The progression of immunoglobulin A nephropathy (IgAN) is currently assessed using the Oxford MEST-C score, which uses five indicators (mesangial and endocapillary hypercellularity, segmental sclerosis, interstitial fibrosis/tubular atrophy, and the presence of crescents) but has not yet included any risk factors related to glomerular size. Therefore, we tested whether adding another indicator, maximal glomerular diameter (Max GD), would improve the prognostic ability of this scoring system. The data of 101 adult patients diagnosed with IgAN between March 2002 and September 2004 were reviewed. We used McFadden’s pseudo-R2 and the corrected Akaike information criterion to assess model fit and the concordance (C)-statistic to assess discriminatory ability. A 10 μm increase in Max GD was significantly associated with a composite outcome (≥50% decline in the estimated glomerular filtration rate or end-stage renal disease). The receiver operating characteristic analysis determined the cut-off for high vs. low Max GD at 245.9 μm, and adding high Max GD to the MEST-C score significantly improved the model’s discrimination of renal outcomes at 5 and ≥10 years. Thus, including the Max GD in the Oxford classification of IgAN might increase its robustness and provide a more comprehensive prognostic system for clinical settings.

Whether the visceral-to-subcutaneous fat ratio (V/S ratio) is associated with renal prognosis in patients with chronic kidney disease (CKD) remains unclear. Furthermore, little is known about the effect of sex and the absolute amount of visceral fat accumulation such as visceral fat area (VFA) ≥100 cm2 on the V/S ratio in relation to renal prognosis. In this study, 200 patients with CKD were evaluated for renal prognosis. Survival analyses and logistic regression analyses were conducted, generating time-series pseudo-R2 values. The mean and percent change of the pseudo-R2 values from the 6th year to the 10th year (6Y–10Y Mean and 6Y–10Y Change, respectively) were calculated for determining the cut-off points for the medium-term renal prognosis. Multivariate Cox regression analysis revealed that the V/S ratio was significantly associated with renal outcomes and that the VFA category (VFA ≥ 100 cm2) had significant interactions with the V/S ratio regarding renal prognosis. The hazard ratio (HR) of the V/S ratio was higher in the sub-cohort of VFA < 100 cm2 than in the sub-cohort of VFA ≥ 100 cm2 (HR: 6.42 vs. 1.00). Regarding sex differences, a strong association was noted between the V/S ratio and renal prognosis in women but not in men (HR: 2.40 vs. 1.10). On the other hand, 6Y–10Y Mean of the pseudo-R2 values indicated differences in the cut-off points of the V/S ratio between men and women (V/S ratio: 0.75 vs. 0.5). Our findings indicate that it may be clinically meaningful to consider the differences in sex and the amount of VFA ≥100 cm2 for the V/S ratio in relation to renal outcomes in patients with CKD. The 6Y–10Y Mean of the pseudo-R2 values contributed to determining the cut-off points of the V/S ratio according to the sex difference.

Results: Seven of 18 patients had pathogenic NPH-causing mutations in NPHP1, NPHP3, NPHP4, or CEP164. Compared with patients without pathogenic mutations, those with pathogenic mutations were significantly younger but did not significantly differ in the classic NPH pathologic findings, such as tubular cysts. On the other hand, the number of tubules with thick tubular basement membrane (TBM) duplication, which was defined as >10-mm thickness, was significantly higher in patients with genetically proven adult NPH than in those without pathogenic mutations. a-Smooth muscle actin (a-SMA)-positive myofibroblasts were detected inside thick TBM duplication. Conclusions:In adult patients with NPH, thick TBM duplication was the specific finding. Our analysis also suggested that older patients tended to have no pathogenic mutations, even when they were suspected to have NPH by renal biopsy. These findings could be the novel clinical clue for the diagnosis of NPH in adult patients.

Background: Acute decompensated heart failure (ADHF) is the most common cause of readmissions in the hospital. ADHF patients are associated with polypharmacy. It is a common problem among elderly patients due to frequently occurring multiple morbidities and is associated with the use of potentially inappropriate medications (PIMs). The aim of this study was to examine the association between PIMs and all-cause mortality in elderly ADHF patients. Methods: This retrospective study included ADHF patients who were admitted to the Showa University Fujigaoka Hospital between January 2015 and August 2016. We investigated the proportion of patients taking at least one PIM at admission and the characteristics of patients at admission. PIMs were defined based on the Screening Tool of Older People's potentially inappropriate Prescriptions (STOPP). Multiple Cox regression analysis was performed to examine the association between PIM use and all-cause mortality. Results: A total of 193 elderly patients (median age 81 years, interquartile range (IQR) 65-99 years) were included in the study. Allcause death occurred in 30 patients. The median number of medications at admission was 7 (IQR 0-18). The number of medications (greater than or equal to six) at admission was associated with mortality. Multivariate Cox regression analysis revealed that systolic blood pressure (SBP) < 100 mm Hg at admission, chronic obstructive pulmonary disease (COPD), and use of non-steroidal anti-inflammatory drugs (NSAIDs) at admission were independent predictors for allcause mortality. Conclusions: The medical staff should attempt to stop unnecessary medications that are prone to be inappropriate prescribing. In particular, prescription of NSAIDs should be carefully assessed and monitored.

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