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Vitamin D deficiency and insufficiency are highly prevalent in CKD, affecting over 80% of hemodialysis (HD) patients and requiring therapeutic intervention. Nephrological societies suggest the administration of cholecalciferol according to the guidelines for the general population. The aim of the observational study was to evaluate the efficacy and safety of the therapy with a high dose of cholecalciferol in HD patients with 25(OH)D deficiency and insufficiency to reach the target serum 25(OH)D level > 30 ng/mL. A total of 22 patients (16 M), with an average age of 72.5 ± 13.03 years and 25(OH)D concentration of 13.05 (9.00-17.90) ng/mL, were administered cholecalciferol at a therapeutic dose of 70,000 IU/week (20,000 IU + 20,000 IU + 30,000 IU, immediately after each dialysis session). All patients achieved the target value > 30 ng/mL, with a mean time of 2.86 ± 1.87 weeks. In the first week, the target level of 25(OH)D (100%) was reached by 2 patients (9.09%), in the second week by 15 patients (68.18%), in the fourth week by 18 patients (81.18%), and in the ninth week by all 22 patients (100%). A significant increase in 1,25(OH)2D levels was observed during the study. However, only 2 patients (9.09%) achieved a concentration of 1,25(OH)2D above 25 ng/mL-the lower limit of the reference range. The intact PTH concentrations remained unchanged during the observation period. No episodes of hypercalcemia were detected, and one new episode of hyperphosphatemia was observed. In conclusion, our study showed that the administration of a high-therapeutic dose of cholecalciferol allowed for a quick, effective, and safe leveling of 25(OH)D concentration in HD patients.

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There are several forms of maintenance high-efficiency hemodialysis (HD), including hemodiafiltrations (HDF) in different technical modes and expanded HD, using dialyzers with medium cut-off membranes. The aim of the study was to assess the intradialytic tolerance and length of dialysis recovery time (DRT) in these modalities. This is an exploratory, crossover study in maintenance HD patients with low comorbidity and no clinical indications for the use of high-efficiency HD, who were exposed to five intermittent dialyses in random order: high-flux hemodialysis (S-HD), expanded HD (HDx), pre-dilution HDF (PRE-HDF), mix-dilution HDF (MIX-HDF) and post-dilution HDF (POST-HDF). Twenty-four dialysis sessions of each method were included in the analysis. Dialysis parameters, including blood flow rate, dialysis fluid flow rate and temperature, and pharmacological treatment were constant. Average total convection volume for post-HDF, pre-HDF and mix-HDF were 25.6 (3.8), 61.5 (7.2) and 47.1 (11.4) L, respectively. During all therapies, patients were monitored for the similarity of their hydration statuses using bioimpedance spectroscopy, and for similar variability over time in systemic blood pressure and cardiac output, while peripheral resistance was monitored using impedance cardiography. The lowest frequency of all intradialytic adverse events were observed during HDx. Delayed DRT was the shortest during PRE-HDF. Patients were also more likely to report immediate recovery while receiving PRE-HDF. These differences did not reach statistical significance; however, the study results suggest that intradialytic tolerance and DRT may depend on the dialysis method used. This supports the need of taking into account patient preferences and quality of life while individualizing high-efficiency therapy in HD patients.

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The aim of the study was to provide a state-of-the-art review with regard to neuropsychiatric disorders associated with tuberous sclerosis complex (TSC). TSC is a rare genetic disease classified as a phacomatosis. Due to the wide spectrum of clinical symptoms of the disease, many cases remain undiagnosed. The vast majority of people with a mutation in the TSC1 or TSC2 genes develop some of the neuropsychiatric symptoms during their lifetime. Diagnostic criteria, neuroanatomical pathology and pathophysiology of psychiatric, neuropsychological, developmental and psychosocial symptoms present in TSC are described. The specificity of epilepsy in TSC and its role in neuropsychiatric and neuropsychological development are presented. All levels (intellectual, developmental, behavioral, psychiatric, school, neuropsychological and psychosocial) of tuberous sclerosis complex-associated neuropsychiatric disorders (TAND) are discussed in detail. The TAND Checklist - a tool for assessing all potentially disturbed aspects of functioning - was presented. The importance of proper diagnosis of neuropsychiatric disorders and multidisciplinary patient care was emphasized.

