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Biomedical subjects

F Girard

Publications and source records attributed to F Girard.

At least 181 records · Page 10Linked to original sources

[Pulmonary function and clinical pattern in homozygous (PiZ) alpha1-antitrypsin deficiency (author's transl)].

A group of 6 males with severe alpha1-antitrypsin deficiency, underwent clinical and pulmonary function evaluation. Findings were compared to those in a group of males with different degrees of airflow obstruction, comparable ages and tobacco consumption, but with normal serum levels of alpha1-antitrypsin. The deficient group was characterized by: (1) a relatively early appearance of symptoms; (2) disturbed lung scans, mostly in the basal zones; (3) radiological evidence, in most cases, of pulmonary emphysema with, in particular, bullae in the lower lung zones; (4) hypoxemia without hypercapnia and a decreased TCO/VA, and (5) a more or less severe reduction of maximal expiratory flows largely, but not exclusively due to a decrease in lung elastic recoil. Clinical and functional parameters did not permit a clear distinction between the deficient and non-deficient groups.

Adult↗

Lung function in children with recurrent bronchitis.

Lung function was studied in 29 children suffering from recurrent bronchitis (average number of bronchitis attacks per year: 4.2 +/- 0.5). Most of them (73%) were 1--7 years old. The date of exploration in relation to the last acute bronchitis was on average 6 weeks. Lung mechanics, residual functional capacity, blood gases and regional lung function were measured. Most of the children were suffering from some kind of functional disorder; alteration of lung mechanics -- increase of lung resistance (22/29) decrease of dynamic compliance (15/23); increase of functional residual capacity (6/10), non specific bronchial hypersensitivity (12/17); regional ventilatory and perfusion malfunction. These functional disorders could be the first evidence of the bronchial lesions which cause chronic obstructive bronchitis in adults.

Bronchitis↗

Transient partial hGH deficiency in prepubertal children with delay of growth.

UNLABELLED: The hGH response to an ornithin or an insulin test was measured in 105 children from 11-18 yr old with delay of growth more than 2 SD. Besides 74 subjects with normal values and 7 with complete lack of response, 24 subjects exhibited a partial rise of GH. Most of the latter had decreasing growth rate and no sign of puberty. Out of 15 assessed for GH function after onset of puberty, 14 showed a normal response accompanying a markedly increased growth velocity. Four other subjects with partial response who were receiving sexual hormones for 48 hr before the second test showed a normal response also. An exogenous hGH treatment administered in two of these patients resulted in a marked and sustained growth increase far before entering puberty. SPECULATION: The findings that plasma GH responses to stimulation tests is reduced in some children with decreasing growth rate before puberty and return to normal after onset of sexual development, would suggest a transient and functional defect in growth hormone secretion and the physiologic role of sexual hormones on GH release mechanism at that period of life.

Adolescent↗

Characterization of lipotropin-, corticotropin-, and beta-endorphin-immunoreactive materials secreted in vitro by a human pituitary adenoma responsible for a case of Nelson's syndrome.

A human pituitary adenoma responsible for a case of Nelson's syndrome was maintained in organ culture and the incubation medium was examined with four different RIAs; human corticotropin (ACTH), beta-MSH, lipotropin (LPH), and beta-endorphin (beta-End). All four immunoreactivites (IRs) were present in the medium obtained after 24 h of incubation. Gel exclusion chromatography under denaturing conditions (6 m guanidine HCl) revealed several immunoreactive components. Two components having both human beta-MSH (beta-hMSH) and human LPH (hLPH) IR coeluted with beta-hLPH and gamma-hLPH; a component with beta-hMSH IR but no hLPH IR coeluted with [125I]beta-hMSH; a component with human ACTH (hACTH) IR eluted at the position of hACTH. Sephadex G-50 gel exclusion chromatography revealed that approximately 80% and 20% of human beta-End (beta-hEnd) IR were accounted for by components coeluting with beta-hLPH and beta-hEnd, respectively. These data demonstrate the presence in this incubation medium of materials similar to if not identical with beta-hLPH, gamma-hLPH, hACTH, beta-hMSH, and beta-hEnd; they suggest that all of these peptides may be secreted in the circulation of patients with Nelson's syndrome.

