Cases reported "Cystic Fibrosis"

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1/23. Combined lung and liver transplantation in a girl with cystic fibrosis.

    PURPOSE: To describe the anesthetic considerations of a combined lung and liver transplant in a 14-yr-old girl with cystic fibrosis. CLINICAL FEATURES: A 14 yr-old girl with cystic fibrosis presented for combined liver and lung transplantation. Anesthetic management was complex in that the pulmonary, hemodynamic, and hematological changes after cardiopulmonary bypass and lung transplantation made the management of the subsequent liver transplant unique. We used a moderate dose fentanyl and isoflurane anesthetic with invasive monitoring including a pulmonary artery catheter. Upon reperfusion of the new liver our patient exhibited severe pulmonary hypertension that was associated with a decrease in cardiac output and systemic hypotension. Utilizing a pulmonary artery catheter, this episode was treated with an increase of prostaglandin E1 (PGE1) infusion to 0.025 microg x kg(-1) x min(-1) and the initiation of 3 microg x kg(-1) x min(-1) dobutamine. The pulmonary hypertension resolved and the cardiac output and blood pressure returned to baseline levels. CONCLUSION: The anesthetic considerations for a combined lung and liver transplant are complex because of the interactions and alterations in cardiovascular, pulmonary and hemostatic systems. The use of a pulmonary artery catheter was critical to the management of our patient because it allowed us to accurately treat an episode of hypotension occurring during liver transplantation. This episode was secondary to acute pulmonary hypertension which is common after pulmonary transplantation but unusual during liver transplantation. It is also critical that a team approach is used to consider all of the concerns of the multiple services managing these complex patients.
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2/23. Fatal pulmonary haemorrhage during anaesthesia for bronchial artery embolization in cystic fibrosis.

    Three children with cystic fibrosis (CF) had significant pulmonary haemorrhage during anaesthetic induction prior to bronchial artery embolization (BAE). Haemorrhage was associated with rapid clinical deterioration and subsequent early death. We believe that the stresses associated with intermittent positive pressure ventilation (IPPV) were the most likely precipitant to rebleeding and that the inability to clear blood through coughing was also an important factor leading to deterioration. Intermittent positive pressure ventilation should be avoided when possible in children with CF with recent significant pulmonary haemorrhage.
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3/23. pneumothorax in adults with cystic fibrosis dependent on nasal intermittent positive pressure ventilation (NIPPV): a management dilemma.

    The management of pneumothorax in three adult patients with cystic fibrosis dependent on nasal intermittent positive pressure ventilation is described.
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4/23. Negative extrathoracic pressure in infants with cystic fibrosis and respiratory failure.

    Respiratory support using negative extrathoracic pressure or high-frequency chest wall oscillation was used to treat 3 infants with cystic fibrosis and respiratory failure who had not responded to maximal medical therapy. Beneficial clinical effects were noted in all three cases. Pulmonary function testing was performed in 2 cases, and measures of compliance increased.
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5/23. noninvasive ventilation using bilevel positive airway pressure to treat impending respiratory failure in the postanesthesia care unit.

    The author presents the use of noninvasive positive pressure ventilation using bilevel positive airway pressure (BiPAP) to treat respiratory insufficiency and hypoxemia in three patients in the postanesthesia care unit. BiPAP improved respiratory function with a decreased respiratory rate, decreased PaCO2, and improved oxygenation in the three patients. The applications of these techniques and equipment required are reviewed.
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6/23. noninvasive ventilation in respiratory failure due to cystic fibrosis.

