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  1. Crigler-Najjar Syndrome

1. MedlinePlus Medical Encyclopedia: Crigler-Najjar Syndrome
criglernajjar syndrome is an inherited disorder in which bilirubin (a substance crigler-najjar syndrome is caused by an abnormal gene which fails to
http://www.nlm.nih.gov/medlineplus/ency/article/001127.htm
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Crigler-Najjar syndrome
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Illustrations
Liver anatomy Alternative names Return to top Glucuronyl transferase deficiency (type I Crigler-Najjar); Arias syndrome (type II Crigler-Najjar) Definition Return to top Crigler-Najjar syndrome is an inherited disorder in which bilirubin (a substance made by the liver) cannot be changed into its water-soluble form, bilirubin glucuronide. This causes jaundice (yellow discoloration of skin and eyes) and organ malfunctions. Causes, incidence, and risk factors Return to top Crigler-Najjar syndrome is caused by an abnormal gene which fails to produce a functional enzyme (bilirubin glucuronyltransferase) capable of converting bilirubin into a water-soluble and therefore, easily excreted form. As a result, bilirubin can build up in the body, which can damage the brain and other organs. The syndrome is inherited as an autosomal recessive trait. This means that the child must get the defective gene from both parents to develop the severe form of the condition. Parents who are carriers (with just one defective gene) have about half the enzyme activity of a normal adult.

2. ? Crigler-Najjar Syndrome
A medical encycopedia article on the topic crigler-najjar syndrome
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3. EMedicine - Crigler-Najjar Syndrome : Article By Alessio Pigazzi, MD, PhD
criglernajjar syndrome - crigler-najjar syndrome (CNS) is a rare disorder ofbilirubin metabolism and has 2 distinct forms type 1 and type 2.
http://www.emedicine.com/med/topic476.htm
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Crigler-Najjar Syndrome
Last Updated: August 26, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: CNS, Crigler-Najjar disease, Gilbert syndrome, Arias syndrome, congenital nonhemolytic jaundice, neonatal jaundice, inherited unconjugated hyperbilirubinemias, uridine diphosphate glycosyltransferase, UGT, kernicterus, bilirubin encephalopathy, plasma exchange transfusion AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography
Author: Alessio Pigazzi, MD, PhD , Head, Minimally Invasive Surgery Program, Division of Surgery, Department of General Oncologic Surgery, City of Hope National Medical Center Coauthor(s): Stefano Ravalli, MD , Assistant Professor, Department of Medicine, Division of Cardiology, Columbia University College of Physicians and Surgeons Editor(s): Tushar Patel, MD

4. Crigler-Najjar Syndrome - A Medical Reference Article
Medical Encyclopdia article about criglernajjar syndrome
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5. EMedicine - Gilbert Syndrome : Article By Sandeep Mukherjee, MD
of hyperbilirubinemia in family members of patients with criglernajjar syndrome.Gilbert syndrome can also frequently coexist with the conditions
http://www.emedicine.com/med/topic870.htm
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Gilbert Syndrome
Last Updated: January 21, 2005 Rate this Article Email to a Colleague Synonyms and related keywords: constitutional hepatic dysfunction, constitutional hyperbilirubinemia, familial nonhemolytic jaundice, hereditary nonhemolytic bilirubinemia, low-grade chronic hyperbilirubinemia AUTHOR INFORMATION Section 1 of 9 Author Information Introduction Clinical Differentials ... Bibliography
Author: Sandeep Mukherjee, MD , Assistant Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center Sandeep Mukherjee, MD, is a member of the following medical societies: American College of Gastroenterology American Society for Gastrointestinal Endoscopy American Society of Transplantation , and Royal College of Physicians and Surgeons of Canada Editor(s): Manoop S Bhutani, MD, FACG, FACP

6. Dr. Koop - Crigler-Najjar Syndrome
criglernajjar syndrome. Injury Disease Nutrition Poison Symptoms Surgery Test Special Topic
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7. EMedicine - Crigler-Najjar Syndrome Article By Alessio Pigazzi
criglernajjar syndrome - crigler-najjar syndrome (CNS) is a rare disorder of bilirubin metabolism and has 2 distinct forms type 1 and type 2. Type
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

