• Childhood Kidney Diseases
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• v.6 no.1
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• pp.48-55
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• 2002

Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.136-143
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• 1997

Purpose : To evaluate the prevalence and clinical manifestations of various glomerulonephritis(GN) in children with asymptomatic urinary abnormalities, a clinicopathological analysis of 134 biopsied cases which were subdivided into 3 groups of proteinuria with hematuria, isolated hematuria and isolated proteinuria was done. Methods : We conducted retrospective study with review of histopathologic findings and clinical manifestations of the 134 cases with asymptomatic urinary abnormalities diagnosed by percutaneous renal biopsy which were done between January 1986 and December 1996 at department of pediatrics, Pusan Paik hospital. Results : 1) The proportion of children with asymptomatic urinary abnormalities was 43.2% of all biosied cases. 2) Among these, primary GN were 95 cases and secondary GN were 39 cases, it's ratio was 2.44:1. As a whole, the most common pathologic diagnosis was IgA nephropathy(IgAN, 26.9%), which was followed by $Henoch-Sch\"{o}nlein$ purpura nephritis(HSPN, 17.9%), minimal change lesion(MC, 17.2%), thin GBM disease(12.7%), Hepatitis B associated glomerulonephritis(HBGN, 6.0%), poststreptococcal glomerulonephritis(PSAGN, 3.0%), mesangial proliferative glomerulonephritis(MesPGN, 2.2%), membranoproliferative glomerulonephritis (MPGN, 2.2%), Alport syndrome (1.5%) and Fibrillary nephritis(0.7%). 3) In proteinuria with hematuria, the most common pathologic diagnosis was IgAN(34.6%), which was followed by HSPN(19%), MC(17.7%), thin GBM disease(8.9%), HBGN(6.3%), PSAGN(3.6%), MesPGN(1.2%), MPGN(1.2%) and Alport syndrome(1.2%). 4) Major causes of isolated hematuria were thin GBM disease(19.6%), IgAN(17.6%), HSPN(17.6%), MC(11.8%). 5) Isolated proteinuria was due to of 3 cases of MC and 1 case of HBGN. Conclusion : The prevalence of glomerulonephritis with asymptomatic urinary abnormalities in children were 43.2% of all biopsed cases. When these children were subdivided into 3 groups, proteinuria with hematuria was accounted 58.9%(79 cases) and then isolated hematuria was 38.1%(51 cases), isolated proteinuria was only 3%(4 cases) respectively. The most common pathologic diagnosis was IgA nephropathy in patient with proteinuria and hematuria, and thin GBM disease in patient with isolated hematuria.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.157-165
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• 2003

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is a systemic vasculitis that involves multiple organs, especially the kidney, which is the most important organ in determining the prognosis of the disease. The morbidity of HSP nephritis in adults is low and there have been little research done on its clinical course so far. Therefore, we have compared the clinical course of HSP nephritis in children and adults in Korea. Methods : We retrospectively analyzed 81 cases of HSP nephritis in children younger than 15 years of age, and 25 cases of adults older than 15 years of age who were admitted to Yonsei University Medical College Severance Hospital from Jan. 1986 to May 2003. Results : The male to female ratio was 1.5 : 1 in children and 1.3 : 1 in adults. The incidence of HSP nephritis for both age groups was found to be increased during the autumn and winter. Infection was the predisposing factor in 39 cases(48.1%) of children, 16 cases(64.0%) of adults, and drugs were the predisposing factor in 8 cases(9.9%) of children and 4 cases (16.0%) of adults. All patients initially presented with microscopic hematuria. Thirteen cases (16.0%) of children and 7 cases(28.0%) of adults initially showed proteinuria of nephrotic range. Thirty four cases(42.0%) of children and 4 cases(16.0%) of adults showed normal urinalysis after treatment. Asymptomatic urinary abnormalities were found in 41 cases(50.6%) of children and 18 cases(72.0%) of adults. Complications such as nephrotic syndrome and hypertension were found in 3 cases(3.7%) of children and 2 cases(8.0%) of adults. Three children(3.7%) and 1(4.0%) adult required dialysis or renal transplantation. Follow-up renal biopsies were performed on 21 children, of whom 10 cases(47.6%) did not show any histologic change, 9 cases(42.9%) showed low grade changes, and 2 cases(9.5%) showed high grade changes. Prognosis was gloomy when proteinuria of nephrotic range and high grade of abnormal histology were present at diagnosis, and there was no significant difference between the two groups(P<0.05) Conclusion : This study showed that there was no difference in terms of the clinical features and courses between the children and adults with HSP nephritis. Proteinuria of nephrotic range and the severity of abnormal histologic changes at diagnosis were found to be associated with a bad prognosis, therefore we recommend that patients with these features require long term follow-up and management.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.22-31
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• 2010

