The Experts below are selected from a list of 117 Experts worldwide ranked by ideXlab platform
Alain Verloes - One of the best experts on this subject based on the ideXlab platform.
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child with manifestations of Nager Acrofacial Dysostosis and the murcs vacterl and pulmonary agenesis associations complex defect of blastogenesis
American Journal of Medical Genetics, 1996Co-Authors: Albert David, Jacques Mercier, Alain VerloesAbstract:Nager Acrofacial Dysostosis (NAFD) combines the facial anomalies of mandibulofacial Dysostosis (Treacher-Collins-Francescetti) with hypoplastic/aplastic or triphangeal thumbs. The MURCS association consists of Mullerian duct aplasia, renal aplasia, cervicothoracic somite Dysostosis with Klippel-Feil anomaly, and often defects of the facio-auriculo-vertebral (Goldenhar) spectrum. We describe a child with NAFD, MURCS anomaly (Klippel-Feil anomaly, vertebral synostoses, left renal agenesis), and left pulmonary agenesis. Our proband appears to express a unique anomaly of blastogenesis, simultaneously affecting the acrorenal, the mandibulofacial, and the cervicothoracic developmental fields, combining clinical manifestations of the MURCS, NAFD, VACTERL, and pulmonary agenesis associations. All anomalies may be traced back to abnormal blastogenesis, occurring during the third or the fourth week of embryonic development, and show that NAFD is a polytopic developmental field defect. © 1996 Wiley-Liss, Inc.
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Child with manifestations of Nager Acrofacial Dysostosis, and the MURCS, VACTERL, and pulmonary agenesis associations: Complex defect of blastogenesis?
American Journal of Medical Genetics, 1996Co-Authors: Albert David, Jacques Mercier, Alain VerloesAbstract:Nager Acrofacial Dysostosis (NAFD) combines the facial anomalies of mandibulofacial Dysostosis (Treacher-Collins-Francescetti) with hypoplastic/aplastic or triphangeal thumbs. The MURCS association consists of Mullerian duct aplasia, renal aplasia, cervicothoracic somite Dysostosis with Klippel-Feil anomaly, and often defects of the facio-auriculo-vertebral (Goldenhar) spectrum. We describe a child with NAFD, MURCS anomaly (Klippel-Feil anomaly, vertebral synostoses, left renal agenesis), and left pulmonary agenesis. Our proband appears to express a unique anomaly of blastogenesis, simultaneously affecting the acrorenal, the mandibulofacial, and the cervicothoracic developmental fields, combining clinical manifestations of the MURCS, NAFD, VACTERL, and pulmonary agenesis associations. All anomalies may be traced back to abnormal blastogenesis, occurring during the third or the fourth week of embryonic development, and show that NAFD is a polytopic developmental field defect.
Albert David - One of the best experts on this subject based on the ideXlab platform.
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child with manifestations of Nager Acrofacial Dysostosis and the murcs vacterl and pulmonary agenesis associations complex defect of blastogenesis
American Journal of Medical Genetics, 1996Co-Authors: Albert David, Jacques Mercier, Alain VerloesAbstract:Nager Acrofacial Dysostosis (NAFD) combines the facial anomalies of mandibulofacial Dysostosis (Treacher-Collins-Francescetti) with hypoplastic/aplastic or triphangeal thumbs. The MURCS association consists of Mullerian duct aplasia, renal aplasia, cervicothoracic somite Dysostosis with Klippel-Feil anomaly, and often defects of the facio-auriculo-vertebral (Goldenhar) spectrum. We describe a child with NAFD, MURCS anomaly (Klippel-Feil anomaly, vertebral synostoses, left renal agenesis), and left pulmonary agenesis. Our proband appears to express a unique anomaly of blastogenesis, simultaneously affecting the acrorenal, the mandibulofacial, and the cervicothoracic developmental fields, combining clinical manifestations of the MURCS, NAFD, VACTERL, and pulmonary agenesis associations. All anomalies may be traced back to abnormal blastogenesis, occurring during the third or the fourth week of embryonic development, and show that NAFD is a polytopic developmental field defect. © 1996 Wiley-Liss, Inc.
