61. Government And Academic Resources On Arterio-Hepatic Dysplasia Alagille s Syndrome / arteriohepatic dysplasia Right elbow - Xanthomata. Alagille syndrome (arteriohepatic dysplasia with retinal degeneration in some http://books.mongabay.com/health/conditions/Arterio-Hepatic_Dysplasia.html

Arterio-Hepatic Dysplasia Resources U.S. Government resources on Arterio-Hepatic Dysplasia CHAPTER 6 REFERENCES General Aspects 1. Alagille D, Odi¨vre M ... Adams PC: Hepatocellular carcinoma associated with arteriohepatic dysplasia. Cholestasis is a common manifestation of hepatic disease in ... The most common and best defined condition within this group is usually known Alagille Syndrome / The Family Village Harriet Lane Links: Search Results Penn State Faculty Research Expertise Database (FRED) Arteriohepatic Dysplasia, Dysplasia, Arteriohepatic. Alagille-Watson Syndrome, Radiology Cases In Pediatric Emergency Medicine Subject Index V6C18 appendicolith series: V6C18 Apt test: V2C14 arteriohepatic dysplasia: Table Alagille syndrome (arteriohepatic dysplasia with retinal degeneration in some Acid Maltase Deficiency Liver Tissue Procurement and Distribution System Stored Specimen ... File Format: PDF/Adobe Acrobat - View as HTML Medical Abbreviations AHD, arterio-hepatic dysplasia. AHD, arteriosclerotic heart disease. AHD, UT Division of Gastroenterology and Hepatology Arteriohepatic dysplasia: a benign syndrome of intrahepatic cholestasis with CURRICULUM VITAE Jose S. Pulido, MD, MS Personal Data US ...

62. KliMo Issue 212/1999 - 3 Alagille syndrome (arteriohepatic dysplasia) follow up of 23 years of Background Alagille syndrome is a arteriohepatic dysplasia which is in most cases http://www.onjoph.com/global/klimo/english/iss212-3.html

A UGENHEILKUNDE I SSUE Abstracts: "Vertical tilt" after penetrating keratoplasty - Comparison between nonmechanical trephination with the excimer laser and motor trephination Achim Langenbucher, Berthold Seitz, Murat M Kus, Gottfried OH Naumann Optical coherence tomography of age-related macular degeneration. Correlation of diagnostic techniques of fluorescence angiography and OCT Christoph W. Spraul, Gabriele E. Lang, Gerhard K. Lang Early posterior capsular fibrosis after combined cataract and vitreoretinal surgery with air/SF6-gas tamponade Kerstin Scharwey, Sima Pavlovic, Karl Wilhelm Jacobi Lattice corneal dystrophy type I. Clinical and molecular genetic analysis in a large family Moritz Meins, Markus Kohlhaas, Gisbert Richard, Andreas Gal Intracanalicular trabeculostomy - A new approach in glaucoma surgery Juergen Kampmeier, Karl Stock, Raimund Hibst, Gabriele E. Lang, Rudolf Steiner, Gerhard K. Lang Eye injury during a laser show Helmut G Sachs, N Baumgathuber, Chris P Lohmann Consequent use of Aachen's therapeutic concept after severe chemical burn of both eyes Thomas Herboth, Gerd Geerling, Gernot Duncker, Gerd-Otto Bastian, Theodor Seiler

63. Alagille Watson Syndrome Alagille Syndrome, sometimes called arteriohepatic dysplasia, is an autosomaldominant disease with highly variable expressivity. http://ibis-birthdefects.org/start/alagsyn.htm

Alagille Watson Syndrome About I.B.I.S. Home Search Topics Search all contents ... "In the News" Messages... Questions/comments Report Dead Links S.O.S. - Exchange Join I.B.I.S. ... Etchings Notice! For more about parental and patient support resources explore Support Groups . For general sources of information see Professional Associations and Information Sources AHD Alagille - Watson Syndrome Arteriohepatic Dysplasia AWS Cholestasis With Peripheral Pulmonary Artery Stenosis Syndromatic Hepatic Ductular Hypoplasia Notice: You may consider searching for Liver or Hepatic disorders Special Resources Alagille Watson Syndrome A Selection of Internet Sites [*] Outstanding [P] For Professionals [S] Support Group [Japanese] [Ukrainian] [*] [P] Alagille Syndrome from GENE Clinics by Lynn Bason, MS, et al., January 5, 2000 Clinical Finding, % of Patients: Bile duct paucity, 85% Chronic cholestasis, 96% Cardiac murmur, 97% Eye findings, 78% Vertebral anomalies, 51% Characteristic facies, 96% Renal disease, 40%

