소장폐색증 259례에 대한 임상적 고찰

정해일(Hae Il Chung),박성일(Snng Il Park),장선택(Sun Taik Chang) 대한외과학회 1979 Annals of Surgical Treatment and Research Vol.21 No.2

소아 급속 진행성 사구체 신염의 임상-병리학적 고찰

조희연,정대림,강주영,하일수,최용,정해일,Cho Hee-Yeon,Chung Dae-Lim,Kang Ju-Hyung,Ha Il-Soo,Choi Yong,Cheong Hae-Il 대한소아신장학회 2004 Childhood kidney diseases Vol.8 No.2

목적: 급속 진행성 사구체신염은 병리조직학적으로 사구체가 광범위한 반월상을 형성하며 임상적으로는 수개월내에 급성 신부전에 빠지게 되는 신질환이다. 저자들은 이 질환의 임상 경과의 이해와 치료 방침의 결정에 도움을 주고자 소아급속 진행성 사구체 신염 환자들의 임상양상과 병리 소견을 후향적으로 고찰하였다. 방법: 1991년부터 2003년까지 소아과에 내원하여 임상 양상과 신생검 소견을 종합하여 급속 진행성 사구체 신염으로 진단 받고 추적 관찰이 가능하였던 12명을 대상으로 임상양상 및 병리소견에 대해 후향적으로 조사하였다. 신부전으로 진행한 군과 정상 신기능이 유지된 군으로 나누어 임상-병리학적 지표들을 비교하였다. 결과: 12명의 환자 중 남자는 4명, 여자는 8명이었다. 발병 당시 연령은 평균 11세 5개월이었고 경과 관찰 기간은 평균 25개월이었다. 신조직 검사 결과에 따라 분류하면 면역복합체 매개성 사구체 신염인 경우가 10예(83%), 무면역 침착 사구체 신염이 2예(17%)였고 항사구체 기저막 항체 신염인 경우는 없었다. 모든 환자가 경구 스테로이드 투여를 받았고 10명(83.3%)에서 methylprednisolone pulse therapy를 시행하였고 이중 8명(65.7%)은 cyclophosphamide 병합 투여도 시행하였고 4명(33.3%)은 혈장교환을 병행하였다. 경과 관찰하는 동안 1예에서 정상 신기능으로 회복되었고 7예는 정상 신기능은 유지되나 신증후군 범위 이하의 단백뇨가 지속되는 부분 회복을 보였다. 2예에서는 지속되는 단백뇨와 혈청 크레아티닌 상승을 보이는 만성 신부전 상태를 보였고 2예에서는 말기 신질환으로 진행하였다. 진단시 높은 혈청 크레아티닌과 낮은 헤모글로빈 수치를 보인 경우와 나이가 어린 경우 예후가 불량하였다. 결론: 소아에서 급속 진행성 사구체신염은 면역 복합체 신염이 대부분을 차지하고 조기 진단과 적극적인 치료를 시행하였을 때 대부분의 환자에서 신기능의 호전이 관찰되었다. 이 질환의 임상 경과와 치료 방침의 확립을 위하여 다기관의 전향적 연구가 필요하다. Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

20대 직장 및 항문주위 양성질환에 대한 임상적 고찰

김창렬(Chang Yeol Kim),정해일(Hae Il Chung),변종하(Jong Ha Beon) 대한외과학회 1982 Annals of Surgical Treatment and Research Vol.24 No.3

소아에서 발생한 전신성 모세혈관 누출 증후군(Systemic Capillary Leak Syndrome) 1례

이택영,정유미,강희경,하일수,정해일,최용,Lee, Taeg Young,Chung, Yoo Mi,Kang, Hee Gyung,Ha, Il Soo,Cheong, Hae Il,Choi, Yong 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.10

전신성 모세혈관 누출 증후군(Systemic Capillary Leak Syndrome)은 Clarkson 등이 처음 기술한 이래 50여명의 증례가 보고된 매우 드문 질환이며, 소아에서는 1995년 Ivan Foeldvari 등이 보고한 예가 유일하다. 저자들은 빈호흡, 청색증, 부종, 소변량 감소를 주소로 내원한 6세 여아에서 저혈압, 혈액농축, 저알부민혈증의 소견을 확인하고 전신성 모세혈관 누출 증후군으로 진단한 예를 문헌 고찰과 함께 보고하는 바이다. Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34-year-old woman, about 50 cases have been reported in the literature. However, most of the cases were of adult age, and the mean age of onset in the reported cases was 42.6 years. In literature review, we could refer only one pediatric case of SCLC by Foeldvari et al. in 1995. We report another pediatric case of SCLS.

