bluuebunny/crossref_metadata_2025_split huggingface dataset:
Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis: Frequency, extent, and specificity
Authors: Kuwabara Satoshi , Sonoo Masahiro , Komori Tetsuo , Shimizu Toshio , Hirashima Fumiko , Inaba Akira , Misawa Sonoko , Hatanaka Yuki ,   Tokyo Metropolitan Neuromuscular Electrodiagnosis Study Group
Published: January 2008

AbstractPrevious studies suggest that in amyotrophic lateral sclerosis (ALS) the abductor pollicis brevis (APB) and first dorsal interosseous (FDI) are more severely involved than abductor digiti minimi (ADM). To elucidate the pattern, frequency, extent, and specificity of such dissociated muscle atrophy in ALS, compound muscle action potentials recorded from APB, FDI, and ADM were analyzed in 77 ALS patients, 171 normal controls, and 196 disease controls. Compared with normal controls, ALS patients had a reduced APB/ADM amplitude ratio (P < 0.001) and FDI/ADM ratio (P < 0.001), whereas patients with other anterior horn diseases showed similar APB/ADM and FDI/ADM ratios to normal values. Decreased APB/ADM ratio was found in 41% of ALS patients, 5% of normal controls, and 4% of disease controls. Prominent muscle atrophy in APB and FDI, with relatively preserved ADM, appears to be specific to ALS. Dissociated hand muscle atrophy presumably reflects part of the pathophysiology and supports the diagnosis of ALS. Muscle Nerve, 2008
Antibodies to myelin‐associated glycoprotein (anti‐Mag) in IgM amyloidosis may influence expression of neuropathy in rare patients
Authors: Garces‐Sanchez Mercedes , Dyck Peter J. , Kyle Robert A. , Zeldenrust Steven , Wu Yanhong , Ladha Shafeeq S. , Klein Christopher J. 
Published: January 2008

AbstractWe have examined whether antibodies to myelin‐associated glycoprotein (anti‐MAG) influence neuropathy occurrence and phenotype in primary (AL IgM) amyloidosis. Anti‐MAG and the cross‐reacted sulfoglucuronyl paragloboside antibodies (SGPG) were studied in 46 patients with IgM amyloidosis (21 with polyneuropathy), and 21 matched IgM MGUS (monoclonal gammopathies of undetermined significance) controls without neuropathy. We assessed the occurrence, phenotype of neuropathy, and attributes of nerve conduction and their relation to antibody activity. Twenty of 46 patients with IgM amyloidosis (7 with and 13 without polyneuropathy) had elevation of anti‐MAG or SGPG by enzyme‐linked immunosorbent assay (ELISA). Two of the polyneuropathy patients with IgM amyloidosis had antibodies to MAG based on Western blot (WB) positivity. One of these patients, with the highest anti‐MAG titer, had a painful sensory ataxia, with prominent demyelination, and amyloid deposition in sural nerve. The other anti‐MAG WB‐positive amyloid patient had an axonal neuropathy and dysautonomia. Low levels of anti‐MAG antibodies were found in 12 of 21 IgM MGUS controls without neuropathy (mean follow‐up, 11 years). We conclude that finding serum anti‐MAG antibodies does not exclude the diagnosis of primary amyloidosis. They do not appear to affect the occurrence or expression of polyneuropathy, except possibly in occasional cases with WB positivity. Muscle Nerve, 2007
Stimulus pulse‐width influences H‐reflex recruitment but not Hmax/Mmax ratio
Authors: Lagerquist Olle , Collins David F. 
Published: January 2008

AbstractIt has been proposed that pulse‐widths of 0.5–1.0 ms should be used to evoke H‐reflexes in humans; however, the influence of pulse‐width on H‐reflex recruitment over a range of stimulus intensities has not been well characterized. We constructed soleus H‐reflex vs. M‐wave recruitment curves using 50, 200, 500, and 1000 μs pulses in 12 subjects. In contrast to previous findings, changing the pulse‐width did not significantly alter maximal H‐reflex (Hmax) or M‐wave (Mmax) amplitudes or Hmax/Mmax ratios. In fact, the 1000 μs pulses resulted in larger H‐reflexes when the M‐wave was 5% Mmax; smaller M‐waves at Hmax; and lower H‐reflex thresholds compared with 50 μs pulses. These differences reflect a leftward shift in the H‐reflex vs. M‐wave recruitment curve when using wide vs. narrow pulses and, combined with no change in the Hmax/Mmax ratios, suggest that factors other than antidromic collision in motor axons limit Hmax. These results support the idea that 1000 μs pulses should be used to evoke H‐reflexes and suggest that wider pulses may be beneficial to generate contractions with a greater reflex contribution when using neuromuscular stimulation for rehabilitation. Muscle Nerve, 2007
Inclusion body myositis functional rating scale: A reliable and valid measure of disease severity
Authors: Jackson C.E. , Barohn R.J. , Gronseth G. , Pandya S. , Herbelin L. ,   The Muscle Study Group (MSG)
Published: January 2008

