61. Pathology Cases For Diagnosis lymphoblastic leukemia Alpha1-antitrypsin deficiency Ataxia telangiectasia Anomaliesof abdominal venous drainage arteriohepatic dysplasia Atypical retinitis http://wwwpath.usuf2.usuhs.mil/Surg_Path/s96-07/96-7.html

Case 96-7: Pediatric Liver Tumors VI Contributed by J. Thomas Stocker, M.D., COL, MC, USA Date Available: June 4, 1996 - December 18, 1996 Objectives: Please note that this case is review only. CME credit is no longer available for this case. History: This eleven year old white male presented with a week long complaint of vague upper abdominal pain and a mild fever. On physical examination, a firm, slightly tender mass was palpable in the right upper quadrant. Laboratory studies included the following: total bilirubin - 2.1 mg/dL, AST - 65 mg/dL, alpha fetoprotein - 1750 ng/dL (normal <50 ng/dL). An arteriogram was performed prior to resection of a 475 gm mass. Illustrations Figure 1 A selective arteriogram reveals a hypervascular mass in the right lobe of the liver. Figure 2 At surgery the mass partially protrudes from the posterior portion of the right lobe. Figure 3 Cut section of the resected lesion displays a multilobulated an-white mass extending into the adjacent parenchyma. Figure 4 The mass is composed of broad trabeculae of large cells with hyperchromatic nuclei and abundant cytoplasm. Figure 5 The trabeculae are 6-10 cells in thickness. Note the anisocytosis and nuclear hyperchromasia with prominent nucleoli. Mitoses were abundant and occasional bizarre.

62. Alagille Syndrome Alliance arteriohepatic dysplasia cholestasis with peripheral pulmonary stenosis intrahepaticbild duct paucity syndromatic hepatic ductular hypoplasia hepatic http://www.medhelp.org/amshc/amshc238.htm

Title: Aicardi Syndrome Newsletter, Inc. Description: Support for families of daughters with Aicardi syndrome, a rare, seizure disorder that affects only females and is characterized by retinal lesions. Information and referrals, resources, research projects. Phone support network, research group, newsletters. Dues $25/year. Scope: International network Founded: Address: c/o Denise M. Parsons 1510 Polo Fields Ct. Louisville, Kentucky, 40245 United States Telephone: Fax: Email: aicnews@aol.com Web Address: http://www.aicardi.com This information has been generously provided by The American Self Help Clearinghouse and hosted by Med Help International . Please send corrections/updates to ed@selfhelpgroups.org Updated: 02/2003

63. Centro Medico Teknon : Library ....... Translate this page Alagille syndrome from other liver and bile duct diseases in infants.It is also known as arteriohepatic dysplasia. http://www.teknon.es/Library/Library.asp?563

64. Rare Diseases List - Office Of Rare Diseases AGS; Watson Alagille syndrome. Alagille syndrome. Cholestasis with peripheralpulmonary stenosis. arteriohepatic dysplasia. Disease Information. http://ord.aspensys.com/diseaseinfo.asp?ID=804

65. CNP Course 1.3 of hepatocellular bile acid transport.(54) Another rare cause is from fibrosis seenin Jeune Syndrome.(55) arteriohepatic dysplasia (Alagille's Syndrome) is http://www.peponline.com/cnp1_3.htm

Umbilical cord alpha-fetoprotein levels may be useful to identify those at risk for hyperbilirubinemia.(34) It was found in 4.5% of Chinese, 3.5% of Malay, and 1.5% of Indian babies. However, under 1% required treatment for hyperbilirubinemia (standards different in U.S.?) and had average peak bilirubins of 12 mg./dl.- not an overwhelming problem.(35) Hereditary spherocytosis is another uncommon cause that should be considered once blood group incompatibilities have been ruled out.(36) Rare intrauterine hemolytic diseases have been reported: Intrauterine pyknocytosis is reported as a rare cause of intrauterine hemolysis.(37) Also, a rare case of anti-C hemolysis requiring aggressive treatment has been reported.(38) A case of erythrocyte glutathione S-transferase deficiency associated with hemolysis and hyperbilirubinemia has been described, but its significance in the newborn population has not been defined.(39) It has been shown, however, that the activity of enzymes that scavenge oxygen radicals, glutathione, peroxidase, and superoxide dismutase, are lower in infants with hyperbilirubinemia. A deficiency of factors protecting from oxygen toxicity may play a role in hemolysis and jaundice.(40) There is a case report of a mother with autoimmune hemolytic disease due to Hodgkin's disease whose baby carried the same IgG antibody and required aggressive treatment for hemolytic disease.(41) Relatively excessive hemolysis occurs in cases of polycythemia wherein baby simply has excess red cells. Of the 1.5% of newborns with polycythemia, 22-33% develop hyperbilirubinemia.(42) Miscellaneous causes of hemolysis that may affect newborns include Vitamin C administered to premature infants(43) and blood heated in IV tubing(44). Phenolic cleaners were exonerated as the cause of 2 cases of Heinz body anemia and hyperbilirubinemia in one institution(45), but an oxidant in food has been postulated as a cause of this rare problem(46). Benzyl alcohol used in neonatal intensive care units has been associated with increased brain hemorrhages, but not with hyperbilirubinemia.(47)

