소아 담석증에 대한 임상적 연구

은호선,백승연,정기섭,Eun, Ho Seon,Baek, Seoung Yon,Chung, Ki Sup 대한소아소화기영양학회 2005 Pediatric gastroenterology, hepatology & nutrition Vol.8 No.2

Purpose: Gall bladder (GB) stones in children are rarely observed, and so reports of them have been quite limited in Korea. Therefore, we tried to assess the epidemiology and clinical features of children with GB stone diagnosed at Severance Hospital. Methods: A clinical study was performed on 18 patients below 15 years of age diagnosed with GB stone by abdominal ultrasonography or CT scan at Severance hospital from January 1999 to May 2005. Results: Concerning patients' profile, their sex ratio of male to female in total 18 cases was 2 : 1 with the mean age of $6{\pm}4.3years$. 15 patients (83.3%) were asymptomatic. Inducing factors were found in 14 children (77.8%) including ceftriaxone therapy in 6 cases (33.3%), infection in 4 cases (22.2%), spherocytosis in 3 cases (16.7%), Down syndrome and abdominal operation in 1 case (5.6%) respectively. Single stone was found in 13 cases (76.5%) and multiple in 4 cases (23.5%). 15 cases (88.2%) had the stones less than 5 mm in size, and 2 cases (11.8%) between 5 and 10 mm. 17 patients received conservative treatment, and one patient had cholecystectomy due to Meckel diverticulum. Conclusion: In this report, Pediatric GB stones are predominant in male children, without typical symptoms, having inducing factors, single and small. Recently the diagnosis of them has been increased because of the development of imaging study. But the reports for them were still rare in Korea. Therefore the study for them is more necessary to find it's clinical characteristics.

Serological investigation of Ureaplasma urealyticum in Korean preterm infants

은호선,이순민,박민수,박국인,남궁란,이철 대한소아청소년과학회 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.11

Purpose: Ureaplasma colonization is related with perinatal complications in preterm infants. Little is known about the difference in virulence among various Ureaplasma urealyticum serovars. The aim of this study was to determine U. urealyticum serovars of preterm infants in order to assess whether any of the serovars were associated with bronchopulmonary dysplasia (BPD). Methods: Three hundred forty-four preterm infants with a gestational age less than 34 weeks admitted to Gangnam Severance Hospital neonatal intensive care unit from July 2011 to December 2012 were included in this study. Tracheal and gastric aspirations were conducted on infants to confirm Ureaplasma colonization. Ureaplasma colonization was confirmed in 9% of infants, of these, serovars were determined by real-time polymerase chain reaction. Results: A total of 31 infants (gestational age, 29.3±3.1 weeks; birth weight, 1,170±790 g) were U. urealyticum positive. The Ureaplasma positive group treated for more days with oxygen and ventilation than the negative group (P<0.05). Histologic chorioamnionitis and moderate to severe BPD were more frequent in the Ureaplasma positive group than in the negative group (P<0.05). U. urealyticum isolates were either found to be a mixture of multiple serovars (32%), serovar 9 alone or combined with other serovars (39%), serovar 11 (26%), 2 (13%), 8 (10%), 10 (13%), and 13 (25%). No individual serovars were significantly associated with moderate to severe BPD and chorioamnionitis. Conclusion: This is the first study to describe the distribution of U. urealyticum serovars from Korean preterm infants. Ureaplasma -colonized infants showed higher incidence of BPD and chorioamnionitis.

Intracranial Pial Arteriovenous Fistula Presenting as Brain Hemorrhage in Newborn Infants

김수연,은호선,신정은,이순민,박민수,남궁란,박국인 대한신생아학회 2015 Neonatal medicine Vol.22 No.4

Congenital intracranial pial arteriovenous fistulas (AVFs) are rare cerebrovascular lesions. Their clinical manifestations tend to vary according to age, with pediatric populations being more likely to have symptoms like congestive cardiac failure and seizures because of arteriovenous shunting; hemorrhage is the major presentation in adult populations. Pediatric populations, especially newborn infants, seldom experience a hemorrhagic event. Here, we report two rare cases of neonates with congenital pial AVF presenting as intraventricular and subdural hemorrhage, respectively, which were treated with endovascular embolization.

