bluuebunny/crossref_metadata_2025_split huggingface dataset:
Successful Control of Intractable Hypoglycemia Using Rapamycin in an 86-Year-Old Man with a Pancreatic Insulin-Secreting Islet Cell Tumor and Metastases
Authors: Bourcier Matthew E. , Sherrod Amanda , DiGuardo Margaret , Vinik Aaron I. 
Published: July 2009

Context: Insulinomas are rare tumors of the pancreatic islet cells that produce insulin. Approximately 5 to 10% of these tumors are cancerous, and control of insulin secretion and hypoglycemia may be difficult in these patients. Malignant insulinomas generally respond poorly to traditional chemotherapeutic agent regimens. At present, streptozotocin is the only approved drug for the treatment of pancreatic islet cell tumors.
Setting and Patient: This report describes a case of an elderly gentleman with a metastatic pancreatic insulinoma and severe hypoglycemia. A continuous infusion of octreotide lowered the blood glucose levels further. He required diazoxide, a thiazide diuretic, phenytoin, and a constant infusion of glucose to control the hypoglycemia and elevated insulin levels.
Intervention: Rapamycin was administered at an oral dose of 2 mg/d.
Results: On the mTOR (mammalian target of rapamycin) agent rapamycin, he was weaned off all drugs except for the thiazide diuretic and maintained euglycemia with a reduction of circulating insulin levels. He remained euglycemic for the past year with no evidence of tumor progression based on Octreoscan. His quality of life is excellent, and he remains active having recently completed a triathlon.
Conclusions: Rapamycin may provide a useful means of abrogating tumor growth and controlling hypoglycemia in malignant insulinomas by reducing the malignant β-cell growth and proliferation as well as inhibiting insulin production.
Rapamycin may abrogate tumor growth and control hypoglycemia in malignant insulinomas by reducing malignant β-cell growth and proliferation, as well as inhibiting insulin production.
Dentoskeletal Effects of Maxillary Protraction in Cleft Patients with Repetitive Weekly Protocol of Alternate Rapid Maxillary Expansions and Constrictions
Authors: Vieira Gustavoda Luz , de Menezes Luciane Macedo , de Lima Eduardo Martinelli S. , Rizzatto Susana 
Published: July 2009

Objective: To evaluate the amount of maxillary protraction with face mask in complete unilateral cleft lip and palate patients submitted to two distinct rapid maxillary expansion (RME) protocols. Material and Methods: The sample consisted of 20 individuals (nine boys and 11 girls; mean age of 10.4 ± 2.62 years) with unilateral complete cleft lip and palate who had a constricted maxilla in the vertical and transverse dimensions. Ten patients underwent 1 week of RME with screw activation of one complete turn per day, followed by 23 weeks of maxillary protraction (group 1). The other 10 patients underwent 7 weeks of alternate rapid maxillary expansion and constriction, with one complete turn per day, followed by 17 weeks of maxillary protraction (group 2); both groups underwent a total of 6 months of treatment. Cephalometric measurements were taken at different times: pretreatment (T1), soon after RME (T2), and after 6 months of treatment (T3). Each measurement was analyzed with mixed models for repeated measures, and the covariance structure chosen was compound symmetry. Results: The maxilla displaced slightly forward and downward with a counterclockwise rotation; the mandible rotated downward and backward, resulting in an increase in anterior facial height; the sagittal maxillomandibular relationship was improved; the maxillary molars and incisors were protruded and extruded; and the mandibular incisors were retroclined. Conclusion: There was no significant difference between the groups in evaluation time.
A Survey of Cleft Team Patient Experience in Obtaining Dental Care
Authors: Becker Devra B. , Lee Frances , Hill Sean , Nissen Richard , Huebener Donald , Scheve Sibyl , Gordon Shayna , Kane Alex A. 
Published: July 2009

