영아기 신증후군의 임상적 고찰

조성윤,이상훈,박성근,양문호,고영태,조병수,차성호 대한신장학회 1996 Kidney Research and Clinical Practice Vol.15 No.1

We performed clinical observation on infantile nephrotic syndrome in 9 cases which were managed in the department of pediatrics at Kyung Hee University Hospital from Jan. 1980 to Jan. 1995. The results were as follows: 1) The mean age of infantile nephrotic syndrome were 5.9 months, and male to female ratio was 1.25:1. 2) The etiologic classification was Finnish type in 2, French type in 1, congenital syphilitis in 2, renal vein thrombosis(RVT) in 1 and MCNS in 3 cases. 3) Among 9 cases, 8 showed microscopic hematuria. 4) As for the effect of treatment and prognosis, all 2 Finnish type and one French type patients who were treated with supportive therapy died at 2, 10, and 16 months, respectively. And all others were recovered by proper treatment with penicillin, dipyridamole, and prednisolone, respectively. In conclusion, correct and early diagnosis is very important for the proper therapy and better prognosis because congenital nepnrotic syndrome developed before the first 6 months of life had a poor prognosis except the secondary ones. And infantile nephrotic syndrome after 6 months of life, except French type, was steroid sensitive minial change nephrotic syndrome, in our cases.

Impact of Enzyme Replacement Therapy on Linear Growth in Korean Patients with Mucopolysaccharidosis Type II (Hunter Syndrome)

조성윤,허림,장미선,이지은,Younghee Kwun,Se-Hyun Maeng,김수진,손영배,박성원,권은경,Sun Ju Han,정주연,진동규 대한의학회 2014 Journal of Korean medical science Vol.29 No.2

Hunter syndrome (or mucopolysaccharidosis type II [MPS II]) arises because of a deficiency in the lysosomal enzyme iduronate-2-sulfatase. Short stature is a prominent and consistent feature in MPS II. Enzyme replacement therapy (ERT) with idursulfase (Elaprase®) or idursulfase beta (Hunterase®) have been developed for these patients. The effect of ERT on the growth of Korean patients with Hunter syndrome was evaluated at a single center. This study comprised 32 patients, who had received ERT for at least 2 yr; they were divided into three groups according to their ages at the start of ERT: group 1 (<6 yr, n=14), group 2 (6-10 yr, n=11), and group 3 (10-20 yr, n=7). The patients showed marked growth retardation as they got older. ERT may have less effect on the growth of patients with the severe form of Hunter syndrome. The height z-scores in groups 2 and 3 revealed a significant change (the estimated slopes before and after the treatment were -0.047 and -0.007, respectively: difference in the slope, 0.04; P<0.001). Growth in response to ERT could be an important treatment outcome or an endpoint for future studies.

전통사회의 변화와 사상 : 19세기 서울의 상비군 제도와 하급군병

조성윤 연세대학교 사회발전연구소 1990 연세사회학 Vol.10 No.-

약결합 방식의 INS/UWB 실내항법장치를 위한 MCC 기반 UKF

조성윤,이재홍,박찬국 제어로봇시스템학회 2022 제어로봇시스템학회 국내학술대회 논문집 Vol.2022 No.6

18 P- 증후군 1례

조성윤,배종우,오보훈 대한산부인과학회 1991 대한산부인과학회 학술대회 Vol.67 No.-

Pseudomonas fluorescens Malate Synthase 의 Acetyl - CoA 결합부위에 있는 필수 Cysteine 잔기에 대한 입증

조성윤,김유삼 ( Sung Yoon Cho,Yu Sam Kim ) 생화학분자생물학회 1991 BMB Reports Vol.24 No.2

Malate synthase from Pseudomonas fluorescens was inhibited by iodoacetamide and N-ethylmaleimide with second-order rate constant of 43.9 M^(-1) and of 1,784 M^(-1), respectively. A double logarithmic plot of the reciprocal of the half-time of inactivation against concentration yield reaction order of 1.275 and 0.94 with respect to iodoacetamide and N-ethylmaleimide, respectively, indicating that there may be one essential residue per active enzyme. The enzyme was also rapidly inhibited by 5,5`-dithiobis (2-nitro-benzoic acid) and by 2-nitro-5-(thiocyanato)benzoate. pK_a of the functional group was determined to be 8.3 by using iodoacetamide. The inactivation by iodoacetamide and by N-ethylmaleimide was protected by acetyl-CoA but not by glyoxylate. The total number of thiol groups in the enzyme was determined to be five. These results strongly suggest that there is one essential group at the acetyl-CoA binding region among five thiol groups of the malate synthase.

스마트 항로표지 정보 수집 및 정보협력시스템 간의 정보 연계 방안

조성윤,성주형,김양섭,권기원 한국항해항만학회 2023 한국항해항만학회 학술대회논문집 Vol.2023 No.1

본 논문은 해상 항로 표지 내 탑재되는 다양한 해양 센서, 등명기, 전원 모듈을 분리하여 각각의 서브 플랫폼으로 관리하고 각각의서브 플랫폼들은 통합적으로 운영하는 메인 플랫폼 구조를 갖는 계층적 형태의 스마트 항로표지 구조안을 제시한다. 제안된 구조안을 바탕으로 기존에 제공되는 다양한 장비 및 센서 뿐만 아니라 항로표지 신 서비스 요구사항에 따른 신규 센서의 확장 및 설치가 용이하며, 서브 플랫폼 별 독립적인 운영을 통한 전체 시스템의 안정성 향상 기대할 수 있다. 또한, 목적에 따른 숫자, 음성, 영상 등 다양한 형태의 데이터를 전송하기 위한 선택적 통신 플랫폼 구조를 제시함으로써 육상 정보협력시스템과의 원활한 정보 교환 체계를 구축할 수 있는 방안을 제안한다.

