bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treatment of an Infant with a Rare Cleft Resolved with Use of an Orthopedic Appliance
Authors: Romero Martín , Latham Ralph , Romance Ana , Salvan Rafael 
Published: June 2004

Objective Cases of bilateral complete clefts of the primary palate and unaffected secondary palate are very rare. One of these cases as well as a new method of presurgical orthopedics to solve the protruding premaxilla protrusion is presented.
Evaluation of Temporomandibular Joint in Patients with Hemifacial Microsomia
Authors: Kitai Noriyuki , Murakami Shumei , Takashima Mariko , Furukawa Souhei , Kreiborg Sven , Takada Kenji 
Published: June 2004

Objective The purpose of this study was to elucidate positional relationships between temporomandibular joint (TMJ) components, including the articular discs, using magnetic resonance imaging (MRI) in patients with hemifacial microsomia (HFM). Subjects and Methods Twenty TMJs in 10 patients with HFM were examined at closed- and open-mouth positions using an MRI scanner. The condyle-fossa and disc-condyle relationships, disc configuration at the closed-mouth position, and the reduction of the disc at the open-mouth position were evaluated. Results On the unaffected side, the condyle-fossa and disc-condyle relationships appeared fairly normal at the closed mouth position. The disc-condyle relationship at the open-mouth position was also normal. The TMJ disc showed normal biconcave configurations at both closed- and open-mouth positions. On the affected side, there was considerable variation in the state of the TMJ. At the closed-mouth position, 5 of the 10 patients revealed fairly normal disc-condyle relationships, one patient showed anterior displacement of the disc, and four patients had no disc. Two patients appeared biconcave, three patients appeared biplanar, and one patient was hemiconvex. At the open-mouth position, the condyle and disc moved in harmony in five patients with normal disc-condyle relationships, but the disc was reduced in a patients with anterior disc displacement. The degree of the TMJ disc dysplasia did not necessarily correspond with the degree of mandibular dysplasia. Conclusion The present study contributes to an improved understanding of TMJ pathology in patients with HFM. The results suggest that, in HFM patients, the examination of the TMJ using MRI is helpful for determining treatment procedures in mandibular distraction osteogenesis.
Facial Characterization of Infants with Cleft Lip and Palate Using a Three-Dimensional Capture Technique
Authors: Hood C. A. , Hosey M. T. , Bock M. , White J. , Ray A. , Ayoub A. F. 
Published: June 2004

Objective To characterize the soft tissue features of infants with unilateral cleft lip (UCL) and unilateral complete cleft lip and palate (UCLP) prior to primary surgery and compare with noncleft controls. Design Prospective controlled capture of the facial morphology of infants using a noninvasive three-dimensional stereophotogrammetry method. Participants 23 children with presurgical cleft: 11 UCL (M = 6, F = 5); 12 UCLP (M = 9, F = 3), and 21 noncleft controls (M = 7, F = 14) were imaged at approximately 3 months of age (range 10 to 16 weeks). Main Outcome Measure Accurate, repeatable quantification of facial soft tissues in infants with clefts prior to surgery. Results Significant differences (p < .05) were found between the UCLP group and UCL and control groups in anatomical and soft nose width, cleft-side alar wing length, and nasal tip horizontal displacement. Both cleft groups were significantly different from controls and from each other in cleft-side nostril dimensions, alar wing angulation, columella angle, and alar base to corner of mouth dimension; alar base width; and soft tissue defect in nose and the lip and philtrum length bordering the cleft. Significant differences between clefts and controls were identified in the nostril and philtrum on the noncleft side. Conclusions The use of children with UCL as controls for UCLP studies is inappropriate. This technique overcame the limitations of direct measurement of infant faces to aid the surgeon in the planning and subsequent re-evaluation of surgical rationale.
A Comparison of Craniofacial Form in Northern Irish Children with Unilateral Cleft Lip and Palate Treated with Different Primary Surgical Techniques
Authors: Johnston Chris D. , Leonard Alan G. , Burden Donald J. , McSherry Patrick F. 
Published: June 2004

