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The parathyroid gland is one of the main organs that regulate calcium and phosphorus metabolism. It is mainly composed of chief cells and oxyphil cells. Oxyphil cell counts are low in the parathyroid glands of healthy adults but are dramatically increased in patients with uremia and secondary hyperparathyroidism (SHPT). Increased oxyphil cell counts are related to drug treatment resistance, but the origin of oxyphil cells and the mechanism of proliferation remain unknown. Herein, three types of parathyroid nodules (chief cell nodules, oxyphil cell nodules and mixed nodules, respectively) excised from parathyroid glands of uremic SHPT patients were used for single-cell RNA sequencing (scRNA-seq), other molecular biology studies, and transplantation into nude mice. Through scRNA-seq of parathyroid mixed nodules from three patients with uremic SHPT, we established the first transcriptomic map of the human parathyroid and found a chief-to-oxyphil cell transdifferentiation characterized by gradual mitochondrial enrichment associated with the uremic milieu. Notably, the mitochondrial enrichment and cellular proliferation of chief cell and oxyphil cell nodules decreased significantly after leaving the uremic milieu via transplantation into nude mice. Remarkably, the phenotype of oxyphil cell nodules improved significantly in the nude mice as characterized by decreased mitochondrial content and the proportion of oxyphil cells to chief cells. Thus, our study provides a comprehensive single-cell transcriptome atlas of the human parathyroid and elucidates the origin of parathyroid oxyphil cells and their underlying transdifferentiating mechanism. These findings enhance our understanding of parathyroid disease and may open new treatment perspectives for patients with chronic kidney disease.
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Hiperparatireoidismo Secundário , Glândulas Paratireoides , Adulto , Animais , Camundongos , Humanos , Glândulas Paratireoides/metabolismo , Células Oxífilas , Camundongos Nus , Transdiferenciação Celular , Hiperparatireoidismo Secundário/genética , Hiperparatireoidismo Secundário/terapia , Análise de Sequência de RNARESUMO
Chronic kidney disease (CKD) is a strong risk factor for peripheral artery disease (PAD) that is associated with worsened clinical outcomes. CKD leads to the accumulation of tryptophan metabolites that are associated with adverse limb events in PAD and are ligands of the aryl hydrocarbon receptor (AHR), which may regulate ischemic angiogenesis. To test if endothelial cell-specific deletion of the AHR (AHRecKO) alters ischemic angiogenesis and limb function in mice with CKD subjected to femoral artery ligation. Male AHRecKO mice with CKD displayed better limb perfusion recovery and enhanced ischemic angiogenesis compared with wild-type mice with CKD. However, the improved limb perfusion did not result in better muscle performance. In contrast to male mice, deletion of the AHR in female mice with CKD had no impact on perfusion recovery or angiogenesis. With the use of primary endothelial cells from male and female mice, treatment with indoxyl sulfate uncovered sex-dependent differences in AHR activating potential and RNA sequencing revealed wide-ranging sex differences in angiogenic signaling pathways. Endothelium-specific deletion of the AHR improved ischemic angiogenesis in male, but not female, mice with CKD. There are sex-dependent differences in Ahr activating potential within endothelial cells that are independent of sex hormones.NEW & NOTEWORTHY This study provides novel insights into the mechanisms by which chronic kidney disease worsens ischemic limb outcomes in an experimental model of peripheral artery disease. Deletion of the aryl hydrocarbon receptor (AHR) in the endothelium improved ischemic angiogenesis suggesting that AHR inhibition could be a viable therapeutic target; however, this effect was only observed in male mice. Subsequent analysis in primary endothelial cells reveals sex differences in Ahr activating potential independent of sex hormones.
