bluuebunny/crossref_metadata_2025 huggingface dataset:
Ecological Genetics of Plant Invasion: What Do We Know?
Authors: Ward Sarah M. , Gaskin John F. , Wilson Linda M.
Published: February 2008

The rate at which plant invasions occur is accelerating globally, and a growing amount of recent research uses genetic analysis of invasive plant populations to better understand the histories, processes, and effects of plant invasions. The goal of this review is to provide natural resource managers with an introduction to this research. We discuss examples selected from published studies that examine intraspecific genetic diversity and the role of hybridization in plant invasion. We also consider the conflicting evidence that has emerged from recent research for the evolution of increased competitiveness as an explanation for invasion, and the significance of multiple genetic characteristics and patterns of genetic diversity reported in the literature across different species invasions. High and low levels of genetic diversity have been found in different invading plant populations, suggesting that either selection leading to local adaptation, or pre-adapted characteristics such as phenotypic plasticity, can lead to aggressive range expansion by colonizing nonnative species. As molecular techniques for detecting hybrids advance, it is also becoming clear that hybridization is a significant component of some plant invasions, with consequences that include increased genetic diversity within an invasive species, generation of successful novel genotypes, and genetic swamping of native plant gene pools. Genetic analysis of invasive plant populations has many applications, including predicting population response to biological or chemical control measures based on diversity levels, identifying source populations, tracking introduction routes, and elucidating mechanisms of local spread and adaptation. This information can be invaluable in developing more effectively targeted strategies for managing existing plant invasions and preventing new ones. Future genetic research, including the use of high throughput molecular marker systems and genomic approaches such as microarray analysis, has the potential to contribute to better understanding and more effective management of plant invasions.
Toward an Understanding of Why Students Contribute in Asynchronous Online Discussions
Authors: Cheung Wing Sum , Hew Khe Foon , Ng Connie Siew Ling
Published: February 2008

The use of online learning is growing very fast in universities. Consequently, understanding how to promote student contribution in asynchronous online discussions, which is considered an integral part of online learning, has become increasingly crucial. Previous research has examined how factors, such as instructor facilitation techniques may influence student contribution. However, issues such as when the online discussion is student facilitated, and students are given a freedom of choice to choose, why students choose to contribute in some forums but not in others are not fully understood. In this article, we report a case study involving pre-service teachers in Singapore that examined this issue. Data were collected from the pre-service teachers' online postings, reflection logs, questionnaires, and interview data. Results suggested six themes that influenced students' decision to contribute or not to contribute: a) relational capital; b) knowledge about the subject or topic; c) discussion activity or topic; d) availability of time; e) reward; and f) random choice. Practical recommendations to motivate students to contribute and future research directions pertaining to asynchronous online discussions are suggested.
From the Social-Cognitive Career Theory Perspective: A College of Education Faculty Model for Explaining Their Intention to Use Educational Technology
Authors: Sahin Ismail
Published: February 2008

A model, based on Social-Cognitive Career Theory (SCCT) (Lent, Brown, & Hackett, 1994, 2002), was developed to test the influence of the College of Education (COE) faculty members' self-efficacy in, outcome expectations from, and interest in educational technology on their intentions to use the technology. While outcome expectations affect faculty members' intentions to use educational technology both directly and indirectly, the influence of self-efficacy beliefs on their willingness to use the technology is exerted through outcome expectations and interest. Overall, the findings from this study displayed a significant contribution of self-efficacy in, outcome expectations from, and interest in educational technologies in the formation of faculty members' intentions to use these technologies. Efforts to develop COE faculty into the leaders of educational technology might benefit from the findings of this study. When levels of faculty confidence in and awareness of educational technology increase, faculty interest in the technology will grow and eventually result in a higher level of faculty willingness to use educational technology.
Hormesis [Biological Effects of Low Level Exposures (Belle)] and Dermatology
Authors: Thong Haw-Yueh , Maibach Howard I.
Published: February 2008

Hormesis, or biological effects of low level exposures (BELLE), is characterized by nonmonotonic dose response which is biphasic, displaying opposite effects at low and high dose. Its occurrence has been documented across a broad range of biological models and diverse type of exposure. Since hormesis appears to be a relatively common phenomenon in many areas, the objective of this review is to explore its occurrence related to dermatology and its public health and risk assessment implication. Hormesis appears to be a common phenomenon in in-vitro skin biology. However, in vivo data are lacking and the clinical relevance of hormesis has yet to be determined. Better understanding of this phenomenon will likely lead to different strategies for risk assessment process employed in the fields of dermatologic toxicology and pharmacology. We believe that hormesis is a common phenomenon and should be given detailed consideration to its concept and its risk assessment implications, and how these may be incorporated into the experimental and regulatory processes in dermatology. The skin, with its unique characteristics, its accessibility, and the availability of non-invasive bioengineering and DNA microarray technology, will be a good candidate to extend the biology of hormesis.
Staging Turkish Women’s Emancipation
Authors: Libal Kathryn
Published: February 2008