중심정맥 도관 관련 감염의 역학 고찰

김정현,은호선,최경민,김도수,용동은 대한소아청소년과학회 2006 Clinical and Experimental Pediatrics (CEP) Vol.49 No.2

Purpose : The purpose of this study is to investigate the pathogens of central venous catheter-related blood stream infections and search for the association among the insertion site, the duration and the underlying conditions with the prevalence of central venous catheter-related blood stream infections under 15 years old. Methods : A retrospective study was performed from Jan, 2003 to Dec, 2003 in Severance Hospital on 112 patients who undertook central venous catheter insertions. Results : We examined 112 patients who undertook central venous catheter insertion. The mean age of patients was 4.77±4.12 years old. Coagulase negative Staphylococci was the most common organism of central venous catheter-related blood stream infections accounting for 33.9 percent, followed by Eenterococcus faecium(9.3 percent), and Staphylococcus aureus(7.5 percent), The most common insertion site was the right femoral vein, followed by the right jugular vein and the left femoral vein. The mean insertion period was 14.17±12.00 days. Conclusion : Central venous catheter-related blood stream infections were not only related to the underlying conditions, but also to the insertion site. We need to study the clinical importance of coagulase negative Staphylococci as it is part of the normal flora of the skin. In future, more studies are needed to take preventive measures and improve treatment methods. 목 적 : 15세 이하 소아의 중심정맥 도관 관련 감염의 임상적 검토를 통해 원인균을 밝히고 삽입위치, 유치기간, 기저 질환 등에 따른 감염 발생과의 연관성을 조사하고자 하였다. 방 법 : 2003년 1월 1일부터 12월 31일까지 연세의료원에 입원하여 중심정맥 도관을 삽입술을 시행 받은 15세 이하 112명을 대상으로 의무기록의 후향적 검토를 시행하였다.결 과 : 총 139례의 배양검사가 시행되었으며, 45례(34.2%)에서 중심정맥 도관 감염이 발생했고(Table 1), 53가지 균주가 동정되었다. 발생 균주는 그람 양성인 coagulase negative Staphylococcus가 18례(33.9%)로 가장 많았고 Enterococcus faecium 5례(9.3%), Staphylococcus aureus 4례(7.5%), Enterococcus faecalis 2례(3.8%) 순으로 발생했고, 그람 음성은 Acinetobacter anitratus 3례(5.7%), Pseudomonas aeruginosa 3례(5.7%), Klebsiella pneumonia 2례(3.8%) 순이었으며, 이외 Yeast 4례(7.5%), Candida albicans 3례(5.7%), Acinetobacter species, α-streptococcus, Candida species, Citrobacter freundii, Diphtheroid 각 1례(1.9%)의 빈도를 보였다. 삽입 부위는 우측 대퇴 정맥이 72례로 가장 많았고, 우측 경정맥 21례, 좌측 대퇴 정맥 20례였다. 중심정맥 도관 삽입 유치기간은 평균 14.17±12.00일이었으며, 감염이 발생하지 않은 군은 13.19±9.10일, 감염이 발생한 군은 15.98±15.98일로 두 그룹 사이에 관련성이 있을 것으로 판단된다(P=0.06). 중심정맥 도관 삽입 환자 중 9명이 사망하였는데 4명에서 중심정맥 도관 감염이 있었으나, 중심정맥 도관 감염과 사망과는 통계적으로 유의한 관계는 없었다(P>0.05).

소아 및 청소년 비만증에서 인슐린 저항성에 미치는 요인

김덕희,은호선,최인경,김호성,차봉수,김동기 대한소아청소년과학회 2005 Clinical and Experimental Pediatrics (CEP) Vol.48 No.10

Purpose : Insulin resistance is the most important risk factor linked to the development of impaired glucose tolerance(IGT), diabetes mellitus and cardiovascular diseases in childhood and adolescent obesity, The purpose of this study was to see whether insulin resistance of obese adolescent is higher than that of obese children. and to analyze gender difference and affecting factors of insulin resistance. Methods : Of the 9,837 school children from 5 to 16 tears old, 92 obese children and 187 adolescent, underwent a two-hour oral glucose tolerance test and plasma glucose, insulin, lipid profiles, leptin and high sensitive C-reactive protein(hs-CRP) were measure. Results : Plasma insulin levels of female were higher compared to those of males during oral glucose tolerance test(P<0.05). Four(4.3%) in obese children and twenty five(13.3%) in obese adolescents met the criteria of IGT. Female, leptin, adiponectin and triglyceride concentrations were strongly correlated with homeostatic model assessment insulin-resistance(HOMA-IR) by multiple linear regression analysis(P<0.05). Conclusion : Obese adolescents might have higher insulin concentrations compared to obese children and obese girls higher insulin concentrations than obese boys. Obese boys and children with impaired glucose tolerance have higher insulin concentrations than those with normal glucose tolerance. HOMA-IR was significantly correlated with female, plasma leptin, adiponectin and triglyceride concentrations. 목 적 : 인슐린 저항성은 소아 및 청소년 비만증에서 내당능장애, 당뇨병증, 심혈관계질환을 일으키는 중요한 위험요인 중 하나로 알려져 있다. 따라서 이 연구의 목적은 비만증 청소년이 소아에 비해 인슐린저항성이 증가하는가 그리고 성별에 따른 차이점을 규명하며 인슐린저항성에 영향을 미치는 요인들을 분석하고자 한다.방 법 : 5-16세의 학생 9,837명을 신체검사하여 92명의 비만증 소아와 187명의 비만증 청소년을 선별하였고, 이들을 대상으로 당부하검사, 공복상태의 혈당, 인슐린농도, 총콜레스테롤, LDL-콜레스테롤, HDL-콜레스테롤, 중성지방의 농도, leptin, hs-CRP 및 adiponectin 농도를 측정하였다.결 과 : 당부하검사를 통한 비만증 소아 및 청소년에서 성별에 따른 인슐린의 농도는 여자에서 인슐린의 농도가 높았다(P< 0.05). 비만증 소아에서 4명(4.3%), 비만증 청소년에서 25명(13.3 %)에서 내당능 장애로 나타났으며, 당뇨병으로 진단된 경우는 1례도 없었다. 인슐린 저항성은 여자, leptin, adiponectin, 중성지방의 농도와 밀접한 상관관계가 있었다(P<0.05). 결 론:비만증에서 인슐린 저항성은 소아보다 청소년에서 컸으며, 남자보다 여자에서 컸다. 또한 leptin 및 중성지방의 농도가 높을수록, adiponectin의 농도가 낮을수록 인슐린 저항성이 컸다. 소아 및 청소년 비만증에서 인슐린 저항성에 미치는 요인들에 관하여 좀 더 많은 연구가 필요하다

