bluuebunny/crossref_metadata_2025 huggingface dataset:
Influence of Controlled Rheumatoid Arthritis on the Action and Disposition of Verapamil: Focus on Infliximab
Authors: Ling Spencer , Lewanczuk Richard Z. , Russell Anthony S. , Ihejirika Brendan , Jamali Fakhreddin
Published: January 2009

Active rheumatoid arthritis (RA), obesity, and old age are associated with reduced responsiveness to the calcium channel antagonist verapamil despite increased drug concentrations. The diminishing effect appears to be associated with the severity of inflammation. We examined pharmacodynamics and pharmacokinetics of verapamil in patients with controlled RA. Volunteers included RA patients in remission: 12 on infliximab, 8 on other antirheumatic therapy, and 12 healthy subjects. Verapamil plasma concentrations and selected inflammatory mediators as well as blood pressure and electrocardiographic parameters were recorded after a single 80‐mg dose of verapamil. Inflammatory mediators were all below what is reported for active RA, confirming that RA was controlled. The tumor necrosis factor‐α concentration, however, was significantly higher in the infliximab group compared with other groups and the literature value for active RA. No significant difference was observed between groups in terms of percentage prolongation of PR interval despite a trend toward a lower response in the RA groups, the mean plasma concentrations, and the total and unbound area under the curve of verapamil. However, the slope of the S‐verapamil concentration—effect curve was steeper for controls compared with the RA patients. Remission from active disease appears to restore plasma protein levels and hepatic drug metabolism activity in patients with RA, resulting in relatively normal verapamil pharmacokinetics.
The Practice of Theory Borrowing in Organizational Studies: Current Issues and Future Directions
Authors: Whetten David A. , Felin Teppo , King Brayden G.
Published: January 2009

The borrowing and application of concepts and theories from underlying disciplines, such as psychology and sociology, is commonplace in organization theory. This article critically reviews this practice in organizational research. It discusses the borrowing of theoretical perspectives across vertical (cross-level) and horizontal (cross-context) boundaries and makes an associated distinction between theories in organizations and theories of organizations. It also explicates several unintended consequences and metatheoretical challenges associated with theory borrowing and highlights the legitimate reasons and ways for borrowing theories. By way of example, this article reviews how theories and concepts have been borrowed and applied in organizational research from two different literatures: individual identity and social movements. Overall, it is argued that treating organizations as social actors is the key to appropriate horizontal and vertical theory borrowing in organizational studies, in that it highlights the distinctive features of the organizational social form and organizational social context.
Constructing the Organ of Deceit
Authors: Littlefield Melissa
Published: January 2009

Functional magnetic resonance imaging (fMRI) and the electroencephalography (EEG)-based technology of Brain Fingerprinting have been hailed as the next, best technologies for lie detection in America, particularly in the context of post-9/11 anxiety. In scientific journals and the popular press, each has been juxtaposed and deemed superior to traditional polygraphy, which measures changes in the autonomic nervous system and correlates these fluctuations with emotions such as anxiety, fear, and guilt. The author contends that the juxtaposition of polygraphy and brain-based detection is a rhetorical strategy that foregrounds the corrective advantage of brain-based techniques, creates an artificial rupture between contiguous technologies, and ignores the shared assumptions foundational to fMRI, EEG, and two older ``truth telling'' technologies: polygraphy and fingerprinting. Far from describing the brain and its functions, fMRI and Brain Fingerprinting produce models of the brain that reinforce social notions of deception, truth, and deviance.
Study Characteristics and Recidivism Base Rates in Juvenile Sex Offender Recidivism
Authors: Caldwell Michael F.
Published: January 2009

This study reports on the results of a review and meta-analysis of 63 data sets that examine sexual recidivism among juvenile sex offenders. The studies include a total of 11,219 juvenile sex offenders, followed for a weighted mean of 59.4 months ( SD = 36.1 months). Recidivism is identified through official records of arrest or conviction. The weighted mean sexual recidivism rate is 7.08% ( SD = 3.9%). The weighted mean rate of general recidivism is 43.4% ( SD = 18.9%). Studies that examine sexual recidivism during adolescence find monthly sexual recidivism rates that are more than 4 times higher than those found in studies that rely only on adult recidivism records. Neither the level of secured placement (community, residential, or secured custody) nor the use of arrest versus conviction as an outcome significantly influences sexual recidivism rates.
Substance Use Disorders in Perpetrators of Intimate Partner Violence in a Forensic Setting
Authors: Kraanen Fleur L. , Scholing Agnes , Emmelkamp Paul M. G.
Published: January 2009

This study investigates the point prevalence of substance use disorders in 150 perpetrators of intimate partner violence (IPV) in a forensic setting and compares participants with and without substance use disorders on demographic and offence-related variables. Furthermore, it investigates the frequency of IPV perpetrated under the influence of substances. Half the sample (50.0%) meets diagnostic criteria for at least one substance-related diagnosis. Significantly more IPV perpetrators without substance use disorders compared with IPV perpetrators with substance use disorders have children living at home and have abused their children. Relative to IPV perpetrators without substance use disorders, significantly more IPV perpetrators with substance-related disorders are found to be under the influence of substances at the time of the offence. Results highlight the importance of understanding the prevalence of substance use disorders in IPV perpetrators in forensic settings.
Repair of Distal Biceps Tendon Rupture Using a Suture Anchor
Authors: Grégory Thomas , Roure Philippe , Fontès Didier
Published: January 2009

Background

Repair of a distal biceps tendon rupture is a challenging procedure and, to date, there is no consensus as to which technique should be used because of the specific complications reported for each.

