The Cleft palate journal最新文献

The nature and severity of congenital heart disease in 78 patients who presented with clefts of the lip and/or palate is reported. The prevalence of bilateral cleft lip and palate in patients with heart lesions was much higher than in cleft patients with normal hearts. Cardiac defects were predominantly conotruncal. Tetralogy of Fallot was present in 24 percent of patients; the prevalence of transposition, atrioventricular septal defect, and truncus arteriosus was also disproportionately high. Patients with conotruncal defects had a greater prevalence of either unilateral or bilateral cleft lip and palate. Most patients with congenital heart disease and clefting had additional abnormalities. Anomalies of other systems were found to be present in 87 percent of patients.

Using an extra-oral approach, subperiosteal pockets were created bilaterally over zygomatic and mandibular areas in six Rhesus monkeys. One side of each anatomic site received a Proplast I implant and the contralateral a nonporous hydroxylapatite (NPHA) block. Two animals were killed postoperatively at 3, 6, and 12 months, and the implants retrieved en bloc for histologic evaluation. Clinical evaluation showed the Proplast implants more stable than NPHA implants. Histologic evaluation for Proplast implants demonstrated complete encapsulation, fibrous tissue infiltration, fragmentation of implants, and some giant cell reaction. The NPHA implants were completely encapsulated with fibrous tissues, and no giant cell response, fragmentation, biodegradation, or bone formation was observed. We concluded that the Proplast was more stable than NPHA implants, but the NPHA produced less inflammatory cell and giant cell reaction.

The pressure-flow method introduced by Warren and DuBois is a useful method for estimating velopharyngeal orifice area. However, this investigation shows that unless geometric similarity of subject and model exists, the value of the flow coefficient k used in the equation to estimate velopharyngeal orifice area cannot be established from model tests. Use of k = 0.65 is questioned as that value is typical of thin plate orifices, a geometry that is not a good representation of the velopharyngeal passage. Values of k from the literature and from steady state tests using a model similar to Warren's are presented to show the effect of various inlet shapes. The influence of inlet shape supports the conclusion that k may be significantly higher than 0.65, conceivably approaching values in the range of 0.9 to 0.97, depending on the orifice geometry.

A number of inherited craniofacial diseases are known to be associated with gene mutations. Inherited genetic disorders of enamel formation called amelogenesis imperfecta (AI) affect the human population with a prevalence of 1 in 14,000 in the United States. Amelogenins, the major proteins in developing enamel matrix of mammalian teeth, have been suggested to participate in normal enamel matrix biomineralization, as well as with abnormal biomineralization such as seen in AI. The complementary DNA for mouse amelogenin gene (AMEL) has been cloned, characterized, and used as a probe to establish the chromosomal locations of AMEL for mouse and man. The human AMEL gene sequences have been located to the distal short arm p22.1----p22.3 region of the X chromosome, and the pericentromeric region of the Y chromosome. An assignment of human AMEL gene to the X chromosome p22 region together with a recent assignment of the X-linked AI disease locus to the Xp22.2 region support the association of the AMEL-X gene with AI. This also leads us to propose that a mutated AMEL-X gene produces altered amelogenin polypeptide, which is defective in its ability to participate in mineralization of enamel matrix, thus giving rise to the X-linked phenotypes of AI.

The contemporary management of patients with craniofacial anomalies involves a team approach of multiple specialists. Interaction between the basic scientist, developmental biologist, and the clinician should be an important aspect of interdisciplinary research if clinically relevant questions are to be addressed under rigorous laboratory conditions. The clinical investigator may be unfamiliar with the terminology and methodology of basic biomedical research. As a result, there can be a lack of communication between the clinician and the basic scientist. The role of the orthodontist in a multidisciplinary craniofacial team has evolved largely from an interest in dysmorphology as applied to craniofacial growth and development. The predictability of orthognathic surgery has been derived from the close collaboration between the orthodontist and surgeon and may be applied to the more comprehensive management of patients with craniofacial anomalies. To determine the mechanisms underlying the pathogenesis and response to treatment will require a collaborative approach by the clinician and research scientist to elucidate causal and putative risk factors in the complex genetic and environmental associations involved in craniofacial anomalies.

The maxillary arch-form of 41 Sri Lankan adults with unoperated complete unilateral cleft lip and palate (8 female and 33 male) were compared to a control group of 100 normal Sri Lankan adults (45 female and 55 male). The teeth in the cleft group were smaller than their equivalents in the control group, the most marked difference being found in the central and lateral incisors. Arch widths of the cleft groups were reduced, more anteriorly than posteriorly, resulting in more V-shaped arches. No differences were found in the arch length or chord lengths between the groups. Crossbites occurred in 19.5 percent of the cleft group as compared to none of the controls. The overjet was greater in the cleft group than in the controls. A higher percentage of missing teeth was recorded in the cleft group.

This paper describes the training, which took place in Sri Lanka, of five speech therapy assistants in cleft palate speech therapy. The context in which this training occurred is described. The original aims of the training and the criteria drawn up for the selection of the assistants are contrasted with the modifications required to these, once the British therapists started working in Sri Lanka. Information is given on the training objectives, syllabus, curriculum, and methods of evaluation of the assistants' progress. The achievements of the assistants are described. Factors relating to the difficulties and ease with which the assistants were trained are highlighted. The paper concludes with a discussion of the cultural, political, and clinical problems encountered in this type of training, and highlights the special difficulties encountered in the training of assistants in the field of speech therapy in developing countries.