bluuebunny/crossref_metadata_2025_split huggingface dataset:
The clinical utility of MRI in patients with neurodevelopmental disorders of unknown origin
Authors: Engbers H. M. , Nievelstein R. A. J. , Gooskens R. H. J. M. , Kroes H. Y. , Van Empelen R. , Braams O. , Wittebol‐Post D. , Hendriks M. M. W. B. , Visser G. 
Published: January 2010

Introduction:  Neuroimaging of the brain in the diagnostic work‐up of patients with neurodevelopmental disorders is a matter of continuing debate. Recommendations range from performing brain imaging in all patients with neurodevelopmental disorders to performing an MRI only in those with indication on clinical examinations. Important indications for neuroimaging are head size abnormalities and focal neurological findings.Methods:  Patients with neurodevelopmental disorders of unknown origin (n = 410), referred to a specialized tertiary diagnostic center for neurodevelopmental disorders were included in a retrospective analysis. A 1‐day work‐up, including an MRI of the brain was performed. Studied were the: (i) yield of MRI scans of the brain and (ii) associations of specific clinical symptoms/signs with abnormal and diagnostic MRI scans.Results:  (i) In 30.7% of the 410 patients with neurodevelopmental disorders (n = 126), abnormal MRI scans were observed, leading to an etiological diagnosis in 5.4% of the patients (n = 22). (ii) Pyramidal disorders (P = 0.001), epilepsy (P = 0.04) and an abnormal head circumference (P = 0.02) were associated with an abnormal MRI scan. The presence of one of the following neurological symptoms/signs: movement disorders, pyramidal disorders, epilepsy, or an abnormal head circumference was associated with a diagnostic MRI scan (P < 0.001) (diagnostic MRI % in neurological versus no neurological symptoms/signs, 13.0% versus 1.9%).Conclusion:  Neuroimaging of the brain in a tertiary care center for patients with neurodevelopmental disorders of unknown origin is useful, especially in case of neurological symptoms/signs.
Rapidly progressive Creutzfeldt–Jakob disease in patients with Familial Mediterranean Fever
Authors: Appel S. A. , Chapman J. , Kahana E. , Rosenmann H. , Prohovnik I. , Pras E. , Reznik‐Wolf H. , Cohen O. S. 
Published: January 2010

Background:  The largest cluster of familial Creutzfeldt–Jakob disease (fCJD) exists in Jews of Libyan origin. Familial Mediterranean fever (FMF) is an inflammatory disease also common in this population.Objectives:  We hypothesized that FMF, as a pro‐inflammatory condition, may affect the course of CJD.Methods:  Three hundred and seventy‐two consecutive patients diagnosed clinically and genetically as CJD were included in the study. Two hundred and thirty‐six had fCJD, and 136 had sporadic disease (sCJD). Review of the patient’s records revealed three patients with FMF–CJD co‐morbidity. In addition, 50 DNA samples of patients with CJD were genotyped as homozygote, heterozygote, and non‐carriers of the FMF mutation. The demographic and clinical variables of the groups were compared.Results:  The three patients with FMF had an earlier age of onset and significantly shorter disease duration than the patients without FMF. Heterozygote carriers did not differ in disease onset and duration from patients without FMF.Conclusions:  The shorter disease duration of CJD patients with FMF may indicate the importance of pro‐inflammatory factors in the disease.
Cerebrospinal fluid hypocretin levels are normal in idiopathic REM sleep behaviour disorder
Authors: Anderson K. N. , Vincent A. , Smith I. E. , Shneerson J. M. 
Published: January 2010

Background:  Rapid eye movement (REM) sleep behaviour disorder (RBD) is a common sleep disorder that can be associated with a number of neurodegenerative conditions as well as with narcolepsy. Current diagnostic criteria require overnight polysomnography, and there are no other biomarkers available. The control of REM sleep is complex with a putative on/off switch within the brainstem activated, amongst other things, by hypocretinergic pathways from the lateral hypothalamus.Methods:  Cerebrospinal fluid hypocretin levels were measured in five patients with idiopathic RBD.Results:  Hypocretin levels were between 254 and 450 pg/ml and therefore within the normal range of >100 pg/ml.Conclusion:  Hypocretin levels in patients with idiopathic RBD are normal.
Mood and motor effects of thalamic deep brain stimulation surgery for essential tremor
Authors: Graff‐Radford J. , Foote K. D. , Mikos A. E. , Bowers D. , Fernandez H. H. , Rosado C. A. , Rodriguez R. L. , Malaty I. A. , Haq I. U. , Jacobson C. E. , Okun M. S. 
Published: January 2010

