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1.
J Cyst Fibros ; 17(4): 542-547, 2018 07.
Article in English | MEDLINE | ID: mdl-29254823

ABSTRACT

BACKGROUND: Hypoglycaemia in cystic fibrosis (CF) is known to occur during oral glucose tolerance tests (OGTT) and continuous glucose monitoring, however demographic, clinical and mechanistic data are limited. The aims of this study were to review patient electronic medical records (EMR) in order to 1) describe patient characteristics of a university teaching hospital CF clinic, 2) determine the prevalence of hypoglycaemia on OGTT and explore associations with demographic and clinical characteristics, and 3) explore patient reported symptoms suggestive of hypoglycaemia documented in the EMR. METHODS: Adults who attended the RPA CF clinic between January 2009 to April 2016 were included in the study. The prevalence of hypoglycaemia on OGTT was determined and clinical and demographic data were compared to age, sex and glucose tolerance matched controls. Reported symptoms suggestive of hypoglycaemia documented in EMR were qualitatively explored. RESULTS: Hypoglycaemia on OGTT was prevalent in 25 (3 fasting and 22 reactive) of 169 patients who had an OGTT. They were heavier, less likely to have pancreatic insufficiency and had a lower insulin response at 2-h. Another 14 patients reported symptoms suggestive of hypoglycaemia in their EMR. No patient appropriately suppressed insulin at 2-h on OGTT. CONCLUSIONS: This study identified two potentially different presentations of hypoglycaemia occur in different clinic sub-populations. Knowledge gaps in the aetiology and triggers of hypoglycaemia remain.


Subject(s)
Cystic Fibrosis , Hypoglycemia , Adult , Australia/epidemiology , Cystic Fibrosis/blood , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Cystic Fibrosis/physiopathology , Demography , Female , Glucose Tolerance Test/methods , Humans , Hypoglycemia/diagnosis , Hypoglycemia/epidemiology , Hypoglycemia/etiology , Male , Prevalence , Risk Assessment
2.
N Engl J Med ; 354(3): 229-40, 2006 Jan 19.
Article in English | MEDLINE | ID: mdl-16421364

ABSTRACT

BACKGROUND: Inhaled hypertonic saline acutely increases mucociliary clearance and, in short-term trials, improves lung function in people with cystic fibrosis. We tested the safety and efficacy of inhaled hypertonic saline in a long-term trial. METHODS: In this double-blind, parallel-group trial, 164 patients with stable cystic fibrosis who were at least six years old were randomly assigned to inhale 4 ml of either 7 percent hypertonic saline or 0.9 percent (control) saline twice daily for 48 weeks, with quinine sulfate (0.25 mg per milliliter) added to each solution to mask the taste. A bronchodilator was given before each dose, and other standard therapies were continued during the trial. RESULTS: The primary outcome measure, the rate of change (slope) in lung function (reflected by the forced vital capacity [FVC], forced expiratory volume in one second [FEV1], and forced expiratory flow at 25 to 75 percent of FVC [FEF25-75]) during the 48 weeks of treatment, did not differ significantly between groups (P=0.79). However, the absolute difference in lung function between groups was significant (P=0.03) when averaged across all post-randomization visits in the 48-week treatment period. As compared with the control group, the hypertonic-saline group had significantly higher FVC (by 82 ml; 95 percent confidence interval, 12 to 153) and FEV1 (by 68 ml; 95 percent confidence interval, 3 to 132) values, but similar FEF25-75 values. The hypertonic-saline group also had significantly fewer pulmonary exacerbations (relative reduction, 56 percent; P=0.02) and a significantly higher percentage of patients without exacerbations (76 percent, as compared with 62 percent in the control group; P=0.03). Hypertonic saline was not associated with worsening bacterial infection or inflammation. CONCLUSIONS: Hypertonic saline preceded by a bronchodilator is an inexpensive, safe, and effective additional therapy for patients with cystic fibrosis. (ClinicalTrials.gov number, NCT00271310.)


Subject(s)
Cystic Fibrosis/drug therapy , Saline Solution, Hypertonic/administration & dosage , Absenteeism , Administration, Inhalation , Adolescent , Adult , Albuterol/therapeutic use , Anti-Bacterial Agents/therapeutic use , Bronchodilator Agents/therapeutic use , Child , Cough/etiology , Cystic Fibrosis/physiopathology , Disease-Free Survival , Double-Blind Method , Drug Therapy, Combination , Female , Forced Expiratory Volume/drug effects , Humans , Isotonic Solutions , Male , Maximal Midexpiratory Flow Rate/drug effects , Premedication , Saline Solution, Hypertonic/adverse effects , Sodium Chloride/administration & dosage , Sodium Chloride/adverse effects , Treatment Outcome , Vital Capacity/drug effects
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