PubMed Health⌕ Search

Biomedical subjects

F Fanfulla

Publications and source records attributed to F Fanfulla.

31 records · Page 2Linked to original sources

Pulmonary function in children with systemic lupus erythematosus.

BACKGROUND: Abnormalities of pulmonary function have been found in children with systemic lupus erythematosus (SLE) even in the absence of clinical or radiographic evidence of pulmonary involvement. It is unknown whether these abnormalities represent an early sign of progressive lung disease or whether they are associated with disease activity. METHODS: After a mean of 4.5 years, respiratory function (forced vital capacity (FVC) and single breath gas transfer factor (TLCO)) and disease activity were reexamined in 13 of 15 previously studied children with SLE. Disease activity was assessed by a validated index of SLE activity (SLE activity measure (SLAM)). RESULTS: In spite of the high prevalence of abnormalities of respiratory function at the baseline investigation, no chest radiographic abnormalities or overt clinical signs of lung disease were found at baseline, in the interval between the two investigations, or at the re-evaluation in any patient. From baseline to the second investigation the mean value of SLAM decreased and there was a trend toward an improvement in FVC and TLCO. TLCO was more severely impaired than FVC, being found as an isolated abnormality in a high percentage of patients (45% at baseline and 35% at follow up). There was a relationship between baseline TLCO and disease activity, expressed as a SLAM score. Moreover, there was a correlation between the changes in the SLAM score from baseline to the second investigation and the corresponding changes in the TLCO value, but not with the corresponding changes in the FVC value. CONCLUSIONS: In this series of patients the decrease in SLE activity from the first to the second investigation was associated with an improvement in pulmonary function. The presence of early isolated functional abnormalities was not associated with subsequent development of lung disease.

Adolescent↗

Evaluation of methacholine dose-response curves by linear and exponential mathematical models: goodness-of-fit and validity of extrapolation.

Several models have been proposed to analyse dose-response curves recorded in bronchoprovocation challenge tests. The aims of the present work were: 1) to investigate which model (linear vs exponential) and which minimization method (trials and errors vs Levenberg-Marquardt) gives better results in terms of data interpolation (goodness-of-fit); and 2) to verify the validity of extrapolation by comparing forced expiratory volume in one second (FEV1) observed after 4 mg methacholine with values extrapolated after truncation of the curves at 2 mg. For these purposes, methacholine dose-response curves were obtained in 832 subjects from a random population sample, as part of the European Community Respiratory Health Survey (ECRHS) in Italy. Methacholine was inhaled up to a maximum dose of 6 mg by dosimeter technique. The coefficient of determination (r2) was significantly higher with the exponential model (0.81 +/- 0.22; mean +/- SD) than with the linear model (0.69 +/- 0.27). With both models, extrapolated values were usually lower than observed values. As a consequence, a 20% fall in FEV1 with respect to postsaline FEV1 was observed in only 24% and 21% of the tests, where a 20% fall had been predicted, respectively, according to the linear and exponential model. In conclusion, exponential models are better than linear models with respect to data interpolation of methacholine dose-response curves. However, they are worse with respect to extrapolation to higher doses. With any model, extrapolation of dose-response curves by one doubling-dose should be avoided.

Adult↗

Breathing during sleep in myasthenia gravis.

The aim of this study was to assess breathing patterns during sleep in patients with generalized mild myasthenia gravis. Fourteen patients (13 F; 1M, age range 20-40 years) in a stable clinical and functional state underwent a sleep questionnaire, baseline respiratory function tests and standard nocturnal polysomnography. All of the patients had normal daytime blood-gas values, except one who showed mild hypoxemia. No patient complained of disturbed sleep; six patients reported snoring. In five patients nocturnal polysomnography showed the occurrence of short and infrequent central apneas mainly during REM sleep, together with a drop in HbSaO2 levels of more than 5% of the baseline wakefulness value. Our data indicate that, in patients with mild generalized MG in a stable functional state, breathing pattern instability during sleep is infrequent and, when it occurs, is mild and mainly related to REM sleep.

Adult↗

Airway reactivity in parents of infants and young children with recurrent wheeze: a case-control study.

Increased airway reactivity has been found in family members of school age children and adults with asthma. As the relation between recurrent wheeze in infancy and bronchial reactivity is not yet clear, it was decided to test bronchial reactivity to methacholine in both parents of 50 preschool age children with recurrent wheeze and in 200 population based controls matched for sex, age, smoking habits, and atopy. Wheezy children fulfilled the following criteria: first attack of wheezing before the age of 2 years, at least four wheezing episodes triggered by a respiratory infection, negative skin prick tests, and no symptoms related to allergy. Four parents and five controls did not undergo the methacholine challenge because their forced expiratory volume in one second was < 80% of the predicted value. Methacholine reactivity was not significantly different in parents and controls. In summary, an increased bronchial responsiveness was not found in parents of infants and young children with recurrent wheeze triggered by infection.

