Alagille syndrome natural history
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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]. In alagille syndrome, the bile may builds up in the liver and causes scarring that prevents the liver from working properly to eliminate wastes from the bloodstream.
Prognosis
The outlook for people with Alagille syndrome depends on several factors, including the severity of liver damage and heart problems and the early correction of malabsorption. Predicting who will experience improved bile flow and who will progress to end-stage liver failure is difficult. Fifteen percent of people with Alagille syndrome will eventually require a liver transplant.
Survival rates for people receiving liver transplants have improved over the past several years because of newer drugs that suppress the immune system and keep it from attacking and damaging the new liver.
Research studies report that 75 percent of children diagnosed with Alagille syndrome live to at least 20 years of age.[2] Because of improvements in liver and heart therapies, this survival rate is increasing. Many adults with Alagille syndrome who improve with treatment lead normal, productive lives. Deaths in people with Alagille syndrome are most often caused by liver failure, heart problems, and blood vessel abnormalities.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 [3].
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.
- ↑ Emerick KM, Rand EB, Goldmuntz E, Krantz ID, Spinner NB, Piccoli DA. Features of Alagille syndrome in 92 patients: frequency and relation to prognosis. Hepatology. 1999;29(3):822–829.
- ↑ 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.