• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.784-789
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• 2006

Purpose : $Henoch-Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis involving small vessels of skin, gastrointestinal(GI) tract and kidney. Digestive involvement of HSP can be serious with massive GI bleeding, perforation, and intussusception. However, some patients do not respond to conventional corticosteroid therapy. In this study, we investigated the efficacy of intravenous immunoglobulin (IVIG) for serious digestive manifestations not responding to steroid. Methods : From April 1999 to January 2005, 22 children diagnosed as HSP with severe GI symptoms were included. Initially, all patients were treated with intravenous methylprednisolone. IVIG 2 g/kg of body weight was infused in children refractory to steroid therapy. Clinical data were reviewed retrospectively. Results : Among 22 children, 12 children underwent IVIG therapy. The mean duration of corticosteroid therapy was $5.61{\pm}4.9$ days before IVIG therapy, and 11 of 12 patients experienced disappearance of GI manifestations after the initiation of IVIG infusion. In one patient, IVIG was ineffective in relieving abdominal pain, but melena subsided. Comparison of the duration of hospitalization between IVIG group and corticosteroid group revealed no significant difference($12.8{\pm}7.6$ days vs. $13.2{\pm}7.8$ days, P=0.777). But, the total duration of abdominal pain decreased in IVIG group although the difference between two groups was not significant($8.8{\pm}8.1$ days vs. $14.8{\pm}16.9$ days, P=0.306). Among 10 children treated with steroid only, 2 children were operated for bowel perforation and intussusception. In contrast, there was no perforation in 12 children who underwent IVIG therapy. Conclusion : IVIG could be the alternative therapy to corticosteroids in children with severe digestive manifestations of HSP.

• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.295-302
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• 2009

Purpose : To report our experience of gastrointestinal (GI) operations (OP) performed in very low birth weight infants (VLBWI) and to evaluate their clinical characteristics. Methods : Among the 1,117 VLBWI admitted to the SMC neonatal intensive care unit from November 1994 to February 2007, the medical records of 37 infants who underwent GI OP (except inguinal hernia OP) and 1,080 VLBWI without GI OP were retrospectively reviewed. Results : The mean gestational age ($27^{+6}2^{+3}$ vs. $28^{+5}{\pm}$2^{+6}$) and birth weight (979${\pm}$241 g vs. 1,071${\pm}$271 g) of the 37 VLBWI who underwent the GI OP was lower than the VLBWI without GI OP group (n=1,080). Mortality rates in the GI OP group were significantly higher than in the non GI OP group (28% vs. 15%, P<0.001). The incidence of cholestasis, retinopathy of prematurity and periventricular leukomalacia were higher in the GI OP group than in the non GI OP group, but the incidence of bronchopulmonary dysplasia was not significantly different between the GI OP group and the non GI OP group. For GI OP indications, focal intestinal perforation was most common and showed a more favorable outcome than necrotizing enterocolitis. Compared with an earlier 7-year period, 1994-2000, the incidence and survival rates increased in the subsequent 2001-2007 period. Conclusion : GI OP was associated with high mortality and morbidity in VLBWI. Further efforts to improve outcomes of GI OP in VLBWI should be investigated to improve the quality of care in VLBWI.

• Investigative Magnetic Resonance Imaging
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• v.4 no.2
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• pp.100-106
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• 2000

