BACKGROUND AND OBJECTIVES: Clinical data for Korean patients with bicuspid aortic valve (BAV) that underwent aortic valve (AV) surgery are currently limited. METHODS: Data for 1,160 consecutive adult BAV patients who underwent AV surgery from 2000 to 2014 in 4 tertiary referral centers were retrospectively analyzed. A standard case report form was used for clinical and echocardiographic parameters. RESULTS: Mean age at the time of AV surgery was 59±13 years. The most common cause of AV surgery was aortic stenosis (AS, 892 [77%]), followed by aortic regurgitation (AR, 199 [17%]), and infective endocarditis (69 [6%]). AS showed a skewed peak in the aged population and was the predominant cause of AV surgery (87%) in patients ≥50 years of age, whereas AR (46%) and active infective endocarditis (19%) were more common in younger patients (p < 0.001). Echocardiographic determination of the BAV phenotype revealed that fusion of the right coronary cusp (RCC) and left coronary cusp (LCC) was most common (622 [53%]), followed by fusion of RCC and non-coronary cusp (NCC) (313 [27%]), and fusion of LCC and NCC (42 [4%]); the BAV phenotype could not be determined in the remaining 183 patients (16%). Fusion of RCC and LCC was more commonly observed in patients with AR than in those with AS (74% vs. 49%; p < 0.001). CONCLUSION: BAV patients were characterized by distinct surgical indications according to their age. Possible associations between BAV phenotypes and surgical indications with potential impacts of ethnicity need to be tested in further studies.
Adult ; Aortic Valve Insufficiency ; Aortic Valve Stenosis ; Aortic Valve ; Bicuspid ; Cardiac Surgical Procedures ; Echocardiography ; Endocarditis ; Heart Valve Diseases ; Humans ; Phenotype ; Retrospective Studies ; Tertiary Care Centers

BACKGROUND AND OBJECTIVES: Clinical data for Korean patients with bicuspid aortic valve (BAV) that underwent aortic valve (AV) surgery are currently limited. METHODS: Data for 1,160 consecutive adult BAV patients who underwent AV surgery from 2000 to 2014 in 4 tertiary referral centers were retrospectively analyzed. A standard case report form was used for clinical and echocardiographic parameters. RESULTS: Mean age at the time of AV surgery was 59±13 years. The most common cause of AV surgery was aortic stenosis (AS, 892 [77%]), followed by aortic regurgitation (AR, 199 [17%]), and infective endocarditis (69 [6%]). AS showed a skewed peak in the aged population and was the predominant cause of AV surgery (87%) in patients ≥50 years of age, whereas AR (46%) and active infective endocarditis (19%) were more common in younger patients (p < 0.001). Echocardiographic determination of the BAV phenotype revealed that fusion of the right coronary cusp (RCC) and left coronary cusp (LCC) was most common (622 [53%]), followed by fusion of RCC and non-coronary cusp (NCC) (313 [27%]), and fusion of LCC and NCC (42 [4%]); the BAV phenotype could not be determined in the remaining 183 patients (16%). Fusion of RCC and LCC was more commonly observed in patients with AR than in those with AS (74% vs. 49%; p < 0.001). CONCLUSION BAV patients were characterized by distinct surgical indications according to their age. Possible associations between BAV phenotypes and surgical indications with potential impacts of ethnicity need to be tested in further studies.

The original version of this article contained wrong informations of some authors which should be changed.

BACKGROUND AND PURPOSE: Nonmotor symptoms (NMS) in Parkinson's disease (PD) have multisystem origins with heterogeneous manifestations that develop throughout the course of PD. NMS are increasingly recognized as having a significant impact on the health-related quality of life (HrQoL). We aimed to determine the NMS presentation according to PD status, and the associations of NMS with other clinical variables and the HrQoL of Korean PD patients. METHODS: We surveyed patients in 37 movement-disorders clinics throughout Korea. In total, 323 PD patients were recruited for assessment of disease severity and duration, NMS, HrQoL, and other clinical variables including demographics, cognition, sleep scale, fatigability, and symptoms. RESULTS: In total, 98.1% of enrolled PD subjects suffered from various kinds of NMS. The prevalence of NMS and scores in each NMS domain were significantly higher in the PD group, and the NMS worsened as the disease progressed. Among clinical variables, disease duration and depressive mood showed significant correlations with all NMS domains (p<0.001). NMS status impacted HrQoL in PD (rS=0.329, p<0.01), and the association patterns differed with the disease stage. CONCLUSIONS: The results of our survey suggest that NMS in PD are not simply isolated symptoms of degenerative disease, but rather exert significant influences throughout the disease course. A novel clinical approach focused on NMS to develop tailored management strategies is warranted to improve the HrQoL in PD patients.
Cognition ; Demography ; Humans ; Korea ; Movement Disorders* ; Parkinson Disease* ; Prevalence ; Quality of Life*

We present a case of 48-year-old male who presented with coronary artery fistula draining into left ventricle. Transthoracic echocardiography showed abnormal blood flow draining into left ventricle, with enlarged coronary arteries and multiple vascular structures around ventricular myocardium. Coronary computed tomography revealed dilatation of entire left coronary artery which was wrapping around left ventricle, and draining into the posterior side of left ventricle. He did not undergo any invasive treatment, because he was not symptomatic.
Coronary Vessels* ; Dilatation ; Echocardiography ; Fistula* ; Heart Ventricles* ; Humans ; Male ; Middle Aged ; Myocardium

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.
Action Potentials ; Axons ; Charcot-Marie-Tooth Disease ; Cohort Studies ; Humans ; Muscles ; Neural Conduction

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.

