• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.596-602
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• 2002

Purpose : The purpose of this study was to analyze the association of peripheral neutrophil count with the development of respiratory failure in preterm infants. Methods : A retrospective study was conducted from January 1993 to December 1999 on 44 preterm infants, who were admitted to the neonatal intensive care unit of St. Francisco hospital. Preterm infants(birth weight 500 to 1,350 gm) who had a complete blood count obtained within 2 hours after delivery. Patients in the lowest of neutrophil count(early neutropenia, < $1.0{\times}10^9/L$) were compared with patients in the remaining group. Results : Low neutrophil count were transient in early neutropenia group. The concentration the circulating neutrophil count rose from $0.85{\pm}0.11{\times}10^9/L$ at average of 2 hours after delivery to $5.3{\pm}2.7{\times}10^9/L$ at 24 hours after delivery in the early neutropenia group and from $3.6{\pm}1.6{\times}10^9/L$ to $5.8{\pm}3.2{\times}10^9/L$ in the non-neutropenia group during the same time period. Compare to the non-neutropenia group, the neutropenia group had a lower birth weight($1,046.50{\pm}180.76gm$ Vs $1,156.70{\pm}124.99gm$), a lower Apgar score(1 min : $3.41{\pm}1.18$ Vs $4.30{\pm}1.46$, 5 min : $5.41{\pm}0.87$ Vs $6.15{\pm}0.95$), and a higher incidence of bronchopulmonary dysplasia(27.27% Vs 7.0%). Patients who had early neutropenia were more likely to require mechanical ventilation, supplemental oxygen and hospital stay. Also, main effect factors for the two groups were birth weight(Odds ratio=5.457, 95% CI=1.551-27.525), initial peripheral blood white cells(odds ratio=8.308, 95% CI=2.054-52.699), and bronchopulmonary dysplasia(odds ratio=0.099, 95% CI=0.017-0.397). Conclusion : A low count of neutrophil in the systemic circulation of premature infants within 2 hours of birth is associated with more severe respiratory distress.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.170-179
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• 1999

Purpose: To evaluate whether or not sodium restriction had its own beneficial effect and increased the efficiency of the anti-hypertensive drugs on the progression of renal failure. Methods: We studied using the excision remnant kidney model. Treatment groups were as follows: 5/6 nephrectomy and a 0.49% (normal-high) sodium diet (NN); 5/6 nephrectomy and a 0.25% (normal-low) sodium diet (LN); 5/6 nephrectomy, a 0.49% sodium diet and enalapril (NNE); 5/6 nephrectomy, a 0.49% sodium diet and nicardipine (NNN); 5/6 nephrectomy, a 0.25% sodium diet and enalapril (LNE); 5/6 nephrectomy, a 0.25% sodium diet and nicardipine (LNN). Both diets were isocaloric and had the same content of protein, phosphorus and calcium. Proteinuria, remnant kidney weight, mesangial expansion scores, and glomerular volume were assessed. Results: Blood pressure tended to be lower in LN compared to NN (P<0.05). NN developed progressive hypertension. LNE, LU, NNE, and NNN reduced blood pressure. LNE, LNN, NNE, NNN, and LN had significantly less proteinuria than NN at 16 weeks (P<0.05). At 24 weeks, LN developed proteinuria (82 mg/day), which were lessened in LNE (54 mg/day) and not lessened in LNN (76 mg/day). Mesangial expansion scores were significantly less in LN rats compared to those in NN rats. Glomerular volumes at 24 weeks in LN rats were significantly less compared to those at 16 weeks in NN rats. Mesangial expansion scores and glomerular volumes at 4, weeks, 12 weeks, and 24 weeks were not different among LN, LNE, and LNN groups. Conclusion: Dietary salt restriction lessens renal damage, at least in part, by inhibiting compensatory renal growth and reducing blood pressure. Enalapril was particularly successful in reducing proteinuria and glomerular injury when combined with dietary salt restriction.

• 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.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.45 no.10
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• pp.1204-1212
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• 2002

Purpose : Early surfactant therapy with either gentle ventilation, high-frequency ventilation or aggressive weaning of mechanical ventilation are principles for the treatment of respiratory distress syndrome(RDS). We studied to determine the accessibility of noninvasive nasal continuous positive airway pressure(CPAP) rather than mechanical ventilation by invasive intubation after early surfactant therapy. Methods : The study group consisted of 14 infants who were born and diagnosed with moderate respiratory distress syndrome and received early surfactant therapy with nasal CPAP of PEEP 5-6 cm $H_2O$ within two hours after birth in the Fatima neonatal intensive care unit for two years from January 1999 to August 2001. The control group consisted of 15 infants who were diagnosed with the disease and could be weaned from mechanical ventilator within five days after birth during the same period. Results : The characteristics, the severity of clinical symptoms and laboratory findings in the two groups at birth showed no significant difference. Neither did the interim analysis of laboratory data in two groups. Of 14 infants in the study group who received nasal CPAP after early surfactant therapy, only two infants showed weaning failure with this therapy. In the response cases, duration of CPAP was five days and mean airway pressure was $5.4{\pm}0.5cm$ $H_2O$. Two had the complication of CPAP with abdominal distension. Final complications and outcomes in the two groups showed no signifcant difference(P>0.05). Conclusion : The clinical courses in the two groups showed no significant difference. Therefore, we suggest that early surfactant therapy with noninvasive nasal CPAP is a simple and safe method rather than aggressive weaning after invasive mechanical ventilation in moderate respiratory distress syndrome.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.8-17
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• 2006

