• Biomolecules & Therapeutics
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• v.29 no.4
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• pp.365-372
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• 2021

Type 2 diabetes mellitus (T2DM) leads to many health problems like diabetic nephropathy (DN). One of the key factors for chronic kidney disease and end-stage renal disease (ESRD) is T2DM. Extensive work is being done to delineate the pathogenesis of DN and to extend possible remedies. This review is intended to understand the nature of DN risk factors, progression, effects of glycemic levels, and stages of DN. We also explored the novel diagnostic and therapeutic approaches for DN such as gene therapy and stem cell treatments.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.125-132
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• 2003

Purpose : Lipoprotein(a) is a genetically determined risk factor for atherosclerotic vascular disease and is elevated in patients with renal disease. Especially the patients with nephrotic syndrome exhibit excessively high Lp(a) plasma concentrations. Also the patients with end-stage renal disease have elevated Lp(a) levels. But the mechanism underlying this elevation is unclear. Thus, in this study, by measuring the level of serum Lp(a) in common renal diseases in children, we hoped to see whether there would be a change in Lp(a) in renal diseases other than nephrotic syndrome. Then, we figured out its implications, and looked for the factors that affect the Lp(a) concentrations. Methods : A total of 75 patients(34 patients with hematuria of unknown etiology, 10 with hematuria and hypercalciuria, 8 with IgA nephropathy, 8 with poststreptococcal glomerulone phritis, 3 with $Henoch-Sch\"{o}nlein$ nephritis, 7 with urinary tract infection, and 5 with or- thostatic proteinuria) were studied. The control group included 20 patients without renal and liver disease. Serum Lp(a), total protein, and albumin levels, 24-hour urine protein and calcium excretions, creatinine clearance and the number of RBCs and WBCs in the urinary sediment were evaluated. Data analysis was peformed using the Student t-test and a P-value less than 0.05 was considered to be statistically significant. Results : LP(a) was not correlated with 24-hour urine calcium and creatinine. Lp(a) level had a positive correlation with proteinuria and negative correlation with serum albumin and serum protein. Among the common renal diseases in children, Lp(a) was elevated only in orthostatic proteinuria (P<0.05). Conclusion : Lp(a) is correlated with proteinuria, serum protein, and serum albumin, but not with any kind of specific renal disease. Afterward, Lp(a) needs to be assessed in patients with orthostatic proteinuria and its possible role as a prognostic factor could be confirmed.

• 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.15 no.2
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• pp.125-137
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• 2011

Purpose : Men are generally more prone to chronic renal disease and progression to end stage renal disease than women. The purpose of this study is to prove the effect of gender and sex hormone on renal fibrosis in mice with unilateral ureteral obstruction (UUO) and to elucidate the specific underlying mechanisms. Methods :We compared the expression of ${\alpha}$-smooth muscle actin (${\alpha}$-SMA) in female and male mice with complete UUO (day 7). After this, we estimated the changes of renal fibrosis in the female mice with oophorectomy and in the female mice with oophorectomy and replacement of $17{\beta}$-estradiol, respectively. Results : The level of ${\alpha}$-SMA in the female kidney with UUO was significantly lower than that in the male kidney with UUO. oophorectomy and replacement of $17{\beta}$-estradiol did not change the expression of angiotensin II type 1 (AT1) receptor in the female kidney with UUO, whereas the expression of angiotensin II type 2 (AT2) receptor was significantly more elevated in the intact female (IF) and the oophorectomized female with estrogen (OF+E) than that in the oophorectomized female (OF). The expressions of inducible nitric oxide synthase (iNOS) in the IF and OF+E mice were significantly more elevated than that in the OF mice, which was similar to the expression of AT2 receptor. Conclusion : The female gender is associated with resistance to renal fibrosis in obstructive uropathy and this gender difference may originate from the existence of $17{\beta}$-estradiol, which has an anti-fibrotic effect via upregulation of the AT2 receptor and iNOS.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.43-50
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• 2001

