bluuebunny/crossref_metadata_2025 huggingface dataset:
Mannose-binding lectin does not act as an acute-phase reactant in adults with community-acquired pneumococcal pneumonia
Authors: Perez-Castellano M , Peñaranda M , Payeras A , Milà J , Riera M , Vidal J , Pujalte F , Pareja A , Villalonga C , Matamoros N
Published: June 2006

The objective of this work was to study the role of mannose-binding lectin (MBL) and C-reactive protein (CRP) in pneumococcal pneumonia, to determine whether MBL acts as an acute-phase reactant and whether the severity of the disease correlates with MBL levels. The study comprised 100 patients with pneumococcal pneumonia. The pneumonia severity score was calculated and graded into a risk class of mortality (Fine scale). The MBL genotypes and the levels of MBL and CRP at the acute and recovery phases were determined. Fifty patients with the wild-type MBL genotype showed higher MBL levels in each phase (P < 0·001) and an increased risk to developing bacteraemia, odds ratio (OR) 2·74, 95% confidence interval (CI) 1·01–7·52) (P = 0·02), but this did not correlate with the pneumonia severity class. CRP levels in the acute phase, 79·53 mg/l [standard deviation (s.d.) 106·93], were higher in the subjects with positive blood cultures (P = 0·003), and remained higher [20·12 mg/l (s.d. 31·90)] in the group of patients with an underlying disease (P = 0·01). No correlation was observed between the levels of MBL and CRP in each phase, or with the pneumonia severity score. We cannot conclude that MBL acts uniformly as an acute-phase reactant in pneumococcal pneumonia. MBL levels do not correlate well with the severity of the pneumonia. The risk of developing bacteraemia could be enhanced in individuals with the wild-type MBL genotype.
CD134 expression on CD4+ T cells is associated with nephritis and disease activity in patients with systemic lupus erythematosus
Authors: Patschan S , Dolff S , Kribben A , Dürig J , Patschan D , Wilde B , Specker C , Philipp T , Witzke O
Published: June 2006

Systemic lupus erythematosus (SLE) is characterized by a deviation of the immune system that involves T cell-dependent autoantibody production. The aim of this study was to investigate the role of co-stimulatory markers on T cells in this disease. Twenty-eight patients with SLE as defined by the American College of Rheumatology (ACR) criteria and 11 healthy controls were included into the study. Eleven patients had biopsy-proven lupus nephritis while 17 patients had no clinical evidence of lupus nephritis. Clinical disease activity was assessed according to the systemic lupus erythematosus disease index (SLEDAI). CD4+ T cell populations in the peripheral blood were analysed for the expression of co-stimulatory markers CD45RO, CD70, CD80, CD86, CD137, CD137L, CD134, CD152, CD154 and ICOS. SLE patients showed an increased frequency of peripheral CD4+ T cells expressing high levels of CD80, CD86 and CD134 compared to healthy controls (7·1 ± 1·5%versus 1·7 ± 0·9%; P < 0·005; 2·3 ± 0·4%versus 1·0 ± 0·2%; P = 0·008, 20·2 ± 2·0%versus 10·6 ± 1·9%; P < 0·005, respectively). Significantly higher levels of CD80 on CD4+ T cells were detected in SLE patients with lupus nephritis compared to patients without nephritis (11·9 ± 3·3%versus 4·0 ± 0·7%; P < 0·005). There was an increased presence of CD134+ CD4+ cells in SLE patients with lupus nephritis (27·5 ± 4·0%versus 15·5 ± 1·3%; P < 0·005). CD80 and CD134 expression was significantly correlated with SLEDAI (r = 0·42, P = 0·03; r = 0·56, P < 0·005). Co-stimulatory molecules on CD4+ T cells are associated with renal disease and disease activity in patients with systemic lupus erythematosus.
Echinococcus multilocularis metacestodes modulate cellular cytokine and chemokine release by peripheral blood mononuclear cells in alveolar echinococcosis patients
Authors: Hübner M P , Manfras B J , Margos M C , Eiffler D , Hoffmann W H , Schulz-Key H , Kern P , Soboslay P T
Published: June 2006

Infection with the cestode Echinococcus multilocularis causes human alveolar echinococcosis (AE), a life-threatening disease affecting primarily the liver. Despite the severity of AE, clinical symptoms often develop only many years after infection, which suggests that E. multilocularis has developed mechanisms which depress anti-parasite immune response, thus favouring immune evasion. In this study we examined the production of cytokines, chemokines and the expression of CD molecules on peripheral blood mononuclear cells (PBMC) from AE patients and healthy controls in response to E. multilocularis metacestode culture supernatant, viable E. multilocularis vesicles and E. multilocularis vesicle fluid antigen in vitro. After 48 h of co-culture, E. multilocularis metacestode culture supernatant and E. multilocularis vesicles depressed the release of the proinflammatory cytokine interleukin (IL)-12 by PBMC. This effect was dose-dependent and a suppression of tumour necrosis factor (TNF)-α and IL-12 was observed even when PBMC were activated with lipopolysaccharide (LPS). Comparing proinflammatory cytokine release by AE patients and controls showed that the release of IL-12 and TNF-α was reduced in AE patients, which was accompanied by an increased number of CD4+ CD25+ cells and a reduced release of the Th2 type chemokine CCL17 (thymus and activation regulated chemokine, TARC), suggesting an anti-inflammatory response to E. multilocularis metacestode in AE patients. Instead the production of interferon (IFN)-γ and the expression of CD28 on CD4+ T cells were increased in PBMC from AE patients when compared to controls. This was accompanied by a higher release of the Th2-type chemokine CCL22 (macrophage derived chemokine, MDC) supporting that E. multilocularis also generates proinflammatory immune responses. These results indicate that E. multilocularis antigens modulated both regulatory and inflammatory Th1 and Th2 cytokines and chemokines. Such a mixed profile might be required for limiting parasite growth but also for reducing periparasitic tissue and organ damage in the host.
Highly active anti-retroviral therapy (HAART)-induced maintenance of adaptive but not innate immune parameters is associated with protection from HIV-induced mortality
Authors: Stebbing J , Bower M , Mandalia S , Nelson M , Gazzard B
Published: June 2006

