[Family pediatricians and obstructive sleep disorders]. / Il pediatra di famiglia e i disturbi respiratori ostruttivi nel sonno.

UNLABELLED: BREATHING: Problems related to the Obstructive Sleep Disordered Breathing (OSDB) are so many: 1) a noso- graphic setting has still to be defined and this leads to contrasting results concerning the prevalence of the OSDB; 2) the absence of a single pathogenetic trigger which can explain the sudden increase of the number of cases of the OSDB since the 1980's; 3) a poor integration between clinical and diagnostic tests; 4) a not well defined role of the family pediatrician in approaching the OSDB. OBJECTIVES: From the above introduction we can deduce four objectives of the study: 1) verifying the prevalence of the OSDB; 2) studying if an early development of the adenotonsillar tissues can influence the on-set of the OSDB; 3) a better definition of the clinical diagnosis; 4) knowing what decisions the family pediatrician do take as concerns the diagnostic tests and therapy. MATERIAL AND METHOD: This study was carried out on questionnaires completed by 8 family pediatricians which consisted of two parts: the first section regarded the whole population interviewed (2.271 children) and the second more specific was reserved only to the 42 children classified as affected by the OSDB. These 42 children presented at least 3 of the following 4 features during sleep: (1) the parents are worried about the way their child breaths (2) snoring (3) apnea (4) paradoxical rib cage movement in inspiration. RESULTS: The prevalence of the OSDB was 1.8%. However considering how suggested by some authors even those children who snored and also presented oral respiration, the prevalence increased to 10.3%. These values are similar to the international results with a prevalence of 2-3% for the more severe forms defined as Obstructive Sleep Apnea Syndromes (OSAS) and of 8-11% considering all the forms of the OSDB. Grouping these patients according to their ages, it resulted that the highest incidence of the OSDB was in children between 3-5 years. This observation supports the hypothesis that at the base of the OSDB is an early development of the adenotonsillar tissues, thus in constrast which the classical course which identifies the peak of adenotonsillar hypertrophy between 4 and 6 years of age. The frequency of the single signs and symptoms in the various ages permits the improvement of the clinical diagnosis: in particular snoring, oral respiration and tonsillar hypertrophy are less frequent in the first three years of life, while in the older children the percentage of growth inhibition decreased and it becomes more difficult observing paradoxical rib cage movement in inspiration. Concerning the diagnostic tests, the family pediatrician asks only exceptionally specific test during sleep (5% of the patients). Concerning therapy, many were the indications for adenotonsillectomy even during the first three years of age (82% of the patients) proving that the family paediatrician has overcome the old attitude of not indicating operation in the first 4-5 years of age. CONCLUSION: The confirmed high prevalence of the OSDB, the possibility of further improving the clinical diagnosis, the good capacity of the family pediatrician concerns diagnosis and therapy are all factors which favour the direct management of most of the children with adenotonsillar hypertrophy by the family pediatrician. The diagnosis and therapeutic choice can find support in sleep tests when necessary. These tests have to be carried out in a specialized laboratory and the results be interpreted together with the clinical signs and symptoms. Patients who have to be managed by Pediatric sleep laboratory are: 1) children with OSDB due to organic and functional alterations on genetic basis; 2) children in whom adenotonsillectomy presents a high risk such as a severe respiratory insufficiency and the young age of the patient (less 12-18 months of life).

[Thirty years of activity at the center for cystic fibrosis in Parma]. / Trenta anni di attività del Centro per la fibrosi cistica di Parma.

One hundred fourteen patients (52 males, age range 1 to 40 yr, median age 16 yr) affected by Cystic Fibrosis (CF) are currently followed by the CF Centre of Parma. Forty two percent of them are adults. Most patients live in Emilia Romagna (59%) and Lombardia (19%). With respect to CF genotype, delta F508 mutation has been found in 54% of patients. Despite of the improvement in knowledge of CF, its diagnosis is still based on the clinical features and sweat test is usually used to confirm the diagnosis. Recent diagnostic tools, such as the genetic analysis and the potential difference measurement, could also be helpful in ambiguous situations. In the CF Centre of Parma, in agreement with the literature, the disease is now diagnosed earlier than in the past. Particularly, in the last year the median age of diagnosis was seven months, while it was 20 months in the 1980s. The early diagnosis and the improvement in the treatment of lung disease has significantly increased the median survival age of our patients, changing from 7.5 years in 1975 to 20 years in 1999. A significant improvement in CF prognosis could be further achieved by potential investigational agents, such as "protein-repair" addressed to CFTR activation. However, the gold standard for CF therapy is represented by gene therapy, although it is not yet available.

[The tonsils and adenoids as a site of infection and the cause of obstruction]. / Tonsille e adenoidi come sede di infezione e causa di ostruzione.

