• Journal of Chest Surgery
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• v.43 no.4
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• pp.375-380
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• 2010

Background: Primary cardiac tumors are extremely rare. The most common type are benign myxomas, and these are almost completely curable with early surgery. Malignant tumors, however, such as sarcomas, are difficult to remove surgically, and their prognosis is known to be poor. In this study, data on patients who had undergone surgical treatment of cardiac tumor in the authors' hospital were collected and analyzed. Material and Method: The subjects included 28 patients who had undergone surgical treatment of cardiac tumor from August 1993 to December 2008. Their medical records were reviewed and retrospectively analyzed. Result: The patients were aged from 20 to 76 years (mean age: $54.2{\pm}15.6$), and 11 were male (39%) and 17 female (61%). Fifteen of them (54%) underwent emergency surgery to improve heart failure symptoms. The most common preoperative symptom was dyspnea (15 cases, 54%). Preoperative echocardiography was performed on all the patients. The average size of the tumor as measured during the operation was $7.0{\pm}6.9cm$ (the average length of the long axis was 2∼40 cm), and the sites of tumor attachment were the interatrial septum (18 cases, 64%), the left atrium (9 cases, 32%), the mitral valve annulus (2 cases, 7%), and the left ventricle (2 cases, 7%). The operation was performed with an incision through both atria in all the patients, and a complete excision was made in 25 cases (89%). According to the biopsy results, there were 4 cases of sarcoma (14%), 1 case of lipoma (4%), and 23 cases of myxoma (82%). The three cases in which the tumors were not completely excised were sarcomas. No operative deaths occurred after the operations. Outpatient follow-up was possible for 24 cases (86%), with a mean follow-up period of $46.8{\pm}42.7$ months. Late death occurred in 3 of the 24 patients; each of these patients had sarcomas. Of these patients, the first had undergone two repeat surgeries, the second had metastatic sites removed, and the last had only chemotherapy. The average recurrence time was $12.7{\pm}10.8$ months, and the average metastasis time was $20.5{\pm}16.8$ months. Conclusion: Most cardiac tumors are benign myxomas. In principle, they should be surgically treated because they can create risks such as embolism, and can be radically treated when surgically removed. In most cases, however, malignant sarcomas are already considerably advanced with severe infiltration into the neighboring tissues at the time of diagnosis. The surgical removal of malignant sarcomas is known to be difficult because of the advanced stage and degree of infiltration. We suggest that excision of the removable portion of the tumor sites to alleviate symptoms such as heart failure can improve quality of life.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.46-52
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• 1997

Urinary tract infection (UTI) in children has been known to be a cause of renal damage, leading to scar formation, hypertension and renal failure. And vesico-ureteral reflex (VUR), frequently accompanying UTI in young children, has been incriminated as the main factor causing scar formation. This retrospective study has been undertaken to see the relationship among UTI, VUR and renal scar formation. Study population consisted of 291 children (boy 134, girl 42) with UTI, who have been admitted to the Pediatric Department of Kyungpook University Hospital during 6 1/2 year period from January 1990 to June 1996. VUR was diagnosed by VCUG and renal scar by ultrasonogram, DMSA scan (or DMSA SPECT) and IVP. The following result were obtained. Sexual difference showed male predominance (male to female, 134:42) below 1 year of age, and female predominance (male to female, 11:35) over 5 years of age were rioted. VUR has been found in 64 children (22%) and the degree of reflux, classfied by the method proposed by 'International Reflux Study in Children', were as follows ; Grade I : 4.0%, Grade II : 3.0%, Grade III : 2.7%, Grade IV : 5.8% and Grade V : 6.2%. There was no sexual difference E.coli was the most predominant infecting agent occurring in 167 children (57%), and end-stage renal failure was diagnosed at the time of first admission in 5 children with Grade V VUR. Renal scar has been noted in 49 out of 582 kidneys (8.4%), and the incidence of scar foramation according to the degree of VUR were as follow ; Grade 0 (No reflux) : 1.2%, Grade I : 6.7%, Grade II 27.3%, Grade III 29.4%, Grade IV : 57.1%, and Grade V : 100%. In summary, present study shows that renal scar formation in UTI has close correlation with the severity of VUR occurring more frequently in severe reflux, so that early diagnosis and proper treatment of UTI and VUR is of paramount importance in preventing renal damage in children with UTI.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.136-143
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• 2000