Purpose:  The aim of this study was to evaluate the effects of unilateral and bilateral ventralis intermedius (Vim) deep brain stimulation (DBS) on mood and motor function.Methods:  Thirty‐one consecutive medication refractory patients with essential tremor who underwent unilateral or bilateral Vim DBS at University of Florida and returned for at least 6 ‐month follow‐up completed the Visual Analog Mood (VAMS), the Beck Depression Inventory (BDI), and the Tremor Rating Scale (TRS) before and after surgery. We excluded all patients who were implanted at other institutions.Results:  The tense subscale of the VAMS improved significantly in both the unilateral and bilateral DBS groups (P < 0.001). On the VAMS afraid subscale, only the bilateral group trended toward improvement (P = 0.075). There were no significant changes for either group for the happy, confused, sad, angry, energetic or tired VAMS scores. TRS subscale scores all improved after unilateral and bilateral Vim DBS surgery (P < 0.001).Conclusions:  Feelings of tenseness, tremor severity and ADLs improved following unilateral or bilateral Vim DBS for ET.
The clandestine uterus: or how the uterus escapes detection prior to puberty
Authors: Michala L , Aslam N , Conway GS , Creighton SM 
Published: January 2010

Please cite this paper as: Michala L, Aslam N, Conway G, Creighton S. The clandestine uterus: or how the uterus escapes detection prior to puberty. BJOG 2010;117:212–215.Uterine agenesis is one of the differential diagnoses in adolescent girls with delayed menstruation. It may also be suspected earlier in childhood during investigations for other genitourinary conditions. However, accurate confirmation that the uterus is absent can be extremely difficult before puberty because of its small size. We describe ten girls referred to a specialist centre with a presumed diagnosis of an absent uterus which was later found to be incorrect. We conclude that imaging should be undertaken by clinicians with experience in management of this age group and in some girls it may be necessary to delay final diagnosis until after puberty.
Reproductive endocrinology of adolescent polycystic ovary syndrome
Authors: Shayya R , Chang RJ 
Published: January 2010

Please cite this paper as: Shayya R, Chang R. Reproductive endocrinology of adolescent polycystic ovary syndrome. BJOG 2010;117:150–155.Polycystic ovary syndrome (PCOS) is the most common endocrinopathy among reproductive‐aged women, and it typically presents during adolescence. The objective of this review is to describe the clinical manifestations of PCOS in adolescent girls and the underlying basis for the altered reproductive physiology. Recognising adolescents at risk for PCOS and taking the appropriate steps to reduce circulating androgen levels is critical in reducing the clinical symptomatology of this disorder, and the development of adulthood infertility, diabetes, and metabolic syndrome in patients with PCOS.
The histology and management of ovarian cysts found in children and adolescents presenting to a children’s hospital from 1991 to 2007: a call for more paediatric gynaecologists
Authors: Hernon M , McKenna J , Busby G , Sanders C , Garden A 
Published: January 2010

Please cite this paper as: Hernon M, McKenna J, Busby G, Sanders C, Garden A. The histology and management of ovarian cysts found in children and adolescents presenting to a children’s hospital from 1991 to 2007: a call for more paediatric gynaecologists. BJOG 2010;117:181–184.Objective  To determine the nature and surgical management of ovarian cysts.Design  Retrospective case‐note study.Setting  Large Childrens’ Hospital: Alder Hey NHS Foundation Trust.Sample  Children undergoing surgery for ovarian cysts between 1991 and 2007.Methods  Cases identified using coding and the pathology database, and analysed using snap 9.Main outcome measures  Extent of surgery performed. Size and histological features of cysts removed.Results  A total of 155 cases were identified. There were 62 ovarian cysts in children under nine who were prepubertal. There were 58 neoplastic cysts in total. Most were benign teratomas (36). Ten cysts were malignant, including five granulosa cell tumours, one yolk sac tumour, one endodermal sinus tumour and one dysgerminoma. Tumour markers were performed in only 16 cases (10%). Sixty‐one (39%) had an ultrasound scan and 16 (10%) had a computed tomography (CT) or magnetic resonance imaging (MRI) scan. Ninety girls (58%) had an oophorectomy and 40 (25%) had an ovarian cystectomy. Oophorectomies were performed for all cases of malignancy, but 75 were also performed for benign or normal pathology. Only 16% of cases were referred to the paediatric gynaecologist and all were for post‐operative management.Conclusions  We recommend the greater use of imaging of the pelvis and tumour markers preoperatively. There should be greater use of conservative expectant management or ovarian‐sparing surgery in view of the low risk of malignancy in this age group. The practice of removing ovaries for benign cysts may be overcome by appointing more gynaecologists with advanced training skills training in paediatric and adolescent gynaecology.
Adenosine inhibits paraventricular pre‐sympathetic neurons through ATP‐dependent potassium channels
Authors: Li De‐Pei , Chen Shao‐Rui , Pan Hui‐Lin 
Published: January 2010

