FP805 CARDIAC ARRHYTHMIAS IN BOYS WITH DENT DISEASE: SINGLE-CENTER EXPERIENCE Larisa Prikhodina 1 , Papizh Svetlana 1 , Natalia Mironenko 1 , Michael Ludwig 2 1 Research & Clinical Institute for Pediatrics, Russian National Research Medical University, Moscow, Russia and 2 Institute of Clinical Chemistry & Clinical Pharmacology, Bonn, Germany INTRODUCTION: Dent disease (DD) is an X-linked proximal tubulopathy characterized by low molecular weight proteinuria, hypercalciuria and nephrocalcinosis with progression to end-stage kidney disease in males. DD type 1 caused by mutations in CLCN5 gene (OMIM 300008) encoding chloride voltage-gated channel 5 (CLC-5). DD type 2 caused by mutations in OCRL (OMIM 300555) gene encoding inositol polyphosphate 5-phosphatase - pivotal regulator of intracellular trafficking. Recent genetic studies have shown that disorders of chloride channels may contribute to cardiac arrhythmogenesis. The aim of the study was to investigate the frequency of arrhythmia in boys with DD. METHODS: We conducted prospective study of 12 boys aged 12.0 (IQR: 7.3; 14.8 years) including 10 patients with DD1 and 2 with DD2 types. The baseline eGFR was decreased to CKD 2 stage in 7/12 (58%) boys. The median follow-up was 4.5 (2.1; 7.8) years. Direct Sanger sequencing of CLCN5 and OCRL genes was performed and DD was confirmed in all patients. RESULTS: We identified asymptomatic sinus arrhythmia in 11/12 (92%) boys at first and last follow-up. Severe arrhythmia had 4/11(36%) patients. Nobody of the boys with DD had hypertension, left ventricular hypertrophy, acid-base disorders or electrolyte serum disturbances, except 6/12 (50%) patients with hypophosphatemia. Elevated serum level of PTH had 3/12 (25%) boys with DD. Decreased eGFR at the last follow-up was found in 9/12 (75%) boys, including CKD 2 and CKD 3a stage in 6/9 (67%) and 3/9 (33%) patients, respectively. CONCLUSIONS: The study demonstrated a high frequency of asymptomatic cardiac arrhythmia in boys with DD. We speculate that arrhythmia in patients with DD might be a clinical manifestation of channelopathies with kidney and heart involvement. A multidisciplinary approach with collaboration of nephrologists and cardiologists is vital for understanding the mechanisms of arrhythmias in DD as well as for evaluating therapies and improving clinical care. FP806 OUTCOME OF YOUNG ADULTS TRANSITIONED FROM PAEDIATRICS TO ADULT RENAL SERVICES Wan Shun Wong 1 , Tara Collidge 1 1 N H S Greater Glasgow & Clyde, Glasgow, United Kingdom INTRODUCTION: Transition from paediatric to adult services is a period of increased risk for non-adherence and allograft failure. UK Registry data demonstrated 1 in 10 young adults died by 5 years after starting renal replacement therapy (RRT). The number of young adults transferring to adult services is increasing. We reviewed the outcomes of young adults transitioned from paediatric to adult nephrology services in the West of Scotland. METHODS: Patients aged 18 to 25 followed up within the standard adult renal service with previous paediatric renal service attendance were identified from the Strathclyde Electronic Renal Patient Record (SERPR). Data was extracted from SERPR and NHS Greater Glasgow Clinical Portal system. Scottish Index of Multiple Deprivation was used to assess deprivation status. RESULTS: 90 young adults transitioned from paediatric to adult services between May 2008 and June 2017. Mean age at the time of transition was 17.7 years. Two patients died from conditions unrelated to CKD during adult follow up. 23 of the 90 patients were on RRT (22 transplant and 1 PD) at transition. Mean transplant eGFR was 68.7ml/min/1.73m2 and median time post transplantation was 112 months. 11 of the 22 transplanted patients had at least one occasion of undetectable tacrolimus or sirolimus levels. 42.9% (n=9) patients had deterioration of their graft function and 7 deteriorations started post-transition. 18.2% (n=4) suffered graft loss. 67 patients were not on RRT at transition with median eGFR of 84.9ml/min/1.73m2. 