bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intracranial aneurysms: optimized diagnostic tools call for thorough interdisciplinary treatment strategies
Authors: Mueller Oliver M. , Schlamann Marc , Mueller Daniela , Sandalcioglu I. Erol , Forsting Michael , Sure Ulrich 
Published: September 2011

Objective: Intracranial aneurysms (IAs) require deliberately selected treatment strategies as they are incrementally found prior to rupture and deleterious subarachnoid haemorrhage (SAH). Multiple and recurrent aneurysms necessitate both neurointerventionalists and neurosurgeons to optimize aneurysmal occlusion in an interdisciplinary effort. The present study was conducted to condense essential strategies from a single neurovascular centre with regard to the lessons learned. Method: Medical charts of 321 consecutive patients treated for IAs at our centre from September 2008 until December 2010 were retrospectively analysed for clinical presentation of the aneurysms, multiplicity and treatment pathways. In addition, a selective Medline search was performed. Results: A total of 321 patients with 492 aneurysms underwent occlusion of their symptomatic aneurysm: 132 (41.1%) individuals were treated surgically, 189 (58.2%) interventionally; 138 patients presented with a SAH, of these 44.2% were clipped and 55.8% were coiled. Aneurysms of the middle cerebral artery were primarily occluded surgically (88), whereas most of the aneurysms of the internal carotid artery and anterior communicating artery (114) were treated endovascularly. Multiple aneurysms (range 2–5 aneurysms/individual) were diagnosed in 98 patients (30.2%). During the study period 12 patients with recurrent aneurysms were allocated to another treatment modality (previously clip to coil and vice versa). Conclusions: Our data show that successful interdisciplinary occlusion of IAs is based on both neurosurgical and neurointerventional therapy. In particular, multiple and recurrent aneurysms require tailored individual approaches to aneurysmal occlusion. This is achieved by a consequent interdisciplinary pondering of the optimal strategy to occlude IAs in order to prevent SAH.
Cervical carotid artery disease in sickle cell anemia: clinical and radiological features
Authors: Telfer Paul T. , Evanson Jane , Butler Paul , Hemmaway Claire , Abdulla Chenya , Gadong Nimze , Whitmarsh Simon , Kaya Banu , Kirkham Fenella J. 
Published: September 2011

AbstractCervical internal carotid artery (cICA) occlusion is a recognized cause of acute ischemic stroke (AIS) in sickle cell disease (SCD), but the associated clinical and radiologic features are not well described. We reviewed data on cervical magnetic resonance angiography (cMRA) performed prospectively in 67 patients (55 children) for indications including transcranial Doppler (TCD) abnormalities, AIS, or previous AIS. cICA lesions were seen in 10 (15%) patients, including 4 of 7 patients presenting with AIS, and appear to have been missed on first presentation in 4 of 10 patients with previous AIS. Radiologic features in 7 patients were consistent with dissection. In 2 patients, there was strong clinical and radiologic evidence for thromboembolic AIS, and this was also considered possible in 4 other patients. Three of the 4 AIS patients were anticoagulated acutely, and the nontreated patient had recurrent, probably thromboembolic, AIS. TCD findings were variable, but in 4 patients there were high velocities in the cerebral vessels contralateral to the cICA stenosis. We suggest that all patients with AIS should have cMRA during acute evaluation to identify cICA occlusions that may require anticoagulation. Routine screening of children with SCD should also include evaluation of neck vessels by carotid Doppler followed by cMRA if a cervical vascular lesion is suspected.
Proof of principle for transfusion of in vitro–generated red blood cells
Authors: Giarratana Marie-Catherine , Rouard Hélène , Dumont Agnès , Kiger Laurent , Safeukui Innocent , Le Pennec Pierre-Yves , François Sabine , Trugnan Germain , Peyrard Thierry , Marie Tiffany , Jolly Séverine , Hebert Nicolas , Mazurier Christelle , Mario Nathalie , Harmand Laurence , Lapillonne Hélène , Devaux Jean-Yves , Douay Luc 
Published: September 2011

