bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intracavity optical parametric oscillator pumped by passively Q-switched Nd:YLF laser
Authors: Żendzian W. , Jabczyński J.K. , Kwiatkowski J. 
Published: November 2008

AbstractWe report on a passively Q-switched end pumped Nd:YLF laser including a noncritically phase-matched KTP singly resonant intracavity optical parametric oscillator (IOPO-KTP). For the Q-switching operation we have used Cr:YAG saturable absorber. The optimized passively Q-switched Nd:YLF laser without IOPO generated linearly polarized pulses of 11.5 ns and 1.07 mJ at 1047 nm. The conversion efficiency of the optimized Q-switched pulse energy at 1047 nm to 1547 nm of a signal approached about 47%. For optimizing both Nd:YLF laser and IOPO we have numerically solved a theoretical model. We have achieved 1.6-ns duration pulses at 1547 nm with energy of 0.5 mJ and peak power of above 300 kW. The beam quality was excellent (M2 ≈1).
Thalassemias and Other Hemoglobinopathies in Former Yugoslavia
Authors: Efremov G 
Published: November 2008

Thalassemias and Other Hemoglobinopathies in Former YugoslaviaThis review summarizes our results on the epidemiology and molecular basis of thalassemias and other hemoglobinopathies in the republics and provinces of the Former Yugoslavia. Over the past 40 years, surveys of more than 37,000 school children and more than 1,600 adults, from all over Former Yugoslavia, except Slovenia, have shown an average incidence of β-thalassemia (β-thal) trait of 1.2%, ranging from 2.9% in the south (Macedonia) to 0.8% in the northwest (Croatia). The frequency of δβ-thal was 0.2%, while that of Swiss type hereditary persistence of fetal hemoglobin (HPFH) was 0.4%. Screening of 12,680 newborns has shown that the frequency of α-thal trait was 1.5%. The molecular basis of the thalassemias in the populations of Former Yugoslavia has been completely defined. More than 700 β-thal chromosomes have been studied and their molecular defect was determined. In the Macedonian population, 16 different β-thal mutations were detected, four of which (IVS-I-110, G→A; IVS-I-6, T>C; IVS-I-1, G>A and codon 39, C>T) accounted for 85% of all β-thal chromosomes. In the Croatian population, 18 different β-thal alleles were detected. Four new mutations [nucleotide (nt) -87, C>A; IVS-II-850, G>C; initiation codon mutation T>C; polyadenylation signal (poly A), AATAAA>AATGAA)] and one new deletion (1605 bp), were characterized. Molecular analyses of DNA from over 50 unrelated cases with δβ-thal have shown that this condition was mainly caused by a 13 kb deletion (Sicilian type); in one family, a deletion of >18 to 23 kb (Macedonian-Turkish type), and in another, a deletion of 148 kb (Yugoslavian type of εγδβ-thal) of the β-globin gene complex, were discovered. Molecular analyses of α-thal from Former Yugoslavia revealed the following defects: the -20.5, -17.5 and -3.7 kb deletions, a 5 nt deletion, and Hb Icaria [α142, Term→Lys (TAA>TCA in α2)]. The incidence of abnormal hemoglobins (Hbs) in Former Yugoslavia was 0.3%. Five different α chain variants in 16 families, 16 different β chain variants in 61 families, one δ chain variant in one family, two types of Hb Lepore in 122 families and two γ chain variants, have been characterized.
Molecular Analysis of Friedreich's Ataxia in Macedonian Patients
Authors: Kocheva S , Trivodalieva S , Vlaski-Jekic S , Kuturec M , Efremov G 
Published: November 2008

