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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.

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.

Background: The incidence of asthma in infancy is rising but its clinical and physiological components remain unclear., Methods: A total of 24 infants, aged less than 48 months, in whom the first wheezing episode (WE) appeared before the age of 30 months (mean age: 9 months) underwent clinical examination and pulmonary function tests at least 2 weeks after the last WE., Results: The mean WE frequency was 1.1 per month and the mean number of admissions for WE was 1.8. 63% of patients showed symptoms between WE and 50% had an allergic profile. There was no evidence of thoracic distension. Bronchial obstruction (BO) occurred in 71% of patients; among these, BO was distal or generalized in 59% and medium or severe in 47%. 12.5% of patients were hypoxemic at testing. BO was less severe in patients treated with theophylline; it was more frequent (87%) in those with symptoms between WE and/or several admissions, and/or admission to the intensive care unit., Conclusion: This study provides additional evidence that infants presenting with asthma at an early age have severe clinical and physiological profiles.

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.

We report the follow-up of 13 infants aged 2.5 to 24 months in whom cystic fibrosis disease presented as respiratory distress. All infants received an intensive treatment. Mean clinical, radiological and microbiological follow-up was 3.1 years. Ten children aged 1.5 to 8.5 years are still alive. One of them presents with serious respiratory failure, with a Shwachman score of 50. The Shwachman score is between 85 and 70 for 8 of 13 and 65 for one. The prognosis of this group did not differ from the one usually observed in the disease. It depends partially on early intensive care management and is not altered by mechanical ventilation.

A 4 1/2 month-old infant presented with severe alveolo-interstitial pneumonitis needing intubation and mechanical ventilation. Legionella pneumophila infection was established by a significant increase in antibody titers. The clinical picture was consistent with that of legionnaires' disease. No immune defect could be proven.

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.

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.