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AIMS: The determination of ideal weight in hemodialysis patients remains a common problem. The use of Lung Ultrasound (LUS) is an emerging method of assessing the hydric status of hemodialysis patients. LUS combined with Inferior Vena Cava (IVC) ultrasonography can define the fluid status in hemodialysis patients. METHODS: This study included 68 hemodialysis patients from the Dialysis Unit of Papageorgiou General Hospital in Thessaloniki. The patients underwent lung and IVC ultrasound 30 min before and after the end of the dialysis session by a nephrology trainee. Patients' ideal weight was modified based on daily clinical practice rather than ultrasound findings. The presence of B lines and ultrasound findings of the IVC were evaluated. RESULTS: The average B line score was 11.53 ± 5.02 before dialysis and became 5.57 ± 3.14 after the session. The average diameter of the IVC was 14.266 ± 0.846 mm before dialysis and 12.328 ± 0.879 mm after the session. The patients were categorized based on the magnitude of overhydration and the findings were evaluated. In addition, findings after the session showed a statistically significant correlation between the b line score and the diameter of the IVC adjusted for the body surface area. (p = 0.009 < 0.05). CONCLUSIONS: A high rate of hyperhydration was detected before the dialysis session (25%). While it is the first study conducted by a nephrology trainee highlighting that it is a feasible technique. Intervention studies should be carried out in the future to draw more precise conclusions.

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PURPOSE: It is unclear whether normal white blood cell (WBC) counts are predictive of subsequent mortality in hemodialysis patients. METHODS: All patients aged 17 years or more, who initiated hemodialysis at a tertiary Hospital from January 2000 to August 2017 with a dialysis vintage of greater than 90 days and normal median WBC count of their first dialysis year were included in the study. They were followed until they died, transferred to other dialysis facilities, switched to peritoneal dialysis, received a renal transplant or reached the end of the study (August 31, 2018). Cox regression was used to estimate hazard ratios for mortality of tertiles of WBC counts, adjusting for baseline demographic, clinical and laboratory variables. RESULTS: 611 patients [median (interquartile range) age 65.2 (53.3-72.6) years, 62.4% male] were studied. During a median follow-up of 3.9 (1.6-7.2) years, 270 participants died. Patients in the mid- (6.25-7.73 × 103/µL, n = 203) and top-tertile (7.73-10.50 × 103/µL, n = 203) of normal WBC counts had significantly higher mortality than patients in the bottom-tertile (3.50-6.25 × 103/µL, n = 205). The adjusted hazard ratio for mortality relative to the bottom-tertile was 1.54, 95% confidence interval (CI) 1.05-2.25 and 2.20, 95% CI 1.46-3.32, for the mid- and top-tertiles, respectively. CONCLUSIONS: In hemodialysis patients, higher WBC count within the normal range is associated with increased long-term mortality. This finding is described for the first time and provides further insight into the clinical significance of a "normal" WBC count result in dialysis patients.

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OBJECTIVES: Our aim is to report four novel α-gal A gene (GLA) mutations resulting in Fabry disease (FD) and provide evidence of pathogenicity of the D313Y mutation regarding which contradictory data have been presented in the literature. SETTING AND PARTICIPANTS: Twenty-five family members of nine unrelated patients with definite FD diagnosis, 10 clinically suspected cases and 18 members of their families were included in this polycentric cohort study. PRIMARY AND SECONDARY OUTCOME MEASURES: Genotyping and measurement of lyso-Gb3 was performed in all individuals. The α-Gal A activity was measured in all men as well as plasma and urine Gb3 concentration in selected cases. Optical and electron microscopy was performed in kidney biopsies of selected patients. All the above were evaluated in parallel with the clinical data of the patients. RESULTS: Fourteen new cases of FD were recognised, four of which were carrying already described GLA mutations. Four novel GLA mutations, namely c.835C>T, c.280T>A, c.924A>C and c.511G>A, resulting in a classic FD phenotype were identified. Moreover, FD was definitely diagnosed in five patients carrying the D313Y mutation. Eight D313Y carriers were presenting signs of FD despite not fulfilling the criteria of the disease, two had no FD signs and two others were apparently healthy. CONCLUSIONS: Four novel GLA pathogenic mutations are reported and evidence of pathogenicity of the D313Y mutation is provided. It seems that the D313Y mutation is related to a later-onset milder phenotype than the typical phenotype with normal lysoGb3 concentration. Our study underlines the significance of family member genotyping and newborn screening to avoid misdiagnoses and crucial delays in diagnosis and treatment of the disease.

