7 research outputs found
Early transplantation into a vesicostomy: a safe approach for managing patients with severe obstructive lesions who are not candidates for bladder augmentation
Introduction
Management of severe antenatally detected oligohydramnios with and without obstruction is improving with the result that more fetuses are surviving with early renal failure. Significant advances have occurred in all specialties involved in the management of these patients. All these specialties working together have resulted in the survival of more patients born with renal failure.
Objective
The aim of this study is to highlight the medical advances in antenatal management of fetal oligohydramnios and pulmonary hypoplasia and to demonstrate that transplantation into a diverted urinary system is safe and leads to good outcomes.
Study design
A case series of five patients were presented who, at the study center's respective facilities, recently underwent renal transplantation into bladders drained by cutaneous vesicostomy after extensive bladder evaluation and whose clinical cases highlight the aim of this study.
Results
A total of 5 patients were reviewed. Renal failure was caused by posterior urethral valves in four patients, and in one patient Eagle-Barrett syndrome. One patient received an amnio-infusion and attempted antenatal bladder shunt. One patient was ventilator dependent until 24 months, and required a tracheostomy, while two patients were ventilator dependent for the first few months of life. Three of five patients were dialysis dependent. Patient age at transplantation ranged from 20 to 61 months. All patients were poorly compliant pre-transplant and had bladder capacities ranging from 10 mL to 72 mL. Months since follow-up ranged from 3 to 64 months. Creatinine levels prior to transplant ranged from 1.9 to 5.6. During the follow up period, this range decreased to 0.13 to 0.53. Two of five patients had UTI episodes since transplantation. Patient A showed Banff Type 1A acute T-cell mediated rejected approximately two months after transplant, but subsequent biopsies have been negative for rejection. Patient A also required a vesicostomy revision approximately two months after transplant and balloon dilation of UVJ anastomosis three months after transplant.
Discussion
Vesicostomy is an especially attractive option to manage children with small bladders to accommodate the high urinary output that occurs after transplantation in infants who require an adult kidney. Recent advances in antenatal management such as amnioinfusion for oligohydramnios have made significant impacts in pulmonary and renal management of this patient population over recent years.
Conclusion
This report provides further support for the use of vesicostomy as an option for surgical management of patients with renal failure with oligohydramnios and severe obstructive lesions identified antenatally. It also indicates the need to update the criteria for antenatal management of oligohydramnios in obstructive and anephric patients
Real-world effectiveness of burosumab in children with X-linked hypophosphatemic rickets
Background: X-linked hypophosphatemic rickets (XLH) is the most common cause of inherited rickets. Historically, XLH was treated with oral phosphate and calcitriol (conventional treatment). Burosumab, a fibroblast growth factor 23 (FGF-23) monoclonal antibody, was approved by the United States Food and Drug Administration (FDA) in 2018 for XLH treatment. Nevertheless, conventional treatment of XLH continues to be recommended by some specialists due to lack of published experience with burosumab in the clinical setting. We compared laboratory and radiographic changes observed following transition from conventional therapy to burosumab in pediatric XLH patients as part of routine care.
Methods: This retrospective single-center study identified and retroactively studied twelve patients aged 1-18 years old with XLH previously treated with conventional therapy and transitioned to burosumab. Laboratory studies and radiographs were obtained routinely as standard of care during two treatment periods: (1) conventional therapy and (2) burosumab treatment. Laboratory values and radiologic rickets severity scores were compared between periods.
Results: All laboratory values demonstrated improvement following 1 month of burosumab treatment, findings which were sustained over the 2-year study period. Rickets severity scores and height z-scores also improved with burosumab. There were no serious adverse events with burosumab, and adverse events overall were very infrequent and mild. One patient developed an asymptomatic mild elevation of serum phosphate while taking burosumab resulting in a temporary pause in therapy.
Conclusions: Safety and effectiveness of burosumab in treatment of XLH were demonstrated as burosumab yielded statistically significant improvement in laboratory and radiographic markers of rickets and height compared to conventional therapy. A higher resolution version of the Graphical abstract is available as Supplementary information
Cinacalcet ameliorates aortic calcification in uremic rats via suppression of endothelial-to-mesenchymal transition
Osteopontin protects against high phosphate-induced nephrocalcinosis and vascular calcification
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Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulopathy Network
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Association of COVID-19 Versus COVID-19 Vaccination With Kidney Function and Disease Activity in Primary Glomerular Disease: A Report of the Cure Glomerulonephropathy Study
Patients with glomerular disease (GN) may be at increased risk of severe COVID-19, yet concerns over vaccines causing disease relapse may lead to vaccine hesitancy. We examined the associations of COVID-19 with longitudinal kidney function and proteinuria and compared these to similar associations with COVID-19 vaccination.
Observational cohort study from July 1, 2021 to Jan. 1, 2023.
& Participants: A prospective observational study network of 71 centers from North America and Europe (CureGN) with children and adults with primary minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy.
COVID-19 and COVID-19 vaccination.
Repeated measure of estimated glomerular filtration rate (eGFR); recurrent time-to-event outcome of GN disease worsening as defined by doubling of UPCR to at least 1.5g/g or increase in dipstick urine protein by two ordinal levels to 3+ (300mg/dL) or above.
Interrupted time series analysis for eGFR. Prognostic matched sequential stratification recurrent event analysis for GN disease worsening.
Among 2,055 participants, 722 (35%) reported COVID-19; of these, 92 (13%) were hospitalized and 3 died (<1%). eGFR slope pre-COVID-19 was -1.40ml/min/1.73m2 (SD 0.29), and -4.26ml/min/1.73m2 (SD 3.02) within 6 months post-COVID-19, which were not significantly different (p=0.34). COVID-19 was associated with increased risk of worsening GN disease activity (HR 1.35, 95% CI 1.01-1.80). Vaccination was not associated with change in eGFR (-1.34ml/min/1.73m2, SD 0.15 vs -2.16ml/min/1.73m2, SD 1.74; p=0.6) or subsequent GN disease worsening (HR 1.02, 95% CI 0.79–1.33) in this cohort.
Infrequent or short follow-up.
Among patients with primary GN, COVID-19 was severe for 1 in 8 cases and was associated with subsequent worsening of GN disease activity, as defined by proteinuria. In contrast, vaccination against COVID-19 was not associated with change in disease activity or kidney function decline. These results support COVID-19 vaccination for patients with GN.
In this cohort study of 2,055 patients with minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy, COVID-19 resulted in hospitalization or death for 1 in 8 cases and was associated with a 35% increase in risk for worsening proteinuria. In contrast, vaccination did not appear to adversely affect kidney function or proteinuria. Our data support vaccination for COVID-19 in patients with glomerular disease