1. MedlinePlus Medical Encyclopedia: Crigler-Najjar Syndrome criglernajjar syndrome. Prevention Return to top. Genetic counseling is recommended for prospective parents with a family history of crigler-najjar syndrome. http://www.nlm.nih.gov/medlineplus/ency/article/001127.htm

@import url(http://www.nlm.nih.gov/medlineplus/images/advanced.css); Skip navigation Medical Encyclopedia Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Crigler-Najjar syndrome Contents of this page: Illustrations Alternative names Definition Causes, incidence, and risk factors ... Prevention 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 normal enzyme activity of a normal adult.

2. EMedicine - Crigler-Najjar Syndrome : Article By Alessio Pigazzi, MD, PhD criglernajjar syndrome - crigler-najjar syndrome (CNS) is a rare disorder of bilirubin metabolism and has 2 distinct forms type 1 and type 2. Type 1 CNS is associated with neonatal jaundice and http://www.emedicine.com/med/topic476.htm

(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Medicine, Ob/Gyn, Psychiatry, and Surgery Gastroenterology 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 , Staff Physician, Department of General Surgery, New York Presbyterian Hospital 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

3. CRIGLER-NAJJAR SYNDROME Welcome to . Advertisement. criglernajjar syndrome - crigler-najjar syndrome - A rare genetic defect (autosomal recessive) where there is the inability to form bilirubin 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

4. EMedicine - Crigler-Najjar Syndrome : Article Excerpt By: Alessio Pigazzi, MD, P eMedicine World Medical Library. (advertisement). Excerpt from criglernajjar syndrome. Please click here to view the full topic text crigler-najjar syndrome. http://www.emedicine.com/med/byname/crigler-najjar-syndrome.htm

(advertisement) Excerpt from Crigler-Najjar Syndrome Synonyms, Key Words, and Related Terms: 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 Please click here to view the full topic text: Crigler-Najjar Syndrome Background: Crigler-Najjar syndrome (CNS) is a rare disorder of bilirubin metabolism and has 2 distinct forms: type 1 and type 2. Type 1 CNS is associated with neonatal jaundice and neurologic manifestations, whereas type 2 CNS (also called Arias syndrome) manifests as a lower serum bilirubin level. Affected individuals may survive to adulthood without any neurological impairment. The differential diagnosis of hyperbilirubinemia can be divided into 3 broad groups: (1) disorders of excessive bilirubin production (eg, hemolysis, ineffective erythropoiesis), (2) impaired hepatic handling of bilirubin (eg, hepatitis, cirrhosis, inherited syndromes), and (3) defective bile outflow (eg, intrahepatic or extrahepatic biliary obstruction). A markedly elevated unconjugated (indirect) hyperbilirubinemia is observed in inherited disorders such as Gilbert syndrome and CNS. Among the inherited unconjugated hyperbilirubinemias, Gilbert syndrome is believed to affect approximately 3-7% of the adult population. CNS is a much rarer disorder, with only a few hundred cases described in the literature.

5. ► Crigler-Najjar Syndrome A medical encycopedia article on the topic crigler-najjar syndrome crigler-najjar syndrome is an inherited disorder in which bilirubin (a substance made by the liver) cannot be crigler-najjar syndrome is caused by an abnormal gene which fails to http://www.umm.edu/ency/article/001127.htm

Disease Nutrition Surgery Symptoms Injury ... Z Maryland Medical Center Programs UM Hospital for Children Home Medical Reference Encyclopedia (English) Toggle English Spanish 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

6. Crigler Najjar Syndrome Type I criglernajjar syndrome type I is a very rare inherited metabolic disorder characterized by the life, most infants with crigler-najjar syndrome type I develop persistent yellowing http://www.bchealthguide.org/kbase/nord/nord1084.htm