Purpose : To evaluate the usefulness of cerebral blood flow measurement applied to perfusion weighted image with short-scan time single shot gradient echo-planar technique in measuring cerebral blood volume(rCBV) of normal rabbits. Materials and methods : With 2.1-3.6 kg weighted rabbits, image is acquired when they are in supine position in children positioner. Perfusion weighted image is acquired to 44 seconds per 1 second successively. After 4 seconds later, Gd-DTPA 2ml are injected into int. jugular vein with 2 ml per second and normal saline is also injected after that. Same technique is applied 2 times per 30 minites in same rabbit. After Image is obtained in two part of cerebral cortex at vertex, convexity, in one of basal ganglia with choosing about $3-5{\textrm{mm}^2}$ areas. Curve of signal intensity changes in time sequence is drawn. After this images are transmitted by PC and software IDL, regional cerebral blood volume is measured with imaging processing program made by us. Results : With 22 of 24 rabbits, satisfactory 1-2 signal intensity versus time curve is made. Cerebral blood capacity and contrast media stay time (ST) is measured in two cerebral cortex and basal ganglia refering in parietal cerebral cortex. Mean focal cerebral blood flow capacity ratio in cortex was $0.97{\pm}0.35$ and in basal ganglia, $0.99{\pm}0.37$, mean contrast media stay time in cortex was $9.83{\pm}1.63$ sec and in basal gaiglia, $9.42{\pm}1.14$ sec, but there was no statistically significant difference between two areas ($\rho$=0.05). Conclusion : In cerebral cortex and basal ganglia, there is no difference in mean focal blood volume and mean contrast stay time. Therefore, PWI is useful in cerebral blood flow and early diagnosis, prognosis of cerebral ischemic disease. Hereafter, it is helpful in analysing cerebral blood flow changes with comparison difference in rCBV between normal tissue and ischemic tissue, and that with DWI finding in infarcted patient.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1503-1511
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• 2002

Purpose : Although the short- and long-term outcomes of low birth weight(LBW) neonatal intensive care unit(NICU) survivors have been extensively studied, much less information is available for normal birth weight(NBW) infants(greater than 2,500 gm) who require NICU care. Methods : We retrospectively examined the neonatal hospitalizations and one year health status of 302 NBW and 131 LBW admissions to our NICU. Information on the neonatal hospitalization was obtained from a review of medical records. Postdischarge health status was collected by using telephone surveys and medical records. Results : After initial discharge, 21.2% of the NBW infants and 23% of the LBW infants required rehospitalization during the first year of life and there was no significant difference between the two groups. The reasons for rehospitalization of the NBW infants included respiratory disorders (32.1%), G-I problems(26.2%), genitourinary problems(11.9%), surgery(10.7%), cardiac problems(7.1%), and congenital/developmental problems(1.2%). For the LBW infants, the order of frequency was the same, with the percentages slightly different. Neonatal risk factors related to the rehospitalization of the NBW infants included mechanical ventilation, duration of mechanical ventilation, and congenital anomaly. But no positive significant correlation of neonatal risk factors with rehospitalization of LBW infants was found. Conclusion : Low and normal birthweight NICU survivors were rehospitalized at similar rates. The most common cause of rehospitalization was respiratory problems. Neonatal risk factors related to rehospitalization of NBW infants were mechanical ventilation, duration of mechanical ventilation, and congenital anomaly. However, no positive significant correlation of neonatal risk factors with rehospitalization of LBW infants was found. The data suggests that NBW infant survivors, as well as LBW infant NICU survivors, require close follow up.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1551-1558
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• 2002

Purpose : Loss of hippocampal interneurons in dentate gyrus has been reported in patients with severe temporal lobe epilepsy and in animals treated with kainic acid(KA). Interneurons contain $Ca^{2+}$- binding protein parvalbumin(PV). The effects of kainic acid on parvalbumin-immunoreactive (PV-IR) interneurons in dentate gyrus were investigated in organotypic hippocampal slice cultures. Methods : Cultured hippocampal slices from postnatal day nine C57/BL6 mice were exposed to $10{\mu}M$ KA, and were observed at 0, 8, 24, 48, 72 hours after a one hour KA exposure. Neuronal injury was determined by morphologic changes of PV-IR interneuron in dentate gyrus. Results : Transient(1 hour) exposure of hippocampal explant cultures to KA produced marked varicosities in dendrites of PV-IR interneuron in dentate gyrus and the shaft of interbeaded dendrite is often much thinner than those in control. The presence of varicosities in dendrites was reversible with KA washout. The dendrites of KA treated explants were no longer beaded at 8, 24, 48 and 72 hours after KA exposure. The number of cells in PV-IR interneurons in dentate gyrus was decreased at 0, 8 hours after exposure. But there was no significant difference in 24, 48 and 72 hours recovery group compared with control group. Conclusion : The results suggested that loss of PV-IR interneurons in dentate gyrus is transient, and is not accompanied by PV-IR interneuronal cell death.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.592-597
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• 2010

Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$ $aureus$) and $Pseudomonas$ $aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.77-84
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• 2014

Purpose: The purpose of this study was to compare the validity of Onen's alternative grading system (AGS) with that of the APDRP and SFU grading systems in patients with isolated and complicated congenital hydronephrosis. Methods: We retrospectively reviewed the medical records of 153 patients (204 renal units) diagnosed with congenital hydronephrosis between January 2002 and December 2011. We classified patients into 2 groups; isolated or complicated hydronephrosis. All renal units were graded according to anterior-posterior diameter of renal pelvis (APDRP), Society for Fetus Urology (SFU) and Onen's grading systems. We analyzed the prognosis of hydronephrosis, according to each grading system, at 2 years of age. Results: There were 152 renal units with isolated hydronephrosis and 52 renal units with complicated hydronephrosis. The isolated hydronephrosis group had a lower grade according to Onen's AGS, and showed more frequent spontaneous remission by 2 years of age. There was more frequent obstruction (P =0.000) and surgical treatment (P =0.000) of units with high-grade hydronephrosis according to Onen's AGS. In the complicated hydronephrosis group the frequencies of spontaneous remission (P =0.015) and renal dysfunction (P =0.013) were significantly higher than those in high-grade hydronephrosis, as indicated by Onen's AGS. There were no significant differences in clinical outcomes among the highest grade groups, according to the 3 systems, in either isolated or complicated hydronephrosis. Conclusion: Onen's AGS reflects the prognosis of hydronephrosis as well as other grading systems in those with isolated hydronephrosis. It was better predictor of renal dysfunction in those with complicated hydronephrosis. However, Onen's AGS was not superior to the other grading systems in terms of predicting prognosis, especially in high-grade hydronephrosis.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.143-153
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• 2010

Purpose: Recently, massive proteinuria has been observed in some transplant patients after switching cyclosporine A (CsA) to sirolimus. To evaluate the pathogenesis of sirolimus-associated proteinuria, we investigated the early changes in slit diaphragm molecules by various administrative conditions of sirolimus and CsA. Methods: In vitro-Mouse podocytes were incubated with buffer (C), sirolimus ($10\;{\mu}g/mL$) after CsA ($10\;{\mu}g/mL$) (C-S), sirolimus only (S) and CsA and sirolimus simultaneously (C+S) for 12, 24, and 48 hours. In vivo- twenty four SPF female Wistar rats were divided into 4 groups buffer (C), sirolimus after 2 weeks of CsA (C-S), sirolimus only (S) and CsA and sirolimus simultaneously (C+S). All groups were treated by intraperitoneal injection every other day for 4 weeks (CsA: 25 mg/kg, sirolimus: 0.5 mg/kg). The changes in mRNA of slit diaphragm molecules were examined by RT-PCR. Results: The mRNA of nephrin was significantly decreased in group C-S and C+S in vitro. In vivo, the mRNA of nephrin in all groups using sirolimus and the mRNA of podocin in group C-S and C+S were decreased. Microscopically, group C-S and C+S showed small vacuolization and calcification in proximal tubular epithelial cells. Immunohistochemistry using nephrin and podocin antibodies did not show remarkable decrease of staining along the glomerular capillaries. Electron-microscopically, focal fusion of foot processes was seen in group C-S and C+S. Conclusion: This study suggests the decrease of slit diaphragm molecules (nephrin and podocin) in podocyte may be one of the causes of sirolimus associated proteinuria, and podocyte injury by sirolimus may need a primary hit by CsA to develop the proteinuria.

