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Toczeń rumieniowaty układowy (SLE) to choroba autoimmunologiczna, której objawy wynikają z odkładania się kompleksów immunologicznych w różnych narządach i prowadzą do przewlekłego procesu zapalnego. Nie jest znana etiologia choroby. Predysponuje ona także do wystąpienia innych chorób z autoagresji. Jedną z nich jest zespół antyfosfolipidowy (APS) związany z obecnością przeciwciał antyfosfolipidowych oraz występowaniem zakrzepicy żylnej i niepowodzeń położniczych. Innym przykładem jest nabyta hemofilia A (AHA) wynikająca z obecności przeciwciał przeciwko VIII czynnikowi krzepnięcia. Głównym jej objawem jest skaza krwotoczna. Różnorodność i mnogość schorzeń objawiających się w konsekwencji tocznia sprawia wiele problemów w diagnostyce i leczeniu. W artykule został przedstawiony rzadki przypadek pacjentki chorującej na SLE z manifestacją głównie nerkową, a także z zajęciem innych narządów, która rozwinęła zarówno zespół antyfosfolipidowy, jak i nabytą hemofilię A o ciężkim przebiegu.

Malignant hypertension (MH) is a state which can rapidly progress to multi-organ failure. It can develop in the course of both primary and secondary type of hypertension. In our study, we present a case of a 36-year-old man diagnosed with renal failure and MH complicated with retinopathy. As the secondary nature of the condition was suspected extensive diagnostics has been conducted. In spite of excluding plenty of pathologies, the underlying cause remained unknown. Eventually, the renal biopsy has been performed, leading to a diagnosis of IgA nephropathy. However, symptoms and course of the disease has not been typical. Nevertheless, IgA nephropathy should be considered as a possible cause of secondary hypertension as well as malignant hypertension. The diagnosis is not easy and a crucial role is played by renal biopsy.

Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener’s disease, is a form of ANCA-associated vasculitis. It manifests mainly in the kidneys and the upper respiratory tract, but ocular involvement is not uncommon. In this article, four cases with ocular manifestations are presented with comprehensive photographic documentation. We describe the way to proper diagnosis, which may be long, the possible treatment, and the final outcomes. Our patients had the following ocular manifestations of GPA: retinal vasculitis, anterior necrotizing scleritis, medial orbital wall and orbital floor erosion with middle face deformation, compressive optic neuropathy due to retrobulbar inflammatory mass, and the abscess of the eyelids, inflammatory intraorbital mass causing exophthalmos and diplopia. This manuscript includes the description of severe forms of GPA, the initial signs and symptoms, relapses, and difficulties in achieving remission. The extraocular involvement is described with diagnostic modalities and laboratory findings. One of the reported cases was diagnosed by an ophthalmologist on the basis of ocular symptoms in the early stages of the disease. Our outcomes are compared with those discussed in the literature.

Diabetes mellitus is currently the leading cause of end-stage renal disease. Assessing nutritional status is an important component of care in this group. This prospective observational study aimed to assess the nutritional status of type 1 and type 2 diabetes patients on hemodialysis or peritoneal dialysis and its relationship with hospitalizations and all-cause death. Adult patients with end-stage renal disease, treated with dialysis, and suffering from type 1 or type 2 diabetes, being treated with insulin, were included in the study. Exclusion criteria comprised other types of diabetes, the patient’s refusal to participate in the study, and severe disorders impacting verbal-logical communication. The nutritional status based on the Nutritional Risk Index, the Geriatric Nutritional Risk Index, fat distribution measures, and the Charlson Comorbidity Index was estimated for 95 Caucasian dialysis patients with type 1 (n = 25) or type 2 (n = 70) diabetes. Patients with type 1 diabetes exhibited significantly inferior nutritional status and increased nutritional risk than those with type 2 diabetes. Lower values of nutritional indices significantly differentiated patients with type 1 from those with type 2 diabetes, with ≥84% sensitivity and specificity. Inferior nutritional status was related to all-cause hospitalizations, whereas higher comorbidity was associated with a greater likelihood of cardiovascular hospitalizations and all-cause death. The significant difference between patients with type 1 and type 2 diabetes being treated with dialysis indicates that these patients should not be considered as a homogeneous group, while also considering the greater age of patients with type 2 diabetes.

(1) Background: It was examined whether glucose-induced changes in the relative blood volume are suitable to identify subjects with and without type-2 diabetes mellitus (T2D) during hemodialysis. (2) Methods: The relative blood volume was continuously recorded during hemodialysis and perturbed by the infusion of glucose comparable to the dose used for intravenous glucose tolerance tests. Indices of glucose metabolism were determined by the homeostatic model assessment (HOMA). Body composition was measured by a bioimpedance analysis. The magnitude and the time course of hemodilution were described by a modified gamma variate model and five model parameters. (3) Results: A total of 34 subjects were studied, 14 with and 20 without T2D. The magnitude of the hemodilution and the selected model parameters correlated with measures of anthropometry, body mass index, absolute and relative fat mass, volume excess, baseline insulin concentration, and HOMA indices such as insulin resistance and glucose disposition in a continuous analysis, but were not different in a dichotomous analysis of patients with and without T2D. (4) Conclusions: Even though the parameters of the hemodilution curve were correlated with measures of impaired glucose metabolism and body composition, the distinction between subjects with and without T2D was not possible using glucose-induced changes in the relative blood volume during hemodialysis.

