The Experts below are selected from a list of 22047 Experts worldwide ranked by ideXlab platform
Frederick S. Kaplan - One of the best experts on this subject based on the ideXlab platform.
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conductive hearing loss in individuals with fibrodysplasia ossificans progressiva
American Journal of Audiology, 1999Co-Authors: Charles E Levy, Albert T Lash, Hal B Janoff, Frederick S. KaplanAbstract:Fibrodysplasia ossificans progressiva (FOP) is a very rare genetic disorder that is characterized by progressive heterotopic ossification of soft tissues and Congenital Malformation of the great to...
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overexpression of an osteogenic morphogen in fibrodysplasia ossificans progressiva
The New England Journal of Medicine, 1996Co-Authors: Adam B Shafritz, Francis H Gannon, Eileen M Shore, Michael Zasloff, Rebecca Taub, Maximilian Muenke, Frederick S. KaplanAbstract:Background Fibrodysplasia ossificans progressiva is a heritable disorder of connective tissue characterized by Congenital Malformation of the great toes and postnatal formation of ectopic bone. Alt...
Amelie Forget - One of the best experts on this subject based on the ideXlab platform.
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asthma exacerbations during the first trimester of pregnancy and Congenital Malformations revisiting the association in a large representative cohort
Thorax, 2015Co-Authors: Lucie Blais, Mariefrance Beauchesne, Amelie Forget, Fatimazohra Kettani, Catherine LemiereAbstract:Background We previously reported an increased prevalence of any Congenital Malformation among women experiencing moderate-to-severe asthma exacerbations during the first trimester of pregnancy, based on a study in which 90.1% of the cohort of women were social welfare recipients. This study re-examined the association between asthma exacerbations and Congenital Malformations in a new large representative cohort of asthmatic pregnant women. Methods A cohort of 36 587 pregnancies in asthmatic women was reconstructed from Quebec Province administrative databases (1998–2009). Occurrences of asthma exacerbations during the first trimester of pregnancy were assessed and categorised into severe, moderate and no such exacerbations. For comparison, we also considered moderate and severe asthma exacerbations combined. Congenital Malformations were identified using diagnoses recorded in the hospitalisation database. Generalised estimation equations were used to estimate adjusted ORs of Congenital Malformations. Results The prevalence of any Congenital Malformation was 19.1%, 11.7% and 12.0% among women with severe, moderate and no such exacerbations during the first trimester, respectively. The adjusted OR for all Malformations was 1.64 (95% CI 1.02 to 2.64) when women with severe exacerbations were compared with those in the reference group, while no association was seen for moderate exacerbations. Also, no association was observed between cases of moderate and severe asthma exacerbations combined and any Congenital Malformation. Conclusions Only severe asthma exacerbations were found to significantly increase the risk of Congenital Malformations in this representative study. Previous studies possibly overestimated the risk because they were based mainly on women at a lower socioeconomic status.
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asthma exacerbations during the first trimester of pregnancy and the risk of Congenital Malformations among asthmatic women
The Journal of Allergy and Clinical Immunology, 2008Co-Authors: Lucie Blais, Amelie ForgetAbstract:Background Uncontrolled maternal asthma during pregnancy has been hypothesized as a cause of Congenital Malformation, but literature is scare on this topic. Objective The aim of this study was to investigate whether asthmatic women who had an exacerbation during the first trimester of pregnancy were more at risk of having a baby with a Congenital Malformation. Methods From the linkage of 3 Canadian administrative databases, we reconstructed a cohort of 4344 pregnancies of asthmatic women. Asthma exacerbations were assessed during the first trimester of pregnancy and were defined as a filled prescription for oral corticosteroids, an emergency department visit, or a hospitalization for asthma. Congenital Malformations were assessed at birth and during the first year of life of the newborn by using diagnoses recorded in the databases. Generalized estimating equation models were used to estimate adjusted odds ratios of Congenital Malformations in association with asthma exacerbations. Results In the cohort we identified 398 (9.2%) babies with at least 1 Malformation and 261 (6.0%) with a major Malformation. The crude prevalences of Malformations were 12.8% and 8.9%, respectively, for women who had and those who did not have an exacerbation. The adjusted odds ratio for all Malformations was 1.48 (95% CI, 1.04-2.09) when comparing women who had and those who did not have an exacerbation. The corresponding figures were 1.32 (95% CI, 0.86-2.04) for major Malformations. Conclusion Asthma exacerbations during the first trimester of pregnancy were found to significantly increase the risk of a Congenital Malformation.
