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

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.376-381
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• 2003

Purpose : This study aimed to demonstrate the possible pathogenesis of granulopoiesis in patients of Kawasaki disease(KD) using quantitative analysis of G-CSF, GM-CSF and their CSFr. Methods : The plasma levels of G-CSF, GM-CSF, G-CSFr and GM-CSFr were studied in 14 patients in the acute phase of KD; 13 children with normal peripheral white blood cell counts were used as the normal control group. The plasma concentration of G-CSF, GM-CSF were analyzed by ELISA. The G-CSFr and GM-CSFr on the peripheral granulocytes were analyzed by a quantitative flow cytometric assay and QuantiBRITE, and the quantitative changes of receptors which did not combine with G-CSF and GM-CSF were measured. Results : The total number of leukocytes in KD was similar to normal control group, but the leukocytes increased according to the number of neutrophils. The plasma concentration of G-CSF were decreased similar to normal control group(P=0.133), but that of GM-CSF decreased more than the normal control group(P=0.227). The quantity of G-CSFr, GM-CSFr were revealed to be no less than the normal control(P=0.721, P=0.912). After incubation with excessive G-CSF, the expressed G-CSFr on the neutrophils were decreased in both groups(P=0.554). The quantities of expressions of GM-CSFr on the neutrophil after incubation with the excessive GM-CSF were always increased in both groups(P=0.255). The amount of GM-CSFr of neutrophils are in proportion to total white blood cells (r=0.788, P=0.035), but it wasn't in the case of KD(P=0.644). Conclusion : The leukocytosis in KD that mediated by increasing neutrophil was not correlated with the plasma concentrations of G-CSF and GM-CSF, and the amount of expression of G-CSFr and GM-CSFr on granulocyte. It is possible that the reduction of concentration of GM-CSF results by increasing the active GM-CSFr.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.271-276
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• 2003

Purpose : Granulocyte-colony stimulating factor(G-CSF) and granulocyte macrophage-colony stimulating factor(GM-CSF) are principal cytokines in granulopoiesis and their physiologic effects are mediated through binding to specific cell surface receptors. Although it is known that the level of serum G-CSF and GM-CSF, and presentation of the receptors are increased in infectious diseases, there have been no studies to find the correlation between the granulopoiesis and leukocytosis. This study was designed to measure G-CSF and GM-CSF in leukocytosis and in control and to demonstrate the possible pathogenesis of granulopoiesis in leukocytosis using quantitative analysis of G-CSF, GM-CSF and their CSFr. Methods : The plasma levels of G-CSF, GM-CSF of 13 children without leukocytosis and 14 children with leukocytosis were measured. Counts of cell surface G-CSFr and GM-CSFr were measured by combining anti G-CSFr and anti GM-CSFr monoclonal antibodies to their respective receptors by using quantitative flow cytometric assay. Results : There was no significant difference betweeen the plasma concentration of G-CSF and GM-CSF in acute leukocytosis and in the control group. However, levels of G-CSFr in acute leukocytosis decreased significantly compared to the control(P=0.012) and the levels of GM-CSFr in both groups revealed no significant difference. Conclusion : Increase in the number of leukocyte in leukocytosis was mediated by increasing the number of neutrophil, and increased plasma concentration of G-CSF may be the cause of neutrophilia. But GM-CSF did not have any influence on leukocytosis.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1200-1206
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• 2003

Purpose : This study was conducted for the use of nasal continuous positive airway pressure (CPAP), by comparing the early use of non-invasive nasal CPAP with low intermittent mandatory ventilation(low IMV) and endotracheal CPAP in weaning a mechanical ventilator from infants with moderate respiratory distress syndrome(RDS). Methods : Thirty infants in the study group, with moderate RDS from November 2001 to June 2002, were administered surfactants and treated with the mechanical ventilator, and applied the nasal CPAP in weaning. Thirty infants of the control group, from January 1999 to September 2001, were applied low IMV and endoctracheal CPAP in weaning. Results : There were no significant differences in the characteristics, the severity of clinical symptoms, the initial laboratory findings and settings of the mechanical ventilator. After weaning, the study group showed no significant changes in $PaCO_2$. However, the control group showed a slight $CO_2$ retension after one and 12 hours. Twenty eight infants(93.3%) of the study group and 24 infants(80%) of the control group were successfully extubated. The primary cause of failure was apnea. There were no significant differences in the duration of weaning and the mechanical ventilator treatment between the groups. Complications in weaning were related to the fixation of nasal CPAP and the mechanical problems caused by endotracheal tube. Conclusion : Aggressive weaning is possible for moderate RDS, in which the nasal CPAP was used without the low IMV and the endotracheal CPAP process. It had no difficulties. In conclusion, the nasal CPAP is an adequate weaning method for moderate RDS.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.317-325
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• 2006

