21. 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

22. Arteriohepatic Dysplasia - Talk Medical Humanfriendly medical definition of arteriohepatic dysplasia. http://www.talkmedical.com/medical-dictionary/1220/Arteriohepatic-Dysplasia

Home Medications Medical Dictionary Talk Medical ... Medical Dictionary Newsletter Subscribe to the free monthly health digest. Relevant health articles just for you. Tell a friend Arteriohepatic Dysplasia Arteriohepatic dysplasia: Print this page About Talk Medical Help Contact Us ... Terms and Conditions

23. Arteriohepatic Dysplasia (Alagille's Syndrome): A Common Cause Of Conjugated Hyp The etiology of arteriohepatic dysplasia is unclear. The main pathogenic mechanismsare discussed. It is felt that the syndromatic duct paucity represents http://www.annclinlabsci.org/cgi/content/abstract/14/6/480

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: 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 Articles Arteriohepatic dysplasia (Alagille's syndrome): a common cause of conjugated hyperbilirubinemia E Kahn and F Daum HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS

24. 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

HOME HELP FEEDBACK SUBSCRIPTIONS ... SEARCH TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: Receive this page by email each issue: [Sign up for eTOCs] Contents: November 1984, Volume 14, Issue 6 [Index by Author] Other Issues: Articles Find articles in this issue containing these words: [Search ALL Issues] To see an article , click its [Full Text] link. To review many abstracts , check the boxes to the left of the titles you want, and click the 'Get All Checked Abstract(s)' button. To see one abstract at a time , click its [Abstract] link. Articles: MH Mark and PM Farmer Articles: The human subfornical organ: an anatomic and ultrastructural study Ann Clin Lab Sci 1984 14: 427-442. [Abstract] KK Kane Articles: Fibrinolysisa review Ann Clin Lab Sci 1984 14: 443-449. [Abstract] KJ Lauenstein, FR Davey, C Hubbell, A Burleson, RP Oates, and RL Burleson Articles: Association of renal graft survival with matching at the HLA-DR locus Ann Clin Lab Sci 1984 14: 450-457. [Abstract] MS Laks, SE Kahn, MJ Favus, and Bermes EW, Jr Articles: Case report: clinical pathological correlations in a case of primary parathyroid carcinoma Ann Clin Lab Sci 1984 14: 458-463.

25. 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

26. Ateriohepatic Dysplasia arteriohepatic dysplasia. (See ALAGILLE SYNDROME). Home HotLines Resources Printed Guide Child Care for the 90 s Links. © 1997 ABC F Press http://www.childhealthinfo.com/arteriohepatic-dysplasia.htm

ARTERIOHEPATIC DYSPLASIA (See ALAGILLE SYNDROME) [Home] [Printed Guide] [Child Care for the '90's] [Links] ... MoreInfo@ChildHealthInfo.com

27. Arteriohepatic Dysplasia: Association Of Liver Disease With Pulmonary Arterial S arteriohepatic dysplasia association of liver disease with pulmonary arterialstenosis as well as facial and skeletal abnormalities http://pediatrics.aappublications.org/cgi/content/abstract/66/6/876

QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: 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. Arteriohepatic dysplasia: association of liver disease with pulmonary arterial stenosis as well as facial and skeletal abnormalities 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

28. Abdominal Coarctation And Alagille Syndrome -- Quek Et Al. 106 (1): 9 -- Pediatr the short arm of chromosome 20 in arteriohepatic dysplasia (Alagille syndrome) . OstmanSmith I Middle aortic syndrome with arteriohepatic dysplasia. http://pediatrics.aappublications.org/cgi/content/full/106/1/e9

QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: 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 Surgery PEDIATRICS Vol. 106 No. 1 July 2000, p. e9 ELECTRONIC ARTICLE: 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. ABSTRACT Top Abstract Discussion Conclusion References Structural cardiac defects such as peripheral pulmonary stenosis are well-described in Alagille syndrome (AS), which is transmitted in an autosomal dominant inheritance. The genetic defect, with

29. Dr. Koop - Arteriohepatic Dysplasia- Health Encyclopedia And Reference arteriohepatic dysplasia Symptoms, Treatments and Medications. http://www.drkoop.com/encyclopedia/93/766.html

Home Diseases Cancer Liver Sep 08, 2005 Search: Dr.Koop MEDLINE Diseases Symptoms Procedures Natural Medicine ... Drug Library Inside DrKoop News Archive Animations Health Videos Health Tools ... Newsletters Health Encyclopedia - Diseases and Conditions A B C D ... Y Arteriohepatic Dysplasia What is the Definition of Arteriohepatic Dysplasia? Description of Arteriohepatic Dysplasia Causes of Arteriohepatic Dysplasia Symptoms of Arteriohepatic Dysplasia ... What Questions to ask Your Doctor About Arteriohepatic Dysplasia? What is the Definition of Arteriohepatic Dysplasia? 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 ^ Description of Arteriohepatic Dysplasia 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 ^ Causes of Arteriohepatic Dysplasia 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.

