• Childhood Kidney Diseases
• /
• v.9 no.2
• /
• pp.222-230
• /
• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Journal of Chest Surgery
• /
• v.39 no.8 s.265
• /
• pp.588-597
• /
• 2006

Background: The surgical repair of a tetralogy of Fallot (TOF) has been performed successfully with a favorable early and late outcome. However, the later development of pulmonary regurgitation and stenosis remains a problem. The development of pulmonary regurgitation and stenosis may be changed by the size of right ventricular outflow tract (RVOT) reconstruction at the initial total correction. Hence, it is necessary to investigate the ideal size of RVOT reconstruction. Material and Method: This prospective study was carried out to determine how a surgical strategy and the RVOT size affect the occurrence of pulmonary regurgitation and stenosis. From January 2002 to December 2004, 62 patients underwent the TOF total correction. The RVOT size (diameter of pulmonary valve annulus) of each case was measured after the RVOT reconstruction and converted to a Z value. A pre-scheduled follow up (at discharge, 6 months, 1 year, 2 years and 3 years) was carried out by echocardiography to evaluate the level of pulmonary regurgitation and stenosis. Result: The patients were divided to two groups (transannular group n=12, nontransannular group n=50) according to the method of a RVOT reconstruction. The Z value of RVOT=iameter of pulmonary valve annulus) (transannular group -1, $range\;-3.6{\sim}-0.8;$ nontransannular group -2.1, $range\;-5.2{\sim}-1.5)$ and the average pRV/LV after surgery ${(transannular group 0.44{\pm}0.09,\;nontransannular group\;0.42{\pm}0.09)}$ did not show any significant difference between two groups. The occurrence of pulmonary regurgitation above a moderate degree was more frequent in the transannular group (p<0.01). In nontransannular group, the development of pulmonary regurgitation more than moderate degree occurred to the patients with larger RVOT size (Z value>0, p<0.02) and the progressing pulmonary stenosis more than mild to moderate degree developed in the patients with smaller RVOT size (Z value<-1.5, p<0.05). A moderate degree of pulmonary stenosis developed for 4 nontransannular patients. Three underwent additional surgery and one underwent a balloon valvuloplasty. Their Z value of RVOT were -3.8, -3.8 -2.9, -1.8, respectively. Conclusion: When carring out a TOF total correction, transannular RVOT reconstruction group has significantly more pulmonary regurgitation. In the nontransannular RVOT reconstruction. the size of the RVOT should be maintained from Z value -1.5 to 0. If the Z value is less than -1.5, we should follow up carefully for the possibility of pulmonary stenosis.

• Clinical and Experimental Pediatrics
• /
• v.45 no.6
• /
• pp.773-782
• /
• 2002

Purpose : To identify the necessity of more reasonable diagnostic criteria and the possibility of early prediction of coronary involvement in the higher risk group, we investigated and compared clinical and laboratory findings in the acute phase and coronary involvements in those younger (n=17) and older(n=53) than one year of age in Kawasaki disease(KD). Methods : Retrospective chart reviews were performed on 70 patients with KD who were admitted to the Chung-Ang University Hospital from April 1997 to May 2001. Results : Male were significantly higher in the younger age group(M : F ratio 3.3 : 1 vs. 1.0 : 1, P=0.004). Fever durations before intravenous immunoglobulin(IVIG) and echocardiography were significantly shorter in the younger group($4.6{\pm}1.3$ vs. $6.2{\pm}2.5$, P=0.004 vs. 0.01, respectively). Cases meeting typical diagnostic criteria were significantly less in the younger group(P=0.006). In the laboratory findings, serum albumin, BUN and $K^+$ levels in the acute febrile phase were significantly higher in the younger group(P=0.002, 0.006, <0.001, respectively) and incidences of coronary artery dilatation in the acute phase were significantly higher in the younger group(P=0.01). Conclusion : Although less met the typical diagnostic criteria of KD, infants younger than one year of age are more susceptible to coronary artery change in the acute febrile phase. Therefore, KD should be entertained as a diagnostic possibility in young infants with prolonged fever without distinct fever focus, and echocardiography should be considered as part of the evaluation of these patients, and then early diagnosis and prompt IVIG should be conducted.