3개월 이하 영아기 열성 요로감염증에 대한 임상적 관찰

은병욱,정유미,강희경,하일수,정해일,이환종,최용,Eun, Byung Wook,Chung, Yoo Mi,Kang, Hee Gyung,Ha, Il Soo,Cheong, Hae Il,Lee, Hoan Jong,Choi, Yong 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.3

목 적 : 3개월 이하 연령의 요로감염증 환자 41례의 임상 양상과 특히 균혈증과 뇌수막염의 동반 양상을 관찰하고자 하였다. 방 법 : 5년 9개월 동안 서울대학교병원 소아과에서 요로감염증을 진단 받고 퇴원한 환자 중에서 연령이 3개월 이하이고 요세균 배양 검사에서 집락형성단위가 $10^5/mL$ 이상이면서 내원 당시 발열과 농뇨가 있었던 예의 의무기록을 분석하였다. 이전에 알려진 비뇨기계 기형이나 면역결핍증이 있거나 원내감염의 경우는 조사대상에서 제외하였다. 결 과 : 남녀 비는 5.8 : 1이었다. 요 세균 배양 검사 결과 41례의 요로감염증 환자에서 40례(97.6%)에서는 단일 균주가, 1례에서는 2개의 균주가 배양되었다. 이 중 E. coli가 34주(83%)로 가장 많았다. 5례(12%)에서 균혈증이 동반되었다. 이중 E. coli가 4주, Enterococcus faecalis가 1주였다. 배양검사 양성의 뇌수막염이 동반된 예는 없었고 뇌척수액의 세포증다증(pleocytosis)도 없었다. 균혈증이 동반된 군과 동반되지 않은 군간에 임상 양상의 차이는 없었다. 방관요관역류의 빈도는 배뇨중 방광요도 조영술을 시행받은 환자의 44%였다. 초음파검사의 방광요관 역류 발견에 대한 민감도는 38%, 특이도는 50%였다. 결 론 : 임상적 특징과 검사 결과는 증상 발현 당시에 균혈증이 동반된 환자를 발견하는데 도움을 주지 못했다. 따라서 모든 3개월 이하 열성 요로감염증 환자에서 혈액배양검사는 필요하다. 열성 요로감염증 소아 환자에서 뇌척수액 검사의 적응증을 밝히기 위한 대규모의 연구를 비롯하여 3개월 이하 연령의 요로감염증 환자군의 진단적 접근과 치료에 대한 연구가 진행되어야 하겠다. Purpose : To characterize the infants under 3 months of age with urinary tract infections(UTIs), and especially patients with bacteremia or meningitis Methods : Hospital records of all the infants under 3 months of age discharged from our hospital for 69 consecutive months with the diagnosis of initial episode of UTI were reviewed. UTI was defined when patients had fever with pyuria, and had urine culture results of ${\geq}10^5$ colony forming units/mL from a bag specimen. Patients with previously known urologic abnormality or immunodeficiency were excluded. Nosocomial infections were also excluded from the study. Results : The male:female ratio was 35 : 6. Of the urine cultures, 40(97.6%) yielded single pathogen, one yielded two pathogens. Escherichia coli was the predominant isolate from the urine. Five patients(12%) also had bacteremia. Pathogens isolated from the blood cultures were E. coli(4) and Enterococcus faecalis(1). No patient had culture-positive meningitis or cerebrospinal fluid pleocytosis. Clinical or laboratory findings between patients with and without bacteremia were not different significantly. The rate of vesicoureteral reflux(VUR) was 44%. The sensitivity of ultrasound for detection of VUR was 38%; specificity was 50%. Conclusion : Clinical and laboratory data were not helpful for identifying patients with bacteremia at the time of presentation. Consequently, blood cultures need to be obtained from all febrile infants under 3 months of age with UTIs. A large-scale study including the indication of lumbar puncture for infants with a febrile UTI and study of evaluation and treatment of infants under 3 months of age with UTIs are required.

학교 집단 소변 검사로 발견 된 막증식성 사구체신염 I형의 특성

최정연,박미영,이용직,하일수,정해일,최용,박영서,한혜원,진동규,정우영,김기혁,유기환,박용훈,Choi, Jung-Youn,Park, Mi-Young,Lee, Yong-Jik,Ha, Il-Soo,Cheong, Hae-Il,Choi, Yong,Park, Young-Seo,Han, Hye-Won,Jin, Dong-Kyu,Chung, Woo-Yeong,Kim, Ke 대한소아신장학회 2006 Childhood kidney diseases Vol.10 No.2

