bluuebunny/crossref_metadata_2025_split huggingface dataset:
Coronary microvascular dysfunction is an early feature of cardiac involvement in patients with Anderson–Fabry disease
Authors: Tomberli Benedetta , Cecchi Franco , Sciagrà Roberto , Berti Valentina , Lisi Francesca , Torricelli Francesca , Morrone Amelia , Castelli Gabriele , Yacoub Magdi H. , Olivotto Iacopo 
Published: July 2013

AimsMale patients with Anderson–Fabry disease (AFD) often exhibit cardiac involvement, characterized by LV hypertrophy (LVH), associated with severe coronary microvascular dysfunction (CMD). Whether CMD is present in patients without LVH, particularly when female, remains unresolved. The aim of the study was to investigate the presence of CMD by positron emission tomography (PET) in AFD patients of both genders, with and without evidence of LVH.Methods and resultsWe assessed myocardial blood flow following dipyridamole infusion (Dip‐MBF) with 13N‐labelled ammonia by PET in 30 AFD patients (age 51 ± 13 years; 18 females) and in 24 healthy controls. LVH was defined as echocardiographic maximal LV wall thickness ≥13 mm. LVH was present in 67% of patients (n = 20; 10 males and 10 females). Dip‐MBF was reduced in all patients compared with controls (1.8 ± 0.5 and 3.2 ± 0.5 mL/min/g, respectively, P < 0.001). For both genders, flow impairment was most severe in patients with LVH (1.4 ± 0.5 mL/min/g in males and 1.9 ± 0.5 mL/min/g in females), but was also evident in those without LVH (1.8 ± 0.3 mL/min/g in males and 2.1 ± 0.4 mL/min/g in females; overall P = 0.064 vs. patients with LVH). Analysis of variance (ANOVA) for the 17 LV segments showed marked regional heterogeneity of MBF in AFD (F = 4.46, P < 0.01), with prevalent hypoperfusion of the apical region. Conversely, controls showed homogeneous LV perfusion (F = 1.25, P = 0.23).ConclusionsCoronary microvascular function is markedly impaired in AFD patients irrespective of LVH and gender. CMD may represent the only sign of cardiac involvement in AFD patients, with potentially important implications for clinical management.
Right ventricular hypertrophy and failure abolish cardioprotection by ischaemic pre‐conditioning
Authors: Andersen Asger , Povlsen Jonas Agerlund , Bøtker Hans Erik , Nielsen‐Kudsk Jens Erik 
Published: July 2013

AimsWe aimed to investigate the response to ischaemia–reperfusion (IR) and ischaemic pre‐conditioning (IPC) in the hypertrophic and failing right heart.Methods and resultsMale Wistar rats underwent sham operation, moderate pulmonary trunk banding (mPTB), or severe PTB (sPTB). Four weeks after surgery, hearts were quick‐frozen (n = 28) for biochemical analysis of key salvage pathways or isolated and perfused in a Langendorff set‐up (n = 46). We randomized perfused hearts to IPC (2 × 5 min of global ischaemia) or no preceding ischaemia (CON), before 40 min of global ischaemia and 120 min of reperfusion. The infarct size/area at risk (IS/AAR) ratio and post‐ischaemic right ventricular (RV) function were used to evaluate the effect of IPC. mPTB induced compensated RV hypertrophy and sPTB induced RV hypertrophy with failure. Hypertrophy of the right ventricle increased IS in hearts from mPTB and sPTB animals compared with sham (IS/AAR, 73.1 ± 2.9% and 59.3 ± 2.4% vs. 35.6 ± 2.9%, P < 0.0001). IPC reduced IS in sham and mPTB hearts (IS/AAR, 35.6 ± 2.9% vs. 17.4 ± 1.2% and 73.1 ± 2.9% vs. 56.9 ± 3.5%, P < 0.01) and improved recovery of RV contractile function. IPC did not alter IS/AAR (59.3 ± 2.4% and 59.3 ± 2.9%, P = 0.999) or haemodynamic recovery in sPTB hearts. RV cyclic guanosine monophosphate (cGMP) and the cGMP‐dependent protein kinase were increased after sPTB.ConclusionRight ventricular hypertrophy increases IR injury. Cardioprotection by IPC is abolished in the failing but not the compensated hypertrophic right ventricle of the rat heart.
Accuracy of Plateau Pressure and Stress Index to Identify Injurious Ventilation in Patients with Acute Respiratory Distress Syndrome
Authors: Terragni Pier Paolo , Filippini Claudia , Slutsky Arthur S. , Birocco Alberto , Tenaglia Tommaso , Grasso Salvatore , Stripoli Tania , Pasero Daniela , Urbino Rosario , Fanelli Vito , Faggiano Chiara , Mascia Luciana , Ranieri V. Marco 
Published: July 2013