Purpose

A new endoscopic technique is described that uses a suture anchor to repair distal biceps tendon ruptures.

Study Design

Case series; Level of evidence, 4.

Methods

The results of a cohort of 23 patients (25 elbows) are reported with a median follow-up of 26 months. All patients were male and their median age was 44 years (range, 30-58). Ten of the patients (12 ruptures) were professional athletes or had a high level of physical activity. All repairs were performed via a 3-cm incision made in the “safe area” of the anterior crease of the forearm. The whole procedure was performed within the tendon sheath. The tendon was reinserted using a single anchor.

Results

Of the 23 patients, 22 were satisfied and 20 patients returned to their preinjury sports and jobs. There was a mean loss of 8.6° of pronation and 5° of supination. A single severe neurologic complication, which required a second surgical procedure, was reported. There were also 2 ectopic ossifications without clinical consequences and a transitory radial nerve paralysis.

Conclusions

This study clearly demonstrated that endoscopic repair of the ruptured distal biceps tendon is safe, effective, and reproducible. It provides good functional outcome and early recovery with few complications. Postoperative median nerve palsy due to edema is a possible concern for patients involved in athletic activity and with a history of nerve entrapment; thus this technique should be used with caution in this group of patients.
Biomechanical Comparison of the Docking Technique with and without Humeral Bioabsorbable Interference Screw Fixation
Authors: Hurbanek Jason G. , Anderson Kyle , Crabtree Stephanie , Joshua Karnes G.
Published: January 2009

Background

Surgical reconstruction of the ulnar collateral ligament has evolved since Frank Jobe's original description. The “docking technique” is a popular modification that allows for securing the graft within a single humeral tunnel. More recently, interference screw fixation has been introduced as a means of improving the ultimate strength, stiffness, and kinematics of these constructs.

Purpose

This study was conducted to compare the biomechanical performance of the docking technique with and without interference screw fixation in the humerus.

Study Design

Controlled laboratory study.

Methods

Nine matched pairs of human cadaveric elbows (age 49.9 ± 8.0 years) were reconstructed with a tendon graft using the docking technique (group 1) or the docking technique with the addition of a 4.75-mm bioabsorbable humeral interference screw (group 2). Before the reconstruction, joint laxity was measured on each specimen with the ulnar collateral ligament intact and then after transection of the ligament. Laxity measurements were repeated after the reconstruction. Failure testing was then performed at 70° of elbow flexion. The specimens were preloaded with a 1-N·m moment and then loaded to failure at a displacement rate of 14 mm/s to approximate 50% strain per second.

Results

Within group 1, the elbow laxity of the reconstructed state was significantly greater than the intact state at all tested flexion angles (P <. 021). Within group 2, no statistically significant difference existed in elbow laxity between the intact state and the reconstructed state. When comparing laxities between groups, group 1 tended to be more lax at all tested flexion angles but was only significantly greater at 105° of flexion. The most common mode of failure for both groups involved the sutures pulling out of the tendon. No significant difference was found for ultimate moment of failure between the 2 groups. However, the moment associated with 3 mm of gap formation for group 2 (12.8 ± 4.2 N·m) was statistically greater than that of group 1 (7.5 ± 1.2 N·m) (P =. 001). The stiffness of group 2 (14.7 ± 6.4 N/mm) was significantly greater than group 1 (9.9 ± 3.1 N/mm) (P =. 044).

Conclusion

The biomechanical performance of the docking technique with and without a humeral interference screw is similar.

Clinical Relevance

The stiffness of the construct, along with the difference in moment that allows a 3-mm gap formation, suggests that the addition of a humeral interference screw is potentially beneficial. Further research in a healing model will help clarify this benefit.
Primary Repair Combined with Bone Marrow Stimulation in Acute Anterior Cruciate Ligament Lesions
Authors: Gobbi Alberto , Bathan Lyndon , Boldrini Lorenzo
Published: January 2009

Background

The anterior cruciate ligament has been shown to have poor healing ability, and reconstruction is the standard treatment.

Hypothesis

Primary anterior cruciate ligament repair combined with bone marrow stimulation could restore stability and function in athletes with acute anterior cruciate ligament incomplete tears.

Study Design

Case series; Level of evidence, 4.

Methods

Among a group of 99 patients with clinically diagnosed anterior cruciate ligament acute lesions, 26 athletes with arthroscopically confirmed incomplete anterior cruciate ligament proximal tears were treated with primary repair combined with bone marrow stimulation of the anterior cruciate ligament femoral attachment site. Postoperatively, all patients underwent a specific rehabilitation program. All patients were prospectively evaluated; outcome measures were assessed using Marx, Noyes, Tegner, Single Assessment Numeric Evaluation, Lysholm, and International Knee Documentation Committee scores. Anterior tibial translation was measured using Rolimeter instrument under anesthesia and at final follow-up.

