Long-Term Effects of Kasai Portoenterostomy for Biliary Atresia Treatment in Russia

Degtyareva, Anna; Razumovskiy, A. Yu.; Kulikova, N. V.; Ratnikov, Sergey; Filippova, E.A.; Gordeeva, E. A.; Albegova, M.B.; Rebrikov, Denis V.; Пучкова, А. А.

doi:10.3390/diagnostics10090686

Cited by 5 publications

(9 citation statements)

References 25 publications

(40 reference statements)

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“…Patients with BA treated with KP often show different lengths of native liver survival time (NLST), which ranges from 59.6% to 63.5% at 1 year, (13,14) approximately 50% at 2 years, (15,16) and 31% to 36% at 10 years post KP (P-KP). (7,17) The literature on the effect of NLST on post-LT outcomes is scarce and varied.…”

Section: Original Articlementioning

confidence: 99%

Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia

Liu¹,

Lin²,

Qu³

et al. 2021

Liver Transpl

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The aim is to explore the impact of the Kasai procedure (KP) and the length of native liver survival time (NLST) on outcomes of liver transplantation (LT). Patients with biliary atresia (BA), who underwent LT in Beijing Friendship Hospital from January 2017 to December 2019, were enrolled and divided into non-KP (N-KP) and post-KP (P-KP) groups. The patients in the P-KP group were further divided into early failure (KP-EF) defined by NLST <1 year, medium failure (KP-MF, NLST 1-5 years), and late failure (KP-LF, NLST >5 years) subgroups. Clinical data at baseline and during follow-up were collected. The inverse probability of treatment weighting method was used to evaluate the independent effect of KP and the length of NLST on clinical outcomes. Among 197 patients with BA, the N-KP group accounted for 43 (21.8%), KP-EF 71 (46.1%), KP-MF 59 (38.3%), and KP-LF 24 (15.6%) cases, respectively. The N-KP and KP-EF groups had significantly longer hospitalization and intensive care unit stays after LT. Graft and overall survival rates were 93.0% in the N-KP group and 97.4% in P-KP group, respectively. The mortality rate in the P-KP group were significantly lower compared with that of the N-KP group with a hazard ratio (HR) of 0.2 (P = 0.02). The risks of biliary and vascular complications and cytomegalovirus (CMV) infection after LT were significantly higher in KP-EF group than those in the KP-MF and KP-LF groups (HRs = 0.09, 0.2, and 0.3, respectively; all P < 0.001). The KP significantly improved after LT overall survival. Patients with early native liver failure after KP have significantly higher risks for biliary and vascular complications and CMV infection.

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Section: Original Articlementioning

confidence: 99%

Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia

Liu¹,

Lin²,

Qu³

et al. 2021

Liver Transpl

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“…Post-LT, hospital LOS was 15 days, while ICU LOS was 6 days. Compared to the no-ACLF group, a higher proportion of ACLF patients needed vasopressors for hemodynamic support post-LT (30.8% vs. 10.6%, p = .04), had higher total LOS (178 [126-236] vs. 20 days, p = .001), higher LOS post-LT (106 [45-151] vs. 13 days, p = .023), and higher ICU LOS post-LT (50 vs. 5 [2][3][4][5][6][7][8][9][10][11][12][13][14] days, p < .001) (Table 3). Abbreviation: ACLF, acute-on-chronic liver failure.…”

Section: Frequency Of Aclf and Impact On Intraoperative And Postopera...mentioning

confidence: 99%

Postoperative outcomes of acute‐on‐chronic liver failure in infants and children with biliary atresia

