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Impact of the A (H1N1) pandemic influenza (season 2009-2010) on patients with cystic fibrosis.
No full textAssael BM, Barreto C, Bentur L, Bhatt JM, Repetto T, Chipps B, Colombo C, De Boeck K, Derelle J, Desager KN, Salvatore D, Escribano A, Kerem E, Lebecque P, Lischka A, Lucidi V, Malfroot A, Minicucci L, Munck A, Padoan R, Pardo F, Collura M, Raia V, Lutz N, Van Daele S, Vazquez C.
Source
Dipartimento di Medicina del Lavoro Clinica del Lavoro L. Devoto, Sezione di Statistica Medica e Biometria G.A.Maccacaro, Università degli Studi di Milano, Italy.
Abstract
BACKGROUND:
Influenza, like other respiratory viral infections, can cause acute deterioration of lung function in patients with cystic fibrosis (CF). Previous studies on a small number of patients reported that most people with CF infected with A (H1N1) influenza experienced a mild course of disease.
AIM:
To characterise the impact of A (H1N1) infection on CF in a large number of patients from different centres and countries.
METHODS:
CF centres accessing the web-site of the European Cystic Fibrosis Society (ECFS) were asked to report clinical data on patients with an ascertained diagnosis of influenza caused by the A (H1N1) virus. The study was web-based and data were collected through an electronic data sheet on the ECFS website.
RESULTS:
Twenty-five centres from 10 countries caring for 4698 patients with CF reported data on 110 patients (2.3%), median age 13 years (range 1-39 years). The prevalence of infection in each centre ranged from 0% to 9.4%. Only 8.8% of the patients had been vaccinated. The main symptoms were fever and respiratory exacerbation requiring IV antibiotics in 53% of the patients; 48% of the patients were hospitalised for an average of 12.9 days (range 2-56) and 31% required oxygen treatment during the time of the infection. Most of the patients recovered and FEV(1) 1 month after the infection was similar to that before the infection. However, 6 patients were admitted to ICU, 5 with mechanical ventilation. Three patients with severe respiratory disease died.
CONCLUSIONS:
A (H1N1) influenza infection caused transient but significant morbidity in most of the patients with CF. However, in a small number of patients with severe lung disease, A (H1N1) influenza was associated with respiratory deterioration, mechanical ventilation and even death
Impact of the A (H1N1) pandemic influenza (season 2009-2010) on patients with cystic fibrosis.
No full textBACKGROUND:
Influenza, like other respiratory viral infections, can cause acute deterioration of lung function in patients with cystic fibrosis (CF). Previous studies on a small number of patients reported that most people with CF infected with A (H1N1) influenza experienced a mild course of disease.
AIM:
To characterise the impact of A (H1N1) infection on CF in a large number of patients from different centres and countries.
METHODS:
CF centres accessing the web-site of the European Cystic Fibrosis Society (ECFS) were asked to report clinical data on patients with an ascertained diagnosis of influenza caused by the A (H1N1) virus. The study was web-based and data were collected through an electronic data sheet on the ECFS website.
RESULTS:
Twenty-five centres from 10 countries caring for 4698 patients with CF reported data on 110 patients (2.3%), median age 13 years (range 1-39 years). The prevalence of infection in each centre ranged from 0% to 9.4%. Only 8.8% of the patients had been vaccinated. The main symptoms were fever and respiratory exacerbation requiring IV antibiotics in 53% of the patients; 48% of the patients were hospitalised for an average of 12.9 days (range 2-56) and 31% required oxygen treatment during the time of the infection. Most of the patients recovered and FEV(1) 1 month after the infection was similar to that before the infection. However, 6 patients were admitted to ICU, 5 with mechanical ventilation. Three patients with severe respiratory disease died.