Results

All athletes were followed up for a mean 25.3 months (range, 17-38 months). Mean age was 26.6 years. Mean preinjury Tegner was 7.1 (SD, 1.1) and final Tegner 6.7 (SD, 1.4); mean preinjury Marx was 11.0 (SD, 3.4) and final Marx 9.6 (SD, 3.1); mean preinjury Noyes was 82.5 (SD, 5.8) and final Noyes 83.3 (SD, 7.2). These scores were not statistically different at P values of. 020,. 011, and. 303, respectively. Final Single Assessment Numeric Evaluation rating was significantly lower than was preinjury Single Assessment Numeric Evaluation rating. This was mainly related to decreased self-confidence in high-risk sports and fear of new injury. Final Single Assessment Numeric Evaluation rating and Tegner scores were significantly higher than were their respective preoperative values. Mean Rolimeter side-to-side difference of anterior knee translation was significantly reduced from 3.5 mm (SD, 0.7) preoperatively to 1.3 mm (SD, 0.8) postoperatively.

Conclusion

In this athletic population, anterior cruciate ligament primary repair in acute incomplete lesion combined with bone marrow stimulation effectively restored knee stability and function.
Comparison of Single- versus Double-Tunnel Tendon-to-Bone Healing in an Ovine Model
Authors: Lu Yan , Markel Mark D. , Nemke Brett , Wynn Shawn , Graf Ben
Published: January 2009

Background

The double-bundle technique has recently gained much interest in ligament reconstruction. In addition to potential kinematic advantages, perhaps double tunnels have the potential for faster and more secure tendon-to-bone healing.

Hypothesis

Placement of tendons in 2 osseous tunnels, as opposed to 1, will enhance tendon fixation as determined biomechanically and histologically.

Study Design

Controlled laboratory study.

Methods

Fourteen sheep were used, and an extra-articular tendon graft reconstruction was performed on both knees of each sheep. In 1 randomly selected knee, the long digital extensor tendon was released from the femur and placed into a single tunnel in the proximal tibia. In the contralateral knee, the tendon was split and placed into 2 tibial tunnels. Ten sheep were analyzed by mechanical testing, and the remaining 4 were subjected to histologic evaluation at 6 weeks after surgery. Paired t tests were used for statistical analysis.

Results

Mechanical testing demonstrated that the peak load (981.8 ± 143.2 N, mean ± SD) and stiffness (570.9 ± 114.6 N/mm) at 6 weeks after surgery in the double-tunnel group were significantly greater than for the single-tunnel group (714.8 ± 94.2 N and 432.2 ± 56.7 N/mm, respectively; load, P =. 007; stiffness, P =. 03). Histologic analysis suggested similar tendon-to-bone healing for both groups.

Conclusion

This study demonstrated enhanced biomechanical fixation of the tendon to the surrounding bone in the double-tunnel compared with the single-tunnel technique in this ovine model.

Clinical Relevance

The double-tunnel technique may provide better fixation and healing in human ligament reconstruction.
Biomechanical Evaluation of Bioabsorbable versus Metallic Screws for Posterior Cruciate Ligament Inlay Graft Fixation
Authors: Gupta Aman , Lattermann Christian , Busam Matthew , Riff Andrew , Bach Bernard R. , Wang Vincent M.
Published: January 2009

Background

Although a tibial inlay technique for posterior cruciate ligament reconstruction is advantageous, metallic screw fixation of the bone block is required. This may pose problems for future surgery (eg, osteotomies, total knee replacement).

Hypothesis

There is no significant difference in the biomechanical integrity of bone block fixation using stainless steel versus bioabsorbable screw fixation of the tibial inlay graft in posterior cruciate ligament reconstruction.

Study Design

Controlled laboratory study.

Methods

Fourteen human cadaveric knees were randomized to receive either stainless steel or bioabsorbable screw fixation of a bone—patellar tendon—bone graft. Cyclic tensile testing of each construct was performed, followed by a load-to-failure test. Digital video digitization was used to optically determine tendon graft deformation.

Results

Cyclic creep deformation showed no significant difference between the 2 groups ( P = .8). The failure load (stainless steel, 461 ± 231 N; bioabsorbable, 638 ± 492 N; P = .7) and linear stiffness (stainless steel, 116 ± 22 N/mm, bioabsorbable, 106 ± 44 N/mm; P = .6) also showed no significant difference between the 2 groups. Optically measured graft deformation was not significant for distal ( P = .7) and midsubstance ( P = .8) regions, while proximal deformation was significantly higher for bioabsorbable fixation ( P = .02). All samples failed at the tibial insertion site with the tibial bone block fracturing at the screws.

Conclusion

Bioabsorbable screw fixation using a tibial inlay technique does not compromise the strength and stiffness characteristics afforded by metallic fixation. From a biomechanical perspective, bioabsorbable screws are a viable alternative to metal in the context of tibial inlay reconstruction.

Clinical Relevance

Use of bioabsorbable fixation can potentially eliminate future hardware problems after posterior cruciate ligament reconstruction using a tibial inlay technique.
Football Playing Surface and Shoe Design Affect Rotational Traction
Authors: Villwock Mark R. , Meyer Eric G. , Powell John W. , Fouty Amy J. , Haut Roger C.
Published: January 2009

Background

High rotational traction between football shoes and the playing surface may be a potential mechanism of injury for the lower extremity.

Hypothesis

Rotational traction at the shoe-surface interface depends on shoe design and surface type.

Study Design

Controlled laboratory study.

Methods

A mobile testing apparatus with a compliant ankle was used to apply rotations and measure the torque at the shoe-surface interface. The mechanical surrogate was used to compare 5 football cleat patterns (total of 10 shoe models) and 4 football surfaces (FieldTurf, AstroPlay, and 2 natural grass systems) on site at actual surface installations.