• Journal of Trauma and Injury
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• v.23 no.2
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• pp.68-74
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• 2010

Purpose: The purpose of this study is, first, to analyze the risk factors that influence the severity of injury in pediatric multiple trauma patients and, second, to present solutions for the problems related to the treatment of such patients. Our living situations are so complicated that the danger of accidents is truly open to children who are not prepared. We need to draw attention to the increased numbers of various accidents involving children. Methods: We studied patients who visited the Emergency Medical Center at Chosun University Hospital from January 1, 2006, to December 31, 2008. Using medical records, we evaluated the general characteristics: the mechanism of injury, the vital signs, the revised trauma score (RTS), the injury severity score (ISS), and the hemoglibin value, which was checked at the time of visit, and the presence or the absence of emergent on regular surgery. We divided the level of injury as follows: light level (1-8 points), mid level (9-15 points), and serious level (above 16 points). We analyzed the medical data by using SPSS 17.0 for windows. Results: The average age of the patients examined was 8.6 years. The number of 6- to 11-year-old patients was 96, which was the largest, but the degree of injury severity was the highest among infant (0-2 years), according to ISS 7.95(${\pm}6.85$). The frequency of accidents was highest on sunny days, and most accidents occurred from 16:00 to 20:00. The cause of multiple trauma for children was the greatest in the traffic accident, (95 patients, 49.0%). In addition, the trauma caused by traffic accidents showed the highest ISS value ($9.02{\pm}6.42$) and the most serious degree (P=0.004). The ISS level of injury ($8.40{\pm}6.64$) for patients moved from a secondary hospital was higher than that ($6.49{\pm}5.57$) for patients who visited the medical center directly. The severity of injury was highest for patients who used a 119 ambulance ($8.84{\pm}5.80$). According to the injured parts of body, Injuries to the arms and the feet most frequent (79 patients, 40.7%), but the level of injury was the highest for internal organs and chest, $16.42{\pm}8.56$ and $11.23{\pm}6.97$, respectively. Conclusion: We used Abbreviated Injury Scale (AIS) in order to examine the characteristics by injured body part for pediatric multiple trauma patients. Because the degree of injury was the highest for internal organs or the chest, we need to more seriously examine and provide for patients who are suspected of having injuries to the internal organs or the chest. We need to quickly determine the need for surgery in patients with serious injuries to the arms and the feet, which is the greatest in frequency. In particular, we need to consider the surgical care of patients who are not very high in the severity of injury to their brains.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.615-621
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• 2002

Purpose : The neonatal cardiac catheterization and angiogram for transcatheter therapy are still essential methods in congenital heart disease, so we reviewed our experience with neonatal cardiac catheterization over 10 years at a single institution. Methods : A retrospective review of all 139 neonatal catheterizations from January 1991 to December 2000 at Sejong Heart Institution was performed. The purpose of the catheterizations, ages, body weights and the complications by the exam was surveyed. Results : The mean age of our 139 patients was 14.9 days and the mean body weight was 3.3 kg. As for the diagnosis : TGA in 49 patients, PAIVS in 26 patients, DORV in 14 patients, PS in 14 patients, PAVSD in 11 patients. For the cardiac catheterization, general anesthesia was performed in 65% of total and fluoroscopy time was $20.1{\pm}14.5$ minutes during cardiac catheterization. In the interventional cardiac catheterization which was 75% of the total, the age and body weight were statistically the same but the irradiation time was longer than the diagnostic catheterization( P=0.001). There were 48 cases of atrial septostomy, 16 cases of balloon pulmonary valvuloplasty and 25 cases of transcatheter pulmonary valvotomy. Complications of cardiac catheterization were found in 16.3%, but there was no difference between interventional catheterization and diagnostic catheterization. Conclusion : Therapeutic cardiac catheterization in neonates is a relatively safe and effective method in congenital heart disease.