• 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.

• Childhood Kidney Diseases
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• v.27 no.2
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• pp.55-63
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• 2023

In 2021, a new chapter on the general management of glomerulonephritis (GN) was added to the Kidney Disease: Improving Global Outcomes (KDIGO). It emphasizes the importance of early general management of GN for improving long-term kidney outcomes and prognosis. The chapter introduces the management of glomerular diseases in 18 subchapters. Here, kidney biopsy for the diagnosis and evaluation of kidney function and the management of complications, such as hypertension, infection, and thrombosis, are presented. Moreover, the adverse effects of glucocorticoids and immunosuppressive therapy, which are commonly used drugs for glomerular disease, are mentioned, and a guideline for drug selection is presented. Each subtheme focused on items reflecting the interpretation of the "practice points" of the expert working group are introduced. In this review of the general treatment for GN in the KDIGO guidelines, excluding pregnancy and reproductive health, we focused on and compared various references pertaining to pediatric GN management.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.75-83
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• 2009

Purpose : Chronic kidney disease (CKD) and obesity are the worldwide public health problem. Obesity is an already well-established risk factor for CKD. The objective of this study is to evaluate the relationship between high BMI and increased risk for nephropathy by clinical data. Methods : Study group were 26 patients who had $BMI{\geq}25\;kg/m^2$ and control group were 49 patients with BMI<$25\;kg/m^2$. Both groups received renal biopsy in Kyung Hee Medical Center between 2003. Jan.-2007. Dec. BMI was calculated from measured weight and height when they were admitted to the hospital. We collected laboratory data such as CBC and blood chemistry. Results : Our hypothesis was that overweight and obesity are associated with incidence and progression of CKD. From kidney biopsy, we found IgAN 17, MesPGN 5, HSPN 2, Intestitial nephritis 1, IgMN 1 (total 26) in the study group whereas IgAN 22, MesPGN 17, HSPN 3, MGN 3, benign hematuria 2, MPGN 1, Intestitial nephritis 1, (total 49) were found in the control group. There was no significant difference between the two groups. Overweight patients demonstrated significantly higher platelet, TG, ALT, and uric acid level compared to control group. Conclusion : We identified a significant relationship between overweight and development of CKD. These results suggest that overweight children have an increased risk for CKD than those who are not obese. So, we should pay attention to children with overweight who have CKD and earlier weight management is crucial to prevent aggravation of CKD.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.213-218
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• 2001

Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN l17 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 213-18)

• Clinical and Experimental Pediatrics
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• v.59 no.3
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• pp.145-148
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• 2016

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially fatal condition characterized by skin rash, fever, eosinophilia, and multiorgan involvement. Various drugs may be associated with this syndrome including carbamazepine, allopurinol, and sulfasalazine. Renal involvement in DRESS syndrome most commonly presents as acute kidney injury due to interstitial nephritis. An 11-year-old boy was referred to the Children's Hospital of Pusan National University because of persistent fever, rash, abdominal distension, generalized edema, lymphadenopathy, and eosinophilia. He previously received vancomycin and ceftriaxone for 10 days at another hospital. He developed acute kidney injury with nephrotic range proteinuria and hypocomplementemia. A subsequent renal biopsy indicated the presence of acute tubular necrosis (ATN) and late exudative phase of postinfectious glomerulonephritis (PIGN). Systemic symptoms and renal function improved with corticosteroid therapy after the discontinuation of vancomycin. Here, we describe a biopsy-proven case of severe ATN that manifested as a part of vancomycin-induced DRESS syndrome with coincident PIGN. It is important for clinicians to be aware of this syndrome due to its severity and potentially fatal nature.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.79-81
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• 1997

Minimal change nephrotic syndrome is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia. Children with onset of nephrotic syndrome between the age of 1 and 8 year are likely to have steroid response to minimal chage disease, but we experienced one case of minimal change disease which failed to respond to steroid therapy at beginning and subsequently developed acute renal failure. It was seen in a 5 year-old male child that presented with edema and gross hematuria. Peritoneal dialysis was performed for acute renal failure for 11 days. Patient was completely recorvered from acute renal failure and renal biopsy was done at 27th day after onset of disease which revealed typical picture of minimal change disease complicated by acute tubular necrosis. We beleive this case is very unusual and it may be the first case in the literature in terms of pediatric cases.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.196-205
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• 2001

Purpose : Membranous glomerulopathy is a glomerular disease characterized by the presence of subepithelial immune deposits with thickening of the capillary wall of the glomerulus without inflammatory change. The pathogenesis of membranous glomerulopathy is still unknown. Its incidence is higher in males, and it is rarely found in infants and adolescents. Among the clinical manifestations proteinuria is most common, while edema and hematuria are present. According to reports from other countries, among few patients diagnosed with membranous glomerulopathy by renal biopsy, show isolated microscopic hematuria without the clinical manifestations. Little research in this area has been performed in Korea, and so we conducted retrograde studies on membranous glomerulopathy associated with isolated microscopic hematuria. Materials and Methods : We analyzed retrogradely 109 cases of asymptomatic isolated microscopic hematuria that were diagnosed as membranous glomerulopathy by renal biopsy at Yonsei University Severance hospital from January, 1992 to July, 2001. Results : In 87 of the 109 cases patients were over 15 years old while in 22 cases patients were under 15 at the time of dignosis. Only three patients showed isolated microscopic hematuria without the clinical manifestations and abnormal laboratory findings and they were all male patients under 15 years old. Conclusion : Few cases of the membranous glomerulopathy show only asymptomatic isolated microscopic hematuria However, since membranous glomerulopathy can be found in patients who present with asymptomatic isolated microscopic hematuria only, if adequate indication for renal biopsy is present, we conclude that renal biopsy must be aggresively pursued in order to find the underlying disease. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 196-205)

• 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.

• Korean Journal of Transplantation
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• v.28 no.3
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• pp.135-143
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• 2014

Background: Kidney injury molecule-1 (KIM-1) is known as a good ancillary marker of acute kidney injury (AKI) and its expression has also been observed in acute rejection and chronic graft dysfunction. We tested usefulness of KIM-1 as an indicator of acute and chronic renal graft injury by correlating KIM-1 expression with renal graft function and histology. Methods: A total of 133 zero-time biopsies and 42 follow-up biopsies obtained within 1 year posttransplantation were selected. Renal tubular KIM-1 staining was graded semiquantitatively from 0 to 3 and the extent of staining was expressed as the ratio of KIM-1 positive/CD10 positive proximal tubules using Image J program. Results: KIM-1 was positive in 39.8% of zero-time biopsies. KIM-1 positive cases were predominantly male and had received grafts from donors with older age, deceased donors, and poor renal function at the time of donation, compared with KIM-1 negative cases. KIM-1 expression showed correlation with delayed graft function and acute tubular necrosis. In comparison of KIM-1 expression between stable grafts (n=23) and grafts with dysfunction (n=19) at the time of repeated biopsy, the intensity/extent of KIM-1 staining and renal histology at zero-time did not differ significantly between the two groups. Histologically, KIM-1 expression was significantly increased with both acute and chronic changes of glomeruli, tubules and interstitium, peritubular capillaritis, and arteriolar hyalinosis. Conclusions: KIM-1 can be used as an ancillary marker of AKI and a nonspecific indicator of acute inflammation and tubulointerstitial fibrosis. However, KIM-1 expression at zero-time is not suitable for prediction of long-term graft dysfunction.