8. ► Crigler-Najjar Syndrome
A medical encycopedia article on the topic crigler-najjar syndrome.
http://www.umm.edu/ency/article/001127.htm
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Crigler-Najjar syndrome
Overview Symptoms Treatment Prevention Definition:
Crigler-Najjar syndrome is an inherited disorder in which bilirubin (a substance made by the liver) cannot be changed into its water-soluble form, bilirubin glucuronide. This causes jaundice (yellow discoloration of skin and eyes) and organ malfunctions.
Alternative Names: Glucuronyl transferase deficiency (type I); Arias syndrome (type II Crigler-Najjar)
Causes, incidence, and risk factors: Crigler-Najjar syndrome is caused by an abnormal gene which fails to produce a functional enzyme (bilirubin glucuronyltransferase) capable of converting bilirubin into a water-soluble and therefore, easily excreted form. As a result, bilirubin can build up in the body, which can damage the brain and other organs. The syndrome is inherited as an autosomal recessive trait. This means that the child must get the defective gene from both parents to develop the severe form of the condition. Parents who are carriers (with just one defective gene) have about half the normal enzyme activity of a normal adult. Infants who inherit the trait from both parents (this is called homozygous for the abnormal gene) develop severe jaundice (hyperbilirubinemia) beginning a few days after birth. If these infants are not treated, they may develop

9. Crigler-Najjar Syndrome (www.whonamedit.com)
criglernajjar syndrome A familial form of congenital hyperbilirubinemia associated with severe disorders of the central nervous system and
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

10. ► Crigler-Najjar Syndrome
A medical encycopedia article on the topic crigler-najjar syndrome.
http://www.umm.edu/ency/article/001127sym.htm
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Crigler-Najjar syndrome
Overview Symptoms Treatment Prevention Alternative Names:
Glucuronyl transferase deficiency (type I); Arias syndrome (type II Crigler-Najjar)
Symptoms:
  • A family history of Crigler-Najjar syndrome Yellow skin (jaundice) and eyes (icterus) that begins on the 2nd or 3rd day of life and progressively worsens Jaundice that persists beyond 2 weeks of life without an obvious cause Confusion and changes in thinking (resulting from brain toxicity of bilirubin)

Signs and tests: Tests used to evaluate the liver function include:
Liver anatomy
Review Date: 11/8/2002
Reviewed By: David G. Brooks, M.D., Ph.D., Division of Medical Genetics, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

11. RU News First International Conference Treatment Of Crigler-Najjar
What Treatment of criglernajjar syndrome First international conference on rare, fatal liver disease that affects Amish, Mennonites and
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12. Crigler-Najjar Syndrome
Article describes criglernajjar syndrome, its symptoms, diagnosis, and treatment.
http://rarediseases.about.com/od/rarediseasesc/a/021305.htm
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Elsewhere on the Web The Crigler-Najjar Association MedlinePlus: Crigler-Najjar Syndrome Suggested Reading Kernicterus: Preventable Infant Brain Damage on the Rise Most Popular Muscular Dystrophy ALD and Lorenzo's Oil Progeria Syndromes Leprosy (Hansen's Disease) ... The Elephant Man What's Hot Muscular Dystrophy Autoimmune Kidney Disease Minamata Disease Charcot-Marie-Tooth Disease ... Meckel-Gruber Syndrome adunitCM(150,100,'x55')

13. CRIGLER-NAJJAR SYNDROME
criglernajjar syndrome - A rare genetic defect (autosomal recessive) where there is the inability to form bilirubin glucuronide due to the
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

14. Crigler-Najjar Syndrome
criglernajjar syndrome is an inherited disorder of bilirubin metabolism.Prompt recognition of the syndrome in infancy and treatment of the excess
http://rarediseases.about.com/b/a/146277.htm
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February 13, 2005
Crigler-Najjar Syndrome
Crigler-Najjar syndrome is an inherited disorder of bilirubin metabolism. Prompt recognition of the syndrome in infancy and treatment of the excess bilirubin in the body prevents brain, muscle, and nerve damage. Email to a Friend
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15. Crigler-Najjar3bis
Mutations in criglernajjar syndrome patients.
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16. Crigler-Najjar Syndrome (www.whonamedit.com)
criglernajjar syndrome A familial form of congenital hyperbilirubinemia associatedwith severe disorders of the central nervous system and resembling
http://www.whonamedit.com/synd.cfm/86.html

