• Journal of Interdisciplinary Genomics
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• v.2 no.1
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• pp.5-9
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• 2020

von Willebrand disease (VWD) is a genetic bleeding disorders caused by a deficiency of von Willebrand factor (VWF). Diagnosis or exclusion of VWD is not an easy task for most clinicians. These difficulties in diagnosis or exclusion of VWD may be due to preanalytic, analytical and postanalytic laboratory issues. Analytical systems to diagnose VWD may produce misleading results because of limitations in their dynamic range of measurement and low sensitivity. However, preanalytical issues such as sample collection, processing, and transportation affect the diagnosis of VWD profoundly. We will review here the common preanlytical issues that may impact the laboratory diagnosis of VWD.

• Journal of Veterinary Clinics
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• v.30 no.5
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• pp.359-362
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• 2013

A 6-year-old castrated male Maltese weighing 4.16 kg presented with a history of bleeding from the mouth after scaling and dental extraction 2 days prior. The von Willebrand factor antigen (vWf:Ag) level was 54% (reference range, 70-180%), indicating that the dog was homozygous for an inherited von Willebrand disease (vWD). The dog received whole-blood transfusion as initial treatment, consequently showing markedly improved clinical signs, and is currently in good condition. To our knowledge, this is the first reported case of type 1 vWD in a Maltese in Korea.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.30 no.1
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• pp.41-51
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• 2008

Mucoepidermoid carcinoma (MEC) is the most common malignant salivary gland tumor which compromises about 6$\sim$8% of all tumors followed by the adenoid cystic carcinoma (ACC) and adenocarcinoma. Most deaths from salivary carcinomas are caused by recurrent or metastatic lesions that are resistant to conventional therapy. Therefore, knowledge of cellular properties and tumor-host interactions that influence the vascular metastasis is important for the design of more effective therapy of salivary carcinomas. Neoangiogenesis is essential for tumor growth, which is postulated to be fundamentally dependent on the induction of stromal neovascularization. However, how neovascularization takes place in live tissue has not been fully established, especially in recruitment and differentiation of endothelial cells in the salivary gland tumors. Vascular endothelial growth factor (VEGF) is a heparin-binding, dimeric polypeptide growth factor known to exert its mitogenic activity specifically on endothelial cells. VEGF has been shown th be directly involved in angiogenesis, which in essential for the pathogenesis of many solid tumors. von Willebrand factor (vWF) is a large multimeric protein synthesized by megakaryocytes and endothelial cells that enable platelets to adhere to exposed subendothelium and, as well, to respond to changes in the blood flow. Recent studies suggest that increased levels of vWF correlate with progression of disease, metastasis, or survival time and thus may have a prognostic significance. vWF is explained as an acute phase proteins which is increased in cancer or as a result of increased endothelial cell synthesis associated with tumor-induced angiogenesis. Due to adhesive properties of vWF, its increased concentrations may also contribute metastasis of tumor. In this study, we determined the mRNA expression of VEGF and vWF in salivary ACC, MEC and pleomorphic adenoma by in situ hybridization. As a result, stronger expression of VEGF and vWF was seen in salivary ACC and MEC which has more invasive nature than the salivary benign tumor.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.126-132
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• 2005

Glanzmann's thrombasthenia is an autosomal recessively inherited hemorrhagic disorder that results from quantitative and qualitative abnormalities in platelet membrane glycoprotein IIb-IIIa, also known as ${\alpha}_{IIb}{\beta}_3$ integrin which is an adhesion receptor for fibrinogen and von Willebrand factor. We describe here a 4-year-old girl who had Glanzmann's thrombasthenia with the ${\beta}_3$ subunit missense mutation.

• Korean Journal of Veterinary Service
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• v.30 no.4
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• pp.539-544
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• 2007

A 7-year-old intact female Golden Retriever was presented for examination. The dog had large irregular subcutaneous masses in the abdomen which were ruptured or encapsulated. Those were removed surgically. Histopathologically, the masses consisted of spindle cells that often formed distinct whorls around a central capillary. Immuno-histochemical analysis revealed that the neoplastic cells were strong diffuse cyto-plasmic immunolabelling for vimentin and focal immunoreactivity for smooth muscle actin, whereas not immunoreactive for cytokeratin, desmin, von Willebrand factor, glial fibrillary acidic protein, or S-100. The neoplastic cells ultrastructurally had processes attached by desmosome-like structures, swollen mitochondria and dilated rough endoplasmic reticulum. Based on the above results, this case was diagnosed as a canine hemangiopericytoma in the abdominal subcutis of a Golden Retriever.

• Journal of Korean Neurosurgical Society
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• v.58 no.2
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• pp.137-140
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• 2015

The most common neurologic manifestations of polycythemia vera (PV) are cerebral infarction and transient ischemic attacks, while cerebral hemorrhage or intracranial dissection has been rarely associated with PV. Here we report the first case of a 59-year-old patient with intracranial supraclinoid internal carotid artery (ICA) dissection causing cerebral infarction and concomitant subarachnoid hemorrhage due to pseudoaneurysm rupture as clinical onset of PV. This case report discusses the possible mechanism and treatment of this extremely rare condition.

