A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy

Abstract:
Studies have endeavored to reconcile whether dysfunction of neutrophils in people with cystic fibrosis (CF) is a result of the genetic defect or is secondary due to infection and inflammation. In this study, we illustrate that disrupted function of the CF transmembrane conductance regulator (CFTR), such as that which occurs in patients with ∆F508 […]

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Is there a risk for kidney stone formation in cystic fibrosis?

Abstract:
Patients with malabsorption syndromes have an increased risk of kidney stone formation. Those with cystic fibrosis (CF) suffer from extreme forms of steatorrhea, but they are not reported to be prone to kidney stone formation. Risk parameters for renal stone formation were studied in the urine of 43 patients with cystic fibrosis and compared […]

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Aminoglycoside-associated hypomagnesaemia in children with cystic fibrosis

Abstract:
Hypomagnesaemia in children with cystic fibrosis (CF) is under-recognized. We report a child with CF who developed significant hypomagnesaemia following intravenous (i.v.) treatment with aminoglycosides for exacerbations of Pseudomonas aeruginosa infection. Three additional cases have also been observed. Investigations in two patients have revealed excessive renal loss of magnesium. It is postulated that renal […]

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Hypomagnesaemia in cystic fibrosis patients referred for lung transplant assessment

Abstract:
BACKGROUND: Hypomagnesaemia in patients with cystic fibrosis (CF) is under-recognized although the true incidence is unknown. Many patients are asymptomatic, although severe deficiency may be associated with muscle weakness, cramps and tetany. Hypomagnesaemia may be a risk factor for post-transplant complications including convulsions, which may be exacerbated by the use of calcineurin inhibitors. The […]

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Role of magnesium in the failure of rhDNase therapy in patients with cystic fibrosis

Abstract:
BACKGROUND: In the management of cystic fibrosis (CF), rhDNase-I inhalation is widely used to facilitate the removal of the highly viscous and elastic mucus (often called sputum) from the lungs. However, an important group of CF patients does not benefit from rhDNase-I treatment. A study was undertaken to elucidate the reason for the failure […]

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Magnesium deficiency in cystic fibrosis

Abstract:
We have described the first case of symptomatic magnesium deficiency in a patient with cystic fibrosis and have discussed its causes. Such deficiency probably occurs with significant frequency in cystic fibrosis and should be considered in such patients so that important morbidity can be prevented.

Orenstein SR, Orenstein DM
South. Med. J. 1983 Dec;76(12):1586
PMID: 6648626

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Oral magnesium supplementation in children with cystic fibrosis improves clinical and functional variables: a double-blind, randomized, placebo-controlled crossover trial

Abstract:
BACKGROUND: Magnesium is one of the most important minerals in the body. Although some studies reported that patients with cystic fibrosis (CF) lack magnesium, no international study has assessed the importance of oral magnesium supplementation in CF patients.OBJECTIVE: We prospectively investigated the long-term effect of oral magnesium supplementation on respiratory muscle strength by using […]

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