Adenoma↗

Immunoreactive beta-MSH in human plasma and in a corticotrophic adenoma culture medium. Its relation to the lipotrophins.

In order to characterize more accurately the relationship between immunoreactive beta-MSH ("BETA-MSH") and the lipotrophins (LPH) we attempted to investigate the gel filtration and the immunological characteristics of "beta-MSH" in the plasma of patients with Nelson's syndrome and Addison's disease as well as in the culture medium from a human corticotrophic adenoma using a sensitive radioimmunoassay from human beta-MSH. When added either to hormone free plasma or to a plasma from a patient with Nelson's syndrome all the human beta-MSH (hbeta-MSH) elutes from a Sephadex G-50 column as a single peak in a volume corresponding to its molecular weight. In contrast plasma "beta-MSH" in 3 patients with Nelson's syndrome and one patient with Addison's disease almost completely elutes in a volume corresponding to a molecular weight range of 6000-10 000; no "beta-MSH" can be detected in its normal elution volume. Drastic pH change (8.2 to 2.3) does not significantly alter the elution pattern. Chromatography of a corticotrophic adenoma culture medium gave a similar pattern of "beta-MSH" with a main peak in the molecular weight range of 6000-10 000. In our radioimmunoassay the culture medium and purified hbeta-LPH gave parallel displacement curves for [125I]hbeta-MSH. It is suggested that hbeta-LPH or a closely related substance is the main material responsible for "beta-MSH" immunoactivity.

Addison Disease↗

17-Hydroxyprogesterone in the cosyntropin test: results in normal and hirsute women and in mild congenital adrenal hyperplasia.

The variations in plasma cortisol, testosterone and 17-hydroxyprogesterone (17-OHP) induced by an im injection of 0.25 mg cosyntrophin were studied in three groups of subjects: 16 healthy women, 16 hirsute women (HW) and 10 mild cases of congenital adrenal hyperplasia (CAH). The basal values of cortisol and testosterone were comparable between the three groups. In the patients with mild CAH, the mean 17-OHP concentration was increased: 483.9 ng/100 ml (113-1200 ng), but it should be noted that the individual values could overlap with the normal concentrations found in the controls and the HW during the luteal phase of the cycle. One hour after the injection of cosyntropin, a massive response of 17-OHP was observed in the mild cases of CAH, the mean basal concentration was multiplied by ten: 4843 ng/100 ml. The minimum concentration reached was 1740 ng/100 ml which is still 3-fold the highest level seen either in normal women (400 ng/ml) or in hirsute women (550 ng/100 ml). Determination of 17-OHP following a short-term ACTH stimulation, therefore provides evidence of partial 21-hydroxylase deficiency.

Adrenal Hyperplasia, Congenital↗

[Value of 17-hydroxyprogesterone determination in exploration of adrenal cortex enzyme deficiencies].

17-hydroxyprogesterone (17-OH-P) was measured in various populations by radioimmunoassay, using a highly specific antibody produce in the rabbit. Dynamic tests were performed with ACTH, dexamethasone and estroprogestative drugs and the role played by the adrenals and the ovaries in 17-OH-P production could be assessed. 17-OH-P determination is of interest in that, it allows the diagnosis of 21-hydroxylase deficiency, where values above 10 ng/ml are often found. Associated with the measure of testosterone and delta 4-androstenedione, it is also useful in the management of the disease. In the mild form of congenital adrenal hyperplasia with late revelation of the symptoms, determination of 17-OH-P following ACTH stimulation allows of relative diagnosis.

Adrenal Hyperplasia, Congenital↗

[Pituitary adenomas with cells containing bêta MSH, alpha and bêta endorphins and without ACTH (author's transl)].

