bluuebunny/crossref_metadata_2025_split huggingface dataset:
The effect of double‐ and triple‐apheresis platelet product donation on apheresis donor platelet and white blood cell counts
Authors: Richa Elie , Krueger Paul , Burgstaler Edwin A. , Bryant Sandra C. , Winters Jeffrey L. 
Published: March 2008

BACKGROUND: Concerned about the effect of multiple platelet (PLT) product donation on donor health, in 2005 the FDA proposed restrictions limiting the number of PLT products donated in 12 months. This was based on limited published evidence. To provide information on the effect of PLT donation on PLT and lymphocyte (LYM) count, hematologic variables were examined in a group of multiple PLT product donors.STUDY DESIGN AND METHODS: Donors with at least two apheresis PLT donations, one of which was a double‐ or triple‐PLT product, were examined. Donor demographics, number of blood donations, and number of apheresis PLT products donated were determined. Hematologic variables were evaluated at the first and last donation.RESULTS: A total of 471 donors (median age, 48.8 years; 55% male; median time between first and last donations, 72 weeks) were studied. The median number of PLT donations was 4 (range, 1‐34) with the median number of PLT products donated being 7 (range, 2‐65). The median PLT count demonstrated a significant increase (14 × 109/L, p = 0.0001) while both white blood cell and LYM counts showed significant decreases (−0.2 × 109/L, p = 0.0052; and −0.06 × 109/L, p = 0.0001, respectively). After adjusting for sex and whole‐blood donations, LYM count demonstrated a significant decline associated with both number of donations (−0.01 × 109/L, p = 0.01) and number of products donated (−0.005 × 109/L, p = 0.02). PLT count demonstrated a significant increase associated with number of products donated (0.42 × 109/L, p = 0.03).CONCLUSION: Significant but small LYM decrease and PLT increase were seen. Limitations on the number of apheresis PLT products donated within 12 months do not seem warranted due to PLT or LYM count changes.
A comparison of hemolysis and red cell mechanical fragility in blood collected with different cell salvage suction devices
Authors: Yazer Mark H. , Waters Jonathan H. , Elkin Kyle R. , Rohrbaugh Max E. , Kameneva Marina V. 
Published: March 2008

BACKGROUND: Suction pressure is one variable that can affect the efficiency of red blood cell (RBC) recovery for intraoperative autotransfusion. This study compared a constant‐suction‐pressure system with a device that is expected to minimize hemolysis by automatically varying the suction pressure.STUDY DESIGN AND METHODS: Twenty‐two 50‐mL reconstituted whole‐blood samples were collected from a flat surface with either a constant‐pressure suction device (BRAT 2 autologous blood recovery system, COBE Cardiovascular) set to a pressure of −200 mmHg or a variable‐pressure suction device (SmartSuction Harmony surgical suction pump, Haemonetics, Inc.). Time of blood collection was recorded and plasma free hemoglobin (PFHb) was spectroscopically measured in the aspirated blood. Postcollection blood was subjected to RBC mechanical fragility test to characterize potential sublethal blood trauma. Relative hemolysis and the mean fragility index (MFI) were calculated.RESULTS: Hemolysis was significantly reduced with the variable‐pressure suction compared to the constant‐pressure suction (2.17% vs. 3.20%, respectively; p < 0.001). There was no significant difference between both suction devices in either the MFI of RBCs (0.632 vs. 0.673, respectively; p > 0.05) or the collection time at −200 mmHg (54.2 seconds vs. 52.8 seconds, respectively; p > 0.05).CONCLUSIONS: Although the variable‐pressure device produced a significant reduction in hemolysis during one‐pass blood collection, the clinical significance of this reduction is not clear. In relative terms, the variable‐pressure device would recover an extra 10 mL of RBCs for every liter of salvaged RBCs, which is negligible compared to the blood loss in major surgery.
Risk factors for hepatitis C virus infection among blood donors in Beijing and implications for improving the pretesting donor screening process
Authors: Qiu Yan , Shi Ling , Wang Yi , Zhang Gui , Zheng Jing , Gong Xiaoyan , Xia Hongying , Zhang Ping , Ness Paul , Shan Hua 
Published: March 2008