This article examines debates over Turkish women’s emancipation and women’s independent organizing in Turkey during the 1930s. It traces the troubled history of Turkey’s most prominent independent women’s organization, the Turkish Women’s Union (Türk Kadın Birliği), focusing especially on the Twelfth Congress of the International Alliance of Women (IAW) held in Istanbul in 1935. Despite the renown of the Women’s Union, it was forced to disband shortly after the Istanbul Congress. Drawing upon popular press accounts, official records of the Istanbul Congress, and correspondence between the Women’s Union and IAW members, this analysis underscores how deeply contested the question of women’s emancipation was, not only within urban elite society, but also among those in municipal and state office. It also provides insights into how actively Turkish feminists engaged questions of peace, disarmament, and Turkey’s role in geopolitics, challenging the view that women were best suited to contribute to social and family policies rather than foreign policy.
Globalizing Iranian Feminism, 1910–1950
Authors: Amin Camron Michael
Published: February 2008

The author examines how the Women’s Party (Hizb-i Zanan) used globalization to signal its departure from the tendency of women’s organizations to prioritize national goals over feminist goals in Iran. In explicitly holding the Iranian government to emerging international standards of equal rights feminism, the Women’s Party contributed to an enduring aspect of Iranian culture in which national conversations imagine a global audience. The thesis rests on two elements: 1) the use of recently published Iranian document sets and a previously unused press source to illustrate the party’s innovation in feminist advocacy in the context of Iran’s first women’s suffrage campaign from 1943 to 1946, and in direct comparison with an earlier organization, the Patriotic Women’s League and its hosting of the Second Eastern Women’s Congress in Tehran in 1932, and 2) the development of criteria for historicizing “globalization” that may be applicable beyond this particular case.
Between Nationalism and Feminism
Authors: Weber Charlotte
Published: February 2008

This essay examines the Eastern Women’s Congresses in Damascus (1930) and Tehran (1932) to show how Middle Eastern women attempted to stake their own claim to modernity within the established terms of nationalist and international feminist discourses. I argue that in organizing across national boundaries, in seeking recognition from the international women’s movement, and above all in articulating a uniquely “Eastern” framework in which to ground women’s rights, the delegates tried to create an autonomous women’s movement that was allied with but independent from both Middle Eastern male nationalists and Western feminists. Simultaneously drawing inspiration and seeking autonomy from Western models, theirs was an attempt to define feminism in their own terms—one that defied the presumption of male reformers who tried to arrogate to themselves the task of modernizing Eastern womanhood.
Spina bifida occulta: is it a predictor of underlying spinal cord abnormality in patients with lower urinary tract dysfunction?
Authors: Nejat Farideh , Radmanesh Farid , Ansari Saeed , Tajik Parvin , Kajbafzadeh Abdolmohammad , Khashab Mostafa El
Published: February 2008

Object

The purpose of this study was to evaluate the importance of spina bifida occulta in radiographs of children with lower urinary tract or bowel dysfunction.

Methods

The authors prospectively investigated the presence of spinal cord abnormalities in 176 patients with functional urinary and bowel problems: 88 children with radiographic evidence of spina bifida occulta (SBO) and 88 age-and sex-matched controls. Each group included 46 boys and 42 girls (age range 5–14 years). Nocturnal enuresis, isolated diurnal enuresis, enuresis during both day and night, urinary tract infection, urinary frequency, encopresis, intractable constipation, and vesicoureteral reflux were assessed in all patients. Magnetic resonance (MR) images were obtained in all patients and evaluated for spinal cord abnormalities. Sacral ratios (SRs) were calculated on the basis of plain radiographs.

Results

There was no statistically significant difference between the 2 groups on any of the clinical measures. The most common sites of SBO on radiographs were the S-1 (47%) and L-5 and S-1 (25%). The mean SRs (± standard deviations) in the SBO and control groups, respectively, were 0.64 ± 0.45 and 0.68 ± 0.51 (no statistically significant difference). Sacral agenesis was found in 17 children (7 in the SBO group and 10 in the control group, p = 0.44). Abnormal MR imaging findings were observed in 9 children (10.22%) in the SBO group and 3 (3.4%) in the control group. Abnormalities included tethered spinal cord in 5 children, syringomyelia in 4, and club-shaped conus in 2. No significant association was found between the presence of SBO and spinal cord abnormalities identified on MR images (p = 0.13, paired t-test).

Conclusions

Among children with functional bowel and urinary problems, there was no statistically significant difference in the prevalence of abnormal spinal MR imaging findings in those with radiographic SBO and an age- and sex-matched control group. Spina bifida occulta was not shown to be a reliable indicator of spinal cord structural abnormalities. Its probable role as a finding associated with spinal cord dysfunction remains unclear.
Ventriculoperitoneal shunt blockage due to spontaneous knot formation in the peritoneal catheter
Authors: Eftekhar Behzad , Hunn Andrew
Published: February 2008

✓The authors report the third case of ventriculoperitoneal shunt blockage due to spontaneous knot formation in the peritoneal catheter that had been placed in a 3.5-year-old boy 8 months earlier. On surgical exploration a double knot was found 10 cm from the distal end of the peritoneal catheter. Although the underlying mechanism remains unknown, the authors used the analogy of related physical studies and true knot formation in the umbilical cord and determined the possible causes as related to the catheter, volume and configuration of the abdomen, and kinetics of the catheter movements. If further study should reveal a significantly higher incidence of this complication, the authors suggest further in vitro studies, designed to investigate the optimal characteristics and safe range of length of peritoneal catheters in different situations.
Epidural abscess treated with a medial supraorbital craniotomy through an incision in the eyebrow
Authors: Rosen David S. , Shafizadeh Stephen , Baroody Fuad M. , Yamini Bakhtiar
Published: February 2008