The failure to eradicate group A beta-hemolytic streptococci from the pharynx is partly due to a low compliance, but above all, an alteration of the oropharyngeal microbiological flora: reduction of alpha-haemolytic streptococci which inhibit group A beta-hemolytic streptococci and increase of microorganisms such as Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis. These latter act indirectly destroying the beta-lactamic ring of penicillins. However, this obstacle is overcome by the use of antibiotics which do not contain beta-lactamic rings such as macrolides or associating amoxicillin with clavulanic acid or with new cephalosporins which are more resistant to beta lactamases. To restrict the diffusion of resistance to antibiotics, it is essential to limit their use diagnosing streptococcal tonsillopharyngitis more precisely, thanks to an improved use of micro-biological diagnostic tests and by a more extended use of tonsillectomy in recurrent tonsillitis (more than 6-7 in 1-2 years). Adenoiditis is closely related to the post nasal drip syndrome, to recurrent otitis media and to otitis media with effusion. All these situations could, therefore, represent an indication, although not well defined, for adenoidectomy. Nasopharyngeal obstruction due to adeno-tonsillar hypertrophy becomes critical during sleep when the hypotony of the upper airway muscles becomes additional to the anatomical obstruction. At this point the inspiratory effort required and the consequent decrease of intra airway pressure increase the pharyngeal obstruction suctioning the pharyngeal walls toward the median line. The resulting clinical picture is defined as sleep-disordered breathing (SDB) due to adenotonsillar hypertrophy (idiopathic), to be distinguished from SDB due to cranio-facial abnormalities or neuromuscular diseases. SDB includes both the more serious sleep apnea syndrome and the less severe upper airway respiratory resistance syndrome. A combination of symptoms and clinical data detectable both while awake or asleep, make the diagnosis simple. During sleep, both apnea and paradoxical inspiratory movements are highly specific while snoring is highly sensitive. To evaluate nasopharyngeal obstruction radiography and optic fibre endoscopy are both equally reliable. The gold standard test for non idiopathic SDB is the polysomnography, whereas for SDB, due to adenotonsillar hypertrophy, one is limited today to the recording during sleep of O2 saturation or of end tidal CO2. These investigations are, however, generally used up to 2 years of age, when the decision to carry out an adenoidectomy and especially a tonsillectomy is more difficult because of the greater risks which surgery involves at this age. The pharmacological therapy has a purely palliative function and is based on antibiotics, local vasoconstrictors, steroids and theophylline which acts more as an antiflogistic than as a breath stimulant. O2 therapy and nasal continuous positive airway pressure (CPAP) give better results, but are more difficult to carry out, in particular on a long term basis. Adenoidectomy especially if associated with tonsillectomy, leads to the resolution of the symptoms, but not always to a normalization of functional alterations (hypoxia and hypercapnia). For this reason, it is necessary to act on other factors which cause oedema of the nasopharyngeal mucosa contributing to the obstruction. In this area, the prevention of viral infections can be achieved by vaccination against influenza and by preventing the child from attending crowded day care centers. With regard to allergic inflammation, skin prick tests could be a first step in view of allergens avoidance measures. With regard to indoor air pollution, passive smoke must be stopped and the child kept out of the kitchen.

[Circadian variations of bronchoconstriction in asthmatic children]. / Variazioni circadiane della broncoostruzione nel bambino asmatico.

In order to determine the magnitude of circadian variations in bronchoconstriction, we examined modifications of the spirometry observed during the 24-hr in a group of asthmatic (31 males, mean age = 8 years, 6 months). Particular attention was paid to the forced expiratory volume in 1 sec. (FEV1) and the forced vital capacity (FVC). We were thus able to compare in single patients 43 spirometry pairs between morning (between 8 and 10 am) and early afternoon (between 1 and 4 pm), 46 pairs between morning and evening (between 6 and 10 pm) and 54 between 2 consecutive mornings. The results of this study, substantially similar for the FEV1 and the FVC, show that the mean values obtained at different hours during the day and in two consecutive mornings were almost superimposable. On the other hand, the mean values and the standard deviations of the differences between two determinations in single patients are high. The percentage of patients who present significant modifications (greater than 10%) of FEV1 or of FVC at different hours of the day is also quite high. Moreover these variations seem directly related to the baseline spirometric value. Although the literature frequently outlines the existence of a circadian rhythm in the modifications or airways patency (maximum bronchoconstriction in the morning and minimum in the evening), in the asthmatic, however, the fast variations in the bronchial tone can be such as to mask this cyclic rhythm.(ABSTRACT TRUNCATED AT 250 WORDS)

[Respiratory physio-kinesitherapy in cystic fibrosis: the parents' viewpoint]. / La fisiochinesiterapia respiratoria nella fibrosi cistica vista dai genitori.

The aim of this study was to evaluate the problems found in performing chest physiotherapy (PKT) by patients with Cystic Fibrosis (CF) and by their families. The research has been based upon processing 389 questionnaires (46 items) that were compiled by the families. Thirteen CF centers all over the nation have participated in this study. Patients' mean age was 7.4 years (range 2 months to 14 years). Data about the socio-economic status and illness severity were available for every patient. Many graphs (line connects points with a line = n. 8; bar charts = n. 17; pie charts = n. 13) show the results and their statistical processing. Our purpose was to solve the following problems: 1) What is the extent of the compliance? 2) Who perform, or should perform, PKT in the family environment? 3) What is the kind and extent of the help request? 4) What are the mistakes made in executing PKT? 5) What is the usefulness of precursors and aerosol therapy? 6) What is the link between physical activity and PKT? 7) What are the main difficulties in performing PKT? 8) How effective is PKT? 9) What is the extent of the parents' faith in their capabilities? 10) Are there any prejudices against PKT? Three major findings emerge from this study: 1) the compliance appeared good; 2) the average family understood the meaning of PKT correctly and 3) PKT is usually executed properly. As for negative results are concerned, it is to be noted that the burden of performing PKT is usually up to the mother. This finding is linked both to the socio-economics status of the family and to the severity of the illness. On the other hand, a home PKT service is only asked in very extreme situations such as sudden worsening of the patient's illness. The difficulties met in performing PKT range from the patient's refusal to logistical and organization problems, which are correlated with the socio-economic status of the family. The relationship between PKT and sport is understood well and there is a high percentage of patients doing regular physical activity, even if it is often hindered by the severity of the disease. In conclusion, although there are many problems, there are also many methods to use in order to improve the acceptance and the effectiveness of the PKT.