AbstractIn vitro RBC production from stem cells could represent an alternative to classic transfusion products. Until now the clinical feasibility of this concept has not been demonstrated. We addressed the question of the capacity of cultured RBCs (cRBCs) to survive in humans. By using a culture protocol permitting erythroid differentiation from peripheral CD34+ HSC, we generated a homogeneous population of cRBC functional in terms of their deformability, enzyme content, capacity of their hemoglobin to fix/release oxygen, and expression of blood group antigens. We then demonstrated in the nonobese diabetes/severe combined immunodeficiency mouse that cRBC encountered in vivo the conditions necessary for their complete maturation. These data provided the rationale for injecting into one human a homogeneous sample of 1010 cRBCs generated under good manufacturing practice conditions and labeled with 51Cr. The level of these cells in the circulation 26 days after injection was between 41% and 63%, which compares favorably with the reported half-life of 28 ± 2 days for native RBCs. Their survival in vivo testifies globally to their quality and functionality. These data establish the proof of principle for transfusion of in vitro–generated RBCs and path the way toward new developments in transfusion medicine. This study is registered at http://www.clinicaltrials.gov as NCT0929266.
How I treat autoimmune lymphoproliferative syndrome
Authors: Rao V. Koneti , Oliveira João Bosco 
Published: September 2011

AbstractAutoimmune lymphoproliferative syndrome (ALPS) represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis, permitting accumulation of lymphoid mass and persistence of autoreactive cells that often manifest in childhood with chronic nonmalignant lymphadenopathy, hepatosplenomegaly, and recurring multilineage cytopenias. Cytopenias in these patients can be the result of splenic sequestration as well as autoimmune complications manifesting as autoimmune hemolytic anemia, immune-mediated thrombocytopenia, and autoimmune neutropenia. More than 300 families with hereditary ALPS have now been described; nearly 500 patients from these families have been studied and followed worldwide over the last 20 years by our colleagues and ourselves. Some of these patients with FAS mutations affecting the intracellular portion of the FAS protein also have an increased risk of B-cell lymphoma. The best approaches to diagnosis, follow-up, and management of ALPS, its associated cytopenias, and other complications resulting from infiltrative lymphoproliferation and autoimmunity are presented. This trial was registered at www.clinicaltrial.gov as #NCT00001350.
Pulmonary vascular resistances during exercise in normal subjects: a systematic review
Authors: Kovacs G. , Olschewski A. , Berghold A. , Olschewski H. 
Published: September 2011

The physiological range of pulmonary vascular resistance (PVR) and total pulmonary resistance (TPR), and the impact of exercise, age and posture have been a matter of debate for many years.We performed a systematic literature review including all right heart catheterisation data where individual PVR and TPR of healthy subjects both at rest and exercise were available. Data were stratified according to age, exercise level and posture.Supine resting PVR in subjects aged <24 yrs, 24–50 yrs, 51–69 yrs and ≥70 yrs was 61±23, 69±28, 86±15 and 90±39 dyn·s·cm−5, respectively. Corresponding TPR was 165±50, 164±46, 226±64 and 223±45 dyn·s·cm−5, respectively. During moderate exercise in subjects aged ≤50 yrs, an 85% increase in cardiac output was associated with a 25% decrease in TPR (p<0.0001) and a 12% decrease in PVR (p<0.01). At 51–69 yrs of age there was no significant decrease in TPR and PVR. In individuals aged ≥70 yrs TPR even increased by 17% (p=0.01), while PVR did not change significantly. At higher exercise levels, TPR decreased in all age groups. In the upright position, based on a limited number of data, resting TPR and PVR were higher than in the supine position and decreased more prominently during exercise, suggesting the release of resting pulmonary vasoconstriction.These data may form a basis to define normal PVR at rest and exercise.
ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre
Authors: Hurdman J. , Condliffe R. , Elliot C.A. , Davies C. , Hill C. , Wild J.M. , Capener D. , Sephton P. , Hamilton N. , Armstrong I.J. , Billings C. , Lawrie A. , Sabroe I. , Akil M. , O’Toole L. , Kiely D.G. 
Published: September 2011