Background: Intradialytic hypotension (IDH) is a frequent complication of hemodialysis (HD). Current methods of IDH prevention are insufficient. Methods: We analyzed the intradialytic time course of systolic (SBP), diastolic (DBP), mean arterial (MAP), pulse pressure (PP), and heart rate (HR) in a group of chronic kidney disease (CKD) patients. First, 30 min into HD, a 40% glucose solution was injected into the venous line of the extracorporeal circulation at a dose of 0.5 g/kg of dry weight. Pressures and HR were measured in frequent intervals. Relative volume overload was determined by bioimpedance spectroscopy. Results: Thirty-five participants were studied. SBP increased after 5, 10, and 20 min of glucose infusion. DBP increased after 2 and 3 h and also at the end of HD. PP increased after 5, 10, and 20 min of glucose infusion and fell after the 2nd and 3rd hour and also at the end of HD. MAP increased after 2 and 3 h of glucose injection and at the end of HD. Significant interactions of the time course of SBP, DBP, MAP, with HR at baseline and of the time course of PP with fluid overload were observed. Symptomatic hypotensive episodes were absent. Conclusions: Glucose infusions during HD prevent symptomatic IDH and do not cause severe hypertensive episodes.

Sclerostin is an inhibitor of the Wnt-beta-catenin pathway. The relationship between sclerostin and adipose tissue or between sclerostin and nutritional status has been the subject of research interest in the last decade. Sclerostin concentrations are elevated in patients with chronic kidney disease (CKD). Leptin is an adipocytokine which inhibits food intake by stimulating the satiety center in the hypothalamus. Leptin concentrations rise with the reduction of eGFR (glomerular filtration rate). The aim of this study was to investigate the possible association between sclerostin and leptin, between sclerostin and selected poor prognostic factors of CKD progression, and between sclerostin and nutritional parameters in non-dialysis CKD male patients. 101 men with non-dialysis CKD stage 3–5 were included in the study. Bioimpedance spectroscopy (BIS) was used to measure body composition. Blood samples were drawn to measure the serum concentrations of sclerostin, leptin, creatinine, hemoglobin (Hgb), parathormone (PTH), inflammatory markers, and markers of nutritional status. We also measured homeostatic model assessment of insulin resistance (HOMA-IR) as well as blood pressure. We observed a significant, positive relationship between sclerostin and age, leptin, and glycated hemoglobin (HgbA1c) concentrations. A significant, negative association was observed between sclerostin and eGFR. Sclerostin is associated with leptin in non-dialysis CKD male patients. Sclerostin is also related to metabolic disturbances such as hyperglycemia in this population.

Background: The quality of autonomic blood pressure (BP) control can be assessed by the entropy of serial BP data. The aim of this study was to evaluate the effect of hemodialysis (HD) and glucose infusion (GI) on amplitude aware permutation entropy (AAPE) of hemodynamic variables during HD in chronic kidney disease patients with and without type-2 diabetes mellitus (DM). Methods: Twenty-one patients without DM (NDO) and ten with DM were studied. Thirty minutes after the start of HD, a 40% glucose solution was administered. Hemodynamic data were extracted from continuous recordings using the Portapres® system. Results: AAPE decreased during HD in all patients and all hemodynamic signals with the exception of AAPE of mean and diastolic BP in DM patients. GI led to an increase in AAPE for cardiac output in all patients, while AAPE for heart rate and ejection time increased only in DM studies, and AAPE for systolic, diastolic, and mean arterial pressure, as well as total peripheral resistance, increased only in NDO patients. Conclusions: The reduction in entropy during HD indicates impaired autonomic control in response to external perturbations. This state is partially reversed by the infusion of glucose with differences in central and peripheral responsiveness in DM and NDO patients.

Background: Neuroendocrine neoplasms are a group of tumors deriving from neural crest. They can be located in every tissue, but most commonly in gastrointestinal tract. Targeted therapy with use of radionuclides is an available and acceptable way of treatment, but its long-term safety is still to be determined, especially with sensitive methods. Methods: Study was performed on a group of 42 patients. They underwent full cycle (4 courses; 8–12 weekly intervals) of radioligand therapy with [177Lu]Lu-DOTATATE alone or tandem therapy with [177Lu]Lu-DOTATATE+[90Y]Y-DOTATATE. Late and long-term marrow and renal complications were assessed. Analysis focused on comparing data before first, fourth, and one year after the last course of RLT. Results: Study showed decreasing of all blood parameters in long-term observation, especially in lymphocytes line. Type of radioisotope, other diseases, primary tumor location, BMI, gender or age did not affect results. The only factor that had influence on hemoglobin and erythrocytes was decreased renal filtration. In long-term observation almost 10% decrease of renal filtration was observed. Type of isotope, gender, age, BMI did not affect these results. Moreover, reduction of urine IL-18, KIM-1, and albumin concentration has been observed. Conclusions: Though low-grade complications of radioligand therapy are possible, it stay a safe method of NEN treatment where benefits outweigh the risk.

BACKGROUND The genetic backgrounds of diabetic kidney disease (DKD) and end-stage kidney disease (ESKD) have not been fully elucidated. AIM To examine the individual and cumulative effects of single-nucleotide polymorphisms (SNPs) previously associated with DKD on the risk for ESKD of diabetic etiology and to determine if any associations observed were specific for DKD. METHODS Fourteen SNPs were genotyped in hemodialyzed 136 patients with diabetic ESKD (DKD group) and 121 patients with non-diabetic ESKD (NDKD group). Patients were also re-classified on the basis of the primary cause of chronic kidney disease (CKD). The distribution of alleles was compared between diabetic and non-diabetic groups as well as between different sub-phenotypes. The weighted multilocus genetic risk score (GRS) was calculated to estimate the cumulative risk conferred by all SNPs. The GRS distribution was then compared between the DKD and NDKD groups as well as in the groups according to the primary cause of CKD. RESULTS One SNP (rs841853; SLC2A1) showed a nominal association with DKD (P = 0.048; P > 0.05 after Bonferroni correction). The GRS was higher in the DKD group (0.615 ± 0.260) than in the NDKD group (0.590 ± 0.253), but the difference was not significant (P = 0.46). The analysis of associations between GRS and individual factors did not show any significant correlation. However, the GRS was significantly higher in patients with glomerular disease than in those with tubulointerstitial disease (P = 0.014) and in those with a combined group (tubulointerstitial, vascular, and cystic and congenital disease) (P = 0.018). CONCLUSION Our results suggest that selected SNPs that were previously associated with DKD may not be specific for DKD and may confer risk for CKD of different etiology, particularly those affecting renal glomeruli.

