81. INABIS '98 - The Coloboma Mouse Mutation As An Animal Model Of ADHD 1 on mouse chromosome 2. Interestingly, the semidominant herited human disorder Alagille'ssyndrome, characterized by arteriohepatic dysplasia, cardiac, ocular http://www.mcmaster.ca/inabis98/sadile/wilson0247/two.html

Invited Symposium: What Can Genetic Models Tell Us About Attention-Deficit Hyperactivity Disorder (ADHD)? Abstract Introduction Results References ... New Search The Coloboma Mouse Mutation as an Animal Model of ADHD Contact Person: Michael c. Wilson ( mwilson@salud.unm.edu Introduction Back to the top. Results The Coloboma Mutant Mouse Coloboma mutant mice display a variety of behavioral, neurophysiological and developmental deficits, of which a subset may be compared with those presented by ADHD children. Although the Cm mutation is early embryonic lethal when homozygous (9), adult heterozygote mice (genotype, Cm The coloboma mutation is a chromosomal deletion that arose from neutron irradiation mutagenesis. Although neutron irradiation generally causes substantial chromosomal deletion and rearrangement, no chromosomal abnormality can be distinguished for the Cm locus at the cytological level (14). Initial mapping experiments placed the Cm locus on mouse chromosome 2 (15). Our more recent studies evaluating the inclusion of genetically mapped polymorphic simple sequence repeats and local gene sequences in an interspecies cross showed that the deletion spans 1-2 cM, comparable to 1-2 x 106 base pairs, towards the distal end of Ch2 (16). While a deletion of this sized segment of genomic DNA may comprise more than 30 genes, currently only four known genes (encoding phospholipase beta 1, and 4, jagged and SNAP-25 proteins) have been identified within the mutation (16, and M.C.W. unpublished data). The suggestion that a significant number of genes may be affected by the

82. UTL. About The Libraries. AV septal defect, see atrioventricular septal defect. AV valve, see atrio-ventricularvalve. arteriohepatic dysplasia, syn. Alagille syndrome. http://www.achd-library.com/nevil_thomas/consensus/app_6.htm

UTL Home Library Directory General Information Resources ... Site Guide Object not found! The requested URL was not found on this server. If you entered the URL manually please check your spelling and try again. If you think this is a server error, please contact marc.lalonde@utoronto.ca Error 404 General Information Resources Services Site Guide ... Home This page was last updated January 15, 2003. Your comments and questions are welcomed.

83. Diseases Database Disease, Symptom, Sign, Etc Alphabetical Index : A Diseases Da Artemether Artemisine see Artemisinin Artemisinin Arteriovenous malformation seeArteriovenous malformation arteriohepatic dysplasia see Alagille syndrome http://www.diseasesdatabase.com/sieve/disease_index_a.asp

Diseases Database [Previous page] [Search] [Index] [Feedback] Diseases Database disease, symptom, sign, etc alphabetical index : A AA amyloidosis Aagenaes syndrome see Cholestasis-oedema syndrome, Norwegian type Aarskog Ose Pande syndrome see SHORT syndrome Aarskog syndrome Aarskog-Scott syndrome see Aarskog syndrome Aase syndrome Abacavir Abciximab Abdo distended see Abdominal distention Abdo mass see Abdominal mass Abdo pain see Abdominal pain Abdomen distended see Abdominal distention Abdominal and pelvic pain see Abdominal pain Abdominal decompression Abdominal distention Abdominal mass Abdominal obesity-metabolic syndrome see Reaven syndrome X Abdominal pain Abdominal swelling see Abdominal distention Abducens nerve lesion see 6th cranial nerve disorder Abducent nerve pathology see 6th cranial nerve disorder Abetalipoproteinaemia Abetalipoproteinemia see Abetalipoproteinaemia Abnormal heart rhythm see Cardiac arrhythmia Abnormal heart sounds Abnormal reflexes Abnormal splitting of heart sounds ... Abnormal sputum ABO incompatibility (feto-maternal) see ABO isoimmunization of fetus and newborn ABO isoimmunization of fetus and newborn Abortion, missed

84. Alagille Syndrome Today Riely CA, Cotlier E, Jensen PS, Klatskin G. arteriohepatic dysplasia a benignsyndrome of intrahepatic cholestasis with multiple organ involvement. http://collection.nlc-bnc.ca/100/201/300/cdn_medical_association/cim/vol-19/0325

