Background: Hemophilia A (HA) treatment strategies aim to manage bleeding episodes and improve patients' quality of life. This study investigates the effectiveness of a preventative approach using intermediate-dose prophylaxis with standard half-life FVIII products in reducing bleeding rates and enhancing the quality of life for patients with severe HA.
Methods: A 4-year prospective longitudinal study followed 35 patients with severe HA (without FVIII inhibitors) who transitioned from a reactive treatment approach to intermediate-dose prophylaxis in Taiwan from 2014 until 2018.
Introduction: Emicizumab mimicking the cofactor function of activated factor VIII (FVIII) restores haemostasis.
Methods: This nationwide observational study aimed to retrospectively investigate efficacy, safety, and cost in 1 year before and up to 3 years after emicizumab prophylaxis for haemophilia A (HA) patients with FVIII inhibitors.
Results And Discussion: A total of 39 severe HA patients with a median age of 23.
Background: Hesperetin has been reported to have anticancer properties. However, the molecular mechanisms underlying its action on leukemia cells remain unclear. This in vitro study evaluated the possible mechanisms of hesperetin in leukemia cells (HL-60 and U937).
View Article and Find Full Text PDFRationale: Hereditary spherocytosis (HS) has a defect in the vertically connected proteins on the cell membrane of red blood cells (RBC). Hereditary elliptocytosis (HE) has a defect in proteins that connect the cell membrane horizontally. We reported two families of RBC membrane disorders in Taiwanese, one was HS and the other was HE.
View Article and Find Full Text PDFVery few studies have shown the real origin and timing of de novo variants (DNV) implicated in von Willebrand disease (VWD). We investigated four families with type 2 VWD. First, we conducted linkage analysis using single nucleotide variant genotyping to recognize the possible provenance of DNV.
View Article and Find Full Text PDFIntroduction: Liver health is essential for persons with hemophilia (PWH) in order to maintain access to new therapies, such as gene therapy. Non-alcoholic fatty liver disease (NAFLD) is seldom reported in the hemophilia population. The study aimed to investigate the prevalence of NAFLD and associated factors in PWH.
View Article and Find Full Text PDFCongenital coagulation factor V deficiency (FVD) is a rare, autosomal recessive bleeding disorder. We characterized the clinical presentations, laboratory features, and genetic alterations of Taiwanese patients with FVD. From 1983 to 2010, five women, one man, and one boy diagnosed with FVD were enrolled in this study.
View Article and Find Full Text PDFBackground: Factor XII (FXII) deficiency is an interesting condition that causes prolonged activated partial thromboplastin time without bleeding diathesis. FXII may be not important in hemostasis, but still plays roles in thrombosis and inflammation. In order to raise clinical awareness about this condition, we studied patients with severe FXII deficiency and their relatives.
View Article and Find Full Text PDFBackground/purpose: Postoperative venous thromboembolism is an important complication in Taiwan. We prospectively investigated the occurrence of deep vein thrombosis (DVT) after major orthopedic surgery without pharmacologic thromboprophylaxis in a cohort of 120 patients (46 males, 74 females, median age 71 years) at our institute.
Methods: Color duplex compression ultrasonography (CUS) was used to detect DVT before and after the operation, while contrast venography was performed postoperatively for comparison and validation.
Introduction: Congenital fibrinogen disorders (CFDs) are caused by mutations in fibrinogen-encoding genes, FGA, FGB, and FGG, which lead to quantitative or qualitative abnormalities of fibrinogen. Although the diagnosis of CFDs is based on antigenic and functional level of fibrinogen, few genotypes are clearly correlated with phenotype.
Methods: In this study, we investigated all of the referred patients diagnosed as CFDs in Taiwan's population between 1995 and 2020.
Inferior vena cava thrombosis (IVCT) is rare and can be under-recognized. However, the associated complications and mortality may be severe. We report the first case series of IVCT observed in Taiwan with a brief literature review.
View Article and Find Full Text PDFCongenital thrombotic thrombocytopenic purpura (cTTP) is a rare disease that is defined as biallelic mutations of ADAMTS13 causing persistent absence of ADAMTS13 activity. The confirmed diagnosis requires a genetic study, and cTTP has never been previously reported in Taiwan. Our patient was a 29-year-old Taiwanese woman who presented with severe hyperbilirubinemia at birth.
View Article and Find Full Text PDFA 66-year-old woman had experienced abnormal bleeding since the age of 7. Thrombocytopenia was not detected until she was 48, and immune thrombocytopenia was diagnosed at age 66. She also reported experiencing hearing disturbance since the age of 30 and acute renal failure since the age of 61 but reported no visual disturbance.
View Article and Find Full Text PDFIntroduction: Acquired factor XIII (FXIII) inhibitor is a rare but possibly underdiagnosed bleeding disorder. To date, less than one hundred cases have been reported, but the number has increased rapidly in recent years, especially in Japan. Because of the rarity of this disorder, no treatment guidelines are available.
View Article and Find Full Text PDFHereditary coagulation factor XI (FXI) deficiency is a rare bleeding disorder, but information on FXI deficiency in Taiwanese patients remains scarce. We evaluated clinical and genetic features of severe FXI deficiency patients in Taiwan. We collected clinical information and performed coagulation laboratory tests and genetic studies in ten unrelated Taiwanese families with severe FXI deficiency.
View Article and Find Full Text PDFOral submucous fibrosis (OSF) is an oral precancerous condition associated with the habit of areca nut chewing and the TGF-β pathway. Currently, there is no curative treatment to completely heal OSF, and it is imperative to alleviate patients' symptoms and prevent it from undergoing malignant transformation. Arctigenin, a lignan extracted from , has been reported to have a variety of pharmacological activities, including anti-fibrosis.
View Article and Find Full Text PDFAims/hypothesis: Chronic kidney disease (CKD) is a known complication of diabetes mellitus, and insulin resistance is a well-known complication of CKD. However, there is no consensus in the published data on the association of CKD with incident diabetes.
Methods: A total of 15,403 people with CKD were identified from the Taiwan National Health Insurance Research Database to determine their risk of incident diabetes compared with that of 15,403 matched individuals without CKD.
Developments in computational chemistry, bioinformatics, and laboratory evolution have facilitated the de novo design and catalytic optimization of enzymes. Besides creating useful catalysts, the generation and iterative improvement of designed enzymes can provide valuable insight into the interplay between the many phenomena that have been suggested to contribute to catalysis. In this work, we follow changes in conformational sampling, electrostatic preorganization, and quantum tunneling along the evolutionary trajectory of a designed Kemp eliminase.
View Article and Find Full Text PDFBackground: Hydroxychloroquine (HCQ), one of the disease-modifying antirheumatic drugs, may lead to an inhibition of autophagy. Autophagy, an intracellular self-defense mechanism for the lysosomal degradation of cytoplasmic components such as damaged organelles, plays a role in protecting against neoplasm growth but is also vital for cancer cells due to an increased intracellular metabolic waste.
Methods: Taiwan National Health Insurance Database was subjected to analysis to investigate the effect of HCQ exposure on cancer risk in patients with autoimmune diseases.
Background: Health burdens of hepatocellular carcinoma (HCC) are emerging quickly in the world, including in Taiwan. Surgical resection has been recognized as the first-line treatment for early tumors. This study aimed to investigate the prognostic risk factors for mortality and recurrence rate in Taiwan, which has a high prevalence of chronic viral hepatitis.
View Article and Find Full Text PDF© LitMetric 2025. All rights reserved.