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use of inhaled corticosteroids during the first trimester of pregnancy and the risk of Congenital Malformations among women with asthma
Thorax, 2007Co-Authors: Lucie Blais, Mariefrance Beauchesne, Evelyne Rey, Jeanluc Malo, Amelie ForgetAbstract:Aim: To investigate whether the maternal use of different doses of inhaled corticosteroids (ICSs) during the first trimester of pregnancy for the treatment of asthma increases the risk of Congenital Malformations in the offspring. Methods: From the linkage of three administrative Canadian databases, a cohort of 4561 pregnancies from women with asthma who delivered between 1990 and 2000 was reconstructed. A two-stage sampling cohort design was used to acquire additional data from the woman’s medical chart. Cases of Congenital Malformation were identified from the medical services database or the hospital database. Using refill patterns of medications, the average daily dose of ICSs used during the first trimester was calculated and categorised as follows: 0, 1–500, 500–1000 and >1000 μg/day in beclomethasone–chlorofluorocarbon equivalent. A Generalized Estimation Equation model was used to estimate the adjusted odds ratio of Congenital Malformation as a function of ICS daily dose. All analyses were performed for all Malformations and major Malformations separately. Results: Within the cohort 418 babies were identified with a Congenital Malformation (9.2%), 278 of which had a major Malformation. About 40% of women used ICSs during the first trimester, but only 5.3% of women used >500 μg/day. The adjusted odds ratio (95% CI) for all Malformations associated with the use of ICSs during the first trimester was: 0.77 (0.53 to 1.13) for 1–500, 0.41 (0.19 to 0.92) for 501–1000 and 1.00 (0.42 to 2.36) for >1000 μg/day. The corresponding figures for major Malformations were 0.90 (0.64 to 1.24), 0.56 (0.22 to 1.43) and 1.67 (0.56 to 5.03). Conclusion: This study adds evidence to the safety of ICSs for the treatment of asthma during pregnancy, with regard to the likelihood of Congenital Malformation.
Yucel Oztan - One of the best experts on this subject based on the ideXlab platform.
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idiopathic isolated clitoromegaly a report of two cases
Reproductive Health, 2004Co-Authors: Eray Copcu, Ozgen Copcu, Alper Aktas, Nazan Sivrioglu, Yucel OztanAbstract:Background Clitoromegaly is a frequent Congenital Malformation, but acquired clitoral enlargement is relatively rare.
Lucie Blais - One of the best experts on this subject based on the ideXlab platform.
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asthma exacerbations during the first trimester of pregnancy and Congenital Malformations revisiting the association in a large representative cohort
Thorax, 2015Co-Authors: Lucie Blais, Mariefrance Beauchesne, Amelie Forget, Fatimazohra Kettani, Catherine LemiereAbstract:Background We previously reported an increased prevalence of any Congenital Malformation among women experiencing moderate-to-severe asthma exacerbations during the first trimester of pregnancy, based on a study in which 90.1% of the cohort of women were social welfare recipients. This study re-examined the association between asthma exacerbations and Congenital Malformations in a new large representative cohort of asthmatic pregnant women. Methods A cohort of 36 587 pregnancies in asthmatic women was reconstructed from Quebec Province administrative databases (1998–2009). Occurrences of asthma exacerbations during the first trimester of pregnancy were assessed and categorised into severe, moderate and no such exacerbations. For comparison, we also considered moderate and severe asthma exacerbations combined. Congenital Malformations were identified using diagnoses recorded in the hospitalisation database. Generalised estimation equations were used to estimate adjusted ORs of Congenital Malformations. Results The prevalence of any Congenital Malformation was 19.1%, 11.7% and 12.0% among women with severe, moderate and no such exacerbations during the first trimester, respectively. The adjusted OR for all Malformations was 1.64 (95% CI 1.02 to 2.64) when women with severe exacerbations were compared with those in the reference group, while no association was seen for moderate exacerbations. Also, no association was observed between cases of moderate and severe asthma exacerbations combined and any Congenital Malformation. Conclusions Only severe asthma exacerbations were found to significantly increase the risk of Congenital Malformations in this representative study. Previous studies possibly overestimated the risk because they were based mainly on women at a lower socioeconomic status.