Results

Both artificial surfaces yielded significantly higher peak torque and rotational stiffness than the natural grass surfaces. The only cleat pattern that produced a peak torque significantly different than all others was the turf-style cleat, and it yielded the lowest torque. The model of shoe had a significant effect on rotational stiffness.

Conclusion

The infill artificial surfaces in this study exhibited greater rotational traction characteristics than natural grass. The cleat pattern did not predetermine a shoe's peak torque or rotational stiffness. A potential shoe design factor that may influence rotational stiffness is the material(s) used to construct the shoe's upper.

Clinical Relevance

The study provides data on the rotational traction of shoe-surface interfaces currently employed in football. As football shoe and surface designs continue to be updated, new evaluations of their performance must be assessed under simulated loading conditions to ensure that player performance needs are met while minimizing injury risk.
Effect of Water on Mechanical Properties of Unsaturated Polyester—Acetylated Hydroxypropyl Guar Gum Composites
Authors: Shenoy M.A. , D'Melo D.J.
Published: January 2009

The use of natural, eco-friendly, renewable resources in composites, as a reinforcing material, requires chemical or physical treatment to improve compatibility with the polymer matrix. In the present study hydroxypropyl guar gum (HPG) was acetylated to investigate its influence on the behavior of its unsaturated polyester (UPR) composites on exposure to water. The kinetics of water diffusion and effect on mechanical properties were studied with respect to the degree of acetylation and filler concentration. The absorption behavior of all composites was found to follow Fick’s law. Mechanical properties of HPG composites were found to be superior compared to pure UPR and acetylated HPG composites.
Effects of Wood Preheat Treatment on Thermal Stability of HDPE Composites
Authors: Kaboorani Alireza , Faezipour Mehdi
Published: January 2009

In this study, wood treated at different temperatures (175, 190, and 205°C) was used to ease the thermal instability caused by the introduction of wood to the composites. Composites consisting of different amounts of wood and coupling agent and HDPE were produced by the injection molding method. The mass loss measured by thermogravimetric analysis (TGA) was used as a tool to evaluate thermal stability of the materials. The results of this study show that heat treatment of wood increased DTGmax degradation temperatures and ash contents of wood especially when treatments took place at 190 and 205°C. An increase in wood content from 25 to 50% made the composites more sensitive to high temperatures. In comparison with untreated wood/HDPE composites, adding heat treated wood to the composites increased the thermal stability and ash contents of the composites. Coupling agent enhanced thermal stability of the composites when untreated wood was used as filler. The role of coupling agent on thermal stability was imperceptible when heat treated wood was used as filler.
Wear Characteristics of Plasma Sprayed Nickel—Aluminum Composite Coatings
Authors: Mishra S.C. , Satapathy Alok , Chaithanya M. , Ananthapadmanabhan P.V. , Sreekumar K.P.
Published: January 2009

In the present investigation plasma spray intermetallic coating of nickel—aluminide was deposited on mild steel substrates. The response of plasma sprayed nickel—aluminide coatings to the impingement of solid particles has been presented in this study. Nickel pre-mixed with alumina powder is deposited on mild steel substances by atmospheric plasma spraying at various operating power level. The coatings are subjected to erosion wear test. Dry silica sand of average particle size 400 μm is used as the erodent. The erosion rate is calculated on the basis of coating mass loss. The erosion studies are made at different velocities and impingement angles. It is observed that, the erosion wear is strongly influenced by the angle of impact. The test is conducted at room temperature, i.e., at 27°C and 60% RH. Coatings deposited at different power levels are found to exhibit different wear rates under similar test conditions. Microstructure of the coating is analyzed with SEM.
Two-body Abrasive Wear Behavior of Nano-clay Filled LDPE/EVA Composites
Authors: Ravi Kumar B.N. , Venkataramareddy M. , Suresha B.
Published: January 2009

The present article summarizes an experimental study on two-body abrasive wear behavior of nano-clay filled LDPE/EVA composites with and without compatibilizer. Poly (ethylene-co-glycidyl methacrylate) was used as the compatibilizer. Nano-clay filled LDPE/EVA composites with and without compatibilizer were melt-mixed using Brabender Co-Twin extruder followed by compression molding. Two-body abrasive wear studies were carried out using a Pin-on-Disc machine under multi-pass condition against the waterproof silicon carbide (SiC) abrasive papers in dry conditions. The effect of grit size, load and different abrading distance on the abrasive wear behavior was reported. The results indicate that the nano-clay filled LDPE/EVA with compatibilizer composite exhibits superior abrasion resistance. In addition, the worn surfaces of the samples were examined by scanning electron microscopy and the morphology was also discussed.
Influence of Multilayer vdW Interaction on Torsional Buckling of Multi-walled Carbon Nanotube under Thermal Fields
Authors: Saha Rohit , Maiti Pabitra R.
Published: January 2009

