Nephrotic syndrome (NS) is the most common glomerular disorder in childhood, and a vast majority of cases are idiopathic. The precise cause of this common childhood disease is not fully elucidated despite significant advancements in our understanding of podocyte biology. Idiopathic NS has been considered "a disorder of T-cell function" mediated by a circulating factor that alters podocyte function resulting in massive proteinuria since the last four decades. Several circulatory factors released from T-cells are considered to be involved in pathophysiology of NS; however, a single presumptive factor has not been defined yet. Extended evidence obtained by advances in the pathobiology of podocytes has implicated podocytes as critical regulator of glomerular protein filtration and podocytopathy. The candidate molecules as pathological mediators of steroid-dependent NS are CD80 (also known as B7-1), hemopexin, and angiopoietin-like 4. The "two-hit" hypothesis proposes that the expression of CD80 on podocytes and ineffective inhibition of podocyte CD80 due to regulatory T-cell dysfunction or impaired autoregulation by podocytes results in NS. Recent studies suggest that not only T cells but also other immune cells and podocytes are involved in the pathogenesis of MCNS.

Nephrotic syndrome (NS) is a common chronic glomerular disease in children characterized by significant proteinuria with resulting hypoalbuminemia, edema, and hyperlipidemia. Renal biopsy findings of diffuse foot processes effacement on electron microscopy and minimal change disease, focal segmental glomerulosclerosis (FSGS), or diffuse mesangial proliferation on light microscopy. It has been speculated that circulating permeability factors would be implicated in the pathogenesis of NS because they have been reportedly detected in the sera of patients and in experimental models of induced proteinuria. Moreover, a substantial portion of the patients with primary FSGS recurrence shortly after transplantation. This report reviews the current knowledge regarding the role of circulating permeability factors in the pathogenesis of proteinuria in NS and suggests future targeted therapeutic approaches for NS.

Purpose: We studied the pathogens and trends in antibiotic sensitivity pattern in children with urinary tract infection (UTI) over 8 years in order to evaluate adequate treatment. Methods: We performed a retrospective review of medical records of children with UTI from January 2009 to December 2016 in Daegu Fatima Hospital. Uropathogens and antibiotic sensitivity patterns were selected. Only 1 bacterial species with a colony count of ${\geq}105CFU/mL$ was considered a positive result. We compared 2 periods group (A: 2009~2012, B: 2013~2016) to investigate trends of antibiotic sensitivity pattern. Results: During the 8 year period, 589 cases are identified (E. coli was cultured in 509 cases, 86.4%). Among all patients, this study investigated the antibiotic sensitivity of E. coli. Antimicrobial susceptibility to ampicillin was steadily low for both periods (A: 32.6%, B: 40.1%, P=0.125), and to amikacin was consistently high for both periods (A: 99.4%, B: 99.3%, P=1.000). Antibiotic sensitivity to third-generation cephalosporin decreased from period A to B (A: 91.7%, B: 75.5%, P=0.000). Antibiotic sensitivity to quinolone significantly decreased from A to B (A: 88.4%, B: 78.2%, P=0.003). The prevalence of extended-spectrum ${\beta}$-lactamase-producing E. coli increased from period A to B (A: 6.1%, B: 17.1%, P=0.000). Conclusion: This study showed that conventional antibiotic therapy for the treatment of pediatric UTI needs to be reevaluated. A careful choice of antibiotic is required due to the change in antibiotic sensitivity and the emergence of antibiotic-resistant bacteria.

Purpose: We aimed to determine characteristics of host, causative organisms, and antibiotic susceptibility of bacteria in pediatric patients with UTI living in metropolitan area of Korea. Methods: Retrospective investigation was done for the causative organisms of UTI in 683 pediatric cases treated at Ajou University Hospital from 2012 to 2017. Patients were classified into Escherichia coli and non-E. coli group, where E. coli group was subdivided into ESBL(+) and ESBL(-) groups based on whether the bacteria could produce extended spectrum beta-lactamase (ESBL). Antibiotic susceptibility of the causative organism was also determined. Results: A total of 683 UTIs occurred in 550 patients, of which 463 (67.8%) were first-time infection and 87 (32.2%) were recurrent ones (2-7 recurrences, 2.52 average), and 64.9% were male and 35.1% were female. The most common causative organism was E. coli (77.2%) and ESBL(+) E. coli was found in 126 cases. The susceptibility of E. coli to 3rd or 4th generation cephalosporin was relatively higher than that to ampicillin or amoxicillin/clavulanic acid. ESBL(+) E. coli showed higher resistance rate to 3rd or 4th generation cephalosporin than ESBL(-) E. coli. Conclusion: New treatment guideline should be considered due to the incidence of ESBL(+) E. coli increased up to one quarter of UTI cases.

