Pediatric kidney disease has a relatively lower prevalence than do other pediatric conditions and has a notably different etiology from kidney diseases observed in adults. Furthermore, the pediatric population is unique in that they experience ongoing growth and development, distinguishing them from adult patients. Consequently, pediatric patients with kidney disease require more specialized and meticulous nutritional management than do adults. To address this need and promote optimal dietary practices for pediatric patients with kidney disease, pediatric nephrologists from the Korean Society of Pediatric Nephrology and nutritionists from the Korean Society of Clinical Nutrition have collaborated to establish nutritional guidelines specifically tailored to Korean dietary patterns. These guidelines offer detailed, nutrient-specific recommendations covering energy, protein, calcium, phosphorus, and potassium consumption while providing practical, culturally relevant guidance intended to support both pediatric patients and their caregivers.

Pediatric kidney disease has a relatively lower prevalence than do other pediatric conditions and has a notably different etiology from kidney diseases observed in adults. Furthermore, the pediatric population is unique in that they experience ongoing growth and development, distinguishing them from adult patients. Consequently, pediatric patients with kidney disease require more specialized and meticulous nutritional management than do adults. To address this need and promote optimal dietary practices for pediatric patients with kidney disease, pediatric nephrologists from the Korean Society of Pediatric Nephrology and nutritionists from the Korean Society of Clinical Nutrition have collaborated to establish nutritional guidelines specifically tailored to Korean dietary patterns. These guidelines offer detailed, nutrient-specific recommendations covering energy, protein, calcium, phosphorus, and potassium consumption while providing practical, culturally relevant guidance intended to support both pediatric patients and their caregivers.

Here, we present the case of a 2-month-old male infant with hyponatremic hypertensive syndrome resulting from stenosis of the right proximal and mid-renal arteries. The patient exhibited nephrotic-range proteinuria, low serum albumin, increased serum creatinine, and elevated renin and aldosterone levels. Doppler ultrasonography and computed tomography angiography revealed decreased vascular flow in the small right renal artery. Following a successful percutaneous balloon angioplasty, the patient experienced a decrease in blood pressure and normalization of serum electrolyte levels within a few days. However, it took 3 months for the proteinuria to resolve completely. This case is significant as it represents the first reported instance of a neonate presenting with clinical features resembling congenital nephrotic syndrome caused by renal artery stenosis that was successfully treated with percutaneous renal angioplasty.

C1q nephropathy is a rare glomerulopathy that typically presents with nephrotic syndrome in children. Treatment with immunosuppressive agents renders patients vulnerable to infection and its complications. Gastroenteritis is common in children, and rotavirus is a leading cause. Extraintestinal manifestations of rotavirus have recently been reported; however, there is a paucity of cases exploring the involvement of a rotavirus on the respiratory system. Acute respiratory distress syndrome (ARDS) is a rapid onset respiratory failure characterized by noncardiogenic pulmonary edema and hypoxemia. Causes of ARDS include sepsis, pneumonia, pancreatitis, aspiration, and trauma. In this paper, we report a case of ARDS after rotavirus infection in a child with C1q nephropathy who had been treated with immunosuppressive agents.

Chronic kidney disease (CKD)-mineral and bone disorder (CKD-MBD) is a common complication of CKD, often accompanied by extra-skeletal calcification in adult patients. As increased vascular calcification is predicted to increase cardiovascular mortality and morbidity, the revised Kidney Disease: Improving Global Outcomes guidelines recommend avoiding calcium-containing phosphate chelators. However, extra-skeletal calcification is less commonly noticed in pediatric patients. Here, we report our experience of such a complication in pediatric patients receiving maintenance peritoneal dialysis. Extra-skeletal calcification was noticed at the corneas, pelvic cavity, and soft tissues of the lower leg in 4 out of 32 patients on maintenance peritoneal dialysis. These patients experienced the aggravation of extra-skeletal calcifications during peritoneal dialysis, and 2 of them underwent excisional operations. It is required to monitor extra-skeletal calcifications in children on kidney replacement therapy.

Purpose: Nephrotic syndrome (NS) is the most common form of glomerulopathy in children. Most pediatric patients respond to glucocorticosteroid treatment (steroid-sensitive NS, SSNS), while approximately 10–15% will remain unresponsive or later become steroid-resistant. There has been a long-standing effort to find biomarkers that may predict steroid responsiveness. Methods: We systematically reviewed current studies which investigated clinically relevant biomarkers for predicting steroid responsiveness in pediatric NS. We performed a PubMed and EMBASE search to identify eligible articles. We collected data on urinary markers, blood/serum markers (including cellular phenotypes and mRNA expression), genotypes and HLA allele frequency. Results: A total of 659 articles were identified following electronic and manual searches. After reviewing the titles, abstracts, and full texts, 72 eligible articles were finally included. Vitamin D-binding protein (VDBP) seemed to be significantly elevated in SRNS than in SSNS, in both serum and urine specimen, although further validation is required. Conclusions The present paper narratively illustrates current understandings of potential biomarkers that may help predict steroid responsiveness. Further investigation and collaboration involving a larger number of patients are necessary.