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INTRODUCTION: Tuberous Sclerosis Complex (TSC) is a rare genetic disease. Around 90% of individuals with TSC present some neuropsychiatric manifestations (TSC-associated neuropsychiatric disorders, TAND). To date, none of the studies have focused on the TAND profile of the adult population. Thus, the aim of the study was to describe their potential specific needs and difficulties, including differences in cohorts with or without epilepsy and/or intellectual disability. METHOD: The Polish version of the TAND Checklist was used for assessment of individuals with TSC. Participants had to meet the criteria for diagnosis of TSC. One hundred adult participants (forty-eight males/ fifty-two females; mean age 32.33 ± 11.29) were enrolled in the study. Epilepsy was present in 71% of patients; intellectual disability occurred in a total of 37%. RESULTS: Only 11% of individuals received complete TAND features examination in the past. Moreover, 91.5 of the subjects had four and more TAND symptoms. Intellectually disabled patients and those with epilepsy had more neuropsychiatric problems than epilepsy-free subjects. CONCLUSIONS: Findings reveal that TANDs are common in adults with TSC and are underdiagnosed. Most individuals present several behavioural and cognitive problems. Among psychiatric disorders, the most common are ASD, depression, and anxiety disorder. TAND screening should be widely disseminated and applied in clinical practice for early identification, prevention, and rehabilitation of their difficulties. TAND is one of the most significant issues affecting the quality of life of TSC patients and their carers.

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BACKGROUND: The efficacy of SARS-CoV-2 vaccination among kidney transplant recipients (KTR) is low. The main goal of this study was to analyze factors that may influence the humoral response to vaccination. METHODS: We analyzed the titer magnitude of IgG antibodies directed against spike (S)-SARS-CoV-2 antigen after the second dose of the mRNA vaccine in 142 infection naïve KTR (83 men, i.e., 58.4%) with a median age (IQR) of 54 (41-63), and 36 respective controls without chronic kidney disease. mRNA-1273 or BNT162b2 were applied in 26% and 74% of KTR, respectively. RESULTS: S-specific immune response (seroconversion) was seen in 73 (51.41%) of KTR, and in all controls 36 (100%). Independent predictors of no response were elder age, shorter transplantation vintage, and a more than two-drug immunosuppressive protocol. In subgroup analyses, the seroconversion rate was highest among KTR without MMF/MPS treatment (70%), treated with no more than two immunosuppressants (69.2%), treated without corticosteroid (66.7%), younger patients aged <54 years (63.2%), and those vaccinated with the mRNA-1273 vaccine (62.16%). The independent predictors of higher S-antibody titer among responders were younger age, treatment with no more than two immunosuppressants, and the mRNA-1273 vaccination. CONCLUSIONS: Our study confirmed a low rate of seroconversion after vaccination with the mRNA vaccine in KTR. The major modifiable determinants of humoral response were the composition of the immunosuppressive protocol, as well as the type of vaccine. The latter could be taken into consideration when initial vaccination as well as booster vaccination is considered in KTR.

