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Biomedical subjects

A Clément

Publications and source records attributed to A Clément.

At least 37 records · Page 2Linked to original sources
PubMed2000

[Comparison of behavior characteristics of the spontaneously alert state and the liberated state in the term newborn].

Occupational therapists evaluate the capacities of infants at younger ages than ever before. The variability of the infants' state has an inevitable influence on the expression of their behaviours. In order to counter the effect of this instability, Grenier (1985) suggests inducing the "liberated state," which optimizes the infant's level of attention. The aim of this study was to identify the behaviours that discriminate this liberated state from the alert state (state 4) as described by Brazelton (1973). Some of the parameters that were defined in the Synactive Theory of Development Model (Als, 1982) were used in order to document the behaviours of the infants observed in the two different states. The average age of the infants, at the time of observation, was 62 hours. The duration of the observation was 5 minutes. Among the 54 newborns that were evaluated, 21 were observed in the two states under study; 33 were evaluated in one state only. The results confirm a greater stability of the "liberated state". Stress and self-regulation behaviours, and the need for external regulation are less frequent in this state; these differences are statistically significant (p < 0.05). Goal-directed movements are more frequent in the "liberated state" (p < 0.001). The authors suggest the use of the "liberated state" to further document the early capacities of infants.

Attention↗
PubMed1999

[Clinical and respiratory function follow-up of 39 infants treated with neonatal respiratory extracorporeal assistance].

UNLABELLED: The aim of this prospective study was to evaluate the consequences of neonatal treatment with a venovenous extracorporeal respiratory assistance. POPULATION AND METHODS: Thirty nine neonates (28 boys) with acute respiratory failure were included. Extracorporeal respiratory assistance consisted of an apnoeic oxygenation with low frequency positive pressure ventilation and extracorporeal membrane CO2 removal through a venous single canula perfusion circuit. The causes of respiratory distress were: 15 meconium aspiration syndrome, 12 respiratory distress syndrome, six hyaline membrane disease, three sepsis, two diaphragmatic hernia, and one post-surgery Mendelson syndrome. The mean duration of mechanical ventilation was 18 days, including 5 days of extracorporeal respiratory assistance. The prospective follow-up included physical examination, chest radiographs, scintigraphy and pulmonary function tests. These tests studied the following parameters: functional residual capacity by helium dilution technique, lung resistance and dynamic lung compliance by the esophageal balloon technique and blood gases with arterialized blood samples. RESULTS: The mean duration of the follow-up was 21.3 months (6 months to 5 years). Results showed in the first year 33% of children with chronic obstructive pulmonary disease and chest (X-ray abnormalities, such as bronchopulmonary dysplasia in 23% of children. Data of pulmonary function test at the end of the first year: lung resistance and functional residual capacity were within limits of predicted values for height, and dynamic lung compliance was slightly decreased; according to the analysis of the functional profile: 31% without abnormality, 41% of obstructive syndrome and 26% with restrictive pattern. Blood gases were normal in 37 children. At the end of the second year, we noticed normal functional residual capacity, an increase of lung resistance while lung compliance was normalized; functional profile was quite different: with a decrease of the number of patients without abnormality (22%) and increase of those with obstructive syndrome (56%). CONCLUSION: The percentage of abnormalities is high but these are moderate in most cases, especially if we compare with the initial seriousness of the pulmonary pathology. We suggest a regular follow-up to study the respective incidence of pulmonary disease and/or extracorporeal respiratory assistance over these abnormalities.

Acute Disease↗
PubMed1999

Loss-of-function mutations in a human gene related to Chlamydomonas reinhardtii dynein IC78 result in primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is a group of heterogeneous disorders of unknown origin, usually inherited as an autosomal recessive trait. Its phenotype is characterized by axonemal abnormalities of respiratory cilia and sperm tails leading to bronchiectasis and sinusitis, which are sometimes associated with situs inversus (Kartagener syndrome) and male sterility. The main ciliary defect in PCD is an absence of dynein arms. We have isolated the first gene involved in PCD, using a candidate-gene approach developed on the basis of documented abnormalities of immotile strains of Chlamydomonas reinhardtii, which carry axonemal ultrastructural defects reminiscent of PCD. Taking advantage of the evolutionary conservation of genes encoding axonemal proteins, we have isolated a human sequence (DNAI1) related to IC78, a C. reinhardtii gene encoding a dynein intermediate chain in which mutations are associated with the absence of outer dynein arms. DNAI1 is highly expressed in trachea and testis and is composed of 20 exons located at 9p13-p21. Two loss-of-function mutations of DNAI1 have been identified in a patient with PCD characterized by immotile respiratory cilia lacking outer dynein arms. In addition, we excluded linkage between this gene and similar PCD phenotypes in five other affected families, providing a clear demonstration of locus heterogeneity. These data reveal the critical role of DNAI1 in the development of human axonemal structures and open up new means for identification of additional genes involved in related developmental defects.