Purpose : This study was performed to evaluate the relationship between glomerular basement membrane (GBM) alterations to epithelial cell (EpC) structure and renal function in Alport Syndrome (AS) patients. Methods : Fifteen patients diagnosed with AS (4-26yrs) were examined. The GBM in AS was categorized as : C1) normal, C2) minor alterations (widening of lamina rara interna or externa without lamina densa change), C3) nonspecific splitting of lamina densa, C4) basket-weaving pattern of lamina densa splitting. The length of each GBM portion along the epithelial side was measured on the systematically obtained electron microscopic photographs. Furthermore to obtain an objective assessment of the degree of glomerular EpC foot process change, the number of slit pores along $10\;{\mu}m$ of peripheral GBM in each category was obtained. Results : The percentage of normal GBM portion (C1) correlated inversely with daily protein excretion (g/day/$m^2$, P<0.05) and sum of the percentage of abnormal GBM portion (C2+C3+C4) had direct correlation with daily protein excretion (g/day/$m^2$, P<0.05). There were no significant relationships between the percentages of other categories of GBM alterations and creatinine clearance or protein excretion. There were no significant relationships between of creatinine clearance in relation to normal GBM(C1) portion as well as that in relation to sum of the percentage of abnormal GBM portion (C2+C3+C4). GBM abnormality did not correlate with age at biopsy. Conclusion : The extent of GBM structural abnormality is related to proteinuria in AS but the epithelial response is uniform even though the GBM ultrastructural lesions are not.

• Archives of Plastic Surgery
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• v.34 no.5
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• pp.659-662
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• 2007

Purpose: Multiple skin leiomyoma and uterine myoma bearing autosomal dominant traits are benign smooth muscle tumors which originate in skin or female uterus. Skin leiomyoma occurs after gene mutation originating from arrector pili muscle of hair follicle where its clinical manifestations vary significantly from person to person. Our department hereby reports the histological findings and genetic evaluations of this very rare disease. Methods: A 57-year-old woman presented in our institute with multiple tumors in the left and central parts of her back that started to appear since 19 years ago. The patient was diagnosed as having uterine myoma 15 years ago and underwent hysterectomy. Biopsy has been done on the specimen, and genomic DNA was separated from Fumarate hydratase gene for it to go through PCR amplification. The results of PCR amplification were aligned by sequencer. Results: According to the results of biopsy, tumor cells were spindle-shaped and were aligned in a bundle where there was no dysplasia or mitosis. Moreover, these cells had abundant eosinophilic cytoplasm with elongated nucleus, and benign leiomyoma that showed positive reactions to SMA stain were found. In genetic examination, mutations such as heterozygous single nucleotide substitutions were found in alignments of amplified DNA. Conclusion: Multiple skin leiomyoma and uterine myoma are relatively uncommon diseases that are transmitted through autosomally dominant traits from genetic mutations. When a patient's chief complaint lies upon skin-colored or brown masses that occur in multiples appearing in the trunk or extremities with characteristic clinical symptoms and histological findings, and when the patient's family history is acknowledged such as skin or uterine leiomyoma or renal tumor, necessary genetic examination on multiple skin leiomyoma and uterine myoma could be done, and thereby precise diagnosis could also be made.

• Journal of Veterinary Clinics
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• v.28 no.6
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• pp.598-602
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• 2011

A 4-year-old female Shih-tzu dog weighing 5.1 kg was referred with abdominal distension and diagnosed as cystic papillary adenocarcinoma with metastasis to serosal surface of abdomen by biopsy. Intraperitoneal chemotherapy with cisplatin (50 $mg/m^2$) diluted in normal saline (250 $ml/m^2$) was initiated following removal of ascites one month after ovariohysterectomy (OHE). Clinical response was great and had no side effects during the chemotherapy period (3 relapses, 33 months after diagnosis). However, pleural effusion occurred with ascites and renal failure at the end of treatment. Eleven treatments of cisplatin were performed during 35 months after diagnosis. Intraperitoneal cisplatin instillation has been effective to control malignant ascites and pleural effusion in ovarian cancers and could be a reasonable palliative treatment. This is the first case report describing intraperitoneal cisplatin chemotherapy in metastatic ovarian cyst papillary adenocarcinoma in Korea.

