Alagille syndrome natural history: Difference between revisions
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The prognosis for Alagille syndrome patients varies largely on choice of treatment. | The prognosis for Alagille syndrome patients varies largely on choice of treatment. | ||
==Complications== | ==Complications== | ||
Children with Alagille syndrome may be at risk for pathologic fractures, which manifest at an early age and in a unique distribution favoring the lower extremity long bones <ref name="pmid20453673">{{cite journal| author=Bales CB, Kamath BM, Munoz PS, Nguyen A, Piccoli DA, Spinner NB et al.| title=Pathologic lower extremity fractures in children with Alagille syndrome. | journal=J Pediatr Gastroenterol Nutr | year= 2010 | volume= 51 | issue= 1 |pages= 66-70 | pmid=20453673 | doi=10.1097/MPG.0b013e3181cb9629 | pmc=PMC2893241 | url= }} </ref>. | |||
==Prognosis== | ==Prognosis== | ||
Revision as of 15:37, 24 July 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The prognosis for Alagille syndrome patients varies largely on choice of treatment.
Complications
Children with Alagille syndrome may be at risk for pathologic fractures, which manifest at an early age and in a unique distribution favoring the lower extremity long bones [1].
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 [2].
References
- ↑ Bales CB, Kamath BM, Munoz PS, Nguyen A, Piccoli DA, Spinner NB; et al. (2010). "Pathologic lower extremity fractures in children with Alagille syndrome". J Pediatr Gastroenterol Nutr. 51 (1): 66–70. doi:10.1097/MPG.0b013e3181cb9629. PMC 2893241. PMID 20453673.
- ↑ Kamath BM, Spinner NB, Emerick KM, Chudley AE, Booth C, Piccoli DA; et al. (2004). "Vascular anomalies in Alagille syndrome: a significant cause of morbidity and mortality". Circulation. 109 (11): 1354–8. doi:10.1161/01.CIR.0000121361.01862.A4. PMID 14993126.