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MO772PRIMARY AND SECONDARY HYPEROXALURIA IN CHRONIC DIALYSIS PATIENTS: FOCUS ON VASCULAR ACCESS
Abstract Background and Aims Hyperoxaluria is a rare metabolic disorder chacterized by widespread calcium oxalate deposition, if plasmatic oxalate level (pOx) overcomes saturation threshold. It could be primary (PH), a recessive disease associated with mutation of the liver enzyme LDH, or secondary...
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| Published in: | Nephrology, dialysis, transplantation dialysis, transplantation, 2021-05, Vol.36 (Supplement_1) |
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| Main Authors: | , , , , , , , , , |
| Format: | Article |
| Language: | English |
| Online Access: | Get full text |
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| Summary: | Abstract
Background and Aims
Hyperoxaluria is a rare metabolic disorder chacterized by widespread calcium oxalate deposition, if plasmatic oxalate level (pOx) overcomes saturation threshold. It could be primary (PH), a recessive disease associated with mutation of the liver enzyme LDH, or secondary (SH), in 75% due to intestinal malabsorption. Urolithiasis could be the first complication, and renal involvement could progress until end-stage renal disease (ESRD). Mean pOx is higher in chronic dialysis patients than healthy individuals because of impaired renal clearance. Oxalate molecular weight (MW) is 88 Da and its clearance is that of low MW uremic toxins. Our aim is to assess an association between arteriovenous fistula (AVF) failures and Hyperoxaluria.
Method
In the period between 1/1/2004 and 12/31/2020, diagnosis of Hyperoxaluria was carried out in 6 patients out of 1530 chronic dialysis patients (0.39%). The median age of patients was 65.5 [26-72] years, the male/female ratio was 3:3 and the median dialysis vintage was 36 [1-181] months. Three patients come to nephrological referral at stage 5 of CKD while 1 patient came to Italy when chronic HD was already started. At that time pOx was not considered. In these 4 patients the presumptive diagnosis of ESRD was urolithiasis and diagnosis of Hyperoxaluria was carried out after the beginning of chronic dialysis, when pOx and genetic analysis were performed. In 1 case biopsy on the kidney graft and in 1 case kidney biopsy after nephrectomy were performed: oxalate deposition was detected. For 2/6 patients diagnosis was carried out after biopsy of native kidneys before starting dialysis. All patients started medical therapy for hyperoxaluria. Five out of 6 patients were eligible to HD, 1/6 to PD. AVF was judged the first choice vascular access.
Results
PH was diagnosed in 3/6 patients: mutations were detected on chromosome 2. Malabsorption secondary to short bowel syndrome and chronic pancreatitis could be assumed as causes of SH in 3 patients. Patient 1 underwent 2 combined liver-kidney transplantation: the first failed by arterial grafts thrombosis and the second by renal primary non-function but the liver was still functioning, he died on HD after 12 years. Patient 2 underwent liver and kidney transplantation and she is nowadays dialysis-free. The median pOx pre-dialysis and before diagnosis was 165 [98-259] umol/L (Table 1).
Patients on chronic HD underwent median of 3 [2-5] AVF interventions. Nine AVF |
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| ISSN: | 0931-0509 1460-2385 |
| DOI: | 10.1093/ndt/gfab103.0010 |