Sex differences in thoracic adaptation to pulmonary hyperinflation in cystic fibrosis.

Sex differences in thoracic adaptation have been reported in patients with cystic fibrosis (CF). The interplay between the pattern of thoracic adaptation and the function of the respiratory muscles in male and female CF patients with pulmonary hyperinflation was investigated. Thoracic dimensions and diaphragm length were measured at residual volume, functional residual capacity and total lung capacity using chest radiography in 23 CF (12 males) and 18 normal (11 males) subjects. Respiratory muscle recruitment during resting breathing was assessed by recording intrathoracic and intra-abdominal pressures. In female CF patients, ribcage expansion was predominant, tending to preserve diaphragm length. In male CF patients, thoracic configuration was normal and diaphragm shortening consequently greater. Ribcage cross-sectional area for a given rib inclination was greater in CF patients, indicating a structural expansion of the ribcage 2.5 times greater in females than males. The contribution of inspiratory ribcage muscles to inspiratory pressure was also greater relative to the diaphragm in the CF group. In conclusion, a structural expansion of the ribcage occurs in cystic fibrosis patients with lung hyperinflation that is greater in females than males. This is associated with an apparent greater contribution of inspiratory ribcage muscles to inspiratory pressure.

Diabetes: a major co-morbidity of cystic fibrosis.

Cystic fibrosis-related diabetes (CFRD) is a frequent complication of cystic fibrosis, its prevalence increases with age of patient and is close to 30% at the age of 30 years. As life expectancy greatly increases, the number of cystic fibrosis patients developing diabetes will increase too. CFRD shares some features with type 1 and type 2 diabetes, initial phase is characterised by postprandial hyperglycaemia followed by a progression toward insulin deficiency. Insulin deficiency is an essential factor in the development of diabetes with an additional contribution of insulin resistance. Systematic screening with an oral glucose tolerance test is recommended from the age of 14 years because clinical signs of CFRD are often confused with signs of pulmonary infection and CFRD occurrence is associated with weight and pulmonary function deterioration. In observational studies CFRD diagnosis is associated with a significant increase in mortality, while treatment allow correction of weight and lung deterioration suggesting that CFRD has a significant impact on CF evolution. Microvascular complications are recognised, although paucity of data does not permit a clear description of their natural history. Annual screening for microvascular complication is recommended. There is no evidence by now that CF patients develop macrovascular complications. The only recommended pharmacological treatment is insulin therapy.

Carcinoma of the lung in three brothers and two sisters.

Documentation of familial aggregation of five siblings with lung cancer is presented. Lung cancer was not observed in their parents nor among their children, which is consistent with the hypothesis of recessive inheritance, although strong environmental factors such as tobacco smoking may contribute to the development of lung cancer in such families.

[Yellow nail syndrome. Apropos of a case. A review of the main radiologic manifestations]. / Le syndrome des ongles jaunes. A propos d'un cas. Revue de ses principales manifestations radiologiques.

The authors report a case of "yellow nail syndrome" and emphasize the radiological features of this recently described entity which physiopathology has not yet been established. A new feature to be described: a non symptomatic pericardial effusion in association with long standing bilateral pleural effusions.

[Extrinsic allergic alveolitis and hematologic diseases (lymphoproliferative syndromes)]. / Alvéolite allergique extrinsèque et affections hématologiques (syndromes lympho-prolifératifs).

The authors report 2 cases of hypersensitivity pneumopathy associated with haematological abnormalities: benign lymphoproliferative syndrome in one case, and malignant lymphadenopathy with fatal outcome in the other case. Such cases, now observed with increasing frequency, can be regarded either as chance associations of 2 types of disease, or as the results of immune disorders with repercussions on the blood.

Leukocyte migration inhibition in farmer's lung.

The leukocyte migration inhibition test (LMIT) was studied in 20 patients with farmer's lung (FL) and in 24 asymptomatic farmers. In the first group, the test proved to be 95% positive (19/20), whereas it was positive in only 44% (11/25) of the control group; this difference was statistically significant (p less than 0.005). The LMIT was also studied in eight patients with FL who had not been in contact with the antigen during the year prior to testing and was found to be 87% positive (7/8). The test therefore proved to be more effective in the diagnosis of FL compared with tests for specific antibodies, even when subjects had not been in contact with the antigen for 1 yr.

[Recent findings on occupational allergic alveolitis]. / Acquisitions récentes sur les alvéolites allergiques professionnelles.

Occupational allergic alveolitis may be observed in agricultural or industrial environment. The presence of serum precipitins to specific antigens remains a valuable symptom. But other immunologic tests, such as inhibition of leucocyte migration, may be performed in doubtful cases or for retrospective diagnosis. It shows the role of delayed hypersensitivity in the pathogenesis of the disease. The functional investigation confirms the alveolar involvement but also reveals in some cases (by flow/volume curves and compliance tests) evidence of small airways disease. The broncho-alveolar lavages show the high percentage of lymphocytes (mainly T lymphocytes) in in the cell population recovered and a very typical phospholipid profile in the supernatant (total absence of lecithin whereas the two other fractions phosphatidyl-inositol and phosphatidyl-ethanolamine are considerably enhanced). The tensioactive properties of the phospholipids were investigated by fluorescence polarisation technique. High values of microviscosity were found in patients. These findings provide a new diagnostic guidance and a new therapeutic approach of these diseases.

[Doxapram. Pharmocokinetic and clinical study]. / Le doxapram. Etude pharmacocinétique et clinique.

Two series of work are reported. The first concerns the pharmacocinetic study of the product after it was administered through different ways and the corresponding evolution of PaCO2. The second study concerns the effect on pulmonary arterial pressure, blood gases and some parameters of ventilatory mechanics, of a perfusion of 200 mg of this product over a two hours period.

[Applications of bidimensional immunoelectrophoresis to hypersensitivity pneumonia by inhalation of organic antigens. Its interest in relation to the classical immunodiffusion and immunoelectrophoresis methods (author's transl)]. / Applications de la technique d'immunoélectrophorèse bidimensionnelle aux pneumopathies d'hypersensibilité par inhalation d'antigènes organiques. Son intérêt par rapport aux méthodes classiques d'immunodiffusion et d'immunoélectrophorèse

Bidimensional electrophoresis is of most interest for the diagnosis of extrinsic allergic alveolitis. In fact, this detecting method is more specific than classical immunoelectrophoresis. Thus patients showing with the classical methods precipitation lines with one or several antigens do not present everytime the corresponding clinical signs. These positive but false reactions cannot in any case appear with bidimensionnal electrophoresis. Generally, the patient showing at least two precipitation lines opposite one antigen in bidimensional electrophoresis always followed by an increase of IgG, does also present the clinical signs of the disease.