bluuebunny/crossref_metadata_2025_split huggingface dataset:
Noninvasive model of sciatic nerve conduction in healthy and septic mice: Reliability and normative data
Authors: Osuchowski Marcin F. , Teener James , Remick Daniel 
Published: July 2009

AbstractNeuromuscular disorders frequently complicate sepsis and other critical illnesses in patients. Mice are the major species used as a model for sepsis. Nerve conduction studies (NCS), the primary tool for noninvasive assessment of nerve and muscle function, is challenging to perform in small animals. A reliable method for noninvasive, repeated NCS testing has not been reported in mice. We developed and validated a method for the repeated measurement of mouse sciatic nerve conduction in normal and septic mice. Our sedated and awake NCS system enabled minimally invasive long‐term repeated measurements. The mean compound muscle action potential (CMAP) amplitude and latency were 17.4 mV and 1.11 ms, respectively (n = 59). There was an excellent intertester reproducibility by linear regression in both normal (r = 0.95) and septic (r = 0.98) mice. We also showed significant, time‐dependent isoflurane‐induced CMAP suppression in all animals, which was further exacerbated in septic mice. This study provides a new tool for the assessment of peripheral nerve/muscle function in mouse neuromuscular disease models that require repeated, long‐term, and minimally invasive monitoring. Muscle Nerve, 2009
SOD1 (A4V)‐mediated ALS presenting with lower motor neuron facial diplegia and unilateral vocal cord paralysis
Authors: Salameh Johnny S. , Atassi Nazem , David William S. 
Published: July 2009

AbstractAmyotrophic lateral sclerosis (ALS) is a progressive degenerative neuromuscular disease that presents with upper and lower motor neuron signs. Although the majority of ALS cases are sporadic, 10% are familial, of which 20%–25% result from mutations in the superoxide dismutase (SOD1) gene. We describe a novel case of SOD1 (A4V)‐mediated ALS that presented with lower motor neuron facial diplegia and unilateral vocal cord paralysis. This case expands the phenotypic expression of the A4V mutation. Muscle Nerve, 2009
Sensorimotor neuronopathy in ataxia with oculomotor apraxia type 2
Authors: Gazulla José , Benavente Isabel , López‐Fraile Isabel Pérez , Modrego Pedro , Koenig Michel 
Published: July 2009

AbstractTwo siblings with ataxia with oculomotor apraxia type 2 (AOA2) exhibited electrophysiological findings suggestive of a sensorimotor neuronopathy, and primary ovarian failure was detected in one of them. Genetic analysis disclosed a novel, homozygous frameshift mutation in the senataxin gene, 2755_2756delGT, responsible for a premature stop codon at position 2760. It is suggested that a neuronopathy might cause the neuromuscular disturbance in AOA2, and that ovarian failure should be looked for in female patients with the disease. Muscle Nerve, 2009
Masseter reflex in the study of spinocerebellar ataxia type 2 and type 3
Authors: García Antonio , Alvarez Silvia , Infante Jon , Berciano José 
Published: July 2009

AbstractIn this investigation we assess the utility of the masseter reflex for diagnostic purposes in autosomal dominant cerebellar ataxias. We studied the masseter reflex electrophysiologically in spinocerebellar ataxia type 2 (SCA2, 10 patients) and type 3 (SCA3/MJD, 13 patients). In SCA2, the masseter reflex was abnormal in 9 (90%) patients. In SCA3/MJD, the masseter reflex was normal in all 13 patients. Our findings suggest that the masseter reflex is a reliable test that provides additional data prior to molecular study in the differentiation between SCA2 and SCA3/MJD. Masseter reflex abnormalities in SCA2 patients could be better explained by dysfunction of the mesencephalic trigeminal nucleus/tract. Muscle Nerve, 2009
Optimizing acquisition time in quantitative electromyography
Authors: Brownell Alexander A. , Bromberg Mark B. 
Published: July 2009

