• Korean Journal of Biological Psychiatry
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• v.8 no.1
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• pp.96-105
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• 2001

Objective : Lithium inhibits the action of inositol monophosphatase(IMPase) in phosphoinositide(PI) signal transduction system at therapeutically relevant concentration. The depletion of inositol by lithium itself cannot explain the lithium's therapeutic effect. However, attention has focused on the abnormality of PI signal transduction system as the pathophysiology of bipolar affective disorder(BPD). We investigated whether IMPase mRNA levels of lymphocytes would be different between BPD patients(n=16) and age, sex-matched normal controls(n=16). We also investigated the change of IMPase mRNA level by lithium during 4 weeks to probe the possibility that IMPase mRNA levels could predict the therapeutic response to lithium and clinical course. Method : Relative IMPase mRNA levels in lymphocyte were quantified by reverse transcriptase(RT)-PCR in sixteen drug-free BPD patients and sex, age-matched normal controls. The psychopathology of patients were measured using YMRS (Young Mania Rating Scale) and CGI(Clinical Global Impression). Results : There was no significant difference in IMPase mRNA levels between BPD patients and normal controls. And the IMPase mRNA levels were not significantly changed by 4 week treatment with lithium. However, the basal IMPase mRNA levels were negatively correlated with the changes of CGI after 4 weeks. Furthermore, the patients with relatively high basal IMPase mRNA levels showed much more improvement during 4 weeks. Conclusions : BPD patients and normal controls were not distinguished by lymphocyte IMPase mRNA level. Although we do not support the hypothesis that lymphocyte IMPase activity would be related with the pathogenesis of BPD and the action of lithium, these data raise the possibility that lymphocyte IMPase mRNA levels could function as a predictor of therapeutic response and clinical course of BPD.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.305-310
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• 2009

Background: The Ross procedure is known as a good surgical option for a young age group with aortic valve problems, but few reports on the Ross procedure are available in the Korean literature. This study is a review of our midterm results of 10 year experience with the pediatric Ross operation in Asan Medical Center. Material and Method: From March 1997 to October 2008, eighteen patients who were aged less than 16 years underwent the Ross procedure. There were 11 males and 7 females. The patients median age was 8.5 years (range: $0.5\sim14.0$). The aortic valve pathophysiology was 6 patients with aortic insufficiency, 4 patients with aortic stenosis, 7 patients with mixed aortic stenoinsufficiencey and 1 patient with infective endocarditis. The valve morphology was bicuspid in 11 and tricuspid in 7. All the patients were operated on with the root replacement technique. All the pumonic valves were replaced with an allograft except for one pericardial monocusp valve. The mean follow up duration was 52.8 months (range: 5.8$\sim$138.2 months). We reviewed the echocardiographic data with focusing on the, auto-graft dysfunction and reoperation. Result: There was no hospital mortality and late mortality. According to the last echocardiographic data, 2 autografts showed aortic regurgitation grade 2, 4 autografts showed aortic regurgitation grade 1 and the others were less than trivial. Reoperation of the pulmonic position conduit was performed 4 times in three patients. The rate of freedom from reoperation at 5 years was 72.2%. On the serial follow up, the Z-values of the aortic annulus/aortic sinus were changed from $1.6{\pm}1.7/0.9{\pm}1.7$ at preoperation to $1.8{\pm}1.6$(p=0.64)/$2.2{\pm}0.9$ (p=0.01) at the last follow-up. There was no significant relation between the growth of the neoaortic root and neoaortic insufficiency. Conclusion: Our midterm results of the Ross procedure in pediatric patients showed good autograft function and growth potential. Vet reoperation due to allograft dysfunction was a major concern.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.292-298
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• 2009

