BACKGROUND: Beta-adrenergic receptor Kinase 1(betaARK1) is a serine/threonine kinase attached, which inhibits the coupling of beta-adrenergic receptor with G-protein. Myocardial betaARK1 level is usually elevated in heart failure and hypertrophy, but it is not known whether the circulating betaARK1 level is related with the degree of cardiac hypertrophy. This study was performed to evaluate the association of the betaARK1 level in circulating mononuclear leukocytes(MNL) in untreated hypertension with left ventricular mass in hypertensive patients. Method: Nineteen non-treated hypertensive patients were included for this study. High blood pressure was confirmed when systolic BP is over 150 mmHg or diastoli BP is over 95 mmHg. Echocardiography was performed to evaluate the degree of hypertrophy by measuring the left ventricular mass index(LVMI) and relative wall thickness(RWT), and test the LV function by measuring the ejection fraction(EF) according to ASE guideline. At the same time, blood was collected from each patient and MNL were isolated by gradient centrifuge with Ficoll-400. Total RNA was purified from MNL and semi-quantitative RT-PCR was performed. After reverse transcription, PCR was done with primers for human betaARK1 and GAPDH as external control. betaARK1 levels were expressed by ratio to GAPDH level and estimated the relations with clinical and Echocardiographic parameters. Result: We studied confirmed 19 hypertensive patients(10 men and 9 women, mean age of 50.6 years). Echocardiographically measured indices(mean+/-SD) were as follows; LVMI(137.3+/-30.6g/m2), PWT(0.53+/-0.09) and EF(54.6+/-8.5%). Ratio of betaARK1 levels to GAPDH was from 0.10 to 0.96 (0.62+/-0.25). betaARK1 levels were correlated with LVMI(correlation coefficient: r=.502, p=.029) and RWT(r=.627, p=.004). But Systolic BP(r=0.009, p=.93), diastolic BP(r=.07, p=.85) or EF(r=.045, p=.84) were not related to level of betaARK1. CONCLUSIONS: The betaARK1 level of circulating MNL was correlated well with the degree of the cardiac hypertrophy estimated by LVMI and RWT. This data suggests that activation of sympatho-adrenal system would exert a major role in developing cardiac hypertrophy and we can expect the decreased responsiveness to catecholamine in the heart of hypertensive patients. betaARK1 in circulating MNL might be used as a predictor or marker for LV hypertrophy in hypertensive patients.
beta-Adrenergic Receptor Kinases ; Cardiomegaly ; Echocardiography ; Female ; GTP-Binding Proteins ; Heart ; Heart Failure ; Humans ; Hypertension ; Hypertrophy ; Leukocytes, Mononuclear* ; Male ; Phosphotransferases ; Polymerase Chain Reaction ; Reverse Transcription ; RNA

No abstract available.
Antiphospholipid Syndrome* ; Lupus Erythematosus, Systemic* ; Spine* ; Venous Thrombosis*

No abstract available.
Antiphospholipid Syndrome* ; Lupus Erythematosus, Systemic* ; Spine* ; Venous Thrombosis*

BACKGROUND: Trichorhinophalangeal syndrome (TRPS) patients tend to have alopecia that appears to be androgenetic, and this genetic model might give clues to the pathogenesis of hair loss or hair morphogenesis. OBJECTIVE: This study was conducted to identify additional genetic evidence of TRPS and hair morphogenesis from a TRPS patient. METHODS: From one TRPS type I patient, we extracted RNA and profiled whole transcriptome in non-balding and balding scalp areas using high-throughput RNA sequencing. RESULTS: We found a total of 26,320 genes, which comprised 14,892 known genes with new isoforms and 4,883 novel genes from the non-balding and balding areas. Among these, a total of 1,242 genes showed different expression in the two scalp areas (p<0.05 and log2 fold-change >0). Several genes related to the skin and hair, alopecia, and the TRPS1 gene were validated by qRT-PCR. Twelve of 15 genes (KRT6C, KRTAP3-1, MKI67, GPRC5D, TYRP1, DSC1, PMEL, WIF1, SOX21, TINAG, PTGDS, and TRPS1) were down-regulated (10 genes: p<0.01; SOX21 and PTGDS: p>0.05), and the three other genes (HBA2, GAL, and DES) were up-regulated (p<0.01) in the balding scalp. Many genes related to keratin and hair development were down-regulated in the balding scalp of the TRPS type I patient. In particular, the TRPS1 gene might be related to androgen metabolism and hair morphogenesis. CONCLUSION: Our result could suggest a novel perspective and evidence to support further study of TRPS and hair morphogenesis.
Alopecia ; Gene Expression Profiling* ; Hair ; High-Throughput Nucleotide Sequencing ; Humans ; Metabolism ; Models, Genetic ; Morphogenesis ; Protein Isoforms ; RNA ; Scalp* ; Skin ; Transcriptome*

