• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.178-185
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• 2005

Purpose : The purpose of this study was to evaluate the outcome of children with juvenile myelomonocytic leukemia(JMML) treated with allogeneic hematopoietic stem cell transplantation(allo-HSCT). Methods : Eleven JMML patients aged 8-39 months underwent allo-HSCT. The sources of grafts were unrelated donors(n=7), HLA-matched siblings(n=3) and an HLA 1-antigen mismatched familial donor. All patients had received chemotherapy ${\pm}13$-cis-retinoic acid(CRA) before transplant, and CRA was used, posttransplant, in six patients. Results : Only three patients were in complete remission(CR) at the time of transplantation. Initial chimeric status revealed complete donor chimerism(CC) in five patients, mixed chimerism(MC) in five and autologous recovery(AR) in one. One patient with MC having persistent splenomegaly eventually turned to CC and CR after rapid tapering of cyclosporine, combined with daily use of CRA. An AR case relapsed shortly after transplant but was rescued with second, unrelated cord blood transplantation. Ultimately, six patients are alive, event-free, with a median follow-up of 15.5 months posttransplant. All three deaths occurred in patients who failed to achieve CC, leading to disease progression. Conclusion : We suggest that graft-versus-leukemia effect play an important role and CRA a possible role in posttransplant leukemic involution in JMML. In patients whose leukemic burden is still high with MC after transplant, early tapering of immunosuppressants and introduction of CRA might provide a chance of a cure for some patients.

• Journal of Korean Ophthalmic Optics Society
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• v.19 no.1
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• pp.31-42
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• 2014

Purpose: The present study was conducted to investigate the effect of the physical properties of RGP lens induced by lens polishing on the actual wearer's subjective comfort, the tear film break-up time and the blinking rate as a follow-up study that revealed the relationship between the lens physical properties during lens manufacturing and lens wearer's factors. Methods: RGP lenses made from the three different polishing conditions (25, 50 and 100 seconds) were applied on 28 eyes of 17 subjects, aged 20 to 29 years, without any known disease and surgical history in the eyes. While the subjects were asked to wear the RGP lenses longer than a week, the questionnaire for the comfortability was administered everyday. Subjective and objective tear break-up time and blinking rate of the wearers were further measured. Results: The wearer's subjective comfortability showed some difference in the type of discomfort and satisfaction score according to the polishing status when wearing RGP lenses made from different polishing conditions longer than a week, and a bigger difference in satisfaction score induced by polishing condition was especially shown in experienced RGP lens wearer rather than un-experienced wearer. In the case of RGP lens wearer compared with the ones without the wearing experience, as the wearing time increased subjective and objective tear break-up time were increased and the blinking rate was decreased. However, subjective and objective tear break-up time were tended to decrease with even longer wearing time when wearing the RGP lens made from the polishment for 100 seconds. Conclusions: These results confirmed that the optimization of physical properties of the lens may not give the same effect on the wearer's subjective and objective symptoms and other factors when actual wearing. From the results, it can suggest that the success rate of RGP lens wear may be changed by physiological factors such as the stabilization of wearer's tear film, comfortability and lens wearing experience when wearing RGP lens based on 'The manufacturing standard for soft contact lens/hard contact lens' provided by Korea Food and Drug Administration.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.197-203
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• 2005

Purpose : Changes in metalloproteinases(MMP) activity have been demonstrated in several disease states, including rheumatoid arthritis and tumor metastasis. More importantly, increased myocardial MMP activity has been reported to occur in both clinical and experimental forms of dilated cardiomyopathy. There was no report about MMP in adriamycin(ADR)-induced cardiomyopathy. The purpose of this study was to investigate gene expression of MMP and tissue inhibitor of metalloproteinases(TIMP) in ADR-induced cardiomyopathy and clarify the relationship between MMP and cytokines. Methods : Male Sprague-Dawley rats were divided into two groups. The first group was control. The second group was given intraperitoneal injections of ADR(5 mg/kg) twice a week over two weeks. Serum concentrations of MMP, TIMP, interleukin(IL)-6 and tumor necrosis factor(TNF)-${\alpha}$ were measured. RNA extraction was performed from frozen rat hearts. Reverse transcription polymerase chain reaction(RT-PCR) was employed. cDNA Microarray analysis was performed by using a set of 5,184 sequence-verified rat cDNA clones. Results : Serum MMP and TIMP levels were not significantly different between the two groups. IL-6 was $36.8{\pm}2.8pg/mL$ and TNF-${\alpha}$ $2.2{\pm}2.7pg/mL$ in the ADR group. They were significantly higher than in the control group. Serum MMP correlated significantly with TNF-${\alpha}$(r=0.41, P<0.05). There was no gene expression of MMP, IL-6 or TNF-${\alpha}$ in the hearts of both groups. Gene expression of TIMP was significantly depressed in the hearts of the ADR group. Conclusion : These results suggested a potential role for TNF-${\alpha}$ in the regulation of extracellular matrix remodeling in ADR induced cardiomyopathy. Rapid screening of multiple decreased gene expression by DNA chip may be a useful diagnostic test to detect early cardiac injury before developing ADR induced cardiomyopathy.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1389-1396
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• 2002