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Doença Arterial Periférica , Insuficiência Renal Crônica , Masculino , Feminino , Camundongos , Animais , Receptores de Hidrocarboneto Arílico/genética , Receptores de Hidrocarboneto Arílico/metabolismo , Células Endoteliais/metabolismo , Isquemia , Doença Arterial Periférica/metabolismo , Insuficiência Renal Crônica/genética , Insuficiência Renal Crônica/metabolismo , Hormônios Esteroides GonadaisRESUMO
OBJECTIVE: This study aimed to explore the serum levels of gasdermin D (GSDMD) in uremic (end-stage kidney disease, ESKD) patients and their correlation with vascular calcification (VC) and clinical results. METHODS: This prospective observational cohort study enrolled 213 ESKD patients who were undergoing regular maintenance hemodialysis (MHD) for > 3 months in our hospital from August 2019 to July 2022. The abdominal aortic calcification score (AACS) was used to assess the VC condition of patients with ESKD. Serum GSDMD, caspase-1, interleukin (IL)-6, IL-1ß, IL-18 and C-reactive protein (CRP) levels were measured using enzyme-linked immunosorbent assay (ELISA). Demographic and clinical data were obtained. All patients were followed up for 1 year, and patients with major adverse cardiovascular events (MACE) were defined as having a poor prognosis. All data used SPSS 26.0 to statistical analyses. RESULTS: The serum total cholesterol (TC) levels of patients in the AACS > 4 group were significantly elevated compared with those in the AACS ≤ 4 group. In addition, ESKD patients with an AACS > 4 had significantly higher serum levels of GSDMD, caspase-1, IL-6, IL-18 and IL-1ß. Moreover, Pearson's analysis supported a positive correlation between GSDMD and caspase-1, IL-6, and IL-1ß. In addition, we found that GSDMD levels were positively correlated with the clinical data (AACS scores and serum TC levels) of patients with ERSD. Additionally, ROC curves showed that the serum levels of GSDMD could be a potential predictive biomarker of moderate/severe VC and prognosis in patients with ESKD. Finally, the results of logistic regression indicated that GSDMD and AACS scores were risk factors for poor prognosis in patients with ESKD. CONCLUSION: Serum GSDMD levels were remarkably elevated in patients with ESKD with moderate/severe calcification. In addition, serum levels of GSDMD could be a potential predictive biomarker of moderate/severe VC and prognosis in patients with ESKD.
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Gasderminas , Interleucina-18 , Humanos , Estudos Prospectivos , Interleucina-6 , Peptídeos e Proteínas de Sinalização Intracelular , Piroptose , Biomarcadores , CaspasesRESUMO
Heart failure and chronic kidney disease (CKD) share several mediators of cardiac pathological remodelling. Akin to heart failure, this remodelling sets in motion a vicious cycle of progressive pathological hypertrophy and myocardial dysfunction in CKD. Several decades of heart failure research have shown that beta blockade is a powerful tool in preventing cardiac remodelling and breaking this vicious cycle. This phenomenon remains hitherto untested in CKD. Therefore, we set out to test the hypothesis that beta blockade prevents cardiac pathological remodelling in experimental uremia. Wistar rats had subtotal nephrectomy or sham surgery and were followed up for 10 weeks. The animals were randomly allocated to the beta blocker metoprolol (10 mg/kg/day) or vehicle. In vivo and in vitro cardiac assessments were performed. Cardiac tissue was extracted, and protein expression was quantified using immunoblotting. Histological analyses were performed to quantify myocardial fibrosis. Beta blockade attenuated cardiac pathological remodelling in nephrectomised animals. The echocardiographic left ventricular mass and the heart weight to tibial length ratio were significantly lower in nephrectomised animals treated with metoprolol. Furthermore, beta blockade attenuated myocardial fibrosis associated with subtotal nephrectomy. In addition, the Ca++- calmodulin-dependent kinase II (CAMKII) pathway was shown to be activated in uremia and attenuated by beta blockade, offering a potential mechanism of action. In conclusion, beta blockade attenuated hypertrophic signalling pathways and ameliorated cardiac pathological remodelling in experimental uremia. The study provides a strong scientific rationale for repurposing beta blockers, a tried and tested treatment in heart failure, for the benefit of patients with CKD.
