41. Bradandkathy.com: Revisiting, Again (27 June 2002) arises. Henry has what appears to be a very mild version of Alagille'ssyndrome, aka arteriohepatic dysplasia. An inherited disease. http://www.bradandkathy.com/archives/00000057.html

Navigation Home Photos Remodel Genealogy ... Blog Archive Revisiting, Again I never really made a point in that last entry. My computer was dying and so I just posted it, unfinished. I'm not sure I really had a point, now. I think I was pissed off at the pro-induction doctors for making it sound like induction was a sure way to know what day your baby will arrive and which doctor will be able to deliver your baby; they give you these drugs and voila! A few hours pass and you have a baby. It doesn't always work that way. So, all this has got me thinking about Henry's birth and newborn days. And, it's got me wondering if I could go through it all again. I don't know. Brad doesn't know. We haven't decided anything, but neither one of us is seeming too eager for another child at this point. Before, I always thought two children would be right. Now, I don't know. I guess the genetic issues are hanging over me whenever this subject arises. Henry has what appears to be a very mild version of Alagille's syndrome , aka arteriohepatic dysplasia . An inherited disease. There's a chance any other children we have could also have it. Why don't Brad or I have any symptoms? We don't know. Maybe one of us did and no one realized it. It's all very uncertain. If there was a guarantee that any future children would be disease free or have only very mild versions...

42. KliMo Issue 212/1999 - 3 274). Alagille syndrome (arteriohepatic dysplasia) follow up of 23 years ofstable ocular findings Michael JM Groh, Hartmut Wenkel, Ursula M Mayer. 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

43. The EOPS Author Index - J 9148 Alagille's syndrome (arteriohepatic dysplasia) hepatocellularcarcinoma associated with the syndrome in a 4-year old girl; http://www.helsinki.fi/laak/silk/perus/EOPSAUTJ.html

The EOPS Author Index Go to Home A B C ... Notes Alphabetical List of All Members and Guests Alphabet J Jakobiec, Frederick A. Joint meeting, 1986 Guest-of-Honour Joint meeting, 1991 Joint meeting, 1996 Previous: New York, New York, U.S.A. Massachusetts Eye and Ear Infirmary 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 Jensen, Ove A. Guest Ordinary Member Corresponding Secretary Organising Secretary Emeritus Member, 1994 Rigshospitalet 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.

44. The EOPS Source Index 1991 9148 Jensen O A Alagille's syndrome (arteriohepatic dysplasia) hepatocellularcarcinoma associated with the syndrome in a 4- year old girl 91-49 Stefani FH http://www.helsinki.fi/laak/silk/perus/EOPS1991.html

The EOPS Source Index Go to Home Previous Next 60's ... Notes Nuerenberg, May 8th - 11th, 1991 30th/6th Joint Meeting Jakobiec F A Adult extra-renal rhabdoid tumor of the lacrimal gland Hidayat A A Congenital malignant rhabdoid tumor of the eye and orbit Garner A Lacrimal gland myoepithelioma Friedman A H Fibrous histiocytoma of the lacrimal sac Lee W R Malignant fibrous histiocytoma of orbit: infarction of optic nerve Kock E Chondroma of the orbit Font R L Recurrent well differentiated orbital liposarcoma with myxoid areas occurring in a teenager Tarkkanen A Malignant fibrous histiocytoma of the orbit with spontaneous resolution Latkovic Z Orbital carcinoma of unknown origin - a case for diagnosis Prause J U Castleman's disease in one orbit Goder G J Kimura's disease of the orbit McLean I Primary histiocytoid carcinoma of the eyelid Boniuk M Unusual bluish tumors of the eyelid and conjunctiva: angioleiomyoma and glomangioma Sterkers M An atypical case of tumour of the eyebrow: melanoma or sarcoma? Mullaney J Conjunctival nodule with testicular tumour Conjunctival malignant melanoma Frayer W C Granulomatous conjunctivitis Ferry A P Teddy bear granuloma of conjunctiva (synthetic fiber granuloma) Manschot W A Not-predictable melanoma reaction on empirical radiation doses Naeser P Proton beam treated malignant melanoma Crawford J B Spindle cell tumor of eyelid in patient with lymphoma de Wolff-Rouendaal D Ciliary body and iris metastasis of cutaneous melanoma in a patient with dysplastic naevus syndrome Albert D M Hepatoblastoma metastatic to the eye

