• Title/Summary/Keyword: 이형성증

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The efficacy of ketogenic diet in childhood intractable epilepsy with malformation of cortical development (대뇌 피질 발달 기형을 동반한 난치성 소아 간질에서 케톤생성 식이요법의 효과)

  • Lee, Young-Mock;Kang, Du Cheol;Chung, Da Eun;Kang, Hoon Chul;Kim, Heung Dong
    • Clinical and Experimental Pediatrics
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    • v.49 no.2
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    • pp.187-191
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    • 2006
  • Purpose : Malformation of cortical development(MCD) constitutes an important etiology of intractable epilepsy and is considered an indication for surgical treatments, though their efficacy is limited and variable depending on MCD's location or distribution. Ketogenic diets are widely known to be effective, but as little study has been made concerning their efficacy on epilepsy with MCD, we evaluated the efficacy of ketogenic diets on MCD patients compared with that of epileptic surgery, which is more invasive. Methods : We performed retrospective studies and analyse on 30 patients with MCD diagnosed by brain MRI and treated with ketogenic diets for intractable epilepsy since 1998, checking decreases in their seizure episodes after starting the diets. Results : Cortical dysplasia was observed in 24(80.0 percent) patients as the most common type of MCD. Also, MCD was observed in unilateral hemisphere most commonly, in 23(76.7 percent) patients; it was observed in both hemispheres in 7(23.3 percent) patients. Nine(30.0 percent) out of 30 patients became seizure-free after starting ketogenic diets, and 14(46.7 percent) patients experienced 50 percent seizure reductions as well. Age of starting the diet or the duration of epilepsy period before starting showed no statistical relationship with the efficacy of the diet. Though the younger the patient and the longer the treatment the more effective the diet seemed to be, there was no statistical correlation between them. The location of MCD showed no significance neither. Conclusion : Considering various limits and invasiveness of surgical treatment, a ketogenic diet could be a good tool in treating children with intractable epilepsy with MCD.

Correlation of serum total hydroperoxide levels and diseases of prematurity (미숙아 합병증 발생과 혈중 활성 산화물 농도와의 관계)

  • Kim, Shin-Hye;Hur, Hae Young;Lee, Kyu Hyoung;Moon, Ja Young;Chae, Kyu Young
    • Clinical and Experimental Pediatrics
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    • v.50 no.8
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    • pp.746-752
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    • 2007
  • Purpose : Total hydroperoxide (TH), free radical-mediated oxidation product can be used as a measure of free radical injury. The aim of the present study was to see if preterm newborns are at increased risk for oxidative stress compared with term newborns, and to determine whether oxidative stress during postnatal first 1 week is associated with clinical outcomes in preterm infants. Methods : Serum TH levels of preterm infants (n=39) were compared with those of term infants (n=24) on the postnatal day 1. Among the preterm infants, serum TH levels of uncomplicated group (n=23) were also compared with those of complicated group (n=16) who developed oxygen radical related diseases on the postnatal day 1 and 7. Retrospective analysis was performed to find out risk factors for oxygen radical injuries based on birth history, laboratory data, neuroimaging findings and clinical progress in two preterm groups. Results : Serum TH levels on postnatal day 1 were higher in the preterm infant group than the term infant group. Serum TH levels on postnatal day 1 in the complicated preterm infant group were significantly higher compared with uncomplicated group, but there was no significant difference in serum TH levels on postnatal day 7. Also, there was no significant difference in serum TH levels between uncomplicated preterm infants and term infants. Serum TH level on postnatal day 1 was independently associated with higher morbidity after adjusting for gestational age, Apgar score (5 min), arterial blood gas analysis. Conclusion : Complicated preterm newborns are at increased risk for oxidative stress compared with uncomplicated newborns and term newborns. Oxidative injury during the prenatal or postnatal day 1 is associated with adverse outcomes in preterm infants. Elevated TH levels on postnatal day 1 may have a value to predict clinical outcomes in preterm infants.