• Journal of Nutrition and Health
• /
• v.41 no.5
• /
• pp.439-447
• /
• 2008

There is a limitation to estimate vitamin $B_{12}$ intake due to lack of data on vitamin $B_{12}$ content in many Korean foods. In this study, vitamin $B_{12}$ content was determined in some soybean or vegetable-fermented foods, edible seaweeds and other frequently consumed foods in Korea by microbioassay using Lactobacillus delbruecki ATCC 7830. The traditional type of Doenjang and Chungkookjang contained 1.85 ${\mu}g/100$ g and 0.69 ${\mu}g/100$ g of vitamin $B_{12}$, respectively, while the factory-type of Doenjang and Chungkookjang contained 0.04-0.86 ${\mu}g/100$ g and 0.06-0.15 ${\mu}g/100$ g. Vitamin $B_{12}$ was not detected in steamed soybeans and Tofu which is a not-fermented soybean product, indicating that vitamin $B_{12}$ in Doenjang and Chungkookjang might be produced during the fermentation process. The Korean-style soy sauce contained 0.04 ${\mu}g$ vitamin $B_{12}$/100 mL, but vitamin $B_{12}$ was not detected in Japanese-style soy sauce and white miso. Commercial Kimchi, a representative Korean vegetable- fermented food, made of Korean cabbage, Yeolmu, or Mustard leaves contained 0.013-0.03 ${\mu}g$ vitamin $B_{12}$/100 g, while Kimchi without red pepper and fermented fish sauce (White Kimchi) did not. Vitamin $B_{12}$ content was very high in some edible seaweeds such as laver (66.76 ${\mu}g/100$ g dry weight) and sea lettuce (84.74 ${\mu}g/100$ g dry weight), and it was 17.12 ${\mu}g/100$ g of dried small anchovy, 1.07 ${\mu}g/100$ g of whole egg, and 0.02 ${\mu}g/100$ g of coffee mix. From these results, it is assumed that Koreans take substantial amount of vitamin $B_{12}$ from plant-origin foods. And, with these data, we will be able to calculate dietary vitamin $B_{12}$ content more correctly than before. In conclusion, soybean-fermented foods, Kimchi, laver and sea lettuce are recommendable as good sources of vitamin $B_{12}$ for vegetarians or Korean elderly on grain and vegetable based diet.

• Journal of the Korean Society of Food Science and Nutrition
• /
• v.41 no.10
• /
• pp.1346-1355
• /
• 2012

This study was carried out to investigate the characteristics of black jujube and Zizyphus jujube extracts during lactic acid fermentation. Both extracts were fermented using Lactobacillus fermentum YL-3. As a result, viable cell number rapidly increased until 24 hours, after which it gradually decreased. Before lactic acid fermentation, the $IC_{50}$ of black jujube, which was 0.014 mg/mL, was lower than that of Zizyphus jujube. Further, black jujube showed stronger antioxidant activity (374.21 mg AA eq/g) than Zizyphus jujube. Contents of total polyphenolics in both extracts were 15.46 mg/g and 13.61 mg/g, respectively, whereas contents of total flavonoids were 374.21 ${\mu}g/g$ and 64.25 ${\mu}g/g$. After lactic acid fermentation, there was no significant increase in DPPH or ABTS free radical scavenging activity. Total polyphenolic content of Zizyphus jujube decreased to 12.39 mg/g upon fermentation, whereas flavonoid content significantly increased to 291.58 ${\mu}g/g$. Further, polyphenolic and flavonoid contents of black jujube increased from 15.46 mg/g to 17.46 mg/g and from 374.21 ${\mu}g/g$ to 1,135.29 ${\mu}g/g$, respectively. These results demonstrate that 9-Times Steamed and Dried increased functional components. Especially, lactic acid fermented black jujube showed remarkably high antioxidant activity. These results confirm the potential use of lactic acid fermented black jujube as a valuable resource for the development of functional foods.

• Tuberculosis and Respiratory Diseases
• /
• v.41 no.2
• /
• pp.103-110
• /
• 1994

To find out the predictors of nocturnal arterial oxygen desaturation in patients with respiratory diseases, transcutaneous oxygen saturation($StcO_2$) monitoring studies using a pulse oximeter were performed during sleep in 20 patients. $StcO_2$ was decreased more than 4% from the baseline value in 18 patients(90%) and more than 10%("Desaturator") in 8(40%). Five of the seven patients(71.4%) with awake $PaO_2$<60mmHg and three of the thirteen patients(23.1%) with awake $PaO_2{\geq}60mmHg$ were "desaturators". The awake $PaO_2/FIO_2$ and $PaO_2/PAO_2$ could distinguish "desaturator" from "nondesaturator", and $PaO_2,\;SaO_2$ or $StcO_2$ could not. These results suggest that the nocturnal oxygen desaturation depends on the severity of the underlying disease rather than the baseline $PaO_2$. Anthropomorphic and lung function factors could not separate between "desaturator" and "non-desaturator", and about a quater of patients with a wake $PaO_2{\geq}60mmHg$ developed significant desaturation. Therefore, it is necessary to monitor the nocturnal arterial oxygen saturation in patients with respiratory diseases regardless of their severity of airflow obstruction or awake $PaO_2$.

