PURPOSE: The aims of this study were to assess the clinical and laboratory profiles of chronic kidney disease-mineral bone disorder (CKD-MBD) and to assess the effects of treatment of active vitamin D analogs on severe hyperparathyroidism (SHPT) in pediatric patients on chronic peritoneal dialysis. METHODS: This is a retrospective study included 53 patients who had been undergoing dialysis for more than 1 year, between January 2003 and December 2012. RESULTS: Even after treatment with phosphate binders and active vitamin D analogs, the mean+/-standard deviation of the percentage of time during peritoneal dialysis that the patients' serum concentrations of phosphorus, corrected total calcium, and parathyroid hormone (PTH) fell within the Kidney Disease Outcomes Quality Initiative recommended ranges was 25.06+/-17.47%, 53.30+/-23.03%, and 11.52+/- 9.51%, respectively. Clinical symptoms or radiological signs of CKD-MBD were observed in 10 patients (18.9%). There were significant differences in percentage of time that the serum intact PTH concentration was outside of the recommended range between patients with and without symptoms or signs of CKD-MBD (below recommended range, 11.74+/-7.37% vs. 40.77+/-25.39%, P<0.001; above the recommended range, 63.79+/-27.86% vs. 37.09+/-27.76%, P=0.022). Of the 25 patients with SHPT, high-dose alfacalcidol treatment was required in 13 patients that controlled SHPT in 7 of these patients, without marked complications. CONCLUSION: Despite our efforts to manage CKD-MBD, patients' met the recommended ranges from relevant guidelines at a low frequency. The treatment of high-dose active vitamin D analogs was required in about half of the patients with SHPT and effective in about half of them.
Calcium ; Child* ; Dialysis ; Humans ; Hyperparathyroidism* ; Kidney Diseases ; Kidney* ; Parathyroid Hormone ; Peritoneal Dialysis* ; Phosphorus ; Retrospective Studies ; Vitamin D*

A number of kidney diseases of childhood may present as isolated proteinuria or/and hematuria, without any overt signs or symptoms. Urinalysis is a simple and inexpensive test used to evaluate various renal disorders. A school urine screening (SUS) program for kidney disease was conducted in Korea in 1998. Several research reports, including case reports and systemic reviews of SUS data, claimed that early detection and confirmatory diagnosis by renal biopsy seems to be helpful for determining the prognosis and intervention of progressive chronic renal disease. However, there is no global consensus as to whether screening for chronic kidney disease (CKD) should be undertaken in children and adolescents. This paper reviews the SUS for CKD in Korea, including the history and structure of the program, its assessment, related research, and associated problems.
Adolescent ; Biopsy ; Child ; Consensus ; Diagnosis ; Hematuria ; Humans ; Kidney Diseases ; Korea ; Mass Screening* ; Prognosis ; Proteinuria ; Renal Insufficiency, Chronic ; Research Report ; Urinalysis

Tuberous sclerosis complex (TSC) is a genetic disorder that affects multiple organ systems and causes tumors. It is important that physicians are aware of the manifestations of TSC, and that they follow the recommendations for screening and evaluation. Several types of renal abnormalities may develop in individuals with TSC. Individuals with TSC may require ongoing treatment that can be adapted for each arising manifestation of renal disease. Herein, we report 4 patients with TSC who presented with a range of different renal manifestations, including angiomyolipoma, renal cell carcinoma, renal infarction, renal cyst, and nephrolithiasis.
Angiomyolipoma ; Carcinoma, Renal Cell ; Child* ; Humans ; Infarction ; Mass Screening ; Nephrolithiasis ; Tuberous Sclerosis*

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.
Antigen-Antibody Complex ; Autoantibodies ; Biopsy ; Child ; Fluorescent Antibody Technique ; Follow-Up Studies ; Hematuria ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Male ; Prognosis ; Proteinuria ; Urinalysis

Erythema multiforme (EM) is an acute mucocutaneous disorder involving the skin, mouth, eyes, and genital organs. It is classified into EM minor and EM major according to the involvement of the mucosal membrane. Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) belong to EM major. Compared to EM minor, SJS presents with more severe and progressive symptoms, and has a higher mortality rate. Corticosteroids are used in the treatment of EM. We report three cases of EM (two cases of EM minor and one case of SJS) that developed during treatment with oral corticosteroid (deflazacort; Calcort(R)) in children with nephrotic syndrome.
Adrenal Cortex Hormones ; Child* ; Erythema Multiforme* ; Genitalia ; Humans ; Membranes ; Mortality ; Mouth ; Necrosis ; Nephrotic Syndrome* ; Skin ; Stevens-Johnson Syndrome

Renal and perinephric abscesses are a rare but potentially fatal complication of urinary tract infection (UTI). Diagnosing renal and perinephric abscesses has been known to be difficult. These abscesses may occur when the appropriate antibiotic treatment for a UTI is delayed, or in cases with a congenital malformation of the urinary system, especially in children. In the present report, we describe 2 cases of renal abscesses with extra-capsular invasion in children with febrile UTI. A 4-month-old male infant with vesicoureteral reflux developed a renal abscess that infiltrated the perinephric area and the left psoas muscle, despite early antibiotic treatment. A 9-year-old boy with prolonged fever also showed a multi-loculated renal abscess that infiltrated the spleen and diaphragm. Both patients were successfully treated with appropriate antibiotics and percutaneous drainage.
Abscess* ; Anti-Bacterial Agents ; Child* ; Diaphragm ; Drainage ; Fever ; Humans ; Infant ; Male ; Psoas Muscles ; Spleen ; Urinary Tract Infections ; Vesico-Ureteral Reflux

