Background
Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) has been recognized as a distinct entity in recent years. To the best of our knowledge, all patients with PGNMID ...reported thus far were older than 20 years of age. We now report five cases of PGNMID in patients under 20 years of age.
Methods
The clinical database was searched for patients with native kidney biopsies from 9/2011 to 8/2017, and cases with a diagnosis of PGNMID were retrieved. Light microscopy specimens and immunofluorescence and electron microscopy images were revisited. Clinical data and kidney biopsy findings for patients under the age of 20 were recorded.
Results
Five (0.78%) of a total of 637 patients younger than 20 with native renal biopsies had a diagnosis of PGNMID, including three males and two females with an average age of 14 years old (range 10–19). All five patients presented with microscopic hematuria and proteinuria. Three patients were nephrotic and their C3 levels were low. All five cases showed a membranoproliferative pattern with abundant mesangial and subendothelial monoclonal IgG3 deposits (3 κ and 2 λ light chain, respectively). The patients were followed up to 56 months. Two patients had re-biopsies 28 and 18 months after initial diagnosis and both showed similar morphologic changes. Various treatments were attempted including prednisone, mycophenolate mofetil, tacrolimus, rituximab, and eculizmab, with mixed responses.
Conclusions
PGNMID does occur in children and young adults. Membranoproliferative glomerulonephritis pattern with monoclonal IgG3 deposits is a common feature. Despite various immunosuppressive treatments, the disease appears slowly progressive.
Infections cause significant morbidity and mortality for children receiving maintenance hemodialysis (HD). The Standardizing Care to Improve Outcomes in Pediatric End-Stage Kidney Disease (SCOPE) ...Collaborative is a quality-improvement initiative aimed at reducing dialysis-associated infections by implementing standardized care practices. This study describes patient-level risk factors for catheter-associated bloodstream infections (CA-BSIs) and examines the association between dialysis center-level compliance with standardized practices and risk of CA-BSI.
Prospective cohort study.
Children enrolled in SCOPE between June 2013 and July 2019.
Data were collected on patient characteristics and center-level compliance with HD catheter care practices across the study period. Centers were categorized as consistent, dynamic (improved compliance over the study period), or inconsistent performers based on frequency of compliance audit submission and changes in compliance with HD care practices over time.
CA-BSIs.
Generalized linear mixed models were used to evaluate (1) patient-level risk factors for CA-BSI and (2) associations between change in center-level compliance and CA-BSIs.
The cohort included 1,277 children from 35 pediatric dialysis centers; 1,018 (79.7%) had a catheter and 259 (20.3%) had an arteriovenous fistula or graft. Among children with a catheter, mupirocin use at the catheter exit site was associated with an increased rate of CA-BSIs (rate ratio RR, 4.45; P = 0.004); the use of no antibiotic agent at the catheter exit site was a risk factor of borderline statistical significance (RR, 1.79; P = 0.05). Overall median compliance with HD catheter care practices was 87.5% (IQR, 77.3%-94.0%). Dynamic performing centers showed a significant decrease in CA-BSI rates over time (from 2.71 to 0.71 per 100 patient-months; RR, 0.98; P < 0.001), whereas no significant change in CA-BSI rates was detected among consistent or inconsistent performers.
Lack of data on adherence to HD care practices on the individual patient level.
Improvement in compliance with standardized HD care practices over time may lead to a reduction in dialysis-associated infections.
A term male neonate presented to his paediatrician for a routine follow-up after hospital discharge. Prenatal care had been excellent and labour and delivery had been unremarkable. He had been ...feeding, gaining weight and was not in distress though significant abdominal distention was noted. Lab tests revealed electrolytes derangements, metabolic acidosis and renal failure. An ultrasound revealed severe unilateral hydronephrosis and echogenic kidneys. A voiding cystourethrogram revealed the definitive diagnosis which was posterior urethral valves.
A term male neonate presented to his paediatrician for a routine follow-up after hospital discharge. Prenatal care had been excellent and labour and delivery had been unremarkable. He had been ...feeding, gaining weight and was not in distress though significant abdominal distention was noted. Lab tests revealed electrolytes derangements, metabolic acidosis and renal failure. An ultrasound revealed severe unilateral hydronephrosis and echogenic kidneys. A voiding cystourethrogram revealed the definitive diagnosis which was posterior urethral valves.
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