bluuebunny/crossref_metadata_2025_split huggingface dataset:
Perceived Social Support of Mothers of Children with Clefts
Authors: Sank Jessica R. , Berk Nancy W. , Cooper Margaret E. , Marazita Mary L. 
Published: October 2018

Objective To identify factors that may predict perceived social support in mothers of children with clefts. Design A comparison was conducted from a sample of mothers of children with oral clefts identified from a single institution (multidisciplinary craniofacial clinic). The Interpersonal Support Evaluation List and Beck Depression Inventory were completed by all participants. Results Mothers of children with visible clefts were more likely to report higher perceived social support than mothers of children with clefts that were not visible (p = .005). Mothers of female children reported higher levels of perceived social support than mothers of male children (p = .02). Mothers who had completed more years of education reported significantly higher levels of perceived social support than mothers who had completed fewer years of school (p = .03). Mothers with more children were more likely to report lower levels of perceived social support (p = .009). Maternal depression was highly correlated with perceived social support (p < .0001). Conclusions Cleft visibility, family size, child sex, and maternal education level were important factors in predicting perceived social support in mothers of children with clefts. Child age, syndrome status, and cleft family history were not significant predictors.
Report of a Patient with Hypoglossia-Hypodactylia Syndrome and a Review of the Literature
Authors: Yasuda Yoshitaka , Kitai Noriyuki , Fujii Yuka , Murakami Shumei , Takada Kenji 
Published: October 2018

Objective To present the morphology of the extremities, craniofacial structures, and the oral cavity based on roentgencephalometry and three-dimensional computed tomography and magnetic resonance imaging scanning in a patient with hypoglossia-hypodactylia syndrome, discuss the orthodontic treatment method, and review the literature for the syndrome. Patient The patient was a 6-year-old boy diagnosed with hypoglossia-hypodactylia syndrome at birth. He had hypodactylia as well as micrognathia with steep inclination of the anterior surface of the mandible in relation to the lower mandibular plane. He had missing mandibular incisors with concomitant bone defect limited to the associated alveolar ridge and an absence of any malformations in the mandibular ramus and condylar head. The patient had a bilateral scissors bite with an extremely constricted mandibular dental arch, skeletal Class II jaw relationship with an average mandibular plane angle and maxillary incisors inclined palatally. He had extremely reduced tongue size and hypertrophy of the floor of the mouth. Anomalies of the central nervous system were not observed. There was no evidence of hearing loss.
Rigid External Distraction Osteogenesis for a Patient with Maxillary Hypoplasia and Oligodontia
Authors: Kitai Noriyuki , Kawasaki Keishi , Yasuda Yoshitaka , Kogo Mikihiko , Murakami Shumei , Kreiborg Sven , Takada Kenji 
Published: October 2018

Objective In this report, the orthodontic treatment combined with rigid external distraction osteogenesis in a 5.5-year-old girl with midfacial hypoplasia and oligodontia is described. Patient The child presented with a reduced maxilla, protruding lower lip, skeletal Class III jaw relationship with a low mandibular plane angle, a short and flattened nose, anterior crossbite, and aplasia of 16 permanent teeth. The patient was treated with rigid external maxillary distraction osteogenesis, maxillary protraction headgear, and Class III elastics. Following treatment, the maxilla was displaced in a forward direction with new bone formation at the tuberosities and the mandible rotated backward in relation to the anterior cranial base. The anterior crossbite was corrected, and the skeletal jaw relationship changed from a Class III to a Class I skeletal pattern. The soft tissue facial profile showed that the nasal projection had been increased, the nasolabial angle increased, and the lower lip protrusion was reduced. Postoperative treatment results were acceptable. Conclusion This report documents that early maxillary advancement with rigid external osteogenesis offers a promising treatment alternative for a very young patient with maxillary hypoplasia and oligodontia.
Genetic Association Studies of Cleft Lip and/or Palate with Hypodontia outside the Cleft Region
Authors: Slayton Rebecca L. , Williams Laura , Murray Jeffrey C. , Wheeler James J. , Lidral Andrew C. , Nishimura Carla J. 
Published: October 2018