BACKGROUND: The objective was to determine risk factors of hepatitis C virus (HCV) infection in blood donors in Beijing.STUDY DESIGN AND METHODS: A cross‐sectional study was conducted in a total of 112 volunteer blood donors at Beijing Red Cross Blood Center (BRCBC) who were identified between January 2002 and December 2003 due to isolated HCV antibody reactivity at a donor screening procedure. The donors were recalled and interviewed about possible risk factor history. Additional blood samples were tested in repeated enzyme immunoassay, recombinant immunoblot assay, and nested reverse transcription–polymerase chain reaction. Chi‐square test, analysis of variance, and multivariate logistic regression analysis were conducted to examine associations between HCV serostatus and potential risk factors.RESULTS: Among donors identified with isolated HCV antibody reactivity at donor screening, the prevalence of confirmed HCV infection was 31.3 percent (95% confidence interval [CI], 22.0%‐39.2%) and indeterminate HCV infection was 22.3 percent (95% CI, 14.7%‐30.3%), and the negative prevalence was 46.4 percent (95% CI, 37.5%‐56.1%). The confirmed HCV cases were more likely than the indeterminate HCV cases to have the histories of previous transfusion, plasma donation, and hepatitis infection or elevated alanine aminotransferase (ALT), whereas the HCV‐negative subjects were least likely to have the above histories (p  < 0.05).CONCLUSION: Histories of blood transfusion before 1995, plasma donation, previous hepatitis infection, or elevated ALT were risk factors for HCV infection among blood donors in Beijing.
Distribution of chemo‐ and mechanoreceptors on the antennae and maxillae of Busseola fusca larvae
Authors: Juma Gerald , Chimtawi Mathayo , Ahuya Peter O. , Njagi Peter G. N. , Le Rü Bruno , Magoma Gabriel , Silvain Jean‐François , Calatayud Paul‐André 
Published: March 2008

AbstractThe stem borer Busseola fusca (Fuller) (Lepidoptera: Noctuidae) is a major pest of maize, Zea mays L., and sorghum, Sorghum bicolor (L.) Moench (both Poaceae), in sub‐Saharan Africa. Like in many other lepidopteran insects, the success of B. fusca in recognizing and colonizing a limited variety of plants is based on the interaction between its sensory systems and the physicochemical characteristics of its immediate environment. The sensilla on the maxillary galeae of B. fusca larvae are typical of Lepidoptera and comprise two uniporous styloconic sensilla, which are contact chemoreceptors, three basiconic sensilla, and two aporous sensilla chaetica. The maxillary palp is two‐segmented and has eight small basiconic sensilla at the tip, which were also found to be gustatory. The antennae of B. fusca larvae are short and simple. The sensilla of the antenna are composed of two aporous sensilla chaetica, three multiporous cone‐shaped basiconic sensilla, three small basiconic sensilla, and one aporous styloconic sensillum. The basiconic sensillum located on the third antennal segment displayed a contact chemoreception response. The other basiconic sensilla did not show any action potential activity in tip‐recording tests. The significant and positive dose–response curve obtained for the antennal basiconic sensillum with sucrose indicated for the first time the presence of gustatory chemoreceptors on the antennae of a lepidopteran larva.
Efficacy of entomopathogenic fungi against soil‐dwelling life stages of western flower thrips, Frankliniella occidentalis, in plant‐growing media
Authors: Ansari M. A. , Brownbridge M. , Shah F. A. , Butt T. M. 
Published: March 2008