✓The authors describe a medial supraorbital craniotomy performed through a medial eyebrow skin incision to approach an epidural abscess located in the medial anterior fossa of the skull. An 8-year-old boy presented with fevers and facial swelling. Imaging demonstrated pansinusitis and an epidural fluid collection adjacent to the frontal sinus. A medial supraorbital craniotomy was performed to access and drain the epidural abscess. The supraorbital nerve laterally and the supratrochlear nerve medially were preserved by incising the frontalis muscle vertically, parallel to the course of the nerves, and dissecting the subperiosteal plane to mobilize the nerves. This approach may be a useful access corridor for other lesions located near the medial anterior fossa.
Desmoplastic infantile astrocytoma and characteristics of the accompanying cyst
Authors: Beppu Takaaki , Sato Yuichi , Uesugi Noriyuki , Kuzu Yasutaka , Ogasawara Kuniaki , Ogawa Akira
Published: February 2008

✓A desmoplastic infantile astrocytoma (DIA) is an extremely rare tumor that comprises a solid astrocytic tumor accompanied by a large cyst and involves the superficial cerebral cortex and leptomeninges in infants. The solid part of this type of tumor has been well described in various reports and books, but characteristics of the cystic portion have remained unclear. Because adequate resection is required to ensure a favorable prognosis, information about the cyst is very important for diagnostic purposes and surgical planning. The authors report on the clinical and histological features of the cyst in a case of a DIA. A 12-month-old boy presented with vomiting. Contrast-enhanced magnetic resonance imaging revealed a strongly enhancing single-lobed large cyst located in the deep white matter, under the solid part of the tumor attached to the dura mater of the left frontal lobe. Both the solid and cystic portions of the tumor were surgically removed. The border between the cyst wall and surrounding white matter was unclear. Histologically, the cyst wall was composed of gliosis representing a rough accumulation of reactive astrocytes, lymphocytes, and small capillary vessels in edematous parenchyma, but no tumor cells. The present case and previous reports suggest that the cyst does not contain tumor cells, even if strongly depicted on contrast-enhanced neuroimaging, and that a thickly enhancing cyst wall indicates gliosis with accumulation of numerous small vessels.
Development of an acquired Chiari malformation Type I in the setting of an untreated lipomyelomeningocele
Authors: Waldau Ben , Grant Gerald , Fuchs Herbert
Published: February 2008

✓The authors present the case of a child with an untreated lipomyelomeningocele who developed an acquired Chiari malformation Type I (CM-I) with a large syrinx over the course of 3 years. To the best of the authors' knowledge, this is the first report to document a case in which an acquired CM-I evolved in a patient with an untreated tethered cord.
Measurement of spinal range of motion in healthy individuals using an electromagnetic tracking device
Authors: Hsu Chien-Jen , Chang Yi-Wen , Chou Wen-Ying , Chiou Chou-Ping , Chang Wei-Ning , Wong Chi-Yin
Published: February 2008

Object

The authors measured the range of motion (ROM) of the spine in healthy individuals by using an electromagnetic tracking device to evaluate the functional performance of the spine.

Methods

The authors used the Flock of Birds electromagnetic tracking device with 4 receiver units attached to C-7, T-12, S-1, and the midthigh region. Forward/backward bending, bilateral side bending, and axial rotation of the trunk were performed in 18 healthy individuals.

Results

The average ROM was calculated after 3 consecutive measurements. The thoracic spine generated the greatest angle in axial rotation and smallest angle in backward bending. The lumbar spine generated the greatest angle in forward bending and smallest angle in axial rotation. The hip joints generated the greatest angle in forward bending and smallest angle in backward bending. Additionally, 40% of forward-bending motion occurred in the lumbar spine and 40% occurred in the hip joints. Approximately 60% of backward bending occurred in the lumbar spine; 60% of axial rotation occurred in the thoracic spine; and 45% of side bending occurred in the thoracic spine.

Conclusions

The Flock of Birds electromagnetic tracking device cannot only measure the ROM of spine but also easily differentiate the 6-degree contributions by different segments.
Biomechanical comparison of occipitoatlantal screw fixation techniques
Authors: Bambakidis Nicholas C. , Feiz-Erfan Iman , Horn Eric M. , Gonzalez L. Fernando , Baek Seungwon , Yüksel K. Zafer , Brantley Anna G. U. , Sonntag Volker K. H. , Crawford Neil R.
Published: February 2008

Object

The stability provided by 3 occipitoatlantal fixation techniques (occiput [Oc]–C1 transarticular screws, occipital keel screws rigidly interconnected with C-1 lateral mass screws, and suboccipital/sublaminar wired contoured rod) were compared.