Torsional buckling of multi-walled carbon nanotube embedded in an elastic matrix subjected to thermal fields is studied using a continuum-based multilayered shell model. The model takes into account the multilayer van der Waals (vdW) interaction between any two layers of the MWCNT as radius dependent function. The elastic matrix surrounding the outermost tube is modeled as Pasternak foundation to account for not only the normal stresses, but also the shear stress between the outermost tube and the surrounding medium. The effect of inner tube radius, number of tubes, stiffness of surrounding elastic medium and temperature change on the critical torsional load is examined. Numerical results show that the vdW interaction will lead to a higher critical torsional load and at room or lower temperature the critical torsional load for infinitesimal buckling of a multi-walled carbon nanotube increases as the value of temperature change increases, while at a temperature higher than room temperature the critical torsional load for infinitesimal buckling of a multi-walled carbon nanotube decreases as the value of temperature change increases.
Effect of Steel Fibers on the Properties of Concrete
Authors: Kumutha R. , Vijai K.
Published: January 2009

An attempt has been made to study the effect of steel fibers on the compressive and flexural behavior of conventional concrete. Thirty cubes and 10 flexure beams were cast, out of which three cubes and a flexure beam belonged to the control mix. The variables in the study were aspect ratio (15, 25, and 35) and vol% fraction of fibers (0.25, 0.50, and 0.75). A marginal improvement in the ultimate strength was observed due to the addition of fibers. The optimum volume fraction of fibers for better performance in terms of strength and ductility was found to be 0.5%. Accelerated curing was adopted for all the specimens. Indian standards give only the correlation of 28-day characteristics compressive strength from the accelerated compressive strength. There is no relationship between the 28-day flexural strength and accelerated flexural strength. Hence an analytical model representing the relationship between 28-day flexural strength and accelerated flexural strength was suggested. Also models for compressive strength and flexural strength for varying aspect ratio and percentage of volume fraction were given.
One Story at a Time
Authors: Gardner Paula J. , Poole Jennifer M.
Published: January 2009

Various factors including social isolation and financial worries put older adults at risk for addictions. Indeed, older adults are the largest consumers of medication, and alcohol consumption is rising. Yet interventions are limited and problems often go unreported. Unearthing “problem” stories in people’s lives (i.e., “the addiction story”) and retelling them in more empowering ways, narrative therapy offers a viable therapeutic alternative, and research on narrative therapy has proven encouraging. However, little is known about narrative therapy with older adults and with addictions. Seeking to address these gaps, an ethnographic study was conducted in Toronto, Canada, with a group of older adults receiving narrative therapy for addictions. Findings suggest that the therapy was “helpful” and participants were able to reduce or halt their substance misuse. Most important, aspects of narrative therapy such as storytelling may be particularly well suited to older adults, offering powerful possibilities for applied gerontology.
Botulinum Toxin A Injections for the Treatment of Schwartz-Jampel Syndrome: A Case Series
Authors: Aburahma Samah Khalil , Al-Khateeb Taiseer , Alrefai Ali , Amarin Zouhair
Published: January 2009

Schwartz-Jampel syndrome is a rare autosomal recessive disorder characterized by myotonia and skeletal dysplasia. Botulinum toxin A is emerging as a therapeutic option for patients with this syndrome. This study describes the use of botulinum toxin A in 4 children with Schwartz-Jampel syndrome. The response to this therapeutic modality was limited and variable.
CDG Type Ia and Congenital Cytomegalovirus Infection: Two Coexisting Conditions
Authors: Jamroz Ewa , Adamek Dariusz , Paprocka Justyna , Adamowicz Maciej , Marszał Elżbieta , Wevers Ron A.
Published: January 2009

Congenital disorders of glycosylation are a heterogeneous group of disorders with multisystemic involvement. The most common form is phosphomannomutase deficiency or congenital disorders of glycosylation type Ia with an autosomal recessive inheritance and incidence estimated at 1/20000— 1/50000 live born. Congenital disorders of glycosylation Ia can manifest as severe multisystemic disease of infancy or milder disorder with only neurological problems including ataxia, hypotonia, and psychomotor retardation. The brain pathological findings in congenital disorders of glycosylation type Ia patients corroborate with cerebellar dysfunction. Usually the most affected part is the anterior lobe of the vermis. Microscopic analysis demonstrates the prominent Purkinje cell loss and subtotal loss of the external and internal granule cell layers. The authors present clinical and pathological picture of a 4-month-old girl with congenital disorders of glycosylation type Ia, additionally complicated by congenital cytomegalovirus infection. The diagnosis was confirmed by low phosphomannomutase activity in patient's fibroblasts and mutations on both alleles of phosphomannomutase 2 gene.
Mutations in the MECP2 Gene Are Not a Major Cause of Rett Syndrome-Like or Related Neurodevelopmental Phenotype in Male Patients
Authors: Santos Mónica , Temudo Teresa , Kay Teresa , Carrilho Inês , Medeira Ana , Cabral Helena , Gomes Roseli , Lourencço Maria Teresa , Venâncio Margarida , Calado Eulália , Moreira Ana , Oliveira Guiomar , Maciel Patrícia
Published: January 2009

Rett syndrome is a genetic neurodevelopmental disorder that affects mainly girls, but mutations in the causative MECP2 gene have also been identified in boys with classic Rett syndrome and Rett syndrome-like phenotypes. We have studied a group of 28 boys with a neurodevelopmental disorder, 13 of which with a Rett syndrome-like phenotype; the patients had diverse clinical presentations that included perturbations of the autistic spectrum, microcephaly, mental retardation, manual stereotypies, and epilepsy. We analyzed the complete coding region of the MECP2 gene, including the detection of large rearrangements, and we did not detect any pathogenic mutations in the MECP2 gene in these patients, in whom the genetic basis of disease remained unidentified. Thus, additional genes should be screened in this group of patients.
A Sleep Habits Questionnaire for Children With Autism Spectrum Disorders
Authors: Malow Beth A. , Crowe Crystal , Henderson Lynnette , McGrew Susan G. , Wang Lily , Song Yanna , Stone Wendy L.
Published: January 2009

