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To assess the results of inlay butterfly cartilage tympanoplasty in children.Before-and-after trial; follow-up duration, 26.6 +/- 19.9 months (mean +/- standard deviation).Tertiary referral center.Fifty-nine pediatric cases of tympanic membrane perforation.Inlay butterfly cartilage tympanoplasty was performed under general anesthesia according to the technique originally described by Eavey and modified by Lubianca-Neto (i.e., without any associated split-thickness skin graft).Percentage of perforation closures, surgical complications, preoperative and postoperative puretone hearing thresholds; the results of inlay butterfly cartilage tympanoplasty were compared with those obtained in a retrospective series of 29 underlay fascia temporalis myringoplasties.The 71% "take rate" of inlay butterfly cartilage tympanoplasty was not significantly different from the 83% take rate obtained with underlay fascia temporalis tympanoplasty (p = 0.23, chi test). The anatomic results were improved when the graft diameter was at least 2 mm larger than the size of the perforation (81% take rate) (p = 0.009, chi test). No iatrogenic cholesteatoma was observed. Pure-tone hearing thresholds were improved at 0.5, 1, and 2 kHz, and stable at 4 kHz. Hearing levels were not different from those obtained with underlay fascia temporalis tympanoplasty.Inlay butterfly cartilage tympanoplasty is a safe, efficient, time-saving, and easy technique of tympanoplasty in children. Anatomic results may be improved by associating a split-thickness skin graft and/or by trimming a tragal graft much larger than the size of the perforation.

Progression of pulmonary sarcoidosis in children remains poorly documented. The aim of this work was to gather follow-up information on pulmonary outcomes in children with sarcoidosis and to obtain data of relevance to a discussion of the optimal length and regimen of glucocorticoid therapy.In the present study, the authors experience of pulmonary sarcoidosis in 21 children referred to the paediatric pulmonary department over a 10-yr period is reported with a documented follow-up of at least 4 yr. Evaluation of the disease during the follow-up included analysis of clinical manifestations, chest radiographs, pulmonary function tests with measurements of the vital capacity (VC), dynamic lung compliance (CL,dyn), lung transfer for CO (TL,CO), and arterial blood gases, as well as bronchoalveolar lavage (BAL) with determination of total and differential cell counts.After initial evaluation the decision was a careful observation of four children without therapy. Corticosteroid treatment was initiated in 17 children. Analysis of results indicated that after 6–12 months of treatment most clinical manifestations of the disease and chest radiograph abnormalities disappeared, and beneficial effects on VC andTL,COwere apparent. After 18 months of steroids no benefit on pulmonary function tests could be noticed, with mainly persistence of alterations ofCL,dyn. Results of BAL studies documented the presence of an alveolitis with increased lymphocyte populations throughout the follow-up. Relapses were observed in four children during tapering of prednisone; they were not reported after discontinuation of steroid therapy.Taken together data obtained in the presented population can lead to the following suggestions for the management of pulmonary sarcoidosis in children. BAL should be performed at the initial evaluation to document alveolitis; however, nothing seems to be gained from repeating this investigation during follow-up in the absence of specific reasons. Once the decision to initiate glucocorticoid therapy is made, 18 months may be a reasonable treatment duration. Discontinuation of therapy can be decided even if the pulmonary function tests remain abnormal, but the child should then be carefully monitored for a relapse.

Objective To evaluate the sensitivity of multidetector computed tomography for confirming suspected foreign body aspiration into the airways in children. Method We conducted a multicentre prospective study of 303 children evaluated using multidetector computed tomography with axial analysis complemented by multiplanar reconstruction when required. The images were read by a radiologist before endoscopy then reviewed later by a senior radiologist blinded to the endoscopy findings. Endoscopy was performed routinely. Results Foreign bodies were found by endoscopy in 70 of the 303 children. The initial multidetector computed tomography reading was 94% sensitive and 95% specific. For the review, the images for 91 patients were excluded because of motion blurring or absence of larynx visualisation; in the remaining 212 patients, sensitivity was 98% and specificity 97%. Conclusion Multidetector computed tomography as performed in our patients cannot replace endoscopy, which remains the reference standard. Nevertheless, multidetector computed tomography is sufficiently sensitive to be of value when foreign body aspiration is not considered initially or when endoscopy is likely to prove challenging.