Indication of Bone Marrow Aspiration in Acute Idiopathic Thrombocytopenic Purpura in Children (소아 급성 특발성 혈소판 감소성 자반증에서 골수흡인 검사의 적응)

  • Kim, Won-Duck;Hah, Jeong-Ok
    • Journal of Yeungnam Medical Science
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    • v.18 no.2
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    • pp.239-245
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    • 2001
  • Background: Acute idiopathic thrombocytopenic purpura(ITF) is one of the common hematologic disorders in children. Bone marrow aspiration (BMA) is often performed in children with acute ITP to rule out leukemia, aplastic anemia or other hematologic diseases. However, whether BMA is needed in children with typical clinical and hematological features of acute ITP have been questioned. This study was performed to examine the proper indication of BMA in acute childhood ITF. Materials and Methods: The medical records and BMA reports of children with the provisional diagnosis of acute ITP were reviewed from January 1984 to December 2000. Patients were divided into two groups, one with typical and another with atypical clinical and hematological features of acute ITP. Typical acute ITP group was characterized by the history of previous viral infection, well being appearance, no hepatosplenomegaly, no lymphadenopathy, normal Hb, WEC, neutrophil count and peripheral blood smear except thrombocytopenia. A platelet count of $50{\times}l0^9/L$ or lower was the cutoff level. Results: Total 120 children with the provisional diagnosis of acute ITP were included. One hundred eighteen of them were confirmed to have acute ITP by BMAs. Of these, 66 had typical and 54 had atypical features. All of typical features and 52 of 54 with atypical features of acute ITP were confirmed to have acute ITP by BMAs. Two patients with atypical features of acute ITP were diagnosed as aplastic anemia and myelodyspalstic syndrome, respectively, by BMAs. Conclusion: This study concludes that BMA is not needed for the children with typical features of acute ITP but it is needed for the children with atypical features of acute ITP to rule out other hematologic disorders.

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Effect of prophylactic indomethacin in extremely low birth weight infants (초극소 저출생체중아에서 예방적 indomethacin 투여효과)

  • Lee, Bo Lyun;Kim, Su Jin;Koo, Soo Hyun;Jeon, Ga Won;Chang, Yun Sil;Park, Won Soon
    • Clinical and Experimental Pediatrics
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    • v.49 no.9
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    • pp.959-965
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    • 2006
  • Purpose : The purpose of this study was to investigate the effect of prophylactic indomethacin on reduction of patent ductus arteriosus(PDA) and intraventricular hemorrhage(IVH) in extremely low birth weight infants(ELBWI). Methods : Retrospective review of 84 ELBWI who were admitted to our neonatal intensive care unit from June 2004 to April 2006 was performed. Patients were divided into prophylactic group(n=28) and control group(n=56), where prophylactic indomethacin were given within 6 hours after birth. Clinical outcomes were compared between these groups. Results : There were no significant differences in gestational age, birth weight, incidence of hemodynamically significant PDA and severe IVH, and mortality between prophylactic group and control group. However, there were more frequent indications for therapeutic indomethacin, higher incidence of intestinal perforation, and longer time to achieve full enteral feeding in prophylactic group than control group. The incidence of other adverse events attributed to indomethacin prophylaxis did not differ between two groups. Conclusions : Prophylactic indomethacin may not prevent hemodynamically significant PDA and severe IVH in ELBWI. On the contrary, it may be associated with increased risk of adverse events. Further efforts should be investigated to decrease PDA and severe IVH in ELBWI.

Brain F-18 FDG PET for localization of epileptogenic zones in frontal lobe epilepsy: visual assessment and statistical parametric mapping analysis (전두엽 간질에서 F-18-FDG PET의 간질병소 국소화 성능: 육안 판독과 SPM에 의한 분석)

  • Kim, Yu-Kyeong;Lee, Dong-Soo;Lee, Sang-Kun;Chung, Chun-Kee;Yeo, Jeong-Seok;Chung, June-Key;Lee, Myung-Chul
    • The Korean Journal of Nuclear Medicine
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    • v.35 no.3
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    • pp.131-141
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    • 2001
  • Purpose: We evaluated the sensitivity of the F-18 FDG PET by visual assessment and statistical parametric mapping (SPM) analysis for the localization of the epileptogenic zones in frontal lobe epilepsy. Materials and Methods: Twenty-four patients with frontal lobe epilepsy were examined. All patients exhibited improvements after surgical resection (Engel class I or II). Upon pathological examination, 18 patients revealed cortical dysplasia, 4 patients revealed tumor, and 2 patients revealed cortical scar. The hypometabolic lesions were found in F-18 FDG PET by visual assessment and SPM analysis. On SPM analysis, cutoff threshold was changed. Results: MRI showed structural lesions in 12 patients and normal results in the remaining 12. F-18 FDG PET correctly localized epileptogenic zones in 13 patients (54%) by visual assessment. Sensitivity of F-18 FDG PET in MR-negative patients (50%) was similar to that in MR-positive patients (67%). On SPM analysis, sensitivity decreased according to the decrease of p value. Using uncorrected p value of 0.05 as threshold, sensitivity of SPM analysis was 53%, which was not statistically different from that of visual assessment. Conclusion: F-18 FDG PET was sensitive in finding epileptogenic zones by revealing hypometabolic areas even in MR-negative patients with frontal lobe epilepsy as well as in MR-positive patients. SPM analysis showed comparable sensitivity to visual assessment and could be used as an aid in the diagnosis of epileptogenic zones in frontal lobe epilepsy.

