Presse Med
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Thrombotic thrombocytopenic purpura (TTP) is a particular form of thrombotic microangiopathy typically characterized by microangiopathic hemolytic anemia, profound peripheral thrombocytopenia, and a severe deficiency of the von Willebrand factor-cleaving protease ADAMTS13 (acronym for A Disintegrin And Metalloproteinase with ThromboSpondin-1 motifs [13th member of the family]). ADAMTS13 deficiency is usually severe (<10% of normal activity) and results from autoantibodies directed to ADAMTS13 (acquired TTP) or from biallelic mutations of the encoding gene. In some cases, acquired TTP occurs in association with specific conditions that must be identified for appropriate management: a HIV infection, a connective tissue disease, a pregnancy, a cancer or a treatment with antiplatelet agents. ⋯ Prophylactic infusions of plasma should be performed in chronic relapsing forms to prevent long-term organ complications, which have to be assessed accurately and regularly. In the upcoming years, new targeted therapies evaluated through international trials should further improve the management of these diseases. Consensual guidelines for the treatment of very specific and rare situations (such as management during pregnancy in USS patients and prevention of relapses in chronic relapsing acquired TTP) should arise from the shared experience of national groups.
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The objectives are to slow the progression of chronic kidney disease (CKD), to take all the cardiovascular risk factors into account, to screen for and treat specific complications and to prepare, if necessary, for renal replacement treatment (transplantation or dialysis). The principal treatment targets are: blood pressure less than 130/80 mmHg and proteinuria less than 0.5 g/day (ratio of proteinuria/creatinuria <50mg/mmol). ⋯ The periodicity of clinical and laboratory assessments depends on the CKD stage, the speed of disease progression and the need to reassess the impact of therapeutic interventions. Comprehensive multidisciplinary management can slow or even stop the progression of CKD and reduce its cardiovascular complications, which are the leading cause of death in these patients.
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Antibodies to neutral endopeptidase, a podocyte protein, are responsible for rare alloimmune neonatal membranous nephropathy that develops in children from neutral endopeptidase-deficient mothers. Neutral endopeptidase was the first podocyte antigen described in human membranous nephropathy. PLA2R1, the type-M receptor of soluble phospholipase A2, is a major target antigen in so-called idiopathic membranous nephropathy in adults. ⋯ Immunization against cationic bovine serum albumin is a cause of early childhood membranous nephropathy. This finding points to a possible role of food and environmental antigens in membranous nephropathy. The newly identified antigen-antibody systems should be considered as molecular signatures challenging the uniform histological definition and having a major impact on patient care in a near future.
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Screening of chronic kidney disease (CKD) that includes estimation of the glomerular filtration rate (GFR) and evaluation proteinuria should be performed in all HIV-infected patients and these parameters have to be monitored annually in patients at higher risk for CKD. Black patients have a genetic predisposition to develop HIV-associated nephropathy. ⋯ Dosing antiretroviral agents with kidney metabolism should be adjusted when eGFR is bellow 50 mL/min/1.73 m(2). eGFR and serum phosphorus at baseline and during treatment should be carefully assessed in patients receiving tenofovir. Proximal renal tubular toxicity must be further evaluated in the presence of eGFR decrease and/or hypophosphatemia under tenofovir therapy.
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Comparative Study
Using actigraphy versus polysomnography in the clinical assessment of chronic insomnia (retrospective analysis of 27 patients).
The current study was conducted in order to investigate whether several different methods of characterizing sleep and insomnia symptoms produce different diagnoses. ⋯ Although this was a preliminary and retrospective study, our results seemed to indicate that actigraphy and PSG were able to lead to a similar output particularly with regard to type of insomnia. Beyond PSG, actigraphy might have a clinical utility in assessing sleep disorders in adults complaining of chronic insomnia.