discussions as one ich patient whose pt and aptt suggest a coagulation disorder secondary to vitamin k deficiency or coagulation factor deficiency, unresponsiveness to vitamin k therapy should be useful to take fx deficiency into consideration. background inborn factor x deficiency (fxd) is a very rare (1: 500,000) hereditary coagulation disorder, which is characterized by clinical manifestatio...

INTRODUCTION Inherited defects in RUNX1 are important causes of platelet function disorders. AIM Our goals were to evaluate RUNX1-related platelet disorders among individuals evaluated for uncharacterized, inherited platelet function disorders and test a proof of concept that bleeding risks could be quantitatively estimated for typical families with an inherited platelet function disorder. ...

Introduction: Acquired factor VIII (FVIII) inhibitors, also known as acquired hemophilia A, are rare with an annual incidence of 1-4 per million people [1,2]. Although the majority of cases of acquired FVIII inhibitors are idiopathic, this disorder is associated with underlying malignancies, autoimmune disease, pregnancy, infections, and medications. Bleeding complications are reported in over ...

End-stage renal disease patients, particularly those treated with hemodialysis (HD), suffer from complex hemostatic disorders. Patients with uremia may experience two opposite he‐ mostatic complications: bleeding diathesis and thrombotic tendencies. Bleeding diathesis in uremic patients is primarily seen due to abnormalities in primary hemostasis, particularly platelet function disorder and imp...

BACKGROUND Factor V deficiency is a rare autosomal recessive coagulation disorder. Awareness of presenting features and management is important to avoid bleeding complications associated with mortality and neurodisability. CASE PRESENTATION A 6-day-old Pakistani boy was admitted with bleeding from the left nipple. His parents were first cousins. A coagulation screen showed a prothrombin time ...

Haemophilia A is a bleeding disorder that has a spectrum of manifestations ranging from persistent bleeding after minor trauma to spontaneous haemorrhage. As an X-linked disease, it has a rare occurrence in females. We report a case of a pregnant patient with severe haemophilia A, who received epidural analgesia during labour. The prepartum, intrapartum and postpartum care of a patient with suc...

Surgical interventions for vesicoureteral reflux (VUR) include open or laparoscopic ureterocystoneostomy (UCN) and endoscopic injection. Historically, open UCN has been the gold standard in surgical treatment of VUR with high success and low complication rates. [1] Although open UCN causes bleeding from the incised bladder wall or dissected trigone, the bleeding is usually not severe unless a b...

Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a fre...

Background: Critically ill children admitted to pediatric intensive care unit (PICU) are at increased risk of gastrointestinal bleeding due to stress related mucosal injury. Reducing gastric acid by acid suppressant medication is the accepted prophylaxis treatment, but there is not any definitive guideline for using prophylaxis in PICU patients. The present study aimed to assess the effect of P...

Type 2N Von Willebrand's disease (2N VWD) is a rare, recessively inherited bleeding disorder, comprising 1-2% of all VWD patients, usually manifesting as a mild bleeding diathesis. Treatment includes desmopressin (DDAVP) or intermediate purity plasma-derived FVIII concentrates containing residual VWF. We present a case of a 75-year-old gentleman, incidentally diagnosed with type 2N VWD in 2002 ...