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asthma exacerbations during the first trimester of pregnancy and the risk of Congenital Malformations among asthmatic women
The Journal of Allergy and Clinical Immunology, 2008Co-Authors: Lucie Blais, Amelie ForgetAbstract:Background Uncontrolled maternal asthma during pregnancy has been hypothesized as a cause of Congenital Malformation, but literature is scare on this topic. Objective The aim of this study was to investigate whether asthmatic women who had an exacerbation during the first trimester of pregnancy were more at risk of having a baby with a Congenital Malformation. Methods From the linkage of 3 Canadian administrative databases, we reconstructed a cohort of 4344 pregnancies of asthmatic women. Asthma exacerbations were assessed during the first trimester of pregnancy and were defined as a filled prescription for oral corticosteroids, an emergency department visit, or a hospitalization for asthma. Congenital Malformations were assessed at birth and during the first year of life of the newborn by using diagnoses recorded in the databases. Generalized estimating equation models were used to estimate adjusted odds ratios of Congenital Malformations in association with asthma exacerbations. Results In the cohort we identified 398 (9.2%) babies with at least 1 Malformation and 261 (6.0%) with a major Malformation. The crude prevalences of Malformations were 12.8% and 8.9%, respectively, for women who had and those who did not have an exacerbation. The adjusted odds ratio for all Malformations was 1.48 (95% CI, 1.04-2.09) when comparing women who had and those who did not have an exacerbation. The corresponding figures were 1.32 (95% CI, 0.86-2.04) for major Malformations. Conclusion Asthma exacerbations during the first trimester of pregnancy were found to significantly increase the risk of a Congenital Malformation.
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use of inhaled corticosteroids during the first trimester of pregnancy and the risk of Congenital Malformations among women with asthma
Thorax, 2007Co-Authors: Lucie Blais, Mariefrance Beauchesne, Evelyne Rey, Jeanluc Malo, Amelie ForgetAbstract:Aim: To investigate whether the maternal use of different doses of inhaled corticosteroids (ICSs) during the first trimester of pregnancy for the treatment of asthma increases the risk of Congenital Malformations in the offspring. Methods: From the linkage of three administrative Canadian databases, a cohort of 4561 pregnancies from women with asthma who delivered between 1990 and 2000 was reconstructed. A two-stage sampling cohort design was used to acquire additional data from the woman’s medical chart. Cases of Congenital Malformation were identified from the medical services database or the hospital database. Using refill patterns of medications, the average daily dose of ICSs used during the first trimester was calculated and categorised as follows: 0, 1–500, 500–1000 and >1000 μg/day in beclomethasone–chlorofluorocarbon equivalent. A Generalized Estimation Equation model was used to estimate the adjusted odds ratio of Congenital Malformation as a function of ICS daily dose. All analyses were performed for all Malformations and major Malformations separately. Results: Within the cohort 418 babies were identified with a Congenital Malformation (9.2%), 278 of which had a major Malformation. About 40% of women used ICSs during the first trimester, but only 5.3% of women used >500 μg/day. The adjusted odds ratio (95% CI) for all Malformations associated with the use of ICSs during the first trimester was: 0.77 (0.53 to 1.13) for 1–500, 0.41 (0.19 to 0.92) for 501–1000 and 1.00 (0.42 to 2.36) for >1000 μg/day. The corresponding figures for major Malformations were 0.90 (0.64 to 1.24), 0.56 (0.22 to 1.43) and 1.67 (0.56 to 5.03). Conclusion: This study adds evidence to the safety of ICSs for the treatment of asthma during pregnancy, with regard to the likelihood of Congenital Malformation.
Shouqin Zhao - One of the best experts on this subject based on the ideXlab platform.