Immunosuppression induced by the human immunodeficiency virus (HIV-1) increases the risk of death. We measured the influence of immunological and virological factors and the type of highly active anti-retroviral therapy (HAART) on this risk. Adaptive (lymphocyte) and innate (natural killer) immune correlates and maximum HIV viral loads were assessed for association with mortality using univariate and multivariate analyses. The protective effect of HAART regimens, containing protease inhibitors (PI) and/or non-nucleoside reverse transcriptase inhibitors (NNRTI) on mortality were also examined in a prospectively recorded cohort of 9621 HIV-infected individuals. From this entire cohort, 5873 HIV infected individuals (61%) have been followed-up in the HAART era and of these 499 (8·5%) have died. In multivariate analyses, CD4 counts below the 50th centile and CD8 and CD19 counts below the 25th centile were significantly associated with mortality, as was increased age (P < 0·001). Innate immune subset levels had no effect on mortality. A maximum HIV viral load greater than the 75th centile was also associated independently with mortality (P < 0·035). Exposure to either a PI or an NNRTI-containing HAART regimen, or both together, was protective against death compared with no anti-retrovirals (P < 0·001). Effective HAART-induced maintenance of the adaptive immune system (CD4, CD8 and CD19 counts) protects from HIV-related mortality.
Levels of serum chemokines discriminate clinical myelopathy associated with human T lymphotropic virus type 1 (HTLV-1)/tropical spastic paraparesis (HAM/TSP) disease from HTLV-1 carrier state
Authors: Guerreiro J B , Santos S B , Morgan D J , Porto A F , Muniz A L , Ho J L , Teixeira A L , Teixeira M M , Carvalho E M
Published: June 2006

Approximately 5% of people infected with human T lymphotropic virus type 1 (HTLV-1) develop clinical myelopathy or tropical spastic paraparesis (HAM/TSP) that is associated with high-levels of Th1 cytokines, interferon (IFN)-γ and tumour necrosis factor (TNF)-α. Chemokines are known to induce cytokine secretion and direct the trafficking of immune cells to sites of disease. The present study measured serum chemokines correlated with autonomously released IFN-γ in cell cultures. HTLV-1 infection was defined by enzyme-linked immunosorbent assay (ELISA) and confirmed by Western blot. Subjects included HTLV-1 carriers (n = 56), patients with HAM/TSP (n = 31) and healthy HTLV-1 seronegative volunteer controls (n = 20). Serum chemokines and IFN-γ autonomously released by mononuclear cells in culture were quantified by ELISA. Compared to HTLV-1 carriers, serum chemokines in HAM/TSP patients showed significantly increased levels of CXCL9 and CXCL10, significantly diminished levels of CCL2 and similar amounts of CCL11 and CCL24. In contrast, CCL11 and CCL24 were significantly lower in serum of HAM/TSP patients than either control. IFN-γ was positively correlated with CXCL9 and CXCL10 when HAM/TSP and HTLV-1 carriers were used as a combined group. However, despite a large proportion of HTLV-1 carriers having high IFN-γ levels, these chemokines were not increased in carriers. This study showed that high levels of CXCL9 and CXCL10 in the systemic circulation and low serum CCL2 levels are features of HAM/TSP. HTLV-1 infection and Tax and/or additional viral encoded factor-mediated pathological processes triggering T cell activation with autogenous IFN-γ release are probably involved in regulating chemokine release.
Association between mannan-binding lectin and impaired lung function in cystic fibrosis may be age-dependent
Authors: Muhlebach M S , MacDonald S L , Button B , Hubbard J J , Turner M L , Boucher R C , Kilpatrick D C
Published: June 2006

An association between mannan-binding lectin (MBL) status and severity of lung function impairment in cystic fibrosis (CF) has been found in several studies, but not in others. To explore the possible basis for discrepancies in the literature, we related both MBL and l-ficolin concentrations to lung function and examined the results in relation to the age of the patients. For patients under 15 years of age, those with MBL < 200 ng/ml had better lung function than those with MBL > 200 ng/ml [median forced expiratory volume in 1 s (FEV1), 99%versus 83%; P = 0·05]. For patients over 15 years of age, those with MBL < 200 ng/ml had poorer lung function than those with MBL > 200 ng/ml (median FEV1, 44%versus 55%; P = 0·1). Also, for the over 15-year-olds, the proportion of patients with FEV1 values below the median was greater in the MBL-insufficient subgroup (P < 0·04). In other words, relative deficiency of MBL appears to accelerate the age-related decline in lung function in CF patients. No corresponding relationships could be found between l-ficolin concentration and lung function. These findings and interpretation lend support to the potential value of MBL replacement therapy in a small minority of cystic fibrosis patients.
Growth hormone treatment and adverse events in Prader–Willi syndrome: data from KIGS (the Pfizer International Growth Database)
Authors: Craig Maria E. , Cowell Christopher T. , Larsson Pontus , Zipf William B. , Reiter Edward O. , Albertsson Wikland Kerstin , Ranke Michael B. , Price David A. ,   on behalf of the KIGS International Board
Published: June 2006

Objective  To evaluate the response to recombinant GH treatment and adverse events in children with Prader–Willi syndrome (PWS) from KIGS, the Pfizer International Growth Database.