J. Neurochem.(2010)113, 530–542.AbstractAdenosine produces cardiovascular depressor effects in various brain regions. However, the cellular mechanisms underlying these effects remain unclear. The pre‐sympathetic neurons in the hypothalamic paraventricular nucleus (PVN) play an important role in regulating arterial blood pressure and sympathetic outflow through projections to the spinal cord and brainstem. In this study, we performed whole‐cell patch‐clamp recordings on retrogradely labeled PVN neurons projecting to the intermediolateral cell column of the spinal cord in rats. Adenosine (10–100 μM) decreased the firing activity in a concentration‐dependent manner, with a marked hyperpolarization in 12 of 26 neurons tested. Blockade of A1receptors with the adenosine A1receptor antagonist 8‐cyclopentyl‐1,3‐dipropylxanthine or intracellular dialysis of guanosine 5′‐O‐(2‐thodiphosphate) eliminated the inhibitory effect of adenosine on labeled PVN neurons. Immunocytochemical labeling revealed that A1receptors were expressed on spinally projecting PVN neurons. Also, blocking ATP‐dependent K+(KATP) channels with 100 μM glibenclamide or 200 μM tolbutamide, but not the G protein‐coupled inwardly rectifying K+channels blocker tertiapin‐Q, abolished the inhibitory effect of adenosine on the firing activity of PVN neurons. Furthermore, glibenclamide or tolbutamide significantly decreased the adenosine‐induced outward currents in labeled neurons. The reversal potential of adenosine‐induced currents was close to the K+equilibrium potential. In addition, adenosine decreased the frequency of both spontaneous and miniature glutamatergic excitatory post‐synaptic currents and GABAergic inhibitory post‐synaptic currents in labeled neurons, and these effects were also blocked by 8‐cyclopentyl‐1,3‐dipropylxanthine. Collectively, our findings suggest that adenosine inhibits the excitability of PVN pre‐sympathetic neurons through A1receptor‐mediated opening ofKATPchannels.
Metallocorroles as cytoprotective agents against oxidative and nitrative stress in cellular models of neurodegeneration
Authors: Kupershmidt Lana , Okun Zoya , Amit Tamar , Mandel Silvia , Saltsman Irena , Mahammed Atif , Bar‐Am Orit , Gross Zeev , Youdim Moussa B. H. 
Published: January 2010

J. Neurochem.(2010)113, 363–373.AbstractWater‐soluble iron, and manganese(III) complexes of corroles and porphyrins were examined with regard to their neuroprotective/neurorescue activities by using various neuronal cytotoxic models of oxidative and nitrative stress. The present study demonstrates that the metallocorroles significantly protect human neuroblastoma SH‐SY5Y and mouse motor neuron‐neuroblastoma fusion NSC‐34 cell lines against neurotoxicity induced by either the peroxynitrite donor 3‐morpholinosydnonimine or the parkinsonism‐related neurotoxin 6‐hydroxydopamine. The neuronal survival effect is further reflected by the prevention of 3‐morpholinosydnonimine‐induced protein nitration, inhibition of caspase 3 activation, as well as attenuation of 6‐hydroxydopamine‐mediated decrease in growth associated protein‐43 levels. The iron(III) corrole, but not manganese (III) corrole, also significantly promotes neuronal survival of hydrogen peroxide (H2O2)‐impaired SH‐SY5Y and NSC‐34 cells. A substantial superiority of the metallocorroles relative to the corresponding porphyrin complexes is revealed in all examined aspects. These results highlight the large potential of corrole complexes as novel agents for therapeutic approaches in degenerative disorders of the central and peripheral nervous systems, where oxidative and nitrative stresses are involved.
The identification and distribution of the enigmatic Large‐billed Reed WarblerAcrocephalus orinus
Authors: SVENSSON LARS , PRYS‐JONES ROBERT , RASMUSSEN PAMELA C. , OLSSON URBAN 
Published: January 2010

