bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prevalence of familial malignancy in a prospectively screened cohort of patients with lymphoproliferative disorders
Authors: Brown Jennifer R. , Neuberg Donna , Phillips Kimberly , Reynolds Hazel , Silverstein Jason , Clark Jennifer C. , Ash Megan , Thompson Christina , Fisher David C. , Jacobsen Eric , LaCasce Ann S. , Freedman Arnold S. 
Published: August 2008

SummaryIncreasing evidence points to a heritable contribution in the development of lymphoma. The goal of this study was to determine the rate of familial lymphoproliferative malignancy among consecutive lymphoma patients presenting to a tertiary care center and to enrol families with multiple affected first‐degree relatives on a data and tissue collection study. Beginning in 2004 all new patients presenting to the Dana‐Farber Cancer Institute with non‐Hodgkin (NHL) or Hodgkin lymphoma (HL) or chronic lymphocytic leukaemia (CLL) were asked to complete a one‐page self‐administered family history questionnaire. 55·4% of 1948 evaluable patients reported a first‐degree relative with a malignancy, with the highest rate among CLL probands. Lymphoid malignancies were particularly common, with 9·4% of all probands reporting a first‐degree relative with a related lymphoproliferative disorder (LPD). This frequency was again highest for CLL, at 13·3% of CLL probands, compared to 8·8% of NHL probands and 5·9% of HL probands (P = 0·002). The prevalence of CLL was significantly increased in parents of CLL probands (P < 0·05), and a greater risk of NHL was seen in fathers of NHL probands than in mothers (P = 0·026). We conclude that familial aggregation of LPDs is common among newly diagnosed patients, varies significantly by diagnosis and contributes meaningfully to the population disease burden.
Secondary non‐Hodgkin lymphoma (NHL) in children and adolescents after childhood cancer other than NHL
Authors: Landmann Eva , Oschlies Ilske , Zimmermann Martin , Moser Olga , Graf Norbert , Suttorp Meinolf , Greiner Jeannette , Reiter Alfred ,   on behalf of the Berlin‐Frankfurt‐Münster group
Published: August 2008

SummaryThe emergence of non‐Hodgkin lymphoma (NHL) during childhood and adolescence as a secondary neoplasm (SN) after previous cancer other than NHL is rare. To describe the characteristics and outcome of NHL following previous cancer other than NHL in children and adolescents, this study analysed the data of patients reported to the NHL‐Berlin‐Frankfurt‐Münster study centre from 1986 to 2005. Out of the total of 2968 NHL‐patients registered, 11 patients were assessed as having suffered from NHL as a proven SN. Four additional children had most likely suffered from NHL as an SN, but a late relapse of the first neoplasm could not be ruled out unequivocally. In the patients with proven SN, median age at diagnosis of the primary malignancy was 3·9 years (range 2–11·7). The median age at diagnosis of NHL was 7·6 years (range 4·7–18). Only lymphoblastic (n = 7) and diffuse large B‐cell (n = 4) lymphomas were diagnosed as SN. The estimated 5‐year event‐free survival from time of diagnosis of NHL was 91% [95% confidence interval (CI) 74–100%] in patients with proven SNs and 84% (95% CI 63–100%) when the patients with probable SNs were included in the analysis. We concluded that secondary NHL in children and adolescents confers a favourable prognosis.
Diagnostic performance of serum free light chain measurement in patients suspected of a monoclonal B‐cell disorder
Authors: Vermeersch Pieter , Van Hoovels Lieve , Delforge Michel , Mariën Godelieve , Bossuyt Xavier 
Published: August 2008

SummaryThe present study aimed to determine the diagnostic performance of different testing strategies to diagnose malignant B‐cell disorder or monoclonal gammopathy of unknown significance (MGUS). Sensitivity and specificity were determined in 833 consecutive patients investigated for a monoclonal gammopathy. Serum protein electrophoresis (PE), serum κ/λ free light chain (FLC) ratio, and serum and urine immunofixation electrophoresis (IFE) were performed in all patients. Twenty‐eight patients were diagnosed with a malignant plasma cell disorder, 25 with B‐cell non‐Hodgkin lymphoma and 156 with MGUS. Serum PE (with follow‐up IFE) plus FLC had a sensitivity of 82·3% and a specificity of 96·8% and missed one plasmacytoma and 23 patients with MGUS. Serum IFE plus urine IFE had a sensitivity of 92·3% and a specificity of 100% and missed two MGUS patients. Serum IFE plus FLC had a sensitivity of 93·8% and a specificity of 96·8% and missed one MGUS patient. Serum PE plus FLC had a significantly lower sensitivity than serum IFE plus FLC or serum IFE plus urine IFE for the diagnosis of MGUS. The sensitivity of serum IFE plus FLC was comparable to the sensitivity of serum IFE plus urine IFE. The specificity of serum IFE plus FLC, however, was lower than the specificity of serum IFE plus urine IFE.
Ghrelin and the differential regulation of des‐acyl (DSG) and oct‐anoyl ghrelin (OTG) in human adipose tissue (AT)
Authors: Kos K. , Harte A. L. , O’Hare P. J. , Kumar S. , McTernan P. G. 
Published: August 2008

