bluuebunny/crossref_metadata_2025_split huggingface dataset:
p.Tyr365Cys change in factor VIII: haemophilia A, but not as we know it
Authors: Bowyer Annette E. , Goodeve Anne , Liesner Ri , Mumford Andrew D. , Kitchen Steve , Makris Mike 
Published: July 2011

SummaryHaemophilia A is caused by a reduction in clotting factor VIII (FVIII). FVIII coagulant activity (FVIII:C) can be measured by three methods; the one‐stage activated partial thromboplastin time‐based clotting assay, the two‐stage Xa generation‐based clotting assay and the chromogenic Xa generation‐based assay. The FVIII:C of most patients with haemophilia A are concordant regardless of the assay method employed. Up to a third of patients show assay discrepancy, usually with the two‐stage and chromogenic assays being much lower than the one‐stage assay. Very rarely, patients have been reported with lower one‐stage compared to two‐stage and chromogenic assays, but here we report that the mutation p.Tyr365Cys accounts for most of these patients and, at least in the UK, is not rare. We have identified this mutation in 23 different families. Affected male index individuals had a lower mean one‐stage FVIII:C of 27 iu/dl compared to two‐stage FVIII:C mean of 77 iu/dl. Affected individuals had minimal or absent bleeding symptoms and when these were present they were usually in patients with another co‐inherited bleeding disorder. Affected individuals often had surgery without the need to correct the one‐stage FVIII:C.
Short‐term warfarin reversal for elective surgery – using low‐dose intravenous vitamin K: safe, reliable and convenient*
Authors: Burbury Kate L. , Milner Alvin , Snooks Belinda , Jupe David , Westerman David A. 
Published: July 2011

SummaryPeri‐procedural management of warfarin reflects an intricate balance between the restoration of haemostasis and appropriate thromboprophylaxis. This prospective single‐arm study assessed the safety and efficacy of a convenient schedule, incorporating low‐dose intravenous vitamin K (vitKIV) for short‐term warfarin reversal prior to elective surgery, as well as vitK‐dependent factor levels (vitK‐Factors) and International Normalized Ratio (INR) pre‐ and post‐vitKIV. One seventy eight patients on long‐term warfarin received 3 mg vitKIV 12–18 h pre‐procedure with no adverse reactions. 167/178 (94%) achieved an INR ≤ 1·5 post‐vitKIV on the day of surgery, while all achieved INR ≤ 1·7. Four patients had procedure‐associated major bleeding, but importantly had achieved a pre‐procedure INR < 1·5 and vitK‐Factors >0·30 iu/ml. No patient suffered a symptomatic thromboembolism during the 6‐week follow‐up. Median days to re‐establish a therapeutic INR were 4 (range 2–11). VitKIV near normalized all vitK‐Factors, with a uniform pattern of depletion and repletion in association with an increase and decrease in INR, respectively; and from the data, INR < 1·5 correlated with vitK‐Factors >0·30 iu/ml. Low‐dose vitKIV for short‐term warfarin reversal was reliable and safe, and successfully lowered the INR to an acceptable level for planned surgery, with no excess of bleeding, thromboembolism, delayed discharge, or resistance to warfarin. The protocol was simple and convenient for both the patients and the healthcare institution.
Emotional availability and attachment across generations: variations in patterns associated with infant health risk status
Authors: Cassibba R. , van IJzendoorn M. H. , Coppola G. 
Published: July 2011