서울의 한 대학병원에서 경험한 소아의 세균성 수막염: 1993-2006

조성윤,김태연,이현주,유은선,김혜순,박은애,유경하,손세정,서정완,이승주,김경효 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.10

Purpose:Despite the seriousness of bacterial meningitis in children, there is little information on the incidence, causative organisms, mortality rate and age distribution. We studied the frequency by age group and causal pathogens, and clinical characteristics in children with bacterial meningitis in the private sector in Korea. Methods:The medical records containing the data on bacterial meningitis patients under 18 years of age confirmed by cerebrospinal fluid (CSF) findings were retrospectively analyzed from September, 1993 to August, 2006 at Ewha Womans University Mokdong Hospital. Results:Eighty-one cases of bacterial meningitis were observed. Overall the most common organism was Streptococcus agalactiae (group B streptococcus, GBS) (30 cases, 37.0%) followed by Haemophilus influenzae (22 cases, 27.2%), Streptococcus pneumoniae (12 cases, 14.8%), Escherichia coli (3 cases, 3.7%), Neisseria meningitidis (1 case, 1.2%) and others (13 cases, 16.0%). In neonates and young infants under 2 months, the most common organism was GBS. In children between 3 months, and 5 years, the most common organism was H. influenzae. S. pneumoniae was the most common organism in children over 5 years of age. Thirty-one patients (38.3%) had complications. Of all ages, the mortality rate of bacterial meningitis markedly decreased compared with the previously reported rate. Conclusion:In neonates, GBS meningitis was most common. The frequency of H. influenzae meningitis decreased after the introduction of H. influenzae type b vaccination. A strategy for the prevention of GBS meningitis in neonates should be established. The influence of the pneumococcal conjugate vaccine on S. pneumoniae meningitis should be studied. (Korean J Pediatr 2008;51:1077-1084) Purpose:Despite the seriousness of bacterial meningitis in children, there is little information on the incidence, causative organisms, mortality rate and age distribution. We studied the frequency by age group and causal pathogens, and clinical characteristics in children with bacterial meningitis in the private sector in Korea. Methods:The medical records containing the data on bacterial meningitis patients under 18 years of age confirmed by cerebrospinal fluid (CSF) findings were retrospectively analyzed from September, 1993 to August, 2006 at Ewha Womans University Mokdong Hospital. Results:Eighty-one cases of bacterial meningitis were observed. Overall the most common organism was Streptococcus agalactiae (group B streptococcus, GBS) (30 cases, 37.0%) followed by Haemophilus influenzae (22 cases, 27.2%), Streptococcus pneumoniae (12 cases, 14.8%), Escherichia coli (3 cases, 3.7%), Neisseria meningitidis (1 case, 1.2%) and others (13 cases, 16.0%). In neonates and young infants under 2 months, the most common organism was GBS. In children between 3 months, and 5 years, the most common organism was H. influenzae. S. pneumoniae was the most common organism in children over 5 years of age. Thirty-one patients (38.3%) had complications. Of all ages, the mortality rate of bacterial meningitis markedly decreased compared with the previously reported rate. Conclusion:In neonates, GBS meningitis was most common. The frequency of H. influenzae meningitis decreased after the introduction of H. influenzae type b vaccination. A strategy for the prevention of GBS meningitis in neonates should be established. The influence of the pneumococcal conjugate vaccine on S. pneumoniae meningitis should be studied. (Korean J Pediatr 2008;51:1077-1084)

Osteogenesis Imperfecta Type VI with Severe Bony Deformities Caused by Novel Compound Heterozygous Mutations in SERPINF1

조성윤,기창석,손영배,김수진,맹세현,진동규 대한의학회 2013 Journal of Korean medical science Vol.28 No.7

Osteogenesis imperfecta (OI) comprises a heterogeneous group of disorders characterized by bone fragility, frequent fractures, and low bone mass. Dominantly inherited COL1A1 or COL1A2 mutations appear to be causative in the majority of OI types, but rare recessively inherited genes have also been reported. Recently, SERPINF1 has been reported as another causative gene in OI type VI. To date, only eight SERPINF1 mutations have been reported and all are homozygous. Our patient showed no abnormalities at birth, frequent fractures,osteopenia, and poor response on pamidronate therapy. At the time of her most recent evaluation, she was 8 yr old, and could not walk independently due to frequent lowerextremity fractures, resulting in severe deformity. No clinical signs were seen of hearing impairment, blue sclera, or dentinogenesis imperfecta. In this study, we describe the clinical and radiological findings of one Korean patient with novel compound heterozygous mutations (c.77dupC and c.421dupC) of SERPINF1.

양발 PDR 의 융합을 통한 소방관용 안정적인 실내 위치추정 기술

조성윤,이재홍,박찬국 제어로봇시스템학회 2020 제어로봇시스템학회 국내학술대회 논문집 Vol.2020 No.7