45. Tenet Healthcare Corporation - Library ....... syndrome from other liver and bile duct diseases in infants. It is alsoknown as arteriohepatic dysplasia. (Back to Top). http://www.etenet.com/Apps/Library/default.asp?ID=521

46. Health Library - Alagille Syndrome Synonyms. AHD; arteriohepatic dysplasia; Cholestasis with Peripheral PulmonaryStenosis; Syndromatic Hepatic Ductular Hypoplasia. Disorder Subdivisions. None. http://health_info.nmh.org/Library/HealthGuide/IllnessConditions/topic.asp?hwid=

47. WebMD - Del Castillo Syndrome Arhinencephaly Armenian Syndrome Arnold Chiari Syndrome ArnoldChiariMalformation ARSA arteriohepatic dysplasia Arteriosclerosis, Retina http://my.webmd.com/content/healthwise/150/37234.htm

WebMD Today Home WebMD Newscenter Member Services WebMD University My WebMD Find a Doctor, Clinic Medical Info Check Symptoms Medical Library Quizzes, Calculators Clinical Trials ... Family Genetics Who We Are About WebMD Health Mall Sponsored: Lose Lbs Naturally Heart Failure? Trouble Focusing? You are in Medical Library Choose a Topic Our Content Sources Health Guide A-Z Ask A Question Clinical Trials Health Topics Symptoms Medical Tests Wellness ... Support Organizations Search the Help Health Topics Click a letter to see a list of topics beginning with that letter A B C D ... back to top AA Aarskog Syndrome Aarskog-Scott Syndrome AAS Aase Syndrome ... back to top AB Abdominal Migraine Abdominal Muscle Deficiency Syndrome Abdominal Wall Defect Abercrombie Syndrome ... back to top AC ACADL ACADM Deficiency Acanthocheilonemiasis Acanthocytosis ... back to top AD Adamantiades-Behcet's Syndrome Adamantinoma Adams Oliver Syndrome ADD (Attention Deficit Disorder) ... back to top AE AE AEC Syndrome back to top AF AF AFD Afibrinogenemia, Congenital back to top AG AGA Agammaglobulinemias, Primary Aganglionic Megacolon Age-Related Macular Degeneration ... back to top AH AHC AHD AHDS Ahumada Del Castillo Syndrome ... back to top AI Aicardi Syndrome AIDS AIDS Dysmorphic Syndrome AIDS-related lymphoma - Health Professional Information - [CancerNet] ... back to top AK Akureyri Disease back to top AL Alagille Syndrome Albers-Schonberg Disease Albinism Albinism with Hemorrhagic Diathesis and Pigmented Reticuloendothelial Cells ... Alcohol Abuse, Teen

48. Malattie Rare E Genetiche Lettera "W" Miller Sindrome•Watson syndromeAlagille syndrome (AGS)/AlagilleWatson syndrome(AWS)/Watson-Miller syndrome/ arteriohepatic dysplasia (AHD)/cardiovertebral http://utenti.lycos.it/fmfpc/W.htm

HOME FORUM Site Map / Mappa del sito FMFPC's International Guest/Book ... E-mail Dal 22 Marzo 2003 il server Lycos, che ospita le pagine FMFPC, subirà un *fermo tecnico*. Le pagine resteranno accessibili ma non verranno aggiornate per circa una settimana. - Tutti i servizi di FMFPC continueranno a funzionare. - È ora possibile accedere al sito anche attraverso l'indirizzo alternativo di FMFPC (sito mirror) all'URL http://digilander.libero.it/fmfpc/portale.htm ultimi aggiornamenti Elenco malattie rare e genetiche"lettera W" © FMFPC è un sito ideato, realizzato ed aggiornato da un malato di FMF (Febbre Mediterranea Familiare) © FMFPC is a site conceived, realized and updated by a patient of FMF (Familial Mediterranean Fever) aggiornato 12 Marzo 2003 Cerca nel menu aree FMFPC AREE FMFPC Malattie Rare Febbre Mediterranea Familiare Laboratori Diagnosi Genetica Associazioni Malati Disabilità/Handicap Categorie Medicina (Directory) Ricerche WEB (Medicina) FMFPC è partner AidWeb.org Cerca nel sito / Search in this site I contatti fino ad oggi possibili con FMFPC: Cerca in questa pagina / Search in this page con i tasti / with the keys Ctrl+F A B C D ... V - W - X Y Z Anomalie Cromosomiche ... legenda W W Sindrome W Syndrome[Pallister syndrome 1/Median cleft upper lip-mental retardation-pugilistic facies syndrome] W syndrome[Pallister W, syndrome de]

