• Biomolecules & Therapeutics
• /
• v.28 no.1
• /
• pp.98-103
• /
• 2020

Marfan syndrome (MFS), a connective tissue disorder caused by mutations in the fibrillin-1 (Fbn1) gene, has vascular manifestations including aortic aneurysm, dissection, and rupture. Its vascular pathogenesis is assumed to be attributed to increased transforming growth factor β (TGFβ) signaling and blockade of excessive TGFβ signaling has been thought to prevent dissection and aneurysm formation. Here, we investigated whether galunisertib, a potent small-molecule inhibitor of TGFβ receptor I (TβRI), attenuates aneurysmal disease in a murine model of MFS (Fbn1^C1039G/+) and compared the impact of galuninsertib on the MFS-related vascular pathogenesis with that of losartan, a prophylactic agent routinely used for patients with MFS. Fbn1^C1039G/+ mice were administered galunisertib or losartan for 8 weeks, and their ascending aortas were assessed for histopathological changes and phosphorylation of Smad2 and extracellular signal-regulated kinase 1/2 (Erk1/2). Mice treated with galunisertib or losartan barely exhibited phosphorylated Smad2, suggesting that both drugs effectively blocked overactivated canonical TGFβ signaling in Fbn1^C1039G/+ mice. However, galunisertib treatment did not attenuate disrupted medial wall architecture and only partially decreased Erk1/2 phosphorylation, whereas losartan significantly inhibited MFS-associated aortopathy and markedly decreased Erk1/2 phosphorylation in Fbn1^C1039G/+ mice. These data unexpectedly revealed that galunisertib, a TβRI inhibitor, showed no benefits in aneurysmal disease in MFS mice although it completely blocked Smad2 phosphorylation. The significant losartan-induced inhibition of both aortic vascular pathogenesis and Smad2 phosphorylation implied that canonical TGFβ signaling might not prominently drive aneurysmal diseases in MFS mice.

• Korean Journal of Clinical Laboratory Science
• /
• v.53 no.1
• /
• pp.131-136
• /
• 2021

Duplex sonography is used widely in various medical fields because of its repeatability and low cost. In particular, the carotid duplex sonography is a useful non-invasive test for diagnosing cerebrovascular disease and predicting the prognosis. In clinical practice, it is very important to reduce the test time and improve accuracy. The patient's clinical information must be known in advance to perform carotid duplex sonography quickly and accurately. Despite this, there are often difficulties finding new cervical vascular diseases that are not mentioned in the clinical information. Therefore, knowing a variety of cases can lead to fast and accurate results. In this context, this paper reports three cases of cervical vascular disease discovered unexpectedly during carotid duplex sonography: CASE 1, internal carotid artery occlusion and cerebral arteries branched from the external carotid artery; CASE 2, internal jugular vein thrombosis; CASE 3, microembolism observed in the vertebral artery.

• BMB Reports
• /
• v.51 no.2
• /
• pp.79-84
• /
• 2018

Niemann-Pick type C disease (NP-C) is a fatal neurodegenerative disorder caused by a deficiency of NPC1 gene function, which leads to severe neuroinflammation such as astrogliosis. While reports demonstrating neuroinflammation are prevalent in NP-C, information about the onset and progression of cerebellar astrogliosis in this disorder is lacking. Using gene targeting, we generated vascular endothelial growth factor (VEGF) conditional null mutant mice. Deletion of VEGF in cerebellar Purkinje neurons (PNs) led to a significant increase of astrogliosis in the brain of NP-C mice in addition to the loss of PNs, suggesting PN-derived VEGF as an important factor in NP-C pathology. Moreover, replenishment of VEGF in neurons improved brain pathology in NP-C mice. Overall, our data provide a new pathological perspective on cerebellar astrogliosis in NP-C and suggest the importance of VEGF as a therapeutic target for this disease.

• Journal of Chest Surgery
• /
• v.25 no.11
• /
• pp.1192-1202
• /
• 1992

Postoperative cardiac outputs and other hernoaynamic values were serially measured in fifteen patients of cyanotic congenital heart disease, after use of blood cardioplegic solution. Cardiac indices showed no change untill eight postoparative hours, then it began to decline to reach as low as 3.22$\pm$0.7L/min/m2 at 12 hours. After then gradual increse occ-ured to recover upto immediate postoperative value at 20 hours. Sharp decrese of heart rate and increse of systemic vascular resistance during 8~16 hours and steady increase of stroke indices during the whole study periods were observed. These observations suggested that the myocardium recovered gradually after open heart surgery, and that the decrease of cardiac indices during 8~16 hours could be a result of decrease of heart rates and increase of afterload. The changes of cardiac indices correlated with the changes of heart rate, postoperative time and mixed venous oxygen saturation [p<0.05]. No other hemodynamic values found to be in statistically significant correlation with the changes of cardiac indices. Left ventricular dysfunction seemed to occur more frequently during 8~12 hours, but it was not statistically significant. [p=0.73] In conclusion, great care must be taken during 2~3 days after the operation of cyanotic congenital heart disease, not to fall into a low cardiac output state, by maintaing adequate heart rates and reducing afterload especially when the systemic vascular resistance increases.

