• Childhood Kidney Diseases
• /
• 제18권2호
• /
• pp.57-63
• /
• 2014

소아들의 여러 신장 질환 특히 만성신장병은 초기에는 뚜렷한 증세나 징후를 나타내지 않고 단독 단백뇨 또는 현미경적 혈뇨로만 나타나는 경우가 많다. 이런 신장 질환을 발견하기 위하여 소변검사는 여러 장단점에도 불구하고 간단하고 비용이 적게 드는 검사로서 선별검사에 이용하게 되었다. 우리나라에서는 신장 질환의 조기 발견을 위한 학교 집단소변검사가 1998년에 개정된 학교신체검사규칙에 근거하여 전체 초 중 고등학생을 대상으로 실시되었다. 그 이후에 많은 연구와 보고들이 학교 집단소변검사가 만성신질환의 조기 발견에 유용할 것이라고 하였지만 아직도 여기에 대한 이견이 있을 뿐만 아니라 비용 효과 등에 대하여서도 충분한 의견 일치를 얻지 못하였기에 코호트 연구 등의 장기적인 관찰적 역학 연구가 반드시 필요하다. 이를 위하여서는 신장학자들의 지속적인 연구뿐만 아니라 제도 개선 및 개발들이 반드시 동반되어야 할 것이다.

• Journal of Preventive Medicine and Public Health
• /
• 제28권1호
• /
• pp.225-243
• /
• 1995

Benzidine Industry in Korea has started after Japan has banned its production in early 1970's, and it has been in operation in Korea for over 20 years. However, it is not known yet whether any bladder cancer has developed from benzidine exposure. This study was done to screen benzidine-exposed workers for bladder cancer, and to examine the feasibility of employing screening test at the workplace. All the workplaces that manufacture or use benzidine for more than 20 years in Korea have been covered in this study, and they include 2 benzidine manufacturing factories, 5 benzidine using factories, as well as 2 benzidine free factories as an outside control. In total, 516 workers were screened with urine stick test and urine cytology test for the evidence of hematuria and abnormal urothelial cells. Each worker was also asked about risk factors and symptoms of bladder cancer including past medical history, smoking, medication and occupational history Benzidine in the air was measured by personal and area sampling. Out of 516 screened workers, 84(16.3%) workers showed positive hematuria in urine stick test, and 7(1.4%) workers showed degenerative cells in urine cytology tests. Those workers with abnormal urine test results who have been exposed to benzidine fo more than 10 years were further screened, and, in total, 23 workers were examined with intra-venous pyelography and cystoscopy. None of those screened had any evidence of bladder cancer When workers with only past hematuria history were included in the positive hematuria group, 96(18.5%) had positive hematuria. On the multiple logistic regression analysis, positive hematuria was significantly associated with benzidine exposure, history of other occupations with elevated bladder cancer risk, pyuria and glycosuria. The association got stronger as direct benzidine exposure was accounted through individual task analysis, and as exposure duration was accounted with tenure analysis. For those with benzidine exposure with more than 10 years of tenure, the odds of having positive hematuria was elevated 2.14(95%C.I is 1.08 to 4.25) times more than for those without exposure. Even though bladder cancer was not detected for several limitations including short observation period, majority of studied workers with short latency, healthy worker effect, and low sensitivity of single screening test in a cross-sectional study, the study results suggest that hematuria screening is a feasible and very useful test for bladder cancer screening among benzidine exposed workers.

• Childhood Kidney Diseases
• /
• 제10권2호
• /
• pp.152-161
• /
• 2006

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.