Abstract

Background:
Guidelines suggest a plateau pressure (P

plat
) of 30 cm H2O or less for patients with acute respiratory distress syndrome, but ventilation may still be injurious despite adhering to this guideline. The shape of the curve plotting airway pressure versus time (S

tress

I

ndex
) may identify injurious ventilation. The authors assessed accuracy of P

plat
and S

tress

I

ndex
to identify morphological indexes of injurious ventilation.

Methods:
Indexes of lung aeration (computerized tomography) associated with injurious ventilation were used as a “reference standard.” Threshold values of P

plat
and S

tress

I

ndex
were determined assessing the receiver-operating characteristics (“training set,” N = 30). Accuracy of these values was assessed in a second group of patients (“validation set,” N = 20). P

plat
and S

tress

I

ndex
were partitioned between respiratory system (Pplat,Rs and S

tress

I

ndex
,
rs
) and lung (P

plat
,
l
and S

tress

I

ndex
,
l
; esophageal pressure; “physiological set,” N = 50).

Results:
Sensitivity and specificity of P

plat
of greater than 30 cm H2O were 0.06 (95% CI, 0.002–0.30) and 1.0 (95% CI, 0.87–1.00). P

plat
of greater than 25 cm H2O and a S

tress

I

ndex
of greater than 1.05 best identified morphological markers of injurious ventilation. Sensitivity and specificity of these values were 0.75 (95% CI, 0.35–0.97) and 0.75 (95% CI, 0.43–0.95) for P

plat
greater than 25 cm H2O versus 0.88 (95% CI, 0.47–1.00) and 0.50 (95% CI, 0.21–0.79) for S

tress

I

ndex
greater than 1.05. Pplat,Rs did not correlate with P

plat
,
l
(R
2 = 0.0099); S

tress

I

ndex
,
rs
and S

tress

I

ndex
,
l
were correlated (R
2 = 0.762).

Conclusions:
The best threshold values for discriminating morphological indexes associated with injurious ventilation were Pplat,Rs greater than 25 cm H2O and S

tress

I

ndex
,
rs
greater than 1.05. Although a substantial discrepancy between Pplat,Rs and P

plat
,
l
occurs, S

tress

I

ndex
,
rs
reflects S

tress

I

ndex
,
l
.
Incidence, Paris Classification, and Follow‐up in a Nationwide Incident Cohort of Pediatric Patients With Inflammatory Bowel Disease
Authors: Müller Katalin E. , Lakatos Péter L. , Arató András , Kovács Judit B. , Várkonyi Ágnes , Szűcs Dániel , Szakos Erzsébet , Sólyom Enikő , Kovács Márta , Polgár Marianne , Nemes Éva , Guthy Ildikó , Tokodi István , Tóth Gergely , Horváth Ágnes , Tárnok András , Csoszánszki Noémi , Balogh Márta , Vass Noémi , Bódi Piroska , Dezsőfi Antal , Gárdos László , Micskey Éva , Papp Mária , Cseh Áron , Szabó Dolóresz , Vörös Péter ,   Hungarian IBD Registry Group (HUPIR), Veres Gabor 
Published: July 2013