Methods

Seven human cadaveric specimens received transarticular screws and 7 received occipital keel–C1 lateral mass screws. All specimens later underwent contoured rod fixation. All conditions were studied with and without placement of a structural graft wired between the skull base and C-1 lamina. Specimens were loaded quasistatically using pure moments to induce flexion, extension, lateral bending, and axial rotation while recording segmental motion optoelectronically. Flexibility was measured immediately postoperatively and after 10,000 cycles of fatigue.

Results

Application of Oc–C1 transarticular screws, with a wired graft, reduced the mean range of motion (ROM) to 3% of normal. Occipital keel–C1 lateral mass screws (also with graft) offered less stability than transarticular screws during extension and lateral bending (p < 0.02), reducing ROM to 17% of normal. The wired contoured rod reduced motion to 31% of normal, providing significantly less stability than either screw fixation technique. Fatigue increased motion in constructs fitted with transarticular screws, keel screws/lateral mass screw constructs, and contoured wired rods, by means of 19, 5, and 26%, respectively. In all constructs, adding a structural graft significantly improved stability, but the extent depended on the loading direction.

Conclusions

Assuming the presence of mild C1–2 instability, Oc–C1 transarticular screws and occipital keel–C1 lateral mass screws are approximately equivalent in performance for occipitoatlantal stabilization in promoting fusion. A posteriorly wired contoured rod is less likely to provide a good fusion environment because of less stabilizing potential and a greater likelihood of loosening with fatigue.
Transplantation of human bone marrow–derived stromal cells into the contused spinal cord of nude rats
Authors: Sheth Rishi N. , Manzano Glen , Li Xiuming , Levi Allan D.
Published: February 2008

Object

Human bone marrow stromal cells (hMSCs) constitute a potential source of pluripotent stem cells. In the present study, hMSCs were transplanted into an area of spinal cord contusion in nude rats to determine their survival, differentiation, potential for neuroprotection, and influence on axonal growth and functional recovery.

Methods

Twenty-nine animals received 6 × 105hMSCs in 6 μl medium 1 week after a contusion, while 14 control animals received an injection of 6 μl medium alone. Basso–Beattie–Bresnahan (BBB) tests were performed weekly. The spinal cords were collected at 6 weeks posttransplantation for histological analysis and assessment of tissue injury.

Results

Immunostaining with anti–human mitochondria antibody and pretransplantation labeling with green fluorescent protein demonstrated that the grafted hMSCs survived and were capable of achieving a flattened appearance in the grafted area; however, none of the transplanted cells stained positively for human-specific neuronal, anti–neurofilament H or glial fibrillary acidic protein within the sites of engraftment. While neuronal or astrocytic differentiation was not seen, cells lining blood vessels in the vicinity of the transplant stained positively for anti–human endothelium CD105 antibody. Staining for anti–neurofilament H antibody demonstrated abundant axonlike structures around the transplanted area in the hMSC group. Tissue sparing analysis showed that animals with grafted hMSCs had a smaller area of contusion cyst compared with controls, but there was no significant difference between the two groups in BBB scores.

Conclusions

The grafted hMSCs survived for ≥ 6 weeks posttransplantation, although they did not differentiate into neural or glial cells. Cells with human endothelial characteristics were observed. Spinal cord–injured rats grafted with hMSCs had smaller contusion cavities, which did not have a significant influence on functional recovery.
Syringomyelia caused by intrathecal remnants of oil-based contrast medium
Authors: Kubota Mayumi , Shin Masahiro , Taniguchi Makoto , Terao Toru , Nakauchi Jun , Takahashi Hiroshi
Published: February 2008

✓ Oily contrast medium had been in use since the early 19th century as a radiographic agent for detecting spinal lesions and spinal cord tumors until the late 20th century. At that point computed tomography scanning and magnetic resonance imaging, or other hydrophilic contrast medium substituted for it. Adverse effects of oil-based dye, both acute and chronic, had been reported since the middle of the 20th century. In this paper the authors report the case of syringomyelia that seemed to be caused mainly by remaining oily contrast medium for 44 years.

Syringomyelia secondary to adhesive arachnoiditis caused by oily contrast medium after a long period of time is well known. In the present case, however, surgery revealed only mild arachnoiditis at the level of syringomyelia as well as both solid and liquid remnants of contrast medium.

Generally, cerebrospinal fluid (CSF) blockage due to an arachnoid adhesion is considered to cause syringomyelia following adhesive arachnoiditis. The authors speculated that in the present case syringomyelia was induced by a mechanism different from that in the previously reported cases; the oily contrast medium itself seems to have induced the functional block of CSF and impaired the buffer system of the intrathecal pressure. No reports on thoracic adhesive arachnoiditis and syringomyelia caused by oil-based dye referred to this mechanism in reviewing the literature.
Extradural arteriovenous fistulas involving the vertebral artery in neurofibromatosis Type 1
Authors: Paolini Sergio , Colonnese Claudio , Galasso Vittorio , Morace Roberta , Tola Serena , Esposito Vincenzo , Cantore Giampaolo
Published: February 2008

