Weber-Christian disease associated with the tip variant of focal segmental glomerulosclerosis: a case report.

Weber-Christian disease (W-CD) is associated with relapsing nodular panniculitis and a variety of systemic findings. Renal parenchymal involvement has been rarely reported. The authors describe a case of nephrotic syndrome in an African-American man with a W-CD flare. The patient had chills and low-grade fever with painful lower extremity skin lesions. A renal biopsy demonstrated the tip variant of focal segmental glomerulosclerosis (FSGS). The kidney biopsy also suggested parenchymal involvement by W-CD disease, with supportive ultrastructural findings. The synchronous W-CD flare and biopsy-proven FSGS and the rapid and sustained response of both to limited treatment suggest a causative association.

The immunoregulatory function of vitamin D: implications in chronic kidney disease.

Cardiovascular and infectious diseases remain the most common causes of death among patients with chronic kidney disease (CKD) and end-stage renal disease (ESRD). Basic science and epidemiological studies indicate that vitamin D has importance not only for cardiovascular health, but also for the immune response. Vitamin D signaling pathways regulate both innate and adaptive immunity, maintaining the associated inflammatory response within physiological limits. Levels of both the inactive as well as active form of vitamin D (25-hydroxyvitamin D and 1,25-dihydroxyvitamin D, respectively) are decreased in patients with CKD and ESRD. It is reasonable to hypothesize, therefore, that the immune dysfunction associated with vitamin D deficiency in patients with CKD and ESRD in part explains the misdirected inflammatory response and increased susceptibility to infection seen in this population. Indeed, observational studies show that vitamin D deficiency in patients with ESRD is associated with increased mortality, and treatment with vitamin D is associated with a decreased risk of infection, as well as reduced all-cause mortality. However, whether different vitamin D preparations have differential effects on physiological function and clinical outcomes is still unclear. A proper understanding of the immune regulatory function of vitamin D is important for the development of future therapeutic strategies.

Examining associations of circulating endotoxin with nutritional status, inflammation, and mortality in hemodialysis patients.

OBJECTIVE: Lipopolysaccharide or endotoxin constitutes most part of the outer portion of the cell wall in the gram-negative bacteria. Subclinical endotoxemia could contribute to increased inflammation and mortality in hemodialysis (HD) patients. Endotoxin level and clinical effect are determined by its soluble receptor sCD14 and high-density lipoprotein. We examine the hypothesis that endotoxin level correlates with mortality. METHODS: In this cohort study, endotoxin levels were measured in 306 long-term HD patients who were then followed up for a maximum of 42 months. Soluble CD14 and cytokines levels were also measured. RESULTS: The mean (±SD) endotoxin level was 2.31 ± 3.10 EU/mL (minimum: 0.26 EU/mL, maximum: 22.94 EU/mL, interquartile range: 1.33 EU/mL, median: 1.27 EU/mL). Endotoxin correlated with C-reactive protein (r = 0.11, P < .04). On multivariate logistic regression analysis, high body mass index and low high-density lipoprotein (HDL) cholesterol levels were associated with higher endotoxemia (endotoxin below or above of median). In multivariate Cox regression analysis adjusted for case-mix and nutritional/inflammatory confounders, endotoxin levels in the third quartile versus first quartile were associated with a trend toward increased hazard ratio for death (hazard ratio: 1.83, 95% confidence interval: 0.93 to 3.6, P = .08). CONCLUSIONS: In this HD cohort, we found associations between endotoxemia and C-reactive protein, body composition, and HDL. Moderately high endotoxin levels tended to correlate with increased mortality than the highest circulating endotoxin level. Additional studies are required to assess the effect of endotoxemia on mortality in dialysis population.

Clinical significance and preventive strategies for contrast-induced nephropathy.

PURPOSE OF REVIEW: Contrast-induced nephropathy continues to be a common cause of in-hospital acute kidney injury. Published studies on pathogenesis, clinical significance, diagnosis, and preventive measures have dramatically increased significantly in the past several years. This review will focus on new developments in contrast-induced nephropathy. RECENT FINDINGS: Studies on the clinical significance of contrast-induced nephropathy are reviewed along with initial reports of biomarkers in diagnosing this complication of iodinated contrast administration. Emerging literature on the relative nephrotoxicity of iso-osmolar versus low-osmolar contrast media and the value of bicarbonate hydration are discussed. More recent preventive measures using prostacyclin, 'statins', and erythropoietin are also reviewed. SUMMARY: Contrast-induced nephropathy is an increasing cause of acute kidney injury and is associated with significant mortality and morbidity. Future developments in this field will focus on refining the clinical significance of this complication, earlier diagnosis with biomarkers, clarifying the role for bicarbonate and iso-osmolar contrast agents as preventive strategies, and the introduction of new prophylactic techniques on the basis of an improved understanding of pathogenesis at the cellular level.