Alagille syndrome: Difference between revisions

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   MeshID        = D016738 |
   MeshID        = D016738 |
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{{Alagille syndrome}}
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''


{{CMG}}
{{CMG}}; '''Associate Editor-In-Chief:'''[[Sandeep Basnet| Sandeep Basnet, MBBS]], [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]


{{Editor Help}}
{{SK}} Alagille's syndrome; Alagille-Watson syndrome; arteriohepatic dysplasia (AHD); cardiovertebral syndrome; cholestasis with peripheral pulmonary stenosis; hepatic ductular hypoplasia; hepatofacioneurocardiovertebral syndrome; paucity of interlobular bile ducts; Watson-Miller syndrome


==[[Alagille Syndrome Overview|Overview]]==
==[[Alagille syndrome overview|Overview]]==


==[[Alagille Syndrome Pathophysiology|Pathophysiology]]==
==[[Alagille syndrome historical perspective|Historical Perspective]]==


==[[Alagille Syndrome Associated Conditions|Associated Conditions]]==
==[[Alagille syndrome pathophysiology|Pathophysiology]]==
==[[Alagille syndrome epidemiology and demographics|Epidemiology and Demographics]]==


==[[Alagille Syndrome Diagnosis|Diagnosis]]==
==[[Alagille syndrome risk factors|Risk Factors]]==


==[[Alagille Syndrome Treatment|Treatment]]==
==[[Alagille syndrome causes|Causes]]==


==[[Alagille Syndrome Medical Therapy|Medical Therapy]]==
==[[Alagille syndrome differential diagnosis|Differentiating Alagille syndrome from Other Diseases]]==


==Surgery==
==[[Alagille syndrome natural history|Natural History, Complications & Prognosis]]==
Corrective surgery is sometimes needed to repair heart defects associated with Allagile Syndrome. Also, because the pulmonary arteries are often narrow in Alagille patients, a catheterization process similar to [[angioplasty]] may be used to widen the arteries to reduce pressure on the heart's pumping valves. In moderate to severe cases, [[stents]] may be placed in the arteries to increase their diameter. Transplantation of the liver has been a successful alternative to medication in severe cases. However, liver transplantation from donor parents should always be preceded by genetic testing because cases have been known where donor mother was also found to have  alagille syndrome post transplant owing to the autosomal dominant inheritance pattern of the syndrome. Death from graft failure, neurological, and cardiac complications is significantly higher in patients with Alagille Syndrome than patients with Biliary Atresia <ref name="pmid20070561">{{cite journal| author=Arnon R, Annunziato R, Miloh T, Suchy F, Sakworawich A, Sogawa H et al.| title=Orthotopic liver transplantation for children with Alagille syndrome. | journal=Pediatr Transplant | year= 2010 | volume= 14 | issue= 5 | pages= 622-8 | pmid=20070561 | doi=10.1111/j.1399-3046.2009.01286.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20070561  }} </ref>.
==Diagnosis==
[[Alagille syndrome diagnostic criteria|Diagnostic Criteria]] | [[Alagille syndrome history and symptoms | History and Symptoms]] | [[Alagille syndrome physical examination | Physical Examination]] | [[Alagille syndrome laboratory findings | Laboratory Findingd]] | [[Alagille syndrome electrocardiogram |Electrocardiogram]] | [[Alagille syndrome x ray|X Ray]] | [[Alagille syndrome CT|CT]] | [[Alagille syndrome MRI|MRI]] | [[Alagille syndrome other imaging findings |Other Imaging Findings]] | [[Alagille syndrome other diagnostic studies|Other Diagnostic Studies]]


Recently, a procedure called partial biliary diversion has been used to significantly reduce pruritus, jaundice, and xanthomas caused by poor bile flow. A portion of the bile produced by the liver is directed through a surgically created [[stoma]] into a plastic pouch on the patient's lower right abdomen. The pouch is periodically drained as it fills with bile.
==Treatment==
[[Alagille syndrome medical therapy|Medical Therapy]] | [[Alagille syndrome surgery|Surgery]] | [[Alagille syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Alagille syndrome future or investigational therapies|Future or Investigational Therapies]]


The Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with Alagille Syndrome and actually appears to worsen outcome <ref name="pmid20601899">{{cite journal| author=Kaye AJ, Rand EB, Munoz PS, Spinner NB, Flake AW, Kamath BM| title=Effect of Kasai procedure on hepatic outcome in Alagille syndrome. | journal=J Pediatr Gastroenterol Nutr | year= 2010 | volume= 51 | issue= 3 | pages= 319-21 | pmid=20601899 | doi=10.1097/MPG.0b013e3181df5fd8 | pmc= | url= }} </ref>.
==Related Chapters==
 
==Prognosis==
Mortality is approximately 10%, with vascular accidents, cardiac disease, and liver disease accounting for most of the deaths. Vascular anomalies account for 34% of the mortality in this population <ref name="pmid14993126">{{cite journal| author=Kamath BM, Spinner NB, Emerick KM, Chudley AE, Booth C, Piccoli DA et al.| title=Vascular anomalies in Alagille syndrome: a significant cause of morbidity and mortality. | journal=Circulation | year= 2004 | volume= 109 | issue= 11 | pages= 1354-8 | pmid=14993126 | doi=10.1161/01.CIR.0000121361.01862.A4 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14993126  }} </ref>.
 
==See also==
*[[Progressive familial intrahepatic cholestasis]]
*[[Progressive familial intrahepatic cholestasis]]


==References==
{{Congenital malformations and deformations of digestive system}}
{{Reflist|2}}
[[fr:Syndrome d'Alagille]]
[[pl:Zespół Alagille'a]]
[[pt:Síndrome de Alagille]]


==External links ==
{{WikiDoc Help Menu}}
 
{{WikiDoc Sources}}
*[http://www.cincinnatichildrens.org/svc/alpha/l/liver/diseases/alagille-syndrome.htm Alagille Syndrome, Liver Diseases and Treatments, Cincinnati Children's Hospital Medical Center]
 
==Support groups ==
*[http://www.alagille.org/  Alagille Syndrome Alliance]
 
*[http://www.liverfamilies.org/ LIVER FAMILIES: An online support group for families whose lives have been touched by pediatric liver disease and transplant. ]
 
{{Congenital malformations and deformations of digestive system}}


[[Category:Pediatrics]]
[[Category:Pediatrics]]
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[[Category:Rare diseases]]
[[Category:Rare diseases]]
[[Category:Hepatology]]
[[Category:Hepatology]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:Up-To-Date]]
 
[[de:Alagille-Syndrom]]
[[fr:Syndrome d'Alagille]]
[[nl:Syndroom van Alagille]]
[[pl:Zespół Alagille'a]]
[[pt:Síndrome de Alagille]]
 
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

Latest revision as of 20:08, 27 August 2012

Alagille syndrome
ICD-10 Q44.7 (EUROCAT Q44.71)
ICD-9 759.89
OMIM 118450
DiseasesDB 29085
MeSH D016738

Alagille syndrome Microchapters

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Overview

Historical Perspective

Pathophysiology

Epidemiology & Demographics

Risk Factors

Causes

Differentiating Alagille syndrome from other Diseases

Natural History, Complications & Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Cost-Effectiveness of Therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Sandeep Basnet, MBBS, Prashanth Saddala M.B.B.S

Synonyms and keywords: Alagille's syndrome; Alagille-Watson syndrome; arteriohepatic dysplasia (AHD); cardiovertebral syndrome; cholestasis with peripheral pulmonary stenosis; hepatic ductular hypoplasia; hepatofacioneurocardiovertebral syndrome; paucity of interlobular bile ducts; Watson-Miller syndrome

Overview

Historical Perspective

Pathophysiology

Epidemiology and Demographics

Risk Factors

Causes

Differentiating Alagille syndrome from Other Diseases

Natural History, Complications & Prognosis

Diagnosis

Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findingd | Electrocardiogram | X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Related Chapters

Template:Congenital malformations and deformations of digestive system


Template:WikiDoc Sources

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