21. Home criglernajjar syndrome is caused by a liver enzyme deficiency. Crigler-NajjarSyndrome (CNS) is an inherited metabolic disorder caused by a liver enzyme http://www.criglernajjar.com/

Our Site Last Updated June 29, 2003 Crigler-Najjar Syndrome is caused by a liver enzyme deficiency Crigler-Najjar Syndrome (CNS) is an inherited metabolic disorder caused by a liver enzyme deficiency which prevents the usual metabolic breakdown of bilirubin; a normal by-product in our body’s disposal of worn-out red blood cells. Children with CNS are unable to eliminate bilirubin from their bodies and therefore must undergo daily 12-hour exposure to special blue lights, just to survive. Without daily treatments, a child would suffer brain damage, muscle and nerve damage and death due to bilirubin toxicity. A Rare Orphan Disease Crigler-Najjar is a very rare disease. There are less than 50 known cases in the USA, and less than 200 worldwide. It is named after Dr. Crigler and Dr. Najjar who discovered the disease. Dr. John F. Crigler is a Special Medical Two-year-old Melanie has to sleep under intense blue lights to help combat the disease Crigler-Najjar Syndrome Advisor to the CNA. The Crigler-Najjar Association Providing Support and Helping Others Our group represents approximately 30 families in the U.S.A. who have family members affected by Crigler-Najjar syndrome. Started in 2002 with a handful of Crigler-Najjar families, our group has grown and expanded to represent all families who are interested in providing support, information and helping others who are less fortunate that may not have the ability to pay for expensive phototherapy lights and medications required just to live.

22. Crigler-Najjar Syndrome Medical Information criglernajjar syndrome Information from Drugs.com. http://www.drugs.com/enc/crigler_najjar_syndrome.html

Home New Drugs Latest News Drug Interactions ... Forum Drugs.com - prescription drug and medicine information available on over 24,000 approved- medications and pharmaceuticals, including side effects and drug interactions. Log-in Register Advertisement Buy a Link Now Special Offers From our sponsors Categories Diabetes Gastro Center Weight Loss AIDS/HIV ... Sexual Health Advertisement Advanced Search Or click the first letter of a drug name: A B C D ... Z Crigler-Najjar syndrome Injury Disease Nutrition Poison ... Liver anatomy Crigler-Najjar syndrome 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 Crigler-Najjar); Arias syndrome (type II Crigler-Najjar) Causes 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.

23. Crigler-Najjar Syndrome criglernajjar syndrome Type II, Arias syndrome, is an autosomal dominantform; one parent will have elevated bilirubin. Kernicterus has been reported http://www.5mcc.com/Assets/SUMMARY/TP0237.html

Crigler-Najjar syndrome DESCRIPTION: Two distinct, inherited, syndromes marked by deficiencies of hepatic glucuronyl transferase. Both produce non-hemolytic unconjugated hyperbilirubinemia with jaundice. Type I is an autosomal recessive form. Kernicterus may develop at any age (homozygotes develop severe hyperbilirubinemia in the first few days of life). Survival into childhood is possible with intensive therapy. Patients have no response to phenobarbital. Type II, Arias syndrome, is an autosomal dominant form; one parent will have elevated bilirubin. Kernicterus has been reported in infants, but hyperbilirubinemia may be consistent with physiologic jaundice. Patients are responsive to phenobarbital. CAUSES: no uridine diphosphoglucuronic transferase Synonyms: congenital hyperbilirubinemia glucuronyl transferase deficiency type I familial unconjugated hyperbilirubinemia ICD-9-CM: 277.4 disorders of bilirubin excretion Author(s): Mark R. Dambro, MD

24. Crigler-Najjar Syndrome Information Diseases Database criglernajjar syndrome,UDP glucuronyl transferase deficiency, Disease DatabaseInformation. http://www.diseasesdatabase.com/ddb3176.htm

Diseases Database Index Sponsors Contact ... Previous Page Crigler-Najjar syndrome information Search 2 synonyms or equivalents were found. Crigler-Najjar syndrome aka/or UDP glucuronyl transferase deficiency may cause or feature (Follow link for list.) may be a risk factor for (Follow link for list.) may be allelic with (Follow link for list.) belong(s) to the category of (Follow link for list.) Crigler-Najjar syndrome: Definition(s) via UMLS Code translations and terms via UMLS Crigler-Najjar syndrome: specific web sites Send Crigler-Najjar syndrome to medical search engines (JavaScript enabled browsers only.) If your browser has no JavaScript you can still use these: Search using Internet medical databases Search using Internet search engines (non-specialist) We subscribe to the HONcode principles of the Health On the Net Foundation Valid XHTML 1.0 Served 2005-09-09 01:56:02 View metadata Last major update 2005-09-03. The medical information here is presented for education, background reading and general interest. The Diseases Database is not a diagnostic or clinical decision-making tool. Please consult your own licensed physician regarding diagnosis and treatment of any medical condition!

