Respiratory outcomes in children with congenital myotonic dystrophy

2021 ◽  
pp. 1-5
Author(s):  
Jaclyn C. Omura ◽  
Maida Chen ◽  
Miriam Haviland ◽  
Susan Apkon
Keyword(s):  
Respiratory Distress ◽  
Myotonic Dystrophy ◽  
Invasive Mechanical Ventilation ◽  
Retrospective Chart Review ◽  
Mortality Rates ◽  
Respiratory Support ◽  
Non Invasive ◽  
Congenital Myotonic Dystrophy ◽  
Respiratory Outcomes

PURPOSE: Congenital myotonic dystrophy (CDM) results in hypotonia and acute respiratory distress at birth. Previous studies show that prolonged periods of intubation (>4 weeks) correlate with increased mortality rates. The objective is to describe the use and duration of respiratory support in newborns with CDM and how these relate to mortality. METHODS: A retrospective chart review was performed at a tertiary pediatric hospital among children with confirmed diagnosis of CDM. The main outcome measures were: mortality, duration of invasive mechanical ventilation (IMV) and non-invasive partial pressure ventilation (NIPPV), along with long-term use of respiratory support and equipment. RESULTS: A total of 18 subjects met inclusion criteria, 83%.f which had documented respiratory distress at birth, 39%.equired NIPPV, and 50%.equired intubation in the neonatal period. The earliest NIPPV was initiated at day one of life, and the latest extubation to NIPPV was at 17 days of life. CONCLUSION: This cohort required IMV for shorter periods with earlier transitions to NIPPV which suggests a possible change in practice and earlier transition to NIPPV recently. Further data are needed to determine if there is a possible correlation between the need for NIPPV/IMV and mortality rates.

scholarly journals Risk Factors Associated with Mechanical Ventilation in Critical Bronchiolitis

Children ◽  
2021 ◽  
Vol 8 (11) ◽  
pp. 1035
Author(s):  
Rachel K. Marlow ◽  
Sydney Brouillette ◽  
Vannessa Williams ◽  
Ariann Lenihan ◽  
Nichole Nemec ◽  
...  
Keyword(s):  
Intensive Care Unit ◽  
Mechanical Ventilation ◽  
Intensive Care ◽  
Medical Center ◽  
Invasive Mechanical Ventilation ◽  
Severity Of Illness ◽  
Pediatric Intensive Care ◽  
Respiratory Support ◽  
Non Invasive ◽  
Lengths Of Stay

The American Academy of Pediatrics (AAP) recommends supportive care for the management of bronchiolitis. However, patients admitted to the intensive care unit with severe (critical) bronchiolitis define a unique group with varying needs for both non-invasive and invasive respiratory support. Currently, no guidance exists to help clinicians discern who will progress to invasive mechanical support. Here, we sought to identify key clinical features that distinguish pediatric patients with critical bronchiolitis requiring invasive mechanical ventilation from those that did not. We conducted a retrospective cohort study at a tertiary pediatric medical center. Children ≤2 years old admitted to the pediatric intensive care unit (PICU) from January 2015 to December 2019 with acute bronchiolitis were studied. Patients were divided into non-invasive respiratory support (NRS) and invasive mechanical ventilation (IMV) groups; the IMV group was further subdivided depending on timing of intubation relative to PICU admission. Of the 573 qualifying patients, 133 (23%) required invasive mechanical ventilation. Median age and weight were lower in the IMV group, while incidence of prematurity and pre-existing neurologic or genetic conditions were higher compared to the NRS group. Multi-microbial pneumonias were diagnosed more commonly in the IMV group, in turn associated with higher severity of illness scores, longer PICU lengths of stay, and more antibiotic usage. Within the IMV group, those intubated earlier had a shorter duration of mechanical ventilation and PICU length of stay, associated with lower pathogen load and, in turn, shorter antibiotic duration. Taken together, our data reveal that critically ill patients with bronchiolitis who require mechanical ventilation possess high risk features, including younger age, history of prematurity, neurologic or genetic co-morbidities, and a propensity for multi-microbial infections.

