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Objective: In patients with end-stage kidney disease, kidney transplantation is the kidney replacement therapy option that provides the most successful survival. However, immunosuppression agents administered after kidney transplantation can increase the risk of opportunistic infections. Microsporidia are obligate intracellular pathogens that can be fatal in immunosuppressed patients. The present study aimed to determine the prevalence of microsporidia in kidney transplantation recipients and the molecular characterization of the detected species. Methods: To evaluate the prevalence of renal microsporidiosis in kidney transplant recipients, the urine samples from a total of 325 patients were analyzed by real-time and nested polymerase chain reaction for Encephalitozoon spp. and Enterocytozoon bieneusi. Results: Only one (0.4%) sample from the adult patient was positive for the Encephalitozoon species, while no positivity was found in pediatric patients. It was determined as Encephalitozoon intestinalis by ITS rRNA gene region sequence analysis. A microsporidia species obtained from humans in Türkiye has been characterized for the first time and registered in GenBank. Conclusion: Our epidemiological results show that the prevalence of renal microsporidiosis in kidney transplant recipients is very low. In addition, as a result of the phylogenetic analysis of the detected isolate, it was observed that it was 100% identical to the isolates reported from dogs in Kayseri, Türkiye. This situation provided essential data regarding the zoonotic transmission dynamics of microsporidia.
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Encephalitozoon , Encefalitozoonosis , Trasplante de Riñón , Microsporidiosis , Filogenia , Humanos , Trasplante de Riñón/efectos adversos , Prevalencia , Masculino , Adulto , Encefalitozoonosis/epidemiología , Femenino , Encephalitozoon/genética , Encephalitozoon/aislamiento & purificación , Niño , Turquía/epidemiología , Microsporidiosis/epidemiología , Persona de Mediana Edad , Adolescente , Adulto Joven , Reacción en Cadena de la Polimerasa , Huésped Inmunocomprometido , Preescolar , Anciano , Enterocytozoon/genética , Enterocytozoon/aislamiento & purificación , AnimalesRESUMEN
INTRODUCTION: Cardiovascular diseases constitute a significant cause of morbidity and mortality in individuals with autosomal dominant polycystic kidney disease (ADPKD). This study aimed to assess the long-term effects of tolvaptan on the kidneys and heart in rapidly progressing ADPKD. METHODS: Among 354 patients diagnosed with ADPKD, 58 meeting the eligibility criteria for tolvaptan were included in the study. The study comprised two groups with similar demographic and clinical characteristics: 29 patients receiving tolvaptan treatment and 29 in the control group. Several included genetic analysis, magnetic resonance imaging, and echocardiography. Clinical and cardiac changes were recorded in both groups after a 3-year follow-up. RESULTS: Tolvaptan treatment demonstrated a significant reduction in the rate of eGFR decline compared to the control group. Furthermore, it was observed that tolvaptan could prevent the development of cardiac arrhythmias by inhibiting an increase in QTc interval and heart rate. CONCLUSION: These findings suggest that, in addition to slowing kidney progression in ADPKD management, tolvaptan may potentially benefit in preventing cardiac complications.
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Antagonistas de los Receptores de Hormonas Antidiuréticas , Tasa de Filtración Glomerular , Riñón Poliquístico Autosómico Dominante , Tolvaptán , Humanos , Tolvaptán/uso terapéutico , Riñón Poliquístico Autosómico Dominante/tratamiento farmacológico , Riñón Poliquístico Autosómico Dominante/complicaciones , Masculino , Femenino , Antagonistas de los Receptores de Hormonas Antidiuréticas/uso terapéutico , Adulto , Persona de Mediana Edad , Tasa de Filtración Glomerular/efectos de los fármacos , Progresión de la Enfermedad , Imagen por Resonancia Magnética , Ecocardiografía , Riñón/efectos de los fármacos , Riñón/fisiopatología , Enfermedades Cardiovasculares/prevención & control , Enfermedades Cardiovasculares/etiología , Estudios de SeguimientoRESUMEN
INTRODUCTION: This study aimed to evaluate the effect of warm water footbaths on comfort, fatigue, and dialysis symptoms in patients undergoing hemodialysis. METHODS: Data were collected from a total of 58 patients, 31 in the intervention group and 27 in the placebo group. The data in the study are collected using the intervention and control group informed volunteer Form, Patient Demonstration Form, foot Bath Application Monitoring Chart, fatigue VAS Scale Form, Dialysis Symptom Index, and Hemodialysis Comfort Scale (HCS). RESULTS: In the second follow-up in the intervention group, HCS was determined to significantly increase all sub-size and total score averages by the first trace (p < 0.05). VAS fatigue point averages were significantly lower (p < 0.05) in the intervention group. CONCLUSION: It was determined that the footbath applied to patients who received hemodialysis treatment increased comfort and reduced fatigue and dialysis symptoms.