64. Alagille Syndrome With Prominent Skin Manifestations Sengupta Sujata, Das Jayant Alagille syndrome (arteriohepatic dysplasia) is the syndrome of paucity ofintrahepatic bile ducts. It is probably inherited in an autosomal dominant http://www.ijdvl.com/article.asp?issn=0378-6323;year=2005;volume=71;issue=2;spag

65. Daniel Alagille (www.whonamedit.com) ducts (Alagille syndrome or arteriohepatic dysplasia) review of 80 cases . arm of chromosome 20 in arteriohepatic dysplasia (Alagille syndrome). http://www.whonamedit.com/doctor.cfm/153.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. A recommendation: Hypography is an open community about science and all things related Daniel Alagille French paediatrician, born January 24, 1925, Paris. Associated eponyms: Alagille's syndrome A congenital familial syndrome marked by clinical picture with intrahepatic cholestasis, neonatal jaundice, and hepatomegaly. Biography: Daniel Alagille in 1954 graduated from the University of Paris, where he also obtained a diploma in biochemistry. From 1954 to 1964 he worked in the Hôpial Saint-Vincent-de Paul and was appointed associate professor in 1963. He as elevated to full professor of paediatrics and clinical genetics at the Université Paris-Sud in 1971, also being staff physician and chairman of the department of paediatrics, Hôpital de Bicêtre, where he founded a paediatric liver unit. From 1964 he was chief of the paediatric liver research unit of the Institut National de la Santé et de la Recherche. He remained with the Hôpital de Bicêtre until his retirement in 1990. Alagille was chief editor of the Revue internationale d'hépatologie (1954-1971) and Archives françaises de pédiatrie (1964-1990). He has published more than 500 articles and several books. He became Chevalier de l'Ordre National du Mérite in 1967 and Chevalier de la Légion d'Honneur in 1988. By 1996 he was professor emeritus at the Université Paris-Sud in Bicêtre.

66. Alagille's Syndrome (www.whonamedit.com) arteriohepatic dysplasia. Familial pulmonary artery stenosis with neonatal liverdisease. Archives of Disease in Childhood. London, 1973, 48 459466. http://www.whonamedit.com/synd.cfm/729.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. A recommendation: Hypography is an open community about science and all things related Alagille's syndrome Also known as: Watson-Miller syndrome Synonyms: Arterio-hepatic dysplasia, cardiovertebral syndrome, cholestasis-peripheral pulmonary stenosis, cholestasis-pulmonary artery stenosis, cholestasis with peripheral pulmonary stenosis, hepatic ductular hypoplasia, hepatic ductal hypoplasia-multiple malformations syndrome, hepatofacial-neurocardiac-vertebral syndrome, cholestasis-pulmonary artery stenosis syndrome, hepatofacioneurocardiovertebral syndrome, paucity of interlobular ducts. Associated persons: Daniel Alagille V. Miller

67. The American Journal Of Surgical Pathology - UserLogin Distinct hepatic retention of Tc99m IDA in arteriohepatic dysplasia arteriohepatic dysplasia in infancy and childhood a longitudinal study of 6 http://www.ajsp.com/pt/re/ajsp/fulltext.00000478-200506000-00013.htm

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68. ADULT CONGENITAL HEART DISEASE GLOSSARY Alagille syndrome, see arteriohepatic dysplasia arteriohepatic dysplasia,An autosomal dominant multisystem syndrome consisting of intrahepatic http://www.cachnet.org/achd_a.html