Objective: To assess patient experiences in obtaining dental care in a team setting without an affiliated dental school. Design and participants: Three hundred seventy-four patients in the cleft team database met inclusion criteria of diagnosis of cleft lip and/or palate, and current age of 7 to 12 years. Demographic information and experiences in obtaining dental care were ascertained using a standardized series of questions. A callback protocol was employed to maximize response rate. Results were analyzed with t-tests using contingency tables. Outcome measures and results: One hundred seventy-one parents/caregivers were interviewed of a possible 374 (response rate 45.7%). Mean age was 9.87 years. The insurance distribution was as follows: 113 private insurance (66.1%), 35 Medicaid (20.5%), and 23 had no insurance (13.5%). The dental checkup distribution was as follows: 145 regular dental checkups (84.8%) and 26 no regular checkups (15.2%). Patients with private insurance were more likely to obtain dental care than were patients with Medicaid (p  =  .002) or patients without insurance (p  =  .0027). Patients with Medicaid were more likely to report provider refusal of care than were patients with private insurance (p  =  .0001) or patients without insurance (p  =  .0001). Patients with private insurance were more likely to report satisfaction with their dental care than were patients with Medicaid (p  =  .0003). Conclusions: We report an 84.8% regular checkup rate among our study population and a significantly different reported experience in obtaining care depending on insurance type. The reasons underlying the differences between privately insured patients and Medicaid patients appear multifactorial.
Esthetic Analysis of Gingival Components of Smile and Degree of Satisfaction in Individuals with Cleft Lip and Palate
Authors: Esper Luis A. , Sbrana Michyele C. , Ribeiro Ingrid W. J. , Siqueira de Erick N. , de Almeida Ana L. P. F. 
Published: July 2009

Objective: To evaluate soft tissue characteristics in individuals with cleft lip and palate and the degree of satisfaction of these individuals after rehabilitation. Setting: Hospital for Rehabilitation of Craniofacial Anomalies, Brazil. Patients: Forty-five individuals with repaired complete unilateral cleft lip and palate, aged 15 to 30 years. Interventions: One hundred thirty-five frontal facial photographs were obtained at rest and in natural and forced smile. Specialists in periodontics evaluated the soft tissue characteristics. Both patients and specialists evaluated the smiles and scored them as esthetically unpleasant, acceptable, or pleasant. Main Outcome Measures: Comparison of the cleft area with the contralateral region was performed for evaluation of soft tissue. The results of the degree of satisfaction with smile were expressed as percentages and means. The findings between patients and periodontists experienced or inexperienced with cleft care were compared. Results: Statistically significant differences were observed for alveolar process deficiency and absence of papilla in the esthetic area between groups (p < .05). Results show 84.4% of individuals considered their smile as esthetically pleasant. Specialists in periodontics of both groups scored the natural smile and forced smile as esthetically acceptable. There was a statistically significant difference in the mean of patients compared with both groups of specialists in periodontics (p < .05). Conclusions: Evaluation and knowledge of the soft tissue characteristics is extremely important for successful rehabilitation. The esthetic values and degree of patient satisfaction are essential for treatment success, since smile reconstruction should be esthetically pleasant to the patient.
Causal Attributions in Parents of Babies with a Cleft Lip and/or Palate and Their Association with Psychological Well-Being
Authors: Nelson Jonathan , O'Leary Catherine , Weinman John 
Published: July 2009

Objective: This study aimed to assess causal attributions of parents of babies with a cleft lip and/or palate. Evidence from causal attribution theory and attribution studies in other medical conditions led to the hypothesis that parents who make internal attributions (self-blame) will have poorer psychological well-being. Design: A cross-sectional survey. Setting: Postal questionnaires were sent to parents of children under the care of the South Thames Cleft Service at Guy's Hospital. Participants: Participants were recruited if they had a baby between 12 and 24 months old with a cleft lip and/or palate. Of 204 parents, 42 responded. Main Outcome Measures: A semistructured questionnaire about causal beliefs was completed alongside validated questionnaires measuring anxiety, depression (Hospital Anxiety and Depression Scale), and perceived stress (Perceived Stress Scale). Results: Causal attributions were grouped according to type (environmental, chance, self-blame, and no belief) and loci (external or internal). The most common attribution made was to external factors (54.4%), followed by no causal attribution (38.1%). Parents making an internal (self-blaming) attribution (16.7%) had significantly (p < .05) higher scores on the Hospital Anxiety and Depression Scale anxiety measure (r  =  .32) and Perceived Stress Scale (r  =  .33), but not on the Hospital Anxiety and Depression Scale depression measure (p  =  .283). Conclusions: The high number of parents making an external attribution can be explained by causal attribution theory. However, the percentage of parents making no causal attribution was higher than seen in previous research. Surprisingly, no parents blamed others. The main hypothesis was tentatively accepted because there were significantly higher anxiety and stress scores in parents who self-blamed; although, depression scores were not significantly higher.
Three-Dimensional Morphology of the Palate in Patients with Bilateral Complete Cleft Lip and Palate at the Stage of Permanent Dentition
Authors: Šmahel Zbyněk , Velemínská Jana , Trefný Pavel , Müllerová Živa 
Published: July 2009