Molecular Analysis of Friedreich's Ataxia in Macedonian PatientsFriedreich's ataxia (FRDA) is rare a progressive neurodegenerative disorder of autosomal recessive inheritance, which is associated with an unstable expansion of a GAA trinucleotide repeat in the first intron of the frataxin gene on chromosome 9q13. We have performed molecular analyses of the frataxin gene of 40 patients with spinocerebellar ataxia from the Republic of Macedonia. Fifteen had early onset of progressive ataxia (before the age of 25), while the remainder were over 25 years old at the time of diagnosis. Only 14 patients had a mutation in the frataxin gene and all of these had early onset ataxia. The number of GAA repeats was in the normal range in 50 healthy individuals.
A Randomized Clinical Trial of Aquatic versus Land Exercise to Improve Balance, Function, and Quality of Life in Older Women with Osteoporosis
Authors: Arnold C.M. , Busch A.J. , Schachter C.L. , Harrison E.L. , Olszynski W.P. 
Published: November 2008

Purpose: Despite the decreased gravitational loading that is experienced in an aquatic environment, little research has been conducted on this exercise medium for women with osteoporosis (OP). Aquatic exercise (AE) may improve function and balance, thus ultimately decreasing fall risk and the potential for hip fractures in this high-risk population.Method: A total of 68 women with OP, aged 60 years or older, were recruited into a randomized clinical trial evaluating the impact of AE, land exercise (LE), and no exercise (NE) on balance, functional mobility, and quality of life (QOL).Results: Only one balance measure (backward tandem walk) significantly improved with AE compared to LE, but this did not translate into a greater improvement in self-report function. There were no significant differences between the exercise interventions and NE, except for in ratings of global change, where participants in the AE group were three times more likely to report improvement than those in the NE group.Conclusion: There were no differences in balance, function, or QOL in women with OP who followed an AE or LE programme compared to those in an NE control group. However, the significant differences in backward tandem walk between the AE and LE groups and self-reported global change between the AE and NE groups warrant further investigation. Significant improvements in balance and global change suggest that AE is a viable alternative for older women with OP who have difficulty exercising on land.
Intracytoplasmic Cytokine Expression and T Cell Subset Distribution in the Peripheral Blood of Patients with Ankylosing Spondylitis
Authors: SZÁNTÓ SÁNDOR , ALEKSZA MAGDOLNA , MIHÁLY ERZSÉBET , LAKOS GABRIELLA , SZABÓ ZOLTÁN , VÉGVÁRI ANIKÓ , SIPKA SÁNDOR , SZEKANECZ ZOLTÁN 
Published: November 2008

ObjectiveTo determine the role of inflammatory mediators in the pathogenesis of ankylosing spondylitis (AS), we investigated peripheral blood lymphocyte subsets and their intracellular cytokine production.MethodsThe percentages of T and B lymphocytes, natural killer (NK) cells, activated T lymphocytes, CD4+ T helper (Th), and CD8+ T cytotoxic (Tc) cells were determined by flow cytometry in 42 patients with AS compared to 52 healthy controls. In order to assess circulating Th1/Th2 and Tc1/Tc2 subsets, we used a whole-blood cytometric assay based on the intracellular interferon-γ, interleukin 4 (IL-4), and IL-10 expression of the cells.ResultsIn the peripheral blood, the frequencies of CD4+ T helper and CD56+ NK cells were higher in AS (54.8% and 16.2%, respectively) compared to controls (45.3% and 10.8%) (p < 0.05). The frequencies of Th0 (1.9% vs 0.8%) and Tc0 (2.1% vs 0.8%) cells were higher, while that of Tc1 cells was lower (26.6% vs 40.1%) in patients with AS versus controls (p < 0.05). The percentage of IL-10-producing Tc cells was significantly higher in AS (18.4%) versus controls (8.5%) (p < 0.05). Finally, the active phase of AS was associated with significantly lower percentage of IL-10-producing Tc cells in the peripheral blood (6.6%) compared to patients with inactive AS (23.1%).ConclusionOur results provide further evidence for an altered T cell subset distribution and intracytoplasmic cytokine balance in AS.
Systemic Lupus Erythematosus Features in Rheumatoid Arthritis and Their Effect on Overall Mortality
Authors: ICEN MURAT , NICOLA PAULO J. , MARADIT-KREMERS HILAL , CROWSON CYNTHIA S. , THERNEAU TERRY M. , MATTESON ERIC L. , GABRIEL SHERINE E. 
Published: November 2008