Objective The purpose of this study was to determine whether the candidate genes previously studied in subjects with cleft lip, cleft palate, or both are associated with hypodontia outside the region of the cleft. Subjects One hundred twenty subjects from the Iowa Craniofacial Anomalies Research Center were selected based on the availability of both dental records and genotype information. Method The type of orofacial clefting and type and location of dental anomalies (missing teeth, supernumerary teeth, or peg laterals) were assessed by dental chart review and radiographic examination. Genotype analysis of candidate genes was performed using polymerase chain reaction/single-strand conformation polymorphism analysis. Results The prevalence of hypodontia in this sample was 47.5%, with 30.0% of subjects having missing teeth outside the cleft. There was a positive association between subjects with cleft lip or cleft lip and palate who had hypodontia outside the cleft region (compared with noncleft controls) and both muscle segment homeo box homolog 1 (MSX1) (p = .029) and transforming growth factor beta 3 (TGFB3) (p = .024). It was not possible in this analysis to determine whether this association was specifically associated with orofacial clefting combined with hypodontia or whether it was due primarily to the clefting phenotype. Conclusions In this sample, there was a significantly greater incidence of hypodontia outside the cleft region in subjects with cleft lip and palate, compared with cleft lip only or cleft palate only. Cleft lip and/or palate with hypodontia outside the cleft region was positively associated with both TGFB3 and MSX1, compared with noncleft controls.
Negative Transcriptional Regulation of Connexin 43 by Tbx2 in Rat Immature Coronal Sutures and ROS 17/2.8 Cells in Culture
Authors: Borke James L. , Chen Jung-Ren , Yu Jack C. , Bollag Roni J. , Orellana Maria F. , Isales Carlos M. 
Published: October 2018

Background Tbx2 is a member of the T-box family of transcriptional regulatory genes with an extensive but not yet fully understood role in embryonic development. This study explores the potential role of Tbx2 in calvarial morphogenesis. Objectives To explore the hypothesis that Tbx2 has a negative regulatory effect on the expression of connexin 43 (Cx43), a protein necessary for cell-to-cell communication; document the presence of Tbx2 protein in the developing cranial sutures; and determine the spatial pattern of expression of this developmentally regulated transcription factor in calvariae. Design The osteoblast-like cell line ROS 17/2.8 was stably transfected with sense or antisense Tbx2. Immunohistochemistry and Western blotting was used to study Tbx2 and Cx43 expression in these cells and sections of embedded developing coronal sutures. Results The ROS 17/2.8 cells transfected with antisense Tbx2 showed a decrease in expression of Tbx2 protein and an increase in expression of endogenous Cx43. The reverse is seen with sense-transfected cells. Both of these proteins are expressed in rat developing coronal sutures. The pattern of Tbx2 expression in the developing was also reciprocal to the pattern of Cx43 expression. Tbx2 protein is concentrated in the center of the sutural blastema, an area devoid of Cx43 protein localization. Conversely, Tbx2 protein expression is low in the periphery of the sutures, in which there is high Cx43 protein expression. Conclusions Taken together, these studies suggest that Tbx2 protein is a negative regulator of Cx43 expression at the transcriptional level in cranial sutures in vivo.
Audiologic and Tympanometric Findings in Children with Cleft Lip and Palate
Authors: Tunçbilek Gökhan , Özgür Figen , Belgin Erol 
Published: October 2018

Objective To evaluate the otologic and audiologic status of 50 children with repaired cleft lip, cleft palate, or both in Hacettepe University, Ankara, Turkey. Design Audiometric and tympanometric evaluation of 100 ears in 50 children were performed. Hearing levels ≤ 15 dB and middle ear pressures between −50 to +50 decaPascals were considered to be normal. Results were examined according to cleft type and laterality. The least and most affected frequencies were calculated. A simple evaluation of speech characteristics including nasal resonance, nasal air escape, and errors of articulation was also performed. Results Sixty-three of the 100 ears had normal hearing status, whereas 40 had normal middle ear pressures. No evidence was found to suggest that individual cleft type and laterality of the ear had any effect on hearing loss or middle ear disease. Two-thirds of the patients had normal or acceptable degree of language skills. Conclusion The final hearing status of patients with cleft palate is a result of a combination of surgical correction, developmental factors, and treatment of middle ear disease. Early and aggressive ventilation tube placement is the standard of cleft care in many countries. Our long-term hearing outcome is relatively good in a population not treated with routine insertion of ventilation tubes. The majority of patients also have satisfactory speech. Patients with cleft palate should have close follow-up for middle ear disease, but further research is warranted to determine the aggressive usage of ventilation tubes.
Unilateral Advancement of the Maxillary Minor Segment by Distraction Osteogenesis in Patients with Repaired Unilateral Cleft Lip and Palate: Report of Two Cases
Authors: Kuroe Kazuto , Iino Shoichiro , Shomura Kenji , Okubo Akiro , Sugihara Kazumasa , Ito Gakuji 
Published: October 2018