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audiological effectiveness of bonebridge implantation for bilateral Congenital Malformation of the external and middle ear
European Archives of Oto-rhino-laryngology, 2019Co-Authors: Shouqin Zhao, Xinxing Fu, Yi Li, Danni Wang, Yuling Li, Peiwei ChenAbstract:PURPOSE: To evaluate the audiological effectiveness of Bonebridge implantation in patients with bilateral Congenital Malformation of the external and middle ear. METHODS: Twenty-eight cases [17 boys and 11 girls; median age, 12 years (range 8-36 years)] had unilateral Bonebridge implantation. Audiological tests were performed preoperatively and postoperatively, and included the pure-tone audiometry test, speech discrimination score (SDS), and evaluation of the unaided and aided hearing thresholds in sound fields. For the group of patients aged > 12 years, Mandarin Speech Test Materials were used to determine the SDS. For the other cases, the Mandarin Lexical Neighborhood Test was used. The daily life efficacy was assessed using the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire postoperatively. The t and t' tests were used in the statistical analyses. RESULTS: The hearing threshold with the Bonebridge improved by 25-35 dB HL, compared to that of the unaided condition. The SDS of patients aged > 12 years improved by about 50%; the SDS also improved by 10-20% in the three patients aged < 12 years. According to the APHAB, the mean Bonebridge scores of ease of communication, background noise, and reverberation decreased by 60.3 ± 15.7, 50.2 ± 11.1, and 59.4 ± 7.8, respectively, compared to the preoperative scores, and the Bonebridge score of aversiveness was higher (69.8 ± 10.8) than the unaided score. CONCLUSIONS: The audiological effectiveness of the Bonebridge was significant and noticeable in bilateral Congenital Malformation of the external and middle ear. The Bonebridge can be an alternative and effective solution for those patients to improve hearing.
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audiological effectiveness of bonebridge implantation for bilateral Congenital Malformation of the external and middle ear
European Archives of Oto-rhino-laryngology, 2019Co-Authors: Ran Ren, Shouqin Zhao, Danni Wang, Peiwei Chen, Jingmin DouAbstract:To evaluate the audiological effectiveness of Bonebridge implantation in patients with bilateral Congenital Malformation of the external and middle ear. Twenty-eight cases [17 boys and 11 girls; median age, 12 years (range 8–36 years)] had unilateral Bonebridge implantation. Audiological tests were performed preoperatively and postoperatively, and included the pure-tone audiometry test, speech discrimination score (SDS), and evaluation of the unaided and aided hearing thresholds in sound fields. For the group of patients aged > 12 years, Mandarin Speech Test Materials were used to determine the SDS. For the other cases, the Mandarin Lexical Neighborhood Test was used. The daily life efficacy was assessed using the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire postoperatively. The t and t′ tests were used in the statistical analyses. The hearing threshold with the Bonebridge improved by 25–35 dB HL, compared to that of the unaided condition. The SDS of patients aged > 12 years improved by about 50%; the SDS also improved by 10–20% in the three patients aged < 12 years. According to the APHAB, the mean Bonebridge scores of ease of communication, background noise, and reverberation decreased by 60.3 ± 15.7, 50.2 ± 11.1, and 59.4 ± 7.8, respectively, compared to the preoperative scores, and the Bonebridge score of aversiveness was higher (69.8 ± 10.8) than the unaided score. The audiological effectiveness of the Bonebridge was significant and noticeable in bilateral Congenital Malformation of the external and middle ear. The Bonebridge can be an alternative and effective solution for those patients to improve hearing.
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classification of facial nerve aberration in Congenital Malformation of middle ear implications for surgery of hearing restoration
Journal of otology, 2018Co-Authors: Liping Xu, Shuling Li, Xinxing Fu, Shouqin ZhaoAbstract:Abstract Objectives Facial nerve aberration is the most troublesome situation in Congenital Malformations of middle ear. The aim of our study is to investigate its imaging and clinical features as well as relevant choice of surgical techniques for hearing improvement. Methods A retrospective study involving review of clinical data of 227 patients (256 ears) with Congenital middle ear anomaly was undertaken, including preoperative computed tomography (CT) data, surgical records and videos. Results Aberration involving intratemporal facial nerve was found in 82/256 ears (32.03%) with Congenital middle ear anomaly. The most common forms of aberration included overhanging over the oval window (50/82 ears, 60.98%), bifurcation (3/82 ears, 3.66%) and transverse over the promontory (3/82 ears, 3.66%), counting for 68.29% (56/82) of the cases with facial nerve aberration. Concomitant stapes Malformation was found in 76/82 ears (92.68%) and atresia or stenosis of the oval window in 27/82 ears (32.93%). In 9/82 ears (10.98%) both stapes and oval window was absent. Elective surgeries for the purpose of hearing improvement included stapodotomy + piston implantation, labyrinthotomy, labyrinthotomy + total ossicular replacement prosthesis (TORP) implantation and Vibrant Soundbridge (VSB) implantation. Conclusion The majority of facial nerve aberration in Congenital Malformation of middle ear involves displacement of facial nerve, in addition to concomitant Malformations of the stapes and/or oval window, which may influence the choice of surgery for hearing improvement. VSB implantation may be considered as a useful option.