ABSTRACTObjectives:The aim of the study was to evaluate the incidence, baseline disease characteristics, and disease location based on the Paris classification in pediatric inflammatory bowel disease (IBD) in the Hungarian nationwide inception cohort. In addition, 1‐year follow‐up with therapy was analyzed.Methods:From January 1, 2007 to December 31, 2009, newly diagnosed pediatric patients with IBD were prospectively registered. Twenty‐seven pediatric gastroenterology centers participated in the data collection ensuring the data from the whole country. Newly diagnosed patients with IBD younger than 18 years were reported. Disease location was classified according to the Paris classification.Results:A total of 420 patients were identified. The incidence rate of pediatric IBD was 7.48/105 (95% confidence interval [CI] 6.34/105–8.83/105). The incidence for Crohn disease (CD) was 4.72/105 (95% CI 3.82–5.79), for ulcerative colitis (UC) 2.32/105 (95% CI 1.71–3.09), and for IBD‐unclassified 0.45/105 (95% CI 0.22–0.84). Most common location in CD was L3 (58.7%); typical upper gastrointestinal abnormalities (ulcer, erosion and aphthous lesion) were observed in 29.9%. Extensive colitis in patients with UC (E4, proximal to hepatic flexure) was the most common disease phenotype (57%), whereas only 5% of children had proctitis. A total of 18.6% of patients had ever severe disease (S1). Frequency of azathioprine administration at diagnosis was 29.5% in patients with CD, and this rate increased to 54.6% (130/238) at 1‐year follow‐up. In UC, only 3.3% received azathioprine initially, and this rate elevated to 22.5% (25/111). Use of corticosteroid decreased from 50% to 15.3% in patients with UC. Rate of bowel resection in patients with CD during the first year of follow‐up was 5%.Conclusions:The incidence of pediatric IBD in Hungary was among the higher range reported. This is the first large, nationwide incident cohort analyzed according to the Paris classification, which is a useful tool to determine the characteristic pediatric CD phenotype.
Management of Portal Hypertension in Children With Portal Vein Thrombosis
Authors: Giouleme Olga , Theocharidou Eleni 
Published: July 2013

ABSTRACTPortal vein thrombosis (PVT) is a common cause of portal hypertension in children. Predisposing conditions for PVT are obscure in more than half of the cases. Variceal bleeding and splenomegaly are the most frequent initial manifestations. Radiologic imaging studies are the mainstay for diagnosis. Treatment includes pharmacologic, endoscopic, and surgical modalities. β‐Adrenergic blockers are not routinely used in children because of unproven efficacy and significant adverse effects. Endoscopic methods, such as sclerotherapy and endoscopic variceal ligation (EVL), are highly effective in the treatment of acute variceal bleeding and eradication of varices. EVL is the treatment of choice because of minimal complications and the need for few endoscopic sessions. EVL facilitates portal decompression either by the formation of collateral vessels or by surgical portosystemic shunting, when vessels grow to the proper diameter for anastomosis. Surgical portosystemic shunts are reserved for refractory cases because of significant complications and technical difficulties. Transjugular portosystemic shunts have an emerging role in the management of portal hypertension caused by PVT. PVT may occur in the posttransplant setting, but optimal management is not defined yet.
Estimation of Fish and ω‐3 Fatty Acid Intake in Pediatric Nonalcoholic Fatty Liver Disease
Authors: St‐Jules David E. , Watters Corilee A. , Brunt Elizabeth M. , Wilkens Lynne R. , Novotny Rachel , Belt Patricia , Lavine Joel E. ,   Nonalcoholic Steatohepatitis Clinical Research Network
Published: July 2013