Background: Surgery for mitral valve disease in children carries both technical and clinical difficulties that are due to both the wide spectrum of morphologic abnormalities and the high incidence of associated cardiac anomalies. The purpose of this study is to assess the outcome of mitral valve surgery for treating congenital mitral regurgitation in children. Material and Method: From 1997 to 2007, 22 children (mean age: 5.4 years) who had congenital mitral regurgitation underwent mitral valve repair. The median age of the patients was 5.4 years old and four patients (18%) were under 12 months of age. 15 patients (68%) had cardiac anomalies. There were 13 cases of ventricular septal defect, 1 case of atrial septal defect and 1 case of supravalvar aortic stenosis. The grade of the preoperative mitral valve regurgitation was II in 4 patients, III in 15 patients and IV in 3. The regurgitation was due to leaflet prolapse in 12 patients, annular dilatation in 4 patients and restrictive leaflet motion in 5 patients. The preoperative MV Z-value and the regurgitation grade were compared with those obtained at follow-up. Result: MV repair was possible in all the patients. 19 patients required reduction annuloplasty and 18 patients required valvuloplasty that included shortening of the chordae, papillary muscle splitting, artificial chordae insertion and cleft closure. There were no early or late deaths. The mitral valve regurgitation after surgery was improved in all patients (absent=10, grade I=5, II=5, III=2). MV repair resulted in reduction of the mitral valve Z-value ($2.2{\pm}2.1$ vs. $0.7{\pm}2.3$, respectively, p<0.01). During the mid-term follow-up period of 3.68 years, reoperation was done in three patients (one with repair and two with replacement) and three patients showed mild progression of their mitral reguration. Conclusion: our experience indicates that mitral valve repair in children with congenital mitral valve regurgitation is an effective and reliable surgical method with a low reoperation rate. A good postoperative outcome can be obtained by preoperatively recognizing the intrinsic mitral valve pathophysiology detected on echocardiography and with the well-designed, aggressive application of the various reconstruction techniques.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.862-868
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• 1997

Shone's syndrome is a congenital cardiac malformation that consists of multiple levels of left heart obstruction including supravalvular mitral ring, congenital mitral stenosis(parachute mitral valve), subaortic stenosis, and coarctation of aorta. This syndrome is a very rare congenital anomaly and its prognosis is poor. We experienced 9 patients with Shone's syndrome between 1985 and 1994. There were 8 male and 1 female patients, and mean age was 33.0$\pm$31.0 months ranged from 2 months to 1 1 years. The congenital mitral, stenosis and coarctation of aorta existed in all patients and the supravalvular mitral ring and subaortic stenosis in 4 patients. Two patients had all four anatomic lesions. 3 patients underwent one stage total correction and the other 6 patients underwent two staged operation that was initial coarctoplasty with thoracotomy and later correction of intracardiac anomalies with median sternotomy. A third operation was performed in 2 patients. These procedures included reoperation for coarctation and replacement of mitral valve for persistent mitral stenosis. There was no operative death at the first operation but two operative deaths at the second operation. The cause of death in two cases was severe heart failure secondary to left ventricular hypoplasia. There was no operative death at the third operation. The seven survivors have beeli followed from 11 months to 12 years(mean follow-up 6.7 $\pm$ 3.6 years). There was no late death and the New York HeArt Association activity level was class I for all patients. We conclude that a food lone-term outcome can be expected by proper surgical treatment tailred to each individual's anatomy and pathophysiology although the operative mortality and morbidity of Shone's syndrome are high.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.143-148
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• 2005

Purpose : Hypogammaglobulinemia has been observed in nephrotic syndrome, but its pathophysiology remains unknown. We evaluated serum immunoglobulins, IgG subclasses, and vaccine-induced viral antibodies(anti-hepatitis B surface IgG and anti-measles IgG) in children with minimal change nephrotic syndrome(MCNS). Methods : Using the stored sera, the levels of immunoglobulin(IgC, IgM, IgA, and IgC) and IgG subclasses(IgG 1, 2, ,3, and 4), anti-HBs Ab and anti-measles IgG of 21 children with MCNS were analyzed and compared to those of 25 age-matched healthy children. Results : The mean values of IgG and IgG1 were $390{\pm}187\;mg/dL$ and $287{\pm}120\;mg/dL$ in nephrotic children, and $1,025{\pm}284\;mg/dL$ and $785{\pm}19\;mg/dL$ in control children, respectively. The values of the total IgG and the 4 IgG subclasses in nephrotic children were all significantly depressed(P<0.001), but the IgM($251{\pm}183\;mg/dL\;vs. 153{\pm}55\;mg/dL$, P=0.02) and IgE values(P=0.01) were elevated, and the IgA values were not changed. The seropositivity of anti-HBs IgG was 42.9$\%$(9 of 21 cases) in the MCNS group and 52$\%$(13/25) in the control group, and that of anti-measles IgG was 75$\%$(16/21) and 92$\%$(23/25), respectively, but there was no statistical difference between the two groups. Conclusion : IgG and IgG subclass levels in MCNS children are all depressed without significant seronegativity of the vaccine-induced viral antibodies. Further studies are needed to resolve the cause of hypogammaglobulinemia in MCNS. (J Korean Soc Pediatr Nephrol 2005;9:143-148)

