• Childhood Kidney Diseases
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• v.12 no.1
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• pp.23-29
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• 2008

Kidney allograft transplantation is the most effective method of renal replacement for end stage renal disease patients. Still, it is another kind of 'disease', requiring immunosuppression to keep the allograft from rejection(allograft immune reaction). Immune system of the allograft recipient recognizes the graft as a 'pathogen (foreign or danger)', and the allograft-recognizing commanderin-chief of adaptive immune system, T cell, recruits all the components of immune system for attacking the graft. Proper activation and proliferation of T cell require signals from recognizing proper epitope(processed antigen by antigen presenting cell) via T cell receptor, costimulatory stimuli, and cytokines(IL-2). Thus, most of the immunosuppressive agents suppress the process of T cell activation and proliferation.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.165-168
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• 2017

Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis.

• 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.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.67-76
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• 2019

Kidney disease is a major global health issue. Hemodialysis and kidney transplantation have been used in the clinic to treat renal failure. However, the dialysis is not an effective long-term option, as it is unable to replace complete renal functions. Kidney transplantation is the only permanent treatment for end-stage renal disease (ESRD), but a shortage of implantable kidney tissues limits the therapeutic availability. As such, there is a dire need to come up with a solution that provides renal functions as an alternative to the current standards. Recent advances in cell-based therapy have offered new therapeutic options for the treatment of damaged kidney tissues. Particularly, cell secretome therapy utilizing bioactive compounds released from therapeutic cells holds significant beneficial effects on the kidneys. This review will describe the reno-therapeutic effects of secretome components derived from various types of cells and discuss the development of efficient delivery methods to improve the therapeutic outcomes.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.94-99
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• 2010

Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index $35\;kg/m^2$) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin- converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.86-92
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• 2019

Steroid-resistant nephrotic syndrome (SRNS) has long been a challenge for clinicians due to its poor responsiveness to immunosuppressants, and rapid progression to end-stage renal disease. Identifying a monogenic cause for SRNS may lead to a better understanding of podocyte structure and function in the glomerular filtration barrier. This review focuses on genes associated with slit diaphragm, actin cytoskeleton, transcription factors, nucleus, glomerular basement membrane, mitochondria, and other proteins that affect podocyte biology.

• Journal of Dental Rehabilitation and Applied Science
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• v.40 no.2
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• pp.46-54
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• 2024

Purpose: The purpose of this study was to compare the periodontal status of end-stage renal disease patients undergoing dialysis and referred for intraoral evaluation prior to renal transplantation surgery with those having normal kidney function. Materials and Methods: Patients who had been undergoing dialysis for end-stage renal disease and been referred to the Dental Clinic Center by the Department of Nephrology at University Hospital for intraoral evaluation prior to kidney transplantation surgery. For comparison of periodontal status, subjects without abnormalities in kidney function were matched with the patients by age and gender and selected as healthy controls. The patients' age, gender, comorbidities, type of dialysis received, and duration of dialysis were investigated by reference to their medical records, and data on their periodontal status were analyzed via the relevant periodontal records. Results: A total of 102 patients, including 51 dialyzed patients and 51 healthy control group subjects, participated in this study. In the patients with end-stage renal disease undergoing dialysis with periodontal probing depth of 5 mm or more, percentage of sites with clinical attachment level of 4 mm or more, percentage of teeth with bleeding on probing, number of missing teeth, and ratio of moderate to severe periodontitis were all significantly greater than in the healthy controls. Conclusion: The periodontal status of end-stage renal disease patients undergoing dialysis and referred for intraoral evaluation prior to kidney transplantation was worse than that of healthy controls.

• Clinical and Experimental Pediatrics
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• v.54 no.8
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• pp.317-321
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• 2011

Children who suffer from steroid-resistant nephrotic syndrome (SRNS) require aggressive treatment to achieve remission. When intravenous high-dose methylprednisolone fails, calcineurin inhibitors, such as cyclosporine and tacrolimus, are used as the first line of treatment. A significant number of patients with SRNS progress to end-stage renal disease if remission is not achieved. For these children, renal replacement therapy can also be problematic; peritoneal dialysis may be accompanied by significant protein loss through the peritoneal membrane, and kidney allograft transplantation may be complicated by recurrence of SRNS. Plasmapheresis and rituximab were initially used for treatment of recurrent SRNS after transplantation; these are now under consideration as rescue therapies for refractory SRNS. Although the prognosis of SRNS is complicated and unfavorable, intensive treatment in the early stages of the disease may achieve remission in more than half of the patients. Therefore, timely referral of pediatric SRNS patients to pediatric nephrology specialists for histological and genetic diagnosis and treatment is highly recommended.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.51-56
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• 2004

Hypertensive encephalopathy is an acute neurologic syndrome that occurs in association with abrupt and marked elevation of blood pressure and is characterized by headache, vomiting, seizure, visual disturbances and altered mental status. Hypertensive encephalopathy is most commonly associated with renal disease in children, including acute glomerulonephritis, reno-vascular hypertension, and end-stage renal disease. Hypertensive encephalopathy associated with nephrolithiasis has not been reported. We have experienced a 10-year-old boy with hypertensive encephalopathy associated with ureteral stone.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.232-239
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• 2002

Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with renal cystic diseases in the Department of Pediatrics, Asan Medical Center from October 1989 to June 2001. Results : In 95 patients, there were 55 cases(58.0%) with multicystic dysplastic kidney(MCDK), 19 cases(20.0%) with simple renal cysts, 13 cases(13.7%) with hereditary polycystic kidney diseases( 7 with autosomal recessive type, 5 with autosomal dominant type, 1 with undetermined), 6 cases(6.3%) with renal cysts in tuberous sclerosis and 1 case(1.0%) with medullary cystic disease. All MCDK patients had no renal dysfunction and hypertension during the follow-up period. Three out of 13 with polycystic kidney diseases had progressed to end-stage renal disease during the follow-up period. One case with a simple cyst underwent laparoscopic malsupialization for decompression. Conclusion : Renal cystic diseases have diverse clinicopathologic features and variable prognosis. We emphasize that routine follow-up should be performed to prevent and to detect early treatable complication in renal cystic diseases. Therefore, their natural history and treatment need further investigation and long term follow-up is required.