Background/Aims: Both iron deficiency and chronic inflammation are highly prevalent in patients with chronic kidney disease (CKD). The effect of intravenous iron infusion on mineral metabolism in CKD may be modified by inflammation. Intravenous iron theraphy may reduce peripheral degradation, secretion, clearence of iFGF23 and lead to hypophosphatemia. The aim of the study was to evaluate the effect of intravenous iron on mineral metabolism in CKD patients. Methods: 35 non-dialysis patients with CKD stages 3-5. received 100 mg/24h of ferric oxide saccharated solution for 5 days. Serum calcium (Ca), phosphorus (P), parathormone (PTH), intact-FGF23 (iFGF23), C-terminal-FGF23 (cFGF23), bone alkaline phosphatase (BAP) and high-sensitive CRP were assessed on day 1 and 3 at baseline and 2 hours after each dose administration and once on day 6. Plasma iFGF23 and cFGF23, as well as serum BAP were measured with ELISA and other parameters with standard automated laboratory methods. Results: Serum iFGF23 increased after iv iron on day 1 and 6 (from 268.9±446.5 to 326.3±529.9 on day 1; p=0.05 and to 451.4±601 pg/mL on day 6; p=0.03). cFGF23 was reduced only on day 1 (from 654.3±441.3 to 473.6±414 RU/mL; p=0.016). P concentration decreased significantly two hours after the first iron infusion (from 1.69±0.5 to 1.54±0.35 mmol/l; p=0.003). In following days the changes of cFGF23, P and of other calcium-phosphate metabolism were not significant. Serum CRP correlated neither with iFGF-23 nor cFGF-23. Conclusion: Intravenous iron supplementation may only transiently affect the production and degradation of FGF23 resulting in hypophosphatemia at the commencement of iron therapy. Chronic low-grade inflammation does not seem to play a role in that mechanism.

Spontaneous rupture of renal pelvis (SRRP) is a rare condition resulting in an extravasation of urine into retroperitoneal space. Due to the uncharacteristic symptoms, often mimicking renal colic, its diagnosis may be complicated. Herein, we report a case of a 73-year-old male with a solitary functioning kidney who presented with malaise and right-sided abdominal pain, rapidly followed by anuria. Laboratory tests showed the signs of AKI. Contrast-enhanced CT performed soon after the admission showed nonspecific abnormalities in the right middle abdomen suspected to be either inflammatory infiltration or surgical scarring. Symptomatic treatment was started, and an acute hemodialysis treatment was commenced. After a temporal improvement, the patient’s general condition worsened significantly, with exacerbated pain and massive increase in plasma creatinine. A second contrast-enhanced CT was performed with an addition of urography phase, revealing the extravasation of the contrast media in the location suggesting the rupture of the renal pelvis. The patient was treated successfully by the placement of a double-J ureteral stent into the ureter. Usually, a clear etiology of SRRP can be determined, that is, urinary tract obstruction, but in this case, we could not find a definite cause. It is important to remember that in the presence of a nonspecific abdominal pain and laboratory signs of AKI, a rare cause like SRRP should be taken into consideration. Performing a contrast CT scan with urography phase can save time in establishing a diagnosis and enable immediate urological intervention.

Departmental sources Background: Kidney transplantation (KTx) reverses most abnormalities related to chronic kidney disease (CKD), but sedentary lifestyle persists in most kidney graft recipients. Physical inactivity has been associated with altered adipokine profile and inflammation in CKD. We postulated that increased physical activity achieved through an individually-tailored program can reverse these changes. Material/Methods: We included 25 clinically stable KTx recipients at least 12 months after transplantation and with eGFR >30 mL/min and 15 age-matched non-dialysis patients with CKD stage 3. Body composition, pattern of daily physical activity, and serum concentrations of leptin, adiponectin, NT-proBNP, and hsCRP were assessed at baseline. All patients in both groups participated in a 12-week supervised exercise program with short cell phone text reminders. All measurements were repeated after 3 months. Results: Active energy expenditure increased significantly during the 3 months in both the KTx and CKD patients, compared with baseline by 47% (p<0.001) and 20% (p=0.01), respectively. Time spent daily on physical activity was also increased (129±83 vs. 194±142 and 81±56 vs. 124±57 min, respectively, p<0.001). Adipose tissue mass decreased significantly in the KTx group (from 40.8±11 to 38.5±10.3 kg, p=0.01). Serum leptin decreased significantly in both KTx and CKD patients (from 11.5±7.0 to 10.0±5.6, p=0.03 and from 14.1±8.3 to 12.2±6.1 ng/mL, p=0.01, respectively). Serum adiponectin increased only in the KTx group (from 1900±953 to 2015±1133 ng/L, p=0.004). Serum CRP decreased in both groups (from 15.1±5.2 to 14.0±5.6 mg/L, p=0.01 in the KTx group and from 16.5±3.9 to 15.4±4.3 mg/L in the CKD group p=0.05). NTpro-BNP was unchanged during the study. Conclusions: Increased physical activity induces beneficial effects on adipokine profile and inflammation but does not seem to affect volume overload in kidney transplant recipients and CKD patients.

