• Neonatal Medicine
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• v.17 no.2
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• pp.254-261
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• 2010

Little is known about neonatal mitochondrial disease, though mitochondrial metabolic disorders may often present in the neonatal period because of the high energy requirement of neonate. In newborn period, common presentations are not specific and the disease course may be rapid and fatal. In this study, we report three cases of neonatal mitochondrial disease. The first case was strongly suspected because of sudden seizure and mental change with severe lactic acidosis, and multiorgan failure. Plasma lactate/pyruvate (L/P) ratio was increased to 55.6 with marked lactic aciduria and increased plasma alanin up to 2,237 nmol/mL. In the second patient, a peritoneal dialysis was performed for acute adrenal and renal failure, but metabolic acidosis persisted. Plasma L/P ratio was increased to 23.9, and MRC I (mitochondrial respiratory chain defect) was diagnosed through the enzymatic analysis of the muscles. The third case showed repetitive episode of lactic acidosis during the first two months of life, hypotonia, failure to thrive and feeding difficulties. We found markedly increased cerebrospinal fluid L/P ratio up to 57 though plasma L/P ratio(19.4) was borderline with increased plasma lactate. The lactate peak was prominent in brain magnetic resonance spectroscopy (MRS). MRC II was confirmed through muscle biopsy. Plasma lactate level and lactate peak of brain MRS were normalized after conservative treatment.

• Journal of Chest Surgery
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• v.22 no.1
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• pp.134-140
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• 1989

A clinical evaluation was made on total 207 cases of corrosive esophageal stricture after ingestion of various corrosive substances and 173 cases of neoplasms in the esophagus and the cardia. The various complications associated with esophageal corrosion were observed on 28 cases [13.5%] in a total of 207 cases. Pathoanatomic findings of complication may be classified to the five category as follow; [1] stenosis in the pharynx due to adhesion of the epiglottis, [2] esophagobronchial fistula, [3] esophageal perforation with bougienation, [4] necrotic rupture of the esophagus and the stomach, and [5] so-called chronic corrosive gastritis. The comparative studies were done on a total of 165 cases of the various procedures of esophagoplasty to the reconstruction of the esophagus, which consists of antethoracal esophagoplasty with jejunum, retrosternal esophagoplasty with jejunum, retrosternal esophagoplasty with right colon, and retrosternal esophagoplasty with left colon. There is no hard and fast rule in selection of jejunum, right colon or left colon as the transplanting bowel and an operative method either antethoracal or retrosternal approach. When there was no possibility of the complication and no any defect of the anatomical states, one stage retrosternal esophagoplasty using right colon was better in various points of view. The 173 patients of the neoplasm of the esophagus consist of 28 cases of benign tumors and 145 cases of malignant tumors in the esophagus and cardia. 28 cases of benign tumors in the esophagus received the surgical treatment and they obtained with excellent results postoperatively. Of the 145 patients of esophageal carcinoma who received surgical managements, 101 cases [69.6%] were found to be operable and 44 cases [30.3%] were inoperable. Due to the various level of carcinoma in the esophagus, the following different surgical procedure was properly used case by case to get the best results in each case. Esophageal carcinoma in the upper and middle third segment received the total esophagectomy and the reconstruction of the esophagus using right colon by substernal procedure. Esophageal carcinoma in the lower third segment received an esophagojejunostomy in the mediastinum after the resection of lower third segment of the esophagus. Carcinoma in the esophago cardia and the stomach received also an esophagojejunostomy after the resection of the lower third segment of the esophagus and subtotal gastrectomy. For the 44 patients with inoperable carcinoma, the several palliative surgical managements such as gastrostomy or jejunostomy for feeding and esophagojejunostomy for bypass of the lower esophagus and the stomach were properly performed case by case for their maximum improvement.

• Proceedings of the Korean Vacuum Society Conference
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• 2010.08a
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• pp.143-144
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• 2010

