• Childhood Kidney Diseases
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• 제21권1호
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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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• 제20권2호
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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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• 제33권4호
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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.

• 대한세포병리학회지
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• 제7권1호
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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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• 제1권2호
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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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• 제21권2호
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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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• 제27권1호
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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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• 제9권2호
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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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• 제15권1호
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• pp.29-37
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• 2011

신장질환은 같은 병명의 환자 일지라도 개개인의 증상과 치료에 대한 반응 정도가 다를 수 있기 때문에 신장병을 전공하는 임상의사는 신장병리에 대한 폭 넓은 지식이 치료에 많은 도움을 줄 수 있다. 이 논문에서는 생검 현장에서의 조직을 나누는 방법부터 기본적인 조직처리 과정까지를 설명함으로써 병리의 이해를 넓히고자 하였다. 광학현미경 판독을 위해서는 기본적인 특수 염색의 색깔 감별, 정상 사구체와 비정상 사구체의 감별점 등을 설명하였다. 면역형광 현미경 소견의 이해를 돕기 위하여 전형적인 막성사구체신병증과 IgA 신증의 침착 형태를 대표적으로 설명하였다. 전자현미경 판독을 위해서 면역 복합체인 고전자 밀도의 침착이 잘 생기는 부위를 질환별로 설명하여 참고가 되도록 하였고 전자현미경으로만 감별 가능한 질환들을 설명하였다. 이 논문은 신장질환의 병리학적 이해를 돕기 위한 소개에 불과하므로, 개별 질환의 판독을 위해서는 다른 신장병리 교과서나 문헌을 참고하여야 한다.

• Journal of Yeungnam Medical Science
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• 제29권2호
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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.