CONCLUSIONS:
A (H1N1) influenza infection caused transient but significant morbidity in most of the patients with CF. However, in a small number of patients with severe lung disease, A (H1N1) influenza was associated with respiratory deterioration, mechanical ventilation and even death
Cystic fibrosis carriers have higher neonatal immunoreactive trypsinogen values than non-carriers
No full textFollowing cystic fibrosis (CF) neonatal screening implementation, a high frequency of heterozygotes has been reported among neonates with elevated immunoreactive trypsinogen (IRT) and normal sweat chloride levels. We studied the relationship between normal IRT values and CF heterozygosity: 10,000 neonates were screened for CF by IRT measurement and tested for 40 CF mutations; the 294 carriers detected were coupled with newborns negative to the same genetic testing, and the two groups' IRT levels compared. Heterozygotes had higher IRT levels than their controls (mean 35.32 vs. 27.58 microg/L, P<0.001). Even within normal trypsinogen range, the probability of being a CF carrier increases with neonatal IRT concentration
Biodistribution and transgene expression with nonviral cationic vector/DNA complexes in the lungs
No full text
Interaction of adenovirus type 5 fiber with the coxsackievirus and adenovirus receptor activates inflammatory response in human respiratory cells
No full textThe innate immune response to adenovirus (Ad)-derived gene transfer vectors has been shown to initiate immediately after interaction of Ad with respiratory epithelial cells, through the induction of extracellular signal-regulated kinase 1 and 2 (ERK1/2) and JNK mitogen-activated protein kinase (MAPK), nuclear factor κB (NF-κB), and different proinflammatory genes. Ad serotypes 2 or 5 (Ad2/5) enter respiratory epithelia after initial binding of fiber with the coxsackievirus-adenovirus receptor (CAR) or, alternatively, with cell surface heparan sulfate glycosaminoglycans. Ad2/5 internalization is triggered by binding of penton base to cellular RGB-binding integrins. Here we investigated the role of the Ad5 surface domain proteins constituting the vector capsid, namely, the fiber, the penton base, and the hexon, on the transmembrane signals leading to the transcription of the different proinflammatory genes in the human respiratory A549 cell line. Interaction of Ad fiber with CAR activates both ERK1/2 and JNK MAPK and the nuclear translocation of NF-κB, whereas no activation was observed after exposing A549 cells to penton base and hexon proteins. Moreover, interaction of Ad fiber with CAR, but not heparan sulfate proteoglycans, promotes transcription of the chemokines interleukin-8, GRO-α, GRO-γ, RANTES, and interferon-inducible protein 10. These results identify the binding of Ad5 fiber with the cellular CAR as a key proinflammatory activation event in epithelial respiratory cells that is independent of the transcription of Ad5 genes
SOCIOECONOMIC STATUS AND USE OF CF CARRIER SCREENING
No full textIn northeastern Italy, CF neonatal screening has been performed for
many years, and is regularly used to monitor CF birth prevalence. A significant progressive decrease of CF birth rates has been recorded in a sub-area where a CF Carrier Screening (CS) programme has been implemented (CS area), but not where CS is not in place (NO CS area). In the CS area a negative correlation has been found between CF incidence and the number of carrier tests performed, suggesting that CF CS may be connected with a downward birth prevalence trend
Extensive carrier testing and CF birth prevalence: evidence for a negative correlation
Full text linkAim of the study was to evaluate if extensive CF carrier testing may be connected
with the progressive decrease of CF birth incidence recorded in North Eastern Italy.
From 1993 to 2007 an average 52,000 newborns per year underwent Neonatal
Screening (NS), and 198 newborns with CF were detected (1/3937). A time related
contraction in birth prevalence was confirmed, with an average annual percent
decrease of 0.15 per 10,000 neonates (Poisson regression analysis p 0.003). In
the NS area two sections were identified: the Western Region (WR), where CF
carrier testing is not offered to couples from the general population, and the
Eastern Region (ER), where CF carrier testing is widely offered to couples from
the general population. In ER from 1995 to 2007 such testing practice has been
steadily expanding, with a total of 87,721 CF carrier tests performed, 3460 carriers
identified, and 238 carrier couples detected (data collection in progress). The
prevalence of CF decreased by time (p<0.001) but the rate of decrease was more
enhanced in ER as suggested by the existence of a statistically significant (p = 0.014)
interaction term between time and region in the Poisson regression model. The
overall negative trend in North Eastern Italy is due to a contraction of CF births
in its Eastern part. In ER a negative correlation was found between CF incidence
and the number of carrier tests (p 0.012). Prenatal diagnosis data collection is in
progress. These data support the hypothesis that carrier screening may modify the
incidence of CF
Frequency of large CFTR gene rearrangements in Italian CF patients
No full textIn most populations, an appreciable fraction of cystic fibrosis transmembrane regulator (CFTR) gene mutations in patients affected by cystic fibrosis (CF) cannot be identified, and large gene rearrangements
might be missed by standard analyses. We have searched large gene rearrangements in a sample of 25 North East Italian CF patients who, after an extensive gene analysis of 188 patients, still bear one or two
unidentified CF mutations. A systematic gene screening by quantitative multiplex PCR of short fluorescent fragments was performed. Overall, 5/26 (19.2%) rearranged alleles were detected, bearing mutation 3120þ1Kbdel8.6Kb (three patients), and c.4_IVS1þ69del119bpins299bp (two patients). These mutations were observed in compound heterozygotes with F508del or termination mutations, and a pancreatic insufficient form of CF. These findings confirm the frequency of CFTR gene rearrangements recently observed in French CF patients
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