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Diálisis Renal , Proyectos de Investigación , Humanos , Diálisis Renal/efectos adversos , Fatiga/etiología , Fatiga/terapiaRESUMEN
BACKGROUND: Microscopic polyangiitis (MPA), a kind of antineutrophil cytoplasmic autoantibody associated vasculitis (AAV), predominantly affects small-sized vessels. MPA is a significant cause of the pulmonary-renal syndrome. Pauci-immune necrotizing and crescentic glomerulonephritis is the typical renal histological feature of AAV. Tubulointerstitial lesions may occur and mostly form with inflammatory cell infiltration in the interstitium. However, a few cases reported only tubulointerstitial involvement without glomerular lesions in patients with MPA. CASE PRESENTATION: We present an MPA case, a 70-year-old male patient diagnosed with acute kidney injury accompanying the dialysis requirement. Only acute tubulointerstitial nephritis was revealed in kidney biopsy without evidence of glomerular injury. Also, interstitial pulmonary fibrosis was determined on computerized tomography, and myeloperoxidase antineutrophil cytoplasmic autoantibody was positive. Consequently, we have considered the main diagnosis as MPA. We did not prefer a standard tubulointerstitial nephritis treatment regimen due to the presence of life-threatening systemic vasculitis. Treatment was established like crescentic glomerulonephritis. Induction therapy consisted of pulse steroid, cyclophosphamide, and plasmapheresis. Unfortunately, severe SARS-CoV-2 infection caused death during induction therapy in this case. CONCLUSIONS: The lack of glomerular injury and solely interstitial inflammation is atypical regarding AAV involvement in the kidney. This diversity might be initially considered as only a simple histological elaboration. However, it is a significant entity for guiding the treatment of AAV.
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COVID-19 , Glomerulonefritis , Poliangitis Microscópica , Nefritis Intersticial , Masculino , Humanos , Anciano , Poliangitis Microscópica/complicaciones , Poliangitis Microscópica/diagnóstico , Diálisis Renal/efectos adversos , COVID-19/complicaciones , SARS-CoV-2 , Riñón/patología , Glomerulonefritis/complicaciones , Glomerulonefritis/diagnóstico , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Anticuerpos Anticitoplasma de NeutrófilosRESUMEN
BACKGROUND: Several factors play a role in the pathogenesis of pruritus in uremic patients. The pathophysiology is complex and many factors have been identified in these patients. The aim of this study was to investigate the presence, severity, and possible causes of pruritus in patients with peritoneal dialysis (PD) . METHODS: Eighty patients, who received continuous ambulatory peritoneal dialysis (CAPD) treatment, were included in this study. Biochemical measurements, parathormone, C-reactive protein (CRP), and vitamin B12 levels of all the patients were recorded. Furthermore, substance P (SP) levels were measured by ELISA methods. Patients were examined by a dermatologist and pruritus degrees were queried using the visual analog score (VAS) with skin dryness. RESULTS: In generalized linear model analysis, total urea clearance and SP independently predicted VAS scores. SP was significantly predictive in ROC analysis in identifying the VAS score in patients with peritoneal dialysis. The sensitivity and specificity of SP were 80% and 67% (cut-off > 364), respectively, with an area under the ROC curve of 0.757 (95% CI 0.650-0.865, p < 0.001). SP also was significantly predictive in ROC analysis in identifying xerosis in PD patients. CONCLUSION: Pruritus was proportional to the amount of substance P and total urea clearance was another reason affecting pruritus in peritoneal dialysis patients.
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Diálisis Peritoneal Ambulatoria Continua/efectos adversos , Prurito/etiología , Adulto , Anciano , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Índice de Severidad de la EnfermedadRESUMEN
INTRODUCTION: Arteriovenous fistula (AVF) stenosis is one of the most important clinical problems in hemodialysis patients. The histopathological findings of neointimal hyperplasia and impaired angiogenesis have been well established in stenotic AVFs. Soluble vascular endothelial growth factor receptor-1 (sVEGFR-1) has been implicated in pathological angiogenesis. Thus, we aimed to investigate the association between sVEGFR-1 and AVF stenosis in hemodialysis patients. METHODS: This prospective cohort study included 70 patients with end-stage renal disease. Forty-five patients were included in the final analysis, and the median follow-up period was 36 months. Venous stenosis was detected by physical examination and documented by fistulography. Blood samples were analyzed a day before the fistula operation, and serum levels of sVEGFR-1 were measured. FINDINGS: The median sVEGFR-1 level was higher in the stenosis group than in the nonstenosis group (17 pg/mL [89.5%] vs. 5 pg/mL [19.2%], respectively; P < 0.001]. According to body mass index (BMI) categories, obese patients (BMI > 30 kg/m2 ) had the shortest stenosis-free survival (20 months [9.35-30.65]). Multivariate Cox analysis showed that sVEGFR-1, serum creatinine, and parathyroid hormone levels were associated with AVF stenosis risk. Kaplan-Meier survival curves showed that patients with less than the median value of sVEGFR-1 (<6093.07 pg/mL) had longer cumulative stenosis-free survival than patients with sVEGFR-1 levels above the median value (P < 0.001). DISCUSSION: Increased levels of sVEGFR-1 and obesity were found to be associated with AVF stenosis in hemodialysis patients.