aberrant innominate artery A rare abnormality associated with right aortic arch wherein the sequence of arteries arising from the aortic arch is: right carotid artery, right subclavian artery, then (left) innominate artery. The latter passes behind the esophagus. This is in contrast to the general rule that the first arch artery gives rise to the carotid artery contralateral to the side of the aortic arch (i.e.: right carotid artery in left aortic arch and left carotid artery in right aortic arch). syn . retro-esophageal innominate artery. aberrant subclavian artery The right subclavian artery arises from the aorta distal to the left subclavian artery. Left aortic arch with (retroesophageal) aberrant right subclavian artery is the most common aortic arch anomaly, first described 1735 by Hunauld, and occurring in 0.5% of the general population. absent pulmonary valve syndrome Pulmonary valvular tissue is absent, resulting in pulmonary regurgitation. This rare anomaly uncommonly may be isolated; or it may be associated with ventricular septal defect, obstructed pulmonary valve annulus and massive dilation and distortion of the pulmonary arteries. Absent pulmonary valve may also occur in association with other simple or complex congenital heart lesions. ACHD Adult Congenital Heart Disease Alagille syndrome see arteriohepatic dysplasia ALCAPA Anomalous left coronary artery arising from the pulmonary artery.

69. CANADIAN ADULT CONGENITAL HEART NETWORK Noonan syndrome, VSD, arteriohepatic dysplasia or congenital Rubella syndrome . also called arteriohepatic dysplasia) may have pulmonary stenosis, http://www.cachnet.org/managing_recomp2.html

RECOMMENDATIONS FOR THE MANAGEMENT OF ADULTS WITH CONGENITAL HEART DISEASE - 2001 (PART 2) Click here to view other parts. Table of Contents Section VI - Coarctation of the Aorta Section VII - Right Ventricular Outflow Tract Obstruction Section VIII - Tetralogy of Fallot Section IX - Ebstein Anomaly ... Bibliography Section V - Left Ventricular Outflow Tract Obstruction Part l - Background Information Definition: This section concerns left ventricular outflow tract obstruction (LVOTO) in the setting of concordant atrioventricular and ventriculoarterial connections. (Neither hypertrophic cardiomyopathy nor interrupted aortic arch will be considered here.) LVOTO can occur at several levels: Supravalvar LVOTO may occur rarely in isolation as an hourglass deformity. It is more often diffuse however, involving the major arteries to varying degrees and begins at the superior margin of the sinuses of Valsalva. Valvar LVOTO in the adult patient with CHD is usually due to bicuspid aortic valve (rheumatic and trileaflet calcific aortic stenosis are excluded here). It usually occurs in isolation but is associated with other abnormalities, the most common being coarctation of the aorta (which should be sought), PDA, or ascending aortopathy. Subvalvar LVOTO is usually either a discrete fibromuscular ridge which partially or completely encircles the left ventricular outflow tract or is a long fibromuscular narrowing beneath the base of the aortic valve. Occasionally, there is a tunnel-like narrowing of the whole left ventricular outflow tract with a small aortic root. Rarely, abnormal insertion of the mitral valve or accessory mitral leaflet may cause significant obstruction.

70. Blackwell Synergy - Cookie Absent intrahepatic bile hypoplasia, and arteriohepatic dysplasia. bile ducts (Alagillesyndrome or arteriohepatic dysplasia) review of 80 cases. http://www.blackwell-synergy.com/doi/abs/10.1111/j.1525-1470.2005.22102.x

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71. Blackwell Synergy - Cookie Absent 2, Watson OH, Miller V. arteriohepatic dysplasia familial pulmonary arterialstenosis with neonatal liver disease. Arch Dis Child 1973; 48 459 466. http://www.blackwell-synergy.com/doi/abs/10.1111/j.1460-9592.2004.01535.x

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72. PharmGKB: Alagille Syndrome Alternate Names, arteriohepatic dysplasia; arteriohepatic dysplasias; Dysplasia,Arteriohepatic; Dysplasias, Arteriohepatic; Syndrome, Alagille http://www.pharmgkb.org/do/serve?objId=PA446376&objCls=Disease

73. Alagille Syndrome We report a case of Alagille syndrome (arteriohepatic dysplasia) with the unusualradiological abnormality of synostosis of the proximal portions of the http://www.thedoctorsdoctor.com/diseases/alagille_syndrome.htm