Adult↗

Reference values of arterial oxygen tension in the middle-aged and elderly.

The lack of available reference values of arterial PO2, particularly for elderly persons, led us to study a sample of 194 normal nonsmoking subjects, equally distributed over all age ranges from 40 to 90 yr. The radial artery was punctured and blood samples were taken and analyzed on an automated, computerized gas-analyzer. The trend of the mean values of PaO2 in the 5-yr class intervals of age showed a clear decline up to the 70- to 74-yr class, and then an inversion. The two regression lines intersecting at this point provided a better fit to the data than did a single regression line (R22 - R12 = 0.918 - 0.678 = 0.24; F = 20.49, p = 0.0027). The relationship of PaO2 with age was thus subsequently considered for the two subgroups (40 to 74 yr; > or = 75 yr) identified on the basis of this cutoff. Because of the significant influence on Pao2 of age, body-mass index (BMI), and PaCO2 in the group 40 to 74 yr of age, the following reference equation was constructed: Pao2 (mm Hg) = 143.6 - (0.39 . age) - (0.56 . BMI) - (0.57 . PaCO2); R2 = 0.28; SEE = 7.48; p < 0.0001. For subjects > or = 75 yr old, for whom there was no correlation with age, BMI, or PaCO2, only the mean +/- SD and 5th percentile of PaO2 were reported (83.4 +/- 9.15 mm Hg and 68.4 mm Hg, respectively). PaCO2 values were not correlated with either age or BMI; the mean +/- SD was 35.79 +/- 3.87 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Chronic respiratory symptoms, bronchial responsiveness and dietary sodium and potassium: a population-based study.

A possible relationship between Na+ intake and increased prevalence and mortality from asthma and chronic obstructive pulmonary disease (COPD) has been suggested but not clearly proven for several reasons (difficulty in assessing Na+ and K+ both by 24 h excretion and dietary recall, too small an effect of these ions on the pathology, and the role of potential confounders). We wanted to determine the relationship of Na+ and K+ intake, assessed by means of a 7 day recall, with chronic respiratory symptoms and bronchial responsiveness in a sample of the general population. Two hundred and five subjects were studied, with complete dietary and respiratory questionnaires, and baseline respiratory function tests, together with a subsample of 146 subjects who underwent histamine challenge. The 7 day recall consisted of two parts: the first assessed discretionary Na+; and the second assessed Na+ and K+ contained in food. The whole sample was split into two groups based on the levels of consumption, and the statistical analysis was performed contrasting the three lower quartiles vs the highest. Smoking habit, social economic status, age and body mass index (BMI) were not confounders for Na+ and K+ intake. The prevalence of symptomatic subjects and baseline respiratory function values were not significantly different in the two groups of quartiles for Na+ and K+. Baseline respiratory values and dose-response slope of the subsample were also not significantly different. We did not prove a relationship between these dietary factors and either bronchial responsiveness or chronic respiratory symptoms. Although we consider that our questionnaire is more reliable than other methods for Na+ and K+ assessment, several potential biases still remain.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Sleep related breathing patterns in patients with spinal muscular atrophy.

A clinical and polygraphic study of nocturnal sleep was performed in 8 (4 males, 4 females; age range 10-37 years) patients with spinal muscular atrophy, whose baseline respiratory function assessment during wakefulness showed restrictive ventilatory syndrome but blood-gas tension within normal limits. No patient reported any significant sleep complaint suggestive of sleep-disordered breathing. However, in 4 patients HbSaO2 desaturations below 90% (HbSaO2 nadir 68%) were detected during nocturnal polysomnography. The HbSaO2 desaturations occurred during brief central apneas or hypopneas, mainly during REM sleep, the apnoea hypopnea index being within normal limits in all cases. The data suggest that nocturnal polysomnography can detect otherwise clinically silent hypoxemia in SMA patients without any predisposing factor to sleep-disordered breathing other than their illness and still showing normal blood-gas tensions during wakefulness. Further studies are needed to determine the long-term evolution and the prognostic significance of nocturnal hypoxemia in these patients.

Adolescent↗

Sleep disorders in neuromuscular diseases.

This article reports the results of several studies on neuromuscular patients (9 Duchenne's muscular dystrophy, 10 myotonic muscular dystrophy, 8 mitochondrial myopathies, 6 spinal muscular atrophy). An evaluation of respiratory function during wakefulness, a sleep questionnaire and an overnight polysomnography were performed in all patients. Recurrent hypoxaemia of variable degree was observed during sleep. In most cases, night-time hypoxaemia appears not to be predictable during wakefulness. Nocturnal hypoxaemia occurs in relation to apnoeas or hypopnoeas, mainly of central type, especially when these breathing irregularities occur during rapid eye movement (REM) sleep. Moreover, polygraphic sleep-apnoea patterns, as defined by international criteria, seem to be an infrequent condition, except for those neuromuscular diseases, characterized by an involvement of the central nervous system.

Adolescent↗

Respiratory function in precapillary pulmonary hypertension.

