The Experts below are selected from a list of 6417 Experts worldwide ranked by ideXlab platform
Dietmar Müller - One of the best experts on this subject based on the ideXlab platform.
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Wiedemann-Steiner syndrome: three further cases.
American journal of medical genetics. Part A, 2010Co-Authors: Rainer Koenig, Peter Meinecke, Alma Kuechler, Dieter Schäfer, Dietmar MüllerAbstract:We describe three patients with a syndrome comprising arched, thick eyebrows, hypertelorism, narrow palpebral fissures, broad Nasal Bridge and tip, long philtrum, thin upper lip, stubby hands and feet, hirsutism, and severe psychomotor retardation. These patients expand the phenotype of the Wiedemann-Steiner syndrome and delineate it as an entity.
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Wiedemann-Steiner syndrome: three further cases.
American Journal of Medical Genetics Part A, 2010Co-Authors: Rainer Koenig, Peter Meinecke, Alma Kuechler, Dieter Schäfer, Dietmar MüllerAbstract:We describe three patients with a syndrome comprising arched, thick eyebrows, hypertelorism, narrow palpebral fissures, broad Nasal Bridge and tip, long philtrum, thin upper lip, stubby hands and feet, hirsutism, and severe psychomotor retardation. These patients expand the phenotype of the Wiedemann–Steiner syndrome and delineate it as an entity. © 2010 Wiley-Liss, Inc.
Anita K. Simonds - One of the best experts on this subject based on the ideXlab platform.
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Mask pressure effects on the Nasal Bridge during short-term noninvasive ventilation.
ERJ open research, 2018Co-Authors: Anne-kathrin Brill, Rachel Pickersgill, Mohammad Moghal, Mary J. Morrell, Anita K. SimondsAbstract:The aim of this study was to assess the influence of different masks, ventilator settings and body positions on the pressure exerted on the Nasal Bridge by the mask and subjective comfort during noninvasive ventilation (NIV). We measured the pressure over the Nasal Bridge in 20 healthy participants receiving NIV via four different NIV masks (three oroNasal masks, one Nasal mask) at three different ventilator settings and in the seated or supine position. Objective pressure measurements were obtained with an I-Scan pressure-mapping system. Subjective comfort of the mask fit was assessed with a visual analogue scale. The masks exerted mean pressures between 47.6±29 mmHg and 91.9±42.4 mmHg on the Nasal Bridge. In the supine position, the pressure was lower in all masks (57.1±31.9 mmHg supine, 63.9±37.3 mmHg seated; p Objective measurement of pressure on the skin during mask fitting might be helpful for mask selection. Mask fitting in the supine position should be considered in the clinical routine.
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Mask pressure effects on the Nasal Bridge during short-term noninvasive ventilation
European Respiratory Society, 2018Co-Authors: Anne-kathrin Brill, Rachel Pickersgill, Mohammad Moghal, Mary J. Morrell, Anita K. SimondsAbstract:The aim of this study was to assess the influence of different masks, ventilator settings and body positions on the pressure exerted on the Nasal Bridge by the mask and subjective comfort during noninvasive ventilation (NIV). We measured the pressure over the Nasal Bridge in 20 healthy participants receiving NIV via four different NIV masks (three oroNasal masks, one Nasal mask) at three different ventilator settings and in the seated or supine position. Objective pressure measurements were obtained with an I-Scan pressure-mapping system. Subjective comfort of the mask fit was assessed with a visual analogue scale. The masks exerted mean pressures between 47.6±29 mmHg and 91.9±42.4 mmHg on the Nasal Bridge. In the supine position, the pressure was lower in all masks (57.1±31.9 mmHg supine, 63.9±37.3 mmHg seated; p
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Mask pressure effects on the Nasal Bridge during noninvasive Ventilation: impact of mask design, Ventilator Settings and body position