ObjectiveFeatures of systemic lupus erythematosus (SLE) are commonly observed in patients with rheumatoid arthritis (RA). However, their frequency and clinical significance are uncertain. We examined the frequency of SLE features in RA and their effect on overall mortality.MethodsWe assembled a population-based incidence cohort of subjects aged ≥ 18 years first diagnosed with RA [1987 American College of Rheumatology (ACR) criteria] between 1955 and 1995. Information regarding disease characteristics, therapy, comorbidities, and SLE features (1982 ACR criteria) were collected from the complete inpatient and outpatient medical records. Cox regression models were used to estimate the mortality risk associated with lupus features.ResultsThe study population comprised 603 subjects with incident RA (mean age 58 yrs, 73% women) with a mean followup time of 15 years. By 25 years after RA incidence, ≥ 4 SLE features were observed in 15.5% of the subjects with RA. After adjustment for age and sex, occurrence of ≥ 4 SLE features was associated with increased overall mortality [hazard ratio (HR) 5.54, 95% confidence interval (CI) 3.59–8.53].With further adjustment for RA characteristics, therapy, and comorbidities, the association weakened but remained statistically significant (HR 2.56, 95% CI 1.60–4.08). After adjustment for age, sex, RA characteristics, therapy, and comorbidities, thrombocytopenia (2.0, 95% CI 1.2, 3.1) and proteinuria (1.8, 95% CI 1.3, 2.6) were significantly associated with mortality.ConclusionSLE features were common in RA, given sufficient observation time. Subjects with RA who developed ≥ 4 SLE features had an increased risk of death. Proteinuria and thrombocytopenia were individually associated with an increased mortality risk.
Serum Autoantibodies and Their Clinical Associations in Patients with Childhood- and Adult-Onset Linear Scleroderma. A Single-Center Study
Authors: ARKACHAISRI THASCHAWEE , FERTIG NOREEN , PINO SALLY , MEDSGER THOMAS A. 
Published: November 2008

ObjectiveTo determine the frequency of selected serum autoantibodies and their clinical associations in patients with childhood-onset (ChO) or adult-onset (AO) linear scleroderma (LiScl) evaluated at a single institution.MethodsSeventy-two patients (ChO = 40, AO = 32), including 12 with en coup de sabre, were studied. All ChO patients had disease onset before age 16 years. Clinical features (extent of cutaneous disease, activity, and joint contractures) were recorded. Antinuclear antibodies (ANA) were identified by indirect immunofluorescence (HEp-2 cells), and anti-single-stranded DNA (anti-ssDNA), antihistone (AHA), and antichromatin (AChA) autoantibodies were detected by ELISA.ResultsThere were no significant differences between groups in regard to gender, proportion with LiScl/E, or clinical features except joint contractures (ChO > AO; p = 0.04). There were no differences in the frequency of ANA or other autoantibodies between the groups except for AHA (ChO > AO). AHA was more frequently found with anti-ssDNA (p < 0.0001). LiScl patients with positive anti-ssDNA and/or AHA had more extensive cutaneous involvement and more often had joint contractures (p < 0.05). Anti-ssDNAwas present more frequently inAO than in ChO patients with active lesions (p = 0.04). ANA and AChA were not associated with any clinical features. Both AHA and anti-ssDNA levels showed good correlation with disease severity.ConclusionOver two-thirds of LiScl patients had ANA. Patients with ChO were similar to those with AO with regard to the frequency of selected serum autoantibodies. Anti-ssDNA and AHA were frequently found together and both were associated with more extensive skin disease with joint contractures. LiScl disease severity correlated with the serum levels of both these antibodies.
Fibromyalgia, Systemic Lupus Erythematosus (SLE), and Evaluation of SLE Activity
Authors: WOLFE FREDERICK , PETRI MICHELLE , ALARCÓN GRACIELA S. , GOLDMAN JOHN , CHAKRAVARTY ELIZA F. , KATZ ROBERT S. , KARLSON ELIZABETH W. 
Published: November 2008

