• Food Science and Preservation
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• v.19 no.3
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• pp.337-343
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• 2012

The objective of this study was to characterize three different commercial (A, B and C) and two handmade (HM-AP, atmospheric pressure; HM-RP, reduced pressure) strawberry jams in relation to soluble solids, pH, total acid, total polyphenol, anthocyanin, color values, texture properties, and sensory evaluation. The soluble solid contents varied from 62.33 to 68.33 $^{\circ}Brix$, and the pH ranged from 3.59 to 3.70. The color L and a values were the highest in the HM-RP strawberry jam (p<0.05). The total polyphenol contents of commercial jams A, B, and C were 56.10, 97.59, and 105.85 mg GAE/100 g, respectively, and those of the HM-AP and HM-RP of handmade jams were 156.13 and 189.94 mg GAE/100 g. The anthocyanin contents of A, B, and C commercial jams were 1.51, 0.95, and 0.80 mg/100 g, respectively, and those of the HM-AP and HM-RP handmade jams were 2.64 and 9.16 mg/100 g. The phenolic contents of the HM-RP jam were significantly much higher than those of the other jams. The hardness ranged from $5.67{\times}10^3$ (HM-AP jam) to $41.91{\times}10^3$ (jam B) dyne/$cm^2$, the jelly strength ranged from 40.08 (HM-AP jam) to 180.33 (jam B) dyne, and the strength ranged from 83.84 (jam C) to 302.93 (jam B) g. The sensory evaluation of the color, flavor, sweetness, sourness, viscosity and overall acceptability of the HM-RP jam showed higher values than those of the other jams. Especially, the highest value of the color score was found in the HM-RP jam. The electon donating abilities of jams A, B, and C and of the HM-AP and HM-RP jams were 44.27, 41.70, 53.06, 69.08, and 73.21%, respectively. These results indicated that the HM-RP strawberry jam prepared with reduced pressure using micro-oxygen technology was a good source of phenolic compounds, total polyphenols and anthocyanin, and had a high level of antioxidant activity.

• Journal of Chest Surgery
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• v.38 no.2 s.247
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• pp.110-115
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• 2005

Background: The aims of this paper were to review the mid term clinical results and to analyze the preoperative risk factors of isolated aortic valve replacement (AVR). Material and Method: Between January 1992 and February 2003, 80 patients underwent isolated AVR. 58 were male and 22 were female patients, raging from 12 to 75 years of age (mean :$46.8{\pm}13.0$ years). 74 patients except one early death and 5 follow-up loss were contacted by OPD or by telephone. The mean duration of follow-up was $44.2{\pm}29.7$ months and the total cumulative period was 272.8 patient-year. Result: The complications in hospital occurred in 35 cases : 12 wound problems (11 superficial, 1 deep), 11 arrhythmias (9 temporary, 2 persistent), 3 low cardiac output, and so forth. The late deaths were 4 cases : the heart-related deaths were 2 cases ($0.7\%$ patient-year). Conclusion: The risk factors that influenced the early mortality and morbidity were older age (> 60 years)(p=0.04), poor preoperative NYHA functional class (> 3) (p=0.048), high preoperative serum creatinin level (> 1.2 mg/100 ml)(p=0.031), long operation time (aortic clamping time>90 min)(p=0.042). The same factors influenced the late mortality and morbidity. Freedom from valve-related complication was $86.4{\pm}5.3\%,$ actuarial survival rate were $96.8{\pm}2.3\%$ at 3 years and $90.8{\pm}4.6\%$ at 10 years.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.133-141
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• 2003

Purpose : Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN. Methods : Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed aster 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN. Results : The median onset age was 13.4 years. Clinical manifestations were nephrotic syn-drome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups. Conclusion : With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• Journal of Chest Surgery
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• v.32 no.7
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• pp.621-627
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• 1999

Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.