25. Crigler-Najjar3bis Clinical description of the criglernajjar syndrome from the Online Mendelian Laboratories specialized in the diagnosis of Crigler -Najjar syndrome http://it.geocities.com/criglernajjar/trebis.htm

CRIGLER-NAJJAR SYNDROME Please refer to the following link: Crigler-Najjar Web pages Other useful links: OMIN Clinical description of the Crigler-Najjar syndrome from the Online Mendelian Inheritance in Man database. NORD Clinical description of the Crigler-Najjar syndrome from the National Organization for Rare Disorders, Inc. database. Dr. Namita Roy-Chowdhury Home page of Dr. N. Roy-Chowdhury at the Albert Einstein College of Medicine of Yeshiva University, Bronx, NY. The Human Gene Mutation Database Mutations in Crigler-Najjar syndrome patients. B-UDPGT Human UDP-glucuronosyltransferase from the OMIN database. Online Medical Dictionary Medical dictionary from the CancerWeb. Yahoo.health Clinical description of the Crigler-Najjar syndrome from the database of Adam.com. Yahoo.health. Glossary of Genetic Terms Glossary of genetic term from the NIH site. Cholestyramine Information on cholestyramine frome On Health web site. Download Acrobat file data sheet of Questran. More Liver Links Foundations: American Liver Foundation Children's Liver Disease Foundation Children's Liver Alliance Inc. (Australia) Center for Liver Disease and Transplantation ... Italian Crigler-Najjar Association CI AMI (Onlus) Crigler-Najjar Italia Associazione Malati Iperbilirubinemici 40033 Casalecchio di Reno (BO) Italy Telephone and Facsimile ++39 - 051- 575835 Personal Web Pages: Gabriella's Crigler-Najjar Page Personal page of a Crigler-Najjar patient.

26. Crigler-Najjar3news News about criglernajjar syndrome from the following sources Crigler-NajjarSyndrome type I is a rare congenital disease with high mortality and http://it.geocities.com/criglernajjar/trenews.htm

CRIGLER NAJJAR SYNDROME News: News about Crigler-Najjar syndrome from the following sources: Science by Gary Taubes, December 2002, Vol. 298, 2116-2120. The strange case of Chimeraplasty. Indegene discussion board: Prolonged Neonatal Unconjugated Hyperbilirubinemia. Mosaic: by Leslie Virostek, November 2001. Dr. Holmes Morton: An innovative pediatrician in an insular community Reuters Health: by Rossella Lorenzi, October 19, 2001. Crigler-Najjar treated with anesthetic-free hepatocyte infusion. E-medicine Journal: by Alessio Pigazzi, July 18, 2001. Vol. 2, number 7. Crigler-Najjar Syndrome. E-medicine Journal: by Shirley Donelson, May 3, 2001. Vol. 2, number 5. Bilirubin, impaired conjugation - Saudi Medical Journal: Management of Crigler-Najjar Syndrome type I. by Al-Shurafa H.A. et al . Department of Hepatobiliary Surgery and Transplantation, University Hospital Hamburg-Eppendorf, Martini Street 52, 20246 Hamburg, Germany. Tel. +49 (40) 42803 6135/6136. Fax. +49 (40) 42803 3431. Email: al-shurafa@uke.uni-hamburg.de From Saudi. Med. J. (2001). 22(6):486-489.

27. CRIGLER-NAJJAR SYNDROME Dr. Koop crigler-najjar syndromecrigler-najjar syndrome, Jul 29, 2005 Genetic counseling is recommended forprospective parents with a family history of crigler-najjar syndrome. http://www.medhelp.org/glossary/new/GLS_1354.HTM

Welcome to .... Advertisement CRIGLER-NAJJAR SYNDROME - A rare genetic defect ( autosomal recessive ) where there is the inability to form bilirubin glucuronide due to the absence of the enzyme bilirubin-glucuronoside glucuronosyltransferase; finding include jaundice , and irreversible brain damage in the severe form. Med Help International Search Ask the Doctor Forums Patient Network The medical glossary has been made possible by a generous donation from: (revised: 1996) Advertisement

28. AllRefer Health - Crigler-Najjar Syndrome (Arias Syndrome (Type II Crigler-Najja criglernajjar syndrome (Arias Syndrome (Type II Crigler-Najjar), GlucuronylTransferase Deficiency (Type I Crigler-Najjar)) information center covers http://health.allrefer.com/health/crigler-najjar-syndrome.html