The serine/threonine kinase Akt has been shown to play a role of multiple cellular signaling pathways and act as a transducer of many functions initiated by growth factor receptors that activate phosphatidylinositol 3-kinase (PI3K). It has been reported that phosphorylated Akt activates eNOS resulting in the production of NO and that NO stimulates soluble guanylate cyclase (sGC), which results in accumulation of cGMP and subsequent activation of the protein kinase G (PKG). It has been also reported that PKG activates PI3K/Akt signaling. Therefore, it is possible that PI3K, Akt, eNOS, sGC, and PKG form a loop to exert enhanced and sustained activation of Akt. However, the existence of this loop in eNOS-expressing cells, such as endothelial cells or astrocytes, has not been reported. Thus, we examined a possibility that Akt phosphorylation might be enhanced via eNOS/sGC/PKG/PI3K pathway in astrocytes in vivo and in vitro. Phosphorylation of Akt was detected in astrocytes after KA treatment and was maintained up to 72 h in mouse hippocampus. 2 weeks after KA treatment, astrocytic Akt phosphorylation was normalized to control. The inhibition of eNOS, sGC, and PKG significantly decreased Akt and eNOS phosphorylation induced by KA in astrocytes. In contrast, the decreased phosphorylation of Akt and eNOS by eNOS inhibition was significantly reversed with PKG activation. The above findings in mouse hippocampus were also observed in primary astrocytes. These data suggest that Akt/eNOS/sGC/PKG/PI3K pathway may constitute a loop, resulting in enhanced and sustained Akt activation in astrocytes.
Animals ; Astrocytes ; Cyclic GMP-Dependent Protein Kinases ; Endothelial Cells ; Guanylate Cyclase ; Hippocampus ; Kainic Acid ; Mice ; Nitric Oxide ; Phosphatidylinositol 3-Kinase ; Phosphorylation ; Phosphotransferases ; Receptors, Growth Factor ; Transducers

BACKGROUND AND OBJECTIVES: The purpose of this study was to investigate 1) the beneficial effect of bosentan treatment (125 mg twice daily) on exercise capacity and echocardiographic variables and 2) the profiles and frequency of adverse events in Korean patients with World Health Organization (WHO) class III or IV pulmonary artery hypertension (PAH). SUBJECTS AND METHODS: Twelve patients who received bosentan treatment were investigated in an open label manner. One patient was excluded in the final analyses due to a prohibited concomitant medication. A 6-minute walk test and echocardiography were performed at baseline and after 12 weeks of treatment. RESULTS: The administration of bosentan for 12 weeks resulted in a significant improvement in exercise capacity (measured with the 6-minute walking distance), WHO functional capacity, and in echocardiographic variables. Bosentan treatment was associated with a decrease in the maximal tricuspid regurgitation jet velocity {from 4.7 m/sec (95% confidence interval, 3.89-5.45) at baseline to 4.4 m/sec (95% confidence interval, 3.61-5.1) at 12 weeks, p=0.03} and systolic pulmonary arterial pressure {from 105 mmHg (95% confidence interval, 74.4-135.6) at baseline to 93 mmHg (95% confidence interval, 66.3-120.1) at 12 weeks, p=0.04}. Treatment with bosentan at a dose of 125 mg twice a day was not associated with life-threatening side effects, although a higher incidence of elevated liver enzymes compared to previous studies was noted. CONCLUSION: Bosentan at a dose of 125 mg twice daily is considered a clinically optimal, safe dose and can be used as a valuable treatment option in Korean PAH patients with WHO functional capacity III or IV, though close monitoring of liver function is required.
Arterial Pressure ; Echocardiography ; Humans ; Hypertension ; Hypertension, Pulmonary ; Incidence ; Liver ; Pulmonary Artery ; Sulfonamides ; Tricuspid Valve Insufficiency ; Walking ; World Health Organization

Paragonimus harinasutai metacercariae were found in a species of freshwater crab, Indochinamon ou, collected in a small stream of Namback District, Luang Prabang Province, Lao PDR. Adult flukes were recovered after experimental infection of the metacercariae to dogs. Metacercariae were round or slightly elliptical, 0.666 x 0.626 mm in average size, and had a thin cyst wall of about 20 microm in thickness, a black excretory bladder, convoluted ceca, and some pinkish materials in the body. Adults were somewhat elongated, 95.2 x 36.5 mm in average size, covered with single-tipped tegumental spines, had a smaller oral sucker than the ventral sucker, a moderately branched ovary, and 5-6 lobulated testes. Eggs were ovoid and bilaterally symmetrical in shape, 79 x 45 microm in average size, and had a uniformly thickened shell. By the present study, it has been confirmed that I. ou is a new second intermediate host for P. harinasutai.
Animals ; Crustacea/*parasitology ; Disease Reservoirs/*parasitology ; Dogs ; Female ; *Host-Parasite Interactions ; Humans ; Laos ; Male ; Paragonimiasis/*parasitology ; Paragonimus/anatomy & histology/*isolation & purification/ultrastructure ; Shellfish/*parasitology