SummaryObjectives  Ghrelin, an important central acting orexigenic hormone, is predominantly secreted in the gastrointestinal tract. However little is known about the action of ghrelin in human adipose tissue (AT).Aim  To study the expression of ghrelin in AT, the effects of octanoyl–(OTG) and des‐acyl (DSG) ghrelin on lipolysis and lipogenesis, leptin release and potential peripheral signalling through the Y1 receptor.Methods  Ex vivo human AT was obtained from women undergoing elective surgery (46 (mean ± SD) 6·8 years, body mass index (BMI): 25·6 ± 5·0 kg/m2, n = 20). Abdominal‐subcutaneous (AbdSc) adipocytes were isolated and treated with recombinant human (rh) OTG and DSG to assess lipid metabolism leptin release and the influence of Y1‐receptor blocker.Results  Ghrelin was expressed in AbdScAT and negatively correlated with BMI (lean: 3·6 ± 0·74 optical‐density‐units (OD), obese: 1·64 ± 0·45OD, *P < 0·05). Only DSG significantly suppressed glycerol release (Control (C): 286 ± 58 µl/l; DSG 1 nm: 224 ± 38 µl/l↓*; DSG 100 nm: 172 ± 13 µl/l↓*,*↓P < 0·05, n = 7) and reduced hormone sensitive lipase expression (C: 1·0 ± 0·3OD; DSG 1 nm: 0·8 ± 0·3OD↓*; DSG 100 nm: 0·6 ± 0·1OD↓*, n = 4). However, both isoforms increased lipoprotein lipase expression (C: 1·0 ± 0·3OD; DSG 100 nm: 0·2 ± 0·4OD↑*; OTG 100 nm: 2·5 ± 0·3OD↑*, n = 4), whilst blockade of Y1 eliminated this effect in both. Leptin was down‐regulated by DSG only (DSG 1 nm: 5·3 ± 0·7 ng/ml; DSG 100 nm: 4·1 ± 0·7 ng/ml*) and was significant after BMI adjustment (P = 0·029).Conclusion  Ghrelin was expressed in human AbdSc AT. In vitro, both OGT and DSG appear to mediate fat deposition with the lipogenic effects in part mediated by the Y1 receptor, whilst the influence of DSG affected lipolysis, lipogenesis and leptin secretion. Taken together, these studies support a local action for ghrelin isoforms on lipid and adipokine metabolism that further supports a cross talk between organs.
Functional studies of new TSH receptor (TSHr) mutations identified in patients affected by hypothyroidism or isolated hyperthyrotrophinaemia
Authors: De Marco Giuseppina , Agretti Patrizia , Camilot Marta , Teofoli Francesca , Tatò Luciano , Vitti Paolo , Pinchera Aldo , Tonacchera Massimo 
Published: August 2008

SummaryObjectives  To establish the role of new TSH receptor (TSHr) variants (P27T, E34 K, R46P, D403N, W488R and M527T) recently identified in children with congenital hypothyroidism (CH) or subclinical hypothyroidism (SH) with a thyroid gland of normal size.Design and measurements  TSHr variants were obtained by mutagenesis. Wild‐type (wt) and TSHr mutants were expressed in COS cells and cAMP assay, 125I‐TSH binding and microchip flow cytometry analyses were performed.Results  D403N and M527T mutants showed a lower cAMP response to bovine TSH (bTSH) with respect to the wtTSHr. R46P and W488R mutants did not show any response to bTSH stimulation in terms of cAMP production. The E34 K mutant showed a significantly lower cAMP response to stimulation with bTSH, while P27T had a lower cAMP response only to the highest dose of bTSH used. P27T, E34 K, D403N and M527T mutants showed a lower TSH binding capacity with respect to the wtTSHr. R46P and W488R mutants did not show any TSH binding.Conclusions  E34 K, D403N, M527T, R46P and W488R TSHr variants seem to cause a functional abnormality of the receptor which is responsible for the observed phenotype. The P27T TSHr variant does not seem to play a functional role in the pathogenesis of CH and should be considered as a polymorphism.
Anaemia and marrow fibrosis in patients with primary hyperparathyroidism before and after curative parathyroidectomy
Authors: Bhadada Sanjay K. , Bhansali Anil , Ahluwalia Jasmina , Chanukya G. V. , Behera Arunanshu , Dutta Pinaki 
Published: August 2008