Purpose : The hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia thrombocytopenia, and acute renal failure. It is ole of tile most common cause of acute renal failure in children but few reports are available in Korea. Thus we investigated the 23 patients diagnosed as HUS during last 14 years. Method : We retrospectively investigated the etiologic factor, clinical manifestations laboratory findings, treatment modalities, and final outcomes of the patients. Then patients were divided into two groups according to outcome, md comparison was performed. Group A(8) comprised patients who progressed to end-stage renal disease or expired. Group B(15) comprised patients who completely recovered after dialysis treatment. Result The number of patients aged less than 4 years were 17; between 5 and 10 were 4 and more than 10 were 2. The gende ratio was M:F=2 : 1. The etiologic factors were as follows: acute gastroenteritis in 14 patients including 4 bloody diarrhea, upper respiratory tract infection in 7 patients, and 1 patient with herbal mediation. The overall mortality rate was 22$\%$: 2 patients died of US complications, 2 patients died of sepsis, and 1 patient died of pulmonary hemorrhage. Group A (Hb 4.8${\pm}$1.2 g/dL) showed lower value in hemoglobin than group B (Hb 6.3${\pm}$1.7 g/dL) during hospital stay (P< 0.05), And the time interval between tile disease onset and dialysis treatment was significantly longer in group A ($11.9{\pm}9.1\;days\;vs\;2.8{\pm}2.1\;days$) (P< 0.05). Conclusion : Overall mortality rate was 22$\%$. Low hemoglobin value and the prolonged time interval between the disease onset and dialysis treatment were related with poor prognosis. So early diagnosis and appropriate intensive care including dialysis treatment is essential to achieve better outcome in children.

• Journal of radiological science and technology
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• v.34 no.4
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• pp.323-331
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• 2011

We evaluated clinical usefulness of Arterial spin labeling perfusion MR imaging on the acute ischemic cerebral infarction patients through this study. We compared 22 patients who were done with DSC imaging and ASL imaging in admitted emergency room with acute ischemic cerebral infarction, with 36 normal comparison persons (DSC image on 21persons, ASL images on 15persons). Siemens Magnetom Verio 3.0T with 12 channel head coil was used for this study. DSC image obtained 4 maps(rCBV, rCBF, rMTT, TTP) through post-processing. For qualitative analysis we compared the area of lesion macro-diagonal with the size of diffusion weighted MR image for rMTT, TTP, rCBF, rCBV, ASL maps. For Quantitative analysis we analyzed significant correlations between less than 3 cm infarction group and normal comparison group using mean relative value of flowing image with Mann-Whitney U test. TTP(95.5%) and rCBF(95.5%) maps showed high recognition rate in qualitative analysis for >3cm infarction group. The rCBF and rCBV map tests were highly related with final stage stroke areas. Mean relative value of infarction group showed a significant correlations in quantitative analysis(p<0.05). As a conclusion, arterial spin labeling image showed high lesion recognition rate in the >3cm infarction group. Mean relative values in quantitative evaluation were used for reference data. If we do more sustainable researches, ASL image will be useful for an early diagnosis of cerebral infarction, determination of the range of ischemic pneumbra and effective treatments.

• Proceedings of the Korean Vacuum Society Conference
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• 2013.08a
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• pp.88-89
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• 2013