Objective Collapse of the maxillary minor segment with lateral crossbite is a common feature in patients with repaired unilateral cleft lip/palate because of maxillary alveolar bony defect and palatal scar tissue. Distraction osteogenesis (DOG) is an effective technique of lengthening and augmentation for bone and gingiva. This case report describes the effects of unilateral advancement of the maxillary minor segment by DOG in two patients with the repaired unilateral cleft lip/palate.
Patterns of Maxillary Alveolar Arch Growth Changes of Infants with Unilateral Cleft Lip and Palate: Preliminary Findings
Authors: Braumann Bert , Keilig Ludger , Stellzig-Eisenhauer Angelika , Bourauel Christoph , Bergé Stefaan , Jäger Andreas 
Published: October 2018

Objective Because of significant differences in pathomorphology at birth, it may be that a better treatment outcome prognosis exists for patients with incomplete versus complete cleft forms. Can reaction patterns be extracted from changes in maxillary dimensions of different cleft forms within the first year of life to support this hypothesis? Subjects Chronologically consecutive casts of the maxilla (1 week and 3, 6, and 12 months of life) of 15 patients with complete unilateral cleft lip and palate (cUCLP) and 13 patients with incomplete unilateral cleft lip and palate (iUCLP). All patients were treated with passive palatal plates. Cheiloplasty was performed at 6 months of age. No primary osteoplastic surgery was carried out. Interventions Following digitizing with a three-dimensional laser scanner, all cast surfaces were computer reconstructed, aligned, and superimposed. Distances between the surfaces were determined and expressed graphically. Computer-aided determination of defined maxillary dimensions was performed. The volumes of segmented surfaces were determined and compared. Results Within the first year of life, decreased sagittal but increased transverse alveolar growth for patients with iUCLP was found. The increase in alveolar crest length in patients with iUCLP was 50% less within the first year of life than in patients with cUCLP. In the same patients, the volumes of the molar segments were, on average, larger at each registration stage and the increase in these volumes larger within the first year of life. Conclusion Conclusions regarding the direction and extent of growth cannot be drawn from the visible level of severity of the malformation.
Dental Occlusion after Veau-Wardill-Kilner versus Minimal Incision Technique Repair of Isolated Clefts of the Hard and Soft Palate
Authors: Karsten Agneta , Larson Margareta , Larson Ola 
Published: October 2018

Objective To compare the Veau-Wardill-Kilner technique with a technique similar to the minimal incision technique described by Mendosa et al. on the basis of surgical complications and dentoalveolar status in the deciduous dentition. Design Retrospective study of medical and dental records and casts. Patients A consecutive series of 129 Caucasian children born with isolated cleft palate between 1980 and 1992. Main Outcome Measures From medical records, the variables of time for surgery, blood loss, complications in the immediate postoperative period, and frequency of fistulas were evaluated. On dental casts, the variables of sagittal, transversal, and vertical relations; structure of the palatal mucosa; and height of the palatal vault were studied. Results Time for surgery was shorter in the extensive clefts repaired with a Veau-Wardill-Kilner technique. Blood loss was higher using the Veau-Wardill-Kilner technique. The width of the upper jaw was significantly narrower in the Veau-Wardill-Kilner group, compared with the minimal incision group. Scar tissue and pits of the palate were more frequently found in the Veau-Wardill-Kilner group. Conclusions The minimal incision technique in this study has been shown to result in better development of the upper jaw with a better dental occlusion and palatal mucosa with significantly less scar tissue.
A Quantitative Three-Dimensional Assessment of Abnormal Variations in Facial Soft Tissues of Adult Patients with Cleft Lip and Palate
Authors: Ferrario Virgilio F. , Sforza Chiarella , Dellavia Claudia , Tartaglia Gianluca M. , Sozzi Davide , Carù Armando 
Published: October 2018

