Browsing by Author "Paloian, Neil"

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    Early transplantation into a vesicostomy: a safe approach for managing patients with severe obstructive lesions who are not candidates for bladder augmentation
    (Elsevier, 2018) Viswanathan, Aravind; Leffler, Thomas; Paloian, Neil; Cain, Mark; McKenna, Patrick H.; Urology, School of Medicine
    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.
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    Nephrocalcinosis and kidney function in children and adults with X-linked hypophosphatemia: baseline results from a large longitudinal study
    (Oxford University Press, 2024) Portale, Anthony A.; Ward, Leanne; Dahir, Kathryn; Florenzano, Pablo; Ing, Steven W.; Jan de Beur, Suzanne M.; Martin, Regina M.; Meza-Martinez, Adriana I.; Paloian, Neil; Ashraf, Ambika; Dixon, Bradley P.; Khan, Aliya; Langman, Craig; Chen, Angel; Wang, Christine; Scott Roberts, Mary; Tandon, P. K.; Bedrosian, Camille; Imel, Erik A.; Medicine, School of Medicine
    Background: In patients with X-linked hypophosphatemia (XLH), conventional therapy with oral phosphate salts and active vitamin D has been associated with nephrocalcinosis. However, the nature of the relationships among XLH, its treatment, nephrocalcinosis, and kidney function remain poorly understood. Methods: Renal ultrasounds were performed and glomerular filtration rates were estimated (eGFR) at baseline in burosumab-naïve patients with XLH who participated in burosumab clinical trials (NCT02181764, NCT02526160, NCT02537431, NCT02163577, NCT02750618, NCT02915705) or enrolled in the XLH Disease Monitoring Program (XLH-DMP; NCT03651505). In this cross-sectional analysis, patient, disease, and treatment characteristics were described among patients with and without nephrocalcinosis. Results: The analysis included 196 children (mean [SD] age 7.6 [4.0] yr) and 318 adults (40.3 [13.1] yr). Mean (SD) height z-score was -1.9 (1.2) for children and -2.3 (1.7) for adults. Nearly all children (97%) and adults (94%) had previously received conventional therapy. Nephrocalcinosis was detected in 22% of children and 38% of adults. In children, reduced eGFR <90 mL/min/1.73 m2 was more prevalent in those with nephrocalcinosis (25%) than in those without (11%), a finding that was not observed in adults. Children with nephrocalcinosis had lower mean values of TmP/GFR (p<.05), serum 1,25(OH)2D (p<.05), and eGFR (p<.001) and higher mean serum calcium concentrations (p<.05) than did those without nephrocalcinosis. Adults with nephrocalcinosis had lower mean serum phosphorus (p<.01) and 1,25(OH)2D (p<.05) concentrations than those without. Exploratory logistic regression analyses revealed no significant associations between the presence of nephrocalcinosis and other described patient or disease characteristics. Conclusions: Nephrocalcinosis was observed in nearly one-quarter of children and more than one-third of adults with XLH. Further study is needed to better understand the predictors and long-term consequences of nephrocalcinosis, with surveillance for nephrocalcinosis remaining important in the management of XLH.