AbstractQuantitative electromyography (QEMG) relies on a number of discharges of the same motor unit action potential (MUAP) from a train to create an averaged MUAP considered to be representative of a true potential. The train of potentials may be affected by changes in position of the electrode relative to contributing muscle fibers of the motor unit due to operator or subject movement. The effect of changes in electrode position, along with consideration for patient comfort, prompted this study to determine the shortest duration of recording time necessary for sufficient data acquisition for QEMG studies. We determined that 10 seconds of moderate muscle activity is the most reasonable acquisition duration to isolate up to 6 MUAPs at a given electrode site and minimize the effects of movement artifact in the signal. Muscle Nerve, 2009
Clinical and genetic analysis of Korean patients with congenital insensitivity to pain with anhidrosis
Authors: Lee Seung‐Tae , Lee Jeehun , Lee Munhyang , Kim Jong‐Won , Ki Chang‐Seok 
Published: July 2009

AbstractCongenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disease characterized by anhidrosis, insensitivity to noxious stimuli, and mental retardation. Mutations in the NTRK1 gene are associated with the pathogenesis of CIPA. In this study, we performed a clinical and genetic analysis on the NTRK1 gene in four Korean patients with CIPA. All patients had typical clinical manifestations of CIPA, including anhidrosis, recurrent fever, absent pain perception, and developmental delay. Sequencing analysis revealed one predominant mutation, c.851‐33T>A, in four affected alleles and three novel mutations, including c.287+2dupT, c.2155G>A (p.Glu719Lys), and c.1218delC (p.Pro407ArgfsX), in each affected allele. For one patient, who was heterozygous for c.851‐33T>A, another mutation could not be identified, suggesting that a possible hidden intronic or large genomic mutation may have been present. This study extends the spectrum of mutations in the NTRK1 gene and confirms that Korean patients with CIPA have the same genetic background as other ethnicities. Muscle Nerve, 2009
Phenotypic diversity associated with the mitochondrial m.8313G>A point mutation
Authors: O'Rourke Killian , Buddles Mark R. , Farrell Michael , Howley Rachel , Sukuraman Sunita , Connolly Sean , Turnbull Douglass M. , Hutchinson Michael , Taylor Robert W. 
Published: July 2009

AbstractWe report the clinical, histochemical, and molecular genetic findings in a patient with progressive mitochondrial cytopathy due to the m.8313G>A point mutation in the mitochondrial tRNALys (MTTK) gene. The clinical features in this case are severe, including short stature, myopathy, peripheral neuropathy, and osteoporosis, while extensive analysis of maternal relatives indicate that the mutation has arisen de novo and was not maternally inherited. This report of a second case, together with single muscle fiber mutation analysis that shows clear segregation of mutation load with cytochrome c oxidase deficiency, confirms that the mutation is pathologic. Muscle Nerve, 2009
Autophagy, lithium, and amyotrophic lateral sclerosis
Authors: Pasquali Livia , Longone Patrizia , Isidoro Ciro , Ruggieri Stefano , Paparelli Antonio , Fornai Francesco 
Published: July 2009

AbstractIn this article we provide an overview of the intersection between amyotrophic lateral sclerosis (ALS) and the autophagy pathway and discuss the potential protective effects of lithium through mechanisms that recruit autophagy and other effects. The autophagy pathway is recruited during motor neuron (MN) death both in vitro and in vivo. Despite a few controversial issues concerning the significance (detrimental/protective) of autophagy in ALS, recent findings indicate a protective role. Lithium in low doses is a well‐known autophagy inducer that clears misfolded proteins and altered mitochondria from MNs. Moreover, lithium preserves mitochondria and sustains their genesis. This effect is replicated by rapamycin, which is an autophagy inducer but with a different mechanism from lithium. Lithium also increases the number of Renshaw cells that are affected early during the progression of experimental ALS. Again, lithium has been reported to decrease glial proliferation in the ALS spinal cord and induces sprouting in corticospinal fibers. Muscle Nerve 40: 173–194, 2009
Genome‐based expression profiling study following spinal cord injury in the rat: An array of 48‐gene model
Authors: Tseng Ling‐Hong , Chen Ilene , Lin Yi‐Hao , Liang Ching Chung , Lloyd L. Keith 
Published: July 2009

