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Background: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Furthermore, SCN is an understudied condition with relatively few symptoms and therefore requires close surveillance. In this review, we sought to explore the epidemiology, natural history, and treatment options for SCN with an emphasis on the pediatric population., Summary: SCN invariably begins in childhood with evidence of structural changes detected as early as infancy. These indolent changes can progress undetected to advanced chronic kidney disease by late adolescence or early adulthood. The risk factors for progression are not well defined, but significant albuminuria (which is also the most common presentation in childhood) is a key factor in progression. One of the main challenges in understanding SCN in children is the poor correlation between estimated and measured glomerular filtration rates. Another challenge is the lack of large-scale longitudinal studies that track the clinical outcomes of pediatric patients over time. Several studies aim to identify early biomarkers of SCN in children, as albuminuria presents only following significant chronic damage. The utility of angiotensin converting enzyme inhibitors and hydroxyurea in treating albuminuria is addressed here as well as novel treatments that may be of benefit., (© 2018 S. Karger AG, Basel.)

Aim: Uncontrolled hypertension is an independent risk factor for cardiovascular disease and is the leading cause of mortality in haemodialysis patients. The aim of this study was to examine the effectiveness of self-management support (SMS) for blood pressure (BP) control and health behaviours., Methods: We conducted a cluster randomized controlled trial (RCT) in which 90 adult haemodialysis patients were assigned to either an SMS or common intervention (CI) group. The SMS group received an intervention consisting of self-management education and motivational interviewing. The CI group received standard care and routine health education. The primary outcome was the BP monitored before each haemodialysis. Secondary outcomes included salt intake (measured using a balance formula), home BP monitoring (HBPM) (assessed using two self-administered questions), and medication adherence (measured using the Medication-taking Behavior Scale). Data were collected at baseline and at 1, 3 and 6 months post-intervention., Results: The SMS group showed continuous reductions in systolic BP from baseline: -9.2, -8.7, and -8.4 mmHg at 1, 3 and 6 months after the intervention, respectively (P < 0.01). Compared with the CI group, the SMS group had a greater decrease in systolic BP at 1 month: -5.9 mmHg (P = 0.0388), but no significant difference was found at 3 or 6 months (P > 0.05). SMS patients showed an improvement in health behaviours relative to baseline (less salt intake, more consistent HBPM, and greater medication adherence) (P < 0.05)., Conclusions: Self-management support obtained short-term success in improving salt restriction, regular performance of HBPM and medication adherence, which led to better BP control., (© 2017 Asian Pacific Society of Nephrology.)

