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1.
Jose W Ruiz Jose Guzman Marek Polak Adrien A Eshraghi Thomas J Balkany Thomas R Van De Water 《Otolaryngology--head and neck surgery》2006,135(5):792-797
OBJECTIVE: Test the ability of glutathione monoethyl ester (GSH(e)) to protect auditory hair cells against the ototoxic effects of 4-hydroxy-2,3-nonenal (HNE). STUDY DESIGN AND SETTING: Organ of Corti explants were either untreated or treated with one of a series of four concentrations of GSH(e) for one day, then exposed to HNE. Counts of FITC-phalloidin-labeled hair cells determined both HNE ototoxicity and GSH(e) otoprotection. RESULTS: HNE was toxic to hair cells at physiologically relevant levels, eg, 400 muM, and GSH(e) provided a significant level of protection against HNE ototoxicity (P < 0.05) at all levels tested, ie, 1.16 to 9.3 mM. CONCLUSION: GSH(e) protects auditory hair cells from damage and loss initiated by a naturally occurring ototoxic molecule, ie, HNE (a by-product of oxidative stress). SIGNIFICANCE: Treatment with GSH(e) may be an effective therapy to protect the cochlea against the adverse effects of traumas (eg, electrode insertion) that generate oxidative stress. 相似文献
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J. L. Adrien C. Barthélémy A. Perrot S. Roux P. Lenoir L. Hameury D. Sauvage 《Journal of autism and developmental disorders》1992,22(3):375-394
The Infant Behavioral Summarized Evaluation (IBSE) is a rating scale adapted from the Behavioral Summarized Evaluation (BSE) and specifically related to the assessment of behaviors of young children having autistic disorders. Content validity and reliability studies described in the paper were made from behavior ratings of videotapes for 89 children aged from 6 to 48 months. Results show a significant group of 19 items including some characteristic early autistic behaviors (communicative and social abnormalities) and some that are less commonly described in the syndrome (attentional, perceptive, and adaptive disorders). The value of the use of this scale for clinicians and professionals involved in behavioral evaluations and treatment of young children with developmental disorders and the necessity for further psychometric investigations are discussed.This study was supported by INSERM U.316 Nervous System from the Foetus to the Child. Development, Circulation, Metabolism, L. Pourcelot, INSERM, Network INSERM No. 489001, Grant INSERM No. 911182 (D. Savrage) No. 911008 (L. Hameury) MRT. No. 9906 Fondation Langlois. Gratitude is expressed to Professor G. Lelord who initiated and supervised this study. The authors thank Professor J. Fermanian, Department of Biostatistics, CHU Necker, Paris, for his helpful suggestions and assistance. They also thank Dr. Larmande, Dr. Leddet, Dr. Glorion, and Dr. Garreau for their medical participation in the study and C. Richard, C. Fillatre, J. Rabant, and F. Etourneau for the psychological assessments of children. Special thanks to G. Calzas, M. Barré, A. Lardeux, and D. Lioret for their technical assistance. 相似文献
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Nicole Bruneau Frédérique Bonnet-Brilhault Marie Gomot Jean-Louis Adrien Catherine Barthélémy 《International journal of psychophysiology》2003,51(1):17-25
The purpose of the present study was to investigate the relations between late auditory evoked potentials (AEPs) recorded at temporal sites (the N1c wave or Tb) and verbal and non-verbal abilities in children with autism. The study was performed in 26 mentally retarded children with autism (AUT) aged 4-8 years (mean age +/- S.E.M. = 71 +/- 2 months; mean verbal and non-verbal developmental quotient +/- S.E.M. = 36 +/- 4 and 48 +/- 3). The stimuli used were 750 Hz tone bursts of 200 ms duration delivered binaurally at different intensity levels (50, 60, 70, 80 dB SPL) with 3-5 s interstimulus intervals. Temporal AEPs were first compared to those of a group of 16 normal children (NOR) in the same age range (mean age +/- S.E.M. = 69 +/- 3 months). We then focused on the AUT group and considered relations between temporal AEPs and the severity of disorders of verbal and non-verbal communication assessed using a behavior rating scale. AEPs recorded on left and right temporal sites were of smaller amplitude in the AUT group than in the NOR group. Increasing intensity-related amplitude was observed on both sides in NOR and only on the right side in AUT. The lack of intensity effect on the left side resulted in a particular pattern of asymmetry at the highest level of intensity (80 dB SPL) with greater N1c amplitude on the right than on the left side (the reverse was found in the NOR group). Electro-clinical correlations indicated that the greater the amplitude of the right temporal N1c responses, the higher the verbal and non-verbal communication abilities. This suggests a developmental reorganization of left-right hemisphere functions in autism, with preferential activation of the right hemisphere for functions usually allocated to the left hemisphere, particularly those involving the secondary auditory areas situated on the lateral surface of the superior temporal gyrus where the N1c/Tb wave is generated. 相似文献