AbstractAimTo explore the potential molecular mechanisms underlying experimental neurogenic bladder dysfunction.MethodsWith the aid of Affymetrix GeneChip Rat Genome U34A arrays, we examined microarray gene expression profiles in bladder wall tissue from female Sprague–Dawley rats within the first 3 weeks following spinal cord injury. Gene transcripts expressed in rat bladder wall tissue at 3 days, 7 days, and 3 weeks following spinal cord injury were compared to normal rat bladder wall tissue.ResultsThe Mahalanobis distance in hierarchical cluster analysis revealed a 48‐gene model, which contained high expressions in rat bladder wall tissue at 3 days, 7 days, and 3 weeks following spinal cord injury. According to gene ontology, plausible molecular alterations in rat bladder wall tissue following spinal cord injury include: (1) the release of nerve growth factor (NGF) and transforming growth factor beta 1 (Tgfb1) (2) the secretion of histamine from mast cells, (3) the occurrence of blood coagulation, (4) the occurrence of N‐terminal protein myristoylation, and (5) Axon guidance mediated by Ena/Vasodilator‐stimulated phosphoprotein (Ena/VASP) promotes reestablishment of the bladder reflex following spinal cord injury. Such changes, jointly termed “bladder remodeling,” can constitute an important long‐term consequence of neurogenic bladder dysfunction.ConclusionThe success of this innovation has supported the use of microarray‐based expression profiling as a commonplace platform for the pathogenesis and therapeutic interventions of experimental neurogenic bladder dysfunction. dysfunction. Neurourol. Urodynam. 29:1439–1443, 2010. © 2009 Wiley‐Liss, Inc.
Limited value of bladder sensation as a trigger for conditional neurostimulation in spinal cord injury patients
Authors: Martens F.M.J. , van Kuppevelt H.J.M. , Beekman J.A.C. , Rijkhoff N.J.M. , Heesakkers J.P.F.A. 
Published: July 2009

AbstractAimsConditional stimulation of dorsal genital nerves suppresses undesired detrusor contractions (UDC) and consequently increases bladder capacity and prevents incontinence. No clinically applicable sensor exists for reliable bladder activity monitoring as a trigger for conditional stimulation. Primary objective of this study was to determine whether bladder sensation concomitant with UDC may be used for spinal cord injury (SCI) patients to trigger neurostimulation in daily life.MethodsNineteen male and 7 female SCI patients suspected of detrusor overactivity (DO) underwent conventional and 6‐hr ambulatory urodynamics. Patients were instructed to do normal daily activities and to activate event buttons of the ambulatory recorder to mark events: physical activity, bladder sensation, micturition or intermittent catheterization, and urinary incontinence. Detection rate was defined as the number of recorded bladder sensation divided by the total number of recorded UDC during ambulatory urodynamics.ResultsBladder sensation was reported by 73% of patients in daily life. Only 41% of patients had analyzable bladder sensation concomitant with UDC during ambulatory urodynamics. For ambulatory and conventional urodynamics, mean detection rates were 23% and 72%, respectively, with mean recording delays of 57 and 16 sec after UDC onset, respectively.ConclusionsBladder sensation only occurs in a small group of SCI patients combined with a rather low detection rate and long reaction time. Therefore, bladder sensation as a trigger for conditional stimulation does not seem to be suitable for SCI patients with DO. Reliable techniques for chronic bladder activity monitoring are a prerequisite for successful clinical application of conditional stimulation. Neurourol. Urodynam. 29:395–400, 2010. © 2009 Wiley‐Liss, Inc.
Stimulation of skin menthol receptors stimulates detrusor activity in conscious rats
Authors: Chen Zhong , Ishizuka Osamu , Imamura Tetsuya , Aizawa Naoki , Kurizaki Yoshiki , Igawa Yasuhiko , Nishizawa Osamu , Andersson Karl‐Erik 
Published: July 2009

AbstractAimsThe Transient Receptor Potential Melastatin (TRPM)8 channel, activated by menthol and cool temperatures, has been proposed to be a cool receptor involved in mediation of the bladder response in the diagnostic ice water test. We examined the expression of TRPM8 in the rat skin and investigated if stimulation of skin menthol receptors by menthol can induce detrusor activity in conscious rats.MethodsImmunofluorescence staining for TRPM8 was performed in samples of the skin from the leg and back of the rats. Continuous cystometry was performed in conscious animals. Voiding interval (VI), micturition volume (MV), and bladder capacity (BC) were evaluated before and after spraying menthol solution to the skin of the leg and back of the rats.ResultsImmunohistochemical analysis revealed that TRPM8 was expressed in the investigated tissues. The expression of TRPM8‐positive receptors was not significantly different between the leg and back skin. Saline caused no significant change in cystometric parameters. After spraying menthol solution (50% and 99% to the skin of the leg, and 99% to the back skin), VI, MV, and BC decreased significantly.ConclusionsTRPM8 is expressed in rat skin. Spraying menthol solution onto the skin‐induced detrusor activity, an effect we suggest is mediated by stimulation of TRPM8 receptors. Neurourol. Urodynam. 29:506–511, 2010. © 2009 Wiley‐Liss, Inc.
Regulatory reform and governance: A survey of selected developing and transition economies
Authors: Zhang Yin‐Fang , Thomas Margo 
Published: July 2009