66. 1Up Health > Health Links Directory > Conditions And Diseases: A Disease (11) Apert Syndrome (10) Aphasia (177) Aplastic Anemia (13) Apnea, Sleep(43) Appendicitis (9) Arrhythmia (77) arteriohepatic dysplasia (5) Arthritis http://www.1uphealth.com/links/conditions-and-diseases-a.html

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67. Alagille Syndrome From Pediatrics / Gastroenterology Syndrome Synonyms, Key Words, and Related Terms AS, Alagille's syndrome, AlagilleWatsonsyndrome, arteriohepatic dysplasia, syndromic bile duct paucity. http://author.emedicine.com/ped/topic60.htm

eMedicine Journal Pediatrics Gastroenterology Alagille Syndrome Synonyms, Key Words, and Related Terms: AS, Alagille's syndrome, Alagille-Watson syndrome, arteriohepatic dysplasia, syndromic bile duct paucity Author Information Introduction Clinical Differentials ... Bibliography AUTHOR INFORMATION Section 1 of 11 Authored by Ann Scheimann, MD , Assistant Professor, Department of Pediatrics, Section of Nutrition and Gastroenterology, Baylor College of Medicine and Johns Hopkins Medical Institution Ann Scheimann, MD, is a member of the following medical societies: North American Society for Pediatric Gastroenterology and Nutrition Edited by Robert Baldassano, MD , Director, Center for Pediatric Inflammatory Bowel Disease, Assistant Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania and Children's Hospital of Philadelphia; Robert Konop, PharmD , Clinical Assistant Professor, Department of Pharmacy, Section of Clinical Pharmacology, University of Minnesota; Carmen Cuffari, MD

68. Page 2 INTRODUCTION Alagille syndrome is also known as AlagilleWatson syndrome,syndromic bile duct paucity, and arteriohepatic dysplasia. http://www.geocities.com/anaivette_morales/ALAGILLE/Page_2x.html

This site is intended to complement professional advice HOME DISEASES ABOUT THIS WEBSITE ABOUT THE AUTHOR Alagille Syndrome ABOUT THE AUTHOR ABOUT THIS WEBSITE DISEASES HOME ... References INTRODUCTION Alagille syndrome is also known as Alagille-Watson syndrome, syndromic bile duct paucity, and arteriohepatic dysplasia. Some of the most common problems associated with this condition are liver disease, heart disease, and problems in the spine, eyes, and kidneys. Although genetic testing is available for Alagille syndrome, the diagnosis is mainly based on clinical findings. GENETICS Alagille syndrome is caused by an alteration in a gene called JAG-1. This gene is located on chromosome 20 and it plays a role in early human development. The condition is inherited in an autosomal dominant fashion, which means that there is a

69. Service Page - Pathologie Information détaillée de la maladie,...... DISEASE Bile ducts paucity, syndromic form, Synonym(s) Alagille syndromearteriohepatic dysplasia, CIM Q44.7, http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=52

70. ORPHANET® : Bile Ducts Paucity Translate this page ORPHANET. ORPHANET database access. Bile ducts paucity. Direct access todetails Alias Alagille syndrome,arteriohepatic dysplasia. Home Page. http://www.orpha.net/static/GB/bile_ducts_paucity.html

ORPHANET database access Bile ducts paucity Direct access to details Alias : Home Page