We re‐evaluate the characteristics of the little‐known Large‐billed Reed WarblerAcrocephalus orinusbased on plumage and biometrical comparisons of 10 newly discovered specimens ofA. orinuswith a large sample of Blyth’s Reed WarblerAcrocephalus dumetorum, the species most closely resemblingA. orinus. Using specimens whose identity was verified by DNA analyses, we found that the most important criteria for identifyingorinusare its long and comparatively broad bill, with typically uniformly dark upper mandible, and on average longer tarsi and claws with a thin and pointed hind claw. In contrast, previously suggested differences in wing formula, plumage colour and shape of flight‐feathers are very minor, variable and seem of limited use for identification oforinus. Graphical analysis of hind claw length against bill to skull length should assist researchers in locating museum or live specimens oforinusthat otherwise might be overlooked asdumetorum, and multivariate analysis unambiguously separates the two species based on our present dataset. A review of the scattered literature and specimen records suggests that the breeding distribution oforinuslies between southeastern Kazakhstan and northeastern Afghanistan, within the breeding range ofdumetorum, but thatorinusmay well winter predominantly east of the main wintering range ofdumetorum.Further evidence to support this assessment of the species' breeding range was obtained in June 2009, with the discovery of a population in the Pamir Mountains of Afghanistan.
Does Silence Speak? An Empirical Analysis of Disclosure Choices During Conference Calls
Authors: HOLLANDER STEPHAN , PRONK MAARTEN , ROELOFSEN ERIK 
Published: January 2010

ABSTRACTIn this paper, we exploit the open nature of conference calls to explore whether managers withhold information from the investing public. Our evidence suggests that managers regularly leave participants on the conference call in the dark by not answering their questions. We find that the best predictors of such an event are firm size, a CEO's stock price–based incentives, company age, firm performance, litigation risk, and whether analysts are actively involved during the call's Q&A section. Finally, we document strong support for the assumption maintained in the literature that investors interpret silence negatively. That is, investors seem to interpret no news as bad news.
Kinetic analysis of evoked IPSCs discloses mechanism of antagonism of synaptic GABAAreceptors by picrotoxin
Authors: Korshoej AR , Holm MM , Jensen K , Lambert JDC 
Published: January 2010

Background and purpose: Although picrotoxin is a well‐established antagonist of GABAAreceptors, detailed studies of its action on inhibitory synaptic transmission have not previously been made.Experimental approach: Electrophysiological techniques were used to study the action of picrotoxin on inhibitory postsynaptic currents (IPSCs) evoked in hippocampal neurones, in culture and slice preparations prepared from Wistar rat embryos and juveniles, respectively.Key results: Picrotoxin gradually reduced the amplitude of GABAAreceptor‐mediated eIPSCs in a concentration‐dependent manner. This was accompanied by a marked acceleration of the eIPSC decay kinetics, which, in contrast to the effect on amplitude, developed immediately and was completely reversed on washing. The decaying phase of the IPSC could be resolved into two components; 30 µM picrotoxin reduced τfastby 34% and increased its relative amplitude, while τslowwas reduced by 38%, and its relative amplitude decreased. The area under the decaying phase of the normalized eIPSC showed an immediate reduction by 36% in 30 µM picrotoxin. With increasing concentrations of picrotoxin, this normalized area converged towards 55% of the control, indicating that the rate of relaxation and block has a finite maximum. This implies that picrotoxin does not act by a pore‐occluding mechanism (open‐channel blocking), and suggests allosteric stabilization of desensitized receptor states as a more likely alternative. This was corroborated by modelling, based on two established microscopic GABAAreceptor transition schemes.Conclusions and implications: Although the identity of the stabilized state has not been determined unequivocally, picrotoxin effectively traps synaptic GABAAreceptors in a desensitized state.
Effects of peroxisome proliferator‐activated receptor γ agonists on Na+ transport and activity of the kinase SGK1 in epithelial cells from lung and kidney
Authors: Wilson Stuart M , Mansley Morag K , Getty Jennet , Husband Elaine M , Inglis Sarah K , Hansen Michael K 
Published: January 2010