A variety of influenza A viruses from animal hosts are continuously prevalent throughout the world which cause human epidemics resulting millions of human infections and enormous industrial and economic damages. Thus, early diagnosis of such pathogen is of paramount importance for biomedical examination and public healthcare screening. To approach this issue, here we propose a fully integrated Rotary genetic analysis system, called Rotary Genetic Analyzer, for on-site detection of influenza A viruses with high speed. The Rotary Genetic Analyzer is made up of four parts including a disposable microchip, a servo motor for precise and high rate spinning of the chip, thermal blocks for temperature control, and a miniaturized optical fluorescence detector as shown Fig. 1. A thermal block made from duralumin is integrated with a film heater at the bottom and a resistance temperature detector (RTD) in the middle. For the efficient performance of RT-PCR, three thermal blocks are placed on the Rotary stage and the temperature of each block is corresponded to the thermal cycling, namely $95^{\circ}C$ (denature), $58^{\circ}C$ (annealing), and $72^{\circ}C$ (extension). Rotary RT-PCR was performed to amplify the target gene which was monitored by an optical fluorescent detector above the extension block. A disposable microdevice (10 cm diameter) consists of a solid-phase extraction based sample pretreatment unit, bead chamber, and 4 ${\mu}L$ of the PCR chamber as shown Fig. 2. The microchip is fabricated using a patterned polycarbonate (PC) sheet with 1 mm thickness and a PC film with 130 ${\mu}m$ thickness, which layers are thermally bonded at $138^{\circ}C$ using acetone vapour. Silicatreated microglass beads with 150~212 ${\mu}L$ diameter are introduced into the sample pretreatment chambers and held in place by weir structure for construction of solid-phase extraction system. Fig. 3 shows strobed images of sequential loading of three samples. Three samples were loaded into the reservoir simultaneously (Fig. 3A), then the influenza A H3N2 viral RNA sample was loaded at 5000 RPM for 10 sec (Fig. 3B). Washing buffer was followed at 5000 RPM for 5 min (Fig. 3C), and angular frequency was decreased to 100 RPM for siphon priming of PCR cocktail to the channel as shown in Figure 3D. Finally the PCR cocktail was loaded to the bead chamber at 2000 RPM for 10 sec, and then RPM was increased up to 5000 RPM for 1 min to obtain the as much as PCR cocktail containing the RNA template (Fig. 3E). In this system, the wastes from RNA samples and washing buffer were transported to the waste chamber, which is fully filled to the chamber with precise optimization. Then, the PCR cocktail was able to transport to the PCR chamber. Fig. 3F shows the final image of the sample pretreatment. PCR cocktail containing RNA template is successfully isolated from waste. To detect the influenza A H3N2 virus, the purified RNA with PCR cocktail in the PCR chamber was amplified by using performed the RNA capture on the proposed microdevice. The fluorescence images were described in Figure 4A at the 0, 40 cycles. The fluorescence signal (40 cycle) was drastically increased confirming the influenza A H3N2 virus. The real-time profiles were successfully obtained using the optical fluorescence detector as shown in Figure 4B. The Rotary PCR and off-chip PCR were compared with same amount of influenza A H3N2 virus. The Ct value of Rotary PCR was smaller than the off-chip PCR without contamination. The whole process of the sample pretreatment and RT-PCR could be accomplished in 30 min on the fully integrated Rotary Genetic Analyzer system. We have demonstrated a fully integrated and portable Rotary Genetic Analyzer for detection of the gene expression of influenza A virus, which has 'Sample-in-answer-out' capability including sample pretreatment, rotary amplification, and optical detection. Target gene amplification was real-time monitored using the integrated Rotary Genetic Analyzer system.

• Journal of Gastric Cancer
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• v.6 no.3
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• pp.146-153
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• 2006

Purpose: Gastrointestinal stromal tumorsm (GISTs) are the most common mesenchymal tumors that arise anywhere in the tubular GI tract. The prognosis for GSTIs is important because f GISTs may metastasiwx in the liver or the abdominal cavity in an early stage. For the reason we examined the tumor size, the mitotic number, ki 67, p53, and c-kit mutation as independent prognostic factor for GISTs. Materials and Methods: A retrospective study was conducted in 76 patients who had been re-evaluated for confirmation of diagnosis between Jan 1998 and Dec. 2001. at Catholic University of medicine. Results: There were significant difference between the turner size, mitotic indices, ki 67, c-kit mutations and the 5-years survival rates. Tumor size (${\geq}5\;cm$) and mitotic index (${\geq}5/50\;HPF$) were statistically related to a significantly poor prognosis (P=0.017 and P=0.042, respectively). c-kit mutations in exon 11 were found in 7 cases c-kit mutation was observed more frequently in high risk patients, and there was a significant difference between c-kit mutation and survival (P=0.037). Elevated ki 67 was noted in 34 out of the 76 cases. High risk patients showed elevated ki67 index more frequently and there was significant relation with the survival rate (P=0.0417). Conclusion: We think that tumor size, mitotic index, Ki 67 and c-kit mutation are as independent prognostic factors for GISTs, but more research is needed.