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Insuficiência Cardíaca , Insuficiência Renal Crônica , Humanos , Ratos , Animais , Ratos Wistar , Metoprolol/farmacologia , Insuficiência Renal Crônica/tratamento farmacológico , Hipertrofia , FibroseRESUMO
BACKGROUND: Atrial fibrillation (AF) can lead to stroke, heart failure, and mortality and has a greater prevalence in dialysis patients than in the general population. Several studies have suggested that uremic toxins may contribute to the development of AF. However, the association between dialysis adequacy and incident AF has not been well established. METHODS: In this retrospective nationwide cohort study, we analyzed data from the Korean National Periodic Hemodialysis Quality Assessment from 2013 to 2015 of patients who received outpatient maintenance hemodialysis 3× a week. The main exposure was single pooled Kt/V (spKt/V), which is the dialysis adequacy index, and the primary outcome was the development of AF. For the primary analysis, patients were categorized into quartiles according to baseline spKt/V. The lowest quartile, representing the lowest adequacy, was used as the reference group. Fine-Gray subdistribution hazard models were used, treating all-cause mortality as a competing risk. RESULTS: Of 25 173 patients, the mean age was 60 (51-69) years, and 14 772 (58.7%) were men. During a median follow-up of 5.7 years, incident AF occurred in a total of 3883 (15.4%) patients. Participants with a higher spKt/V tended to have lower AF incidence. In survival analysis, a graded association was observed between the risk of incident AF and spKt/V quartiles: subdistribution hazard ratios and 95% CIs for the second, third, and the highest quartile compared with the lowest quartile were 0.90 (95% CI, 0.82-0.98), 0.84 (95% CI, 0.77-0.93), and 0.79 (95% CI, 0.72-0.88), respectively. CONCLUSIONS: This nationwide cohort study showed that a higher spKt/V is associated with a reduced risk of incident AF. These findings suggests that reducing uremic toxin burden through enhanced dialysis clearance may be associated with a lower risk of AF development in patients undergoing maintenance hemodialysis.
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A 14-year-old male tiger developed anorexia with elevated blood urea nitrogen and creatinine levels. The patient had a palpable abdominal mass and demonstrated neutrophilic leukocytosis and anaemia. Leukocytes, yeast and bacteria were present in the urine. The animal was non-responsive to therapy and was subsequently euthanised. Extensive acute renal papillary necrosis (RPN) with pyelonephritis, chronic nephritis and polycystic renal disease were evident during gross and microscopic pathology examinations. The histologic occurrence of fungal spores and pseudohyphae morphologically consistent with Candida species were observed within the necrotic papillary regions of the kidney and within multiple foci of mild parakeratotic hyperkeratosis present in the gingiva and tongue. Candida albicans along with a slight growth of Escherichia coli were recovered from kidney cultures. Possible contributory factors for the renal candidiasis and associated RPN include predisposing oral candidiasis, polycystic renal disease, ischaemic nephrosclerosis, age-associated or other forms of immunodeficiency and therapy with meloxicam, a non-steroidal anti-inflammatory drug. The absence of apparent lower urinary tract involvement coupled with the presence of intravascular renal 'Candida emboli' suggest that chronic oral candidiasis was the probable source of the kidney infection.
