Outcomes of biliary atresia in the Nordic countries – a multicenter study of 158 patients during 2005–2016☆,☆☆
Section snippets
Study design and data collection
This was an international multicenter retrospective observational survey. All children with confirmed BA born in the Nordic countries during 1.1.2005–30.6.2016 and followed-up at least for 4 months were included. Data were collected locally by pediatric surgeons, pediatric hepatologists and pediatricians in all Nordic centers involved with the treatment of BA according to a collectively approved questionnaire and data extraction sheet. The participating centers included one from Denmark
Center characteristics
The median number of patients treated in the participating six centers annually ranged from 0.7 to 3.7, while only two centers managed over three patients annually (Supplementary Table 1). Cholescintigraphy, operative cholangiography and ultrasound were uniformly used diagnostic investigations, whereas preoperative diagnostic liver biopsy, MRI, PTC and ERC were less commonly employed. In five centers one surgeon or surgical team operated all BA patients and none used laparoscopic surgical
Discussion
The results of this study show that the clearance of jaundice rate, the 5-year native liver survival after PE and overall survival in the Nordic countries compared well with previous European studies (Table 5). Following PE, serum bilirubin concentration normalized in 64%, and 55% of the Nordic patients were alive with their native livers at 5 years. Their median serum bilirubin and blood platelet levels were in the normal range, reflecting well-preserved native liver function. The diagnostic
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Systematic review: The quality of life of patients with biliary atresia
2022, Journal of Pediatric SurgeryCitation Excerpt :In Japan, on the other hand, 49% of BA patients live with their native livers for at least 20 years. All studies found that a younger age at HPE was associated with longer native liver survival [7–15]. Despite the advent of research on the long-term fate of BA patients in terms of HrQoL, the disease's rarity, as evidenced by the paucity of big, multicenter studies, calls for care in interpreting the published variable findings.
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2022, Best Practice and Research: Clinical GastroenterologyCitation Excerpt :Aspired short-term outcome of PE entails normalization of serum bilirubin level, reflecting successful restoration of adequate bile flow from the liver to the intestine, a discernible prerequisite for long-term NLS [2]. In a Nordic multicenter study of 148 patients, only 10% of the patients who failed to reach serum bilirubin below 20 μmol/l following PE survived with NL for 2 years, as opposed to over 90% 2-year NLS among those who normalized their serum bilirubin [5]. The positive effect of bilirubin normalization on NLS only increased with longer follow-up.
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2021, Annals of Medicine and SurgeryCitation Excerpt :The effect of age at KPE that is affected by many factors (i.e. access to medical care, diagnosis efficiency, surgery scheduling, etc) on post-operative jaundice resolution, short-, mid-and long-term both NLS and overall survivals has been a matter of debate for years in the literature as some reports have been against its benefit, while most others have ensured its importance [60]; those ensuring studies have documented that the operation has had better outcomes when has been performed before 90 days of life due to the presence of an association between younger age at KPE and both increased porta-hepatis remnant ducts NO and decreased fibrosis grade [8,61,62]; therefore, performing it before 90 days has been the goal of most centers. Similarly, and despite the need for more improvement, only 23% of our infants underwent the operation after 90 days of life as we did our best for early detection of the disease, moreover, age<90 days at KPE was a significant predictor of postoperative jaundice resolution and independent predictor of long-term NLS in our study, also, younger age at KPE was a significant predictor of jaundice resolution in Chardot et al., 2013 [13], Pakarinen et al., 2018 [20], Parolini et al., 2019 [22], Hanalioğlu et al., 2019 [27], and Ihn et al., 2018 [63] retrospective studies. Moreover, it was an independent predictor of 4- and 5-year TFS in de Vries et al., 2012 [15] and Qiao et al., 2015 [64] retrospective studies respectively.
Diagnostic and prognostic value of the gut microbiota and its metabolite butyrate in children with biliary atresia
2024, Pediatric Surgery International
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No conflicts of interest to declare.
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Financial support: MPP was supported by grants from the Finnish Pediatric Research Foundation, the Sigrid Juselius Foundation and the Helsinki University Central Hospital research funds. JFS and MA were supported by grants from the Crown Princess Lovisa Foundation and Sällskapet Barnavård.