bluuebunny/crossref_metadata_2025_split huggingface dataset:
Strain and wafer curvature of 3C‐SiC films on silicon: influence of the growth conditions
Authors: Zielinski M. , Ndiaye S. , Chassagne T. , Juillaguet S. , Lewandowska R. , Portail M. , Leycuras A. , Camassel J. 
Published: March 2007

AbstractWe study the influence of the growth conditions on the residual strain and related optical and structural properties in the case of 3C‐SiC films grown on (001) silicon substrates. We show that two possible mechanisms compete to manage the final sample bow: one is by controlling the composition of the gaseous phase (C/Si ratio) the other one by adjusting the growth temperature and duration (creep effect). In both cases, we compare the low temperature photoluminescence spectra of samples grown under tensile or compressive final stress. We show that better results can be obtained when using the creep effect. (© 2007 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Transparent ohmic contacts to GaSb/In(Al)GaAsSb photovoltaic cells
Authors: Golaszewska K. , Kamińska E. , Piotrowska A. , Rutkowski J. , Kruszka R. , Kowalczyk E. , Papis E. , Wawro A. , Piotrowski T. T. 
Published: March 2007

AbstractIn this paper we present the results of study of thin oxide films: CdO, ZnO and RuSiO4 used as transparent ohmic contacts to GaSb/InGaAsSb/AlGaAsSb photodiodes. Thin oxide films with thickness of 50 nm were deposited by magnetron sputtering. CdO and RuSiO4 were formed in a reactive process in Ar–O2 atmosphere, from Cd and Ru1Si1 targets, respectively. ZnO films were deposited directly from ZnO target by rf sputtering. We have shown that application of CdO, ZnO and RuSiO4 transparent films instead of conventional metal‐based contacts enables to improve of photodiode properties. As a result, GaSb/InGaAsSb/AlGaAsSb photodiodes with detectivity D * increased by factor of 2 and reduced by factor of 3 the series resistance were obtained. (© 2007 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Islet Vasculature as a Regulator of Endocrine Pancreas Function
Authors: Ballian Nikiforos , Brunicardi F. Charles 
Published: March 2007

AbstractThe islets of Langerhans consist of endocrine cells embedded in a network of specialized capillaries that regulate islet blood flow. Despite evidence for a critical role of islet perfusion in endocrine pancreas function, there is information to support no fewer than three models of endocrine cell perfusion, emphasizing the lack of a universally accepted physiological theory. Islet blood flow is regulated by signals, such as hormones and nutrients that reach the islet vasculature from distant tissues via the bloodstream. In addition, islet perfusion determines communication between endocrine and exocrine cells and between different types of endocrine cells within islets. Interest in islet microcirculation has increased after improvements in islet transplantation, a therapy for diabetes mellitus that requires revascularization of grafted islets in a new host organ. Abnormal revascularization is thought to be partly responsible for differences in graft and native islet function. Similarly, angiogenesis has been shown to be a critical step in the transformation of islet hyperplasia to neoplasia.
Central Lymph Node Dissection in Differentiated Thyroid Cancer
Authors: White Matthew L. , Gauger Paul G. , Doherty Gerard M. 
Published: March 2007

AbstractBackgroundThere has been renewed interest in extensive lymph node dissection for papillary thyroid cancer (PTC), and a number of reports have been published concerning compartment‐oriented dissection of regional lymph nodes in PTC. A comprehensive review of this body of literature using evidence‐based methodology is pending.MethodsSystematic review of the literature using evidence‐based criteria.ResultsIssue 1: Systematic compartment‐oriented central lymph node dissection (CLND) may decrease recurrence of PTC (Levels IV and V data, no recommendation) and likely improves disease‐specific survival (grade C recommendation). Limited level III data suggest survival benefit with the addition of prophylactic dissection to thyroidectomy (grade C recommendation). The addition of CLND to total thyroidectomy can significantly reduce levels of serum thyroglobulin and increase rates of athyroglobulinemia (level IV data, no recommendation). Issue 2: There may be a higher rate of permanent hypoparathyroidism and unintentional permanent nerve injury when CLND is performed with total thyroidectomy than for total thyroidectomy alone (grade C recommendation). Issue 3: Reoperation in the central neck compartment for recurrent PTC may increase the risk of hypoparathyroidism and unintentional nerve injury when compared with total thyroidectomy with or without CLND (grade C recommendation), supporting a more aggressive initial operation.ConclusionEvidence‐based recommendations support CLND for PTC in patients under the care of experienced endocrine surgeons.
Genetics of focal segmental glomerulosclerosis
Authors: Woroniecki Robert P. , Kopp Jeffrey B. 
Published: March 2007