2.2 Noninvasive Ventilatory Support, 2016Co-Authors: Anne-kathrin Brill, Rachel Pickersgill, Mary J. Morrell, Mohammad Mogal, Anita K. SimondsAbstract:Background: Masks for noninvasive ventilation (NIV) must provide a good seal but can exert high pressure on the face, particularly over the Nasal Bridge (NB) which can limit treatment tolerability. We assessed the effects of different mask models, ventilator settings and body position on NB skin pressure. Methods: The pressure exerted by the NIV mask on the NB was measured with an I-Scan system in 20 healthy volunteers in a seated position with 4 different commercially available masks. Each mask was measured in a seated position with the NIV ventilator set at IPAP/EPAP: 15/5 cmH 2 0, 20/5 cmH 2 0 and 25/5 cmH 2 0 in a random order. Additionally NB pressure was measured in the supine position at 20/5 cmH 2 0. Air-leak was kept Results: The 4 different masks resulted in very variable NB pressures between median 37 mmHg (IQR: 23-59) in an oroNasal dual wall cushion mask and 94 mmHg (IQR: 53-129) in a gel cushioned mask. There was no significant change in NB pressure with the different IPAP levels. NB pressure was similar (3 masks) or significantly lower in the supine position (1 mask). Subjective discomfort was correlated with both, IPAP level and NB pressure. Conclusion: Mask choice does not have to be adapted to the IPAP level. Mask fitting in the supine position can be considered in the clinical routine. A range of different masks should be available within an NIV service to reduce pressure effects over the Nasal Bridge.
Dae H Lew - One of the best experts on this subject based on the ideXlab platform.
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Removal of Nasal Silicone Implant and the Impact of Subsequent Capsulectomy.
Plastic and reconstructive surgery, 2020Co-Authors: Won S Kook, Chae E Yang, Dae H LewAbstract:BACKGROUND Little is known about contributory factors of unremoved periimplant capsule causing Nasal deformities after postrhinoplasty silicone implant extraction. This study investigated the impact of retained capsule causing contracture deformity and effect of subsequent capsulectomy in preventing and correcting the deformity. METHODS A total of 103 patients underwent secondary surgery for silicone implant removal and grafted cartilage between May of 2015 and June of 2017. Among them, 67 patients without septal extension graft or open wound and with 8-week or more follow-up were analyzed retrospectively. All operations were approached with an intraNasal incision. Three procedures were performed: (1) removal of implant plus tip graft only (n = 12), (2) removal of implant plus tip graft plus subtotal capsulectomy (n = 47), and (3) removal of implant plus tip graft plus subtotal capsulectomy in patients with contraction and thick capsule (n = 8). Preremoval/postremoval of Nasal Bridge length index and nasolabial angle were measured with the lateral view. RESULTS In group 1, Nasal Bridge length index decreased by 6.2 percent and nasolabial angle increased by 5.7 percent. In group 2, Nasal Bridge length index increased by 2.5 percent and nasolabial angle decreased by 2.2 percent. In group 3, Nasal Bridge length index increased by 8.6 percent and nasolabial angle decreased by 7.9 percent. CONCLUSIONS For patients undergoing surgical removal of a Nasal silicone implant with or without cartilage, a concomitant capsulectomy is required to prevent potential contractures and to minimize tip deformity. Capsulectomy can release and lengthen the contracted nose without septal extension or derotation grafting. Dorsal skin irregularity did not occur regardless of whether capsulectomy was performed. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, IV.
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Removal of Nasal Silicone Implant and the Impact of Subsequent Capsulectomy.
