Guy Tournier

Mycobacterial lung disease in cystic fibrosis: a prospective study.

BACKGROUND Patients with cystic fibrosis (CF) may be predisposed to airway infections with unusual organisms, such as mycobacteria. The aim of the study was to determine the incidence and clinical picture of mycobacterial infection in CF children. METHODS At least 2 acid-fast bacillus (AFB) smears and mycobacterial cultures were performed on a prospective basis on 682 sputum specimens from 106 patients during a 1-year period. RESULTS Thirty-three percent of the cultures were contaminated with other bacteria. Seven children had at least one sputum culture positive for one mycobacterium. Five children had only one positive AFB culture. Their clinical status and lung function remained stable during follow-up. Two teenagers with severe lung disease had several positive AFB smears and cultures for Mycobacterium chelonae and Mycobacterium abscessus. The isolation of M. chelonae and M. abscessus was associated with a clinical and functional decline. Clarithromycin treatment resulted in temporary improvement with the disappearance of the mycobacteria after 6 months of treatment. This prospective study shows an incidence of 2.3% for positive cultures. The prevalence was 6.6% for mycobacterial colonization but only 1.9% for mycobacterial lung disease in our pediatric population. CONCLUSIONS We recommend performing AFB smears and cultures in CF children with severe lung disease and/or during a lung exacerbation. In these patients persistence of M. chelonae or M. abscessus in sputum should lead to consideration of treatment with clarithromycin.

Idiopathic pulmonary fibrosis in infants

Idiopathic pulmonary fibrosis is a poorly characterized disease in infants. In the present report, we reviewed our experience with 10 infants during a 10‐year period. In 9 patients, onset of symptoms occurred before the age of 2 months and included tachypnea, cough, and inadequate weight gain. However, despite the presence of these symptoms, diagnosis was delayed for 3 months at which time the infants were referred to the pediatric pulmonary department, when the diagnosis was confirmed by open lung biopsy. At the time of admission, bronchoscopy with alveolar lavage was performed in 9 children and showed severe alveolitis with an increase in the neutrophil count. Nine infants were treated with prednisone alone or in combination with chloroquine, colchicine, or cyclophosphamide; all these patients died despite treatment. One infant was treated with pulses of methylprednisolone because of a failure in response to oral prednisone. This girl who displayed similar clinical, radiological, and histological abnormalities as the other children at the time of diagnosis is the only child still alive after 3 years of follow‐up. She is now free of respiratory symptoms and has a normal growth curve. The present report raised two important points: (1) a thorough evaluation of characteristic symptoms should lead to an early diagnosis of pulmonary fibrosis in infants; and (2) administration of pulse therapy using corticosteroids has been helpful and needs to be evaluated further. Pediatr Pulmonol. 1997; 23:49–54.

Chronic interstitial lung disease in children: Response to high-dose intravenous methylprednisolone pulses

The prognosis for children with chronic interstitial lung disease is poor and the mortality rate is high, especially in infants. This explains the many therapeutical protocols which have been proposed and investigated by several authors. In the present work, we evaluated the response of three infants with idiopathic pulmonary fibrosis to high‐dose intravenous prednisolone pulses. The patients were referred to the department at the age of 4, 17, and 3 months, respectively. The diagnosis was confirmed by open lung biopsy and intravenous pulse methyl prednisolone therapy was started with the following protocol: 300 mg/m2 methylprednisolone daily for 3 days, repeated every 4 to 6 weeks. Because of the extreme severity of the respiratory distress at the time of diagnosis, the intravenous pulse treatments were initially complemented by oral prednisone. Clinical improvement was noticed within 6 months with progressive correction of hypoxemia. After follow‐up for 3.5 to 4 years, with a total number of pulses of 37, 26, and 32, respectively, the children are symptom‐free and do not require oxygen supplementation. During this period, no side effects and no adrenal insufficiency could be documented. Based on current knowledge of steroid action, it can be speculated that the response to intermittent high‐dose intravenous methylprednisolone may explain the ability of this mode of hormone administration to maintain an adequate level of glucocorticoid receptor expression. More information and trials through multicenter collaborations are needed to assess therapeutical protocols of repeated high‐dose intravenous steroid treatment. Pediatr Pulmonol. 1998; 26:332–338.