✓ Spinal extradural arteriovenous fistulas (AVFs) are rare lesions that may be associated with neurofibromatosis Type 1 (NF1). In these patients, the shunt typically occurs between the V2 segment of the vertebral artery and the epidural venous plexus. Previously, reported cases have been treated either by using endovascular embolization or, sporadically, by open surgery. In surgical reports, proximal deafferentation or manipulation of the venous portion of the shunt—including suture, resection, or open embolization of the epidural ectasia—was attempted with variable results. The authors report on a case of a young patient with NF1 who underwent emergency surgical disconnection of a cervical extradural AVF after previously unsuccessful endovascular and surgical therapy. The lesion drained into a giant intrathecal varix, causing severe myelopathy. After surgery, the patient recovered almost completely. This experience clarified the surgical anatomy of these malformations and showed that, when surgery is necessary, the optimal treatment providing complete and permanent cure of this condition is direct closure of the epidural shunt pedicle.
A minimally invasive technique for L5–S1 intraforaminal disc herniations: microdiscectomy with a tubular retractor via a contralateral approach
Authors: Yeom Jin S. , Kim Kyeong Hwan , Hong Soon Woo , Park Kun-Woo , Chang Bong-Soon , Lee Choon-Ki , Buchowski Jacob M.
Published: February 2008

✓ Surgical treatment of intraforaminal disc herniations at the L5–S1 level is technically demanding. The 2 most commonly used procedures involve either a medial or lateral ipsilateral approach and often require a partial or even complete facet resection, which may in turn result in vertebral instability and/or back pain, as well as, in some cases, a fusion or stabilization procedure. In this report, the authors present a new minimally invasive technique for the treatment of L5–S1 intraforaminal disc herniations. Using this technique, which involves tubular retractors and an operative microscope to approach the neural foramen from the contralateral side, the authors could easily visualize and remove the herniated disc material and perform a thorough microdiscectomy with minimal resection of osseous and ligamentous structures. To illustrate this new minimally invasive technique for the treatment of intraforaminal disc herniations at L5–S1, they describe the cases of 2 patients who underwent the procedure and in whom successful results were achieved.
The clinical atlas of Greig cephalopolysyndactyly syndrome
Authors: Balk Katherine , Biesecker Leslie G.
Published: February 2008

Greig cephalopolysyndactyly syndrome (GCPS) is a rare multiple congenital anomaly syndrome that is inherited in an autosomal dominant pattern and is caused by haploinsufficiency of the GLI3 gene. The syndrome typically includes preaxial or mixed pre‐ and postaxial polydactyly and cutaneous syndactyly, ocular hypertelorism, and macrocephaly in its typical forms, but sometimes includes hydrocephalus, seizures, mental retardation, and developmental delay in more severe cases. Patients with milder forms of GCPS can have subtle craniofacial dysmorphic features that are difficult to distinguish from normal variation. This article presents the spectrum of dysmorphic findings in GCPS highlighting some of its key presenting features to familiarize clinicians with the variable expressivity of the condition. © 2008 Wiley‐Liss, Inc.
Perrault syndrome: Report of four new cases, review and exclusion of candidate genes
Authors: Marlin Sandrine , Lacombe Didier , Jonard Laurence , Leboulanger Nicolas , Bonneau Dominique , Goizet Cyril , Billette de Villemeur Thierry , Cabrol Sylvie , Houang Muriel , Moatti Lucien , Feldmann Delphine , Denoyelle Françoise
Published: February 2008

We report on two sporadic and two familial new cases with sensorineural hearing impairment and ovarian dysgenesis which are the cardinal signs of Perrault syndrome in females. Only one of them has a nervous system defect. We reviewed all the published cases of Perrault syndrome in order to define the clinical variability and to evaluate the frequency of the neurological anomalies in this clinical entity. Moreover we excluded GJB2, POLG, and FOXL2 as candidate genes in Perrault syndrome. © 2008 Wiley‐Liss, Inc.
Genotype–phenotype analysis and natural history of left ventricular hypertrophy in LEOPARD syndrome
Authors: Limongelli Giuseppe , Sarkozy Anna , Pacileo Giuseppe , Calabrò Paolo , Digilio Maria Cristina , Maddaloni Valeria , Gagliardi Giulia , Di Salvo Giovanni , Iacomino Maria , Marino Bruno , Dallapiccola Bruno , Calabrò Raffaele
Published: February 2008

Because it is unclear whether the genotype may influence the clinical course in patients with LEOPARD syndrome (LS), we analyzed clinical and molecular predictors of adverse cardiac events in patients with left ventricular hypertrophy (LVH). A comprehensive cardiovascular evaluation, including baseline electrocardiogram, echocardiography, exercise test and 24 hr Holter monitoring at the time of clinical diagnosis and during follow‐up was conducted on 24 patients referred to our departments. Phenotypical examination and diagnosis were performed by expert clinical geneticists. The entire PTPN11 and RAF1 coding regions were screened for mutations by DHPLC analysis, followed by sequencing. Patients without PTPN11 mutations (34%) showed a higher frequency of family history of sudden death (P = 0.007), increased left atrial dimensions (P = 0.05), bradyarrhythmias (P = 0.04), episodes of supraventricular tachycardias (P = 0.06), atrial fibrillation (P = 0.009), and nonsustained ventricular tachycardias (P = 0.05) during Holter monitoring. Six patients (25%) had adverse cardiac events during follow‐up (including sudden deaths, resuscitated cardiac arrest, septal myectomy, and heart failure). LVH, New York Heart Association Class, left ventricular outflow tract obstruction, and nonsustained ventricular tachycardias were associated to adverse cardiac events. Of note, three patients with mutations in exon 13 showed a severe obstructive cardiomyopathy, with serious cardiac complications during follow‐up (heart failure, septal myectomy, and sudden death). In conclusion, patients with LVH associated with LS seem to carry a relatively high risk of adverse (arrhythmic and nonarrhythmic) events. Further genotype–phenotype studies are warranted to fully elucidate the impact of the genotype on the natural history of patients with LS and LVH. © 2008 Wiley‐Liss, Inc.
Clinical and radiographic delineation of odontochondrodysplasia
Authors: Unger Sheila , Antoniazzi Franco , Brugnara Milena , Alanay Yasemin , Caglayan Ahmet , Lachlan Katherine , Ikegawa Shiro , Nishimura Gen , Zabel Bernhard , Spranger Jürgen , Superti‐Furga Andrea
Published: February 2008