The aim of our study was to determine if rectal sedation with pentobarbital sodium provides safe and effective sedation for children undergoing auditory brainstem response (ABR) testing.A prospective study was conducted in the ENT pediatric department of Robert Debre's hospital (APHP, Paris). 68 children under 8 years of age were given rectal pentobarbital for ABR testing at a dosage of about 5 mg/kg.61 children of 68 (89.7%) were adequately sedated with rectal pentobarbital. The mean elapsed time from drug administration to full sedation was 36,1 minutes. No adverse event was reported in 84.1% of children.Pentobarbital provides safe and effective sedation. Rectal administration is easy, painless and with brief duration of action. It's a good alternative to general anesthesia for young children undergoing ABR testing.

Four-quadrant cricoid division in laryngotracheal reconstruction is indicated in case of very tight laryngotracheal stenosis. The principle of this operation is to section the anterior, posterior and lateral walls of the cricoid and to increase the diameter of the laryngeal lumen by interposition of a cartilaginous graft in the anterior and/or posterior cricoid wall.From 1991 to 2001, 25 children underwent a laryngotracheoplasty with four-quadrant cricoid division. Two techniques were used: a single-stage procedure with a 10 days post-operative intubation (n=6), or a two-stage procedure with prolonged post-operative intraluminal stenting and tracheostomy (n=19).In the two-stage procedure the decannulation rate was 89% (17/19) after the first operation, and 100% after revision surgery using the same technique. In the single-stage procedure the extubation rate was 83.3% (5/6).Laryngotracheal reconstruction with four-quadrant cricoid division gives excellent airway patency especially in case of severe subglottic stenosis with altered laryngeal mobility. The single-stage procedure decreased the tracheostomy related morbidity. This technique is particularly adapted to children under three.

Epistaxis is a frequent emergency for which guidelines and treatment are well known. Nevertheless few studies have evaluated the factors that may influence the decision for hospitalisation. Through a retrospective study we have searched to define potential factors which lead to taking such a decision.Retrospective analysis of charts of 260 patients hospitalized for epistaxis. For each patient we have reviewed several data including, age, associated pathologies, current treatment, duration of the stay, complications, recurrences etc...If the severity of the bleeding is one of the major factor, socio-economic factors are also taken into account and may influence the decision to hospitalize. Age, associated pathologies (hypertension, hemopathy) and their treatment (anti-coagulant) also play a role in this decision. Furthermore an obvious strategy in the choice of the different treatment may help to reduce the length of stay and the morbidity of each treatment.We propose some modifications to our strategy to improve the management of epistaxis. This includes the use of endoscopic techniques for diagnosis and treatment.

We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leucocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. Light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1–11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. Conclusion Renal failure, proteinuria, leucocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.

Mycoplasma pneumoniae, a gram-negative bacteria, is an important cause of lower respiratory tract infection in children (20% of cases). The infection tends to be endemic and is punctuated by epidemic episodes every 4 to 7 years. Its frequency seems to be higher in children between 5 and 9 years of age, but is probably underestimated before 5 years. M.pneumoniae may cause multisystem infection. Diagnosis is established upon clinical data and laboratory findings. Usually, the infection is associated with leucocyte count under 15,000/mL and C-reactive protein under 50 m/L. Detection of M. pneumoniae DNA in clinical samples appears to have advantages over serological tests. Severe infections have been described in patients with humoral and cellular immunodeficiencies, sickle cell disease, cystic fibrosis. Treatment with macrolids and tetracyclines (after 8 years of age) is indicated. Respiratory functional sequelae are possible.

