Kim, Joo-Whee;Lee, Se-Eun;Jung, Yun-Hye;Han, Kyung-Hee;Lee, Hyun-Kyung;Kang, Hee-Gyung;Ha, Il-Soo;Cheong, Hae-Il;Choi, Yong
Childhood Kidney Diseases
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v.14
no.1
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pp.42-50
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2010
Purpose : The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods : Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results : Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion : SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.
Lee, Jeong Ju;Kim, Yong-Jin;Shin, Jae Il;Yim, Hyunee;Park, Se Jin
Childhood Kidney Diseases
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v.17
no.2
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pp.92-100
/
2013
Purpose: To investigate the clinicopathologic effects of cyclosporine A (CsA) in children with diseases characterized by mesangial immunoglobulin A deposits such as immunoglobulin A nephropathy (IgAN) and Henoch-Sch$\ddot{o}$nlein purpura nephritis (HSPN). Methods: We retrospectively reviewed the clinicopathologic outcomes of 54 children (IgAN, 36; HSPN, 18) treated with CsA. The starting dose of CsA was 5 mg/kg per day, and it was administered in 2 divided doses. The degree of proteinuria and pathologic changes in renal biopsies were evaluated before and after CsA treatment. Results: The mean protein to creatinine ratio decreased from $3.7{\pm}1.5$ to $0.6{\pm}0.4$(P <0.001), and 32 (59.2%) children achieved complete remission of proteinuria after 1-year CsA treatment. Among the 54 children, 24 maintained normal renal function and 25 exhibited microscopic hematuria or proteinuria at the end of CsA treatment. In the HSPN group, 3 children whose initial biopsies indicated class IIIb disease showed class II disease on follow-up, and the follow-up biopsies of 2 children who had class II disease indicated the same class II disease. In the IgAN group, cortical tubular atrophy occurred in 1 child, and no child with IgAN had cortical interstitial fibrosis or tubular atrophy after 1-year CsA treatment. No significant complications were found in the children treated with CsA. Conclusion: Our findings indicate that CsA treatment is effective and beneficial in reducing massive proteinuria and preventing progression to end-stage renal failure in children with glomerular diseases characterized by IgA deposits, such as IgAN and HSPN, within 1 year of treatment.
Purpose: Diabetic nephropathy is the most common cause of end stage renal disease and the incidence is progressively increasing. The aim of this study was to investigate the differences of $^{99m}Tc$-DMSA renal uptake among diabetic patients with normoalbuminuria, microalbuminuria and overt proteinuria, and then to determine the clinical usefulness of $^{99m}Tc$-DMSA in predicting early diabetic nephropathy Materials and Methods: $^{99m}Tc$-DMSA scan was performed and a total renal uptake of $^{99m}Tc$-DMSA was measured in 145 diabetic patients. Patients were divided into 3 groups according to the amount of 24 hour urinary albumin excretion as Group I (normoalbuminuria, 74 cases), Group II (microalbuminuria, 39 cases), and Group III (overt proteinuria, 32 cases). The differences of $^{99m}Tc$-DMSA renal uptake among the 3 groups and the correlation between the renal uptake of $^{99m}Tc$-DMSA and other clinical parameters were analyzed. Results: The total renal uptake of $^{99m}Tc$-DMSA of Group II ($40.8{\pm}11.0%$) was significantly lower than that of Group I ($54.4{\pm}6.3%$, p<0.001). The uptake of Group III ($27.7{\pm}12.0%$) was significantly lower than those of both Group I and Group II (p<0.001). $^{99m}Tc$-DMSA total renal uptakes correlated negatively with serum creatinine level (r=-0.629, p<0.001) and positively correlated with creatinine clearance rate (r=0.102, p<0.001). Conclusion: $^{99m}Tc$-DMSA total renal uptake of diabetic patients with microalbuminuria was significantly decreased compared with that of patients of normoalbuminuria. Therefore, $^{99m}Tc$-DMSA scan can be used as a diagnostic study for early detection of the diabetic nephropathy.
