Fatigue in cystic fibrosis: a novel prospective study investigating subjective and objective factors associated with fatigue.

Fatigue is a debilitating symptom in patients with cystic fibrosis (CF). Although fatigue is commonly reported in these patients, an effective treatment for this symptom has not been found. The factors associated with fatigue in CF have not been investigated. We conducted a prospective, case-control study in adult patients with CF. All the patients were chronically infected with Pseudomonas aeruginosa and were enrolled in the study during disease stability. A gender and age-matched control group was also recruited. Subjective assessment included three questionnaires: the Chalder fatigue questionnaire, St Mary's Hospital sleep questionnaire (SQ), and the scaled general health and Hillier questionnaire (GHQ). For patients with CF, spirometry, body mass index (BMI), haemoglobin level, C-reactive protein, and the burden of pulmonary exacerbations (PExs) were assessed. The control group completed all the three questionnaires, and their BMI was measured. A total of 78 participants were enrolled in the study (44 patients with CF and 34 control). Female patients with CF received antibiotics for more days than male patients with CF. The fatigue score did not differ between female and male participants in either the patients with CF or the control group; however, the fatigue score was greater for both the sexes in the patients with CF compared with the control group: p = 0.038 for female and p = 0.048 for male. The scores for the SQ and the GHQ did not differ between the two study groups. The fatigue score correlated with the total score for SQ (p < 0.0001) in patients with CF, but not in control participants. In patients with CF and the individuals in the control group, a close correlation was found between the fatigue score and the GHQ domain-specific scores and with the total score; p < 0.0001 for patients with CF and p = 0.001 for control. No correlations were found between the fatigue score and any of the objective parameters studied.

A novel respiratory symptom scoring system for CF pulmonary exacerbations.

BACKGROUND: There is currently no simple scoring system to evaluate change in symptoms during a pulmonary exacerbation (PEx) in adult cystic fibrosis (CF) patients. PATIENTS AND METHODS: We evaluated 265 episodes in 58 adult CF patients. A simple symptom score was administered at the start and the end of each PEx. The score evaluated four symptoms: cough, sputum, breathlessness and fatigue. Each symptom was scored from one (mild symptoms) to four (severe symptoms). The total symptom score was the summation of all the four symptoms. The total symptom score was compared with CF Respiratory Questionnaire (CFRQ) and with spirometry. RESULTS: There was significant internal correlation between scores for each pair of symptoms. The total symptom score correlated with the functional activity score and the respiratory score domains and with the summary score for CFRQ. The total symptom score correlated with spirometry values. Symptom score improved after 2-week treatment with intravenous (IV) antibiotics in 88.3%, remained unchanged in 7.3% and worsened in 4.4% of all episodes. Changes in symptom score after IV treatment correlated with changes of all main spirometry measurements. CONCLUSION: This new symptom score is simple and sensitive to change over a short period. It correlates with established quality-of-life questionnaires and with spirometry. The changes of symptom score over a short period correlate with changes in spirometry. This score can be used as an added tool to assess the outcome of CF PExs.

MRI appearance of the pancreas in patients with cystic fibrosis: a comparison of pancreas volume in diabetic and non-diabetic patients.

We investigated differences in the volume of the pancreas in cystic fibrosis (CF) patients with and without diabetes using MRI to study the natural history of CF-related diabetes (CFRD). We investigated 29 pancreas-insufficient adult CF patients, 13 with CFRD and 16 without diabetes. Patients with CFRD were receiving insulin therapy at the time of study. None of the non-diabetic CF patients had evidence of impaired glucose tolerance. Pancreas volume was estimated by MRI scans using T1 weighted fat-suppression sequences and assessed by an examiner who was unaware of the patients' diabetes status. Pancreas volume of CF patients was measured and subsequently compared with that of non-CF age-matched Type 1 diabetes (T1DM) patients and healthy controls previously investigated. The two CF groups were matched for age and gender. There were no differences in spirometry values, body mass index or pancreatic exocrine function. The pancreas was visible by MRI in only 3 of 13 (23.1%) patients with CFRD and in 5 of 16 (31.3%) patients without diabetes (p-value = 0.7). In total, the pancreas was not detected by MRI as an anatomical entity in 21 of 29 (72.4%) CF patients, irrespective of their diabetes status. When comparing the four study groups, the pancreas was significantly smaller in CF patients than in T1DM patients and healthy controls.

