PubMed · 14822609
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M C OLDFIELD. 1951. Cleft palate.. https://pubmed.ncbi.nlm.nih.gov/14822609/
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Recent studies have unveiled disrupted metabolism in the progression of cleft palate (CP), a congenital anomaly characterized by defective fusion of facial structures. Nonetheless, the precise composition of this disrupted metabolism remains elusive, prompting us to identify these components and elucidate primary metabolic irregularities contributing to CP pathogenesis. We established a murine CP model by retinoic acid (RA) treatment and analyzed control and RA-treated embryonic palatal tissues by LC-MS-based proteomic approach. We identified 220 significantly upregulated and 224 significantly downregulated proteins. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analysis revealed that these differentially expressed proteins (DEPs) were involved in translation, ribosome assembly, mitochondrial function, mRNA binding, as well as key metabolic pathways like oxidative phosphorylation (OXPHOS), glycolysis/gluconeogenesis, and amino acid biosynthesis. These findings suggest that dysregulated ribosome-related pathways and disrupted metabolism play a critical role in CP development. Protein-protein interaction analysis using the STRING database revealed a tightly connected network of DEPs. Furthermore, we identified the top 10 hub proteins in CP using the Cytohubba plugin in Cytoscape. These hub proteins, including RPL8, RPS11, ALB, PA2G4, RPL23, RPS6, CCT7, EGFR, HSPD1, and RPS28, are potentially key regulators of CP pathogenesis. In conclusion, our comprehensive proteomic analysis provides insights into the molecular alterations associated with RA-induced CP in Kun Ming mice. These findings suggest potential therapeutic targets and pathways to understand and prevent congenital craniofacial anomalies.
The Royal College of Surgeons Cleft Steering Group and the Craniofacial Society of Great Britain and Ireland have recommended that, for patients with a cleft of the lip and/or palate, a model is made of the deformity before primary surgery. This provides a record for audit and can be used to compare the results of treatment. There is no standardisation in the way in which a model of the cleft deformity is made, which makes inter-unit comparison difficult. In this paper, we describe our technique for making a composite model of the cleft palate, lip and nose. This method is adaptable and quick to perform, and the models are easy to store.
Orthognathic surgery has become a standard procedure in cranio-maxillo-facial surgery during the past five decades. Based upon the elementary works by Obwegeser who introduced sagittal split ramus osteotomy in the early 1950s, this procedure has become the worldwide gold standard in mandibular orthognathic procedures by now. When devices for ensuring the centric condylar position throughout the entire surgery were introduced by Luhr in the early 1980s, modern understanding of complete functional rehabilitation in orthognathic surgery was further enhanced. Even though Le Fort I osteotomy was performed by Wassmund already in the 1920s, it took almost another 50 years until this procedure became accepted in surgery, mainly due to concerns for severe haemorrhage that may occur during surgery. Application of a compound condylar positioning device which was established to ensure and keep the exact condylar position throughout maxillary, mandibular, as well as during bimaxillary osteotomies, and to permit the targeted use of distraction devices. Among those, cleft patients also sometimes need orthognathic correction of typical micrognathia and pseudoprogenia in late adolescence or at early adult age as secondary cleft surgery procedures. In this overview, special emphasis will be placed on orthodontic treatment as well as on the technical prerequisites for the surgical procedure.