• Journal of Gastric Cancer
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• v.7 no.4
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• pp.185-192
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• 2007

Purpose: RUNX3, a novel tumor suppressor, is frequently inactivated in gastric cancer. In the present study, we examined the pattern of RUNX3 expression in gastric cancer cells from gastric cancer specimens and the impact of its alteration on clinical outcome. Materials and Methods: A total of 124 samples of both gastric cancer and normal tissue were obtained from 124 patients who underwent curative gastrectomy at the Seoul Medical Center from January 2001 to December 2005. RUNX3 expression was determined by immunohistochemical staining, and the results were analyzed. Statistical analysis wabased on clinicopathological findings and differences in survival rates. Results: The mean age of the patients was 61 years, and the male:female ratio was 1.9:1. The expression rate of RUNX3 was 59.7% (74/124). The expression rate was higher in differentiated gastric cancers (nucleus: 9.1%, cytoplasm: 57.6%) than in the undifferentiated types (nucleus: 5.2%, cytoplasm: 46.6%) (P=0.133). The 5-year survival rates according to RUNX3 expression determined from cancer tissue were 88.9% for the nucleus $\pm$ cytoplasm(+) group of patients, 76.1% for the cytoplasm only (+) group of patients, and 65.3% for the RUNX3 negative expression group of patients (P=0.626). Only UICC TNM staging showed statistical significance related to the survival rate, as determined by multivariate analysis. Conclusion: The RUNX3 expression rate was higher in differentiated gastric cancer than in the undifferentiated types without significance. Although RUNX3 expression predicted better survival, based on multivariate analysis, the finding was not statistically significant. More cases should be further evaluated.

• The Journal of the Korean bone and joint tumor society
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• v.13 no.1
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• pp.60-66
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• 2007

Purpose: The purpose of this study was to evaluate the functional outcomes of periacetabular malignant bone tumors treated by internal pelvectomy without reconstruction. Materials and methods: Between January 1996 and December 2005, eight patients with primary malignant or metastatic periacetabular bone tumors were treated by internal pelvectomy without reconstruction. There were 6 men and 2 women. Mean age was 42 years old. There were 3 osteosarcomas, 3 chondrosarcomas and 2 metastatic carcinomas. The type of pelvic resections were 6 type I+II+III, 1 type I+II and 1 type II+II resection. The functional outcomes were evaluated with ISOLS revised criteria. The follow up period ranged from 6 to 84 months. Results: At last follow up, 5 patients showed CDF, 2 patients, AWD and the remained 1, DOD. The mean functional score for pain, functional activity, emotional acceptance, use of external support, walking ability and gait were 4.9, 2.9, 2.9, 1.5, 2.3 and 2.5 respectively. The total functional score ranged from 37% to 70%(average: 56%). There were three temporary nerve palsies. Conclusion: The internal pelvectomy without reconstruction for selective difficult periacetabular malignant tumors could be a viable option with fewer complications and fair functional outcomes.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1000-1006
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• 2002

Purpose : In the course of treatment, patients with hematological or oncological disorders often develop pulmonary complication. The patients who develop a severe pulmonary complication have a poor outlook. The causes of pulmonary complication are either infectious or non-infectious in origin. We have analyzed the etiology and outcome of these patients admitted to the pediatric intensive care unit of Asan Medical Center. Methods : Medical records of 95 patients on Pediatric oncology service who were admitted to pediatric intensive care unit(PICU) of Asan Medical Center from Jan 1997 to May 2000 were retrospectively reviewed. Results : The mean age of the patients was 8.5 years(2 months-18 years). The underlying malignancies of these 95 patients were as following; acute lymphoblastic leukemia(31 cases), lymphoma (11 cases), acute myeloid leukemia(nine cases), brain tumor(eight cases) and other solid tumors(25 cases). Pulmonary complications included pneumonia, acute respiratory failure, pneumothorax and pleural effusion. The most common cause of pulmonary complication was infection(88%) in etiology. The overall mortality rate was 56.8%. Pulmonary complications in these patients carried high rates of mortality regardless of whether they were immune compromised(76%) or not(69%). Even without pulmonary complications, the hematological or oncological patients admitted to PICU had high mortality rates of 43%. Conclusion : Pulmonary complications are frequent finding in the hematological or oncological patients admitted to Intensive Care Unit. The main etiology of these pulmonary complications was infection, which carried a high mortality rate regardless of their immune status at the time when they were admitted to PICU.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.80-84
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• 2010

Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs) and 95% confidence interval (95% CIs) of risk factors for unfavorable outcomes.Results : The mean follow-up duration for these 43 children was $7.2{\pm}1.5$ years (range, 4.5 to 13.0 years). Of these, 13 (30.2%) had cryptogenic and 30 (69.8%) had symptomatic IS. Eleven (25.6%) children were initially treated with adrenocorticotrophic hormone (ACTH) therapy, with a mean treatment lag of $1.3{\pm}1.9$ months (range; 0.1 to 7.0 months). Eighteen (41.8%) children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2%) children had at least moderate neurodevelopmental disorders and 2 (4.8%) died. In univariate analysis, etiology (symptomatic) and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

• 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.

• Women's Health Nursing
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• v.6 no.2
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• pp.234-257
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• 2000

At present in the medical care, the study and effort for producing health service to consider efficiency, effectiveness, and quality are urgently called for because of the difficulty in the keen competition according to the inter- nationalization and opening, the operation in the medical institution service testing system, the change in the medical policy of KDRGs, and the lack of the health care cost increasing rate. As an alternative, the case management for the new management system is introduced in the U.S., and the Critical Pathway that is the method designing the contents of activity and its result has been developed and applied in order to anticipate and manage the patient-outcome for the realization of the cost-effective case-management. Thus, this study intended to analyze the effectiveness to obtain by developing the Critical Pathway presented as the method to improve the quality-betterment and cost effectiveness through the continuous and consistent patient management for the hysterectomy patient and applying it to the real practice. As a study method, this author formed a conceptual framework through considering five Critical Pathway used in the current U.S. and three Critical Pathway presented in the literature to develop the Critical Pathway for the hysterectomy patient, and made out the preliminary Critical Pathway through reviewing the old chart. This author made the verified the validity of the expert group about the developed Critical Pathway, and to confirm the possibility of practice application, completed and settled the final Critical Pathway after using the Critical Pathway to the hysterectomy patient from March 1st to 15th, 1997. Finally, to analyze the application-effect of the developed Critical Pathway, this author offered health care service applying the Critical Pathway to the hysterectomy patient from April 15th to August 31th, 1997. The guide for the Critical Pathway was carried out in advance by outpatient setting nurse for outpatient setting visit before the operation, and after hospitalization the primary nurse monitored the execution degree on the every duty. After discharge this author surveyed the complication through phone visiting, and one month after discharge surveyed the patient's reaction about the offered service when outpatient setting visit and analyzed the result. The source for health care cost was obtained by the statistics about the hospital charge which was offered by the General Business Department. The results were as follows. 1. It was decided that the vertical line of the Critical Pathway was made up of eight items such as monitoring/assessment, treatment, line/drains, activity, medication, lab test, diet, patient teaching, and the horizontal line of the Critical Pathway was made up of from hospitalization to discharge. 2. After the analysis of service contents through reviewing the old chart, it was decided that the horizontal line of the preliminary Critical Pathway was made up of from hopitalization to fourth postoperative day, and the vertical line of it was divided into eight items which were the contents to occur with the time frame of the horizontal line. 3. After the verifying the validity of the expert group about the preliminary Critical Pathway, the horizontal line was amended from hopitalization to third postoperative day, and taking their consensus, some contents of the horizontal line was amended and deleted. 4. From March 1st to 15th, 1997, to confirm the clinical suitability, this author offered eight hysterectomy patients the medical service through the Critical Pathway. The result was that three of them could be discharged at the expected discharge day, and the others later than that day. Supplementing the preliminary Critical Pathway through analyzing the cause of that delay- case, this author developed the final Critical Pathway. 5. There were no significant differences between the experimental and the control group in the incidence of complication(P > 0.05). 6. The 92.4% of experimental group was satisfied with the Critical Pathway service. 7. The length of hospital stay of the experimental group offered with the Critical Pathway service was 4.6 days and there was a significant difference that it was 1.3 days shorter than that of the control group(t=-29.514, P=0.000). 8. There wsa a significant difference that the mean medical charge per one patient of the experimental group offered the Critical Pathway service was cheaper \124,150 than that of the control group(t=-9.826, P=0.000). 9. The result that the author assumed and analyzed hospital income with the rate of turning bed was assumed that the increase of hospital income was \63,245,072 for that study, and the income increase was expected with \68,704,864 for a year. The result that this author applied the Critical Pathway to the hysterectomy patient have no differences in the incidence of complication, high satisfaction with that service, and the length of hospital stay decreased in the experimental group, and the mean hospital charge per one patient decreased, but hospital income increased. Suggestions for further study and nursing practice are as follows. 1. The study to apply the Critical Pathway for a year, verify the validity, and measure the effect repeatedly is needed. 2. To apply and manage the Critical Pathway effectively, the study to computerize it is needed. 3. The study to develop hospital-based Critical Pathway about other diseases or procedure, and measure the effect is needed.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.15
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• pp.6627-6632
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• 2015