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Fístula Arteriovenosa , Derivación Arteriovenosa Quirúrgica , Fístula Arteriovenosa/etiología , Derivación Arteriovenosa Quirúrgica/efectos adversos , Constricción Patológica , Humanos , Estudios Prospectivos , Diálisis Renal/efectos adversos , Factor A de Crecimiento Endotelial Vascular , Receptor 1 de Factores de Crecimiento Endotelial VascularRESUMEN
OBJECTIVE: This study aimed to investigate the relationship between fluid and sodium excretion and blood pressure, echocardiographic parameters, and arterial stiffness in peritoneal dialysis (PD) patients and to evaluate the effect of sodium excretion on patients' survival. METHOD: This study was conducted as a single-centered, prospective study in the Department of Nephrology in Erciyes University. The patients included in the study were followed up for 3 years. Seventy PD patients were included in the study. We recorded demographic characteristics, biochemical parameters, urine and peritoneal ultrafiltration volumes, peritoneal equalization tests, ambulatory blood pressure measurements, and echocardiographic measurements. We measured the amount of total sodium excretion of the patients and arterial stiffness using pulse wave velocity (PWV). Patients were divided into two groups based on the amount of total sodium excretion: low group and high group. We compared these groups in terms of cardiac and biochemical parameters. RESULTS: When demographic data and biochemical values were compared, there was no significant difference between the two groups. NT-proBNP level, systolic blood pressure, and mean arterial pressure were significantly higher in the low group (p: 0.02, p: 0.031, p: 0.05, respectively). Net ultrafiltration was significantly higher in the high group (p: 0.03), was also found to be high in patients with high sodium excretion (p: 0.001). Negative correlations were found between sodium excretion and net ultrafiltration, NT-Pro BNP, and PWV. At the end of the 3-year follow-up, the survival rate was shorter and the mortality rate was higher in the low group (p: 0.042). DISCUSSION AND CONCLUSION: Fluid status in PD patients can affect arterial stiffness both directly and through hypertension. Correction of hypervolemia has the potential to not only prevent hypertension and left ventricular hypertrophy, but also to improve arterial stiffness, a well-known cardiovascular risk factor. The mortality rate was higher in PD patients with low total sodium excretion. Therefore, these patients should be followed more closely to ensure volume control.
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Presión Arterial , Endotelio Vascular/fisiopatología , Fallo Renal Crónico/fisiopatología , Fallo Renal Crónico/terapia , Diálisis Peritoneal , Sodio/metabolismo , Rigidez Vascular , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Soluciones para Hemodiálisis , Humanos , Fallo Renal Crónico/metabolismo , Fallo Renal Crónico/mortalidad , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Tasa de Supervivencia , Adulto JovenRESUMEN
Autosomal-dominant polycystic kidney disease (ADPKD) is associated with oxidative stress and hypertension development before renal function decline and cardiovascular disease development. Oxidative stress-responsive kinase-1 (OSR-1) participates in the signaling regulating Na+ transport during oxidative stress and also plays a role in the regulation of cell volume and blood pressure. Therefore, we aimed to investigate the potential role of OSR-1 in ADPKD patients. Eighty ADPKD patients, 80 healthy controls, and 80 non-ADPKD patients with hypertension were enrolled in this cross-sectional study. Twenty-four-hour ambulatory blood pressure monitoring was conducted in all participants. Blood samples were taken after 12-h fasting for the measurement of biochemical parameters and OSR-1 gene expression. Vascular dysfunction was assessed using ischemia-induced forearm flow-mediated vasodilation (FMD). Briefly, of the 80 ADPKD patients, 41(51%) were male, and 53(66%) of them were hypertensive. The mean age of the 80 controls was 35.3 ± 12.6 years, and 37(46%) of them were male. The mean age of the 80 non-ADPKD patients with hypertension was 44.6 ± 11.9 years, and 38(47.5) of them were male. There were significant differences in serum OSR-1 gene expression between the ADPKD patients and the control subjects. Serum OSR-1 gene expression was also significantly increased in hypertensive ADPKD patients in comparison with both normotensive ADPKD counterparts and non-ADPKD hypertensive subjects. Serum OSR-1 gene expression was increased in patients with ADPKD than healthy subjects. Low estimated glomerular filtration rate (eGFR), OSR-1 gene expression, total kidney volume (TKV), and high-density lipoprotein (HDL) were also independently associated with hypertension in ADPKD patients.