The development of BAL in children for both research and clinical purposes has been limited so far by the difficulty in establishing reference values. The aim of the study was (1) to define composition of BAL cellular components in control children and to evaluate the ability of these cells to express various cytokines, and (2) to study modifications of differential cytology and BAL cell cytokine responses in children with interstitial lung disorders.Two groups were investigated: a control group of 16 children who were concluded to be free of parenchymal lung disease after complete pulmonary investigation, and a group of 11 children with pulmonary sarcoidosis. Differential cytology was evaluated by standard techniques. BAL cell cytokine expression was studied at the level of messenger RNA (mRNA) by reverse transcription-polymerase chain reaction (RT-PCR) methods.In the control group, differential cell counts appeared to be similar to values reported in adult populations with normal distribution of the data and no influence of age. In this group, no transcripts for interleukin-1beta (IL-1beta), tumor necrosis factor-alpha (TNF-alpha), IL-6, and transforming (correction of tranforming) growth factor-beta (TGF-beta) could be detected. In children with sarcoidosis, different profiles of IL-1beta, TNF-alpha, IL-6, and TGF-beta expression were individualized which seemed to be related to the activity and/or severity of the disease, IL-6 and TGF-beta mRNA being observed only in the more severe forms.These data provide information on BAL cell number and function in children. Characterization of BAL cytokine expression patterns during the course of interstitial lung diseases in children may be of great interest for evaluation of disease activity and/or severity and therefore for planning of therapy.

Bronchodual is a combination of two bronchodilators, a beta-2 adrenergic substance (fenoterol, 50 micrograms per dose), and an anticholinergic substance (ipratropium bromide, 20 micrograms per dose), administered by metered aerosol. According to different studies carried out with adults and children, the bronchodilating action of this combination is greatly superior to that obtained with each substance individually; the beta-2 adrenergic substance can be spared; its action is longer and involves a decrease in the number of drug intake and lastly its tolerance is excellent. A French multicentre study was performed with 74 children (7 to 15 years old, mean: 11.8 +/- 2.6 years), 53 boys and 21 girls with allergic or non-allergic asthma: 41 children presented a moderate asthma (one crisis per month) and 33 children moderately severe asthma (one crisis per week). These children had been given no corticosteroid therapy (per os or inhaled) for at least 15 days, their usual long-term treatment (cromoglycate, anti H1, theophylline LP, antibiotics) was given as normal. Their FVC wasor = 80% of predicted values and they presented an intercritical airway obstruction. Hypoxemia was noted in 41.2% of the children. A reversibility test was performed with Bronchodual: 68 of 74 children (i.e. 91.9%) were responders, ie after two doses of Bronchodual at least one of the FEFs had increased by at least 15% when compared with the initial values. The responders were given a 2 month treatment with two inhalations 3 times/day with a spacer. Fifty-seven patients were considered for the final evaluation.(ABSTRACT TRUNCATED AT 250 WORDS)

Right pneumonectomy can lead to severe respiratory impairment due to stenosis of the left main bronchus. This syndrome is usually treated by inserting a fixed-volume prosthesis but, in children, expandable prostheses have the advantage of being adaptable to growth and permit progressive recentering of the mediastinum. We report 3 such cases, with the results of pulmonary function tests. The patients were aged 11, 17, and 22 years at the time of implantation and had undergone pneumonectomy during childhood for either bronchiectasis or complete pulmonary sequestration. All 3 patients are doing well, with a follow-up of 1 to 3 1 2 years. Pulmonary function tests have shown a substantial improvement in the obstructive syndrome in 2 patients whereas, in the third patient, in whom the contralateral lung was not perfectly healthy, the functional improvement was only moderate.

The clinical course of sarcoidosis in children has not been well defined. Eight children with symptomatic sarcoidosis included in this study underwent pulmonary function tests and bronchoscopy with bronchoalveolar lavage (BAL) before treatment and during steroid therapy. At the start of therapy, functional parameters, mostly dynamic lung compliance and lung transfer factor for CO, were impaired. This was associated with abnormalities of BAL cell populations: increased total cell number with a high proportion of lymphocytes, modifications of lymphocyte subpopulation with an elevated CD4+/CD8+ ratio, and enhanced ability of alveolar macrophages to release hydrogen peroxide. Although respiratory abnormalities seemed to be similar at the initial stage of sarcoidosis in children and adults, the course of the disease appeared to be different. Despite the absence of respiratory symptoms and disappearance of chest radiographic abnormalities on prolonged steroid treatment, we found slow improvement of pulmonary functions associated with persistence of BAL lymphocytosis and elevated CD4+/CD8+ ratios. However, the ability of alveolar macrophages to release hydrogen peroxide was significantly reduced after 6 months of steroid treatment, and it remained identical to the control group. Therefore, the evaluation of disease activity appears to be critical for therapy in pediatrics, and for this purpose studies of alveolar macrophage activation may be of particular interest. Pediatr Pulmonol. 1993; 16:41–47. © 1993 Wiley-Liss, Inc.

