Extractions: Alagille Syndrome, AHD, Arteriohepatic Dysplasia, Cholestasis with Peripheral Pulmonary Stenosis, Syndromatic Hepatic Ductular Hypoplasia,alagille syndrome, ahd, arteriohepatic dysplasia, cholestasis with peripheral pulmonary stenosis, syndromatic hepatic ductular hypoplasia,Alagille Syndrome, AHD, Arteriohepatic Dysplasia, Cholestasis with Peripheral Pulmonary Stenosis, Syndromatic Hepatic Ductular Hypoplasia Alagille Syndrome is a genetic liver disorder usually present at birth. It is characterized by insufficient passage of bile due to a lower than normal number of bile ducts inside the liver. In some cases, the child may be born with no bile ducts. Major symptoms include prolonged yellow skin discoloration (jaundice), eye and heart structure anomalies, abnormally shaped vertebrae of the spine, compression of nerve space inside the lower spine, an absence of deep tendon reflexes, mental deficiency, facial and kidney (renal) abnormalities, shortened fingers, and pancreatic insufficiency.
Arteriohepatic Dysplasia arteriohepatic dysplasia arteriohepatic dysplasia is a genetic disorder that mimics other forms of prolonged liver disease in infants and young children. http://ww3.komotv.com/global/story.asp?s=1230821&ClientType=Printable
Entrez PubMed arteriohepatic dysplasia a benign syndrome of intrahepatic cholestasis with multiple organ involvement. Riely CA, Cotlier E, Jensen http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstra
Entrez PubMed Ocular findings in arteriohepatic dysplasia (Alagille s syndrome). Persons with arteriohepatic dysplasia typically present with prolonged neonatal jaundice. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstra
Alagille Syndrome arteriohepatic dysplasia OR ALAGILLE SYNDROME. Alagille Medicine. Alagille sSyndrome / arteriohepatic dysplasia Right elbow - Xanthomata. http://www.health-nexus.com/alagille_syndrome.htm
Extractions: Health-Nexus.Net Health-Nexus.Org The #1 Health information site 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 Alagille Syndrome WebMD/Lycos - Article - Alagille Syndrome With Lycos, WebMD offers a comprehensive online health resource. Whether you want to get information about a medical condition, learn more about leading a healthier lifestyle, or talk to others...
Alagille Syndrome New Register, Dr Ira Shah MD, DCH(Gold Medalist), FCPS, DNB. Key words arteriohepatic dysplasia , chronic cholestasis, pulmonary artery hypoplasia http://www.pediatriconcall.com/fordoctor/casereports/alagille_syndrome.asp
Extractions: M.D, DCH(Gold Medalist), FCPS, DNB Key words:- Arteriohepatic dysplasia , chronic cholestasis, pulmonary artery hypoplasia Introduction: Case :- 18 month old male child BONCM to normal parents presented with jaundice since 2 months of age, clay colored stools with high colored urine, and pruritis since l year. Patient was noticed to have cyanosis, which increased, on crying. He had dysmorphic features in form of deep-seated eyes, with prominent forehead and small pointed chin. Patient had hepatomegaly with 5cm palpable liver. He also had an ejection systolic murmur with soft and single second heart sound suggestive of tetralogy of fallot physiology. Patient was clinically diagnosed as a case of Arteriohepatic dysplasia or Alagille syndrome . 2 D echo showed subvalvalar pulmonary stenosis with left pulmonary artery not seen. USG Abdomen was suggestive of gall bladder sludge with hepatomegaly. His liver enzymes were elevated. There was hyperbilirubinemia with increased direct compound (2.4mg% D= 1.2 mg%). Serum cholesterol (316 mg%) and Triglyceride (352 mg%) were markedly elevated. Liver scan (HIDA) showed decreased excretion. Patient was advised cardiac catheterisation to assess the anatomical malformation and evaluate further treatment. Liver biopsy was not undertaken in this patient. He was treated with fat soluble vitamins, cholestyramine, ureodeoxycholic acid. His pruritis markedly subsided with above management. He was started on T. Propranalol for his cyanotic heart disease and discharged. He was advised surgery for heart disease and option for liver transplantation was explained.
Alagille Syndrome / The Family Village See also arteriohepatic dysplasia, WatsonAlagille Syndrome, Syndromic Hepatic Ductular Hypoplasia, Syndromic Intrahepatic Biliary Hypoplasia, Cholestasis http://www.familyvillage.wisc.edu/lib_alag.htm
Extractions: See also: Arteriohepatic Dysplasia, Watson-Alagille Syndrome, Syndromic Hepatic Ductular Hypoplasia, Syndromic Intrahepatic Biliary Hypoplasia, Cholestasis with Peripheral Pulmonary Stenosis, Syndromic Bile Duct Paucity, Intrahepatic Biliary Artresia or Dysgenesis. ASA's mission is to provide information about Alagille syndrome, networking services, and a forum for exchange of experiences for families with the syndrome. They also disseminate information to all those interested in this rare liver disorder, and will refer families to others in the same geographical area. The Alliance publishes a quarterly newsletter, LiverLinks , and has a brochure and a fact sheet available that explain the syndrome and treatment that is available. ASA offers a new parent packet that includes, the newsletter, fact sheet, brochure, and an information form for referrals. ASA collects information on physicians and research being done and makes this information available to its members on a limited basis. They are in the process of developing a bibliography of articles and they have a scientific advisory board.
