Vascular calcification in chronic kidney disease associated with pathogenic variants in ABCC6.

Vascular calcification is prevalent in chronic kidney disease (CKD). Genetic causes of CKD account for 10-20% of adult-onset disease. Vascular calcification is thought to be one of the most important risk factors for increased cardiovascular morbidity and mortality in CKD patients and is detectable in 80% of patients with end stage kidney disease (ESKD).

Reassuring Data on the Cardiovascular Risk in Adults With X-linked Hypophosphatemia Receiving Conventional Therapy.

Context: X-linked hypophosphatemia (XLH) is a rare genetic disorder that results in increased plasma levels of fibroblast growth factor 23 (FGF23). Several studies have demonstrated a direct association between FGF23 and cardiovascular mortality in cohorts of patients with chronic renal failure. However, in patients with XLH, studies on the cardiovascular impact of the disease are rare, with contradictory results.

Predictors of relapse and preventative strategies in immune thrombotic thrombocytopenic purpura.

Introduction: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune blood disorder, which presents with microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombosis and is caused by severe deficiency of ADAMTS13. iTTP may result in both acute and chronic complications and is rapidly fatal without expedient treatment. Life-time risk of relapse is approximately 40%.

Blood-brain barrier permeability in survivors of immune-mediated thrombotic thrombocytopenic purpura: a pilot study.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening disorder of systemic microthrombosis and organ ischemia. The etiology of chronic cerebrovascular outcomes in iTTP survivors is largely unknown. In this pilot study, we measured blood-brain barrier (BBB) permeability in patients with iTTP at the start of remission and 6 months later.

Spot urine protein to creatinine ratio.

In a recent article in Pediatric Nephrology, EM Yang and colleagues (Pediatr Nephrol 2017: doi: 10.1007/s00467-016-3587-6 ) published a retrospective cross-sectional study involving a cohort of 442 children with an mean estimated glomerular filtration rate of >60 mL/min/1.73 m.

Minimum mycophenolic acid levels are associated with donor-specific antibody formation.

Although de novo DSA are associated with inferior graft survival, there are no effective strategies to prevent their formation. Underexposure to MPA (prodrug: MMF) also contributes to rejection rates early after transplantation, but the effect of this phenomenon on the formation of DSA long-term post-transplantation is unknown. Data are expressed as mean (standard deviation).

"Drink at least 8 glasses of water a day to be healthy???".

The saying "drink at least 8 glasses of water per day to be healthy" has had little supporting evidence. The purpose of this article was to briefly introduce the role of hydration in health in order to explore in more detail recent observational studies of hyperhydration in chronic kidney disease (CKD) in man. The Modification of Diet in Renal Disease study initially noted a negative association between increasing urine output and progression of CKD that was absent when corrected for baseline variables.

Using individual DSA titers to assess for accommodation after late humoral rejection.

Management of late humoral rejection remains challenging, and DSA may persist. A case report illustrates how individual DSA titers using solid-phase-based assays may help to assess for accommodation. A male cystinosis patient received a cadaveric renal transplant at the age of 12 yr with a daclizumab, tacrolimus, MMF, and steroids-based immunosuppression.

ACE levels may affect cystatin C measurements.

Objective: To assess the relationship between angiotensin-converting enzyme (ACE) levels and cystatin C in a patient with the presumed diagnosis of sarcoidosis.

Design And Methods: Case report with correlation analysis between ACE levels and cystatin C concentrations while considering gold-standard nuclear medicine glomerular filtration rate (GFR) methods for the measurement of renal function.

Results: We observed a strong correlation between ACE levels and cystatin C concentration in 12-year old with the presumed diagnosis of sarcoidosis in the absence of renal pathology and abnormal renal function.

Should we consider MMF therapy after rituximab for nephrotic syndrome?

The management of steroid-dependent nephrotic syndrome, especially in patients who have failed to respond to cytotoxic drugs, such as cyclophosphamide, remains challenging. Rituximab represents a new (off-label) therapeutic option. In a significant portion of patients, it has a short serum half-life following the recovery of CD20-positive cells.

Impaired GFR is the most important determinant for FGF-23 increase in chronic kidney disease.

Objectives: It is unclear whether fibroblast growth factor-23 (FGF-23) increases in response to phosphate accumulation or to decrease clearance in chronic kidney disease (CKD) as is the case with other low molecular weight proteins such as cystatin C (CysC).

Design And Methods: This cross-sectional study measured serum FGF-23, CysC, and other serum markers of bone metabolism in 69 patients, aged 18 months-24 years, with various stages of CKD (eGFR=11-214mL/min).

Results: FGF-23 levels were significantly correlated with CysC and parathyroid hormone levels (PTH) on univariate non-linear regression analysis.

The use of dietary supplements and medications by Canadian athletes at the Atlanta and Sydney Olympic Games.

Objective: To learn more about the prevalence of dietary supplement and medication use by Canadian athletes in the Olympic Games in Atlanta 1996 and Sydney 2000.

Setting And Participants: Data were collected from personal interviews with Canadian athletes who participated at the 1996 Atlanta and 2000 Sydney Olympic Games. The athletes were interviewed by Canadian physicians regarding the use of vitamins, minerals, nutritional supplements, and prescribed and over-the-counter medications.