document.write(''); var hwPrint=1; var hwDocHWID="nord1084"; var hwDocTitle="Crigler Najjar Syndrome Type I"; var hwRank="1"; var hwSectionHWID="nord1084"; var hwSectionTitle=""; var hwSource="cn6.0"; var hwProdCfgSerNo="wsh_html_031_s"; var hwDocType="NORD"; National Organization for Rare Disorders, Inc. Crigler Najjar Syndrome Type I Important It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Bilirubin Glucuronosyltransferase Deficiency Type I Uridine Diphosphate Glucuronosyltransferase, Severe Def. Type I Congenital Familial Nonhemolytic Jaundice Type I Disorder Subdivisions None Related Disorders List Information on the following diseases can be found in the Related Disorders section of this report: Crigler-Najjar Syndrome Type II Gilbert Syndrome Rotor Syndrome Rh Disease General Discussion Crigler-Najjar syndrome type I is a very rare inherited metabolic disorder characterized by the complete absence of the enzyme uridine diphosphate glucuronosyltransferase (UDPGT), which is normally found in the liver. This enzyme is required for the conversion (conjugation) and subsequent excretion of bilirubin from the body. When UDP-glucuronosyltransferase activity is absent, these metabolic processes are hampered and abnormally high levels of bilirubin accumulate in the blood (hyperbilirubinemia). Within the first few days of life, most infants with Crigler-Najjar syndrome type I develop persistent yellowing of the skin, mucous membranes, and whites of the eyes (jaundice). In addition, some affected individuals may develop kernicterus, a potentially life-threatening neurological condition in which toxic levels of bilirubin accumulate in the brain, causing damage to the central nervous system. Early signs of kernicterus may include lack of energy (lethargy), vomiting, fever, and/or unsatisfactory feedings. More serious signs of kernicterus may develop, including abnormal muscle rigidity, resulting in muscle spasms (dystonia) and involuntary movements of the limbs and/or entire body (athetosis). Crigler-Najjar syndrome type I is inherited as an autosomal recessive genetic trait.

7. Crigler-Najjar Syndrome criglernajjar syndrome. DESCRIPTION Two distinct, inherited, syndromes marked by deficiencies of hepatic Type II, Arias syndrome, is an autosomal dominant form; one parent will 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

8. Austin Health Information An overview of criglernajjar syndrome including symptoms, treatment and prevention. http://www.austin360.com/shared/health/adam/ency/article/001127.html

9. Medical Encyclopedia: Crigler-Najjar Syndrome (Print Version) Medical Encyclopedia criglernajjar syndrome. Genetic counseling is recommended for prospective parents with a family history of crigler-najjar syndrome. http://www.nlm.nih.gov/medlineplus/print/ency/article/001127.htm

To print this page, use the print option from your browser. To close this window, click on the "x" in the upper right hand corner of the window. Medical Encyclopedia: Crigler-Najjar syndrome URL of this page: http://www.nlm.nih.gov/medlineplus/ency/article/001127.htm Alternative names Glucuronyl transferase deficiency (type I Crigler-Najjar); Arias syndrome (type II 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. 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 kernicterus, which is bilirubin toxicity of the brain and which can be fatal.

10. Crigler-Najjar Syndrome Information Diseases Database criglernajjar syndrome UDP glucuronyl transferase deficiency, Disease Database Information Sponsors Contact Previous Page. crigler-najjar syndrome Information. crigler-najjar syndrome http://www.diseasesdatabase.com/sieve/item1.asp?glngUserChoice=3176

11. Crigler-Najjar Syndrome - Information / Diagnosis / Treatment / Prevention home genetic disorders criglernajjar syndrome crigler-najjar syndrome. NORD Crigler Najjar Syndrome Type I General information and further resources. http://www.healthcyclopedia.com/genetic-disorders/crigler-najjar-syndrome.html

Home Health cyclopedia All Topics by Category The Good Health Search Engine Health Conditions A-Z Gurus ... genetic disorders > crigler-najjar syndrome Crigler-Najjar Syndrome Information / Diagnosis / Treatment / Prevention External links (marked with an arrow ) open in a new window. This site is a web directory and does not offer medical advice. We cannot take responsibility for information found on listed sites. This Page Related Topics Medical Definition Health News Web Directory: Related Topics: Chronic Illness Nutrition and Metabolism Disorders Medical Definition: University of Newcastle-upon-Tyne Medical Dictionary: "Crigler-Najjar syndrome" Health News: Search millions of published articles for news on Crigler-Najjar Syndrome Modern Medicine Aging The Ardell Wellness Report HealthFacts Medical Post Medical Update Men's Health and the National Women's Health Report Note: Subscription required to access the full text of articles. Web Directory: Austin Health Information An overview of crigler-najjar syndrome including symptoms, treatment and prevention. Crigler-Najjar Disease Bi-lingual site with information and links on this disease.

12. Redirect http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?218800

13. ► Crigler-Najjar Syndrome criglernajjar syndrome. Prevention Genetic counseling is recommended for prospective parents with a family history of crigler-najjar syndrome. http://www.umm.edu/ency/article/001127prv.htm

Disease Nutrition Surgery Symptoms Injury ... Z Maryland Medical Center Programs UM Hospital for Children Home Medical Reference Encyclopedia (English) Toggle English Spanish Crigler-Najjar syndrome Overview Symptoms Treatment Prevention Alternative Names: Glucuronyl transferase deficiency (type I); Arias syndrome (type II Crigler-Najjar) Prevention: Genetic counseling is recommended for prospective parents with a family history of Crigler-Najjar syndrome. People who carry the gene can be recognized by blood testing. 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. Home Health Info Our Physicians Our Services ... Contact Us UNIVERSITY OF MARYLAND MEDICINE © 2001 UNIVERSITY OF MARYLAND MEDICAL SYSTEM This site developed and maintained by Public Affairs. Privacy Statement Diversity Statement The information on this web site is provided as a public service by the university of Maryland Medical System, a regional health care delivery system that includes an academic medical center (the University of Maryland Medical Center in downtown Baltimore) as well as five community and specialty hospitals and outpatient sites throughout Baltimore and central Maryland. This information is not intended to be a substitute for individual medical advice in diagnosing or treating a health problem. Please consult with your health care provider about your health care concerns. If you would like to make an appointment with a University of Maryland physician, please call 1-800-492-5538

