Pediatric glomerulopathy after COVID-19 vaccination: A case series and review of the literature.

BACKGROUND: Cases of glomerulopathy after COVID-19 vaccination have been reported in the adult population, while only a few cases have been reported in children and adolescents. For better understanding of this association in pediatric population, we aimed to describe clinical course of patients with glomerulopathy within 60 days of COVID-19 vaccination who were under followed up in the pediatric nephrology department of National Taiwan University Children's Hospital. METHODS: We reviewed the clinical characteristics, vaccine types, and outcomes of patients with newly diagnosed glomerular diseases or relapse of underlying glomerulopathy within 60 days after COVID-19 vaccination at our facility between January 2021 and July 2022. RESULTS: Thirteen pediatric patients were found to have newly diagnosed glomerular diseases or relapse from their underlying glomerulopathy after receiving their first, second, or third COVID-19 vaccines in our facility. Of the five pediatric patients with newly diagnosed glomerulopathy after vaccination, thin basement membrane nephropathy, idiopathic nephrotic syndrome, and hematuria have been identified. Seven patients had relapse episodes of underlying nephrotic syndrome and one patient with underlying isolated microscopic hematuria developed subnephrotic proteinuria after COVID-19 vaccination. All patients experienced remission or improvement with either immunosuppressive or conservative treatment during the follow-up period. CONCLUSIONS: This is the largest case series to date of pediatric glomerulopathy after COVID-19 vaccination. From our report, patients with either newly diagnosed or relapse of glomerulopathy after vaccination had good outcomes, and receiving vaccination to prevent COVID-19 infection or complications should be encouraged in pandemic era under close monitoring kidney manifestations.

Risk factors for in-hospital mortality and acute kidney injury in neonatal-pediatric patients receiving extracorporeal membrane oxygenation.

BACKGROUND: Acute kidney injury (AKI) is the most frequent complication in critically ill neonatal and pediatric patients receiving extracorporeal membrane oxygenation (ECMO) support. This study analyzed risk factors for in-hospital mortality and the incidence of AKI in neonatal and pediatric patients received ECMO support. METHODS: We reviewed the medical records of 105 neonatal and 171 pediatric patients who received ECMO support at the intensive care unit (ICU) of a tertiary care university hospital between January 2008 and December 2015. Demographic, clinical, and laboratory data were retrospectively collected as survival and AKI predictors, utilizing the Kidney Disease Improving Global Outcome (KDIGO) consensus definition for AKI. RESULTS: In the 105 neonatal and 171 pediatric patients, the overall in-hospital mortality rate were 58% and 55% respectively. The incidence of AKI at post-ECMO 24 h were 64.8% and 61.4%. A greater KDIGO24-h severity was associated with a higher in-hospital mortality rate (chi-square test; p < 0.01) and decreased survival rate (log-rank tests, p < 0.01). In univariate logistic regression analysis of in-hospital mortality, the CVP level at post ECOMO 24-h increased odds ratio (OR) (OR = 1.27 [1.10-1.46], p = 0.001) of in-hospital mortality in neonatal group; as for pediatric group, elevated lactate (OR = 1.12 [1.03-1.20], p = 0.005) and PT (OR = 1.86 [1.17-2.96], p = 0.009) increased OR of in-hospital mortality. And the KDIGO24h stage 3 had the strongest association with in-hospital mortality in both neonatal (p = 0.005) and pediatric (p = 0.001) groups. In multivariate OR of neonatal and pediatric groups were 4.38 [1.46-13.16] (p = 0.009) and 3.76 [1.70-8.33] (p = 0.001), respectively. CONCLUSION: AKI was a significant risk factor for in-hospital mortality in the neonatal and pediatric patients who received ECMO support. A greater KDIGO24-h severity was associated with higher mortality rates and decreased survival rate in both neonatal and pediatric groups. Of note, KDIGO24h can be an easy and early tool for the prognosis of AKI in the neonatal and pediatric patients.