71. zŠÂŠíÇŒóŒQIV 208. I, Contiguous gene (deletion) syndrome, 1, arteriohepatic dysplasia(AHD), , 211. 2, DiGeorge?, , 214. http://www.nippon-rinsho.co.jp/backnum/s_mokuji/jyunkanki_4.html

zŠÂŠíÇŒóŒQ@IV •W‘è ƒgƒbƒvƒI[ƒT[ ‰ª“c —¹ŽO ‚w‚h‚h‚h ŠeŽíŽ¾Š³¥•a‘Ô‚É‚Ý‚ç‚ê‚éS¥ŒŒŠÇ¥ŒŒˆ³ˆÙ‰c A ŽRè’mŽq ŠŒF×–EŽî ²“¡ W ƒJƒ‹ƒ`ƒmƒCƒhŽîᇠŽO•i’¼Žq bó‘BŽ¾Š³ Ô’‘ñ•F •›bó‘BŽ¾Š³ •›t”玿Ž¾Š³‚É‚Ý‚ç‚ê‚éS ¥ ŒŒŠÇ ¥ ŒŒˆ³ˆÙí –TŽ ‹ ‘̍זEŽî “c•Ó»‘ã B ‚”AŽ_ŒŒÇ ¬Œ@ËŽO ƒVƒgƒXƒeƒ[ƒ‹ŒŒÇ ‚‹´³s “d‰ðŽ¿ˆÙí ‹´’ܗǍK “œ”A•a ”ª–ØŽõ•v ƒrƒ^ƒ~ƒ“ˆÙíÇ‚É‚Ý‚ç‚ê‚éSŒŒŠÇŽ¾Š³ ˆ»D½l ”ì–žÇ, “à‘ŸŽ‰–bÇŒóŒQ ’†‘ºŽ¡—Y C æ“V«‘ãŽÓˆÙíÇ ƒAƒ~ƒmŽ_‘ãŽÓˆÙí ’†—ÑŒ[‹L “œŒ´•a ––ΗT ƒ~ƒgƒRƒ“ƒhƒŠƒAˆÙíÇ —é–ØŽO˜aŽq —L‹@Ž_‘ãŽÓˆÙí ’“c³•F ‘å˜a“c‘€ D ˆâ“`«_Œo ‹Ø‹Ù’£«ƒWƒXƒgƒƒtƒC[ “c‘º‘ñ‹v ‹ØƒWƒXƒgƒƒtƒB[ ÎŒ´˜úK Šâè‘×—Y E ˆâ“`«Œ‹‡DŽ¾Š³ Ehlers-Danlos ÇŒóŒQ ^“cƒˆê MarfanÇŒóŒQ ^“cƒˆê MASS phenotype œŒ`¬•s‘SÇ ‰œ ÍŽO ’oŠÉ«”畆 = ‰œ ÍŽO ‘å“®–¬‹NŽn•”Šg’£ “c’†N”Ž ’e«üˆÛ«‰¼«‰©FŽî F ˆ««ƒŠƒ“ƒpŽî “cŒû”Žš  Œ´”­«ŒŒ¬”ÂŒŒÇ, Ž”­«ŒŒ¬”ÂŒ¸­«Ž‡”Á•a DŽ_‹ ‘‘½ÇŒóŒQ ‚‘º‰ë”V œ‘ˆÙŒ`¬ÇŒóŒQ £ìˆè•v ¬lT×–E”’ŒŒ•a(ATL) ‘½”­«œ‘Žî‚¨‚æ‚э‚ŒŽ•a Ž©ŒŒ•a‚É‚¨‚¯‚ézŠÂŠíŽ¾Š³ –¼‰z‰·Œ •nŒŒÇ –ƉuƒOƒƒuƒŠƒ“ˆÙíÇ(‚IgEÇŒóŒQ‚È‚Ç) ™ŽR‰·l G äPŒ´•a ¥ ŒŒŠÇ‰Š‚Ƌ߉Ž¾Š³ Behcet•a ’JŒû—²O Overlap ÇŒóŒQ‚É‚¨‚¯‚éS Still•a “V–쏻•v Wegener “÷‰èŽîÇ ‹g“c‰ëŽ¡ ¬‡«Œ‹‡‘gD•a(MCTD) is«‘Sg«d‰»Ç (PSS) ‚Œ³r•F ‘Sg«‰óŽ€«ŒŒŠÇ‰Š, ‰ß•q«ŒŒŠÇ‰Š

