• Childhood Kidney Diseases
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• v.6 no.2
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• pp.243-250
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• 2002

Amyloidosis comprises a diverse group of systemic and local diseases characterized by organ involvement by the extracellular deposition of fibrils composed of subunits of a variety of normal serum proteins. Secondary amyloidosis is caused by the deposition of amyloid A(AA) protein in chronic inflammatory disease. Juvenile rheumatoid arthritis(JRA) has been known to be the most common cause of secondary amyloidosis. We experienced one case of secondary renal amyloidosis in a 12-year-old girl who had suffered from JRA for several years who had visited our renal clinic to evaluate the proteinuria with microscopic hematuria which was detected by chance at school urine screening examination. Apple green birefringence was observed under polarized light with Congo red stain at)d characteristic electron microscopic findings was also noted in renal tissues which was obtained by percutaneous renal biopsy. In our knowledge, this is the first case report of secondary renal amyloidosis developed in pediatric age in Korea.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.49-56
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• 2013

Purpose: IgA nephropathy (IgAN) is one of the major causes of end-stage renal disease. Mass school urine screening (SUS) has been performed to enable early detection of chronic renal diseases, including IgAN. We wanted to evaluate the patients with IgAN, including those diagnosed through SUS. Methods: Between 1998 and 2010, 64 children were diagnosed with IgAN based on renal biopsy results obtained at the Pediatric Nephrology Department, ${\bigcirc\bigcirc}$ University Hospital. We divided these patients into the SUS group (37 cases), diagnosed through SUS, and the symptomatic (Sx) group (27 cases), diagnosed clinically. The medical records of both groups were analyzed retrospectively. Results: The mean age of the SUS and Sx groups was $10.8{\pm}2.7$ and $9.5{\pm}3.4$ years (P >0.05), respectively. Both groups had a higher proportion of male patients. The time from the notification of an abnormal urinary finding to a hospital visit or renal biopsy was shorter in the Sx group than in the SUS group. Regarding clinical manifestations, there were fewer cases with gross hematuria (P <0.001) and edema (P =0.008) in the SUS group, but there were no differences in terms of the therapeutic regimen and treatment duration. Regarding laboratory parameters, the Sx group had a higher white blood cell count (P =0.007) and lower hemoglobin (P =0.007) and albumin (P =0.000) levels. There were no differences in the renal biopsy findings in both groups, based on the history of gross hematuria or the severity of proteinuria. However, in all 64 patients with IgAN, the light microscopy findings (Hass classification) were related to a history of gross hematuria or the severity of proteinuria. Conclusion: There were no significant clinical and histological differences between the groups, as both had early stage IgAN. Although SUS facilitates the early detection of IgAN, long-term, large-scale prospective controlled studies are needed to assess the benefits of early diagnosis and treatment in chronic renal disease progression.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.186-194
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• 2004