49. Alagille Syndrome Alagille Syndrome. AHD, arteriohepatic dysplasia, Cholestasis withPeripheral Pulmonary Stenosis Authors Nancy B Spinner, PhD http://ribosome.geneclinics.org/profiles/alagille/details.html

Alagille Syndrome [AHD, Arteriohepatic Dysplasia, Cholestasis with Peripheral Pulmonary Stenosis] Authors: Nancy B Spinner, PhD Ian D Krantz, MD Children's Hospital of Philadelphia Initial Posting: 19 May 2000 Last Update 4 February 2003 Summary Disease characteristics. Alagille syndrome is characterized by potentially life-threatening involvement of the liver and heart and by skeletal, ophthalmological, and facial changes that may not be medically significant but can be useful in diagnosis. Hepatic involvement typically presents in the first three months of life as cholestasis, jaundice, and pruritis with a paucity of bile ducts observed histologically; some patients develop liver failure. The most common cardiac manifestations are peripheral and branch pulmonic stenosis (67% of patients) and tetralogy of Fallot (7-16%). Posterior embryotoxon (prominent Schwalbe's ring) of the eye and butterfly vertebrae do not cause symptoms. Diagnosis/testing. The diagnosis of Alagille syndrome is primarily based on clinical findings. Fluorescence in situ hybridization (FISH) detects a microdeletion of 20p12, including the entire

50. NORD - National Organization For Rare Disorders, Inc. To purchase fulltext report ($7.50) View Cart/Checkout. Copyright 1987, 1990,1996, 1998, 1999 Synonyms of Alagille Syndrome AHD; arteriohepatic dysplasia; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Alagille Synd

51. EMedicine - Alagille Syndrome Article By Ann Scheimann, MD Synonyms and related keywords AS, Alagille's syndrome, AlagilleWatsonsyndrome, arteriohepatic dysplasia, syndromic bile duct paucity. http://www.emedicine.com/ped/topic60.htm

52. EMedicine - Nevus Comedonicus : Article By Joseph J Shaffer, MBBS syndrome, hemangiomas. Eye Congenital cataracts (unilateral and bilateral).Other - arteriohepatic dysplasia, rare systemic malignancies. http://www.emedicine.com/derm/topic294.htm

(advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Dermatology Benign Neoplasms Nevus Comedonicus Last Updated: November 15, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: comedo nevus, nevus actiniformis unilateralis, nevus follicularis keratosus, nevus unilateralis comedonicus, nevus actiniformis, zoniform nevus AUTHOR INFORMATION Section 1 of 9 Author Information Introduction Clinical Differentials ... Bibliography Author: Joseph J Shaffer, MBBS , Fellow, Dermatologic Surgery, Department of Cutaneous Surgery, Fairview University Medical Center Coauthor(s): Vincent De Leo, MD , Chair, Program Director, Department of Dermatology, St Luke's and Roosevelt Hospital Center Joseph J Shaffer, MBBS, is a member of the following medical societies: American Academy of Dermatology Editor(s): Barbara Reed, MD , Clinical Associate Professor, Department of Dermatology, University of Colorado Health Sciences Center; Michael J Wells, MD

53. Tissue Type Total Alagille's (Arteriohepatic Dysplasia) Tissue Type. Total. Alagille's (arteriohepatic dysplasia). 15 (2483g). A1AT. 53 (3729g).Alc cirrhosis. 23 (3194g). Alcoholic Liver disease (unspec). 2 (360g). ALD/Hep C. http://www.peds.umn.edu/Centers/ltpads/specim.htm

54. Brooklyn Birthing Center - Medical Terms Arterial blood gas, Arterial tension. Arteriogram, arteriohepatic dysplasia.Arteriosclerosis, Arteriosclerotic aneurysm. Artery, Arthritis. http://www.brooklynbirthingcenter.com/medicalTerms