AbstractBackground  The presence of limits or distortions in the children's communicative behaviours (due to a chronic illness) may interfere with the possibility to build secure attachment relationships. Moreover, the distress that the atypical chronic illness condition brings to family life may interfere the intergenerational transmission of attachment.Methods  This study evaluated the associations between maternal attachment representations, emotional availability and mother–child attachment in a clinical and in a comparison group. Forty infants (23 female) in their 14th month of life and their mothers participated in this study, 20 dyads with clinical infants (10 premature infants and 10 infants affected by atopic dermatitis) and 20 full‐term and healthy comparison infants. The Adult Attachment Interview, the Emotional Availability Scales (EAS) and the Strange Situation Procedure were used to assess, respectively, the security of mothers' attachment representations, the emotional availability and the quality of mother–child attachment.Results  We found that the two groups (clinical vs. comparison) did not differ with respect to the Adult Attachment Interview and the Emotional Availability Scales measures. A significant difference was found in the distribution of the infant–mother attachment patterns, with a higher incidence of insecure infants in the clinical group. In the typically developing group, more secure maternal attachment representations predicted more emotional availability in mother–infant interactions, which predicted more secure infant–mother attachments. However, we did not find similar support for intergenerational transmission of attachment in the clinical group.Conclusions  We speculate that constant concerns about the child's health condition and communicative difficulties of clinical infants may hamper or even mitigate the intergenerational transmission of attachment.
Cerebral palsy registers and high‐quality data: an evaluation of completeness of the4Childregister using capture–recapture techniques
Authors: Surman G. , da Silva A. A. M. , Kurinczuk J. J. 
Published: July 2011

AbstractBackground As the survival of very preterm and low‐birthweight infants increases, so does the importance of monitoring the birth prevalence of childhood impairments; disease registers provide a means to do so for these rare conditions. High levels of ascertainment for disease research registers have become increasingly difficult to achieve in the face of additional challenges posed by consent and confidentiality issues.4Child –Four Counties Database of Cerebral Palsy, Vision Loss and Hearing Loss in Children has been collecting data and monitoring these three major childhood impairments since 1984.Methods This study used capture–recapture and related techniques to identify areas which are particularly affected by low ascertainment, to estimate the magnitude of missing cases on the4Childregister and to provide birth prevalence estimates of cerebral palsy which allow for these missing cases.Results Estimates suggest that while overall around 27% of cerebral palsy cases were not reported to4Child, ascertainment for severely motor‐impaired children (93% complete) and those born in two of the four counties was good (Oxfordshire: 90%, Northamptonshire: 94%). After allowing for missing cases, adjusted estimates of cerebral palsy birth prevalence for 1984–1993 were 3.0 per 1000 live births versus 2.5 per 1000 live births in 1994–2003.Conclusions Capture–recapture techniques can identify areas of poor ascertainment and add to information around the provision of cerebral palsy birth prevalence estimates. Despite variation in ascertainment over time, capture–recapture estimates supported a decline in cerebral palsy birth prevalence between the earlier and later study periods in the four English counties of the geographical area covered by4Child.
Developing a programme for healthy growth and nutrition during infancy: understanding user perspectives
Authors: Lakshman R. , Landsbaugh J. R. , Schiff A. , Cohn S. , Griffin S. , Ong K. K. 
Published: July 2011

AbstractBackground  Avoiding excess energy intake and rapid weight gain during infancy may be effective in preventing childhood obesity. We developed a programme for healthy growth and nutrition in formula milk‐fed babies. The aim of this study was to understand users' perspectives about the programme and planned trial.Methods  We conducted three focus group discussions (10 mothers) and nine individual interviews (seven health visitors, one midwife and one mother) discussing the programme materials and trial protocol. All sessions were transcribed verbatim and a thematic analysis was performed using the framework approach.Results  Mothers reported receiving conflicting messages about infant feeding and were keen for consistent advice. They welcomed the support that the programme would offer to mothers who gave their babies formula milk, but some were sceptical about the feasibility of limiting formula milk quantities. They suggested that recommended quantities should be presented as general guidelines rather than rigid rules. Some mothers said that it was too early to intervene to prevent obesity, that babies could not be overfed and that the risks of formula milk feeding had been exaggerated. Because of the routine advice to feed on demand, babies were fed in response to crying, and crying was equated with ‘hunger’. Some mothers said that growth was genetically determined so they ignored the growth charts. Health visitors used the growth charts to assess adequate weight gain rather than to identify excess weight gain. Health visitors said that mothers would need a lot of education and support to limit formula milk quantities.Conclusions  Efforts to prevent childhood obesity by avoiding excess weight gain during infancy have to address mothers' beliefs that babies cannot be overfed, that crying always signals hunger and that growth is determined by genes rather than nutrition. Mothers and healthcare providers have different motivations and understanding these are important in the development of any intervention.
The neuropeptide calcitonin gene‐related peptide affects allergic airway inflammation by modulating dendritic cell function
Authors: Rochlitzer S. , Veres T. Z. , Kühne K. , Prenzler F. , Pilzner C. , Knothe S. , Winkler C. , Lauenstein H.‐D. , Willart M. , Hammad H. , Müller M. , Krug N. , Lambrecht B. N. , Braun A. 
Published: July 2011

