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The correction of cleft palate with primary veloplasty and delayed repair of the hard palate.

Optimal repair of the cleft palate requires meticulous balance of the effects of the procedure on craniofacial growth, dentition, and speech. Aggressive, early closure of the entire palate was practiced by the senior author many years ago. Successful closure with reasonable speech was produced at the price of severe facial and dental disturbance. Patients were adversely affected for many years before the "antisurgery war-cry" of dentists and orthodontists tempered our enthusiasm. In the past 5 or 6 years, the din of speech therapists again advocating early closures has arisen. Besieged on both sides, the plastic surgeon must temper the demands of both camps, seeking the overall benefit of the patient. We believe that primary veloplasty and delayed hard-palate closure as demonstrated in this series provide superior speech with minimal deleterious side effects. The results of intravelar veloplasty, when combined with two-step closure, are especially gratifying. It is hoped that the further refinement of this technique in the past several years will improve results even further.

Child, Preschool↗

Relevance of the palatal protein kinase A pathway to the pathogenesis of cleft palate by secalonic acid D in mice.

Secalonic acid-D (SAD) is a teratogenic mycotoxin inducing cleft palate (CP) in the offspring of the exposed mice by reducing palatal shelf size secondary to reduced proliferation of the palatal mesenchymal (PM) cells. Co-administration of dimethylsulfoxide (DMSO) reversed the CP-inducing effect of SAD. Although SAD has been shown to affect both protein kinases A (PKA) and C (PKC) pathways, the relevance of each of these pathways to its CP induction is unknown. The present studies were designed to test the hypothesis that the protective effect of DMSO is mediated by its specific reversal of the effect(s) of SAD on one of these two pathways using ELISA-based activity assays, Western blot analysis, electrophoretic mobility shift assays (EMSA), and murine embryonic PM (MEPM) cell growth in culture. Within the PKA pathway, SAD inhibited the activity of the catalytic subunit of PKA and its migration into the nucleus, elevated phosphorylated cyclic AMP (cAMP) response element (CRE)-binding protein (pCREB) level, and reduced the binding of CREB to CRE. In the PKC pathway, SAD reduced the activity of PKC and the binding of transcription factors (TF) to 12-O-tetradecanoate-13 phorbol acetate-response element (TRE). SAD also inhibited MEPM cell growth and the expression of the CRE- and TRE-containing gene, proliferating cell nuclear antigen (PCNA). Reversal, by DMSO, of the effects of SAD on MEPM cell growth, on PCNA expression and on all components of the PKA, but not of PKC, pathway suggests that the perturbation of the PKA pathway by SAD is relevant to its induction of CP in mice.

Animals↗

Development of articulation before delayed hard-palate closure in children with cleft palate: a cross-sectional study.

The development of articulation before surgical closure of the hard palate was compared in 75 preschool children with cleft lip and palate and 40 preschool children born without clefts. The children were aged 2 years to 5 years 11 months. The patients had significantly poorer articulation skills than the controls at each age level. Substitutions were the most frequent error, and they did not decrease with age in the patients. Fistula size and a history of speech therapy were significant factors in the articulation error scores only in 5-year-olds. No advantage in articulation proficiency was found for those who had worn a prosthesis to occlude the hard-palate defect.

Articulation Disorders↗

A comparison of babbling and speech at pre-speech level, 3, and 5 years of age in children with cleft lip and palate treated with delayed hard palate closure.

Babbling and speech in 21 children with cleft palate were compared at pre-speech level, 3, and 5 years of age. The aims were to study if misarticulations in pre-school speech appear to be articulatorily related to the sound productions in pre-speech, whether the feeding technique influenced the prevalence of anterior articulation, and if there was a relationship between speech and the size of the residual cleft at 3 and 5 years of age. All the children had the soft palate closed, whereas the cleft in the hard palate was left open to be closed later on. Perceptual judgement of speech revealed a high prevalence of hypernasality, nasal escape and retracted oral articulation of dental or alveolar plosives. The latter was correlated with the size of the residual cleft area. There was a tendency towards a relationship between absence of anterior sound productions in babbling and retracted oral articulation in speech. The feeding technique, however, appeared not to have had any influence on articulatory place.

Articulation Disorders↗

Tongue-palate contact during selected vowels in children with cleft palate.