Women's Health Drug Database Medical Terms Discussion Groups ... HealthyWomen Medical Terms Sometimes doctors use words only they understand. It's difficult enough to understand health information without struggling with unfamiliar words. In order to help you better understand your health and improve your communication with your healthcare provider, we have provided an extensive list of medical terms defined using clear, understandable language. Term Search Type in a Term: Term Select A B C D ... Z A Aarskog-Scott syndrome Aase-Smith syndrome I Aase-Smith syndrome II Abdomen ... Azotemia B Bacillary angiomatosis Back pain Backbone Bacteremia ... Bypass C Caesarian section Caffeine Calcaneal spur Calcific bursitis ... Cytoplasm D D and C Dactyledema Dactylitis Dandruff ... Dysuria E E. coli E. coli hemorrhagic diarrhea Eagle syndrome Ear piercing ... Eyetooth F Fabry disease Face Presentation Facial nerve Fainting ... Fungus G Galactorrhea Gallbladder Gallstones Gamete Intra-Fallopian Tube Transfer (GIFT) ... Gynecomastia H Habitual abortion Hair follicle Hair follicles Hairy-cell leukemia ... Hysteroscopy I Ibuprofen Ichthyosis Ichthyosis simplex Ichthyosis vulgaris ... IV J Jadassohn-Lewandowski Syndrome Jakob-Creutzfeldt disease Jaundice Jejunum ... Juxtaglomerular apparatus K Kanner syndrome Kaposi's Sarcoma Kartagener syndrome Kawasaki disease ... Kyphosis L Labia Labile diabetes Labor LAC encephalitis ... Lymphoproliferative disorders M Macrogenitosomia Macrosomia Macula Macular degeneration ... Myriad N Nail-patella syndrome Nanism Narcolepsy Nasal septum ... Nystagmus O Oat cell cancer Obesity Obsessive-compulsive disorder Obstetrician ... Oxytocin P

55. Untitled Paucity of Interlobular Bile Ducts. 116. Adams PC Hepatocellular carcinoma associatedwith arteriohepatic dysplasia. Dig Dis Sci 31 438442, 1986. 117. http://tpis.upmc.edu/tpis/dlp/CHAP6REF.htm

General Aspects J Pediatr 106; 171-184, 1985 Semin Liver Dis 7: 61-66, 1987 J Hepatology 1: 545-559, 1985 Semin Liver Dis 7: 67-76, 1987 ... Prog Pediatr Surg 6: 113-139, 1974 Biliary Atresia Radiology 163: 377-379, 1987 J Pediatr Surg 13: 389-391, 1978 Hepatology 4: 7S-10S, 1984 J Pediatr Surg 20: 529-534, 1985 ... J Pediatr 100: 399-401, 1982 -antitrypsin deficiency (Pi SZ) and biliary atresia. J Pediatr Gastroenterol Nutr 9: 256-260, 1989 J Pediatr Surg 24: 48-51, 1989 Hum Pathol 14: 512-537, 1983 Arch Dis Child 66: 121-123, 1991 ... J Pediatr Surg 25: 153-162, 1990 Other Causes of Biliary Obstruction Gastroenterology 80: 380-383, 1981 World J Surg 9: 244-249, 1985 Hepatology 4: 678-683, 1984 Ann Surg 191: 626-634, 1980 ... Idiopathic perforation of the biliary tract in infancy . J Pediatr Surg 18: 546-550, 1983. Neonatal Hepatitis Prog Liver Dis 6: 471-485, 1979 Appl Pathol 6: 49-55, 1988 J Pediatr Gastroenterol Nutr 6: 203-207, 1987 -dihydroxy- and 3 -trihydroxy-5-cholenoic acids. J Clin Invest 79: 1021-1038, 1987 Arch Dis Child 52: 360-367, 1977 Arch Dis Child 60: 447-451, 1985 Arch Dis Child 60: 512-516, 1985 ... J Pediatr Gastroenterol Nutr 5: 492-500, 1986 -3-oxosteroid 5 -reductase deficiency described in identical twins with neonatal hepatitis. A new inborn error in bile acid synthesis.

56. Answer: Six Month Old With Cholestasis Answer for Case 1 a six month old with cholestasis. The most likely diagnosisis Alagille's Syndrome which is also called arteriohepatic dysplasia. http://hsc.virginia.edu/medicine/clinical/pediatrics/CMC/cases/answer1.html

Answer for Case 1: a six month old with cholestasis The most likely diagnosis is Alagille's Syndrome which is also called arteriohepatic dysplasia Alagille's Syndrome is inherited as an autosomal dominant trait. The syndrome is characterized by paucity of intrahepatic ducts resulting in cholestatic liver disease a variety of cardiac anomalies, most typically peripheral pulmonic stenosis butterfly vertebrae an ocular anomaly termed posterior embryotoxin a characteristic facies consisting of a triangular shaped face with a pointed chin and wide and deeply set eyes. Affected patients may also be mentally retarded. The prognosis and natural history of the disease largely depend on the severity of the liver disease. Between one third and one half of affected children will develop chronic liver disease ultimately necessitating liver transplantation. Return to the Case Return to Children's Medical Center Home Page Witz@Virginia.edu 11/94

