Zangger Halvorsen RA Jr; Garrity S; Kuni C; du Cret RP; Letourneau JG; Bloomer J arteriohepatic dysplasia (Alagille s syndrome) unusual hepatic architecture and http://radiologie-dijon.chez.tiscali.fr/Biblio/Halvorsen.htm
Daniel Alagille (www.whonamedit.com) M. Gautier, M. Odièvre, JP Dommergues Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia) review of 80 cases. http://www.whonamedit.com/doctor.cfm/153.html
Extractions: 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. 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.
Alagille's Syndrome (www.whonamedit.com) Bibliography GH Watson, V. Miller arteriohepatic dysplasia. Familial pulmonary artery stenosis with neonatal liver disease. Archives of Disease in Childhood. http://www.whonamedit.com/synd.cfm/729.html
Extractions: 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. 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. A congenital familial syndrome with onset during first three months of life, affecting both sexes. Marked by clinical picture with intrahepatic cholestasis due to hypoplasia of the interlobular biliary duct, neonatal jaundice, hepatomegaly (from 3rd month). Long list of symptoms include dysmorphic (flat) facies sometimes referred to as cholestasis facies, with prominent forehead, mongoloid slant, mild hypertelorism, straight or bulbous nose, and skeletal anomalies; occasionally diffuse xanthomas (palms, extensor areas, creases), and pruritus, Harsh mesosystolic murmur. Growth and mental retardation. In males, hypogonadism. Etiology unknown. Inheritance is autosomal dominant with reduced penetrance and variable expressivity.
Extractions: WWW Medical.WebEnds.com Arteriohepatic Dysplasia; Dysplasia, Arteriohepatic; Arteriohepatic Dysplasias; Dysplasias, Arteriohepatic; Syndrome, Alagille Hypoplasia of the hepatic ducts, congenital pulmonary artery stenosis, facial abnormalities , and other congenital malformations, particularly skeletal. It is often presented as jaundice during the neonatal period. It is an autosomal recessive disease generally manifesting during childhood. "Arteriohepatic" refers to the pulmonary artery and the intrahepatic bile ducts, not to the hepatic artery
Alagille Watson Syndrome Alagille Syndrome, sometimes called arteriohepatic dysplasia, is an autosomal dominant disease with highly variable expressivity. http://ibis-birthdefects.org/start/alagsyn.htm
Right Ventricular Outflow Tract Obstruction It may occur in tetralogy of Fallot, Williams syndrome, Noonan syndrome, VSD, arteriohepatic dysplasia or congenital Rubella syndrome. http://www.rbh.nthames.nhs.uk/Cardiology/Consensus/rightventricular.htm
Extractions: SECTION VII - RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION (RVOTO) Part I - Background Information Supravalvar RVOTO seldom occurs in isolation. It may occur in tetralogy of Fallot, Williams syndrome, Noonan syndrome, VSD, arteriohepatic dysplasia or congenital Rubella syndrome. Valvar RVOTO, the most common form of RVOTO, is almost always congenital in origin. Typically, the stenotic pulmonic valve is a thin, pliable, dome-shaped structure, with a narrow opening at its apex. In 10-15% of cases, the valve is dysplastic with thickened and immobile cusps. In adults, the valve may calcify late in life. Subvalvar (infundibular) RVOTO usually occurs in combination with other lesions, particularly ventricular septal defect, and as part of tetralogy of Fallot. A separate but somewhat similar entity is "double-chambered right ventricle" with mid-cavity obstruction, often from a prominent moderator band. This may be associated with a VSD.
Alagilles Syndrom - Små Och Mindre Kända Handikappgrupper Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia) review of 80 cases. J Pediatr 1987; 110 195200. http://www.sos.se/smkh/1998-29-079/1998-29-079.HTM
Extractions: Version: 2.2 Socialstyrelsen Detta är ett utdrag ur Socialstyrelsens kunskapsdatabas om små och mindre kända handikappgrupper. Med små och mindre kända handikappgrupper avses ovanliga sjukdomar/skador som leder till omfattande funktionshinder och som finns hos högst 100 personer per miljon invånare. Syftet med databasen är att ge aktuell information om små och mindre kända handikappgrupper och om det stöd och den service som dessa grupper behöver. För ytterligare information om aktuell diagnos hänvisas till informationsmaterial, litteratur och databaser som anges under resp diagnos. Orsak till sjukdomen/skadan Alagilles syndrom orsakas vanligtvis av en nymutation Praktiska tips Resurspersoner Professor Antal Nemeth, Barnmedicinska kliniken, Huddinge Universitetssjukhus, 141 86 Stockholm, tel 08-585 800 00.
.: In There, Always There, RadioThere :. Bone dysplasia overran. That shall arteriohepatic dysplasia by against. These atrio digitalis dysplasia your. Cemental dysplasia periapical forward. http://www.radiothere.net/?w=dysplasia
ADULT CONGENITAL HEART DISEASE GLOSSARY ACHD, Adult Congenital Heart Disease. Alagille syndrome, see arteriohepatic dysplasia. ALCAPA, Anomalous left coronary artery arising from the pulmonary artery. http://www.cachnet.org/achd_a.html
Extractions: 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.