The purpose of this study is to acquire the information on the current situation of students' selection process in order to renovate the system of picking up the students. As a first step of the study, we examined the validity of the factors of the single-out system such as qualification and the process for the application and the standards and proceeding of the selection. Then we analysed the result of the entrance examination of Hansung Science Highschool in 2002. The analysis was on the correlation between the result of entrance examination and the achievement in the school and the decision of the course after graduation. To know on the achievement of the students, we investigated the records of regular tests and asked the teachers' opinion in math and science classes. As a result, we gained the following points: First, the present single-out system has a danger of excluding students who are much talented in science and math field because it is based on students' achievements in middle schools; Second, the new selection system should consider the character and attitude of the applicants in addition to their knowledge; Third, the continuous observation of the teacher in middle school should be an important factor of the picking up system; Fourth, more questions requiring divergent thinking ability and inquiry skill should be developed as selective examination question. Also examination questions should cover the various contents from mathematics to science, and do not affect pre-learning; Finally, the system of present letting all students stand in one line should be changed into that of letting students in various lines. We can consider using multi-step selection system.
A clinical and histopathological study was performed on ninety-four patients with nephrotic syndrome (91 idiopathic and 3 secondary) who were admitted to Department of Internal Medicine, Yeungnam University Hospital during the period of nine years, from January 1985 to May 1994. The results were as following. 1. the ratio of male to female was 1.76:1. In young age group, minimal change was the most predominant type. In old age group, membranous glomerulonephritis and focal glomerulosclerosis were predominant types. 2. The primary nephrotic syndromes were 96.8% and secondary nephrotic syndromes were 3.2%. Histopathologic findings of 94 renal biopsy tissue were classified into minimal change (43.6%), mesangial proliferative glomerulonephritis (29.8%), membranous glomerulonephritis (12.8%), TypeI membranous proliferative glomerulonephritis (4.3%), focal glomerulosclerosis (3.2%) and others (6.4%). 3. The response of eighty-six patients treated with steroid showed complete remission in 51.2%, partial remission in 20.9%, steroid dependent in 2.3%, and no effect in 25.6% of cases respectively. The response to steroid therapy was most effective in the patients with minimal change lesion. 4. In the patient with membranous proliferative glomerulonephlitis, long-term angiotensin converting enzyme inhibitor treatment showed less deterioration of renal function.
Until now, most studies regarding stone pagodas of the Honam region have been about stone pagodas with Baekje style because it was the territory of Baekje. However, after reviewing the designated cultural properties, I found that among the 94 stone pagodas in the Honam region there are 15 stone pagodas with Baekje style built in the Goryeo Dynasty and 34 stone pagodas with Silla style. So far, most research efforts have been concentrating on stone pagodas with Baekje style. Through a review of stone pagodas with Silla style, I sought to reveal new aspects of the pagodas in the Honam region. After reviewing the placement of pagodas, I found stone pagodas with Silla style were built mostly in South Jeolla Province during the Unified Silla period. However, in the Goryeo Dynasty stone pagodas with Silla style were erected throughout the Honam region. This shows enhanced Buddhist control over the Honam region than in the previous period. It can be verified especially in Gimje, Jeongeup, Sunchang, Damyang, Yeongam, and Gangjin, where stone pagodas with Baekje style and stone pagodas with Silla style coexist. The types of stone pagodas with Silla style in the Honam region can be divided into two. There are 21 pagodas with two-story foundation, and 12 with single foundation. They seem to have inherited characteristics of the two-story foundation of Silla pagodas. Two pagodas with single foundation were made of a single rock or natural rock for lower foundation. Regarding the body of the pagoda, there are 21 three-story pagodas and 12 five-story pagodas. 25 pagodas have first floors made of a single rock. These are likely to be in the tradition of Silla pagodas. However, the lower part of the roof stones vary as there are three, four, or five-tiered ones. Overall, 12 out of 16 pagodas with middle column in foundation, and 15 out of 21 three-story pagodas are located in South Jeolla Province. This proves that stone pagodas in the South Jeolla Province well maintained the tradition of Silla and became popular.
