P0171NON COMPLIANCE AND ACUTE DEHYDRATION ARE MAIN REASON FOR ACUTE KIDNEY FAILURE IN NON-INFANTILE PRIMARY HYPEROXALURIA TYPE I (PH I)

Abstract

Abstract Background and Aims Patients with primary hyperoxaluria type 1 have an increased risk of acute or chronic kidney failure (AKF/CKF). There are two risk groups: first the infantile oxalosis patients with an unremarkable family history and second, the adult patients with only an oligosymptomatic course (minor stone events), who get into end-stage renal disease (ESRD) due to oxalate-induced chronic inflammatory processes in the kidney. We were interested in the prevalence of acute kidney failure in the PH1 patients seen in our Hyperoxaluria Center over the last 10 years. Method We retrospectively analyzed the database of the German Hyperoxaluria Center for those patients being routinely seen in our outpatient clinic for AKF and its specific reason. AKF was defined as patients with a sudden onset of kidney failure and the necessity of dialysis installment, but a documented stable kidney function (no worse than stage 2-3 CKD) prior to onset of AKF. Results Currently, we routinely follow (3-4 times a year) 49 of 117 patients with genetically proven PH1. For the patients not routinely seen, we act as the reference center and primary lab. Out of the 117 PH1 patients, 6 had infantile oxalosis and thus early ESRD; 8 patients are currently on dialysis; and 6 patients have died, 2 patients each either post liver or liver/kidney transplantation (LTx, LKTx), under dialysis or as sudden death, the latter two due to systemic oxalosis. Transplantations were performed in 35 patients with PH1 (LKTx in 28 patients, LTx in 4 patients and 3 had an isolated kidney transplantation). Out of the 49 PH1 patients we routinely follow, AKF was diagnosed in 8 patients aged 11-56 years (one pediatric patient, 3 patients 18-19 years old, and 4 patients 29-56 years old), which has led to CKF and hence, maintenance hemodialyis in 7/8 and even death in one patient. Non-compliance regarding either medication or recommended fluid intake was the reason for AKF in 6 of the 8 patients, the 11 year-old girl, 2 adult patients and the 3 young adults being on the edge of leaving pediatric care. Acute massive diarrhea without adequate fluid substitution led to AKF in 2 middle aged male patients. Conclusion AKF is not uncommon in patients with PH1 and is frequently related to either non-compliance or to situations of severe fluid losses with inadequate fluid substitution. In the situations described, AKF is not based on already extremely altered renal function. Therefore, interruption of medication or lack of fluid intake, even for short periods of time, can lead to severe clinical consequences in these patients.