University Of Miami School Of Medicine - Glossary - Arteriohepatic arteriohepatic dysplasia Also known as Alagille syndrome, this ia a geneticdisorder characterized by jaundice in the newborn period, liver disease with http://www.med.miami.edu/glossary/art.asp?articlekey=6754
Arteriohepatic Dysplasia - Talk Medical Humanfriendly medical definition of arteriohepatic dysplasia. http://www.talkmedical.com/medical-dictionary/1220/Arteriohepatic-Dysplasia
Extractions: This Article Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Alert me to new issues of the journal Download to citation manager PubMed Articles by Kahn, E Articles by Daum, F Annals of Clinical and Laboratory Science, Vol 14, Issue 6, 480-486 E Kahn and F Daum
ACLS -- Table Of Contents (November 1984, 14 [6]) E Kahn and F Daum Articles arteriohepatic dysplasia (Alagille s syndrome) acommon cause of conjugated hyperbilirubinemia http://www.annclinlabsci.org/content/vol14/issue6/index.shtml
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MedCirca.com | Lippincott Williams & Wilkins Index Search Results for arteriohepatic dysplasia What s an Index Search? arteriohepatic dysplasia (ALL) (3). Results 13 http://www.medcirca.com/search/indexsearch.asp?root_id=12064&index=yes
Extractions: This Article P Rs: Submit a response Alert me when this article is cited Alert me when P ... Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Levin, S. E. Articles by Schmaman, A. SE Levin, P Zarvos, S Milner and A Schmaman The clinical features and course of five children with the recently described syndrome of arteriohepatic dysplasia are presented. All had bilateral pulmonary arterial stenosis, proven at cardiac catheterization, as well as associated liver disease of varying severity. In one of the fatal cases, a hitherto undescribed anomaly was foundstenosis of the right coronary artery ostium. A viral etiology, eg, the congenital rubella
Extractions: This Article Abstract Full Text (PDF) P ... Citation Map Services E-mail this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to My File Cabinet ... Download to citation manager PubMed Articles by Quek, S. C. Articles by Quak, S. H. Related Collections Abdominal Coarctation and Alagille Syndrome Swee Chye Quek, MBBS, MMED(PAED), DCH(Lond), FACC FAMS*; Lenny Tan, MBBS, FRCR FRACR, FAMS Swee Tian Quek, MBBS, FRCR William Yip, MBBS, MD, FRCP FAMS*; Marion Aw, MBBS, MMED(PAED), MRCP and Seng Hock Quak, MBBS, MD, FRCP(Glasg), FRCPCH From the Department of Pediatrics, Division of Cardiology, National University of Singapore, and Department of Diagnostic Imaging, National University Hospital, Singapore, Singapore.
Extractions: and Conditions A B C D ... Y Arteriohepatic dysplasia is a genetic disorder that mimics other forms of prolonged liver disease in infants and young children. However, a group of unusual features in other organ systems distinguishes Arteriohepatic dysplasia from other liver and bile duct diseases in infants. It is also known as Allagile syndrome. The gene for this disorder has been discovered on chromosome 20 in band 20p12. top ^ Children with arteriohepatic dysplasia usually have a liver disease characterized by a progressive loss of the bile ducts within the liver over the first year of life and narrowing of the bile ducts outside the liver. This leads to a buildup of bile in the liver, causing damage to liver cells. Scarring may occur and lead to cirrhosis in about 30 to 50 percent of affected children. Arteriohepatic dysplasia was first described in 1969 by D. Alagille, in French medical literature. top ^ Arteriohepatic dysplasia is generally inherited from one parent and there is a 50 percent chance that each child will develop the syndrome. Each affected adult or child may have all or only a few of the features of the syndrome.
Birth Disorder Information Directory - A Alagille (Watson) Syndrome (arteriohepatic dysplasia, HepatofacioneurocardiovertebralSyndrome, Watson Miller Syndrome). List of Sites http://www.bdid.com/defectag.htm
Extractions: HOME Aganglionosis Aganthia Agammaglobulinemia Agenesis of the Cerebellar Vermis Agenesis of the Corpus Callosum Aglossia Adactylia (Oromandibular Limb Hypoplasia) Agnathia Agyria-Pachygyria Aicardi Syndrome Aicardi-Goutieres Syndrome (Encephalopathy-Basal Ganglia Calcification) Aksu Stockhausen Syndrome (Branchial Arch Defects) Al-Awadi Farag Teebi Syndrome (Primary Hypogonadism with Partial Alopecia) Al awadi farag teebi syndrome HYPOGONADISM, PRIMARY, AND PARTIAL ALOPECIA
Arteriohepatic Dysplasia - Medicine Terms Health and medicine terms and glossary detailed Information on ArteriohepaticDysplasia. http://www.encyclopedia-wiki.org/encyclopedias/medicine/Arteriohepatic-Dysplasia
Extractions: Arteriohepatic Dysplasia Medicine and health Arteriohepatic dysplasia is a genetic disorder that mimics other forms of prolonged liver disease in infants and young children. However, a group of unusual features in other organ systems distinguishes Arteriohepatic dysplasia from other liver and bile duct diseases in infants. It is also known as Allagile syndrome. The gene for this disorder has been discovered on chromosome 20 in band 20p12.
Portal Toolkit Invalid Site URL arteriohepatic dysplasia and cardiovascular malformations. AM HEART J 1994;1276959.Context Link. 2. Alagille D, Odievre M, Gautier M, Dommerguess JP. http://ppv.ovid.com/pt/re/amhj/fulltext.00000406-199512000-00044.htm
Alagille's Syndrome - 2 Alagille s Syndrome / arteriohepatic dysplasia Elbow - Xanthomata. For moreinformation, see the American Liver Foundation and Online Mendelian http://tray.dermatology.uiowa.edu/Alagil02.htm
Alagille's Syndrome - 5 Alagille s Syndrome / arteriohepatic dysplasia Back (Date 6/1981) - XanthomataCompare with Alagille s Syndrome - 4 http://tray.dermatology.uiowa.edu/Alagil05.htm
Entrez PubMed PURPOSE It has been stated that arteriohepatic dysplasia is a form of biliarypaucity with a good p http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1
Entrez PubMed The diagnosis of arteriohepatic dysplasia may be difficult, particularly in veryyoung patients with http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=3
Alagille's Syndrome Tubulointerstitial nephropathy associated with arteriohepatic dysplasia. arteriohepatic dysplasia in infancy and childhood A longitudinal study of six 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).
Dorlands Medical Dictionary arteriohepatic dysplasia, Alagille syndrome. bronchopulmonary dysplasia, a chroniclung disease of infants, possibly related to oxygen toxicity or http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS
