Background Self-management intervention aims to facilitate an individual’s ability to make lifestyle changes. The effectiveness of this intervention in non-dialysis patients with chronic kidney disease (CKD) is limited. In this study, we applied a systematic review and meta-analysis to investigate whether self-management intervention improves renoprotection for non-dialysis chronic kidney disease. Methods We conducted a comprehensive search for randomized controlled trials addressing our objective. We searched for studies up to May 12, 2018. Two reviewers independently evaluated study quality and extracted characteristics and outcomes among patients with CKD within the intervention phase for each trial. Meta-regression and subgroup analyses were conducted to explore heterogeneity. Results We identified 19 studies with a total of 2540 CKD patients and a mean follow-up of 13.44 months. Compared with usual care, self-management intervention did not show a significant difference for risk of all-cause mortality (5 studies, 1662 participants; RR 1.13; 95% CI 0.68 to 1.86; I 2 = 0%), risk of dialysis (5 studies, 1565 participants; RR 1.35; 95% CI 0.84 to 2.19; I 2 = 0%), or change in eGFR (8 studies, 1315 participants; SMD -0.01; 95% CI -0.23 to 0.21; I 2 = 64%). Moreover, self-management interventions were associated with a lower 24 h urinary protein excretion (4 studies, 905 participants; MD − 0.12 g/24 h; 95% CI -0.21 to − 0.02; I 2 = 3%), a lower blood pressure level (SBP: 7 studies, 1201 participants; MD − 5.68 mmHg; 95%CI − 9.68 to − 1.67; I 2 = 60%; DBP: 7 studies, 1201 participants; MD − 2.64 mmHg, 95% CI -3.78 to − 1.50; I 2 = 0%), a lower C-reactive Protein (CRP) level (3 studies, 123 participants; SMD -2.8; 95% CI -2.90 to − 2.70; I 2 = 0%) and a longer distance on the 6-min walk (3 studies, 277 participants; SMD 0.70; 95% CI 0.45 to 0.94; I 2 = 0%) when compared with the control group. Conclusions We observed that self-management intervention was beneficial for urine protein decline, blood pressure level, exercise capacity and CRP level, compared with the standard treatment, during a follow-up of 13.44 months in patients with CKD non-dialysis. However, it did not provide additional benefits for renal outcomes and all-cause mortality. Electronic supplementary material The online version of this article (10.1186/s12882-019-1309-y) contains supplementary material, which is available to authorized users.

Von Hippel-Lindau (VHL) disease is a rare autosomal dominant cancer syndrome. A phenomenon known as genetic anticipation has been documented in some hereditary cancer syndromes, where it was proved to relate to telomere shortening. Because studies of this phenomenon in VHL disease have been relatively scarce, we investigated anticipation in 18 Chinese VHL disease families. We recruited 34 parent-child patient pairs (57 patients) from 18 families with VHL disease. Onset age was defined as the age when any symptom or sign of VHL disease first appeared. Anticipation of onset age was analyzed by paired t test and the other two special tests (HV and RY2). Relative telomere length of peripheral leukocytes was measured in 29 patients and 325 healthy controls. Onset age was younger in child than in parent in 31 of the 34 parent-child pairs. Patients in the first generation had older onset age with longer age-adjusted relative telomere length, and those in the next generation had younger onset age with shorter age-adjusted relative telomere length (P < 0.001) in the 10 parent-child pairs from eight families with VHL disease. In addition, relative telomere length was shorter in the 29 patients with VHL disease than in the normal controls (P ¼ 0.003). The anticipation may relate to the shortening of telomere length in patients with VHL in successive generations. These findings indicate that anticipation is present in families with VHL disease and may be helpful for genetic counseling for families with VHL disease families and for further understanding the pathogenesis of VHL disease. Cancer Res; 74(14); 3802-9. Ó2014 AACR.

