SoVerNet---Page Unavailable Or Access Forbidden Welcome to the criglernajjar syndrome Web Site This is a web site devoted tochildren and adults with the crigler-najjar syndrome and their families. http://www.crigler-najjar.com/
Health Ency.: Disease: Crigler-Najjar Syndrome An overview of criglernajjar syndrome including symptoms, treatment and prevention. http://www.austin360.com/shared/health/adam/ency/article/001127.html
Extractions: Important notice Ency. home Disease C Crigler-Najjar syndrome Overview Symptoms Treatment Prevention Alternative names: Glucuronyl transferase deficiency (type I); Arias syndrome (type II Crigler-Najjar) Definition: Crigler-Najjar syndrome is an inherited disorder of bilirubin metabolism in which bilirubin cannot be changed into its water-soluble form, bilirubin glucuronide. This is caused by abnormal genes which fail to produce a functional enzyme (bilirubin glucuronyltransferase) capable of converting bilirubin into a water-soluble and therefore, easily excreted form. As a result of the defect, bilirubin can build up in the body causing organs to not work properly and jaundice (a yellow discoloration of the skin and eyes because bilirubin is yellow colored). Causes and Risks Crigler-Najjar syndrome is inherited as an autosomal recessive trait. Parents who are carriers of this condition 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
Extractions: Crigler-Najjar Syndrome is a nearly always fatal genetic disorder that afflicts newborns. The disease is a deficiency in an enzyme that people need to control the production of bilirubin. When too much bilirubin accumulates, jaundice and severe brain damage occurs. Bilirubin is produced during the breakdown of hemoglobin, the blood's oxygen-carrying red pigment. Currently the disease is treated with phototherapy, to breakdown the bilirubin, followed by liver transplantation. However, liver transplantations may take place after permanent brain damage occurs. Investigators at The Rockefeller University have developed tin-mesoporphyrin, a drug that inhibits hemoglobin from breaking down. Currently, scientists are testing the drug in clinical trials. While the disease occurs worldwide, populations such as the Amish and Mennonites in which family and genetic histories can be established, afford an excellent opportunity for scientists and health care professionals to learn more about Crigler-Najjar Syndrome. Expected as attendees are a 31-year-old woman who is the oldest known living patient with Crigler-Najjar Syndrome, five Mennonite children with the disorder and their families as well as parents of patients from the United States, Canada, England, Germany and India.
Pediatric Database A look at criglernajjar syndrome, a definition, epidemiology, pathogenesis, clinical features, investigation and management. http://www.icondata.com/health/pedbase/files/CRIGLER-.HTM
Extractions: (advertisement) 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 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.
OMIM ENTRY 218800 Le mot clé CriglerNajjar, syndrome crigler-najjar syndrome est présent au sein de plusieurs arborescences du http://www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?218800
MEDLINEplus Medical Encyclopedia: Crigler-Najjar Syndrome criglernajjar syndrome. Prevention Return to top Genetic counseling is recommendedfor prospective parents with a family history of crigler-najjar syndrome. http://www.nlm.nih.gov/medlineplus/ency/article/001127.htm
Extractions: Skip navigation Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Contents of this page: Liver anatomy Alternative names Return to top Glucuronyl transferase deficiency (type I); 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. 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
404 - File Not Found A brief description of criglernajjar syndrome along with some links for further study. http://www.bio.davidson.edu/biology/Courses/Molbio/Mol Students/spring2000/rober
Extractions: The page you requested could not be found. Target URL http://www.bio.davidson.edu/courses/Molbio/Mol%20Students/spring2000/roberts/Pages/Crigler-Najjar.html Referring URL The Biology Department pages have been moved to a new server and reorganized. Every effort has been made to preserve existing links. Please email the webmaster to report broken links. In your email, please indicate which pages the link is to and from. The Biology Department web site starts here Davidson College Home Page Search Davidson Email Webmaster
MEDLINEplus Medical Encyclopedia: Topics Beginning With Aq-Az stimulation test; Arginine vasopressin see ADH; Arias syndrome (typeII CriglerNajjar) see crigler-najjar syndrome; Arisaema triphyllum http://www.nlm.nih.gov/medlineplus/ency/encyclopedia_Aq-Az.htm
