• Childhood Kidney Diseases
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• v.21 no.1
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• pp.8-14
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• 2017

Purpose: To classify the results of renal biopsy in pediatric patients and to compare pathological findings with clinical features. Methods: This study included data of 318 children who underwent renal biopsy at our hospital between December 1987 and November 2014. Biopsy specimens were examined histopathologically using light, immunofluorescence, and electron microscopy. Results: Asymptomatic urinary abnormalities was the most common clinical diagnosis (35.9%), followed by nephrotic syndrome (29.3%), and acute glomerulonephritis (18.0%). Glomerular disease was identified in 98.1% of the renal biopsy specimens. The most common primary cause of glomerulonephritis was IgA nephropathy, with gross hematuria in 61.9% of the patients, hypertension in 14.2%, proteinuria >1.0 gm/24-hr in 33.3%, and impaired renal function in 3.6% patients. Conclusion: The most common clinical diagnosis was asymptomatic urinary abnormalities, with primary glomerular disease being the most common renal biopsy finding, and IgA nephropathy the most common histopathological lesion. This study provides a 27-year overview of pediatric renal disease at our center and underlines the importance of renal biopsy for accurate diagnosis and proper management.

• Childhood Kidney Diseases
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• v.20 no.2
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• pp.79-82
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• 2016

Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme ${\alpha}-galactosidase$ A, resulting in the accumulation of glycosphingolipids within the lysosomes of various cell types. It has a wide spectrum of clinical phenotypes, and renal failure is a serious complication. Fabry disease is confirmed either by measurement of ${\alpha}-galactosidase$ A activity or by genetic testing for GLA mutations. Renal biopsy findings on light microscopy, specifically enlarged podocytes with foamy cytoplasm, and osmiophilic inclusion bodies in the cytoplasm in all types of renal cells on electron microscopy, are characteristic of this disease. The predominant differential diagnosis is iatrogenic phospholipidosis in association with certain drugs that can cause cellular injuries indistinguishable from Fabry disease. Here, we report the case of a 10-year-old boy with microscopic hematuria who underwent a renal biopsy that showed morphological findings consistent with Fabry disease, although the patient had neither a GLA mutation nor a history of drug consumption. Six years later, spontaneous regression of this renal pathology was observed in a second renal biopsy examination.

• Journal of Chest Surgery
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• v.33 no.4
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• pp.338-341
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• 2000

Renal transplant recipients who received immunosuporessive agent are in high risk of development Kaposi's sarcoma. In Korea a few report of Kaposi's sarcoma has been pubilshed but any report of intrathoracic Kaposi's sarcoma provedn by open lung biopsy has not been pulbilshed until now. We report a case of intrathoracic Kaposi's sarcoma developed in a 25 year old Korean man, who had been operated renal transplantation due to end stage renal disease and received cyclosporine and prednisolone as immunosuppessive agent, without any other organ involvment and was proven by open lung biopsy. Although discontinuation of immunosuppressive agent, temporary symptomatic and radilolgic improvement were observed, he died 11 days later after open lung biopsy because of intractable resiratory failure.

• The Korean Journal of Cytopathology
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• v.7 no.1
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• pp.31-37
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• 1996

It is well known that fine needle aspiration biopsy(FNAB) is very useful and has a high accuracy rate in the diagnosis of renal neoplasms. Although there is some indecision to perform the FNAB for a rare possibility of tumor seeding along the biopsy needle tract, it tends to be used increasingly. As in the cytologic diagnosis of metastatic lesion through-out the body, renal cell carcinoma should nearly always be considered in the differential diagnosis, the precise understanding of cytologic features of renal cell carcinoma with various cell types and architectural patterns is necessarily required. In this report, we present three cases of primary renal cell tumors, two of renal cell carcinomas and one of oncocytoma, preponderantly emphasizing the cytologic differential points in the FNAB specimen.

• 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.

• Investigative Magnetic Resonance Imaging
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• v.21 no.2
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• pp.119-124
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• 2017

Chromophobe renal cell carcinoma (RCC) is an uncommon subtype of RCC having a better prognosis than clear cell RCC. Although there are several reports of seeding metastasis of RCC after biopsy, seeding metastasis of chromophobe RCC after surgical resection has seldom been reported. Here, we describe a case of multiple seeding metastases in the abdomen and pelvis 78 months after robot-assisted laparoscopic partial nephrectomy, without prior history of biopsy for chromophobe RCC in the right kidney. As magnetic resonance imaging (MRI) of the pelvic mass showed a similar appearance to the primary renal mass and displayed separate margins with the rectum and prostate gland, we were able to make a diagnosis before pathologic confirmation.