Pulmonary hypertension (PH) is a heterogeneous condition. To date, no registry data exists reflecting the spectrum of disease across the five diagnostic groups encountered in a specialist referral centre.Data was retrieved for consecutive, treatment-naïve cases diagnosed between 2001 and 2010 using a catheter-based approach. 1,344 patients were enrolled, with a mean follow-up of 2.9 yrs.The 3-yr survival was 68% for pulmonary arterial hypertension (PAH), 73% for PH associated with left heart disease, 44% for PH associated with lung disease (PH-lung), 71% for chronic thromboembolic PH (CTEPH) and 59% for miscellaneous PH. Compared with PAH, survival was inferior in PH-lung and superior in CTEPH (p<0.05). Multivariate analysis demonstrated that diagnostic group independently predicted survival. Within PAH, Eisenmenger’s survival was superior to idiopathic PAH, which was superior to PAH associated with systemic sclerosis (p<0.005). Within PH-lung, 3-yr survival in sleep disorders/alveolar hypoventilation (90%) was superior to PH-lung with chronic obstructive pulmonary disease (41%) and interstitial lung disease (16%) (p<0.05). In CTEPH, long-term survival was best in patients with surgically accessible disease undergoing pulmonary endarterectomy.In this large registry of consecutive, treatment-naïve patients identified at a specialist PH centre, outcomes and characteristics differed between and within PH groups. The current system of classification of PH has prognostic value even when adjusted for age and disease severity, emphasising the importance of systematic evaluation and precise classification.
The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension
Authors: Nickel N. , Golpon H. , Greer M. , Knudsen L. , Olsson K. , Westerkamp V. , Welte T. , Hoeper M.M. 
Published: September 2011

Current guidelines for the treatment of patients with idiopathic pulmonary arterial hypertension (IPAH) recommend basing therapeutic decision-making on haemodynamic, functional and biochemical variables. Most of these parameters have been evaluated as risk predictors at the time of diagnosis. The aim of the present study was to assess the prognostic impact of changes in these parameters after initiation of targeted therapy.A cohort of 109 patients with IPAH who had undergone haemodynamic, functional and biochemical assessments at baseline and 3–12 months after initiation of pulmonary arterial hypertension (PAH)-targeted therapy, were followed for a median 38 months in order to determine predictors of mortality at baseline and during the course of their disease.Within the observation period, 53 (48.6%) patients died and four (3.7%) underwent lung transplantation. Kaplan–Meier estimates for transplantation-free survival were 92%, 67%, and 51% at 1, 3, and 5 yrs, respectively. Among baseline variables, 6-min walk distance, right atrial pressure, cardiac index, mixed-venous oxygen saturation (Sv,O2) and N-terminal-pro brain natriuretic peptide (NT-proBNP) were independent predictors of survival. During follow-up, changes in World Health Organization functional class, cardiac index,Sv,O2and NT-proBNP proved significant predictors of outcome. When assigned to prognostic groups, improvements as well as deteriorations in these parameters after initiation of PAH-targeted therapy had a strong impact on survival. Measurements obtained at follow-up had a higher predictive value than variables obtained at baseline.Changes in established predictors of outcome during the course of the disease provide important prognostic information in patients with IPAH.
Overweight is not a comorbidity factor during childhood asthma: the GrowthOb study
Authors: Mahut Bruno , Beydon Nicole , Delclaux Christophe 
Published: September 2011

While being overweight is a risk factor for subsequent asthma in children, the importance of body mass index (BMI) as a comorbidity factor remains debated. The aim of this study was to assess the relationships between being overweight and the characteristics of childhood asthma.The BMI, BMI z-scores and International Obesity Task Force (IOTF) grades were evaluated in asthmatic children according to atopic status, symptoms during the past 3 months, exercise breathlessness, treatment and lung function in 6–15-yr-old children with confirmed asthma.491 asthmatic children (mean±sdage 10.8±2.6 yrs; 179 females) were prospectively enrolled. There were 78 (15.5%) overweight (IOTF grade 1) and eight (1.6%) obese (grade 2) children. The children's BMI z-scores did not differ according to atopy, exacerbation, symptom-free days or treatment. The BMI z-score correlated positively with forced vital capacity and forced expiratory volume in 1 s in females, which could be related to earlier puberty in overweight females (growth spurt with increased volumes). Compared with normal weight children, overweight and obese children had reduced lung volume ratios (functional residual capacity/total lung capacity (TLC) and residual volume/TLC), no evidence of airflow limitation and similar symptoms.In conclusion, the observed functional relationships with BMI are not specific to asthma and being overweight is not associated with significant clinical impacts on asthma during childhood.
Titrated oxygen requirement and prognostication in idiopathic pulmonary fibrosis
Authors: Hook J.L. , Arcasoy S.M. , Zemmel D. , Bartels M.N. , Kawut S.M. , Lederer D.J. 
Published: September 2011