Patients  A total of 328 children (274 prepubertal, median age 6·0 years; 54 pubertal, median age 12·7 years) were treated for 1 year and 161 children were treated for 2 years with GH.

Results   Height standard deviation score (SDS) increased significantly during treatment; the response was greater in prepubertal (−0·7 vs.−1·8 pretreatment) compared with pubertal children (−1·5 vs.−1·8). Predictors of first‐year height velocity in multiple regression analysis were GH dose, body weight (positively correlated), height SDS minus mid‐parental height SDS and chronological age (negatively correlated), together accounting for 39% of the variation in response to GH. Body mass index (BMI) SDS did not change significantly during 2 years of treatment. Of all the 675 GH‐treated PWS patients in KIGS, there were five cases of sudden death (age range 3–15 years). Three were obese (weight for height > 200%) and causes of death included bronchopneumonia, respiratory insufficiency and sleep apnoea. Scoliosis was the most commonly reported adverse event (n = 24), four children developed hyperglycaemia and six had presumptive diabetes (type 2 in five, and one case of type 1).

Conclusions   Short‐term growth improved in response to conventional doses of GH in children with PWS. Prior to commencement of GH, examination of the upper airways and sleep studies should be performed in PWS patients. GH should be used with caution in those with extreme obesity or disordered breathing and all patients should be closely monitored for adverse events.
Lifestyle factors and forearm bone density in young Greek men
Authors: Kyriazopoulos P. , Trovas G. , Charopoulos J. , Antonogiannakis E. , Galanos A. , Lyritis G
Published: June 2006

Objective  The aim of this study was to evaluate the effects of dietary factors (calcium, proteins, alcohol, coffee and tea intake), exercise, sunlight exposure and immobilization on bone mineral content (BMC) and bone mineral density (BMD) in young men.

Patients and measurements  We examined a group of 300 healthy men, aged 18–30. Mean weight was 80‐81 kg (53–125 kg range) and height 179 cm (160–195 cm range). Distal BMC (dBMC), distal BMD (dBMD) and ultradistal BMD (udBMD) at the radius were measured by single X‐ray absorptiometry (Osteometer DTX). The data concerning lifestyle factors were obtained through a questionnaire. The 300 men were divided in four groups according to calcium intake, four groups taking into account protein and three groups alcohol intake. There also were five groups of exercise level, six groups of sun exposure and two groups of duration of immobilization.

Results  In the group with the lowest levels of calcium intake (< 400 mg/day), dBMD and udBMD were lower than in the other groups of calcium intake (P = 0·002). dBMC and udBMD were lower (P = 0·043 and 0·015, respectively) in subjects with low physical activity (< 2 h/week), whereas dBMC and udBMD were higher (P < 0·0005) in subjects with frequent sun exposure (group labelled ‘very often’). Multiple regression analysis on bone mineral density of the forearm showed that, calcium intake, exercise and sunlight were also independent predictors of bone mass. No significant correlation between the other examined factors and BMD or BMC was detected.

Conclusions  Calcium intake, exercise level and sun exposure showed a statistically significant correlation with distal BMD and BMC in young adult men.
Presentation and evolution of organic central precocious puberty according to the type of CNS lesion
Authors: Trivin Christine , Couto‐Silva Ana‐Claudia , Sainte‐Rose Christian , Chemaitilly Wassim , Kalifa Chantal , Doz François , Zerah Michel , Brauner Raja
Published: June 2006

Objective  To evaluate the influence of the type and treatment of CNS lesion causing central precocious puberty (CPP) on the presentation, hypothalamic‐pituitary function and final height.

Patients  One hundred patients with CPP caused by central nervous system (CNS) lesion.

Results  The CPP was the presenting symptom of the lesion in 25 (10 boys) and occurred in 75 patients (23 boys) previously treated for lesions. These were optic glioma or astrocytoma (n = 45), hydrocephalus (n = 22), hypothalamic hamartoma (n = 15), suprasellar arachnoid cyst (n = 10) and others (n = 8).

The percentages of patients with increased height, bone age advance, testicular volume, LH/FSH peaks ratio after gonadotrophin‐releasing hormone (GnRH) test and plasma testosterone concentration in boys and oestradiol in girls varied from one aetiology to another. The boys with hamartoma were significantly taller and had greater bone age advance, LH peak and testosterone than boys with optic glioma. The girls with hamartoma and suprasellar arachnoid cyst were significantly younger and had greater LH peak than girls in the other groups.