Summary
Background The neuropeptide calcitonin gene‐related peptide (CGRP) is released in the lung by sensory nerves during allergic airway responses. Pulmonary dendritic cells (DC) orchestrating the allergic inflammation could be affected by CGRP.
Objective To determine the immunomodulatory effects of CGRP on DC function and its impact on the induction of allergic airway inflammation.
Methods CGRP receptor expression on lung DC was determined by RT‐PCR and immunofluorescence staining. The functional consequences of CGRP receptor triggering were evaluated in vitro using bone marrow‐derived DC. DC maturation and the induction of ovalbumin (OVA)‐specific T cell responses were analysed by flow cytometry. The in vivo relevance of the observed DC modulation was assessed in a DC‐transfer model of experimental asthma. Mice were sensitized by an intrapharyngeal transfer of OVA‐pulsed DC and challenged with OVA aerosol. The impact of CGRP pretreatment of DC on airway inflammation was characterized by analysing differential cell counts and cytokines in bronchoalveolar lavage fluid (BALF), lung histology and cytokine responses in mediastinal lymph nodes.
Results RT‐PCR, immunofluorescence and cAMP assay demonstrated the expression of functionally active CGRP receptors in lung DC. RT‐PCR revealed a transcriptional CGRP receptor down‐regulation during airway inflammation. CGRP specifically inhibited the maturation of in vitro generated DC. Maturation was restored by blocking with the specific antagonist CGRP8−37. Consequently, CGRP‐pretreated DC reduced the activation and proliferation of antigen‐specific T cells and induced increased the numbers of T regulatory cells. The transfer of CGRP‐pretreated DC diminished allergic airway inflammation in vivo, shown by reduced eosinophil numbers and increased levels of IL‐10 in BALF.
Conclusions and Clinical Relevance CGRP inhibits DC maturation and allergen‐specific T cell responses, which affects the outcome of the allergic airway inflammation in vivo. This suggests an additional mechanism by which nerve‐derived mediators interfere with local immune responses. Thus, CGRP as an anti‐inflammatory mediator could represent a new therapeutic tool in asthma therapy.
Cite this as: S. Rochlitzer, T. Z. Veres, K. Kühne, F. Prenzler, C. Pilzner, S. Knothe, C. Winkler, H.‐D. Lauenstein, M. Willart, H. Hammad, M. Müller,
N. Krug, B. N. Lambrecht and A. Braun, Clinical & Experimental Allergy, 2011 (41) 1609–1621.
Glucose activation of islets of Langerhans up-regulates Toll-like receptor 5: possible mechanism of protection
Authors: Weile C , Josefsen K , Buschard K 
Published: July 2011