This study reports tongue-palate contact recorded using electropalatography (EPG) during five vowels /i/, /theta/, /I/, /o/ and /backwards c/ spoken by school-aged children with cleft palate and a group of normal speakers. All the children had articulation disorders affecting consonants but none had obvious vowel errors. Two measures were taken from the EPG data at the temporal midpoint of the vowels. The first identified the percentage of vowels produced with complete coronal constriction and the second calculated amount of contact. The results showed that children with cleft palate frequently produced the high vowel /i/ with complete constriction, with 40% of /i/ targets articulated in this way. There were lower percentages for /theta/ and /I/ and no complete constrictions during the lower vowels /o/ and /backwards c/. None of the normal speakers produced any vowels with complete constriction. In terms of amount of contact, the vowels ranked /i/>/theta/>/I/>/o/>/backwards c/, with /i/ having the most and /backwards c/ the least contact. Although this ranking held for both groups, the cleft group had more contact than normal speakers, especially during high vowels. Complete constriction is viewed as a clinically relevant phenomenon that blocks oral airflow and as a result increases nasal airflow during vowels.

Adolescent↗

Dexamethasone receptor levels in palatal and lung fibroblasts of adult A/J and C57BL/6J mice: relationship to glucocorticoid-induced cleft palate.

Glucocorticoid-induced cleft palate (CP) has been used as an animal model for hormonal teratogenesis. In mice, the susceptibility to glucocorticoid-induced CP varies with the strain, A/J being very sensitive and C57/BL6J relatively resistant. Studies in adult and embryonic murine tissues have attempted to correlate the number of glucocorticoid receptors and CP susceptibility, with conflicting results. The relative quantities of dexamethasone receptors were now studied in established palatal and lung fibroblast cell cultures obtained from adult C57 and A/J mice. A rapidly saturable, stable binding system was demonstrated. Scatchard plots were linear indicating a single class of high affinity receptors. The glucocorticoid receptor number ranged from 6.2 x 10(-16) mole/microgram prot to 8.6 x 10(-16) mole/microgram prot, while the KD varied from 1.0 x 10(-8) M to 2.8 x 10(-8) M. The differences in receptor characteristics between murine strains were not significant (p greater than 0.05). The absence of a difference in receptor number between the two strains may reflect the limitation of fibroblast cell culture in assessing glucocorticoid binding in vivo. Alternatively, if a difference in palatal dexamethasone receptor levels between mice strains exists, it may occur only in the embryo.

Animals↗

Closure of the soft palate for persistent otorrhea after placement of pressure equalization tubes in cleft palate infants.

Four case reports of infants with cleft palate and intractable otorrhea following the placement of pressure equalization tubes are presented. In one patient, liquids taken orally were noted to reflux through her ears. Otorrhea was refractory to medical management in all cases and was controlled only after closure of the soft palate. Persistent otorrhea may be an indication for early closure of the soft palate in these infants.

Cleft Palate↗

Susceptibility of mice to phenytoin-induced cleft palate correlated with inhibition of fetal palatal RNA and protein synthesis (41255).

Phenytoin administered to pregnant mice during the critical embryonic period of palatal differentiation produced 50% cleft palates in the Ajax (A/J) strain compared to 1.6% clefts in the C57BL/6 (B6) strain of mice. Furthermore, a single maternal injection of phenytoin produced a significantly greater and more persistent decrease in fetal palatal RNA and protein synthesis in the sensitive A/J strain compared to that in the insensitive B6 strain of mice. Thus, these differential effects of phenytoin on RNA and protein synthesis are associated with the differential susceptibility to the teratogenic action of phenytoin in the two strains.

Animals↗

Effects of variation in the timing of palatal repair on sagittal craniofacial morphology in complete cleft lip and palate children.

The complete cleft lip and palate children, ranging from 6-14 years of age were studied to evaluate the effect of variation in the timing of palatal repair on craniofacial morphology and compared to the noncleft children. It was observed that all the groups early (8 to < or = 24 months), medium (> 24 to < or = 36 months) and late repair (> 36 to < or = 78 months) had significantly larger cranial base, retruded maxillomandibular relations, skeletodental and incisal relationships compared to the noncleft children. However, intercomparison among the cleft groups showed insignificant difference amongst them suggesting that the timing of palatal repairs does not effect the anterioposterior (sagittal) relationship.

Adolescent↗

Distribution of palatal and other arteries in cleft and non-cleft human palates.