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Endotelio Vascular/patología , Hipertensión/fisiopatología , Riñón Poliquístico Autosómico Dominante/fisiopatología , Proteínas Serina-Treonina Quinasas/genética , Adulto , Monitoreo Ambulatorio de la Presión Arterial , Estudios de Casos y Controles , Estudios Transversales , Femenino , Tasa de Filtración Glomerular , Humanos , Hipertensión/genética , Masculino , Persona de Mediana Edad , Estrés Oxidativo/fisiología , Riñón Poliquístico Autosómico Dominante/genéticaRESUMEN
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition that consisted of disorders that share particular clinical, serologic and pathologic properties. The common presentation of disease includes tumor-like swelling of involved organs and the histopathological findings are a lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells, and a variable degree of fibrosis that has a characteristic "storiform" pattern in biopsy specimens of tumor-like masses. Major presentations of this disease, which often affects more than one organ, include autoimmune pancreatitis, salivary gland disease (sialadenitis), orbital disease and retroperitoneal fibrosis. The steroid treatment is essential for the treatment of the disease however, other immunosuppressive drugs including cyclophosphamide or rituximab could be an option in resistant cases. CASE SUMMARY: Herein, we reported a 34-year-old woman whom previously had diagnosed with asthma, rheumatoid arthritis and Sjögren's syndrome (SS) referred our nephrology department due to acute kidney failure development at the last rheumatology visit. After kidney biopsy she has been diagnosed with IgG4-RD and tubuluointerstitial nephritis. She had been accepted resistant to steroid, mycophenolate mofetil, methotrexate and azathioprine therapies due to receiving in last two years. She refused to receive cyclophosphamide due to potential gonadotoxicity of the drug. Thus, rituximab therapy was considered. She received 1000 mg infusion, 15 d apart and 6 mo later it has been administered same protocol. After one year from the last rituximab dose serum creatinine decreased from 4.4 mg/dL to 1.6 mg/dL, erythrocyte sedimentation rate decreased from 109 mm/h to 13 mm/h [reference range (RR) 0-20], and C-reactive protein decreased from 55.6 mg/L to 5 mg/L (RR 0-6). All pathologic lymph nodes and masses were also disappeared. CONCLUSION: Patients with IgG4-RD usually misdiagnosed with rheumatologic diseases including systemic lupus erythematous or SS and also they were screened for the presence of malignancy. Rituximab could be an important treatment option in cases with steroid resistant tubulointerstitial nephritis in IgG4-RD.
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BACKGROUND AND OBJECTIVES: Cyst pressure induces renin-angiotensin-aldosterone system activation and kidney hypoxia in autosomal dominant polycystic kidney disease (ADPKD). Lipopolysaccharide-induced Toll-like receptor activation causes metabolic disturbances that are triggered by increased succinate levels and hypoxia inducible factors, which results in inflammation via IL-1ß activation. Since we aimed to investigate the role of both inflammation and hypoxia in the clinical course of ADPKD, via succinate levels from sera samples, HIF-1α gene expression from whole blood and urine samples and IL-1ßgene expression from whole blood were measured. METHODS: One hundred ADPKD patients and 100 matched healthy controls were enrolled to this cross-sectional study. Twenty-four-hour ambulatory blood pressure monitoring was conducted in all participants. Blood, serum, and urine samples were taken after 12-h fasting for the measurement of biochemical parameters and succinate levels. Whole blood and urine samples were used for HIF-1α and IL-1ß geneexpression by using quantitative real-time PCR. RESULTS: There were significant differences in whole blood HIF-1α, IL-1ß geneexpression, and serumsuccinate levels between the ADPKD patients and the control subjects. Whole blood HIF-1αgene expression, IL-1ß geneexpression, and serumsuccinate levels were also significantly different in ADPKD patients with hypertension in comparison with normotensive ones (p < 0.05). Serum succinate levels and blood IL-1ß geneexpression were increased in ADPKD patients with high levels of HIF-1α geneexpression (p = 0.018 and p = 0.029, respectively). CONCLUSIONS: Increased age,low eGFR, and HIF-1α and IL-1ß geneexpressions were also independently associated with hypertension in ADPKD patients. Inflammation and hypoxia are both relevant factors that might be associated with hypertension in ADPKD.