ABSTRACTAims:Fish and ω‐3 fatty acids are reported to be beneficial in pediatric nonalcoholic fatty liver disease (NAFLD), but no studies have assessed their relation to histological severity. The objectives of this study were to evaluate the dietary intake of fish and ω‐3 fatty acids in children with biopsy‐proven NAFLD, and examine their association with serological and histological indicators of disease.Methods:This was a cross‐sectional analysis of 223 children (6–18 years) who participated in the Treatment of Nonalcoholic Fatty Liver Disease in Children trial or the NAFLD Database study conducted by the Nonalcoholic Steatohepatitis Clinical Research Network. The distribution of fish and ω‐3 fatty acid intake was determined from responses to the Block Brief 2000 Food Frequency Questionnaire, and analyzed for associations with serum alanine aminotransferase, histological features of fatty liver disease, and diagnosis of steatohepatitis after adjusting for demographic, anthropometric, and dietary variables.Results:The minority of subjects consumed the recommended 8 ounces of fish per week (22/223 [10%]) and 200 mg of long‐chain ω‐3 fatty acids per day (12/223 [5%]). Lack of fish and long‐chain ω‐3 fatty acid intake was associated with greater portal (P = 0.03 and P = 0.10, respectively) and lobular inflammation (P = 0.09 and P = 0.004, respectively) after controlling for potential confounders.Conclusions:Fish and ω‐3 fatty acid intake was insufficient in children with NAFLD, which may increase susceptibility to hepatic inflammation. Patients with pediatric NAFLD should be encouraged to consume the recommended amount of fish per week.
Home range and resource selection by animals constrained by linear habitat features: an example ofBlakiston's fish owl
Authors: Slaght Jonathan C. , Horne Jon S. , Surmach Sergei G. , Gutiérrez R.J. 
Published: July 2013

SummaryTypically in resource selection studies, the spatial extent of a home range is defined first and then the available resources within that perimeter are estimated. However, the home ranges (or habitats) of some animals are constrained by linear environmental features (e.g. rivers, shorelines). Traditional home range estimators often overestimate home range extent for such species, which can lead to spurious estimation of resource availability and selection.We used a synoptic model of space use to explicitly account for resource selection of a species constrained by linear features in its environment to compare with traditional home range estimators. We used the endangered Blakiston's fish owlBubo blakistoniin the Russian Far East as our example.Mean annual home range size (± standard error) was more than three times larger when using kernel methods (30·3 ± 15·1 km2) than when using the synoptic model (9·4 ± 2·0 km2,n = 7).Fish owls showed strong selection for areas within valleys, closer to waterways, closer to patches of permanently open water and with greater channel complexity than available sites.Synthesis and applications. The synoptic model solves a long‐standing problem in home range and resource selection studies because it provides an objective way to estimate the space use of a species whose habitat is constrained by linear features in its environment. Improvements in the accuracy of such estimations can lead to identification of important resources across landscapes, the development of more rigorous site‐specific or landscape‐scale management plans, and to scientifically defensible conservation or threat mitigation measures.
The New National Quality Framework: Quantifying Some of the Effects on Labour Supply, Child Care Demand and Household Finances for Two‐Parent Households
Authors: Breunig Robert V. , Gong Xiaodong , Trott Declan 
Published: July 2013

New regulations to improve the quality of early childhood education and care came into force in Australia in 2012. Using a simultaneous, structural model of labour supply and child care demand we predict the effects on the labour supply of partnered women, on demand for child care and on household finances for two‐parent households in Australia. Using estimated cost impacts of this new National Quality Framework from government and non‐government sources, we find modest effects on household behaviour. For a mid‐range cost scenario, we find that partnered women's labour force participation will decrease by just over one‐half of one percentage point, a change of less than 1 per cent. Working hours for partnered women decrease by 20 minutes or approximately 2 per cent. Household disposable income decreases by $12.50, a 0.6 per cent decrease. Given widespread agreement about the benefits of investing in children, these quantitatively small effects for two‐parent households strengthen the case for the National Quality Framework.
Autoimmune hepatitis in southern Israel: A 15‐year multicenter study
Authors: Delgado Jorge‐Shmuel , Vodonos Alina , Malnick Stephen , Kriger Ofra , Wilkof‐Segev Renana , Delgado Bertha , Novack Victor , Rosenthal Alexander , Menachem Yoram , Melzer Ehud , Fich Alexander 
Published: July 2013