ObjectiveTo determine if fibromyalgia (FM) or fibromyalgia-ness (the tendency to respond to illness and psychosocial stress with fatigue, widespread pain, general increase in symptoms, and similar factors) is increased in patients with compared to those without systemic lupus erythematosus (SLE); to determine whether FM or fibromyalgia-ness biases the SLE Activity Questionnaire (SLAQ); and to determine if the SLAQ is overly sensitive to FM symptoms.MethodsWe developed a 16-item SLE Symptom Scale (SLESS) modeled on the SLAQ and used that scale to investigate the relation between SLE symptoms and fibromyalgia-ness in 23,321 patients with rheumatic disease. FM was diagnosed by survey FM criteria, and fibromyalgia-ness was measured using the Symptom Intensity (SI) Scale. As comparison groups, we combined patients with rheumatoid arthritis and noninflammatory rheumatic disorders into an “arthritis” group and also utilized a physician-diagnosed group of patients with FM.ResultsFM was identified in 22.1% of SLE and 17.0% of those with arthritis. The SI scale was minimally increased in SLE. The correlation between SLAQ and SLESS was 0.738. SLESS/SLAQ scale items (Raynaud’s phenomenon, rash, fever, easy bruising, hair loss) were significantly more associated with SLE than FM, while the reverse was true for headache, abdominal pain, paresthesias/stroke, fatigue, cognitive problems, and muscle pain or weakness. There was no evidence of disproportionate symptom-reporting associated with fibromyalgia-ness. Self-reported SLE was associated with an increased prevalence of FM that was unconfirmed by physicians, compared to SLE confirmed by physicians.ConclusionThe prevalence of FM in SLE is minimally increased compared with its prevalence in patients with arthritis. Fibromyalgia-ness does not bias the SLESS and should not bias SLE assessments, including the SLAQ.
Inhibition of Src Homology 2 Domain-Containing Protein Tyrosine Phosphatase Substrate-1 Reduces the Severity of Collagen-Induced Arthritis
Authors: TANAKA KONAGI , HORIKAWA TATSUYA , SUZUKI SATSUKI , KITAURA KAZUTAKA , WATANABE JUNKO , GOTOH AKITO , SHIOBARA NORIYUKI , ITOH TSUNETOSHI , YAMANE SHOJI , SUZUKI RYUJI , FUKUI NAOSHI , OCHI TAKAHIRO 
Published: November 2008

ObjectiveTo investigate whether the blockade of Src homology 2 domain-containing protein tyrosine phosphatase substrate-1 (SHPS-1) has any therapeutic effects on rheumatoid arthritis.MethodsA functional blocking monoclonal antibody for SHPS-1 (anti-SHPS-1 mAb) was administered at various doses to collagen-induced arthritis (CIA) mice, and severity of the arthritis was evaluated by clinical and histological scores of the limbs. To clarify the mechanisms of action of the antibody, the serum concentration of anti-type II collagen antibody was measured in those mice, and in vitro experiments were conducted to determine the effects of the antibody on the induction of osteoclasts and the release of cytokines from mouse spleen cells.ResultsCompared with mice given control IgG, the administration of anti-SHPS-1 mAb significantly reduced the severity of inflammation and destruction of bone and cartilage in CIA mice. This therapeutic effect was observed even when the antibody treatment was started after the onset of arthritis. The appearance of anti-type II collagen antibody in CIA mice was not altered by the antibody treatment. In in vitro experiments, the anti-SHPS-1 mAb significantly inhibited osteoclastogenesis of bone marrow cells, and significantly reduced the release of interleukin 1β (IL-1β), IL-2, IL- 12, interferon-γ, and tumor necrosis factor-α, but not that of IL-4 or IL-10, from the spleen cells after stimulation with concanavalin A.ConclusionAdministration of a monoclonal antibody for SHPS-1 reduced the severity of arthritis in CIA mice. Regulation of biological functions of SHPS-1 may be a novel and potent strategy to treat patients with rheumatoid arthritis.
Cost Comparison Between Mycophenolate Mofetil and Cyclophosphamide-Azathioprine in the Treatment of Lupus Nephritis
Authors: TSE KAI CHUNG , TANG COLIN S.O. , LAM MAN FAI , YAP DESMOND Y.H. , CHAN TAK MAO 
Published: November 2008