• Journal of the Korean Society of Food Science and Nutrition
• /
• v.43 no.5
• /
• pp.631-640
• /
• 2014

The inhibitory effects of Boswellia serrata (BW) extracts on degenerative osteoarthritis were investigated in primary-cultured rat cartilage cells and a monosodium-iodoacetate (MIA)-induced osteoarthritis rat model. To identify the protective effects of BW extract against $H_2O_2$ ($800{\mu}M$, 2 hr) in vitro, cell survival was measured by MTT assay. Cell survival after $H_2O_2$ treatment was elevated by BW extract at a concentration of $20{\mu}g/mL$. In addition, BW extract treatment significantly reduced and normalized the productions of pro-inflammatory factors, nuclear transcription factor ${\kappa}B$, cyclooxygenase-2, tumor necrosis factor-${\alpha}$, and interleukin-6 at a concentration of $20{\mu}g/mL$. Treatment of chondrocytes with BW extract significantly reduced 5-lipoxygenase activity and production of prostaglandin E2, especially at a concentration of $10{\sim}20{\mu}g/mL$. For the in vivo animal study, osteoarthritis was induced by intra-articular injection of MIA into knee joints of rats. Consumption of a diet containing BW extract (100 and 200 mg/kg) for 35 days significantly inhibited the development and severity of osteoarthritis in rats. To determine the genetic expression of arthritic factors in articular cartilage, real-time PCR was applied to measure matrix metalloproteinases (MMP-3, MMP-9, and MMP-13), collagen type I, collagen type II, and aggrecan, and BW extract had protective effects at a concentration of 200 mg/kg. In conclusion, BW extract was able to inhibit articular cartilage degeneration by preventing extracellular matrix degradation and chondrocyte injury. One can consider that BW extract may be a potential therapeutic treatment for degenerative osteoarthritis.

• Clinical and Experimental Pediatrics
• /
• v.52 no.5
• /
• pp.557-566
• /
• 2009

Purpose : To evaluate the clinical features and characteristics of childhood periodic syndromes (CPS) in Korea using the new criteria of the International Classification of Headache Disorders (ICHD)-II. Methods : The study was conducted at pediatric neurology clinics of five urban tertiary-care medical centers in Korea from January 2006 to December 2007. Patients (44 consecutive children and adolescents) were divided into three groups (cyclic vomiting syndrome [CVS], abdominal migraine [AM], and benign paroxysmal vertigo of childhood [BPVC]) by recurrent paroxysmal episodes of vomiting, abdominal pain, dizziness, and/or vertigo using the ICHD-II criteria and their characteristics were compared. Results : Totally, 16 boys (36.4%) and 28 girls (63.6%) were examined (aged 4-18 yr), with 20 CVS (45.5%), 8 AM (18.2%), and 16 BPVC (36.4%) patients. The mean age at symptom onset was $6.3{\pm}3.6$ yr, $8.5{\pm}2.7$ yr, and $8.5{\pm}2.9$ yr in the CVS, AM, and BPVC groups, respectively, showing that symptoms appeared earliest in the CVS group. The mean age at diagnosis was $8.0{\pm}3.4$ yr, $10.5{\pm}2.6$ yr, and $10.1{\pm}3.2$ yr the CVS, AM, and BPVC groups, respectively. Of the 44 patients, 17 (38.6%) had a history of recurrent headaches and 11 (25.0%) showed typical symptoms of migraine headache, with 5 CVS (25.0%), 2 AM (25.0%), and 4 BPVC (25.0%) patients. Family history of migraine was found in 9 patients (20.4%): 4 in the CVS group (20.0%), 2 in the AM group (25.0%), and 3 in the BPVC group (18.8%). Conclusion : The significant time lag between the age at symptom onset and final diagnosis possibly indicates poor knowledge of CPS among pediatric practitioners, especially in Korea. A high index of suspicion may be the first step toward caring for these patients. Furthermore, a population-based longitudinal study is necessary to determine the incidence and natural course of these syndromes.