Objective The quality of outcomes in patients with unilateral cleft lip and palate (UCLP) was compared between two senior plastic surgeons. One surgeon carried out a one-stage Wardill-Kilner palate repair. The other surgeon employed a vomer flap hard palate repair followed by a von Langenbeck soft palate closure (Oslo protocol). Design Retrospective analysis. Patients Thirty-four children (mean age 9.7 years) born with complete skeletal UCLP in Northern Ireland from 1983 to 1991 who received primary repair surgery from one of the two surgeons. Main Outcome Measures Cephalometric analysis was used to determine the craniofacial form and soft tissue profile. The quality of the dental arch relationships was independently assessed using the Goslon ranking system. Results Twenty-nine of the 34 subjects had good or satisfactory arch relationships. Comparison between the surgeons revealed that the Wardill-Kilner group had a greater proportion of Goslon grades of greater than 3, indicating poor arch relationships. However, this difference failed to reach statistical significance. There were no significant cephalometric differences between patients treated by the two surgeons. Conclusions Although the difference was not statistically significant, the highest proportion of patients likely to require orthognathic surgery was found in those treated using the Wardill-Kilner technique.
Mathematical modeling and kinetic parameter estimation in batch crystallization
Authors: Quintana‐Hernández P. , Bolaños‐Reynoso E. , Miranda‐Castro B. , Salcedo‐Estrada L. 
Published: June 2004

AbstractA dynamic model of a batch crystallizer was used to estimate the kinetic parameters in a sugar cane crystallization process. The mathematical model included mass, energy, and population balance differential equations, and empirical relationships (power‐law–type) for nucleation, growth, and production–reduction rates. Experimental data were obtained from runs carried out at different agitation and cooling profiles. A nonlinear optimization process was used to fit 11 kinetic parameters to experimental data. The overall experimental error was estimated in 7.71% and the average asymptotic error of all kinetic parameters was less than 2%. The nucleation rate was favored by high agitation speed and natural cooling profiles. The maximum growth rate was reached when supersaturation was at a maximum. There was a no clear effect of agitation profiles on growth. The overall effect of the term production–reduction was in all cases positive, with the largest values found for natural cooling profiles. © 2004 American Institute of Chemical Engineers AIChE J, 50: 1407–1417, 2004
Does bilirubin protect against hemochromatosis gene (HFE) related mortality?
Authors: Alizadeh Behrooz Z. , Njajou Omer T. , Houwing‐Duistermaat Jeanine J. , Jong Gerard de , Vergeer Jeannette M. , Hofman Albert , Pols Huibert A.P. , Duijn Cornelia M. van 
Published: June 2004

AbstractSerum bilirubin is an important antioxidant that is found at increased levels in hereditary hemochromatosis patients. We hypothesized that increased levels of serum bilirubin may play a protective role against oxidative stress induced by iron overload in carriers of mutations in the hereditary hemochromatosis gene (HFE). We studied the relation between serum total bilirubin, serum iron levels, the HFE C282Y and H63D mutations, and mortality. The study was conducted in 2,332 randomly selected subjects from the Rotterdam Study, a population‐based follow‐up study of people aged 55 years or over. Serum bilirubin levels were significantly correlated with serum iron (Pearson's correlation coefficient (r) = 0.4, P < 0.001), transferrin saturation (r = 0.4, P < 0.001), and serum ferritin (r = 0.2, P < 0.05). Carriers of the HFE mutations had higher levels of bilirubin compared to wild‐type homozygotes. The relation was the strongest in H63D heterozygotes or homozygotes and C282Y heterozygotes. High levels of serum bilirubin were associated with a 2.8 (95% CI 0.9–8.8) fold reduction in mortality in H63D homozygotes and a 2.2 (1.0–4.7) fold reduction in mortality in C282Y heterozygotes. Taken together, our data suggest that the high levels of the antioxidant bilirubin may counteract the adverse effect of oxidative stress induced by iron overload. This may explain in part the reduced penetrance of the HFE mutations. © 2004 Wiley‐Liss, Inc.
Circulating COMP is decreased in pseudoachondroplasia and multiple epiphyseal dysplasia patients carrying COMP mutations
Authors: Mabuchi Akihiko , Momohara Shigeki , Ohashi Hirofumi , Takatori Yoshio , Haga Nobuhiko , Nishimura Gen , Ikegawa Shiro 
Published: June 2004

AbstractMutations in the gene encoding cartilage oligomeric matrix protein (COMP) cause two common skeletal dysplasias, pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED). At present, diagnosis of these diseases is based primarily on clinical and radiographic findings and is sometimes erroneous, particularly in adult patients. However, genetic diagnosis is difficult, because COMP mutations are scattered throughout the gene and five additional disease genes for MED exist. There is evidence that circulating COMP may serve as a molecular indicator of a variety of diseases affecting cartilage. Therefore, we investigated plasma COMP concentrations in 21 patients with PSACH or MED. Of these, six PSACH and seven MED patients carried COMP mutations, and the remaining eight MED patients lacked mutations in COMP. We observed significantly decreased plasma COMP levels in patients with COMP mutations compared with controls (P < 0.0001). In addition, plasma COMP levels were significantly decreased in MED patients carrying mutations in COMP relative to those who lacked COMP mutations (P = 0.001). Our results indicate that circulating COMP levels reflect genetic abnormalities in COMP, providing an easier, more rapid and cost‐efficient method for diagnosing PSACH and particularly for MED. © 2004 Wiley‐Liss, Inc.
Variants of the orexin2/hcrt2 receptor gene identified in patients with excessive daytime sleepiness and patients with Tourette's syndrome comorbidity
Authors: Thompson Miles D. , Comings David E. , Abu‐Ghazalah Rashid , Jereseh Yousef , Lin Leo , Wade Judy , Sakurai Takeshi , Tokita Shigeru , Yoshida Tetsuo , Tanaka Hirokazu , Yanagisawa Masashi , Burnham W. McIntyre , Moldofsky Harvey 
Published: June 2004