72. CCS Supravalvar RVOTO seldom occurs in isolation. It may occur in tetralogy of Fallot,Williams syndrome, Noonan syndrome, VSD or arteriohepatic dysplasia. http://www.ccs.ca/society/conferences/archives/1996/1996part-07.asp

Abstracts Awards 2003 Canadian Cardiovascular Congress Canadian Training Programs ... Stethoscoop Canadian Consensus Conference on Adult Congenital Heart Disease 1996 Title: Section VII - Right ventricular outflow tract obstruction PART 1 - BACKGROUND INFORMATION RVOTO can occur at any level. Supravalvar RVOTO seldom occurs in isolation. It may occur in tetralogy of Fallot, Williams syndrome, Noonan syndrome, VSD or arteriohepatic dysplasia. Valvar RVOTO, the most common form of RVOTO, is almost always congenital in origin. In 15% of cases, the valve is dysplastic as well as stenotic. In adults, the valve may calcify from the fourth decade onwards. Subvalvar (infundibular) RVOTO usually occurs in combination with other lesions, particularly ventricular septal defect, and as part of tetralogy of Fallot. RVOTO (either valvar or subvalvar) may occur in association with subaortic stenosis. A separate but somewhat similar entity is ''double-cham- bered RV' with midcavity obstruction. This may be associated with a small VSD (see indications for intervention). Branch PA stenosis is not considered here.

73. Nature Publishing Group cholangitis, extrahepatic biliary arresia, idiopathic adulthood ductopenia, idiopathicneonatal hepatitis, Byler's disease, and arteriohepatic dysplasia. http://www.nature.com/cgi-taf/DynaPage.taf?file=/gt/journal/v9/n3/full/3301638a.

74. Other Names Of Alagille Syndrome most frequently encountered in medical literature and in talking with healthcare professionals include arteriohepatic dysplasia (AHD) WatsonAlagille http://www.alagille.org/othernames.html

Other names for Alagille Syndrome most frequently encountered in medical literature and in talking with health care professionals include: Arteriohepatic Dysplasia (AHD) Watson-Alagille Syndrome Syndromic Bile Duct Paucity (SBDP) Syndromic Hepatic Ductular Hypoplasia Syndromic Intrahepatic Biliary Hypoplasia Cholestasis with Peripheral Pulmonary Stenosis Intrahepatic Biliary Atresia or Dysgenesis Printable version This page was last updated 03/04/02. When more information becomes available, it will be added.

75. 227: ALAGILLE SYNDROME WITH HEMOCHROMATOSIS Alagille syndrome is a rare inherited condition of arteriohepatic dysplasiausually recognized within the first year of life. Jaundice http://www.pulsus.com/cddw2003/abs/abs227.htm

ALAGILLE SYNDROME WITH HEMOCHROMATOSIS J Leonard , M Borgaonkar Department of Medicine, Memorial University of Newfoundland, St. John's, Newfoundland A case report of a patient with both Alagille syndrome and hemochromatosis will be discussed. Alagille syndrome is a rare inherited condition of arteriohepatic dysplasia usually recognized within the first year of life. Jaundice and cholestasis resulting from biliary tract hypoplasia are the predominant features. Alagille syndrome may result in long-term complications including cirrhosis, hepatocellular carcinoma, and pancreatic insufficiency. Hemochromatosis is also a genetic disorder characterized by increased gut iron absorption. Eventually there can be iron deposition in several areas including the liver, heart, and pancreas leading to organ dysfunction. Until now, there have been no reports of a patient with both Alagille syndrome and hemochromatosis. This is the first case of a patient with both Alagille syndrome and hemochromatosis. This case represents an interesting association of two genetic disorders. Although almost certainly incidental, this association has never before been reported. NEXT ABSTRACT

76. COLESTASIS EN EL NIÑO Translate this page M, Gautier M, Odievre M, Dommergues J. Syndromic paucity of interlobular bileducts (Alagille syndrome or arteriohepatic dysplasia) review of 80 cases. http://www.comtf.es/pediatria/Congreso_AEP_2000/Ponencias-htm/Luis_Peña.htm