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BACKGROUND: Adjustment of the initial dose of paricalcitol in hemodialysis patients with secondary hyperparathyroidism (SHPT) on the basis of severity of SHPT generally is preferred in current practice. Whether the proposed dose, based on the formula baseline intact parathyroid hormone (iPTH [picograms per milliliter]) divided by 80, is the most appropriate has not been assessed adequately. METHODS: A double-blind randomized trial comparing iPTH/80 dose with the immediately lower iPTH/120 dose was undertaken. Forty-three hemodialysis patients with iPTH levels between 300 and 900 pg/mL (300 and 900 ng/L) were followed up for 12 weeks. The primary outcome was control of iPTH levels within a target range between 150 and 300 pg/mL (150 and 300 ng/L). RESULTS: No difference between the 2 dose groups was noted in time to achieve target iPTH levels of 150 to 300 pg/mL (150 to 300 ng/L). More episodes of excessive decrease in iPTH levels occurred in the iPTH/80 group compared with the iPTH/120 group (P = 0.003). Nine patients in the iPTH/80 group (45%) versus 2 patients in the iPTH/120 group (10%) had iPTH levels less than 150 pg/mL (<150 ng/L) in at least half the measurements performed during the second half of the study (P = 0.034). Increases in calcium levels were greater in the iPTH/80 group at all times during the study (P < 0.05 at weeks 4 and 10). The number of required dose reductions was significantly greater in the iPTH/80 group compared with the iPTH/120 group (P = 0.008). CONCLUSION: In hemodialysis patients with SHPT, a lower initial dose of iPTH/120 shows efficacy similar to that of the already widely used iPTH/80 scheme in reaching target iPTH levels (150 to 300 pg/mL [150 to 300 ng/L]), with less required dose adjustments, lower increase in calcium levels, and lower cost. In addition, the initial dose of paricalcitol based on the iPTH/80 formula leads significantly more patients to excessive suppression of iPTH (<150 pg/mL [<150 ng/L]) than the iPTH/120 dose.

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AIM: To investigate the abnormalities of cellular immune responses in patients on hemodialysis (HD) and in those on continuous ambulatory peritoneal dialysis (CAPD). PATIENTS AND METHODS: Forty-five (45) healthy volunteers, 34 patients on HD therapy, and 37 patients on CAPD were recruited for the present study. Lymphocyte subpopulations (CD2+, CD3+, CD3+/CD4+, CD3+/CD8+, CD3-/16+56+, CD19, and CD4/CD8) were determined by flow cytometry. RESULTS: Lymphopenia, decreased absolute counts, and altered percentage values of CD3+, CD3+/ 4+, and CD19+ subpopulations were found in both patient groups. The HD and CAPD patients showed increased percentages of natural killer cells (CD3-/16+56+) compared to controls but CD4+/CD8+ ratio showed no significant changes among uremic patients and controls. CONCLUSIONS: Replacement therapy may contribute to the quantitative alterations of immune subsets found in HD and CAPD patients compared to normal subjects. We speculate that these changes account, at least in part, for the immune dysregulation observed in patients with chronic renal failure. Analysis of lymphocyte subsets will help the research and the evaluation of the possible causes of immunodeficiency in uremic patients undergoing replacement therapy and will probably contribute to more efficient and preventive strategies.

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OBJECTIVE: The clinical efficacy of therapeutic apheresis is still controversial. We undertook a retrospective review of apheresis treatment to ascertain its safety and efficacy. METHODS: We reviewed 31 patients (13 male, 18 female). Plasmapheresis was performed on 7 patients with hematologic disorders, 5 patients with neurologic disorders, 6 patients with systemic diseases, and 3 patients with Lyell syndrome. Immunoadsorption onto protein A sepharose was evaluated as rescue therapy in 7 patients. Low-density lipoprotein (LDL) apheresis was performed on 3 patients. RESULTS: There were five mortalities due to serious complications of their primary disease. Most complications were mild such as hypotension and hypocalcemia. Two patients who received LDL apheresis had severe anaphylactic reactions. Apheresis was effective in the remaining 24 patients. CONCLUSIONS: The therapeutic apheresis consists of a continuously improving therapeutic method for diseases with high mortality and morbidity, especially in cases with poor outcome by using current medications.

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Reduction of pathological autoantibodies may be useful in the treatment of systemic lupus erythematosus (SLE). On the other hand clinically manifested myocarditis in SLE, though uncommon, may be life-threatening and its pathogenesis has been ascribed to autoimmunity. The aim of this study is to present a rare case of a patient with severe lupus myocarditis, where immunoadsorption (IA) was evaluated as rescue therapy. A case of SLE with initial manifestation of myocarditis is reported in a 29-year-old male who presented with arthritis, fever, lymphadenopathy, joint swelling and morning stiffness. Laboratory evaluation revealed increased antinuclear antibody (ANA), slightly decreased complement and positive anticoagulant panel. From the above clinical and laboratory features, criteria of SLE seemed applicable. During his hospitalization, the patient developed pericardial effusion and cardiogenic shock. Although pericardiotomy was performed and was treated with immunosuppressive agents, plasmapheresis and supported with current medications, his clinical condition remained critical with an ejection fraction of 20%. At this point it was decided to receive IA onto staphylococcal protein A. After 6 sessions with IA and concomitant immunosuppression, the patient responded well, his condition improved and was dismissed with an ejection fraction of 50%. Fulminant lupus myocarditis is a severe and rare situation lacking a satisfying specific therapy available today. In our presented case, IA in addition to immunosuppressive therapy was beneficial. Considering the benefits of our case and the current knowledge, it might be useful to clarify the open question in scale pilot studies.

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