AbstractRegulation is now considered an integral instrument in developing policy toolkit to support market‐led, pro‐poor growth in developing and transition economies. Institutional environment in general and regulatory governance in particular have increasingly been viewed as a factor of competitiveness. In search for better governance, regulatory reform is critical. This article assesses regulatory reform in selected developing and transition economies by reporting the results of a survey on the application of regulatory governance policies, tools and institutions. It is found that in these countries regulatory reform has not shifted in approaches and objectives to taking a systematic view of regulatory governance and the means of promoting and enhancing it. It is suggested that, in order to improve regulatory governance, focus should be put on each of the three elements: regulatory policies, tools and institutions, and that centralised and concerted efforts are needed to integrate the elements. Copyright © 2009 John Wiley & Sons, Ltd.
Pulmonary tuberculosis presenting as fever without source in a pediatric patient with acute lymphoblastic leukemia
Authors: Lancioni Christina , LaBeaud A. Desiree , Esper Frank , Abughali Nazha , Auletta Jeffery 
Published: July 2009

AbstractChildren who undergo treatment for malignancies are at high for infection with both typical and opportunistic pathogens. Fever in these children prompts extensive evaluation and empiric treatment with broad‐spectrum antimicrobials. In the United States (US), tuberculosis is an infrequently reported cause of fever in the pediatric cancer patient and has not been well described. In this report we describe a case of primary pulmonary tuberculosis (TB) in a boy with precursor B‐cell acute lymphoblastic leukemia (ALL) and review the pertinent literature. Pediatr Blood Cancer 2009; 53:1318–1320. © 2009 Wiley‐Liss, Inc.
Acute malnutrition is common in Malawian patients with a Wilms tumour: A role for peanut butter
Authors: Israëls Trijn , Borgstein Eric , Jamali Monica , de Kraker Jan , Caron Huib N. , Molyneux Elizabeth M. 
Published: July 2009

AbstractBackgroundChildren with cancer in resource limited countries are often malnourished at diagnosis. Acute malnutrition is associated with more infectious complications and an increased risk of morbidity and mortality in major surgery.MethodsAll new patients with the clinical diagnosis of a Wilms tumour admitted in the Queen Elizabeth Central Hospital, Blantyre, Malawi from January 2007 until June 2008 were included. We documented anthropometric parameters, tumour size and serum levels of micronutrients at diagnosis. Corrected weight (body weight − tumour weight) was repeated after 4 weeks of preoperative chemotherapy. During therapy oral feeds were encouraged and a locally made ready to use therapeutic peanut butter‐based food (chiponde) supplied.ResultsA high rate of acute malnutrition was found in patients with Wilms tumour at diagnosis (45–55%), much higher than in community controls (11%). Patients (40%) and community controls (37%) had a similar, high rate of stunting (low height for age), a sign of chronic malnutrition. Tumour size at diagnosis and the degree of acute malnutrition at diagnosis was correlated; patients with a larger tumour had more severe acute malnutrition (r = −0.88, P < 0.01). With a supply of chiponde, 7 of 18 patients had a >5% increase in corrected weight during preoperative chemotherapy. Patients with a more positive nutritional course had a better tumour response to chemotherapy (r = 0.52, P < 0.05). Surprisingly, few micronutrient deficiencies were found, except for low serum levels of vitamin A (44% of patients).ConclusionAcute malnutrition, superimposed on chronic malnutrition, is common in patients with Wilms tumour in Malawi. Earlier presentation needs to be encouraged. Chiponde, a peanut butter based ready‐to‐use‐therapeutic‐food, is an attractive means of nutritional support which needs further study. Pediatr Blood Cancer 2009; 53:1221–1226. © 2009 Wiley‐Liss, Inc.
Magnetic resonance imaging assessment of cardiac and liver iron load in transfusion dependent patients
Authors: Leung Alex Wing Kwan , Chu Winnie Chiu Wing , Lam Wynnie Wai Man , Lee Vincent , Li Chi Kong 
Published: July 2009

