Factors influencing jaundice-free survival with the native liver in post-portoenterostomy biliary atresia patients: Results from a single institution

Koga, Hiroyuki; Wada, Mariko; Nakamura, Hiroki; Miyano, Go; Okawada, Manabu; Lane, Geoffrey J.; Okazaki, Tadaharu; Yamataka, Atsuyuki

doi:10.1016/j.jpedsurg.2013.08.007

Cited by 62 publications

(39 citation statements)

References 26 publications

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“…5,10,[14][15][16][17] Moreover, its occurrence has been found to significantly reduce the survival rate of patients with either good or inadequate bile flow 7,16,17 , and repeated episodes-especially within the first 2 years of surgery-have been associated with a decrease in native liver survival and, ultimately, with the need for liver transplantation. [18][19][20][21] In the current study, the overall incidence of cholangitis was 87.5%, and perioperative intravenous antibiotics were universally provided (usually for 1-2 weeks, but even longer if the cholangitis worsened). The majority of the antibiotics were combinations of third generation cephalosporins and metronidazole.…”

Section: Discussionmentioning

confidence: 71%

“…These findings are consistent with previous studies which reported that, firstly, an increased number of cholangitis episodes negatively affected the native liver survival, and secondly, having episodes more than 2 years after the Kasai portoenterostomy appeared to be a prognostic marker for a future liver transplantation. 18,20,21 In addition to cholangitis, the univariate analysis demonstrated other significant factors affecting the long-term outcome, namely, an age at operation of less than 80 days, the achievement of jaundice clearance, a prolonged provision of a prophylactic antibiotic, and a shorter duration to attain jaundice clearance. In a longterm study of native liver patients who had survived for more than 20 years after the Kasai operation, Nio et al 25 concluded that the age at operation, the early development of cholangitis, and the time to jaundice resolution were prognostic factors influencing longterm native liver survival.…”

Section: Discussionmentioning

confidence: 99%

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Factors Related to the Clinical Outcomes of the Kasai Procedure in Infants with Biliary Atresia

Tantemsapya

2020

SMJ

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Objective: The Kasai portoenterostomy has been accepted as the primary therapeutic treatment for biliary atresia. However, successful bile drainage does not always promise a long-term native liver survival. Several clinical factors were evaluated to discover associations with the outcomes. Methods: A retrospective chart review was conducted of infants with biliary atresia who underwent the conventional Kasai portoenterostomy at Siriraj Hospital, January 2006-August 2018. The patient demographics, perioperative clinical and laboratory data, adjuvant therapies, complications, and interventions were analyzed in correlation to the short-and long-term outcomes. The short-term outcome evaluated was the resolution of jaundice, while the long-term outcome was remaining jaundice-free with the native liver. Results: The complete medical records of 80 patients were retrospectively reviewed. Their mean age at the time of the Kasai portoenterostomies was 97 days. Overall, 66.3% achieved jaundice clearance. The mean follow-up duration was 50.5 months, with 51.3% of the patients remaining jaundice-free with their native liver. A prolonged duration of a prophylactic antibiotic was significantly related to the jaundice clearance, with a p-value of 0.002. Moreover, a lower number of episodes of cholangitis was significantly related to a good long-term outcome (p-value, 0.024). Conclusion: The prolonged provision of a prophylactic antibiotic as adjuvant therapy after the Kasai portoenterostomy was associated with jaundice clearance. An increased incidence of cholangitis episodes was associated with poor long-term outcomes. Postoperative adjuvant therapy should be standardized and maintained for the care of biliary atresia patients to improve their outcomes.

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Section: Discussionmentioning

confidence: 71%

Section: Discussionmentioning

confidence: 99%

Factors Related to the Clinical Outcomes of the Kasai Procedure in Infants with Biliary Atresia

Tantemsapya

2020

SMJ

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“…In line with our findings, greater levels of serum bilirubin, AST, APRI, and GT at 3 to 5 months after PEostomy have been suggested to be associated with decreased survival of the native liver after COJ. 29,30 In our patients who underwent protocol liver biopsies not warranted by their clinical status, postoperative bilirubin outperformed other liver function tests in the prediction of survival of the native liver, followup cirrhosis, and progression of fibrosis; whereas other patientrelated and treatment-related factors had no association with these outcomes. Very low serum bilirubin levels at 3 and 6 months after PEostomy were associated with a 100% 5-year survival of the native liver, demonstrating that instead of the commonly used cutoffs of 34 or 20 μmol/L (1.2 or 1.7 mg/dL), only bilirubin levels approaching the lower limit of normal reflect an optimal postoperative outcome.…”

Section: Discussionmentioning

confidence: 76%

Very low bilirubin after portoenterostomy improves survival of the native liver in patients with biliary atresia by deferring liver fibrogenesis

