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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.

The effects of vitamin D receptor activation on cardiovascular diseases, especially hypertension and cardiac dysfunction, are areas of active investigation. This article reviews the current state of knowledge about vitamin D receptor activation with respect to blood pressure, heart, and vascular health, as well as to chronic kidney disease and end-stage renal disease. Potential biological mechanisms, the role of vitamin D-binding protein, and data from observational and randomized controlled trials on this topic are summarized. [ABSTRACT FROM AUTHOR]

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]

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. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article on late referral of patients with chronic kidney disease.

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]