All patients treated for optic glioma had hypothalamic–pituitary deficiencies, including GH (100%), thyrotrophin (71·4%), corticotrophin (12·5%) and pubertal (34·3%) deficiencies. Sixty percent of those with suprasellar cysts lacked GH.

Final height was below −2 SD in 15/59 (25%) patients, including 5/11 not treated with GnRH analogue, 3/5 not treated with GH despite GH deficiency, and 2 with hydrocephalus as a result of meningomyelocele.

Conclusions  The type of CNS lesion influences the presentation of CPP. This is probably caused by differences in the mechanisms inducing puberty and to the hypothalamic‐pituitary deficiencies associated with the CPP as a result of a lesion and/or its treatment.
A retrospective postlicensure survey of FEIBA efficacy and safety
Authors: DIMICHELE D. , NÉGRIER C.
Published: June 2006

Summary.   Patients with haemophilia who develop inhibitors have unique treatment needs; bypassing agents such as Factor Eight Inhibitor Bypassing Activity, Anti‐Inhibitor Coagulant Complex (FEIBA; Baxter AG, Vienna, Austria) are part of this therapeutic armamentarium. This study sought to increase comprehension of the full therapeutic profile of FEIBA by evaluating its safety and efficacy in the settings of acute bleeding, surgery, and prophylaxis. Information was collected through a postmarketing surveillance study; questionnaire booklets were distributed to 72 treatment centers in the United States and Europe. The booklets contained questions related to patient demographics, inhibitor titre determinations, and FEIBA treatment. Information comprising 200 FEIBA treatment periods and representing >4500 infusions was available for 63 patients with inhibitors (n = 60, haemophilia A; n = 3, haemophilia B). Twelve patients were in more than one treatment group. Efficacy was determined by a subjective global evaluation and was good or excellent in 82% of all acute, and 91% of all surgical, treatments. Additionally, prophylactic treatment resulted in improved or stabilised clinical orthopaedic status in 11 of 13 patients (85%). Based on available data, FEIBA was judged safe in all treatment situations by the small number of adverse events (<0.04%). No thrombotic complications occurred during any treatment episode. Results indicated that FEIBA was safe and effective in acute, surgical, and prophylactic treatment settings, supporting the utility of FEIBA as a treatment option for patients with inhibitors. However, prospective studies are advised.
Spinal epidural haematoma in a patient with haemophilia B
Authors: BALKAN C. , KAVAKLI K. , KARAPINAR D.
Published: June 2006

Summary.  Spinal epidural haematoma (SEH) is a rare complication in haemophiliacs. We report the case of a 17‐year‐old boy with severe haemophilia B who presented with acute onset of neck/back pain, walking impairment and urinary retention because of an extensive SEH. The haematoma was identified by magnetic resonance imaging of the spinal column. Prompt and aggressive treatment with factor IX concentrate led to complete recovery at 3 weeks. This case calls attention to the clinical manifestation, radiological features and management options of the rarely reported SEH in haemophiliacs. Despite evidence of extensive SEH, factor replacement therapy and a multidisciplinary team approach result in complete neurological recovery without the need for surgical decompression.
Liver haemorrhage in haemophilia – a case report and review of the literature
Authors: HAMILTON M. , FRENCH W. , RHYMES N. , COLLINS P.
Published: June 2006

Summary.  We present two patients with severe haemophilia A, who presented with haemorrhagic shock, secondary to a liver haematoma in the neonatal period. Both required emergency treatment and diagnosis of the hereditary coagulation defect. Major organ bleeding in severe haemophilia A in the newborn period is rare, and this unusual complication is not well recognized. Failure to recognize that the bleeding is due to a bleeding disorder, particularly in the absence of a family history, may lead to delay in appropriate management.
The Haemo‐QoL Index: developing a short measure for health‐related quality of life assessment in children and adolescents with haemophilia
Authors: POLLAK E. , MÜHLAN H. , VON MACKENSEN S. , BULLINGER M. ,   THE HAEMO‐QOL GROUP
Published: June 2006

Summary.  As quality of life (QoL) research is increasingly focusing on children and adolescents with haemophilia, the need for both age‐appropriate and disease‐specific assessment tools becomes apparent. Therefore, a set of questionnaires measuring QoL in children and adolescents with haemophilia was simultaneously developed in six languages within the European Haemo‐QoL project (Haemophilia, 8, 2002, 47; Haemophilia, 10, 2004, 17). For implementation in larger studies and for use in daily clinical routine, a both short and psychometrically robust version of the questionnaire is needed. Using from the Haemo‐QoL field study complete data sets of 306 children and adolescents (4–16 years) and their parents, a multivariate approach of item selection was applied to construct an eight‐item instrument, the Haemo‐QoL Index. The instrument is applicable to different age groups and represents the core content as well as the multidimensional structure of the original long versions. According to preliminary analyses, the index's psychometric performance concerning reliability and convergent validity is good. Further validation of the instrument's performance on a new and independent sample is needed.
Preliminary results of HBV DNA testing of Polish haemophilia patients – lack of occult HBV infection
Authors: WINDYGA J. , BROJER E. , GRONOWSKA A. , GRABARCZYK P. , MIKULSKA K. , SZCZEPANIK A. B. , STEFAŃSKA E. , BUCZMA A.
Published: June 2006