Hukkinen

Kerola

Jahnukainen

et al. 2019

Surgery

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Background: Progression of fibrosis and ensuing complications determine the postoperative course of patients operated on for biliary atresia. We evaluated predictors of the progression of fibrosis in the native liver after operative treatment. Methods: Among patients whose bilirubin decreased to < 34 μmol/L after portoenterostomy (n = 41), predictors of follow-up cirrhosis and the progression of fibrosis were analyzed with logistic regression and survival of their native liver was evaluated with the Kaplan-Meier method. Areas under receiving operating characteristic were used to define optimal cutoffs. Results: After median follow-up of 5.2 years (interquartile range 1.6-10.2) after portoenterostomy, liver biopsies showed cirrhosis in 53% of patients, and the Metavir stage remained stable or decreased in 38%. The development of cirrhosis was predicted by total or conjugated bilirubin ≥170/120 μmol/L at the time of portoenterostomy (P ≤ .009); normalization of bilirubin within 1.9 months (P = .002); total or conjugated bilirubin ≥ 12.5/7.5 μmol/L (P = .002) and aspartate aminotransferase-to-platelet ratio ≥ 0.55 at 3 months postoperatively (P = .001); and total or conjugated bilirubin ≥ 7.5/2.5 μmol/L (P ≤ .001), aspartate aminotransferase-to-platelet ratio ≥ 0.63 (P = .004), and gamma glutamyl transferase ≥ 266 U/L (P = .007) at 6 months postoperatively. In multiple regression analysis, conjugated bilirubin ≥ 2.5 μmol/L at 6 months increased the risk of cirrhosis 35-fold (P = .020), and other predictors were not predictive. Total or conjugated bilirubin < 12.5/7.5 μmol/L (P ≤ .014), aspartate aminotransferase-to-platelet ratio < 0.55 at 3 months (P = .006), and total or conjugated bilirubin < 7.5/2.5 μmol/L at 6 months postoperatively (P ≤ .014) were the strongest predictors of a stable, nonprogressive fibrosis. Decreases in total or conjugated bilirubin to < 12.5/7.5 μmol/L by 3 months and to < 7.5/2.5 μmol/L by 6 months improved survival of the native liver (log-rank P ≤ .025). Age at follow-up or at portoenterostomy, anatomic type of biliary atresia, use of postoperative steroids, and episodes of cholangitis were unrelated to the progression of fibrosis or the development of cirrhosis (P = not significant). Conclusion: Among patients whose serum bilirubin normalizes after portoenterostomy, its rapid decrease to very low levels prolongs the survival of their native liver by delaying the progression of fibrosis.

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“…Postoperatif 3. ayın sonunda "sarılığın düzelmesi" serum total bilirubin düzeylerinin 2 mg/dl veya daha düşük olması olarak tanımlanır ve portoenterostomi sonrası başarının iyi bir göstergesidir. Hepatik portoenterostomi sonrası 60 günden daha kısa sürede sarılı-ğın düzelmesi kendi karaciğeri ile uzun dönem yaşam süresini etkileyen en önemli faktörlerden birisidir (14) . Ayrıca, postoperatif 7. günde serum total bilirubin değerlerinin preoperatif değerlerin %20'sinden daha fazla düzelme göstermesi iyi bir sonuç göstergesi ola-rak kabul edilmekte ve kendi karaciğeri ile 5 yıllık yaşam oranlarının daha iyi olduğu belirtilmektedir (4) .…”

Section: Portoenterostomi Sonuçlarıunclassified

“…Ayrıca, postoperatif 7. günde serum total bilirubin değerlerinin preoperatif değerlerin %20'sinden daha fazla düzelme göstermesi iyi bir sonuç göstergesi ola-rak kabul edilmekte ve kendi karaciğeri ile 5 yıllık yaşam oranlarının daha iyi olduğu belirtilmektedir (4) . Ancak, sarılığı düzelse de, portoenterostomiyi izleyen ilk 3 ay içinde geçirilen kolanjit(ler), AST ve GGT değerlerinin yüksek seyretmesi gelecekte karaciğer transplantasyonu gereksinimi olacağını destekleyen bulgular arasında yer almaktadır (14) .…”

Section: Portoenterostomi Sonuçlarıunclassified

biliary atresia

Ergün¹

2017

tcdd

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Factors influencing jaundice-free survival with the native liver in post-portoenterostomy biliary atresia patients: Results from a single institution

Cited by 62 publications

References 26 publications

Factors Related to the Clinical Outcomes of the Kasai Procedure in Infants with Biliary Atresia

Factors Related to the Clinical Outcomes of the Kasai Procedure in Infants with Biliary Atresia

Very low bilirubin after portoenterostomy improves survival of the native liver in patients with biliary atresia by deferring liver fibrogenesis

biliary atresia

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