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RTX administration was safe and effective in patients with difficult-to-treat NS. One or 2 doses of RTX may be sufficient to deplete B cells and achieve better control of pediatric NS.

<i>HNF1B</i> mutations, one of the most common causes of congenital anomalies of the kidney and urinary tract, manifest as various renal and extrarenal phenotypes. We analyzed the genotype-phenotype correlations in 14 pediatric patients with <i>HNF1B</i> mutations. Genetic studies revealed total gene deletion in six patients (43%). All patients had bilateral renal abnormalities, primarily multiple renal cysts. Twelve patients exhibited progressive renal functional deterioration, and six of them

Congenital anomalies of the kidney and urinary tract (CAKUT) are the most common cause of chronic kidney disease in children. The search for genetic causes of CAKUT has led to genetic diagnosis in approximately 5-20 % of CAKUT patients from Western countries. In this study, genetic causes of CAKUT in Korean children were sought using targeted exome sequencing (TES) of 60 genes reported to cause CAKUT in human or murine models. We identified genetic causes in 13.8% of the 94 recruited patients. P

The strategy against tumour lysis syndrome (TLS) had been hyperhydration, urine alkalinization, and allopurinol. Recently, rasburicase was added to the armament against this life-threatening condition. In Korea, rasburicase is used as a rescue therapy for cases with allopurinol-resistant hyperuricemia, because of the restriction by the National Health Insurance. We reviewed our experiences to re-assess the risk factors of TLS and the efficacy of rasburicase. Medical records were retrospectively

Several urine biomarkers were significantly different between AKI and no AKI groups, and some had changed before the onset of AKI. These groups were distinct according to causative factors of AKI and GA. Urine biomarkers could be useful for monitoring the development of AKI in premature infants.

Rituximab (RTX), anti-CD20 monoclonal antibody, has been proposed as a rescue therapy for refractory nephrotic syndrome (NS) on the basis of favorable clinical observations. While reported efficacy of RTX on refractory RTX is promising, the long-term effect obtained from randomized clinical trial is limited, let alone the long-term safety profile of RTX in these patients. To obtain solid evidence of efficacy and safety of this medication, we conducted a clinical trial to evaluate the efficacy an

A retrospective review was performed to assess the risk factors and outcomes of BK virus infection and nephropathy (BKVN), an early complication in pediatric kidney allograft recipients. The study investigated the incidence, risk factors, and clinical outcomes of BK viremia and BKVN in a Korean population of pediatric patients who received renal transplantation from 2001-2015 at the Seoul National University Hospital. BKVN was defined as biopsy-proven BKVN or plasma BK viral loads >10,000 copies

Key message• Although the basic concept of hemodialysis (HD) is similar in adults and children, specific factors must be considered in the latter, including the small dialyzer and circuit, difficult vascular access, and frequent complications.• HD-associated complications include catheter-related problems, hemodynamic instability, and neurodevelopmental and cognitive dysfunction.• Pediatric HD is challenging, and steady efforts are needed to perform it safely and reduce its complications, thereb

<b>Objectives:</b> Gordon syndrome (GS), also known as pseudohypoaldosteronism type II, is a rare tubular disease characterized by hypertension, hyperkalemia, and metabolic acidosis. Its causative genes are <i>CUL3</i>, <i>KLHL3</i>, <i>WNK1</i>, and <i>WNK4</i>, and they are associated with varying severity of the disease. Herein, we report the first case of GS caused by a <i>CUL3</i> mutation in a patient with short stature in Korea.<b>Case presentation:</b> A 7-year-old boy had hypertension,

Bioimpedance spectroscopy (BIS) is a noninvasive method used to evaluate body fluid volume status in dialysis patients, but reports on its effectiveness in pediatrics are scarce. We investigated the correlation between BIS and clinical characteristics and identified the changes in patients whose dialysis prescription was modified based on BIS. The medical records of children on maintenance dialysis who had undergone BIS between 2017 and 2019 were reviewed. Of the 49 patients, 14 were overhydrate

Chronic kidney disease (CKD) in children is associated with various complications, including poor growth and development, mineral bone disorder, cardiovascular disease, kidney failure, and mortality. Slowing down the progression of CKD is important since CKD is often not curable. Prospective cohort studies have been conducted to understand the progression and outcomes of CKD in children, and these studies have identified non-modifiable and modifiable risk factors. Recognition of known risk facto