AbstractWe developed a disease‐specific, 10‐point functional rating scale for patients with inclusion body myositis (IBMFRS). The IBMFRS was utilized as a secondary outcome measure in a multicenter pilot trial of the clinical safety and tolerability of high‐dose beta interferon‐1a. In this trial, 28 IBM patients completed the IBMFRS at baseline and monthly for 6 months. The IBMFRS showed statistically significant correlations (P < 0.001) with maximal voluntary isometric contraction, manual muscle testing, handgrip dynamometry, and the amyotrophic lateral sclerosis (ALS) functional rating scale (ALSPRS). Compared to these other outcome measures, the IBMFRS was also the most sensitive measure of change over the course of the study. Muscle Nerve, 2008
Reliability of quantifying foot and ankle muscle strength in very young children
Authors: Rose Kristy J. , Burns Joshua , Ryan Monique M. , Ouvrier Robert A. , North Kathryn N. 
Published: January 2008

AbstractPreschool‐age children with neuromuscular disorders are often excluded from clinical trials due to the lack of reliable and objective strength measures. We evaluated the reliability of measuring foot and ankle muscle strength in 60 healthy children age 2–4 years. The strength of foot inversion and eversion, ankle plantarflexion, and dorsiflexion was measured using a hand‐held dynamometer. Intrarater and interrater reliability of two assessors was determined for each muscle group using intraclass correlation coefficients (ICCs), 95% confidence intervals (CIs), and standard error of measurement (SEM). For all muscle groups intrarater (ICC2,2 = 0.85‐0.94, 95% CI = 0.75–0.96, SEM = 1.5–4.7 N) and interrater (ICC2,1 = 0.88–0.96, 95% CI = 0.81–0.98, SEM = 1.2–4.6 N) reliability was high for all children. As expected, reliability was generally highest in 3‐ and 4‐year‐old children and lowest in 2‐year‐old children. Hand‐held dynamometry can reliably measure foot and ankle strength in very young children and may help aid in diagnosis and in characterizing disease progression in disorders affecting the foot and ankle. Muscle Nerve, 2008
Gene and protein expression associated with protein synthesis and breakdown in paraplegic skeletal muscle
Authors: Drummond Micah J. , Glynn Erin L. , Lujan Heidi L. , Dicarlo Stephen E. , Rasmussen Blake B. 
Published: January 2008

AbstractSpinal cord injury reduces the rate of skeletal muscle protein synthesis and increases protein breakdown, resulting in rapid muscle loss. The purpose of this study was to determine whether long‐term paraplegia would eventually result in a downregulation of muscle mRNA and protein expression associated with both protein synthesis and breakdown. After 10 weeks of spinal cord transection, soleus muscle from 12 rats (6 sham‐control, 6 paraplegic) was studied for mRNAs and proteins associated with protein synthesis and breakdown using real‐time polymerase chain reaction and immunoblotting techniques. Protein kinase B (PKB/Akt), ribosomal S6 kinase 1 (S6K1), and myogenin mRNA were downregulated, whereas muscle ring finger 1 (MuRF1) and phospho‐forkhead transcription factor 4 (FoxO4) protein were increased in paraplegic rats. We conclude that gene and protein expression of pathways associated with protein synthesis are reduced, whereas some markers of protein breakdown remain elevated following chronic paraplegia. Clinical interventions designed to increase muscle protein synthesis may be helpful in preventing excessive muscle loss during long‐term paraplegia. Muscle Nerve, 2008
An FPTAS for a supply scheduling problem with non‐monotone cost functions
Authors: Ng Chi To , Kovalyov Mikhail Yakovlevich , Cheng Tai Chiu Edwin 
Published: January 2008

