ABSTRACT
Cystic Fibrosis (CF) is a recessive autosomic genetic disease with an incidence in mediterranean countries of about 1:3500 born alive. In Italy the considerable genetic variability makes it difficult to identify all the homozygous subjects and, consequently, to estimate the incidence of the disease in healthy carriers. The disease is evolutive and affects various systems, most of all the respiratory and gastrointestinal systems. Not many years ago, when the clinical definition of CF was first introduced, average survival did not exceed the pediatric age. Nowadays with ever advancing diagnostic and therapeutical techniques many CF patients survive until an adult age. It is therefore necessary to plan adequate health service interventions so as to satisfy as much as possible the needs of both the patients and their families. To this end data collected since 1.1.1988 by the Italian registry for CF (year of birth, sex, region of birth and residence, diagnosis procedures, results of sweat test, pancreatic insufficiency, DNA analysis, status: alive, dead, lost to follow up) of all the patients, diagnosed in the 18 Reference Centres and the 3 local Centres for CF, have proved to be extremely useful. Since the birth of the Registry on 31.12.1997, data relating to 2458 patients alive on 1.1.1988 and 1159 born during the last ten years, for a total of 3617 subjects (1756 females and 1861 males), have been recorded. As already mentioned a considerable increase in life expectancy of CF patients (from 1988 to 1990 the average age of death was 14 years, from 1994 to 1997 it was 19) and a consequent increase in the percentage of adult patients have been observed.
Subject(s)
Cystic Fibrosis/epidemiology , Registries , Adolescent , Adult , Age Distribution , Age of Onset , Child, Preschool , Cystic Fibrosis/diagnosis , Cystic Fibrosis/genetics , Female , Humans , Infant , Infant, Newborn , Italy/epidemiology , Male , Time FactorsABSTRACT
Forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1.0), forced expiratory flow between 25 and 75% of FVC (FEF25-75), and maximal expiratory flow at 50% of FVC (MEF50) were measured in 1,880 school children who lived in urban areas before and after a decrease of air pollution. A group of 162 children from a suburban area served as controls. In the first survey, FEV1.0, FEF25-75, and MEF50 of children from urban areas were significantly lower, while in the second survey they were not significantly different from those of controls. The slopes over time of FEV1.0, FEF25-75, and MEF50, adjusted for sex and anthropometric variables, were closely related to the decrease of pollutants concentration. Our results suggest that a decrease of air pollution may produce an improvement of lung function.
Subject(s)
Air Pollutants/poisoning , Lung/drug effects , Adolescent , Air Pollutants/analysis , Child , Cross-Sectional Studies , Forced Expiratory Volume , Humans , Italy , Maximal Expiratory Flow Rate , Maximal Midexpiratory Flow Rate , Suburban Population , Sulfur Dioxide/analysis , Urban Population , Vital CapacityABSTRACT
The duration of action of procaterol, a beta-2-adrenoceptor agonist with an entirely new chemical structure, was assessed, in comparison to that of salbutamol, by inhalation of 43 inhalation units of methacholine at time intervals of 1, 3, 5, 7, and 9 hours after intake of the drugs. Procaterol and salbutamol were given in a double-blind random fashion, on different days, to 12 asthmatic children; pulmonary function tests (FVC, FEV1, MEF50, MEF25), skeletal muscle tremor, and vital signs (heart rate and blood pressure) were measured. Although there was no statistical significant difference between procaterol and salbutamol, protection of large and medium airways lasted for about five hours, while normal small airway patency was still present at seven hours. It is concluded that procaterol can be a good alternative beta-2-adrenoceptor agonist when the oral route is needed.
Subject(s)
Adrenergic beta-Agonists/pharmacology , Airway Resistance/drug effects , Ethanolamines/pharmacology , Methacholine Compounds/antagonists & inhibitors , Albuterol/pharmacology , Asthma/physiopathology , Child , Female , Humans , Male , Methacholine Chloride , Procaterol , Respiratory Function TestsABSTRACT
To investigate the effects of air pollution on the respiratory health of children, a subject of some controversy, a comparative study was undertaken of 2,385 school children who lived in central urban, peripheral urban, and suburban areas. Daily monitoring of sulphur dioxide and total suspended particle concentrations in all areas showed that pollutant concentrations in central and peripheral urban areas were above commonly accepted safety levels for respiratory health, while concentrations in the suburban area were within acceptable limits. A questionnaire administered to each mother assessed environmental exposure to pollutants in the household, the occurrence of respiratory symptoms as well as lung diseases as diagnosed by a physician, and general information. Children were interviewed about smoking habits and any acute respiratory symptoms. Children also performed standard lung function tests. Results showed that children from both urban areas had lessened pulmonary function and a higher prevalence of bronchial secretion with common colds than did those from the suburban area. These differences persisted after corrections for exposure to indoor pollutants, active or passive smoking, socioeconomic status, and sex. Parental cigarette smoking was related to a fall in forced expiratory volume in 1 second and an increased incidence of acute respiratory illnesses and chronic cough in children. Although boys had higher lung volumes and lower air flow, regression analysis showed no significant influence of the interactions "sex-geographic area" and "sex-smoking" on lung function. It was concluded that air pollution has a significant effect on the respiratory health of children.
Subject(s)
Air Pollutants/adverse effects , Lung Diseases/etiology , Child , Cooking , Cross-Sectional Studies , Environmental Monitoring , Female , Humans , Italy , Male , Respiratory Function Tests , Seasons , Smoking , Socioeconomic Factors , Suburban Population , Sulfur Dioxide/adverse effects , Tobacco Smoke Pollution/adverse effects , Urban PopulationABSTRACT
Fasting serum pepsinogen 1 level was determined by radioimmunoassay in 30 patients with cystic fibrosis and in on equal number of age-matched healthy children. Serum pepsinogen 1 basal levels were significantly higher (49 +/- 18 ng/ml; p less than 0.01) in cystic fibrosis patients than in controls (38 +/- 12 ng/ml). The increased pepsinogen 1 level did not correlate with the levels of Po2 and Pco2 tensions, fat balance or basal and maximum acid outputs. It is concluded that the mechanisms which lead to a rise of serum pepsinogen 1 in cystic fibrosis are unknown. However these findings could provide useful information with regard to the biology of a disease whose physiopathology is still unknown.
Subject(s)
Cystic Fibrosis/enzymology , Pepsinogens/blood , Adolescent , Child , Child, Preschool , Humans , Infant , RadioimmunoassayABSTRACT
Two different nebulizers, the DeVilbiss 646 and the Mefar were tested for differences in output characteristics (air flow rate, amount of nebulized liquid and particle size) and it was found that there is a high variability between nebulizers of both brands. In the light of these findings, neither of these devices can be chosen as a standard nebulizer for bronchial provocation.
Subject(s)
Aerosols/standards , Bronchial Provocation Tests/instrumentationABSTRACT
Respiratory function (FVC, FEV1, FMF and FEF200-1200) was measured before and after nebulized distilled water in 68 subjects divided in the five following groups: a) 21 healthy children (age range 7 to 15 years); b) 10 children with asthma (age range 6 to 11 years); c) 10 children with cystic fibrosis (age from 5 to 15 years); d) 7 children with different diseases of the respiratory system (age range from 6 to 11 years); e) 20 healthy adults (aged from 18 to 33 years). The only statistically significant changes were noted in the asthmatic children group. We conclude that a specifically induced bronchoconstriction by nebulized distilled water is a good mean of assessing bronchial lability.