Objective.To compare the healthcare expenditure associated with mycophenolate mofetil (MMF)-based immunosuppression in contrast to conventional therapy in patients with lupus nephritis.Methods.Our retrospective single-center study compared the major healthcare costs during the first 24 months of treatment incurred by immunosuppressive medications, hospitalization, and complications in patients with severe lupus nephritis who had been treated with prednisolone and either MMF or sequential cyclophosphamide induction followed by azathioprine maintenance (CTX-AZA).Results.Forty-four patients were studied (22 in each group). Baseline demographic and clinical measures, and remission rates after treatment, were similar between the 2 groups. Immunosuppressive drug cost was 13.6-fold higher in the MMF group (US$4168.3 ± 1176.5 per patient, compared with $285.0 ± 70.6 in the CTX-AZA group, mean difference $3883.2 ± 251.3; p < 0.001). MMF treatment was associated with a lower incidence of infections (12.0 episodes/1000 patient-months, compared with 32.4 in the CTX-AZA group; p = 0.035). Combined cost of hospitalization and treatment of infections was 82.5% lower in the MMF group (mean difference –2208.7 ± 1700.6; p = 0.120). Overall treatment expenditure on immunosuppressive drugs, hospitalization, and treatment of infections was 1.57-fold higher in the MMF group (mean US $4635.9 compared with $2961.5 in the CTX-AZA group; p < 0.001).Conclusion.While the cost of MMF treatment for severe lupus nephritis is much higher compared with CTX-AZA, the increased drug cost is partially offset by savings from the reduced incidence of complications.
Geroderma osteodysplastica maps to a 4 Mb locus on chromosome 1q24
Authors: Newman William G. , Clayton‐Smith Jill , Metcalfe Kay , Cole Rachel , Tartaglia Marco , Brancati Francesco , Morara Sara , Novelli Antonio , Liu Xiangdong , Siminovitch Katherine A. , Mundlos Stefan , Tassabehji May , Black Graeme C.M. 
Published: November 2008

AbstractImportant insights into the etiology of osteoporosis have been gained by the study of single gene disorders, including osteogenesis imperfecta. We report on the genetic mapping of geroderma osteodysplastica (GO), a rare autosomal recessive disorder of the connective tissue, characterized by wrinkly skin and severe osteoporosis. We undertook autozygosity mapping in one Libyan and four consanguineous Pakistani families with a total of 10 affected individuals to define a 4 Mb homozygous region on chromosome 1q24, which harbors the GO causative gene. No obvious candidate genes that encode known protein constituents of the extracellular matrix are found in the linked region. Importantly, our study demonstrates that GO is not allelic to wrinkly skin syndrome caused by mutations in ATP6V0A2. © 2008 Wiley‐Liss, Inc.
Paternal somatic mosaicism of a TGFBR2 mutation transmitting to an affected son with Loeys–Dietz syndrome
Authors: Watanabe Yoriko , Sakai Haruya , Nishimura Akira , Miyake Noriko , Saitsu Hirotomo , Mizuguchi Takeshi , Matsumoto Naomichi 
Published: November 2008

