[Recurrent respiratory infections in children].
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Biomedical subjects
Publications and source records attributed to G Tournier.
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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.
BACKGROUND: Extraocular, facial, bulbar and intercostal muscles are frequently affected in juvenile myasthenia gravis, especially during exacerbations. Acetylcholine receptor antibodies are often present in the blood in this type of myasthenic syndrome. CASE REPORT: A girl presented with an exertional dyspnea at the age of 13 years, that improved after rest. All investigations were negative, except for lung function tests that showed a restrictive pattern. The diagnosis of juvenile myasthenia gravis was finally made at the age of 15 years because of the recurrence of sudden exertional dyspnea and a history of subtle weakness on repetitive movement leading to poor suckling, together with vocal and occasional swallowing difficulties. Dyspnea immediately improved after intravenous injection of 1 mg neostigmine, but the response was transient. No acetylcholine receptor antibody was found and a search for thymoma proved negative. Ambenonium chloride (Mytelase) was effective on clinical exacerbations, but the only improved test after 9 months of treatment was the functional residual capacity. CONCLUSION: Anticholinesterase drugs must be tried in patients who present exertional dyspnea without bronchopulmonary or cardiac disease on the presumption of myasthenia gravis even when ocular or bulbar manifestations are absent.
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127 cases of tracheal dyskinesia were seen in infants and children out of which 87 were 1 to 12 months of age. The diagnosis was based on the existence of a collapse reducing the tracheal diameter of more than 50% on endoscopy. Endoscopic examination was performed without general anesthesia. This material represents 5,8% of the patients submitted to this procedure. 85 patients had "primitive" dyskinesia and 42 had major associated abnormalities. Uni or bilateral bronchial dyskinesia was associated in 43% of the cases. The four commonest presenting symptoms were a stridulous or wheezing respiration, recurrent bronchitis, chronic cough, cyanosis. The frequency of associated digestive troubles: gastroesophageal reflux aspiration was noteworthy. Several functional consequences were encountered: hypoxemia, hypercapnia, abnormalities of FRC, increased RL, lowering of dynamic compliance, alterations of perfusion and ventilation on scintiscans. The prognosis was good in primitive cases. Two deaths occurred, in the group with associated abnormalities. The pattern of the patient with primitive dyskinesia and that of the patient with dyskinesia and associated abnormalities are outlined. Some features remarkable in this series of patients are pointed out in a discussion of the pathophysiology of the syndrome. Increased transmural pressure is not a common cause of tracheal dyskinesia and infection as well. The possibility of a temporary intrinsic anomaly of the tracheal wall is suggested. Even if its exact mechanism remains unknown, tracheal dyskinesia is a distinct entity observed in infants and children. It appears as a common cause of recurrent bronchopulmonary disease in the young.
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.
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.
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.
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Three children presenting with vascular abnormalities of the right lungs are reported. Clinical symptoms were not specific. Total lack of perfusion associated with normal ventilation at pulmonary scintiscanning was highly suggestive. Diagnosis was confirmed by pulmonary catheterism and angiography. The congenital or acquired nature of these abnormalities is not established. Development of pulmonary arterial hypertension during follow-up may need pneumonectomy.
The effect of naloxone on occlusion pressure (P0.1), the pattern of breathing and the expiratory flows during spontaneous ventilation was studied in 16 children with chronic obstructive pulmonary disease under control conditions, after isotonic saline injection and 5 (N5) and 25 (N25) min after i.v. injection of naloxone (2 micrograms X kg-1). At N5, no change was observed in tidal volume normalized for body weight (VTBW), inspiratory time (TI), respiratory frequency (f), mean inspiratory flow (VTBW/TI) and the ratio of TI and total duration of the respiratory cycle (TI/TT). P0.1 decreased significantly (p less than 0.001) at N5 and returned to control values at N25. The decrease in P0.1 without any change in the breathing pattern suggests that naloxone has an effect on respiratory mechanics. Indeed, at N5, the expiratory flows generated at 25% of VT, measured on the flow-volume curves during spontaneous ventilation, increased significantly when compared to control values. Moreover, the decrease of P0.1 after naloxone was found to be correlated to the reduction of dynamic lung compliance (CLdyn) (p less than 0.02). It is speculated that peripheral airway obstruction, as reflected by the decrease in CLdyn, might be a triggering factor for the release of endorphins. The bronchodilation observed after naloxone could then be due to naloxone's antagonistic effect on endorphin-induced bronchoconstriction.
BACKGROUND: 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. METHODS AND PATIENTS: 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. RESULTS: 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. CONCLUSIONS: 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.