AbstractThe orexin‐2/hypocretin‐2 (OX2R) receptor gene is mutated in canine narcolepsy and disruption of the prepro‐orexin/hypocretin ligand gene results in both an animal model of narcolepsy and sporadic cases of the human disease. This evidence suggests that the structure of the OX2R gene, and its homologue, the OX1R gene, both members of the G protein‐coupled receptor (GPCR) family, and the gene encoding the peptide ligands, the prepro‐orexin/hypocretin gene, may be variables in the etiology of sleep disorders. We report a single stranded conformational polymorphism (SSCP) analysis of the coding regions of these genes in idiopathic sleep disorder patients diagnosed with excessive daytime sleepiness (EDS) (n = 28), narcolepsy (n = 28), Tourette's syndrome/chronic vocal or motor tic disorder (n = 70), and control subjects (n = 110). Two EDS patients showed a Pro11Thr change. One Tourette's syndrome patient was found to have a Pro10Ser alteration. The Pro10Ser and Pro11Thr variants were not found in non‐disease populations. Analysis of the ability of the mutant receptors to mobilize calcium compared to the wild‐type receptor in response to orexin agonists indicated that they resulted in decreased potency at high (ηM) concentrations of orexin ligands. Further work is warranted to study the variability of the orexin/hypocretin system in a variety of disorders characterized by EDS. © 2004 Wiley‐Liss, Inc.
Genomic screen for loci associated with alcohol dependence in Mission Indians
Authors: Ehlers Cindy L. , Gilder David A. , Wall Tamara L. , Phillips Evelyn , Feiler Heidi , Wilhelmsen Kirk C. 
Published: June 2004

AbstractAlcohol dependence is a leading cause of morbidity and mortality in Native Americans, yet biological factors underlying the disorder in this ethnic group remain illusive. This study's aims were to map susceptibility loci for DSM‐III‐R alcohol dependence and two narrower alcohol‐related phenotypes in Mission Indian families. Each participant gave a blood sample and completed an interview using the Semi‐Structured Assessment for the Genetics of Alcoholism (SSAGA) that was used to make alcohol dependence diagnoses and the narrower phenotypes of withdrawal, and drinking severity. Genotypes were determined for a panel 791 microsatellite polymorphisms. Analyses of multipoint variance component LOD scores for the dichotomous DSM‐III‐R phenotype revealed no peak LOD scores that exceeded 2.0 at any chromosome location. Two chromosomes, 4 and 12, had peak LOD scores that exceeded 2 for the alcohol use severity phenotype and three chromosomes 6, 15, 16 were found to have peaks with LOD scores that exceeded 2 for the withdrawal phenotype. Evidence for linkage to chromosomes 4 and 15, and 16 have been reported previously for alcohol related phenotypes whereas no evidence has as yet been reported for chromosomes 6 and 12. Combined linkage and association analysis suggest that alcohol dehydrogenase 1B gene polymorphisms are partially responsible for the linkage result on chromosome 4 in this population. These results corroborate the importance of several chromosomal regions highlighted in prior segregation studies in alcoholism and further identify new regions of the genome that may be unique to either the restricted phenotypes evaluated or this population of Mission Indians. © 2004 Wiley‐Liss, Inc.
Using nonword repetition to distinguish genetic and environmental influences on early literacy development: A study of 6‐year‐old twins
Authors: Bishop Dorothy V. M. , Adams Caroline V. , Norbury Courtenay Frazier 
Published: June 2004

