The vascular endothelium and coagulation: homeostasis, disease, and treatment, with a focus on the von willebrand factor and factors VIII and V

JAD Pablo-Moreno, LJ Serrano, L Revuelta… - International Journal of …, 2022 - mdpi.com
The vascular endothelium has several important functions, including hemostasis. The
homeostasis of hemostasis is based on a fine balance between procoagulant and …

A comprehensive overview of coagulation factor V and congenital factor V deficiency

S Tabibian, Y Shiravand, M Shams… - … in thrombosis and …, 2019 - thieme-connect.com
Coagulation factor (F) V is a glycoprotein that plays an essential role in the formation of the
prothrombinase complex, which is critical for progressing clot formation. FV deficiency is a …

The magic of proteases: from a procoagulant and anticoagulant factor V to an equitable treatment of its inherited deficiency

JA De Pablo-Moreno, A Miguel-Batuecas… - International Journal of …, 2023 - mdpi.com
Proteostasis, ie, the homeostasis of proteins, responsible for ensuring protein turnover, is
regulated by proteases, which also participate in the etiopathogenesis of multiple conditions …

Transfusion in neonatal patients: review of evidence-based guidelines

PE Zerra, CD Josephson - Clinics in laboratory medicine, 2021 - labmed.theclinics.com
Although a critical therapy in the management of hospitalized pediatric patients, 1, 2
transfusion of red blood cells (RBCs), platelets, and fresh frozen plasma (FFP) in neonatal …

Diagnosis, clinical manifestations and management of rare bleeding disorders in Iran

A Dorgalaleh, SER Alavi, S Tabibian, S Soori… - …, 2017 - Taylor & Francis
ABSTRACT Background: Rare bleeding disorders (RBDs) are heterogeneous disorders,
mostly inherited in an autosomal recessive pattern. Iran is a Mideast country with a high rate …

Rare diseases with periodontal manifestations

M Hanisch, T Hoffmann, L Bohner, L Hanisch… - International Journal of …, 2019 - mdpi.com
Background: The object of this paper was to provide an overview of rare diseases (RDs) with
periodontal manifestations and allocate them to relevant categories. Methods: In ROMSE, a …

High mutational heterogeneity, and new mutations in the human coagulation factor V gene. Future perspectives for factor V deficiency using recombinant and …

S Bernal, I Pelaez, L Alias, M Baena… - International journal of …, 2021 - mdpi.com
Factor V is an essential clotting factor that plays a key role in the blood coagulation cascade
on account of its procoagulant and anticoagulant activity. Eighty percent of circulating factor …

Intracranial hemorrhage in congenital bleeding disorders

S Tabibian, H Motlagh, M Naderi… - Blood Coagulation & …, 2018 - journals.lww.com
Intracranial hemorrhage (ICH), as a life-threatening bleeding among all kinds of congenital
bleeding disorders (CBDs), is a rare manifestation except in factor XIII (FXIII) deficiency …

Transfusion therapy of neonatal and paediatric patients: They are not just little adults

RR Gammon, N Al‐Mozain, M Auron… - Transfusion …, 2022 - Wiley Online Library
Patient blood management (PBM) strategies are needed in the neonate and paediatric
population, given that haemoglobin thresholds used are often higher than recommended by …

Acquired factor V inhibitor: a nation‐wide study of 38 patients

T Goulenok, C Vasco, D Faille… - British Journal of …, 2021 - Wiley Online Library
Acquired factor V inhibitor (AFVI) is an extremely rare disorder that may cause severe
bleeding. To identify factors associated with bleeding risk in AFVI patients, a national …