• Childhood Kidney Diseases
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• v.28 no.1
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• pp.44-50
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• 2024

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that affects multiple organs. More than half of the patients with SLE have kidney involvement, and up to 10% of patients with lupus nephritis develop end-stage renal disease (ESRD). Central nervous system (CNS) involvement in SLE occurs in 21% to 95% of patients. Severe neurological manifestations such as seizures, cerebrovascular disease, meningitis, and cerebrovascular accidents can develop in childhood-onset SLE, but cerebral infections, such as brain abscess and hemorrhage, are seldom reported in lupus nephritis, even in adults. Here, we report a rare case of childhood-onset SLE with ESRD, cerebral abscess, and hemorrhage. A 9-year-old girl diagnosed with lupus nephritis was administered high-dose steroids and immunosuppressant therapy to treat acute kidney injury (AKI) and massive proteinuria. The AKI deteriorated, and after 3 months, she developed ESRD. She received hemodialysis three times a week along with daily peritoneal dialysis to control edema. She developed seizures, and imaging showed a brain abscess. This was complicated by spontaneous cerebral hemorrhage, and she became unstable. She died shortly after the hemorrhage was discovered. In conclusion, CNS complications should always be considered in clinical practice because they increase mortality, especially in those with risk factors for infection.

• The Korean Journal of Food And Nutrition
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• v.25 no.4
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• pp.755-761
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• 2012

In this study, we examined immuno-modulatory activities of crude polysaccharides from wild ginseng adventitious roots (WGAR). The crude polysaccharide (WGAR-CP) was isolated from WGAR by hot water extraction, ethanol precipitation, and dialysis. The major constituents in WGAR-CP were neutral sugar (64.77%), and uronic acid (34.32%). WGAR-CP demonstrated anti-complementary activity dose-dependently. The immuno-modulatory effects of WGAR-CP were also analyzed by measuring nitric oxide and cytokines in the supernatants of mouse peritoneal macrophages. Mouse peritoneal macrophages stimulated with WGAR-CP produced nitric oxide and various cytokines such as interleukin (IL)-6 and IL-12 in a dose-dependent manner. In conclusion, WGAR-CP may have immuno-modulatory activities by activating a complementary system and macrophages, which produces cytokines.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.136-143
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• 2000

Purpose : The hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia thrombocytopenia, and acute renal failure. It is ole of tile most common cause of acute renal failure in children but few reports are available in Korea. Thus we investigated the 23 patients diagnosed as HUS during last 14 years. Method : We retrospectively investigated the etiologic factor, clinical manifestations laboratory findings, treatment modalities, and final outcomes of the patients. Then patients were divided into two groups according to outcome, md comparison was performed. Group A(8) comprised patients who progressed to end-stage renal disease or expired. Group B(15) comprised patients who completely recovered after dialysis treatment. Result The number of patients aged less than 4 years were 17; between 5 and 10 were 4 and more than 10 were 2. The gende ratio was M:F=2 : 1. The etiologic factors were as follows: acute gastroenteritis in 14 patients including 4 bloody diarrhea, upper respiratory tract infection in 7 patients, and 1 patient with herbal mediation. The overall mortality rate was 22$\%$: 2 patients died of US complications, 2 patients died of sepsis, and 1 patient died of pulmonary hemorrhage. Group A (Hb 4.8${\pm}$1.2 g/dL) showed lower value in hemoglobin than group B (Hb 6.3${\pm}$1.7 g/dL) during hospital stay (P< 0.05), And the time interval between tile disease onset and dialysis treatment was significantly longer in group A ($11.9{\pm}9.1\;days\;vs\;2.8{\pm}2.1\;days$) (P< 0.05). Conclusion : Overall mortality rate was 22$\%$. Low hemoglobin value and the prolonged time interval between the disease onset and dialysis treatment were related with poor prognosis. So early diagnosis and appropriate intensive care including dialysis treatment is essential to achieve better outcome in children.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.78-86
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• 2001

Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.117-122
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• 1997

Purpose : To clarify the clinical findings, laboratory findings and disease course of EP complicated with CAPD and to find out possible predisposing factors to EP. Methods : The medical records of 34 children who underwent CAPD at our hospital between Jan. '94 and Dec. '96 were retrospectively reviewed. The clinical features and laboratory findings of EP were analyzed, and several parameters were evaluated as predisposing factors of EP. Results : EP developed in 7(21%) out of 34 patients. The major symptom of EP was turbid peritoneal fluid without fever, abdominal pain or disturbance of drainage in all cases. The microbiologic culture studies of the peritoneal fluid resulted negative in all cases. Patients with peripheral blood eosinophilia before insertion of CAPD catheter had higher risk of EP than those without eosinophilia (P=0.002). And peripheral blood eosinophilia, noted after insertion of hemodialysis catheter in cases with previous hemodialysis before CAPD, showed significant correlation with the occurrence of EP (P=0.016), too. However, there was no significant correlation between peripheral blood eosinophilia noted after insertion of CAPD cathter and the occurrence of EP. Identification of eosinophils in peritoneal fluids was more accurate with cytospin analysis. Conclusions : An early and accurate diagnosis of EP in patients with CAPD can prevent unnecessary treatment of antibiotics. Peripheral blood eosinophilia before insertion of CAPD catheter is one of the predisposing factors of EP. And, cytospin analysis of peritoneal fluid is an accurate method for diagnosis of EP.

