A comparison of the buttonhole and rope-ladder AVF cannulation techniques and infection rates within the SCOPE collaborative.

BACKGROUND: Studies regarding hemodialysis (HD) arteriovenous fistula (AVF) cannulation in adults indicate a higher risk of infection with the buttonhole (BH) technique compared to the rope-ladder (RL) technique. Pediatric data on this issue is sparse. METHODS: We compared infection rates within the Standardizing Care to Improve Outcomes in Pediatric End stage kidney disease (SCOPE) centers performing BH cannulation versus RL cannulation of AVF. Generalized linear mixed modeling was used to assess differences in access-related blood stream infection (BSI) and access site infection (ASI) rates between the centers. RESULTS: Data was available from 211 AVF enrollments among 210 children. There were 61 AVF enrollments at 6 BH centers and 150 enrollments at 13 RL centers. Demographics were similar between the two groups. There were 12 total infections in 3383 patient months. BH centers had 3 infections (0 BSI, 3 ASI) and RL centers had 9 infections (5 BSI, 3 ASI). Mean [95% confidence interval] infection rates per 1000 patient months were not different between BH and RL centers (BH: 3.1 [0.6,15.6], RL: 3.2 [1.3,9.4], p = 0.947). A survey was also completed by the BH centers to describe their BH practices. The BH procedure at the majority of sites was characterized by a small patient/nurse ratio and strict antiseptic protocols. CONCLUSIONS: This data provides evidence of a low BSI rate associated with BH cannulation in pediatric HD patients. Further studies are needed to better delineate the differences in the pediatric and adult experience with the BH cannulation technique.

Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis.

BACKGROUND: Focal segmental glomerulosclerosis (FSGS) recurs in 20-40 % of allografts. Plasmapheresis (TPE) has been one of the mainstays of treatment with variable results. Rituximab (RTX), a monoclonal antibody to the protein CD20, is being used for treatment of recurrent FSGS (recFSGS) but pediatric experience is limited. METHODS: We conducted a retrospective review of eight patients with recFSGS, treated with RTX (1-4 doses) after having minimal response to TPE. Complete response was defined as a decrease in urine protein creatinine ratio (Up/c) to less than 0.2 and partial response was a decrease in Up/c ratio by 50 % of baseline and in the sub-nephrotic range (U p/c <2). RESULTS: Complete response was seen in two of eight patients, and partial response was seen in four of eight patients. Two patients had no response. At last follow-up, all the partial responders had sub-nephrotic range proteinuria (Up/c ratios ranging from 0.29 to 1.6). Delayed response, up to 9 months post-RTX, was also seen in some of the patients. Significant complications such as rituximab-associated lung injury (RALI), acute tubular necrosis, and central nervous system(CNS) malignancy were also observed in our case series. CONCLUSIONS: Rituximab can be used with caution as a treatment for recFSGS. Efficacy is variable from none to complete response. Even partial reduction in proteinuria is of benefit in prolonging the life of the allograft. Long-term, multicenter studies are needed to prove its sustained efficacy in those who respond and to monitor for serious adverse effects.

Acute kidney injury in COVID-19 pediatric patients in North America: Analysis of the virtual pediatric systems data.

BACKGROUND: Despite extensive research into acute kidney injury (AKI) in adults, research into the epidemiology, associated risk factors, treatment, and mortality of AKI in pediatric COVID-19 patients is understudied. Advancing understanding of this disease is crucial to further developing treatment and preventative care strategies to reduce morbidity and mortality. METHODS: This is a retrospective analysis of 2,546 COVID-19 pediatric patients (age ≤ 21 years) who were admitted the ICU in North America. Analysis of the Virtual Pediatric Systems (VPS) COVID-19 database was conducted between January 1, 2020, and June 30, 2021. RESULTS: Out of a total of 2,546 COVID positive pediatric patients, 10.8% (n = 274) were diagnosed with AKI. Significantly higher continuous and categorical outcomes in the AKI subset compared to the non-AKI cohort included: length of stay at the hospital (LOS) [9.04 (5.11-16.66) vs. 5.09 (2.58-9.94) days], Pediatric Index of Mortality (PIM) 2 probability of death [1.20 (0.86-3.83) vs. 0.96 (0.79-1.72)], PIM 3 probability of death [0.98 (0.72-2.93) vs. 0.78 (0.69-1.26)], mortality [crude OR (95% CI): 5.01 (2.89-8.70)], airway and respiratory support [1.63 (1.27-2.10)], cardio-respiratory support [3.57 (1.55-8.23)], kidney support [12.52 (5.30-29.58)], and vascular access [4.84 (3.70-6.32)]. CONCLUSIONS: This is one of the first large scale studies to analyze AKI among pediatric COVID-19 patients admitted to the ICU in North America. Although the course of the COVID-19 virus appears milder in the pediatric population, renal complications may result, increasing the risk of disease complication and mortality.