Purpose : This study was done to analyze the changes in the clinical conditions and the diagnosis of hypertrophic pyloric stenosis. Methods : We report a retrospective clinical analysis of 39 patients with hypertrophic pyloric stenosis from Jan. 1992 to Aug. 2001. The age and sex distribution, family and birth history, clinical symptoms, the ultrasonographic and the operative sizes of pyloric canals were compared. Results : The body weight was below the 3 percentile at admission in eight cases(20.5%). "Olive like mass" in right upper quadrant was palpated during physical examination in 23 cases(59%) and gastric peristaltic wave observed in six cases(15%). The ultrasonographic measurements showed that the pyloric muscle thickness to be $4.95{\pm}0.99mm$($mean{\pm}SD$), pyloric diameter $14.42{\pm}2.64mm$, and pyloric length $20.17{\pm}3.92mm$. Fredet-Ramstedt pyloromyotomy was employed in all cases. The operative measurements of the pyloric muscle thickness was $5.11{\pm}1.01mm$, pyloric diameter $15.01{\pm}2.47mm$, and pyloric length $22.32{\pm}3.43mm$. Conclusion : There was no significant difference between the ultrasonographic and operative measurements. Currently, the hypertrophic pyloric stenosis patients showed lesser clinical hallmarks of the disease. The earlier diagnosis using imaging studies before development of significant metabolic abnormalities is becoming an important factor that change the future outcomes of hypertrophic pyloric stenosis.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1512-1518
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• 2002

Purpose : Twins have a higher mortality and morbidity than singletons. Co-twin with one fetal death is particularly at risk. We investigated the neonatal outcome of live co-twins when one fetus had died after the 20th gestational week, and associated risk factors. Methods : A retrospective study was performed in fifteen cases of twin pregnancy with single intrauterine fetal deaths after the 20th gestational week during the period from January 1996 to December 2000 at Chonnam University Hospital. Results : Gestational age was $33.7{\pm}3.2weeks$, birth weight was $1,992{\pm}592g$. Interval between one fetal death being detected and the delivery of a live co-twin was $32.4{\pm}29.5days$. There were 11 cases(73.3%) of premature babies less than 37 gestational weeks. Main causes of preterm delivery were preterm labor and premature rupture of membranes. Hematologic findings suggesting disseminated intravascular coagulopathy(DIC) were not found in all mothers before delivery, and was not associated with DIC and encephalomalacia of the live co-twin. Perinatal outcome of fifteen live co-twins was as follows : six were normal(40%), three were DIC(20.0%), three were encephalomalacia(20.0%), one suffered intrauterine growth retardation, there was one case of twin to twin transfusion syndrome, and one of congenital heart disease(atrial septal defect with pulmonary stenosis). The occurrence of DIC and encephalomalacia in live co-twins was not related to placental chorionicity, birth weight, gestational week, and the interval between the detection one fetal death and the delivery of a live co-twin. Conclusion : We could not find any maternal hematologic problems in twin pregnancies complicated by one fetal death. Twenty percent of live co-twins showed DIC and encephalomalacia. However, its associated risk factors were not found. We need to investigate more closely the cases of live co-twins with one intrauterine fetal death.

• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.989-995
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• 2003

Purpose : Dexamethasone is frequently administered to prevent or treat chronic lung disease in human neonates who are also prone to hypoxic-ischemic(HI) insults. Recently, meta-analysis of the follow-up studies reveals a significantly increased odd ratio for the occurrence of cerebral palsy or an abnormal neurologic outcome, and there is conflicting evidence regarding the impact of dexamethasone exposure on HI brain injury. This study was conducted to explore the effect of post-HI dexamethasone administration on neuronal injury in neonatal rats. Methods : HI was produced in seven-day-old rats by right carotid artery ligation followed by two hours of 8% oxygen exposure. At the end of HI, the animals were injected intraperitoneally either with dexamethasone(0.5 mg/kg) or saline. Neuronal injury was assessed seven days after the HI by the area of infarction, TUNEL reactivity, Bcl-2 and Bax expression in brain. Results : Post-insult dexamethasone administration resulted in reduction of weight gain and a higher mortality rate during seven days after HI. Dexamethasone treatment revealed no effect on the size of brain infarction induced by HI. Bax protein expression increased in dexamethasone treated brain but Bcl-2 protein expression and TUNEL reactivity revealed no significant differences between dexamethasone treated and non treated brain. Increased Bax protein expression suggest upregulation of the apoptosis by dexamethasone. Conclusion : The result suggests the adverse role of Post-HI administration of dexamethasone in neonatal HI.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.548-553
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• 2010