Objective: Three-dimensional analysis of palate size and shape in 30 patients with complete bilateral cleft lip and palate (BCLPc) at the stage of permanent dentition. Design: Cross-sectional study based on laser scanning. Subjects: Thirty dental casts of boys approximately 15 years old with BCLPc and 28 dental casts of healthy boys of the same age. Interventions: Arched-lip suture with periosteoplasty and push-back of the palate with pharyngeal-flap surgery. Main Outcome Measures: Data on palate height in the 210 defined locations and on palate widths and profile area in 10 transverse sections. Results: The palate in patients with BCLPc was conical and narrower than in control study subjects, much more anteriorly than posteriorly. From the canines posteriorly, the palate was of almost constant height of 10 mm in the midline, being higher than in control study subjects at this location and lower more posteriorly (by 24% to 29% between molars). The area of transverse sections was reduced as compared with control study subjects from the first premolars posteriorly and reached more than 40% between molars. The length of the palate up to the first molars was not changed. Conclusion: BCLPc subjects exhibited narrow, low, and flat palate. Palate size and shape differences indicate a substantial reduction of the space for the tongue.
Maxillofacial Aspects in Malignant Osteopetrosis
Authors: da Silva Santos Paulo Sérgio , Esperidião André Passaglia , de Freitas Ronaldo Rodrigues 
Published: July 2009

Objective: Malignant osteopetrosis (MO) is a rare hereditary disease that affects young children. Its physiopathology is explained by a basic defect in osteoclast precursor cells, with a radiographic image of diffuse sclerosis and increased bone density. The bone contains an increased number of structurally abnormal osteoclasts. Infections commonly occur because the neutrophils exhibit reduced chemotactic response and decreased ability of bacterial phagocytosis. Patient: A white female patient aged 9 months, of mixed descent, was diagnosed at birth as having MO after bone marrow biopsy. Dental examination revealed the presence of four teeth, all of which had enamel hypoplasia and no carious lesions. After bone marrow transplantation (BMT), an erythematous lesion appeared on the alveolar ridge on day 2, with signs of tooth exfoliation. On day 7, there was gingival bleeding, edema, and erythema at the region of the maxillary incisors with grade 3 mobility, without changes in food intake. Results: Concerning the oral cavity, the patient maintained grade 1 tooth mobility without tooth exfoliation or other oral alterations. Conclusion: Accurate diagnosis and careful therapeutic planning are important to avoid the secondary complications of the disease.
Intraoral Photographs for Rating Dental Arch Relationships in Unilateral Cleft Lip and Palate
Authors: Liao Yu-Fang , Huang Chiung-Shing , Lin I-Feng 
Published: July 2009

Background and Purpose: The Goslon Yardstick is one of the most commonly used methods to assess dental arch relationships of patients with unilateral cleft lip and palate. This system was originally applied to dental casts. For reasons of economy and convenience, we aimed to determine whether intraoral photographs could substitute for dental casts for rating dental arch relationships. Methods: Records of 58 patients with nonsyndromic complete unilateral cleft lip and palate from the Chang Gung Craniofacial Center, Taipei, Taiwan, were used in this study. A set of dental casts and digital intraoral photographs taken at around 9 years of age were available for all patients. An experienced examiner rated the dental casts using the Goslon Yardstick to provide the reference scores. The other three examiners rated the intraoral photographs and repeated the rating 1 week later to calculate inter- and intraexaminer reliability. The photographic scores for each examiner were then compared with the reference scores to determine the validity of the photographs. Results: The results showed no significant difference between the rating of dental casts and photographs using the Goslon Yardstick. Reliability was also high for rating on photographs. Conclusions: Intraoral photographs appear to be a viable alternative to the application of the Goslon Yardstick on dental casts.
Transport Distraction Osteogenesis for Closing Full-Thickness Calvarial Defects in Sheep
Authors: Icekson M. , Regev E. , Neuman R. , Margulis A. 
Published: July 2009