Pediatric Immunization Practices in Nephrotic Syndrome: An Assessment of Provider and Parental Knowledge.

Background: Children with nephrotic syndrome (NS) are at high risk for vaccine-preventable infections due to the immunological effects from the disease and concurrent treatment with immunosuppressive medications. Immunizations in these patients may be deferred due to their immunosuppressive treatment which may increase the risk for vaccine-preventable infections. Immunization practices in children with NS continue to vary among pediatric nephrologists. This raises the question of whether children with NS are receiving the recommended vaccinations at appropriate times. Therefore, it is critical to understand the practices and patient education provided by physicians to patients on the topic of vaccinations. Methods: After informed consent, parents/guardians of 153 pediatric patients (<18 years old) diagnosed with NS from 2005 to 2018 and 50 pediatric nephrologists from 11 participating centers completed anonymous surveys to evaluate immunization practices among pediatric nephrologists, assess the vaccine education provided to families of children with NS, assess the parental knowledge of immunization recommendations, and assess predictors of polysaccharide pneumococcal vaccine adherence. The Advisory Committee on Immunization Practices (ACIP) Immunization 2019 Guideline for those with altered immunocompetence was used to determine accuracy of vaccine knowledge and practices. Results: Forty-four percent of providers self-reported adherence to the ACIP guidelines for inactive vaccines and 22% to the guidelines for live vaccines. Thirty-two percent of parents/guardians reported knowledge that aligned with the ACIP guidelines for inactive vaccines and 1% for live vaccines. Subjects residing in the Midwest and provider recommendations for vaccines were positive predictors of vaccine adherence (p < 0.001 and p 0.02, respectively). Conclusions: Vaccine recommendation by medical providers is paramount in vaccine adherence among pediatric patients with NS. This study identifies potential educational opportunities for medical subspecialty providers and family caregivers about immunization recommendations for immunosuppressed patients.

Dextran-Sulfate Plasma Adsorption Lipoprotein Apheresis in Drug Resistant Primary Focal Segmental Glomerulosclerosis Patients: Results From a Prospective, Multicenter, Single-Arm Intervention Study.

Background: Focal segmental glomerulosclerosis (FSGS) causes end stage renal disease (ESRD) in significant proportion of patients worldwide. Primary FSGS carries poor prognosis and management of FSGS patients, refractory to standard treatments or resistant to steroids, remains a major challenge. Lipoprotein apheresis is a therapeutic approach for drug resistant primary FSGS and post-renal transplant primary FSGS recurrence. Objectives: To examine the safety and probable benefit at 1, 3, 6, 12, and 24-months following completion of apheresis treatment using Liposorber® LA-15 system in patients with nephrotic syndrome (NS), due to refractory primary FSGS or primary FSGS associated NS, in post renal transplant children. Material and Methods: Prospective, multicenter, single-arm intervention study using Liposorber® LA-15 system. Patients ≤21 years old with drug resistant or drug intolerant NS secondary to primary FSGS with glomerular filtration rate (GFR) ≥60 ml/min/1.73 m2 or post renal transplant patients ≤21 years old with primary FSGS associated NS were included in the study. Each patient had 12 dextran-sulfate plasma adsorption lipoprotein apheresis sessions over a period of 9 weeks. All patients were followed up at 1, 3, 6, 12, and 24-months following completion of treatment. Results: Of 17 patients enrolled, six were excluded from the outcome analysis (protocol deviations). Of the remaining 11 patients, all but one have completed apheresis treatments. Three patients were lost to follow-up immediately after completion of apheresis and excluded from outcome analysis. At one-month follow-up, 1 of 7 patients (14.3%) attained partial remission of NS while 2 of 4 subjects (50%) and 2 of 3 subjects (66.7%) had partial/complete remission at 3- and 6-months follow-up, respectively. One of two patients followed up for 12 months had complete remission and one patient had partial remission of NS after 24 months. Improved or stable eGFR was noted in all patients over the follow-up period. Conclusion: The results of our multicenter study showed improvement in the response rates to steroid or immunosuppressive therapy and induced complete or partial remission of proteinuria in some of the patients with drug resistant primary FSGS. The main limitation of our study is the small number of subjects and high dropout rate.

Demographics and response to therapeutic plasma exchange in pediatric renal transplantation for focal glomerulosclerosis: a single center experience.