Purpose : This study was carried out to elucidate the effects of nitric oxide synthase(NOS) inhibitor, NG-monomethyl-L-arginine(L-NMMA) and nitric oxide precursor, L-arginine(L-Arg) on cerebral hemodynamics and energy metabolism during reoxygenation-reperfusion(RR) after hypoxia-ischemia(HI) in newborn piglets. Methods : Twenty-eight newborn piglets were divided into 4 groups; Sham normal control(NC), experimental control(EC), L-NMMA(HI & RR with L-NMMA), and L-Arg(HI & RR with L-Arg) groups. HI was induced by occlusion of bilateral common carotid arteries and simultaneously breathing with 8 percent oxygen for 30 mins, and followed RR by release of carotid occlusion and normoxic ventilation for one hour. All groups were monitored with cerebral hemodynamics and cytochrome $aa_3$ (Cyt $aa_3$) using near infrared spectroscopy(NIRS). $Na^+$, $K^+$-ATPase activity, lipid peroxidation products, and tissue high energy phosphate levels were determined biochemically in the cerebral cortex. Results : In experimental groups, mean arterial blood pressure, $PaO_2$, and pH decreased, and base excess and blood lactate level increased after HI compared to NC group(P<0.05). These variables subsequently returned to baseline after RR except pH. There were no differences among the experimental groups. In NIRS, oxidized hemoglobin($HbO_2$) decreased and hemoglobin(Hb) increased during HI(P<0.05) but returned to base line immediately after RR; 40 min after RR, the $HbO_2$ had decreased significantly compared to NC group(P<0.05). Changes of Cyt $aa_3$ decreased significantly compared to NC after HI and recovered at the end of the experiment. Significantly reduced cerebral cortical cell membrane $Na^+$, $K^+$-ATPase activity and increased lipid peroxidation products(P<0.05) were not improved with L-NMMA or L-Arg. Conclusion : These findings suggest that NO is not involved in the mechanism of HI and RR brain damage during the early acute phase of RR.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.64-71
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• 1999

Purpose : The protective effects of dietary protein on the progression of renal failure were studied in subtotally nephrectomized rats. Methods : Treatment groups were as follows; 5/6 nephrectomy and a normal protein ($18.5\%$) diet (NP); 5/6 nephrectomy and a low protein ($6\%$) diet (LP): 5/6 nephrectomy, a normal protein diet and converting enzyme inhibitor, enalapril (NPE): 5/6 nephrectomy, a low protein diet and enalapril (LPE). Both diets were isocaloric and had the same phosphorus content. Proteinuria, remnant kidney weight, mesangial matrix expansion score and glomerular volume were assessed at 4, 12 and 16 weeks after renal ablation. Results : LP and NP developed progressive hypertension. Eight weeks after surgery, LPE and NPE controlled hypertension. LP, LPE, and NPE had significantly less proteinuria than NP at 16 weeks (P<0.05). Kidney weight in LP were markedly less enlarged than NP (P<0.05). There was no difference in kidney weight between LPE and NPE. At 12 and 16 weeks the mesangial matrix expansion score was significantly less in LP, LPE, and NPE compared to NP (P<0.05). At 12 and 16 weeks mean glomerular volume was significantly less in LP compared to NP (P<0.05). At 12 and 16 weeks mean glomerular volume in LPE was significantly less compared to NPE. Conclusion : Dietary protein restriction afforded considerable protection from renal injury in the rat remnant kidney model. During the enalapril treatment, there was no additional protective effect of dietary protein restriction against the development of renal lesions.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.24-30
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• 1997

