• Childhood Kidney Diseases
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• v.27 no.2
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• pp.133-138
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• 2023

Nutcracker syndrome (NCS) is a disease caused by compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by the predominance of IgA deposits in the glomerular mesangial area. Hematuria and proteinuria can be present in both diseases, and some patients can be concurrently diagnosed with NCS and IgAN; however, a causal relationship between the two diseases has not yet been clarified. Here, we report two pediatric cases of NCS combined with IgAN. The first patient presenting with microscopic hematuria and proteinuria was diagnosed with NCS at the initial visit, and the second patient was later diagnosed with NCS when proteinuria worsened. Both patients were diagnosed with IgAN based on kidney biopsy findings and treated with angiotensin-converting enzyme inhibitors and immunosuppressants. A high index of suspicion and timely imaging or biopsy are essential for the proper management of NCS combined with glomerulopathy.

• 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.8 no.2
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• pp.205-213
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• 2004

Purpose: To evaluate the clinical manifestations of various glomerular diseases in children, a clinicopathological study was performed in 52 children who had renal biopsy. The type and relative incidence of the glomerular pathologies were analyzed, and the clinical predictability and usefulness of renal biopsy in glomerular diseases were assessed. Methods: Medical records of fifty two children with renal disease who had undergone percutaneous renal biopsy under ultrasonic guidance at Chungnam University Hospital from October 1995 to August 2003 were reviewed. In addition, we compared the clinical findings before renal biopsy with the pathological diagnosis. Results: The male to female ratio was 1.6:1 and they were $9.8\pm2.6$ years old on average. The chief complaints for biopsy were hematuria in 22 cases which was the most common (42.3%), proteinuria in 16 cases(30.8%), and hematuria & proteinuria(26.9%). Among the 22 cases of hematuria, there were 15 cases of gross hematuria(68.2%) and 7 cases of microscopic hematuria(31.8%). In terms of histopathologic diagnosis, most of them were primary glomerular diseases(84.6%), which included IgA nephropathy(28.8%), thin glomerular basement membrane disease(25.0%), focal segmental glomerulosclerosis(FSGS)(11.5%), membranous proliferative glomerulonephritis(7.7%), minimal change lesion(3.8%), acute poststreptococcal glomerulonephritis(3.8%) and membranous glomerulonephritis(3.8%). The clinical manifestations and pathologic diagnosis were not correlated. Conclusion: The clinical manifestations could not predict the pathological diagnosis. Therefore, renal biopsy would be inevitable in diagnosis of glomerular diseases for effective management and assessment of prognosis.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.174-181
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• 2006

Purpose : To analyse the results of the renal biopsies and the clinical diagnoses of patients who had undergone percutaneous kidney biopsies in the department of pediatrics at Kyunghee University Hospital for 22 years from 1984 to 2005. Methods : We retrospectively reviewed the medical records of 1559 patients and analyzed the chief complaints that led to a renal biopsy, age, sex, histopathologic findings and diagnosis. Routine kidney biopsies were performed by automated gun biopsy guided by real time ultrasonography. The diagnoses were made based on the specimen's light microscopy, immunofluorescence microscopy and electron microscopy findings and clinical symptoms and signs. Results : The mean age of the patients was 10 years with the male to female ratio being 1.3:1. The chief complaints that led to a renal biopsy included hematuria only(753 cases, 48.3%), proteinuria only(125 cases, 8.0%) and hematuria combined with proteinuria(537 cases, 34.4%). The most frequent histopathological finding was primary glomerular disease(75.4%) which included IgA nephropathy(30.1%) and mesangial proliferative glomerulonephritis(27.6 %). Systemic disease comprised 11.4% which included Henoch-$Sch\ddot{o}nlein$ nephritis(10.5%) and lupus nephritis(0.8%). Alport syndrome was found in 1.1% of cases which was attributed to hereditary causes. 628 children(40.3%) visited the clinic due to abnormal school urine screening abnormalities and among these, 237 children had mesangial proliferative glomerulonephritis and 234 children who had IgA nephropathy were managed thereafter. Conclusion : IgA nephropathy and mesangial proliferative glomerulonephritis were the two major forms of primary glomerulonephritis found in Korean children who had kidney biopsies from 1984 to 2005.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.1-8
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• 2001