Chronic respiratory failure (CRF) with hypoxia and hypercapnia is the last ineluctable phase in cystic fibrosis (CF). Nasal positive pressure ventilation (NPPV), a non-invasive method, may be given to CF children with CRF, especially to patients accepted for transplantation (T). This method improves ventilatory function by resting the chronically exhausted respiratory muscles, facilitates bronchial drainage by physiotherapy, prevents the exacerbations of the illness and prepares patients for T.NPPV was used in 6 CF patients (mean age 13 years 6 months). One of them was transplanted 15 days later, two of them were accepted for T. All had hypoxia. Five of them had hypercapnia.NPPV was given to four patients for 3 to 14 months. The preliminary results were positive. One patient gained weight, two had more fluid sputum. One patient showed an increase in functional respiratory tests (FRT: PaO2, vital capacity, FEV-1) while these tests were stabilized in the others.NPPV in an effective non-invasive method for use with CF children. It is indicated for CF patients accepted for T and also earlier, for CF patients with CRF in order to prevent acute exacerbations and functional respiratory deterioration.

A series of 27 children (mean age: 12 yrs, 5 mos.) presenting with thoracic sarcoidosis is reported. This series, collected from 1961 to 1988 shows the rarity of the disease at that age. However the low rate of asymptomatic forms (22%) suggests that the frequency of the disease is underestimated, as it is not diagnosed. The histological proof is necessary for the diagnosis. When peripheral lesions available for biopsy are lacking, a liver needle biopsy is helpful (93% of positivity). This study shows the frequency of multivisceral types, the intensity of the macrophagic and lymphocytic alveolitis. The therapeutic indications depend on the comparison of the radiological stage, the results of pulmonary function tests (PFT), those of the bronchoalveolar lavages (BAL) and of the serum granulomatous activity markers, especially concerning angiotensin converting enzyme (ACE). When present at the beginning of evolution, several risk factors lead to use a corticosteroid treatment: age of onset before 4 years, multivisceral involvement, presence of functional pulmonary signs, delayed diagnosis and onset of treatment, impaired respiratory function (especially concerning the alveolo-capillary diffusion), PMN cells greater than or equal to 2% in the initial BAL, and IgG proteins greater than 4 SD. Thus sarcoidosis in children differs from that seen in adults as it has a more marked evolutive tendency and leaves severe sequelae in one third of patients.

Background. Ciliary ultrastructural defects with total lack of dynein arms (DA) cause abnormal mucociliary function leading to the chronic infections observed in primary ciliary dyskinesia. The role of partial ciliary ultrastructural defects, especially those involving the central complex, and their relationship with respiratory symptoms have been less thoroughly investigated. Objective. In a pediatric population with partial ciliary defects, we determined the relationship(s) between ultrastructural findings, ciliary motility, and clinical and functional features, and evaluated the outcome of this population. Design. We analyzed the clinical presentation and pulmonary function of 43 children with chronic bronchitis and partial ultrastructural defects (from 15% to 90%) of their respiratory cilia demonstrated on bronchial biopsies. The study population was divided into 3 groups according to ciliary ultrastructure: the main ultrastructural defect concerned the central complex in 23 patients (CC group), peripheral microtubules in 8 patients (PMT group), and DA in 12 patients (DA group). Results. The percentage of ciliary defects was lower in the PMT group than in the CC and DA groups. Patients in the PMT group had less severe disease with frequent normal ciliary motility. Patients in the CC group had initially a higher incidence of respiratory tract infections, extensive bronchiectasis frequently requiring surgery, and arguments in favor of a congenital origin (high proportion of sibling form). Partial absence of DA, although of congenital origin, was associated with a good prognosis. In all groups, follow-up showed that the functional prognosis remained good with appropriate treatment. Conclusions. In children with chronic respiratory infections, presence of situs inversus, sibling form, obstructive pulmonary syndrome, or bronchiectasis required ultrastructural analysis, regardless of ciliary motility. Detection of CC abnormalities is a marker of severity and required intensive therapy and close follow-up.

