Baseline Characteristics of Pediatric Patients With Heart Failure Due to Systemic Left Ventricular Systolic Dysfunction in the PANORAMA-HF Trial.

Background: Sacubitril/valsartan has been approved for the management of heart failure (HF) with reduced ejection fraction in adults. PANORAMA-HF trial (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) investigated its effects on clinical outcomes in pediatric patients with HF.

Methods: PANORAMA-HF is a multicenter, Phase II/III study using an adaptive, seamless, 2-part design.

Lack of interaction between NEMO and SHARPIN impairs linear ubiquitination and NF-κB activation and leads to incontinentia pigmenti.

Background: Incontinentia pigmenti (IP; MIM308300) is a severe, male-lethal, X-linked, dominant genodermatosis resulting from loss-of-function mutations in the IKBKG gene encoding nuclear factor κB (NF-κB) essential modulator (NEMO; the regulatory subunit of the IκB kinase [IKK] complex). In 80% of cases of IP, the deletion of exons 4 to 10 leads to the absence of NEMO and total inhibition of NF-κB signaling. Here we describe a new IKBKG mutation responsible for IP resulting in an inactive truncated form of NEMO.

Rapid curbing of a vancomycin-resistant Enterococcus faecium outbreak in a nephrology department.

Background And Objectives: Vancomycin-resistant enterococci (VRE) are recovered with increasing frequency among patients with chronic renal failure, making VRE a major concern in nephrology departments, especially for patients who are treated by hemodialysis. We report herein the successful aggressive management of a VRE outbreak in a nephrology department.

Design, Setting, Participants, & Measurements: An Enterococcus faecium vanB strain was isolated from a peritoneal dialysis solution from an inpatient.