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Candidíase , Tigres , Animais , Masculino , Candidíase/veterinária , Candidíase/tratamento farmacológico , Candidíase/microbiologia , Necrose Papilar Renal/veterinária , Necrose Papilar Renal/etiologia , Candida albicans/isolamento & purificação , Animais de Zoológico , Nefropatias/veterinária , Nefropatias/microbiologia , Nefropatias/patologia , Nefropatias/etiologiaRESUMO
OBJECTIVE: To determine whether urea nitrogen and creatinine levels differ in lacrimal fluid (LF) and serum (SER) in nonazotemic (control) and azotemic dogs and whether there is an agreement between LF and SER. METHODS: A prospective observational study was performed at the Auburn University Small Animal Teaching Hospital between May 2023 and March 2024. Forty control and 38 azotemic dogs were enrolled. Twenty microliters of LF per eye was collected with microcapillary tubes, and 3 mL of blood was drawn. Bland-Altman plot and intraclass correlation coefficient (ICC) were used to evaluate the agreement between LF and SER. RESULTS: There was good agreement between LF and SER levels of urea nitrogen in the control group (Bland-Altman plot mean bias of -0.8108 ± 2.407 mg/dL; ICC of 0.874 [95% CI, 0.773 to 0.934]) and the azotemic group (Bland-Altman plot mean bias of -9.681 ± 23.89 mg/dL; ICC of 0.82 [95% CI, 0.658 to 0.906]). There was poor agreement between LF and SER concentrations for creatinine in the control and azotemic groups, with only 26 dogs with creatinine detectable in LF. CONCLUSIONS: Lacrimal fluid and SER concentrations of urea nitrogen showed good agreement in both the control and azotemic groups, whereas poor agreement was found for creatinine in both groups. CLINICAL RELEVANCE: Measurement of urea nitrogen in LF may provide an alternative to blood for diagnosing uremia. However, additional research is necessary before substituting LF for SER.
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Uremia, also known as uremic syndrome, refers to the clinical symptoms in the final stage of renal failure. The definition of the term has changed over time due to an improved comprehension of the kidney's function and the advancement of dialysis technology. Here, we aim to present an overview of the various concepts that have developed regarding uremia throughout the years. We provide a comprehensive review of the historical progression starting from the early days of Kolff and his predecessors, continuing with the initial research conducted by Niwa et al., and culminating in the remote sensing hypothesis of Nigam. Additionally, we explore the subsequent investigation into the function of these toxins as signaling molecules in various somatic cells.
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Uremia , Toxinas Urêmicas , Uremia/história , Uremia/metabolismo , Humanos , História do Século XX , Toxinas Urêmicas/metabolismo , Toxinas Urêmicas/história , História do Século XXI , AnimaisRESUMO
Immunoglobulin G-4 related disease (IgG4-RD) is an immune-mediated, inflammatory disease that often involves multiple organ systems. IgG4-RD can be classified according to the organs involved. Type 1 IgG4-RD is related to acute pancreatitis and sclerosing cholangitis. Disease manifestation is also seen in the retroperitoneal region, pelvic organs, and orbital space. Here we describe a rare case of IgG4-RD causing isolated acute kidney injury.
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BACKGROUND: In males with end stage renal disease biochemical hypogonadism is a frequent finding. Testosterone and sex hormone binding globulin (SHBG) have been associated with insulin resistance, a well-known condition in uremia. The aim of the present study was to investigate in males on chronic hemodialysis the relationship of testosterone and SHBG serum levels with insulin resistance. METHODS: In a cross-sectional study we enrolled men treated with chronic hemodialysis who did not suffer from an acute illness or other endocrinopathy, as well as primary hypogonadism, and were not hospitalised. Diabetes mellitus, diabetic nephropathy or previous transplantation were not exclusion criteria. As controls we used a community-based group of healthy males matched for age and Body Mass Index (BMI). We assessed the BMI (kg/m2) from body weight and height, the body fat content (%) by bioelectrical impedance and serum testosterone (ng/ml), SHBG (nmol/L) and estradiol (pg/ml) by standard methods. Testosterone < 3.25 ng/ml defined biochemical hypogonadism. In non-diabetic males, we calculated the homeostasis model assessment index (HOMA-R), an estimate of insulin resistance, from serum fasting insulin and glucose. RESULTS: 27 men (age 54.4 ± 19 years) on chronic hemodialysis (treatment duration 29.1 ± 14.4 months) and 51 healthy men (age 47.1 ± 9.6 years) were included. In men on hemodialysis vs. healthy men there were increased serum levels of SHBG (40.9 ± 26.9 vs. 27.6 ± 11.9 nmol/L; p = 0.031) and a significantly enhanced frequency of biochemical hypogonadism (22.2 vs. 3.9%; p = 0.011). In cases without diabetes (n = 22) a significant correlation was observed between the HOMA-R (r = -0.586, p = 0.004) and the fasting insulin levels (r = -0.650, p = 0.001) on the one hand and the serum SHBG levels on the other. CONCLUSIONS: Our findings confirm enhanced prevalence of biochemical hypogonadism in males on chronic hemodialysis. In non-diabetic cases the serum levels of SHBG correlated with serum insulin and insulin resistance.