Background: We conducted a study exploring the clinical safety and efficacy of decitabine in patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS), combined with a complex karyotype. Materials and Methods: From April 2009 to September 2013, a total of 35 patients with AML/MDS combined with a complex karyotype diagnosed in the First Affiliated Hospital of Soochow University were included for retrospective analysis. All patients were treated with decitabine alone ($20mg/m^2$ daily for 5 days) or combination AAG chemotherapy (Acla 20mg qod*4d, Ara-C $10mg/m^2$ q12h*7d, G-CSF $300{\mu}g$ qd, the dose of G-CSF adjusted to the amount in blood routinely). Results: In 35 patients, 15 exhibited a complete response (CR), and 6 a partial response (PR), the overall response rate (CR+PR) being 60% (21 of 35). Median disease-free survival was 18 months and overall survival was 14 months. In the 15 MDS patients with a complex karyotype, the CR rate was 53.3% (8 of 15); in 20 AML patients with complex karyotype, the overall response rate was 65% (13 of 20). The response rate of decitabine alone (22 cases) was 56.5% (13 of 22), while in the combination chemotherapy group (13 cases), the effective rate was 61.5% (8 of 13)(P>0.05). There are 15 patients with chromosome 7 aberration, after treatment with decitabine, 7 CR, 3 PR, overall response rate was 66.7% (10 of 15). Of 18 patients with 3 to 5 kinds of chromosomal abnormalities, 66.7% demonstrated a response; of 17 with more than 5 chromosomal abnormalities, 52.9% had a response. In the total of 35 patients, with one course (23 patients) and ${\geq}$two courses (12 patients), the overall response rate was 40.9% and 92.3% (P<0.05). Grade III to IV hematological toxicity was observed in 27 cases (75%). Grade III to IV infections were clinically documented in 7 (20%). Grades I to II non-hematological toxicity were infections (18 patients), haematuria (2 patients), and bleeding (3 patients). With follow-up until September 2013, 7 patients were surviving, 18 had died and 10 were lost to follow-up. In the 6 cases who underwent allogeneic hematopoietic stem cell transplantation (HSCT) all were still relapse-free survivors. Conclusions: Decitabine alone or combination with AAG can improve outcome of AML/MDS with a complex karyotype, there being no significant difference decitabine in inducing remission rates in patients with different karyotype. Increasing the number of courses can improve efficiency. This approach with fewer treatment side effects in patients with a better tolerance should be employed in order to create an improved subsequent chance for HSCT.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.4
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• pp.1811-1815
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• 2014