Amino Acid Sequence↗
PubMed1999

Chest physiotherapy in cystic fibrosis: improved tolerance with nasal pressure support ventilation.

OBJECTIVE: Chest physiotherapy (CPT) is an integral part of the treatment of patients with cystic fibrosis (CF). CPT imposes additional respiratory work that may carry a risk of respiratory muscle fatigue. Inspiratory pressure support ventilation (PSV) is a new mode of ventilatory assistance designed to maintain a constant preset positive airway pressure during spontaneous inspiration with the goal of decreasing the patient's inspiratory work. The aim of our study was 1) to evaluate respiratory muscle fatigue and oxygen desaturation during CPT and 2) to determine whether noninvasive PSV can relieve these potential adverse effects of CPT. METHODS: Sixteen CF patients in stable condition with a mean age of 13 +/- 4 years participated to the study. For CPT, we used the forced expiratory technique (FET), which consisted of one or more slow active expirations starting near the total lung capacity (TLC) and ending near the residual volume. After each expiration, the child was asked to perform a slow, nonmaximal, diaphragmatic inspiration. After one to four forced breathing cycles, the child was asked to cough and to expectorate. A typical 20-minute CPT session consisted of 10 to 15 FET maneuvers separated by rest periods of 10 to 20 breathing cycles each. During the study, each patient received two CPT sessions in random order on two different days, at the same time of day, with the same physiotherapist. During one of these two sessions, PSV was provided throughout the session (PSV session) via a nasal mask using the pressure support generator ARM25 designed for acute patients (TAEMA, Antony, France). The control session was performed with no nasal mask or PSV. Both CPT sessions were performed without supplemental oxygen. Lung function and maximal inspiratory pressures (PImax) and expiratory pressures (PEmax) were recorded before and after each CPT session. RESULTS: Mean lung function parameters were comparable before the PSV and the control sessions. Baseline pulse oximetry (SpO2) was significantly correlated with the baseline vital capacity (% predicted) and forced expiratory volume in 1 second (FEV1) (% predicted). PSV was associated with an increase in tidal volume (Vt) from 0.42 +/- 0.01 liters to 1.0 +/- 0.02 liters. Respiratory rate was significantly lower during PSV. SpO2 between the FET maneuvers was significantly higher during PSV as compared with the control session. SpO2 decreases after FET were significantly larger during the control session (nadir: 91.8 +/- 0. 7%) than during the PSV session (93.8 +/- 0.6%). Maximal pressures decreased during the control session (from 71.9 +/- 6.1 to 60.9 +/- 5.3 cmH2O, and from 85.3 +/- 7.9 to 77.5 +/- 4.8 cmH2O, for PImax and PEmax, respectively) and increased during the PSV session (from 71.6 +/- 8.6 to 83.9 +/- 8.7 cmH2O, and from 80.4 +/- 7.8 to 88.0 +/- 7.4 cmH2O, for PImax and PEmax, respectively). The decrease in PEmax was significantly correlated with the severity of bronchial obstruction as evaluated based on baseline FEV1 (% predicted). Forced expiratory flows did not change after either CPT session. The amount of sputum expectorated was similar for the two CPT sessions (5.3 +/- 5.3 g vs 4.6 +/- 4.8 g after the control and PSV session, respectively; NS). Fifteen patients felt less tired after the PSV session. Ten patients reported that expectoration was easier with PSV, whereas 4 did not note any difference; 2 patients did not expectorate. Nine patients expressed a marked and 5 a small preference for PSV, and 2 patients had no preference. The physiotherapists found it easier to perform CPT with PSV in 14 patients and did not perceive any difference in 2 patients. DISCUSSION: Our study in CF children shows that respiratory muscle performance, as evaluated based on various parameters, decreased after CPT and that significant falls in oxygen saturation occurred after the FET maneuvers despite the quiet breathing periods between each FET cycle. These unwanted effects of CPT were

Adolescent↗
PubMed1999

[Pathophysiology of acute thoracic syndrome].

Pulmonary complications are the leading cause of morbidity and mortality in sickle cell disease patients. Acute chest syndrome (ACS), in which chest pain and dyspnea, occurs in combination with a recent chest radiograph abnormality, raises both diagnostic and therapeutic challenges. The pathogenesis of ACS involves alterations in blood rheology, increased coagulability, and, above all, increased adhesion of sickle cells to the vascular endothelium and nitric oxide-mediated dysregulation of vascular reactivity. Sickle cell disease thus impacts all the cells in the vascular environment. Recently gained insights into pathophysiology offer hope that new treatments for preventing and treating acute and chronic pulmonary complications will soon become available.

