BackgroundSaliva and sweat are modified by cystic fibrosis (CF). In both cases the chloride and sodium ion concentrations for healthy subjects and CF patients differ, this representing a possible alternative tool for CF diagnosis. In this context, the aim of this study was to compare the concentrations of these ions in saliva samples taken from CF patients and healthy subjects.MethodsA case–control study was carried out at a university CF center, in which the saliva samples were analyzed on an ABL 835 Radiometer® to determine the ion concentration.ResultsFor the CF patients (n = 80) the values for the biochemical parameters of chloride, potassium and sodium ion concentration were higher (p < 0.009) and the volume and pH of the saliva were lower than in the case of healthy subjects (p < 0.009). For the healthy subjects group (n = 84) versus CF patients, according to the ROC curve, the values for sodium were: cutoff: 13.5 mmol/L, sensitivity: 73.4%, specificity: 70.6%; and for chloride: cutoff: 20 mmol/L, sensitivity: 68.1%, specificity: 72.9%.ConclusionsThe chloride and sodium concentrations in the saliva samples were higher for CF patients in comparison with healthy subjects. Thus, saliva as a tool for CF diagnosis can be considered a new challenge, and a population study including patients in all age classes needs to be performed, in different countries over the world, to extend the database to include a broad spectrum of information in order to identify normal ion concentration ranges for CF patients according to age, genotype and environment.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2614233148750145

Saliva chloride concentration and saliva sodium concentration are candidates to be used in cystic fibrosis diagnosis, mainly in cases where it is difficult to achieve the correct sweat amount, and/or CFTR mutation screening is difficult, and/or reference methods for sweat test are unavailable to implement or are not easily accessible by the general population.

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ObjectiveThis study aimed to determine the glycaemic profile of patients with cystic fibrosis using a continuous glucose monitoring system (CGMS), and to evaluate the associations of glycaemic abnormalities with sex, age, pubertal stage, CFTR gene mutations, nutritional status, lung function, oral glucose tolerance test, glycated haemoglobin concentrations, fasting insulin concentrations, C peptide concentrations and exocrine pancreatic function.Study designThis observational study evaluated CGMS data from 39 patients with cystic fibrosis who were treated at a referral centre. The patients were 10–19.9 years old, and were categorised according to whether they had normal results (27 patients) or glucose intolerance (12 patients) during the oral glucose tolerance test.ResultsThe maximum interstitial glucose concentration among individuals with normal oral glucose tolerance test results was 174.9±65.1 mg/dL (9.7–3.61 mmol/L), compared with 170.4±40.9 mg/dL (9.46–2.27 mmol/L) among individuals with glucose intolerance. The CGMS revealed that 18 of the 27 patients with normal oral glucose tolerance test results had peak interstitial glucose concentrations of >140 mg/dL (7.8 mmol/L), and that 4 of these individuals had peak levels of >200 mg/dL (11.1 mmol/L). None of the analysed clinical or laboratory characteristics predicted the occurrence of hyperglycaemic peaks on CGMS.ConclusionsThe present study revealed that CGMS could detect hyperglycaemia among patients with cystic fibrosis and ‘normal’ oral glucose tolerance test results, and that their clinical and laboratory characteristics were not useful in discerning between patients who did and did not exhibit these excursions.

Objective:To elucidate whether insulin is effective or not in patients with cystic fibrosis before the diabetes mellitus phase.Data source:The study was performed according to the Prisma method between August and September 2014, using the PubMed, Embase, Lilacs and SciELO databases. Prospective studies published in English, Portuguese and Spanish from 2002 to 2014, evaluating the effect of insulin on weight parameters, body mass index and pulmonary function in patients with cystic fibrosis, with a mean age of 17.37 years before the diabetes mellitus phase were included.Data synthesis:Eight articles were identified that included 180 patients undergoing insulin use. Sample size ranged from 4 to 54 patients, with a mean age ranging from 12.4 to 28 years. The type of follow-up, time of insulin use, the dose and implementation schedule were very heterogeneous between studies.Conclusions:There are theoretical reasons to believe that insulin has a beneficial effect in the studied population. The different methods and populations assessed in the studies do not allow us to state whether early insulin therapy should or should not be carried out in patients with cystic fibrosis prior to the diagnosis of diabetes. Therefore, studies with larger samples and insulin use standardization are required.