Sleep difficulties in children with autism spectrum disorders are common, with poor sleep hygiene a contributing factor. We developed the Family Inventory of Sleep Habits to measure sleep hygiene in this population. Its validity and reliability in 2 groups of children aged 4 to 10 years, those with a clinical diagnosis of autism spectrum disorders, and those who are typically developing are described. In both groups, total and modified (reflecting insomnia subscales) scores on the Children's Sleep Habits Questionnaire showed significant negative correlations with the total score. The Peabody Picture Vocabulary Test-III was significantly correlated with total score in the autism spectrum group but not in the typically developing group. Age and socioeconomic status were not correlated with total score in either group. This preliminary work suggests that the Family Inventory of Sleep Habits is a valid and reliable measure of sleep hygiene in autism spectrum disorders.
Prenatal Microcephaly: Can We Be More Accurate?
Authors: Berger Itai
Published: January 2009

Consultation regarding isolated fetal microcephaly is especially difficult in a genetically heterogeneous population, as one of the factors associated with head circumference difference is ethnicity. The study presents four women referred to the neuro-fetal clinic for isolated fetal microcephaly. All fetuses had normal chromosomes, no structural abnormalities, or evidence of intrauterine infection or intoxication. Their head circumference measurements were 2 to 3 standard deviations below average for age on repeat ultrasound and fetal magnetic resonance imaging. Striking differences in chart placements were found between different professionals using different charts. In all children, measured head circumference after birth/termination was within normal limits. This study suggests that national charts should be built measuring fetal size adjusted for ethnicity and based on a genetically heterogeneous population. The charts should be customized between different professionals assessing prenatal and postnatal head circumference.
Role of TNF-α Gene Variation in Idiopathic Childhood Ischemic Stroke: A Case-Control Study
Authors: Shi Kai-Li , He Bing , Wang Jian-Jun , Zou Li-Ping
Published: January 2009

Etiology of the idiopathic childhood stroke remains unknown. In previous studies, the immunologic process may be involved in the idiopathic stroke. Tumor necrosis factor— alpha (TNF-α), an important immune mediator, may contribute to the initiation and progression in the stroke. The main purpose of this study was to investigate correlation of TNF-α genetic variation and idiopathic childhood ischemic stroke. Using the direct DNA sequencing method, polymorphisms in the TNF-α promoter region were genotyped in 67 Chinese patients with idiopathic childhood stroke and 70 controls. Among totally 7 single nucleotide polymorphisms identified in the TNF-α promoter region, the variant of the -863C/A is associated with increased risk of idiopathic childhood ischemic stroke in our study group. TNF-α molecule may have genetically as well as functionally an important role in the pathogenesis of idiopathic childhood ischemic stroke in the Chinese population.
Magnetic Resonance Imaging, Magnetic Resonance Spectroscopy, and Facial Dysmorphism in a Case of Lowe Syndrome with Novel OCRL1 Gene Mutation
Authors: Yuksel Adnan , Karaca Ender , Albayram M. Sait
Published: January 2009

Lowe syndrome is a multisystem disorder characterized by anomalies of the eye, the nervous system, and the kidney. It is an uncommon, X-linked disease. Bilateral cataract and severe hypotonia are present at birth. Psychomotor retardation is evident in childhood, while renal complications arise in adolescence. The mutation of the gene OCRL1 localized at Xq26.1 is responsible for the disease. The authors report on a 12-year-old male with mental retardation, facial dysmorphism as prominent forehead, long and slender-shaped face, prominent eyebrows, epicanthus, microphthalmia, low-posterior set ears with prominent helix and antihelix, long philtrum, and mild prognathia. He also had history of neonatal hypotonia and congenital cataracts. His cranial magnetic resonance imaging showed increased signal intensity in white matter on T2-weighted images, and magnetic resonance spectroscopy revealed elevation of the myoinositol peak at 3.56 ppm. Molecular analysis of OCRL1 gene revealed novel N574K mutation on 17th exon.
Cranial Ultrasound Lesions in the NICU Predict Cerebral Palsy at Age 2 Years in Children Born at Extremely Low Gestational Age
Authors: Kuban Karl C. K. , Allred Elizabeth N. , O'Shea T. Michael , Paneth Nigel , Pagano Marcello , Dammann Olaf , Leviton Alan , Du Plessis Adré , Westra Sjirk J. , Miller Cindy R. , Bassan Haim , Krishnamoorthy Kalpathy , Junewick Joseph , Olomu Nicholas , Romano Elaine , Seibert Joanna , Engelke Steve , Karna Padmani , Batton Daniel , O'Connor Sunila E. , Keller Cecelia E.
Published: January 2009