Multicenter, Open-Label Study of Recombinant Human DNase in Cystic Fibrosis Patients With Moderate Lung Disease

Cystic fibrosis is characterized by the accumulation of thick viscous purulent secretions. Recombinant human deoxyribonuclease I (rhDNase) breaks down extracellular DNA, which contributes to the increased viscosity of sputum. A multinational, open‐label study was conducted in 974 cystic fibrosis patients with moderate lung disease [forced vital capacity (FVC) 40–70% of predicted values] to examine the safety and efficacy of aerosolized rhDNase, 2.5 mg, once daily over a period of at least 12 weeks. Patients were assessed under conditions reflecting routine clinical practice.

Carcinomes mucoépidermoïdes bronchiques: à propos de trois observations

BACKGROUND: Mucoepidermoid carcinomas (MEC) are very rare (less than 20 cases reported in the literature) and potentially malignant bronchial gland carcinomas. PATIENTS: Three children, two boys (11 and 7 years old) and one girl (5 years old) suffered from respiratory symptoms such as cough, recurrent pneumonia and/or hemoptysis for 2 to 12 months. Bronchial endoscopy showed a mass into the left (two cases), or the right main bronchus (one case). Chest CT scan identified local extension, and lung-associated lesions. Histopathological study concluded to MEC in the three cases. The patients were treated by segmental bronchial resection, completed with left upper lobectomy (two cases), bronchotomy (one case). All the tumor could be removed; there was no metastasis. The outcome was uneventful with a 8 to 24 months follow-up. CONCLUSION: Bronchial tumors of children must be considered in patients with chronic cough, recurrent pneumonia and/or hemoptysis and require bronchial endoscopy for their diagnosis.

Adrenobronchial fistula complicating a neonatal adrenal abscess: treatment by percutaneous aspiration and antibiotics

A case of retroperitoneal pulmonary fistula caused by a neonatal adrenal abscess is reported. The adrenal abscess was diagnosed by means of needle aspiration which guided the choice of antibiotic therapy. The fistula was demonstrated by direct injection of contrast medium into the adrenal abscess. Treatment by needle aspiration of the adrenal abscess and intravenous antibiotics was successful.

Mucoviscidose: les stratégies thérapeutiques se multiplient

Resume Depuis la decouverte du gene defectueux dans la mucoviscidose, des progres tres rapides ont ete accomplis dans la comprehension des fonctions cellulaires de la proteine CFTR ( cystic fibrosis transmembrane conductance regulator ) et des mecanismes regulant son expression. A partir de la caracterisation des processus impliques dans la progression de la maladie au niveau pulmonaire, des projets therapeutiques varies ont ete developpes, fondes sur la therapie genique et sur l’utilisation de differents agents pharmacologiques. Certains de ces agents ont pour role de retablir une fonction CFTR adequate; d’autres agiraient en court-circuitant la proteine CFTR par induction de l’expression de proteines proches. Dans la presente mise au point, les strategies therapeutiques actuellement en cours d’investigation sont discutees

Sarcoidosis of the upper respiratory tract in children

Sarcoidosis is a multisystemic granulomatosis of unknown etiology which mainly affects young adults. It is characterized primarily by bilateral hilar adenopathies, a pulmonary infiltrate and cutaneous and ocular lesions. It rarely occurs in children under the age of 16. Localization in the upper respiratory tract (URT) is infrequent and sarcoidosis of the URT in children is exceptional, with only 13 cases reported in the literature. In the present report we describe the clinical, diagnostic explorations, histological and therapeutic aspects of 2 new cases in children.