Background Alagille syndrome is a rare inherited syndrome characterized by a reduction in intrahepatic bile ducts associated with multiple abnormalities in many other organ systems such as the cardiovascular system, skeleton, and kidneys. Recently, the syndrome has been associated with mutations in the JAGGED gene, mapped to chromosome 20. OUTLINE Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/ ... Internet Links EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Alagille-Watson syndrome Arteriohepatic dysplasia Syndromic bile duct paucity INCIDENCE/ PREVALENCE 1/100,000 live births AGE SEX GEOGRAPHY EPIDEMIOLOGIC ASSOCIATIONS DISEASE ASSOCIATIONS CHARACTERIZATION COAGULOPATHY Bleeding tendency in children with Alagille syndrome. Lykavieris P, Crosnier C, Trichet C, Meunier-Rotival M, Hadchouel M. Service d'Hepatologie pediatrique, Hopital de Bicetre, Le Kremlin Bicetre Cedex, France. Pediatrics. 2003 Jan;111(1):167-70. Abstract quote OBJECTIVE: Spontaneous intracranial bleeding is now a widely recognized complication and cause of mortality in patients with Alagille syndrome. The pathogenesis of intracranial bleeding in these patients remains unclear. The aim of the study was to look for other sites of bleeding in these patients that could suggest a factor of multiorgan morbidity. METHODS: The records of 174 patients with Alagille syndrome were reviewed, and 38 (22%) patients without liver failure who experienced hemorrhage that led to a drop in hemoglobin level of at least 3 g/dL or to blood transfusion were identified.

74. Clinical Nuclear Medicine - UserLogin We encountered a patient with arteriohepatic dysplasia (Alagille s syndrome),whose scan appearance was significantly different from the few other described http://www.nuclearmed.com/pt/re/cnm/fulltext.00003072-199602000-00003.htm

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75. Harriet Lane Links Alagille s Syndrome/arteriohepatic dysplasia Dermatologic Finding 5http//tray.dermatology.uiowa.edu/Alagil05.htm 0314-2000 http://derm.med.jhmi.edu/poi/search.cfm?MolecularBiology=-1&SearchTitle=Molecula

76. Radiology Cases In Pediatric Emergency Medicine Subject Index V6C18 appendicolith series V6C18 Apt test V2C14 arteriohepatic dysplasiaV6C5 arthrocentesis ankle V3C6 ascites V2C4 atelectasis, pulmonary V2C7, http://www.hawaii.edu/medicine/pediatrics/pemxray/zindex.html

SUBJECT INDEX Editors: Loren G. Yamamoto, MD, MPH Alson S. Inaba, MD Robert M. DiMauro, MD Kapiolani Medical Center For Women And Children Dept. Pediatrics, University of Hawaii John A. Burns School of Medicine 1319 Punahou Street, Honolulu, HI 96826 Return to Univ. Hawaii Dept. Pediatrics Home Page Return to the Radiology Cases In Pediatric Emergency Medicine Home Page Vol 1 - Vol 6 Index A abdominal abscess: abdominal distention: abdominal pain appendicitis: see also: appendicitis bowel obstruction - see bowel obstruction cholecystitis: imperforate hymen: intussusception: see also intussusception Meckel's diverticulitis: non-specific: leukemia: pneumonia: psoas abscess: spinal fracture: volvulus - see volvulus abdominal radiographs, series, test your skill: abscess: abdominal - see abdominal abscess prevertebral - see retropharyngeal abscess psoas - see psoas abscess retropharyngeal - see retropharyngeal abscess absent liver edge sign of intussusception: acetabular fracture: Achille's tendonitis - see Sever's disease acquired immunodeficiency syndrome - see HIV acromioclavicular (AC) injury: AIDS - see HIV airway obstruction: Alagille's syndrome: aneurysmal bone cyst: angiography: aortography - see aortogram cerebral: magnetic resonance: ankle sprain: ankle fracture: ankle radiographs, series, test your skill:

77. Radiology In Ped Emerg Med, Vol 6, Case 5 Teaching Points 1) Alagille syndrome (arteriohepatic dysplasia) is characterizedby a paucity of intrahepatic bile ducts. Clinical manifestations include http://www.hawaii.edu/medicine/pediatrics/pemxray/v6c05.html

Elbow Swelling In a 2 Year Old With Liver Disease Radiology Cases in Pediatric Emergency Medicine Volume 6, Case 5 Donna Mendez, MD Children's Medical Center of Dallas University of Texas Southwestern School of Medicine The radiograph demonstrates a healing right radial midshaft fracture. Also noted are multiple lytic lesions with cortical scalloping along the metaphysis and diaphysis of the forearm bones with generalized severe demineralization. A long bone survey is obtained. View his lower extremity radiographs. View femur radiographs. View tibia radiographs. Return to Radiology Cases In Ped Emerg Med Case Selection Page Return to Univ. Hawaii Dept. Pediatrics Home Page Web Page Author: Loren Yamamoto, MD, MPH Professor of Pediatrics University of Hawaii John A. Burns School of Medicine Loreny@hawaii.edu