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Adrien JL Rossignol-Deletang N Martineau J Couturier G Barthelemy C 《Developmental psychobiology》2001,39(2):124-136
Based on the Piagetian framework, this study examined regulation of cognitive activity and developmental communication profiles and their interrelationship in groups of autistic, mentally retarded, and normal children of comparable overall, verbal, and oculo-manual developmental ages (from 6 to 24 months). Regulation of activity was assessed with both an object permanence test and an original behavior grid, and development of communication skills with the Guidetti-Tourrette scales (French adaptation of the Seibert-Hogan scales). The results showed evidence of certain types of dysregulation of cognitive activity and a general delay in communication ability in autistic children compared to the other two groups. Moreover, although the intensity of some of these disorders decreased in relation to the developmental levels of social interaction and joint attention in normal children, they were related to both high and low levels of development of social interaction only in autistic children. These findings raise the hypothesis of a relationship between a disorder of disengaging from an activity and developmental levels of social interaction noted at two transitory periods of early development (12 and 24 months) only in children with autism. Developmental and neuropsychological interpretations of this particular pattern are proposed. 相似文献
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C. Barthelemy J. L. Adrien P. Tanguay B. Garreau J. Fermanian S. Roux D. Sauvage G. Lelord 《Journal of autism and developmental disorders》1990,20(2):189-204
The Behavioral Summarized Evaluation (BSE), is a 20-item paper- and-pencil rating scale specifically designed for the measurement of behavioral parameters which could be related to biological data in autistic children involved in educational programs, neurophysiological studies, and therapeutic trials. The development of the scale, the validity, and reliability studies are presented in this paper. The results suggest that the BSE is an acceptable tool for the assessment of autistic behaviors, easy to handle, and accessible to both professionals and paraprofessionals of the medico-educative staff. It is a useful addition to the bioclinical researcher's evaluation battery for bioclinical and therapeutic studies. However, more work is suggested to further investigate the psychometric properties of this behavior assessment instrument.This study was supported by CNRS, by INSERM U 316 Nervous System from the Foetus to the Child, Development, Circulation, Metabolism, L. Pourcelot, CNAMTS No. 2748/88, FRM 1986, Conseil Régional Région Centre, Contrat No.7.040.000, INSERM, and Fondation Langlois. The authors thank S. Lancrenon, Department of Biophysics and Biomathematics (J. Fermanian), C.H.U. Pitie-Salpetriere, Paris, for her helpful suggestions and assistance. They also thank Mrs. Barre and Miss Lioret for their technical work. 相似文献
10.
Thierry Berney M.D. Giorgio La Scala M.D. Denise Vettorel M.D. Dagmar Gumowski M.D. Conrad Hauser M.D. Pascal Frileux M.D. Dr. Patrick Ambrosetti M.D. Adrien Rohner M.D. 《Diseases of the colon and rectum》1994,37(10):1038-1042
PURPOSE: This paper intends to stress the importance of early diagnosis and discuss surgical treatment of Type IV Ehlers-Danlos syndrome (EDS-4), an autosomal dominant connective tissue disease characterized by typical features of the face and extremities, inappropriate and easy bruising, and extreme tissue fragility, which may lead to dramatic and often fatal complications, mostly spontaneous arterial or intestinal rupture. METHODS: We report the case of a 41-year-old female who presented with spontaneous perforation of the sigmoid colon. RESULTS: The patient was seen over a nine-year period, during which time she required six operations and presented with a great number of surgical complications including stenosis of an end-colostomy, repeated subocclusive episodes caused by intraperitoneal adhesions, and enterocutaneous fistulas, finally ending with an ileostomy and short bowel syndrome. It is only after a difficult laparotomy for ovarian cyst excision, marked by numerous adhesions and friable bowel, that the diagnosis of EDS-4 was considered and established. CONCLUSIONS: In case of “idiopathic” spontaneous perforation of the colon in a young adult, features of EDS-4 should be thoroughly looked into and, if found, skin fibroblast culture with collagen Type III analysis performed. The surgical treatment of choice consists of subtotal colectomy and permanent endileostomy. In case of patient refusal, a second-stage ileorectal anastomosis can be performed but carries the high risk of anastomotic leakage. 相似文献