Introduction: Tertiary hyperparathyroidism (tHP) may develop in patients treated with hemodialysis or peritoneal dialysis. Parathyroidectomy may result in a significant reduction in the severity of symptoms. For the effective surgical treatment of hyperparathyroidism, proper localization of the parathyroid glands prior to surgery is essential. The sensitivity of scintigraphy in the diagnosis of tHP is lower than in the diagnosis of primary hyperparathyroidism. In recent years, positron emission tomography (PET/CT) has been gaining importance, usually as a complementary technique. Aim: The aim of this study was to determine the usefulness of PET/CT with [11C]MET in the preoperative localization diagnosis of patients with tertiary hyperparathyroidism caused by chronic kidney disease, in whom first-line diagnostic methods did not allow the localization of pathologically parathyroid glands. Material and methods: The study was conducted in a group of 19 adult patients with severe tHP who were resistant or intolerant to non-invasive treatment, with negative results of scintigraphy and ultrasonography of the neck. The study protocol included measurement of the concentration of calcium, phosphorus, and PTH in the blood serum and performing PET/CT with [11C]MET. Results: A positive result of PET/CT was obtained in 89.5% of the patients (17/19). Parathyroidectomy was performed in 52.9% of the patients (9/17) with positive results of PET/CT with [11C]MET, which were fully consistent with the results of the histopathological examinations of the removed parathyroid glands. On this basis, the sensitivity of PET/CT with [11C]MET in the preoperative localization diagnosis of patients with tHP was found to be 100%. Multiple lesions were visualized in 57.9% of the patients (11/19). Ectopic lesions were visualized in 21.1% of the patients (4/19). Conclusions: PET/CT with [11C]MET is a sensitive technique for the second-line preoperative imaging of parathyroid glands in patients with tertiary hyperparathyroidism in whom first-line examinations, such as ultrasound and scintigraphy, has failed.

IgG4-related disease belongs to the group of immune-mediated diseases. It is a relatively new condition, classified in 2003. It is characterized by involvement of multiple organs, over time causing organ failure. The observed radiological changes are slow growing. As a rule, the course of the disease is subclinical, which means that the diagnosis is often made incidentally, retrospectively and many years after the onset of symptoms. In the initial stage of diagnosis, it is often confused with Sjögren's syndrome, systemic vasculitis, or neoplastic disorders.We report the case of a female patient who was diagnosed with glomerulonephritis at the age of 34. The patient was qualified for renal replacement therapy with chronic hemodialyses in 2020 due to end-stage renal disease. In the same year, the patient was hospitalized for pneumonia of unknown etiology. After discharge from the hospital, the patient developed fever up to 39oC, chills, and pleural fluid. A suspicion of systemic disease was raised. Computed tomography of the chest showed enlargement of the anterior and middle mediastinum with increased densitization of adipose tissue and enlargement of mediastinal lymph nodes. Antibiotic therapy was introduced, followed by systemic corticosteroids, resulting in clinical improvement and remission of infiltrative lesions in radiological studies. The differential diagnosis excluded neoplastic lesions of the left lung, systemic connective tissue diseases, e.g. systemic lupus erythematosus and systemic vasculitis. Taking into account the whole clinical picture the suspicion of IgG4-related disease was raised. After the treatment, serum IgG4 level was found to be normal at 36.6 mg/ dl, anti-nuclear antibodies (ANA) were negative, rheumatoid factor was not elevated, complement component 4 (C4) was normal, complement component 3 (C3) decreased to 71 mg/dl (n: 90-180 mg/dl). Treatment with prednisone 10 mg/day was maintained. Histopathologic reanalysis of the renal biopsy (from a biopsy performed in 2012) was performed, which showed lymphocytic infiltration in the renal interstitium with segmental vascular loop sclerosis in the glomeruli and the presence of abundant granular IgG4 deposits in the glomerular mesangium by immunofluorescence.IgG4-related disease poses great diagnostic difficulty due to its non-specific and long-term course. Patients usually seek help with various specialists many years before the full picture of the disease is presented. It is poorly understood as it is a recently described condition (two decades ago). The classification criteria of IgG4 disease developed in 2019, the growing number of publications and recommendations being developed give hope for better understanding and more efficient diagnosis of the disease.

Background: Chronic kidney disease (CKD) is associated with an accelerated risk of cardiovascular mortality. Hormonal and metabolic disorders in CKD may constitute novel risk factors. Our objective was to characterize and evaluate prognostic implications of circulating sex steroids and selected nutritional parameters in patients at different stages of CKD. Methods: Studied groups were composed of 78 men: 31 on hemodialysis (HD), 17 on peritoneal dialysis (PD), 30 with CKD stage G3-G4. Total testosterone (TT), dehydroepiandrosterone sulphate (DHEA-S), androstenedione, luteinizing hormone (LH), prolactin (PRL), and biochemical parameters were measured; Free testosterone (FT) was calculated. Results: The lowest TT and FT were observed in HD, the highest- in CKD (p = 0.006 for TT, p = 0.005 for FT). TT positively correlated with total cholesterol in HD (p = 0.012), FT negatively correlated with BMI in CKD (p = 0.023). During the 12 months, 9 patients died (5 in the HD, 4 in the PD group). The deceased group had significantly lower concentrations of albumin (p = 0.006) and prealbumin (p = 0.001), and a significantly higher concentration of androstenedione (p = 0.019) than the surviving group. In the group of men on dialysis, a serum TT concentration