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Cardiovascular disease (CVD) frequently occurs in patients with chronic kidney disease (CKD), particularly those undergoing dialysis. The mechanisms behind this may be related to traditional risk factors and CKD-specific factors that accelerate atherosclerosis and vascular calcification in CKD patients. The accumulation of uremic toxins is a significant factor in CKD-related systemic disorders. Basic research suggests that indoxyl sulfate (IS), a small protein-bound uremic toxin, is associated with macrophage dysfunctions, including increased oxidative stress, exacerbation of chronic inflammation, and abnormalities in lipid metabolism. Strategies to mitigate the toxicity of IS include optimizing gut microbiota, intervening against the abnormality of intracellular signal transduction, and using blood purification therapy with higher efficiency. Further research is needed to examine whether lowering protein-bound uremic toxins through intervention leads to a reduction in CVD in patients with CKD.
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Aterosclerose , Indicã , Macrófagos , Insuficiência Renal Crônica , Uremia , Indicã/toxicidade , Humanos , Macrófagos/efeitos dos fármacos , Animais , Toxinas Urêmicas , Microbioma Gastrointestinal/efeitos dos fármacos , Estresse Oxidativo/efeitos dos fármacosRESUMO
OBJECTIVES: This study aimed to investigate risk factors for major adverse cardiovascular and cerebrovascular events (MACCEs) in patients with uremia and hypertension during maintenance hemodialysis (MHD). METHODS: Clinical data of patients with uremia and refractory hypertension admitted to Changzhou Fourth People's Hospital (Changzhou Tumor Hospital) from February 2018 to February 2022 were retrospectively collected and analyzed. All patients were treated with MHD and categorized into an MACCE group and a non-MACCE group according to whether MACCEs occurred during the treatment cycle. Univariate analysis and multivariate logistic regression analysis were applied to identify the risk factors for MACCEs in the patients during the treatment period. RESULTS: (1) A total of 156 patients were included in this study, among whom 75 patients were in the MACCE group and 81 in the non-MACCE group, with an MACCE incidence of 48.08%. (2) Diabetes, body mass growth rate, triglyceride (TG), N-terminal pro-brain natriuretic peptide (NT-proBNP), as well as the standard deviation (SD) and coefficient of variability (CV) for both systolic (SBP) and diastolic blood pressure (DBP) showed significant differences between the two groups, with P<0.05. (3) Diabetes, body mass growth rate ≥5.54%, TG≥1.40 mmol/L, NT-proBNP≥5.82 ng/L, SBP-SD≥13.52, SBP-CV≥8.63, DBP-SD≥8.14, and DBP-CV≥8.82 were found to be risk factors for MACCEs in the patients. CONCLUSIONS: The incidence of MACCEs in patients with uremia and hypertension during MHD was associated with diabetes, body mass growth rate, TG, NT-proBNP, SBP-SD, SBP-CV, DBP-SD, and DBP-CV.Early screening for high-risk patients and positive intervention measures should be given to reduce the risk of MACCEs to enhance the safety of dialysis procedures.
