LONG-TERM USE OF INHALED HYPERTONIC SALINE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS: EXPERIENCE FROM A TERTIARY CARE CENTER IN A LOW AND MIDDLE INCOME COUNTRY
Keywords:Hypertonic Saline, Pulmonary Exacerbation, Pseudomonas colonization
AbstractBackground Long term hypertonic saline use has been found to improve mucus transport, airway hydration, and mucociliary clearance in patients with cystic fibrosis. However, the effect of hypertonic saline on the outcomes of patients with cystic fibrosis is not well established. The aim of our study was to determine the long-term use of hypertonic saline in reducing pulmonary exacerbations, length of hospital stay and pseudomonas colonization in patients with cystic fibrosis admitted for treatment at a tertiary care referral center. Methods: Retrospective cohort study was conducted on 71 patients with cystic fibrosis. Patients ranged in age between 3–18 years. All patients with two to five pulmonary exacerbations in the preceding six months were included in the study. Those who received regular inhaled 3–7% hypertonic saline twice daily during their admission and till 6 months after discharge from hospital were categorized as hypertonic saline (HTS) group. Patients who did not receive regular hypertonic saline for 6 months were included in the non-hypertonic saline (NHTS) group. Data was analyzed at the end of one year. Results: The HTS group had 37 patients whereas, the NHTS group had 34 patients. Mean number of exacerbation episodes was significantly lower in HTS group (2.18±0.84) as compared to NHTS group (3.67±0.91) (p<0.01) whereas, length of hospital stays and frequency of pseudomonas colonization did not significantly differ between the two groups (p=0.78 and p=0.12 respectively). The mean number of pulmonary exacerbations also significantly reduced from 3.11±1.07 to 2.18±0.84 p-value <0.01 in the HTS group over the follow-up period of one year. Conclusion: Long term hypertonic saline therapy is beneficial in patients with cystic fibrosis in preventing pulmonary exacerbations and subsequently reducing morbidity.
Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J 2004;23(1):146–58.
Bonfield TL, Panuska JR, Konstan MW, Hilliard KA, Hilliard JB, Ghnaim H, et al. Inflammatory cytokines in cystic fibrosis lungs. Am J Respir Crit Care Med 1995;152(6):2111–8.
Konstan MW, Hilliard KA, Norvell TM, Berger M. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med 1994;150(2):448–54.
Maiuri L, Raia V, Kroemer G. Strategies for the etiological therapy of cystic fibrosis. Cell Death Differ 2017;24(11):1825–44.
Shah U, Frossard P, Moatter T. Cystic fibrosis: defining a disease under-diagnosed in Pakistan. Trop Med Int Health 2009;14(5):542–5.
Cogen JD, Oron AP, Gibson RL, Hoffman LR, Kronman MP, Ong T, et al. Characterization of Inpatient Cystic Fibrosis Pulmonary Exacerbations. Pediatrics 2017;139(2):e20162642.
Aziz DA, Billoo AG, Qureshi A, Khalid M, Kirmani S. Clinical and laboratory profile of children with Cystic Fibrosis: Experience of a tertiary care center in Pakistan. Pak J Med Sci 2017;33(3):554–9.
Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC, et al. The future of cystic fibrosis care: a global perspective. Lancet Respir Med 2020;8(1):65–124.
Wark P, McDonald VM, Jones AP. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database Syst Rev 2005;20(3):CD001506.
Donaldson SH, Danielle Samulski T, LaFave C, Zeman K, Wu J, Trimble A, et al. A four week trial of hypertonic saline in children with mild cystic fibrosis lung disease: Effect on mucociliary clearance and clinical outcomes. J Cyst Fibros 2020;19(6):942–8.
Rosenfeld M, Emerson J, Williams-Warren J, Pepe M, Smith A, Montgomery AB, et al. Defining a pulmonary exacerbation in cystic fibrosis. J Pediatr 2001;139(3):359–65.
Flume PA, Mogayzel PJ, Robinson KA, Goss CH, Rosenblatt RL, Kuhn RJ, et al. Cystic Fibrosis Pulmonary Guidelines: Treatment of Pulmonary Exacerbations. Am J Respir Crit Care Med 2009;180(9):802–8.
Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006;354(3):229–40.
Mandelberg A, Amirav I. Hypertonic saline or high volume normal saline for viral bronchiolitis: Mechanisms and rationale. Pediatr Pulmonol 2010;45(1):36–40.
Robinson M, Hemming AL, Regnis JA, Wong AG, Bailey DL, Bautovich GJ, et al. Effect of increasing doses of hypertonic saline on mucociliary clearance in patients with cystic fibrosis. Thorax 1997;52(10):900–3.
Assouline G, Leibson V, Danon A. Stimulation of prostaglandin output from rat stomach by hypertonic solutions. Eur J Pharmacol 1977;44(3):271–3.
Dmello D, Nayak RP, Matuschak GM. Stratified assessment of the role of inhaled hypertonic saline in reducing cystic fibrosis pulmonary exacerbations: a retrospective analysis. BMJ Open 2011;1(1):e000019.
Rosenfeld M, Ratjen F, Brumback L, Daniel S, Rowbotham R, McNamara S, et al. Inhaled Hypertonic Saline in Infants and Children Younger Than 6 Years with Cystic Fibrosis: The ISIS Randomized Controlled Trial. JAMA 2012;307(21):2269–77.
Stahl M, Wielpütz MO, Ricklefs I, Dopfer C, Barth S, Schlegtendal A, et al. Preventive Inhalation of Hypertonic Saline in Infants with Cystic Fibrosis (PRESIS). A Randomized, Double-Blind, Controlled Study. Am J Respir Crit Care Med 2019;199(10):1238–48.
Zhang L, Mendoza-Sassi RA, Wainwright C, Klassen TP. Nebulised hypertonic saline solution for acute bronchiolitis in infants. Cochrane Database Syst Rev 2017;12:CD006458.
Smith JJ, Travis SM, Greenberg EP, Welsh MJ. Cystic Fibrosis Airway Epithelia Fail to Kill Bacteria Because of Abnormal Airway Surface Fluid. Cell 1996;85(2):229–36.
Goldman MJ, Anderson GM, Stolzenberg ED, Kari UP, Zasloff M, Wilson JM. Human β-Defensin-1 Is a Salt-Sensitive Antibiotic in Lung That Is Inactivated in Cystic Fibrosis. Cell 1997;88(4):553–60.
Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.
USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.
AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.