Adult Respiratory Distress Syndrome in a Pediatric Intensive Care Unit: Predisposing Conditions, Clinical Course, and Outcome

PEDIATRICS ◽  
1981 ◽  
Vol 67 (6) ◽  
pp. 790-795
Author(s):  
Raymond K. Lyrene ◽  
William E. Truog
Keyword(s):  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Adult Respiratory Distress Syndrome ◽  
Distress Syndrome ◽  
Clinical Course ◽  
Pediatric Intensive Care ◽  
Retrospective Chart Review ◽  
Trauma Mortality ◽  
Predisposing Conditions

Adult respiratory distress syndrome, commonly seen in adults, is not well recognized in children. A retrospective chart review was carried out to determine the relative incidence, predisposing conditions, clinical course, and outcome of children with adult respiratory distress syndrome. Fifteen patients were identified. The most common predisposing conditions were near-drowning and near-strangulation with a noticeable absence of major trauma. Mortality was 60%. Death was most often secondary to central nervous system complications. Air leak was the most common complication of treatment. Two of six survivors suffered major neurologic handicaps. Long-term pulmonary sequelae were minimal.

Association of Antenatal Terbutaline and Respiratory Support Requirements in Preterm Neonates

2021 ◽  
Author(s):  
Buranee Yangthara ◽  
Visanu Kittiarpornpon ◽  
Pitiporn Siripattanapipong ◽  
Walaiporn Bowornkitiwong ◽  
Ratchada Kitsommart ◽  
...  
Keyword(s):  
Care Center ◽  
Tertiary Care ◽  
Lung Compliance ◽  
Respiratory Support ◽  
Preterm Neonates ◽  
Non Invasive ◽  
Antenatal Steroids ◽  
Respiratory Outcomes ◽  
Record Review ◽  
Retrospective Medical Record Review

Background: Before the advent of antenatal steroids, early non-invasive respiratory support (NIV) and intratracheal surfactant, antenatal terbutaline was also used to improve lung compliance and reduce the incidence of respiratory distress syndrome (RDS). Objectives: To study the association between antenatal terbutaline and endotracheal intubation (ET) within the first 24 hours of life, RDS, bronchopulmonary dysplasia (BPD), and intraventricular hemorrhage (IVH) in infants with gestational age (GA) of < 32 weeks. And to study the association between antenatal terbutaline, and ET or NIV within the first 24 hours of life, and RDS in infants with GA of 32 to 36 weeks. Method: A retrospective medical record review of preterm infants delivered at a single tertiary care center from October 2016 to December 2020. Multivariable logistic regression was used to explore the association between antenatal terbutaline and neonatal respiratory support. Result: 1,794 infants were included, 234 (13.0%) had GA < 32 weeks and 1,560 (86.9%) had GA 32 to 36 weeks. Antenatal terbutaline, corticosteroid, or both agents were administered in 561 (31.3%), 1,461 (81.4%), and 555 (30.9%), respectively. Antenatal terbutaline was significantly associated with a reduction in ET (adjusted odds ratio (aOR) = 0.40, 95% confident interval (CI) 0.19 to 0.82, p = 0.012) in infants of GA < 32 weeks, but not in infants with GA 32 to 36 weeks. Antenatal terbutaline was not associated with RDS or BPD, but was significantly associated with a reduction in grade III-IV IVH (aOR 0.11, CI 0.01 to 0.98; p = 0.048), in infants of GA < 32 weeks. Conclusion: In a state-of-the-art neonatal care setting, antenatal terbutaline was associated with a reduction in ET during the first 24 hours in infants of GA < 32 weeks. The use of antenatal terbutaline to improve acute neonatal respiratory outcomes merits reconsideration.

scholarly journals Protocolized Post-Extubation Respiratory Support to prevent reintubation: protocol and statistical analysis plan for a clinical trial

BMJ Open ◽  
2019 ◽  
Vol 9 (8) ◽  
pp. e030476 ◽  
Author(s):  
Jonathan Dale Casey ◽  
Erin R Vaughan ◽  
Bradley D Lloyd ◽  
Peter A Bilas ◽  
Eric J Hall ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Usual Care ◽  
Invasive Mechanical Ventilation ◽  
High Flow ◽  
Respiratory Support ◽  
Invasive Ventilation ◽  
High Flow Nasal Cannula ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
Risk Patients