Summary.  Identification of hepatitis B virus (HBV) infection in the absence of surface antigen (HbsAg) became possible with the introduction of HBV DNA detection methods. Such occult HBV infection was diagnosed recently in about half of the Japanese HBsAg‐negative haemophilia patients. The aim of our study was to assess the prevalence of occult HBV infection in Polish severe haemophilia population on the sample of 115 haemophilia A and B patients (mean age 34.9 ± 10.9) treated with non‐virus inactivated clotting factor preparations before 1995. HBV DNA was detected in nine HBsAg‐positive patients (7.8%). The mean HBV DNA load was 72 800 IU mL−1 (250–400 000 IU mL−1). Hepatitis C virus (HCV) RNA was found in six out of nine HBV‐positive patients. In conclusion, HBV DNA was identified only in HBsAg‐positive patients. Unlike in Japan, the frequency of occult HBV infection in Polish haemophilia population seems extremely rare or absent.
Current European practice in immune tolerance induction therapy in patients with haemophilia and inhibitors
Authors: ASTERMARK J. , MORADO M. , ROCINO A. , VAN DEN BERG H. M. , VON DEPKA M. , GRINGERI A. , MANTOVANI L. , GARRIDO R. P. , SCHIAVONI M. , VILLAR A. , WINDYGA J. ,   ON BEHALF OF THE EHTSB
Published: June 2006

Summary.  The management of patients with inhibitors is an important challenge in haemophilia care. The lack of randomized controlled trials means that clinical decisions are generally based on subjective opinions, and purchasers’ attention is likely to focus on the costs of treatment. In order to assess the current management of inhibitor patients and use of immune tolerance induction therapy (ITI) in Europe, we performed a survey within a European network of 21 comprehensive care centres from 14 countries (the European Haemophilia Therapy Standardisation Board). The survey identified a total of 381 patients with inhibitors attending the centres, 211 (55.4%) of whom had never been exposed to ITI. Between 1998 and 2003, the centres performed 233 procedures and 114 (48.9%) were successful. The survey demonstrated that dosing, which is the time to start and stop the ITI, the type of concentrate to use and the definition of success varied among the centres. Well‐designed trials are warranted to guide decision‐making, but in the absence of these studies we have developed consensus guidance for the management of inhibitor patients based on current clinical practice, as identified by the survey, and review of the literature.
Periodontal status and IOTN interventions among young hemophiliacs
Authors: AZHAR S. , YAZDANIE N. , MUHAMMAD N.
Published: June 2006

Summary.   Fifty‐two young individuals suffering from severe haemophilia A and B volunteered to be compared with school‐ and college‐going students for oral health status description and subsequent management. A total of 244 students (84.42% boys and 15.58% girls) with the age group of 13–23 years were divided into two groups, A and B (controls). The purpose of this study was to increase awareness about evidence‐based dental practices by oral examinations followed by comparisons of periodontal health and prevalence of malocclusions among medically compromised students and healthy controls. Results described the oral health in severe haemophilic population to be compromised with combined simplified health index score of 0.50 and Decayed/Modified/Filled Teeth (DMFT) index score of 2.07 when compared with 0.42 and 0.95, respectively, among group B. Although prevalence of malocclusion and orthodontic treatment needs among group A were higher, yet it was not confirmed as a reason for degraded dental and periodontal status. However, spontaneous and/or toothbrush (trauma)‐induced gingival bleeding episodes among group A could be explained as factors discouraging oral hygiene maintenance, particularly self‐administered measures. Four haemophiliacs presented with symptoms of Temporomandibular Joint Dysfunction Syndrome (TMPDS). Evidence‐based oral medicine and clinical practices need to be encouraged and applied to enhance the quality of life among haemophiliacs, particularly in developing world. Dental treatment needs of haemophilic population appear to be greater and maybe incorporated in routine dental practices, at institutional and individual levels.
Treatment and outcome of acquired haemophilia A with a standard conventional regimen in a cohort without associated conditions
Authors: NG H. J. , TAN D. C. L. , LEE L. H.
Published: June 2006

Summary.  Acquired haemophilia A (AH) is a rare bleeding disorder with significant risk of mortality. We conducted a study on the treatment and outcomes of patients with AH diagnosed between 1998 and 2004 in our institution. Fourteen patients (age range 17–89, median 64) were followed‐up from between 10–64 months (average 32 months). Inhibitor levels ranged from 5 to 450 BU. Ten patients required either recombinant activated factor VIIa or prothrombin complexes for control of bleeding. All patients received a standard immunosuppressive regimen of prednisolone 1 mg kg day−1 and oral cyclophosphamide 50–100 mg day−1. In addition, nine patients received intravenous immunoglobulin. Complete remission (CR) was achieved in 88% (eight of nine evaluable patients) over durations ranging from 5–78 days (average 35 days). There were three mortality; from bleeding, sepsis and cerebral infarct. Two patients were lost to follow‐up. There were no relapses among patients achieving CR. Conditions associated with AH were not identified at diagnosis or subsequent follow‐up in all patients. This series represent one of the largest aetiologically and treatment‐wise, uniformed cohort of AH patients. Despite toxicity concerns, the combination of prednisolone and cyclophosphamide remain an effective regimen and should be among the first line of treatment choices, with careful patient selection.
The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization
Authors: LEE C. A. , CHI C. , PAVORD S. R. , BOLTON‐MAGGS P. H. B. , POLLARD D. , HINCHCLIFFE‐WOOD A. , KADIR R. A.
Published: June 2006