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular and lymphatic vessels. A 15-year-old boy was initially diagnosed with Gorham-Stout at the age of 8 years based on clinical and radiological findings. Following diagnosis, he was treated with pamidronate, interferon alfa, propranolol, oral corticosteroids, and sirolimus. He developed proteinuria at the age of 15 and progressed into the nephrotic range 2 years later. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified variant. The sequential increase in proteinuria associated with medications suggested that the focal segmental glomerulosclerosis may be caused by pamidronate and sirolimus, but cannot completely rule out the possibility of kidney involvement of GSS itself.

Background: Hearing loss (HL) in children may adversely affect their development. HL is more prevalent in patients with chronic kidney disease (CKD) than in the general population.This study evaluated the prevalence of HL and its underlying diseases in patients with childhood-onset in CKD. Methods: In this retrospective study of a tertiary referral center, childhood-onset CKD patients (stage 2–5, age at onset of renal symptom < 18 years) were recruited. We referred to the “renal” syndromic HL as cases with genetic or syndromic diseases, or extra-renal anomalies in addition to HL and CKD. Results: A total of 421 patients (male:female = 279:142) were reviewed according to the causes of CKD: congenital anomalies of the kidney and urinary tract (CAKUT; n = 184, 43.7%), glomerulopathies (GP; n = 105, 24.9%), cystic kidney diseases (CYST; n = 39, 9.3%), perinatal problems (PP; n = 29, 6.9%), and others (n = 64, 15.2%). HL was detected in 82 (19.5%) patients, including 51 (12.1%) patients with sensorineural hearing loss (SNHL), 30 (7.1%) with conductive hearing loss (CHL), and 1 patient with mixed HL. The prevalence of HL in each group was as follows: 16.8% in the CAKUT group, 28.6% in the GP group, 12.8% in the CYST group, 24.1% in the PP group, and 14.1% in the others group. HL was more common in higher CKD stages, especially CHL in end-stage renal disease. SNHL was more prevalent in CKD from GP. Of the 82 patients with HL, 50% had renal syndromic HL: 58.8% of SNHL and one-third of CHL were renal syndromic HL. Conclusion One-fifth of the childhood-onset CKD had HL. Collectively, renal syndromic HL comprised half of the HL in this study. To improve the quality of life in patients with childhood-onset CKD, we suggest that HL should be considered, requiring surveillance, and if necessary, early intervention.

PURPOSE: The quality of bowel preparation is a major determinant of the quality of colonoscopy. This study evaluated lifestyle factors, including usual dietary style, associated with bowel preparation. METHODS: This retrospective study evaluated 1,079 consecutive subjects who underwent complete colonoscopy from December 2012 to April 2014 at National Cancer Center of Korea. Questionnaires on bowel preparation were completed by the subjects, with the quality of bowel preparation categorized as optimal (excellent or good) or suboptimal (fair, poor or inadequate). Lifestyle factors associated with bowel preparation were analyzed. RESULTS: The 1,079 subjects included 680 male (63.0%) and 399 female patietns (37.0%), with a mean age of 49.6 ± 8.32 years. Bowel preparation was categorized as optimal in 657 subjects (60.9%) and as suboptimal in 422 (39.1%). Univariate analyses showed no differences between groups in lifestyle factors, such as regular exercise, alcohol intake, smoking, and dietary factor. Body mass index (BMI) > 25 kg/m2 was the only factor associated with suboptimal bowel preparation on both the univariate (P = 0.007) and the multivariate (odds ratio, 1.437; 95% confidence interval, 1.104–1.871; P = 0.007) analyses. CONCLUSION: Most lifestyle factors, including dietary patterns, exercise, alcohol intake and smoking, were not associated with suboptimal bowel preparation in Koreans. However, BMI > 25 kg/m2 was independently associated with suboptimal bowel preparation. More intense preparation regimens before colonoscopy can be helpful in subjects with BMI > 25 kg/m2.
Body Mass Index ; Colonoscopy* ; Dietary Supplements ; Female ; Humans ; Korea ; Life Style* ; Male ; Mass Screening* ; Retrospective Studies ; Smoke ; Smoking