Our prospective cohort study of extremely low gestational age newborns evaluated the association of neonatal head ultrasound abnormalities with cerebral palsy at age 2 years. Cranial ultrasounds in 1053 infants were read with respect to intraventricular hemorrhage, ventriculomegaly, and echolucency, by multiple sonologists. Standardized neurological examinations classified cerebral palsy, and functional impairment was assessed. Forty-four percent with ventriculomegaly and 52% with echolucency developed cerebral palsy. Compared with no ultrasound abnormalities, children with echolucency were 24 times more likely to have quadriparesis and 29 times more likely to have hemiparesis. Children with ventriculomegaly were 17 times more likely to have quadriparesis or hemiparesis. Forty-three percent of children with cerebral palsy had normal head ultrasound. Focal white matter damage (echolucency) and diffuse damage (late ventriculomegaly) are associated with a high probability of cerebral palsy, especially quadriparesis. Nearly half the cerebral palsy identified at 2 years is not preceded by a neonatal brain ultrasound abnormality.
Blood Flow Velocities Are Reduced in the Optic Nerve of Children with Elevated Intracranial Pressure
Authors: Miller Marijean M. , Chang Taeun , Keating Robert , Crouch Eric , Sable Craig
Published: January 2009

The authors previously used spectral Doppler imaging to determine optic nerve blood flow velocities in normal children. In the current study, we measured central retinal artery and central retinal vein blood flow velocities by spectral Doppler imaging in 38 healthy children and 18 children with elevated intracranial pressure between ages 4 and 17. We found central retinal artery systolic blood flow velocity was significantly reduced in children with elevated increased intracranial pressure; ANOVA P = .01 (normal children 8.9 cm/s [SD 1.1] versus children with elevated intracranial pressure 7.5 cm/s [SD 1.3]). Central retinal vein maximal blood flow velocity was also significantly reduced in children with elevated intracranial pressure; ANOVA P < .02 (normal children 4.2 cm/s [SD 0.9] versus children with elevated intracranial pressure 3.6 cm/s [SD 0.7]). Spectral Doppler imaging is a noninvasive test well tolerated in children that identifies blood flow velocity changes in elevated intracranial pressure.
From Fossil to Fetus: Nonhemorrhagic Germinal Matrix Echodensity Caused by Mineralizing Vasculitis—Hypothesis of Fossilizing Germinolysis and Gliosis
Authors: van Baalen Andreas , Rohr Axel
Published: January 2009

Subventricular echodensities medioventral to the caudate nucleus near the caudothalamic groove are regular ultrasonographic findings in preterm infants with germinal matrix hemorrhages. The transformation into pseudocysts is termed germinolysis. Interestingly, identical ultrasonographic findings are rarely even reported for near-term infants. We examined 5 cases of near-term infants presenting with various localized subventricular echodensities after various noncerebral infections (intra-amniotic infection, gastroenteritis of unidentified pathogens, septic wound, and a twin pair with rotavirus gastroenteritis), suggesting an inflammatory or ischemic rather than a hemorrhagic etiology. With high-resolution ultrasonography, these lesions showed similar characteristics: all were space-occupying, homogeneous, echodense, and ultimately transformed into pseudocysts. Additionally, Doppler ultrasonography showed linear perivascular echodensities in the basal ganglia of one infant. All infants were also examined by cerebral magnetic resonance imaging (MRI) including T2*-weighted gradient-echo sequences so that subventricular hemorrhages could be excluded in 4 infants. One of these 4 infants died unexpectedly at home. Brain autopsy revealed nonhemorrhagic germinal matrix pseudocysts with gliotic walls. Based on our findings and the original literature, we favored an ischemic infarction of the strongly vascularized and highly metabolic germinal matrix as cause of this germinolysis and gliosis. We postulated that the infarction was the result of an obstructive and possibly mineralizing, or even fossilizing vasculopathy due to an inflammatory response. The precise pathogenesis of the suspected immune-mediated vasculitis remains unknown. We concluded that cerebral ultrasonography should be performed in infants with noncerebral infection and that it should be complemented by MRI in cases of subventricular echodensities to prove or to exclude a germinal matrix hemorrhage.
Unilateral Venous Thalamic Infarction in a Child Mimicking a Thalamic Tumor
Authors: Haug Verena , Linder-Lucht Michaela , Zieger Barbara , Korinthenberg Rudolf , Mall Volker , Mader Irina
Published: January 2009

Unilateral thalamic infarction is a rare condition in adults. This is a case report of a young child presenting with left-sided hemiparesis of sudden onset due to an unilateral venous thalamic infarction. This was attributed to an asymmetric thrombosis of the cerebral internal veins, a partial thrombosis of the vein of Galen and straight sinus. Magnetic resonance imaging resulted primarily in the differential diagnosis of a cerebral tumor or an intracerebral abscess, leading to stereotactic puncture. Subsequent magnetic resonance venography facilitated the correct diagnosis. Heparin-induced thrombocytopenia necessitated anticoagulation treatment with hirudin and later, warfarin. The patient made a complete recovery. We conclude that unclear unilateral thalamic lesions might be symptomatic of a cerebral deep venous thrombosis and might mimic a thalamic tumor. In uncertain cases, we suggest rapid performance of magnetic resonance angiography.
Cerebral Sinus Venous Thrombosis Complicated by Cerebellar Hemorrhage in a Child With Acute Promyelocytic Leukemia
Authors: Beslow Lauren A. , Abend Nicholas S. , Smith Sabrina E.
Published: January 2009