• Journal of Chest Surgery
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• v.42 no.1
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• pp.1-8
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• 2009

Background: The pathophysiology of acute respiratory distress syndrome with sepsis is acute lung injury (ALI) that's' caused by endotoxin (LPS). We evaluate effects of moxifloxacin on LPS-induced ALI in a rat model. Material and Method: The rats were divided into 3 groups as the control group (C), the LPS insult group (L), and the LPS+moxifloxacin treated group (L-M). ALI was induced by endotracheal instillation of E.coli LPS, then moxifloxacin was given in 30 minutes. Five hours later, we checked the lung weight/body weight ratio(the L/BW ratio), the protein & neutrophils in the bronchoalveolar lavage fluid (BALF), the myeloperoxidase (MPO) activity & the malondialdehyde (MDA) content, the expressions of cytosolic and secretory phospholipase $A_2$ (c, $sPLA_2$), and the morphology of the lung with using a light microscope. Result: The L/BW ratio, the protein content and the neutrophil count in the BALF, and the MPO activity and the MDA content in lung were significantly increased in group L compared to group C, and these factors were markedly decreased in group L-M compare to group L. The $cPLA_2$ expression and the $sPLA_2$ expression were increased in group L and the $cPLA_2$ expression was decreased in group L-M. Yet the $sPLA_2$ expression was not changed in group L-M. Morphologically, many inflammatory findings were observed in group L, but not in group L-M. Conclusion: Many of the inflammatory changes of ALI that were caused by LPS insult were ameliorated by moxifloxacin treatment.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.363-369
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• 2003

Purpose : Pharmacologic provocation test of growth hormone(GH) is a non-physiologic method and has several limitations for diagnosing growth hormone(GH) deficiency. Spontaneous GH release studies could be important in understanding the pathophysiology of children with poor growth but normal responses to GH provocation tests. Also, the relationship between nocturnal GH secretions and sleep patterns in short stature children is poorly understood. The aim of this study is to determine whether there are differences in sleep patterns and nocturnal GH secretory profiles between idiopathic short stature children and a normal stature group. Methods : Spontaneous nocturnal GH secretions and sleep patterns were evaluated in 12 prepubertal idiopathic short stature children with normal responses to provocation tests and 9 normal stature controls. Blood samples were taken every 30 minutes from 22:00-06:30 and sleep patterns were analyzed by polysomnography. Results : The mean GH level during sleep was significantly lower in short stature children than in controls. The peak GH level after sleep, coincident with the first slow wave sleep, was lower in the short stature group. The slow wave sleep times of short stature children were decreased compared with those of normal subjects. Conclusion : These results suggest that overnight serial GH sampling is helpful to identify short stature children with subnormal GH secretions, and sleep structure differences may be associated with decreased overnight GH secretions in short stature children.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.903-908
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• 2003

