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We have conducted a pilot trial of a home telemonitoring system involving 60 adult patients with cystic fibrosis (CF) enrolled at four Italian CF centres. Thirty randomly selected patients received a home device to perform spirometry and to measure oxygen saturation. The data were transferred to a data centre which forwarded them to the patient's CF clinic. The telemonitoring system was evaluated using questionnaires completed by patients and their doctors. The doctors reported that telemonitoring was useful in monitoring their patients' health status and in decreasing the CF centre workload. The data transfer procedure posed no particular problems for the patients. The major difficulties reported by the patients were night-time saturimetry measurements, and in the first week of the study, data transmission to the data centre. The pilot trial was positive and therefore merits a larger study. [ABSTRACT FROM AUTHOR]

Background: The existence of gender-related differences since childhood in survival of cystic fibrosis (CF) patients has been recently challenged., Methods: We evaluated the effect of gender on survival of 2293 CF patients born after 01/01/1988, followed up by 29 CF centres until 31/12/2004 and recorded in the Italian Registry for CF (IRCF)., Results: We observed similar annual mortality rates in females (3.59‰) and males (4.00‰), similar survival curves (log-rank test p=0.64) and similar hazards of death (hazard ratio adjusted for presence of symptoms at diagnosis, meconium ileus, F508del mutation and age at diagnosis: 1.29, 95%CI: 0.60; 2.76). However, excess mortality due to CF was higher for females (5.9) than males (5.1)., Conclusions: In our population CF females do not experience higher mortality than males but, due to the disease, they lose the expected survival advantage occurring in the general population at this age. We do not exclude, however, that differences in mortality will establish after adolescence., (Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Cystic Fibrosis is the most common life-shortening autosomal recessive disease of Caucasian population. The clinical expression of the disease is heterogeneous and the treatment of CF patients requires high qualification of medical and paramedical staff. In 1992 and 1993, in Italy, two national laws (104/92 and 548/93) were enacted in favour of people affected by CF. Data gathered in the Italian Registry for CF (started on 1.1.1988) allow us to monitor changes occurred in the age structure of patients and diagnosis methods. This also thanks to the laws mentioned above. Data here presented refer to 4,209 patients, 1,964 of whom were alive on 1.1.1988, and 2,245 were diagnosed afterwards. A remarkable increase in early diagnosis (within the 3rd month of life) was noticed (from 41% to 65%), together with an increasing percentage of subjects who underwent a neonatal screening program (+34%). An increase in late diagnoses is also apparent: the frequency for patients diagnosed after 18 years of life ranged from 8% to 12%. The proportion of adult patients is more than doubled between 1988 (17%) and 2000 (41%) and the number of patients over 30 is now about 400. 512 patients died during the study period. Median age at death increased from 14 (1988) to 22 (2000) years. This trend can be observed also for patients diagnosed within the first year of life. All these changes have contributed in modifying age structure of CF population. In late 80's, the latter was mostly composed by children, while now 50% of patients are adolescents or adults. Therefore, it is now necessary to involve specialists of adult diseases in CF care.