Purpose : In Korea, the school urine screening program is a useful tool for screening urine abnormalities. It is particularly useful in early detection of membranoproliferative glomerulonephritis(MPGN) I, which frequently progresses to chronic renal failure. In this study, we studied the medical history, laboratory findings, and histologic findings of MPGN to gain helpful information on early detection and treatment. Methods : The subjects were 19 children, who were diagnosed with MPGN from kidney biopsies that were performed in ten nationwide university hospitals because of abnormal urine findings from school urine screening programs conducted from July 1999 to April 2004. We divided the patients into 2 groups, a nephrotic range proteinuria group(n=8) and a non-nephrotic proteinuria group(n=11), and retrospectively analyzed the clinical features, laboratory findings, histologic findings, treatment, and clinical course. Results : The mean age at the first abnormal urinalysis was $10.6{\pm}2.2$ years in the nephrotic proteinuria group and $9.6{\pm}3.2$ years in the non-nephrotic proteinuria group. The mean age at the time of kidney biopsy was $11.3{\pm}2.3$ years in the nephrotic range proteinuria group and $10.4{\pm}3.2$ years in the non-nephrotic proteinuria group respectively. There was no significant difference in the mean age and sex between the two groups. In the nephrotic proteinuria group, 6 children had a low plasma C3 level and in the non-nephrotic proteinuria group, 8 children had a low plasma C3 level, but there was no significant difference between the 2 groups. There was no significant difference in the laboratory test results(including WBC count, RBC count, platelet count and other serologic tests) between the 2 groups except for 24 hour urine protein secretion. There was no difference between the 2 groups with regard to the acute and chronic changes in the glomerulus on light microscopic findings, IgG, IgA, Ig M, C1q, C3, C4, fibrogen deposition on immunofluoroscence findings, and mesangial deposits, subendothelial deposits, and subepithelial deposits on electron microscopic findings. The children were treated with corticosteroids, ACE(angiotensin-converting enzyme) inhibitors, dipyridamole and other immunosuppressive agents. During the course of treatment, there were no children whose clinical condition worsened. Among 19 children, 3 children went into remission(2 in the nephrotic proteinuria group, 1 in the non-nephrotic proteinuria group) and 9 children went into a partial remission(4 in the nephrotic proteinuria group, 5 in the non-nephrotic proteinuria group) on urinalysis. There was no significant difference in the treatment results between the two groups. Conclusion : The 73.7% of children who were incidentally diagnosed with MPGN by the school urine screening program had reduced C3. 42.1% of the children had nephrotic range proteinuria. There were no significant differences in clinical features, laboratory test results, light microscopic, immunofluorescence microscopic, and electron microscopic findings between the nephrotic proteinuria group and the non-nephrotic proteinuria group except for the 24 hour urine protein secretion. Therefore, for early detection of MPGN during the school urine screening program, we strongly recommend a kidney biopsy if children have abnormal urine findings such as persistent proteinuria and persistent hematuria, or if the serum C3 is reduced.

소아 연령에서 발견된 medullary sponge kidney

정우영,조민현,구영란,임선희,정해일,Chung, Woo Yeong,Cho, Min Hyun,Gu, Young-Ran,Leem, Sun-Hee,Cheong, Hae Il 대한소아신장학회 2012 Childhood kidney diseases Vol.16 No.2

Medullary sponge kidney disease (MSK)는 신장 수질 피라미드부위에서 야기되는 희귀질환으로 collecting precalyceal duct의 낭종성 확장(dilatation)과 ectasia을 특징으로 한다. MSK 환자의 발생 빈도에 대해서는 명확히 알려진 바가 없으며, 특히 소아 청소년 연령에서는 매우 드물게 발견된다. 연구자들은 국내 소아신장학회 회원들을 대상으로 MSK 환자의 전수 조사를 실시하였고 현재까지 문헌상으로 보고된 관련 유전자들인 GDNF, ATP6V1B1, ATP6V0A4 유전자에 대한 분석을 실시하였기에 이를 보고하는 바이다. Purpose: Medullary sponge kidney (MSK) is a rare congenital disease characterized by diffuse ectasia or dilatation of precalyceal collecting tubules. MSK incidence and prevalence in the general population is uncertain and only a few patients are reported especially in the pediatric age. There has been increasing reports of patients with MSK who have other malformative disorders. Also several case reports concerning about etiological association of some genes. Methods: Collaborative study through nation-wide survey was done to investigate the incidence and etiological association of some genes such as GDNF gene, ATP6V1B1, ATP6V0A4 gene in developing MSK in Korean children. Results: Four cases of MSK who have various other malformative disorders were collected. There are no mutations of GDNF gene, ATP6V1B1, ATP6V0A4 gene in all patients. Conclusion: MSK is one of the very rare diseases in pediatric age. The etiological association of GDNF gene, ATP6V1B1, ATP6V0A4 gene in developing MSK in Korean children is not proved.