Acute Disease↗
PubMed1999

[Computed tomography imaging of abdominal wall metastases after laparoscopy].

We report a case of abdominal wall metastases which occurred after diagnostic laparoscopy in a 64-year-old man presenting with gallbladder carcinoma and peritoneal carcinomatosis. CT showed enhancing masses along the laparoscopic tracts, suggesting metastatic seeding at the time of the surgery. This unusual complication fuels the current debate regarding indications of laparoscopic surgery in patients with malignancy.

Abdominal Muscles↗
PubMed1998

[Mucoviscidosis: therapeutic strategies are multiplying].

Since the cloning of the defective gene in cystic fibrosis, much has been learned on the function of CFTR and on the mechanisms regulating its expression. Based on the current understanding of the processes involved in lung disease progression, a number of approaches have been developed using gene therapy and pharmacological agents. Several of these agents have been reported to restitute a function to CFTR with specific mutations. Other molecules act on channels other than CFTR, and may be effective by bypassing CFTR itself. In the present review the various therapeutical strategies currently investigated are discussed.

Child↗
PubMed1998

[Value of high-frequency (20 MHz)in the diagnosis of cutaneous tumors].

The purpose of the present study is to asses the value of high frequency ultrasonography (20 MHz), a new noninvasive imaging technique, in cutaneous tumors. Cutaneous tumors are clinically varied and their diagnosis is still based on histopathological analysis. 140 cutaneous tumors have been examined with US and imaging findings have been compared to the results of clinical and histological examinations. This study shows that high frequency ultrasonography, even though it cannot replace pathological analysis, may help the dermatologist in the positive diagnosis of some cutaneous tumors and in accessing the location and the in depth-extension of the lesions in the different layers of the skin.

Adolescent↗
PubMed1998

[Imaging of adrenal incidentalomas].

Imaging plays an important role in evaluating fortuitously discovered adrenal masses. These unanticipated masses (incidentalomas) are usually discovered at CT examinations. Although most incidentally discovered adrenal tumors are non-hyperfunctioning adenomas, they must be distinguished from primary or secondary malignant lesions. Recent studies have established that both unenhanced CT scans and chemical shift MR imaging can accurately differentiate benign adenomas from nonadenomatous masses by features that reflect the large amount of lipid found in most adenomas and absent in most nonadenomas. We review here the current imaging issues concerning adrenal incidentalomas including diagnostic approaches and management.

Adenoma↗
PubMed1997

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.

Adolescent↗
PubMed1996

[Pulmonary toxicity of drugs and thoracic irradiation in children].

The pathology of drug-induced pulmonary toxicity in children is poorly understood and probably under-estimated, in the absence of any prospective studies evaluating in a systematic fashion the side effect of medication on the respiratory apparatus. The pulmonary toxicity of thoracic irradiation has markedly receded with more restricted indications for this sort of treatment. Three clinical patterns are most commonly encountered in drug induced lung disease in children: interstitial lung disease, hypersensitivity lung disease and non-cardiogenic pulmonary oedema. The diagnosis is a diagnosis of exclusion and rests on a group of clinical arguments and also on the progress of the disease. Broncho-alveolar lavage rules out infectious disease. Respiratory function tests show non-specific anomalies. A lung biopsy may be indicated. The mechanism of the pulmonary toxicity are associated with disequilibrium of the oxidant/antioxidant and protease/antiprotease system as well as disturbance of the immune response or alteration of the pulmonary matrix by disease of the collagen system. Increased toxicity may be seen in children because of a very significant cumulative dose. The cytotoxic drugs which are most often implicated in causing this are bleomycin, methotrexate, cyclophosphamide and busulfan. Other drugs which are responsible for toxic lung disease are nitrofurantoin, sulfasalazine, D-penicillamine, betalactams, Diphenyl-hydantoin and carbamazepine. Acute post-radiation lung disease is rare. Post-radiation fibrosis is found six months after irradiation and hinders thoraco-pulmonary growth in the child. It is important to assess lung function in all children before any chemotherapy or thoracic irradiation. Cytotoxic drugs are the most common cause of toxic lung disease. This iatrogenic disease requires a multi-discipline approach to ensure the quality of care for these children.

Anti-Bacterial Agents↗
PubMed1996

[Bronchopulmonary dysplasia].

