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In a personal series of 107 patients, we describe clinical presentations, methods of recognition and therapeutic management of inherited fatty acid oxidation (FAO) defects. As a whole, FAO disorders appear very severe: among the 107 patients, only 57 are still living. Including 47 siblings who died early in infancy, in total 97 patients died, of whom 30%(More)
CONTEXT Between March and July 2009, the largest number of confirmed cases of 2009 influenza A(H1N1) infection occurred in North America. OBJECTIVE To describe characteristics, treatment, and outcomes of critically ill patients in Canada with 2009 influenza A(H1N1) infection. DESIGN, SETTING, AND PATIENTS A prospective observational study of 168(More)
BACKGROUND The clinical manifestations of inherited disorders of fatty acid oxidation vary according to the enzymatic defect. They may present as isolated cardiomyopathy, sudden death, progressive skeletal myopathy, or hepatic failure. Arrhythmia is an unusual presenting symptom of fatty acid oxidation deficiencies. METHODS AND RESULTS Over a period of 25(More)
BACKGROUND Fetal distress is a frequent complication of gastroschisis, and could be screened for by home monitoring, as many pregnant women expecting an affected child live far away from a specialized perinatal center. This study was undertaken to audit a policy of fetal home monitoring (FHM) to achieve early detection of fetal heart rate (FHR)(More)
From 1990 to 2000, we performed eight liver-kidney transplants in eight children, aged 1-16 years, with end-stage renal failure (ESRF) due to primary hyperoxaluria (PH1). The duration of dialysis before transplantation ranged from 2 to 42 months (mean 14 months) and was <1 year in four patients. Only the first patient underwent postoperative hemodialysis;(More)
OBJECTIVES The aim of this survey was to determine North American and European pediatric intensivists' knowledge and stated practice in the management of children with acute respiratory distress syndrome with regard to mechanical ventilation settings; blood gas and SO2 targets; and use of adjunctive treatments at sites where actual practice had just been(More)
PURPOSE Duration of weaning from mechanical ventilation is decreased with the use of written protocols in adults. In children, the use of written protocols has not had such an impact. METHODS AND MEASUREMENTS We conducted a single-center trial to assess the feasibility of conducting a multicenter randomized clinical trial comparing the duration of weaning(More)
OBJECTIVE To prospectively evaluate relationships among serum cytokine levels, innate immune responsiveness, and mortality in a multicenter cohort of critically ill children with influenza infection. DESIGN Prospective, multicenter, observational study. SETTING Fifteen pediatric ICUs among members of the Pediatric Acute Lung Injury and Sepsis(More)
INTRODUCTION Heat and moisture exchangers (HMEs) are increasingly used in the ICU for gas conditioning during mechanical ventilation. Independent assessments of the humidification performance of HMEs are scarce. The aim of the present study was thus to assess the humidification performance of a large number of adult HMEs. METHOD We assessed 48 devices(More)
BACKGROUND Invasive mechanical ventilation, if prolonged, may lead to high morbidity and mortality. OBJECTIVE To determine the incidence rate and early risk factors for prolonged acute invasive mechanical ventilation in children. DESIGN Retrospective longitudinal cohort study over a 1-yr period. PATIENTS All consecutive episodes of invasive mechanical(More)