• 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.22 no.2
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• pp.81-85
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• 2018

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of "very early onset inflammatory bowel disease". Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

• Clinical and Experimental Pediatrics
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• v.53 no.7
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• pp.770-773
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• 2010

A 13-year-old girl was diagnosed with non-cystic fibrosis (CF)-related multifocal bronchiectasis accompanied by nephrotic-range proteinuria of unknown cause. On renal biopsy, there were many segmental homogeneous deposits of amyloid tissue with positive Congo red staining in the glomeruli and interstitium. On electron microscopy, relatively straight, non-branching, randomly arranged amyloid fibrils were showed in the mesangium of the glomeruli. These fibrils were approximately 10 nm in diameter, compatible with secondary amyloidosis. Her level of serum amyloid A was remarkably elevated. To our knowledge, this girl is the first case of secondary renal amyloidosis induced by bronchiectasis in Korean children.

• Journal of Yeungnam Medical Science
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• v.32 no.2
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• pp.102-105
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• 2015

Secondary systemic (AA) amyloidosis is a severe complication of progressed Crohn disease (CD) characterized by the deposition of amyloid A in body organs and tissues. Various therapeutic approaches have been recommended, however there is still no effective treatment. Recently, several case reports have demonstrated the effects of anti-tumor necrosis factor-${\alpha}$ therapy in patients with AA amyloidosis associated with CD. We report on a 35-year-old female patient with CD complicated by AA amyloidosis in the gastrointestinal tract and renal involvement, who was treated with infliximab. The infliximab therapy improved the gastrointestinal symptoms and decreased the serum creatinine.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.142-149
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• 2003

Purpose : The urinary mass screening program for the detection of urinary abnormalities in school aged population has been performed in Seoul since 1981. Nation-wide urinary mass screening program was also performed since 1998. The aim of this study was to analyze the cause and nature of isolated proteinuria detected by chance on the urinary mass screening test in Busan and Kyungsangnam-do Province Methods : The medical records of 44 cases of isolated proteinuria detected by chance on the urinary mass screening test in Busan and Kyungsangnam-do Province, and evaluated for urinary abnormalities at the pediatrics outpatients renal clinics of Busan Paik Hospital from April 2002 to August 2003 were reviewed prospectively. Results : The cause and incidence of isolated proteinuria were as follows; transient proteinuria 4 cases(9.1%), orthostatic proteinuria 36 cases(81.8%) and persistent proteinuria 4 cases (9.1%). The total protein amount of the 24 hour urine were $121.0{\pm}136.4\;mg$ in transient proteinuria, $179.1{\pm}130.0\;mg$ in orthostatic proteinuria and $1532.8{\pm}982.5\;mg$ in persistent proteinuria. In the orthostatic proteinuria group, the total protein amount of the 24 hour urine was in the range of 40-616 mg. Spot urine protein/creatinine ratio(PCR) were $0.10{\pm}0.01$ in transient proteinuria, $0.61{\pm}0.61$ in orthostatic proteinuria and $4.35{\pm}4.04$ in persistent proteinuria. In the orthostatic proteinuria group, spot me PCR was in the range of 0.09-2.32. Renal biopsy was peformed in 4 children of the persisitent proteinuria group. They showed minimal change in 1 case, membranoproliferatiye glomerulonephritis in 2 cases and secondary renal amyloidosis in 1 case. Conclusion : The majority of isolated proteinuria which was detected by chance on school urinary mass screening were transient or orthostatic proteinuria. Even though the incidence of persistent proteinuria was much lower, it is necessary to take care of these children regularly and continuously, because persistent proteinuria itself is a useful marker of the progressive renal problems.