Serum Creatinine Reference Limits in Pediatric Population-A Single Center Electronic Health Record-Based Database in Taiwan.

Objective: To assess age- and sex-specific serum creatinine levels in a pediatric population using a hospital-based database in Taiwan. Study Design: Data on serum creatinine levels were obtained from the National Taiwan University Hospital-integrated Medical Database (NTUH-iMD). Due to the possibility of having acute kidney injury or chronic kidney disease, individuals with multiple serum creatinine measurements were excluded, and outliers in each age- and sex-specific group were also subsequently removed. The remaining creatinine measurements in each group were analyzed, and 95% reference limits were established. Results: Serum creatinine data of individuals aged between 1 month and 18 years from May 2011 to January 2018 were retrieved. After applying the exclusion criteria, 27,911 individuals with a single corresponding serum creatinine measurement were enrolled. Creatinine level reference limits for each age- and sex-specific group were generated. The upper reference limits (URLs), which are particularly useful in clinical practice, followed the natural trend of increasing serum creatinine with age. Conclusion: We generated serum creatinine reference limits from a single hospital-integrated medical database in Taiwan for different age- and sex-specific groups of children. Our results will aid physicians in clinical practice regarding renal function evaluation, especially for patients without a recent baseline serum creatinine level.

High Concentration of C5a-Induced Mitochondria-Dependent Apoptosis in Murine Kidney Endothelial Cells.

Patients with a relapse of idiopathic nephrotic syndrome have significantly increased levels of serum complement component 5a (C5a), and proteinuria has been noted in mice treated with C5a via changes in permeability of kidney endothelial cells (KECs) in established animal models. However, the apoptosis of KECs treated with high concentrations of C5a has also been observed. As mitochondrial damage is known to be important in cell apoptosis, the aim of this study was to examine the association between C5a-induced mouse KEC apoptosis and mitochondrial damage. Mouse KECs were isolated and treated with different concentrations of C5a. Cell viability assays showed that a high-concentration mouse recombinant protein C5a (rmC5a) treatment reduced mouse KEC growth. Cell cycle phase analysis, including apoptosis (sub-G1 phase) showed an increased percentage of the subG1 phase with a high-concentration rmC5a treatment. Cytochrome c and caspase 3/9 activities were significantly induced in the mouse KECs after a high-dose rmC5a (50 ng/mL) treatment, and this was rescued by pretreatment with the C5a receptor (C5aR) inhibitor (W-54011) and N-acetylcysteine (NAC). Reactive oxygen species (ROS) formation was detected in C5a-treated mouse KECs; however, W-54011 or NAC pretreatment inhibited high-dose rmC5a-induced ROS formation and also reduced cytochrome c release, apoptotic cell formation, and apoptotic DNA fragmentation. These factors determined the apoptosis of mouse KECs treated with high-dose C5a through C5aR and subsequently led to apoptosis via ROS regeneration and cytochrome c release. The results showed that high concentrations of C5a induced mouse KEC apoptosis via a C5aR/ROS/mitochondria-dependent pathway. These findings may shed light on the potential mechanism of glomerular sclerosis, a process in idiopathic nephrotic syndrome causing renal function impairment.

Transfusion-dependent thalassaemic patients with renal Fanconi syndrome due to deferasirox use.

AIM: Deferasirox is a new oral iron chelating agent with several cases reporting renal adverse events in recent years. Our aim was to identify the incidence of deferasirox-related Fanconi syndrome (FS) and its risk factors. METHODS: All transfusion-dependent thalassaemic patients who received deferasirox at the outpatient department of the National Taiwan University Hospital (NTUH) from January 2006 to February 2014 were evaluated. RESULTS: This cohort study included 57 patients, and mean age of deferasirox initiation was 18.2 ± 7.7 years. After 6.9 ± 1.8 years of follow-up, 5 in 57 (8.8%) thalassaemic patients had FS. Age of starting deferasirox negatively correlated with incidence of FS (correlation coefficient -0.892, P = 0.008). Other factors were not significantly associated with FS. Serum creatinine level at the start of deferasirox compared to at the end of study or onset of FS did not show significant change (P = 0.277). All the deferasirox-related FS manifested with proximal renal tubular acidosis and hypophosphataemia, which needed specific treatment or withdrawal of deferasirox use. CONCLUSIONS: We recommend that children, especially of young age, who regularly use deferasirox should undergo routine urinalysis and blood testing for early detection of FS.