The supplemental oxygen flow rate is a common bedside measure of gas exchange impairment. We aimed to determine whether a titrated oxygen requirement (TOR) predicted mortality in idiopathic pulmonary fibrosis (IPF).We examined 104 adults with IPF enrolled in a prospective cohort study and a validation cohort of 151 adults with a variety of interstitial lung diseases (ILDs). The TOR was defined as the lowest oxygen flow rate required to maintain an oxyhaemoglobin saturation of 96% while standing. Cox proportional hazards models and time-dependent receiver operating characteristic curves were used to examine survival time.A higher TOR was associated with a greater mortality rate independent of forced vital capacity and 6-min walk test results in IPF (adjusted hazard ratio (per 1 L·min−1) 1.16, 95% CI 1.06–1.27). The TOR was at least as accurate as pulmonary function and 6-min walk testing at predicting 1-yr mortality. Findings were similar in other ILDs.The TOR is a simple, inexpensive bedside measurement that aids prognostication in IPF.
Neutrophil chemotaxis in granulomatosis with polyangiitis (Wegener’s) and idiopathic pulmonary fibrosis
Authors: Richter A.G. , Perkins G.D. , Chavda A. , Sapey E. , Harper L. , Thickett D.R. 
Published: September 2011

The presence of antineutrophil cytoplasmic antibodies in granulomatosis with polyangiitis (Wegener’s) (GPA) implicates the neutrophil as a key effector cell. Previous studies have reported elevated neutrophil counts in the lung, although the determinants of neutrophil chemotaxis in the GPA lung are unknown.Bronchoalveolar lavage fluid (BALF) cell counts, myeloperoxidase (MPO) and chemokines were measured in 27 patients with GPA, 20 disease controls with idiopathic pulmonary fibrosis (IPF) and six healthy controls. CXC chemokine ligand (CXCL)8, interleukin (IL)-1β, epithelial neutrophil-activating protein 78, granulocyte colony-stimulating factor (G-CSF) and granulocyte-macrophage colony-stimulating factor were measured by ELISA. The neutrophil chemotactic potential of BALF was investigated using the under-agarose method, and specific antibodies were used to examine the role of CXCL8 and IL-1β.GPA BALF had an increased neutrophil percentage, and elevated MPO, CXCL8 and G-CSF concentrations compared with healthy controls. Chemotaxis of control neutrophils towards BALF from patients with active (p=0.006) and remission (p=0.077) GPA, and IPF (p=0.001) patients was increased compared with normal controls. BALF-induced chemotaxis correlated with BALF IL-1β (r=0.761, p=0.001) and CXCL8 (r=0.640, p=0.012) in GPA, and was inhibited by anti-CXCL8 (85%; p<0.001) and anti-IL-1β (69%; p<0.001).Our study confirms a neutrophilia and pro-inflammatory alveolar milieu that persists in clinical remission. CXCL8 and IL-1β appear to play important roles in the neutrophil chemotactic response to BALF.
MHC polymorphism and disease resistance to vibrio anguillarum in 8 families of half-smooth tongue sole (Cynoglossus semilaevis)
Authors: Du Min , Chen Song-lin , Liu Yan-hong , Liu Yang , Yang Jing-feng 
Published: September 2011

AbstractBackgroundGenes in the major histocompatibility complex (MHC) have a critical role in both the innate and adaptive immune responses because of their involvement in presenting foreign peptides to T cells. However, the nature has remained largely unknown.ResultsWe examined the genetic variation in MHC class IIB in half-smooth tongue sole (Cynoglossus semilaevis) after challenge withvibrio anguillarum. Two thousand and four hundred fry from 12 half-smooth tongue sole families were challenged withVibrio anguillarum. To determine any association between alleles and resistance or susceptibility toV. anguillarum, 160 individuals from four high-resistance (HR, < 40.55% mortality) families and four low-resistance (LR, > 73.27% mortality) families were selected for MHC IIB exon2 gene sequence analysis. The MHC IIB exon2 genes of tongue sole displayed a high level of polymorphism and were discovered at least four loci. Meanwhile, the dN/dS[the ratio of non-synonymous (dN) substitutions to synonymous (dS) substitutions] in the peptide-binding region (PBR) was higher than that in the non-peptide-binding region (non-PBR). Eighty-eight alleles were discovered among 160 individuals, and 13 out of 88 alleles were used to analyze the distribution pattern between the resistant and susceptible families. Certain alleles presented in HR and LR with a different frequency, while other alleles were discovered in only the HR or LR families, not both. Five alleles,Cyse-DBB*6501,Cyse-DBB*4002,Cyse-DBB*6102,Cyse-DBB*5601andCyse-DBB*2801, were found to be associated with susceptibility toV. anguillarumwith a frequency of 1.25%, 1.25%, 1.25%, 1.25% and 2.5% in the HR families, and 35%, 33.75%, 27.5%, 16.25%, 15% in the LR families (p< 0.01, 0.01, 0.01, 0.01, 0.01), respectively. Four alleles,Cyse-DBB*3301,Cyse-DBB*4701,Cyse-DBB*6801andCyse-DBB*5901, were found to be associated with resistance toV. anguillarum, with a frequency of 13.75%, 11.25%, 11.25%, 8.75% in the HR families and 1.25%, 1.25%, 1.25%, 1.25% and 1.25% in the LR families (p< 0.01, 0.05, 0.05 and p = 0.064), respectively.ConclusionsElucidation of the role of MHC II B genes in half-smooth tongue sole should prove to be helpful to the in-depth development of marker-assisted selective breeding in half-smooth tongue sole.
Temporal trends (1977-2007) and ethnic inequity in child mortality in rural villages of southern Guinea Bissau
Authors: Fazzio Ila , Mann Vera , Boone Peter 
Published: September 2011