IntroductionFollowing extubation from invasive mechanical ventilation, nearly one in seven critically ill adults requires reintubation. Reintubation is independently associated with increased mortality. Postextubation respiratory support (non-invasive ventilation or high-flow nasal cannula applied at the time of extubation) has been reported in small-to-moderate-sized trials to reduce reintubation rates among hypercapnic patients, high-risk patients without hypercapnia and low-risk patients without hypercapnia. It is unknown whether protocolised provision of postextubation respiratory support to every patient undergoing extubation would reduce the overall reintubation rate, compared with usual care.Methods and analysisThe Protocolized Post-Extubation Respiratory Support (PROPER) trial is a pragmatic, cluster cross-over trial being conducted between 1 October 2017 and 31 March 2019 in the medical intensive care unit of Vanderbilt University Medical Center. PROPER compares usual care versus protocolized post-extubation respiratory support (a respiratory therapist-driven protocol that advises the provision of non-invasive ventilation or high-flow nasal cannula based on patient characteristics). For the duration of the trial, the unit is divided into two clusters. One cluster receives protocolised support and the other receives usual care. Each cluster crosses over between treatment group assignments every 3 months. All adults undergoing extubation from invasive mechanical ventilation are enrolled except those who received less than 12 hours of mechanical ventilation, have ‘Do Not Intubate’ orders, or have been previously reintubated during the hospitalisation. The anticipated enrolment is approximately 630 patients. The primary outcome is reintubation within 96 hours of extubation.Ethics and disseminationThe trial was approved by the Vanderbilt Institutional Review Board. The results will be submitted for publication in a peer-reviewed journal and presented at one or more scientific conferences.Trial registration numberNCT03288311.

scholarly journals Changing consumption of resources for respiratory support and short-term outcomes in four consecutive geographical cohorts of infants born extremely preterm over 25 years since the early 1990s

BMJ Open ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. e037507
Author(s):  
Jeanie L Y Cheong ◽  
Joy E Olsen ◽  
Li Huang ◽  
Kim M Dalziel ◽  
Rosemarie A Boland ◽  
...  
Keyword(s):  
Retinopathy Of Prematurity ◽  
Periventricular Leukomalacia ◽  
Respiratory Support ◽  
Non Invasive ◽  
Discharge Home ◽  
Nasal Cannulae ◽  
Extremely Preterm ◽  
Additional Costs ◽  
Prospective Longitudinal

ObjectivesIt is unclear how newer methods of respiratory support for infants born extremely preterm (EP; 22–27 weeks gestation) have affected in-hospital sequelae. We aimed to determine changes in respiratory support, survival and morbidity in EP infants since the early 1990s.DesignProspective longitudinal cohort study.SettingThe State of Victoria, Australia.ParticipantsAll EP births offered intensive care in four discrete eras (1991–1992 (24 months): n=332, 1997 (12 months): n=190, 2005 (12 months): n=229, and April 2016–March 2017 (12 months): n=250).Outcome measuresConsumption of respiratory support, survival and morbidity to discharge home. Cost-effectiveness ratios describing the average additional days of respiratory support associated per additional survivor were calculated.ResultsMedian duration of any respiratory support increased from 22 days (1991–1992) to 66 days (2016–2017). The increase occurred in non-invasive respiratory support (2 days (1991–1992) to 51 days (2016–2017)), with high-flow nasal cannulae, unavailable in earlier cohorts, comprising almost one-half of the duration in 2016–2017. Survival to discharge home increased (68% (1991–1992) to 87% (2016–2017)). Cystic periventricular leukomalacia decreased (6.3% (1991–1992) to 1.2% (2016–2017)), whereas retinopathy of prematurity requiring treatment increased (4.0% (1991–1992) to 10.0% (2016–2017)). The average additional costs associated with one additional infant surviving in 2016–2017 were 200 (95% CI 150 to 297) days, 326 (183 to 1127) days and 130 (70 to 267) days compared with 1991–1992, 1997 and 2005, respectively.ConclusionsConsumption of resources for respiratory support has escalated with improved survival over time. Cystic periventricular leukomalacia reduced in incidence but retinopathy of prematurity requiring treatment increased. How these changes translate into long-term respiratory or neurological function remains to be determined.