ObjectiveIn this study we aimed to assess the incidence, prevalence and clinical outcomes of patients with autoimmune hepatitis (AIH) in southern Israel.MethodsCase‐finding methods and population‐based administrative data were used to evaluate the epidemiology and prognostic factors of AIH from 1995 to 2010.ResultsDuring the study period, the average annual prevalence and incidence of AIH in southern Israel were 11.0/100 000 and 0.67/100 000, respectively. We identified 100 AIH cases with a mean age of 47.9 years, including 95 women and five men. Type 1 AIH was found in 77 cases, and liver fibrosis and cirrhosis were found in 73.4% and 22.3% of all patients who underwent liver biopsy. In all, 98 patients were treated with a combination of steroids and azathioprine or steroids alone (prednisone and azathioprine in 71, budesonide and azathioprine in 11, prednisone or budesonide alone in six and ten, respectively). Complete remission was recorded in 56 patients, whereas partial response or failure of treatment was noted in 42 patients. In multivariate analysis the independent predictors of remission were the degree of liver fibrosis (mild vs bridging fibrosis (F3) and cirrhosis [F4]) (P = 0.003) and level of albumin (P = 0.031). The estimated 1‐year and 10‐year survival for AIH were 96.5% and 89.7%, respectively.ConclusionsThe prevalence of AIH in Israel is quite similar to that of other European Caucasian populations, with a relatively long‐term good prognosis, despite a low rate of response to immunosuppressive therapy.
Cd2+ resistance mechanisms in Methanosarcina acetivorans involve the increase in the coenzyme M content and induction of biofilm synthesis
Authors: Lira‐Silva Elizabeth , Santiago‐Martínez M. Geovanni , García‐Contreras Rodolfo , Zepeda‐Rodríguez Armando , Marín‐Hernández Alvaro , Moreno‐Sánchez Rafael , Jasso‐Chávez Ricardo 
Published: July 2013

SummaryTo assess what defence mechanisms are triggered by Cd2+ stress in Methanosarcina acetivorans, cells were cultured at different cadmium concentrations. In the presence of 100 μM CdCl2, the intracellular contents of cysteine, sulfide and coenzyme M increased, respectively, 8, 27 and 7 times versus control. Cells incubated for 24 h in medium with less cysteine and sulfide removed up to 80% of Cd2+ added, whereas their cysteine and coenzyme M contents increased 160 and 84 times respectively. Cadmium accumulation (5.2 μmol/10–15 mg protein) resulted in an increase in methane synthesis of 4.5 times in cells grown on acetate. Total phosphate also increased under high (0.5 mM) Cd2+ stress. On the other hand, cells preadapted to 54 μM CdCl2 and further exposed to > 0.63 mM CdCl2 developed the formation of a biofilm with an extracellular matrix constituted by carbohydrates, DNA and proteins. Biofilm cells were able to synthesize methane. The data suggested that increased intracellular contents of thiol molecules and total phosphate, and biofilm formation, are all involved in the cadmium resistance mechanisms in this marine archaeon.
Evaluation of grafted patients with donor corneas that today are more than 100 years old
Authors: Visby Elizabeth , Hjortdal Jesper , Nielsen Kim 
Published: July 2013

Abstract.Purpose:  Life expectancy is increasing. When corneal donors become older and corneal‐grafted patients live longer with their graft, the need for good‐quality donor tissue becomes more crucial. The aim of the present investigation is to study grafted recipients with a donor cornea with a total tissue age of more than 100 years.Methods:  One thousand consecutive donor records from the Danish Cornea Bank were initially reviewed. After applying different inclusion criteria, 35 recipients with corneal donor tissue of more than 100 years of age were invited for a follow‐up visit. Visual acuity, corneal transparency and thickness, and intraocular pressure were measured. Corneal topography and endothelial photos were taken.Results:  Seventeen of the invited patients attended the examination. The average age of the grafts at examination was 107 years old; the oldest being 118 years. Most grafts were still clear 23–35 years after transplantation, and almost one‐fourth had best spectacle‐corrected visual acuity (BSCVA) ≥ 0.50. Cell morphology showed irregularity in size and shape for both grafted and healthy corneas, but the alterations were more extensive in the grafted corneas. The average endothelial cell density (ECD) was 1360 mm2 in the grafted corneas. Sixty per cent had ECD > 1000 cells/mm2. No signs of decompensation were observed for those with <1000 cells. The average central corneal thickness of the grafts was 0.582 mm (SD = 0.067) compared with 0.494 mm (SD = 0.043) in the fellow cornea.Conclusion:  This study shows a trend of moderate long‐term survival and quality for very old grafts despite low ECD. Most recipients had a clear transplant, and one‐fifth had BSCVA of 0.80.
Human β‐defensin 2 may inhibit internalisation of bacillus Calmette‐Guérin (BCG) in bladder cancer cells
Authors: Kim Jung Hoon , Kim Soon‐Ja , Lee Kyung Mee , Chang In Ho 
Published: July 2013