30. Alagille (Watson) Syndrome (Arteriohepatic Dysplasia, [Hepatofacioneuro]cardiove Translate this page Alagille (Watson) Syndrome (arteriohepatic dysplasia, HepatofacioneurocardiovertebralSyndrome, Watson Miller Syndrome) http://www.bdid.com/alagille.htm

HOME Alagille (Watson) Syndrome (Arteriohepatic Dysplasia, [Hepatofacioneuro]cardiovertebral Syndrome, Watson Miller Syndrome) Alagille syndrome Alagille Syndrome Alagille syndrome (AGS) ALAGILLE SYNDROME; AGS ... HOME

31. Birth Disorder Information Directory - A Alagille (Watson) Syndrome (arteriohepatic dysplasia, HepatofacioneurocardiovertebralSyndrome, Watson Miller Syndrome). List of Sites http://www.bdid.com/defectag.htm

HOME Ag-Am Aganglionosis Total Intestinal Aganglionosis total intestinal AGANGLIONOSIS, TOTAL INTESTINAL Aganthia with Holoprosencephaly and Situs Inversus Aganthia holoprosencephaly situs inversus AGNATHIA-HOLOPROSENCEPHALY Agammaglobulinemia List of Sites Agenesis of the Cerebellar Vermis List of Sites Agenesis of the Corpus Callosum List of Sites Related Books Aglossia Adactylia (Oromandibular Limb Hypoplasia) Aglossia adactylia AGLOSSIA-ADACTYLIA Also see Hanhart Syndrome Agnathia -Holoprosencephaly See Holoprosencephaly-Agnathia Agyria-Pachygyria Type I See Miller-Dieker (Lissencephaly) Syndrome Aicardi Syndrome List of Sites Aicardi-Goutieres Syndrome (Encephalopathy-Basal Ganglia Calcification) Aicardi-Goutieres syndrome Encephalopathy, Familial Infantile, with Calcification of Basal Ganglia and Chronic Cerebrospinal Fluid Lymphocytosis Related Books Cutis verticis gyrata thyroid aplasia mental retardation ... CUTIS VERTICIS GYRATA, THYROID APLASIA, AND MENTAL RETARDATION Aksu Stockhausen Syndrome (Branchial Arch Defects) branchial arch defects Al-Awadi Farag Teebi Syndrome (Primary Hypogonadism with Partial Alopecia) Al awadi farag teebi syndrome HYPOGONADISM, PRIMARY, AND PARTIAL ALOPECIA

32. Arteriohepatic Dysplasia - Medicine Terms Health and medicine terms and glossary detailed Information on ArteriohepaticDysplasia. http://www.encyclopedia-wiki.org/encyclopedias/medicine/Arteriohepatic-Dysplasia

Arteriohepatic Dysplasia Medicine and health Home Encyclopedias Lexika Articles ... Medizin und Gesundheit Medicine terms: Arteriohepatic Dysplasia 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. Medicine terms and glossary navigation: Next Topics: Arteriole Arteriosclerosis Arteriovenous-fistula Arteritis ... Aspartame Back to Medicine Index: Medicine terms Home Web the encyclopedia-wiki.org http://www.encyclopedia-wiki.org

33. 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

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34. Portal Toolkit Invalid Site URL Reply Alagille syndrome (arteriohepatic dysplasia). Silberbach, Michael MD;Terry, Annie MD. HTML. 1318. Carotid sinus hypersensitivity. http://ppv.ovid.com/pt/re/amhj/toc.00000406-199512000-00000.htm

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35. 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

Dept. of Dermatology - University of Iowa College of Medicine Alagille's Syndrome / Arteriohepatic Dysplasia - Elbow - Xanthomata For more information, see the American Liver Foundation and Online Mendelian Inheritance in Man (OMIM) Return to Image Index page. Return to Image Index page. Return to Dermatology's Home page. January, 1996

36. 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

Dept. of Dermatology - University of Iowa College of Medicine Alagille's Syndrome / Arteriohepatic Dysplasia - Back (Date: 6/1981) - Xanthomata Compare with Alagille's Syndrome - 4 For more information, see the American Liver Foundation and Online Mendelian Inheritance in Man (OMIM) Return to Image Index page. Return to Image Index page. Return to Dermatology's Home page. January, 1996

37. 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

38. 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

39. 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

Alagille's syndrome Prof. J. Reichen First described by Alagille in 1969, reported in the English literature in 1975 (1). Synonyms: arteriohepatic dysplasia, syndromatic ductopenia. Genetics: 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).

40. 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

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