Alagille syndrome today 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 Contents Abstract Introduction Clinical and laboratory findings Other manifestations ... References Abstract 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 Introduction 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]

85. Birth Disorder Information Directory - A Al gazali sabrinathan nair syndrome. Alagille (Watson) Syndrome (ArteriohepaticDysplasia, Hepatofacioneurocardiovertebral Syndrome, Watson Miller Syndrome) http://www.bdid.com/defectag.htm

HOME Ag-Am Aganglionosis Total Intestinal Aganglionosis total intestinal AGANGLIONOSIS, TOTAL INTESTINAL Aganthia with Holoprosencephaly and Situs Inversus Aganthia holoprosencephaly situs inversus AGNATHIA-HOLOPROSENCEPHALY Agammaglobulinemia List of Sites Agenesis of the Cerebellar Vermis List of Sites Agenesis of the Corpus Callosum List of Sites Related Books Aglossia Adactylia (Oromandibular Limb Hypoplasia) Aglossia adactylia AGLOSSIA-ADACTYLIA Also see Hanhart Syndrome Agnathia -Holoprosencephaly See Holoprosencephaly-Agnathia Agyria-Pachygyria Type I See Miller-Dieker (Lissencephaly) Syndrome Aicardi Syndrome List of Sites Aicardi-Goutieres Syndrome (Encephalopathy-Basal Ganglia Calcification) Aicardi-Goutieres syndrome Encephalopathy, Familial Infantile, with Calcification of Basal Ganglia and Chronic Cerebrospinal Fluid Lymphocytosis Related Books Cutis verticis gyrata thyroid aplasia mental retardation ... CUTIS VERTICIS GYRATA, THYROID APLASIA, AND MENTAL RETARDATION Aksu Stockhausen Syndrome (Branchial Arch Defects) branchial arch defects Al-Awadi Farag Teebi Syndrome (Primary Hypogonadism with Partial Alopecia) Al awadi farag teebi syndrome HYPOGONADISM, PRIMARY, AND PARTIAL ALOPECIA

86. Alagille Syndrome New Register, Dr Ira Shah MD, DCH(Gold Medalist), FCPS, DNB Key words Arteriohepaticdysplasia , chronic cholestasis, pulmonary artery hypoplasia. http://www.pediatriconcall.com/fordoctor/CaseReports/alagille_syndrome.asp

Question of day Alternative Medicine Journals Queries ... Shopping Mall ALAGILLE SYNDROME UserName Password New Register Dr Ira Shah M.D, DCH(Gold Medalist), FCPS, DNB Key words:- Arteriohepatic dysplasia , chronic cholestasis, pulmonary artery hypoplasia Introduction: Case :- 18 month old male child BONCM to normal parents presented with jaundice since 2 months of age, clay colored stools with high colored urine, and pruritis since l year. Patient was noticed to have cyanosis, which increased, on crying. He had dysmorphic features in form of deep-seated eyes, with prominent forehead and small pointed chin. Patient had hepatomegaly with 5cm palpable liver. He also had an ejection systolic murmur with soft and single second heart sound suggestive of tetralogy of fallot physiology. Patient was clinically diagnosed as a case of Arteriohepatic dysplasia or Alagille syndrome . 2 D echo showed subvalvalar pulmonary stenosis with left pulmonary artery not seen. USG Abdomen was suggestive of gall bladder sludge with hepatomegaly. His liver enzymes were elevated. There was hyperbilirubinemia with increased direct compound (2.4mg% D= 1.2 mg%). Serum cholesterol (316 mg%) and Triglyceride (352 mg%) were markedly elevated. Liver scan (HIDA) showed decreased excretion. Patient was advised cardiac catheterisation to assess the anatomical malformation and evaluate further treatment. Liver biopsy was not undertaken in this patient. He was treated with fat soluble vitamins, cholestyramine, ureodeoxycholic acid. His pruritis markedly subsided with above management. He was started on T. Propranalol for his cyanotic heart disease and discharged. He was advised surgery for heart disease and option for liver transplantation was explained.

87. ORPHANET® : Base De Données Sur Les Maladies Rares Et Les Médicaments Orpheli Translate this page Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepaticdysplasia) Review of 80 cases. J Pediatr, 1987, 110 195-200. http://orphanet.infobiogen.fr/data/patho/FR/fr-alagille.html

Le syndrome d'Alagille Auteur : Docteur Michelle Hadchouel Editeur scientifique : Professeur Serge Erlinger Nom de la maladie et ses synonymes Nom des maladies exclues Incidence Description clinique ... Etiologie Nom de la maladie et ses synonymes Syndrome d’Alagille, AGS, Nom des maladies exclues Jagged Incidence Environ 1/100 000 naissances. Description clinique Manifestations cardiaques Anomalies oculaires Mode de prise en charge et traitement Etiologie Alagille D, Estrada A, Hadchouel M, et al. Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): Review of 80 cases. J Pediatr, 1987, 110 : 195-200. Anad F, Burn J, Matthews D, Cross I, et al. Alagille syndrome and deletion of 20p. J Med Genet, 1990, 27 : 729-37. Cardona J, Houssin D, Gauthier F et al. Liver transplantation in children with Alagille syndrome - a study of twelve cases. Transplantation 1995, 60 : Crosnier C, Lykavieris P, Meunier-Rotival M, Hadchouel M. Alagille syndrome. The widening spectrum of arteriohepatic dysplasia. Clin Liver Dis. 2000, 4:765-78. Hadchouel M. Paucity of interlobular bile ducts. Semin Diagn Pathol, 1992, 9 : 24-3.

88. Radiology In Ped Emerg Med, Vol 6, Case 5 What bony abnormality is present? Teaching Points 1) Alagille syndrome (arteriohepaticdysplasia) is characterized by a paucity of intrahepatic bile ducts. 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

89. Alagilles Syndrom - Små Och Mindre Kända Handikappgrupper Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepaticdysplasia) review of 80 cases. J Pediatr 1987; 110 195200. http://www.sos.se/smkh/1998-29-079/1998-29-079.HTM

Socialstyrelsen 106 30 Stockholm e-post Socialstyrelsen klassificerar sin utgivning i olika dokumenttyper Alagilles syndrom Arteriohepatisk dysplasi, Monosomi 20p12-syndromet Sjukdom/skada/diagnos Orsak till sjukdomen/skadan Symtom Diagnostik ... Databasreferenser Dokumentdatum: 2002-06-03 Version: 2.0 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. Sjukdom/skada/diagnos Orsak till sjukdomen/skadan Alagilles syndrom orsakas vanligtvis av en nymutation Symtom Diagnostik Praktiska tips Resurspersoner Professor Antal Nemeth, Barnmedicinska kliniken, Huddinge Universitetssjukhus, 141 86 Stockholm, tel 08-585 800 00.

90. Zangger Halvorsen RA Jr; Garrity S; Kuni C; du Cret RP; Letourneau JG; Bloomer J Arteriohepaticdysplasia (Alagille's syndrome) unusual hepatic architecture and http://radiologie-dijon.chez.tiscali.fr/Biblio/Halvorsen.htm

Halvorsen RA Jr; Garrity S; Kuni C; du Cret RP; Letourneau JG; Bloomer J Arteriohepatic dysplasia (Alagille's syndrome): unusual hepatic architecture and function. Abdom Imaging 1995 May-Jun;20(3):191-6 Texte Bibliographie

91. Privattandvård I Örebro, Tandläkaren Michel Deaibes, Sjukdomslista På Engels Aphasia. Aplastic Anemia. Apnea, Sleep. Appendicitis. Arrhythmia. ArteriohepaticDysplasia. Arthritis. Arthrogryposis. Asbestosis. Asperger's Syndrome. Aspergillosis. http://www26.brinkster.com/privtand/sjuk.htm