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Hipertensión/complicaciones , Subunidad alfa del Factor 1 Inducible por Hipoxia/metabolismo , Interleucina-1beta/metabolismo , Riñón Poliquístico Autosómico Dominante/genética , Adulto , Hipoxia de la Célula/genética , Estudios Transversales , Femenino , Expresión Génica/fisiología , Humanos , Hipertensión/sangre , Subunidad alfa del Factor 1 Inducible por Hipoxia/genética , Interleucina-1beta/genética , Masculino , Persona de Mediana Edad , Nefritis/genética , Riñón Poliquístico Autosómico Dominante/sangre , Riñón Poliquístico Autosómico Dominante/complicaciones , ARN Mensajero/metabolismo , Sistema Renina-Angiotensina/genéticaRESUMEN
BACKGROUND: Urinary tract infections (UTI) are one of the important clinical presentations in patients with autosomal dominant polycystic kidney disease (ADPKD). The association between UTI among genotypic and phonotypic properties of ADPKD patients is still obscure. Thus, we investigated the relationship between UTI and polycystin gene mutation with total kidney volume. METHODS: Forty patients with ADPKD patients with a history of more than two UTI and age-gender-matched 40 ADPKD patients without UTI history enrolled in the study. Ambulatory blood pressure monitoring was performed in all participants. Magnetic resonance imaging (MRI) was performed with a 1.5-T system, and total kidney volumes were calculated using mid-slice technique. To determine PKD1 and PKD2 genotype, we performed molecular and genetic tests involving the following steps: DNA isolation, next-generation sequencing (NGS) and data analysis. RESULTS: ADPKD patients with UTI had lower eGFR values than those without UTI [64.9 (32.2-100.8) vs 89.5 (59.0-110.0) (p = 0.041)]. In addition, patients with UTI had significantly increased height-adjusted total kidney volume than patients without UTI [950 (290-1350) vs 345 (243-780.0) (p = 0.005)]. Multiple logistic regression analysis showed that the PKD1-truncating mutation and hTKV independently predicted UTI. The sensitivity and specificity of hTKV were 65% and 77% (cutoff > 727 cm3) with an area of under the ROC curve of 0.70 (95% CI 0.56-0.85, p = 005). CONCLUSIONS: ADPKD patients with larger kidneys and PKD1 mutation are susceptible to increased risk of multiple UTI. Additionally, renal function decreased in ADPKD patients with multiple UTI history.
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Riñón Poliquístico Autosómico Dominante/diagnóstico por imagen , Riñón Poliquístico Autosómico Dominante/genética , Infecciones Urinarias/diagnóstico por imagen , Infecciones Urinarias/genética , Adulto , Monitoreo Ambulatorio de la Presión Arterial , Femenino , Estudios de Asociación Genética , Genotipo , Tasa de Filtración Glomerular , Humanos , Riñón/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Mutación , Fenotipo , Canales Catiónicos TRPP/genéticaRESUMEN
Secondary and tertiary hyperparathyroidism is an important problem of chronic kidney disease. Brown tumor is a benign, unusual, reactive lesion as a result of disturbed bone remodeling, from long-standing increase in parathyroid hormone level. Brown tumors may cause morbidity due to pressure symptoms on neural structures and spontaneous bone fractures. Herein, we presented a peritoneal dialysis patient with tertiary hyperparathyroidism under calcand calcitriol treatment for 4 years due to refusing of the parathyroidectomy operation. She admitted to hospital for sudden onset back pain with difficulty in gait and walking, and imaging studies showed an expansile mass lesion in the thoracic spine. She was operated for mass and diagnosed with brown tumor. After operation, she lost the ability of walking than become paraplegic and she underwent rehabilitation program. Preventive measures including calcitriol and cinacalcet may cause a modest decrease in parathyroid hormone levels but it should be remembered for the development of bone complications such as brown tumor formation in patients with moderate elevated PTH levels, especially those with tertiary hyperparathyroidism. Parathyroidectomy should be performed without delay in these cases.