AbstractChauhan et al. Oper Res Lett 33 (2005), 249–254 presented a fully polynomial time approximation scheme (FPTAS) for a supply scheduling problem, which is to minimize a total cost associated with the sizes of deliveries from several providers to one manufacturer. The cost functions are assumed non‐decreasing and their arguments are assumed continuously divisible. In this note, we give a motivation for considering the case of non‐monotone cost functions with continuously divisible or discrete arguments. For this more general case, we suggest a modification of the FPTAS in [Chauhan et al., Oper Res Lett 33 (2005), 249–254]. We also suggest a different FPTAS to handle concave cost functions. © 2008 Wiley Periodicals, Inc. Naval Research Logistics, 2008
Analysis of ellagic acid in pomegranate rinds by capillary electrophoresis and high‐performance liquid chromatography
Authors: Zhou Benhong , Wu Zhenhua , Li Xiaojun , Zhang Jie , Hu Xianming 
Published: January 2008

AbstractTwo novel methods for the analysis of ellagic acid in pomegranate (Punica granatum) rinds are proposed. Capillary electrophoresis (CE) was performed in a bare fused‐silica capillary using a buffer solution of tri(hydroxymethyl)aminomethane:potassium dihydrogen phosphate (pH 8.4) with an applied voltage of 20 kV and UV detection at 254 nm. HPLC analysis was performed with a Zobax SB C18 column and a mobile phase consisting of methanol:ethyl acetate:potassium dihydrogen phosphate: phosphoric acid at a flow rate of 1.0 mL/min. Under optimised conditions, the HPLC retention and the CE migration times for ellagic acid were 10.32 and 12.23 min, respectively. Calibration curves of peak area vs. concentration gave correlation coefficients of 0.9999 for HPLC and 0.9990 for CE. The detection limits for HPLC and CE were 2.8 and 2.2 µg/mL, respectively. Average recoveries were 98.32 ± 1.2% for HPLC and 96.52 ± 2.8% for CE. Both methods were shown to be suitable for the determination of ellagic acid in pomegranate rinds extraction; however, the CE method required less solvent and gave better column efficiency, whilst the HPLC provided superior precision. Copyright © 2008 John Wiley & Sons, Ltd.
Birth after pre‐implantation genetic diagnosis (PGD) of spinocerebellar ataxia 2 (Sca2)
Authors: Moutou Céline , Nicod Jean‐Christophe , Gardes Nathalie , Viville Stéphane 
Published: January 2008

AbstractBackgroundSpinocerebellar ataxia 2 (SCA2) is an autosomal‐dominant neurodegenerative disease caused by an extended polyglutamine sequence in the ATXN2 protein. We describe the development of a new single‐cell multiplex PCR protocol for pre‐implantation genetic diagnosis (PGD) of SCA2 and its successful clinical application.MethodsThree duplex tests have been developed, one, which combines the detection of the CAG repeats in addition to the D12S821 microsatellite, another, the amplification of the CAG repeats and the D12S1333 microsatellite and the last, the combination of both microsatellites D12S821 and D12S1333.ResultsPCR conditions were established using 226 single lymphoblasts or patient lymphocysts. Amplification was obtained in an average of 99.6%, a complete genotype in 86%, a conclusive result in 96% and an allelic drop‐out (ADO) rate of 10.7% was observed. PGD for SCA2 was performed for a couple with a paternal risk of transmitting the pathology. Two cycles were done from which 18 embryos were biopsied, 8 were diagnosed as unaffected, 9 as affected and 1 gave no results. In both cycles 2 embryos were transferred, with no pregnancy at the first attempt, and a twin pregnancy at the second attempt. The patient delivered one girl and one boy at 36 weeks and 3 days. Copyright © 2008 John Wiley & Sons, Ltd.
Outcome following prenatal diagnosis of complete atrioventricular septal defect
Authors: Rasiah S. V. , Ewer A. K. , Miller P. , Wright J. G. , Tonks A. , Kilby M. D. 
Published: January 2008

AbstractObjectiveTo identify in a case cohort study, overall outcome following prenatal diagnosis of complete AVSD (cAVSD) in a tertiary referral fetal cardiology center.MethodWe retrospectively reviewed all pregnancies from 1997 to 2004 in which the fetus was identified on ultrasound examination as having a cAVSD.ResultsA prenatal diagnosis of cAVSD was made using fetal echocardiography in 99 fetuses. The median (range) gestational age at diagnosis was 23 weeks (17–37). In 41 cases, cAVSD was the sole cardiac lesion. The remaining 58 fetuses had associated additional intracardiac malformations. Prenatal karyotype was obtained in 43 fetuses and was abnormal in 23. Extracardiac anomalies were also identified in 25 fetuses. Following prenatal counseling, 35 couples chose termination. Of the 64 continuing pregnancies, 12 were stillbirths and 4 were lost to follow‐up. Of the 48 live births, 16 were neonatal deaths without surgery while 32 babies underwent surgery and 19 have survived to date (follow‐up between 2 years 10 months to 9 years 10 months).ConclusionAt the time of prenatal diagnosis at a regional fetal medicine center, the overall survival rate for fetuses with cAVSD is 32% (excluding termination and those lost to follow‐up). This information has important implication for parents of fetuses with cAVSD and when undergoing prenatal counseling. Copyright © 2008 John Wiley & Sons, Ltd.
SAFE—The Special Non‐invasive Advances in Fetal and Neonatal Evaluation Network: aims and achievements
Authors: Chitty Lyn S. , van der Schoot C. Ellen , Hahn Sinuhe , Avent Neil D. 
Published: January 2008