Recurrence of FSGS following renal transplantation in pediatric patients is reported as 20-57%. Records of 37 pediatric patients transplanted for FSGS between 1990 and 2005 at a single center were reviewed. Recurrence of disease was assessed by nephrotic range proteinuria and/or FSGS on biopsy. Response to TPE was defined as urine protein to creatinine ratio <0.2. Forty-nine percent of patients were African American, 38% were Caucasian. Fifty-four percent received kidneys from deceased donors and 46% from live donors. Seven patients received preemptive TPE prior to transplantation. Two of these seven patients recurred in the transplanted kidney (28%). Recurrent FSGS occurred in 16 of 37 patients (46%), all of whom received TPE. Recurrence occurred within one month in 12 of 37 patients (32%); eight remitted with TPE (67%). Four of 12 patients failed to respond to TPE. Four of 37 patients (14%) recurred more than one month after transplantation and underwent TPE; three-fourths of patients remitted (75%). Twenty-one of 37 patients (54%) did not recur. One and five yr graft survivals were 84% and 67%, respectively. Median graft survival was 6.7 yr (5.2-10.3). Despite recurrence, FSGS patients can achieve sustained graft function.

Recurrent focal segmental glomerulosclerosis in renal allograft recipients: role of human leukocyte antigen mismatching and other clinical variables.

Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation impacts long-term graft survival and limits access to transplantation. We hypothesized that HLA donor/recipient matching could be used as a surrogate marker of recurrence. In a retrospective study of 42 pediatric and 77 adult subjects with primary FSGS, transplanted from 1990 to 2007 at a single center, we analyzed the degree of donor/recipient HLA compatibility and other clinical variables associated with FSGS recurrence. There were total of 131 allografts for primary FSGS (11 subjects were transplanted twice, and 1 had a third allograft) with 20 cases of FSGS recurrence (17 children) in the primary allograft, and two children who had FSGS recurrence in the second allograft. Fifty-two subjects (40%) were African American, and 66 (50%) Caucasians. Recurrent FSGS and controls were not different for age at transplant, gender, donor source, acute/chronic rejection episodes, and HLA matches. Recurrent FSGS was not associated with HLA mismatches; power equals 83%. Immunosuppressive regimen had no effect on recurrence of FSGS, P = .75. Recurrent FSGS is not associated with HLA mismatching, acute cellular or vascular rejection, and occurs primarily in the pediatric population.

Growth hormone axis in chronic kidney disease.

Chronic kidney disease (CKD) in children is associated with dramatic changes in the growth hormone (GH) and insulin-like growth factor (IGF-1) axis, resulting in growth retardation. Moderate-to-severe growth retardation in CKD is associated with increased morbidity and mortality. Renal failure is a state of GH resistance and not GH deficiency. Some mechanisms of GH resistance are: reduced density of GH receptors in target organs, impaired GH-activated post-receptor Janus kinase/signal transducer and activator of transcription (JAK/STAT) signaling, and reduced levels of free IGF-1 due to increased inhibitory IGF-binding proteins (IGFBPs). Treatment with recombinant human growth hormone (rhGH) has been proven to be safe and efficacious in children with CKD. Even though rhGH has been shown to improve catch-up growth and to allow the child to achieve normal adult height, the final adult height is still significantly below the genetic target. Growth retardation may persist after renal transplantation due to multiple factors, such as steroid use, decreased renal function and an abnormal GH-IGF1 axis. Those below age 6 years are the ones to benefit most from transplantation in demonstrating acceleration in linear growth. Newer treatment modalities targeting the GH resistance with recombinant human IGF-1 (rhIGF-1), recombinant human IGFBP3 (rhIGFBP3) and IGFBP displacers are under investigation and may prove to be more effective in treating growth failure in CKD.

Mauriac syndrome in a 3-year-old boy.

OBJECTIVE: To report a case of Mauriac syndrome in a young child with poorly controlled type 1 diabetes mellitus. METHODS: We describe the typical features of Mauriac syndrome and review the clinical, laboratory, and ultrasound findings and follow-up results in our current patient. RESULTS: Hepatomegaly, growth impairment, and cushingoid features characterize Mauriac syndrome. Most frequently, it is seen in adolescents and young adults with a history of poor glycemic control. In our current patient, a 3-year-old boy with type 1 diabetes mellitus of 2 years' duration, Mauriac syndrome developed in the context of a disrupted social environment. His liver enzyme derangement, hepatomegaly, and growth improved once euglycemia was achieved. CONCLUSION: Although Mauriac syndrome is currently uncommon, close monitoring of children with type 1 diabetes is important to maintain optimal glycemic control and prevent such an occurrence.