Original article—liver, pancreas, and biliary tractTwenty-Year Transplant-Free Survival Rate Among Patients With Biliary Atresia
Section snippets
Patients and Methods
Between January 1, 1977, and December 31, 1988, there were 106 patients who were diagnosed with BA in The Netherlands. The diagnosis of BA was confirmed in all patients by intraoperative cholangiography and pathology of the liver and biliary remnants. Surgery was performed in 1 of the 6 Dutch university medical centers that specializes in pediatric surgery. The short-term results of the majority of this cohort were described in 1989.9 The Dutch OLT program started in 1979 in adults and in 1982
Twenty-Year Survival After Surgical Correction
Of the 106 children (62 girls), 2 were lost to follow-up evaluation at the ages of 4 (address not found) and 6 years (moved abroad). Twenty-eight of the 104 patients (27%) survived for 20 years without OLT (Table 1). Sixteen of these patients were female (57%). The overall 20-year survival with and without OLT was 43% (45 of 104). OLT was performed in 23% (24 of 104) of patients before the age of 20. The survival rate after OLT was 71% (17 of 24). Fifty percent (52 of 104) of the patients died
Discussion
Since 1977, the transplant-free 20-year survival rate of BA patients treated with surgical correction has increased from 20% to 33% in The Netherlands. The 20-year survival rate was not significantly associated with the age at surgical correction. Interestingly, one fifth of the 20-year transplant-free survivors had no signs of cirrhosis and a normal liver biochemistry. Female, but not male, transplant-free BA patients had a lower GH when compared with a reference group.
The 20-year
Acknowledgments
The author are grateful to Dr A. P. van den Berg, Professor Dr J. P. H. Drenth, Dr K. J. van Erpecum, Dr R. J. de Knegt, and Dr J. Salemans for their valuable help in retrieving patient data. The authors also thank The Netherlands Study group of Biliary Atresia and Registry (NeSBAR): D. C. Aronson, J. H. Escher, L. W. E. van Heurn, H. A. Heij, A. Kindermann, C. M. F. Kneepkens, Z. J. de Langen, G. Madern, A. M. van den Neucker, P. M. J. G. Peeters, P. N. M. A. Rieu, J. J. M. Tolboom, and D. C.
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Conflicts of interest The authors disclose no conflicts.