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BACKGROUND: Thrombotic microangiopathy (TMA) is associated with carfilzomib, and knowledge of carfilzomib-induced TMA is based mainly on case reports. This study investigated the clinical characteristics of patients with carfilzomib-induced TMA and provided a reference for the rational use of carfilzomib. METHODS: Reports of carfilzomib-induced TMA were collected for retrospective analysis by searching the Chinese and English databases from inception to January 31, 2024. RESULTS: Sixty-six patients were included, with a median age of 63 years (range 39, 85). The median time to onset of TMA was 42 days (range 1, 1825) from initial administration, and the median number of cycles was 3 cycles (range 1, 15). Hemolytic anemia was recorded in 64 patients, with a median of 8.3 g/dL (range 4.6, 13). Sixty-three patients had thrombocytopenia with a median of 18 × 109/L (range 1, 139). The median value of increased LDH was 1192 IU/L (range 141, 5378). ADAMTS13 activity was normal in 41 (62.1 %) of the 42 patients. Mutations were found in 9 (13.6 %) of the 15 patients. Fifty-seven patients achieved a clinical response after discontinuing carfilzomib and receiving therapeutic plasma exchange (53.0 %), eculizumab (24.2 %), or hemodialysis (39.4 %). CONCLUSION: Carfilzomib-induced TMA is an important adverse event that should be considered in patients receiving carfilzomib for multiple myeloma with anemia, thrombocytopenia, and acute kidney injury. Withdrawal of carfilzomib and treatment with eculizumab have proven successful in some patients.
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Oligopeptídeos , Microangiopatias Trombóticas , Humanos , Microangiopatias Trombóticas/induzido quimicamente , Masculino , Pessoa de Meia-Idade , Feminino , Oligopeptídeos/efeitos adversos , Oligopeptídeos/uso terapêutico , Idoso , Adulto , Estudos Retrospectivos , Idoso de 80 Anos ou mais , Resultado do Tratamento , Proteína ADAMTS13/genética , Mieloma Múltiplo/tratamento farmacológico , Anticorpos Monoclonais Humanizados/efeitos adversos , Anticorpos Monoclonais Humanizados/uso terapêutico , Trombocitopenia/induzido quimicamenteRESUMO
Regulatory T cells (Tregs) are crucial immune cells that initiate a tolerable immune response. Transforming growth factor-beta (TGFß) is a key cytokine produced by Tregs and plays a significant role in stimulating tissue fibrosis. Systemic sclerosis, an autoimmune disease characterized by organ fibrosis, is associated with an overrepresentation of regulatory T cells. This review aims to identify Treg-dominant tolerable host immune reactions and discuss their association with scleroderma and end-stage organ failure. End-stage organ failures, including heart failure, liver cirrhosis, uremia, and pulmonary fibrosis, are frequently linked to tissue fibrosis. This suggests that TGFß-producing Tregs are involved in the pathogenesis of these conditions. However, the exact significance of TGFß and the mechanisms through which it induces tolerable immune reactions during end-stage organ failure remain unclear. A deeper understanding of these mechanisms could lead to improved preventive and therapeutic strategies for these severe diseases.
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Background/Objectives: Magnetic Resonance Imaging (MRI) is essential in diagnosing neurological conditions, offering detailed insights into brain pathology. Uremic encephalopathy (UE) is a severe neurological disorder resulting from renal failure, characterized by cognitive impairments and brain abnormalities due to the accumulation of uremic toxins (UTs). Despite extensive research on UTs, there is a significant gap in the detailed characterization of MRI findings in UE patients. This study aims to bridge this gap by conducting a comprehensive literature review of cerebral MRI findings in UE. We hypothesize that specific MRI patterns correlate with the severity and clinical manifestations of UE, thereby enhancing diagnostic accuracy and improving patient outcomes. Methods: A literature review was performed using PubMed, Cochrane Library, and Google Scholar. The search terms included "uremic encephalopathy MRI", "uremia and kidney failure MRI", and "toxic and metabolic or acquired encephalopathies MRI". The inclusion criteria were original articles on UE and MRI findings published in English. Results: Common MRI sequences include T1-weighted, T2-weighted, FLAIR, and DWI. Frequent MRI findings in UE are cytotoxic and vasogenic brain edema in regions such as the basal ganglia and periventricular white matter. Patterns like the "lentiform fork sign" and basal ganglia involvement are key indicators of UE. Conclusions: MRI plays a crucial role in diagnosing UE by identifying characteristic brain edema and specific patterns. A comprehensive diagnostic approach, incorporating clinical, laboratory, and imaging data, is essential for accurate diagnosis and management. The study calls for larger well-designed cohorts with long-term follow-up to improve the understanding and treatment of UE.