15.2% (n=10) patient’s native renal function deteriorated during the follow up period with adult service and in 8, this started post-transition. 10.4% (n=7) of patients started RRT (4 transplants and 3 HD). Social deprivation was not associated with deterioration of native/graft function. Table 1 shows the comparison between those with deteriortaion of renal function and those without. Patients without deterioration of function Patients with deterioration of function Significance Paediatric clinic non-attendance (DNA) rate 5.5% 15.9% 0.001 Adult clinic DNA rate 13.9% 18.9% Not significant Documented concern on clinic/medication compliance 32.4% 60% <0.05 No. of renal unit admission 0 0.5 <0.05 Pill burden (tablets/day) 3 6 <0.05 CONCLUSIONS: Our data confirms that transition from paediatric to adult renal services is associated with high rates of deterioration in native and graft function. Deterioration was significantly associated with documented non-adherence and high pill burden but not social deprivation. Deterioration occurred more commonly post- transition, although paediatric clinic non-attendance suggest that non-adherent behaviour precedes transition. Designing services specifically for young adults that provide high continuity of care and promote self management, may reduce non- adherence in this group. FP807 IMMUNOSUPRESSIVE TREATMENT IN CHILDREN WITH IGA NEPHROPATHY AND EVIDENCE TO SUPPORT THE CLINICAL VALUE OF PODOCYTOPATHIC FEATURES Alexandra Cambier 1 , Rabant Marion 2 , Peuchmaur Michel 1 , Alexandre Hertig 3 , Deschenes Georges 1 , Ce ´ cile Couchoud 4 , Salomon Re ´ mi 2 , Julien Hogan 1 , Robert Thomas 5 1 Ho ˆpital Robert-Debre ´, Paris, France, 2 Hopital Necker, Paris, France, 3 hopital Tenon, Paris, France, 4 Agence de la Biome ´decine , Rennes, France and 5 APHM, Marseille, France, France INTRODUCTION: There is a need for treatment guidelines and prognostic factor identification in children with primary IgA nephropathy. Prospective studies assessing the risk-to-benefit ratio of corticosteroids in pediatric IgAN are urgently needed as steroids are currently central to this debate. We analyzed the causative effect of steroids and the applicability of the Oxford classification. METHODS: A total of 82 consecutive children (mean 10.6 years; median follow-up 3.3 years) were reviewed; 21 patients (25.6%) presented with acute kidney injury, and 6 (7.3%) with nephrotic syndrome. Renal biopsies were scored for Oxford classification and podocytopathic features in 2 groups: a group treated with steroid therapy (some in association with cyclophosphamide) and supportive care (renin angiotensin system blockade) and a group treated by supportive care alone. RESULTS: The 2 groups were not comparable because baseline clinical data were different. Estimated glomerular filtration rate (eGFR) in immunosupressive group significantly improved between M0 (at onset) and M6 (6 months after treatment) from 89.9 [61.2-114.5] to 110.5 [93.7-120] ml/min per 1.73 m2, P < 0.001. Proteinuria also significantly decreased from (1.6 [1-4.3] to 0.3 [0.2-0.7] g/g, P < 0.001). In the supportive care group, eGFR and proteinuria remained stable. Podocytopathic features were predictive of renal function decline by univariable (-4.9 6 14.9 ml/min per 1.73 m2, P = 0.0079) and multivariable analysis and of poor renal prognosis to a combined event (renal function impairment more than 10% of the eGFR baseline or chronic kidney disease stage 3 at 6 months) in univariable analysis. MEST-C score failed to prove its prognostic value. CONCLUSIONS: Immunosuppressive treatment, especially steroid therapy, seems beneficial in children with glomerular inflammation and proliferation. The Oxford classification does not appear to be entirely appropriate in predicting long-term renal prognosis for children, whereas the characteristics of podocytopathy are strongly predictive of renal prognosis. Children IgAN could be a podocytopathy disease. i324 | Abstracts Abstracts Nephrology Dialysis Transplantation Downloaded from https://academic.oup.com/ndt/article/34/Supplement_1/gfz106.FP805/5516012 by guest on 29 September 2021