Hyperkalemia is often detected in young infants, particularly in association with acute pyelonephritis or a urinary tract anomaly. Cases of hyperkalemia in this population may also be due to transient pseudohypoaldosteronism, or immaturity of renal tubules in handling potassium excretion. Symptoms of hyperkalemia are non-specific, but are predominantly related to skeletal or cardiac muscle dysfunction, and can be fatal. Therefore, treatment has to be initiated immediately. Administration of fludrocortisone for hyperkalemia is appropriate in cases with hypoaldosteronism, but is challenging in young infants with hyperkalemia due to renal tubular immaturity, without pseudohypoaldosteronism. We report the case of a 25-day-old male presenting with persistent hyperkalemia with normal serum aldosterone, who was admitted with a first episode of pyelonephritis and unilateral high-grade vesicoureteral reflux. The patient was treated successfully with fludrocortisone.
Aldosterone* ; Fludrocortisone ; Humans ; Hyperkalemia* ; Hypoaldosteronism ; Infant ; Male ; Myocardium ; Potassium ; Pseudohypoaldosteronism ; Pyelonephritis* ; Urinary Tract ; Vesico-Ureteral Reflux

Congenital thoracic ectopic kidney is a very rare developmental disorder and the rarest type of ectopic kidney. This condition is usually asymptomatic and detected incidentally on routine chest radiography. Most cases of thoracic ectopic kidney develop in adulthood and during the neonatal period, and congenital thoracic ectopic kidney rarely develops in children. Most patients are asymptomatic, and the treatment depends on the diagnosis. Herein, we report a rare case of ectopic thoracic kidney associated with a diaphragmatic hernia in a 15-month-old male infant, who presented with periodic severe irritability. The thoracic ectopic kidney was detected as a mass in the right base of the chest on routine chest radiography.
Child ; Diagnosis ; Hernia, Diaphragmatic* ; Humans ; Infant* ; Kidney* ; Male ; Radiography ; Thorax

PURPOSE: Nafamostat mesilate (NM), a synthetic serine protease inhibitor, has been investigated as an anticoagulant for adult patients with a high risk of bleeding, who need chronic renal replacement therapy (CRRT). However, little is known about the use of NM as an anticoagulant in pediatric CRRT. The aim of this study was to evaluate the ideal dosage, efficacy, and safety of NM in pediatric CRRT. METHODS: We conducted a retrospective study of 40 pediatric patients who had undergone at least 24 h of venovenous CRRTs between January 2011 and October 2013. We divided the patients according to risk of bleeding. Those at high risk received no anticoagulation (group 1) or NM as an anticoagulant (group 2), while those at low risk received heparin (group 3). RESULTS: Forty patients (25 male and 15 female; mean age, 8.2+/-6.6 years) were enrolled. The mean duration of CRRT was 13.0 days, and the survival rate was 57.5%. The mean hemofilter lifespan was 39.3 h in group 1 and 11.3 h in group 3. In group 2, hemofilter lifespan was extended from 7.5 h to 27.4 h after the use of NM (P=0.001). The mean hemofilter lifespan with NM was greater than with heparin (P=0.018). No patient experienced a major bleeding event during treatment with NM. CONCLUSION: NM may be a good alternative anticoagulant in pediatric patients with a high risk of bleeding requiring CRRT, and is not associated with bleeding complications.
Adult ; Child* ; Female ; Hemorrhage* ; Heparin ; Humans ; Male ; Mesylates* ; Renal Replacement Therapy* ; Retrospective Studies ; Serine Proteases ; Survival Rate

PURPOSE: Bisphosphonates are widely used for the management steroid-induced osteoporosis (SIO) in children. With the increasing use of bisphosphonates, there have been reports of abnormal radiological findings in the growing skeleton. Therefore, their use in pediatric patients remains controversial. The present study was conducted to evaluate the long-term follow-up radiographic features, particularly metaphyseal sclerotic lines, in children who receive pamidronate therapy for nephropathy. METHODS: Twenty-four children with nephropathy treated with oral calcium and pamidronate (mean duration, 9 months; dose, 100 mg daily), were evaluated retrospectively. All patients had SIO secondary to chronic glucocorticoid therapy for treating nephropathy. Long bone radiographic imaging was performed before treatment with pamidronate, and at follow-up, several years later. Physeal growth rates were estimated by measuring the distance that the sclerotic lines moved on the radiographs during the corresponding time intervals. RESULTS: The mean follow-up period was 138 months. Long bone radiographs showed well-defined sclerotic lines at the metaphyseal ends, progressively moving from the physeal plate to the diaphysis, in all patients. The mean rate of movement of the sclerotic line was 6.21 mm per year. In 12 patients, the lines disappeared. The mean rate of growth in height was 7.33 cm per year. CONCLUSIONS: Results of long-term follow-up suggest that the metaphyseal sclerotic lines associated with pamidronate treatment tend to disappear without affecting overall skeletal growth. Bisphosphonate treatment for SIO in children with nephropathy seems to be safe, although further studies in larger number of patients are needed.
Calcium ; Child* ; Diaphyses ; Diphosphonates ; Follow-Up Studies* ; Humans ; Osteoporosis ; Retrospective Studies ; Skeleton