Summary.  The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. These guidelines have been provided for healthcare professionals for information and guidance and it is also intended that they are readily available for women with bleeding disorders.
Abortive activation precedes functional deletion of CD8+ T cells following encounter with self‐antigens expressed by resting B cells in vivo
Authors: Fraser Joanne M. , Janicki Claire N. , Raveney Ben J. E. , Morgan David J.
Published: June 2006

InsHA mice express the haemagglutinin (HA) protein from influenza virus A/PR/8 H1N1 (PR8) as a self antigen on pancreatic islet β cells. We have utilized these mice to investigate the ability of resting B cells expressing Kd to induce self‐tolerance among naive KdHA‐specific clone 4 CD8+ T cells. Adoptive transfer of KdHA‐peptide‐pulsed resting B cells into clone 4→InsHA recipients resulted in the activation and proliferation of clone 4 CD8+ T cells throughout the peripheral lymphoid tissues. Significantly, proliferation was not associated with the acquisition of T cell effector function; as evidenced by a lack of interferon‐γ production and the complete absence of any autoimmune pathology even after immunization of recipient mice with PR8. These data demonstrate that resting B cells pulsed with self‐epitopes can induce abortive activation of potentially self‐reactive naive CD8+ T cells resulting in their functional deletion from the peripheral T‐cell repertoire in the absence of any associated autoimmunity.
Importance of gastrointestinal ingestion and macromolecular antigens in the vein for oral tolerance induction
Authors: Wakabayashi Ayako , Kumagai Yoshihiro , Watari Eiji , Shimizu Masumi , Utsuyama Masanori , Hirokawa Katsuiku , Takahashi Hidemi
Published: June 2006

Oral administration of a certain dose of antigen can generally induce immunological tolerance against the same antigen. In this study, we showed the temporal appearance of ovalbumin (OVA) antigens in both portal and peripheral blood of mice after the oral administration of OVA. Furthermore, we detected 45 000 MW OVA in mouse serum 30 min after the oral administration of OVA. Based on this observation, we examined whether the injection of intact OVA into the portal or peripheral vein induces immunological tolerance against OVA. We found that the intravenous injection of intact OVA did not induce immunological tolerance but rather enhanced OVA‐specific antibody production in some subclasses, suggesting that OVA antigens via the gastrointestinal tract but not intact OVA may contribute to establish immunological tolerance against OVA. Therefore, we examined the effects of digesting intact OVA in the gastrointestinal tract on the induction of oral tolerance. When mice were orally administered or injected into various gastrointestinal organs, such as the stomach, duodenum, ileum, or colon and boosted with intact OVA, OVA‐specific antibody production and delayed‐type hypersensitivity (DTH) response were significantly enhanced in mice injected into the ileum or colon, compared with orally administered mice. These results suggest that although macromolecular OVA antigens are detected after oral administration of OVA in tolerant‐mouse serum, injection of intact OVA cannot contribute to tolerance induction. Therefore, some modification of macromolecular OVA in the gastrointestinal tract and ingestion may be essential for oral tolerance induction.
Enhancement of CD8 T‐cell function through modifying surface glycoproteins in young and old mice
Authors: Sadighi Akha Amir A. , Berger Scott B. , Miller Richard A.
Published: June 2006

Previous work from our laboratory has shown that modifying cell surface glycosylation with either a Clostridium perfringens‐derived sialidase (CP‐Siase), or an O‐linked glycoprotein endopeptidase (OSGE) can enhance the function of CD4 T cells from both young and old mice at multiple levels. Here we have re‐assessed the effect of age on CD8 T‐cell function, and examined the outcome of enzymatic treatment with CP‐Siase and OSGE on its different aspects. Pre‐treatment of CD8 T cells with either CP‐Siase or OSGE led to a significant increase in anti‐CD3‐mediated Ca2+ response in both young and old mice. Pre‐treated CD8 T cells from both age groups also displayed a significant increase in activation‐induced CD69 and CD25 expression, and produced significantly higher amounts of interleukin‐2 and interferon‐γ in comparison to their untreated counterparts. Furthermore, pretreatment with either enzyme enhanced granzyme B expression in CD8 T cells, and increased their cytolytic activity in vitro. These data support the notion that glycosylated surface proteins hinder CD8 T‐cell activation and function in both young and old mice, and raise the possibility of significantly improving CD8 T cell function in older individuals through enzymatic alteration of surface glycoproteins.
The evaluation of grape pomace distillates from selected red grape varieties
Authors: Gerogiannaki‐Christopoulou Maria , Kyriakidis Nikolaos V. , Athanasopoulos Panagiotis E.
Published: June 2006

This report describes the composition of the volatiles from grape pomace distillated that are commercially available in Greece. These spirits, which are a valuable by‐product of the winemaking industry, were obtained by distillation of grape pomace after fermentation under anaerobic conditions. The volatiles were distilled twice. Each distillate was separated in to 4 equal (by volume) fractions and the changes in volatile concentration during distillation were measured. Assays were done by gas/liquid chromatography (for alcohols, carboxylic acids, esters and aldehydes). The average values for volatile concentrations obtained for Greek grape pomace distillates were compared with the corresponding values for grape pomace manufactured in other Mediterranean countries.
Utilization of peanut fines in the optimization of peanut polvoron using mixture response surface methodology
Authors: San Juan Edith M. , Edra Ermina V. , Sales Jocelyn M. , Lustre Alicia O. , Resurreccion Anna. V. A.
Published: June 2006