Background: Henoch-Schönlein purpura (HSP) is a small vessel IgA-predominant vasculitis., Objective: To describe adult patients with HSP and determine if the distribution of skin lesions (ie, purpura above the waist or purpura below the waist only), is a predictor of significant renal involvement at the time of the skin biopsy and the months following., Methods: A retrospective study on renal function from 72 adult patients with skin-biopsy proven HSP. Longitudinal renal data were analyzed after HSP diagnosis by using baseline renal function for comparison., Results: Statistical analysis adjusted for sex, age, and baseline creatinine revealed a trend between HSP lesions only on the upper and lower extremities and long-term renal involvement (4.767, P = .067). Moreover, in another analysis adjusted for age and baseline creatinine, lesions located only on the upper and lower extremities significantly increased the odds of having long-term significant renal involvement (6.55, P = .049) in men., Limitations: This retrospective study used patient information that was subject to selection bias., Conclusion: In patients with HSP, skin lesion distribution on the extremities might be predictive of significant long-term renal involvement and might be critical for risk stratification and development of personalized diagnostics and therapeutics., (Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background and Objectives: Vitamin D (25-hydroxyvitamin D; 25[OH]D) deficiency is common in patients initiating long-term hemodialysis, but the safety and efficacy of nutritional vitamin D supplementation in this population remain uncertain., Design, Setting, Participants, & Measurements: This randomized, placebo-controlled, parallel-group multicenter trial compared two doses of ergocalciferol with placebo between October 2009 and March 2013. Hemodialysis patients (n=105) with 25(OH)D levels ≤32 ng/ml from 32 centers in the Northeast United States were randomly assigned to oral ergocalciferol, 50,000 IU weekly (n=36) or monthly (n=33), or placebo (n=36) for a 12-week treatment period. The primary endpoint was the achievement of vitamin D sufficiency (25[OH]D >32 ng/ml) at the end of the 12-week treatment period. Survival was assessed through 1 year., Results: Baseline characteristics were similar across all arms, with overall mean±SD 25(OH)D levels of 21.9±6.9 ng/ml. At 12 weeks, vitamin D sufficiency (25[OH]D >32 ng/ml) was achieved in 91% (weekly), 66% (monthly), and 35% (placebo) (P<0.001). Mean 25(OH)D was significantly higher in both the weekly (49.8±2.3 ng/ml; P<0.001) and monthly (38.3±2.4 ng/ml; P=0.001) arms compared with placebo (27.4±2.3 ng/ml). Calcium, phosphate, parathyroid hormone levels, and active vitamin D treatment did not differ between groups. All-cause and cause-specific hospitalizations and adverse events were similar between groups during the intervention period. Lower all-cause mortality among ergocalciferol-treated participants was not statistically significant (hazard ratio, 0.28; 95% confidence interval, 0.07 to 1.19)., Conclusions: Oral ergocalciferol can increase 25(OH)D levels in incident hemodialysis patients without significant alterations in blood calcium, phosphate, or parathyroid hormone during a 12-week period., (Copyright © 2015 by the American Society of Nephrology.)

Background: Calciphylaxis is a rare, painful, and debilitating disorder of vascular calcification and skin necrosis that typically affects patients with advanced kidney disease. During our routine pathology practice, we noted several missed diagnoses on calciphylaxis consultation cases originating from outside institutions and sought to explore factors associated with false‐negative pathologic diagnosis of calciphylaxis. Methods: The pathology database of a large tertiary academic medical center was retrospectively searched for "calciphylaxis" in inside reports on outside surgical consultation cases between 2007 and 2017. Inside and outside pathology reports were compared and medical records were searched for calciphylaxis clinical diagnosis and risk factors. Results: Twenty‐four calciphylaxis patients were identified, with median age of 63.5 years. Seven of 24 (29%) of specimens were inadequate (e.g., lack of subcutaneous adipose tissue for evaluation). Eight of 17 (47%) of adequate specimens had a first false‐negative pathologic diagnosis of calciphylaxis. Histochemical staining for calcium significantly correlated with true‐positive diagnosis (93% vs 55%, P = 0.004). Dermatopathology fellowship training significantly correlated with true‐positive diagnosis (82% vs 38%, P = 0.047). Conclusions: Adequate sampling, dermatopathology training, and use of histochemical stains to identify calcium associate with decreased false‐negative rate for calciphylaxis diagnosis. These findings need further evaluation in larger prospective studies. [ABSTRACT FROM AUTHOR]

The article offers information on calciphylaxis which is a complex disorder of microvascular calcification that is typically manifested as painful cutaneous lesions and results in poor outcomes. It mentions that Calciphylaxis occurs in patients with earlier stages of chronic kidney disease, acute kidney injury, and kidney transplantation. It states that extracutaneous vascular calcifications leads to skeletal myopathy, intestinal bleeding and visual impairment.

Calciphylaxis, a disorder of dermal arteriolar calcification with a distinct predilection for patients with advanced kidney disease, remains an enigmatic condition that challenges clinicians. Observations regarding positive associations between levels of circulating parathyroid hormone (PTH) and the risk of calciphylaxis, combined with experimental data suggesting a pathogenic role of secondary hyperparathyroidism in vascular calcification, have led to the assumption that reducing PTH levels will improve the outcomes in patients with calciphylaxis. This editorial communicates the contemporary data regarding surgical parathyroidectomy as a treatment option for these patients. We raise concerns regarding the quality of available data and discuss the direction of future comparative effectiveness research in this field. [ABSTRACT FROM AUTHOR]