Metabolic Alkalosis in Adults with Stable Cystic Fibrosis

Al-Ghimlas, Fahad; Faughnan, Marie E.; Tullis, Elizabeth

doi:10.2174/1874306401206010059

Cited by 21 publications

(18 citation statements)

References 26 publications

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“…It has been previously suggested that a concomitant metabolic alkalosis contributes to hypercapnic respiratory failure in exacerbations of adult CF and that these patients less frequently exhibit acidosis than COPD patients due to the abnormal electrolyte transport in CF patients compared to COPD, even during exacerbations [ 35 , 36 ]. This may have been present in our subjects as well with a median pH in the normal range NIV which has been clinically used for hypercapnic respiratory failure in CF, but little is known about its physiological effects, particularly in the adult population.…”

Section: Discussionmentioning

confidence: 99%

High-flow nasal oxygen versus noninvasive ventilation in adult patients with cystic fibrosis: a randomized crossover physiological study

Sklar

Dres

Rittayamai

et al. 2018

Ann. Intensive Care

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BackgroundNoninvasive ventilation (NIV) is the first-line treatment of adult patients with exacerbations of cystic fibrosis (CF). High-flow nasal oxygen therapy (HFNT) might benefit patients with hypoxemia and can reduce physiological dead space. We hypothesized that HFNT and NIV would similarly reduce work of breathing and improving breathing pattern in CF patients. Our objective was to compare the effects of HFNT versus NIV in terms of work of breathing, assessed noninvasively by the thickening fraction of the diaphragm (TFdi, measured with ultrasound), breathing pattern, transcutaneous CO2 (PtcCO2), hemodynamics, dyspnea and comfort.MethodsAdult CF patients who had been stabilized after requiring ventilatory support for a few days were enrolled and ventilated with HFNT and NIV for 30 min in crossover random order.ResultsFifteen patients were enrolled. Compared to baseline, HFNT, but not NIV, reduced respiratory rate (by 3 breaths/min, p = 0.01) and minute ventilation (by 2 L/min, p = 0.01). Patients also took slightly larger tidal volumes with HFNT compared to NIV (p = 0.02). TFdi per breath was similar under the two techniques and did not change from baseline. MAP increased from baseline with NIV and compared to HFNT (p ≤ 0.01). Comfort was poorer with the application of both HFNT and NIV than baseline. No differences were found for heart rate, SpO2, PtcCO2 or dyspnea.ConclusionsIn adult CF patients stabilized after indication for ventilatory support, HFNT and NIV have similar effects on diaphragmatic work per breath, but high-flow therapy confers additional physiological benefits by decreasing respiratory rate and minute ventilation.Clinical trial registrationEthics Committee of St. Michael’s Hospital (REB #14-338) and clinicaltrial.gov (NCT02262871).Electronic supplementary materialThe online version of this article (10.1186/s13613-018-0432-4) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

High-flow nasal oxygen versus noninvasive ventilation in adult patients with cystic fibrosis: a randomized crossover physiological study

Sklar

Dres

Rittayamai

et al. 2018

Ann. Intensive Care

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“…Patients with CF are prone to the development of metabolic alkalosis [7-9], which creates a physiologic diminution of respiratory effort that can be detrimental in CF patients , who suffer from chronic lung injury. Metabolic alkalosis could also be the initial presentation or complication of cystic fibrosis in infants and children [10].…”

Section: Introductionmentioning

confidence: 99%

Downregulation of the Cl-/HCO3-Exchanger Pendrin in Kidneys of Mice with Cystic Fibrosis: Role in the Pathogenesis of Metabolic Alkalosis

Kia

Barone

McDonough

et al. 2018

Cell Physiol Biochem

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Background/Aims: Patients with cystic fibrosis (CF) are prone to the development of metabolic alkalosis; however, the pathogenesis of this life threatening derangement remains unknown. We hypothesized that altered acid base transport machinery in the kidney collecting duct underlies the mechanism of impaired bicarbonate elimination in the CF kidney. Methods: Balance studies in metabolic cages were performed in WT and CFTR knockout (CF) mice with the intestinal rescue in response to bicarbonate loading or salt restriction, and the expression levels and cellular distribution of acid base and electrolyte transporters in the proximal tubule, collecting duct and small intestine were examined by western blots, northern blots and/or immunofluorescence labeling. Results: Baseline parameters, including acid-base and systemic vascular volume status were comparable in WT and CF mice, as determined by blood gas, kidney renin expression and urine chloride excretion. Compared with WT animals, CF mice demonstrated a significantly higher serum HCO₃^- concentration (22.63 in WT vs. 26.83 mEq/l in CF mice; n=4, p=0.013) and serum pH (7.33 in WT vs. 7.42 in CF mice; n=4, p=0.00792) and exhibited impaired kidney HCO₃^- excretion (urine pH 8.10 in WT vs. 7.35 in CF mice; n=7, p=0.00990) following a 3-day oral bicarbonate load. When subjected to salt restriction, CF mice developed a significantly higher serum HCO₃^- concentration vs. WT animals (29.26 mEq/L in CF mice vs. 26.72 in WT; n=5, p=0.0291). Immunofluorescence labeling demonstrated a profound reduction in the apical expression of the Cl^-/HCO₃^- exchanger pendrin in cortical collecting duct cells and western and northern blots indicated diminished plasma membrane abundance and mRNA expression of pendrin in CF kidneys. Conclusions: We propose that patients with cystic fibrosis are prone to the development of metabolic alkalosis secondary to the inactivation of the bicarbonate secreting transporter pendrin, specifically during volume depletion, which is a common occurrence in CF patients.

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“…8H, I). Nevertheless, the moderate defect may be related to the metabolic alkalosis observed in CF patients [121][122][123][124].…”

Section: Kidneymentioning

confidence: 99%

Bicarbonate in cystic fibrosis

Kunzelmann

Schreiber

Hadorn

2017

Journal of Cystic Fibrosis

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Metabolic Alkalosis in Adults with Stable Cystic Fibrosis

Cited by 21 publications

References 26 publications

High-flow nasal oxygen versus noninvasive ventilation in adult patients with cystic fibrosis: a randomized crossover physiological study

High-flow nasal oxygen versus noninvasive ventilation in adult patients with cystic fibrosis: a randomized crossover physiological study

Downregulation of the Cl-/HCO3-Exchanger Pendrin in Kidneys of Mice with Cystic Fibrosis: Role in the Pathogenesis of Metabolic Alkalosis

Bicarbonate in cystic fibrosis

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