Arteriohepatic Dysplasia (Alagille Syhdrome,AGS) The summary for this Chinese (Traditional) page contains characters that cannot be correctly displayed in this language/character set. http://www3.vghtc.gov.tw:8082/ped/department/ghc/disease/2002-11-28/Arteriohepat
Alagille's Syndrome J. Reichen. First described by Alagille in 1969, reported in the English literature in 1975 (1). Synonyms arteriohepatic dysplasia, syndromatic ductopenia. http://www.ikp.unibe.ch/lab2/Alagille.htm
Extractions: Autosomal dominant disorder owing to mutations in the JAG1 gene on chromosome 20p12 (2;3). JAG1 codes for a NOTCH receptor ligand important in cell-cell interactions and in development. Different mutations have been described 70 % of which are sporadic (4). During embroygenesis JAG1 is expressed mainly in the cardiovascular system; in the liver it is associated with blood vessels. It is also expressed in other structures of mesenchymal origin suggesting that the variety of associated conditions are not chance associations (5). Clinical presentation, associated features: The classical syndrome consists of jaundice in early infancy, characteristic facies, pulmonary stenosis, butterfly vertebrae, growth and mental retardation and hypogonadism (1). In Emerick's series, 40 % had renal disease and stroke occurred in 14 % (6). The growth retardation is due to resistance to GH (7). Severe pruritus occurs in 45 % but can abate with age (6). Besides posterior embryotoxon a variety of other ocular anomalities have been described (8). Table 1 Frequency of main signs of Alagille's syndrome in the two largest series Alagille (9) (n= 80) Emerick (6) (n=92) Chronic cholestasis Characteristic facies Systolic murmur Butterfly vertebrae Posterior embryotoxon Other associated conditions include congenital mechanical obstruction of the small intestine (10), cystic renal disease (11), tubulo-interstitial nephropathy (12), exocrine pancreatic insufficiency (13), pancreas atrophy with diabetes mellitus (14), coproporphyrin abnormalities with photosensitivity (15).
Alagille's Syndrome Dept. of Dermatology University of Iowa College of Medicine. Alagille s Syndrome / arteriohepatic dysplasia - Right elbow - Xanthomata. http://tray.dermatology.uiowa.edu/Alagil01.htm
Alagille's Syndrome - 4 Medicine. Alagille s Syndrome / arteriohepatic dysplasia Back (Date 10/1980) - Xanthomata Compare with Alagile s Syndrome - 5. http://tray.dermatology.uiowa.edu/Alagil04.htm
Pharmacy Drug Index It is an anticholesterol drug used to reduce abnormally high blood levels of cholesterol and to treat arteriohepatic dysplasia. http://www.mythos.com/pharmacy/Index_DrugName.aspx?L=Q&P=1
Alagille Syndrome arteriohepatic dysplasia (ALAGILLE SYNDROME) EXTREME VARIABILITY AMONG AFFECTED FAMILY MEMBERS. SA Shulman et al.; Am J Med Genet (Oct 1984; 19(2)). Pp. http://www.bchealthguide.org/kbase/nord/nord473.htm
Extractions: 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. Information on the following diseases can be found in the Related Disorders section of this report: Alagille Syndrome is a genetic liver disorder usually present at birth. It is characterized by insufficient passage of bile due to a lower than normal number of bile ducts inside the liver. In some cases, the child may be born with no bile ducts. Major symptoms include prolonged yellow skin discoloration (jaundice), eye and heart structure anomalies, abnormally shaped vertebrae of the spine, compression of nerve space inside the lower spine, an absence of deep tendon reflexes, mental deficiency, facial and kidney (renal) abnormalities, shortened fingers, and pancreatic insufficiency.
PSU Vol 12, 1999 Two types of PILBD are recognized 1) syndromic (arteriohepatic dysplasia or Alagille s syndrome) with characteristic extrahepatic abnormalities (fascial http://home.coqui.net/titolugo/PSU12.htm
Harriet Lane Links 6. Alagille s Syndrome/arteriohepatic dysplasia Dermatologic Finding 5 http//tray.dermatology.uiowa.edu/Alagil05.htm 0314-2000. http://derm.med.jhmi.edu/poi/search.cfm?MolecularBiology=-1&SearchTitle=Molecula
Harriet Lane Links 5. Alagille s Syndrome/arteriohepatic dysplasia Dermatologic Finding 5 http//tray.dermatology.uiowa.edu/Alagil05.htm 0314-2000. http://derm.med.jhmi.edu/poi/search.cfm?PlasticSurgery=-1&SearchTitle=Plastic Su
Dorlands Medical Dictionary tachycardia arising in the right ventricle. arteriohepatic dysplasia, Alagille syndrome. bronchopulmonary dysplasia, a chronic lung http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS
