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         Arteriohepatic Dysplasia:     more detail

1. Alagille's Syndrome
A good, though brief (less than 1024 chars), description of your site. Psychosocial. Gastroenterology. arteriohepatic dysplasia OR ALAGILLE SYNDROME
http://tray.dermatology.uiowa.edu/Alagil01.htm
Dept. of Dermatology - University of Iowa College of Medicine
Alagille's Syndrome / Arteriohepatic Dysplasia - Right elbow - Xanthomata
For more information, see the American Liver Foundation and Online Mendelian Inheritance in Man (OMIM) Return to Image Index page. Return to Image Index page.
Return to Dermatology's Home page.
January, 1996

2. Alagille Syndrome / The Family Village
See also arteriohepatic dysplasia, WatsonAlagille Syndrome, Syndromic Hepatic Ductular Hypoplasia, Syndromic
http://www.familyvillage.wisc.edu/lib_alag.htm
Alagille Syndrome
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Who to Contact
Alagille Syndrome Alliance [ASA]
10630 SW Garden Park Place
Tigard, Oregon, USA 97223
E-mail: alagille@earthlink.net
Web: http://www.alagille.org/
See also: Arteriohepatic Dysplasia, Watson-Alagille Syndrome, Syndromic Hepatic Ductular Hypoplasia, Syndromic Intrahepatic Biliary Hypoplasia, Cholestasis with Peripheral Pulmonary Stenosis, Syndromic Bile Duct Paucity, Intrahepatic Biliary Artresia or Dysgenesis. ASA's mission is to provide information about Alagille syndrome, networking services, and a forum for exchange of experiences for families with the syndrome. They also disseminate information to all those interested in this rare liver disorder, and will refer families to others in the same geographical area. The Alliance publishes a quarterly newsletter, LiverLinks , and has a brochure and a fact sheet available that explain the syndrome and treatment that is available. ASA offers a new parent packet that includes, the newsletter, fact sheet, brochure, and an information form for referrals. ASA collects information on physicians and research being done and makes this information available to its members on a limited basis. They are in the process of developing a bibliography of articles and they have a scientific advisory board.

3. Alagille's Syndrome (www.whonamedit.com)
G. H. Watson, V. Miller arteriohepatic dysplasia. Familial pulmonary artery stenosis with neonatal liver disease.
http://www.whonamedit.com/synd.cfm/729.html

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Alagille's syndrome Also known as:
Watson-Miller syndrome
Synonyms:
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.
Associated persons: Daniel Alagille V. Miller Geoffrey H. Watson Description: 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. Bibliography:
  • G. H. Watson, V. Miller:

4. Arteriohepatic Dysplasia - Medical Dictionary Of Popular Medical Terms To Help Y
arteriohepatic dysplasia Also known as Alagille syndrome, this ia a genetic disordercharacterized by jaundice in the newborn period, liver disease with
http://www.medterms.com/script/main/Art.asp?li=MNI&ArticleKey=6754

5. Alagille (Watson) Syndrome (Arteriohepatic Dysplasia, [Hepatofacioneuro]cardiove
HOME Alagille (Watson) Syndrome (arteriohepatic dysplasia, HepatofacioneurocardiovertebralSyndrome, Watson Miller Syndrome). ARTERIOHEPATIC
http://www.bdid.com/alagille.htm

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Alagille (Watson) Syndrome (Arteriohepatic Dysplasia, [Hepatofacioneuro]cardiovertebral Syndrome, Watson Miller Syndrome)

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6. Ateriohepatic Dysplasia
arteriohepatic dysplasia. (See ALAGILLE SYNDROME)
http://childhealthinfo.com/arteriohepatic-dysplasia.htm
ARTERIOHEPATIC DYSPLASIA (See ALAGILLE SYNDROME) [Home] [Printed Guide] [Child Care for the '90's] [Links] ... MoreInfo@ChildHealthInfo.com

7. Birth Defects - Syndromes - Malformations - Anomalies
Arnold Chiary. Arsenic. arteriohepatic dysplasia. Arthrogryposis. Arthrogryposis Multiplex Congenita
http://www.ibis-birthdefects.org/start/drophealth.htm
Tips for printing Birth Defects - Syndromes - Malformations - Anomalies
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  • By making a selection you agree to the terms of our This page Includes: The words "Syndrome", "Dysplasia", "Dystrophy" are avoided.
Names - Eponyms A B C D ... Search this site powered by FreeFind Requests for additional topics
  • 4p Monosomy 4p- Deletion 5p Monosomy 5p- Kromosom ... Search this site powered by FreeFind Requests for additional topics This site offers information mostly for educational purposes. This site is not intended to alter health care protocols nor to serve as a sole source of medical information. Please read full Always seek the advice of your local health care provider. Sponsor: I.B.I.S. Site Concept: W. W., D. C. Page Content: W. Wertelecki, M. D. Email: Webmaster 07/August/2002 R.C.

8. Gastroenterology - Alagille Syndrome
Gastroenterology. arteriohepatic dysplasia OR ALAGILLE SYNDROME. Alagillefirst described a syndrome consisting of a paucity of interlobular
http://www.mc.vanderbilt.edu/peds/pidl/gi/alagille.htm

PIDL Home/ Contents
Development Nutrition Acute Illness ... Psychosocial
Gastroenterology
ARTERIOHEPATIC DYSPLASIA OR ALAGILLE SYNDROME Alagille first described a syndrome consisting of a paucity of interlobular bile ducts with chronic cholestasis. Patients with this syndrome typically have the following major features: peculiar facies, chronic cholestasis, butterfly- like vertebral arch defects, and peripheral pulmonary artery hypoplasia or stenosis, either isolated or associated with complex cardiovascular abnormalities. Other less frequent features include growth retardation, mental retardation, renal disturbances, other vascular malformations, high- pitched voice, and delayed puberty with hypogonadism. Patients typically present with conjugated hyperbilirubinemia from chronic cholestasis within the first 3 months of life. Hepatomegaly and splenomegaly are almost invariably present. A remarkable feature is the extreme degree of pruritus. The splenomegaly may actually precede the development of portal hypertension. Xanthomas are commonly seen on the extensor surfaces of the fingers, palmar creases, nape of the neck, anal folds, popliteal fossa, and inguinal area. There is marked elevation of cholesterol, phospholipid, and triglyceride levels. Typically liver enzymes are found to be normal.

9. Alagille Syndrome(arteriohepatic Dysplasia)
Alagille syndrome(arteriohepatic dysplasia). the the most common syndrome incorporatingintrahepatic bile duct paucity. progressive destruction of bile ducts.
http://www.vghtc.gov.tw:8082/ped/teaching/inout/alagille syndrome/tsld002.htm

10. Alagille's Syndrome - 3
Dacri, dacrio-, dacria / Dacry-, dacryo-, dacry Componentes iniciales y final de las voces castellanas que denotan relación con las lágrimas. Dacriadenalgia / Dacryadenalgia Dacriadenectomía / Dacryadenectomy Dacriadenitis / Dacryadenitis
http://tray.dermatology.uiowa.edu/Alagil03.htm
Dept. of Dermatology - University of Iowa College of Medicine
Alagille's Syndrome / Arteriohepatic Dysplasia - Palmar Hand - Xanthomata
For more information, see the American Liver Foundation and Online Mendelian Inheritance in Man (OMIM) Return to Image Index page. Return to Image Index page.
Return to Dermatology's Home page.
January, 1996

11. Alagille Syndrome(arteriohepatic Dysplasia)
The summary for this Chinese (Traditional) page contains characters that cannot be correctly displayed in this language/character set.
http://www.vghtc.gov.tw:8082/ped/teaching/inout/alagille syndrome/sld002.htm

12. University Of Miami School Of Medicine - Glossary - Arteriohepatic Dysplasia
Medical Dictionary. arteriohepatic dysplasia. arteriohepatic dysplasiaAlso known as Alagille syndrome, this ia a genetic disorder
http://www.med.miami.edu/patients/glossary/art.asp?articlekey=6754

13. Chromosome Entry Map Table For Chromosomes
20.18. Alagille syndrome (arteriohepatic dysplasia). 118450. Alagille syndrome (2)
http://www.ibmpcug.org/ksxxy/gene20.htm
Chromosome Entry Maps with Pix. Known Disease Entitys Entry Map # Title MIM # Disorders Bone morphogenetic protein-2 ?Fibrodysplasia ossificans progressiva (1) Phosphoenolpyruvate carboxykinase-1 (soluble) ?Hypoglycemia due to PCK1 deficiency (1) Growth hormone releasing factor; somatocrinin ?Isolated growth hormone deficiency , Gigantism due to GHRF hypersecretion (1) Inosine triphosphatase-A [Inosine triphosphatase deficiency] (1) Alagille syndrome (arteriohepatic dysplasia) Alagille syndrome (2) Cystatin C Cerebral amyloid angiopathy (3) Congenital hereditary endothelial dystrophy of cornea Congenital hereditary endothelial dystrophy of cornea Posterior polymorphous corneal dystrophy Corneal dystrophy, posterior polymorphous (2) Prion protein (p27-30) Creutzfeldt-Jakob disease, 123400 (3); Insomnia, fatal familial (3) Arginine vasopressin (neurophysin II, antidiuretic hormone) Diabetes insipidus, neurohypophyseal, 125700 (3) Cholinergic receptor, nicotinic, alpha polypeptide-4 Epilepsy, benign neonatal, type 1, (3); Epilepsy, frontal lobe Protective protein for beta-galactosidase (cathepsin A) Galactosialidosis (3) Glutathione synthetase Hemolytic anemia due to glutathione synthetase Transcription factor 14, hepatic nuclear factor (HNF4)

14. University Of Miami School Of Medicine - Glossary - Alagille Syndrome
Alagille syndrome Also known as arteriohepatic dysplasia, this is a genetic disordercharacterized by jaundice in the newborn period, liver disease with
http://www.med.miami.edu/patients/glossary/art.asp?articlekey=6752

15. Wipro GE Medical
Biliary atresia. c. arteriohepatic dysplasia (Alagille's syndrome). d.
http://www.wipro-ge.com/ultrasound/quiz/quiz_archive_blk2q3.html
UltraGEnius Quiz - Block 2 Quiz #3
Dr. Swaroop Shanbhag, Dr. Bhavin Jankharia
Dept of MRI, Dr. Jankharia's Imaging Centre, Mumbai, India CASE STUDY
A 15-years-old girl presented with pain in the abdomen and vomiting. She had a history of recurrent episodes of pain in the right upper quadrant. Fig.1
USG of the right hypochondrium Fig.2
USG of the right hypochondrium Fig.3
USG of the right hypochondrium 1. What is the complete diagnosis?
Answer: Type 1 choledochal cyst with calculi in the distal CBD 2. Embryologically, the first structure to develop is the. a. Cystic duct
b. Common bile duct
c. Pancreatic duct
d. Gall bladder Answer: Common bile duct 3. Paucity of bile ducts associated with renal tubular acidosis is seen in
a. Caroli's disease

16. Arteriohepatic Dysplasia - General Practice Notebook
medical information from General Practice Notebook. arteriohepatic dysplasia. Alagillesyndrome is defined clinically as the combination of biliary hypoplasia;
http://www.gpnotebook.co.uk/cache/-644218858.htm
arteriohepatic dysplasia Alagille syndrome is defined clinically as the combination of:
  • biliary hypoplasia cardiovascular abnormalities vertebral abnormalities characteristic facies ocular abnormalities
The degree of biliary hypoplasia is variable. Some patients have severe cholestasis, progress to cirrhosis and require liver transplantation.
Click here for more information...

17. Sitemap Of GPnotebook.co.uk
cell crises of the lung / hypostatis dermatitis / vertebrosternal ribs / monocyticleukaemia / white pimples / arteriohepatic dysplasia / pigmentation(linear
http://www.gpnotebook.co.uk/sitemap17.htm
This is the GPnotebook.co.uk sitemap, click here to go to the homepage. drugs causing hypothyroidism esophageal perforation pregnancy (AIDS in) prolapse(rectal) ... adolescent thoracic scoliosis

18. Abnormalities, Multiple
ALAGILLE SYNDROME; AGS; Alagille Syndrome Amer. Liver Found. Alagille's Syndrome/ arteriohepatic dysplasia Back (Date 10/1980) - Xanthomata U. of Iowa;
http://www.ohsu.edu/cliniweb/C16/C16.131.77.html
Abnormalities, Multiple
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19. Ateriohepatic Dysplasia
arteriohepatic dysplasia. (See ALAGILLE SYNDROME). Home HotLines ResourcesPrinted Guide Child Care for the '90's Links. © 1997 ABC F Press.
http://www.childhealthinfo.com/arteriohepatic-dysplasia.htm
ARTERIOHEPATIC DYSPLASIA (See ALAGILLE SYNDROME) [Home] [Printed Guide] [Child Care for the '90's] [Links] ... MoreInfo@ChildHealthInfo.com

20. A Index
ARNOLDCHIARI MALFORMATION. ARNOLD-CHIARI SYNDROME. arteriohepatic dysplasia. ARTERIOVENOUSMALFORMATION (AVMS). ARTHO-OPHTHALMOPATHY. ARTHRITIS. ARTHROGRYPOSIS.
http://www.childhealthinfo.com/a/
A B C D ... Z Just click on the item you want to learn more about.
A
A-BETA-LIPOPROTEINEMIA AARSKOG SYNDROME ABDOMINAL MIGRAINE ABDUCTION/MISSING CHILDREN ... MoreInfo@ChildHealthInfo.com

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