• Childhood Kidney Diseases
• /
• 제21권2호
• /
• pp.53-60
• /
• 2017

Proteinuria is common in pediatric and adolescent patients. Proteinuria is defined as urinary protein excretion at levels higher than $100-150mg/m^2/day$ in children. It can be indicative of normal or benign conditions as well as numerous types of severe underlying renal or systemic disease. The school urine screening program has been conducted in Korea since 1998. Since then, numerous patients with normal or benign proteinuria as well as early stage renal diseases have been referred to the hospital. Benign proteinuria includes orthostatic proteinuria and transient proteinuria. Most causes of proteinuria can be categorized into 3 types: 1) overflow, 2) tubular, and 3) glomerular. Although treatment should be directed at the underlying cause of the proteinuria, prompt evaluation, diagnosis, and long-term monitoring of these pediatric patients can prevent potential progression of the underlying disease process. This article provides an overview of proteinuria: its causes, methods of assessment, and algorithmic suggestions to differentiate benign from pathologic renal disease.

• Childhood Kidney Diseases
• /
• 제24권2호
• /
• pp.131-137
• /
• 2020

Nephrogenic diabetes insipidus (DI) is a rare disease in which the patient cannot concentrate urine despite appropriate or high secretion of antidiuretic hormone. Congenital nephrogenic DI is caused by the arginine vasopressin receptor 2 (AVPR2) or aquaporin 2 (AQP2) gene mutation; the AVPR2 genetic mutation accounts for 90% of the cases. National health screening for infants and children was launched in 2007 in order to prevent accidents and promote public health in infants and children in Korea. The program has been widely used as a primary clinical service in Korea. We treated an infant with faltering growth and delayed development detected by the National health screening program, and diagnosed the problem as nephrogenic DI caused by a rare missense mutation of c.490T>C on the AVPR2 gene. This case can be a good educational nephrogenic DI with a rare AVPR2 mutation, which was well screened and traced by the national health screening program for infants and children in Korea.

• 대한유전성대사질환학회지
• /
• 제23권2호
• /
• pp.31-38
• /
• 2023

Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder caused by a deficiency in branched chain α-keto acid dehydrogenase (BCKAD). Between 1997, when Korea's MSUD case was first reported, and 2023, 14 cases were reported in the literature. 29% of the cases experienced developmental delay, and 29% expired. The prevalence of MSUD in Korea was estimated to be 1 in 230,000. Of 21 MSUD patients currently being treated at the Korea Genetics Research Center, 19 were detected through newborn screening program, and 2 were diagnosed by the symptoms. 14 MSUD patients had confirmed genetic mutations; 6 (43%) were BCKDHA and 8 (57%) were BCKDHB. In one case, a large deletion was observed. 4 patients had leucine levels above 2,000 (umo/L), and post-dialysis diet therapy was initiated in the newborn period. No patient required further dialysis as diet therapy and regular monitoring proved highly effective. Most MSUD patients were growing normally; weight and height growth were above the 50th percentile in 76% of the cases while BMI values were higher than normal in 71% of cases. Developmental delays were observed only in 2 cases (10%) and anticonvulsant use in 3 cases (14%). With newborn screening available to all Korean infants, early diagnosis and intervention should allow most patients to remain asymptomatic. However, ongoing surveillance, dietary management and continued patient compliance as well as rapid correction of acute metabolic decompensations remain critical to a favorable long-term prognosis.

