New aspects on the pathogenesis of renal disorders related to monoclonal gammopathies.

Background: Multiple myeloma and other related monoclonal gammopathies are frequently encountered conditions associated with renal damage, especially in elderly population. They are arising from clonal proliferation of plasma cells in bone marrow producing various quantities of abnormal monoclonal immunoglobulins, or their components/fragments.

Summary: These abnormal proteins differ from normal immunoglobulins in the amino acid sequence and in the three-dimensional structure of the molecule, which may determine their toxicity.

Elevated asymmetric dimethylarginine is associated with oxidant stress aggravation in patients with early stage autosomal dominant polycystic kidney disease.

Background/aims: In experimental models of polycystic kidney disease impaired bioavailability of nitric oxide (NO) and elevated mRNA expression of oxidative stress markers at the kidney level was noted. However, clinical studies investigating the potential role of endothelial dysfunction and oxidative stress in the pathogenesis of autosomal dominant polycystic kidney disease (ADPKD) are limited. We evaluated asymmetric dimethylarginine (ADMA) as marker of NO synthase inhibitor as well as 15-F2t-Isoprostane and oxidized-low density lipoprotein (oxidized-LDL) as measures of oxidative stress in patients with early stages ADPKD.

Role of asymmetrical dimethylarginine in the progression of renal disease.

Asymmetric dimethylarginine (ADMA) is a naturally occurring amino acid found in tissues and cells that circulates in plasma and is excreted in urine. It inhibits nitric oxide synthases (NOs) and produces considerable cardiovascular biological effects. Several studies have suggested that plasma concentrations of ADMA provide a marker of risk for endothelial dysfunction and cardiovascular disease.

Scleroderma renal crisis accompanied by new-onset pulmonary arterial hypertension: an acute systemic endothelial injury? Case report and literature.

Systemic Sclerosis (SSc) is a multisystem connective tissue disease characterized by fibrosis of the skin and internal organs and extensive vasculopathy. We report a case of a 41 year-old white woman with a 10 year-history of limited scleroderma, who developed the rare combination of Scleroderma Renal Crisis (SRC) and Systemic Sclerosis related Pulmonary Arterial Hypertension (SScPAH) in the same time. Although the patient received the proposed antihypertensive treatment, the renal function did not recover, and she initiated on renal replacement therapy.

Right atrium myxoma coexisting with antiphospholipid syndrome: a case report.

In this case report we describe a rare case of right atrium myxoma that coexisted with antiphospholipid syndrome in a young woman. We describe the unusual findings and diagnostic challenges combined with a review of the literature.