Since dyspnoea on exertion is very often the first symptom of precapillary pulmonary hypertension (PPH), either from chronic thromboembolic pulmonary hypertension (CTEPH) or from idiopathic pulmonary hypertension (IPH), these patients are often first examined in a pulmonary function laboratory. We carried out a retrospective study (1987-1992) on pulmonary function in 34 patients diagnosed to have PPH by means of specific diagnostic tools, out of 5,467 patients first attending our laboratory. Nine suffered from IPH, 10 from CTEPH and 15 from Eisenmenger physiology. This last group differed from the others, since its diagnosis had been known for a long time and the stage of the disease was more advanced, when pulmonary function tests were performed in our laboratory (with a view to transplantation). Respiratory function, blood gases and arterial oxyhaemoglobin saturation (HbSaO2) during exercise (Bruce protocol), diffusing capacity of the lungs for carbon monoxide (DLCO), shunt fraction (QS%) (approximation obtained from arterial oxygen tension (PaO2) after 100% oxygen breathing) had been evaluated. In the first two groups, in contrast to other reports, we could observe no obstructive defect. Only 20% of the subjects had restrictive defects, however mild. The typical functional picture of these patients revealed normal lung volumes, normal or slightly reduced DLCO, mild hypoxaemia with hypocapnia, severe HbSaO2 drops during exercise, and pathological QS%. We conclude that every time a patient presents with breathlessness at rest or on exercise, a normal chest X-ray and respiratory function tests, pulmonary hypertension must be suspected and subject to specific and invasive tests. More severe functional impairment was observed in the PPH from the Eisenmenger disorder. This might be due to a more advanced stage of this type of hypertension at the time of our observation and/or to the different mechanisms of the diseases themselves.

Adult↗

Reference values of maximal respiratory mouth pressures: a population-based study.

The aim of our study was to measure values of maximal inspiratory (MIP) and expiratory (MEP) mouth pressures in 625 (266 male, 359 female) clinically and functionally normal subjects drawn out of a sample representative of the general population. MEP (near TLC and FRC) was found to be significantly higher when compared with MIP (near RV and FRC), and pressures in male subjects were significantly higher than those in female subjects. MEP values at TLC and FRC were found to be closely related, as were values of MIP near RV and near FRC. Among the tested body-size variables, body surface area (BSA) for all parameters had the highest degree of correlation. Stepwise linear regression analysis was performed to define the equation of normality for all four parameters, employing BSA, sex, age, and relative interaction terms. R2 values, although the variables employed for the equations were highly significant, were relatively low and didn't fully explain the source of variability. The influence of age was smaller than the influence of BSA, although age did reduce the unexplained variance in MEP and MIP. These results confirm that the most useful employment of MIP and MEP is to monitor their changes in each patient, but they point out, however, the usefulness of reliable reference equations.

Adolescent↗

Pulmonary function in childhood connective tissue diseases.

The term connective tissue diseases (CTD) defines a group of illnesses characterized by the presence of immune abnormalities and by widespread inflammation involving various organs and tissues including the lung. These diseases are not frequent in the paediatric age group. Very few data on pulmonary function are available in paediatric CTD. We investigated possible early lung function abnormalities and any likely relationship with clinical activity of the disease in a group of 81 paediatric CTD patients, without clinical or radiological evidence of pulmonary involvement. Measurement of lung volumes and diffusion lung capacity were performed. A sample of 65 subjects, defined as normal on the basis of history and clinical examination, and matched by age and height with the group of patients, was chosen as control group. CTD patients did not show significant deviations from the control distribution with respect to functional residual capacity (FRC) and maximal expiratory flow at 75% of the forced vital capacity (MEF75) values. On the contrary, both vital capacity (VC) and diffusing capacity of the lungs for carbon monoxide (DLCO) were quite impaired in most CTD during the active phase of the disease. Our results show a functional lung impairment in most children with clinically active CTD, even in absence of abnormalities on chest X-ray pictures.

Adolescent↗

[Cigarette smoke and bronchial hypersensitivity].

Among intrinsic factors which may affect the onset of COPD, bronchial non specific hyperresponsiveness seems to play an important, even though, ill-defined role. Attention is drawn to 2 major points: 1) Cigarette smoking habit may increase airway responsiveness? 2) Any possible hyperresponsiveness induced by smoking may be relevant in the development of COPD? At present, only point 1 may be positively answered; point 2 will be clarified by means of perspective and long-term surveys, we have not achieved yet. Our cross-sectional study showed a significant influence of smoking on bronchial responsiveness in absolutely asymptomatic subjects and with airway caliber absolutely in the normal range. This kind of influence resulted to be dose-dependent. Furthermore a noxious role of smoking has been observed, the greater effect the higher amount of cigarettes/day smoked. Moreover this acute role of smoking has been remarked by the trend of bronchial responsiveness of past-smokers, the more similar to non smoker's, the more far was smoking cessation.

Humans↗