Respiratory Distress

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Douglas F Willson - One of the best experts on this subject based on the ideXlab platform.

  • pediatric acute Respiratory Distress syndrome consensus recommendations from the pediatric acute lung injury consensus conference
    Pediatric Critical Care Medicine, 2015
    Co-Authors: Philippe Jouvet, Douglas F Willson, Simon Erickson, Robinder G Khemani, Lincoln S Smith, Jerry J Zimmerman, Mary K Dahmer, Heidi R Flori, Michael W Quasney
    Abstract:

    Objective: To describe the final recommendations of the Pediatric Acute Lung Injury Consensus Conference. Design: Consensus conference of experts in pediatric acute lung injury. Setting: Not applicable. Subjects: PICU patients with evidence of acute lung injury or acute Respiratory Distress syndrome. Interventions: None. Methods: A panel of 27 experts met over the course of 2 years to develop a taxonomy to define pediatric acute Respiratory Distress syndrome and to make recommendations regarding treatment and research priorities. When published, data were lacking a modified Delphi approach emphasizing strong professional agreement was used. Measurements and Main Results: A panel of 27 experts met over the course of 2 years to develop a taxonomy to define pediatric acute Respiratory Distress syndrome and to make recommendations regarding treatment and research priorities. When published data were lacking a modified Delphi approach emphasizing strong professional agreement was used. The Pediatric Acute Lung Injury Consensus Conference experts developed and voted on a total of 151 recommendations addressing the following topics related to pediatric acute Respiratory Distress syndrome: 1) Definition, prevalence, and epidemiology; 2) Pathophysiology, comorbidities, and severity; 3) Ventilatory support; 4) Pulmonary-specific ancillary treatment; 5) Nonpulmonary treatment; 6) Monitoring; 7) Noninvasive support and ventilation; 8) Extracorporeal support; and 9) Morbidity and long-term outcomes. There were 132 recommendations with strong agreement and 19 recommendations with weak agreement. Once restated, the final iteration of the recommendations had none with equipoise or disagreement. Conclusions: The Consensus Conference developed pediatric-specific definitions for acute Respiratory Distress syndrome and recommendations regarding treatment and future research priorities. These are intended to promote optimization and consistency of care for children with pediatric acute Respiratory Distress syndrome and identify areas of uncertainty requiring further investigation.

  • pediatric calfactant in acute Respiratory Distress syndrome trial
    Pediatric Critical Care Medicine, 2013
    Co-Authors: Robert F Tamburro, Neal J Thomas, Jonathon D Truwit, Mark R Conaway, Christine Traul, Edward J Truemper, Douglas F Willson, Edmund E Egan
    Abstract:

    Rationale Our previous studies in children with acute lung injury/acute Respiratory Distress syndrome demonstrated improved outcomes with exogenous surfactant (calfactant) administration. Sample sizes in those studies were small, however, and the subject populations heterogeneous, thus making recommendations tenuous. Objective To investigate the efficacy of surfactant administration in a larger, more homogenous population of children with lung injury/acute Respiratory Distress syndrome due to direct lung injury. Design and setting Masked, randomized, placebo-controlled trial in 24 children's hospitals in six different countries. Patients and methods Children 37 weeks postconception to 18 years old with lung injury/acute Respiratory Distress syndrome due to direct lung injury were randomized to receive up to three doses of 30 mg/cm height of surfactant (calfactant) versus placebo (air) within 48 hours of intubation and initiation of mechanical ventilation. The primary outcome was mortality at 90 days. Ventilator-free days, changes in oxygenation, and adverse events were also assessed. Results The study was stopped at the sponsor's request after the second interim analysis for presumed futility. A total of 110 subjects were enrolled, with consent withdrawn from one whose data are unavailable. There were no significant differences between groups except in hospital-free days (10.4 ± 7.8 placebo vs 6.4 ± 7.8 surfactant; p = 0.01). Overall 90-day mortality was 11% (seven surfactant, five placebo). No immediate improvement in oxygenation was associated with surfactant administration. Conclusions Surfactant did not improve outcomes relative to placebo in this trial of children with direct lung injury/acute Respiratory Distress syndrome. Differences in concentration of the surfactant, failure to recruit the lung during surfactant administration, or using two rather than four position changes during administration are possible explanations for the difference from previous studies. Exogenous surfactant cannot be recommended at this time for children with direct lung injury/acute Respiratory Distress syndrome.