AbstractWe report on somatic mosaicism of a TGFBR2 missense mutation, c.1336G > A (D446N). The affected son with the heterozygous mutation was previously reported [Sakai et al. (2006); Am J Med Genet A 140A:1719–1725]. Further evaluation indicates his clinical condition is Loeys–Dietz syndrome. Parental blood samples were studied to confirm whether the propositus' mutation was a de novo change, and suggested a trace of the mutation in the father. DNAs extracted from blood leukocytes, buccal cells, hair root cells, and nails in the father indicated 52%, 25%, 0%, and 35% of cells harbored the mutation, respectively. This is the first detailed report of somatic mosaicism of a TGFBR2 mutation. © 2008 Wiley‐Liss, Inc.
Navajo microvillous inclusion disease is due to a mutation inMYO5B
Authors: Erickson Robert P. , Larson‐Thomé Katherine , Valenzuela Robert K. , Whitaker Stacia E. , Shub Mitchell D. 
Published: November 2008

AbstractMicrovillous Inclusion Disease (MID) is a rare, autosomal recessive gastrointestinal disease of increased frequency among the Navajos. Previous work has shown a deficiency of RAB8 in one Japanese patient, while homozygous mutations inMYO5Bwere found in 7 of 10 mostly Middle Eastern families. We have identified a shared homozygous mutation inMYO5Bin seven affected Navajos with the expected heterozygosity in five parents. We have developed a simple restriction enzyme based assay that allows for rapid screening for this mutation. © 2008 Wiley‐Liss, Inc.
Conversion of iminoacyl quinolinylpalladium (II) complexes into novel oxo‐pyrrolo[3,4‐b] quinolines via depalladation reactions
Authors: Hamad Elgazwy Abdel‐Sattar S. 
Published: November 2008

AbstractOxidative addition reactions of quinolines 1a, b with Pd(dba)2 in the presence of PPh3 (1:2) in acetone gave dinuclear palladium complexes [Pd(C,N‐2‐C9 H4N‐CHO‐3‐R‐6)Cl(PPh3)]2 [(R = H (2a), R = OMe (2b), which were reacted with isocyanide XyNC (Xy = 2,6‐Me2C6H3) to give novel iminoacyl quinolinylpalladium complexes 3a, b in good yields (81 and 77%). Cyclopalladated complexes3a, b were also obtained in low yields (39 and 33.5%) via one‐pot reaction of 1a, b with isonitrile XyNC:Pd(dba)2 (4:1). The reaction of 3a,b with Tl(TfO) (TfO = triflate, CF3SO3) in the presence of H2O or EtOH causes depalladation reactions of complexes to provide the corresponding organic compounds 4a, b, 5a, b and 6a, b in yields of 41, 27 and 18 − 19%, respectively. The products were characterized by satisfactory elemental analyses and spectral studies (IR, 1H, 13C and 31P NMR). The crystal structures 2a, 3a and3b were determined by X‐ray diffraction studies. Copyright © 2008 John Wiley & Sons, Ltd.
Estimation of reinforcement in compatibilized polypropylene nanocomposites by extensional rheology
Authors: Laske Stephan , Kracalik Milan , Gschweitl Michael , Feuchter Michael , Maier Günther , Pinter Gerald , Thomann Ralf , Friesenbichler Walter , Langecker Günter Rüdiger 
Published: November 2008

AbstractStructural characterization in polymer nanocomposites is usually performed using X‐ray scattering and microscopic techniques, whereas the improvements in processing and mechanical properties are commonly investigated by rotational rheometry and tensile testing. However, all of these techniques are time consuming and require quite expensive scientific equipment. It has been shown that a fast and efficient way of estimating the level of reinforcement in polymer nanocomposites can be performed by melt extensional rheology, because it is possible to correlate the level of melt strength with mechanical properties, which reflect both the 3D network formed by the clay platelets/polymer chains as well as final molecular structure in the filled system. The physical network made of silicate filler and polymer matrix has been evaluated by X‐ray diffraction and transmission electron microscopy. Extensional rheometry and tensile testing have been used to measure efficiency of the compatibilizer amount in a polypropylene‐nanoclay system. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2009