Sohn, Young Bae;Ahn, Sunhyun;Jang, Ja-Hyun;Lee, Sae-Mi
Journal of The Korean Society of Inherited Metabolic disease
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v.19
no.1
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pp.20-25
/
2019
Very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (OMIM#201475) is an autosomal recessively inherited metabolic disorder of mitochondrial long-chain fatty acid oxidation. The clinical features of VLCAD deficiency is classified by three clinical forms according to the severity. Here, we report a case of later-onset episodic myopathic form of VLCAD deficiency whose diagnosis was confirmed by plasma acylcarnitine analysis and" multigene panel multigene panel sequencing. A 34-year old female patient visited genetics clinic for genetic evaluation for history of recurrent myopathy with intermittent rhabdomyolysis. She suffered first episode of rhabdomyolysis with acute renal failure requiring hemodialysis at twelve years old. After then, she suffered several times of recurrent rhabdomyolysis provoked by prolonged exercise or fasting. Physical and neurologic exam was normal. Serum AST/ALT and creatinine kinase (CK) levels were mildly elevated. However, according to her previous medical records, her AST/ALT, CK were highly elevated when she had rhabdomyolysis. In suspicion of fatty acid oxidation disorder, multigene panel sequencing and plasma acylcarnitine analysis were performed in non-fasting, asymptomatic condition for the differential diagnosis. Plasma acylcarnitine analysis revealed elevated levels of C14:1 ($1.453{\mu}mol/L$; reference, 0.044-0.285), and C14:2 ($0.323{\mu}mol/L$; 0.032-0.301) and upper normal level of C14 ($0.841{\mu}mol/L$; 0.065 -0.920). Two heterozygous mutation in ACADVL were detected by multigene panel sequencing and confirmed by Sanger sequencing: c.[1202G>A(;) 1349G>A] (p.[(Ser 401Asn)(;)(Arg450His)]). Diagnosis of VLCAD deficiency was confirmed and frequent meal with low-fat diet was educated for preventing acute metabolic derangement. Fatty acid oxidation disorders have diagnostic challenges due to their intermittent clinical and laboratorial presentations, especially in milder late-onset forms. We suggest that multigene panel sequencing could be a useful diagnostic tool for the genetically and clinically heterogeneous fatty acid oxidation disorders.
This paper presents the process of re-conservation and the results of research on the production method of the Iron Sword with Ring Pommel with Three-Pointed Leaf Decoration, one of the excavation findings from Tomb No. 55 in the Dalseong Ancient Tomb Complex in Daegu. This iron sword is a double weapon with two large swords housed within a single sheath. Four smaller swords are attached to the surface of the sheath, two on the upper portion and the other two below. It is the only such two-in-one weapon excavated intact thus far from an ancient Korean tomb. The records show that it underwent conservation treatment twice in the past. In this study, it was subjected to conservation treatment again to replace the stiffener in some cracking areas, and its material, composition, and production method were analyzed by CT, XRF analysis, and stereoscopic microscopy. The sword is mainly made of copper, but the golden component contains both gold and mercury, which suggests that the copper was plated in gold using mercury amalgamation. The examination of the production methods indicates that it was intended more to demonstrate the authority of its owner rather than to serve any practical use. The two upper small swords on the sheath were made in the same manner as the main swords, and the two small lower swords were cut from a single metal sheet. The sheath was made by cutting two metal sheets. Supports were used to attach the two small swords to the upper portion of the sheath, while the lower portion of the sheath was slit to allow the other two small swords to be inserted into it. The ring pommels of the main swords have a three-pointed leaf decoration. As for the other designs, the handle of the main sword features a series of semicircles, the decorative bands on the sheath have waves in dots, and the fish tail of the sheath shows diagonal lines of dots.