미숙아 대사성 골 질환

남궁란,이순민,은호선,박민수,박국인,이철 대한신생아학회 2013 Neonatal medicine Vol.20 No.3

Preterm infants are at significant risk of reduced bone mineral content and subsequent bone disease, variably termed osteopenia of prematurity or neonatal rickets. The prevalence varies depending on gestation, occurs up to 30% of Korean extremely low birth weight infants (ELBW). The etiology is multifactoreal: inadequate nutrient intake, prolonged parenteral nutrition, chronic co-morbidities and immobilization. In Korean ELBW infants, rickets of prematurity was significantly increased by 18times with severe parenteral nutrition associated cholestasis, and by 3 times with moderate/severe bronchopulmonary dysplasia. Prolonged hospitalization with ventilator care could lead to lack of physical activity and muscle contraction against resistance in preterm infants, which may increase bone resorption and demineralization,resulting in osteopenia. Low serum P and high alkaline phosphatase (ALP)are suggestive of metabolic bone disease. In Korean ELBW infants, increased serum ALP (>495 IU) at the age of 5 weeks has a 81% of sensitivity and 87% of specificity for radiographic rickets. An early nutritional intervention can reduce both the prevalence and the severity of osteopenia. Provision of adequate nutrition including energy, protein and minerals, and passive physical exercise during first weeks of life may prevent abnormal bone remodeling activity in preterm infants. Early detection by vigorous monitoring of bone homeostasis and prompt treatment of osteopenia are warranted in these high-risk infants. This review is to focus on the recent advances in the understanding of bone metabolism in preterm infants and on the therapeutic approach to prevent and to treat metabolic bone disease of prematurity.

초극소저체중 출생아에서 발생한 태변 연관 장폐색증

유하양,신정은,은호선,이순민,박민수,이미정,김명준,남궁란,이철,박국인 대한신생아학회 2013 Neonatal medicine Vol.20 No.1

Purpose: Meconium-related ileus (MRI) is one of the major causes of bowel obstruction in extremely low-birth weight newborn infants (ELBWI). Hyperos-molar water-soluble contrast (HWSC) enemas been recognized to be an effective treatment for MRI. The purpose of this study is to observe clinical findings of MRI accompanied by ELBWI and evaluate the therapeutic efficacy and complications of HWSC enemas. Methods: A total of 15 ELBWI with MRI were treated with HWSC enemas under the guidance of ultrasonography at the bedside in the NICU between 2008 and 2011. Clinical findings of 15 patients were reviewed and compared with those of 48 ELBWI without MRI administered to NICU during the same period. Radiological findings, therapeutic efficacy and complications of HWSC enemas in patients with MRI were also reviewed. Results: Patients with MRI, compared to those without MRI, showed the following significantly lower Apgar score at 1 minute, higher incidence of preeclampsia, bronchopulmonary dysplasia and sepsis, and longer duration of the first meconium passing and non-feeding per oral. Fourteen patients with MRI had resolved bowel obstruction successfully following 1-2 trials of enema. One case was not relieved following 3 trials of enema, showed no clinical improvement, and died of severe intraventricular hemorrhage and multi-organ failure at 45 days old. No complications associated with HWSC enemas were observed in all cases. Conclusion: Administration of HWSC enemas under the guidance of abdomen ultrasonography in the NICU is safe and efficacious for the rapid diagnosis and treatment of MRI even accompanied by ELBWI.