AbstractThe potential of several entomopathogenic fungi to control soil‐dwelling stages of western flower thrips, Frankliniella occidentalis Pergande (Thysanoptera: Thripidae), was evaluated in various growing media. Two Metarhizium anisopliae (Metsch) Sorokin strains, V275 and ERL700, were the most effective, causing 85–96% mortality of thrips larvae and pupae (as measured by relative adult emergence rates), 11 days after inoculation. Mortality in other M. anisopliae‐treated media ranged from 51–84%; Beauveria bassiana (Balsamo) Vuillemin strains caused 54–84% mortality, and Paecilomyces fumosoroseus (Wize) 63–75% mortality. In contrast, mortality from the insecticide treatment (fipronil 1 g l−1 medium) was only 15–54%. Metarhizium anisopliae V275 was selected for more in‐depth evaluation under greenhouse conditions. There was no significant difference in M. anisopliae efficacy in growing media whether it was applied as drench (84–93%) or premixed into the medium as dry conidia (85–92%). The use of M. anisopliae with reduced rates of either fipronil or imidacloprid did not significantly improve control. Overall, our study shows that M. anisopliae V275 is robust and offers much promise for the control of soil‐dwelling stages of thrips as part of an integrated pest‐management programme.
Biomarkers for lysosomal storage disorders: identification and application as exemplified by chitotriosidase in Gaucher disease
Authors: Aerts Johannes M , Van Breemen Marielle J , Bussink Anton P , Ghauharali Karen , Sprenger Richard , Boot Rolf G , Groener Johanna E , Hollak Carla E , Maas Mario , Smit Suzanne , Hoefsloot Huub C , Smilde Age K , Vissers Johannes PC , De Jong Sheryas , Speijer Dave , De Koster Chris G 
Published: March 2008

AbstractA biomarker is an analyte that indicates the presence of a biological process linked to the clinical manifestations and outcome of a particular disease. An ideal biomarker provides indirect but ongoing determinations of disease activity. In the case of lysosomal storage disorders (LSDs), metabolites or proteins specifically secreted by storage cells are good candidates for biomarkers. Potential clinical applications of biomarkers are found in improved diagnosis, monitoring of disease progression and assessment of therapeutic correction. These applications are illustrated by reviewing the use of plasma chitotriosidase in the clinical management of patients with Gaucher disease, the most common LSD. The ongoing debate on the value of biomarkers in patient management is addressed. Novel analytical methods have revolutionized the identification and measurement of biomarkers at the protein and metabolite level. Recent developments in biomarker discovery by proteomics are described and the future for biomarkers of LSDs is discussed.Conclusion: Besides direct applications for biomarkers in patient management, biomarker searches are likely to render new insights into pathophysiological mechanisms and metabolic adaptations, and may provide new targets for therapeutic intervention.
Methods for assessing neurodevelopment in lysosomal storage diseases and related disorders: a multidisciplinary perspective
Authors: Martin Holly R , Poe Michele D , Reinhartsen Debra , Pretzel Rebecca E , Roush Jackson , Rosenberg Angela , Dusing Stacey C , Escolar Maria L 
Published: March 2008

AbstractLysosomal storage diseases and related disorders (LSRDs) are a heterogeneous group of rare diseases caused by genetic mutations that result in deficiencies of specific lysosomal enzymes. Some of these enzymes are necessary for normal development of the central and peripheral nervous systems. Because of the heterogeneity in clinical presentation and complexity of these disorders, evaluation of disease progression poses unique challenges. In recent years, recombinant enzyme replacement therapy and haematopoietic stem cell transplantation have been developed to treat some of these diseases. With the development of specific therapies and screening programmes, there is a need to systematically follow the natural course and effects of treatment in these disorders with standardized and validated tools. This review describes the limitations of currently available neurobehavioural tools in longitudinally tracking disease outcomes in patients with neurodegenerative LSRDs. A multidisciplinary team reviewed over 750 evaluations in 274 patients. These patients were found to have neurological, sensory and somatic problems that considerably influence the results of neurobehavioural testing.Conclusion: Treatment effects in patients with neurodegenerative LSRDs are best evaluated by repeated measures and longitudinal analysis of each domain of function.
Newborn screening for lysosomal storage disorders
Authors: Matern Dietrich 
Published: March 2008