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Kidney transplantation is the treatment of choice for end-stage kidney diseases. Unfortunately, kidney allograft recipients rarely develop tolerance or accommodation and require life-long immunosuppression. Among many other regulatory mechanisms, CD5+ B lymphocytes (mainly B-1a) seem to be involved in the process of allograft acceptance. These cells are the major source of natural, low-affinity antibodies, which are polyreactive. Thus, we hypothesized that CD5+ B cells could be referred to as a biomarker in those patients who developed accommodation towards kidney allotransplant. In this study, 52 low-immunized kidney transplant recipients were evaluated for transplant outcome up to 8 y post-transplant. The follow up included anti-HLA antibodies, B cells phenotype and cytokines. We have identified a cohort of recipients who produced alloantibodies (Abs+), which was associated with increased levels of CD5+ B cells, mainly during the first year after transplantation but also later on. Importantly, creatinine levels were comparable between Abs+ and Abs- allorecipients at 2 years after the transplantation and graft survival rate was comparable between these groups even eight years post-transplant. So, it seems that despite the presence of alloantibodies the graft function was sustained when the level of CD5+ B cells was increased. Targeting CD5+ B cells may be a valuable therapeutic option to increase transplant success. The phenotype can be also tried as a biomarker to increase the effectiveness of individualized post-transplant treatments.

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INTRODUCTION Sleep disturbances, similarly to constipation­related symptoms, are common problems in patients with chronic kidney disease (CKD) and are associated with worse health-related quality of life. OBJECTIVES The aim of the study was to investigate sleep problems in conservatively treated patients with CKD and to assess association between sleep quality and constipation in that population. PATIENTS AND METHODS In this cross­sectional study, 100 conservatively treated outpatients with CKD filled questionnaires addressing sleep quality (The Medical Outcomes Study 12­item Sleep Scale-Revised [MOS­Sleep­R]) and constipation­related symptoms (PAC­SYM, Rome III criteria). RESULTS The T scores of none of the assessed sleep domains differed across the estimated glomerular filtration rate terciles (all P >0.05). The scores from the PAC-SYM abdominal and stool subscales correlated with all assessed sleep quality domains. In both univariable and multivariable regression models adjusted for key clinical data, functional constipation, less than 7 bowel movements a week, abdominal discomfort, and pain as well as too small bowel movements were independently associated with increased prevalence ratio of decreased sleep quality. CONCLUSIONS In patients with nondialysis CKD, sleep disorders might have common etiological factors with constipation-related symptoms.

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Background: Constipation is a common gastrointestinal disorder that in general population is associated with worse health-related quality of life (HRQoL). The epidemiology of constipation has not been reliably determined in conservatively-treated CKD patients. We aimed to determine the prevalence of constipation and constipation-related symptoms in conservatively-treated CKD patients, to find factors associated with their altered prevalence ratio (PR), and to verify the associations between constipation and HRQoL. Methods: In this cross-sectional study, 111 conservatively-treated CKD outpatients fulfilled questionnaires that included questions addressing HRQoL (SF-36v2®), constipation-related symptoms (The Patient Assessment of Constipation-Symptoms questionnaire), the Bristol stool form scale (BSFS), Rome III criteria of functional constipation (FC), and frequency of bowel movement (BM). Results: Depending on the used definition, the prevalence of constipation was 6.6-28.9%. Diuretics and paracetamol were independently associated with increased PR of BSFS-diagnosed constipation (PR 2.86, 95% CI 1.28-6.37, P = 0.01) and FC (PR 2.67, 95% CI 1.07-6.64, P = 0.035), respectively. The most commonly reported symptoms were bloating (50.9%) and straining to pass a BM (42.7%). Abdominal discomfort (37.3%) was independently associated with worse scores in all analyzed HRQoL domains. In multiple regressions, FC and having <7 BM/week, but not BSFS-diagnosed constipation, were associated with lower scores in several HRQoL domains. Conclusions: Constipation and related symptoms are prevalent in CKD patients. FC and decreased frequency of defecation, but not BSFS-diagnosed constipation, are associated with worse assessment of HRQoL in conservatively-treated CKD patients.

