MO112SYSTEMIC OXALOSIS IN PRIMARY HYPEROXALURIA TYPE 3 – ARE THE PATIENTS AT RISK?

Abstract Background and Aims Primary Hyperoxaluria type 3 (PH3) is said to be the less problematic form of PH with low risk chronic kidney disease (CKD) end stage renal disease. However, a recent OxalEurope registry evaluation reported both urine plasma oxalate levels in comparable range as PH1 PH2 patients. In addition, PH3 patients remain symptomatic recurrent stones, even adulthood, 24% 95 evaluated were on CKD ≥ 2 at last follow up. Hence, it was speculated, that may also develop systemic deposition. Method We retrospectively analyzed imaging procedures performed so far regularly seen German Center, which included: eye exams; x-rays hand; bone MRI (3 Thesla left knee proximal tibia); Speckle tracking echocardiography using 2D Cardiac Performance Analysis VC (TomTec Imaging Systems GmbH, Germany), measures changes global longitudinal strain (GLS), an index ventricular contractibility. The normal for GLS ≤18%. All or parents signed informed consent. Results From 49 registered center, 12 pediatric 4 adult are regular basis, least twice year, rest followed other centers. 16 stable function no than 2. Eye examination six all. Four received x-ray hand, 3, but one patient clinical course (multiple stone removal procedures, decline GFR), tiny sclerosing areas, although true metaphyseal bands, caput MCP IV thumb. Therefore, tibia this another patient, showed signs done 6 abnormal (GLS – 17.3 hypertrophy) borderline twin sibling 18.6). had salivary stones parotid gland, found his other, older routine jaw before orthodontic treatment. Conclusion Although currently only data small cohort patients, parameters available show, deposition occur PH3. Based our experience PH1, we regard best parameter detect early calcium-oxalate depositions. Hence reduction strain, thus contractability, clear proof such deposits. Of course, more needed elucidate therefore screening all