14. AllRefer Health - Crigler-Najjar Syndrome (Arias Syndrome (Type II Crigler-Najja criglernajjar syndrome. Review provided by VeriMed Healthcare Network. Main Page of crigler-najjar syndrome. From Our Sponsors ADAM. ADAM, Inc. http://health.allrefer.com/health/crigler-najjar-syndrome-info.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 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) 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 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.

15. AllRefer Health - Crigler-Najjar Syndrome Symptoms & Signs (Arias Syndrome (Type You are here AllRefer.com Health Diseases Conditions criglernajjar syndrome Symptoms of crigler-najjar syndrome. crigler-najjar syndrome. http://health.allrefer.com/health/crigler-najjar-syndrome-symptoms.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 You are here : AllRefer.com Health Crigler-Najjar Syndrome : Symptoms of 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) 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) 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.

16. Crigler-Najjar Syndrome criglernajjar syndrome. Crigler-Najjar Syndrome crigler-najjar syndrome. crigler-najjar syndrome - Welcome to . Advertisement http://www.health-nexus.com/crigler-najjar_syndrome.htm

Health-Nexus.Net Health-Nexus.Org The #1 Health information site Home ... Up Search Health-Nexus for: Match ALL words Match ANY word Email this page to a friend ! Post a question or comment on our Message Board Home Page Health Specialties Health News ... Alternative Health Options Substance Abuse Animal Health Search: Books Magazines Video Keywords: Find it Here 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 ...

17. Crigler-Najjar3bis Mutations in criglernajjar syndrome patients. B-UDPGT. Laboratories specialized in the diagnosis of Crigler -Najjar syndrome University of Dundee, UK. 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. Gabriella's Crigler-Najjar Page Personal page of a Crigler-Najjar patient. More Liver Links Foundations: American Liver Foundation Children's Liver Disease Foundation Center for Liver Disease and Transplantation Children's Liver Alliance Inc. (Australia)

18. Crigler-Najjar3 Several gene alterations have been discovered in criglernajjar syndrome patients, leading to reduced or absent B-UDPGT activity causing hyperbilirubinemia. http://it.geocities.com/criglernajjar/tre.htm

CRIGLER-NAJJAR SYNDROME Please refer to the following link: Crigler-Najjar Web pages Other useful links Definition Crigler-Najjar disease is an autosomal recessive inherited disease characterized by a persistent unconjugated hyperbilirubinemia. Glucuronidation by the bilirubin-uridine diphosphoglucuronate glucuronosyltransferase (B-UDPGT) is required for the secretion of bilirubin into the bile. In Crigler-Najjar patients the enzyme is either inactive (type I) or severely reduced (type II). The high plasma level of unconjugated bilirubin leads to jaundice and kernicterus (bilirubin encephalopathy). Treatment Current therapy mainly consists of phototherapy (10-12 hours per day) and the patient's survival is dependent on the indefinite continuation of this therapy. Although initially very effective, phototherapy is inconvenient and the efficacy of this treatment is reduced with ageing due to increased thickness of skin and body surface/weight ratio; thus, patients are again at risk for kernicterus around time of puberty. Liver transplantation is an efficacious therapy but the number of donor organs is limited and it requires life-long immune suppression. Genetics Several gene alterations have been discovered in Crigler-Najjar syndrome patients, leading to reduced or absent B-UDPGT activity causing hyperbilirubinemia. Full-length cDNA for human B-UDPGT has been cloned and

19. Crigler-Najjar Syndrome (www.whonamedit.com) criglernajjar syndrome Also Hyperbilirubinaemia in newborn in crigler-najjar syndrome is called Najjar-Crigler icterus. Bibliography http://www.whonamedit.com/synd.cfm/86.html

Home List categories Eponyms A-Z Biographies by country ... Contact 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. 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.

20. Crigler-Najjar Syndrome Information Diseases Database criglernajjar syndrome aka/or UDP glucuronyl transferase deficiency Medical information linkscrigler-najjar syndrome specific sites. 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 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 i-medicine.info - the evidence based medicine, informatics and audit portal Valid XHTML 1.0 Served 2004-06-02 11:13:47 Metadata Updated 2004-05-22

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