Inhibition of Rho-associated kinase relieves C5a-induced proteinuria in murine nephrotic syndrome.

Childhood nephrotic syndrome is mainly caused by minimal change disease which is named because only subtle ultrastructural alteration could be observed at electron microscopic level in the pathological kidney. Glomerular podocytes are presumed to be the target cells whose protein sieving capability is compromised by a yet unidentified permeability perturbing factor. In a cohort of children with non-hereditary idiopathic nephrotic syndrome, we found the complement fragment C5a was elevated in their sera during active disease. Administration of recombinant C5a induced profound proteinuria and minimal change nephrotic syndrome in mice. Purified glomerular endothelial cells, instead of podocytes, were demonstrated to be responsible for the proteinuric effect elicited by C5a. Further studies depicted a signaling pathway involving Rho/Rho-associated kinase/myosin activation leading to endothelial cell contraction and cell adhesion complex breakdown. Significantly, application of Rho-associated kinase inhibitor, Y27632, prevented the protein leaking effects observed in both C5a-treated purified endothelial cells and mice. Taken together, our study identifies a previously unknown mechanism underlying nephrotic syndrome and provides a new insight toward identifying Rho-associated kinase inhibition as an alternative therapeutic option for nephrotic syndrome.

Efficacy and safety of intermittent hemodialysis in infants and young children with inborn errors of metabolism.

BACKGROUND: Intermittent hemodialysis (IHD) is the most efficient form of renal replacement therapy (RRT) for removing toxic substances from patients' bodies. However, the efficacy and safety of IHD in infants and young children with inborn errors of metabolism are still not clear. METHODS: This retrospective study included patients with urea cycle disorders, maple syrup urine disease, and methylmalonic acidemia who received IHD or non-IHD RRT at our hospital between 2001 and 2012 to remove ammonia, leucine, or methylmalonic acid. Both the efficacy and safety of the RRT were evaluated. RESULTS: Thirty-five courses of RRT, including 25 courses of IHD and ten courses of non-IHD RRT, for 15 patients were included in the analysis. Before 2006, non-IHD RRT procedures, including peritoneal dialysis (PD) and continuous venous-venous hemofiltration (CVVH), were the most often used; from 2006 onwards IHD was used. There was one procedure-unrelated death. Catheter penetration occurred in one course of IHD. The efficacy data revealed that both the median duration of dialysis and the median 50 % toxin reduction time were shorter in IHD than in non-IHD RRT. CONCLUSIONS: In infants and young children with inborn errors of metabolism, IHD is safe and more efficient than non-IHD RRT at removing toxins.

Drug susceptibility and treatment response of common urinary tract infection pathogens in children.