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Classification of facial nerve aberration in Congenital Malformation of middle ear: Implications for surgery of hearing restoration
Elsevier, 2018Co-Authors: Jin Hao, Shouqin ZhaoAbstract:Objectives: Facial nerve aberration is the most troublesome situation in Congenital Malformations of middle ear. The aim of our study is to investigate its imaging and clinical features as well as relevant choice of surgical techniques for hearing improvement. Methods: A retrospective study involving review of clinical data of 227 patients (256 ears) with Congenital middle ear anomaly was undertaken, including preoperative computed tomography (CT) data, surgical records and videos. Results: Aberration involving intratemporal facial nerve was found in 82/256 ears (32.03%) with Congenital middle ear anomaly. The most common forms of aberration included overhanging over the oval window (50/82 ears, 60.98%), bifurcation (3/82 ears, 3.66%) and transverse over the promontory (3/82 ears, 3.66%), counting for 68.29% (56/82) of the cases with facial nerve aberration. Concomitant stapes Malformation was found in 76/82 ears (92.68%) and atresia or stenosis of the oval window in 27/82 ears (32.93%). In 9/82 ears (10.98%) both stapes and oval window was absent. Elective surgeries for the purpose of hearing improvement included stapodotomy + piston implantation, labyrinthotomy, labyrinthotomy + total ossicular replacement prosthesis (TORP) implantation and Vibrant Soundbridge (VSB) implantation. Conclusion: The majority of facial nerve aberration in Congenital Malformation of middle ear involves displacement of facial nerve, in addition to concomitant Malformations of the stapes and/or oval window, which may influence the choice of surgery for hearing improvement. VSB implantation may be considered as a useful option. Keywords: Congenital middle ear anomaly, Facial nerve, Stapes, Oval window, Vibrant Soundbridge (VSB
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the implantation of bonebridge in bilateral Congenital Malformation of external and middle ear
Chinese journal of otorhinolaryngology head and neck surgery, 2017Co-Authors: Shouqin Zhao, Yi Li, Danni Wang, Yuling LiAbstract:Objective To evaluate the auditory efficacy of Bonebridge implantation in patients with bilateral Congenital Malformation of external and middle ear. Methods Eleven cases (6 males and 5 females) had unilateral Bonebridge implantation. The age ranged from 8 to 26 and the average age was 16.9. Seven to ten days after operation, the first fitting was undergone. In acoustic sound field, the average auditory thresholds were respectively measured for unaided ears and Bonebridge implanted ears by pure tone auditory (PTA, 0.25, 0.5, 1, 2 and 4 kHz). For the group over 12-year-old, MSTM was applied to evaluate speech discrimination score (SDS). For the other cases, MLNT was used as the test material. The auditory efficacy post Bonebridge implantation would be analyzed and evaluated by comparing the differences between unaided ears and Bonebridge implanted ears. Results The bone conduction audibility threshold after Bonebridge implantation was as well as the preoperative. The auditory threshold with Bonebridge aided was improved to 25-35 dB HL, when compared to that of the unaided ears in the sound field. The SDS in the group over 12-year-old was improved about 50%; the efficacy was slightly limited for the other two cases (both less than 12 years old). Statistical analysis showed that there were significant differences between unaided ears and Bonebridge implanted ears in the sound field and SDS(P<0.05). Conclusions The auditory efficacy of Bonebridge is significant and noticeable in patients with bilateral Congenital Malformation of external and middle ear. Bonebridge provides a new and effective way for patient with Congenital Malformation of external and middle ear to reconstruct hearing. Key words: Ear deformities, Congenital; Hearing loss; Hearing aids; Bonebridge