Cleft lip and cleft palate are two possible effects of fusion failure of embryonic facial processes. Aberrant facial morphogenesis suggest aberrant arterial distribution. Prompted by surgical need, studies detailing major branches of the third or pterygopalatine portion of the maxillary artery acquire practical significance. Therefore postomortem arteriographic studies were undertaken to ascertain location of these major arteries and their branches in twelve near-term human fetuses. Comparison was made between arterial distributions in three cleft and nine non-cleft fetal palates. The question of bilateral symmetry was examined. It is known that these major branches are commonly present on both sides, but the study revealed numberous variations in each facial half in both cleft and non-cleft palates. Variation implies morphologic contradiction with sterotype presentations. So numberous are the variations that occasionally they may account for sudden and unexpected hemorrhage during surgery/and retarded healing or sphacelus of flaps following surgery.

Alveolar Process↗

A lining vomer flap for palate pushback in unilateral cleft palate repair.

A combinaation vomer mucoperiosteal flap and nasal floor mucoperiosteal flap is described which is used to achieve nasal coverage in unilateral cleft palate patients requiring pushbacks. A posteriorly based readily accessible vomer flap is raised on the cleft side and used as nasal lining for the palatal mucoperiosteal flap on the non-cleft side. On the cleft side, a symmetrically sized nasal floor flap is easily elevated under direct vision and used to cover the nasal aspect of the corresponding mucoperiosteal palatal flap.

Cleft Palate↗

Palatal adhesion: the treatment of unilateral palatal paralysis after high vagus nerve injury.

BACKGROUND: Resection of skull base tumors commonly necessitates intraoperative sacrifice of lower cranial nerves at the level of the jugular foramen. Sequelae of unilateral vagus nerve loss include ipsilateral laryngeal paralysis, ipsilateral palatal and pharyngeal paralysis, and velopharyngeal incompetence (VPI) marked by hypernasal speech and nasopharyngeal reflux of liquids during swallowing. METHODS: Palatal adhesion (PA), a procedure whereby the unilaterally paralyzed palate is attached to the posterior pharyngeal wall, decreases the size of the velopharyngeal port and minimizes the symptoms. This study assessed the outcome of PA in 31 patients with VPI secondary to proximal vagus nerve injury. RESULTS: PA decreased postoperative nasality in 96% of patients. Nasopharyngeal reflux was significantly improved in 83%. Three patients (11%) had minor wound breakdown postoperatively, all of which healed completely with conservative management. CONCLUSION: PA offers a favorable result with minimal concomitant morbidity and is recommended for patients with VPI secondary to unilateral proximal vagus nerve paralysis.

Adult↗

Palate development in the mouse: a quantitative method that permits the estimation of time and rate of palate closure.

A quantitative technique is used to express the morphogenetic stages of palate development in the mouse in terms of gestational age and the normal probability distribution. The qualitative palate rating scale for mouse embryos was modified and the numbers of embryos that had reached the different palate stages at specific gestational ages were fitted to a cumulative multinomial frequency distribution by normal probability regression on gestational age. The median time for each stage can be estimated and the standard deviation of this time (slope of the probability regression) provides a measure of the rate of development. A/J and A.B6 F1 embryos (A/J maternal environment) differed significantly in median time but not in rate of development during a restricted examination time.

Animals↗

Phonetic and otological results after early palate closure in 18 consecutive children presenting with cleft lip and palate.

OBJECTIVE: First, to analyze the speech and hearing results at 3.5 years of age when early palate closure has been performed. Second to assess at 7 years of age the need for speech therapy and/or additional surgery in the form of cranial-based pharyngeal flap for obtaining normal speech. METHODS: Retrospective study in a tertiary teaching hospital concerning 18 consecutive cases presenting cleft lip and palate with no associated abnormalities. Interventions included early palatine closure (velum at 3 months, hard palate and lip at 6 months). Speech therapy was initiated at 3.5 years of age when needed. Cranial-based pharyngeal flap was performed when normal nasal emission was not obtained by speech therapy at 7 years of age. Phonetic and otological assessment were performed in all cases. RESULTS: Good to excellent speech in the majority (95%) of children, with only 3/18 undergoing pharyngoplasty to obtain type I or I/II speech by the age of 7 (range: 6.5-8.0). 6/18 children had drain insertion, and 2/18 had hearing loss of 20-40 dB in all frequencies. CONCLUSION: Most children (95%) start school with good or excellent speech. However, the high incidence of middle ear problems shows that more effective screening and treatment are warranted.

