• Clinical and Experimental Pediatrics
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• v.56 no.3
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• pp.125-129
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• 2013

Purpose: The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. Methods: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. Results: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was $14.5{\pm}5.4$ years (range, 2 to 22 years). The follow-up duration was $28.9{\pm}20.4$ months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. Conclusion: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.21 no.8
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• pp.593-602
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• 2020

Power cables installed in domestic factories or underground can cause accidents depending on the manufacturing process, installation, and environmental conditions during use. When an accident occurs in a power cable, it can cause enormous economic loss and social confusion. Hence, the importance of preventive management of the cable through diagnosis is increasing to prevent it. Therefore, in this paper, a diagnostic sample cable was produced by simulating a part that could be a problem due to the installation, manufacturing defects, or deterioration of cables that can occur in the field. Dielectric loss Tangent (tan 𝛿; TD), and Partial Discharge(PD) tests were performed. Partial discharge and AC (60Hz) withstand voltage equipment using High-Frequency Current Transformer (HFCT) were applied After applying a VLF (Very Low Frequency) power supply with a frequency of 0.1Hz was applied. As a result, B and C phase defect samples at a 2.0U₀ voltage through the VLF could measure the internal partial discharge in the A-phase normal sample cable from the noise at a 0.5U₀ to 2.0U₀ voltage. In addition, the 1.5U₀ voltage was measured through the AC (60Hz) withstand voltage equipment of the commercial frequency to verify its effectiveness. Partial discharge in the run-off state was measured at a voltage of 1.0U₀, and there was a risk when installing the equipment. AC power equipment showed a difficulty of movement by volume or weight. The diagnostic method, through the VLF of the quadrant state, revealed its safety and effectiveness.

• Tuberculosis and Respiratory Diseases
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• v.54 no.2
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• pp.230-235
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• 2003

A primary pulmonary artery sarcoma is a rare malignant tumor derived from the intimal layer of the pulmonary artery. Its clinical presentation can lead to a misdiagnosis of more common diseases such as thromboembolic disease. It is known to have a very poor prognosis. Therefore, the correct diagnosis of a primary pulmonary artery sarcoma is difficult and often delayed. We experienced a case of primary pulmonary artery sarcoma mimicking a pulmonary thromboembolism. The patient was admitted as a result of progressive dyspnea and coughing. The lung perfusion scan showed a large perfusion defect involving almost the entire right lung and suspicious small perfusion defects in the left upper lobe. Magnetic resonance imaging of the chest showed an enhancing nodule within the thrombus in the right pulmonary artery. The mass was removed completely by surgery, but the patient died as a result of shock.

• The Journal of Korean Academy of Prosthodontics
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• v.36 no.6
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• pp.900-913
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• 1998

Despite of technical difficulties, the combination of occlusal projection and densitometric digital analysis may ultimately provide a means of detection of subtle bone loss at the facial and lingual side of dental implant (Oblique occlusal view is more useful for $ITI^{(R)}$ dental implant due to its contour of shoulder as like tulip flower). In this study, conventional periapical projections of x-ray beam had shown more high sensitivity to detect the bony defects than oblique occlusal projections in alveolar crest obscured by dental implants or not, even if the difference was not statistically significant. Unlike conventional periapical projections. occusal projections combined with densitometric digital analysis technique may provide a means for detection of subtle bone change at the all around of implants without obscuring effect by implant itself. Although the results from this in vitro study were performed under limited circumstances, these results might afford more possibility and versatile modality of diagnosis options to clinician in the implant practice.

• Journal of Genetic Medicine
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• v.6 no.1
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• pp.81-86
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• 2009

Multiple epiphyseal dysplasia (MED) is a clinically and genetically heterogeneous chondroplasia, characterized by delayed development of the ossification centers and, deformities of the extremities that involve only the epiphysis and result in mild short stature. Mutations in the cartilage oligomeric matrix protein (COMP) gene are most commonly found, and most of the mutations are located in the calmodulin-like repeats and the C-terminal domain. We report a Korean kindred of 12 family members with MED in four generations who were found to have a novel mutation in the COMP gene. A pedigree showed early onset osteoarthritis requiring arthroplasty that was an autosomal dominant inherited trait. Radiological examinations demonstrated the presence of osteochondral defects in the medial femoral condyles, and the knee and hip joints showed variable degrees of precocious degenerative changes. Mutation analysis of the COMP gene in the proband and five other affected family members identified a novel missense mutation, c.1280G>C (p.Gly427Ala) in exon 12, which was not found in three unaffected family members. Direct sequencing of the COMP gene may yield pathogenic mutations in dominantly inherited MED cases, and may provide opportunities of carrier detection among high-risk family members, leading to genetic counseling for early diagnosis and intervention before the onset of complications.