  • the relationship of fluid administration to outcome in the pediatric calfactant in acute Respiratory Distress syndrome trial
    Pediatric Critical Care Medicine, 2013
    Co-Authors: Robert F Tamburro, Neal J Thomas, Jonathon D Truwit, Mark R Conaway, Christine Traul, Edward J Truemper, Douglas F Willson, Edmund E Egan
    Abstract:

    Objectives:Adult studies have demonstrated the relationship between fluid overload and poor outcomes in acute lung injury/acute Respiratory Distress syndrome. The approach of pediatric intensivists to fluid management in acute lung injury/acute Respiratory Distress syndrome and its effect on outcome

Katja Grohmann - One of the best experts on this subject based on the ideXlab platform.

  • infantile spinal muscular atrophy with Respiratory Distress type 1 smard1
    Annals of Neurology, 2003
    Co-Authors: Piroschka Stolz, Catrin Janetzki, Raymonda Varon, Katja Grohmann, Kate Bushby, Enrico Bertini, Francesco Muntoni, Markus Schuelke, Robert A Ouvrier
    Abstract:

    Autosomal recessive spinal muscular atrophy with Respiratory Distress type 1 (SMARD1) is the second anterior horn cell disease in infants in which the genetic defect has been defined. SMARD1 results from mutations in the gene encoding the immunoglobulin mu-binding protein 2 (IGHMBP2) on chromosome 11q13. Our aim was to review the clinical features of 29 infants affected with SMARD1 and report on 26 novel IGHMBP2 mutations. Intrauterine growth retardation, weak cry, and foot deformities were the earliest symptoms of SMARD1. Most patients presented at the age of 1 to 6 months with Respiratory Distress due to diaphragmatic paralysis and progressive muscle weakness with predominantly distal lower limb muscle involvement. Sensory and autonomic nerves are also affected. Because of the poor prognosis, there is a demand for prenatal diagnosis, and clear diagnostic criteria for infantile SMARD1 are needed. The diagnosis of SMARD1 should be considered in infants with non-5q spinal muscular atrophy, neuropathy, and muscle weakness and/or Respiratory Distress of unclear cause. Furthermore, consanguineous parents of a child with sudden infant death syndrome should be examined for IGHMBP2 mutations.

Renato Machado Fiori - One of the best experts on this subject based on the ideXlab platform.

  • lamellar body count and stable microbubble test on tracheal aspirates from infants for the diagnosis of Respiratory Distress syndrome
    Pediatric Critical Care Medicine, 2012
    Co-Authors: Ana Claudia Vieira, Jefferson Pedro Piva, Terezinha Paz Munhoz, Pedro Celiny Ramos Garcia, Humberto Holmer Fiori, Renato Machado Fiori
    Abstract:

    OBJECTIVES: To evaluate the performance of lamellar body count in tracheal aspirates from intubated preterm babies to predict Respiratory Distress syndrome. DESIGN: Case-control study. SETTING: Three neonatal intensive care units. PATIENTS: Seventy-two patients not older than 3 days were included in the study, 38 preterm infants with Respiratory Distress syndrome, 16 preterms without Respiratory Distress syndrome, and 18 term infants. All required mechanical ventilation. INTERVENTIONS: Lamellar body count was performed in an automated cell counter. Tracheal samples were diluted in dithiothreitol without centrifugation and kept frozen at -20°C until use. Samples were placed in a dithiothreitol-containing test tube at a ratio of one part tracheal aspirate to six parts dithiothreitol solution, vortexed for 10 secs, and aspirated by the cell counter. Lamellar body count was performed using the platelet channel. All results were multiplied by seven. The stable microbubble test was done for comparison. MEASUREMENTS: Lamellar body count and stable microbubble test. MAIN RESULTS: Lamellar body count was significantly lower in the Respiratory Distress syndrome group compared with the non Respiratory Distress syndrome preterm group and also with the term group. The median and interquartile range obtained for lamellar body count were 38,500/μL (14,000-112,000) for the Respiratory Distress syndrome group, 822,500/μL (442,000-962,500) for the non Respiratory Distress syndrome preterm group, and 633,000/μL (322,000-1,608,000) for the term group (p < .001). The sensitivity and specificity of lamellar body count and stable microbubble test for the diagnosis of Respiratory Distress syndrome were calculated, taking into consideration the Respiratory Distress syndrome and the non Respiratory Distress syndrome preterm groups. Considering a cutoff point of 200,000 lamellar bodies/μL, lamellar body count sensitivity was 92.1% (95% confidence interval 78.6-98.3) and lamellar body count specificity was 93.8% (95% confidence interval 69.8-99.8). The area under the curve was 0.94 (95% confidence interval 0.84-1.00). CONCLUSIONS: Lamellar body count and stable microbubble test can be rapidly and easily performed on tracheal aspirates and they seem to have very good performance for diagnosing Respiratory Distress syndrome in intubated patients.

  • lamellar body count and stable microbubble test on tracheal aspirates from infants for the diagnosis of Respiratory Distress syndrome
    Pediatric Critical Care Medicine, 2012
    Co-Authors: Ana Claudia Vieira, Jefferson Pedro Piva, Terezinha Paz Munhoz, Pedro Celiny Ramos Garcia, Humberto Holmer Fiori, Renato Machado Fiori
    Abstract:

    OBJECTIVES: To evaluate the performance of lamellar body count in tracheal aspirates from intubated preterm babies to predict Respiratory Distress syndrome. DESIGN: Case-control study. SETTING: Three neonatal intensive care units. PATIENTS: Seventy-two patients not older than 3 days were included in the study, 38 preterm infants with Respiratory Distress syndrome, 16 preterms without Respiratory Distress syndrome, and 18 term infants. All required mechanical ventilation. INTERVENTIONS: Lamellar body count was performed in an automated cell counter. Tracheal samples were diluted in dithiothreitol without centrifugation and kept frozen at -20°C until use. Samples were placed in a dithiothreitol-containing test tube at a ratio of one part tracheal aspirate to six parts dithiothreitol solution, vortexed for 10 secs, and aspirated by the cell counter. Lamellar body count was performed using the platelet channel. All results were multiplied by seven. The stable microbubble test was done for comparison. MEASUREMENTS: Lamellar body count and stable microbubble test. MAIN RESULTS: Lamellar body count was significantly lower in the Respiratory Distress syndrome group compared with the non Respiratory Distress syndrome preterm group and also with the term group. The median and interquartile range obtained for lamellar body count were 38,500/μL (14,000-112,000) for the Respiratory Distress syndrome group, 822,500/μL (442,000-962,500) for the non Respiratory Distress syndrome preterm group, and 633,000/μL (322,000-1,608,000) for the term group (p < .001). The sensitivity and specificity of lamellar body count and stable microbubble test for the diagnosis of Respiratory Distress syndrome were calculated, taking into consideration the Respiratory Distress syndrome and the non Respiratory Distress syndrome preterm groups. Considering a cutoff point of 200,000 lamellar bodies/μL, lamellar body count sensitivity was 92.1% (95% confidence interval 78.6-98.3) and lamellar body count specificity was 93.8% (95% confidence interval 69.8-99.8). The area under the curve was 0.94 (95% confidence interval 0.84-1.00). CONCLUSIONS: Lamellar body count and stable microbubble test can be rapidly and easily performed on tracheal aspirates and they seem to have very good performance for diagnosing Respiratory Distress syndrome in intubated patients.

Edmund E Egan - One of the best experts on this subject based on the ideXlab platform.