A Aarskog Syndrome Aase Syndrome Abetalipoproteinemia Ablepharon-Macrostomia Syndrome Achilles Tendonitis Achondroplasia Acoustic Neuroma Acromegaly Activated Protein C Resistance Acute Idiopathic Polyneuritis ADD and ADHD Addiction and Recovery Addison's Disease Adiposis Dolorosa Adjustment Disorders Adrenoleukodystrophy Agnosia Agoraphobia Aicardi Syndrome AIDS Alagille Syndrome Albinism Alcoholism Alexander Disease Alkaptonuria Allergies Alopecia Alpers' Disease Alpha1 Antitrypsin Deficiency Alport Syndrome Alstrom Syndrome Alternating Hemiplegia Altophobia Alzheimer's Amblyopia Amputee Amyloidosis Amyoplasia Congenita Amyotrophic Lateral Sclerosis Anal Fissures Anemia Anencephaly Aneurysm Angina Pectoris Anophthalmos Anorexia Anosmia Anterior Knee Pain Syndrome Antiphospholipid Syndrome Anxiety Aortic Valve Disease Apert Syndrome Aphasia Aplastic Anemia Apnea, Sleep Appendicitis Arrhythmia Arteriohepatic Dysplasia Arthritis Arthrogryposis Asbestosis Asperger's Syndrome Aspergillosis Asthma Atherosclerosis Athlete's Foot Atrial Fibrillation Attachment Disorder Attention Deficit Disorder Autism Auto Immune Disorders Aviophobia Aviatophobia B Bacillary Angiomatosis Back Disorders Bad Breath Balanitis Baldness Barth Syndrome Bassen Kornzweig Syndrome Batten Disease Beckwith-Wiedemann Syndrome Behcet's Syndrome Bell's Palsy Benign Breast Lumps Benign Prostatic Hyperplasia Berger's Disease Beriberi Beryllium Disease Besnier Boeck Disease Betalipoprotein Deficiency Disease

92. MUMS List Of Disorders - A Syndrome (1); ArnoldChiari Syndrome w/Spina Bifida (22) *; ArteriohepaticDysplasia (AHD) (Alagille Syndrome) (12) *; Arteriosclerosis (3 http://www.netnet.net/mums/mum_a.htm

Return to MUMS Home Page MUMS: List of Disorders A Number in parentheses indicates number of matches. indicates there is a support group which covers that diagnosis. 2 Hydroxic Gluteric Urea (1) 2 Keto Adepic Aciduria (1) 3 Hydroxy 3-Methylglutaryl CoAlyase Deficiency (HMG) (1) * 3 Methycrotonyl CoA Carboxylase Deficiency (3MCCC) (1) 3 Methylglutaconic Aciduria (4) 4 Hydroxybutyric Aciduria (1) 5 Alpha Reductase Deficiency (1) * 5 Oxoprolinuria (Pyroglutamic Aciduria) (1) 18 Hydroxylase Deficiency (Adrenal Hyperplasia) (1) 21 Hydroxylase Deficiency (Adrenal Hyperplasia) (18) ** ACTH Deficiency (1) AIDS (Acquired Immune Deficiency Syndrome) (6) ** Aarskog Syndrome (8) ** Abdominal Chylous Ascites (abdomen fills w/fluid) (4) Abdominal Migraines (2) Abdominal Teratoma Tumor (5) Abetalipoproteinemia (4) * Abetalipoproteinemia (Bassen-Kornzweig Syndrome) (1) * Absence of Arm Below Elbow, Congenital (25) * Absence of Arms (Severed in accident) (2) * Absence of Arms, Hands attached to shoulder (1) * Absence of Extensor Tendons in hands - bilateral (1) * Absence of Feet and Hands, Congenital (2) *

93. HealthCentral.com HealthCentral, SEARCH FOR TOPIC CENTERS Choose a topic. http://www.healthcentral.com/library/library.cfm

WELCOME! Receive Free Newsletters Join a Community SEARCH FOR: TOPIC CENTERS Choose a topic Acupuncture/Chinese Medicine Alcohol Allergies Alzheimer's Andro Anemia Arthritis Asthma Baby and Toddler Health Back Care Bodywork and Mind/Body Breast Cancer Caffeine Cancer Overview Caregiving Chemotherapy Children's Health Cholesterol Circumcision Colds and Flus Colon Cancer Computer-Related Disorders Cosmetic Surgery Death and Dying Dental Depression Diabetes Diet Drugs Digestion Disabilities Divorce Drugs and Medications Eating Disorders Environmental/Occupational Erectile Dysfunction Eye and Vision Fitness Food Safety Genetic Research Getting the Most from Your Provider Headaches and Migraines Health Information Online Health Insurance Hearing Heart and Circulation Hepatitis Herbs Herpes High Blood Pressure HIV and AIDS HMO's and Managed Care Home Remedies Hyperactivity and ADD Immunizations/Vaccines Impotence Infertility Internet Hoax Watch Legislation and Healthcare Policy Leukemia Lung Cancer Medical Breakthroughs Medical Expenses Medical Humor Medical Marijuana Men's Health Menopause Mental Health Multiple Sclerosis Nutraceuticals Nutrition and Healthy Eating Osteoporosis Other Alternative Practices Pain Management Parenting Parkinson's Pet Health Pregnancy and Childbirth Prostate Cancer Prostate Disorders Reduce Stress Safety and First Aid Senior Health Sex and Relationships Sexual Health Skin and Hair Sleep Disorders Smoking Spirituality St. John's Wort