Lemierre's syndrome is characterized by anaerobic bacterial infection in the head and neck, causing thrombophlebitis of the jugular vein. This disease is usually associated with a history of pharyngitis. The most common pathogens are Fusobacterium species, particularly Fusobacterium necrophorum. Lemierre's syndrome is seen most commonly in teenagers and young adults. We present a case report of a 67-year-old man with an atypical clinical manifestation of an uncommon pathogen in Lemierre's syndrome with epilepsia partialis continua.
Adolescent ; Aged ; Bacterial Infections ; Epilepsia Partialis Continua ; Fusobacterium ; Fusobacterium necrophorum ; Head ; Humans ; Jugular Veins ; Lemierre Syndrome* ; Neck ; Pharyngitis ; Thrombophlebitis ; Venous Thrombosis ; Young Adult

BACKGROUND: To assess the myocardial perfusion state after myocardial infarction, Tl-201 SPECT (Thallium-201 Single Photon Emission Computed Tomography) with a repeated "booster" injection before the acquisition of delayed redistribution image is more sensitive and more effective than conventional 4 hour redistribution image. However, this protocol has several disadvantages such as patient inconvenience, additional Tl-201 dose and compromised quantitative analysis. In this study, we compared 4 hour nitrate-augmented redistribution protocol with standard 24 hour delayed redistribution protocol with reinjection to evaluate the usefulness of sublingual nitrate to augment myocardial perfusion and the effectiveness of myocardial assessment for each protocol. METHODS: In 20 myocardial infarction patients, stress-redistribution Tl-201 SPECT was performed. Immediately after resting redistribution image was taken, each patient was administered 0.6 mg of nitroglycerin sublingually without additional Tl-201 and nitrate-augmented SPECT was taken after 30 minutes. Each patient then returned the next day and was injected with a booster dose of Tl-201 30 minutes before the delayed redistribution SPECT acquisition. For the analysis of SPECT study, the myocardium was divided into 22 segments, and the perfusion to each segment was scored on a four-point scale by consensus. An overall cardiac perfusion score was derived by summing the perfusion score for each segment. RESULTS: Reduced stress perfusion was identified in 258 segment among total 440 segments: 61 (23.6%) had improved perfusion after rest redistribution; 145 (56.2%) had improved perfusion after nitrate-augmented redistribution; 140 (54.2%) had improved perfusion after 24 hour delayed redistribution after Tl-201 reinjection. The cardiac perfusion score after stress was 38.2+/-13.1. The score increased to 41.5+/-13.1 after rest redistribution. The perfusion score were improved to 46.3+/-10.4 (p< or =0.05 vs. rest redistribution) after nitrate augmentation. The cardiac perfusion score, 46.2+/-10.8, did not improve further after delayed redistribution. CONCLUSION: Tl-201 SPECT with sublingual nitrate-augmented redistribution is as same or better than 24-hour delayed redistribution with reinjection to detect viable myocardium. Therefore, Tl-201 SPECT with sublingual nitrate-augmented redistribution has economic and time sparing advantage over traditional 24 hour delayed redistribution with reinjection.
Consensus ; Humans ; Myocardial Infarction ; Myocardium* ; Nitroglycerin ; Perfusion* ; Tomography, Emission-Computed, Single-Photon*

