High uric acid (UA) is hypothesized to worsen kidney and cardiovascular disease morbidity activation of systemic inflammation. Clinical trials of UA modification report reduction of the inflammatory marker high sensitivity C-reactive protein (hs-CRP) as an outcome measure, but studies have not demonstrated that hyperuricemia independently increases hs-CRP when adjusted for important confounders such as body mass index (BMI), sex, and age. To identify clinical risk factors for elevated hs-CRP, including but not limited to hyperuricemia, through a cross-sectional analysis of the National Health and Nutrition Examination Survey (NHANES) 2015-2018.
View Article and Find Full Text PDFLysosomal acid ceramidase (Ac) has been shown to be critical for ceramide hydrolysis and regulation of lysosome function and cellular homeostasis. In the present study, we generated a knockout mouse strain (Asah1/Podo) with a podocyte-specific deletion of the α subunit (main catalytic subunit) of Ac. Although no significant morphologic changes in glomeruli were observed in these mice under light microscope, severe proteinuria and albuminuria were found in these podocyte-specific knockout mice compared with control genotype littermates.
View Article and Find Full Text PDFCongenital or primary nephrogenic diabetes insipidus (NDI) is a rare genetic disorder that severely impairs renal concentrating ability, resulting in massive polyuria. There is limited information about prognosis or evidence guiding the management of these patients, either in the high-risk period after diagnosis, or long-term. We describe the clinical presentation, genetic etiology, treatment and renal outcomes in a large group of children <21 years with NDI.
View Article and Find Full Text PDFBackground: Sickle cell nephropathy (SCN) is a progressive disease that contributes significant morbidity and mortality in sickle cell disease (SCD), yet it remains poorly understood. Hyperuricemia negatively impacts renal function in the non-sickle cell population but is understudied in SCD.
Methods: We performed a cross-sectional analysis of the first 78 pediatric SCD patients enrolled in a cohort study.
Objective: To assess practice pattern similarities and differences amongst pediatric rheumatologists and nephrologists in the management of pediatric Granulomatosis with Polyangiitis (GPA).
Methods: A voluntary survey was distributed to the Midwest Pediatric Nephrology Consortium Group (MWPNC) and an international pediatric rheumatology email listserv in 2016-2017. Data were collected on general practice characteristics and preferences for induction management under three clinical scenarios (A-C): newly diagnosed GPA with glomerulonephritis, GPA with rapidly progressive glomerulonephritis, and GPA with pulmonary hemorrhage.