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Hiperparatiroidismo/complicaciones , Osteítis Fibrosa Quística/complicaciones , Osteoclastos/patología , Paraplejía/etiología , Diálisis Peritoneal/efectos adversos , Adulto , Calcitriol/uso terapéutico , Agonistas de los Canales de Calcio/uso terapéutico , Femenino , Humanos , Hiperparatiroidismo/tratamiento farmacológico , Osteítis Fibrosa Quística/diagnóstico por imagen , Osteítis Fibrosa Quística/patología , Osteítis Fibrosa Quística/cirugía , Paraplejía/rehabilitación , Paratiroidectomía/normas , Insuficiencia Renal Crónica/terapiaRESUMEN
BACKGROUND: Overweight and obesity were recently associated with a poor prognosis in patients with autosomal dominant polycystic kidney disease (ADPKD). Whether the metabolic consequences of obesity as defined by the metabolic syndrome (MS) are also linked with disease progression remains untested. METHODS: Eligible ADPKD patients with different stages of CKD (n = 105) and 105 non-diabetic controls matched for CKD stage were enrolled in the study. Groups were evaluated at baseline for presence of MS, blood markers of metabolism, homeostasis model assessment of insulin resistance (HOMA-IR) score, and biochemical markers of inflammation (hs-CRP, IL-1ß, IL-6, TNF-α and PON-1). MS was defined according to the National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III). Patients were followed for 12 months and progression defined as a decrease in baseline eGFR > 10%. RESULTS: MS and hypertension were more prevalent amongst ADPKD patients than in the control group. Meanwhile, markers of inflammation such as hs-CRP (3.63 [3.45-5.17] vs. 4.2 [3.45-8.99] mg/dL; p = 0.014), IL-6 (21.65 [14.1-27.49] vs. 24.9 [16.23-39.4] pg/mL; p = 0.004) and IL-1ß (21.33 [15.8-26.4] vs. 26.78 [18.22-35] pg/mL; p < 0.001) levels were all more elevated in ADPKD patients than in non-diabetic CKD subjects. In multivariate analysis having a truncating PKD1 mutation predicted (OR 1.25 [1.09-1.43]; p = 0.002) fulfilling the MS criteria. Finally, ADPKD patients fulfilling MS criteria had a significantly more rapid progression during 12 months of follow-up than did those that did not (OR 3.28 [1.09-9.87]; p = 0.035). CONCLUSIONS: Our data supports the notion that dysmetabolisms part of the ADPKD phenotype and associated with a poor outcome, especially in patients with a truncating PKD1 mutation.
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Metabolismo Energético , Mediadores de Inflamación/sangre , Síndrome Metabólico/epidemiología , Mutación , Riñón Poliquístico Autosómico Dominante/epidemiología , Insuficiencia Renal Crónica/epidemiología , Canales Catiónicos TRPP/genética , Adulto , Biomarcadores/sangre , Estudios de Casos y Controles , Progresión de la Enfermedad , Femenino , Predisposición Genética a la Enfermedad , Humanos , Hipertensión/diagnóstico , Hipertensión/epidemiología , Masculino , Síndrome Metabólico/sangre , Síndrome Metabólico/diagnóstico , Síndrome Metabólico/genética , Persona de Mediana Edad , Fenotipo , Riñón Poliquístico Autosómico Dominante/diagnóstico , Riñón Poliquístico Autosómico Dominante/genética , Prevalencia , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/genética , Factores de Riesgo , Factores de Tiempo , TurquíaRESUMEN
BACKGROUND: The prominent features of autosomal dominant polycystic kidney disease (ADPKD) are early development of hypertension, chronic kidney disease and cardiovascular problems. Thus, we aimed to investigate the role of endothelin, a vascular biomarker, in the clinical course of ADPKD, including renal and cardiovascular survival. METHODS: In 138 patients with ADPKD and 28 healthy controls, we measured serum endothelin-1 (ET-1) levels by enzyme-linked immunosorbent assay (ELISA). Endothelium-dependent vasodilatation (flow-mediated dilatation, FMD) and endothelium-independent vasodilatation (nitroglycerin-mediated dilatation, NMD) of the brachial artery were assessed non-invasively with high-resolution ultrasound. Magnetic resonance imaging (MRI) was performed with a 1.5-T system, and total kidney volumes were calculated using mid-slice technique. To determine PKD1 and PKD2 genotype, we performed molecular and genetic tests involving the following steps: DNA isolation, next-generation sequencing (NGS) and data analysis. RESULTS: Endothelin levels and height-adjusted total kidney volumes (hTKV) significantly increased while the estimated glomerular filtration rate (eGFR) decreased across CKD stages 1-4. Hypertension was more frequent in ADPKD patients with high serum endothelin. At multivariate Cox analysis, endothelin level, PKD1 truncating mutation, hTKV, high-sensitive C reactive protein (hs-CRP) level and the presence of diabetes mellitus were associated with the risk of overall survival. Moreover, endothelin level, PKD1 truncating mutation, hTKV, age and presence of hypertension were associated with the risk of renal survival. Additionally, body mass index (BMI), FMD, PKD1 truncating mutation, endothelin and triglyceride levels were independently associated with hypertension. CONCLUSIONS: Increased serum endothelin levels independently predict hypertension in ADPKD. Serum endothelin levels are also associated with both renal and overall survival in patients with ADPKD.