Background: Left ventricular diastolic dysfunction (LVDD) is observed in the early stages of chronic kidney disease (CKD) and may lead to heart failure with preserved ejection fraction (HFpEF). The purpose of our study was to investigate the association between metabolic, nutritional and inflammatory parameters and LVDD in CKD and non-CKD patients. Methods: Two groups of patients were recruited to the study: 93 men with CKD and eGFR lower than 60 mL/min/1.73 m2 and 40 men without kidney function decrease with eGFR ≥ 60 mL/min/1.73 m2. Transthoracic echocardiography was performed to evaluate the diastolic function of the left ventricle. Bioimpedance spectroscopy (BIS) was used to measure overhydration and lean body mass. We also measured the serum concentrations of albumin, glucose, haemoglobin A1c (HgbA1c), fibrinogen, C-reactive protein (CRP), tumor necrosis factor-alpha (TNF-alpha) and osteoprotegerin (OPG). Results: We observed that elevated serum fibrinogen and glucose concentrations were associated with LVDD independently of CKD status. Serum fibrinogen concentrations increased with the advancement of LVDD. Low albumin concentrations in CKD were related with LVDD. In the control group, lower muscle mass presented as lean tissue index (LTI) and lean tissue mass (LTM), and overhydration were associated with LVDD. In the group of patients without kidney function decrease the OPG concentrations were significantly higher in those with LVDD, and they rose with the advancement of LVDD. Conclusions: Elevated inflammatory parameters, increased serum glucose concentrations and worse nutritional status are the states that may impair the diastolic function of the left ventricle in CKD and non-CKD patients. Serum OPG levels are elevated in patients without kidney function decrease and LVDD and its concentrations rise with the advancement of LVDD.

Urachus is a tubular connection between the umbilical cord and the bladder of developing foetus and tends to degenerate during perinatal period to form an impatent median umbilical ligament. Failure to degenerate results in patent canal between the bladder and the umbilicus called "patent urachus" which may lead to serious of symptoms such as umbilical discharge, dermatitis, umbilical infection, abdominal pain or recurrent urinary tract infections. The Tenckhoff catheter is a tube used to perform peritoneal dialysis that is inserted through abdominal wall into peritoneum either by open surgery, minilaparotomy, laparoscopy or needle-guidewire technique.A 57-years old man was admitted to the hospital after implantation of Tenckhoff catheter by percutaneous technique in order to start peritoneal dialysis treatment. His medical history was: endstage chronic kidney disease (diabetic nephropathy), type 2 diabetes and hypertension. After the infusion of dialysate the patient experienced sudden urine pressure and passed significant amount of urine. The CT scan showed the tip of catheter being placed inside the urinary bladder. The catheter was introduced through the abdominal wall into the canal of previously undiagnosed patent urachus. The decision about re-surgery was made to stitch urachal remnants and place new the Tenckhoff catheter. Awaiting the surgery patient temporary started hemodialysis. In ongoing observation patient did not present any complications associated with peritoneal dialysis treatment.

Background The secretion, distribution, and elimination of insulin in response to a bolus of glucose injected during regular hemodialysis was modeled to quantify the intra-dialytic mass balance of glucose and insulin in patients without (D0) and with type 2 diabetes (D1). Methods A two-compartment regional blood flow model with shared compartments and dynamics for glucose, insulin and c-peptide was used to identify parameters of insulin and c-peptide co-secretion, first- and second-pass hepatic insulin extraction, as well as insulin-independent and insulin-dependent glucose utilization. Experimental data from a previous study obtained in 21 D0 and 14 D1 were used to identify kinetic model parameters and the fractions of glucose and insulin removed by dialysis. Results Modeled gains for insulin secretion (β1 = 0.015 vs. 0.084 L/min, β2 = 0.004 vs. 0.666 L) were lower in D1, resulting in a lower total insulin secretion (Mi = 6.40 vs. 38.0 nmol). Hepatic insulin extraction was high (Eihep = 0.558 vs. 0.638) and only slightly smaller in D1. The fraction of insulin removed by dialysis (Fid = 0.07 vs. 0.05) was small and comparable between D1 and D0. Modeled gains for insulin-dependent glucose uptake (γ = 0.38 vs. 1.34 L2/nmol/min) were lower whereas those for insulin-independent glucose uptake (λ = 0.14 vs. 0.067 L/min) were higher in D1. The fraction of glucose removed by dialysis (Fgd = 0.31 vs. 0.28) was higher in D1. Conclusion Apart from expected differences in modeled secretion and glucose utilization in patients with and without diabetes an intravenous bolus of glucose causes only small differences in overall glucose and insulin balance during a typical hemodialysis treatment.

The aim of the study was to evaluate the significance of metalloproteinase 3 (MMP-3), chemokine CXC ligand 13 (CXCL-13) and complement component 5a (C5a) in different stages of ANCA associated vasculitis (AAV). 89 adults were included into the study. 28 patients with active AAV (Birmingham Vasculitis Activity Score, BVAS > 3) formed the Active Group. 24 individuals who were in remission after 6 months of induction therapy formed the Short R Group, while 34 patients with longitudinal remission formed the Long R Group. 28 patients without autoimmune diseases similar in terms of age, gender and stage of kidney disease formed the Control Group. Receiver operating characteristic curve analysis (ROC) was used to evaluate MMP-3, CXCL-13 and C5a as markers of the different phases of vasculitis. In ROC analysis, MMP-3, CXCL-13 and C5a presented a good ability in distinguishing active vasculitis (Active Group) from the Control Group (AUC > 0.8), whereas only CXCL-13 displayed potential ability in distinguishing active vasculitis (Active Group) from long term remission (Long R Group, AUC = 0.683). MMP-3 significantly and positively correlated with serum creatinine concentration (r = 0.51, p = 0.011; r = 0.44, p = 0.009; r = −0.66, p p = 0.012; r = −0.35, p = 0.039; r = −0.63, p