Purpose: To evaluate the diagnositc value of the absolute and relative renal uptake rates on $^{99m}Tc-DMSA$ renal scan of children with febrile UTI. Method: The absolute and relative renal uptake rates of $^{99m}Tc-DMSA$ were checked in 68 children with febrile urinary tract infection (35 with unilateral focal defect; 13 with bilateral focal defect; and 20 with diffuse bilateral defect) and 49 children with afebrile UTI and normal $^{99m}Tc-DMSA$ renal scan as control. Results: The renal absolute uptake rate in the control group were $21.8{\pm}3.9%(right),\;22.2{\pm}3.9%(left),\;and\;44.2{\pm}7.8%(total)$. The absolute uptake rate gradually increased until the age 12 months and then was stationary. In febrile UTI with unilateral focal defect on the $^{99m}Tc-DMSA$ renal scan, both relative and absolute uptake rates were similarly diagnostic ($41.2{\pm}9.7%,\;16.5{\pm}5.4%$ vs $50.0{\pm}2.6%,\;22.0{\pm}3.9%$, p<0.01). In acute pyelonephritis with bilateral focal defect on the $^{99m}Tc-DMSA$ renal scan, the absolute uptake rate was significantly more diagnostic than the relative uptake rate[$17.3{\pm}5.3%$ (right), $17.4{\pm}5.3%\;(left),\;vs\;21.8{\pm}3.9%,\;(right)\;22.2{\pm}3.9%$, (left)% p<0.01]. In febrile UTI with bilateral diffuse defects on the $^{99m}Tc-DMSA$ renal scan, the absolute uptake rate was significantly diagnostic than the relative uptake rate [$18.1{\pm}3.9%\;(right),\;18.4{\pm}3.8%\;(left),\;vs\;21.8{\pm}3.9%,\;(right)\;22.2{\pm}3.9%$(left), p<0.01]. Conclusion: In the $^{99m}Tc-DMSA$ renal scan, the absolute uptake rate was more useful than the relative uptake rate to evaluate bilateral acute pyelonephritis.

• The Korean Journal of Nuclear Medicine Technology
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• v.14 no.1
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• pp.133-137
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• 2010

Purpose: The main function of vitamin D is the mineralization of the brain by increase of calcium and phosphorus, in case it is insufficient in children, lime deposition on cartilage cannot occur so it leads to rachitis, and in adults, it leads to osteomalacia or osteoporosis. It is also strongly believed in the academic world that vitamin D can restrict the growth of cancer cells and prevent heart diseases, which is also somewhat proven in epidemiological researches. While the right density of vitamin D is still being studied, 20-32 ng/mL is believed to be the most ideal density. Therefore, I wanted analyze how much density of 25-Hydroxyvitamin D3 that Koreans possess. Materials and Methods: From February 20th, 2008 to April 21st, 2009, the collection of 2800 serums, from medical examination treated subjects by Neodin Medical Institute, have been tested. The targets were tested by 25-Hydroxyvitamin D (125I Kit: Diasorin, USA), and were analyzed by dividing into many different categories (gender, age, season, region). Results: The average density of male were 20 ng/mL, female 17.08 ng/mL. Per age groups, the density of males were as follows: 10~20-18 ng/mL, 21~30-17 ng/mL, 31~40-19 ng/mL, 41~50-21 ng/mL, 51~60-22 ng/mL, 61~70-22 ng/mL, 71~80-22 ng/mL and 81~90-19.9 ng/mL. Average density of females per age groups, were as follows: 10~20-16 ng/mL, 20~30-15.26 ng/mL, 30~40-16 ng/mL, 40~50-17 ng/mL, 50~60-19 ng/mL, 60~70-19 ng/mL, 70~80-19 ng/mL, and 80~90-17 ng/mL. Per seasons, From December to May, the subjects showed the density of 15.97 ng/mL, while from June to November, it showed 21.60 ng/mL. On density of males from January to April regionally, Seoul+Gyeonggi-Do-15.52 ng/mL, Gangwon-Do-15.33 ng/mL, Choongchung-Do-18.03 ng/mL, Jeonla-Do-18.68 ng/mL, Gyungsang-Do-18.76 ng/mL and Cheju Do-21.23 ng/mL. Conclusions: The vitamin D of Koreans is has been insufficient compared to the suggested amount. Ultraviolet rays, which is the main source of vitamin D is critical, therefore it is suggested that more outdoor activities can definitely help.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.27 no.3
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• pp.239-249
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• 2001