AbstractIn this short review, the objectives and work of SAFE—the Special Non‐invasive Advances in Fetal and Neonatal Evaluation Network, a European Union Framework VI network of excellence is described. We demonstrate how this network facilitates the implementation of non‐invasive prenatal diagnosis (NIPD) for single gene disorders, fetal rhesus typing, aneuploidy and pregnancy complications. Copyright © 2008 John Wiley & Sons, Ltd.
OEIS sequence—a rare congenital anomaly with prenatal evaluation and postnatal outcome in six cases
Authors: Tiblad Eleonor , Wilson R. Douglas , Carr Michael , Flake Alan W. , Hedrick Holly , Johnson Mark P. , Bebbington Michael W. , Mann Stephanie , Adzick N. Scott 
Published: January 2008

AbstractObjectiveTo describe the outcome of children prenatally diagnosed with omphalocele, exstrophy of the cloaca, imperforate anus and spinal defects (OEIS).MethodA retrospective study identified six fetuses diagnosed with OEIS from 2001 to 2007 and data were collected from pre‐ and postnatal charts.ResultsPrenatal findings included an infraumbilical wall defect, omphalocele, no visible bladder and spinal defects. Abnormalities of the upper urinary tract were seen in all cases and ambiguous genitalia in four. Four women continued the pregnancy after prenatal counseling and these fetuses were liveborn at a mean gestational age of 36 weeks. Postnatal evaluation confirmed the prenatal findings in the neonates. The children have required multiple surgeries. Common complications are recurrent urinary tract infections and failure to thrive. Neurodevelopment and motor function are normal in all children, except for one child with lower extremity paralysis and global developmental delay.ConclusionPrenatal diagnosis of OEIS is reliable and no significant differences in pre‐ and postnatal findings in the children were found. Correct diagnosis of abnormalities of external and internal genitalia remains a challenge and abnormalities of the upper urinary tract are common. Postnatal survival is good but associated with considerable morbidity and psychosocial consequences. Copyright © 2008 John Wiley & Sons, Ltd.
When to biopsy a foot ulcer? Seven cases of malignant melanoma presenting as foot ulcers
Authors: Kong M‐F , Jogia R , Jackson S , Quinn M , McNally P , Davies M 
Published: January 2008

AbstractOver the last four years we have diagnosed seven cases of malignant melanoma which were referred to our diabetes foot ulcer clinic. In five of our patients who had diabetes the lesion was initially misdiagnosed as a neuropathic foot ulcer. Acral melanoma is frequently misdiagnosed due to its less common location and because it does not fit the ‘ changing mole’ pattern. It has been mistaken for plantar warts, fungal infections, hyperkeratotic lesions and non‐healing ulcers. Early detection of malignant melanoma greatly enhances the prognosis but primary melanoma of the foot has been associated with a poorer survival rate.In the light of our cases we advocate a high index of suspicion for an alternative aetiology such as melanoma in cases where the ulcer looks atypical and refuses to heal with conventional treatment, if the ulcer is in an unusual anatomical site or when one cannot easily postulate a cause for development of the ulcer for any other reason. If the ulcer looks ‘ suspicious' and atypical, a dermatology opinion should be sought early and a biopsy should be considered. Copyright © 2008 John Wiley & Sons.
Diabetic mastopathy
Authors: Ghosh S , Collier A , Simpson C , Malik I , Milne A 
Published: January 2008

AbstractDiabetic mastopathy can be a source of diagnostic confusion for clinicians. It may present with clinical features similar to breast cancer, and fine needle aspirate, core biopsy and mammography maybe unhelpful in excluding malignant disease. Surgical biopsy reveals characteristic features. As the lesion can recur, follow up is necessary. Copyright © 2008 John Wiley & Sons.
The management of type 2 diabetes care: the challenge within primary care
Authors: Mold F , While A , Forbes A 
Published: January 2008