Purpose: Membranoproliferative glomeulonephritis(MPGN) has been diagnosed in an increasing number of asymptomatic cases. These cases have been detected by school urinary screening test even though the total cases of MPGN show a decreasing trend. We have analyzed the clinical and pathological characteristics of children with MPGN according to the clinical manifestations at the time of disease presentation. Methods: A total of 18 patients who had been diagnosed with idiopathic MPGN by percutaneous renal biopsy from January 1990 to February 2004 were involved in our study. The patients were divided into 2 groups as the school urinary screening(A) group and the symptomatic(S) group according to the clinical manifestations at the time of disease presentation. Results: Out of the total 18 patients, 8(44.4%) were in the S group and 10(55.6%) were in the A group. The mean serum total protein, albumin and $C_3$ levels in the S group were significantly lower than those levels of the A group, respectively($4.9{\pm}1.2\;g/dL,\;vs\;7.0{\pm}0.5\;g/dL\;P=0.002,\;2.8{\pm}0.9\;g/dL\;vs.\;4.1{\pm}0.3\;g/dL\;P=0.002,\;63.9{\pm}36.4\;mg/dL\;vs.\;100.8{\pm}39.5\;g/dL\;P=0.041$). The mean total protein amount of 24 hour collected urine in the S group were significantly higher than that of the A group($3684.0{\pm}2601.3\;mg/m^2\;vs.\;559.4{\pm}4.6.9\;mg/m^2$, respectively, P=0.001). Hypocomplementemia was observed in 11(61.1%) out of 18 patients at the time of disease onset, 7(87.5%) in the S group and 4(40%) in the A group. However the hypocomplementemia was decreased in 6(33.3%) out of 18 patients at the time of final follow-up, 3(37.5%) in the S group and 3(30%) in the A group. According to the pathologic type, hypocomplementemia was observed 8 patients(61.5%) with type I disease, 1 patients (100%) with type II disease, 2 patients(50%) in type III disease at the disease onset, but 4 patients(30.8%) in type I disease, 1 patient(100%) in type II disease, 1 patient(33.3%) with type III disease at the time of last follow-up. The incidence of cellular crescent formation and tubular atropy. as observed on light microscopy, were higher in the S group compared to the A group. Mean grade of capillary wall thickening and, mesangial proliferation were significantly higher in the S group. Conclusion: MPGN, as diagnosed in patients with only asymptomatic urinary abnormalities, has been increasing, it is more frequent in asymptomatic patients than in patients with presenting symptoms. Our result suggests that MPGN should be considered in the renal biopsy diagnosis regardless of serum $C_3$ level when urinary abnormalities are found by school urinary screening test.

• Journal of Physiology & Pathology in Korean Medicine
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• v.17 no.1
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• pp.118-122
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• 2003

Oxidative stress and its consequent lipid peroxidation exert harmful effects, which have been currently involved in the generation of carbon tetrachloride (CCl₄)-induced fibrosis(cirrhosis). In this study, it was investigated whether dried extract of 田螺(Concha Cipangopaludinae; CC) has liver functional improvement, antioxidative and antifibrotic effect in rats those were induced liver fibrosis by CCl₄ administration. The female Sprague-Dawley rats were divided into 3 groups(Normal, AC, AC-CC) and were observed in 6 weeks. Except for normal group, liver fibrosis(cirrhosis) in rats were developed by CCl₄ administration(0.8 ㎖/rat/week). And the rats were treated with prepared CC(p. o. 2 ㎖/day/rat). At the time of sacrifice, the liver, kidney and spleen were weighed and the ratio of organ weight/body weight was calculated. The MDA, hyp and biochemical parameters(AST, ALT, ALP, t-bili) were measured in sera and liver tissue of rats. The strong yellow color of urine was observed in all CCl₄-treated group compared with normal group, but jaundice didn't appear in CCl₄-treated group. The mortality of CCl₄-treated group is very low(<13%) during 6 weeks of observation time. The ratio of liver/body as well as the weight of liver in CCl₄-treated rats significantly increased compared with that in normal group(p<0.001). The level of clinical parameters in sera of all liver fibrosis(cirrhosis) developed rats were significantly higher than that of normal group(p<0.001-0.05). Especially the value of BUN, ALP, t-bilirubin in AC-CC group showed 20.9%, 19.6%, 47.9% lower than that in AC group. The content of hyp in CCl₄-treated rats was significantly higher than normal group(p<0.001～<0.05), and showed 12.2% lower value in the AC-CC group than AC group(p<0.05). The production of lipid peroxidation(MDA) in sera and liver tissue significantly increased under the fibrotic(cirrhotic) condition(p<0.001～<0.05). Especially the MDA value of AC-CC group in sera significantly 46.5% decreased compared with that of AC group(p<0.05), and the MDA value of AC-CC in liver tissue showed 21.4% lower than that of AC group. Concha Cipangopaludinae can be improved hepatic function, and maybe have effect of liver protection, antioxidation and antifibrosis.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.189-194
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• 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.

• Journal of Korean Society of Occupational and Environmental Hygiene
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• v.31 no.3
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• pp.213-225
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• 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.