57. Indications For Liver Transplantation In Children Extrahepatic biliary atresia arteriohepatic dysplasia (Alagille syndrome) Neonatalhepatitis Primary sclerosing cholangitis Caroli's disease Lanerhan's cell http://www.classkids.org/library/livertxdx.htm

Diagnostic Indications for Hepatic Transplantation in Children Chronic cholestatic liver disease Extrahepatic biliary atresia Arteriohepatic dysplasia (Alagille syndrome) Neonatal hepatitis Primary sclerosing cholangitis Caroli's disease Lanerhan's cell histiocytosis (histiocytosis X) Inborn errors of metabolism Alpha-1-antitrypsin deficiency Tyrosinemia Wilson's disease Miscellaneous metabolic or genetic liver disease Glycogen storage disease, type 1 Glycogen storage disease, type 4 Type 1 hyperoxaluria Crigler-Najjar syndrome Ornithine transcarbamylase deficiency Familial hypercholesterolemia Protein C deficiency Niemann-Pick and other lipidosis Gaucher's disease Erythropoietic protoporphyria Hemophilia A Hemophilia B Urea cycle enzyme deficiency Hepatocellular injury Viral hepatitis Autoimmune hepatitis Drug-induced liver failure Amanita mushroom poisoning Mass occupying lesions Hepatoblastoma Hepatocellular carcinoma (hepatoma) Polycystic liver disease Hepatic adenomatosis Hemangioendothelioma Miscellaneous Cystic fibrosis Congenital hepatic fibrosis Reviewed on Children’s Liver Association for Support Services

58. A Appendicitis; @ Arrhythmia; @ arteriohepatic dysplasia; @ Arthritis;@ Arthrogryposis; @ Asbestosis; @ Asperger's Syndrome; @ Aspergillosis; http://www.ad.com/Health/Conditions_and_Diseases/A/

search Top Categories: ADD and ADHD @ AIDS Dementia Complex @ Aarskog Syndrome @ Aase Syndrome ... @ Ayerza's Syndrome AD.COM Web Directory is based on ODP - Open Directory Project data. No proprietary software was used in the development of this web site. Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor

59. Pediatric Cardiology Alagille Syndrome. This is also known as arteriohepatic dysplasia.It is a disease mainly of the liver which results in a decreased http://www.ucch.org/sections/cardio/new/alagille.html

Sections/Specialties Palos Heights Merrillville La Rabida [Return to main] Alagille Syndrome This is also known as arteriohepatic dysplasia. It is a disease mainly of the liver which results in a decreased number of tubes (ducts) which usually allow the liver to get rid of a substance known as bile. There is also progressive destruction of the bile ducts. The end result is that bile accumulates within the liver and yellowness of the skin (jaundice) results since the liver cannot get rid of a substance called bilirubin which is one of the components in bile. There are a number of features common to people with Allagile syndrome. They may have a broad forehead with deep set and widely spaced eyes. They may have an underdeveloped mandible, eye abnormalities, abnormalities of the spine, kidney abnormalities, and cardiac abnormalities. The cardiac abnormalities are usually narrowing of the pulmonary arteries (peripheral pulmonary stenosis) which may require a cardiac intervention (such as stenting and dilation). Occasionally, another defect known as

60. Alagille by. Malcolm Cheng, MD, FAAP, FRCPC. A. Classification. 1. “Syndromic”(Alagille Syndrome or arteriohepatic dysplasia). Autosomal Dominant. http://www3.sympatico.ca/malcolm.cheng2/alagille.html

Intrahepatic Biliary Hypoplasia by Malcolm Cheng , MD, FAAP, FRCPC A. Classification 1. “Syndromic” (Alagille Syndrome or Arteriohepatic Dysplasia) - Autosomal Dominant - Anomalies: - Skin: Jaundice - Facial: Triangular facies - Ocular: Posterior embryotoxon, Axenfeld’s anomaly - Cardiac: Valvular or peripheral pulmonic stenosis - Renal: Dysplastic kidneys - Growth failure - Mild mental retardation in some 2. Non-syndromic B. Laboratory Findings C. Treatments - Low cholesterol diet with MCT - Cholestyramine/Rifampin for pruritus D. Prognosis - Better for Alagille syndrome

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