Objective
To investigate whether secretion of human β‐defensin 2 (HBD‐2) is induced by bacillus Calmette‐Guérin (BCG) and to determine whether HBD‐2 affects BCG internalisation in bladder cancer cells.
Materials and Methods
Reverse transcription‐polymerase chain reaction analysis was used to determine whether HBD‐2 mRNA increases after incubation with BCG. HBD‐2 proteins in 5637 and T24 human bladder cancer cell lines were assayed by enzyme‐linked immunosorbent assay.
The internalisation rate was evaluated by double immunofluorescence assay and confocal microscopy to test the optimal dose of HBD‐2 for BCG internalisation.
We also investigated the difference in internalisation rates and cell viability between recombinant HBD‐2 protein, anti‐HBD‐2 antibody, and HBD‐2 plus anti‐HBD‐2 antibody pretreatments.
Results
BCG induced HBD‐2 mRNA expression and HBD‐2 production dose and time‐dependently in bladder cancer cells and affected BCG internalisation.
Pretreatment with recombinant HBD‐2 protein lowered internalisation of BCG dose‐dependently. Moreover, anti‐HBD‐2 antibody prevented the effect of HBD‐2 on BCG internalisation in bladder cancer cells.
The internalisation rate of BCG pretreated with anti‐HBD‐2 antibody was higher than that in the control in 5637 (P < 0.01) and T24 cells (P < 0.05). The BCG internalisation rate in cells pretreated with anti‐HBD‐2 antibody plus recombinant HBD‐2 protein was higher than that in the control in 5637 (P < 0.01) and T24 cells (P < 0.05).
Mycobacterium bovis BCG decreased bladder cancer cell viability, and anti‐HBD‐2 antibody prevented the inhibitory role of HBD‐2 on the anti‐proliferative effects of M. bovis BCG in bladder cancer cells
Conclusion
Bladder cancer cells produce HBD‐2 when they are infected by BCG to defend themselves against BCG internalisation, which plays an important role during the initiation and propagation of the immunotherapeutic response in bladder cancer cells.
Measuring the surgical ‘learning curve’: methods, variables and competency
Authors: Khan Nuzhath , Abboudi Hamid , Khan Mohammed Shamim , Dasgupta Prokar , Ahmed Kamran 
Published: July 2013

Objectives
To describe how learning curves are measured and what procedural variables are used to establish a ‘learning curve’ (LC).
To assess whether LCs are a valuable measure of competency.
Patients and Methods
A review of the surgical literature pertaining to LCs was conducted using the Medline and OVID databases.
Results
Variables should be fully defined and when possible, patient‐specific variables should be used. Trainee's prior experience and level of supervision should be quantified; the case mix and complexity should ideally be constant.
Logistic regression may be used to control for confounding variables.
Ideally, a learning plateau should reach a predefined/expert‐derived competency level, which should be fully defined. When the group splitting method is used, smaller cohorts should be used in order to narrow the range of the LC.
Simulation technology and competence‐based objective assessments may be used in training and assessment in LC studies.
Conclusions
Measuring the surgical LC has potential benefits for patient safety and surgical education. However, standardisation in the methods and variables used to measure LCs is required.
Confounding variables, such as participant's prior experience, case mix, difficulty of procedures and level of supervision, should be controlled.
Competency and expert performance should be fully defined.