AbstractThis study considered whether cognitive profile could distinguish groups of children where genes or environment played a major role in influencing reading level. Same‐sex twin pairs from an epidemiological study were categorized according to parental report at 4 years of age into those with low language skills and a typically developing group. A total of 132 same‐sex twin pairs from the low language group and 66 from the control group were assessed at 6 years of age, to investigate heritability of reading ability adjusted for nonverbal IQ. For pairs where both twins had normal scores on a nonword repetition test, heritability was zero, with environmental influences explaining all the variance. For pairs where one or both twins had low nonword repetition, the heritability estimate was 0.79 and the variance due to shared environment was zero. Future studies of genetics of reading development should treat those with poor nonword repetition skills as a separate subgroup. © 2004 Wiley‐Liss, Inc.
Identification of human UDP‐glucuronosyltransferase enzyme(s) responsible for the glucuronidation of 3‐hydroxydesloratadine
Authors: Ghosal Anima , Yuan Yuan , Hapangama Neil , Su Ai Duen (Iris) , Alvarez Narciso , Chowdhury Swapan K. , Alton Kevin B. , Patrick James E. , Zbaida Shmuel 
Published: June 2004

AbstractDesloratadine is a non‐sedating antihistamine recently approved for the treatment of seasonal allergic rhinitis. The major metabolite of desloratadine in human plasma and urine is the glucuronide conjugate of 3‐hydroxydesloratadine. 3‐Hydroxydesloratadine‐glucuronide is also the major in vitro metabolite of 3‐hydroxydesloratadine formed by incubation of 3‐hydroxydesloratadine with human liver microsomes supplemented with uridine 5′‐diphosphate‐glucuronic acid (UDPGA). The metabolite structure was confirmed by LC‐MS and LC‐MS/MS. Out of ten recombinant human UDP‐glucuronosyltransferases (UGTs), UGT1A1, UGT1A3, UGT1A8 and UGT2B15 exhibited catalytic activity with respect to the formation of 3‐hydroxydesloratadine‐glucuronide. Inhibition studies with known inhibitors of UGT (diclofenac, flunitrazepam and bilirubin) confirmed the involvement of UGT1A1, UGT1A3 and UGT2B15 in the formation of 3‐hydroxydesloratadine‐glucuronide. The results from this study demonstrated that the in vitro formation of 3‐hydroxydesloratadine‐glucuronide from 3‐hydroxydesloratadine was mediated via UGT1A1, UGT1A3 and UGT2B15 in human liver. Copyright © 2004 John Wiley & Sons, Ltd.
Absorption, distribution, metabolism and excretion of YM466, a novel factor Xa inhibitor, in rats
Authors: Mano Yuji , Sonoda Takuya , Nakamura Eiji , Usui Takashi , Kamimura Hidetaka 
Published: June 2004

AbstractYM466 is a novel factor Xa inhibitor for the treatment of thrombosis. The absorption, distribution, metabolism and excretion of YM466 were investigated in male Fisher rats after a single oral administration. YM466 was absorbed rapidly from all segments of the gastrointestinal tract except the stomach. After oral dosing, the plasma concentration of 14C‐YM466 reached a maximum within 0.5h, and declined rapidly with an elimination half‐life of 0.64h. The unchanged YM466 accounted for almost all of its radioactivity, suggesting a minimal metabolism in rats. This was also supported by the finding that no metabolites were observed in bile and urine after oral dosing of 14C‐YM466. The distribution of 14C‐YM466 in tissue was evaluated and the liver and kidney were the organs with radioactivity concentrations consistently higher than that of plasma. Cumulative biliary and urinary excretion of radioactivity in bile duct‐cannulated rats was 29.5% and 7.6%, respectively, indicating prominent excretion into bile after oral dosing. This was consistent with the finding that 76.1% and 25.2% of radioactivity dosed were excreted to faeces and urine, respectively, after i.v. dosing. These results suggest that YM466 was rapidly absorbed and then subjected to biliary excretion with a minimal metabolism after oral dosing to rats. Copyright © 2004 John Wiley & Sons, Ltd.
Pharmacokinetics and dose proportionality of extended‐release metformin following administration of 1000, 1500, 2000 and 2500 mg in healthy volunteers
Authors: Cullen Edward , Liao James , Lukacsko Peter , Niecestro Robert , Friedhoff Lawrence 
Published: June 2004

AbstractThe pharmacokinetics and dose‐exposure relationship of an extended‐release formulation of metformin (ER‐metformin) was investigated in a randomized, single‐dose, four‐period crossover study in 24 healthy male volunteers. During each study period, subjects received a randomly assigned dose containing 1000, 1500, 2000 or 2500 mg metformin. Blood samples were drawn 0–72 h after dosing for pharmacokinetic and dose‐proportionality assessment. Although several pairwise comparisons between dose groups were significant (p<0.05) with respect to dose‐normalized Cmax, AUC0–72 h, and AUC∞, the magnitude of the difference across the dose range was <20% for AUC0–72 h and AUC∞, and was ⩽30% for Cmax. The results indicate a consistent and predictable increase in metformin exposure with an extended‐release formulation of metformin over 1000 to 2500 mg. Copyright © 2004 John Wiley & Sons, Ltd.
Vasoactive exposures, vascular events, and hemifacial microsomia
Authors: Werler Martha M. , Sheehan Jane E. , Hayes Catherine , Mitchell Allen A. , Mulliken John B. 
Published: June 2004