Percentages of peanut fines, sugar and butter in a peanut polvoron formulation were optimized using mixture response surface methodology (RSM). A consumer panel evaluated colour, appearance, flavour, texture and overall acceptance of a control and 15 experimental formulations of peanut polvoron containing various levels of peanut fines, sugar and butter. Texture was found to be the limiting factor for the optimization of peanut polvoron. Formulations with a 9‐point hedonic scale score minima of ≥6 for overall acceptance and acceptance of colour, appearance, flavour and texture were considered optimum. Optimum formulations can be obtained in all blends containing 22–36% peanut fines, 24% butter and 40–54% sugar.
Textural attributes of commercial biscuits. Effect of relative humidity on their quality
Authors: Mandala I. G. , Ioannou C. A. , Kostaropoulos A. E.
Published: June 2006

Two commercial biscuits of semi‐sweet type, ‘Marie’ and ‘Petit Beurre’ were used for investigating: (i) their texture variability as a function of the location and the side of puncturing and (ii) the influence of storage under different relative humidity on their texture, porosity and colour. Significant texture differences were found as a function of the location of puncturing (centre or periphery of biscuit area), as well as between the upper and the opposite side of a biscuit. At small relative humidity changes (11–32%) a distinct relationship between texture and moisture content was not noticed. In ‘Petit Beurre’ at aw 0.32 a peak was observed in the puncture force values. In both biscuits porosity increased with their moisture content, accompanied by an area expansion. Significant differences in colour were mainly noticed between the upper and opposite side of the biscuits. Colour was less influenced by relative humidity changes.
Digital imaging for evaluation of potato after‐cooking darkening and its comparison with other methods
Authors: Wang‐Pruski Gefu
Published: June 2006

After‐cooking darkening (ACD) phenomenon occurs when potato tubers develop greyish dark colour after boiling, frying, or steaming. The pigment responsible for ACD is a complex of chlorogenic acid and iron, which is formed during cooking and oxidised during cooling. The evaluation of ACD is thus based either on the amount of chlorogenic acid in raw tubers or the greyish colour intensity on cooked tuber surfaces. This study introduced a new method, the digital imaging method, for evaluation of potato ACD. The comparison of this new method with visual evaluation and the measurement of chlorogenic acid using HPLC were also presented. The digital imaging method is based on the direct measurement of the degree of dark colour on cut tuber surfaces, expressed by pixel levels. The new method is faster, more reliable and simpler when compared with any methods presently used. It is particularly applicable to handling large sample numbers.
Improving the nutritional quality and maintaining consumption quality of akara using curdlan and composite flour
Authors: Plahar Minerva A. , Hung Yen‐Con , McWatters Kay H.
Published: June 2006

Soybean flour and curdlan were incorporated into cowpea flour to determine their effect on lowering the fat content and on the physical properties of akara. At 20% substitution, soybean flour lowered the fat content of akara by 7.7% and increased the protein content by 28.7% without significantly affecting the firmness or the colour of akara. Addition of 1% curdlan decreased the fat content by 32.2% but significantly increased the firmness of akara and produced a darker‐coloured product. The paste moisture content of akara containing 20% soybean flour and 1% curdlan was modified to obtain product characteristics comparable with the control (100% cowpea flour) while maintaining a lower fat content. Optimum results were obtained for paste with 63% moisture content. Firmness of this modified product was similar to the control and the fat content was lower (17%) compared with the control (26%).
The time‐dependent rheological characteristics of a chickpea flour dispersion as a function of temperature and shear rate
Authors: Ravi Ramasamy , Bhattacharya Suvendu
Published: June 2006

Chickpea or Bengalgram (Cicer arietinum L.) flour is used as a raw material to prepare several oriental snacks. Time‐dependent rheological studies were done on chickpea flour dispersions at different temperatures (10–40 °C) and shear rates (5–200 s−1) by using a controlled stress rheometer with a coaxial cylinder attachment. The loss in shear stress with time of shearing was a function of both the temperature and the shear rate used; low temperatures coupled with high shear rates yielded high losses in shear stress. The common time‐dependent rheological models such as the Weltmann, Hahn‐Ree‐Eyring and Peleg were suitable to characterize the time‐dependent rheological behaviour of chickpea flour dispersions. The Peleg model was marginally the best as evidenced by the lower root mean square error values of 0.77–5.17% (average 1.40%) compared with Weltmann (0.90–5.58%, average 2.00%) and Hahn‐Ree‐Eyring (0.96–5.97%, average 2.74%) models.
Nest ornamentation by female spotless starlings in response to a male display: an experimental study
Authors: POLO VICENTE , VEIGA JOSÉ P.
Published: June 2006

The use of behavioural traits by females in signalling condition has been practically ignored in evolutionary theory. However, females may also exhibit ornaments and behavioural displays, although less elaborated than those of males.

In this study we suggest that the carrying of feathers by spotless starlings Sturnus unicolor Temminck females to decorate the nest represents an elaborated and costly behaviour that is displayed in response to a courtship male behaviour: the carrying of nest green plants.

By experimentally increasing the amount of green plants in the nests, to give the appearance that highly attractive males defended them, we induced females to increase their feather carrying rates.