Purpose : Clinical manifestations and pathologic findings of thin glumerular basement membrane disease, recognized as a common underlying disease of benign, familaiar and asymptomatic hematuria has not been reported systemically in Korera. We analyzed clinical and pathologic findings of patients who were diagnosed as thin glomerular basement membrane disease Methods : We analyzed clinical and pathologic findings of twenty-six patients who were diagnosed as thin glomerular basement membrane disease by renal biopsy among who complained asymptomatic hematuria from 1990 to 2000. Results : The subjects were aged 9.4${\pm}$3.2 (3.0-15.8) years-old at onset of hematuria, and 11.1${\pm}$2.2 (4.7-16.3) years-old at renal biopsy. Sexual discrepancy was more common in girls (eight boys and eighteen girls). A family history of hematuria was found in 8 patients(30.7$\%$). Major clinical manifestation on admission was microscopic hematuria according to the findings of 3case(11.5$\%$) of gross hematuria, 23cases(88.5$\%$9) of microscopic hematuria, and 1 case(3.8$\%$) of proteinuria. Microscopic hematuria persisted in all cases. Kidney biopsy showed few changes by light microscopy, but IgM, C3 and fibrinogen deposit in mesangium was found by immunofluorescent microscopy in a few cases. Electron microscopic findings have revealed thinning of the glomerular basement membrane varied from 180.9${\pm}$35.8nm. Conclusion : Thin glomerular basement membrane disease might be a common cause of microscopic hematuria of children and family history was revealed in about 30$\%$. Clinical progression was good in majorities.(J. Korean Soc Pediatr Nephrol 5 : 1-8, 2001)

• Clinical and Experimental Pediatrics
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• v.53 no.7
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• pp.766-769
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• 2010

Dense deposit disease (DDD) is a rare disorder characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidneys. The diagnosis is made in most patients between 5 and 15 years of age, and within 10 years, approximately half of the affected patients progress to end-stage renal disease. We report a rare case of regressive DDD without C3 deposition after steroid therapy in an 11-year-old boy. The patient presented with edema, gross hematuria, and nephrotic-range proteinuria. Laboratory testing revealed a serum creatinine level of 1.17 mg/dL, albumin level of 2.3 g/dL, and serum C3 level of 125 mg/dL (range 90-180 mg/dL). The results of the renal biopsy were consistent with DDD without C3 deposition. After 6 weeks of steroid therapy, the nephrotic syndrome completely resolved. The follow-up renal biopsy showed a significant reduction in mesangial proliferation and disappearance of electron-dense deposits in the GBM.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.162-173
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• 2006

Purpose : To report the decreasing indicence of HBV(Hepatitis B virus)-associated membranous nephropathy in children after HBV vaccination and to elucidate the clinical course and treatment strategies of IMN(Idiopathic membranous nephropathy). Methods : We retrospectively reviewed the clinico-pathological findings of HBV-MN and IMN patients who underwent a renal biopsy from 1986 to 2005. We compared the HBV-MN and the IMN groups and the remission and the non-remission groups of patients with IMN. Results : Among 24 cases of MN patients, HBV-MN comprised 6 cases(25%) and IMN 18 cases(75%). Clinical masnifestations were nephrotic syndrome(3 cases, 50%), nephritic syndrome(1 case, 16.7%), asymptomatic(2 cases, 33.4%) in the HBV-MN group, asymptomatic(10 cases, 55.5%), nephrotic syndrome(5 cases, 27.8%), and gross hematuria(3 cases, 16.7%) in the IMN groups. From 1996 to 2000, there were 2 cases(28%) of HBV-MN and 5 cases(72%) of IMN. After 2001 all 10 cases were IMN. In the HBV-MN group, 4 cases(66.7%) received interferon and 1 cases received methylprednisolone pulse therapy. In the IMN group, 16 cases(88.9%) received methylprednisolone, 8 cases(44.4%) were in complete remission, 2 cases(11.1%) were in partial remission, 2 cases(11.1%) were in chronic renal failure, and 5 cases(27.8%) were lost to follow-up with sustained proteinuria, 1 case(5.6%) continued to have frequent relapse of nephrotic syndrome without renal insufficiency. In the comparison between remission and non-remission groups, nephrotic range proteinuria and hypertension were more significantly common in the non-remission group(P<0.05). Conclusion : With HBV vaccination, HBV-MN has decreased markedly. IMN is a rare glomerular disease in children. Because the prognosis for patients with nephrotic range proteinuria is poor this group needs more aggressive treatment.

• 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.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.188-195
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• 2001

Type II membranoproliferative glomerulonephritis (Dense deposit disease) is an acquired primary glomerular disease characterized by electron microscopic evidence of a continuous dense membrane deposition replacing the lamina densa. It is a subtype of idiopathic membra- noproliferative glomerulonephritis, and was described as a separate entity by Berger and Galle in 1963. It frequently occurs in older chilren and young adults and the clinical course is variable, but is generally progressive. The presenting feature is nephrotic syndrome in many patients, and proteinuria and hematuria are also seen frequently. The purpose of this paper is to present a case of DDD (Dense deposit disease) from a 10 year old boy who was diagnosed as a acute poststreptococcal glomurulonephritis with protenuria, hematuria, and facial edema by renal biopsy 4 years ago. (J, Korean Soc Pediatr Nephrol 2001 ; 5 : 188-95)

• 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)