AbstractNewborn screening is a public health programme aimed at identifying treatable conditions in pre‐symptomatic newborns to avoid premature mortality, morbidity and disabilities. With the advent of successful treatment options for an increasing number of lysosomal storage disorders (LSDs), such as enzyme replacement or bone marrow transplantation, inclusion of these disorders into newborn screening programmes seems reasonable. However, the success of these programmes depends on the availability of testing methods that are suitable for population screening, have high sensitivity and a low false‐positive rate. In recent years, two methods have been proposed for newborn screening of LSDs, which enable assessment of more than one LSD from a single blood spot sample. One applies tandem mass spectrometry, the other microbead array technology. Now, prospective studies are needed to determine the most effective approach to newborn screening that will identify those patients who require treatment.Conclusion: With the advent of high‐throughput assays for the detection of LSDs, newborn screening for these disorders may soon become a reality. However, careful prospective studies are required to optimize this process before it is used on a larger scale.
Gaucher disease: unmet treatment needs
Authors: Mehta Atul 
Published: March 2008

AbstractGaucher disease is a multisystemic metabolic disorder arising from a deficiency of lysosomal glucocerebrosidase. The predominant clinical manifestations of the disease are hepatosplenomegaly, peripheral blood cytopenias and skeletal disease. Treatment with enzyme replacement therapy (ERT) and substrate reduction therapy (SRT) has been shown to be effective in improving organ volume, anaemia, thrombocytopenia, bone markers and biomarkers in patients with Gaucher disease. However, some patient needs remain unmet because of the limited availability of treatment, the inaccessibility of certain disease sites and emerging disease manifestations. An increase in haematological, lymphoreticular and immune system malignancies has been observed in patients with Gaucher disease, but mechanisms underlying the development of these are not fully understood. Mild neurological manifestations may also affect patients with type 1 Gaucher disease, but treatment with ERT or SRT does not improve neurological function. Potential new treatments for Gaucher disease include small molecules, which may penetrate tissues that are not accessible by ERT.Conclusion: ERT currently remains the most effective treatment for Gaucher disease. New treatments are emerging, but deficiencies in understanding basic pathophysiological mechanisms hinder progress.
Apoptotic abnormalities in differential gene expression in peripheral blood mononuclear cells from children with Fabry disease
Authors: Moore David F , Goldin Ehud , Gelderman Monique P , Robinson Chevalia , Baer Jessica , Ries Markus , Elkahloun Abdel , Brady Roscoe O , Schiffmann Raphael 
Published: March 2008

AbstractAim: This study was designed to examine the effect of enzyme replacement therapy (ERT) on differential gene expression in peripheral blood mononuclear cells (PBMCs) of children with Fabry disease who had not previously been exposed to ERT.Methods: Thirteen children with Fabry disease (age range, 6.5–17.0 years) were studied as part of a 6‐month, open‐label study of ERT with agalsidase alfa. Paired blood samples were taken at the start of the study and after 6 months of ERT. Further blood samples were also taken from 16 age‐matched control subjects. PBMCs were isolated and, following RNA extraction, differential gene expression analysis was performed using the Human Genome U133 Plus 2.0 microarray.Results: Twenty‐one genes were determined to be differentially expressed in PBMCs of ERT‐naïve children with Fabry disease compared with healthy controls; neuronal apoptosis inhibitory protein ranked as the most significantly differentially expressed gene. Comparison of gene expression in children with Fabry disease prior to and after ERT showed that two genes were significantly differentially expressed (p ≤ 0.05) following treatment; the expressed sequence tag (probe set ID, 243259_at) was downregulated, while expression of apoptosis‐inducing factor was increased, possibly as an antioxidant counter‐regulatory response.Conclusion: This study identifies a number of genes that are differentially expressed in a small cohort of children with Fabry disease relative to healthy controls. These genes may relate to the underlying biological abnormalities in Fabry disease.
Fabry disease during childhood: clinical manifestations and treatment with agalsidase alfa
Authors: Ramaswami Uma 
Published: March 2008