BACKGROUND/PURPOSE: To document the trends of sensitivity and to find whether it is necessary to change antibiotics in selected patients according to the sensitivity test results in our clinical practice. METHODS: We collected urine culture results from 0-18-year-old patients in the National Taiwan University Hospital from January 1, 2003 to October 31, 2012. Their medical chart was reviewed to identify true pathogens responsible for their urinary tract infection (UTI). We checked the percentage of susceptibility of these pathogens to ampicillin, amoxicillin-clavulanate (AMC), cefazolin, cefmetazole, ceftriaxone, gentamicin, and trimethoprim-sulfamethoxazole (TMP-SMX) according to the Clinical and Laboratory Standards Institute (CLSI) guideline. The extended-spectrum-beta-lactamases (ESBLs) rate was also checked. In addition, we reviewed the treatment response of different antibiotics. Defervescence within 48 hours after initial antibiotics use was considered responsive. RESULTS: A total of 7758 urine cultures positive for Escherichia coli infection were collected during the 10-year period. The E. coli cefazolin susceptibility rate was 62-73% during 2003-2010, but it dropped to 23% in 2011 and 28% in 2012 after the new CLSI guideline (M100-S21) was released. However, other antibiotics did not show a significant difference. In UTI caused by E. coli, on average, the sensitivity rates for various antibiotics were as follows: cefmetazole, 90%; ceftriaxone, 85%; gentamicin, 77%; AMC, 61%; TMP-SMX, 47%; and ampicillin, 20%. The ESBL rate was also found to increase (2-11%; p < 0.01). The overall response rate of UTI caused by E. coli to first-line antibiotics such as first-generation cephalosporin and/or gentamicin was 78%. CONCLUSION: The susceptibility of common urinary tract pathogens to cefazolin has decreased dramatically since 2010. This trend may be due to the change in the CLSI guideline. Although the susceptibility rate to first-line empirical antibiotics shows a decreasing trend, we found that the clinical response was acceptable for our first-line empirical antibiotics.

Pediatric renal transplantation: results and prognostic factors.

BACKGROUND/OBJECTIVE: As renal transplantation may increase survival rates and improve quality of life for children with end-stage renal disease, we investigated the long-term outcomes and prognostic factors of pediatric renal transplantation. METHODS: A retrospective study was conducted to review 25 pediatric renal transplantations, either from live or deceased donors, in our hospital from 1995 to 2008. The cumulative graft survival rate was calculated using the Kaplan-Meier method. Log rank tests were employed to identify categorical prognostic factors for graft survival of the pediatric renal transplantations, and Cox regression analysis for numeric factors. RESULTS: The mean age of our study subjects was 11.63±3.76 years, and the mean follow-up period was 49.24±33.72 months. The 12-month and 36-month graft survival rates were 92% and 82.14%, respectively. The rejection-free survival rates were 88% and 72.88% in the first and third years, respectively. All of the patients were alive during the follow-up period. Acute rejection (p=0.0175) and male sex (p=0.0384) were found to be significant factors for graft survival. CONCLUSION: For pediatric patients, we found that renal transplantation is now a safe and effective surgical procedure for children with end-stage renal disease. Acute rejection and male gender were identified as prognostic factors for poor graft survival.

A rare cause of childhood-onset nephrotic syndrome: lipoprotein glomerulopathy.

We report on a 12-year-old female patient with lipoprotein glomerulopathy (LPG) who was proven to be heterozygous for ApoE2 Kyoto (Arg25Cys). Her family members have the same variant but do not have obvious signs of renal function impairment. Six months of treatment with a statin caused significant clinical improvement in the lipid profile, proteinuria, and renal function. Our case suggests that administration of a statin is a potential therapeutic strategy for improving nephrotic syndrome in patients with LPG.

Nephromegaly is a significant risk factor for renal scarring in children with first febrile urinary tract infections.

PURPOSE: We determined whether nephromegaly on ultrasound can be used to identify patients with urinary tract infection at increased risk for renal scarring, and we investigated the effect of vesicoureteral reflux on renal scarring. MATERIALS AND METHODS: We enrolled hospitalized patients with a first febrile urinary tract infection. All patients underwent renal ultrasound and most patients underwent voiding cystourethrography. Renal scarring was assessed using (99m)technetium dimercapto-succinic acid renal scintigraphy at least 6 months after treatment. Children with recurrent urinary tract infections before scintigraphy were excluded from the study. RESULTS: A total of 545 children (80 with and 465 without nephromegaly) were enrolled. Infection was more severe in patients with than without nephromegaly. The incidence of renal scarring was significantly higher in patients with nephromegaly (90% vs 32%, p <0.001), in kidneys with nephromegaly (80.5% vs 18.7%, p <0.001) and in kidneys with vesicoureteral reflux (41.5% vs 22.2%, p <0.001). Kidneys with nephromegaly had a greater incidence of reflux. The finding of nephromegaly is associated with a greatly increased likelihood of renal scarring in patients with vesicoureteral reflux. CONCLUSIONS: Our results indicate that ultrasound diagnosis of nephromegaly at onset is associated with a high incidence of renal scarring, and identification of nephromegaly at onset and vesicoureteral reflux are significant risk factors for renal scarring in children with a first febrile urinary tract infection. Nephromegaly is associated with an increased frequency of vesicoureteral reflux and increased likelihood of renal scarring in patients with reflux.