AbstractThe recent advances in understanding the pathophysiology of focal segmental glomerulosclerosis (FSGS) and molecular function of glomerular filtration barrier come directly from genetic linkage and positional cloning studies. The exact role and function of the newly discovered genes and proteins are being investigated by in vitro and in vivo mechanistic studies. Those genes and proteins interactions seem to change susceptibility to kidney disease progression. Better understanding of their exact role in the development of FSGS may influence future therapies and outcomes in this complex disease.
International Morquio A Registry: Clinical manifestation and natural course of Morquio A disease
Authors: Montaño A. M. , Tomatsu S. , Gottesman G. S. , Smith M. , Orii T. 
Published: March 2007

SummaryMucopolysaccharidosis IVA (MPS IVA; Morquio A disease) is a lysosomal storage disorder caused by deficiency of N‐acetylgalactosamine‐6‐sulfate sulfatase. The natural history of this disease is incompletely understood. To study which variables influence the clinical outcome, we conducted a study in which MPS IVA patients were asked to fill out a questionnaire with inquiries regarding family history, diagnosis, signs and symptoms, height, weight, surgical history, physical activity, and general complaints. A total of 326 patients (172 male, 154 female) from 42 countries enrolled in the Morquio A Registry programme. The mean age of patients enrolled was 14.9 years for males and 19.1 years for females, with a wide range of 1–73 years. Sixty‐four per cent of the patients were under 18 years. Initial symptoms were recognized between 1 and 3 years of age (mean age 2.1 years) and mean age at diagnosis for the patients was 4.7 years. A progressive skeletal dysplasia was commonly observed among the MPS IVA patients. Fifty per cent of patients underwent surgical operations to improve their quality of life. The most frequent surgical sites include neck (51%), ear (33%), leg (26%) and hip (25%). The birth length for affected males and females was 52.2 ± 4.7 cm and 52.2 ± 4.5 cm, respectively. The final adult height for affected males and females was 122.5 ± 22.5 cm and 116.5 ± 20.5 cm, respectively. The results of this study provide a reference for assessment of efficacy for studies of novel therapies.
Enzyme, cell and gene‐based therapies for metachromatic leukodystrophy
Authors: Sevin C. , Aubourg P. , Cartier N. 
Published: March 2007

SummaryMetachromatic leukodystrophy (MLD) is a demyelinating storage disease caused by deficiency of the lysosomal enzyme arylsulfatase A (ARSA). Lack of ARSA activity leads to the accumulation of galactosylceramide‐3‐O‐sulfate (sulfatide) in the central and peripheral nervous systems. Based on the age at onset, the disease is usually classified into three forms: the late‐infantile form, which manifests in the second year of life; the juvenile variants (onset between 4 and 12 years), which are subdivided into early‐juvenile (EJ, onset before 6 years) and late‐juvenile (LJ, onset after 6 years); and the adult form (onset after 12 years of age). Currently, there is no efficient therapy for the late‐infantile form of MLD (50% of the patients), death occurring within a few years after onset of neurological symptoms. Allogeneic haematopoietic cell transplantation (HCT), when performed at a very early stage of the disease, may improve selected patients with juvenile or adult forms of MLD. As with other lysosomal storage diseases, the physiopathology of MLD is poorly understood. Demyelination is the main pathological finding, but substantial storage of sulfatides in neurons also occurs, and may contribute to the clinical phenotype. The physiopathological process leading to neuronal and glial cell degeneration and apoptosis involves accumulation of undegraded sulfatides but also secondary abnormalities (storage/mislocalization of unrelated lipids, inflammatory processes). This review summarizes the recent advances in the understanding of the physiopathology of MLD and the new therapeutic perspectives currently under preclinical investigation, including enzyme replacement therapy, gene therapy and cell therapy.
Phenylketonuria: Dietary and therapeutic challenges
Authors: Giovannini M. , Verduci E. , Salvatici E. , Fiori L. , Riva E. 
Published: March 2007