Acoustic Impedance Tests↗

Long-term results of segmental repositioning of the maxilla in cleft palate patients without previously grafted alveolo-palatal clefts.

Eleven patients (9 UCLP, 2 BCLP) were treated with segmental osteotomies with or without osteotomies at the Le Fort I level and simultaneous bone grafting of the alveolo-palatal clefts at adult age. These patients were clinically and radiographically evaluated after a mean follow-up period of 59 months (range 39-110 months). One patient showed complete dentoalveolar relapse, whereas the skeletal stability after miniplate fixation proved to be adequate in all cases. Only one patient presented with a persisting oro-nasal fistula. In six cases, the alar base asymmetry had improved to such an extent that further nasal corrections were not necessary. The procedure described is a reliable technique to graft the alveolo-palatal cleft and reposition the dentoalveolar segments simultaneously in those adult cleft palate patients who had no previous alveolar bone grafting.

Adolescent↗

A six-centre international study of the outcome of treatment in patients with clefts of the lip and palate: the results of a cross-linguistic investigation of cleft palate speech.

Speech samples of 131 subjects with complete unilateral clefts of the lip and palate from six European cleft palate centres were analysed and assessed using a specifically designed phonetic framework. This framework focused on consonants that are "vulnerable" in speech associated with cleft palate and common to the five languages of the project. The methodology used and the results of the reliability study are reported. Consonant articulation, resonance, and voice quality are also evaluated. The results show good outcomes with regard to consonant articulation across the whole study group with common areas of minor difficulty across languages. The results for resonance were less good, with slight hypernasality in 20% of subjects. There were, however, few indications of seriously disordered speech. The detectable differences between centres match the findings of the Eurocleft Orthodontic Group particularly in regard to the ranking of the centres.

Adolescent↗

Genetic analysis of TTF-2 gene in children with congenital hypothyroidism and cleft palate, congenital hypothyroidism, or isolated cleft palate.

Homozygous null mice for thyroid transcription factor (TTF)-2 gene exhibit cleft palate and thyroid malformation. We performed a genetic analysis of the TTF-2 gene in 2 children with congenital hypothyroidism (CH) and cleft palate, 45 children with thyroid dysgenesis, 19 children with isolated cleft palate or cleft lip, 4 patients with thyroid hemiagenesis. The entire coding-region of the TTF-2 gene was analyzed by direct sequencing. Direct sequencing of the TTF-2 gene revealed polymorphisms in the length of the polyalanine tract. The most frequent stretch length was 14 residues and it was found in 50 of 70 (71%) and in 45 of 53 (85%) normal healthy controls. A polyalanine tract of 16 residues in the heterozygous state was seen in 18 of 70 (26%) cases and in 4 of 53 (7%) normal subjects. In 1 of 4 (25%) case of hemiagenesis a polyalanine tract of 16 residues in the homozygous state was observed. In 1 of 26 agenesis the polyalanine tract consisted of 12 residues in the heterozygous state. Direct sequencing also revealed the presence of two silent polymorphisms. No mutations were identified in the TTF-2 gene. In conclusion, our results show that no genetic alteration was present in the TTF-2 gene of these patients, suggesting that defects in the TTF-2 gene are a rare event.

Adolescent↗

The Langerhans cell density of palatal epithelium in denture and non-denture wearers, as correlated with other parameters of the palatal mucosa.

A quantitative histological investigation was carried out on biopsy specimens taken from patients suffering from denture sore mouth. The results were compared with those obtained in investigations on denture and non-denture wearers. The sections were studied by standardized quantitative morphometric methods. After 4 years the denture bearing palatal epithelium from both groups, normal denture wearers and the patients suffering from denture sore mouth did not show changes in mean thickness of the epithelium as compared with the controls. The mitotic index in denture bearing epithelium from patients suffering from denture sore mouth was three times lower than in the epithelium of the normal denture wearers. The number of Langerhans cells correlated with the mitotic indices of the group of denture wearers and non-denture wearers. The group of denture sore mouth patients showing a low mitotic index showed a high number of Langerhans cells in their palatal epithelium. The three groups of patients investigated did not show differences in density of mast cells in the lamina propria of their palatal epithelium.

Adult↗