An overview of international literature from cystic fibrosis registries: 1. Mortality and survival studies in cystic fibrosis.

Patient registries are organized systems of data collection for scientific, clinical or health strategy purposes. Aims of our review were to document scientific literature based on data and information from cystic fibrosis (CF) registries; to understand which clinical problems have been addressed and for which of these the studies concerned have correctly answered the questions raised (i.e. a methodological critique) and to identify clinical issues in need of further investigation. The review included primary studies starting from a formally constituted CF registry of at least national level, using data from the registry to evaluate research hypotheses. This article is an overview of the research undertaken, focusing in detail on the issues of mortality and survival. The studies considered here focused mainly or secondarily on survival in CF, the aim being to ascertain an improving trend, identify any prognostic factors and, in some cases, attempt to provide a predictive model of survival.

[Usefulness of chest X-ray during cystic fibrosis exacerbation in adult patients]. / Utilità del radiogramma del torace negli episodi di riacutizzazione della fibrosi cistica nel paziente adulto.

PURPOSE: Verify the real usefulness of chest X-ray during clinical exacerbation of cystic fibrosis (FC). MATERIAL AND METHODS: The study was based on a group of 46 adult patients affected by FC. For every subject we selected one or more pair of chest films, of which the first was used as reference image (To), whereas the second (T1) was selected among the following controls, either in course of clinical obvious exacerbation, or during another routine control. The 64 pairs of chest films (32 representing phases of clinical stability, 32 documenting evolution towards clinical deterioration) were subjected to evaluation by two radiologists, who were first asked to judge if stability, improvement or worsening of the overall radiographic picture could be observed; then to estimate the improvement, worsening, stability or absence of the 12 radiographic signs, selected among those more frequently correlating with the acute phase of disease. RESULTS: The comprehensive radiological evaluation, which shows a statistically significant difference (p=0.012) between the two groups considered, weakly correlates with the patient clinical status. Among the patients with exacerbation, the radiographic picture appeared worsened in only 18/32 cases (sensibility 56%, specificity 78%), while on the contrary 7/32 clinically stable patients exhibited a radiographic deterioration. Among the considered radiographic-sings, some were exclusively observed during exacerbation (specificity 100%), but with low sensitivity (pneumothorax: 6%, increment of bronchiectasis: 9%, air space disease 22%); the most common alterations (mucoid impactions and bronchial wall thickening) were observed in both groups of patients. DISCUSSION AND CONCLUSIONS: Our results demonstrate that there is not a precise correlation between the radiographic picture and the clinical manifestations of exacerbation and confirm the poor usefulness of chest X-ray in such a phase of disease. Chest X-ray is needed to exclude pnemothorax or extensive air space disease, rather then to accurately diagnose exacerbation.

Circulating adhesion molecules in cystic fibrosis.

A marked influx of inflammatory cells occurs into the airways of patients with cystic fibrosis (CF), which may contribute to the development of lung injury. Leukocyte-endothelial adhesion molecules play a crucial role in the recruitment of inflammatory cells, and soluble forms of these molecules have been shown to increase in several inflammatory diseases. By using a capture ELISA, we determined serum levels of soluble ICAM-1 (sICAM-1), E-selectin (sE-selectin), and VCAM-1 (sVCAM-1) in patients with CF, in stable clinical conditions (n = 29, mean age: 25.8+/-1.5 yr), and healthy control subjects (n = 12, mean age: 27.6+/-1.5 yr). Clinical, spirometric, microbiological, and hematological assessments were made in all subjects. sICAM-1 and sE-selectin concentrations, but not sVCAM-1 levels were significantly increased in CF patients as compared with normal subjects (both p < 0.001). sICAM-1 levels were inversely related to FEV1 values (r = -0.519, p = 0.004) and Schwachman score (r = -0.405, p = 0.03) in CF patients. In 7 of 29 CF patients, soluble adhesion molecule levels were determined not only at the time of stable clinical conditions, but also before and after antibiotic treatment for a pulmonary exacerbation. sICAM-1 and sE-selectin levels increased in all patients at the time of the exacerbation, compared with levels at the time of stable conditions (p < 0.02 for both comparisons); antibiotic treatment induced a significant decrease of both circulating adhesion molecules (p < 0.02). The elevated serum levels of sICAM-1 and sE-selectin in CF patients, even when they are clinically stable, may reflect the marked and persistent inflammatory process in the disease.