Objective To supply quantitative information about the facial soft tissues of adult operated patients with cleft lip and palate (CLP). Design, Setting, and Patients The three-dimensional coordinates of soft tissue facial landmarks were obtained using an electromagnetic digitizer in 18 Caucasian patients with CLP (11 males and 7 females aged 19 to 27 years) and 162 healthy controls (73 females and 89 males aged 18 to 30 years). From the landmarks, 15 facial dimensions and two angles were calculated. Data were compared with those collected in healthy individuals by computing z-scores. Two summary anthropometric measurements for quantifying craniofacial variations were assessed in both the patients and reference subjects: the mean z-score (an index of overall facial size), and its SD, called the craniofacial variability index (an index of facial harmony). Results In treated patients with CLP, facial size was somewhat smaller than in normal individuals, but in all occasions the mean z-score fell inside the normal interval (mean ± 2 SD). Almost all patients had a craniofacial variability index larger than the normal interval, indicating a global disharmonious appearance. Overall, in patients pronasale, subnasale, and pogonion were more posterior, the nose was shorter and larger, the face was narrower, and the soft tissue profile and upper lip were flatter than in the reference population. Conclusions The facial soft tissue structures of adult operated patients with CLP differed from those of normal controls of the same age, sex, and ethnic group. In this patient group, surgical corrections of CLP failed to provide a completely harmonious appearance, even if the deviations from the reference were limited. Further analyses of larger groups of patients are needed.
Craniofacial Morphology in a Patient with Simpson-Golabi-Behmel Syndrome
Authors: Taniyama Tomohide , Kitai Noriyuki , Iguchi Yoshitaka , Murakami Shumei , Yanagi Miho , Takada Kenji 
Published: October 2018

Objective We present the case of a 6-year-old boy with a coarse face, cleft palate, and malocclusion with anterior open bite who had been diagnosed with Simpson-Golabi-Behmel syndrome. Morphology of the craniofacial structures was examined on the basis of conventional radiographs, three-dimensional (3D) computed tomography (CT) and magnetic resonance (MR) scanning. Patient This patient had 13 ribs on the right side, slight scoliosis, supernumerary nipples, a coarse face, hypertelorism, a short broad upturned nose, a wide mouth, a straight facial profile with incompetence of the lips, midline groove of tongue, and cleft palate. The patient also had severe anterior open bite, a distal step-type molar relationship, five congenitally missing teeth, and a supernumerary tooth. Lateral cephalometric analysis revealed a large anterior cranial base, a large maxilla and mandible, a large inferior face height, and skeletal Class I jaw relationship with a high mandibular plane angle and large gonial angle. The 3D CT image showed a large cranium, a long face height, and prominent skull sutures. The MR image showed a large tongue, midline groove of the tongue, and a small space between tongue and palate.
Segment Distraction to Reduce a Wide Alveolar Cleft before Alveolar Bone Grafting
Authors: Binger Thomas , Katsaros Christos , Rücker Martin , Spitzer Wolfgang J. 
Published: October 2018

Objective To demonstrate a method for reduction of wide alveolar clefts prior to bone grafting. This method aims to facilitate bone grafting and achieve adequate soft tissue coverage of the graft with attached gingiva. Case Report Treatment of a patient with bilateral cleft lip and palate with a severe alveolar defect on the left side is illustrated. Distraction osteogenesis was used to mesialize the left segment so that the alveolar cleft was reduced to a minimum. After a 10-week retention period, bone grafting of the reduced alveolar defect was successfully performed. After consolidation of the bone graft, the alveolar cleft was found to have an osseous closure with adequate soft tissue coverage. Conclusion In patients with a wide alveolar cleft, soft tissue coverage with keratinized mucosa can be more readily assured by reducing the alveolar cleft by callus distraction to mesialize the lateral segment prior to bone grafting.
Transcription Factor 21 Is Required for Branching Morphogenesis and Regulates the Gdnf-Axis in Kidney Development
Authors: Ide Shintaro , Finer Gal , Maezawa Yoshiro , Onay Tuncer , Souma Tomokazu , Scott Rizaldy , Ide Kana , Akimoto Yoshihiro , Li Chengjin , Ye Minghao , Zhao Xiangmin , Baba Yusuke , Minamizuka Takuya , Jin Jing , Takemoto Minoru , Yokote Koutaro , Quaggin Susan E. 
Published: October 2018