SummaryObjective To determine the relationship between anaemia and myelofibrosis in patients with symptomatic primary hyperparathyroidism (PHPT) and to assess the effect of curative parathyroidectomy on anaemia and marrow fibrosis.Design and methods In this prospective follow‐up study of 28 consecutive patients with symptomatic PHPT from January 2005 to June 2006, 15 patients were diagnosed with anaemia (haemoglobin < 130 g/l in males and < 120 g/l in females), eight (53%) of whom were finally recruited for the study. Complete blood cell count, serum calcium, phosphorus, alkaline phosphatase, intact PTH and 25‐hydroxyvitamin D and bone marrow examination were performed both before and after parathyroidectomy in all (n = 8) patients, but bone marrow examination after surgery was performed only in those who had marrow fibrosis at baseline (n = 6).Results Anaemia was observed in 15 (53·3%) of the 28 patients with symptomatic PHPT. Normocytic normochromic anaemia that is characteristic of PHPT was found in 14 (50%) patients. Eight of the 15 patients with anaemia had a bone marrow examination and marrow fibrosis was observed in six (75%). Both anaemia and marrow fibrosis improved after successful parathyroidectomy, but improvement in anaemia was significant (P = 0·02) only in those with marrow fibrosis at baseline. Marrow fibrosis did not correlate with duration of the disease (P = 0·17), degree of hypercalcaemia (P = 0·53) or serum levels of intact PTH (P = 0·60).Conclusions Anaemia is common in patients with symptomatic PHPT, and was associated with marrow fibrosis in the majority of the patients who underwent bone biopsy. Both anaemia and marrow fibrosis improved after curative parathyroidectomy, but improvement in anaemia was noticeable only in those who had marrow fibrosis at presentation.
Voice characteristics in women with congenital adrenal hyperplasia due to 21‐hydroxylase deficiency
Authors: Nygren Ulrika , Södersten Maria , Falhammar Henrik , Thorén Marja , Hagenfeldt Kerstin , Nordenskjöld Agneta 
Published: August 2008

SummaryObjective  Congenital adrenal hyperplasia (CAH) is an autosomal recessive inherited disorder resulting in lack of cortisol and often aldosterone and overproduction of androgens. Raised levels of androgens can lead to increased mass in the laryngeal tissues, which may lower the fundamental frequency (F0) of the voice.Design/patients  A follow‐up study of voice characteristics in 38 women with CAH and 24 age‐matched controls between 18 and 63 years of age using subjective self‐ratings of voice symptoms and acoustic and perceptual analyses. The results were correlated to mutation and treatment.Measurements  The subjects rated degree of hoarseness, dark voice, voice problems and vocal fatigue on visual analogue scales. Audio recordings were made of a standard reading passage in a sound treated booth. Acoustic analyses were made of the subjects’ mean, minimum and maximum F0 in the habitual speech range. Three voice clinicians made perceptual evaluations of dark voice in terms of timbre.Results  Women with CAH rated higher values than controls with regard to the statement ‘my voice is a problem in my daily life’. They spoke with significantly lower mean, lower minimum and lower maximum F0, as well as darker voice quality as compared with the controls. Affected voice symptoms were associated with a late diagnosis or problems with medication.Conclusion  The voices in women with CAH can be virilized because of late diagnosis or suboptimal treatment with glucocorticoids. These voice problems may need referral for voice assessment and should be considered when treating CAH.
Serum total antioxidant status and lipid peroxidation marker malondialdehyde levels in overt and subclinical hypothyroidism
Authors: Torun Ayse Nur , Kulaksizoglu Sevsen , Kulaksizoglu Mustafa , Pamuk Baris Onder , Isbilen Elif , Tutuncu Neslihan Bascil 
Published: August 2008