By using immunohistoenzymatic techniques, cells of 4 pituitary adenomas are revealed with beta MSH, alpha and beta endorphins antisera but do not react with 1--24 and 17--39 ACTH antisera. These data suggest that adenomatous cells are able to synthesize and LPH-like molecule without ACTH. The levels of plasmatic LPH and beta MSH, studied by RIA, stay normal. These results are discussed.

Adenoma↗

[Kinetics of the pulmonary clearance of inhaled cadmium and its accumulation in rat liver and kidneys].

Pulmonary clearance rate of cadmium (Cd), inhaled in the form of an aerosol of Cd acetate, is very slow (half-life 53 days). However, results demonstrate that an important part of Cd cleared from the lung is recovered in liver and kidney. Liver binds this metal rapidly but to a limited extent, whereas renal accumulation is slow but continuous throughout the three months following exposure.

Aerosols↗

Criteria for evaluating mucus functions and their disorders in chronic bronchitis.

In chronic bronchitis, disorders of the physical and chemical properties of mucus may contribute to bronchial obstruction. The abnormalities are analysed in sputum collected by physiotherapy. Measurements of the rheological properties (apparent viscosity and strain recovery), content analysis of secretory immunoglobulin A (S-IgA) and of serum albumin, and determination of the transport velocity of sputum on the ciliated frog palate provide reliable indices of the functional capacity of bronchial mucosa. The effectiveness of mucociliary clearance and coughing is analysed in patients by measuring the rate of removal of radioactive particles deposited on proximal airways. Different types of pathological secretions may be observed. In subjects with occasional cough and sputum, the secretions are characterized by a high content of S-IgA and serum albumin, and a high viscosity and low elasticity, reflecting their high degree of cross-linking. In chronic bronchitics, the S-IgA content and viscoelastic properties of sputum decrease as the illness progresses. During infection, purulent sputum exhibits high viscosity and low strain recovery. Such modifications of the rheological properties of mucus may impair mucociliary clearance. The role of the elastic component is predominant. Sputum, characterized by very low strain recovery (SR less than 4 units) or conversely by very high strain recovery (SR greater than 15 units), is transported at a low rate by mucociliary clearance. A marked hyperviscosity (no greater than 200 poises (120 N s m-2)) also appears as a limiting factor of the mucociliary clearance.

Animals↗

Early paradoxical decrease in serum somatomedin activity following injection of growth hormone.

The early effects have been investigated of an intra-muscular growth hormone injection (6 mg/m2) on serum somatomedin activity in 21 children with growth hormone deficiency and in 5 children with constitutional short stature. In cases of hGH total deficiency, there was an early and significant decrease in SM activity, which reached a minimum level 2 hours after the injection (-34% p less than 0.01). After 24 hours, SM activity increased to +64% above basal levels. In cases of hGH partial deficiency and in short, non-hGH deficient children, no similar early changes were observed. Neither was any correlation found between the variations in SM activity and those in hGH or free fatty acid levels. The unexpected early decrease in SM activity following hGH administration seems to be related to GH deficiency.

Child↗

Pituitary-adrenal axis activity in treated congenital adrenal hyperplasia: static and dynamic studies.