AbstractBACKGROUNDBased on experimental evidence and clinical observations, hemifacial microsomia (HFM) is one of several structural anomalies that are postulated to result from vascular disruption. We collected data in a case‐control study to identify whether vasoactive exposures or vascular events during early pregnancy affect the risk of HFM.METHODSCases with a diagnosis of HFM were identified at craniofacial centers in 26 cities across the United States and Canada, from 1996 to 2002. Controls were matched to cases by age and pediatrician practice. Mothers of 230 cases and 678 controls were interviewed about pregnancy events and exposures. Case and control mothers were compared for early pregnancy use of vasoactive medications, cigarettes, and alcohol; singleton or multiple gestation; and diabetes, hypertension, or vaginal bleeding in the first half of pregnancy.RESULTSOdds ratios (ORs) were significantly increased for vasoactive mediation use (OR, 1.9 overall; OR, 4.2 among smokers), multiple gestations (OR, 10.5), and diabetes (OR, 6.0). Vaginal bleeding in the second trimester and heavy alcohol intake were associated with increased risks, but the estimates were based on small numbers and, therefore, are unstable. No associations were observed for cigarette smoking without vasoactive medication use, hypertension, and vaginal bleeding in the first trimester.CONCLUSIONSThe increased risks of HFM associated with vasoactive medication use, multiple gestations, diabetes, and second trimester vaginal bleeding appear collectively to support the hypothesis that vascular disruption is one etiology for HFM, because each of these factors is related to effects on blood vessels. Birth Defects Research (Part A), 2004. © 2004 Wiley‐Liss, Inc.
Base hydrolysis of phosphodiester bonds in pneumococcal polysaccharides
Authors: Pujar Narahari S. , Huang Ngan Fong , Daniels Christopher L. , Dieter Lance , Gayton Marshall G. , Lee Ann L. 
Published: June 2004

AbstractA comprehensive study of the base hydrolysis of all phosphodiester bond‐containing capsular polysaccharides of the 23‐valent pneumococcal vaccine is described here. Capsular polysaccharides from serotypes 6B, 10A, 17F, 19A, 19F, and 20 contain a phosphodiester bond that connects the repeating units in these polysaccharides (also referred to as backbone phosphodiester bonds), and polysaccharides from serotypes 11A, 15B, 18C, and 23F contain a phosphodiester bond that links a side chain to their repeating units. Molecular weight measurements of the polysaccharides, using high performance size exclusion chromatography with tandem multiangle laser light scattering and refractive index detection, was used to evaluate the kinetics of hydrolysis. The measurement of molecular weight provides a high degree of sensitivity in the case of small extents of reaction, thus allowing reliable measurements of the kinetics over short times. Pseudo‐first‐order rate constants for these polysaccharides were estimated using a simple model that accounts for the polydispersity of the starting sample. It was found that the relative order of backbone phosphodiester bond instability due to base hydrolysis was 19A > 10A > 19F > 6B > 17F, 20. Degradation of side‐chain phosphodiester bonds was not observed, although the high degree of sensitivity in measurements is lost in this case, due to the low contribution of the side chains to the total polysaccharide molecular weight. In comparison with literature data on pneumococcal polysaccharide 6A, 19A was found to be the more labile, and hence appears to be the most labile pneumococcal polysaccharide studied to date. The rate of hydrolysis increased at higher pH and in the presence of divalent cation, but the extent was lower than expected based on similar data on RNA. Finally, the differences in the phosphodiester bond stabilities were analyzed by considering stereochemical factors in these polysaccharides. These results also provide a framework for evaluation of molecular integrity of phosphodiester‐bond‐containing polysaccharides in different solution conditions. © 2004 Wiley Periodicals, Inc. Biopolymers, 2004
Crystal Structure of the Compounds LnAl0.5‐xSi0.5+x (Ln: La, Ce, Pr, Nd, Sm, Gd), Ln3Al4Si6 (Ln: La, Pr), and LnAlSi2 (Ln: Pr, Nd).
Authors: Lyaskovska Natalya , Romaniv Oksana , Semus'o Natalya , Gladyshevskii Evgen 
Published: June 2004

AbstractFor Abstract see ChemInform Abstract in Full Text.