(Background) The aim of our study was to evaluate the efficacy and safety of testosterone replacement therapy (TRT) in men with chronic kidney disease and hypogonadism on conservative and hemodialysis treatment. (Methods) The studied population consisted of 38 men on hemodialysis (HD), 46 men with CKD stages II-IV (predialysis group, PreD) and 35 men without kidney disease who were similar in age to others (control group). Serum total testosterone level (TT) was measured, and free testosterone level (fT) was calculated. Hypogonadism criteria according to the EAU definition were fulfilled by 26 men on HD (68.4%) and by 24 men from the PreD group (52%). Testosterone replacement therapy (TRT) with testosterone enanthate in intramuscular injections every 3 weeks was applied in 15 men from HD and in 14 men from PreD. The safety of TRT was monitored by measuring PSA and overhydration. (Results) A significant rise of TT and fT was observed after 3 months of TRT, but no significant changes were observed after 6 and 12 months in the HD and PreD group. An intensity of clinical symptoms of hypogonadism measured by ADAM (androgen deficiency in the ageing male) questionnaire gradually decreased, and the intensity of erectile dysfunction measured by the IIEF-5 (international index of erectile functioning) questionnaire also decreased after 3, 6 and 12 months of TRT in the HD and PreD group. (Conclusions) The applied model of TRT is effective in the correction of clinical signs of hypogonadism without a significant risk of overhydration or PSA changes.

Background. Data concerning central nervous system (CNS) alterations in ANCA-associated vasculitis with renal involvement (AAVR) are sparse. The study aimed to assess vascular and vasogenic brain alterations in patients with acute onset of AAVR and the applicability of non-contrast magnetic resonance imaging (MRI) techniques in this diagnosis. Methods. Thirty-eight patients with acute onset of AAVR were included in the study. BVAS/WG, c-ANCA, p-ANCA, renal function and perfusion, neurological assessment, and brain MRI were performed. Results. Cerebral vascular alternating narrowing and dilatation (VAND) was detected in 42.1% of patients, and the black-blood was significantly more diagnostic than the TOF technique (p < 0.001). VAND occurrence was independently associated with the concentration of p-ANCA. The vasogenic white matter lesions (VWML) were found in 94.4% of patients, and in their detection, SWAN was significantly better than the FLAIR technique (p = 0.002). The number of VWML correlated with age and cranial nerve damage. Hemosiderin deposits were found in 21.6% of patients and were associated with a gait impairment and paresthesia. Conclusions. Vascular and vasogenic alterations in the CNS are frequent in patients with acute onset of systemic ANCA-associated vasculitis with renal involvement. Non-contrast MRI is useful in the diagnosis of brain vasculitis.

Chronic kidney disease (CKD) can be caused by many conditions. The most common reasons are diabetic nephropathy, hypertension - associated nephropathy, cardiovascular disease. Although there are different reasons of deterioration of kidney function, many of them have combined molecular mechanisms by modulating metabolic homeostasis, autophagy, apoptosis, oxidative stress, inflammation. The aim of this paper is to present known molecular bases of CKD development in the course of other selected diseases to research why different patients are more prone to the CKD than others with the same condition. Selected aspects of genetic conditions were conducted, such as gene polymorphism of sirtuins, APOL1 gene polymorphism, the role of reactive oxygen species. More research is needed to understand the genetics of CKD and its' affecting the process of diagnostics and treatment.

Background: Osteoprotegerin (OPG) is a molecule which belongs to the tumor necrosis factor receptor superfamily. OPG concentration is elevated in patients with left ventricle hypertrophy, heart failure and acute myocardial infarction. OPG concentrations rise in chronic kidney disease (CKD). The aim of this study was to investigate the association between OPG concentrations and cardiovascular complications, such as left ventricle hypertrophy, systolic and diastolic dysfunction of left ventricle and dysfunction of right ventricle in chronic kidney disease patients not treated with dialysis. The relation between OPG and the amount of pericardial fluid was also examined. Methods: One hundred and one men with CKD stage 3–5 not treated with dialysis were included in the study. Overhydration, body fat mass and lean body mass were measured using bioimpedance spectroscopy (BIS). Echocardiography was performed to evaluate the amount of pericardial fluid and to measure the thickness of the interventricular septum (IVS), systolic and diastolic function of left ventricle, as well as systolic function of right ventricle. Results: We observed a significant positive association between OPG and the thickness of the interventricular septum, the size of the left atrium (LA) and the presence of pericardial fluid. A negative relationship was observed between OPG and ejection fraction (EF). Conclusions: Our results suggest that OPG can be an independent marker of left ventricular hypertrophy, systolic and diastolic dysfunction of left ventricle and the presence of pericardial fluid in chronic kidney disease patients.

Purpose In patients with chronic kidney disease (CKD), hypogonadism is more frequent than in the general population and its prevalence ranges between 40% and 60%. The aim of the study was to investigate the prevalence of hypogonadism and its association with kidney function, body composition, inflammatory markers and lipid disorders in patients with CKD. Materials and Methods The study population consisted of 112 men aged ≥40 years in different stages of CKD: 33 participants with eGFR ≥60 mL/min/1.73 m2, 27 men with eGFR 30-59 mL/min/1.73 m2, 17 predialysis patients with eGFR

Renal involvement is observed in 30% of sarcoidosis cases, but the exact occurrence is unknown, and the current numbers are estimated to be underestimated. The most common manifestation of renal sarcoidosis is interstitial nephritis, but other presentations are also possible, with specific histopathological and laboratory findings. Glomerulopathies, nephrocalcinosis and nephrolithiasis are among the most commonly seen types of renal involvement.We would like to show a case series describing four patients with varying renal manifestations of sarcoidosis: membranous nephropathy, granulomatous interstitial nephritis, IgA nephropathy and chronic kidney disease. The diagnosis of sarcoidosis can precede, present simultaneously with or follow the onset of renal manifestations. Our patients also showcase varying clinical pictures of renal sarcoidosis with different changes in renal parameters.The involvement of kidneys in sarcoidosis is multifaceted and may pose a diagnostic difficulty, and a diagnostic kidney biopsy is often needed. Chronic sarcoidosis patients should undergo regular screening for renal involvement to introduce proper management quickly and effectively.