Background
The mammalian kidney develops through reciprocal inductive signals between the metanephric mesenchyme and ureteric bud. Transcription factor 21 (Tcf21) is highly expressed in the metanephric mesenchyme, including Six2-expressing cap mesenchyme and Foxd1-expressing stromal mesenchyme. Tcf21 knockout mice die in the perinatal period from severe renal hypodysplasia. In humans, Tcf21 mRNA levels are reduced in renal tissue from human fetuses with renal dysplasia. The molecular mechanisms underlying these renal defects are not yet known.

Methods
Using a variety of techniques to assess kidney development and gene expression, we compared the phenotypes of wild-type mice, mice with germline deletion of the Tcf21 gene, mice with stromal mesenchyme–specific Tcf21 deletion, and mice with cap mesenchyme–specific Tcf21 deletion.

Results
Germline deletion of Tcf21 leads to impaired ureteric bud branching and is accompanied by downregulated expression of Gdnf-Ret-Wnt11, a key pathway required for branching morphogenesis. Selective removal of Tcf21 from the renal stroma is also associated with attenuation of the Gdnf signaling axis and leads to a defect in ureteric bud branching, a paucity of collecting ducts, and a defect in urine concentration capacity. In contrast, deletion of Tcf21 from the cap mesenchyme leads to abnormal glomerulogenesis and massive proteinuria, but no downregulation of Gdnf-Ret-Wnt11 or obvious defect in branching.

Conclusions
Our findings indicate that Tcf21 has distinct roles in the cap mesenchyme and stromal mesenchyme compartments during kidney development and suggest that Tcf21 regulates key molecular pathways required for branching morphogenesis.
Galvanic Tripartite Amazon River Basin Macro- Imagineering
Authors: Cathcart Richard Brook 
Published: October 2018

<p>Referencing basic statements on macro-engineering’s innovation-phase organization penned separately by Panero and Davidson, <em>Team Geographos</em> offers a new global commerce enhancing tripartite transportation macro-project, the postulated Trans-Pacific Brazil-Peru-China Trade Route. The latitudinal TPB-P-CTR may become an economically feasible substitute or supplement to a 21st Century China-supported trans-American railway designed to connect Peru’s Pacific Ocean coast with Brazil’s Atlantic Ocean coast. In effect, a New World-originated concept that could globalize the developed infrastructure of the entire Amazon River Basin. Whilst certainly remarkably speculative, technically, the suggested TPB-P-CTR could create common destinies (yet still multi-national) for tropic zone of Peru and Brazil, operationalized by substantial financial and technical inputs mainly from China’s temperate zone seaport-based communities.</p><p class="-1"><strong>Key words: </strong>Global commerce, macro-engineering, tripartite transportation macro-project. </p><p class="-1">===========================================================================</p><p class="-1">Fazendo referência a declarações básicas sobre a organização da fase de inovação da macroengenharia, escritas separadamente por Panero e Davidson, a <em>Team Geographos</em> oferece um novo macroprojeto de transporte tripartido que fortalece o comércio global, a postulada Transpacífico Brasil-Peru-China. A latitudinal TPB-P-CTR pode se tornar um substituto ou suplemento economicamente viável para uma ferrovia transamericana com suporte da China do século 21, projetada para conectar a costa do Oceano Pacífico no Peru com a costa do Oceano Atlântico no Brasil; com efeito, um conceito originado no Novo Mundo que poderia globalizar a infraestrutura de toda a bacia do rio Amazonas. Ainda que em grande medida especulativo, do ponto de vista técnico o CTR-TPB sugerido poderia criar destinos comuns, mas ainda multinacionais, para a região do Peru e do Brasil, operacionalizados por substanciais insumos financeiros e técnicos, principalmente das comunidades baseadas em portos da zona temperada da China.</p><p><strong>Palavras-chave: </strong>Comércio global, macroengenharia, macroprojeto de transporte tripartite. </p>