    BACKGROUND: Noninvasive positive-pressure ventilation (NIPPV) is increasingly used as an effective means of avoiding endotracheal intubation and mechanical ventilation in patients with respiratory insufficiency or failure. methods: We retrospectively reviewed our experience with NIPPV to treat respiratory failure in five patients with cystic fibrosis (CF). RESULTS: Despite chronic lung disease related to CF, none of our cases were end-stage. All patients had recent pulmonary function tests showing a forced expiratory volume in 1 second (FEV1) of more than 30% predicted for age. All patients had progressive atelectasis, hypoxemia, and impending respiratory failure related to an acute pulmonary exacerbation or upper abdominal surgical procedure (open gastrostomy tube placement). Respiratory rates decreased, oxygen saturation increased, fraction of inspired oxygen (FiO2) requirement decreased, transcutaneous CO2 decreased, and atelectasis resolved with NIPPV. CONCLUSIONS: Use of NIPPV provides effective respiratory support while avoiding the need for endotracheal intubation. The applications of NIPPV, reports of its use in patients with CF, and the equipment required are reviewed.
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7/23. prone position as a life-saving measure for acute pulmonary haemorrhage in a young adult with cystic fibrosis.

    Fatal pulmonary haemorrhage is a rare complication of cystic fibrosis. A case of unexpected life-threatening pulmonary haemorrhage is presented, and the successful management of this problem including immediate prone ventilation. Different anaesthetic techniques, avoiding endotracheal intubation and positive pressure ventilation, which may avoid similar complications, are described.
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8/23. Nebulized morphine as a treatment for dyspnea in a child with cystic fibrosis.

    OBJECTIVE: To shed light on nebulized morphine, a new treatment for dyspnea in children with terminal lung disease. methods: A clinical case study was conducted on a patient in a tertiary care medical center. RESULTS: Nebulized morphine was administered in incremental doses ranging from 2.5 mg to 12.5 mg in a 10-year-old, 20-kg boy with end-stage cystic fibrosis. Before the nebulized morphine treatments were started, a dose of nebulized lidocaine failed to provide the patient with any relief. After each dose of morphine, the following parameters were recorded: visual analog "dyspnea" scores, vital signs, venous blood gases, and blood levels of morphine. The nebulized morphine was found to have a modest effect on the patient's dyspnea, with no significant differences found between the varying doses. Venous carbon dioxide tension levels increased <4 mm Hg for all doses except 12.5 mg, for which there was a 9-mm Hg increase. Systemic blood levels of morphine were <10 ng/mL at all doses. The nebulized morphine did not cause any significant changes in blood pressure or heart rate for doses <12.5 mg. CONCLUSIONS: Inhaled morphine was associated with a mild, beneficial effect on dyspnea, with minimal differences found between the lowest and highest doses. This "ceiling" effect may be the result of saturation of opioid receptors in the lung, the variable bioavailability of inhaled morphine, or a placebo response. More studies are needed to determine what, if any, the optimum dose of nebulized morphine is for children.
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9/23. Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure.

    Nocturnal nasal intermittent positive pressure ventilation (nIPPV) has been used successfully in the management of patients with respiratory failure due to chest wall deformity and neuromuscular disease. In order to determine if nIPPV is useful in patients with cystic fibrosis (CF) complicated by respiratory failure, we treated four hypercapnic patients for up to 18 months. All patients had failed to respond to intensive conventional therapy, including nocturnal nasal CPAP in three of the patients. Within a few days of commencing nIPPV, all reported improved length and quality of sleep. There was lessening of the degree of hypercapnia and an increase in respiratory muscle strength. After stabilization in the hospital, all patients were able to be discharged home receiving nocturnal assisted ventilation. The improvements seen in these patients have been maintained for up to 18 months. We believe nIPPV offers an effective therapeutic approach for patients with end-stage CF in hypercapnic respiratory failure and may be particularly advantageous for those awaiting heart-lung transplant.
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10/23. Aerosolized iloprost as a bridge to lung transplantation in a patient with cystic fibrosis and pulmonary hypertension.

    We describe a patient with cystic fibrosis, end-stage lung disease, and secondary pulmonary hypertension in whom aerosolized iloprost was effective in lowering pulmonary artery pressure and improving functional status, thus proving successful as a bridge to lung transplantation. Inhaled iloprost may be an efficient and selective approach to treat pulmonary hypertension related to end-stage obstructive pulmonary disease.
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