The short term prognosis of the esophageal atresia has been improved by the progress of diagnosis and surgical techniques. The long term prognosis still depends upon the occurrence of bronchopulmonary complications. Having investigated fourteen cases and a review of the literature, the authors studied the cause of these respiratory complication. Two factors seem to play an important role: the gastroesophageal reflux and the tracheomalacia. This has important therapeutical implications: treatment of the gastro-esophageal reflux and long term respiratory kinesitherapy. The authors suggest for these patients a management plan over several years.

The authors report a new case of partial immune deficiency of cellular immunity, associated with chronic mucocutaneous candidiasis in a 12 Years-old boy. The disease began very early during the first few weeks of life, with thrush in the mouth. This candidiasis then evolved intermittently and was still present. Numerous cutaneous, pulmonary and ear infections occured throughout this child's life. This morbid association led to a search for an immune deficiency. Humoral immunity was normal. Abnormalities of cellular immunity were as follows: apart from candidine skin anergy, there was a deficiency in the factor which inhibits leukocyte migration, secretion of a factor favouring this migration (MEF). It was also noted the presence of the patient's serum, of a factor inhibiting lymphocyte transformation in the presence of candidine. In spite of treatment with intravenous route, amphotericin B, followed by transfer factor, the oral candidiasis persisted together with the skin anergy to candidine. On the other hand, the serum inhibitory factor disappeared. Pulmonary cryptococcosis probably favoured by corticosteroid treatment, developed on this background of immune deficiency; as usual it spread to the meninges. Treatment associating intraveinous amphotericin B and 5 fluorocytosine oral and later intravenous, total duration 6 months, grave a recovery maintained on a 8 months follow up.

Among nearly 1 300 infants and children studied for chronic or recurrent respiratory diseases 36 of them had plasma and/or salivary IgA deficiency. They were 1 to 13 years of age with 20 between 3 to 6 years; 11 had total, lack or traces of salivary IgA, 9 had very low or nul plasma IgA titer and 6 had both. The deficiency was transitory in 10. Associated abnormalities with potential liability for the disease: allergy, gastroesophageal reflux, alpha-1-antitrypsin deficiency and other immunologic deficiency were encountered in 26. The often multifactorial pattern of respiratory diseases in pediatrics is emphasized. The pathogenic role of IgA deficiency is discussed. Even though its responsibility is demonstrated, its real magnitude and extent requires further studies.

The present study relates to 39 children, 24 boys and 15 girls, aged 1 to 17 (mean age, 6 1/2 years) suffering from chronic airflow obstruction without muco-viscidosis. The search for a ciliary anomaly was motivated either by the coexistence of situs inversus (group I: 12 cases) or by the negative or scanty aetiological history (group II: 27 cases). The investigation of the cilia consists of a study of ciliary motility and an ultrastructural study of biopsy specimens using the electron microscope. Groups I and II were similar as regards severity of disease, assessed by the incidence of bronchiectasis and chronic hypoxia at rest; a higher incidence of neonatal respiratory distress was noted in group I (7 cases against 4 in group II). Ciliary immotility was particularly noted in group I (7 cases) and one case of weak motility in group II. The major ultrastructural anomalies of the axonemal complex were seen as well in both groups I and II; it was noted that all the structures may be totally or partially missing with the exception of the peripheral microtubules. The discussion centred on three points: 1. In the first analysis, the discordance between the existance of major ultrastructural anomalies of the axonemal complex, a priori incompatible with the conservation of ciliary motility on light microscopy; the link seems to lie in the percentage of cilia affected, the existence of ciliary dyskinesia and finally the disorientation of the basal corpuscles. 2. The immunologic abnormalities sometimes associated with a syndrome of ciliary immotility: that is the anomalies of leucokyte migration under the control of microtubular structures in the peri-centriolar region. 3. The practical consequences. Current treatment rests on daily respiratory physiotherapy and antibiotics adapted for cases of superinfection; also there are drugs stimulating ciliary activity perhaps by the effect on their residual motility; some substances carrying ATP and ATP-ase may re-establish ciliary motility anulled by the absence of dynein arms; also the observations of major ultrastructural abnormalities ought to lead to better genetic counselling than at present, where the mode or modes of transmission are uncertain.

