ABSTRACT
Von Willebrand disease (vWD) is the most common inherited bleeding disorder. The biggest challenge in the management of vWD is the difficulty in performing surgical procedures because of bleeding. Treatment guidelines recommend the use of pure von Willebrand factor or von Willebrand factor/factor VIII (vWF/FVIII) concentrate in patients with type 2 or type 3 vWD undergoing surgery, in patients with type 1 vWD undergoing surgery who are unresponsive, and in patients for whom desmopressin acetate is contraindicated. However, there is no consensus on the dosage and optimum levels of these factors to date. The aim of this study was to evaluate the perioperative management of patients with vWD during surgical procedures. Data pertaining to surgical procedures performed at our center from 2003 to 2014 were analyzed retrospectively. All operations, except one appendectomy, were elective, and a plan for the management of hemostasis was prepared for each patient. During this period, 37 surgical procedures (major, minor, and dental) were performed in 23 patients with vWD. Complications occurred in four out of 37 procedures (10.8%). No deaths or life-threatening bleeding occurred during any of the operations and no thromboembolic events were observed. The results from this retrospective study indicate that surgery can be safely performed by providing adequate and timely hemostasis during and after the procedure in patients with vWD. Perioperative and postoperative bleeding complications are rare when patients are closely and carefully monitored.
Subject(s)
von Willebrand Diseases/surgery , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Retrospective Studies , Treatment Outcome , Young Adult , von Willebrand Diseases/complicationsABSTRACT
PURPOSE: Circumcision is a very common surgical procedure that has been performed for thousands of years. In this paper, we report the long-term results of circumcision performed by using diathermic knife on patients with bleeding diathesis and the amount of blood factors used. MATERIALS AND METHODS: A total of 147 patients with bleeding diathesis circumcised under local anesthesia by using diathermic knife between 1996 and 2010 were recruited into this study. Age of the patients, type of the bleeding diathesis and the treatment protocols were recorded. Postsurgical infection and bleeding rates of 2 different factor replacement protocols were recorded. RESULTS: Mean age of the patients was 11.5 years (range, 1.5-37 years). In the group of protocol 1 applied patients, 3 patients (4%) had bleeding and one patient had infection whereas in group of protocol 2, 4 patients (5.4%) had bleeding. The bleeding cases were taken under control after the factor replacement and elastic bandage. CONCLUSION: Whenever it is necessary for an individual to be circumcised for any reason whatsoever, we think that circumcision can be performed in the patients with bleeding diathesis with lower costs and complication rates by using diathermic knife and the protocol that we used.
Subject(s)
Blood Coagulation Disorders , Circumcision, Male/economics , Circumcision, Male/instrumentation , Diathermy/economics , Diathermy/instrumentation , Adolescent , Adult , Child , Child, Preschool , Circumcision, Male/methods , Cost-Benefit Analysis , Equipment Design , Humans , Infant , Male , Risk Factors , Young AdultABSTRACT
INTRODUCTION: In hemophilia A, factor activity usually correlates with clinical severity; however, there are patients with severe hemophilia who have bleeding less than expected. AIM: The aim of this study is to evaluate the impact of prothrombotic mutations on annual factor consumption in children with hemophilia. METHODS: Factor V Leiden (FVL) G1691A, Prothrombin (PT) G20210A and methylenetetrahydrofolate reductase (MTHFR) C677T and A128C mutations were evaluated in children with moderate-severe hemophilia A (n = 51) and controls (n = 25). RESULTS: None of the cases and controls carried the FVL and PT G20210A in homozygous state. There was no difference in factor consumption between carriers of FVL, PT mutations, and noncarriers. Patients who were homozygous for MTHFR C677T were found to have increased factor consumption compared to noncarriers, and this was a negative association. No decrease in factor consumption was noted in patients with hemophilia having MTHFR A1298C mutation. CONCLUSION: We could not demonstrate a significant decrease in factor concentrate consumption in children with hemophilia having prothrombotic mutations.