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Association between Ureaplasma urealyticum Colonization and Adverse Outcomes in Premature Infants (출생 시 Ureaplasma urealyticum 집락이 조산아의 임상 양상에 미치는 영향)

  • Yun, Jin-Sang;Chang, Sun-Jung;Jo, Heui-Seung;Lee, Kyu-Hyung
    • Neonatal Medicine
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    • v.16 no.1
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    • pp.47-54
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    • 2009
  • Purpose: Present evidences suggest that Ureaplasma urealyticum is a cause of pneumonia, septicemia, and bronchopulmonary dysplasia (BPD) in newborn infants, particularly those born prematurely. The purpose of this work was to examine the relationship between Ureaplasma urealyticum in the tracheal aspirates and adverse outcomes, such as BPD and early onset neonatal sepsis in premature infants. Methods: A polymerase chain reaction (PCR) was performed on tracheal aspirates collected within 24 hour after birth in 176 premature infants less than 35 weeks of gestation and admitted to the neonatal intensive care unit of Bundang CHA Hospital. Results: U. urealyticum was detected in 37 of 176 preterm infants (21.0%). Gestational age ($29^{+5}{\pm}2^{+5}$ wk vs. $30^{+6}{\pm}2^+{-5}$ wk, P=0.0l3) and birth weight (1.39${\pm}$0.44 kg vs. 1.59${\pm}$0.55 kg, P=0.037) were lower in the U urealyticum-positive group compared to the control group. The incidence of early onset neonatal sepsis (16.2% vs. 6.5%, P=0.045) and BPD (45.9% vs. 29.5%, P=0.047) was higher in the U urealyticum-positive group compared to the control group, but the severity of BPD was not different between two groups. However, multiple logistic regression analysis revealed that the presence of U. urealyticum was not independently related to the development of early onset neonatal sepsis and BPD. Conclusion: The results suggest that colonization of the lower respiratory tract by U. urealyticum might not be related to the development of neonatal sepsis and BPD directly in preterm infants.

Evaluation of Perinatal and Management Factors Associated with Improved Survival in Extremely Low Birth Weight Infants (출생 체중 1,000 g 미만의 초극소 미숙아의 생존율 향상과 연관된 산과 및 치료인자)

  • Park, Sung Eun;Jeon, Ga Won;Choi, Chang Won;Hwang, Jong Hee;Koo, Soo Hyun;Kim, Yu Jin;Lee, Chang Hoon;Chang, Yun Sil;Park, Won Soon
    • Clinical and Experimental Pediatrics
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    • v.48 no.12
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    • pp.1324-1329
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    • 2005
  • Purpose : The aim of this study was to evaluate recent improvements in the survival rate of extremely low birth weight (ELBW) infants and to identify perinatal and management factors that are associated with improved survival. Methods : Two groups of ELBW infants who were admitted to our neonatal intensive care unit (NICU) during two distinct eras : November 1994-December 1999 (Period 1 : n=100) and January 2000-April 2004 (Period 2 : n=166) were retrospectively reviewed. Results : Despite the younger gestational age and smaller birth weight of the ELBW infants in period 2, not only did their survival rate increased to 75 percent from 60 percent in period 1, but their incidence of morbidities such as bronchopulmonary dysplasia, confimed sepsis and intraventricular hemorrhage (${\geq}$Grade III) also declined. Factors significantly associated with improved survival included the use of antenatal steroids, a longer duration of nasal continuous positive airway pressure and the absence of intraventricular hemorrhage (${\geq}$Grade III). Conclusion : We believe that optimized clinical practice, that emphasized less invasive care, contributed to the recent improvements in the survival rate of ELBW infants.