Summary
Toll-like receptors are pattern-recognition receptors of the innate immune system that are activated during viral, bacterial or other infections, as well as during disease progression of type 1 and type 2 diabetes. Toll-like receptor 5 (TLR-5) specifically recognizes bacterial infection through binding of flagellin from pathogenic bacteria such as Salmonella and Listeria species. We have found that the expression of TLR5 is up-regulated by glucose activation of isolated islets of Langerhans, in contrast to other investigated TLRs (TLR-2, -3, -4, -6 and -9. Stimulation of islets with 10 mm glucose increased the levels of TLR5 mRNA 10-fold (P = 0·03) and the TLR-5 protein levels twofold (P = 0·04). Furthermore, the protein level of downstream signalling molecule myeloid differentiation primary response gene 88 (MyD88) increased 1·6-fold (P = 0·01). Activation of TLR-5 in islets lead to a marked reduction of both stimulated and basal secretion of insulin, as well as an increase in production of nitric oxide, proinflammatory cytokines, anti-inflammatory heat-shock protein and major histocompatibility complex (MHC) class I transporter. We observe no effects of TLR-5 activation on islet survival. We suggest that this regulation by TLR-5 might be beneficial during serious infection such as sepsis by limiting the activity of beta cells during peaks of insulin demand to counteract beta cell damage.
Endotoxemia affects citrulline, arginine and glutamine bioavailability
Authors: Elwafi Fatimazahra , Curis Emmanuel , Zerrouk Naima , Neveux Nathalie , Chaumeil Jean‐Claude , Arnaud Philippe , Cynober Luc , Moinard Christophe 
Published: July 2011

Eur J Clin Invest 2012; 42 (3): 282–289AbstractBackground  Sepsis considerably alters the intestinal barrier functions, which in turn modify the absorption and bioavailability of nutrients. However, the effects of septic shock on aminoacid (AAs) bioavailability are poorly documented. The aim of this study was to compare the bioavailability of citrulline, arginine and glutamine during endotoxemia.Materials and methods  Thirty‐six rats were randomised into two groups: control and lipopolysaccharides (LPS). The LPS group received an intraperitoneal injection of endotoxins (7·5 mg/kg). After 12 h, each group was again randomised into three subgroups, each of which received an oral bolus of citrulline, arginine or glutamine (5·7 mmol/kg). Blood samples were collected at various times from 0 to 600 min after AA administration. The concentrations of citrulline, arginine, glutamine and their metabolites arginine and ornithine were measured to determine pharmacokinetic parameters Area Under Curve (AUC), Cmax and Tmax.Results  The AUC values of citrulline decreased in LPS rats [citrulline, control: 761 ± 67 and LPS: 508 ± 72 μmol min/mL (P = 0·02)]. Maximum concentrations of citrulline were also significantly decreased by endotoxemia (P = 0·01). The pharmacokinetic parameters of arginine and glutamine were not significantly modified by endotoxemia. The AUC value of arginine from citrulline conversion was diminished in endotoxemic rats. The other pharmacokinetic parameters of arginine were not significantly modified after arginine or citrulline supply in either group (control or LPS).Conclusion  Endotoxemia affects the bioavailability of AAs differently according to the amino acid considered. This feature may be important for nutritional strategy in ICU patients.
Dietary fenugreek and onion attenuate cholesterol gallstone formation in lithogenic diet–fed mice
Authors: Reddy Raghunatha R. L. , Srinivasan Krishnapura 
Published: July 2011

SummaryAn animal study was conducted to evaluate the antilithogenic effect of a combination of dietary fenugreek seeds and onion. Lithogenic conditions were induced in mice by feeding them a high (0.5%) cholesterol diet (HCD) for 10 weeks. Fenugreek (12%) and onion (2%) were included individually and in combination in this HCD. Fenugreek, onion and their combination reduced the incidence of cholesterol gallstones by 75%, 27% and 76%, respectively, with attendant reduction in total cholesterol content by 38–42%, 50–72% and 61–80% in serum, liver and bile respectively. Consequently, the cholesterol/phospholipid ratio was reduced significantly in serum, liver and bile. The cholesterol saturation index of bile was reduced from 4.14 to 1.38 by the combination of fenugreek and onion and to 2.33 by onion alone. The phospholipid and bile acid contents of the bile were also increased. Changes in the hepatic enzyme activities (3‐hydroxy‐3‐methylglutaryl Coenzyme A reductase, cholesterol‐7α‐hydroxylase and cholesterol‐27‐hydroxylase) induced by HCD were countered by fenugreek, onion and their combination. Hepatic lipid peroxides were reduced by 19–22% and 39–45% with fenugreek, onion and their combination included in the diet along with the HCD. Increased accumulation of fat in the liver and inflammation of the gallbladder membrane produced by HCD were reduced by fenugreek, onion and their combination. The antilithogenic influence was highest with fenugreek alone, and the presence of onion along with it did not further increase this effect. There was also no additive effect of the two spices in the recovery of antioxidant molecules or in the antioxidant enzyme activities.
Do nurses have a different physical health profile? A systematic review of experimental and observational studies on nurses’ physical health
Authors: Fronteira Inês , Ferrinho Paulo 
Published: July 2011

