Twenty-Year Transplant-Free Survival Rate Among Patients With Biliary Atresia
| Autor: | Vries, W. de, Homan-van der Veen, J., Hulscher, J.B.F., Hoekstra-Weebers, J.E.H.M., Houwen, R.H.J., Verkade, H.J., Aronson, C., Escher, J.H., Heurn, L.W.E. van, Heij, H.A., Kindermann, A., Kneepkens, C.M., Langen, Z.J. de, Madern, G.C., Neucke, M. van den, Peeters, P.M., Rieu, P.N.M.A., Tolboom, J.J.M., Zee, D.C. van der |
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| Přispěvatelé: | Paediatric Surgery, Other Research, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, Paediatric Gastroenterology, Center for Liver, Digestive and Metabolic Diseases (CLDM), Lifestyle Medicine (LM), Health Psychology Research (HPR) |
| Rok vydání: | 2011 |
| Předmět: |
Adult
Male medicine.medical_specialty Cirrhosis SURGERY medicine.medical_treatment CHILDREN Liver transplantation Gastroenterology DISEASE Liver Function Tests Biliary Atresia QUALITY-OF-LIFE Biliary atresia Internal medicine MANAGEMENT medicine Humans HEPATIC PORTOENTEROSTOMY Child Survival rate Survival analysis Netherlands Ultrasonography OLDER Cholestasis Cardiovascular diseases [NCEBP 14] Hepatology medicine.diagnostic_test business.industry Infant Survival Time LIVER-TRANSPLANTATION medicine.disease Survival Analysis CHOLANGITIS Liver Child Preschool Cohort Quality of Life EXPERIENCE Portal hypertension Female Therapy business Liver function tests The Netherlands Study group on Biliary Atresia Registry (NeSBAR) |
| Zdroj: | Clinical Gastroenterology and Hepatology, 9, 1086-91 Clinical gastroenterology and hepatology, 9(12), 1086-1091. W.B. Saunders Ltd Clinical Gastroenterology and Hepatology, 9, 12, pp. 1086-91 Clinical Gastroenterology and Hepatology, 9(12), 1086-1091. ELSEVIER SCIENCE INC |
| ISSN: | 1542-3565 |
| Popis: | Item does not contain fulltext BACKGROUND & AIMS: Surgical treatment with Kasai portoenterostomy has improved the prognosis for patients with biliary atresia, although most patients ultimately require liver transplantation. Well-described patients with long-term, transplant-free survival are scarce; we assessed liver status and health perception among Dutch patients who survived 20 years after therapy and investigated whether the rate of transplant-free survival increases with time. METHODS: By using the Dutch national database for biliary atresia, we identified 104 patients, born between 1977 and 1988. We collected data on clinical characteristics, liver biochemistry, and ultrasonography from all transplant-free patients who were alive at age 20 years (n = 28; 27% of the patients). General health perception data (RAND-36) were collected at the last examination. RESULTS: The 20-year transplant-free survival rate increased from 20% (10 of 49) in the 1977 to 1982 cohort to 32% (18 of 55) in the 1983 to 1988 cohort (P = .03). Twenty-one percent of the long-term survivors (6 of 28) had normal liver biochemistry test results and no clinical or ultrasonographic signs of cirrhosis. The general health perception of female, but not male, patients, was lower, compared with controls (RAND-36 score, 54 +/- 14 vs 74 +/- 18; P = .005). CONCLUSIONS: More than 25% of patients with biliary atresia survive at least 20 years without liver transplantation in The Netherlands. Women with biliary atresia have a reduced perception of their health, compared with control patients. Twenty percent of long-term survivors are symptom-free, without clinical or ultrasonographic signs of cirrhosis or portal hypertension. 01 december 2011 |
| Databáze: | OpenAIRE |
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