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BACKGROUND: Consultation-liaison (CL) psychiatrists are frequently asked to consult on various abnormal movements(1). CL psychiatrists can be instrumental in aiding the primary teams to identify and manage these movement disorders. In this manuscript, we provide an illustrative case of a patient presenting with myoclonus and offer a review on this important topic. Myoclonus accompanied by delirium represents a rare post-transplant complication and can be associated with heightened morbidity and mortality. The incidence of this complication in solid organ transplant (SOT) recipients is scarcely documented, and its pathophysiology remains inadequately understood. Potential etiologies in the intensive care unit (ICU) are numerous and likely multifactorial. The literature lacks detailed descriptions of the correlation and association between myoclonus and uremia. Management of this condition requires a multimodal approach, focusing on resolving underlying metabolic disturbances and providing symptomatic treatment. OBJECTIVES: This manuscript describes the clinical presentation of myoclonus in a liver transplant recipient accompanied by delirium and precipitated by uremia. We aim to highlight the diagnostic and therapeutic complexities, help providers distinguish myoclonus from other movement disorders, and aid appropriate management. METHODS AND RESULTS: We present a case of acute myoclonus in an elderly female liver transplant recipient precipitated by uremia and improved after continuous renal replacement treatment. In addition, we conducted a systematic review utilizing EMBASSE and PubMed of reported cases of myoclonus, delirium, and/or encephalopathy accompanied by uremia. We included 12 manuscripts in our review and discussed their findings. CONCLUSION: CL psychiatrists are frequently consulted for a range of movement disorders in the ICU, including myoclonus. Accurate diagnosis and identification of contributing etiologies are critical in these cases. Management typically involves addressing the underlying disorder, such as using dialysis for uremia, alongside symptomatic treatment with benzodiazepines to mitigate the frequency and amplitude of myoclonus. This approach helps to alleviate both the physical burden and psychological distress associated with the condition. This case underscores the pivotal role of the CL psychiatrist within a complex multidisciplinary team, contributing to diagnostic precision and optimization of management strategies for movement disorders.
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Acute and chronic kidney disease (CKD) have known neurological associations resulting from uremia, electrolyte disturbances, comorbidities such as hypertension, or other toxin accumulation. Reversible focal neurological deficits are relatively uncommon and poorly understood sequelae of kidney disease. Herein, we describe an unusual case of an adolescent male who developed acute aphasia during his initial presentation for acute kidney injury (AKI) superimposed on progressive CKD stage 5 associated with uremia and multiple electrolyte derangements. Symptoms resolved within one day of initiating continuous renal replacement therapy (CRRT) and gradual electrolyte and uremia correction. Such transient focal neurological deficits in AKI superimposed on progressive CKD in the pediatric population has not been widely reported.
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A patient who had been diagnosed with infantile retinal dystrophy developed renal failure in his twenties, at which time the diagnosis was revised to Senior-Loken syndrome. He was poorly compliant. At 36 years old, he experienced a sudden drop in visual acuity in the setting of cramping and fatigue and was found to be in uremic crisis. Six months after the event and its treatment, his vision failed to improved. Optic nerve pallor was out of proportion to the retinal dystrophy, and the presumed reason for his new visual loss was uremic optic neuropathy. The patient's younger sister also had been diagnosed with infantile retinal dystrophy, and metabolic screening confirmed subclinical renal dysfunction that was to be carefully followed going forward. Infantile retinal dystrophy can be associated with later systemic disease. Early detection of such disease can potentially decrease morbidity. Patients with retinal dystrophy can develop new visual loss from causes other than the retinopathy itself.