The pituitary-adrenal axis activity was evaluated in 43 patients, treated for congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, by measuring plasma ACTH, 17-hydroxyprogesterone (17-OHP), testosterone, and aldosterone. Dynamic studies were performed by injecting 250 micrograms synthetic ACTH im and collecting blood samples 1 h later for steroid analysis. Twelve to fourteen hours after the last hydrocortisone dose given the evening before, plasma ACTH fluctuated widely from less than 10-475 pg/ml, 17-OHP exceeded normal values and varied from 1-275 ng/ml, while testosterone ranged from 3-151 ng/100 ml. The correlations between ACTH and 17-OHP (n equal 61, r equal 0.665, P less than 0.001) and between 17-OHP and testosterone (n = 43, r = 0.761, P less than 0.001) were good, while that between 17-OHP and aldosterone (n = 64, r = 0.512, P less than 0.001) was rather poor. One hour after ACTH injection, the mean level of 17-OHP was significantly increased as compared to the mean basal level [96.8 ng/ml +/- 10.6 (SE) as compared to 67.0 ng/ml +/- 8.1 (SE)]. However, only 12 out of the 48 tests showed a positive response equal to or greater than 100%, and the majority of these responses (10 out of 12) occurred when basal levels of 17-OHP were between 10-70 ng/ml. This suggests that when basal levels fall outside these values, the pituitary-adrenal axis is either too inhibited or too stimulated to react to exogenous ACTH. Of the 48 tests where 17-OHP was measured, 23 had basal level values within these limits, the mean being 40.3 ng/ml. The corresponding mean ACTH level was 99 pg/ml with a wide range (1-230 pg/ml). On the other hand, in prepubertal children who exhibited 17-OHP concentrations between 10-70 ng/ml, testosterone varied from 3-30 ng/100 ml, with a mean of 16.0 ng/100 ml +/- 1.9 (SE) which is not different from the mean level found in normal children [14.0 ng/100 ml +/- 1.3 (SE)]. Thus, under the influence of endogenous ACTH which is moderately increased, 17-OHP concentrations far exceed normal values, whereas plasma testosterone seems to be unaffected.

17-alpha-Hydroxyprogesterone↗

[Recent data on the group of melanotropic and lipotropic pituitary hormones (MSH-LPH) and on the brain morphinomimetic peptides (endorphins)].

It was admitted that human beta-MSH was responsible for the hyper-pigmentation observed in some syndromes associated with ACTH hypersecretion. beta-LPH was a pituitary polypeptide, containing the entire sequence of beta-MSH in its fragment 37-58, and the physiological role of which remained unknown. alpha-MSH and CLIP (Corticotrophin-like Intermediary Peptide) were thought to be specific of certain species possessing a distinct pituitary pars intermedia. Recent data give new insight upon some of these conceptions. beta-MSH seems not to exist in man; it is almost established now that plasma "Immunoreactive beta-MSH" (IR-beta-MSH) is in fact beta- and/or gamma-LPH. In chronic renal failure plasma IR-beta-MSH is elevated because of a decreased plasma disappearance rate, whereas ACTH is normal. Good evidence suggests that both LPH and ACTH are synthesized in the same pituitary cell within a common polypeptidic precursor. Endogenous peptides with morphinomimetic activity (Endorphins) have been isolated from brain and hypophysis; they are all made up of different fractions of beta-LPH-C-terminal fragment 61-91; It is likely that they represent a new class of brain neurotransmitters involved in some functions of the central nervous system, structural similarities suggest that beta-LPH may be the biosynthetic precursor of Endorphins, however such a hypothesis remains to be clearly demonstrated.

Adrenocorticotropic Hormone↗

[Pathophysiology of chronic, bronchial hypersecretion (author's transl)].

The biochemical and rheological analysis of sputum allows one to detect abnormalities of the bronchial secretion and to describe the development of the bronchial disease. The main biochemical components of sputum and their biological activities are first described. The fibrillar structure of the mucus is related to heavy weight glycoproteins and these macromolecules contribute to the viscoelastic properties of the bronchial secretion, by their capacity to combine with other proteins such as secretory immunoglobulin A (S-IgA). As the chronic bronchial hypersecretory state progresses, the glandular synthesis of mucins and/or secretory proteins decreases while the passive filtration of blood-derived proteins, such as serumalbumin, increases. Such biochemical changes are frequently associated with a rise in viscosity and a loss in elasticity of sputum. These rheological modifications result in an impairment of the mucociliary clearance and therefore contribute to airway obstruction. The assessment of different indices such as the equilibrium in mucin secretion, S-IgA/serum-albumin ratio, viscosity and elasticity of sputum and its mucociliary transport rate, should lead to a better knowledge of the defense properties of the bronchial mucosa.

Airway Obstruction↗