• Journal of Chest Surgery
• /
• v.36 no.8
• /
• pp.545-558
• /
• 2003

Adult respiratory distress syndrome (ARDS) is of particular interest because of its severity of the associated lung injury and its high mortality. However, the pathophysiologies of ARDS in infant and childhood groups are still not well clarified inspite of many previous investigations. To investigate the time course of pathophysiology of ARDS in infant and childhood groups, this study was designed with experimental endotoxin-induced ARDS model using young rabbits (8 week-old). Material and Method: Rabbits were divided into the control group (n=8) and the endotoxin-treated group (n=32). The endotoxin group was subdivided into 4 groups by the sampling times as 3, 6, 12 and 24 hr-groups (G- $E_{3,6,12,24,}$ each n=8). The experimental ARDS was made by a bolus injection of endotoxin (Escherichia coli serotype 055 : B5, 0.50 mg/kg) via rabbit ear vein. For evaluation of the hematologic and inflammatory markers, and superoxide dismutase (SOD) concentrations, the blood samples were taken from the heart. The bronchoalveolar lavage fluid (BALF) were obtained for analysis of the leukocytes and protein concentration. With biopsy of the lung, histopathologic changes of the lung were also evaluated. Result: In the endotoxin groups, significant leukopenia (owing to pancytopenia) occurred in 3 and 6-hr groups, which was followed by significant leukocytosis (owing to neutrophilia) in the 12 and 24-hr groups (p<0.05). Serum levels of tumor necrosis factor-$\alpha$ (TNF-$\alpha$) and interleukin-1 $\beta$ (IL-1 $\beta$) in the endotoxin groups were higher than those of control group (p<0.05). Serum levels of superoxide dismutase (SOD) of G- $E_{3}$ and G- $E_{6}$ were higher than those of control group, whereas those of G- $E_{12}$ were lower than those of control groups (p<0.05). Total leukocyte counts and protein con-centrations in BALF were significantly elevated in the endotoxin groups compared to the control group (p < 0.05). The hemorrhagic pattern of BALF showed occurred in the endotoxin groups. The endotoxin groups (in G- $E_{6}$) had severe infiltration of inflammatory cells (lymphocyte and monocyte) in the pulmonary interstitium and parenchyma, migrations of neutrophil and eosinophil into alveolar spaces and interstitial widening, which are the evidences of acute lung injury. In the endotoxin groups, there were significant positive correlations between the BALF findings and the immunologic markers (TNF-$\alpha$, IL-1$\beta$, SOD) (p<0.05). Conclusion: Severe acute lung injury occurred in all the endotoxin-treated rabbits. The pathophysiologic findings were so progressive until 6-hr by time dependant pattern, and then recovered slowly, Variable hematologic, immuno-logic, and pathologic factors were well correlated in the development and progression of endoxin-induced lung injury. The pathophysiologic responses were sensitive and rapid in young rabbit Young rabbit seemed to be a useful experimental animal model for infant and childhood groups.roups.

• Radiation Oncology Journal
• /
• v.13 no.4
• /
• pp.339-348
• /
• 1995

Purpose : Since 1983, authors have conducted a study to evaluate the effect of external radiation therapy and to determine affected factors in management of the patients with malignant obstructive jaundice due to porta hepatis metastasis from stomach cancer. Materials and Methods : Thirty two patients with malignant obstructive jaundice due to porta hepatis metastasis from gastric cancer were presented. We have analysed 23 patients who were treated with external radiation therapy of more than 3000cGy. The radiation dose, disease extent at developement of jaundice, total bilirubin levels before radiation therapy, differentiation of histology, combind treatment, intent of primary surgery, initial stage of gastric cancer were analyzed to determine affected factors in radiation therapy. External radiation therapy was delivered with a daily dose of 180-300cGy, 5 times a week fractionation using 4 MeV linear accelerator. The radiation field included the porta hepatis with tumor mass by the abdominal ultrasonography or CT scan. In twenty three patients received more than 3000cGy, total irradiation dose was ranged from 3000cGy to 5480cGy, median 3770cGy. Among 23 patients, 13 patients were delivered more than equivalant dose of TDF 65(4140cGy/23fx). Results : Among 23 patients, complete, partial and no response were observed in 13, 5, 5 patients, respectively. The median survival for all patients was 5 momths. The significant prolongation of median survival was observed in complete responders(11 months) as compared to partial and no responders(5 months, 5 months respectively) Out of 13 patients with complete response, 6 patients lived more than a year Among 13 patients receiving more than 4140cGy equivalent dose, complete, partial and no response were observed in 10, 2 and 1 patients, respectively. The median survival for all these patients was 9.5 months. The median survival for complete responders(10/13) was 11.5 months. Among 10 patients receiving less than 4140cGy equivalent dose, complete, partial and no response were observed in 3, 3, 4 patients, respetively. The median survival for all these patients was 4.3 months Therefore, the radiation dose affected the results of treatment. For the complete response with prolongation of survival duration, at least 4140cGy equivalant dose should be delivered to porta hepatis. In evaluation of the disease extent, 7 patients of 13 complete responders showed localized disease in porta hepatis or peripancreatic area, but all patients with partial and no response showed wide extensive disease or persistant disease of primary gastric cancer. Therefore. the patients with the localized disease were the higher probability of complete response and long term survival. This study suggested that the radiation dose and the disease extent at developement of jaundice affected in radiation therapy for malignant obstructive jaundice. There were no serious complications related to external radiation therapy. Conclusion : External radiation therapy only could achieve the palliative effect in the patients with malignant obstructive jaundice due to porta hepatis metastasis from stomach cancer. This study suggested that the prolongation of survival duration could be achived in complete responders and radiation dose, extent of disease affected the results of treatment of malignant obstructive jaundice.