Subject(s)
Eosinophilia/complications , Gastroenteritis/complications , Skin Abnormalities/diagnosis , Biopsy , Child, Preschool , Colonoscopy/methods , Diagnosis, Differential , Eosinophilia/diagnosis , Face , Female , Gastroenteritis/diagnosis , Humans , Intestinal Mucosa/pathology , Skin Abnormalities/etiologyABSTRACT
: Angiodysplastic (AD) lesion is the most common cause of recurrent gastrointestinal (GI) bleeding in inherited Von Willebrand disease (VWD) patients lacking high-molecular-weight multimers. Defect or dysfunction of von Willebrand factor (VWF) may lead to enhanced endothelial cell proliferation followed by the development of neoangiogenesis and vascular malformation, which result in severe bleeding. Recurrent bleeding causing by GI AD is a challenging complication of VWD. The management of VWD could be difficult due to frequent recurrence and severity of bleeding episodes. The primary aim of management is not only to stop but also to prevent bleeding. We present two patients of type 3 VWD associated with AD and severe GI bleeding, which were successfully treated by endoscopic coagulation and prophylactic therapy with different regimens of plasma-derived VWF/factor VIII (pdVWF/FVIII) concentrate to maintain a trough level in the patient unresponsive to standard treatment.
Subject(s)
Angiodysplasia/complications , Gastrointestinal Hemorrhage/complications , von Willebrand Disease, Type 3/complications , Adult , Angiodysplasia/therapy , Drug Combinations , Endoscopy, Gastrointestinal , Factor VIII/therapeutic use , Gastrointestinal Hemorrhage/therapy , Humans , Male , Middle Aged , von Willebrand Disease, Type 3/therapy , von Willebrand Factor/therapeutic useSubject(s)
Klippel-Trenaunay-Weber Syndrome/diagnosis , Abdominal Wall , Child, Preschool , Humans , VeinsSubject(s)
Protein S Deficiency , Tomography, X-Ray Computed , Vena Cava, Inferior , Venous Thromboembolism , Adolescent , Female , Humans , Protein S Deficiency/complications , Protein S Deficiency/diagnostic imaging , Protein S Deficiency/drug therapy , Vena Cava, Inferior/abnormalities , Vena Cava, Inferior/diagnostic imaging , Venous Thromboembolism/diagnostic imaging , Venous Thromboembolism/drug therapy , Venous Thromboembolism/etiologyABSTRACT
There has been an ongoing debate as to whether hemophilia A (HA) is more severe than hemophilia B (HB), and there are studies supporting each side of the argument. The study aimed to investigate whether any differences in major bleeding events exist between patients with severe HA and HB. A nationwide, population-based retrospective cohort study using the National Health Insurance Research Database was conducted. We compared 658 patients with severe HA and 137 patients with severe HB without inhibitors from 1997 to 2013, during the period when adult patients older than 18 years old were treated with the on-demand therapy since birth. There was no significant difference between patients with severe HA and HB in the rate of major bleeding events, with an adjusted relative ratio of 0.79 (95% confidence interval [CI]: 0.36-1.71, P = .548). There was also no significant difference in the incidence rate of major bleeding events between adult patients with HA and HB with the on-demand therapy, and an adjusted hazard ratio (HR) of 0.82 (95% CI: 0.65-1.02). However, patients with HA had a lower incidence rate of intracranial hemorrhage, with an adjusted HR of 0.44 (95% CI: 0.25-0.79). In addition, no significant difference in the frequency of major bleeding events requiring hospitalization between patients with HA and HB was found, P > .05. In conclusion, the study demonstrated that patients with severe HB encountered a similar rate of major bleeding events to those with severe HA.