Abstract

Background
Guinea Bissau is one of the poorest countries in the world, with one of the highest under-5 mortality rate. Despite its importance for policy planning, data on child mortality are often not available or of poor quality in low-income countries like Guinea Bissau. Our aim in this study was to use the baseline survey to estimate child mortality in rural villages in southern Guinea Bissau for a 30 years period prior to a planned cluster randomised intervention. We aimed to investigate temporal trends with emphasis on historical events and the effect of ethnicity, polygyny and distance to the health centre on child mortality.

Methods
A baseline survey was conducted prior to a planned cluster randomised intervention to estimate child mortality in 241 rural villages in southern Guinea Bissau between 1977 and 2007. Crude child mortality rates were estimated by Kaplan-Meier method from birth history of 7854 women. Cox regression models were used to investigate the effects of birth periods with emphasis on historical events, ethnicity, polygyny and distance to the health centre on child mortality.

Results
High levels of child mortality were found at all ages under five with a significant reduction in child mortality over the time periods of birth except for 1997-2001. That period comprises the 1998/99 civil war interval, when child mortality was 1.5% higher than in the previous period. Children of Balanta ethnic group had higher hazard of dying under five years of age than children from other groups until 2001. Between 2002 and 2007, Fula children showed the highest mortality. Increasing walking distance to the nearest health centre increased the hazard, though not substantially, and polygyny had a negligible and statistically not significant effect on the hazard.

Conclusion
Child mortality is strongly associated with ethnicity and it should be considered in health policy planning. Child mortality, though considerably decreased during the past 30 years, remains high in rural Guinea Bissau. Temporal trends also suggest that civil wars have detrimental effects on child mortality.

Trial Registration
Current Controlled Trials ISRCTN52433336
Tubular immunostimulating complex based on cucumarioside A2-2 and monogalactosyldiacylglycerol from marine macrophytes
Authors: Kostetsky Eduard Y , Sanina Nina M , Mazeika Andrey N , Tsybulsky Alexander V , Vorobyeva Natalia S , Shnyrov Valery L 
Published: September 2011

Abstract

Background
There is an urgent need to develop safe and effective adjuvants for the new generation of subunit vaccines. We developed the tubular immunostimulating complex (TI-complex) as a new nanoparticulate antigen delivery system. The morphology and composition of TI-complexes principally differ from the known vesicular immunostimulating complexes (ISCOMs). However, methodology for the preparation of TI-complexes has suffered a number of shortcomings. The aim of the present work was to obtain an antigen carrier consisting of triterpene glycosides from Cucumaria japonica, cholesterol, and monogalactosyldiacylglycerol from marine macrophytes with reproducible properties and high adjuvant activity.

Results
The cucumarioside A2-2 - cholesterol - MGalDG ratio of 6:2:4 (by weight) was found to provide the most effective formation of TI-complexes and the minimum hemolytic activity in vitro. Tubules of TI-complexes have an outer diameter of about 16 nm, an inner diameter of 6 nm, and a length of 500 nm. A significant dilution by the buffer gradually destroyed the tubular nanoparticles. The TI-complex was able to increase the immunogenicity of the protein antigens from Yersinia pseudotuberculosis by three to four times.

Conclusions
We propose an optimized methodology for the preparation of homogeneous TI-complexes containing only tubular particles, which would achieve reproducible immunization results. We suggest that the elaborated TI-complexes apply as a universal delivery system for different subunit antigens within anti-infectious vaccines and enhance their economic efficacy and safety.