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<link rel="alternative" hreflang="en" href="https://medlineplus.gov/ency/article/001127.htm"/><meta property="og:title" content="Crigler-Najjar syndrome: MedlinePlus Medical Encyclopedia"/><meta property="og:url" content="https://medlineplus.gov/ency/article/001127.htm"/><meta property="twitter:title" content="Crigler-Najjar syndrome: MedlinePlus Medical Encyclopedia"/><meta name="twitter:card" content="summary_large_image"/><meta name="description" content="Crigler-Najjar syndrome is a very rare inherited disorder in which bilirubin cannot be broken down. Bilirubin is a substance made by the liver."/><meta itemprop="description" name="description" content="Crigler-Najjar syndrome is a very rare inherited disorder in which bilirubin cannot be broken down. Bilirubin is a substance made by the liver."/><meta property="og:description" content="Crigler-Najjar syndrome is a very rare inherited disorder in which bilirubin cannot be broken down. Bilirubin is a substance made by the liver."/><meta property="twitter:description" content="Crigler-Najjar syndrome is a very rare inherited disorder in which bilirubin cannot be broken down. Bilirubin is a substance made by the liver."/><meta property="og:image" content="https://medlineplus.gov/images/share_ency_1.jpg"/><meta property="og:image" content="https://medlineplus.gov/images/share_ency_2.jpg"/><meta property="og:image" content="https://medlineplus.gov/images/share_ency_3.jpg"/><meta property="twitter:image" content="https://medlineplus.gov/images/share_ency_1.jpg"/>
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</div><div><span>Crigler-Najjar syndrome</span></div></div>
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<article><div id="d-article"><div class="page-info"><div class="page-title"><a name="start" id="start">
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</a><h1 class="with-also" itemprop="name">Crigler-Najjar syndrome</h1>
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</noscript></div><div class="main"><div id="ency_summary"><p>Crigler-Najjar syndrome is a very rare inherited disorder in which bilirubin cannot be broken down. Bilirubin is a substance made by the liver.</p></div><section><div class="section"><div class="section-header"><div class="section-title"><h2>Causes</h2></div><div class="section-button"><button type="submit" aria-controls="section-1" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-1"><p>An enzyme converts bilirubin into a form that can easily be removed from the body. Crigler-Najjar syndrome occurs when this enzyme does not work correctly. Without this enzyme, bilirubin can build up in the body and lead to:</p><ul><li>Jaundice (yellow discoloration of skin and eyes)</li><li>Damage to the brain, muscles, and nerves</li></ul><p>Type I Crigler-Najjar is the form of the disease that starts early in life. Type II Crigler-Najjar syndrome may start later in life.</p><p>The syndrome runs in families (inherited). A child must receive a copy of the variant gene from both parents to develop the severe form of the condition. Parents who are carriers (with just one variant gene) have about one half the enzyme activity of a normal adult, but do not have symptoms.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Symptoms</h2></div><div class="section-button"><button type="submit" aria-controls="section-2" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-2"><p>Symptoms may include:</p><ul><li><a test="test" href="./003205.htm">Confusion</a> and changes in thinking</li><li>Yellow skin (jaundice) and yellow in the whites of the eyes (icterus), which begin a few days after birth and get worse over time</li><li>Lethargy</li><li>Poor feeding</li><li>Vomiting</li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Exams and Tests</h2></div><div class="section-button"><button type="submit" aria-controls="section-3" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-3"><p>Tests of liver function include:</p><ul><li>Conjugated (bound) bilirubin</li><li>Total bilirubin level</li><li>Unconjugated (unbound) bilirubin in blood.</li><li><a test="test" href="./003332.htm">Enzyme assay</a></li><li><a test="test" href="./003895.htm">Liver biopsy</a></li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Treatment</h2></div><div class="section-button"><button type="submit" aria-controls="section-4" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-4"><p>Light treatment (phototherapy) is needed throughout a person's life. In infants, this is done using bilirubin lights (bili or 'blue' lights). Phototherapy does not work as well after age 4, because thickened skin blocks the light.</p><p>A liver transplant can be done in some people with type I disease.</p><p>Blood transfusions may help control the amount of bilirubin in blood. Calcium compounds are sometimes used to remove bilirubin in the gut.</p><p>The drug phenobarbitol is sometimes used to treat type II Crigler-Najjar syndrome.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Outlook (Prognosis)</h2></div><div class="section-button"><button type="submit" aria-controls="section-5" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-5"><p>Milder forms of the disease (type II) do not cause liver damage or changes in thinking during childhood. People who are affected with a mild form still have jaundice, but they have fewer symptoms and less organ damage.</p><p>Infants with the severe form of the disease (type I) may continue to have jaundice into adulthood, and may need daily treatment. If not treated, this severe form of the disease will lead to death in childhood.</p><p>People with this condition who reach adulthood will develop brain damage due to jaundice (<a test="test" href="./007309.htm">kernicterus</a>), even with regular treatment. The life expectancy for someone with type I disease is 30 years.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Possible Complications</h2></div><div class="section-button"><button type="submit" aria-controls="section-6" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-6"><p>Possible complications include:</p><ul><li><div>A form of brain damage caused by jaundice (kernicterus)</div></li><li><div>Chronic yellow skin/eyes</div></li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>When to Contact a Medical Professional</h2></div><div class="section-button"><button type="submit" aria-controls="section-7" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-7"><p>Seek genetic counseling if you are planning to have children and have a personal or family history of Crigler-Najjar.</p><p>Contact your health care provider if you or your newborn infant has jaundice that does not go away.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Prevention</h2></div><div class="section-button"><button type="submit" aria-controls="section-8" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-8"><p>Genetic counseling is recommended for people with a personal or family history of Crigler-Najjar syndrome who want to have children. Blood tests can identify people who carry the genetic variant.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Alternative Names</h2></div><div class="section-button"><button type="submit" aria-controls="section-Alt" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Alt"><p>Glucuronyl transferase deficiency (type I Crigler-Najjar); Arias syndrome (type II Crigler-Najjar)</p></div></div></section><section><div class="section sec-mb"><div class="section-header"><div class="section-title"><h2>Images</h2></div><div class="section-button"><button type="submit" aria-controls="section-tnails" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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<li><img src="//medlineplus.gov/ency/images/ency/tnails/8848t.jpg" alt="Liver anatomy" title="Liver anatomy" class="side-img"/><a href="../imagepages/8848.htm">Liver anatomy</a></li>
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</ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>References</h2></div><div class="section-button"><button type="submit" aria-controls="section-Ref" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Ref"><p>Bonn J, Balistreri WF. Metabolic diseases of the liver. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. <em>Nelson Textbook of Pediatrics</em>. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 405.</p><p>Kaplan M, Wong RJ, Bensen R, Sibley E, Stevenson DK. Neonatal jaundice and liver diseases. In: Martin RJ, Fanaroff AA, Walsh MC, eds. <em>Fanaroff and Martin's Neonatal-Perinatal Medicine</em>. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 95.</p><p>Lidofsky SD. Jaundice. In: Feldman M, Friedman LS, Brandt LJ, eds. <em>Sleisenger and Fordtran's Gastrointestinal and Liver Disease</em>. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 21.</p></div></div></section>
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<section><div class="section"><div class="section-header"><div class="section-title"><h2>Review Date 8/18/2024</h2></div><div class="section-button"><button type="submit" aria-controls="section-version" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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