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Whonamedit.com does not give medical advice.
This survey of medical eponyms and the persons behind them is meant as a general interest site only. No information found here must under any circumstances be used for medical purposes, diagnostically, therapeutically or otherwise. If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here: see a doctor.
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Crigler-Najjar syndrome Also known as: Arias’ syndrome Synonyms: Congenital familial non-haeomolytic jaundice, congenital non-hemolytic jaundice, congenital non-heomlytic jaundice with kernicterus. Associated persons: Irwin Monroe Arias John Fielding Crigler Victor Assad Najjar Description: A familial form of congenital hyperbilirubinemia associated with severe disorders of the central nervous system and resembling kernicterus (degeneration of the basal ganglia). Other symptoms include jaundice of the skin, sclera, and mucous membrane. The syndrome is caused by an enzyme deficiency in the liver and faulty bilirubin conjugation. Both sexes affected. Presents with severe jaundice in the first few days after birth. Inheritance is autosomal dominant with incomplete penetration and varied expressivity. A deficiency of glucuronide formation was demonstrated by Irwin Monroe Arias in an anicteric father of two jaundiced children with a defect of hepatic glucuronyl transferase activity. This is sometimes referred to as Crigler-Najjar, type II. In Type I (above) death from kernicterus usually occurs within 15 months after birth. In Type II, which has been called Arias’ syndrome, the patient may survive to young adult life.

17. Crigler-Najjar
Information and useful links on criglernajjar syndrome
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18. John Fielding Crigler (www.whonamedit.com)
Crigler American paediatrician, born September 11, 1919, Charlotte, NorthCarolina. Associated with criglernajjar syndrome,Najjar-Crigler icterus.
http://www.whonamedit.com/doctor.cfm/69.html

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Whonamedit.com does not give medical advice.
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Hypography
is an open community about science and all things related
John Fielding Crigler American paediatrician, born September 11, 1919, Charlotte, North Carolina. Associated eponyms: Crigler-Najjar syndrome A familial form of congenital hyperbilirubinemia associated with severe disorders of the central nervous system and resembling kernicterus (degeneration of the basal ganglia). Najjar-Crigler icterus Hyperbilirubinaemia in newborn in Crigler-Najjar syndrome. Biography: John Fielding Crigler was educated at Duke University and the Johns Hopkins School of medicine, graduating in 1943. He specialised in paediatrics and in 1955 became a member of the staff of the Children’s Hospital, Boston, where he became chief of the division of endocrinology in 1965. Ole Daniel Enersen

19. CLF Gilbert's Syndrome
CLF Regional Offices. Gilbert's Syndrome What is Gilbert's Syndrome? Gilbert's Syndrome is a fairly common, mild liver disorder.
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20. Crigler-Najjar Syndrome
criglernajjar syndrome Web Pages - Welcome to the crigler-najjar syndrome Web Advertisement crigler-najjar syndrome - A rare genetic defect (autosomal
http://www.health-nexus.com/crigler-najjar_syndrome1.htm
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Crigler-Najjar Syndrome
Crigler-Najjar Syndrome Web Pages Welcome to the Crigler-Najjar Syndrome Web Site This is a web site devoted to children and adults with the Crigler-Najjar syndrome and their families. ...
www.icondata.com ...files/CRIGLER- ...PEDBASE) Discipline: GI Last Updated: 12/26/94 CRIGLER-NAJJAR SYNDROME - I DEFINITION: An inherited disorder of bilirubin...bili-rubin in the liver there are two forms of Crigler-Najjar Syndrome...
Crigler-Najjar syndrome
CRIGLER-NAJJAR SYNDROME Welcome to .... Advertisement CRIGLER-NAJJAR SYNDROME - A rare genetic defect (autosomal recessive) where there is the inability to form...
MEDLINEplus Medical Encyclopedia: Crigler-Najjar syndrome Crigler-Najjar syndrome. ... Causes and risks Return to top. Crigler-Najjar syndrome is inherited as an autosomal recessive trait. Parents ...

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