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Item Cleft palate: a genetic and epidemiologic investigation(1978) Shields, Edward D.Item Craniofacial Morphology in familial cases of cleft lip/palate: phenotypic heterogeneity and genetic predisposition in unaffected family members(1993) Litz, Stephanie M.; Bixler, David; Fleener, Donald E.; Hennon, David Kent, 1933-; Sadove, A. Michael; Ward, Richard E.; Avery, David R.This study investigated familial cases of cleft lip with or without cleft palate to determine whether the unaffected members of each family can be identified as gene carriers for the cleft trait. This research presumes that such carriers will have henotypic features identifiable by cephalometric analysis that are associated with an increased risk to cleft offspring. Using population genetics methodology, a pedigree analysis was made for each family member was assigned to one of four groups: (1) obligate normal, (2) affected, (3) carrier, and (4) unknown. LA and PA cephalographs were taken on each subject and a clinical oral-facial examination carried out on participating family members. Various anatomic landmarks located on the LA and PA films were digitized and from them, a total of 28 linear measurements were made. To eliminate the effect of sex and differential age responses, Z scores were calculated. Through univariate analysis, only one variable, NCR-MO, was shown to be significantly different between the two groups. This variable difference by itself is not adequate to differentiate those in the normal group from the carrier group. Even though only one variable was significant, other differences in the variables between these groups become obvious when the group variables were plotted as Z scores. Since Z scores are pure values with no limits (2--the number of standard deviations in a given variable differs from normal). Thereby, age-related growth differences were minimized. Further information is gained when these Z scores are plotted as pattern profiles, Figures 5-7. These profiles of mean Z scores for each variable pointed out areas of the face in which the differences were so great that specific anatomic areas appeared to be associated with one of the four groups. For example, gene carriers demonstrated specific alterations in facial height that might conceivably be used to discriminate that group from the other three groups. The family normals and carriers were then analyzed by using a stepwise multivariate analysis. By this approach, a discriminant function was generated consisting of six variables (three each from the lateral and frontal headplates), which proved to be significant in distinguishing an individual's phenotype. These variables define facial height, width and depth. The specific findings included a decrease in mid-facial height and depth along with an increased lower facial height and width in the gene carrier population as compared to the normals. The function then was used to predict group membership of the same two groups. Comparing this analytical prediction to that of the grouping system that resulted from the pedigree analysis, all but one individual was classified correctly in both the normal and carrier population. A discriminant score was also determined for the unknown population of family members which were defined as non-cleft blood relatives of cleft probands. Thus, they were a mixture of two types--those unaffected who carried a genetic liability for producing a cleft child and those unaffected who did not. A prediction of their placement into either the normal or carrier group was made with the discriminate function. One-third were classed in the normal group and two-thirds as gene carriers. The results of this study confirm that the phenotype of these unaffected family members designated as obligate gene carriers differs significantly from that of the family normals. This information is not only quite useful for genetic counselling but gives both a better understanding or the genetic control of clefting and can lead to molecular research to identify the specific gene in question.Item Long Term Maxillary Growth Following Primary Bone Grafting in Unilateral Cleft Lip and Palate(1996) Gandelsman, Genrikh; Hathaway, Ronald R.; Arbuckle, Gordon R.; Hennon, David K.; Katona, Thomas R.; Shanks, James C.The question of growth attenuation of the maxillary complex arises in connection with primary osteoplasty procedure. The deficiencies associated with the development of the jaws in unilateral complete cleft lip and palate (UCLP) children is an ongoing problem in terms of growth inhibition and orthodontic treatment. Retardation of growth can be attributed to intrinsic cleft factors and/or associated with the treatment of the cleft condition. In this study, the extent of such deficiency, if any, was investigated by assessing arch length, arch width, arch symmetry and inter-arch harmony. Seventeen orthodontic casts of UCLP children (mean age 7 years 11 months) were obtained. All were treated with a primary osteoplasty at James Whitcomb Riley Hospital for Children at Indianapolis, Indiana. The sample was compared to