SummaryBackground  Mitochondria are the main production site of free oxygen radicals, which can cause organ dysfunction by oxidation of cellular macromolecules such as carbohydrates, lipids and proteins. Oxidative stress may result from either overproduction of these species or from failure of the antioxidant defence systems. Thyroid hormones have well‐known effects on mitochondrial oxygen consumption, but data about how hypothyroidism affects oxidative stress are controversial, and little is known about oxidative stress in subclinical hypothyroidism. Total antioxidant status (TAS) gives information about all of the antioxidants in the organism, while malondialdehyde (MDA) is a lipid peroxidation marker used to assess lipid peroxidation due to increased oxidative stress. We aimed to determine how hypothyroidism and subclinical hypothyroidism affect serum MDA and TAS.Subjects and methods  Serum TAS, MDA, C‐reactive protein levels and lipid compositions were studied in 20 hypothyroid, 40 subclinical hypothyroid and 40 healthy subjects.Results  MDA was elevated in both hypothyroid and subclinical hypothyroid patients compared with controls, while TAS levels show no significant differences between groups. Low‐density lipoprotein (LDL) cholesterol levels were significantly high in both hypothyroid and subclinical hypothyroid patients. Triglyceride levels were high only in hypothyroid patients when compared with the controls. MDA showed a correlation with LDL cholesterol, total cholesterol and triglyceride.Conclusions  These results suggest an increased oxidative stress in both hypothyroid and subclinical hypothyroidism states, which can be explained by both the insufficient increase in the antioxidant status and the altered lipid metabolism in these cases.
Down‐regulation of NR4A1 in follicular thyroid carcinomas is restored following lithium treatment
Authors: Camacho Cléber P. , Latini Flavia R. M. , Oler Gisele , Hojaij Flavio C. , Maciel Rui M. B. , Riggins Gregory J. , Cerutti Janete M. 
Published: August 2008

SummaryIntroduction  The identification of follicular thyroid adenoma‐associated transcripts will lead to a better understanding of the events involved in pathogenesis and progression of follicular tumours. Using Serial Analysis of Gene Expression, we identified five genes that are absent in a malignant follicular thyroid carcinoma (FTC) library, but expressed in follicular adenoma (FTA) and normal thyroid libraries.Methods  NR4A1, one of the five genes, was validated in a set of 27 normal thyroid tissues, 10 FTAs and 14 FTCs and three thyroid carcinoma cell lines by real time PCR. NR4A1 can be transiently increased by a variety of stimuli, including lithium, which is used as adjuvant therapy of thyroid carcinoma with 131I. We tested if lithium could restore NR4A1 expression. The expression of other genes potentially involved in the same signalling pathway was tested. To this end, lithium was used at different concentration (10 mm or 20 mm) and time (2 h and 24 h) and the level of expression was tested by quantitative PCR. We next tested if Lithium could affect cell growth and apoptosis.Results  We observed that NR4A1 expression was under‐expressed in most of the FTCs investigated, compared with expression in normal thyroid tissues and FTAs. We also found a positive correlation between NR4A1 and FOSB gene expression. Lithium induced NR4A1 and FOSB expression, reduced CCDN1 expression, inhibited cell growth and triggered apoptosis in a FTC cell line.Conclusions  NR4A1 is under‐expressed in most of FTCs. The loss of expression of both NR4A1 and the Wnt pathway gene FOSB was correlated with malignancy. This is consistent with the hypothesis that its loss of expression is part of the transformation process of FTCs, either as a direct or indirect consequence of Wnt pathway alterations. Lithium restores NR4A1 expression, induces apoptosis and reduces cell growth. These findings may explain a possible molecular mechanism of lithium's therapeutic action.
Application of ISO 22000 and comparison with HACCP on industrial processing of common octopus (Octopus vulgaris) – Part I
Authors: Arvanitoyannis Ioannis S. , Varzakas Theodoros H. 
Published: August 2008