The purpose of this study is to evaluate the relationship of the factors which could be influenced by orthognathic surgery especillay SSRO. We measured the amounts of the maximum opening, lateral movements, maximum velocity and pattern of mandibular path during the opening and closing of mandible at the following times ; preoperative, 1 month after operation, 6 months after operation respectively using MKG. And the results were compared according to the categorized subgroups. Following results were obtained : 1. The change of the amounts of mandibular lateral movement and maximum opening velocity were statistically different between male and female (p<0.05), but the others were not. 2. According to the method of operation, there was no difference in the change of the mandibular movements between the group of SSRO and SSRO plus LeFort I osteotomy (p>0.05). 3. According to the amounts of mandibular movement, the recovery of left lateral movement of the group of $6{\sim}10mm$ was better than the other groups (p<0.05). 4. In the frontal pattern of the opening and closing of the mandible, the complex deflected type (F5), simple deflected type (F4), complex deviated type (F3), simple deviated type (F2), straight type (F1) were obtained in order at the time of preoperative, simple deflected type, simple deviated type, complex deviated type, straight type, complex deflected type in order at the time of 1 month after surgery, and the result at the time of 6 months after surgery was the same with that of the time of preoperative. In the sagittal pattern, non-coincident type (S2) was predominant at the time of preoperative, and coincident type (S1) was predominant at the time of 1 month after surgery. After 6 months, the result was also the same with that of the preoperative in sagittal pattern. 5. There was not a statistical difference in the change of the mandibular movement between group of presence of the preoperative TMJ symptoms and non-presence group (p>0.05). 6. There was not a statistical difference in the change of the mandibular movement between repositioning device applied group and non-applied group (p>0.05). 7. Sixty three percents of the patients who had preoperative TMJ symptoms were improved after surgery and preoperative TMJ symptoms were more improved after operation in the repositioning device non-applied group statistically (p<0.05).

• Pediatric Infection and Vaccine
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• v.5 no.1
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• pp.128-135
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• 1998

Purpose : This study was intended to measure seropositivities and the level of mumps- and rubella-specific IgG of MMR vaccinees from 12 to 17 years of age in Korea. Materials and Methods : From May 1996 to July 1996 we obtained sera from students of 1 middle and 2 high schools in Seoul, who were MMR vaccinees from 12 to 17 years of age and had no evidence of immunodeficiency. These 216 study population include 110 males and 106 females. Mumps- and rubella-specific IgG antibody levels were measured by ELISA. Cut-off values for seropositivity were 20 U(Gamma Unit) in mumps and over 0.17 in rubella. Results : 1) As age increased, seropositivities to mumps increased, being 68.4% in 12 year, 79.3% in 13 year, 72.2% in 14 year, 82.0% in 15 year, 87.5% in 16 year, 87.0% in 17 year, which however has no statistical significance. 2) As age increased, the level of mumps-specific IgG antibody(mean+standard deviation, GU) increased, being $52.0{\pm}49.2$ in 12 year, $65.9{\pm}51.4$ in 13 year, $71.1{\pm}66.0$ in 14 year, $67.8{\pm}53.6$ in 15 year, $82.8{\pm}67.8$ in 16 year, $92.0{\pm}68.9$ in 17 year, which however has no statistical significance. 3) As age increased, seropositivities of rubella-specific IgG increased significantly, being 26.3% in 12 year, 20.7% in 13 year, 50.0% in 14 year, 67.2% in 15 year, 66.7% in 16 year, 65.2% in 17 year(P<0.001). 4) As age increased, rubella-specific IgG increased significantly, being $0.13{\pm}0.145$ in 12 year, $0.087{\pm}0.101$ in 13 year, $0.194{\pm}0.168$ in 14 year, $0.260{\pm}0.187$ in 15 year, $0.305{\pm}0.213$ in 16 year, $0.325{\pm}0.221$ in 17 year(P<0.001). There was positive correlation between age and rubella-specific IgG titer(rubella-specific $IgG=0.0517{\times}age-0.5586$, r=0.3752, P<0.001). Conclusion : In adolescent, seropositivities and the level of mumps-specific IgG remained relatively high, but approximately 20% of study population showed seronegativity. Seropositivities and the level of rubella-specific IgG showed the lowest level at 13 years of age and were increased with age after 14 years of age. Further evaluation may be needed to elucidate the cause of these changes of rubella-specific IgG.