scholarly journals La mère et l'enfant atteints de dystrophie myotonique de Steinert

1989 ◽  
Vol 16 (1) ◽  
pp. 104-108 ◽  
Author(s):  
G. Paris ◽  
R. Laframboise ◽  
J.-P. Bouchard
Keyword(s):  
Mental Retardation ◽  
Respiratory Distress ◽  
Myotonic Dystrophy ◽  
Malformation Syndrome ◽  
Gestational Period ◽  
Congenital Myotonic Dystrophy ◽  
Pregnancy And Delivery

ABSTRACT:The Mother and Infant with Myotonic Dystrophy Pregnancy and delivery present a number of risks for the mother suffering from myotonic dystrophy, and for her infant. Most of the time, she does not even know that she is affected by the disease and a carrier of the gene. We review the complications of pregnancy and delivery in myotonic patients, and propose a simple management with specific items for each gestational period. The child of a dystrophic mother has a 50% risk of inheriting the abnormal gene. He may also exhibit a developmental and malformation syndrome called "congenital myotonic dystrophy". From the beginning, he may show respiratory distress, thereafter inability to swallow and severely hypotonia. Later, he may demonstrate mental retardation. Some of the most obvious signs found in neonates in our practice are illustrated. We also add a few tests to the list of those already recommended for these children.

scholarly journals Limitations of the ARDS criteria during high-flow oxygen or non-invasive ventilation: Evidence from critically ill COVID-19 patients

2021 ◽  
Author(s):  
Michael Hultström ◽  
Ola Hellkvist ◽  
Lucian Covaciu ◽  
Filip Fredén ◽  
Robert Frithiof ◽  
...  
Keyword(s):  
Invasive Mechanical Ventilation ◽  
High Flow ◽  
University Hospital ◽  
Respiratory Support ◽  
Arterial Oxygen ◽  
Invasive Ventilation ◽  
Steady State Condition ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
High Flow Oxygen

Abstract Introduction The ratio of partial pressure of arterial oxygen to inspired oxygen fraction (PaO2/FIO2) during invasive mechanical ventilation (MV) is used as criteria to grade the severity of respiratory failure in acute respiratory distress syndrome (ARDS). During the SARS-CoV2 pandemic the use of PaO2/FIO2 ratio has been increasingly used in non-invasive respiratory support such as high-flow nasal cannula (HFNC) and non-invasive ventilation (NIV). The grading of hypoxemia in non-invasively ventilated patients is uncertain. The main hypothesis, investigated in this study, was that the PaO2/FIO2 ratio does not change when switching between MV, NIV and HFNC. Methods This was a sub-study of a single-center prospective observational study of patients admitted to the intensive care unit (ICU) at Uppsala University Hospital in Sweden for critical COVID-19. In a steady state condition, the PaO2/FIO2 ratio was recorded before and after any change between two of the studied respiratory support techniques (i.e., HFNC, NIV and MV). Results A total of 148 patients were included in the present analysis. We find that any change in respiratory support from or to HFNC caused a significant change in PaO2/FIO2 ratio (up to 48 mmHg, from HFNC to MV). Changes in respiratory support between NIV and MV did not show consistent change in PaO2/FIO2 ratio. In patients classified as mild to moderate ARDS during MV, the change from HFNC to MV showed a variable increase in PaO2/FIO2 ratio ranging between 52 and 140 mmHg (median of 127 mm Hg). This made prediction of ARDS severity during MV from the apparent ARDS grade during HFNC impossible. Conclusion HFNC is associated with lower PaO2/FIO2 ratio than either NIV or MV in the same patient, while NIV and MV provided similar PaO2/FIO2 and thus ARDS grade by Berlin definition. The large variation of PaO2/FIO2 ratio precludes using ARDS grade as a measure of pulmonary damage during HFNC.

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