SummaryPKU subjects need special attention in the definition of optimal supplementation of nutrients, which may be insufficient in relation to the type of diet and may otherwise manifest symptoms of deficit. In particular, it is necessary to pay great attention to the long‐chain polyunsaturated fatty acid (LC‐PUFA) levels in relation to correct development of the central nervous system. On the basis of numerous beneficial effects currently known, a permanent supplementation with LC‐PUFAs, in particular with docosahexaenoic acid, should be considered. Moreover, new formulas, Phe‐free peptides, and ‘modulated’ amino acid preparations might help in preventing nutritional deficiencies and imbalances, with the ultimate aim of improving growth. New strategies—such as supply of tetrahydrobiopterin—need to be optimized in terms of targets, patients and expected outcomes.
Diagnosis of families with familial hypercholesterolaemia and/or Apo B‐100 defect by means of DNA analysis of LDL‐receptor gene mutations
Authors: Widhalm K. , Dirisamer A. , Lindemayr A. , Kostner G. 
Published: March 2007

SummaryBackground:One major problem of using hypercholesterolaemia alone as a primary criterion for diagnosing familial hypercholesterolaemia (FH) is that 15–40% of relatives may be misdiagnosed because plasma lipid levels in FH heterozygotes overlap with those in the general population.Setting:General Hospital/University of Vienna, Department of Pediatrics, Outpatient lipid clinic.Methods:As a part of the MED‐PED (make early diagnosis – prevent early death) project we are currently investigating children, adolescents and their relatives who are suspected to be affected with FH in our out‐patient clinic for metabolic diseases using MED‐PED inclusion criteria and confirming the diagnosis by means of DNA analysis.Patients:263 patients with premature atherosclerosis and/or hypercholesterolaemia: 116 children (mean age 11.6 ± 4.1 years; 57 girls and 59 boys) and 147 adults (64 women, mean age 41.5 ± 13.7 years; 83 men, mean age 42.8 ± 10.8 years).Results:119 patients with mutations have been detected; 56 children with either low density lipoprotein receptor (LDLR) and/or ApoB mutations (27 girls and 29 boys; mean total cholesterol (TC) 275 ± 71 mg/dl, triglycerides (TG) 101 ± 57 mg/dl, high‐density lipoprotein cholesterol (HDL‐C) 49 ± 12 mg/dl, low‐density lipoprotein cholesterol (LDL‐C) 198 ± 67 mg/dl) and one boy with a homozygous. LDLR mutation. A further 62 adults with LDLR and/or ApoB mutations were documented; 33 women (mean age 36.9 ± 11.1 years; mean TC 283 ± 76 mg/dl, TG 137 ± 78 mg/dl, HDL‐C 55 ± 17 mg/dl, LDL‐C 210 ± 67 mg/dl) and 29 men (mean age 45.0 ± 10.6 years; mean TC 301 ± 87 mg/dl, TG 163 ± 112 mg/dl, HDL‐C 42 ± 12 mg/dl, LDL‐C 233 ± 83 mg/dl). In 32 of these subjects (11 children (21%), 21 adults (42%)), serum lipid levels were lower than the diagnostic MED‐PED limits adopted, so that they might have been misclassified without an additional DNA analysis.Conclusion:In our study, diagnosis of FH and related disorders (ApoB‐100 defect) by means of conventional laboratory methods missed at least 21% in children and 42% in adults affected with LDLR and/or ApoB gene mutations. Genetic FH diagnosis provides a tool for specific diagnosis of mutation carrier status.
Risk factors for postoperative pulmonary complications in coronary artery bypass graft surgery patients
Authors: Jensen Louise , Yang Liyan 
Published: March 2007

BackgroundDespite numerous advances in anesthesia, surgical techniques, and postoperative care for coronary artery bypass graft (CABG) surgery, postoperative pulmonary complications (PPCs) still account for postoperative morbidity.ObjectiveTo determine current risk factors for PPCs in CABG surgery patients.MethodsA retrospective cohort design was used. Health records were reviewed for patients ( n=315) who had CABG surgery at a large quaternary healthcare center over a 4 month period. Pre-, peri-, and postoperative risk factors for PPCs were recorded as binary variables. Data were further assessed according to PPCs and non-PPCs using logistic regression models.ResultsPPCs occurred in 99.4% of this CABG surgical cohort. Atelectasis, pleural effusion, atelectasis with pleural effusion, and pneumonia were the most frequent PPCs post CABG surgery. Age >65 years, diabetes, and ASA classification N3 were found to be related to the presence of atelectasis. No significant risk factors were related to the development of pleural effusion or atelectasis with pleural effusion. Postoperative pneumonia was associated with previous myocardial infarction, ventilation >10 h, and hospital stay >5 days. History of bronchitis and COPD were related to postoperative pneumothorax; history of heart failure, COPD, and other lung diseases were related to postoperative pulmonary edema.ConclusionThese findings contribute to the understanding of PPCs in post-CABG surgery patients and assist in identification of patients at risk for developing PPCs.
Caregiver burden in partners of Heart Failure patients; limited influence of disease severity
Authors: Luttik Marie Louise , Jaarsma Tiny , Veeger Nic , Tijssen Jan , Sanderman Robbert , van Veldhuisen Dirk J. 
Published: March 2007