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BACKGROUND: Kidney transplantation (KT) is the preferred treatment for end-stage kidney disease (ESKD), while preemptive (PE) living donor (LD) KT is associated with better survival, quality of life, and lower costs. Tuberous sclerosis complex (TSC) is a genetic multisystem disorder. Renal involvement (multiple bilateral angiomyolipoma [AMLs], cysts, renal cell carcinoma [RCC]) is related to significant morbidity, including ESKD and KT. Nephrectomy in TSC patients before KT is controversial. Affected kidneys carry a risk of hemorrhage or malignancy, while AMLs may be fat-poor and are often hardly distinguishable from RCC in magnetic resonance (MR)/computed tomography. On the other hand nephrectomy impedes PE KT. Mammalian target of rapamycin inhibitors (mTORi) have proved efficacy in many TSC complications, including AMLs, fat-poor AMLs, TSC-related RCC, and immunosuppressive (IS) treatment. CASE REPORT: A 29-year-old female TSC patient was referred for evaluation to the TSC reference center. Her family history was negative for TSC. A clinical evaluation revealed multisystem TSC manifestation (skin, brain, lungs, kidneys). MR disclosed indeterminate fat-poor renal lesions, possibly AMLs, but RCC could not be excluded. A comparison with previous MR did not show any significant progression. Due to ESKD, the patient was qualified for PE LD (mother) KT. mTORi, sirolimus, was used in IS. Creatinine at discharge was 2.1 mg/dL. Sixteen months later, MR showed significant reduction in tumor size. Two years after KT, graft function remained stable (creatinine 1.98 mg/dL). No complications related to renal lesions occurred. CONCLUSIONS: mTORi are the therapy of choice in TSC patients after KT, achieving IS effect and improvement in TSC manifestations while avoiding nephrectomy and management of patients with indeterminate renal lesions, especially in the case of PE KT.

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Human cytomegalovirus (HCMV) is considered to be a major pathogen that affects the outcome of solid organ transplantation (TX). Both recipient and donor may be HCMV positive, therefore HCMV re-infection is possible after TX. However, little is known how cytomegalovirus (CMV) transmitted from an infected donor to an infected recipient modulates the recipient's already suppressed immunity, and what the clinical consequences are. To investigate these issues, 52 kidney recipients were followed up for 2 years after TX. T, B and natural killer (NK) lymphocytes, naive and memory T subsets, CD28 expression, relative telomere length, CMV-specific lymphocytes and serum cytokines were measured several times post-TX. Patients were monitored for signs of CMV viremia and other infections. The most important observation was that CMV-specific lymphocytes expand vastly in HCMV-infected recipients who received kidneys from infected donors, in comparison with uninfected donors. Despite this, a higher rate of HCMV viremia was found. Immune deterioration was confirmed by an increased number of CD28-negative T lymphocytes, inverted CD4/CD8 index and shortened telomeres. This was superior in HCMV-infected recipients transplanted from infected donors, when compared with uninfected. In conclusion, CMV alters the immune system in kidney transplant recipients and promotes immune exhaustion.

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BACKGROUND: CMV infection remains major complication after kidney transplantation, thus diagnostics tools that would improve identification of individuals at risk of development of CMV - related complications are useful. For this reason, searching for proper immunological biomarkers candidates gives hope to individualize antiviral therapy and minimize side effects of antiviral drugs. OBJECTIVES: The purpose of this research was to assess immune assays that can be used to predict the likelihood of CMV viremia after kidney allotransplantation. STUDY DESIGN: In the study, immunological markers of CMV viremia were assessed in 52 kidney transplant recipients during two years lasting follow-up. Immunological markers associated with viral infection, like lymphocytosis, cytotoxic T lymphocytes (CTL) and serum cytokines levels were compared with less common immunological assays, like activated T lymphocytes, CMV-specific CTL stratified according to naïve/memory phenotype. The test to assess expression of CD28 antigen on CTL, as a possible additional marker of CMV-specificity, was developed. RESULTS: CD28-positive CMV-specific CTL have been found the most useful marker for CMV viremia prediction. Tested value of 3 cells/µl was found to be most suitable for CMV activation assessment with acceptable sensitivity and specificity. DISCUSSION: This preliminary report suggests that CD28-positive CMV-specific CTL could be put at the first line, as possible novel marker associated with CMV viremia development.

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