Plastic and reconstructive surgery, 2019Co-Authors: Won S Kook, Chae E Yang, Dae H LewAbstract:Little is known about contributory factors of unremoved periimplant capsule causing Nasal deformities after postrhinoplasty silicone implant extraction. This study investigated the impact of retained capsule causing contracture deformity and effect of subsequent capsulectomy in preventing and correcting the deformity. A total of 103 patients underwent secondary surgery for silicone implant removal and grafted cartilage between May of 2015 and June of 2017. Among them, 67 patients without septal extension graft or open wound and with 8-week or more follow-up were analyzed retrospectively. All operations were approached with an intraNasal incision. Three procedures were performed: (1) removal of implant plus tip graft only (n = 12), (2) removal of implant plus tip graft plus subtotal capsulectomy (n = 47), and (3) removal of implant plus tip graft plus subtotal capsulectomy in patients with contraction and thick capsule (n = 8). Preremoval/postremoval of Nasal Bridge length index and nasolabial angle were measured with the lateral view. In group 1, Nasal Bridge length index decreased by 6.2 percent and nasolabial angle increased by 5.7 percent. In group 2, Nasal Bridge length index increased by 2.5 percent and nasolabial angle decreased by 2.2 percent. In group 3, Nasal Bridge length index increased by 8.6 percent and nasolabial angle decreased by 7.9 percent. For patients undergoing surgical removal of a Nasal silicone implant with or without cartilage, a concomitant capsulectomy is required to prevent potential contractures and to minimize tip deformity. Capsulectomy can release and lengthen the contracted nose without septal extension or derotation grafting. Dorsal skin irregularity did not occur regardless of whether capsulectomy was performed. Therapeutic, IV.
Rainer Koenig - One of the best experts on this subject based on the ideXlab platform.
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Wiedemann-Steiner syndrome: three further cases.
American journal of medical genetics. Part A, 2010Co-Authors: Rainer Koenig, Peter Meinecke, Alma Kuechler, Dieter Schäfer, Dietmar MüllerAbstract:We describe three patients with a syndrome comprising arched, thick eyebrows, hypertelorism, narrow palpebral fissures, broad Nasal Bridge and tip, long philtrum, thin upper lip, stubby hands and feet, hirsutism, and severe psychomotor retardation. These patients expand the phenotype of the Wiedemann-Steiner syndrome and delineate it as an entity.
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Wiedemann-Steiner syndrome: three further cases.
American Journal of Medical Genetics Part A, 2010Co-Authors: Rainer Koenig, Peter Meinecke, Alma Kuechler, Dieter Schäfer, Dietmar MüllerAbstract:We describe three patients with a syndrome comprising arched, thick eyebrows, hypertelorism, narrow palpebral fissures, broad Nasal Bridge and tip, long philtrum, thin upper lip, stubby hands and feet, hirsutism, and severe psychomotor retardation. These patients expand the phenotype of the Wiedemann–Steiner syndrome and delineate it as an entity. © 2010 Wiley-Liss, Inc.
Roger L Ladda - One of the best experts on this subject based on the ideXlab platform.
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previously apparently undescribed syndrome shallow orbits ptosis coloboma trigonocephaly gyral malformations and mental and growth retardation
American Journal of Medical Genetics, 1995Co-Authors: C Jeanette M D Ramer, Robin Winter, Rosanna Pallotta, Ségolène Aymé, Roger L LaddaAbstract:We describe 2 children with severe ptosis, trigonocephaly, broad Nasal Bridge, and major brain malformation. A total of 8 children have been reported who share most of these findings. Two of the individuals have had identical pericentric inversions involving chromosome 2p12-q14. These cases appear to represent a unique malformation syndrome. 14 refs., 6 figs., 1 tab.
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Previously apparently undescribed syndrome: Shallow orbits, ptosis, coloboma, trigonocephaly, gyral malformations, and mental and growth retardation
American journal of medical genetics, 1995Co-Authors: Jeanette C. Ramer, Robin Winter, Rosanna Pallotta, Ségolène Aymé, Angela E. Lin, William B. Dobyns, Roger L LaddaAbstract:We describe 2 children with severe ptosis, trigonocephaly, broad Nasal Bridge, and major brain malformation. A total of 8 children have been reported who share most of these findings. Two of the individuals have had identical pericentric inversions involving chromosome 2p12-q14. These cases appear to represent a unique malformation syndrome.