AbstractBackground:In complying with required life style changes Heart Failure (HF) patients often depend on their partners. However providing care may cause burden and affect the health of these partners. The aim of this study was to investigate determinants of caregiver burden in order to identify caregivers who are at risk.Methods:Using a cross‐sectional design, caregiver burden and potential determinants were measured in partners of HF patients. Demographic and clinical data were assessed in HF patients, partners completed questionnaires on caregiver burden (the Caregiver Reaction Assessment, CRA), caregiving tasks performed, physical and mental health status and quality of the marital relationship.Results:In total 357 partners (75% female, mean age 67 years) participated. The physical health status of HF patients was only significantly associated with two domains of caregiver burden, ‘disruption of daily schedule’ (p<0.01) and ‘loss of physical strength’ (p<0.01). No associations were found with age, co‐morbidity and LVEF. All domains of the CRA were mainly associated with the partner's own mental health (p<0.01) and with providing personal care to HF patients (p<0.01). Gender differences were only found with regard to the domain of ‘feeling a lack of family support’.Conclusion:The assessment of caregiver burden should focus on the mental strength of partners. Furthermore when assistance in personal care is needed, additional support, either informal or professional, may be indicated.
Chronic sildenafil lowers transpulmonary gradient and improves cardiac output allowing successful heart transplantation
Authors: Jabbour Andrew , Keogh Anne , Hayward Christopher , Macdonald Peter 
Published: March 2007

AbstractIntroductionFor patients undergoing heart transplantation, an elevated pulmonary vascular resistance (PVR) increases mortality in the early post‐heart transplant period. This study aimed to assess the effects of chronic sildenafil administration on the PVR, transpulmonary gradient (TPG), and cardiac output (CO) in patients with heart failure awaiting heart transplantation.Method:The data from serial right heart catheterizations (RHC) of six patients were analyzed. All patients demonstrated a reaction to the vasodilators glyceryl trinitrate or inhaled iloprost at initial RHC before commencing sildenafil. A follow‐up RHC was performed as required to guide management.Results:The average total daily dose of sildenafil was 100 mg for a period of 68±58 days (4–145). The average TPG at baseline was 23.7 mmHg and fell in 4 of the 6 patients (67%) with an average reduction of 4.5±7.3 mmHg (–5 to 14). The average PVR at baseline was 571 dyn s cm–5 and fell in 5 of 6 patients (83%), with an average reduction of 167±266 dyn s cm–5 (74–518). The CO at baseline was 3.95 L/min and rose in 5 of 6 patients (83%) with an average improvement of 0.58 L/min (–1.1 to 1.3). The mean pulmonary capillary wedge pressure (MPCWP) at baseline was 26.3 mmHg and fell in 5 of 6 patients (83%) with an average fall of 5.5 mmHg (–1 to 17). Four of the six patients achieved a final TPG<15 mmHg, which we consider to be acceptable for orthotopic heart transplantation, average 11 mmHg (8 to 13). Three of these patients have already undergone successful, uncomplicated heart transplantation.Conclusion:Chronic sildenafil use is safe and effective in reducing an elevated TPG and PVR in patients with heart failure requiring heart transplantation and allows patients to be transplanted who may otherwise have been excluded because of pulmonary hypertension.
Electromechanical effects of cardiac resynchronization therapy during rest and stress in patients with heart failure
Authors: Valzania Cinzia , Gadler Fredrik , Eriksson Maria J. , Olsson Arne , Boriani Giuseppe , Braunschweig Frieder 
Published: March 2007