3-5족 화합물 반도체를 이용한 집광형 삼중 접합 태양전지는 40% 이상의 광변환 효율로 많은 주목을 받고 있다[1]. 삼중 접합 태양전지의 하부 셀은 기계적 강도가 높고 장파장을 흡수할 수 있는 Ge이 사용된다. Ge위에 성장될 III-V족 단결정막으로서 Ge과 격자상수가 일치하는 GaInP나 GaAs가 적합하고, 성장 중 V족 원소의 열확산으로 인해 Ge과 pn접합을 형성하게 된다. 이때 GaInP의 P의 경우 GaAs의 As보다 확산계수가 낮아 태양전지 변환효율향상에 유리한 얇은 접합 형성이 가능하고, 표면 에칭효과가 적기 때문에 GaInP를 단결정막으로 선택하여 p-type Ge기판 위 성장으로 단일접합 Ge구조 제작이 가능하다. 하지만 이종접합 구조 성장으로 인해 발생한 계면사이의 전위나 미세결함들이 결정막내부에 존재하게 되며 이러한 결함들은 광학소자 응용 시 비발광 센터로 작용할 뿐 아니라 소자의 누설전류를 증가시키는 원인으로 작용하여 태양전지 변환효율을 감소시키게 된다. 이에 결함감소를 통해 소자의 전기적 특성을 향상시키고자 수소 열처리나 플라즈마 공정을 통해 수소 원자를 박막내부로 확산시키고, 계면이나 박막 내 결함들과 결합시킴으로서 결함들의 비활성화를 유도하는 연구가 많이 진행되어 왔다 [2][3]. 하지만, 격자불일치를 갖는 GaInP/Ge 구조에 대한 수소 열처리 및 불순물 준위의 거동에 대한 연구는 많이 진행되어 있지 않다. 따라서 본 연구에서는 Ga0.45In0.55P/Ge구조에 수소 열처리 공정을 적용을 통하여 단결정막 내부 및 계면에서의 결함밀도를 제어하고 이를 통해 태양 전지의 변환효율을 향상시키고자 한다. <111> 방향으로 $6^{\circ}C$기울어진 p-type Ge(100) 기판 위에 유기금속화학증착법 (MOCVD)을 통해 Si이 도핑된 200 nm의 n-type GaInP층을 성장하여 Ge과 단일접합 n-p 구조를 제작하였다. 제작된 GaInP/Ge구조를 furnace에서 250도에서 90~150분간 시간변화를 주어 수소열처리 공정을 진행하였다. 저온 photoluminescence를 통해 GaInP층의 광학적 특성 변화를 관찰한 결과, 1.872 eV에서 free-exciton peak과 1.761 eV에서 Si 도펀트 saturation에 의해 발생된 D-A (Donor to Acceptor)천이로 판단되는 peak을 검출할 수 있었다. 수소 열처리 시간이 증가함에 따라 free-exciton peak 세기 증가와 반가폭 감소를 확인하였고, D-A peak이 사라지는 것을 관찰할 수 있었다. 이러한 결과는 수소 열처리에 따른 단결정막 내부의 수소원자들이 얕은 불순물(shallow impurity) 들로 작용하는 도펀트들이나, 깊은 준위결함(deep level defect)으로 작용하는 계면근처의 전위, 미세결함들과의 결합으로 결함 비활성화를 야기해 발광세기와 결정질 향상효과를 보인 것으로 판단된다. 본 발표에서는 상술한 결과를 바탕으로 한 수소 열처리를 통한 박막 및 계면에서의 결함준위의 거동에 대한 광분석 결과가 논의될 것이다.

• The Korean Journal of Mycology
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• v.23 no.2 s.73
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• pp.129-138
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• 1995

DNA fragment being able to restore in vitro activity of ${\beta}-1,3-glucan$ synthase was cloned by transformation of the Saccharomyces cerevisiae LP353 mutant strain with genomic library constructed in the YCp50. For the selection of transformants which showed no detectable phenotype linked to recovery of the defect in ${\beta}-1,3-glucan$ synthase activity, the colony autoradiography was succesfully applied. The restriction map of the cloned DNA fragment, which is 8.5-kb in length, was constructed. Both the YEplac195 and the YCp50 carrying the 8.5-kb fragment increased ${\beta}-1,3-glucan$ synthase activity of LP353 by two fold. Neither the YEplac195 nor the YCp50 carrying the 8.5-kb DNA fragment, however, complemented the temperature-dependent osmotic sensitivity which is another distinctive phenotype of LP353. Subcloning experiments indicated that a functional region was located in 4.8-kb BglII-KpnI fragment. The 4.8-kb fragment was also able to increase the level of ${\beta}-1,3-glucan$ content in cell wall as well as the resistance of cells to cell wall lytic enzyme, ${\beta}-1,3-glucanase$. The growth rate of the LP353 with 4.8-kb fragment was almost same as that of wild type strain in liquid medium with 1.2 M sorbitol at nonpermissive temperature. Taken these results together, the 4.8-kb fragment seemed to contain the BGS2 gene for ${\beta}-1,3-glucan$ synthase activity in yeast S. cerevisiae.

• Reproductive and Developmental Biology
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• v.32 no.1
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• pp.1-7
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• 2008

The SCID-repopulation cells(SRCs) assay has been widely used to determine the self-renewal capacity of hematopoietic stem cells (HSCs). In this study, we tested the repopulating efficiency of porcine bone marrow derived hematopoietic stem cells using nonobese diabetic/severe combined immunodieficient (NOD/SCID) mice which was inherited immunodeficiency mire with defect of T cells, B cells, and low activity of NK cells. We transplanted porcine bone marrow hematopoietic stem/progenitor cells with intraperitoneal injection into neonate NOD/SCID mice. We confirmed efficient reconstitution activity of inoculated porcine hematopoietis cells in variety of organs of NOD/SCID mice. Interestingly, pig $CD3^+$ T lymphocytes detected with high level in liver($15.6{\pm}3.7%$), spleen($5.6{\pm}3.0%$), thymus($1.5{\pm}1.3%$), and BM($2.3{\pm}0.9%$), respectively. These data imply that microenvironment of neonate NOD/SCID mice is very efficient for proliferation and differentiation of porcine T cells, and can be useful for the study of T cells development and renogeneic organ transplantation.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.148-154
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• 2016