Purpose: We investigated whether a renal size discrepancy on a renal sonogram (US) in children with febrile urinary tract infection (UTI) was correlated with the presence of cortical defects on their dimercaptosuccinic acid (DMSA) renal scan. Methods: We examined 911 children who were admitted consecutively to our hospital with their first episode of febrile UTI from March 2001 to September 2014. All enrolled children underwent a US and DMSA scan during admission. According to the US findings, including the renal size discrepancy, data were compared between children with positive and negative DMSA scan results. A positive DMSA scan result was defined as reduced or absent tracer localization and indistinct margins that did not deform the renal contour. Results: Mean renal lengths of the right and left kidneys were larger in children with positive DMSA scan results than in children with negative DMSA scan results ($63.2{\pm}11.3mm$ vs. $58.4{\pm}7.8mm$, P<0.001; $64.9{\pm}11.2mm$ vs. $59.9{\pm}7.9mm$, P<0.001; respectively). A significant difference was observed in both renal lengths between children with positive and negative DMSA scan results ($4.6{\pm}3.8mm$ vs. $3.3{\pm}2.6mm$, P<0.001). A multiple logistic regression analysis, revealed that a small kidney, cortical thinning, and a renal length discrepancy on US findings were significant factors for predicting the presence of cortical defects on an acute DMSA scan [P=0.028, 95% confidence interval (CI) 1.054-2.547; P= 0.004, 95% CI 1.354- 4.810; P<0.001, 95% CI 1.077-1.190, respectively]. Conclusion: In conclusion, a renal size discrepancy on US findings in children with their first episode of febrile UTI was a helpful tool for predicting the presence of cortical defects on an acute DMSA scan.

Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (antiCFH-aHUS) primarily affects children aged 9-13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFH-aHUS) in Laos.

The ketogenic diet (KD) has been used as an effective antiepileptic therapy for intractable childhood epilepsy. However, various adverse effects have been reported with use of the KD. We report a case of a child who developed acute tubular necrosis subsequent to therapy with KD. A 5-year-old girl had myoclonic epilepsy with developmental delay. She was under the treatment with antiepileptic drugs since the age of 3 months and on the KD during the past 18 months. Proteinuria persisted intermittently with the initiation of the KD and subsequently increased in the past 2 months. She was admitted with intermittent mild fever, vomiting, and lethargy for the past 3-4 weeks. At the time of admission, she presented with hypertriglyceridemia, heavy proteinuria, renal Fanconi syndrome, and acute kidney injury. Renal sonography showed a marked increase in the size and parenchymal echogenicity of both kidneys. A renal biopsy revealed acute tubular necrosis accompanied by early interstitial fibrosis. After the withdrawal of the KD and supportive therapy, without changing other anticonvulsants and their dosages, improvement of renal function was observed. Proteinuria had disappeared after 1 month and kidney size returned to normal after 8 months. It is hypothesized that the KD can induce and/or aggravate the renal tubulointerstitial injury in some patients who are under the treatment with anticonvulsants.

Renal cortical necrosis (RCN) is patchy or diffuse ischemic destruction of the renal cortex caused by significantly reduced renal arterial perfusion. It is a rare cause of acute kidney injury (AKI) and is associated with high mortality. Here, we review the case of RCN in a 15-year-old boy who developed AKI. A 15-year-old boy was referred to our hospital from a local hospital due to a sharp decrease in his renal function. He presented with acute flank pain, nausea with vomiting, and oliguria for the past two days. He had taken a single dose of antihistamine for nasal congestion. At our hospital, his peak blood pressure was 148/83 mmHg and he had a high body mass index of $32.9kg/m^2$. The laboratory data showed a blood urea nitrogen (BUN) of 28.4 mg/dL, a creatinine of 4.26 mg/dL, and a glomerular filtration rate estimated from the serum cystatin C of $20.2mL/min/1.73m^2$. Proteinuria (spot urine protein to creatinine ratio 1.66) with pyuria was observed. Kidney sonography showed parenchymal swelling and increased renal echogenicity. Due to rapidly progressing nephritis, steroid pulse therapy (750 mg/IV) was done on the second day of his admission and the patient showed complete recovery with normal renal function. However, the kidney biopsy findings revealed renal cortical hemorrhagic necrosis. Multifocal, relatively well-circumscribed, hemorrhagic necrotic areas (about 25%) were detected in the tubulointerstitium. Although RCN is an unusual cause of AKI, especially in children, pediatricians should consider the possibility of RCN when evaluating patients with rapidly decreasing renal function.