cystic fibrosis/hypoxia

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Predicting hypoxia in cystic fibrosis patients during exposure to high altitudes.

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BACKGROUND For patients with cystic fibrosis (CF)-related partial respiratory insufficiency and reduced arterial oxygen tension at ground level, the mild hypobaric environment on commercial jet aircraft poses the risk of severe hypoxemia. Thus, physicians should be able to estimate the extent of

Lung clearance index evaluation in detecting nocturnal hypoxemia in cystic fibrosis patients: Toward a new diagnostic tool.

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Nocturnal hypoxemia adversely affects outcomes in patients with cystic fibrosis (CF). Although an early detection of this abnormality may be desirable, still its predictability remains uncertain. The Lung Clearance Index (LCI) is a measure of lung ventilation distribution obtained from

The role of nutritional status, airway obstruction, hypoxia, and abnormalities in serum lipid composition in limiting exercise tolerance in children with cystic fibrosis.

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Previous work has shown that impaired exercise tolerance in children with cystic fibrosis (C.F.) is related to the severity of airway obstruction without elucidating the possible roles of hypoxia or malnutrition. It has been suggested that poor nutrition leads to abnormalities in serum fatty acids

Assessment of hypoxia in children with cystic fibrosis.

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Hypoxia during sleep and exercise may occur in an important number of patients with cystic fibrosis (CF). Despite its recognition, no clear definition for hypoxia in CF exists, and nor do guidelines for commencing oxygen therapy. CF patients with hypoxia may have increased pulmonary artery pressure,

Ventilatory responses to hypercapnia and hypoxia in conscious cystic fibrosis knockout mice Cftr-/-.

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This study was designed to examine the ventilatory performance and the lung histopathology of cystic fibrosis knockout mice (Cftr-/-) compared with heterozygous (Cftr+/-) or wild-type (Cftr+/+) littermates. Ventilation was recorded in conscious animals using whole-body plethysmography. Tidal volume

Nocturnal hypoxemia in children and adolescents with cystic fibrosis.

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To determine the prevalence of nocturnal hypoxemia and its association with pulmonary function, nutritional status, sleep macrostructure, and obstructive respiratory events during sleep in a population of clinically stable children and adolescents with cystic fibrosis (CF). This was a

Sleep hypoxemia in young adults with cystic fibrosis.

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Hypoxemia, obstructive apnea, and disordered breathing during sleep have been documented in adult patients with chronic obstructive pulmonary disease. We studied sleep, oxygen saturation, and breathing in nine patients (ages 17 to 26 years) with cystic fibrosis (CF) lung disease by means of

Nocturnal hypoxia and sleep disturbances in cystic fibrosis.

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Disrupted sleep and nocturnal hypoxia are common in cystic fibrosis (CF). However, the predictors of nocturnal hypoxia in CF are still controversial. In order to identify the risk factors for nocturnal desaturation and sleep disturbances, we carried out a clinical and polysomnographic investigation

Accuracy of three pulse oximeters during exercise and hypoxemia in patients with cystic fibrosis.

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To evaluate the reliability of noninvasive oximeters during exercise in patients with cystic fibrosis (CF), we studied 9 patients during exercise tests, using 3 devices (Hewlett-Packard 47201A, Biox-Ohmeda 3700, Nellcor 100), comparing oximetry readings with arterial blood co-oximetry. Fifty samples

Inadequate erythroid response to hypoxia in cystic fibrosis.

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An increase in hemoglobin concentration characterizes the normal compensatory response to chronic tissue hypoxia. We observed no such increase in 42 chronically hypoxic patients with cystic fibrosis, in whom the mean concentration was 12.6 gm/dl; one third of the patients were anemic. Compared with

Postural hypoxemia in cystic fibrosis.

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We measured arterial blood gases in the sitting and supine position in 33 patients with cystic fibrosis (CF). Arterial PO2 ranged from 48 to 97 mm Hg in the sitting position and fell by 1 to 23 mm Hg in 26 patients, rose by 1 to 4 mm Hg in five, and was unchanged in two when supine. Mean PO2 change

Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia.

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OBJECTIVE To determine (1) the prevalence of pulmonary hypertension and cardiac dysfunction in adult cystic fibrosis (CF) patients with severe lung disease, (2) the relationship between these cardiovascular abnormalities and hypoxemia, and (3) the impact of subclinical pulmonary hypertension on

Impaired expression of hypoxia-inducible factor-1α in cystic fibrosis airway epithelial cells - a role for HIF-1 in the pathophysiology of CF?

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The continuous infection-inflammation cycle plays a crucial role in the progression of cystic fibrosis (CF) disease. This noxious loop can be aggravated by a reduced partial pressure of oxygen in the blood, hypoxemia, present in CF patients. These interconnected factors, hypoxia, inflammation and

Hypoxemia and hypercapnia during exercise and sleep in patients with cystic fibrosis.

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BACKGROUND In patients with cystic fibrosis (CF), it has been proposed that hypoxemia and hypercapnia occur during episodes of stress, such as exercise and sleep, and that respiratory muscle weakness because of malnutrition may be responsible. METHODS Pulmonary function, respiratory muscle strength,

Hypoxia promotes danger-mediated inflammation via receptor for advanced glycation end products in cystic fibrosis.

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BACKGROUND Hypoxia regulates the inflammatory-antiinflammatory balance by the receptor for advanced glycation end products (RAGE), a versatile sensor of damage-associated molecular patterns. The multiligand nature of RAGE places this receptor in the midst of chronic inflammatory

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