Alagille syndrome is a genetic disorder that affects the liver, heart, and other systems of the body. Problems associated with the disorder generally become evident in infancy or early childhood. The disorder is inherited in an autosomal dominant pattern, and the estimated prevalence of Alagille syndrome is 1 in every 70,000 live births.
The severity of the disorder can vary within the same family, with symptoms ranging from so mild as to go unnoticed to severe heart and/or liver disease requiring transplantation.
Signs and symptoms arising from liver damage in Alagille syndrome may include a yellowish tinge in the skin and the whites of the eyes (jaundice), itching, and deposits of cholesterol in the skin (xanthomas). A liver biopsy may indicate too few bile ducts (bile duct paucity). Other signs of Alagille syndrome include congenital heart problems, particularly Tetralogy of Fallot, and an unusual butterfly shape of the bones of the spinal column that can be seen in an x-ray. Many people with Alagille syndrome have similar facial features, including a broad, prominent forehead, deep-set eyes, and a small pointed chin. The kidneys and central nervous system may also be affected.
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Chronic Illness
Liver :: Digestive Disorders
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Alagille Syndrome - Discussion of the genetics, clinical presentation, natural history, and treatment of Alagille's syndrome.
Meta Description: [ Description of clinical features, genetics and treatment of Alagille's syndrome. ]
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Alagille Syndrome - A rare inherited disorder in which there are fewer than normal bile ducts in the liver. Information by Cincinnati Children's Hospital Medical Center.
Meta Description: [ Cincinnati Children's Hospital Medical Center contact information, copyright, privacy, endorsement, legal and liability disclaimer policy and information. ]
Alagille Syndrome Alliance - Worldwide support network for people who care about people with alagille syndrome.
Meta Description: [ The official Alagille Syndrome Alliance Worldwide Web Site. This page includes extensive information
about Alagille Syndrome and information on how to contact the Alliance board of directors ]
Canadian Liver Foundation: Alagille Syndrome - Discusses what it is, causes, symptoms, and treatments, including dietary modifications.
Children's Liver Alliance - A description, the diagnosis, symptoms, treatment and complications of Alagille syndrome.
Meta Description: [ biliary atresia,liver disease,liver,children,liverkids ]
Dept. of Dermatology - University of Iowa College of Medicine - Images of Alagille's Syndrome.
NORD: Alagille Syndrome - The synonyms, a general discussion and further resources.
Meta Description: [ National Organization for Rare Disorders is dedicated to helping people with rare, orphan diseases. Rarediseases.org contains information on the prevention, treatment and cure of rare diseases. ]
Pediatric Oncall - Offers an introduction to Alagille syndrome and a case study.
Meta Description: [ ALAGILLE SYNDROME CASE REPORTS PEDIATRIC ONCALL ]
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