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Biomedical subjects

H Trang

Publications and source records attributed to H Trang.

13 recordsLinked to original sources

Long-term noninvasive mechanical ventilation for children at home: a national survey.

Experience with domiciliary noninvasive mechanical ventilation (NIMV) in children is limited. The aim of this study was to determine the number of patients and categorize the use of domiciliary NIMV in children in France. An anonymous cross-sectional national study was performed, using a postal questionnaire sent to all specialist centers utilizing domiciliary NIMV for chronic respiratory failure. Patients aged <18 years and receiving home NIMV were included in the study. Detailed information was obtained from 102 patients from 15 centers: 4/15 centers cared for 84% of patients; 7% of patients were under 3 years old; 35% were between 4-11 years old; and 58% were older than 12 years. Underlying diagnoses included neuromuscular disease (34%), obstructive sleep apnea (OSA) and/or cranio-facial abnormalities (30%), cystic fibrosis (17%), congenital hypoventilation (9%), scoliosis (8%), and other disorders (2%). NIMV was started because of nocturnal hypoventilation (67%), acute exacerbation (28%), and/or failure to thrive (21%). Volume-targeted ventilation was preferred in restrictive disorders (56%) and central hypoventilation (56%), while pressure support ventilation (PSV) was preferred in cystic fibrosis (71%). Patients with OSA and/or cranio-facial abnormalities were ventilated with continuous positive airway pressure (45%) or bilevel PSV (52%). In conclusion, NIMV is currently used in France for the domiciliary management of children with a variety of conditions causing chronic respiratory failure. However, NIMV in children is still performed on a small scale, and combined with the heterogeneity of the patient population, its application would best be served by centers specializing in the initiation and long-term follow-up of these patients.

Adolescent↗

Alleles of the estrogen receptor alpha-gene and an estrogen receptor cotranscriptional activator gene, amplified in breast cancer-1 (AIB1), are associated with quantitative calcaneal ultrasound.

Quantitative bone ultrasound (QUS) has a significant heritable component. Because estrogen is required for attainment of peak bone mass, we studied alleles of two genes, estrogen receptor alpha (ER1) and amplified in breast cancer-1 (AIB1), for their association with QUS. In a volunteer sample of 663 white women aged 18-35 years, bone ultrasound attenuation (BUA), speed of sound (SOS), and heel stiffness index (SI), the latter consisting of the component measures of BUA and SOS, were measured at the right calcaneus by QUS. Subjects were genotyped for the ER1 polymorphisms Xba I and Pvu II and for the AIB1 polyglutamine tract polymorphism. In a multiple regression analysis, ER1 genotype was an independent predictor of QUS-SI (p = 0.03). Because AIB1 and ER1 enhance gene expression in a coordinate manner, we also searched for interactions. A gene-by-gene interaction effect was seen for QUS-SI (p = 0.009), QUS-BUA (p = 0.03), and QUS-SOS (p = 0.004). These remained significant after the inclusion of clinically relevant variables into the final regression model. Overall, these clinical and genetic factors accounted for up to 16% of the variance in peak QUS; the genetic markers alone accounted for 4-7%. This is the first demonstration of specific genetic effects on calcaneal QUS encoded by alleles of genes directly involved in mediating estrogen effects on bone.

Adolescent↗

A new look at the management of the oculo-mandibulo-facial syndrome.

The authors review the literature on the oculo-mandibulo-facial syndrome and present the case of a six-year-old boy with congenital cataracts, microphthalmos, nystagmus, failure to thrive, dysmorphic features with a tiny pinched nose, mandibular hypoplasia, microstomia, double chin, chronic snoring, recurrent respiratory infections and dental problems. Chronic obstructive sleep apnoea with decreased oxygen saturation was present. Optimal medical management of OMFS-patients is described.

Abnormalities, Multiple↗

The pharmacokinetics of droperidol in anesthetized patients.

A pharmacokinetic study of droperidol was performed in ten anesthetized patients receiving an intravenous bolus dose of 150 micrograms/kg of droperidol. Plasma concentrations were measured using a specific radioimmunoassay method. The pharmacokinetics of droperidol can be described according to a three-compartment open model. The mean (+/- SD) half-life for the rapid (t 1/2 pi) and slow distribution t 1/2 alpha) phases was 1.4 +/- 0.5 min and 14.3 +/- 6.5 min, respectively. The mean elimination half-life, t 1/2 beta was 103.8 +/- 20.2 min. The mean (+/- SD) total body clearance was 14.1 +/- 4.4 ml X min-1 X kg-1, and the total apparent volume of distribution (Vd beta) was 2.04 +/- 0.50 l/kg. The short terminal half-life of droperidol does not correlate with the well-known, relatively prolonged duration of its pharmacologic action.

Adult↗

Pharmacokinetics of phenoperidine in anaesthetized patients undergoing general surgery.

The pharmacokinetics of phenoperidine have been studied in five anaesthetized patients receiving a 2-mg bolus dose i.v. Plasma concentrations were measured using a sensitive radioimmunoassay method. The distribution of phenoperidine was described according to a two-compartment open model. The mean distribution half-life (T1/2 alpha) for the five patients was short (2.2 min); the mean elimination half-life (T1/2 beta) was 193 min. The mean whole body clearance was 22 ml min-1 kg-1 and the apparent steady state distribution volume (VSS) was 5.7 litre kg-1. Secondary concentration peaks occurred in all patients; in two patients these were substantial and occurred 80 min after injection.

Anesthesia, General↗

Pharmacokinetics of phenoperidine in patients undergoing cardiopulmonary bypass.

Phenoperidine concentrations were studied, using radioimmunoassay, in five patients submitted to coronary artery bypass graft surgery. Administration of phenoperidine consisted of a 5-mg bolus dose followed by constant infusion of 5 mg h-1. Before cardiopulmonary bypass, phenoperidine concentrations were stable in an individual patient, but there was a large scatter between patients. The concentrations decreased immediately following the start of the bypass, but this decrease was short. During cardiopulmonary bypass, the phenoperidine concentrations increased progressively and were greater at the end of the bypass than before it. The increase in concentration continued following the discontinuation of bypass. The ratios of change of the observed results were in accord with a theoretical evaluation, although the observed concentrations were all greater than those calculated, except at one point. This difference in phenoperidine concentration is probably related to an alteration of liver plasma flow. Haemodilution as a result of the priming of the cardiopulmonary bypass circuit played only a transient role.

Adult↗

[Respiratory form mof myasthenia gravis].

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.

Adolescent↗