Purpose : Kawasaki disease is notorious for coronary arterial complication which is usually developed as a febrile disease in early childhood. Increased polymorphonucleus(PMN) cell levels in acute phases may be associated with the pathophysiology of Kawasaki disease. We studied the relationship between coronary arterial dilatation and elastase activity which was excreted from PMN cell and roles as an important factor for vasculitis. Methods : Ten patients diagnosed with Kawasaki disease in Yonsei University Medical Center were examined between November, 2001 and January, 2002. In addition, 15 patients with other febrile diseases were also examined. Echocardiography was done in patients with Kawasaki disease on the first day of admission and four weeks after the onset of the disease. At each time, venous samples were drawn and separated into plasma and leukocytes. In patients with other febrile disease, samples were drawn on admission. Elastase activities in plasma and neutrophil extracts were measured. Results : The significant increased plasma elastase activity, $6.19{\pm}0.74U/mL$, found in Kawasaki disease patients compared with the other febrile disease patients, $4.86{\pm}1.17U/mL$(P<0.05). And there was no significance between the above two diseases in terms of the elastase activity in neutrophil extracts. The relationship between initial elastase activity and the coronary arterial complication which was shown in subacute phase wasn't significant. Conclusion : Plasma elastase activity was increased in Kawasaki disease significantly, but the initial plasma elastase activity in the acute phase could not reflect the range of coronary arterial complication.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.230-235
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• 2003

Purpose : Lower respiratory tract infections in infant and young children are often due to a virus, especially the Respiratory syncytial(RS) virus. Chest X-ray findings in bronchiolitis and bronchopneumonia are different. The radiographic hallmark of bronchiolitis is pulmonary hyperinflation and similar to that of bronchial asthma. Bronchiolitis is predisposed to later development of bronchial asthma. To evaluate the difference of immuno-pathophysiology between bronchiolitis and bronchopneumonia, we measured $IFN-{\gamma}$(Th1 cytokine), IL-5(Th2 cytokine) and ECP. We also investigated whether X-ray findings in infants with viral infected respiratory disease are useful in predicting the development of asthma. Methods : We measured IL-5, ECP, $IFN-{\gamma}$ levels in serum from 21 infants with bronchiolitis and 21 infants with bronchopneumonia and 16 infants without pulmonary viral diseases. Results : IL-5 levels of bronchiolitis and bronchopneumonia were significantly higher than those of the control(P=0.02, P=0.042). IL-5 levels of bronchiolitis were higher than those of bronchopneumonia but there was no significant difference. $IFN-{\gamma}$ levels of bronchopneumonia were higher than those of bronchiolitis but there was no significant difference. ECP levels of bronchiolitis and bronchopneumonia were higher than those of the control but only those of bronchiolitis were significantly higher than those of the control(P=0.045). IL-5 and ECP levels did not show any significant correlation in bronchiolitis, bronchopneumonia and control groups. Conclusion : We cannot prove the distinct differences in serum Th1/Th2 cytokine profiles between bronchiolitis and bronchopneumonia in infants. These results suggest that the different findings on chest X-ray between bronchiolitis and bronchopneumonia could not be a predictor of later development of asthma.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1140-1146
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• 2009

Purpose:The pathophysiology of magnesium, the second highest common compound in humans, is still unclear, especially in preterm babies. We accessed the association between total magnesium (tMg), ionized Mg (iMg), and gestational age (GA) and that between serum magnesium (sMg) and intraventricular hemorrhage (IVH) in preterm babies. Methods:In all, 119 inborn preterm infants admitted between July 2006 and February 2008 were divided into the IVH group (19) and the control group (100) and were prospectively analyzed. TMg, iMg, pH, total calcium (tCa), and ionized Ca (iCa) levels were determined immediately after delivery or within 3 hours after birth, and their correlation with GA were investigated. Results:TMg was not correlated with GA, tCa, iCa, and pH. IMg was correlated with tMg (r=0.288, P=0.002) and iCa (r=0.212, P=0.021); however, it was not correlated with GA and pH. Mean GA and birth weight were significantly lower (P=0.002) and smaller (P=0.030) in the IVH group. Mean sMg was higher in the IVH group ($2.5{\pm}0.9mg/dL$) than in the control group ($2.1{\pm}0.6mg/dL$) (P=0.021). SMg was a risk factor even after logistic regression analysis (OR, 2.798; 95% C.I., 1.265-6.192; P=0.011). Conclusion:In less than 37-week-old preterm babies, tMg and iMg were similar, regardless of GA. High sMg may be a risk factor for IVH in premature babies, regardless of their exposure to antenatal magnesium.