A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.9 no.2
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• pp.360-369
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• 2008

The solar cells should be operated at the maximum power point because its output characteristics were greatly fluctuated on the variation of insolation, temperature and load. It is necessary to install an inverter among electric power converts by means of the output power of solar cell is DC. The inverter is operated supply a sinusoidal current and voltage to the load and the interactive utility line. In this paper, the proposes a photovoltaic system is designed with a step up chopper and single phase PWM voltage source inverter. Synchronous signal and control signal was processed by one-chip microprocessor for stable modulation. The step up chopper is operated in continuous mode by adjusting the duty ratio so that the photovoltaic system tracks the maximum power point of solar cell without any influence on the variation of insolation and temperature for solar cell has typical dropping character. The single phase PWM voltage source inverter is consists of complex type of electric power converter to compensate for the defect, that is, solar cell cannot be develop continuously by connecting with the source of electric power for ordinary using. It can be cause the efect of saving electric power, from 10 to 20%. The single phase PWM voltage source inverter operates in situation, that its output voltage is in same phase with the utility voltage. The inverter are supplies an ac power with high factor and low level of harmonics to the load and the utility power system.

• Food Science of Animal Resources
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• v.24 no.4
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• pp.361-366
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• 2004

Physicochemical and textural properties, and sensory evaluation of low-fat sausages (LFSs) manufactured with 0.3 and 0.6％ chitosan were investigated and compared with those of regular-fat sausages (RFSs). Although the batter pH was not changed with the addition of chitosan (p>0.05), product pH was reduced (p>0.05) with the addition of chitosan up to 0.6％. Proximate composition and cooking loss (％) were not affected by the addition of chitosan. Expressible moisture 1r91ues (EM, ％) of LESs were lower (p<0.05) than those of RFSs, but not affected by the addition of chitosan. The addition of chitosan in the sausage formulation became harder, springier and more cohesive (p<0.05), whereas, no differences were observed (p>0.05) in these values of sausages containing between 0.3 and 0.6％. Batter viscosity was not affected by the addition of chitosan, but LESs with or without chitosan had lower viscosity than RFSs due to high moisture (％). Sensory evaluation results showed that LFSs containing 0.3％ chitosan had most parameters similar to those of low-fat control. These results indicated that chitosan at the level of 0.3％ can be used for the manufacture of LFSs without quality defect.

• Journal of Life Science
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• v.28 no.5
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• pp.597-604
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• 2018

Although the core mechanisms of Attention Deficit/Hyperactivity Disorder (ADHD) are unknown, several ADHD-associated proteins have been studied. G-protein - coupled receptor kinase interacting protein-1 (GIT1) is a multifunctional adapter protein that affects neuron growth and dendrite formation. GIT1-deficient mice have shown ADHD-like behavior and also recovered through amphetamine treatment. In this study, gliotransmitters were investigated in both intracellular and extracellular space from GIT1-deficient mice. To measure the amount of gliotransmitters, primary astrocyte cultures were taken from the cerebral and cerebellar cortices of wild (WT), hetero (HE), and knock-out (KO) mice. Major gliotransmitters were analyzed using high-performance liquid chromatography. It was observed that the amount of excitatory and inhibitory gliotransmitters were dependent on genotype and showed a change in excitation/inhibition ratios. Interestingly, the major excitatory gliotransmitter, glutamate, existed at the lowest level in WT mice, but the amount of inhibitory gliotransmitters, gamma-aminobutyric acid (GABA) and glycine, varied depending on brain region. Remarkably, an increased amount of GABA was measured at the intracellular cerebrum in WT mice compared with KO mice. It was presumed that KO mice would secrete more inhibitory gliotransmitters to compensate for GIT1 depletion or else acquire a defect to reuptake-secreted GABA. This may be a possible mechanism for ADHD pathology.

• Journal of the Institute of Electronics and Information Engineers
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• v.50 no.5
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• pp.183-190
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• 2013

In this paper, we propose an improved anisotropic diffusion model for micro-crack detection in heterogeneously textured surface of polycrystalline solar wafers. Due to the nature of the image sensor, the gray-level of the diagonal micro-crack is non-uniform. Thus, the conventional algorithms can't fully detect diagonal micro-cracks when the number of iteration is not enough. However, the increasing of the iteration number leads to increase computation time and detects micro-crack thicker than the original micro-crack. In order to overcome this drawback, we use the gradient of north, south, east, and west directions as well as extended directions. To calculate the diffusion coefficients, we compare the gradients of conventional directions and extended directions and apply the larger gradient values to the coefficient function. This is because the proposed method reflects the information of diagonal micro-crack. Comparing to Tsai et al.'s and Ko and Rheem's, the proposed algorithm shows superior efficiency in detecting the diagonal micro-cracks with less iterations in the images of polycrystalline solar wafers. In addition, it also shows that the thickness of segmented micro-crack is similar to the orignal micro-crack.