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131 results on '"Boule M"'

17. Cytokine polymorphisms influence lung disease progression in cystic fibrosis

Author

Corvol, H., primary, Boelle, P.Y., additional, Brouard, J., additional, Knauer, N., additional, Henrion-Caude, A., additional, Chadelat, K., additional, Flamant, C., additional, Charlier, C., additional, Boule, M., additional, Fauroux, B., additional, Vallet, C., additional, Feingold, J., additional, Ratjen, F., additional, Grasemann, H., additional, and Clement, A., additional

Published
2008
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44. Genotype analysis and phenotypic manifestations of children with intermediate sweat chloride test results.

Author

Desmarquest, Pascale, Feldman, Delphine, Tamalat, Aline, Boule, Michele, Fauroux, Brigitte, Tournier, Guy, Clement, Annick, Desmarquest, P, Feldmann, D, Tamalat, A, Boule, M, Fauroux, B, Tournier, G, and Clement, A

Subjects
LUNG diseases, CYSTIC fibrosis
Abstract

Study Objectives: Cystic fibrosis (CF) is one of the most common inherited diseases among whites. Since the cloning of the CF transmembrane conductance regulator (CFTR) gene, a number of studies have focused on associations between the genotype and phenotype in CF. This had led to the progressive identification of new groups of patients, including those who have mild lung disease and those who have normal sweat chloride values (< 60 mEq/L). The aim of the present work was to provide information on the genotype and the phenotypic characteristics of children with intermediate-range sweat chloride test results.Patients and Results: We focused on children referred to the pulmonary department for various types of pulmonary disease and who had several sweat chloride test results with median values in the range of 40 to 60 mEq/L. Twenty-four patients over a 10-year period were enrolled (mean age, 4.8 years). Respiratory manifestations at initial evaluation included recurrent bronchitis, wheezing, chronic cough, and pneumonia. The duration of the follow-up ranged from 0.5 to 10.5 years. Sputum cultures revealed the presence of Haemophilus influenzae (10 children), Staphylococcus aureus (4 children), and Pseudomonas aeruginosa (3 children). Pancreatic insufficiency was found in two patients. Analysis of the entire coding sequence allowed identification of 16 known mutations in CFTR gene. Fifteen chromosomes (31.2%) carried a mutation in CFTR gene and one allele carried two mutations. Three patients were homozygous or double heterozygous (DeltaF508/DeltaF508, DeltaF508/3849 + 10 kb C-->T, S1235R/G551D). The 5-thymidine allele was identified in four children.Conclusion: These results indicate an higher frequency of CFTR gene mutations in patients with borderline sweat chloride test results, compared to data reported in the general population. They lead to the recommendations for complete pulmonary and GI investigations in this group of patients, as well as assiduous care and medical follow-up. [ABSTRACT FROM AUTHOR]

Published
2000
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49. Control of Breathing in Children with Interstitial Lung Disease

Author

Gaultier, Cl, Perret, L, Boule, M, Tournier, G, and Girard, F

Abstract

Summary: Control of breathing at rest was studied in 14 children (4–16 years old) with interstitial lung disease (ILD). Four of them were tested several times. Breathing pattern and the mouth occlusion pressure (P0.1) were measured. Results in ILD children were compared to values in healthy children previously reported. Respiratory frequency and minute ventilation were increased (P < 0.02). Inspiratory time (Tl) was shortened(P < 0.001), the shortening in Ti being significantly related to the increase in lung elastance (EL). The ratio of Tl to the total duration of the respiratory cycle (Tl/TTOT) was lowered (P < 0.01). Tidal volume (VT) both in ml and normalized for body weight (BW) was normal. P0.1was higher than predicted. The increase in P0.1was significantly related to change in arterial O2pressure (PaO2) which was reduced in 10 cases. There was a significant relationship between the increase in P0.1and in EL. The increase in mean inspiratory flow (VTBW/Tl.) was related to the increase in EL. But VTBW/TI was not increased as much as P0.1. Consequently the effective inspiratory impedance was enhanced. This high effective inspiratory impedance was related to the increased lung elastance.Speculation: Increase in the neural drive in ILD children appears to be due to hypoxemia and/or to the increased elastic load. The effects of the elastic load on the control of breathing are probably vagally-mediated.

Published
1982
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50. Ventilatory Strategy in Hypoxic or Hypercapnic Newborns

Author

Bonora, M., Boule, M., and Gautier, H.

Abstract

In conscious newborns, the ventilatory response to hypoxia is characterized by precocious hyperventilation followed by tardive hypoventilation, the latter disappearing with age. The hypoventilation could be mainly related to a weak peripheral drive and to the persistence of the diaphragmatic activity during expiration. Also, a decrease in metabolic rate and body temperature interferes with the response. The hyperventilation in response to hypercapnia increases as maturation proceeds and the maturation of the peripheral chemoreceptors contribute to this effect, as during hypoxia. The responses to both stimuli depend on many factors such as sleep state, anesthesia or ambient temperature.

Published
1994
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