Three cases of cholesterol interstitial pneumonia in patients 3, 9 and 10 years of age respectively are reported. All three were born in Island of Reunion. Two were sisters. All had failure to thrive, dyspnea on rest and clubbing. Respiratory symptoms had appeared early in infancy. Open pulmonary biopsy was diagnostic. Prognosis was poor the boy dying at 4 years of age and severe respiratory insufficiency at 9 or 10 years in the two girls. Current etiological investigations were non contributory. However a profile of chronic infection with Epstein Barr virus (EBV) was found in each case while serological profiles ruled out infection with a virus of the herpes group virus (cytomegalovirus, herpes simplex). The possible role of EBV as an etiological agent of cholesterol pneumonia is discussed and genetic or environmental factors as well.

The authors report the cases of 2 children, 12 and 4 years old respectively, presenting with a lipoma of the postero-superior mediastinum, that were investigated within a 6 year-period. Costal erosions were present in both cases with intraspinal extension in one child. Standard X-ray films should have led to the diagnosis, because of the relative low density of the mass considering its volume. In one case, CT scan confirmed the homogeneous fatty nature of the mass.

The control of breathing at rest was studied in 30 children (3 to 17 years old) with chronic obstructive pulmonary disease (COPD). Five of them were tested several times during the follow-up of the COPD. Breathing pattern was evaluated and mouth occlusion pressure (PO.1) was measured. Results in COPD children were compared to previously reported values in healthy controls. PO.1 was significantly increased. In 11 children breathing O2, PO.1 decreased but remained at higher levels than predicted. However, the decrease in PO.1 in COPD was not significantly greater than in healthy children. These results may be explained by the relative mild hypoxemia in those children. The increase in PO.1 was significantly correlated with the increase in lung resistance (p less than 0.02) and with the decrease in dynamic lung compliance (p less than 0.01). Most of the COPD children were normocapnic. However, modifications in the breathing pattern were observed. The inspiratory time (TI) was significantly shortened, the expiratory time was prolonged and the TI over the total duration of the respiratory cycle was lowered. The respiratory frequency was unchanged. The tidal volume, normalized for body weight (VTBW), was increased. The mean inspiratory flow (VTBW/TI) was significantly augmented, but not as much as PO.1: in consequence, the effective inspiratory impedance was higher than predicted. Thus, as adults, COPD children had a greater inspiratory neural drive. In contrast to normocapnic COPD adults, they had a modified respiratory timing.

The authors describe 7 cases of idiopathic pulmonary hemosiderosis occurring in children, and emphasize the diagnostic value of the association of pulmonary and anemic manifestations, one of which could appear to be an isolated finding and thus delay diagnosis. Complementary investigations are discussed and the relative inocuity of a lung biopsy with a Gerbeaux needle, and the greater value of bronchoscopic samples over those obtained by gastric intubation for evaluating the presence of siderophagic activity emphasized. Functional pulmonary tests, especially for blood gases and ventilatory function, were also conducted. The difficulties in classifying the various types of pulmonary hemosiderosis, especially the "idiopathic" form in children, are outlined. Immunological disorders must be systematically searched for in order to choose the most effective therapy: corticoids or immunodepressants.

A nine-year-old boy was hospitalized for pneumonia of the left lower lobe. A left pleural effusion developed 48 hours later. The same E. coli strain was recovered from five blood cultures, pleural fluid, and middle ear fluid. Recovery was achieved after two months. Pleural lavage was performed twice daily for the first 15 days and parenteral antimicrobial therapy was given for 45 days. Because E. coli is not usually responsible for ENT or lower respiratory tract infections, an immune deficiency was sought for. The index patient had a twin in whom a history of osteomyelitis at the age of 2 and pneumonia at the age of 8 was found. Recurrent otitis had been a problem in both twins from the age of 14 months. In both twins, immunoglobulin assays led to the diagnosis of Bruton agammaglobulinemia. These two children are now receiving intravenous infusions of human immunoglobulins every three weeks. Bruton agammaglobulinemia is infrequently diagnosed at so late an age.