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Endotelina-1/sangre , Hipertensión/etiología , Riñón Poliquístico Autosómico Dominante/sangre , Insuficiencia Renal Crónica/etiología , Adulto , Anciano , Biomarcadores/sangre , Estudios de Casos y Controles , Progresión de la Enfermedad , Femenino , Tasa de Filtración Glomerular , Hemodinámica , Humanos , Hipertensión/sangre , Hipertensión/diagnóstico , Hipertensión/fisiopatología , Riñón/fisiopatología , Masculino , Persona de Mediana Edad , Riñón Poliquístico Autosómico Dominante/complicaciones , Riñón Poliquístico Autosómico Dominante/diagnóstico , Riñón Poliquístico Autosómico Dominante/fisiopatología , Pronóstico , Insuficiencia Renal Crónica/sangre , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/fisiopatología , Factores de Riesgo , Factores de Tiempo , Regulación hacia ArribaRESUMEN
OBJECTIVES: This study aimed to show the effects of thymoquinone, which is known for its antioxidant, anti-inflammatory, and renal protective effects in contrast-induced nephropathy. MATERIALS AND METHODS: This is an experimental study in rats. 7 groups were included within the scope of our study: sham-vehicle (n=3), premedication-control (n=6), model (n=6), isolated thymoquinone (n=3+3), low-dose thymoquinone (n=6), and high-dose thymoquinone (n=7). In addition to 48 hr of water deprivation, we pre-medicated the rats with intra-peritoneal indomethacin and L-NAME administration. After premedication, 12.5 ml/kg dose of a high osmolar contrast agent-diatrizoat (Urografin %76) was administrated. Thymoquinone was administrated in two different doses of 1 mg/kg and 1.75 mg/kg for four days intraperitoneally. Renal functions, histopathological differences, oxidative stress parameters, and inflammatory indicators of rats were evaluated at the end of the study. RESULTS: Significant decreases were observed in levels of serum creatinine and serum BUN with low-dose thymoquinone (1 mg/kg) administration. In light microscopy, significantly less histopathological damage was observed in the low-dose thymoquinone group compared to the contrast agent group. While high-dose thymoquinone is accepted as ineffective biochemically, toxic evidence was identified histopathologically. There were no significant differences between M and TA groups for serum MDA and SOD levels, which were compared to evaluate oxidative stress (P:0.99, P:0.98; respectively). TNF-α, iNOS, and NF-кB gene expressions were not significantly different between all groups (P:0.748, P:0.531, P:0.910; respectively). CONCLUSION: This experimental study has demonstrated for the first time the protective effect of the TQ substance for CIN in 1 mg/kg dose, in the accompaniment of biochemical and histopathological data in rats.
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Skin infections caused by Paecilomyces species have been rarely described in patients with solid organ transplantation. Cutaneous manifestations are highly variable and include erythematous macules, nodules, pustules, and vesicular and necrotic lesions. The diagnosis of these infections is generally made by examination of a skin biopsy. Management of these fungal infections is difficult due to the immunocompromised state of the patients. Moreover, antifungal therapy and immunosuppressive drug interactions should be considered during treatment management. Herein, we reported a case of cellulitis caused by Paecilomyces variotii in a 56-year-old man who had undergone a kidney transplantation. Erythematous macular and nodular lesions on the left hand and left foot appeared first; within 2 months the skin lesions became ulcerated, hemorrhagic, and progressively painful and the patient was admitted to our hospital. The diagnosis was made by skin biopsy and tissue culture. The skin lesions resolved by the sixth week of the treatment with voriconazole.
Asunto(s)
Dermatomicosis/diagnóstico , Trasplante de Riñón/efectos adversos , Paecilomyces/aislamiento & purificación , Piel/patología , Receptores de Trasplantes , Antifúngicos/uso terapéutico , Biopsia , Dermatomicosis/tratamiento farmacológico , Dermatomicosis/etiología , Dermatomicosis/microbiología , Humanos , Huésped Inmunocomprometido , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Paecilomyces/efectos de los fármacos , Piel/microbiología , Resultado del Tratamiento , Voriconazol/uso terapéuticoRESUMEN
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disorder with unclear disease mechanism. Currently, overt hypertension and increased renal volume are the best predictors of renal function. In this study, we assessed the usefulness of selected circulating microRNAs (miRs) to predict disease progress in a cohort with ADPKD. METHODS: Eighty ADPKD patients (44.6 ± 12.7 years, 40% female, 65% hypertensive) and 50 healthy subjects (HS; 45.4 ± 12.7, 44% female) were enrolled in the study. Serum levels of 384 miRs were determined by Biomark Real Time PCR. Groups were compared using the limma method with multiple-testing correction as proposed by Smyth (corrected p < 0.01 considered significant). RESULTS: Comparing ADPKD to HS, we found significant differences in blood levels of 18 miRs (3 more and 15 less abundant). Of these, miR-3907, miR-92a-3p, miR-25-3p and miR-21-5p all rose while miR-1587 and miR-3911 decreased as renal function declined in both cross-sectional and longitudinal analysis. Using ROC analysis, an increased baseline miR-3907 in the circulation predicted a > 10% loss of GFR over the following 12 months (cut-off >2.2 AU, sensitivity 83%, specificity 78%, area 0.872 [95% CI: 0.790-0.953, p < 0.001]). Adjusting for age and starting CKD stage using multiple binary logistic regression analysis did not abrogate the predictive value. CONCLUSION: Increased copy numbers of miR-3907 in the circulation may predict ADPKD progression and suggest pathophysiological pathways worthy of further study.