• The Journal of Society for e-Business Studies
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• v.23 no.2
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• pp.111-121
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• 2018

Many problems in rotating machinery such as aircraft engines, wind turbines and motors are caused by bearing defects. The abnormalities of the bearing can be detected by analyzing signal data such as vibration or noise, proper pre-processing through a few signal processing techniques is required to analyze their frequencies. In this paper, we introduce the condition monitoring method for diagnosing the failure of the rotating machines by analyzing the vibration signal of the bearing. From the collected signal data, the normal states are trained, and then normal or abnormal state data are classified based on the trained normal state. For preprocessing, a Hamming window is applied to eliminate leakage generated in this process, and the cepstrum analysis is performed to obtain the original signal of the signal data, called the formant. From the vibration data of the IMS bearing dataset, we have extracted 6 statistic indicators using the cepstral coefficients and showed that the application of the Mahalanobis distance classifier can monitor the bearing status and detect the failure in advance.

• Clinical and Experimental Reproductive Medicine
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• v.29 no.1
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• pp.57-66
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• 2002

Objective : This study was designed to investigate the interrelationship and clinical usefulness of sperm morphology by strict criteria (SM), acrosome reaction following ionophore challenge test (ARIC) and sperm penetration assay (SPA) using zona-free hamster ova as prognostic factors in in vitro fertilization. Materials and Methods: Semen samples were provided by 83 patients undergoing IVF. We first evaluated the differences between normal fertilization group and poor fertilization group on three andrologic tests. Secondly, we analyzed the relationship between the three andrologic tests and in vitro fertilization on IVF settings. Finally, we evaluated the effectiveness of the three andrologic tests as the prognostic indicators for fertilizing ability. Results: The fertilization rate of all men in the poor fertilization group was less than 30%; but there was no evidence that this poor fertilization was due to oocyte defects. The results of three andrologic tests were significatly higher in normal fertilization group. Fertilization rate (%) in vitro was highly correlated (p<0.001) with % normal sperm by SM, ARIC value (%), and SPA result. By using Receiver-Operator-Characteristic curve (ROC), we evaluated the effectiveness of these three tests. The sensitivity and specificity of SM, ARIC test and SPA in predicting fertilization potential in IVF setting were 76% and 75%, 84% and 90%, and 76% and 95%, respectively. Conclusion: Our data suggest that the three andrologic tests can be reliable tools as prognostic factors of sperm fertilizing ability. Among these test, ARIC test and SPA gave more accurate information on fertilizing capacity. ARIC test was shown to have a predictive value for fertilizing ability comparable to that of SPA that appears to be a simple and cost-effective addition to current andrology laboratory. Combined application of these three tests may give more information on predicting sperm fertilizing capacity.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.47-51
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• 2016