• Childhood Kidney Diseases
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• v.27 no.1
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• pp.19-25
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• 2023

Proteinuria is an important risk factor for renal and cardiovascular disease. It is associated with a risk for glomerulonephritis, chronic kidney disease, and end-stage renal disease. Therefore, if persistent proteinuria is detected, kidney biopsy is considered to diagnose and treat the underlying disease. Recently, variants in the cubilin (CUBN) gene, which is associated with albuminuria, have been reported. This gene encodes cubilin, a membrane glycoprotein receptor expressed in the renal proximal tubules. Cubilin is a component of the megalin and cubilin-amnionless complex that mediates albumin reabsorption into the proximal tubules through endocytosis. A defect in cubilin leads to a reduction in albumin reuptake, resulting in albumin-dominant proteinuria. Although numerous controversies exist, several reports suggest that cubilin defects lead to proteinuria with a high portion of albuminuria but may not impair renal filtration function. If albuminuria due to reduced cubilin function is confirmed as a benign condition, we can consider using genetic studies to detect CUBN mutations in patients with proteinuria and they may not require any treatment or kidney biopsy. Here, we review recent papers on CUBN mutations and discuss the prognosis and management of individuals with this mutation.

• Journal of Yeungnam Medical Science
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• v.9 no.2
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• pp.427-435
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• 1992

We have experienced a case of infantile nephrotic syndrome confirmed by renal biopsy in a 13-month-old female patient who showed growth and develop mental retardation and persistent proteinuria. She revealed mild eyelid edema, joint laxity, delayed speech development and adrenal cortical calcification on the radiologic study. Renal biopsy showed microcystic tubular change, micro-glomeruli and marked mesangial proliferation.

• Childhood Kidney Diseases
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• v.15 no.1
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• pp.29-37
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• 2011

To understand the course of renal diseases well, we must have basic knowledges of histologic procedures of renal biopsy samples as well as basic pathologic changes. This article describes the method of dividing the biopsy samples, fixatives for various pathologic examinations and basic pathologic changes of glomerular diseases. For light microscopic examination, color changes of glomerular structures in PAS, trichrome and PAM stains, normal glomerular patterns compared to various glomerulopathies are introduced. While describing typical staining patterns and intensities of fluorescence in membranous glomerulopathy and IgA nephropathy, basic interpretation of immunofluorescent microscopic examination is described. To understand electron microscopic pictures of renal diseases, preference locations of electron dense deposits in various glomerulonephrites are described with schema. This article is the introduction part of the renal pathology and for the further detail changes of specific entities, we should reference the renal pathology textbooks or articles.

• Journal of Yeungnam Medical Science
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• v.29 no.2
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• pp.102-105
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• 2012

Collapsing glomerulopathy (CG) has become an important cause of end-stage renal disease (ESRD). First delineated from other proteinuric glomerular lesions in the 1980s, CG is now recognized as a common, distinct pattern of proliferative parenchymal injury that portends a rapid loss of renal function and poor responses to empirical therapy. The first cases in the literature trace back to human-immunodeficiency-virus(HIV)-negative patients who underwent biopsy in 1979. A 45-year-old male patient complained of hematuria and proteinuria eight years ago. He showed an abrupt serum creatinine increase from 1.75 to 2.65 mg/dL in the last preceding months. Afterwards, his serum creatinine progressively increased up to 6.82 mg/dL. Moreover, his 24 h urine protein level was determined to have reached 6,171 mg/day, as opposed to 670 mg/day a year earlier. Consequently, renal biopsy was performed, and its result showed collapsing glomerulopathy, compatible with the diagnosis. He has undergone continuous ambulatory peritoneal dialysis as renal replacement therapy. Thus, it is reported herein that a patient clinically diagnosed with chronic kidney disease eight years ago showed a sudden renal-function decrease and was clinicopathologically diagnosed with collapsing glomerulopathy based on the results of his renal biopsy.