AbstractBackground:Haemodynamic and functional effects of cardiac resynchronization therapy (CRT) have been studied mostly at rest. CRT effects on left ventricular (LV) dyssynchrony and function during stress have not been evaluated in detail.Aims:We studied the electromechanical effects of CRT at rest and during Dobutamine stress echocardiography (DSE), during active and withheld CRT.Methods:Twenty‐one responders to CRT (62±12 yr) were assessed by walking test, quality of life, and BNP with active CRT (“off”) and 2 weeks after pacing withdrawal (“off”). DSE (10 μ/kg/min) was performed both at “on” and “off” to evaluate dyssynchrony parameters, systolic and diastolic function.Results:At rest, CRT withdrawal was associated with an increased interventricular mechanical delay (IVMD, from 21±18 ms to 49±24 ms, p<0.001) and impaired intraventricular synchrony. There was a significant decrease in LV systolic function and LV filling time. Dobutamine infusion had no impact on inter‐ and intraventricular synchrony. During stress, there was an improvement in LV performance both at “on” and “off” However, LV dp/dt, aortic VTI, cardiac output, mean systolic peak velocities and LV filling time during dobutamine stress were significantly greater with CRT “on”.Conclusion:In long‐term responders, CRT improves LV performance both at rest and during dobutamine stress. This is attributable to an improvement in LV synchrony, which is maintained during stress.
Comparison of B‐type natriuretic peptide assays for identifying heart failure in stable elderly patients with a clinical diagnosis of chronic obstructive pulmonary disease
Authors: Rutten Frans H. , Cramer Maarten‐Jan M. , Zuithoff Nicolaas P.A. , Lammers Jan‐Willem J. , Verweij Wim , Grobbee Diederick E. , Hoes Arno W. 
Published: March 2007

AbstractAims:To compare the ability of different B‐type natriuretic peptide (BNP) assays to identify heart failure in stable elderly patients with a diagnosis of chronic obstructive pulmonary disease (COPD).Methods:200 patients aged ≥65 years with COPD according to their general practitioner and without known heart failure, underwent a diagnostic work‐up. The final diagnosis of heart failure was established by a panel using the diagnostic principles of the European Society of Cardiology. All available diagnostic results, including echocardiography, but not BNP or NT‐proBNP measurements, were used. The ability of different B‐type natriuretic peptide assays to identify heart failure was estimated using the area under the receiver operating characteristic curves (ROC‐area).Results:The ROC‐areas did not differ significantly between the various assays of NT‐proBNP and BNP, and ranged from 0.68 (95%CI 0.60–0.73) to 0.73 (95%CI 0.64–0.81). For NT‐proBNP the age‐ and gender‐independent “optimal” cut‐point was 15 pmol/l (125 pg/ml) and for BNP 10 pmol/l (35 pg/ml). All assays were much better at excluding than detecting heart failure.Conclusions:All assays of B‐type natriuretic peptide showed reasonable and comparable accuracy in recognising heart failure. At “optimal” cut‐points, all assays performed better at excluding than detecting new cases of heart failure in this population.
Clinical diagnosis of left ventricular dilatation and dysfunction in the age of technology
Authors: Rovai Daniele , Morales Maria‐Aurora , Di Bella Gianluca , De Nes Michele , Pingitore Alessandro , Lombardi Massimo , Rossi Giuseppe 
Published: March 2007

AbstractBackground:The diagnostic process has become increasingly dependent on instrumental and laboratory investigation.Aim:To evaluate the accuracy of symptoms and signs in identifying left ventricular (LV) dilatation and/or systolic dysfunction.MethodsA group of 100 patients in stable clinical condition and scheduled for cardiac magnetic resonance imaging was prospectively examined by two cardiologists, who were unaware of the individual patient's condition. Patients were interviewed and underwent physical examination.Results:Several symptoms and signs were associated with LV dilatation and systolic dysfunction at univariate analysis. Using multiple logistic regression, a mitral systolic murmur, a laterally displaced LV impulse, orthopnoea and hepatomegaly were all independent predictors of LV dilatation (end‐diastolic volume ≥110 ml/m2) (p<0.0001) and LV dysfunction (ejection fraction <45%) (p<0.0001). The combination of the above variables correctly identified 79% of patients with LV dilatation (sensitivity 51%, specificity 92%), and 82% of patients with LV dysfunction (sensitivity 68%, specificity 90%). Considering LV dilatation and dysfunction, 77% of patients were correctly identified after history alone (kappa=0.13), 84% after LV impulse examination (kappa=0.55) and 86% after cardiac auscultation (kappa=0.58).ConclusionSymptoms and signs predict LV dilatation and/or dysfunction with fair sensitivity and excellent specificity.