Radiology Cases In Pediatric Emergency Medicine Subject Index V3C18, V3C19, V4C10, V6C18 appendicolith V3C18, V4C10, V6C18 appendicolith series V6C18 Apt test V2C14 arteriohepatic dysplasia V6C5 arthrocentesis ankle http://www.hawaii.edu/medicine/pediatrics/pemxray/zindex.html
Extractions: 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:
Radiology In Ped Emerg Med, Vol 6, Case 5 What bony abnormality is present? Teaching Points 1) Alagille syndrome (arteriohepatic dysplasia) is characterized by a paucity of intrahepatic bile ducts. http://www.hawaii.edu/medicine/pediatrics/pemxray/v6c05.html
Extractions: 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
International Society For Adult Congenital Cardiac Disease ACHD Adult Congenital Heart Disease Alagille syndrome see arteriohepatic dysplasia ALCAPA Anomalous left coronary artery arising from the pulmonary artery. http://www.isaccd.org/profres/a.php
Extractions: Dylan Taylor MD, FRCPC, FACC Job Board: Search Post Jun 02, 2004 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).
Mental Retardation, Directory Alagille Syndrome, eMedicine, Ann Scheimann Alagille Watson Syndrome, IBIS arteriohepatic dysplasia (Alagille Syndrome), The Transplant Center JAG1, CancerGene http://www.saunalahti.fi/kup/engl/directory.htm
Dy : On Medical Dictionary Online Spastic Dysphonias, Spastic Pseudobulbar Dysplasia Epiphysialis Punctata Dysplasia, Anhidrotic Ectodermal Dysplasia, arteriohepatic dysplasia, Branchio http://www.online-medical-dictionary.org/?q=~Dy
JMG Online -- Table Of Contents (24 [10]) 616620. Abstract. The Alagille syndrome (arteriohepatic dysplasia) RF Mueller J Med Genet 1987; 24 621-626. Brachmann-de Lange http://jmg.bmjjournals.com/content/vol24/issue10/index.shtml
J Med Genet -- Abstracts: Krantz Et Al. 34 (2): 152 Also referred to as the Alagille Watson syndrome, syndromic bile duct paucity, and arteriohepatic dysplasia, it is a significant cause of neonatal jaundice http://jmg.bmjjournals.com/cgi/content/abstract/34/2/152
Extractions: Division of Human Genetics and Molecular Biology, The Children's Hospital of Philadelphia, PA 19104, USA. Alagille syndrome (OMIM 118450) is an autosomal dominant disorder associated with abnormalities of the liver, heart, eye, skeleton, and a characteristic facial appearance. Also referred to as the Alagille- Watson syndrome, syndromic bile duct paucity, and arteriohepatic dysplasia, it is a significant cause of neonatal jaundice and cholestasis in older children. In the fully expressed syndrome, affected subjects have intrahepatic bile duct paucity and cholestasis, in conjunction with cardiac malformations
Alagille Syndrome Today Riely CA, Cotlier E, Jensen PS, Klatskin G. arteriohepatic dysplasia a benign syndrome of intrahepatic cholestasis with multiple organ involvement. http://collection.nlc-bnc.ca/100/201/300/cdn_medical_association/cim/vol-19/0325
Extractions: Daniel Alagille, MD Clin Invest Med Paper reprints may be obtained from: Dr. Daniel Alagille, 75 Denfert-Rochereau, 75014 Paris, France; fax 33 1 43 54-2590 A genetic syndrome causing paucity of interlobular bile ducts (Alagille syndrome) is characterized by five main characteristics: typical, peculiar facies; chronic cholestasis; posterior embryotoxon; butterfly-like vertebral-arch defects; and cardiovascular malformations. In the complete form of the syndrome, all five features are observed. Four or less of these characteristics are present in the incomplete or partial forms of this syndrome. Other, less frequent characteristics (growth retardation, mental retardation, renal and bone abnormalities as well as a high-pitched voice) have also been observed. An autosomal dominant mode of genetic transmission with variable penetrance seems likely. Therapy consists of nutritional supplementation of medium-chain triglycerides, essential fatty acids and fat-soluble vitamins. Liver transplantation has been used successfully to treat patients with liver failure, portal hypertension or severe pruritus and xanthomatosis. Table of contents This syndrome, characterized by the association of five major groups of features, was gradually identified over 15 years. In 1965 Smith, Optiz and Inhorn1 described intrahepatic biliary dysgenesis associated with polycystic kidneys and cardiac anomalies. In 1969 I and my colleagues Habib and Thomasin[2] reported 25 cases of intrahepatic biliary hypoplasia and cardiac and vertebral malformations associated with a peculiar facies. The syndrome was better defined in 1975,[3] and the view that it is a specific entity was further strengthened by the description of posterior embryotoxon in 1979 by Riely and associates.[4]
The EOPS Author Index - J 9148 Alagille s syndrome (arteriohepatic dysplasia) hepatocellular carcinoma associated with the syndrome in a 4-year old girl; http://www.helsinki.fi/laak/silk/perus/EOPSAUTJ.html
Extractions: Boston, Massachusetts, U.S.A. Metastatic breast "colloid" carcinoma simulating a primary ciliary epithelial tumour Anaplastic carcinoma of the lacrimal gland presenting with recurrent subconjunctival hemorrhages and displaying incipient sebaceous differentiation Adult extra-renal rhabdoid tumor of the lacrimal gland Prepartum capillary hemangioma (pseudo-Kaposi sarcoma) arising in a nevus flammeus Guest Copenhagen, Denmark Pseudoepitheliomatous hyperplasia of the retinal pigment epithelium Gliomatosis of the retina with secondary involvement of the orbit (? astrocytoma) Glomus tumour (glomangioma) of eyelid Retinal anlage tumour (progonoma melanoticum) of the maxilla Acute keratomalacia with corneal perforation, retinal protrusion and expulsive haemorrhage.
