Minerva pediatrica
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Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non-immune microangiopathic hemolytic anemia and acute renal failure. HUS is typically classified into two primary types: 1) HUS due to infections, often associated with diarrhea (D+HUS, Shiga toxin-producing Escherichia Coli-HUS), with the rare exception of HUS due to a severe disseminated infection caused by Streptococcus; 2) HUS related to complement, such HUS is also known as "atypical HUS" and is not diarrhea associated (D-HUS, aHUS); but recent studies have shown other forms of HUS, that can occur in the course of systemic diseases or physiopathological conditions such as pregnancy, after transplantation or after drug assumption. Moreover, new studies have shown that the complement system is an important factor also in the typical HUS, in which the infection could highlight an underlying dysregulation of complement factors. ⋯ For the aHUS the initial management is supportive and similar to the approach for STEC-HUS; currently we have moved from the historic plasma therapy to new therapeutic approaches, first of all eculizumab, a monoclonal antibody that blocks the C5 cascade. This drug has shown an improvement in platelet count, cessation of hemolysis, improvement of renal function within a few days after the treatment. In patients with end-stage renal disease (ESRD) renal transplantation from a non-related donor and prophylactic administration of eculizumab to prevent recurrent disease in the allograft could be considered.
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Providing optimal mechanical ventilation to critically-ill children remains a challenge. Patient-ventilator dyssynchrony results frequently with numerous deleterious consequences on patient outcome including increased requirement for sedation, prolonged duration of ventilation, and greater imposed work of breathing. Most currently used ventilators have real-time, continuously-displayed graphics of pressure, volume, and flow versus time (scalars) as well as pressure, and flow versus volume (loops). ⋯ This paper starts with a description of the scalars and loops followed by a discussion of the information that can be obtained from each of these graphics. A review will follow, on the common types of dyssynchronous interactions and how each of these can be detected on the ventilator graphics. The final section discusses how graphics can be used to optimize the ventilator support provided to patients.