Extractions: 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.
Extractions: 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
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
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
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
Extractions: 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)
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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
Extractions: 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
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
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
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
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
Extractions: 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.
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.
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
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
Extractions: 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.
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
Extractions: 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.
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