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As dysbiosis of gut microbiota is recognized as a major risk factor for malnutrition in hemodialysis (HD) patients, we aimed to assess the effects of fortified synbiotic dessert on malnutrition, oxidative stress, inflammation, and quality of life in patients undergoing hemodialysis. A total of 50 hemodialysis patients were randomized into two groups of intervention and control to consume either 50 g of synbiotic dessert fortified with vitamin D (1000 IU) and calcium (500 mg) (FSD) or 50 g of control dessert (CD) for 8 weeks, respectively. Changes in nutritional status [Subjective Global Assessment (SGA)], anthropometric measures, malondialdehyde (MDA), total antioxidant capacity (TAC), high-sensitivity C-reactive protein (hs-CRP), ferritin, biochemistry [serum albumin, vitamin D, creatinine, blood urea nitrogen (BUN), complete blood count (CBC), and electrolytes], and quality of life were assessed before and at the end of the trial. The SGA scores and serum ferritin levels decreased significantly in the FSD group compared to the control group (p = .01 and p = .03, respectively). Regarding other markers, no statistically significant changes were found comparing the two groups. This novel fortified synbiotic dessert as a functional food may be effective in reducing the severity of malnutrition by improving SGA score in short term in hemodialysis patients. Thus, it is suggested to do further studies to elucidate the possible mechanisms related to the effects of this dessert on microbiota, skeletal muscle mass, and inflammation in HD in long term.
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Introducción: Las mioclonías son contracciones musculares paroxísticas de corta duración o pérdida abrupta del tono muscular, denominadas mioclonías positivas y negativas, respectivamente. Se presenta un caso clínico de mioclonías positivas y negativas generalizadas y se pretende describir los múltiples mecanismos fisiopatológicos y etiologías que lo desencadenan. Presentación del caso: Hombre de 35 años, con diabetes mellitus tipo 1 complicada con enfermedad renal diabética en hemodiálisis, desarrolló una bacteriemia asociada a catéter por Staphylococcus aureus y presentó mioclonías positivas y negativas. Se identificaron como posibles desencadenantes la uremia, la infección y los fármacos con potencial promioclónico; el hallazgo incidental de una lesión isquémica en núcleo caudado no explicaba la semiología encontrada en el paciente. Se hizo el control y retiro de todos los factores promioclónicos enunciados, junto a manejo farmacológico con levetiracetam, y con ello se logró el control de los síntomas. Discusión: Los pacientes con enfermedad renal crónica son susceptibles a la acumulación de productos tóxicos de tipo guanidinas, que tienen potencial para producir mioclonías. Además, las infecciones, el uso de fármacos con potencial promioclónico y lesiones estructurales como las isquemias corticales son etiologías que deben considerarse en el diagnóstico diferencial. El mayor impacto en los síntomas se observa con el control del factor desencadenante, y, en caso de persistir, la terapia farmacológica proporciona buenos resultados. Conclusión: Las mioclonías son trastornos del movimiento relativamente comunes en la enfermedad renal crónica. La identificación del desencadenante es crucial para su manejo junto al uso de fármacos con actividad antimioclónica.
Introduction: Myoclonus are paroxysmal muscle contractions of short duration or abrupt loss of muscle tone, called positive and negative myoclonus respectively. A clinical case of generalized positive and negative myoclonus is presented and the aim is to describe the multiple pathophysiological mechanisms and etiologies that trigger it. Case presentation: A 35-year-old man with type 1 diabetes mellitus complicated by diabetic kidney disease on hemodialysis developed catheter-associated bacteremia due to Staphylococcus aureus and presented positive and negative myoclonus. Uremia, infection, and drugs with pro-myoclonic potential were identified as possible triggers; The incidental finding of an ischemic lesion in the caudate nucleus did not explain the semiology found in the patient. The control and removal of all the pro-myoclonic factors mentioned was carried out, along with pharmacological management with levetiracetam, thus achieving control of the symptoms. Discussion: Patients with chronic kidney disease are susceptible to the accumulation of guanidine-type toxic products, which have the potential to produce myoclonus. Furthermore, infections, the use of drugs with pro-myoclonic potential and structural lesions such as cortical ischemia are etiologies that should be considered in the differential diagnosis. The greatest impact on symptoms is observed with the control of the triggering factor and if it persists, pharmacological therapy provides good results. Conclusion: Myoclonus are relatively common movement disorders in chronic kidney disease. Identification of the trigger is crucial for its management along with the use of drugs with anti-myoclonic activity.