Clinical nephrology最新文献

To identify the correlation between the plasma long non-coding RNA maternally expressed gene 3 (lncRNA MEG-3) and inflammatory cytokines in patients with diabetic nephropathy (DN) and search for a potential index for the diagnosis of DN. Quantitative real-time PCR (qPCR) was used to assess lncRNA MEG-3 expression. The levels of plasma cytokines were detected via enzyme-linked immunosorbent assay (-ELISA). 20 patients with type 2 diabetes (T2DM) and DN (DM+DN+ group), 19 patients with T2DM (DM+DN- group), and 17 healthy subjects (DM-DN- group) were finally enrolled. The expression of lncRNA MEG-3 was significantly upregulated in the DM+DN+ group compared to the DM+DN- group (p < 0.05) and the DM-DN- group (p < 0.001). Pearson's correlation analysis showed a positive correlation of lncRNA MEG-3 levels with cystatin C (Cys-C) (r = 0.468, p < 0.05), albumin-creatinine ratio (ACR) (r = 0.532, p < 0.05), and creatinine (Cr) (r = 0.468, p < 0.05), and a negative correlation with estimated glomerular filtration rate (eGFR) (r = -0.674, p < 0.01). Furthermore, the expression level of plasma lncRNA MEG-3 had a significantly positive correlation with the level of interleukin 1β (IL-1β) (r = 0.524, p < 0.05) and interleukin 18 (IL-18) (r = 0.230, p < 0.05). Binary regression analysis showed that lncRNA MEG-3 was a risk factor for DN with odds ration (OR) value of 1.71 (p < 0.05). The area under receiver operation characteristic (ROC) curve (AUC) of DN identified by lncRNA MEG-3 was 0.724. LncRNA MEG-3 was highly expressed in DN patients and showed a positive correlation with IL-1β, IL-18, ACR, Cys-C, and Cr.

Calcific uremic arteriolopathy (CUA) represents a rare but severe disease with high morbimortality. The authors present the case of a 58-year-old male patient with chronic kidney disease due to obstructive uropathy, on hemodialysis (HD). He started HD due to uremic syndrome with a severe renal dysfunction, dysregulation of calcium and phosphate metabolism, and he presented with distal penile ischemia, which was treated with surgical debridement and hyperbaric oxygen therapy. Four months later, painful distal digital necrosis of both hands was observed. Extensive arterial calcification was observed on X-ray. A skin biopsy confirmed the presence of CUA. Sodium thiosulfate was administered for 3 months, HD was intensified, and hyperphosphatemia control was achieved, with progressive improvement of the lesions. This case illustrates an uncommon presentation of CUA in a patient on HD for a few months, non-diabetic and not anticoagulated, but with a severe dysregulation of calcium and phosphate metabolism.

Purpose: Atypical hemolytic uremic syndrome (aHUS) is a genetic-based thrombotic microangiopathy (TMA) that is mediated by the activation of the alternative complement pathway. Heterozygous deletion in CFHR3-CFHR1 occurs in 30% of the general population and has not been classically linked to aHUS. Post-transplant aHUS has been associated with a high rate of graft loss. Herein, we report our case series of patients who developed aHUS after solid-organ transplantation.

Materials and methods: Five consecutive cases of post-transplant aHUS were identified at our center. Genetic testing was performed in all but one.

Results: One patient had a presumed TMA diagnosis before transplant. One heart and 4 kidney (KTx) transplant recipients were diagnosed with aHUS based on the clinical picture of TMA, acute kidney injury, and normal ADAMTS13 activity. Genetic mutation testing revealed heterozygous deletion in CFHR3-CFHR1 in 2 patients and a heterozygous complement factor I (CFI) variant of uncertain clinical significance (VUCS) (Ile416Leu) in a third. Four patients were on tacrolimus, 1 had anti-HLA-A68 donor-specific antibody (DSA), and another had borderline acute cellular rejection at the time of aHUS diagnosis. Four responded to eculizumab, and 1 out of 2 patients came off renal replacement therapy. One KTx recipient died from severe bowel necrosis in the setting of early post-transplant aHUS.

Conclusion: Calcineurin inhibitors, rejection, DSA, infections, surgery, and ischemia-reperfusion injury are common triggers that could unmask aHUS in solid-organ transplant recipients. Heterozygous deletion in CFHR3-CFHR1 and CFI VUCS may be important susceptibility factors acting as the first hit for alternative complement pathway dysregulation.

Aim: In this study, we aimed to investigate the long-term benefits of switching from oral to intravenous calcimimetics in patients on hemodialysis.

Materials and methods: Patients on maintenance hemodialysis at our institution who switched from oral to intravenous calcimimetics between March 1, 2017 and October 31, 2018 were enrolled. We compared tablet number; chronic kidney disease-mineral and bone disorder (CKD-MBD)-related drug cost; and serum corrected calcium, serum phosphorous, and serum intact parathyroid hormone levels before and 1, 2, and 3 years after switching from oral to intravenous calcimimetics.

Results: There were 15 patients (11 males and 4 females; mean age 60.9 ± 9.2 years). The tablet numbers and CKD-MBD-related drug cost before and 3 years after switching to calcimimetics were 12.1 ± 8.1 tablets/day vs. 8.4 ± 5.0 tablets/day (p = 0.0371) and 9,654.5 ± 6,206.8 yen (87.8 ± 56.4 U.S. dollars)/week vs. 7,231.7 ± 3,490.9 yen (65.7 ± 31.7 U.S. dollars)/week (p = 0.0406), respectively.

Conclusion: Switching from oral to intravenous calcimimetics decreased intact parathyroid hormone levels and reduced the tablet numbers and CKD-MBD-related drug cost for a long period without significant adverse effects.

Objective: The aim of the study was to evaluate the clinical course and risk factors of venous thromboembolic complications (VTEC) in children with a first episode of steroid-sensitive nephrotic syndrome (SSNS).

Materials and methods: We retrospectively analyzed the medical records of children hospitalized due to SSNS in one pediatric nephrology unit between 2012 and 2019. Demographic data, clinical symptoms at the onset of NS, and laboratory parameters were compared between patients with and without VTEC.

Results: Among 106 children (4.7 ± 3.06 years of age) with a first episode of SSNS, 5 VTEC were diagnosed during 2 - 60 days after onset of NS, on the basis of clinical symptoms and/or results of imaging studies. These were thromboses of femoral vein, central part of the kidney, dorsal veins of the hand, venous sinuses of the brain, and superficial vein in the popliteal fossa region. We found significant higher serum fibrinogen level (p = 0.022) and D-dimers (p = 0.0001) in children with VTEC vs. those without VTEC, but AUC analysis showed that only D-dimers significantly differentiate thrombosis. The clinical risk factors of VTEC were vascular cannulation (100%), infections (80%), and diuretics (80%). In children with VTEC, low molecular weight heparin was used. The outcome was a full recovery in all patients.

Conclusion: VTEC occurs in 4.72% of children with a first episode of SSNS. The course of VTEC in children with SSNS may be asymptomatic. The clinical risk factors of VTEC in children with SSNS are vascular cannulation, infections, and diuretics. High D-dimer levels are a sensitive indicator of thrombosis.

Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) comprising microangiopathic hemolytic anemia, consumptive thrombocytopenia, and end-organ damage. Risk of end-stage renal disease is increased as HUS usually manifests in native and transplanted kidneys. In transplants, while de novo disease can be seen, recurrent disease is more common. The etiology is variable, being either primary or secondary. aHUS often constitutes a diagnostic and therapeutic challenge, which may lead to a considerable delay in the diagnosis and treatment. During the last decades, great progress has been made in understanding the mechanisms and therapeutic options of this devastating condition. We present a case of a 50-year-old female who received her first kidney transplant from her mother at the age of 9 years. She experienced recurrent losses of transplants, and only after the loss of her fourth transplant did the diagnosis of aHUS become evident.

Purpose: To evaluate the effects of dexmedetomidine (DEX) on outcomes of critically ill patients with acute kidney injury (AKI).

Materials and methods: Data were extracted from the Medical Information Mart for Intensive Care III database (MIMIC III). Propensity score matching (PSM) analysis (1 : 3), Cox proportional hazards model, linear regression and logistic regression model were used to assess the effect of DEX on clinical outcomes.

Results: After PSM, 324 pairs of patients were matched between the patients with DEX administration and those without. DEX administration was associated with decreased in-hospital mortality (hazard ratio (HR) 0.287; 95% CI 0.151 - 0.542; p < 0.001) and 90-day mortality (HR 0.344; 95% CI 0.221 - 0.534; p < 0.001), and it was also associated with reduced length of stay (LOS) in ICU (4.54 (3.13,7.72) vs. 5.24 (3.15,10.91), p < 0.001) and LOS in hospital (11.63 (8.02,16.79) vs 12.09 (7.83,20.44), p = 0.002). Subgroup analysis showed that the above associations existed only in the mild and moderate AKI subgroups, but not in the severe AKI subgroup. Nevertheless, DEX administration was not associated with recovery of renal function (HR 1.199; 95% CI 0.851 - 1.688; p = 0.300).

Conclusion: DEX administration improved outcomes in critically ill patients with mild and moderate AKI and could be a good choice of sedation.

Heparin-induced thrombocytopenia (HIT) is a severe, potentially life-threatening adverse drug reaction. It is an antibody-mediated process involving platelet activation. Heparin and low-molecular-weight heparin (LMWH) are routinely used in uremic patients undergoing hemodialysis. Here, we report a case of HIT that occurred in a hemodialysis patient after she switched from heparin to the LMWH nadroparin for anticoagulation during hemodialysis. The clinical features, incidence, mechanism, and treatment of HIT are discussed.