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  • Title: [Bile duct atresia: outline for a solution].
    Author: Broto J, Asensio M, Gil Vernet JM, Marhuenda C, Boix Ochoa J.
    Journal: Cir Pediatr; 2000 Jul; 13(3):106-9. PubMed ID: 12601939.
    Abstract:
    UNLABELLED: Biliary atresia continues to be a serious and relatively rare disease (1/50,000 newborns) and whose long-term prognosis has changed drastically since the appearance of liver transplant (LT) as a therapeutic weapon. The combination of two factors, early diagnosis and correct application of Kasai's surgical technique, is essential to obtain acceptable results and sufficient biliary drainage allowing the children to overcome the critical 7 kg barrier and place them in the lesser morbi-mortality range in relation to a possible LT. But we must keep in mind that despite its critics, Kasai's technique can guarantee, both in our own experience and in the literature, ten years survival percentages over 50% with correct hepatic function, as well as clinical normality and a quality of life clearly superior to first years post-LT. We present the evolution of a group of 20 patients affected with biliary atresia, diagnosed in our center since 1985, the year when pediatric LT began to be used as a therapeutic procedure in this country. We valued the age of intervention, technique, immediate and long-term results and the evolution and necessity of LT. All 20 patients were analyzed individually, and they currently have an age range from 2-14 years and were all operated by Kasai's technique. We classified the patients as having good, regular or poor results with regards to biliary flow, normalization of billirubin levels and clinical evolution. Sixteen patients presented biliary flow of such an extent that 14 of them, classified as good, completely normalized the billirubin levels. Two others, presently aged 14 and 8 years respectively, present average levels of 2.5-5.5 mg/100 ml and are classified as regular in a situation of advisable transplant, although with an acceptable hepatic function. Only one case, the first in the poor group, did not initially present biliary elimination and died at age six months while on the waiting list. Three other cases in the same group presented insufficient biliary elimination and were transplanted with 7, 11 and 12.5 kg, respectively. The second died in the first year post-transplant. CONCLUSIONS: In our opinion, action in biliary atresia must be early and based on the correct application of Kasai's technique, seeking to achieve a biliar flow that eliminates or distances the patient as far as possible from the necessity of a future LT. Three lines come together to obtain this target: an early diagnosis, a correct application of Kasai's technique, and an implication in the follow-up and treatment of these children by the hepatic transplant groups. All this advises us, as is done in other countries, to create reference centers for the study of neonatal cholestasis where an accumulated experience of a relatively rare pathology can be taken advantage of.
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