[{"@context":"https:\/\/schema.org\/","@type":"Article","@id":"https:\/\/share-dev.upmc.com\/2017\/08\/crigler-najjar-syndrome\/#Article","mainEntityOfPage":"https:\/\/share-dev.upmc.com\/2017\/08\/crigler-najjar-syndrome\/","headline":"Rare, Hereditary Conditions: Crigler-Najjar Syndrome","name":"Rare, Hereditary Conditions: Crigler-Najjar Syndrome","description":"Crigler-Najjar syndrome is a rare hereditary condition that often affects children from Amish and Mennonite families. Learn more about this illness.","datePublished":"2017-08-18","dateModified":"2021-11-26","author":{"@type":"Organization","@id":"https:\/\/www.upmc.com\/services\/transplant","name":"Transplant Services","url":"https:\/\/www.upmc.com\/services\/transplant","sameAs":"https:\/\/share-dev.upmc.com\/transplant-services\/","parentOrganization":"UPMC"},"publisher":{"@type":"Organization","name":"UPMC HealthBeat","logo":{"@type":"ImageObject","@id":"https:\/\/share-dev.upmc.com\/wp-content\/uploads\/2019\/04\/UPMC-HealthBeat-Logo.png","url":"https:\/\/share-dev.upmc.com\/wp-content\/uploads\/2019\/04\/UPMC-HealthBeat-Logo.png","width":600,"height":60}},"image":{"@type":"ImageObject","@id":"https:\/\/share-dev.upmc.com\/wp-content\/uploads\/2017\/06\/082017BLG_AMISHCRIGLERSYNDROME.jpg","url":"https:\/\/share-dev.upmc.com\/wp-content\/uploads\/2017\/06\/082017BLG_AMISHCRIGLERSYNDROME.jpg","height":325,"width":754},"url":"https:\/\/share-dev.upmc.com\/2017\/08\/crigler-najjar-syndrome\/","about":["Living and Wellness","Pediatrics"],"wordCount":462,"keywords":["crigler-najjar syndrome","hereditary conditions","living donor liver transplant"],"articleBody":"Post Updated March, 2021Crigler-Najjar syndrome is a rare hereditary condition that often affects children from Amish and Mennonite families.\u00a0Only 100 cases have been recorded.Crigler-Najjar syndrome occurs when high levels of\u00a0bilirubin\u00a0are in the blood. Bilirubin, a brownish yellow waste product found in bile, is created when the liver breaks down old red blood cells.Normally, the liver works to convert bilirubin and remove it from the body. For children with Crigler-Najjar syndrome, the toxic bilirubin is left in the body, causing the child to become very sick.Through a collaborative approach, UPMC works with partner hospitals to coordinate complex transplant surgery and pre- and post-surgical care for patients who need it most. Learn more at the UPMC Complex Care Connect\u2122 program website.Types of Crigler-Najjar SyndromeDoctors recognize two types of Crigler-Najjar syndrome:Type I:\u00a0These patients are missing an important liver enzyme called glucuronosyl transferase. This form of Crigler-Najjar syndrome can be life-threatening.Type II:\u00a0These patients have much milder symptoms because the liver enzyme is not missing but is severely limited.Signs and Symptoms for Crigler-Najjar SyndromeThe most noticeable symptom of Crigler-Najjar syndrome is jaundice, or when\u00a0a child\u2019s eyes and skin take on a yellow tone. Other symptoms include:Stomach painDiarrhea and vomitingFeverConfusionSlurred speechDifficulty swallowingSeizuresTreatments for Crigler-Najjar SyndromeThe most common treatment for Crigler-Najjar syndrome is\u00a0phototherapy. This is when a child\u2019s skin is exposed to a very bright LED light, similar to a tanning bed.This light breaks down bilirubin so that it is no longer toxic. While helpful, phototherapy is a tedious process requiring many hours of therapy each day.The other treatment option for Crigler-Najjar syndrome is a liver transplant. Because the new liver can convert bilirubin and remove it from the body, this can be a life-saving solution.To learn more about becoming a living donor, visit the Liver Transplant Waiting List.\u00a0Why Living-Donor Liver Transplantation?During a liver transplant, a child\u2019s damaged liver is replaced with a healthy liver from a deceased person or a piece of a healthy liver from a living donor.Unfortunately, the wait for a transplant from a deceased donor can take years. A living-donor liver transplant can eliminate the wait for children with Crigler-Najjar syndrome.During a\u00a0living donor transplant, a piece of liver from a healthy adult is removed and transplanted into a child in need.A living donor must be:Between the ages of 18 and 55In general good healthHave an unselfish desire to contribute to another person\u2019s life in a healthy wayBy\u00a0becoming a living donor for a child\u00a0with Crigler-Najjar syndrome, you can help to reduce the transplant waiting list and give that child a second chance at life. "},{"@context":"https:\/\/schema.org\/","@type":"BreadcrumbList","itemListElement":[{"@type":"ListItem","position":1,"name":"2017","item":"https:\/\/share-dev.upmc.com\/2017\/#breadcrumbitem"},{"@type":"ListItem","position":2,"name":"08","item":"https:\/\/share-dev.upmc.com\/2017\/\/08\/#breadcrumbitem"},{"@type":"ListItem","position":3,"name":"Rare, Hereditary Conditions: Crigler-Najjar Syndrome","item":"https:\/\/share-dev.upmc.com\/2017\/08\/crigler-najjar-syndrome\/#breadcrumbitem"}]}]