Fabry disease (FD) is an ultra-rare genetic lysosomal storage disease caused by pathologic gene variants resulting in insufficient expression of α-galactosidase A. This enzyme deficiency leads to accumulation of globotriaosylceramide and globotriaosylsphingosine in plasma and in different cells throughout the body, causing major cardiovascular, renal, and nervous system complications. Until 2018, reimbursed enzyme replacement therapy (ERT) for FD was available in all European Union countries except Poland.             We present the preliminary results of the first two years of reimbursed ERT in Poland. We obtained data from the seven largest academic centers in Katowice, Cracow, Wrocław, Poznań, Gdańsk, Warsaw, and Łódź. The questionnaire included the following data: number of patients treated, number of patients qualified for ERT, and patient characteristics.             All centers returned completed questionnaires that included data for a total of 71 patients (28 men and 43 women) as of June 2021. Thirty-five patients with the diagnosis of FD confirmed by genetic testing (22 men and 13 women) had already qualified for reimbursed ERT. Mean (SD) age at the commencement of the ERT program was 39.6 (15.5) years (range 18-79 years). Mean time from the first clinical symptoms reported by the patients to the FD diagnosis was 21.1 (8.9) years, and the mean time from the final diagnosis of FD to the beginning of ERT was 4.7 (4.6) years.             FD is still underdiagnosed in Poland. To identify undiagnosed FD patients and to ensure that patients in Poland benefit fully from ERT, implementation of an effective nationwide screening strategy and close cooperation with a network of rare disease centers is advised.

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Background and Aims Iron therapy may induce inflammatory response that may indirectly affect endogenous erythropoietin (EPO) production. We postulated that increased secretion of FGF-23 may provide a link between intravenous iron administration and suppression of endogenous EPO secretion. Evaluation of a short-term effect of intravenous iron sucrose administration on the secretion of endogenous EPO in patients with chronic kidney disease (CKD). Method 35 non-dialysed patients with G3b-5 CKD were included. All patients received 100 mg of intravenous iron infusion (iron (III)-hydroxide sucrose complex) daily for 5 days. Plasma concentration of EPO, iFGF-23, cFGF-23, PTH, bone alkaline phosphatase (BAP), phosphorus (PO4) and calcium (Ca) were measured before and two hours after the first and third iron infusion and at the end of iron therapy. Results EPO concentration at the end of iron treatment was significantly lower compared to 2 h after the first iron infusion (p&lt;0.001) and before the third dose (p&lt;0.001) (12.6 [31.2] mIU/mL, 30.9 [38.3] mIU/mL, 33.4 [41.3] mIU/mL and, respectively). Conversely, serum iFGF-23 increased significantly after the third dose (61.1 [401.5] pg/mL; p&lt;0.05) and after the treatment (92.1 [849.7] pg/mL; p&lt;0.01) compared to pre-treatment value (48.4 [403.8] pg/mL). cFGF-23 concentration decreased significantly after the first dose of iron (491.8 [828.6] vs. 339.2 [875.8] RU/mL; p&lt;0.01) and after the completion of the therapy (398.7 [931.9]) vs. baseline (p&lt;0.05). There was no linear correlation between EPO and FGF-23. Conclusion Intravenous iron therapy increases the secretion of FGF-23 and supresses endogenous EPO production but these two effects do not seem to be related.

Fabry disease (FD) is an ultra-rare genetic lysosomal storage disease caused by pathologic gene variants resulting in insufficient expression of α-galactosidase A. This enzyme deficiency leads to accumulation of globotriaosylceramide and globotriaosylsphingosine in plasma and in different cells throughout the body, causing major cardiovascular, renal, and nervous system complications. Until 2018, reimbursed enzyme replacement therapy (ERT) for FD was available in all European Union countries except Poland.             We present the preliminary results of the first two years of reimbursed ERT in Poland. We obtained data from the seven largest academic centers in Katowice, Kraków, Wrocław, Poznań, Gdańsk, Warszawa, and Łódź. The questionnaire included the following data: number of patients treated, number of patients qualified for ERT, and patient characteristics.             All centers returned completed questionnaires that included data for a total of 71 patients (28 men and 43 women) as of June 2021. Thirty-five patients with the diagnosis of FD confirmed by genetic testing (22 men and 13 women) had already qualified for reimbursed ERT. Mean (SD) age at the commencement of the ERT program was 39.6 (15.5) years (range 18-79 years). Mean time from the first clinical symptoms reported by the patients to the FD diagnosis was 21.1 (8.9) years, and the mean time from the final diagnosis of FD to the beginning of ERT was 4.7 (4.6) years.             FD is still underdiagnosed in Poland. To identify undiagnosed FD patients and to ensure that patients in Poland benefit fully from ERT, implementation of an effective nationwide screening strategy and close cooperation with a network of rare disease centers is advised.