SummaryCritical control points (CCPs) were identified in the risk assessment of octopus (Octopus vulgaris) processing and implemented in the hazard analysis critical control point (HACCP) plan. In the hazard analysis worksheet the different hazards were identified at each processing stage, whereas in the HACCP plan each CCP is identified and accompanied with the relevant significant hazard, critical limit, monitoring of the CCP and corrective actions. In this work comparison of ISO 22000 analysis with HACCP is carried out over octopus processing and packaging. ISO 22000 Analysis Worksheet was employed for determination of some prerequisite programmes (PrPs). Comparison between the two systems has been carried out using the hazard analysis worksheet. The PrPs are the main difference between the two systems. The incorporation of PrPs in the ISO 22000 made the system more flexible as a smaller number of CCPs was introduced.
Viscoelastic properties of reconstituted cassava dough
Authors: Rodríguez‐Sandoval Eduardo , Fernández‐Quintero Alejandro , Sandoval‐Aldana Angélica , Cuvelier Gerard , Bello‐Peréz Luis A. 
Published: August 2008

AbstractThe influence of water (60–70%) and salt (1–2%) content on the viscoelastic properties of cassava dough, reconstituted from cooked flour, was studied using a controlled strain rheometer. Reconstituted cassava dough behaved as a solid‐like material with the storage modulus (G′) predominant over the loss modulus (G″). As the water content was increased, G′ decreased and G″ increased; but tan δ was practically independent of the water content. This behaviour suggested that water had plasticising effects, but probably did not change dough structure. The effect of salt content on the dynamic rheological properties of cassava dough was not significant, except for G″ values at water contents close to 60%.
Study of organic acids, volatile fraction and caseins of a new Halloumi‐type cheese during ripening in whey brine
Authors: Stelios Kaminarides , Paraskevi Stamou , Theophilos Massouras , Aikaterini Georgala 
Published: August 2008

SummaryOrganic acids, fat hydrolysis, volatile compounds and sensory characteristics of a new brine cheese which combines characteristics of Halloumi and Feta cheeses during its ripening in whey brine (100g NaCl L−1) were studied. Thermotolerant protease of Mucor miehei as a coagulant enzyme and a mixture of thermotolerant starter cultures Enterococcus faecium 0165 (0.5% w/w) and Lactobacillus casei 80 10D were used. Good quality new Halloumi‐type cheese was produced with higher proteolysis than traditional Halloumi cheese kept in whey brine. The volatile compounds identified comprised alcohols, aldehydes, ketones, acids, esters, hydrocarbons and sulphur compounds. Ethanol was the dominant volatile compound determined. Lactic acid was the dominant acid produced; its concentration increased during ripening, reaching a maximum value of 9929 mg kg−1 at day 30. Acetic acid was also found in high amounts, which increased during cheese ripening. Lipolysis of cheese was not intense. The most abundant acids of the mature cheese were palmitic, oleic and acetic acid. The Halloumi‐type cheese scored higher in the sensory analysis when fresh than did the mature cheese.
The impact on tigers of poaching versus prey depletion
Authors: Chapron Guillaume , Miquelle Dale G. , Lambert Amaury , Goodrich John M. , Legendre Stéphane , Clobert Jean 
Published: August 2008

Summary

There exists a continuing dilemma in prioritizing conservation actions for large carnivores. Habitat loss, poaching, and prey depletion have often been cited as the three primary threats, but there is debate over the relative importance of each.
We assess the relative importance of poaching and prey depletion rates, and use existing information in the literature and multi‐type branching process and deterministic felid population models to address four lines of evidence used to infer that tiger populations are inherently resilient to high mortality rates.
Our results suggest that tigers, more so than leopards or cougars, require large populations to persist, are quite susceptible to modest increases in mortality, and less likely to recover quickly after population declines. Demographic responses that would ensure population persistence with mortality rates that are sustainable for cougars or leopards are biologically unrealistic for tigers.
We propose alternative interpretations of evidence used to suggest that tigers are inherently resilient to high mortality rates. In contrast to other solitary felids, tigers breed later and their inter‐birth interval is larger, making them less resilient to poaching. A model used to support the contention that prey depletion has greater impact on population persistence than poaching appears to be based on false premises. Camera‐trapping data that suggest positive population growth despite low survival rate cannot differentiate mortality from emigration, and does not differentiate the impact of varying survival rate on different sex‐age classes; for example, low survival rate of dispersers is tolerable if survival rate of adult breeding females is high.
Synthesis and applications. While high prey numbers are essential to sustain tiger populations, our results suggest prey recovery efforts will not be sufficient if mortality rates reach 15%. Extrapolating demographic responses from other, even closely related species to develop conservation strategies can be misleading. Reduction of human‐caused mortality, especially of resident breeding females, appears to be the most essential short‐term conservation effort that must be made. Since mortality rates are usually unknown and generally stochastic in nature, any management policy that might reduce survival rates should be firmly avoided.