• Journal of Nutrition and Health
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• v.31 no.9
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• pp.1422-1432
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• 1998

This study investigated the nutritional status of 32 CAPD patients. Their weight, height, triceps, mid arm circumference were measured and their dietary intake and the blood profiles were evaluated. They were 56.0${\pm}$11.8 years old. Their intake of energy and protein were 78% and 93% of RDA respectively. The energy from dextrose was 376.1${\pm}$83.2kcal. The men's and women's intake of protein was 84.6% and 102.1% of RDA respectively, According to the distribution of BMI, 23.5% of the men and 6.1% of the women were underweight and 5.9% of the men and 20% of the women were overweight. The serum albumin levels of the men and women were 3.30 and 3.71g/41 respectively. However, the average amount of serum ferritin, as a whole, comes within the normal range, which shows that stored iron was not decreased and that their anemia was not caused by iron deficiency. The subjects were divided into three groups according to the level of albumin, and their intakes of nutrients were compared with one another, The group with the high level of albumin showed that energy and protein intake was significantly larger and that BMI was also significantly higher than the other groups. There was a positive correlation between BMI and energy intake. Serum total protein had a positive correlation to energy intake ; hematocrit, to carbohydrate intake. BMI had a positive correlation to energy intake. A relative magnitude of factors affecting albumin level was analyzed by Stepwise multiple regression analysis. Overall results about relative influence of independent variables to dependent variable(albumin) indicated that the blood total protein(p <0.0001) was the most significantly correlated with serum albumin level in all subjects,1311owe4 by creatinine and total cholesterol. (Korean J Nutrition 31(9) : 1422-1432, 1998)

• 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.

• Journal of Microbiology and Biotechnology
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• v.15 no.1
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• pp.105-111
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• 2005

End-stage renal disease is a fatal and devastating disease that is caused by progressive and irreversible loss of functioning nephrons in the kidney. Dialysis and renal transplantation are the common treatments at present, but these treatments have severe limitations. The present study investigated the possibility of reconstructing renal tissues by transplantation of renal precursor cells to replace the current treatments for end-stage renal disease. Embryonic renal precursor cells, freshly isolated from metanephroi of rat fetus at day 15 post-gestation, were seeded on biodegradable polymer scaffolds and transplanted into peritoneal cavities of athymic mice for three weeks. Histologic sections stained with hematoxylin & eosin and periodic acid-Schiff revealed the formation of primitive glomeruli, tubules, and blood vessels, suggesting the potential of embryonic renal precursor cells to reconstitute renal tissues. Immunohistochemical staining for proliferating cell nuclear antigen, a marker of proliferating cells, showed intensive nuclear expression in the regenerated renal structures, suggesting renal tissue reconstitution by transplanted embryonic renal precursor cells. This study demonstrates the reconstitution of renal tissue in vivo by transplanting renal precursor cells with biodegradable polymer scaffolds, which could be utilized as a new method for partial or full restoration of renal structure and function in the treatment of end-stage renal disease.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.92-101
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• 2000

Purpose : We analyzed the demogaphic data md clinical course of Korean children with chronic renal failure (CRF) observed between 1990 and 1999. Patients and Methods : Questionnaires were mailed to all children's hospitals ail through the country. We asked for primary renal disease age and serum creatinine levels at first presentation with CRF and end-stage renal disease (ESRD), and modes of renal replacement therapy (RRT). Results : 401 children (254 boys, 147 girls) with CRF, defined as a permanent increase of serum creatinine above 1.2 mg/dl for at least 3 months or until death, were identified. This represents an incidence of 3.68 per million child population per year. Of these patients, 22$\%$ on younger than 5 years, 28$\%$ 5 to 10 years and 50$\%$ 10 to 15 year. Eight five $\%$ of the patients could be classified with a primary renal disease. The most frequent cause is glomerulonephritis (36$\%$), followed by chronic pyelonephritis (21$\%$), renal hrpo/dylplasia (9$\%$), and hereditary nephropathies (7$\%$). Reflux nephropathy (16$\%$) was the most common single cause of CRF. ESRD was reached in 70$\%$ of all patient. 99.3$\%$ of these started RRT. Hemodialysis (HD, 42$\%$), peritoneal dialysis (PD, 35$\%$) and transplantation (TP, 23$\%$) were performed as the initial mode of RRT. A total of 161 TPs were performed (159 first grafts, 2 second grafts). A total of 32 patients died. The main causes of death were dialysis related complication in HD patients and infections in PD patients. Survival rate on any form of RRT was 88.7$\%$ during the mean follow-up period of 37 months. Conclusion Major efforts should be directed toward earlier diagnosis and treatment of reflux nephropathy to prevent occurrence of Of. Dialysis and TP have now become well accepted forms of treatment in Korean children with ESRD.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.84-91
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• 2009

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene and G the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.