Purpose : The urinary tract infection associated with vesicoureteral reflux(VUR) in children may result in serious complications such as renal scarring, hypertension, proteinuria and end stage renal disease. The purpose of this study was to evaluate the factors affecting renal scar such as age, gender, grade of VUR, and ACE gene polymorphism, and body growth in the patients with and those without renal scar associated with VUR Methods : During the period from January 1994 to July 2000, We had 93 children with urinary tract infection associated with VUR who were admitted to the Department of pediatrics of Chonbuk National University Hospital. The patients were divided into two groups according to follow up 99mTc-DMSA renal scan; patients with renal scar group and those with non-scar group. We analyzed and compared the factors associated with renal scarring between the two groups. Results : There were no significant difference in gender, causative organism, ACE gene polymorphism, height and weight at diagnosis between renal scar group and non-scar group. Fifty four patients were in renal scar group and forty seven of them had VUR. The age at diagnosis was significantly higher in renal scar group (2.48${\pm}$2.64yr) than in non renal scar group (1.26${\pm}$1.83yr). Especially, the infants who were less than 1 year of age with VUR developed relatively more renal scar compared with infants older than 1 tear of age. The incidence of renal scarring showed a direct correlation with the severity of VUR. Conclusion : The factors affecting renal scar formation were age at diagnosis, presence and grade of VUR, but the other factors such as gender, causative organism, ACE gene polymorphism were not associated with renal scarring. Therefore, further evaluation about uropathogenic E coli and foflow up study about body growth associated with severity of renal scar would be necessary. (J. Korean Soc Pediatr Nephrol 5 : 43- 50, 2001)

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.10-21
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• 2010

The overall prognosis of Henoch-Schoenlein purpura (HSP) is favorable, but severe nephritis has a high risk of progression to end stage renal failure. Recent studies emphasize the importance of early treatment in children with severe HSP nephritis, but the treatment of severe HSP nephritis still remains controversial due to the rarity of randomized controlled studies in this field. Nevertheless, several intensive therapies, such as intravenous high-dose methylprednisolone pulse, immunosuppressive/cytotoxic drugs, fibrinolytic therapy, anticoagulants, antiplatelet agent and plasma exchange, have been used in children with severe HSP nephritis. In this review, we focus on the treatment of severe HSP nephritis in children.

• Childhood Kidney Diseases
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• v.28 no.2
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• pp.51-58
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• 2024

Patients with chronic kidney disease (CKD) bear a significant financial burden and face numerous complications and higher mortality rates. The progression of CKD is associated with glomerular injury caused by glomerular hyperfiltration and oxidative stress. Factors such as uncontrolled hypertension, elevated urine protein levels, anemia, and underlying glomerular disease, contribute to CKD progression. In addition to conservative treatment, several medications are available to combat the progression of CKD to end-stage kidney disease. Renin-angiotensin-aldosterone system blockers could slow the progression of CKD by reducing glomerular hyperfiltration, lowering blood pressure, and decreasing inflammation. Mineralocorticoid receptor antagonists inhibit the mineralocorticoid receptor signaling pathway, thereby attenuating inflammation and fibrosis. Sodium-glucose cotransporter 2 inhibitors exhibit protective effects on the kidneys and against cardiovascular events. Tolvaptan, a selective vasopressin V2-receptor antagonist, decelerates the rate of increase in total kidney volume and deterioration of kidney function in patients with rapidly progressive autosomal dominant polycystic kidney disease. The protective effects of AST-120 remain controversial. Due to a lack of evidence regarding the efficacy and safety of these medications in children, it is imperative to weigh the benefits and adverse effects carefully. Further research is essential to establish the efficacy and safety profiles in pediatric populations.