Objective: To prove the hypothesis that transport distraction osteogenesis can be applied to the skull to close critical-size calvarial defects. Design: A sheep model was developed to investigate this hypothesis. In four sheep, bilateral parietal bone windows were created and adjacent osteotomies performed. On the tested side, an adjacent bone segment was transported into the defect. The contralateral side was left untreated as a control. Main Outcome Measures: After completion of the distraction and consolidation period, a computed tomography study was performed, and the animals were sacrificed. The newly formed bone was examined macroscopically and histologically. Results: A successful closure of the defect with transport distraction was achieved in all of the animals. The control side healed spontaneously in one (younger) sheep but did not heal in the other three animals. The closure of the bony defect with transport distraction was evident macroscopically as well as on the computerized tomography. Microscopic examination showed new healthy bone formation on the treated side. Conclusion: We conclude that transport distraction is an effective tool in closing full-thickness calvarial defects in adult sheep. Further investigation is needed before applying this promising technique in humans.
Treatment Outcome after One-Stage Repair in Children with Complete Unilateral Cleft Lip and Palate Assessed with the Goslon Yardstick
Authors: Fudalej Piotr , Hortis-Dzierzbicka Maria , Obloj Barbara , Miller-Drabikowska Dorota , Dudkiewicz Zofia , Romanowska Anna 
Published: July 2009

Objective: To compare results of Golson Yardstick measurement of dental arch relationships in a sample of 10-year-old Polish children with results of the Golson measurement in published reports. Materials and Methods: Plaster models of 28 consecutively treated subjects with unilateral cleft lip and palate (UCLP) that was repaired with a one-stage simultaneous closure performed in the first year of life. All individuals were born between 1994 and 1995. The Goslon score (categories 1 to 5) was allocated. Intra- and interrater agreement was assessed with kappa statistics and Pearson correlation coefficient. Independent t tests were employed to detect difference between the score in the present and other published samples. Results: Mean Goslon score equaled 2.44; 57% of the patients were allocated Goslon category 1 or 2, 32% were rated Goslon 3, and 11% of the patients were assigned category 4 or 5. Intrarater agreement was between 0.75 and 0.77. Interrater agreement was 0.79. Conclusions: Dental arch relationship following one-stage repair was comparable with the results of the centers with the best outcome.
Flexible Laryngeal Mask Airway for Cleft Palate Surgery in Children: A Randomized Clinical Trial on Efficacy and Safety
Authors: Kundra Pankaj , Supraja N. , Agrawal K. , Ravishankar M. 
Published: July 2009

Objective: To evaluate the efficacy of a flexible laryngeal mask airway in children undergoing palatoplasty. Design: Prospective, randomized, single-center study. Setting: Jawaharlal Institute of Postgraduate Medical Education and Research. Patients: Sixty-six children (American Society of Anesthesiologists physical status 1 and 2) scheduled to undergo palatoplasty were assigned randomly to an endotracheal intubation group (RAE group, n  =  33) and a flexible laryngeal mask airway group (FLMA group, n  =  33). Main Outcome Measures: Peak airway pressure, inspired and expired tidal volume, end-tidal carbon dioxide, lung compliance, and airway resistance were continuously measured after placement of the assigned airway. The percentage leak around the airway was quantified as the leak fraction. Parametric data between groups were analyzed using an unpaired Student's t test and within groups using a one-way analysis of variance. Nonparametric variables were analyzed using the Fisher exact test. Results: In two children, the flexible laryngeal mask airway was displaced from its original position; whereas, one endotrachial tube advanced endobronchially. The leak fraction was significantly higher in the RAE group when compared with that in FLMA group (13.34% ± 13.74% versus 5.96% ± 3.78%, p < .05) until the throat pack was applied. Peak airway pressure and resistance were significantly higher in the RAE group compared with the FLMA group at all time intervals, p < .05. During emergence, frequency of coughing, desaturation, and laryngospasm were increased in the RAE group. Conclusion: A flexible laryngeal airway mask is suitable for maintaining the airway and helps in smooth emergence in children undergoing palatoplasty.
Methodology for Speech Assessment in the Scandcleft Project—An International Randomized Clinical Trial on Palatal Surgery: Experiences from a Pilot Study
Authors: Lohmander A. , Willadsen E. , Persson C. , Henningsson G. , Bowden M. , Hutters B. 
Published: July 2009

