Related Experiment Videos

Invasive mechanical ventilation for acute respiratory failure in children with cystic fibrosis: outcome analysis and

Ariel Berlinski1, Leland L Fan, Claudia A Kozinetz

  • 1Section of Pediatric Pulmonology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030, USA.

Pediatric Pulmonology
|September 3, 2002
PubMed

Insights

Outcomes for cystic fibrosis (CF) patients requiring invasive mechanical ventilation (IMV) are poor, especially for those aged 5 and older. Malnutrition and hemoptysis increase the risk of needing IMV in CF patients.

Area of Science:

  • Pulmonology
  • Pediatrics
  • Critical Care Medicine

Background:

  • Cystic fibrosis (CF) patients requiring invasive mechanical ventilation (IMV) for acute respiratory failure (ARF) have historically faced poor outcomes, with mortality rates as high as 60-90%.
  • A comprehensive review of mortality in pediatric CF patients requiring IMV has not been published in two decades, highlighting a need for updated data.

Purpose of the Study:

  • To investigate the outcomes of CF patients who required IMV due to ARF between 1988 and 1998.
  • To compare these outcomes with previously reported data and identify risk factors associated with mortality.
  • To identify factors that may increase the risk of developing ARF requiring IMV in CF patients.

Main Methods:

  • A retrospective cohort study design was employed, analyzing data from CF patients requiring IMV for ARF between 1988-1998.
  • A nested case-control study was used to identify risk factors for ARF leading to IMV.
  • Data collected included patient demographics, clinical status (e.g., steroid use, lung function, nutritional status, comorbidities), and specific events like hemoptysis and pneumothorax.

Main Results:

  • Thirty-eight episodes of IMV occurred in 33 CF patients during the study period.
  • Older age was significantly associated with mortality: patients aged 5-34 years had a 75% mortality rate compared to 22% for those younger than 5 years (P = 0.013).
  • Malnutrition (Odds Ratio = 4.2) and a history of major hemoptysis (Odds Ratio = 6.3) were identified as significant risk factors for requiring IMV due to ARF.

Conclusions:

  • Infants and young children (<5 years) with CF requiring IMV for ARF demonstrate a favorable prognosis.
  • CF patients aged 5 years and older requiring IMV face significantly higher mortality rates.
  • Malnutrition and a history of major hemoptysis are critical risk factors that increase the likelihood of CF patients developing ARF necessitating IMV.

Related Concept Videos

Mechanical Ventilation II: Invasive Ventilation01:23

Mechanical Ventilation II: Invasive Ventilation

Ventilators are essential medical equipment used to aid patients with respiratory difficulties. Their primary function is to assist or replace spontaneous breathing by providing mechanical ventilation. There are two general classes of mechanical ventilators: negative-pressure and positive-pressure ventilators.
Negative-Pressure Ventilators
Negative-pressure ventilators create a vacuum around the chest or body to draw air into the lungs, simulating breathing. This method does not require an...
Mechanical Ventilation I: Indication and Settings01:29

Mechanical Ventilation I: Indication and Settings

Mechanical ventilation is a life-saving technique for managing acute respiratory failure and other respiratory complications. The process involves using a machine known as a ventilator to supply oxygen to the lungs and assist in removing carbon dioxide. It serves as a bridge to long-term mechanical ventilation or a temporary measure until ventilatory support is discontinued. The ventilator can maintain this function for a prolonged period, providing critical support for patients until they can...
Acute Respiratory Failure-V01:29

Acute Respiratory Failure-V

The treatment for acute respiratory failure varies based on factors like the underlying cause, overall health, and severity. A collaborative healthcare team is essential for early detection, often through arterial blood gas analysis. Identifying the cause is the primary goal, with treatment strategies adjusted for ventilation/perfusion (V/Q) mismatch, shunting, or diffusion impairment.
Ensure that patients are monitored continuously for their response to therapy, including changes in...
Mechanical Ventilation III: Noninvasive Ventilation01:23

Mechanical Ventilation III: Noninvasive Ventilation

Noninvasive positive-pressure ventilation (NIPPV), continuous positive airway pressure (CPAP), and bilevel positive airway pressure (BiPAP) are essential methods in respiratory care. These ventilation techniques offer unique benefits for patients with various respiratory conditions, providing adequate support without requiring intubation. Let's explore how each method is crucial in improving patient outcomes and enhancing respiratory therapy.
Noninvasive Positive-Pressure Ventilation (NIPPV)
Acute Respiratory Failure-III01:30

Acute Respiratory Failure-III

Hypercapnic respiratory failure, also known as Type 2 or ventilatory respiratory failure, is a severe condition characterized by the body's inability to effectively remove carbon dioxide (CO2) from the bloodstream. It leads to an arterial CO2 pressure (PaCO2) exceeding 45 mmHg and a blood pH above 7.35. This situation indicates that the body's ventilatory demand, or the ventilation needed to maintain normal PaCO2 levels, surpasses its supply or the maximum gas flow achievable without causing...
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...