Five cases of hypersensitivity pneumonitis in children are reported. Four had the pattern of bird breeder's disease and one of aspergillus lung hypersensitivity. All were clearly defined by clinical, radiological, functional and immunologic features. The possible difficulties in diagnosis are emphasized. The disease might be frequently overlooked and its frequency underestimated in children. The diagnostic value of precipitating antibodies is discussed. Although some immunologic studies suggest immune complex mechanisms, recent experimental and clinical data are in favor of a role for cell-mediated immunity. In addition whether or not immunological damage is to occur and result into disease depends not merely upon the characteristics of the antigen exposure. Genetic and/or acquired individual differences in immune reactivity can play a definite role as well. The risk of secondary pulmonary fibrosis and chronic respiratory failure in untreated patients is emphasized. Immunologic and functional tests for identification of the provoking antigen and ceasing of contamination exposure are urgent : they are also useful for monitoring steroid therapy.

The treatment of childhood asthma should be considered with respect to age, severity and aetiology. Treatment should be instituted early from the first crisis in order to avoid progression to a more severe form. It consists of two aspects: the treatment of the acute episode and the chronic treatment. The treatment of the acute episode consists of using bronchodilators (BD) (rapid release Theophylline and/or beta agonists) to which one might add corticosteroids if the crisis lasts for more than a few hours or seems severe at the outset, an antibiotic should also be used as infection is often a trigger factor in infants. Maintenance treatment is necessary in asthmatics with frequent exacerbations. It should be tailored to the symptomatology and aetiology. The symptomatic treatment consists of a bronchodilator (slow release Theophylline or an atropine-like pharmacological derivative) to which one may add, in severe cases, corticosteroids which may be in the form of aerosol, or as rarely as possible by mouth. The second aspect of treatment relating to the aetiology is the most difficult to apply as childhood asthma is often multi-factorial: in allergic asthma the avoidance of allergens, disodium cromoglycate, ketotifen, and if necessary specific desensitization. In non-allergic asthma, physiotherapy, treatment of infectious foci, particularly ENT (ORL), and attention to psychosomatic features. When asthma is diagnosed and treated early the prognosis is transformed and progress towards chronicity is avoided.

During the last six years, the search for a deficiency in alpha-1-antitrypsin (AAT) was carried out in 106 children aged from 5 months to 14 years, having the obstructive pulmonary syndrome. Nine deficiencies were in this manner detected and phenotyped. These are the observations of three of these children which are reported here. Their age ranged between 4 years and a half for two of them and 8 years for the third one, having asthma. The phenotypes are respectively SS, MZ, and ZZ. Following these observations, the problems brought up by the association in AAT deficiency and the obstructive pulmonary syndrome in children are discussed: clinical symptomatology, interpretation of the basic design, pathogenic signification and possible connections with asthma and therapeutic influence. The functional respiratory disorders noted in these children involved a ventilatory asychronism, an overexpansion, and regional abnormalities of ventilation and perfusion. To answer these still outstanding questions, the need for a systematic prospective study carried out on a wide group of children is emphasized.

Twenty children aged between 5 and 16 suffering from severe asthma were treated with long acting oral theophylline. The minimum duration of treatment was three months and the maximum 12 months. Clinical, pharmacokinetic and pulmonary function studies were made. With a mean dose of 8.75 mg/kg, morning and evening, the theophylline blood levels on the 4th day of treatment (4 hours after the morning dose) were 12.3 +/- 4.36 mg/l. Those taken 4 hours after the evening dose 9.41 +/- 3.75 mg/l, suggesting a circadian rhythm for theophylline blood levels. The importance if fever is stressed in relationship to blood levels, which were increased by 67-100% with fevers of 38.5-39 degrees C. Treatment was very beneficial in 53% of cases. Respiratory function studies were performed in 13 children. After 4 days of treatment there was a significant decrease in airflow obstruction and hypoxemia However, despite carefully adjusted treatment, functional disturbances persisted, in particular hyperinflation.