AllRefer Channels :: Yellow Pages Reference Health Home ... Contact Us Quick Jump ADD/ADHD Allergies Alzheimer's Disease Arthritis Asthma Back Pain Breast Cancer Cancer Colon Cancer Depression Diabetes Gallbladder Disease Heart Attack Hepatitis High Cholesterol HIV/AIDS Hypertension Lung Cancer Menopause Migraines/Headaches Osteoporosis Pneumonia Prostate Cancer SARS Stroke Urinary Tract Infection 1600+ More Conditions Alternative Medicine Health News Symptoms Guide Special Topics ... Medical Encyclopedia Web health.allrefer.com You are here : AllRefer.com Health Crigler-Najjar Syndrome Alternate Names : Arias Syndrome (Type II Crigler-Najjar), Glucuronyl Transferase Deficiency (Type I Crigler-Najjar) 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. Liver Anatomy Jump to a section Definition Prevention Treatment Expectations or Prognosis ... Calling Your Health Care Provider Topics that might be of interest to you Rh Incompatibility Bilirubin ELISA Liver Biopsy Other Topics Confusion Enzyme Glucuronyl Transferase Jaundice - Yellow Skin From Our Sponsors: A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's

29. AllRefer Health - Crigler-Najjar Syndrome Diagnosis & Tests (Arias Syndrome (Typ criglernajjar syndrome (Arias Syndrome (Type II Crigler-Najjar), GlucuronylTransferase Deficiency (Type I Crigler-Najjar)) information center covers http://health.allrefer.com/health/crigler-najjar-syndrome-diagnosis-tests.html

AllRefer Channels :: Yellow Pages Reference Health Home ... Contact Us Quick Jump ADD/ADHD Allergies Alzheimer's Disease Arthritis Asthma Back Pain Breast Cancer Cancer Colon Cancer Depression Diabetes Gallbladder Disease Heart Attack Hepatitis High Cholesterol HIV/AIDS Hypertension Lung Cancer Menopause Migraines/Headaches Osteoporosis Pneumonia Prostate Cancer SARS Stroke Urinary Tract Infection 1600+ More Conditions Alternative Medicine Health News Symptoms Guide Special Topics ... Medical Encyclopedia Web health.allrefer.com You are here : AllRefer.com Health Crigler-Najjar Syndrome Crigler-Najjar Syndrome Definition Prevention Treatment Expectations or Prognosis ... Go To Main Page Alternate Names : Arias Syndrome (Type II Crigler-Najjar), Glucuronyl Transferase Deficiency (Type I Crigler-Najjar) Tests used to evaluate the liver function include: Unconjugated bilirubin in blood (would be highly elevated) Total bilirubin level (would be high) Conjugated bilirubin (would be low to absent) Liver biopsy enzyme assay for low-absent Glucuronyl transferase activity A family history of Crigler-Najjar syndrome Previous Top Next Jump to another section Definition Prevention Treatment Prognosis ... Calling Your Health Care Provider Topics that might be of interest to you Rh Incompatibility Bilirubin ELISA Liver Biopsy Other Topics Confusion Enzyme Glucuronyl Transferase Jaundice - Yellow Skin 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.

30. Crigler-najjar Syndrome Type II Gupta Ravi, Parashar Yogesh - Indian J Pediatr Peerreviewed open access biomedical speciality periodical from India coveringvarious aspects of child health. http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2004;volume=71

31. Home Support site for parents and patients with criglernajjar syndrome Type 1 and Type 2. For physicians and researchers learn about essential treatment to avoid brain damage due to excessive bilirubin concentrations. http://criglernajjar.com

Our Site Last Updated June 29, 2003 Crigler-Najjar Syndrome is caused by a liver enzyme deficiency Crigler-Najjar Syndrome (CNS) is an inherited metabolic disorder caused by a liver enzyme deficiency which prevents the usual metabolic breakdown of bilirubin; a normal by-product in our body’s disposal of worn-out red blood cells. Children with CNS are unable to eliminate bilirubin from their bodies and therefore must undergo daily 12-hour exposure to special blue lights, just to survive. Without daily treatments, a child would suffer brain damage, muscle and nerve damage and death due to bilirubin toxicity. A Rare Orphan Disease Crigler-Najjar is a very rare disease. There are less than 50 known cases in the USA, and less than 200 worldwide. It is named after Dr. Crigler and Dr. Najjar who discovered the disease. Dr. John F. Crigler is a Special Medical Two-year-old Melanie has to sleep under intense blue lights to help combat the disease Crigler-Najjar Syndrome Advisor to the CNA. The Crigler-Najjar Association Providing Support and Helping Others Our group represents approximately 30 families in the U.S.A. who have family members affected by Crigler-Najjar syndrome. Started in 2002 with a handful of Crigler-Najjar families, our group has grown and expanded to represent all families who are interested in providing support, information and helping others who are less fortunate that may not have the ability to pay for expensive phototherapy lights and medications required just to live.

