Cholangitis as a factor affecting the effectiveness of treatment of biliary atresia in children

Abstract

Biliary atresia (BA) is a severe progressive liver disease in neonates, characterized by obliteration of bile ducts, cholestasis, fibrosis, and cirrhosis. Acute cholangitis (AC) after Kasai hepatoportoenterostomy (KPE) is a key factor influencing treatment efficacy and liver function prognosis.

Aim - to determine diagnostic criteria, assess the effectiveness of treatment, and evaluate the role of interventional procedures in children with BA after KPE.

Materials and methods. Sixty-four children with BA who underwent KPE at 1,5-4,5 months of age were included. AC subtypes were classified by episode frequency: isolated (IAC), early (EAC), recurrent (RAC), and non-resolving (NAC). Treatment included third-generation cephalosporins, combinations with meropenem, co-trimoxazole, and intravenous immunoglobulins. Effectiveness was evaluated by clinical symptoms, laboratory parameters (direct bilirubin, ALT, AST, GGT, CRP, PCT), and instrumental methods. Statistical analysis was performed using the t-test and ANOVA.

Results. AC occurred in 100% of children after KPE. IAC and EAC were characterized by moderate

 increases in liver enzymes and inflammatory markers and achieved full normalization after third-generation cephalosporin therapy. In RAC patients, combination therapy with meropenem provided rapid normalization of laboratory parameters: direct bilirubin decreased from 78.5±5.3 to 3.1±0.3 µmol/L, CRP from 42,1±1,1 mg/L to 1,1±0.3 mg/L, PCT from 49,7±1,6 ng/mL to 0,9±0,1 ng/mL. In NAC patients, persistent clinical symptoms and elevated inflammatory markers were observed: direct bilirubin 35,9±0,8 µmol/L, CRP 20,6±0.6 mg/L, PCT 12,8±0,5 ng/mL, indicating a severe course and the need for additional interventional and surgical procedures.

Conclusions. AC subtype after KPE determines the severity of clinical and laboratory disturbances and prognosis in children with BA. Differentiated conservative therapy according to AC subtype allows normalization of clinical and laboratory parameters in mild and recurrent forms, whereas severe cases (NAC) require additional interventional procedures. High levels of CRP and PCT are prognostically unfavorable markers of AC severity.

The study was conducted in accordance with the Declaration of Helsinki. The protocol was approved by the local ethics committee. Written informed consent was obtained from the children’s legal guardians.

The author declares no conflict of interest.