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Hipertensión/sangre , MicroARNs/sangre , Riñón Poliquístico Autosómico Dominante/sangre , Adulto , Área Bajo la Curva , Estudios de Casos y Controles , Progresión de la Enfermedad , Femenino , Tasa de Filtración Glomerular , Humanos , Hipertensión/complicaciones , Masculino , Persona de Mediana Edad , Riñón Poliquístico Autosómico Dominante/complicaciones , Riñón Poliquístico Autosómico Dominante/genética , Valor Predictivo de las Pruebas , Curva ROC , Adulto JovenRESUMEN
Castleman's disease (CD), also known as angiofolicular lymph node hyperplasia, is a rare heterogenous group of lymphoproliferative disorders. Histologically, it can be classified as hyaline vascular type, plasma cell type, or mixed type. Clinically two different subtypes of the CD are present: Unicentric and multicentric. Unicentric CD is generally asymptomatic and associated with hyaline vascular type, and its diagnoses depend on the localized lymphadenopathy on examination or imaging studies. However, multicentric CD presents with generalized lymphadenopathy and systemic symptoms including malaise, fever, night sweats, weight loss, and it is associated with the plasma cell type and mix type. Herein, we report a patient with unicentric CD of the plasma cell type without systemic symptoms, who developed end stage renal failure caused by amyloidosis 6 years after onset of CD.
RESUMEN
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary cause of chronic kidney disease. The intriguing role of innate immune system and inflammation become a target for potential therapeutic approach to slow progression. When toll-like receptors (TLRs) signaling and their receptors activate, they start a cascade of intracellular signaling that induces the production of the inflammatory cytokines and chemokines. Thus, we aim to investigate the association of TLRs between progression of ADPKD. Ninety ADPKD patients and ninety matched controls were enrolled this prospective study and were followed during 3 years. TLR-2 and TLR-4 gene polymorphisms and expressions were measured. Hypertension was diagnosed with ambulatory blood pressure monitoring. Rapid progression was defined as sustained decline in estimated glomerular filtration rate (eGFR) of more than 5 mL/min per 1.73 m2 per year. TLR-4Asp299Gly polymorphisms were significantly different between patient and control group (P < 0.05). Also, TLR-2 and TLR-4 gene expressions were significantly different between the ADPKD patients and the control subjects (P < 0.05). The expression levels of both TLR-2 and TLR-4 were found to be higher in the rapid progression groups comparing the slow progression group (P < 0.05). TLR-2 gene expression, hypertension and uric acid were found to be independent risk factors in identifying rapid progression in ADPKD patients. TLR-2 and TLR-4 gene expressions are associated with rapid progression in ADPKD patients. TLRs may play a role in the progression of ADPKD.
Asunto(s)
Riñón Poliquístico Autosómico Dominante/inmunología , Riñón Poliquístico Autosómico Dominante/fisiopatología , Receptores Toll-Like/inmunología , Adulto , Progresión de la Enfermedad , Femenino , Humanos , Inflamación/complicaciones , Inflamación/inmunología , Inflamación/fisiopatología , Masculino , Persona de Mediana Edad , Riñón Poliquístico Autosómico Dominante/complicacionesRESUMEN
OBJECTIVE: Cisplatin is a potent antineoplastic agent used and its major limiting side effect is nephrotoxicity. The aims of the study are early detection of acute kidney injury (AKI) with biomarkers and investigation of the potential nephron-protective effects of theophylline. METHODS: Glomerular filtration rates (GFR), neutrophil gelatinase-associated lipocalin (NGAL), cystatin C were measured at 5th day of treatment in all of the patients. In addition, these parameters were measured repeatedly after the administration of cisplatin, at 2nd hour, 5th and 20th days. PATIENTS: Sixty patients who are planned to receive cisplatin for the first time were included in the study. Patients were divided into two groups as Group 1 (n = 30) (standard treatment arm) and Group II (n = 30) (theophylline arm). RESULTS: In both groups after the administration of cisplatin, GFR showed a significant decrease within time (p = 0.006). Urine NGAL levels were significantly high after 2 h of cisplatin administration (p < 0.001), no significant difference was observed between groups. However, when the time*group effects were considered together, higher NGAL levels were detected in the group not receiving theophylline (p = 0.025). After 5 days of cisplatin administration, urine protein levels were significantly higher in both groups (p < 0.001). CONCLUSION: Results showed that urine NGAL level is a superior biomarker compared to serum creatinine and serum cystatin C in the detection of early AKI. Theophylline was found not to bring a complete protection for the kidneys, but less nephrotoxicity was developed when compared to the group not receiving theophylline.