポルフィリン症（acute hepatic porphyria：AHP）は、ヘムの生合成過程に異常があることで発症する遺伝性の疾患です。急性間欠性ポルフィリン症（acute intermittent porphyria：AIP）をはじめ、4つの病型に分類されています。<br /> AHPは、激しい腹痛などの症状を伴う急性発作をきっかけに発症しますが、多様な臨床症状を呈することから、他の疾患と間違われて見逃されている可能性もあります。そのため、適切な治療を受けられず、長い間苦しんでいる患者さんが存在すると考えられます。<br /> 未だ不明な点が多いものの、合併症として高血圧や慢性腎臓病（chronic kidney disease：CKD）、肝細胞癌の発症リスクを高めることなどが報告されています。<br /> 本サイトでは、AHPについて知っていただきたい情報をまとめました。</p>  <div class="link-box-list"><a class="link-box-list__item link-box link-box--about-ahp" href="/about-AHP/porphyria-subtypes"><span class="link-box__content"><span class="link-box__content-inner"><span class="link-box__title">AHPについて</span> <span class="link-box__body"> 急性肝性ポルフィリン症は、<br /> QOLに影響を及ぼす遺伝性の疾患です。<br /> 早期に診断し、<br /> 早期から適切な治療を行うことが重要です。 </span> </span> </span> </a> <a class="link-box-list__item link-box link-box--symptom link-box--reverse" href="/symptom/AHP-symptoms"> <span class="link-box__content"> <span class="link-box__content-inner"> <span class="link-box__title">症状</span> <span class="link-box__body"> 腹痛を訴えて受診することが多く、<br /> 9割以上の患者が腹痛を主訴に受診したとの<br /> データもあります。<br /> 加えて、末梢神経系、中枢神経系、自律神経系などに<br /> 1つ以上の症状がみられます。 </span> </span> </span> </a> <a class="link-box-list__item link-box link-box--examination" href="/examination/urinalysis"> <span class="link-box__content"> <span class="link-box__content-inner"> <span class="link-box__title">検査</span> <span class="link-box__body"> AHP診断の手がかりとしては、<br /> 急性発作時に上昇する、尿中のアミノレブリン酸や<br /> ポルフォビリノーゲンを指標とした検査が有用です。<br /> 確定診断には、尿検査に加えて、<br /> 遺伝学的検査が必要になります。 </span> </span> </span> </a></div> <div class="patient_site_link-wrapper"><a href="https://ahp-info.jp/" target="_blank"><span class="patient_site_link-text">患者さん向け急性肝性<span>ポルフィリン症</span>(AHP)情報サイト</span><img alt="AHP-Info" src="/themes/custom/porphyriajp/images/ahp-info.png" /></a></div> </div> </div> </article> </div> </div> </div> </main> <div 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