Extractions: Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Aquarium granuloma see Swimming pool granuloma Arachnodactyly ARDS (acute respiratory distress syndrome) Areolar gland abscess see Subareolar abscess ARF see Acute renal failure Arginine test see Growth hormone stimulation test Arginine vasopressin see ADH Arias syndrome (type II Crigler-Najjar) see Crigler-Najjar syndrome Arisaema triphyllum see Jack-in-the-pulpit Arkless-Graham see Acrodysostosis Arm and leg movements - uncontrollable see Movement - uncontrolled or slow Armpit lump Arnold Pick's disease see Picks disease Arrhenoblastoma see Sertoli-Leydig cell tumor Arrhenoblastoma of ovary Arrhythmias Arterial blood gas see Blood gases Arterial blood gas analysis see Blood gases Arterial embolism Arterial insufficiency Arterial insufficiency of the legs see Recurrent blockage of leg arteries Arterial plethysmography see Plethysmography Arterial stick Arteriogram Arteriolar nephrosclerosis see Malignant hypertension (arteriolar nephrosclerosis) Arteriosclerosis see Atherosclerosis Arteriosclerosis obliterans see Arteriosclerosis of the extremities Arteriosclerosis of the extremities Arteriosclerotic heart disease see Coronary heart disease Arteriovenous malformation - cerebral Arteriovenous malformation - pulmonary see Pulmonary arteriovenous fistula Arthralgia see Joint pain Arthritis Arthritis - psoriatic see Psoriatic arthritis Arthritis - resources Arthrodesis see Spinal fusion Arthroscopic lateral retinacular release see
Health Ency.: Disease: Crigler-Najjar Syndrome notice. Ency. home Disease C criglernajjar syndrome. Crigler-Najjarsyndrome. Ency. home Disease C crigler-najjar syndrome. Please http://www.austin360.com/shared/health/adam/ency/article/001127sym.html
Extractions: Important notice Ency. home Disease C Crigler-Najjar syndrome Overview Symptoms Treatment Prevention Alternative names: Glucuronyl transferase deficiency (type I); Arias syndrome (type II Crigler-Najjar) Symptoms Signs and Tests Tests used to evaluate the liver function include: Ency. home Disease C Please read this Important notice Also Check Out
The Rockefeller University May 23, 1996. Treatment of criglernajjar syndrome First international conferenceon rare, fatal liver disease that affects Amish, Mennonites and others. http://www.rockefeller.edu/news/news96.html
Extractions: News Archives: Nov. 11, 1996 $2.5 Million Grant Supports Training at Biology's Interface with Chemistry and Physics The Rockefeller University has received a $2.5 million, five-year grant from the Burroughs Wellcome Fund (BWF) to establish a program designed to draw gifted young chemists, physicists and mathematicians to the frontiers of biomedical research. Oct. 31, 1996 Gene Identified for Most Common Form of Fanconi anemia The gene involved in the most common form of an inherited, often fatal disease called Fanconi anemia (FA), which causes severe bone marrow failure, birth defects and a type of leukemia, has been isolated and cloned by scientists in an international consortium of six centers, including The Rockefeller University. The work appears in the November Nature Genetics. Oct. 25, 1996 Shrimp OK for Heart Healthy Diets People no longer need avoid shrimp for its high cholesterol. Steamed shrimp, naturally low in fat, can be included in heart-healthy diets for people without lipid problems, report scientists from The Rockefeller University and the Harvard School of Public Health in the November American Journal of Clinical Nutrition Oct. 21, 1996
Crigler-Najjar Syndrome Medical Encyclopdia article about criglernajjar syndrome A resource with information on over 4000 medical topics including crigler-najjar syndrome http://www.marylandms.com/medical-terms/02959.htm
Crigler-Najjar Syndrome (www.whonamedit.com) criglernajjar syndrome Also Hyperbilirubinaemia in newborn in Crigler-Najjarsyndrome is called Najjar-Crigler icterus. Bibliography http://www.whonamedit.com/synd.cfm/86.html
Extractions: 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.
Victor Assad Najjar (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/doctor.cfm/61.html
Extractions: Victor Assad Najjar studied medicine at the American University in Beirut, graduating in 1935. Three years later he came to the USA and trained in paediatrics at the Johns Hopkins Hospital, Baltimore, where he held a faculty appointment as paediatrician from 1949 to 1957. For the next ten years he was professor and chairman of the department of microbiology, Vanderbilt University School of Medicine, Nashville, Tennessee. In 1968 he became professor of molecular biology at the Massachusetts Division of the American Cancer Society, and chief of the division of protein chemistry at Tufts University School of Medicine, Boston, Massachusetts. He was American Cancer Society Research Professor of Molecular Biology and Microbiology at Tufts University School of Medicine, 19781984.
Extractions: (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Medicine, Ob/Gyn, Psychiatry, and Surgery Gastroenterology 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
Crigler-najjar, Syndrome : Sites Et Documents Francophones Translate this page Crigler-najjar, Syndrome. Menu général CISMeF. Arborescence(s) du thesaurus MeSHcontenant le mot-clé Crigler-Najjar, syndrome crigler-najjar syndrome http://www.chu-rouen.fr/ssf/pathol/criglernajjarsyndrome.html
Extractions: 1Up Health Crigler-Najjar syndrome Alternative Medicine Clinical Trials ... Health Topics A-Z Search 1Up Health Crigler-Najjar syndrome Information Crigler-Najjar syndrome Causes, Incidence, and Risk Factors Alternative names : Arias syndrome (type II Crigler-Najjar), Glucuronyl transferase deficiency (type I) 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. 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