Propionic acidemia (PA) is an autosomal recessively inherited disorder of the organic acid metabolism. It is caused by a deficiency of propionyl-CoA carboxylase (PCC). PCC is a heteropolymeric enzyme composed of ${\alpha}$- and ${\beta}$-subunits. The clinical symptoms of PA are heterogeneous and present vomiting, dehydration, hypotonia, and lethargy, and it can result in death. The typical presentations of neonatal onset PA are life-threatening metabolic acidosis and hyperammonemia. Here, we described a case of neonatal onset PA with mild clinical presentations. She was born to a healthy mother without complications. No significant illness was observed until nine days after birth. She started exhibiting poor oral feeding, vomiting, lethargy, and hypotonia at ten days old. Her laboratory results showed mild hyperammonemia and acidosis. The initial diagnosis was neonatal sepsis and she was treated with antibiotics. However, her clinical symptoms didn't improve. So we considered a metabolic disease. She was given nothing by mouth and intravenous hydration and nutrition support was performed. Propionylglycine and 3-hydroxypropionic acid were showed high concentrations in urine by gas chromatograph mass spectrometry (GC-MS). C3 level of acylcarnitine analysis elevated 10.4 uM/L (range, 0.200-5.00) in plasma. We took gene analysis for PA to be based on the symptoms and laboratory results. We detected PCCB gene mutation and diagnosed PA. She survived without severe neurologic defects and complications and was hospitalized only three times with upper respiratory tract infections for 7 years. We report a case of a ten days old neonate with PA presenting without severe metabolic acidosis and hyperammonemia who was effectively treated with early aggressive care and conventional methods.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1211-1216
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• 2008

Purpose : Diamond-Blackfan anemia (DBA) is a rare heterogeneous genetic disorder of infancy and early childhood. It is characterized by red cell aplasia, congenital anomalies, and a predisposition to cancer. Corticosteroids and red cell transfusions are the mainstays of therapy. We describe our experience of 6 cases of DBA that were encountered over a period of 16 years. Methods : Medical records of 6 patients diagnosed to have DBA and admitted to the Chonnam National University Hospital between 1992 and 2008 were retrospectively reviewed. Results : Three patients were males. The age at diagnosis ranged from 3 to 18 months (median, 5.5 months). Heart defects were observed in 4, polydactyly in 2, and strabismus in 1 patient. The median number of transfusions was 3 (range, 2-8). All patients responded to initial treatment with steroids and had a hemoglobin level ${\geq}9g/dL$ with a median of 12.5 days (range, 7-22 days). Three patients are currently not receiving steroid therapy. A minimum dose of oral prednisolone (<5 mg per day) was required to maintain hemoglobin ${\geq}9mg/dL$ in 3 cases. Red cell transfusion was infrequently required in 1 patient. In the median follow-up of 14 years, there was no development of malignancy. No significant side effects of steroids were found, except for short stature in 2. Conclusion : The majority of DBA patients achieved complete response and under maintenance therapy with low dose of steroids. Close observation is needed to monitor steroid side effects, cardiac function, and development of malignancy. A nation-wide survey is necessary to further characterize this rare disease in Korean children.

• Journal of the Korean Society of Surveying, Geodesy, Photogrammetry and Cartography
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• v.34 no.6
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• pp.619-627
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• 2016

Cells of a PV (photovoltaic) module can suffer defects due to various causes resulting in a loss of power output. As a malfunctioning cell has a higher temperature than adjacent normal cells, it can be easily detected with a thermal infrared sensor. A conventional method of PV cell inspection is to use a hand-held infrared sensor for visual inspection. The main disadvantages of this method, when applied to a large-scale PV power plant, are that it is time-consuming and costly. This paper presents an algorithm for automatically detecting defective PV panels using images captured with a thermal imaging camera from an UAV (unmanned aerial vehicle). The proposed algorithm uses statistical analysis of thermal intensity (surface temperature) characteristics of each PV module to verify the mean intensity and standard deviation of each panel as parameters for fault diagnosis. One of the characteristics of thermal infrared imaging is that the larger the distance between sensor and target, the lower the measured temperature of the object. Consequently, a global detection rule using the mean intensity of all panels in the fault detection algorithm is not applicable. Therefore, a local detection rule was applied to automatically detect defective panels using the mean intensity and standard deviation range of each panel by array. The performance of the proposed algorithm was tested on three sample images; this verified a detection accuracy of defective panels of 97% or higher. In addition, as the proposed algorithm can adjust the range of threshold values for judging malfunction at the array level, the local detection rule is considered better suited for highly sensitive fault detection compared to a global detection rule. In this study, we used a panel area extraction method that we previously developed; fault detection accuracy would be improved if panel area extraction from images was more precise. Furthermore, the proposed algorithm contributes to the development of a maintenance and repair system for large-scale PV power plants, in combination with a geo-referencing algorithm for accurate determination of panel locations using sensor-based orientation parameters and photogrammetry from ground control points.