AbstractManaging type 2 diabetes in primary care poses many challenges for health professionals in preventing and managing complications. These challenges are greatly amplified in those environments with high levels of deprivation and ethnic diversity.A retrospective structured audit of patient electronic medical records, with systematic extraction of clinical data, was undertaken in three general practices in an ethnically diverse outer London primary care trust. The records of 646 patients were analysed.Patients from minority ethnic groups were significantly more likely to have poor glycaemic control (p<0.05), and a higher proportion of patients with poor control were aged under 60 (p<0.05). Asian patients showed much higher levels of overall body mass index (BMI) risk, with all Asian patients (100%, n=85) having a sub‐optimal BMI compared to other patient groups (p<0.001). Younger age (50 years and under) was associated with less frequent contact (p<0.05). Patients managed solely in general practice had significantly better glycaemic control than those in shared care schemes (p<0.01). Feedback from practices found that diabetes support services were limited and, as such, influenced health professionals' ability to provide some on‐going care in terms of dietary advice and insulin initiation.The audit findings suggest that there are some significant relationships between diabetes outcomes, patient characteristics and the care process. It may be possible to develop interventions suitable for metropolitan primary care and which reflect the diversity of population needs. Copyright © 2008 John Wiley & Sons.
Unique proteomic features induced by a potential antiglioma agent, Nordy (dl‐nordihydroguaiaretic acid), in glioma cells
Authors: Bian Xiu‐Wu , Xu Jian‐Ping , Ping Yi‐Fang , Wang Yan , Chen Jian‐Hong , Xu Cheng‐Ping , Wu Yu‐Zhang , Wu Jun , Zhou Xiang‐Dong , Chen Yi‐Sheng , Shi Jing‐Quan , Wang Ji Ming 
Published: January 2008

AbstractNordy is a chirally synthesized compound of a natural lipoxygenase inhibitor nordihydroguaiaretic acid. In this study, we found that Nordy inhibited the growth of human glioma cell lines in vitro and their tumorigenicity in mice. In addition, Nordy promoted differentiation of highly malignant human glioma cells. Investigation into the mechanistic basis of Nordy activities revealed that it altered the pattern of protein expression profiles in tumor cells. By using 2‐DE, we found that in human glioma cell lines, at least six proteins were down‐regulated after Nordy treatment, while four proteins were elevated in the same cells. Among the six down‐regulated proteins, microsequencing with MALDI TOF MS confirmed the identity of five: proliferation‐associated gene A (PAG‐A), alternative splicing factor‐3 (ASF‐3), β‐galactoside binding lectin, eukaryotic translation initiation factor 5A (eIF‐5A), and coffilin‐1 (nonmuscle). Four up‐regulated proteins were GST‐π, glyceraldehyde‐3‐phosphate dehydrogenase, α‐enolase, and cyclophilin. All these proteins have been reported to participate in key cellular functions including proliferation, metabolism, differentiation, apoptosis, and gene transcription. Our results suggest that Nordy may constitute a promising drug lead for the development of novel antitumor agents targeting proteins that control tumor cell function at multiple levels.
Differential expression of proteins in response to the interaction between the pathogen Fusarium graminearum and its host, Hordeum vulgare
Authors: Geddes Jennifer , Eudes François , Laroche André , Selinger L. Brent 
Published: January 2008

AbstractUsing proteomic techniques, a study aimed at isolating and identifying proteins associated with resistance to fusarium head blight (FHB) was conducted on six barley genotypes of varying resistance. At anthesis, barley spikelets were point inoculated with Fusarium graminearum macroconidial suspensions or mock inoculum. In total, 43 acidic protein spots out of 600 were detected 3 days postinoculation to be differentially expressed due to FHB and were identified. Identification of proteins responsive to FHB included those associated with oxidative burst and oxidative stress response, such as malate dehydrogenase and peroxidases, and pathogenesis‐related (PR). An increase in abundance of PR‐3 or PR‐5 could be associated with the resistant genotypes CI4196, Svansota, and Harbin, as well as the intermediate resistant genotype CDC Bold. On the contrary, the susceptible genotype Stander showed a decrease in abundance of these acidic PR‐proteins. In the susceptible and intermediate resistant genotypes Stander and CDC Bold, as well as CI4196, the increased abundance of proteins associated with an oxidative response might have prepared the terrain for saprophytic fungal invasion. On the contrary, in the resistant sources Harbin and Svansota we did not observed change in abundance of these proteins. Not a single significant change in acidic protein abundance could be detected in Chevron. Three distinct response patterns are reported from these six barley genotypes.