94. Society For Pediatric Pathology -- Perspectives In Pediatric Pathology Vol. 14, Ruebner. Chapter 8. Paucity of interlobular bile ducts, arteriohepaticdysplasia and nonsyndromic duct paucity 168216, Ellen Kahn http://www.spponline.org/ppp/v14.htm

Perspectives in Pediatric Pathology Volume 14 1991 Foreword: Biliary atresia: Extrahepatic? Intrahepatic? Both? Neither? vii-x, Beverly B. Dahms Chapter 1. Founders of Pediatric Pathology: James Blanding Arey, M.D., Ph.D. Marie Valdes-Dapena Chapter 2. Basic Immunobiology of transplantation Ettenger R and Ferstenberg LB Chapter 3. The role of the clinical laboratory in transplantation Arthur W. Bracey and Susan B Conley Chapter 4. Pediatric cardiac transplantation Suzanne R. Taylor, Eduardo J. Yunis, and F. Jay Fricker Chapter 5. Pancreas transplantation for the treatment of diabetes James A. Schulak and James T. Mayes Chapter 6. Considerations of some architectural properties of the human biliary tree and liver Benjamin H. Landing and Theadis R. Wells Chapter 7. Normal and abnormal development of human intrahepatic bile ducts: An immunohistochemical perspective. Tikoes A Blankenberg, Judi Lund, and Boris H. Ruebner Chapter 8. Paucity of interlobular bile ducts, arteriohepatic dysplasia and nonsyndromic duct paucity

95. Patho hypertension; Renal disease in massive obesity; Alagill syndrome (arteriohepaticdysplasia). Hereditary Nephropathies Alport syndrome; http://www.hosp.go.jp/~sakura2/jinnet/jinneph/patho.html

Œ´•¶i1995 Churgj ˜a–ó Primary Glomerular Diseases (Glomerulonephritis and Related Conditions) Minor glomerular abnormalities Focal/segmental lesions (with only minor abnormalities in other glomeruli), including focal glomerulonephritis Diffuse glomerulonephritis Membranous glomerulonephritis (membranous nephropathy) Proliferative glomerulonephritis Mesangial proliferative glomerulonephritis Endocapillary proliferative glomerulonephritis Mesangiocapillary glomerulonephritis (membaranoproliferative glomerulonephritis type1 and 3) Crescentic (extracapillary) and necrotizing glomerulonephritis Sclerosing glomerulonephritis Unclassified glomerulonephritis Glomerulonephritis of Systemic Diseases Lupus nephritis IgA nephropathy (Berger disease) Nephritis of Henoch-Schonlein purpura (anaphylactoid purpura) Anti-basement glomerulonephritis (Goodpasture's syndrome) Glomerular lesions in systemic infections Septicemia Infective endocarditis Shunt nephritis Syphilis Human immunodeficiency syndrome Hepatitis B and C Chlamydia Rickettsiae Parasitic nephropaties Malarial nephropathy (falciparum malaria, quartan malaria)

96. JAUNDICE - NEONATAL 1. Intrahepatic Cholestasis. 1. Persistent Alagille Syndrome (arteriohepaticdysplasia); Benign Recurrent Intrahepatic Cholestasis; http://www.icondata.com/health/pedbase/files/JAUNDICE.HTM