Purpose : We describe the changes of rat glomerular epithelial cells when exposed to high levels of glucose and advanced glycosylation endproducts(AGE) in the in vitro diabetic condition. We expect morphological alteration of glomerular epithelial cells and permeability changes experimentally and we may correlate the results with a mechanism of proteinuria in DM. Methods : We made 0.2 M glucose-6-phsphate solution mixed with PBS(pH 7.4) containing 50 mg/mL BSA and pretense inhibitor for preparation of AGE. As control, we used BSA. We manufactured and symbolized five culture dishes as follows; B5 - normal glucose(5 mM) + BSA, B30 - high glucose(30 mM) + BSA, A5 - normal glucose(5 mM) + AGE, A30 - high glucose(30 mM) + AGE, A/B 25 - normal glucose(5 mM) + 25 mM of mannitol(osmotic control). After the incubation period of both two days and seven days, we measured the amount of heparan sulfate proteoglycan(HSPG) in each dish by ELISA and compared them with the B5 dish at 2nd and 7th incubation days. We observed the morphological changes of epithelial cells in each culture dish using scanning electron microscopy(SEM). We tried the permeability assay of glomerular epithelial cells using cellulose semi-permeable membrane measuring the amount of filtered BSA through the apical chamber for 2 hours by sandwich ELISA. Results : On the 2nd incubation day, there was no significant difference in the amount of HSPG between the 5 culture dishes. But on the 7th incubation day, the amount of HSPG increased by 10% compared with the B5 dish on the 2nd day except the A30 dish(P<0.05). Compared with the B5 dish on the 7th day the amount of HSPG in A30 and B30 dish decreased to 77.8% and 95.3% of baseline, respectively(P>0.05). In the osmotic control group (A/B 25) no significant correlation was observed. On the SEM, we could see the separated intercellular junction and fused microvilli of glomerular epithelial cells in the culture dishes where AGE was added. The permeability of BSA increased by 19% only in the A30 dish on the 7th day compared with B5 dish on the 7th day in the permeability assay(P<0.05). Conclusion: We observed not only the role of a high level of glucose and AGE in decreasing the production of HSPG of glomerular epithelial cells in vitro, but also their additive effect. However, the role of AGE is greater than that of glucose. These results seems to correlate with the defects in charge selective barrier. Morphological changes of the disruption of intercellular junction and fused microvilli of glomerular epithelial cells seem to correlate with the defects in size-selective barrier. Therefore, we can explain the increased permeability of glomerular epithelial units in the in vitro diabetic condition.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.123-129
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• 1999

Purpose: Acute poststreptococcal glomerulonephritis(APSGN) is a renal disease which is characterized by glomerular proliferation and inflammatory changes due to immune reaction. Although the 95% of patients with APSGN seems to recover fully and present as benign course, the remaining patients show poor prognosis. Therefore comparative retrograde study between APSGN with and without nephrotic syndrome was done to find out the any prognostic indicator to predict the outcome in patients with APSGN. Methods: We had retrospectively analyzed seventy-one patients who were diagnosed as APSGN clinically from Mar.1989 to Feb.1999 in Yonsei university medical center. Sixty-four of the patients was APSGN without nephrotic syndrome(Group A) and seven patients were in APSGN with nephrotic syndrome(Group B). Results: Patients who were diagnosed as APSGN with nephrotic syndrome were seven(9.9%) out of seventy-one. In the comparative study, sex ratio was 1:1 in group A and 1.9: 1 in group B, onset mean age was $8.9{\pm}2.6$ in group A and $8.8{\pm}2.6$ in group B. Following clinical profiles were compared but there were no significant difference between these two groups: WBC count($9413{\pm}2964\;vs\;9368{\pm}2650(/mm^3)$), hemoglobin($10.6{\pm}1.2\;vs\;10.0{\pm}0.9(gm/dL)$), ASO($746.1{\pm}640.7\;vs\;614.9{\pm}475.9(IU/ml)$), $C_3(20.1{\pm}17.0\;vs\;16.9{\pm}13.1(mg/dL)$), $C_4(22.8{\pm}9.5\;vs\;22.6{\pm}6.9(mg/dL)$), BUN($25.8{\pm}26.1\;vs\;28.1{\pm}14.5(mg/dL)$), creatinin($0.8{\pm}0.3\;vs\;0.8{\pm}0.3(mg/dL)$), $C_{cr}(80.6{\pm}28.8{\pm}62.4{\pm}31.4(ml/min/1.73\;m^2$)), the duration of edma, gross hematuria, and hypertension. However, we found that there were a significant difference in the duration of proteinuria($1.95{\pm}2.27\;vs\;13.3{\pm}21.1(months)$)(P<0.05), decreased $C_3$ duration($1.9{\pm}2.9\;vs\;7.3{\pm}5.0(weeks)$)(P<0.05) and especially it was proloned according to the amount of early urine protein excretion. Conclusion: Our study showed markedly prolonged duration of proteinuria and decreased $C_3$ duration in patients with APSGN with nephrotic syndrome. We were not able to find the definite prognostic factor that will guide the outcome of patients with APSGN accompaning nephrotic syndrome, but above findings seemed to represent as a relative indication of the outcome of the disease. All patients recovered completely and we did not experience any cases that progressed into the renal failure.

• 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)

• 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.