Objectives: To evaluate motor dysfunction in infants with Pierre Robin sequence (PRS) who manifest upper airway obstruction and congenital dysphagia.
Study Design: Term infants (n = 28) with nonsyndromic PRS were studied between days 15 and 45. Sucking-swallowing electromyography was used to evaluate suction and coordination between the oral and pharyngeal phases of swallowing. Esophageal manometry was used to study the lower esophageal sphincter, esophageal body, and upper esophageal sphincter functions. Manometry results were compared with those of 16 infants with gastroesophageal reflux disease (GERD).
Results: Electromyography showed incoordination of sucking and swallowing in 24 of 28 patients. The disorder was mild in 6, moderate in 6, and severe in 12 patients. All patients showed manometry disturbances: incomplete or asynchronous lower sphincter relaxation (15), multipeaked esophageal body waves (17), very high amplitude waves (14), and asynchronous upper sphincter relaxation (19). The frequency of disturbances and mean resting pressures of both lower and upper sphincters were significantly higher than GERD patients.
Conclusion: In Pierre Robin sequence, sucking-swallowing electromyography and esophageal manometry reveal dysfunction in the motor organization of the tongue, the pharynx, and the esophagus.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1067/mpd.2002.124313 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Missense variants weakening a SOX9 phosphodegron linked to odontogenesis defects, scoliosis and other skeletal features.
HGG Adv
January 2025
Department of Surgery, Division of Orthopaedics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. Electronic address:
SOX9 encodes an SRY-related transcription factor critical for chondrogenesis and sex determination among other processes. Loss-of-function variants cause campomelic dysplasia and Pierre Robin Sequence, while both gain- and loss-of-function variants cause disorders of sex development. SOX9 has also been linked to scoliosis and cancers, but variants are undetermined.
View Article and Find Full Text PDFFocused Investigation of Facial Nerve Dysfunction After Mandibular Distraction Osteogenesis for Robin Sequence.
J Craniofac Surg
January 2025
Division of Plastic and Reconstructive Surgery, Children's National Hospital.
Facial nerve dysfunction (FND) is a well-recognized but poorly documented complication of mandibular distraction osteogenesis (MDO) for Robin sequence (RS). This study aims to document the authors' experiences with FND and identify risk factors associated with this adverse event. A retrospective review of a prospectively gathered database was performed to identify patients with RS who underwent MDO at the authors' institution from March 2016 to June 2023.
View Article and Find Full Text PDFCDK4 inactivation inhibits apoptosis via mitochondria-ER contact remodeling in triple-negative breast cancer.
Nat Commun
January 2025
Center for Integrative Genomics, University of Lausanne, Faculty of Biology and Medicine, Lausanne, Switzerland.
The energetic demands of proliferating cells during tumorigenesis require close coordination between the cell cycle and metabolism. While CDK4 is known for its role in cell proliferation, its metabolic function in cancer, particularly in triple-negative breast cancer (TNBC), remains unclear. Our study, using genetic and pharmacological approaches, reveals that CDK4 inactivation only modestly impacts TNBC cell proliferation and tumor formation.
View Article and Find Full Text PDFMandibular Distraction Osteogenesis vs. Tracheostomy in the Management of Pierre Robin Sequence: A Systematic Review and Meta-Analysis.
Cleft Palate Craniofac J
January 2025
Department of Physiology and Medical Biochemistry, Faculty of Medicine, Airlangga University, Surabaya, Indonesia.
Objective: This study compares mandibular distraction osteogenesis (MDO) and tracheostomy in managing severe airway obstruction in patients with the Pierre Robin sequence (PRS).
Design: A systematic review and meta-analysis following PRISMA guidelines was performed. Literature searches were conducted across PubMed, ScienceDirect, Cochrane Library, Scopus, E.
CBCT-Based Analysis of Factors Influencing the Quality of New Bone Formation Following Mandibular Distraction Osteogenesis in Children With Pierre Robin Sequence.
J Craniofac Surg
January 2025
Department of Oral and Maxillofacial Surgery, Guangzhou Medical University Affiliated Women and Children's Medical Center, Guangzhou, China.
The aim of this study was to explore the factors influencing the quality of new bone formation after distraction osteogenesis in children with Pierre Robin sequence (PRS). Using cone-beam computed tomography (CBCT), bone density relative grayscale values of the region of new bone formation before and 3 to 4 months after mandibular distraction osteogenesis (MDO) were measured in 80 children with PRS, and correlation analysis was conducted with the potential clinical influencing factors of the children. CBCT reconstruction of the panoramic film showed that the new bone formation was good at 3 to 4 months after MDO.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!