Background: The assessment of kidney perfusion has an emerging significance in many diagnostic applications. However, whether and which of the ultrasound Doppler parameters better express renal cortical perfusion (RCP) was not shown. The study aimed to prove the usefulness of Doppler ultrasound parameters in the assessment of RCP regarding low-dose contrast-enhanced multidetector computer tomography (CE-MDCT) blood flow. Methods: Thirty non-stenotic kidneys in twenty-five hypertensive patients (age 58.9 ± 19.0) with mild-to-severe renal dysfunction were included in the study. Resistive index (RI) and end-diastolic velocity (EDV) in segmental arteries, color Doppler dynamic RCP intensity (dRCP), RI (dRI), pulsatility index (dPI), and CE-MDCT blood flow (CBF) in the renal cortex were estimated. Results: CBF correlated significantly with age, estimated glomerular filtration rate (eGFR), RI, EDV, dRI, dPI, and dRCP. In separate multivariable backward regression analyses, RI (R2 = 0.290, p = 0.003) and dRCP (R2 = 0.320, p = 0.001) were independently associated with CBF. However, in the common ultrasound model, only dRCP was independently related to CBF (R2 = 0.317, p = 0.001). Only CBF and EDV were independently associated with eGFR (R2 = 0.510, p < 0.001). Conclusions: Renal cortical perfusion intensity is the best ultrasound marker expressing renal cortical perfusion. In patients with hypertension and kidney dysfunction, renal resistive index and end-diastolic velocity express renal cortical perfusion and kidney function, respectively.

Background: Overnutrition is one of the risk factors of chronic kidney disease (CKD). The factors related to both obesity and CKD are adiponectin and ghrelin. The aim of the study was to assess if there is a link of nutritional status and selected nutrients intake with adiponectin and ghrelin in patients with diabetic nephropathy (DN). Methods: The study involved 55 patients diagnosed with DN in the pre-dialysis period (two groups: GFR < 30 and >30 mL/min/1.73 m2). In all participants standard blood tests, total ghrelin and total adiponectin plasma concentrations and anthropometric measurements (BMI, WHR- waist–hip ratio, body composition analysis) were performed. The evaluation of energy and nutrient intakes was made using the three-day food record method. Results: Excessive body weight was found in 92.80% patients. The average daily energy intake was 1979.67 kcal/day (14.45% protein energy, 28.86% fat, and carbohydrates 56.89%). In the group with eGFR < 30 mL/min/1.73 m2 the analysis showed a negative relationship between ghrelin and WHR value, and the creatine and albumin concentrations. There was a positive correlation between ghrelin concentration and the consumption of carbohydrates and sucrose. In the group of patients with eGFR > 30 mL/min/1.73 m2, a positive correlation was found between the concentration of ghrelin and the consumption of vegetable protein, carbohydrates, and glucose. Conclusions: The study confirms the high prevalence of obesity in patients with DN-Excessive supply of protein was found in the patients’ diets, which may contribute to the deterioration of the course of the disease and its prognosis. In patients with eGFR < 30 there was a negative correlation between ghrelin concentration and nutritional status, and in patents with eGFR > 30 between ghrelin concentration and some nutrients intake.

Background: Some endocrine disorders, previously considered benign, may be related to a poorer prognosis for patients with renal failure. Both low serum free triiodothyronine (fT3) and low total testosterone (TT) concentrations have been considered as predictors of death in dialysis patients, but the results of studies are inconsistent. In our study, we evaluated the relationships of the serum thyroid hormone levels and the total testosterone levels with survival in male dialysis patients. Methods: Forty-eight male dialysis patients, 31 on hemodialysis (HD) and 17 on peritoneal dialysis (PD), aged 61.4 ± 10.0, 59.2 ± 12.2 years, respectively, were included in the study. Serum thyroid hormones and total testosterone were measured. Results: During the 12-month follow-up, nine all-cause deaths were recorded. The concentrations of fT3 were significantly lower in those who died than in the survivors (p = 0.001). We did not observe any statistically considerable differences between the group of men who died and the rest of the participants in terms of the total serum testosterone concentration (p = 0.350). Total testosterone positively correlated with fT3 (r = 0.463, p = 0.009) in the HD group. Conclusions: In the group of male dialysis patients, the serum concentration of fT3 had a better prognostic value in terms of survival than the total testosterone. A linear relationship between the fT3 levels and testosterone levels in men undergoing hemodialysis may confirm the hypothesis that some of the hormonal changes observed in chronic kidney disease (CKD) may have a common cause.

Data concerning the influence of sex hormones on body composition in women with chronic kidney disease (CKD) is limited.The aim of our study was to define free testosterone levels and their association with body composition, biochemical markers of nutrition in females with CKD.47 women were included into the study. 13 females treated with hemodialysis formed the hemodialysis group (HD), 24 females with CKD stage IV/V (eGFR30 ml/min/1,73 m2) formed the predialysis group (PreD), and 10 females without kidney disease formed the control group (C). Lean tissue mass (LTM) and fat mass (Fat) were measured using bioimpedance spectroscopy. Free testosterone levels were assessed using ELISA (IBL International). Statistical analysis was performed using Statistica v 13.1.The median free testosterone (fT) levels were 0.7, 0.6, 0.85 pg/ml respectively for HD, PreD and C group. The median fT did not differ significantly between the groups (p=0.24). The mean LTM was 28.5 ±5.6, 27.3 ±4.9, 30.6 ±4.3 kg, mean Fat mass was 22.7 ±8.5, 31.3 ±9.8, 31.6 ±8.5 kg for the HD, PreD and C groups respectively. Positive correlations were observed between fT and LTM (r=0.306, p=0.035) in the whole study group. A negative correlation was observed between fT and age (r=-0.284) but was on the border of statistical significance (p=0.052).In women with advanced CKD, median testosterone levels did not differ significantly from those observed in women without kidney failure. Free testosterone levels were associated with the amount of muscle mass in the whole study population.