COLESTASIS EN EL NIÑO L.PEÑA QUINTANA,JC RAMOS VARELA,*H.ARMAS RAMOS. UNIDAD DE GASTROENTEROLOGÍA Y NUTRICIÓN PEDIÁTRICA. *HUC TENERIFE, HOSPITAL UNIVERSITARIO MATERNO-INFANTIL LAS PALMAS DE GRAN CANARIA La colestasis se define como un trastorno en la formación-excreción de la bilis que provoca retención de sus componentes en hígado y sangre. La colestasis en el niño casi siempre denota una alteración hepática, que puede ser secundaria a múltiples procesos (Tabla I). Sin embargo, la Atresia de Vías Biliares Extrahepática (AVBEH), el déficit de Alfa 1 Antitripsina, el Síndrome de Alagille y la Colestasis intrahepática familiar progresiva (PFIC) son las causas mas frecuentes en nuestro medio. Nos referiremos principalmente a las dos últimas entidades así como a los trastornos primarios de la síntesis de ácidos biliares, en donde más progresos recientes han existido, sobre todo en el campo de la genética. El neonato y particularmente el prematuro tiene mayor predisposición a la colestasis, ya que en esta etapa de la vida los ácidos biliares tienen menor síntesis, menor captación, menor excreción y menor resorción ileal. El cuadro clínico se manifiesta con ictericia en mayor o menor grado, coluria y heces despigmentadas. Evolutivamente (a partir del 4º-5º mes) y dependiendo de la patología de base, puede aparecer prurito en ocasiones muy severo.

77. Directory :: Look.com Disease (11) Apert Syndrome (9) Aphasia (172) Aplastic Anemia (13) Apnea, Sleep(41) Appendicitis (9) Arrhythmia (81) arteriohepatic dysplasia (5) Arthritis http://www.look.com/searchroute/directorysearch.asp?p=43343

78. Definitions Of Genetic Disorders-A. chiari1_doc.htm. arteriohepatic dysplasia (Alagille Syndrome) http//www.stepstn.com/nord/rdb_sum/473.htm.Arteriovenous Malformations http://www.icomm.ca/geneinfo/def-a.htm

The GAPS INDEX to Information on the Internet about Genetic Disorders and Birth Defects Genetic Information and Patient Services, Inc. (GAPS) HOME DISORDERS GLOSSARY Definitions of Genetic Disorders beginning with the letter A Click on the link next to the disorder to view its definition. A-Beta-Lipoproteinemia abl.htm A-V avcd.htm A-V congheart.html A Beta-2-Microglobulin Amyloidosis amylo.htm A-T ataxia.htm alpha.htm A1AT Deficiency alpha.htm Aagenaes aagen.htm Aarskog syndrome aarsk.htm Aarskog-Scott Syndrome aarsk.htm Aase-smith syndrome aas.htm Aase Syndrome aas.htm AAT alpha.htm AAT Deficiency alpha.htm Abderhalden-Kaufmann-Lignac Syndrome cystinosis.htm Abdominal Muscle Deficiency Syndrome pbs1.htm Abdominal Wall Defect aparosch.htm Abdominal Epilepsy epilepsy.htm Abdominal Migraine cvs1.htm Abductor Spasmodic Dysphonia csd.htm Abductor Spastic Dysphonia csd.htm Abercrombie Syndrome amylo.htm Ablepharon-Macrostomia Syndrome ams.htm

79. Searchalot Directory For A Aplastic Anemia (13); Apnea, Sleep (41); Appendicitis (9); Arrhythmia(81); arteriohepatic dysplasia (5); Arthritis (176); Arthrogryposis (96 http://www.searchalot.com/Top/Health/ConditionsandDiseases/A/

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80. WebGuest - Open Directory : Health : Conditions And Diseases : A 39); Appendicitis@ (8); Arrhythmia@ (77); arteriohepatic dysplasia@(5); Arthritis@ (174); Arthrogryposis@ (96); Asbestosis@ (7); Asperger's http://directory.webguest.com/index.cgi/Health/Conditions_and_Diseases/A/

Browse thru 1000's of books about health, mind and body: About Us Privacy Statement Acceptable Use Policy Legal Notices ... Contact Us the entire directory only in Conditions_and_Diseases/A Top Health Conditions and Diseases : A A B C D ... Ayerza's Syndrome Last update: 20:30 PT, Sunday, September 22, 2002 Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor

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