• Proceedings of the Korean Society of Veterinary Clinics Conference
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• 2006.05a
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• pp.170-170
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• 2006

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.13 no.1
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• pp.23-29
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• 2010

Purpose: The aim of this study was to assess the benefits of routine pre-endoscopy coagulation screening tests and platelet counts in Korean children. Methods: Between March 2004 and December 2009, children who underwent gastrointestinal endoscopy for the evaluation of various gastrointestinal symptoms were included. All of the subjects included in the study also underwent routine coagulation screening and platelet count determinations prior to endoscopy and biopsy. The clinical records and laboratory tests were retrospectively reviewed in all patients. Results: One hundred sixty-two of 1,476 (11%) patients who underwent endoscopy had abnormal results on pre-screening coagulation tests. Fourteen patients underwent coagulation factor assays due to abnormal clotting results in consecutive tests or due to clinical evidence of a bleeding tendency. Seven patients were diagnosed with factor XII deficiency, one patient was diagnosed with von Willebrand disease, one patient had von Willebrand disease and factor XII deficiency, and one patient was presumed to have mild hemophilia. The remaining 4 patients had normal results with the factor assays. The results of platelet counts were normal with the exception of 1 patient. No patient had significant bleeding during the endoscopic procedures, despite abnormal pre-endoscopic coagulation tests. Conclusion: Routine coagulation screening tests and platelet counts revealed abnormal results in some patients. Most of the patients with abnormal clotting were shown to have a factor XII deficiency, which had no significant associated bleeding tendencies; the other patients were diagnosed with hemophilia or von Willebrand disease. Therefore, although abnormal pre-endoscopic coagulation is not always related to significant bleeding complications, pre-endoscopic coagulation screening may be useful in some children in predicting the risk of bleeding tendency during endoscopic procedures.

• The Korean Journal of Physiology and Pharmacology
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• v.19 no.1
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• pp.35-42
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• 2015

In cardiovascular disorders, understanding of endothelial cell (EC) function is essential to elucidate the disease mechanism. Although the mouse model has many advantages for in vivo and in vitro research, efficient procedures for the isolation and propagation of primary mouse EC have been problematic. We describe a high yield process for isolation and in vitro culture of primary EC from mouse arteries (aorta, braches of superior mesenteric artery, and cerebral arteries from the circle of Willis). Mouse arteries were carefully dissected without damage under a light microscope, and small pieces of the vessels were transferred on/in a Matrigel matrix enriched with endothelial growth supplement. Primary cells that proliferated in Matrigel were propagated in advanced DMEM with fetal calf serum or platelet-derived serum, EC growth supplement, and heparin. To improve the purity of the cell culture, we applied shearing stress and anti-fibroblast antibody. EC were characterized by a monolayer cobble stone appearance, positive staining with acetylated low density lipoprotein labeled with 1,1'-dioctadecyl-3,3,3',3'-tetramethyl-indocarbocyanine perchlorate, RT-PCR using primers for von-Willebrand factor, and determination of the protein level endothelial nitric oxide synthase. Our simple, efficient method would facilitate in vitro functional investigations of EC from mouse vessels.

• Journal of Nutrition and Health
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• v.35 no.1
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• pp.5-14
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• 2002

Alteration in the syntesis or enhanced inactivation of nitric oxide(NO) can induce impairment of endothelial cell function. Insulin dependent diabetes mellitus(IDDM) is characterized by impaired endothelial function and vascular disease. NO is produced through L-arginine pathway To elucidate the hypothesis that the decreased production on NO in IDDM reflects vascular damage and the NO production can be manipulated by either dietary fat(7% of kg diet) or the oral supplementation with L-arginine(2g/kg bw), plasma markers for vascular endothelial damage and plasma lipid profiles were measured in streptozotocin(STZ)-induced diabetic rats. Diabetic or normal Sprague-Dawley rats were fed 6 different experimental diets for 4 weeks(SO : soybean oil, SOA: soybean oil + L-arginine supplementation, BT : beef tallow, BTA_ beef tallow + L-arginine supplementation, OV olive oil, OVA : olive oil + L-arginine supplementation). Plasma glucose, total cholesterel, HDL-cholesterol, LDL-cholesterol and triglyceride were measured. Endothelial markers, plasma von Willebrand factor(vWf), thromboxane B$_2$, and 6-keto PGF1$\alpha$ of aorta were measured by ELISA. Plasma NO production was evaluated through the measurement of nitrite by EIA. Feeding saturated fatty acid(SFA, BT) increased relative liver size(RLS) in diabetic rats compared to either polyunsatunted fatty acid(PUFA, SO) or monounsaturated fatty acid(MUFA, OV) The supplementation of L-arginine inhibited the liver and kidney enlargement in olive oil find diabetic rats. Plasma glucose was lower in diabetic animal find the olive oil compared to fed beef tallow and the supplementation L-arginine decreased it in diabetic rats find beef tallow significantly(p < 0.05). Plasma TXB$_2$ levels were increased due to diabetes and the value of beef tallow group showed highest value. Plasma vWf concentration of beef tallow group was higher value in normal rats and was elevated more in diabetes. In diabetic groups, the vWf concentration of olive oil group was lower than beef tallow or soybean oil group. The supplementation of L-arginine in diabetic rats decreased plasma TXB$_2$ and vWf levels significantly(p < 0.05). NO production was higher in normal olive oil fed rats and was tend to be decreased in diabetic rats and the supplementation of L-arginine recovered to normal value(p < 0.05), Olive oil supplemented with L-arginine tended to lower plasma total cholesterol and LDL-cholesterol after 4 week treatment. These results suggest that generalized vascular endothelial changes based on plasma TXB$_2$and vWf occurs in diabetic rats. and olive oil with L-arginine supplementation contributes to a better control of the hyperglycemia, endothelial changes and hypercholesterolemia accompanying diabetes as compared with beef tallow or soy bean oil in this rat model.