門脈本幹閉塞症（EHPVO）の小児患者における凝固プロファイルと血小板数

The aim of the study was to study coagulation abnormalities and hematimetric indices in pediatric patients with extrahepatic portal vein obstruction (EHPVO).

This was a retrospective and longitudinal study, which included 93 patients diagnosed with EHPVO treated between January 1991 and December 2023 at a reference center for pediatric liver diseases. The research was carried out by collecting data from the medical records of these patients, who were divided into three groups: G1 (n=78; EHPVO without surgery), G2 (n=4; EHPVO with surgery without splenectomy), and G3 (n=11; EHPVO with splenectomy).

The mean age at admission was 5.48 years, with 64.5% male patients and an average follow-up time of 10.42 years. At admission, the prevalence of prolonged R (thromboplastin time Ratio) and INR (international normalized ratio) was 52.32% and 26.8%, respectively; anemia was observed in 67.7% of patients, and thrombocytopenia in 70.45%. In the comparison of each variable between admission and the last follow-up, it was observed that for R, INR, and platelets, there was no significant difference in group 1. However, there was a significant difference in group 3, with a reduction in R and INR at the last follow-up compared to admission, and a significant increase in platelets between admission and the last follow-up. Owing to the reduced sample size, the outcomes of group 2 were excluded from the between-group comparisons due to limitationsin statistical power.

The percentage of anemia, thrombocytopenia, and prolonged INR at admission was high. Splenectomy resulted in an increase in platelet count and a reduction in R and INR. Therefore, the alteration in INR and R at admission is likely not due to the consumption of coagulation factors in the cavernoma region, but rather, probably in the spleen.