A 12-year-old boy who presented with headache and papilledema was diagnosed with cerebral sinus venous thrombosis and promyelocytic leukemia. Cerebral sinus venous thrombosis is an uncommon manifestation of acute promyelocytic leukemia, and acute promyelocytic leukemia is an unusual etiology for cerebral sinus venous thrombosis. This case highlights the importance of a complete evaluation for risk factors in a child presenting with cerebral sinus venous thrombosis. Anticoagulation was initiated for treatment of cerebral sinus venous thrombosis, and new asymptomatic hemorrhage was identified in the cerebellum 1 day later. Anticoagulation was discontinued for 5 days and then reinitiated in the intensive care unit without hemorrhage expansion or other complications. We review the literature regarding anticoagulation in children with cerebral sinus venous thrombosis and issues specific to acute promyelocytic leukemia. This case suggests that in a controlled setting, anticoagulation may be safe in selected patients with cerebellar hemorrhage.
Serum and Muscle Carnitine Levels in Epileptic Children Receiving Sodium Valproate
Authors: Anil Murat , Helvaci Mehmet , Ozbal Ebru , Kalenderer Onder , Anil Ayse Berna , Dilek Mustafa
Published: January 2009

The purpose of this study was to determine whether children with epilepsy undergoing valproate therapy and who are otherwise healthy have lower levels of serum and muscle carnitine. A total of 50 patients with epilepsy, 3 to 14 years of age, who were treated solely with valproate and free of abnormal neurologic findings or nutritional problems were selected. The control group consisted of 30 healthy children. The total carnitine levels in serum were 28.1 ± 10.3 and 55.6 ±7.3 μg/mL, and the free carnitine levels in serum were 16.5 ±10.2 and 44.6 ±7.3 μg/mL, the total carnitine levels in muscle were 12.1 ± 1.8 and 45.3 ± 5.9 μmol/g noncollagen protein and the free carnitine levels in muscle were 5.6 ± 1.6 and 39.3 ± 6.0 μmol/g noncollagen protein in the valproic acid—treated and control groups, respectively (P < .05). In conclusion, valproate monotherapy depletes both muscle and serum carnitine levels in otherwise healthy epileptic children.
From Eugenic Euthanasia to Habilitation of ``Disabled'' Children: Andreas Rett's Contribution
Authors: Ronen Gabriel M. , Meaney Brandon , Dan Bernard , Zimprich Fritz , Stögmann Walter , Neugebauer Wolfgang
Published: January 2009

Although the name of Andreas Rett is familiar to many from his eponymous neurogenetic syndrome, his other achievements involving the care of disabled children deserve special attention. His tireless advocacy helped to bring fundamental changes in the medical and societal attitude toward disabled individuals in a city that had recently seen more than 7500 disabled children and inmates of psychiatric hospitals actively euthanized by National Socialist (Nazi) decree. Most notably, this study demonstrates the remarkable changes that can be achieved single-handedly by a vocal and energetic physician. Yet at the same time, several instances are recorded in which Rett appeared to prioritize his own professional advancement at the expense of truthful disclosure of his own past, as well as that of some of his close associates. Dr Rett's professional life and contributions, now 10 years after his death, presents a compelling object lesson for neurologists and others involved in the care of the disabled.
Megalencephalic Leukoencephalopathy with Subcortical Cysts in a Tunisian Boy
Authors: Tinsa Faten , Farid Olfa , Douira Wiem , Rodriguez Diana , Burglen Lydie , Boussetta Khadija , Bousnina Souad
Published: January 2009

Megalencephalic leukoencephalopathy with subcortical cysts is an autosomal recessive cerebral white-matter disorder in children. Megalencephalic leukoencephalopathy with subcortical cysts is caused by mutation in the megalencephalic leukoencephalopathy with subcortical cysts gene 1. The authors report a case of a 2-year-old Tunisian boy who had presented seizure following minor head trauma. He had megalencephaly, cerebellar ataxia, and mild pyramidal signs at physical examination. Magnetic resonance imaging showed extensive white-matter signal changes involving the corpus callosum and temporal subcortical cysts consistent with megalencephalic leukoencephalopathy with subcortical cysts. The molecular genetic study of the megalencephalic leukoencephalopathy with subcortical cysts gene 1 identified a homozygous mutation C46fsX57 in the patient. The mutation was found to be heterozygous in the parents. To the best of the authors' knowledge, this mutation has never been reported.
Framing Third-Sector Contributions to Service Provision
Authors: Never Brent
Published: January 2009

Third-sector organizations provide essential services, but not all types of organizations operate equally well given different intensities of public problems. This article considers the need to create three-dimensional maps of the sector matching populations of service providers with the intensities of public problems where they operate. It does so by providing a framework for understanding third-sector organizational service provision. It is illustrated by following a case of essential service provision in the face of state failure. It concludes by highlighting the utility of three-dimensional maps for policy makers.
Host Students' Perspectives of Intercultural Contact in an Irish University
Authors: Dunne Ciarán
Published: January 2009

Given the increasing numbers of international students in Ireland and the lack of attention afforded to host culture students in existing research on intercultural relations in higher education, a grounded theory study was conducted in an Irish university exploring host (Irish) students' perspectives on intercultural contact. The study focused on students' construction of cultural difference within the educational environment, the factors influencing intercultural contact, and students' experiences of such contact. The findings suggest that although nationality and age are used to differentiate students, the concept of “maturity”—underpinned by values and behaviors relating to academic motivations, responsibilities, and authority—is central to students' construction of cultural difference on campus.

Diverse factors identified as impacting upon students' intercultural acquaintance prospects and relational development are presented and discussed. The findings highlight the complexity of promoting meaningful intercultural contact among students and also suggest conditions to foster intercultural contact based on students' feedback.