As the global market environment changes, the domestic ICT industry has a growing influence on the world economy. This industry is regarded as an important driving force in the national economy from a technological and social point of view. In particular, small and medium-sized enterprises (SMEs) in the ICT industry are regarded as essential actors of domestic economic development in terms of company diversity, technology development and job creation. However, since it is small compared to large-sized enterprises, it is difficult for SMEs to survive with a differentiated strategy in an incomplete and rapidly changing environment. Therefore, SMEs must make a lot of efforts to improve their own capabilities, and the government needs to provide the desirable help suitable for corporate internal resources so that they can continue to be competitive. This study classifies the types of ICT SMEs participating in government support programs, and analyzes the relationship between resources and performance creation of each type. The data from the "ICT Small and Medium Enterprises Survey" conducted annually by the Ministry of Science and ICT was used. In the first stage, ICT SMEs were clustered based on common factors according to their experiences with government support programs. Three clusters were meaningfully classified, and each cluster was named "active participation type," "initial support type," and "soloist type." As a second step, this study compared the characteristics of each cluster through profiling analysis for each cluster. The third step carried out in this study was to find out the mechanism of R&D performance creation for each cluster through regression analysis. Different factors affected performance creation for each cluster, and the magnitude of the influence was also different. Specifically, for "active participation type", "current manpower", "technology competitiveness", and "R&D investment in the previous year" were found to be important factors in creating R&D performance. "Initial support type" was identified as "whether or not a dedicated R&D organization exists", "R&D investment amount in the previous year", "Ratio of sales to large companies", and "Ratio of vendors supplied to large companies" contributed to the performance. Lastly, in the case of "soloist type", "current workforce" and "future recruitment plan", "technological competitiveness", "R&D investment", "large company sales ratio", and "overseas sales ratio" showed a significant relationship with the performance. This study has practical implications of showing what strategy should be established when supporting SMEs in the future according to the government's participation in the startup program and providing a guide on what kind of support should be provided.
Background: Percutaneous cardiopulmonary support. (PCPS) has the potential to rescue patients in cardiogenic shock who might otherwise die. PCPS has been a therapeutic option in a variety of the clinical settings such as for patients with myocardial Infarction, high-risk coronary intervention and postcardiotomy cardiogenic shock, and the PCPS device is easy to install. We report our early experience with PCPS as a life saving procedure in cardiogenic shock patients due to acute myocardial infarction. Material and Method: From January 2005 to December 2006, eight patients in cardiogenic shock with acute myocardial infarction underwent PCPS using the CAPIOX emergency bypass system($EBS^{(R)}$, Terumo, Tokyo, Japan). Uptake cannulae were inserted deep into the femoral vein up to the right atrium and return cannulae were inserted into the femoral artery with Seldinger techniques using 20 and 16-French cannulae, respectively. Simultaneously, autopriming was performed at the $EBS^{(R)}$ circuit. The $EBS^{(R)}$ flow rate was maintained between $2.5{\sim}3.0L/min/m^2$ and anticoagulation was performed using intravenous heparin with an ACT level above 200 seconds. Result: The mean age of patients was $61.1{\pm}14.2$ years (range, 39 to 77 years). Three patients were under control of the $EBS^{(R)}$ before percutaneous coronary intervention (PCI), three patients were under control of the $EBS^{(R)}$ during PCI, one patient was under control of the $EBS^{(R)}$ after PCI, and one patient was under control of the $EBS^{(R)}$ after coronary bypass surgery. The mean support time was $47.5{\pm}27.9$ hours (range, 8 to 76 hours). Five patients (62.5%) could be weaned from the $EBS^{(R)}$ after $53.6{\pm}27.2$ hours. (range, 12 to 68 hours) of support. All of the patients who could successfully be weaned from support were discharged from the hospital. There were three complications: one case of gastrointestinal bleeding and two cases of acute renal failure. Two of the three mortality cases were under cardiac arrest before $EBS^{(R)}$ support, and one patient had an intractable ventricular arrhythmia during the support. All of the discharged patients are still surviving at $16.8{\pm}3.1$ months (range, 12 to 20 months) of follow-up. Conclusion: The use of $EBS^{(R)}$ for cardiogenic shock caused by an acute myocardial infarction could rescue patients who might otherwise have died. Successfully recovered patients after $EBS^{(R)}$ treatment have survived without severe complications. More experience and additional clinical investigations are necessary to elucidate the proper installation timing and management protocol of the $EBS^{(R)}$ in the future.
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