Bronchopulmonary dysplasia (BPD), a respiratory disorder first described in prematurely born infants with respiratory distress syndrome (RDS) treated with mechanical ventilation and oxygen supplementation, is the most common cause of chronic lung disease in infants. It is defined as the need for increased inspired oxygen at 28 days of age, and is observed with the highest frequency following premature delivery of very low birth weight infants. Indeed, an incidence of 48% has been recently reported in a population with a mean gestational age of 27 weeks. The etiology of BPD is multifactorial including lung immaturity, respiratory distress, oxygen therapy, and mechanical ventilation. Based on the current understanding of the pathogenesis of the disease, several therapeutical strategies are used. One of them is focused on the prevention of BPD by correcting surfactant deficiency in premature infants with RDS using exogenous surfactant, and also by improving the techniques of mechanical ventilation used for the management of RDS. Another approach which is being developed is focused on the factors involved in the processes of repair of the injured immature lung. These factors include the use of inhibitors of the inflammatory cascade, antioxidants, and inhibitors of fibrosis.

Anti-Inflammatory Agents↗
PubMed1995

[Oxidants and antioxidants. Biological effects and therapeutic perspectives].

The main oxidizing free radicals (FR) are oxygen-derived metabolites (superoxide anion O2-, hydrogen peroxide H2O2, hydroxyl radical OH (zero)), hypochloric acid, chloramines, nitrogen dioxide, ozone and lipid peroxides. They are produced continually by living organisms, either in the intracellular compartment by the mitochondrial respiratory chain and mixed function oxidase system, or in the extracellular compartment, especially by phagocytes. The body possesses complex protective antioxidant systems against this potentially toxic production, such as dismutase superoxides, catalase, the glutathione enzyme system, metallic ion sequestration, enzymes degrading proteins injured by FR, metabolising hydroperoxides, and repairing DNA, and vitamins E, C, P, and betacarotene. A physiological steady-state is established under normal conditions between the production of oxidants and their neutralization by antioxidants. Oxidative lesions result from a disturbance of the oxidant-antioxidant balance. Oxygen-derived metabolites act on polyunsaturated cell membrane lipids, induce genetic alterations, and oxidize sulfhydryl groups of proteins, thereby modifying their functions. FR are involved in major physiological mechanisms such as phagocytosis, the inflammatory reaction, and the reperfusion ischaemia phenomenon observed during organ storage. The therapeutic use of enzymatic (SOD, catalase, GSH) and nonenzymatic antioxidants (vitamins E, N-acetylcysteine, allopurinol and oxypurinol) has yet to be evaluated. The current state of our knowledge indicates the extreme complexity of these systems and calls for caution in the therapeutic use of antioxidant substances.

Antidotes↗
PubMed1994

Cytotoxic drug-induced pulmonary disease in infants and children.

The increased survival rate of malignant diseases due to more aggressive treatments contributes to the occurrence of drug-induced pulmonary diseases (DIPD). We reviewed, retrospectively over a 10-year period, 15 children (8 girls) who presented a DIPD. Their mean age was 9 years (range, 1 to 17 years), with an underlying malignant disease in 14 (9 leukemias). Three typical patterns have emerged from this analysis: (1) acute hypersensitivity lung disease caused by methotrexate (in 6 patients) or azathioprine (in 1 patient). This acute syndrome consisted of alveolar-interstitial infiltrate with a hypercellularity on bronchoalveolar lavage (BAL) (mean, 714,286 cells/mL; range, 180,000-2,940,000 cells/mL) and an increase of lymphocyte counts (mean, 39%; range 11-64%) with predominantly CD8-suppressor/cytotoxic lymphocytes. Inhibition of leukocyte migration or leukocyte aggregation in the presence of low drug concentrations was positive in the 5 cases tested. Lung function tests showed a restrictive pattern and the outcome of DIPD was always favorable. (2) Chronic pneumonitis/fibrosis was seen in 6 patients who received a variable association of cyclophosphamide (3 patients), bleomycin (2 patients), BCNU (2 patients), and melphalan (1 patient). Symptoms of an alveolar-interstitial pneumonitis developed progressively. BAL showed a moderate increase of total cell numbers (mean, 495,000 cells/mL; range, 150,000-900,000 cells/mL). Lung function tests showed a restrictive pattern. Despite corticosteroid treatment in 4 children, one died after bleomycin lung injury and 2 had functional lung impairment. (3) Noncardiogenic pulmonary edema occurred in 2 patients with leukemia treated with recombinant interleukin II. BAL showed hypercellularity and outcome was rapidly favorable.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed1992

[Langerhans-cell histiocytosis of the vulva. General review and a case history].

Localisation of Langerhans' cell histiocytosis (HCL) is rare. In solitary form the prognosis for this pathology is good, if the tumour occurs in a woman of 36 years of age. (In this case the woman's age was 91) The appearances may be misleading clinically. The diagnosis is made by histological examination of P-S 100 positive cells and by finding Birbeck's granules on electron microscopy. Treatment may be surgical or as in our case medical.

Administration, Topical↗