Gastrointestinal surgery in very low birth weight infants: Clinical characteristics (극소 저체중 출생아에서 시행한 소화기계 수술의 임상적 특성)

  • Kim, Ji Eun;Yoo, Hye Soo;Kim, Hea Eun;Park, Soo Kyoung;Jeong, Yoo Jin;Choi, Seo Heui;Seo, Hyun Joo;Chang, Yun Sil;Seo, Jeong Meen;Park, Won Soon;Lee, Suk Koo
    • 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.

Cord blood IL-10, IL-12 in preterm newborns as predictors of respiratory distress syndrome and bronchopulmonary dysplasia (호흡곤란증후군과 기관지폐이형성증 예측 인자로서의 미숙아 제대혈 IL-10, IL-12)

  • Park, Jee Yoon;Kim, Ji Young;Cho, Soo Jin;Kim, Young Ju;Park, Hye Sook;Ha, Eun Hee;Park, Eun Ae
    • Clinical and Experimental Pediatrics
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    • v.50 no.3
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    • pp.248-254
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    • 2007
  • Purpose : Inflammation plays a major role in the pathogenesis of RDS and BPD in the immature lung. We investigated the possible role of IL-10 and IL-12 in the cord blood of preterm newborns with RDS or BPD. Methods : Forty preterm newborns whose mothers received antenatal care at Ewha Womans University Mokdong Hospital between January 2003 to June 2005, and agreed to testing their cord blood samples were enrolled. The gestational ages were below 34 weeks. Cord blood level of IL-10 and IL-12 were determined by ELISA. We separated the patients into 2 groups (RDS group and non-RDS group, BPD group and non-BPD group) and compared the cytokine levels and clinical records of the groups. Results : Cord blood IL-10 level showed a significant inverse correlation with gestational age and birth weight (P=0.001, P=0.005). Preterm infants with RDS showed higher IL-10 level (1.0 vs 0.1 pg/mL; P=0.001) in the cord blood than those without RDS. The differences remained statistically significant after correction for the effect of gestational age between both preterm groups. Despite similar cord blood IL-10 levels, preterm infants with BPD showed no significant difference with those without BPD. Conclusion : Cord blood IL-10 levels are increased in preterm infants which may be due to the immuno-suppression occurring during pregnancy and to fetal immaturity because these levels are inversely correlated with the gestational age. So, Cord blood IL-10 level can be used as the predictor of RDS.

Diagnostic Criteria of $^{99m}Tc$-diethylenetriaminepentaacetic acid Captopril Renal Scan for the Diagnosis of Renovascular Hypertension by Unilateral Renal Artery Stenosis ($^{99m}Tc$-diethylenetriaminepentaacetic acid 캅토프릴 신장스캔의 단측 신동맥 협착에 의한 신혈관성고혈압 진단 기준)

  • Choi, Seung-Jin;Hong, Il-Ki;Chang, Jae-Won;Park, Su-Kil;Moon, Dae-Hyuk
    • The Korean Journal of Nuclear Medicine
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    • v.38 no.6
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    • pp.498-505
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    • 2004
  • Purpose: We compared captopril renal scintigraphic criteria for the diagnosis of renovascular hypertension by unilateral renal artery stenosis. Materials and Methods: The study group consisted of 24 patients (m/f : 16/8, age: $39{\pm}18$ years) with unilateral renal artery stenosis who underwent renal artery revascularization and captopril renal scintigraphy with $^{99m}Tc$-diethylenetriaminepentaacetic acid between May 1995 and April 2004. The blood pressure response was classified as cure/improvement or failure. We evaluated captopril-induced changes in relative function (BCfun) and renogram grade (0 to 5: 0=normal, and 5=renal failure pattern without measurable uptake) (CBren) and the difference of renograms between the normal and stenotic kidney on captopril scan (CNren). Results: light of 24 patients were cured and 11 improved and 5 patients were classified as failed revascularization. Significant predictors of a cure or improvement of blood pressure were younger age, stenosis by fibromuscular dysplasia or arteritis, BCfun, CBren and CNren. Areas under the receiver operating characteristic curve of age, BCfun, CBren and CNren were not significantly different. Positive and negative predictive values of predictors were 100% and 42% (age ${\leq}38$): 92% and 50% (BCfun ${\geq}1%$): 92% and 75% (CBren ${\geq}1$), and 90% and 60% (CNren ${\geq}1$), respectively. Conclusion: Captopril induced changes in renal function and renogram can reliably predict hypertension response to revascularization. Renogram pattern on captopril scan can diagnose renovascular hypertension without baseline data in patients with unilateral renal artery stenosis.