Universal hepatitis B vaccination reduces childhood hepatitis B virus-associated membranous nephropathy.

OBJECTIVE: To compare the incidence of hepatitis B virus (HBV)-associated membranous nephropathy (HBVMN) before and after universal HBV vaccination and to identify factors underlying the change. METHODS: This study included 471 hospitalized children with nephrotic syndrome (NS) and 488 long-term follow-up hepatitis B surface antigen (HBsAg)-carrier children. Horizontal transmission (negative maternal HBsAg status) of HBVMN and HBV was assessed, and the incidence of HBVMN was compared before and after initiation of the universal HBV vaccination program started in 1984. RESULTS: The frequency of HBVMN in children with NS was 11.6% between 1974 and 1984, 4.5% between 1984 and 1994, 2.1% between 1994 and 2004, and 0% between 2004 and 2009. Similarly, the number of HBsAg-seropositive children with NS (mainly via horizontal infection) decreased after universal vaccination. The prevaccination frequency of HBV horizontal transmission in chronic HBsAg carriers from the general population was 36.5% compared with 5% in the postvaccination period. The incidence of HBVMN in these carriers revealed a parallel decline. CONCLUSIONS: Our results revealed a significant decrease in the frequency of HBVMN in children with NS and in long-term follow-up HBsAg carriers. Children with HBVMN are primarily infected with HBV via horizontal transmission; thus, the significant reduction in horizontal transmission in HBsAg-carrier children in the general population after universal HBV vaccination may explain the reduction of HBVMN in the vaccinated population. These findings confirm the effectiveness of HBV vaccination on reducing the incidence of HBVMN, possibly through a significant decline in horizontal HBV infection.

Nonenteropathic hemolytic uremic syndrome: the experience of a medical center.

BACKGROUND: Hemolytic uremic syndrome (HUS) is an uncommon cause of acute renal failure in children. In contrast to Western countries, most HUSs in Taiwan are caused by Streptococcus pneumoniae. In this article, we demonstrate the clinical courses of children with HUS in Taiwan and try to explain the pathophysiology of complications. METHODS: We retrospectively reviewed the medical records of children with HUS who were admitted to the Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan, between January 1997 and February 2008. The clinical courses, laboratory data, complications, and prognosis were recorded. We divided the patients into dialysis group and non-dialysis group. The laboratory data for the two groups were expressed as mean, standard deviation, and range. RESULTS: Nine patients were enrolled in the study, and all were diagnosed with S pneumoniae-associated HUS. One patient had meningitis, and the others had pneumonia. The mean durations of anemia and thrombocytopenia were 19.2 days and 10.2 days, respectively. Five patients received renal replacement therapy because of oligouria. The average of the peak total bilirubin levels of patients in the dialysis and non-dialysis groups were 24.6 ± 20.7mg/dL and 3.8 ± 1.9mg/dL, respectively. In addition to one patient who had meningitis, four other patients experienced central nervous system (CNS) complications. The mean durations of hypertension in five patients with CNS manifestations and four patients without CNS manifestations were 16.8 ± 7.8 days and 4.8 ± 6.6 days, respectively. Two patients died in acute stage, and most of the others had regained normal renal function at discharge or during follow-up. CONCLUSION: (1) Streptococcus pneumoniae is the most common causative pathogen of HUS in Taiwan; (2) the mean duration of hypertension in patients with CNS manifestations was much longer than that in patients without CNS manifestations; and (3) the average of the peak total bilirubin levels of patients in the dialysis group was much higher than that in the patients of the non-dialysis group.

