@article{article_968568, title={Hematological Aspects of Extrahepatic Portal Vein Obstruction in Childhood}, journal={Türk Hijyen ve Deneysel Biyoloji Dergisi}, volume={76}, pages={415–422}, year={2019}, author={Karakurt, Neslihan and Demirbaş, Fatma and Çaltepe, Gönül and Albayrak, Canan and Kalaycı, Ayhan Gazi}, keywords={Portal vein thrombosis, thrombocytopenia, gastrointestinal system bleeding, splenomegaly, plasminogen activator inhibitor-1}, abstract={Objective: Extrahepatic portal vein obstruction is rare but considerable cause of portal hypertension PHT . Patients may present with cytopenia s or bleeding, to hematology clinics. The aim of this study is to present our experience of patients with this rare disease and emphasize the value of thrombophilia assessment in portal venous thrombosis PVT .Methods: Children admitted to our hospital between June 2006 and October 2018 with diagnosis of extra-hepatic portal venous anomalies are included. Vascular anomalies were defined as trombus and/or portal cavernoma PC . The medical reports were assessed retrospectively.Results: Twelve patients Female/ Male: 9/ 3 aged 4.6± 3.4 years old are included. The most common complaints on admission were abdominal pain and upper gastrointestinal bleeding. Ten patients 83% had cytopenia s at diagnosis; eight 67% had anemia, five had 42% leukopenia and nine had 75% thrombocytopenia. Imaging studies revealed thrombus in ten 83% and PC in ten 83% patients. Five patients had a history of umbilical catheterization and one was also positive for homozygous mutation of Factor V Leiden. Four patients were positive for heterozygous mutation of PAI- 1. Conclusion: PVT during childhood is rare, it may present with thrombocytopenia, splenomegaly and esophageal varices bleeding. Although PVT it is not a common cause of thrombocytopenia in children, clinicians are encouraged to get information regarding history of umbilical catheterization in neonatal period and obtain portal doppler ultrasound in cases with splenomegaly. Further studies about PVT Plasminogen activator inhibitor 1 mutations are needed.}, number={4}, publisher={T.C. Sağlık Bakanlığı Halk Sağlığı Genel Müdürlüğü}