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Child with manifestations of Nager Acrofacial Dysostosis, and the MURCS, VACTERL, and pulmonary agenesis associations: Complex defect of blastogenesis?
American Journal of Medical Genetics, 1996Co-Authors: Albert David, Jacques Mercier, Alain VerloesAbstract:Nager Acrofacial Dysostosis (NAFD) combines the facial anomalies of mandibulofacial Dysostosis (Treacher-Collins-Francescetti) with hypoplastic/aplastic or triphangeal thumbs. The MURCS association consists of Mullerian duct aplasia, renal aplasia, cervicothoracic somite Dysostosis with Klippel-Feil anomaly, and often defects of the facio-auriculo-vertebral (Goldenhar) spectrum. We describe a child with NAFD, MURCS anomaly (Klippel-Feil anomaly, vertebral synostoses, left renal agenesis), and left pulmonary agenesis. Our proband appears to express a unique anomaly of blastogenesis, simultaneously affecting the acrorenal, the mandibulofacial, and the cervicothoracic developmental fields, combining clinical manifestations of the MURCS, NAFD, VACTERL, and pulmonary agenesis associations. All anomalies may be traced back to abnormal blastogenesis, occurring during the third or the fourth week of embryonic development, and show that NAFD is a polytopic developmental field defect.
Jacques Mercier - One of the best experts on this subject based on the ideXlab platform.
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child with manifestations of Nager Acrofacial Dysostosis and the murcs vacterl and pulmonary agenesis associations complex defect of blastogenesis
American Journal of Medical Genetics, 1996Co-Authors: Albert David, Jacques Mercier, Alain VerloesAbstract:Nager Acrofacial Dysostosis (NAFD) combines the facial anomalies of mandibulofacial Dysostosis (Treacher-Collins-Francescetti) with hypoplastic/aplastic or triphangeal thumbs. The MURCS association consists of Mullerian duct aplasia, renal aplasia, cervicothoracic somite Dysostosis with Klippel-Feil anomaly, and often defects of the facio-auriculo-vertebral (Goldenhar) spectrum. We describe a child with NAFD, MURCS anomaly (Klippel-Feil anomaly, vertebral synostoses, left renal agenesis), and left pulmonary agenesis. Our proband appears to express a unique anomaly of blastogenesis, simultaneously affecting the acrorenal, the mandibulofacial, and the cervicothoracic developmental fields, combining clinical manifestations of the MURCS, NAFD, VACTERL, and pulmonary agenesis associations. All anomalies may be traced back to abnormal blastogenesis, occurring during the third or the fourth week of embryonic development, and show that NAFD is a polytopic developmental field defect. © 1996 Wiley-Liss, Inc.
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Child with manifestations of Nager Acrofacial Dysostosis, and the MURCS, VACTERL, and pulmonary agenesis associations: Complex defect of blastogenesis?
American Journal of Medical Genetics, 1996Co-Authors: Albert David, Jacques Mercier, Alain VerloesAbstract:Nager Acrofacial Dysostosis (NAFD) combines the facial anomalies of mandibulofacial Dysostosis (Treacher-Collins-Francescetti) with hypoplastic/aplastic or triphangeal thumbs. The MURCS association consists of Mullerian duct aplasia, renal aplasia, cervicothoracic somite Dysostosis with Klippel-Feil anomaly, and often defects of the facio-auriculo-vertebral (Goldenhar) spectrum. We describe a child with NAFD, MURCS anomaly (Klippel-Feil anomaly, vertebral synostoses, left renal agenesis), and left pulmonary agenesis. Our proband appears to express a unique anomaly of blastogenesis, simultaneously affecting the acrorenal, the mandibulofacial, and the cervicothoracic developmental fields, combining clinical manifestations of the MURCS, NAFD, VACTERL, and pulmonary agenesis associations. All anomalies may be traced back to abnormal blastogenesis, occurring during the third or the fourth week of embryonic development, and show that NAFD is a polytopic developmental field defect.