The association of dentinogenesis imperfecta (DI) with a distinct form of chondrodysplasia in a boy was reported by Goldblatt et al. [1991; Am J Med Genet 39:170–172] and has been given the name of Goldblatt syndrome or odontochondrodysplasia (ODCD; OMIM#184260). Since the original description, only four further individuals have been reported (one sib pair and two unrelated cases). We report on an additional six individuals, including a second sib pair (brother and sister), with clinical and radiographic features that cluster and thus confirm the nosologic status of this entity. The main radiographic features are congenital platyspondyly with coronal clefts, severe metaphyseal changes particularly of the hands, wrists, and knees, mesomelic limb shortening, and coxa valga. The main physical signs are short stature, joint laxity, narrow chest, scoliosis, and DI. This combination of clinical and radiographic findings allows clear recognition of this syndrome in early childhood. Of note, the signs that are present in the newborn period are not entirely specific and the differential diagnosis includes spondylometaphyseal dysplasia (SMD) Sedaghatian type or platyspondylic lethal dysplasia (PSLD) Torrance type. The occurrence of two sib pairs in a group of only 11 patients suggests an autosomal recessive inheritance pattern. Overmodification of cartilage‐extracted collagen 2 has been reported in two sibs, but mutation analysis of COL2A1 as well as of COMP, FGFR3, RMRP, and SBDS in one or more patients have given negative results, and the molecular etiology is as yet unknown. © 2008 Wiley‐Liss, Inc.
Health‐related quality of life of children with a positive carrier status for inherited cardiovascular diseases
Authors: Smets Ellen M.A. , Stam Marinka M.H. , Meulenkamp Tineke M. , van Langen Irene M. , Wilde Arthur A.M. , Wiegman Albert , de Wert Guido M. , Tibben Aad
Published: February 2008

Familial hypercholesterolemia, hypertrophic cardiomyopathy, and long QT Syndrome are genetic cardiovascular conditions which may lead to sudden cardiac death at a young age. Preventive measures include lifestyle modifications, medications, and/or cardiac devices. Hence, identification of carrier children can protect them for the potentially life threatening consequences at a young age. Yet, informing children about their genetic risk status and subjecting them to treatment may have negative consequences. This preliminary study aimed to explore (1) how the health‐related quality of life of carrier children compares to the quality of life of Dutch children in general; and (2) to what extent the carrier children's quality of life and their parents' perception thereof concur. Our method involved carrier children (n = 35), aged between 8 and 18 years, and their parents (n = 37) who completed a self‐report questionnaire. Children's health‐related quality of life was assessed with a children and parent version of the KIDSCREEN. Dutch reference data were available from a representative national sample. Our results show no statistically significant differences in scores between carrier children and the reference group. Also, no differences were found between carrier children and their parents' ratings, with the exception of the scale “psychological well being”. Parents rated their child's psychological well being significantly lower. We identified no problems with the well‐being of carrier children as compared to a representative sample of peers. This may offer some initial reassurance to those who have concerns about the implications of genetically testing children for one of these cardiovascular conditions. Yet, attention to possible problems in these children remains warranted. © 2008 Wiley‐Liss, Inc.
Fermentative production of l‐glycerol 3‐phosphate utilizing a Saccharomyces cerevisiae strain with an engineered glycerol biosynthetic pathway
Authors: Popp A. , Nguyen H.T.T. , Boulahya K. , Bideaux C. , Alfenore S. , Guillouet S.E. , Nevoigt E.
Published: February 2008