Comparison of virulence variations on MDCK monolayers by Escherichia coli isolated from acute lobar nephronia and acute pyelonephritis.

Escherichia coli is the most common pathogen associated with acute lobar nephronia (ALN), a clinically more severe parenchymal inflammatory disease that requires a longer duration of antibiotic treatment than acute pyelonephritis (APN). This study was conducted to unravel the virulence differences between clinical isolates of E. coli from pediatric ALN and APN patients. A total of 88 urinary isolates of E. coli were investigated. They were identified from radiologically diagnosed ALN and APN patients and had previously been molecularly characterized for important urovirulence genes. Madin-Darby canine kidney (MDCK) epithelial cells were used as an in vitro model. Multivariate logistic regression analyses indicated that ALN isolates were more likely to show adhesion (p<0.05; odds ratio [OR], 3.81; 95% confidence interval [CI], 1.23-11.80) and cytotoxicity (p<0.001; OR, 10.42; 95% CI, 3.03-35.89). However, no difference in the penetration ability was noted. Henceforth, the ability to adhere to and produce cytotoxicity against uroepithelial cells appears a prerequisite factor for E. coli to cause more severe bacterial kidney infection, such as ALN.

Genetic polymorphisms and susceptibility to parenchymal renal infection among pediatric patients.

BACKGROUND: Patient susceptibility to bacterial urinary tract infections, which is determined by complex pathogen-host interactions, varies between individuals due to genetic variation. The neutrophil-dependent innate immune system is an important part of keeping the urinary tract sterile. This study was performed to explore single nucleotide polymorphisms (SNPs) in genes associated with neutrophil-dependent immunity in pediatric patients with severe parenchymal infections. METHODS: The subjects included patients who fulfilled the diagnostic criteria of acute pyelonephritis (APN) and acute lobar nephronia (ALN) without underlying disease or structural anomalies (excluding vesicoureteral reflux). Genotyping of the genes encoding toll-like receptor 4 (TLR-4), interleukin-8 (IL-8), and IL-8 receptors CXCR1 and CXCR2 was performed by matrix-assisted laser desorption/ionization time-of-flight-based mini-sequencing analysis. RESULTS: A total of 17 SNPs, including missense SNPs and those located in promoter regions, were initially selected for genotyping. Only 4 SNPs with a heterozygosity rate >0.01 were evaluated further. The observed genotype frequencies satisfied Hardy-Weinberg equilibrium. Statistical analysis revealed that only IL-8 (rs4073, -251A>T) showed significant differences in genotype and allele frequency between the control and APN or ALN cases. Following the elimination of vesicoureteral reflux, which is a significant risk factor for severe parenchymal infection, a single SNP in IL-8 (rs4073) was found to be associated with clinically severe ALN. CONCLUSIONS: The AA genotype and A allele of the IL-8 SNP is related to patient susceptibility to parenchymal infection and is correlated with the severity of infection in pediatric APN and ALN patients, probably due to the upregulation of IL-8 expression.

Comparison of extended virulence genotypes for bacteria isolated from pediatric patients with urosepsis, acute pyelonephritis, and acute lobar nephronia.

