Fabrice Lesage

Weaning children from mechanical ventilation with a computer-driven system (closed-loop protocol): a pilot study.

Objective: To evaluate the applicability, tolerance, and efficacy of a closed-loop protocol to wean children from mechanical ventilation. Design: Prospective single-center pilot study. Setting: Tertiary care university hospital. Patients: Twenty mechanically ventilated children aged between 1 and 17 yrs, with a body weight ≥10 kg, no inotropes, and no heavy sedation. Interventions: Patients were weaned in pressure support mode by a closed-loop computerized protocol (closed-loop protocol) that interprets clinical data in real time and controls pressure support levels. Measurements and Main Results: The closed-loop protocol applicability and tolerance were evaluated. The efficacy of this protocol was evaluated by comparing the duration of mechanical ventilation with a historical group of 20 patients weaned with a clinician-decision protocol. The closed-loop protocol successfully decreased pressure support ventilation in 16 children, recommended separation from the ventilator in 14 children, and did not cause any serious adverse events. Mechanical ventilation duration was 5.1 ± 4.2 days in the closed-loop group and 6.7 ± 11.5 days (mean ± sd) in the clinician-decision group (p = .33) with no difference in the need for reintubation or noninvasive mechanical ventilation (one of 20 and four of 20, respectively; p = .20). Conclusions: A closed-loop protocol was successfully used to wean children from mechanical ventilation. Further studies are required to assess the impact of this novel therapeutic strategy on the length of mechanical ventilation.

Long-term outcomes in Ornithine Transcarbamylase deficiency: a series of 90 patients

BackgroundThe principal aim of this study was to investigate the long-term outcomes of a large cohort of patients with ornithine transcarbamylase deficiency (OTCD) who were followed up at a single medical center.MethodsWe analyzed clinical, biochemical and genetic parameters of 90 patients (84 families, 48 males and 42 females) with OTCD between 1971 and 2011.ResultsTwenty-seven patients (22 boys, 5 girls) had a neonatal presentation; 52 patients had an “intermediate” late-onset form of the disease (21 boys, 31 girls) that was revealed between 1 month and 16 years; and 11 patients (5 boys, 6 girls) presented in adulthood (16 to 55 years). Patients with a neonatal presentation had increased mortality (90% versus 13% in late-onset forms) and peak plasma ammonium (mean value: 960 μmol/L versus 500 μmol/L) and glutamine (mean value: 4110 μmol/L versus 1000 μmol/L) levels at diagnosis. All of the neonatal forms displayed a greater number of acute decompensations (mean value: 6.2/patient versus 2.5 and 1.4 in infants and adults, respectively). In the adult group, some patients even recently died at the time of presentation during their first episode of coma. Molecular analyses identified a deleterious mutation in 59/68 patients investigated. Single base substitutions were detected more frequently than deletions (69% and 12%, respectively), with a recurrent mutation identified in the late-onset groups (pArg40 His; 13% in infants, 57% in adults); inherited mutations represented half of the cases. The neurological score did not differ significantly between the patients who were alive in the neonatal or late-onset groups and did not correlate with the peak ammonia and plasma glutamine concentrations at diagnosis. However, in late-onset forms of the disease, ammonia levels adjusted according to the glutamine/citrulline ratio at diagnosis were borderline predictors of low IQ (p = 0.12 by logistic regression; area under the receiver operating characteristic curve of 76%, p <0.05).ConclusionsOTCD remains a severe disease, even in adult-onset patients for whom the prevention of metabolic decompensations is crucial. The combination of biochemical markers warrants further investigations to provide additional prognostic information regarding the neurological outcomes of patients with OTCD.

Cerebral vasculitis in severe Kawasaki disease: early detection by magnetic resonance imaging and good outcome after intensive treatment

Kawasaki disease is an acute vasculitis, that has a classic complication of acquired coronary artery aneurysm. Severe forms with multi‐organ involvement or neurological dysfunction are rare. Cerebral vascular involvement has been related to large‐vessel injury or cardioembolism, leading to focal brain infarction. A 4‐year‐old female presented with unusual, rapidly catastrophic Kawasaki disease with refractory shock, acute renal failure, and coma, requiring intensive haemodynamic management. The observation of diffuse micro‐haemorrhages (T2*‐weighted sequence) associated with white matter injury on brain magnetic resonance imaging (MRI) pointed towards lesions of the medium/small blood vessels. Cerebral vasculitis was suspected and the immunosuppressive treatment was increased Subsequently, the patient’s recovery was rapid. On follow‐up severe, bilateral vitritis was evident and surgery improved visual outcome. Early recognition of severe or unusual forms of Kawasaki disease could lead to more favourable outcome using appropriate treatment strategies. Diffuse cerebral micro‐haemorrhages on T2* brain MRI sequences might be a key sign for the diagnosis of medium or small cerebral vessel involvement.

Fatal parvovirus B19 myocarditis in children and possible dysimmune mechanism.

We report 2 cases of previously healthy children, who developed, after a common parvovirus B19 infection, a sudden inflammatory response, involving predominantly T cell, directed against myocardium and leading to fatal outcome. These cases and several published case reports further our understanding of fulminating parvovirus myocarditis in children.

Children with Down syndrome: Clinical course and mortality-associated factors in a French medical paediatric intensive care unit

To investigate clinical course and mortality‐associated factors in children with Down syndrome (DS) managed in a medical paediatric intensive care unit.

Regional Pediatric Acute Stroke Protocol: Initial Experience During 3 Years and 13 Recanalization Treatments in Children

Background and Purpose— To evaluate hyperacute management of pediatric arterial ischemic stroke, setting up dedicated management pathways is the first recommended step to prove the feasibility and safety of such treatments. A regional pediatric stroke alert protocol including 2 centers in the Paris-Ile-de-France area, France, was established. Methods— Consecutive pediatric patients (28 days–18 years) with confirmed arterial ischemic stroke who had acute recanalization treatment (intravenous r-tPA [recombinant tissue-type plasminogen activator], endovascular procedure, or both) according to the regional pediatric stroke alert were retrospectively reviewed during a 40-month period. Results— Thirteen children, aged 3.7 to 16.6 years, had recanalization treatment. Median time from onset to magnetic resonance imaging was 165 minutes (150–300); 9 out of 13 had large-vessel occlusion. Intravenous r-tPA was used in 11 out of 13 patients, with median time from onset to treatment of 240 minutes (178–270). Endovascular procedure was performed in patients time-out for intravenous r-tPA (n=2) or after intravenous r-tPA inefficiency (n=2). No intracranial or peripheral bleeding was reported. One patient died of malignant stroke; outcome was favorable in 11 out of 12 survivors (modified Rankin Scale score 0–2). Conclusions— Hyperacute recanalization treatment in pediatric stroke, relying on common protocols and adult/pediatric ward collaboration, is feasible. Larger systematic case collection is encouraged.