Electrolyte abnormalities in cystic fibrosis: systematic review of the literature |
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Authors: | Elisabetta Scurati-Manzoni Emilio F. Fossali Carlo Agostoni Enrica Riva Giacomo D. Simonetti Maura Zanolari-Calderari Mario G. Bianchetti Sebastiano A. G. Lava |
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Affiliation: | 1. Pediatric Emergency Unit, De Marchi Hospital, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy 2. Integrated Department of Pediatrics, Ente Ospedaliero Cantonale Ticinese, University of Berne, Bern, Switzerland 3. Second Pediatric Clinic, Clinica De Marchi, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy 4. Department of Pediatrics, San Paolo Hospital, University of Milan, Milan, Italy 5. Pediatric Nephrology, University Children’s Hospital Berne, University of Berne, Bern, Switzerland 6. San Giovanni Hospital, 6500, Bellinzona, Switzerland
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Abstract: |
Background Cystic fibrosis per se can sometimes lead to hyponatremia, hypokalemia, hypochloremia or hyperbicarbonatemia. This tendency was first documented 60 years ago and has subsequently been confirmed in single case reports or small case series, most of which were retrospective. However, this issue has not been addressed analytically. We have therefore systematically reviewed and analyzed the available literature on this subject. Methods This was a systematic review of the literature. Results The reports included in this review cover 172 subacute and 90 chronic cases of electrolyte imbalances in patients with cystic fibrosis. The male:female ratio was 1.57. Electrolyte abnormalities were mostly associated with clinically inapparent fluid volume depletion, mainly affected patients aged ≤2.5 years, frequently tended to recur and often were found before the diagnosis of cystic fibrosis was established. Subacute presentation often included an history of heat exposure, vomiting, excessive sweating and pulmonary infection. History of chronic presentation, in contrast, was often inconspicuous. The tendency to hypochloremia, hypokalemia and metabolic alkalosis was similar between subacute and chronic patients, with hyponatremia being more pronounced (P?0.02) in subacute compared to chronic presentations. Subacute cases were treated parenterally; chronic ones were usually managed with oral salt supplementation. Retention of urea and creatinine was documented in 38 % of subacute cases. Conclusions The findings of our review suggest that physicians should be aware that electrolyte abnormalities can occur both as a presenting and a recurring feature of cystic fibrosis. |
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