AbstractBackgroundMagnetic resonance imaging (MRI) is an emerging tool to assess organ‐specific iron load in patients with transfusion dependent anemia.ObjectiveWe performed MRI T2 star (T2*) assessment in 44 transfusion dependent patients to study the prevalence of cardiac and liver iron overload and the relationship of T2* measurement with various clinical and biochemical parameters.ResultMean age of the study subjects was 19.9 years (range 8.8–32.3) and the mean cardiac T2* was 23.4 ± 13.8 msec. Fifty percent of the subjects had abnormal cardiac T2* (below 20 msec). Cardiac T2* was not found to have any correlation with serum ferritin or liver T2*. Liver T2* value was abnormal in 79% of the subjects and it correlated inversely with both current and 12 months average serum ferritin (r = −0.44, P = 0.003; r = −0.46, P = 0.002). Clinical parameters including age, duration of transfusion, age starting iron chelation therapy, and ratio between transfusion volume and desferrioxamine dosage were not correlated with cardiac and liver T2*.ConclusionWe conclude that iron overload in heart and liver is common in our transfusion dependent patients. Liver T2* has inverse correlation with serum ferritin. Cardiac T2* does not have any correlation with the various clinical and biochemical parameters. Pediatr Blood Cancer 2009;53:1054–1059. © 2009 Wiley‐Liss, Inc.
Neuroblastoma involvement of the falx cerebri
Authors: Quackenbush Katherine Elizabeth , Luna‐Fineman Sandra , Magee James Fergall , Gundogan Munire , Golobi Mahin , Irie Takeshi , Fernandez Conrad Vincent 
Published: July 2009

AbstractInvolvement of the falx cerebri in infants with stage 4 neuroblastoma is thought to be rare. The falx is derived from the neural crest and thus may be a location for primary neuroblastoma. Its propensity for metastasis is unknown. Management of neuroblastoma in this location is potentially challenging. We describe two children less than 18 months of age who were successfully managed with chemotherapy alone (without radiation or surgery) for falx involvement with neuroblastoma. Pediatr Blood Cancer 2009; 53:1337–1339. © 2009 Wiley‐Liss, Inc.
Microwave‐assisted water extraction of green tea polyphenols
Authors: Nkhili Ezzohra , Tomao Valerie , El Hajji Hakima , El Boustani Es‐Seddik , Chemat Farid , Dangles Olivier 
Published: July 2009

AbstractIntroduction – Green tea, a popular drink with beneficial health properties, is a rich source of specific flavanols (polyphenols). There is a special interest in the water extraction of green tea polyphenols since the composition of the corresponding extracts is expected to reflect the one of green tea infusions consumed worldwide.Objective – To develop a microwave‐assisted water extraction (MWE) of green tea polyphenols.Methodology – MWE of green tea polyphenols has been investigated as an alternative to water extraction under conventional heating (CWE). The experimental conditions were selected after consideration of both temperature and extraction time. The efficiency and selectivity of the process were determined in terms of extraction time, total phenolic content, chemical composition (HPLC‐MS analysis) and antioxidant activity of the extracts.Results – By MWE (80°C, 30 min), the flavanol content of the extract reached 97.46 (± 0.08) mg of catechin equivalent/g of green tea extract, vs. only 83.06 (± 0.08) by CWE (80°C, 45 min). In particular, the concentration of the most bioactive flavanol EGCG was 77.14 (± 0.26) mg of catechin equivalent/g of green tea extract obtained by MWE, vs 64.18 (± 0.26) mg/g by CWE.Conclusion – MWE appears more efficient than CWE at both 80 and 100°C, particularly for the extraction of flavanols and hydroxycinnamic acids. Although MWE at 100°C typically affords higher yields in total phenols, MWE at 80°C appears more convenient for the extraction of the green tea‐specific and chemically sensitive flavanols. Copyright © 2009 John Wiley & Sons, Ltd.