Type 1 primary hyperoxaluria is a genetic disorder caused by deficiency of the liver-specific peroxisomal enzyme alanine-glyoxylate aminotransferase. This enzyme deficiency leads to excess oxalate production and deposition of calcium oxalate salts, resulting in kidney failure and systemic oxalosis. Aside from combined liver/kidney transplantation, no curative treatment exists. Various strategies for optimizing dialysis treatment have been evaluated, but neither conventional hemodialysis nor peritoneal dialysis can keep pace with oxalate production in this patient population. In this report, we describe a patient with end-stage renal disease from type 1 primary hyperoxaluria managed with nocturnal home hemodialysis. Performing hemodialysis 8-10 hours each night with blood flow of 350 mL/min and total dialysate volume of 60 L, she has maintained pre- and postdialysis serum oxalate levels at or below the level of supersaturation. We also review published literature regarding oxalate removal in various modalities of dialysis in patients with type 1 primary hyperoxaluria. In our patient, nocturnal hemodialysis has controlled serum oxalate levels better than conventional hemodialysis therapies. Home nocturnal hemodialysis should be considered an option for management of patients with end-stage renal disease from type 1 hyperoxaluria who are awaiting transplantation.
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http://dx.doi.org/10.1053/j.ajkd.2013.05.013 | DOI Listing |
J Clin Med
January 2025
Nephrology and Renal Transplantation, Hospital Clínic de Barcelona, 08036 Barcelona, Spain.
: High-volume online hemodiafiltration (OL-HDF) has proven to be the most efficient dialysis modality and to offer better clinical outcomes in patients on hemodialysis. Longer and more frequent dialysis sessions have demonstrated clinical and survival benefits. : A single-center observational study of the first one hundred patients on nocturnal every-other-day OL-HDF was conducted with the aim of reporting the experience with this treatment schedule and evaluating analytical and clinical outcomes as well as the patient and technique survival.
View Article and Find Full Text PDFPediatr Nephrol
December 2024
Department of Pediatric Nephrology, Ghent University Hospital, Ghent, Belgium.
Three-weekly 4-h hemodialysis/hemodiafiltration (HD/HDF) per week has become the "standard HD/HDF" regimen in children across the globe, although increasingly criticized, since crucial determinants such as residual kidney function and patient preferences are not considered. As a consequence, several children fail to achieve adequate dialysis while on a "standard HD/HDF." In these circumstances, an extended dialysis prescription such as short daily (2-3 h/session, 5-7 days a week) or nocturnal HD/HDF (6-9 h/session, 3-5 days a week), either at home or in a dialysis center, may be considered.
View Article and Find Full Text PDFAntioxidants (Basel)
October 2024
Research Laboratory Molecular Bases of Human Pathology LR19ES13, Faculty of Medicine, University of Sfax, Sfax 3029, Tunisia.
Background: This study aimed to investigate the effects of intradialytic concurrent (resistance-endurance) training combined with melatonin (MEL) supplementation on oxidative stress, inflammation, and cellular damage in hemodialysis (HD) patients.
Methods: Thirty-two HD patients were randomly assigned to three groups: Exercise (EX)-MEL, EX-Placebo (PLA), and Control (C)-PLA. Participants in the EX-MEL and EX-PLA groups underwent 12 weeks of concurrent training.
Indian J Otolaryngol Head Neck Surg
December 2024
Sri Ramachandra Medical College and Research Institute, Sri Ramachandra Institute of Higher Education and Research, Porur, Chennai India.
Chronic kidney disease (CKD) is a major cause of morbidity and mortality in India, with a prevalence estimated at 13-15%. Obstructive sleep apnea (OSA) is an emerging threat, significantly contributing to CKD development. Over 50% of end-stage renal disease (ESRD) patients exhibit OSA symptoms.
View Article and Find Full Text PDFCureus
October 2024
Central Clinical Laboratory, University Hospital St. Marina, Varna, BGR.
Introduction One of the most important critical determinants of quality of life and adequacy of hemodialysis (HD) performed in patients is the recorded survival and mortality rates. Nowadays, as an adequately performed HD dialysis, we accept the one with reaching values for the index single pool Kt/V (spKt/V) higher than 1.2.
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