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

Type 1 diabetes mellitus commonly occurs in childhood and adolescence, although the prevalence of type 2 diabetes mellitus in these age groups is now being increased in the western world and Korea. Diabetic nephropathy developing in 15-25% of subjects with type 1 diabetes mellitus and in similar or higher percentage of type 2 diabetes mellitus patients is the leading cause of end-stage renal disease worldwide. Although prepubertal diabetic duration may contribute less to the development of microvascular complications than pubertal and postpubertal duration, diabetic nephropathy in susceptible patients almost certainly begins soon after disease onset and may accelerate during adolescence, leading to microalbuminuria or incipient DN. Type 1 diabetes is commonly associated with a period of hyperfiltration followed by the development of persistent microalbuminuria after as little as 7-10 years of type 1 diabetes. Microalbuminuria is associated with pathologic lesions that are so advanced as to overlap with those seen in patients with overt proteinuria and declining kidney function, therefore, microalbuminuria currently considered the best clinical indicator of overt diabetic nephropathy risk. This review covers the natural history and renal manifestations of diabetic nephropathy in children and adolescents.

• Clinical and Experimental Pediatrics
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• v.57 no.3
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• pp.135-139
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• 2014

Purpose: Adult Korean patients on chronic dialysis have a 9-year survival rate of 50%, with cardiovascular problems being the most significant cause of death. The 2011 annual report of the North American Pediatric Renal Trials and Collaborative Studies group reported 3-year survival rates of 93.4% and relatively poorer survival in younger patients. Methods: In this study, we have reviewed data from Korean Pediatric Chronic Kidney Disease Registry from 2002 to 2010 to assess survival rates and causes of death in Korean children on chronic dialysis. Results: The overall estimated patient survival rates were 98.4%, 94.4%, and 92.1% at 1, 3, and 5 years, respectively. No significant difference was observed in survival rates between patients on peritoneal dialysis and those on hemodialysis. Patients for whom dialysis was initiated before 2 years of age (n=40) had significantly lower survival rates than those for whom dialysis was initiated at 6-11 years of age (n=140). In all, 26 patients had died; the mortality rate was 19.9 per 1,000 patient years. The most common causes of death were infections and comorbidities such as malignancy and central nervous system (CNS) or liver diseases. Conclusion: The outcomes observed in this study were better than those observed in adults and comparable to those observed in pediatric studies in other countries. To improve the outcomes of children on chronic dialysis, it is necessary to prevent dialysis-related complications such as infection, congestive heart failure, or CNS hemorrhage and best control treatable comorbidities.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.75-82
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• 2020

Objectives: We aimed to investigate the incidence, manifestations, and outcomes of malignancy after pediatric kidney transplantation (KT) at our center over 30 years. Methods: We retrospectively reviewed the medical records of 155 patients under 18 years of age who underwent KT between January 1990 and February 2020 at Asan Medical Center. Results: Twelve patients (7.7%) were diagnosed with a malignancy after KT. Malignancy was diagnosed after a mean period of 6.4±5.9 years (median 4.6, range 0.5-20.6 years) after KT. Nine (75.0%) of the 12 cancer patients were diagnosed with post-transplant lymphoproliferative disease (PTLD), and the other three had papillary thyroid cancer, mucoepidermoid cancer of the hard palate, and T-cell acute lymphoblastic leukemia, respectively. PTLD was diagnosed within a mean of 3.7±3.4 years (median 3.7, range 0.5-9.8 years) after KT. Five patients diagnosed with PTLD were cured without recurrence. Three patients with PTLD died from the disease, and one patient with mucoepidermoid cancer from a non-PTLD malignancy died after progression, despite surgical resection and chemotherapy. Three (33.3%) of the nine survivors progressed to end-stage renal disease (ESRD) after completing cancer treatment. No patient with post-transplant malignancy (PTM) experienced critical renal deterioration during cancer treatment. Conclusion: PTLD was the most common PTM, occurring at 5.8% of the pediatric KT patients after KT in our center. Careful follow up is needed particularly considering the risk of PTLD after KT in children.