BACKGROUND: The neurologic deficit is one of the most serious complications after cardiopulmonary bypass (CPB). This complication has reported to be closely associated with arterial partial pressure of carbon dioxide tension (PaCO2). The traditional way to measure PaCO2 is by intermittent arterial gas analysis during CPB. We tested the relationship between PaCO2 and CPB exhausted partial pressure of carbon dioxide tension (exPCO2) which can be monitored continuously during CPB. METHODS: The total 46 patients who underwent cardiac surgery under CPB were studied. Capnography sampling line was connected to CPB exhausted port to monitor exPCO2. We sampled arterial blood from CPB for gas analysis at cooling, stable hypothermia, and rewarming phase and recorded exPCO2 simultaneously at each phase. RESULTS: We found out that exPCO2 was associated with temperature corrected PaCO2 (cPaCO2) at all 3 phases(r = 0.73, 0.70, 0.84, P < 0.05) and with temperature uncorrected PaCO2 (ucPaCO2) at cooling (r = 0.64, P < 0.05) and rewarming phases (r = 0.81, P < 0.05). CONCLUSIONS: We concluded that exPCO2 could be used to monitor either ucPaCO2 or cPaCO2 at cooling and rewarming phase and cPaCO2 at hypothermia during CPB.
Capnography ; Carbon ; Carbon Dioxide ; Cardiopulmonary Bypass ; Humans ; Hypothermia ; Neurologic Manifestations ; Organothiophosphorus Compounds ; Oxygen ; Oxygenators ; Partial Pressure ; Rewarming ; Thoracic Surgery

PURPOSE: To compare the clinical and anterior segment anatomical features in primary angle closure sub-groups based on configurations of iris root insertion. METHODS: Primary angle closure patients were imaged using anterior segment optical coherence tomography. Anterior chamber depth, iris curvature, iris thickness (IT) at the scleral spur and 500, 750, and 1,500 µm from the scleral spur (IT(0), IT(500), IT(750), and IT(1500)), lens vault, iris area, angle opening distance (AOD(500)), angle recess area (ARA(750)), and trabecular iris space area (TISA(750)) were measured. Iris root insertion was categorized into a non-basal insertion group (NBG) and basal insertion group (BG). RESULTS: In total, 43 eyes of 39 participants belonged to the NBG and 89 eyes of 53 participants to the BG. The mean age of participants was greater in the NBG than the BG (62.7 ± 5.7 vs. 59.8 ± 7.3 years, p = 0.043), and the baseline intraocular pressure was higher in the BG than the NBG (16.4 ± 4.4 vs. 14.9 ± 3.3 mmHg, p = 0.037). The BG showed a greater IT(0) (0.265 ± 0.04 vs. 0.214 ± 0.03 mm, p < 0.001) and iris area (1.59 ± 0.24 vs. 1.52 ± 0.27 mm2, p = 0.045), lower ARA(750) (0.112 ± 0.08 vs. 0.154 ± 0.08 mm2, p = 0.017) and AOD(500) (0.165 ± 0.07 vs. 0.202 ± 0.08 mm, p = 0.014) compared to the NBG. CONCLUSIONS: The BG had a narrower anterior chamber angle, thicker peripheral iris, and higher pretreatment intraocular pressure.
Anterior Eye Segment/*diagnostic imaging ; Female ; Glaucoma, Angle-Closure/diagnosis/physiopathology/*surgery ; Gonioscopy ; Humans ; *Intraocular Pressure ; Iridectomy/*methods ; Iris/diagnostic imaging/*surgery ; Lens, Crystalline/diagnostic imaging ; Male ; Middle Aged ; Prospective Studies ; Tomography, Optical Coherence/*methods

The numb chin syndrome (NCS) is characterized by chin or lower lip numbness restricted to the distribution of the mental nerve (the distal trigeminal nerve). The authors report a patient whose initial symptom of tumor recurrence was unilateral numbness of the chin. A 65-year-old male was admitted because of paresthesia around the left chin and left lower lip. Neurologic examination revealed hypesthesia on the left side of chin, lower lip and buccal mucous mem-brane. Bone scan (Tc-99m MDP) showed focal hot uptakes on the left mandible and left first rib. Brain CT with bone window setting showed a focal osteolytic lesion in the bone marrow of the left mandibular canal without destruction of bone cortex. Both coronal T1 weighted image and axial T2 weighted image showed focal low signal intensities on the left ramus. The pathophysiologic mechanism could be understood by identification of the pathologic focus.
Aged ; Bone Marrow ; Brain ; Chin ; Hodgkin Disease* ; Humans ; Hypesthesia ; Lip ; Male ; Mandible ; Neurologic Examination ; Paresthesia ; Recurrence ; Ribs