Aim. To review the evidence on nurses’ health systematically.Background. Nurses are one of the most important resources of a health system. They are subjected to biological, socio‐economic, cultural and health system factors that determine their health. Although mental and physical health problems seem to prevail among nurses, literature is often contradictory. The literature on health styles, health behaviours and self perceived health of nurses is also unclear.Design. Systematic review of experimental and observational studies on nurses’ health.Methods. Forty‐three databases searched. 2692 references identified as of potential interest; 187 studies included. Only the results from studies on physical health are presented. No meta‐analysis was possible. The Scottish Intercollegiate Guidelines Network system was used to rate evidence.Results. Nurses were at greater risk of musculoskeletal injuries and more prone to blood‐borne pathogens infections than other health care workers. They were at greater risk of breast cancer than other female health care workers. Nurses in hospitals were more at risk of tuberculosis. They did not have excess risk of cancer in general, Hodgkin’s disease, stomach, colon, rectum, pancreatic, ovary, kidney, brain or thyroid cancer or of lymphosarcoma. They have a higher prevalence of occupational allergies than clerical workers.Discussion. Nurses suffer more from musculoskeletal disorders which is consistent with the nature of nursing work. They are at greater risk of acquiring tuberculosis, particularly if they work in pulmonary, medicine or Human Immunodeficiency Virus wards which might be due to repeated contact with infected patients. Nurses are also more prone to blood‐borne pathogens infections which might be explained by exposure while working. The proneness to occupational allergies can be explained by exposure to a series of chemical agents.Relevance to clinical practice. Without sound knowledge on nurses’ health and it is not possible to develop specific occupational health programmes.
Patterns of morning and evening fatigue among adults with HIV/AIDS
Authors: Lerdal Anners , Gay Caryl L , Aouizerat Bradley E , Portillo Carmen J , Lee Kathryn A 
Published: July 2011

Aims and objectives.  Describe patterns of morning and evening fatigue in adults with HIV and examine their relationship to demographic and clinical factors and other symptoms.Background.  Most studies of HIV‐related fatigue assess average levels of fatigue and do not address its diurnal fluctuations. Patterns of fatigue over the course of the day may have important implications for assessment and treatment.Design.  A cross‐sectional, correlational design was used with six repeated measures over 72 hours.Method.  A convenience sample of 318 HIV‐infected adults was recruited in San Francisco. Socio‐demographic, clinical and symptom data were collected with questionnaires. CD4+ T‐cell count and viral load were obtained from medical records. Participants completed a four‐item version of the Lee Fatigue Scale each morning and evening for three consecutive days. Participants were grouped based on their diurnal pattern of fatigue (high evening only, high morning only, high morning and evening and low morning and evening). Group comparisons and logistic regression were used to determine the unique predictors of each fatigue pattern.Results.  The high evening fatigue pattern was associated with anxiety and the high morning pattern was associated with anxiety and depression. The morning fatigue pattern showed very little fluctuation between morning and evening, the evening pattern showed the largest fluctuation. The high morning and evening pattern was associated with anxiety, depression and sleep disturbance and this group reported the most fatigue‐related distress and interference in functioning.Conclusions.  These results provide initial evidence for the importance of assessing the patient’s daily pattern of fatigue fluctuation, as different patterns were associated with different symptom experiences and perhaps different aetiologies.Relevance to clinical practice.  Different fatigue patterns may benefit from tailored intervention strategies. Management of depressive symptoms could be tested in patients who experience high levels of morning fatigue.