• Research in Plant Disease
• /
• v.9 no.1
• /
• pp.36-38
• /
• 2003

Stem blight of brunfelsia (Brunfelsia calycina) caused by Fusarium oxysporum was found in greenhouse around Sungnam area, Kyunggi province, Korea in September 2001. The initial infection appeared as a slight wilting of the foliage, turned yellow from the lower leaves. The yellowing leaves were falled, resulting in blight of stem and eventual death of the entire plant. The vascular tissue of a diseased plants became dark brown and browning of the vascular system was a characteristic of the disease and the pith remained healthy, Isolates obtained from the lesions of the diseased plant were identified as F. oxysporum, based on the morphological characteristics of conidia. Symptom by artificial inoculation was same to the symptom of naturally infected plants. This is the first report demonstrating the stem blight on a brunfelsia caused by F. oxysporum in Korea, and we proposed to name this disease "stem blight of brunfelsia".

• Asian Pacific Journal of Cancer Prevention
• /
• v.15 no.1
• /
• pp.175-178
• /
• 2014

Background: Resection rates of lung cancer are low in general and especially in countries like Nepal. Advanced stage at presentation and poor general condition of the patient are the usual causes. Materials and Methods: In this prospective observational study, one hundred cases of lung cancer who presented at the Thoracic Surgery Unit between October 2011 and October 2012 were included. Results: Those aged in the $6^{th}$ and $7^{th}$ decades together accounted for 72/100 patients. The male to female ratio was 2:1. There was a mean-$29.2{\pm}14.2$ pack yrs smoking history with only five non-smokers. Seventy-six patients presented with locally advanced disease while 21 had metastases. Only three had local disease. The average time between onset of symptoms to first contact with a doctor was $2.3{\pm}5.3$ months (range: 0-35.6 months). Average time between first contact to referral was $50.4{\pm}65.7$ days (range-0-365). Only three patients were resected, one after neo-adjuvant chemotherapy. Advanced disease was the cause of unresectability in 95 cases. One of three patients with local disease had pulmonary functions allowing the warranted resection. $N_2$ disease with $T_{1-3}$ on CT scan was found in 47. Three of these patients underwent mediastinoscopy and all confirmed uninvolved $N_2$. Conclusions: Lung resection rates in our center remain low. Late presentation leading to advanced disease and poor pulmonary reserves preclude resection in most cases. More liberal use of mediastinal staging and better assessment of pulmonary functions may allow us to improve resection rates.

• Integrative Medicine Research
• /
• v.3 no.4
• /
• pp.204-210
• /
• 2014

The aim of this review was to understand the effects of ${\beta}$-adrenergic stimulation on oxidative stress, structural remodeling, and functional alterations in the heart and cerebral artery. Diverse stimuli activate the sympathetic nervous system, leading to increased levels of catecholamines. Long-term overstimulation of the ${\beta}$-adrenergic receptor (${\beta}AR$) in response to catecholamines causes cardiovascular diseases, including cardiac hypertrophy, stroke, coronary artery disease, and heartfailure. Although catecholamines have identical sites of action in the heart and cerebral artery, the structural and functional modifications differentially activate intracellular signaling cascades. ${\beta}AR$-stimulation can increase oxidative stress in the heart and cerebral artery, but has also been shown to induce different cytoskeletal and functional modifications by modulating various components of the ${\beta}AR$ signal transduction pathways. Stimulation of ${\beta}AR$ leads to cardiac dysfunction due to an overload of intracellular $Ca^{2+}$ in cardiomyocytes. However, this stimulation induces vascular dysfunction through disruption of actin cytoskeleton in vascular smooth muscle cells. Many studies have shown that excessive concentrations of catecholamines during stressful conditions can produce coronary spasms or arrhythmias by inducing $Ca^{2+}$-handling abnormalities and impairing energy production in mitochondria, In this article, we highlight the different fates caused by excessive oxidative stress and disruptions in the cytoskeletal proteome network in the heart and the cerebral artery in responsed to prolonged ${\beta}AR$-stimulation.

• Journal of Chest Surgery
• /
• v.50 no.5
• /
• pp.378-381
• /
• 2017

Patients with an atrial septal defect (ASD) and severe pulmonary arterial hypertension (PAH) are considered ineligible for defect closure surgery because of the risk of right ventricular decompensation and death after the operation. We report the case of a patient with large ASD and severe PAH who was able to undergo defect closure surgery successfully following long-term use of combined oral sildenafil and beraprost.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.23 no.1
• /
• pp.72-78
• /
• 2020

Infantile hepatic hemangioma, the most common vascular tumor of the liver in infancy, can occur with acute postnatal liver and congestive heart failure. Nevertheless, its course is often benign, and many children can be diagnosed and treated without surgical intervention. The distinction from malignant diseases is not always easy and it not clear whether invasive procedures for diagnosis and therapy should be performed. Here we report our experiences in our Center for Pediatric Liver Disease and postulate that large studies are needed to avoid unnecessary invasive procedures for these patients in the future.

• Anatomy and Cell Biology
• /
• v.56 no.1
• /
• pp.155-159
• /
• 2023

Studies describing the vascular systems and their variations in Situs inversus totalis (SIT) from a whole-body computed tomographic (CT) angiography perspective are lacking. We report a case of SIT in which postmortem CT angiography (PMCTA) was performed as a part of the forensic death investigation and incidentally detected several vascular variations in it. The PMCTA procedure was performed using the multiphase PMCTA protocol. Almost all major vessels were visualized, indeed in a completely reversed pattern. Contrast mixture flow interruptions were noted in the right coronary arterial branches suggesting possible blockage, upon which autopsy revealed >90% vessel occlusions at several locations. As such the cause of death was due to ischemic heart disease. Anomalous origins of the right internal mammary artery; abnormal left thyrocervical trunk and variations in the drainage of testicular veins were noted. Our findings might be helpful to clinicians and add to the body of literature on SIT.