an age and sex matched non-cleft control group (n=38). None of the subjects had undergone orthodontic treatment beyond infant maxillary orthopedics. Anatomic landmarks were identified and digitized by means of optical electronics. The data were automatically fed into a computer which executed preprogrammed data manipulation algorithms. Significant (p<0.05) inhibition of growth has taken place in the maxillary arches of the UCLP group in both anteroposterior and transverse directions. lntercanine width (ICW) was reduced on average by 7.6 mm (23.6 percent) while the intermolar width (IMW) was shortened by 3.9 mm (7.8 percent). Sagittal growth was retarded by 5.5 mm (17.4 percent). A gradient of "normalization" originating at a point of surgical insult was observed anteroposteriorly in the transverse maxillary dimension. In the mandible, ICW was retarded by 2.0 mm (7.8 percent) while IMW and A-P growth vectors were not significantly affected. This investigation also revealed significant (p = 0.0001) differences in the size of the maxillary anterior palatal area (cleft mean = 83.5 mm2, non-cleft mean = 133.9 mm2). These findings lead to the conclusion that primary osteoplasty may contribute to maxillary growth attenuation with concomitant mandibular compensatory growth patterns.Item Relationship of enamel hypoplasia and trauma in repaired cleft lip and palate(1961) Mink, John R.Item Self-concept of parental evaluation of peer relationships in cleft lip and palate children(1983) Jones, James E.; Barton, Paul; Harshman, Hardwick W., 1914-; Starkey, Paul E.; Avery, David R.; Bixler, DavidThis investigation examined the relationship of the self-concept of children with cleft lip and palate to the self-concept of non-cleft children. Fifty cleft lip and palate children between the ages of eight and 18 were individually matched by age, sex and race with 50 non-cleft children. Each child was given the Piers-Harris Children's Self-Concept Scale. Children with clefts, regardless of sex, reported significantly lower global self-concept than non-cleft subjects (p < .005). Further significant differences between cleft and non-cleft subjects were found in five of six cluster scores. These included: behavior (p < .05), school status (p < .05), popularity (p < .05), happiness and satisfaction (p < .001), and physical attributes and appearance (p < .001). A significant effect (p < .01), was found on the popularity score, with cleft males feeling less popular than their non-cleft peers. A significant effect was also found on the anxiety score, with cleft females reporting significantly more anxiety (p < .01) than their non-cleft peers. A questionnaire was completed by the parents of the cleft and non-cleft subjects evaluating their child's relationship with family, peers and progress in school. In general, parents of both groups reported positive ratings of their child's social interactions. Parents of cleft. . subjects reported more negative responses than the parents of non-cleft subjects concerning the teasing the child experiences because of his/her facial appearance (p < .05) and the effect that the child's facial appearance had on progress in school (p < .05).Item A study of the hereditary basis of cleft lip and cleft palate(1966) Coccia, Chester Tullio, 1935-This study attempted to identify the incomplete manifestations of gene(s) producing cleft lip and/or cleft palate. An effort was made to determine how various oral-facial discrepancies, when considered as incomplete manifestations of clefting, might produce an obvious mode of inheritance of the clefting trait i n families with a history of cleft lip and/or cleft palate. Eleven families were studied, selection being made on the basis of the degree of relationship of the affected individuals to the proband. Oral and facial discrepancies including bifid uvula, mandibular lip pits, commissural lip pits, raphe of the upper lip, notching of the alveolus, asymmetrical nasal shape, congenitally missing and malformed maxillary central and lateral incisors, and excessive crowding of the teeth in the anterior segment of the maxillary arch were selected as possible incomplete manifestations of gene action. The results of this study demonstrated that when these manifestations were considered together with the cleft lip and/or cleft palate condition, no additional information on the manner of inheritance of the cleft lip and/or cleft palate trait was produced. Ocular and orbital measurements, which may also be used in determining possible mid-facial discrepancies, were compared between the relatives of families with a history of clefting and a control group of individuals ranging from four to 22 years of age. A total of 166 individuals were compared. Significant differences in the circumference-interorbital index, skeletal interorbital distance, and the canthal index were found. Since these significant differences in the ocular and orbital measurements could not be significantly related to the degree of relationship between affecteds (the test of heritability), they should be held with some reservation until a larger sample can be studied.