• Neonatal Medicine
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• v.17 no.2
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• pp.254-261
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• 2010

Little is known about neonatal mitochondrial disease, though mitochondrial metabolic disorders may often present in the neonatal period because of the high energy requirement of neonate. In newborn period, common presentations are not specific and the disease course may be rapid and fatal. In this study, we report three cases of neonatal mitochondrial disease. The first case was strongly suspected because of sudden seizure and mental change with severe lactic acidosis, and multiorgan failure. Plasma lactate/pyruvate (L/P) ratio was increased to 55.6 with marked lactic aciduria and increased plasma alanin up to 2,237 nmol/mL. In the second patient, a peritoneal dialysis was performed for acute adrenal and renal failure, but metabolic acidosis persisted. Plasma L/P ratio was increased to 23.9, and MRC I (mitochondrial respiratory chain defect) was diagnosed through the enzymatic analysis of the muscles. The third case showed repetitive episode of lactic acidosis during the first two months of life, hypotonia, failure to thrive and feeding difficulties. We found markedly increased cerebrospinal fluid L/P ratio up to 57 though plasma L/P ratio(19.4) was borderline with increased plasma lactate. The lactate peak was prominent in brain magnetic resonance spectroscopy (MRS). MRC II was confirmed through muscle biopsy. Plasma lactate level and lactate peak of brain MRS were normalized after conservative treatment.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.106-111
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• 2007

[ $Henoch-Sch\ddot{o}nlein$ ] Purpura(HSP) is a form of vasculitis that typically affects small arteries in the skin, joints, intestinal tract and kidneys. It usually resolves spontaneously but sometimes can cause serious problems in the kidneys and intestinal tract. A 6-year-old girl with purpura, arthralgia and abdominal pain for 2 weeks was admitted. She also showed gross hematuria, generalized edema and decreased urine output. Blood pressure was in the upper normal range. Initial laboratory findings showed hypoalbuminemia, hyperlipidemia, microhematuria and nephrotic-range proteinuria(27.2 g/day). Initially, she was treated with pulse methylprednisolone, azathioprine, albumin and furosemide. Her renal biopsy revealed diffuse mesangial proliferation with strong IgA deposition. There were no crescents. On the third hospital day, she complained of severe abdominal pain and free peritoneal air was seen on abdominal X-ray. Primary repair of small bowel was performed and two pin-point sized holes were found. One week later, she still showed heavy proteinuria. Therefore, we added an ACE inhibitor and dipyridamole, and changed azathioprine to cyclosporine. One month later, the urine protein/creatinine ratio was decreased to 17.8 from 57, but heavy proteinuria has been still persisted. Here we report a rare case of a patient with HSP who had both severe nephrritc-nephrotic syndrome and small bowel perforation.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.128-131
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• 2014

Histopathologic evidence of "full-house" immune complex deposits is a pathognomonic feature of lupus nephritis. This report presents the case of a 12-year-old boy with persistent microscopic hematuria and proteinuria. He was diagnosed with "full-house" nephropathy based on a renal biopsy. However, there was no other clinical or biological evidence of systemic lupus erythematosus (SLE). Although the potential for isolated "full-house" nephropathy preceding SLE is unclear, such patients should be followed for clinical signs and autoantibodies of SLE. In most cases, microscopic hematuria has a good prognosis, and follow-up usually requires only regular urinalysis. However, we should be aware of isolated "full-house" nephropathy that remains asymptomatic for a long time, as few patients with no clinical signs and negative serology ultimately develop SLE.

• The Korean Journal of Physiology and Pharmacology
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• v.19 no.3
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• pp.229-234
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• 2015

Nafamostat mesilate (NM) is a serine protease inhibitor with anticoagulant and anti-inflammatory effects. NM has been used in Asia for anticoagulation during extracorporeal circulation in patients undergoing continuous renal replacement therapy and extra corporeal membrane oxygenation. Oxidative stress is an independent risk factor for atherosclerotic vascular disease and is associated with vascular endothelial function. We investigated whether NM could inhibit endothelial dysfunction induced by tumor necrosis factor-${\alpha}$ (TNF-${\alpha}$ ). Human umbilical vein endothelial cells (HUVECs) were treated with TNF-${\alpha}$ for 24 h. The effects of NM on monocyte adhesion, vascular cell adhesion molecule-1 (VCAM-1) and intracellular adhesion molecule-1 (ICAM-1) protein expression, p38 mitogenactivated protein kinase (MAPK) activation, and intracellular superoxide production were then examined. NM ($0.01{\sim}100{\mu}g/mL$) did not affect HUVEC viability; however, it inhibited the increases in reactive oxygen species (ROS) production and p66shc expression elicited by TNF-${\alpha}$ (3 ng/mL), and it dose dependently prevented the TNF-${\alpha}$ -induced upregulation of endothelial VCAM-1 and ICAM-1. In addition, it mitigated TNF-${\alpha}$ -induced p38 MAPK phosphorylation and the adhesion of U937 monocytes. These data suggest that NM mitigates TNF-${\alpha}$ -induced monocyte adhesion and the expression of endothelial cell adhesion molecules, and that the anti-adhesive effect of NM is mediated through the inhibition of p66shc, ROS production, and p38 MAPK activation.