78. Arch Pediatr Adolesc Med -- Table Of Contents (Vol. 148 No. 3, March 1994) Alagille syndrome (arteriohepatic dysplasia) L. Marodi; G. Rigo; R. Kaposzta ArchPediatr Adolesc Med. 1994;148287288. Detection of measles virus from http://archpedi.ama-assn.org/content/vol148/issue3/index.dtl

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 148 No. 3, pp. 243-334, March 1994 Content Access Sign in/out Activate online subscription One-time access Individual subscriptions ... Register for E-mail Alerts Advertisement //This is the Right Skyscraper DisplayAds ("Right1,Position2!Right1", "120", "600"); //This is the Left Skyscraper DisplayAds ("Right1,Position2!Position2", "120", "240"); Table of Contents Jump to Section ARTICLES ARTICLES General pediatrics in a time of change. Inventing the future M. E. Wilson Arch Pediatr Adolesc Med. The role of socioeconomic status and injury morbidity risk in adolescents R. Anderson; S. R. Dearwater; T. Olsen; D. J. Aaron; A. M. Kriska; R. E. LaPorte Arch Pediatr Adolesc Med. ABSTRACT Human immunodeficiency virus infection in infants during the first 2 months of life. Reliable detection and evidence of in utero transmission C. D. Brandt; T. A. Rakusan; A. V. Sison; E. S. Saxena; M. Ellaurie; J. L. Sever

79. ISACCD - Glossary A-A Alagille syndrome see arteriohepatic dysplasia; ALCAPA Anomalous left arteriohepatic dysplasia An autosomal dominant multisystem syndrome http://www.isaccd.org/profres/a.php

@import url(../css/hidden.css); Become a Member Professional Resources News Jobs/Training ... Home a Find terms beginning with: A B C D ... Z Revised April 2002 Contact (email) Jack Colman: j.colman@utoronto.ca Erwin Oechslin: erwin.oechslin@usz.ch Dylan Taylor: dtaylor@cha.ab.ca Purpose The purpose of this glossary is to help guide those reading and researching in the area of adult congenital heart disease. It is meant to be a living document, constantly under revision, improvement, correction, as you, its users, find ways to ease the path for those who follow. To this end, if you cannot find a term you think should be here, or if you disagree with a definition, or see a way to improve it, drop us an e-mail before you move on. We promise to consider all feedback carefully, and to make additions and revisions often. We hope you find the glossary helpful. Prepared by: Jack M. Colman MD, FRCPC, FACC Erwin Oechslin MD Dylan Taylor MD, FRCPC, FACC Job Board: Search Post Professional Resources Sep 08, 2005 aberrant innominate artery A rare abnormality associated with right aortic arch wherein the sequence of arteries arising from the aortic arch is: right carotid artery, right subclavian artery, then (left) innominate artery. The latter passes behind the esophagus. This is in contrast to the general rule that the first arch artery gives rise to the carotid artery contralateral to the side of the aortic arch (i.e.: right carotid artery in left aortic arch and left carotid artery in right aortic arch).

80. Triangular Face And Vascular Malformation. The Links To Renal Failure -- Wauters ducts (Alagille syndrome or arteriohepatic dysplasia) review of 80 cases . C. Ocular anomalies in the Alagille syndrome (arteriohepatic dysplasia). http://ndt.oxfordjournals.org/cgi/content/full/18/2/436

JOURNAL HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Extract FREE Full Text (PDF) Alert me when this article is cited ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed ... Request Permissions PubMed PubMed Citation Articles by Wauters, A. Articles by Maes, B. Nephrol Dial Transplant (2003) 18: 436-439 European Renal Association-European Dialysis and Transplant Association Nephroquiz Triangular face and vascular malformation. The links to renal failure Anne Wauters Tom Dejagere Koen Devriendt Yves Vanrenterghem and Bart Maes Department of Nephrology and Department of Human Genetics, University Hospital Gasthuisberg, Leuven B-3000, Belgium Email: Case A 40-year-old woman was referred to our out-patient clinic with moderate renal failure (serum creatinine, 2.35 mg/dl; urea, 76 mg/dl) and slight proteinuria (0.2 g/24 h). Urine microscopy

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