Secondary membranous - proliferative glomerulonephritis most often develops in the course of viral infections (HCV, HBV), autoimmune diseases, paraproteinemia, and also in the course of chronic bacterial infections. Infections with Pseudomonas stutzeri (P. stutzeri) are extremely rare and usually mildly symptomatic. The natural habitat of this bacterium is soil and water. Nevertheless, in the case of P. stutzeri infection, especially in patients frequently hospitalized or receiving immunosuppressive medications, environmental contamination in healthcare facilities should be taken into account when looking for the source of the infection.A 60-year-old man with a previous history of nicotinism and arterial hypertension with a vascular port in the vena cava superior (VCS) after treatment for bladder cancer (stage G2/G3) several years ago was described. The patient underwent the TURBT procedure, and then received intravesical infusions with BCG for 3 years, followed by complications in the form of severe dysuria and lower abdominal pain. Due to severe nausea and the inability to take analgesics orally, the patient was ordered to insert a vascular port into the VCS in order to continue the analgesic and anti - inflammatory therapy. Several years later, after the onset of massive edema of lower limbs, the patient was subjected to a 24-hour urine collection, in which proteinuria amounted to approx. 13 g/day, followed by a diagnostic kidney biopsy. Histopathological examination described membranoproliferative glomerulonephritis (MPGN). Other renal parameters were also abnormal, i.e. serum creatinine concentration was 1.9 mg/ dl and serum urea concentration was 116 mg/dl. Immunosuppressive treatment was initiated. Patient received methylprednisolone intravenously followed by prednisone orally and cyclosporine orally. During the initial period of immunosuppressive therapy, the serum levels of cyclosporine were insufficient (starting from 26.34 ng/ml), which resulted in increasing its dose, ultimately reaching 175 mg/day. After several months of therapy, the patient was hospitalized again, due to infection of the respiratory tract that had lasted for several weeks and was not amenable to antibiotic therapy. Deterioration of renal parameters and increased inflammatory markers suggested diagnosis of catheter - related sepsis. P. stutzeri was grown from the material collected from the catheter and the patient's blood. Appropriate antibiotic therapy was initiated and after the patient's condition improved, cyclosporine therapy was restarted, which was discontinued after the diagnosis of bacteremia. Rapid remission was achieved, allowing the discontinuation of immunosuppressive drugs.Chronic, asymptomatic infection with a rare pathogen, like Pseudomonas stutzeri, was probably the cause of the glomerulonephritis. After removal of the port and antibiotic therapy, disease remission was achieved.

BackgroundThe ability of extrarenal tissues to convert 25(OH)D (calcidiol) into 1,25(OH)2D (calcitriol) and dependence of the conversion on substrate levels provide the rationale for supplementing vitamin D in dialysis patients who usually have severe depletion of both: 25(OH)D and 1,25(OH)2D. The primary aim of the study was to compare effects of small doses of cholecalciferol (12,000 IU/week) with frequently used in Europe, small doses of alfacalcidol (1.5 μg/week) or placebo, given for 12 weeks, on serum 1,25(OH)2D in hemodialysis patients with 25(OH)D deficiency. Secondary outcomes were changes in serum calcium, phosphate, 25(OH)D, parathyroid hormone (PTH), fibroblast growth factor 23 (FGF23) and sclerostin during the treatment.MethodsThis was a prospective, randomized, partly double-blind (cholecalciferol vs. placebo) study. Out of 522 patients dialyzed in 5 centers in the Mazovian Province, 93 gave informed consent and met the inclusion criteria: any vitamin D metabolites and calcimimetics naïve; no history of liver or intestinal disease; serum 25(OH)D 110 pg/ml, calcium ResultsTo our knowledge, this is the first study comparing head-to-head these drugs in the hemodialysis population. There were no significant differences between the groups at baseline. 81 patients completed the study. Cholecalciferol normalized serum 25(OH)D, with a mean rise from 12.9 ± 6.7 to 31.3 ± 10.1 ng/ml (p < 0.0001). This was accompanied by a marked increase of 1,25(OH)2D from 13.8 ± 9.3 to 25.1 ± 14.2 pmol/l (p < 0.0001). A rise in serum 1,25(OH)2D was also observed in alfacalcidol treated patients, however much smaller (from 13.5 ± 10.1 to 18.5 ± 11.0 pmol/l; p = 0.02). Neither cholecalciferol nor alfacalcidol treatment resulted in significant changes in serum PTH and the remaining parameters.ConclusionsIn most patients, treatment with cholecalciferol in a 12,000 IU/week dose permits safe correction of 25(OH)D deficiency and is more effective than 1.5 μg/week dose of alfacalcidol in rising serum 1,25(OH)2D. This, together with a lack of influence on circulating iPTH the usefulness of such small alfacalcidol doses in hemodialysis patients is debatable.

Testosterone deficiency is a common disorder among men treated with hemodialysis. The aim of our study was to evaluate the relationship between free testosterone levels and body composition, biochemical markers of nutritional status, and inflammation in men on hemodialysis.Prospective analysis of men treated with hemodialysis for more than 3 months in one hemodialysis center.A total of 56 men-41 men undergoing hemodialysis treatment thrice-weekly over a period of at least 3 months (HD group) and 15 men without kidney disease, with estimated glomerular filtration rate60 mL/min/1.73 mNone, observational study.Free testosterone level.The mean free testosterone level was significantly lower in the HD group than that in the C group and positively correlated with lean tissue index (LTI, r = 0.51, P = .001) and body cell mass (BCM, r = 0.57, P .001). Significant, negative correlations were observed between free testosterone level and age (r = -0.4, P = .004) as well as fat tissue index (r = -0.36, P = .018). In a subgroup of men on hemodialysis who have low testosterone levels (9.4 pg/mL), we observed a lower LTI and BCM and higher age, fat tissue index, and loginterleukin-6. In our receiver operating characteristic curve analysis, LTI and BCM were shown to be good predictors of a low testosterone level with cutoff points of 13.3 kg/mLow lean tissue mass and BCM can be predictors of low testosterone level.