Background and purpose:  Peroxisome proliferator‐activated receptor γ (PPARγ) agonists, such as rosiglitazone and pioglitazone, sensitize cells to insulin, and are therefore used to treat type 2 diabetes. However, in some patients, these drugs induce oedema, and the present study tests the hypothesis that this side effect reflects serum and glucocorticoid‐inducible kinase 1 (SGK1)‐dependent enhancement of epithelia Na+ absorption.Experimental approach:  Na+ absorbing epithelial cells (H441 cells, mpkCCD cells) on permeable membranes were mounted in Ussing chambers, and the effects of rosiglitazone (2 µM) and pioglitazone (10 µM) on transepithelial Na+ absorption were quantified electrometrically. Changes in SGK1 activity were assessed by monitoring phosphorylation of residues within an endogenous protein.Key results:  Both cell types absorbed Na+ via an electrogenic process that was enhanced by insulin. In mpkCCD cells, this stimulation of Na+ transport was associated with increased activity of SGK1, whereas insulin regulated Na+ transport in H441 cells through a mechanism that did not involve activation of this kinase. Rosiglitazone and pioglitazone had no discernible effect on transepithelial Na+ absorption in unstimulated or insulin‐stimulated cells and failed to alter cellular SGK1 activity.Conclusions and implications:  Our results do not support the view that PPARγ agonists stimulate epithelial Na+ absorption or alter the control of cellular SGK1 activity. It is therefore likely that other mechanisms are involved in PPARγ‐mediated fluid retention, and a better understanding of these mechanisms may help with the identification of patients likely to develop oedema or heart failure when treated with these drugs.
Tetrandrine blocks cardiac hypertrophy by disrupting reactive oxygen species‐dependent ERK1/2 signalling
Authors: Shen Di‐Fei , Tang Qi‐Zhu , Yan Ling , Zhang Yan , Zhu Li‐Hua , Wang Lang , Liu Chen , Bian Zhou‐Yan , Li Hongliang 
Published: January 2010

Background and purpose:  Tetrandrine, a well‐known naturally occurring calcium antagonist with anti‐inflammatory, antioxidant and anti‐fibrogenetic activities, has long been used clinically for treatment of cardiovascular diseases such as hypertension and arrhythmia. However, little is known about the effect of tetrandrine on cardiac hypertrophy. The aims of the present study were to determine whether tetrandrine could attenuate cardiac hypertrophy and to clarify the underlying molecular mechanisms.Experimental approach:  Tetrandrine (50 mg·kg−1·day−1) was administered by oral gavage three times a day for one week and then the mice were subjected to either chronic pressure overload generated by aortic banding (AB) or sham surgery (control group). Cardiac function was determined by echocardiography.Key results:  Tetrandrine attenuated the cardiac hypertrophy induced by AB, as assessed by heart weight/body weight and lung weight/body weight ratios, cardiac dilatation and the expression of genes of hypertrophic markers. Tetrandrine also inhibited fibrosis and attenuated the inflammatory response. The cardioprotective effects of tetrandrine were mediated by blocking the increased production of reactive oxygen species and the activation of ERK1/2‐dependent nuclear factor‐κB and nuclear factor of activated T cells that occur in response to hypertrophic stimuli.Conclusions and implications:  Taken together, our results suggest that tetrandrine can improve cardiac function and prevent the development of cardiac hypertrophy by suppressing the reactive oxygen species‐dependent ERK1/2 signalling pathway.
Why Isn’t More US Farmland Organic?
Authors: Kuminoff Nicolai V. , Wossink Ada 
Published: January 2010

AbstractWe develop a theoretical model to assess the dollar compensation required to induce conventional growers to convert to organic. The model incorporates the uncertainty in producers’ expectations about future returns and about the impact of policy changes on these expectations in particular. We demonstrate that a new policy which favours organic can have opposing effects on the rate of conversion. An increase in relative returns to organic today will increase conversion rates. However, if the future of the policy programme is uncertain, its introduction can increase the value of waiting to switch, which will decrease conversion rates. We then develop an empirical switching regression model that enables direct estimation of the value associated with being able to postpone the conversion decision until some of the uncertainty is resolved. The model is applied to data on organic and conventional soybeans before and after major changes in US farm policy toward organic growers. The results suggest that sunk costs associated with conversion to organic coupled with uncertainty about future returns can help to explain why there is so little organic farmland in the USA.
The Effect of Cooperatives on Quality‐Enhancing Innovation
Authors: Drivas Kyriakos , Giannakas Konstantinos 
Published: January 2010

AbstractThis paper develops game‐theoretic models of heterogeneous consumers to analyse the effect of cooperatives on quality‐enhancing product innovation activity, the pricing of food products and the welfare of the groups involved, in the context of a mixed duopoly where an open‐membership consumer co‐op competes with an investor‐owned firm in markets for horizontally differentiated products. Analytical results show that the involvement of the member welfare‐maximising co‐op in innovation activity can change the nature of product differentiation and the structure of the market, and be quality and welfare enhancing by increasing innovation activity and reducing the prices of food products. The effects of co‐operative involvement are shown to depend on the degree of consumer heterogeneity and the size of innovation costs.