• Childhood Kidney Diseases
• /
• 제7권2호
• /
• pp.142-149
• /
• 2003

목적 : 초, 중, 고등학생들을 대상으로 실시한 집단뇨 검사의 전반적인 분석에 대한 연구 결과들은 여러 연구자들에 의해 지속적으로 보고되었으나, 단독 단백뇨(isolated proteinuria)에 대한 연구는 소수에 불과하다. 국내에서는 매년 전체학생의 0.3-0.5% 정도를 차지하는 것으로 보고되어 있다. 단백뇨는 과도한 양의 단백이 소변으로 배출되는 것으로, 증상이 없는 환자에서 발견된 단백뇨는 심각한 신장질환이나 전신 질환의 초기 발현이거나 혹은 임상적으로 의미 없는 일시적인 소견일 수 있다. 그러나 지속적인 단백뇨의 경우는 향후 진행성 신질환의 경과를 취하는 경우가 적지 않으므로 진단 당시 정확한 검사 및 지속적인 외래 추적 관찰이 필수적이다. 이에 저자들은 부산 경남지방에 거주하는 초, 중, 고등학교 학생들 중학교 신체검사에서 우연히 발견된 단독 단백뇨를 주소로 본 원을 방문하였던 학생들을 대상으로, 단독 단백뇨의 원인을 규명하고, 전국적인 자료 구축의 기본적인 데이터를 제공하고자 본 연구를 시행하였다. 방법 : 2002년 4월부터 2003년 8월까지 학교 신체 검사에서 발견된 소변 검사상의 이상 소견을 주소로 부산백병원 소아과를 방문하였던 학생 중 단독 단백뇨의 소견을 보였던 초, 중, 고등학생 44명을 대상으로 전향적으로 조사하였다. 성별은 남자가 15명, 여자가 29명으로 남녀 비는 1:1.93이었다. 연령은 7세부터 16.9세까지로 평균 연령은 12.0세였다. 모든 학생들은 소아신장학회의 프로토콜에 의거하여 검사를 시행하였다. 결과 : 단독 단백뇨를 보였던 44명 학생들에서 단백뇨의 원인적 분석 결과는 일시적 단백뇨가 4례(9.1%), 기립성 단백뇨가 36례(81.8%), 지속성 단백뇨가 4례(9.1%)로, 기립성 단백뇨가 가장 많았다. 본원에 의뢰된 학생들의 학교 신체검사상에서의 단백뇨 정성검사(urine dipstick test) 결과를 보면, 전체적으로 2+가 24례(54.5%)로 가장 많았으며, Trace와 3+가 각각 6례, 1+ 5례, 4+ 3례였다. 기립성 단백뇨 군에서는 2+가 21례(58.3%)로 가장 많았다. 24시간 채집뇨상의 총단백량은 일시적 단백뇨 군에서는 평균 $121.0{\pm}136.4\;mg$이었고, 기립성 단백뇨 군은 평균 $179.1{\pm}130.0\;mg$, 지속성 단백뇨 군은 $1,532.8{\pm}982.5\;mg$이었다. 기립성 단백뇨 군에서는 24시간 채집뇨상의 총단백량의 범위가 40 mg에서 616 mg까지로 비교적 넓은 범위를 나타내었다. 단회뇨를 이용하여 측정한 PCR은 일시적 단백뇨군에서는 평균 $0.10{\pm}0.01$이었고, 기립성 단백뇨군은 평균 $0.61{\pm}0.61$, 지속성 단백뇨 군은 평균 $4.35{\pm}4.04$이었다. 기립성 단백뇨 군에서는 PCR의 범위가 0.09에서 2.32까지의 편차를 나타내었다. 지속성 단백뇨를 보이는 경우가 4례에서 관찰되었다. 이들 모두는 1년 동안 계속하여 단백뇨가 나타났으며, 결국 신조직 검사를 시행하였다. 1례는 미세병변이었고, 나머지 2례는 막증식성 사구체 신염이였으며, 나머지 1례는 신유전분증이었다. 결론 : 단백뇨의 검출은 신장질환의 존재를 확인해 줄 뿐만이 아니라 지속적인 단백뇨는 진행성 경과의 위험성을 동시에 암시해 주는 매우 유용한 지표이다. 본 연구 결과 학교 신체 검사상에서 발견된 단독 단백뇨의 주된 원인은 기립성 단백뇨로 81.8%를 차지하였다. 그러나 지속성 단백뇨의 경우 비록 9.1%의 빈도를 보였지만, 신장 조직검사를 시행한 결과 진행성 경과를 취할 수 있는 막 증식성 사구체 신염과 매우 희귀한 증례인 신유전분증 등으로 진단됨으로써 지속성 단백뇨의 경우 정확 진단적 접근이 필수적임을 알 수 있다. 기립성 단백뇨의 경우는 간단한 소변 검사만으로도 진단이 가능하므로 필요하지 않은 검사의 무분별한 시행이나 신장 질환에 대한 막연한 불안감을 해소할 수 있다. 초, 중, 고등학생을 대상으로 시행하고 있는 집단 소변 검사는 예방적인 차원에서의 의료 관리의 전형적인 사례로 볼 수 있다. 이의 성공적이면서도 확고한 정착을 위해서는 각 지역을 중심으로 시행되어진 자료의 수집과 관리를 일원화하는 작업이 요구되고 있다. 이를 통해 얻어진 자료들의 통계적 분석과 다양한 예시들은 소아 신질환의 관리의 귀중한 이정표를 제시해 줄 것이다.