Pediatric Database (PEDBASE) Discipline: APP Last Updated: 5/18/94 JAUNDICE - NEONATAL DEFINITION: A disorder caused by an excess of unconjugated or conjugated bilirubin in the newborn period. EPIDEMIOLOGY: incidence: ? age of onset: newborn period risk factors: see differential diagnosis DIFFERENTIAL DIAGNOSIS FOR UNCONJUGATED HYPERBILIRUBINEMIA: 1. Increased Production 1. Hemolytic Diseases Intrinsic Membrane Enzyme Hemoglobin Synthesis Extrinsic Immune Non-immune 2. Infection Sepsis TORCH infections 3. Extravesated bruising cephalohematoma delayed cord clamping transfusion (twin-twin, maternal-fetal) 4. Enterohepatic Circulation Increase delayed feeding delayed stooling dehydration GI obstruction 5. Polycythemia 2. Decreased Conjugation 1. Crigler-Najjar Syndrome - I 2. Gilbert Syndrome 3. Lucy-Driscoll Syndrome 4. Breast Milk Jaundice DIFFERENTIAL DIAGNOSIS OF CONJUGATED HYPERBILIRUBINEMIA: 1. Intrahepatic Cholestasis 1. Persistent Alagille Syndrome (arteriohepatic dysplasia) Benign Recurrent Intrahepatic Cholestasis Byler Disease Cholestasis - Lymphedema Neonatal Hepatitis NSP of Interlobular Bile Ducts 2. Acquired

97. Untitled Document The summary for this Japanese page contains characters that cannot be correctly displayed in this language/character set. http://radiol-03.hospital.okayama-u.ac.jp/case/NMED/13/ans13.htm

ÅIf’fF @‘å“®–¬•Ùã•”‹·ó(supravalvular AS)‚ÉŠ¥“®–¬‹·ó‚ð‡•¹‚µ‚½Alagille syndrome ‚Ì1—á lŽ@F ‹ž“s‘åEŠj@‹v•Û Ž l, ‘½“c‘º ‰h“ñ, ¬¼ ~“ñ

98. New Page 0 a. Paucity of interlobular bile ducts. (1) Syndromic. Alagille syndrome (arteriohepaticdysplasia) consists of hypoplastic intrahepatic bile ducts, chron. http://www.bol.ucla.edu/~ofattal/neonatalcholestasis.htm

NEONATAL CHOLESTASIS Home CHOLESTASIS (REDUCTION IN BILE FLOW) A. General considerations. 1. Cholestasis results from extrahepatic obstruction or hepatocellular injury, either primary or secondary to many infectious, or metabolic and toxic causes. 2. The newborn is particularly susceptible because of immature hepatobiliary function, with decrease in bile acid pool size, rate of synthesis, intraluminal concentration, and ileal uptake. Consequently, intraluminal fat digestion is impaired, and cholestatic effects of various endogenous and exogenous substances are enhanced. 3. Conjugated hyperbilirubinemia is always a problem and is accompanied by elevation of bile salts and phospholipids, indicating cholestasis. Early recognition allows prompt diagnosis and effective therapy. B. Clinical syndromes (Box 19-3). Neonatal cholestasis occurs in 1:2500 births, with extrahepatic obstruction accounting for half of the cases. Neonatal hepatitis, biliary atresia and at-antitrypsin deficiency are the three most common causes, with an approximate incidence of 1:5000, 1:10,000, and 1:20,000, respectively. 1. Extrahepatic biliary disease.

99. Full Time Faculty Pulido LaBrecque DR. Ophthalmic features of Alagille syndrome (arteriohepaticdysplasia). J Pediatr Ophthalmol Strabismus 30130135, 1993. http://www.uic.edu/com/eye/department/facultypages/Full Time Faculty/Pulido.html

FULL TIME FACULTY Jose S. Pulido, MS, MD Professor and Head Ophthalmology and Visual Sciences Service Email : josepuli@uic.edu Phone: 312-996-6590 Fax: 312-996-7770 US Mail: University of Illinois at Chicago. Department of Ophthalmology and Visual Sciences 1855 W. Taylor Street M/C 648 Chicago, IL 60612 Click here to view Dr. Pulido's complete CV in Adobe Acrobat PDF Education / Employment Appointments Professional Experience ... Posters and Presentations EDUCATION / EMPLOYMENT BA University of Chicago, Chicago, IL (in 3 yrs; with Honors in the Department and College) MS University of Chicago, Chicago, IL MD Tulane University School of Medicine, New Orleans, LA MBA The University of Iowa, Iowa City, IA back to top Intern, Internal Medicine Tulane Affiliated Hospitals Charity Hospital, New Orleans, LA

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