P. Ayliffe - One of the best experts on this subject based on the ideXlab platform.
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Nager Acrofacial Dysostosis a case series with literature review and update
Journal of Oral and Maxillofacial Surgery, 2013Co-Authors: O. Sheikh, G. Logan, R. Pabla, P. AyliffeAbstract:pathology by an oral and maxillofacial surgeon or TMJ specialist. Ordinal variables obtained included maximal incisal opening (MIO) at preoperative, 1 month, 3-6 months and 1+ year follow up visits. Secondary variables obtained included prior history of TMJ surgery and incidence of surgical complications. Other variables recorded included age at time of surgery and gender. SPSS statistical software, version 21 was used. Oneway analysis of variance (ANOVA) and non-parametric tests were utilized and statistical significance was set at a p-value of 1year intervals were equivalent (p>0.05). Average improvement in range of motion after 1 year when compared to preoperative values was greater in the DJD group (mean = 8mm increase in MIO) than painless hypomobility/trauma/malocclusion group (mean = 3mm increase in MIO). Interestingly, a prior history of open joint surgery affects mandibular range of motion at all time intervals except 1-month post TJR surgery (p<0.05). The rate of postoperative complications was 58%, although the vast majority were neurologic deficits that recovered by 1-year postoperative.
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Nager Acrofacial Dysostosis: A Case Series with Literature Review and Update
Journal of Oral and Maxillofacial Surgery, 2013Co-Authors: O. Sheikh, G. Logan, R. Pabla, P. AyliffeAbstract:pathology by an oral and maxillofacial surgeon or TMJ specialist. Ordinal variables obtained included maximal incisal opening (MIO) at preoperative, 1 month, 3-6 months and 1+ year follow up visits. Secondary variables obtained included prior history of TMJ surgery and incidence of surgical complications. Other variables recorded included age at time of surgery and gender. SPSS statistical software, version 21 was used. Oneway analysis of variance (ANOVA) and non-parametric tests were utilized and statistical significance was set at a p-value of 1year intervals were equivalent (p>0.05). Average improvement in range of motion after 1 year when compared to preoperative values was greater in the DJD group (mean = 8mm increase in MIO) than painless hypomobility/trauma/malocclusion group (mean = 3mm increase in MIO). Interestingly, a prior history of open joint surgery affects mandibular range of motion at all time intervals except 1-month post TJR surgery (p
Peter Ring - One of the best experts on this subject based on the ideXlab platform.
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Nager Syndrome Problems and Possibilities of Therapy
Journal of Orofacial Orthopedics Fortschritte der Kieferorthopädie, 2000Co-Authors: Charlotte Opitz, Christian Stoll, Peter RingAbstract:Es wird das angeborene Krankheitsbild des Nager–Syndroms dargestellt, und anschließend werden die Probleme und Möglichkeiten bei der Behandlung dieser Patienten beschrieben. Anhand des Berichts über einen Patienten, der vom Neugeborenen- bis in das Erwachsenenalter therapeutisch begleitet worden ist, werden die kieferchirurgisch/kieferorthopädischen Maßnahmen dargestellt und die Grenzen der Therapie aufgezeigt. The congenital Nager Acrofacial Dysostosis syndrome is presented, and possibilities and problems in the treatment of these patients are described. A case study of a patient who has been followed from birth to adulthood illustrates the surgical/orthodontic course of treatment and its limits.
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Nager syndrome. Problems and possibilities of therapy.
Journal of Orofacial Orthopedics Fortschritte der Kieferorthopädie, 2000Co-Authors: Charlotte Opitz, Christian Stoll, Peter RingAbstract:The congenital Nager Acrofacial Dysostosis syndrome is presented, and possibilities and problems in the treatment of these patients are described. A case study of a patient who has been followed from birth to adulthood illustrates the surgical/orthodontic course of treatment and its limits.