• Childhood Kidney Diseases
• /
• 제1권2호
• /
• pp.189-194
• /
• 1997

Oligomeganephronia is a rare congenital form of bilateral renal hypoplasia histologically characterized by reduction in number and hypertrophy of nephrons. Clinically, this condition is presented in early infancy with vomiting, polyuria, polydipsia and dehydration. The problems are readily corrected, but slowly progressive renal failure follows accompanied by failure to thrive, short stature, and renal osteodystrophy. We experienced three cases of oligomeganephronia. Case 1. : A 3 2/12 years old female child was incidentally diagnosed as renal failure at age of 2 months when she was hospitalized due to pneumonia. She had open renal biopsy and was diagnosed as bilateral dysplastic kidney. On OPD follow-up, she progressed to end-stage renal failure (BUN/Cr 114/4.6 mg/dl) and had renal transplantation. The specimen was shrunk remarkably and light microscopy showed oligomeganephronia. Case 2. : A 14 8/12 years old female child with proteinuria was detected in an annual urine screening program for school children, she was diagnosed as renal failure (BUN/Cr 33.9/4.1 mg/dl), and had $5{\times}4{\times}3\;cm$ sized mass on abdominal CT scan. She had renal biopsy, and the specimen showed oligomeganephronia. She had hemodialysis for six months, and renal transplantation along with bilateral nephrectomy was performed. Case 3. : A 14 8/12 years old male child was diagnosed having chronic nephritis and chronic renal failure at 3 years old, progressed to end-stage renal failure (BUN/Cr 87/9.6 mg/dl) on OPD follow-up, and had a rephrectomy and renal transplantation. The biopsy specimen showed oligomeganephronic hypoplasia, secondary focal segmental glomerolosclerosis, and chronic interstitial nephritis. We report 3 cases of oligomeganephronia that progressed to end-stage renal failure and had successful renal transplantation with a brief review of related literatures.

• 한국산업보건학회지
• /
• 제31권3호
• /
• pp.213-225
• /
• 2021

Objectives: This study identifies the trends in possible and probable occupational disease among chromium workers and reviews the literature on domestic and foreign chromium workers to review the industries, biological exposure levels, and major results. Methods: The results of the Ministry of Employment and Labor's special health-screening program for hazard agents among workers from 2009 to 2019 were used. Also, the industries, biological exposure levels, and major results on chromium workers were reviewed using PubMed and RISS. Results: The average annual proportion of both possible and probable occupational disease for chromium workers has recently increased. The average annual proportion of possible and probable occupational disease that can occur was high for both men and women in their 60s or older by age and 10 to 14 years by work duration. By industry, possible occupational disease showed high in manufacturing. In the literature review, many electroplating-related chromium-workers reported high levels of exposure to blood and urine chromium, as did dental technicians; tannery, tile factory or glass mill workers; cement workers; and sodium bichromate workers. Furthermore, a number of main findings in recent studies have reported that chromium exposure is related to genetic toxicity among workers. Conclusions: In this study, the average annual rate of both possible and probable occupational disease in domestic chromium workers is increasing, and a body of literature shows that chromium exposure is related to genetic toxicity and associated indicators among workers, which requires more systematic study.