AbstractFabry disease is a rare X‐linked disorder that leads to widespread and progressive disease manifestations, with patients at risk of premature mortality as a result of renal, cardiovascular or cerebrovascular complications. In recent years there has been a growing awareness that the first signs and symptoms of Fabry disease may begin during childhood. Studies show that clinical manifestations such as pain, hypohidrosis, gastrointestinal disturbances, angiokeratomas, cornea verticillata and acroparaesthesiae may be common in childhood and that such manifestations may become apparent during the first few years of life. Despite the early onset of these signs and symptoms, however, diagnosis is often delayed. Interest is now focused on whether enzyme replacement therapy can slow or prevent the onset of these disease manifestations. Preliminary data from two studies suggest that treatment with agalsidase alfa is well tolerated in children and that it may have beneficial clinical effects; however, further research is needed to determine whether enzyme replacement therapy can prevent the development of disease manifestations.Conclusion: The manifestations of Fabry disease first become apparent during childhood. It is well known that disease‐associated manifestations are progressive; however, it has yet to be determined whether specific treatment with enzyme replacement therapy can prevent the development of the associated severe and life‐threatening complications.
CNS‐directed gene therapy for lysosomal storage diseases
Authors: Sands Mark S , Haskins Mark E 
Published: March 2008

AbstractLysosomal storage diseases (LSDs) are a group of inherited metabolic disorders usually caused by deficient activity of a single lysosomal enzyme. As most lysosomal enzymes are ubiquitously expressed, a deficiency in a single enzyme can affect multiple organ systems, including the central nervous system (CNS). At least 75% of all LSDs have a significant CNS component. Approaches such as bone marrow transplantation (BMT) or enzyme replacement therapy (ERT) can effectively treat the systemic disease associated with LSDs in some patients. However, CNS disease remains a major challenge. Gene therapy represents a promising approach for the treatment of CNS disease as it has the potential to provide a permanent source of the deficient enzyme. Direct intracranial injection of viral gene transfer vectors has resulted in reduced lysosomal storage and functional improvement in certain small (rodent) and large (canine and feline) animal models of LSDs. The addition of protein transduction domains (PTDs) to the recombinant enzymes increased the distribution of enzyme and the extent of correction. Therapeutic levels of lysosomal enzymes can also be delivered to distant sites in the brain by anterograde and retrograde axonal transport. Finally, combining disparate approaches such as BMT and CNS‐directed gene therapy can increase treatment efficacy in LSDs with severe CNS disease that are refractory to more conventional approaches.Conclusion: The development of gene transfer vectors that mediate persistent expression in vivo, the addition of PTDs, a better understanding of lysosomal enzyme trafficking and combining different therapies provide hope that the CNS component of LSDs can be effectively treated.
Effects of Perinatal, Combined Exposure to 1,4‐Dichlorobenzene and 1,1‐Dichloro‐2,2‐bis(p‐chlorophenyl)ethylene on Rat Female Reproductive System
Authors: Makita Yuji 
Published: March 2008

Abstract:  Prenatal or early postnatal exposure to some synthetic chemicals may affect the later reproductive system of the offspring. 1,4‐Dichlorobenzene (DCB) is used as an air freshener and a moth repellent and 1,1‐dichloro‐2,2‐bis(p‐chlorophenyl) ethylene (p,p′‐DDE) is a persistent metabolite of 1,1,1‐trichloro‐2,2‐bis(p‐chlorophenyl)ethane that was used as a pesticide before. DCB concentrations of residential air and oral p,p′‐DDE intake through marine products are demonstrated to be rather high in Japan. Such situations lead to high body burden of these pollutants in pregnant women. Consequently, foetuses and neonates will be exposed much more to DCB and p,p′‐DDE via the mother. Therefore, the effects of the perinatal, combined exposure to DCB and p,p′‐DDE on the female reproductive system have been investigated in mature rat female offspring of dams ingesting 25 p.p.m. DCB (approximately 2 mg/kg) and 125 p.p.m. p,p′‐DDE (approximately 10 mg/kg) during the gestational and lactational period. Sexual maturation was fully developed in the rat female offspring perinatally exposed to DCB and/or p,p′‐DDE through maternal exposure. The combined effect of DCB and p,p′‐DDE was observed, and the ovarian weight was seen to decrease to approximately 80% of the control rat in matured female offspring following perinatal exposure to DCB and p,p′‐DDE. This alteration might lead to reproductive dysfunction in matured female offspring. However, biological relevance of the alteration in the ovary remained uncertain in the present study. Further investigations concerning the reproductive function and mechanistic implication are required for elucidating the combined effects of perinatal exposure to DCB and p,p′‐DDE on the later female reproductive system entirely.