32. Crigler-najjar Syndrome Type II,Gupta Ravi, Parashar Yogesh: Indian Journal Of P Indian Journal of Pediatrics is an open access http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2004;volume=71

33. Crigler-Najjar Syndrome criglernajjar syndrome is caused by a disorder in the metabolism of bilirubin,the chemical that causes jaundice. http://digestive-disorders.health-cares.net/crigler-najjar-syndrome.php

34. UpToDate Crigler-Najjar Syndrome CLASSIFICATION — criglernajjar syndrome, also referred to as congenital TYPE I DISEASE — crigler-najjar syndrome type I was described by Crigler and http://patients.uptodate.com/topic.asp?file=hep_dis/12983

35. Crigler-Najjar Syndrome Treatment Options At Mayo Clinic (type I CriglerNajjar); Arias syndrome (type II Crigler-Najjar). Crigler-Najjarsyndrome (CNS) is a rare genetic condition in which the liver cannot http://www.mayoclinic.org/genetic-liver-diseases/crigler-najjar.html

Home About Mayo Clinic Jobs Contact Us Mayo Clinic Locations: Arizona Florida Minnesota Mayo Clinic ... Medical Services Genetic Liver Diseases Genetic Liver Diseases Overview Treatment Options Treatment in Jacksonville Treatment in Rochester ... Medical Services Treatment of Crigler-Najjar Syndrome Synonyms: Glucuronyl transferase deficiency (type I Crigler-Najjar); Arias syndrome (type II Crigler-Najjar) Crigler-Najjar syndrome (CNS) is a rare genetic condition in which the liver cannot process bilirubin (a pigment produced when the liver processes waste products). Diagnosis This extremely rare syndrome is usually diagnosed by jaundice testing shortly after birth. Factors which lead to a suspicion of the disease are a family history of Crigler-Najjar syndrome and jaundice (yellow skin or eyes) that persists beyond two weeks without an obvious cause. The condition is marked by confusion and changes in thinking, resulting from the effects of excess bilirubin in the brain. Besides bilirubin tests, other diagnostics may include enzyme assays (another blood test) or liver biopsies. Treatment Children with CNS are unable to eliminate bilirubin from their bodies. Blood transfusions are used to eliminate excess bilirubin. Long-term treatment for this condition is phototherapy in which patients lay under lights for as many as 12 hours per day to break down the bilirubin. Without daily treatments, CNS patients may suffer brain damage, muscle and nerve damage or death due to bilirubin toxicity. Oral calcium phosphate may be used in conjunction with phototherapy. In school-age children, phototherapy may increase to approximately 15 hours a day, which interferes considerably with daily life. In these circumstances, liver transplantation either whole liver replacement or partial liver transplantation is the treatment of choice. A variant of the condition (type 2) is less serious and responds to drugs such as phenobarbitone.

36. Crigler-Najjar Syndrome - Patient UK criglernajjar syndrome - Patient UK. A directory of UK health, disease, illnessand related medical websites that provide patient information. http://www.patient.co.uk/showdoc/40001363/

PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people. Crigler-Najjar Syndrome Two syndromes of severe unconjugated hyperbilirubinaemia: Type 1 - only 100 cases reported, autosomal recessive inheritance with neonatal jaundice leading to kernicterus or brain damage and death within 12 months (bilirubin 350-950(mol/l) Type 2 Defect in the gene encoding for UDP-glucuronyl transferase responsible for conjugating bilirubin. Epidemiology Incidence Rare Risk Factors Family history Presentation Symptoms Jaundice Differential Diagnosis Other causes of jaundice Investigations Serum bilirubin Management Non-Drug Type 1 whole-body blue light phototherapy or plasmapheresis until orthotopic liver transplantation Type 2 - No treatment necessary, avoid drugs that displace unconjugated bilirubin from protein binding e.g. sulphonamides, salicylates, penicillin. References Used Thompson RPH in Oxford Textbook of Medicine, 4th Edition. Eds; Warrell DA et al. OUP 2003.