Interest in l‐glycerol 3‐phosphate (l‐G3P) production via microbial fermentation is due to the compound's potential to replace the unstable substrate dihydroxyacetone phosphate (DHAP) in one‐pot enzymatic carbohydrate syntheses. A Saccharomyces cerevisiae strain with deletions in both genes encoding specific l‐G3Pases (GPP1 and GPP2) and multicopy overexpression of l‐glycerol 3‐phosphate dehydrogenase (GPD1) was studied via small‐scale (100 mL) batch fermentations under quasi‐anaerobic conditions. Intracellular accumulation of l‐G3P reached extremely high levels (roughly 200 mM) but thereafter declined. Extracellular l‐G3P was also detected and its concentration continuously increased throughout the fermentation, such that most of the total l‐G3P was found outside the cells as fermentation concluded. Moreover, in spite of the complete elimination of specific l‐G3Pase activity, the strain showed considerable glycerol formation suggesting unspecific dephosphorylation as a mechanism to relieve cells of intracellular l‐G3P accumulation. Up‐scaling the process employed fed‐batch fermentation with repeated glucose feeding, plus an aerobic growth phase followed by an anaerobic product accumulation phase. This produced a final product titer of about 325 mg total l‐G3P per liter of fermentation broth. Biotechnol. Bioeng. 2008;100: 497–505. © 2008 Wiley Periodicals, Inc.
Viral clearance using disposable systems in monoclonal antibody commercial downstream processing
Authors: Zhou Joe X. , Solamo Felix , Hong Tony , Shearer Michael , Tressel Tim
Published: February 2008

Once highly selective protein A affinity is chosen for robust mAb downstream processing, the major role of polishing steps is to remove product related impurities, trace amounts of host cell proteins, DNA/RNA, and potential viral contaminants. Disposable systems can act as powerful options either to replace or in addition to polishing column chromatography to ensure product purity and excellent viral clearance power for patients' safety. In this presentation, the implementation of three disposable systems such as depth filtration, membrane chromatography, and nanometer filtration technology in a commercial process are introduced. The data set of viral clearance with these systems is presented. Application advantages and disadvantages including cost analysis are further discussed. Biotechnol. Bioeng. 2008;100: 488–496. © 2008 Wiley Periodicals, Inc.
Biomaterial‐loaded allograft threaded cage for the treatment of femoral head osteonecrosis in a goat model
Authors: Yang Shuhua , Wu Xinghuo , Mei Rongcheng , Yang Cao , Li Jin , Xu Weihua , Ye Shunan
Published: February 2008

The purpose of this study was to evaluate the efficacy of core decompression with a biomaterial‐loaded allograft threaded cage (ATC) for the treatment of femoral head osteonecrosis in an established goat model. First, bilateral early‐stage osteonecrosis was induced. After core decompression, the remaining goats were randomly divided into three groups: Group A, the goats were left without any treatment; Group B, the goats were treated with implanting a composite of autologous bone and decalcified bone matrix (DBM); Group C, the goats were treated using insertion of ATC loaded with DBM and autogenous bone graft. Then radiographic, histological and biomechanical analysis were taken in each group at 5, 10, and 20 weeks postoperation. In Group A, the classical signs of osteonecrosis of the femoral head were identified 10 weeks after the induction. Twenty weeks later, the density, surface and biomechanical stability of the femoral head were normal in Group C, while an irregular surface and an inhomogeneous microstructure or variation of density/hardness were identified in Group B. The specimens revealed a continuous trabaecular bone structure throughout the cage and extensive bone ingrowth and remodeling in Group C, while fibrous tissue was evident in Group B. Core decompression with a biomaterial loaded ATC almost uniformly delays or arrests the progression of the disease before articular collapse, and it could help to get the balance between bone resorption and new bone formation, strengthen structural mechanics of the femoral head, provide structure support of articular cartilage. Biotechnol. Bioeng. 2008;100: 560–566. © 2008 Wiley Periodicals, Inc.
Impact of target mRNA structure on siRNA silencing efficiency: A large‐scale study
Authors: Gredell Joseph A. , Berger Angela K. , Walton S. Patrick
Published: February 2008

The selection of active siRNAs is generally based on identifying siRNAs with certain sequence and structural properties. However, the efficiency of RNA interference has also been shown to depend on the structure of the target mRNA, primarily through studies using exogenous transcripts with well‐defined secondary structures in the vicinity of the target sequence. While these studies provide a means for examining the impact of target sequence and structure independently, the predicted secondary structures for these transcripts are often not reflective of structures that form in full‐length, native mRNAs where interactions can occur between relatively remote segments of the mRNAs. Here, using a combination of experimental results and analysis of a large dataset, we demonstrate that the accessibility of certain local target structures on the mRNA is an important determinant in the gene silencing ability of siRNAs. siRNAs targeting the enhanced green fluorescent protein were chosen using a minimal siRNA selection algorithm followed by classification based on the predicted minimum free energy structures of the target transcripts. Transfection into HeLa and HepG2 cells revealed that siRNAs targeting regions of the mRNA predicted to have unpaired 5′‐ and 3′‐ends resulted in greater gene silencing than regions predicted to have other types of secondary structure. These results were confirmed by analysis of gene silencing data from previously published siRNAs, which showed that mRNA target regions unpaired at either the 5′‐end or 3′‐end were silenced, on average, ∼10% more strongly than target regions unpaired in the center or primarily paired throughout. We found this effect to be independent of the structure of the siRNA guide strand. Taken together, these results suggest minimal requirements for nucleation of hybridization between the siRNA guide strand and mRNA and that both mRNA and guide strand structure should be considered when choosing candidate siRNAs. Biotechnol. Bioeng. 2008;100: 744–755. © 2008 Wiley Periodicals, Inc.
Increased phenotypic stability and ethanol tolerance of recombinant Escherichia coli KO11 when immobilized in continuous fluidized bed culture
Authors: Zhou Bin , Martin Gregory J.O. , Pamment Neville B.
Published: February 2008