  • pediatric calfactant in acute Respiratory Distress syndrome trial
    Pediatric Critical Care Medicine, 2013
    Co-Authors: Robert F Tamburro, Neal J Thomas, Jonathon D Truwit, Mark R Conaway, Christine Traul, Edward J Truemper, Douglas F Willson, Edmund E Egan
    Abstract:

    Rationale Our previous studies in children with acute lung injury/acute Respiratory Distress syndrome demonstrated improved outcomes with exogenous surfactant (calfactant) administration. Sample sizes in those studies were small, however, and the subject populations heterogeneous, thus making recommendations tenuous. Objective To investigate the efficacy of surfactant administration in a larger, more homogenous population of children with lung injury/acute Respiratory Distress syndrome due to direct lung injury. Design and setting Masked, randomized, placebo-controlled trial in 24 children's hospitals in six different countries. Patients and methods Children 37 weeks postconception to 18 years old with lung injury/acute Respiratory Distress syndrome due to direct lung injury were randomized to receive up to three doses of 30 mg/cm height of surfactant (calfactant) versus placebo (air) within 48 hours of intubation and initiation of mechanical ventilation. The primary outcome was mortality at 90 days. Ventilator-free days, changes in oxygenation, and adverse events were also assessed. Results The study was stopped at the sponsor's request after the second interim analysis for presumed futility. A total of 110 subjects were enrolled, with consent withdrawn from one whose data are unavailable. There were no significant differences between groups except in hospital-free days (10.4 ± 7.8 placebo vs 6.4 ± 7.8 surfactant; p = 0.01). Overall 90-day mortality was 11% (seven surfactant, five placebo). No immediate improvement in oxygenation was associated with surfactant administration. Conclusions Surfactant did not improve outcomes relative to placebo in this trial of children with direct lung injury/acute Respiratory Distress syndrome. Differences in concentration of the surfactant, failure to recruit the lung during surfactant administration, or using two rather than four position changes during administration are possible explanations for the difference from previous studies. Exogenous surfactant cannot be recommended at this time for children with direct lung injury/acute Respiratory Distress syndrome.

  • the relationship of fluid administration to outcome in the pediatric calfactant in acute Respiratory Distress syndrome trial
    Pediatric Critical Care Medicine, 2013
    Co-Authors: Robert F Tamburro, Neal J Thomas, Jonathon D Truwit, Mark R Conaway, Christine Traul, Edward J Truemper, Douglas F Willson, Edmund E Egan
    Abstract:

    Objectives:Adult studies have demonstrated the relationship between fluid overload and poor outcomes in acute lung injury/acute Respiratory Distress syndrome. The approach of pediatric intensivists to fluid management in acute lung injury/acute Respiratory Distress syndrome and its effect on outcome

Robert A Ouvrier - One of the best experts on this subject based on the ideXlab platform.

  • infantile spinal muscular atrophy with Respiratory Distress type 1 smard1
    Annals of Neurology, 2003
    Co-Authors: Piroschka Stolz, Catrin Janetzki, Raymonda Varon, Katja Grohmann, Kate Bushby, Enrico Bertini, Francesco Muntoni, Markus Schuelke, Robert A Ouvrier
    Abstract:

    Autosomal recessive spinal muscular atrophy with Respiratory Distress type 1 (SMARD1) is the second anterior horn cell disease in infants in which the genetic defect has been defined. SMARD1 results from mutations in the gene encoding the immunoglobulin mu-binding protein 2 (IGHMBP2) on chromosome 11q13. Our aim was to review the clinical features of 29 infants affected with SMARD1 and report on 26 novel IGHMBP2 mutations. Intrauterine growth retardation, weak cry, and foot deformities were the earliest symptoms of SMARD1. Most patients presented at the age of 1 to 6 months with Respiratory Distress due to diaphragmatic paralysis and progressive muscle weakness with predominantly distal lower limb muscle involvement. Sensory and autonomic nerves are also affected. Because of the poor prognosis, there is a demand for prenatal diagnosis, and clear diagnostic criteria for infantile SMARD1 are needed. The diagnosis of SMARD1 should be considered in infants with non-5q spinal muscular atrophy, neuropathy, and muscle weakness and/or Respiratory Distress of unclear cause. Furthermore, consanguineous parents of a child with sudden infant death syndrome should be examined for IGHMBP2 mutations.