Background: In clinical trials with no upper age limit, the proportion of older patients is usually small, probably reflecting the more conservative approach adopted by clinicians when treating the elderly. An exploratory analysis of elderly patients in the RECORD-1 Trial showed that patients ${\geq}$ 65 y.o. had superior median PFS than overall RECORD-1 population (5.4 months and 4.9 months, respectively). We investigated the efficacy, relative benefit and safety of Everolimus (EVE) as sequential therapy after failure of VEGFr-TKI therapy for older patients with metastatic renal cell cancer (mRCC), in daily practice. Materials and Methods: 172 consecutive IRB approved patients with mRCC (median age 65, M:F 135/37, 78% clear cell) who received salvage EVE at 39 tertiary institutions between October 2009 and August 2011 were included in this analysis. Some 31% had progressed on sunitinib, 22% on sorafenib, 1% on axitinib, 41% on sequential therapy, and 5% had received other therapy. Patients with brain metastases were not included and 95% of the patients had a ECOG (Eastern Cooperative Oncology Group) performance status (PS) of 0 or 1. Previous radiotherapy was an exclusion criterion, but prior chemotherapy was permitted. Adequate organ function and hematologic parameters were mandatory. EVE administration was approved by the institutional review board at each participating institution and signed informed consent was obtained from all patients. Results: Median time of the whole cohort to last follow-up was 3.5 months (range 0.4-15.2 months). Forty four percent were continuing to take EVE at last followup. There were 86 (50%) patients ${\geq}$ 65 y.o. and 86 (50%) <65 y.o. The percentage of patients who showed PR/SD was higher in the older group than in the younger one (5.9%/61.2% vs 1.2%/46.5%, respectively). Median survival of older patients was also significantly longer (3.5 +/- 0.31 vs 3.1 +/- 0.34, hazard ratio=0.45, CI; 0.255-0.802). Analysis using Cox regression model adjusted for gender, PS, number of metastases, site of metastases, histology, smoking history and age detected an association between age and PFS (p=0.011). The frequency of adverse events in elderly patients treated with EVE was no greater than that in younger patients, although such toxicity may have had a greater impact on their quality of life. Conclusions: Older patients should not generally be excluded from accepted therapies (mTOR inhibitors after failure of VEGFr-TKI therapy) for mRCC.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.178-187
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• 2002

Purpose : The detection of hydronephrosis(HN) with antenatal ultrasonography was first reported in the 1970s. Prenatal HN is diagnosed with an incidence of 1:100 to 1:500 on antenatal screening. Recently, the purpose of antenatal screening has changed from simple detection to selection for specific diagnosis-based management. this study is to evaluate the usefulness of antenatal sonography for HN and to investigate the differential causes of HN and their clinical outcomes. Patients and methods : 11,783 live neonates with prenatal ultrasonographic examination at Ajou University School of Medicine, from Sep. 1994 to Aug. 2001 were analyzed. Results and conclusion : Hydronephrosis (>10 mm) was detected in 119 (1.0%) cases antenatally and among these, 91 were proved to have HN postnatally Males were three times more affected than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis (47%), followed by multicystic dysplastic kidney, vesicoureteral junction obstruction and vesicoureteral reflux. During 20 months' follow-up(3 to 72 months), 58(48%) renal units showed spontaneous resolution and surgical interventions were necessary in 10 (7.4%) of postnatally confirmed hydronephrotic renal units.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.17-23
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• 1997

After the first description of IgA nephropathy by Berger in 1968, the prognosis of this disease was considered favourable. However recent studies have revealed that IgA nephropathy result in end stage renal desease in 25-30% by 20 years. Heavy proteinuria, hypertension, histological high class are regarded as poor prognostic factors. In 1996, Yagame et al reported the new histopathologic grading with a strong correlation between the grading, heavy proteinuria, high s-Cr level and renal survival. The aims of this study are to determine whether the pathological grading and other clinical parameters could contribute to predicting the outcome of this disease eventhough pediatric patients. Seventy nine patients (59 males, 20 females) with IgA nephropathy were examined. Patients were 2.08-15.17 years of age ($9.85{\pm}2.83$). The mean follow-up duration were $27{\pm}28$ months. Six of seventy nine patients progressed to chronic renal failure during the follow-up periods. High 24h urinary protein excretion at diagnosis were significantly higher in chronic renal failure patients (p<0.05). Hypertension at diagnosis were the significant associated factors in progression of chronic renal failure (p<0.05). Histological changes of IgA nephropathy in light microscopy were classified into five classes by WHO classification, four grades in Yagame's gradings. Among the seventy nine patients, 24 were as class 1, 30 as class 2, 23 as class 3; 4 as class 4, 0 as class 5 by WHO classification. 23 were classified grade 1, 31 as grade 2, 24 as grade 3, 1 as grade 4 by Yagame's grading. Among six patients who progressed to chronic renal failure, 1 clssified as class 1, 1 as class 2, 3 as class 3, 1 as class 4, 0 as class 5 by WHO Classification. 1 patients were classified as grade 1, 1 as grade 2, 3 as grade 3, 1 as grade 4 by Yagame's grading. (p>0.05) In conclusion, hypertension and heavy proteinuria at initial presentation were significantly associated with progression of chronic renal failure. The classification of WHO & Yagame's grading has no significant association with the progression of chronic renal failure in pediatric patients.