The recombinant Escherichia coli B strain KO11, containing chromosomally‐integrated genes for ethanol production, was developed for use in lignocellulose‐to‐ethanol bioconversion processes but suffers from instability in continuous culture and a low ethanol tolerance compared to yeast. Here we report the ability cell immobilization to improve its phenotypic stability and ethanol tolerance during continuous culture on a 50 g/L xylose feed. Experiments conducted in a vertical tubular fermentor operated as a liquid‐fluidized bed with the cells immobilized on porous glass microspheres were compared to control experiments in the same reactor operated as a chemostat without the support particles. Without cell immobilization the ethanol yield fell sharply following start‐up, declining to 60% of theoretical after only 8–9 days of continuous fermentation. While immobilizing the cells did not prevent this decline, it delayed its onset and slowed its rate. With immobilization, a stable high ethanol yield (>85%) was maintained for at least 10 days, thereafter declining slowly, but remaining above 70% even after up to 40 days of fermentation. The ethanol tolerance of E. coli KO11 cells was substantially increased by immobilization on the glass microspheres. In ethanol tolerance tests, immobilized cells released from the microspheres had survival rates 2.3‐ to 15‐fold higher than those of free cells isolated from the same broth. Immobilization is concluded to be an effective means of increasing ethanol tolerance in E. coli KO11. While immobilization was only partially effective in combating its phenotypic instability, further improvements can be expected following optimization of the immobilization conditions. Biotechnol. Bioeng. 2008;100: 627–633. © 2008 Wiley Periodicals, Inc.
Neural differentiation and potential use of stem cells from the human umbilical cord for central nervous system transplantation therapy
Authors: Low Choon Bing , Liou Yih‐Cherng , Tang Bor Luen
Published: February 2008

The human umbilical cord is a rich source of autologous stem and progenitor cells. Interestingly, subpopulations of these, particularly mesenchymal‐like cells from both cord blood and the cord stroma, exhibited a potential to be differentiated into neuron‐like cells in culture. Umbilical cord blood stem cells have demonstrated efficacy in reducing lesion sizes and enhancing behavioral recovery in animal models of ischemic and traumatic central nervous system (CNS) injury. Recent findings also suggest that neurons derived from cord stroma mesenchymal cells could alleviate movement disorders in hemiparkinsonian animal models. We review here the neurogenic potential of umbilical cord stem cells and discuss possibilities of their exploitation as an alternative to human embryonic stem cells or neural stem cells for transplantation therapy of traumatic CNS injury and neurodegenerative diseases. © 2008 Wiley‐Liss, Inc.
Investigation of Barcelona and Du Chilly filbert nuts. I. Chemical study of Barcelona and Du Chilly filbert nuts and oils
Authors: Fang S. C. , Bullis D. E.
Published: February 2008

Barcelona and Du Chilly fibert nuts have been analyzed for their principal constituents. The characteristics of the oils obtained by solvent extraction and by cold expression have been determined and their fatty acid composition has been ascertained. Most of the chemical and physical characteristics of the oils as determined in this laboratory agree well with values previously reported by other investigators on European varieties. However, our study of the oil components shows a considerable difference in fatty acid composition.
Clarifying co‐rumination: Associations with internalizing symptoms and romantic involvement among adolescent girls
Authors: Starr Lisa R. , Davila Joanne
Published: February 2008

Co‐rumination, or excessive discussion of problems within friendships, has been associated with internalizing symptoms and is especially prevalent among adolescent girls. Eighty‐three early adolescent girls participated in a prospective study further examining this construct. Co‐rumination was positively correlated with depressive symptoms and positive aspects of friendship, but did not predict longitudinal changes in depressive symptoms. Co‐rumination was negatively related to social anxiety when controlling for depressive symptoms. Co‐rumination correlated positively with romantic experiences, and the two interacted to predict longitudinal changes in depressive symptoms, implying that co‐rumination may only be depressogenic under certain circumstances. Theoretical ramifications for the construct of co‐rumination and interpersonal aspects of adolescent internalizing symptoms are discussed.
Book review
Authors: Skoglund Kristofer
Published: February 2008

Heart Failure: Oxford Specialist Handbooks in Cardiology by Roy S Gardner, Theresa A McDonagh, Niki L Walker, ISBN: ‐13: 978–0199205738
Developmental expression of glycine receptor subunits in rat cerebellum
Authors: García‐Alcocer Guadalupe , Mejía Carmen , Berumen Laura C. , Miledi Ricardo , Martínez‐Torres Ataúlfo
Published: February 2008

The distribution of the glycine receptor subunits α1–3 and β in the developing rat cerebellum was studied from postnatal day 1 to adulthood by means of quantitative RT‐PCR and immunohistochemistry. qRT‐PCR of postnatal cerebella indicated the presence of mRNA for each subunit, with a relative expression of α2 > α3 > α1 > β. The immunohistochemistry indicated a strong α2 signal in the Purkinje cells, internal and external granular layers. The α1–3 subunits had weak signals in the Purkinje cells and molecular layer. The α1 subunit was expressed at a low level and was also found in the white matter. The function of these receptors in neuronal and glial plasma membranes in early postnatal development remains to be determined.