Objective: To present the methodology for speech assessment in the Scandcleft project and discuss issues from a pilot study. Design: Description of methodology and blinded test for speech assessment. Speech samples and instructions for data collection and analysis for comparisons of speech outcomes across five included languages were developed and tested. Participants and Materials: Randomly selected video recordings of 10 5-year-old children from each language (n  =  50) were included in the project. Speech material consisted of test consonants in single words, connected speech, and syllable chains with nasal consonants. Five experienced speech and language pathologists participated as observers. Main Outcome Measures: Narrow phonetic transcription of test consonants translated into cleft speech characteristics, ordinal scale rating of resonance, and perceived velopharyngeal closure (VPC). A velopharyngeal composite score (VPC-sum) was extrapolated from raw data. Intra-agreement comparisons were performed. Results: Range for intra-agreement for consonant analysis was 53% to 89%, for hypernasality on high vowels in single words the range was 20% to 80%, and the agreement between the VPC-sum and the overall rating of VPC was 78%. Conclusions: Pooling data of speakers of different languages in the same trial and comparing speech outcome across trials seems possible if the assessment of speech concerns consonants and is confined to speech units that are phonetically similar across languages. Agreed conventions and rules are important. A composite variable for perceptual assessment of velopharyngeal function during speech seems usable; whereas, the method for hypernasality evaluation requires further testing.
Enamel Defects in Maxillary Central Incisors of Infants with Unilateral Cleft Lip
Authors: Gomes Alessandra Cristina , Neves Lucimara Teixeira das , Gomide Marcia Ribeiro 
Published: July 2009

Objective: To evaluate the presence of enamel alterations in deciduous maxillary central incisors of infants with unilateral cleft lip and alveolar ridge, with or without cleft palate, and to compare the occurrence and location of these alterations between the central incisor adjacent to the cleft and the contralateral incisor. Design: Intraoral clinical examination was performed after tooth cleaning and drying by a single examiner with the aid of a dental mirror, dental probe, and artificial light, with the child positioned on a dental chair. The defects were recorded in a standardized manner according to the criteria of the Modified Developmental Defects of Enamel Index. Setting: Hospital for Rehabilitation of Craniofacial Anomalies (HRAC) at Bauru, São Paulo, Brazil. Patients: One hundred one infants were evaluated. All were white, of both genders, aged 12 to 36 months and had at least two thirds of the crowns of maxillary incisors erupted. Results: Demarcated opacity was the most common defect at both cleft and noncleft sides, followed by diffuse opacity. The occurrence of hypoplasia at the cleft side was 11.8%. Most defects affected less than one third of the crown. Conclusion: The occurrence of enamel defects in deciduous maxillary central incisors of patients with unilateral cleft lip was 42.6%, mainly affecting the cleft side as to both number and severity.
Dental Development in Children with Growth Hormone Insensitivity Syndrome: Demirjian Analysis of Serial Panoramic Radiographs
Authors: Campbell Richard , Weinshel Randy , Backeljauw Philippe , Wilson Stephen , Bean Judy , Shao Mingyuan 
Published: July 2009

Objective: This study evaluates the effects of 8 years of insulinlike growth factor-I therapy on tooth development in patients with growth hormone insensitivity syndrome. Methods: Forty-nine panoramic radiographs were evaluated from eight patients (six boys, two girls). Seven teeth in the mandibular left region were graded according to the Demirjian system. Radiographs were taken at the start of insulinlike growth factor-I therapy and were continued at approximately yearly intervals for 8 years. Results: Three of six boys and one of two girls who began treatment with insulinlike growth factor-I at earlier ages experienced an increase in the rate of tooth development. One of six boys who began treatment with insulinlike growth factor-I at a later age had a slower rate of dental development. The patients had more rapid tooth maturation during the beginning of treatment. By the end of treatment, all patients had normal dental maturity for their age. Conclusions: Treatment of growth hormone insensitivity syndrome with insulinlike growth factor-I appears to lead to an increase in dental maturation, particularly in younger patients. After 8 years all patients had achieved normal dental development.