The size of the spleen by magnetic resonance imaging in patients with cystic fibrosis; with and without diabetes--a novel observational study.

BACKGROUND: Magnetic resonance imaging (MRI) has been shown to be a useful tool to evaluate the volume of the pancreas. There is currently no information about the size of the spleen in cystic fibrosis (CF) patients. PATIENTS AND METHODS: We investigated 51 adult volunteers: 28 pancreatic insufficient CF patients [13 with CF-related diabetes (CFRD) and 15 non-diabetic] and 23 male non-CF patients [12 with type 1 diabetes mellitus (T1DM) and 11 healthy control subjects]. Patients with known liver cirrhosis or portal hypertension were excluded. The size of the spleen was measured in all subjects by an investigator unaware of patients' clinical status. For comparison of spleen size in the four study groups only male CF patients were included. For CF patients, spleen size was compared with forced expiratory volume in 1 s (FEV(1)), body mass index (BMI), total number of days of intravenous (IV) antibiotic treatment for pulmonary exacerbations in year previous to study, levels of circulating white blood cells, glycosylated haemoglobin A1c (HbA1c), and exocrine function of the pancreas, as assessed by daily requirement of oral lipase. RESULTS: Amongst the four study groups, spleen size was greatest in the male non-diabetic CF patients (P = 0.01). For CF patients, spleen size was greater in male compared to female patients (P = 0.012). For patients with CFRD, there was an inverse correlation between the spleen size and HbA1c (r = -0.59, P = 0.04) and the daily intake of supplementary lipase (r = -0.63, P = 0.02). The size of the spleen in patients with CFRD, but not in CF patients without CFRD, inversely correlated with the days of IV antibiotic treatment received in the year previous to the study (r = -0.67, P = 0.012). There was no correlation between spleen size and BMI, FEV1 and white blood cell counts in any group. CONCLUSION: On MRI, the spleen size was greatest in male non-diabetic CF patients in comparison with other groups. The size of the spleen in CFRD patients was smaller when diabetes was poorly controlled, when exocrine pancreatic function was greatly impaired and in those with greater need for IV antibiotics in the year prior to the study.

Prognostic factors to predict outcome following the administration of hypertonic/hyperoncotic solution in hypovolemic patients.

Hypertonic solutions effectively improve hemodynamic parameters in patients admitted to the emergency room. However, no significant differences in outcome were observed compared with standard isotonic treatment in most previously published studies. This study evaluates pretreatment prognostic factors that predict a beneficial effect of hypertonic solution in patients admitted to the emergency room with hemorrhagic hypovolemia in a prospective double-blind fashion. The patients (n = 212) were randomized upon admission to receive 250 mL intravenous (i.v.) bolus of hypertonic 7.5% NaCl + 6% dextran (HSD, n = 101), or isotonic 0.9% NaCl solutions (IS, n = 111) as the first treatment, followed by standard resuscitation. Pretreatment factors assessed were sex, age, cause of hypovolemia, revised trauma score (RTS), Glasgow index, and mean arterial pressure (MAP) on admission. Both groups were compared for survival at 24 h and 30 days postadmission. Infused volumes were registered. HSD administration significantly increased MAP and reduced i.v. crystalloid infusions to maintain hemodynamic parameters, compared with IS. There was no difference between groups in the number of blood transfusions administered. Overall complication rates in both groups were similar (24%). There was a significant difference (p < .03) in overall (30 days) survival rate between HSD (73%) and IS (64%) groups. The 24 h survival rate was significantly lower in IS (72%) compared with HSD (87%); p < .01. Multivariate analyses showed that RTS and MAP were identified as independent predictors for 24 h survival in the group that received HSD. When evaluated for overall survival rate, hypertonic infusion benefited significantly only patients with MAP < 70 mmHg (p < .01).