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Background Acute kidney injury (AKI) is an important complication of coronavirus disease 2019 (COVID-19), which could be caused by both systematic responses from multi-organ dysfunction and direct virus infection. While advanced evidence is needed regarding its clinical features and mechanisms. We aimed to describe two phenotypes of AKI as well as their risk factors and the association with mortality. Methods Consecutive hospitalized patients with COVID-19 in tertiary hospitals in Wuhan, China from 1 January 2020 to 23 March 2020 were included. Patients with AKI were classified as AKI-early and AKI-late according to the sequence of organ dysfunction (kidney as the first dysfunctional organ or not). Demographic and clinical features were compared between two AKI groups. Their risk factors and the associations with in-hospital mortality were analyzed. Results A total of 4020 cases with laboratory-confirmed COVID-19 were included and 285 (7.09%) of them were identified as AKI. Compared with patients with AKI-early, patients with AKI-late had significantly higher levels of systemic inflammatory markers. Both AKIs were associated with an increased risk of in-hospital mortality, with similar fully adjusted hazard ratios of 2.46 [95% confidence interval (CI) 1.35–4.49] for AKI-early and 3.09 (95% CI 2.17–4.40) for AKI-late. Only hypertension was independently associated with the risk of AKI-early. While age, history of chronic kidney disease and the levels of inflammatory biomarkers were associated with the risk of AKI-late. Conclusions AKI among patients with COVID-19 has two clinical phenotypes, which could be due to different mechanisms. Considering the increased risk for mortality for both phenotypes, monitoring for AKI should be emphasized during COVID-19.

Regulation of microRNAs (miRNA) has been extensively investigated in diseases; however, little is known about the roles of miRNAs in cleidocranial dysplasia (CCD). The aim of the present study was to investigate the potential involvement of miRNAs in CCD. In vitro site-directed mutagenesis was performed to construct three mutant Runx2 expression vectors, which were then transfected into LS8 cells and MC3T3-E1 cells, to determine the impact on amelogenesis and osteogenesis, respectively. miRCURY LNA miRNA microarray identify miR-185-5p as a miRNA target commonly induced by all three Runx2 mutants. Real-time quantitative PCR was applied to determine the expression of miR-185-5p and Dlx2 in samples. Dual-luciferase reporter assays were conducted to confirm Dlx2 as a legitimate target of miR-185-5p. The suppressive effect of miR-185-5p on amelogenesis and osteogenesis of miR-185-5p was evaluated by RT-PCR and western blot examination of Amelx, Enam, Klk4, and Mmp20 gene and protein expression, and by Alizarin Red stain. We found that mutant Runx2 suppressed amelogenesis and osteogenesis. miR-185-5p, induced by Runx2, suppressed amelogenesis and osteogenesis. Furthermore, we identified Dlx2 as direct target of miR-185-5p. Consistently, Dlx2 expression was inversely correlated with miR-185-5p levels. This study highlights the molecular etiology and significance of miR-185-5p in CCD, and suggests that targeting miR-185-5p may represent a new therapeutic strategy in prevention or intervention of CCD.

This largest VHL survival analysis indicates that onset age, family history, mutation type and first presenting symptom have an effect on the survival of patients with VHL disease, which is helpful to genetic counselling and clinical decision-making.

The prognostic significance of PD-L1 in renal cell carcinoma (RCC) had been investigated in previous studies; however, the results remain controversial. The primary aim of this meta-analysis was to investigate the prognostic and clinicopathological significance of the PD-L1 expression in patients with RCC. Relevant literature was identified form PubMed, Embase, Web of Science and Cochrane library, which compared the prognostic significance between PD-L1 expression and RCC. Hazard ratios (HRs) for survival outcomes and odds ratios (ORs) for clinical parameters associated with PD-L1 were extracted from eligible studies. Heterogeneity was assessed using the I value. The fixed-effects model was used if there was no evidence of heterogeneity; otherwise, the random-effects model was used. Publication bias was evaluated using Begg's funnel plots and Egger's regression test. A total of 1863 patients from ten eligible studies were analyzed. The results showed that PD-L1 expression is associated with poor overall survival in clear cell RCC (ccRCC) (HR = 2.76, 95%CI: 2.25-3.38, I = 14.4%, P < 0.001) and non-clear cell RCC (non-ccRCC) (HR = 2.77, 95%CI: 1.62-4.72, I = 28.8%, P < 0.001). In addition, PD-L1 expression was found to be significantly associated with primary tumor stage (OR = 1.76, 95%CI: 1.39-2.23; I = 56.3%), regional lymph node involvement (OR = 2.10, 95%CI: 1.48-2.98; I = 14.9%), distant metastases (OR = 2.69, 95%CI: 2.05-3.54; I = 0.0%), nuclear grade (OR = 1.72, 95%CI: 1.32-2.23; I = 79.4%) and histologic tumor necrosis (OR = 2.25, 95%CI: 1.59-3.18; I = 66.1%) in patients with RCC. The outcome stability was confirmed by sensitivity analysis. Both the Begg's funnel plot test (P = 0.276) and the Egger's (P = 0.388) verified that there was no publication bias within the included studies. This study suggests that PD-L1 expression is correlated with poor prognosis and advanced clinicopathological features in RCC patients.

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