Naeem,

Ayub,

Coss‐Bu

et al. 2024

Pediatric Transplantation

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IntroductionAcute‐on‐chronic liver failure (ACLF) is associated with increased mortality and morbidity in patients with biliary atresia (BA). Data on impact of ACLF on postoperative outcomes, however, are sparse.MethodWe performed a retrospective analysis of patients with BA aged <18 years who underwent LT between 2011 and 2021 at our institution. ACLF was defined using the pediatric ACLF criteria: ≥1 extra‐hepatic organ failure in children with decompensated cirrhosis.ResultsOf 107 patients (65% female; median age 14 [9–31] months) who received a LT, 13 (12%) had ACLF during the index admission prior to LT. Two (15%) had Grade 1; 4 (30%) had Grade 2; and 7 (55%) had Grade ≥3 ACLF. ACLF cohort was younger at time of listing (5 [4–8] vs. 9 [6–24] months; p < .001) and at LT (8 [8–11] vs. 16 [10–40] months, p < .001) compared to no‐ACLF group. Intraoperatively, ACLF patients had higher blood loss (40 [20–53] vs. 10 [6–19] mL/kg; p < .001) and blood transfusion requirements (33 [21–69] vs. 18 [7–25] mL/kg; p = .004). Postoperatively, they needed higher vasopressor support (31% vs. 10.6%; p = .04) and had higher total hospital length of stay (106 [45–151] vs. 13 [7–30] days; p = .023). Rate of return to the operating room, hospital readmission rates, and 1‐year post‐LT survival rates were comparable between the groups.ConclusionDespite higher perioperative complications, survival outcomes for ACLF in BA after LT are favorable and comparable to those without ACLF. These encouraging data reiterate prioritization during organ allocation of these critically ill children for LT.

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“…In Type 1, there is obstruction of the common bile duct, and in type 2, there is obstruction at the level of the common hepatic duct. And type 3 comprises 90% of cases is non‐correctable type associated with obstruction of entire biliary tree involving porta hepatitis 9 …”

Section: Introductionmentioning

confidence: 99%

“…And type 3 comprises 90% of cases is non-correctable type associated with obstruction of entire biliary tree involving porta hepatitis. 9 Kasai portoenterostomy (KPE) restores bile flow and improves jaundice in 70-80% of cases when performed before 60 days of life. 10 Factors that affect outcome of KPE include patient's age at time of presentation, presence of cirrhosis at time of surgery, surgical unit size and experience of performing KPE and development of post-operative cholangitis.…”

mentioning

confidence: 99%

Biliary atresia—An experience from the first pediatric liver transplant center in Pakistan

Khan

Ali

Dar

et al. 2022

Pediatric Transplantation

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Background: Biliary atresia (BA) is the most common cause of neonatal cholestatic syndrome. The true incidence of BA in Pakistan is largely unknown.Aim: This study aimed to report the clinical features, age at diagnosis and outcomes of biliary atresia from the first pediatric liver transplant center in Pakistan. Methods:The study was done in Shifa International hospital from 2013 to 2020. All babies who had biliary atresia confirmed by laboratory investigation were included.Demographic data, age of presentation, clinical presentation, supporting investigations like liver function tests, ultrasound abdomen, HIDA scan and liver biopsy were noted. Outcome related to Kasai portoenterostomy, liver transplant, complications and immunosuppressant agents were noted.Result: A total of 42 children were included, 23 (54.7%) males and 19 (45.2%) were females. Jaundice was seen in all patients (100%) followed by acholic stools (81%). Associated malformations were noted in 6 (14.2%) patients. Liver function tests confirmed obstructive cholestasis (p 0.04). Kasai was done in 19 (45%) patients only, living donor liver transplant was performed in 6 (14%) patients. Age range of transplant patients was from 3 months to 1 year. Indication for liver transplant was failed Kasai in 1(16.7%) patient and chronic liver disease in 5 (83.3%) patients. LDLT survivors were 10 months to 1 year of age at the time of transplant, mean age was 10.6 months.Maximum survival noted so far is 7 years. Acute complications seen post-transplant were sepsis (three patients), surgical site infections (two patients), biliary leaks and acute cellular rejection in one patient each. Chronic graft rejection, portal vein stricture needing stenting was done in one patient.Discussion: All patients underwent LDLT from related donors wih no donor related mortality. All are deceased patients were yonger and had advanced disease. BA remains third most commo indication of transplant in our center. Conclusion:Liver transplant is the only lifesaving procedure after failed Kasai or as primary liver transplant due to advance liver disease. The advent of liver transplantation services offers survival and improving outlook of the disease.

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Long-Term Effects of Kasai Portoenterostomy for Biliary Atresia Treatment in Russia

Cited by 5 publications

References 25 publications

Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia

Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia

Postoperative outcomes of acute‐on‐chronic liver failure in infants and children with biliary atresia

Biliary atresia—An experience from the first pediatric liver transplant center in Pakistan

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