The amount of feathers carried to the nest was correlated to female reproductive experience and laying date, two variables correlated with female body condition. These results suggests that this behaviour may work as an honest indicator of female quality.

We conclude that male carrying plants and female carrying feathers can be viewed as two sex‐specific functionally related signalling behaviours involved in mutual courtship or status signalling.
Resource partitioning in sympatric delphinids: space use and habitat preferences of Australian snubfin and Indo‐Pacific humpback dolphins
Authors: PARRA GUIDO J.
Published: June 2006

Many species of delphinids co‐occur in space and time. However, little is known of their ecological interactions and the underlying mechanisms that mediate their coexistence.

Snubfin Orcaella heinsohni, and Indo‐Pacific humpback dolphins Sousa chinensis, live in sympatry throughout most of their range in Australian waters. I conducted boat‐based surveys in Cleveland Bay, north‐east Queensland, to collect data on the space and habitat use of both species. Using Geographic Information Systems, kernel methods and Euclidean distances I investigated interspecific differences in their space use patterns, behaviour and habitat preferences.

Core areas of use (50% kernel range) for both species were located close to river mouths and modified habitat such as dredged channels and breakwaters close to the Port of Townsville. Foraging and travelling activities were the dominant behavioural activities of snubfin and humpback dolphins within and outside their core areas.

Their representative ranges (95% kernel range) overlapped considerably, with shared areas showing strong concordance in the space use by both species. Nevertheless, snubfin dolphins preferred slightly shallower (1–2 m) waters than humpback dolphins (2–5 m). Additionally, shallow areas with seagrass ranked high in the habitat preferences of snubfin dolphins, whereas humpback dolphins favoured dredged channels.

Slight differences in habitat preferences appear to be one of the principal factors maintaining the coexistence of snubfin and humpback dolphins. I suggest diet partitioning and interspecific aggression as the major forces determining habitat selection in these sympatric species.
Application of random effects to the study of resource selection by animals
Authors: GILLIES CAMERON S. , HEBBLEWHITE MARK , NIELSEN SCOTT E. , KRAWCHUK MEG A. , ALDRIDGE CAMERON L. , FRAIR JACQUELINE L. , SAHER D. JOANNE , STEVENS CAMERON E. , JERDE CHRISTOPHER L.
Published: June 2006

Resource selection estimated by logistic regression is used increasingly in studies to identify critical resources for animal populations and to predict species occurrence.

Most frequently, individual animals are monitored and pooled to estimate population‐level effects without regard to group or individual‐level variation. Pooling assumes that both observations and their errors are independent, and resource selection is constant given individual variation in resource availability.

Although researchers have identified ways to minimize autocorrelation, variation between individuals caused by differences in selection or available resources, including functional responses in resource selection, have not been well addressed.

Here we review random‐effects models and their application to resource selection modelling to overcome these common limitations. We present a simple case study of an analysis of resource selection by grizzly bears in the foothills of the Canadian Rocky Mountains with and without random effects.

Both categorical and continuous variables in the grizzly bear model differed in interpretation, both in statistical significance and coefficient sign, depending on how a random effect was included. We used a simulation approach to clarify the application of random effects under three common situations for telemetry studies: (a) discrepancies in sample sizes among individuals; (b) differences among individuals in selection where availability is constant; and (c) differences in availability with and without a functional response in resource selection.

We found that random intercepts accounted for unbalanced sample designs, and models with random intercepts and coefficients improved model fit given the variation in selection among individuals and functional responses in selection. Our empirical example and simulations demonstrate how including random effects in resource selection models can aid interpretation and address difficult assumptions limiting their generality. This approach will allow researchers to appropriately estimate marginal (population) and conditional (individual) responses, and account for complex grouping, unbalanced sample designs and autocorrelation.
Mitigating human disturbance: can protection influence trajectories of recovery in benthic assemblages?
Authors: BEVILACQUA STANISLAO , TERLIZZI ANTONIO , FRASCHETTI SIMONETTA , RUSSO GIOVANNI FULVIO , BOERO FERDINANDO
Published: June 2006

Understanding whether Marine Protected Areas (MPAs) can be considered as a suitable tool for restoring the structure and function of populations and assemblages is urgently needed to achieve an effective policy of mitigation of human impact in coastal management. However, to date, the role played by MPAs in enhancing ecosystems resilience has been more advocated than unambiguously documented.

This study was designed to test whether full protection in marine reserves facilitates recovery of benthos impacted by the date mussel Lithophaga lithophaga fishery, one of the most harmful human activities affecting subtidal rocky habitats in the Mediterranean Sea.

The effects of this destructive fishery were reproduced at one fully protected location (P) and at two unprotected control locations (Cs) in the SW Mediterranean Sea. At each location, three plots (4 m2) of rocky surface at 4–6 m depth were disturbed experimentally, while another three plots served as reference. In each plot, the species composition and relative cover of the sessile benthic assemblages were sampled photographically on each of five occasions during a period of 20 months.

Over and above variation in habitat features among locations, multivariate and univariate analyses revealed significant differences between P‐vs.‐Cs in patterns of assemblage recovery and showed that, at the fully protected location, recovery was faster than at the unprotected control locations.

Our results suggest that MPAs have the potential to change the trajectories of recovery of disturbed assemblages by accelerating the processes of recolonization and call for further investigation to identify the specific mechanisms underlying increased resilience.