37. NEJM -- Treatment Of The Crigler-Najjar Syndrome Type I With Hepatocyte Transpla Original Article from The New England Journal of Medicine Treatment of thecriglernajjar syndrome Type I with Hepatocyte Transplantation. http://content.nejm.org/cgi/content/short/338/20/1422

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Please sign in for full text and personal services Previous Volume 338:1422-1427 May 14, 1998 Number 20 Next Ira J. Fox, M.D., Jayanta Roy Chowdhury, M.D., Stuart S. Kaufman, M.D., Timothy C. Goertzen, M.D., Namita Roy Chowdhury, Ph.D., Phyllis I. Warkentin, M.D., Kenneth Dorko, B.S., Bernhard V. Sauter, M.D., and Stephen C. Strom, Ph.D. Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings. Full Text PDF Editorial by Lake, J. R. ... PubMed Citation disorder characterized by severe unconjugated hyperbilirubinemia beginning at birth. The syndrome results from an absence of hepatic uridine diphosphoglucuronate (UDP) glucuronosyltransferase activity, which is essential for the conjugation and excretion of bilirubin. Because of the accumulation of unconjugated bilirubin in plasma, patients are at risk for kernicterus. Although phototherapy successfully reduces serum bilirubin levels, patients are again at risk for kernicterus around the time of puberty, when phototherapy

38. NEJM -- Sign In Original Article from The New England Journal of Medicine Treatment of thecriglernajjar syndrome Type I with Hepatocyte Transplantation. http://content.nejm.org/cgi/content/full/338/20/1422

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Access to this article requires sign-in. Subscribers have access to all content. Other registered users have access to research articles six months old and older. If you're a registered user or an activated subscriber SIGN IN User Name Password Forgot your Password? Click here and we'll e-mail it to you. If you do not use cookies, sign in here. Remember my User Name and Password. Log in via Athens. PURCHASE THIS ARTICLE Purchase a single article and get immediate online access for just $10. If you're a subscriber but have not yet activated your full online access If you'd like to purchase a subscription to NEJM If you're not a subscriber and want FREE limited access... ACTIVATE YOUR SUBSCRIPTION Subscribers to NEJM are entitled to full access to all online content and features, including 20 FREE online CME exams. OR Receive full access to ALL current content and online features including Personal Archives, PDF article downloads, PDA access, E-mail alerts and 20 FREE online CME exams. OR Receive FREE online access to NEJM Original and Special Articles 6 months after publication and choose to receive the Table of Contents and notification of early release articles via e-mail.

39. Crigler Najjar Syndrome (Bilirubin Uridinediphosphate Glucuronosylyltransferase Current therapy for criglernajjar syndrome type 1 report of a world registry.crigler-najjar syndrome type II is inherited both as a dominant and as a http://www.bdid.com/crigler.htm

HOME Crigler Najjar Syndrome (Bilirubin Uridinediphosphate Glucuronosylyltransferase Deficiency) Type I Treatment of Crigler-Najjar Syndrome Crigler-Najjar syndrome : sites francophones CRIGLER crigler-najjar syndrome ... SINDROME DI CRIGLER-NAJJAR Type II See Gilbert('s) Syndrome HOME

40. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Genetic_Disorders/ Austin Health Information An overview of crigler-najjar syndrome NORDCrigler Najjar Syndrome Type I - General information and further resources. http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Genetic_Dis

@import "/styles/navbar.css"; @import "/styles/tabStyles.css"; Set home page Bookmark site Add search Latest News ... Email to friend Text Size A A A Front Page ... Genetic Disorders : Crigler-Najjar Syndrome Subtopics See Also: Health: Conditions and Diseases: Chronic Illness Health: Conditions and Diseases: Nutrition and Metabolism Disorders Search Google: Crigler-Najjar Syndrome News Books Links ... Mayo Clinic Develops New Coma Measurement System (September 8, 2005) full story Nitric Oxide Could Extend Fertility (September 8, 2005) Biochemistry , an American Chemical Society journal. full story Flipped, Expelled, Copied, And Shrunk: Researchers Document Dramatic Genome Alterations During Primate Evolution (September 6, 2005) Genome Research presents a series of studies that provide insight into the evolution and variation of primate genomes. The issue will appear online and in print on September 1, concomitant with the publication of the chimpanzee genome sequence in the journal Nature full story Discovery Will Aid Identification Of Misregulated Genes In Rett Syndrome (September 4, 2005) Molecular Cell that the "Rett Syndrome protein", MeCP2, only binds to genes with a specific sequence of nucleotide bases. This knowledge will aid in the identification of the genes that are regulated by the gene MECP2. This work was supported, in part, by the Rett Syndrome Research Foundation (RSRF).

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