유전자 돌연변이로 확진된 상염색체 열성 다낭신질환 신생아 1례

백재은,이순민,은호선,박민수,박국인,남궁란,이철 대한신생아학회 2014 Neonatal medicine Vol.21 No.1

Autosomal recessive polycystic kidney disease (ARPKD) is a severe form of polycystic kidney disease that is characterized by enlarged kidneys and congenital hepatic fibrosis. The clinical spectrum of this condition shows wide variation. Approximately 30-50% of affected individuals die in the neonatal period, while others survive into adulthood. ARPKD is caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6p12, which consists of 86 exons variably assembled into many alternatively spliced transcripts. We report a case of a pathogenic PKHD1 frameshift mutation, c.889_931del43, which was identified using direct full sequencing, associated with enlarged cystic kidneys and dilatation of intrahepatic bile duct, as observed on imaging studies.

만성폐질환을 가진 미숙아에서 폐동맥고혈압증의 위험인자

라정진,이순민,은호선,박민수,박국인,남궁란,이철 대한신생아학회 2013 Neonatal medicine Vol.20 No.1

Purpose: Pulmonary hypertension (PH) is known as a major risk factor for preterm infants deaths, and associated with low gestational age, low birth weight, longer duration of oxygen therapy, low Apgar scores, and oligohydramnios. The aim of this study was to determine the risk factors for PH in preterm infants with chronic lung disease. Methods: A retrospective review was performed of data from 86 infants (<32 weeks of gestation) who diagnosed with chronic lung disease at Severance Children’s Hospital from July 2006 to June 2011. PH was diagnosed on the basis of echocardiogram demonstrating elevated right ventricle pressure. The infants were divided into two groups: PH group (n=18) or control group (n=67). Results: PH group showed lower gestational age and underwent longer duration of oxygen therapy, mechanical ventilation, and hospitalization compared with control group. PH group received more surfactant therapy, and the frequency of the development of intraventricular hemorrhage (IVH) (≥G3) and severe chronic lung disease was higher in PH group than control group. According to multivariate analysis, IVH (≥G3) (OR=5.00, 95% CI=1.10-22.63, P=0.037) and severe BPD (OR=2.43, 95% CI=1.03-15.41, P=0.045) were found to be significant risk factors for PH in preterm infants with chronic lung disease. Conclusion: Pulmonary hypertension was significantly increased in preterm infants with chronic lung disease when associated with IVH (≥G3). We suggest that effort for early diagnosis and management for PH would be helpful in preterm infants with chronic lung disease when severe IVH was combined.

Usefulness of serum cystatin C to determine the dose of vancomycin in neonate

신정은,이순민,은호선,박민수,박국인,남궁란 대한소아청소년과학회 2015 Clinical and Experimental Pediatrics (CEP) Vol.58 No.11

Purpose: The vancomycin dosage regimen is regularly modified according to the patient’s glomerular filtration rate (GFR). In the present study, we aimed to assess the usefulness of serum cystatin C (Cys-C) concentration, compared with serum creatinine (SCr) concentration, for predicting vancomycin clearance (CLvcm) in neonates. Methods: We retrospectively analyzed the laboratory data of 50 term neonates who were admitted to the neonatal intensive care unit and received intravenous vancomycin, and assessed the pharmacokinetic profiles. Creatinine clearance (CLcr) and GFR based on Cys-C (GFRcys-c) were estimated using the Schwartz and Larsson formulas, respectively. Results: The mean CLvcm (±standard deviation) was 74.52±31.17 L/hr, the volume of distribution of vancomycin was 0.67±0.14 L, and vancomycin half-life was 9.16±17.42 hours. The SCr was 0.46±0.25 mg/dL and serum Cys-C was 1.43±0.34 mg/L. The peak and trough concentrations of vancomycin were 24.65±14.84 and 8.10±5.35 mcg/mL, respectively. The calculated GFR based on serum creatinine concentration (GFR-Cr) and GFRcys-c were 70.2±9.45 and 63.6±30.18 mL/min, respectively. The correlation constant for CLvcm and the reciprocal of Cys-C (0.479, P=0.001) was significantly higher than that for CLvcm and the reciprocal of SCr (0.286, P=0.044). GFRcys-c was strongly correlated with CLvcm (P=0.001), and the correlation constant was significantly higher than that for CLvcm and CLcr (0.496, P=0.001). Linear regression analysis showed that only GFRcys-c was independently and positively correlated with CLvcm (F=41.9, P<0.001). Conclusion: The use of serum Cys-C as a marker of CLvcm could be beneficial for more reliable predictions of serum vancomycin concentrations, particularly in neonates.