BACKGROUND: Despite recent advances in molecular epidemiology and pathogenecity analyses of extraintestinal Escherichia coli infections, detailed analyses identifying virulence factors of E. coli isolates from pediatric urosepsis patients have not been reported. This study was conducted to explore and differentiate bacterial virulence factors associated with urosepsis and 2 other severe parenchymal infections, acute pyelonephritis (APN) and acute lobar nephronia (ALN), in pediatric patients. METHODS: Patients included in this study were those who fulfilled the diagnostic criteria of urosepsis, APN, and ALN, without underlying disease or structural anomalies, excluding those with vesicoureteral reflux. Patients with cystitis were included as controls. E. coli isolates from urine (cystitis, APN, and ALN) or blood (urosepsis) specimens were analyzed using polymerase chain reaction (PCR) for 25 virulence genes. RESULTS: A total of 147 children (24 cystitis, 45 APN, 48 ALN, and 30 urosepsis) were enrolled in the study. Distinct syndrome-specific differences in the distribution for certain virulence genes, but conservation across syndromes for others, were found. In addition, urosepsis isolates presented higher aggregate virulence factor scores (P < 0.0001) compared with cystitis, APN, and ALN isolates. By contrast, cystitis isolates showed significantly lower aggregate virulence factor scores than all 3 invasive urinary bacterial infections; APN (P < 0.01), ALN (P < 0.01), and urosepsis (P < 0.0001). CONCLUSIONS: Our findings suggested that urosepsis isolates carry more virulence factors and are likely more urovirulent compared with cystitis, APN, and ALN isolates.

Treatment course of steroid-dependent nephrotic syndrome: emphasized on treatment effect.

AIM: Children with steroid-dependent nephrotic syndrome (SDNS) need long-term steroid usage to maintain sustained remission. Cyclophosphamide is a well-known alternative agent to spare the use of steroids and avoid the side-effects that result from long-term steroid therapy. Most children may continue to have SDNS despite receiving cyclophosphamide. Additional alternative drugs may be needed. In the present study, the effects on SDNS of sequential treatment after cyclophosphamide usage were established. METHODS: Forty-six children with SDNS were enrolled in this retrospective uncontrolled study. In addition to prednisolone, patients were treated with cyclophosphamide as a first-line alternative drug. Children who still had SDNS despite cyclophosphamide therapy received chlorambucil, levamisole or another course of cyclophosphamide. The treatment responses were recorded and the mean duration of follow up was 96 months. RESULTS: Seventeen patients (37%) experienced no relapse after cyclophosphamide therapy. Twenty-five patients (54%) had varied responses. Only four patients showed no effect. Children who still had SDNS despite cyclophosphamide therapy received second or more alternative drugs. Cyclophosphamide with or without chlorambucil resolved steroid-dependency in 33 of 46 (72%) children who either had complete remission or developed steroid-sensitive, rather than steroid-dependent, nephrotic syndrome. CONCLUSION: With the exception of four patients who were lost to follow up and four who were refractory and needed other treatment, most children with SDNS could spare the steroid (complete remission or steroid sensitive nephrotic syndrome) after using one or more of these modulating agents.

Acute lobar nephronia is associated with a high incidence of renal scarring in childhood urinary tract infections.

BACKGROUND: Acute lobar nephronia (ALN) is a severe nonliquefactive inflammatory renal bacterial infection, and requires a longer duration of treatment. The aim of this prospective study was to investigate renal scarring after ALN and to examine the risk factors for renal scarring in children with ALN compared with those with acute pyelonephritis (APN). METHODS: Patients with computed tomography-diagnosed ALN were enrolled and randomly allocated, with serial entry, to either a 2- or 3-week antibiotic treatment regimen. Age- and gender-matched APN patients served as comparators. Patients underwent dimercaptosuccinic acid scintigraphy at least 6 months later to assess renal scarring. RESULTS: A total of 218 children (109 ALN, 109 APN) were enrolled. The incidence of renal scarring was similar between 2- and 3-week treatment groups and was higher in ALN patients than in APN patients (89.0% vs. 34.9%, P < 0.001). Renal scarring was prone to occur in children with higher inflammatory indices and longer duration of fever before and after treatment. Multiple regression analysis on independent variables showed that only ALN was significantly associated with a higher incidence of renal scarring. CONCLUSIONS: Our results showed a new finding that ALN is associated with a very high incidence of renal scarring, in comparison to APN, irrespective of the duration of antibiotic treatment.