Purpose : IgA nephropathy (IgAN) is the most commonly occurring form of chronic glomerulonephritis in pediatric cases. Human leukocyte antigen (HLA) genes have been implicated in various inflammatory and autoimmune diseases. The present study was conducted to investigate the association between 2 single nucleotide polymorphisms (SNPs) of the HLA-G gene and childhood IgAN. Methods : The authors analyzed and compared $HLA-G$ gene SNPs (rs1736936 and rs2735022) in 174 patients with childhood IgAN and in 438 healthy controls. In addition, IgAN patients were dichotomized and compared with respect to proteinuria (< and >$4mg/m^2/hour$), the presence or absence of podocyte foot process effacement, and the presence of pathologically early and advanced disease markers such as interstitial fibrosis, tubular atrophy, or global sclerosis. Results : No significant SNP frequency differences were observed for the $HLA-G$ gene between IgAN patients and the control group. Moreover, no significantly associated SNP was observed with the presence of proteinuria, podocyte foot process effacement, or pathologically advanced markers. However, the haplotype, composed of rs1736936 and rs2735022, showed a significant association with the susceptibility to develop childhood IgAN (haplotype T/C: dominant model, $P$=0.049; haplotype C/T: recessive model, $P$=0.030). Conclusion : Our results indicate that rs1736936 and rs2735022 as the $HLA-G$ gene promoter haplotype might be associated with the susceptibility to develop childhood IgAN in the Korean population.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.592-597
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• 2010

Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$ $aureus$) and $Pseudomonas$ $aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.

• Clinical and Experimental Pediatrics
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• v.50 no.3
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• pp.268-271
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• 2007

Purpose : Premature narrowing of the foramen ovale is rare but serious clinical entity. Prenatal narrowing or obstruction of the foramen ovale shows symptoms such as right heart failure, fetal hydrops, triscupid regurgitation, left heart obstructive disease, and supraventricular tachycardia. This study aimed to assess the prenatal diagnosis and postnatal clinical course of restrictive foramen ovale in utero in otherwise normal heart. Methods : The subjects were five patients diagnosed with restrictive foramen ovale in utero from January 2001 to June 2005 at Chungnam National University Hospital. The diagnostic criteria was defined when the maximum diameter in a 4-chamber view is less than 2.5 mm and there is a continuous doppler velocity at the foramen ovale of more than 0.6m/s. Results : At the time of diagnosis of restrictive foramen ovale, gestation age was 34~37 wks, and chief complaints were fetal arrhythmia(2 cases), pericardial effusion, Ebstein anomaly and subaortic stenosis. Two cases which were diagnosed fetal hydrops and supraventricular tachycardia delivered by emergent cesarian section. Five cases were found to have right heart dilatation on echocardiogram after birth, but right heart dilatation became normalized at day 7 after birth and the clinical courses were not eventful. Conclusion : Identifying an obstructed foramen ovale in the fetus warrants the further search for additional cardiac and extracardiac anomalies, which may alter the prognosis. Delivery should be induced if possible in cases of foramen ovale obstruction with signs of cardiac decompensation.

• Journal of the Korean Society of Food Science and Nutrition
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• v.34 no.9
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• pp.1398-1406
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• 2005

The purpose of this study was to investigate effects of web-based nutrition counseling on nutrient intake and blood glucose in type ll diabetic Patients. Forty type II diabetic patients, twenty one of them were diabetic patients without complication (Ncx-DM) and nineteen of them were diabetic patients with complication (Cx-DM), participated in a web-based nutrition counseling program. At the first nutrition counseling, the patients were counselled through interview and then follow up nutrition counseling was accomplished four times during eight weeks through tile web-based internet program. Various markers of disease risk including anthropometric indices, nutrient intake and blood glucose were measured before and after the nutrition counseling. After the nutrition counseling, body mass index and waist circumference decreased in both group but did not change significantly. Fasting blood glucose significantly decreased from 153.9 mg/dL to 139.0 mg/dL (p<0.05) in NCX-DM and from 178.2 mg/dL to 128.5 mg/dL (p<0.01) in Cx-DM after the nutrition counseling. Glycosylated hemoglobin level decreased from $9.3\%$ to $8,7\%$ in Ncx-DM and significantly decreased from $9.7\%$ to $7.8\%$ (p<0.01) in Cx-DM after the nutrition counseling. In addition, total cholesterol and LDL-cholesterol level significantly decreased in both group (P<0,05) after the nutrition counseling. Energy intake decreased significantly in Ncx-DM (P<0.05) and Cx-DM (p<0.01). Although the nutrient intake did not change significantly, the nutrient intake was improved after the nutrition counseling. Therefore, this study shows that the web-based nutrition counseling is effective in improving energy and nutrient intake and influences positively in blood glucose and serum lipids of the patients. In addition, these results indicate that the internet presents us with potential as a new medium for nutrition counseling in informationized society.