Background Chronic kidney disease (CKD) is associated with a subclinical inflammatory state, which contributes to increased mortality in CKD patients. The purpose of this study was to examine the association between chosen cytokines, such as IFN-γ, IL-10, IL-2p70, IL-6, and kidney function as well as the body composition and nutritional markers in patients with CKD and diabetes mellitus type 2. Materials and methods 21 patients with diabetes mellitus type 2 and CKD stage 3b - 5, with estimated glomerular filtration rate (eGFR) lower than 45 mL/min/1.72m2, not being treated with dialysis were included in the study. Body composition was assessed by bioimpedance spectroscopy (Body Composition Monitor - Fresenius Medical Care). Results Significant, negative correlations between lean tissue index (LTI) and IFN-γ concentrations (r = -0.52, p = 0.021) as well as IL-6 concentrations (r = -0.46, p = 0.047) were observed. Only the IL-6 levels significantly correlated with kidney function expressed by eGFR (r = -0.47, p = 0.034). We observed a significant positive correlation between IL-6 level and IFN-γ (r = 0.51, p = 0.019) as well as with high-sensitivity C-reactive protein (hsCRP) levels (r = 0.48, p = 0.029). The IL-10 level significantly correlated with hsCRP (r = 0.53, p = 0.015). Conclusion In CKD patients with diabetes mellitus type 2 during conservative treatment, IL-6 levels were associated with kidney function expressed by eGFR. IL-6 levels and IFN-γ levels negatively correlated with the amount of muscle mass. Cytokines did not show any association with the amount of fat tissue this study.

Introduction: The increasing number of patients with end-stage renal disease (ESRD) requires seeking new opportunities to improve their quality of life, not only because of kidney disease but also due to other disturbances, such as thyroid hormone disorders. The objective of the study was to evaluate the influence of coexisting hypothyroidism and thyroid hormone therapy in patients with ESRD on thyroid hormone conversion ratios and rT3 concentration. Material and methods: The study involved 85 patients aged 26 to 87 years, with a mean age of 59.62 ± 15.45 years. Four groups of patients were examined: G1 group — 25 persons without RF and hypothyroidism, G2 — 26 patients with ESRD treated with haemodialysis (HD), G3 — 12 patients with ESRD treated with HD and newly diagnosed hypothyroidism, and G4 — 22 HD patients with hypothyroidism treated with thyroid hormones substitution. The concentrations of TSH, T4, T3, fT4, fT3, and rT3 were measured and the fT3/fT4, T3/T4, and rT3/T4 conversion ratios and rT3/T3 ratio were calculated. Concentrations of protein, hsCRP, Hg, and blood gases were also checked; the anion gap was calculated. Results: Patients from group G1 through G2 to G3 were older (p trend = 0.002), with lower Hb level (p trend < 0.001), with lower pH (p trend < 0.001), with increased anion gap (p trend < 0.013) and CRP concentrations (p trend < 0.001), and decreased total protein level (p trend < 0.001). There were increased TSH values (p trend < 0.001) and lower T4 (p trend = 0.024), fT3 (p trend < 0.001), T3 (p trend < 0.001), and rT3 (p trend = 0.008) levels. rT3/T3 ratio did not change, the rT3/T4 ratio tended to decrease (p trend = 0.065) similarly to T3/T4 ratio (p trend = 0.063), and the fT3/fT4 ratio also decreased (p trend = 0.005). It seems that the treatment of thyroid disease in patients with renal failure, treated with haemodialysis, is not associated with change of rT3 and conversion factor levels. Conclusions: The concentration of rT3 in HD patients in relation to healthy persons tends to decrease, and hypothyroidism increases this tendency in these patients. Hormone substitution treatment does not eliminate the influence of RF on inhibition of rT3 production. In patients with ESRD, hypothyroidism additionally reduces the conversion of thyroid hormones examined by fT3/fT4 and to a lesser extent T3/T4 ratios.

Ksymena Le&sacute;niak, Aleksandra Rymarz, Arkadiusz Lubas, Stanis&lstrok;aw Niemczyk Department of Internal Medicine, Nephrology and Dialysis, Military Institute of Medicine, Warsaw, PolandCorrespondence: Ksymena Le&sacute;niakDepartment of Internal Medicine, Nephrology and Dialysis, Military Institute of Medicine, Warsaw, PolandEmail klesniak@wim.mil.plBackground: The management of nonviral cryoglobulinemic vasculitis (CV) has not been established yet. Randomized control trials are challenging to perform because of the rarity of the disease. The most promising biological therapy is rituximab (RTX), an anti-CD 20 monoclonal antibody. The aim of the study was to assess rituximab treatment’s safety and effectiveness in patients with severe noninfectious cryoglobulinemic vasculitis.Materials and Methods: We retrospectively reviewed 8 courses of RTX treatment in three patients with severe noninfectious CV. In 2 patients, the indication for the start of RTX therapy was the relapse of the disease despite the maintenance treatment, for the third patient, it was the first-line therapy.Results: Clinical, renal, and immunologic efficacy was observed in all evaluable RTX courses. We found a significant decrease of cryoglobulins in the 3-rd month from RTX treatment. However, 5 clinical relapses occurred and two patients experienced severe adverse events (SAEs) after RTX therapy. Patients with SAEs were relatively older and had a longer duration of disease. Lower levels of hemoglobin, C3 component of complement and eGFR as well as higher rheumatoid factor (RF) concentration were observed before RTX treatments complicated with SAEs.Conclusion: Data from our observation show the efficacy of rituximab in the refractory, nonviral cryoglobulinemic vasculitis with a severe course of the disease. However, the therapy is associated with the risk of SAEs, especially in elderly patients with kidney failure and significant immunologic alterations.Keywords: cryoglobulinemia, rituximab, systemic vasculitis