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Nadine?AME?van der BeekEmail author Juna?M?de Vries Marloes?LC?Hagemans Wim?CJ?Hop Marian?A?Kroos John?HJ?Wokke Marianne?de Visser Baziel?GM?van Engelen Jan?BM?Kuks Anneke?J?van der Kooi Nicolette?C?Notermans Karin?G?Faber Jan?JGM?Verschuuren Arnold?JJ?Reuser Ans?T?van der Ploeg Pieter?A?van Doorn 《Orphanet journal of rare diseases》2012,7(1):88
Background
Due partly to physicians’ unawareness, many adults with Pompe disease are diagnosed with great delay. Besides, it is not well known which factors influence the rate of disease progression, and thus disease outcome. We delineated the specific clinical features of Pompe disease in adults, and mapped out the distribution and severity of muscle weakness, and the sequence of involvement of the individual muscle groups. Furthermore, we defined the natural disease course and identified prognostic factors for disease progression.Methods
We conducted a single-center, prospective, observational study. Muscle strength (manual muscle testing, and hand-held dynamometry), muscle function (quick motor function test), and pulmonary function (forced vital capacity in sitting and supine positions) were assessed every 3–6 months and analyzed using repeated-measures ANOVA.Results
Between October 2004 and August 2009, 94 patients aged between 25 and 75 years were included in the study. Although skeletal muscle weakness was typically distributed in a limb-girdle pattern, many patients had unfamiliar features such as ptosis (23%), bulbar weakness (28%), and scapular winging (33%). During follow-up (average 1.6 years, range 0.5-4.2 years), skeletal muscle strength deteriorated significantly (mean declines of ?1.3% point/year for manual muscle testing and of ?2.6% points/year for hand-held dynamometry; both p<0.001). Longer disease duration (>15 years) and pulmonary involvement (forced vital capacity in sitting position <80%) at study entry predicted faster decline. On average, forced vital capacity in supine position deteriorated by 1.3% points per year (p=0.02). Decline in pulmonary function was consistent across subgroups. Ten percent of patients declined unexpectedly fast.Conclusions
Recognizing patterns of common and less familiar characteristics in adults with Pompe disease facilitates timely diagnosis. Longer disease duration and reduced pulmonary function stand out as predictors of rapid disease progression, and aid in deciding whether to initiate enzyme replacement therapy, or when.16.
Branson DH 《Environmental entomology》2008,37(3):686-695
The complex interplay between grasshoppers, weather conditions, and plants that cause fluctuations in grasshopper populations remains poorly understood, and little is known about the ecological processes that generate grasshopper outbreaks. Grasshopper populations respond to interacting extrinsic and intrinsic factors, with yearly and decadal weather patterns and the timing of precipitation all potentially important. The effects of initial and increasing grasshopper densities on grasshopper survival and reproductive correlates were examined at a northern mixed-grass prairie site through manipulations of grasshopper densities inside 10-m2 cages. High-quality grass growth occurred after a 9.1-cm mid-August rain. Reduced proportional survival was apparent in the two higher density treatments before the rain, indicative of food-limited density-dependent mortality. However, the large late summer rainfall event mediated the effects of exploitative competition on demographic characteristics because of the high-quality vegetation growth. This led to weaker effects of food limitation on survival and reproduction at the end of the experiment. The results indicate a direct link between weather variation, resource quality and grasshopper population dynamics led to a severe grasshopper outbreak and show that infrequent large precipitation events can have significant effects on population dynamics. Additional research is needed to examine the importance of infrequent large precipitation events on grasshopper population dynamics in grassland ecosystems. 相似文献
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Herman Branson 《Bulletin of mathematical biology》1942,4(3):131-137
The behavior of the diffusion coefficient of a solute which can be adsorbed by a colloid only after the colloid has aggregated
to a certain size is deduced on the basis of a few assumptions. Some relations of such a mechanism to cell reactions are indicated. 相似文献
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Hutchinson AB Patel P Sansom SL Farnham PG Sullivan TJ Bennett B Kerndt PR Bolan RK Heffelfinger JD Prabhu VS Branson BM 《PLoS medicine》2010,7(9):e1000342
Background
Detection of acute HIV infection (AHI) with pooled nucleic acid amplification testing (NAAT) following HIV testing is feasible. However, cost-effectiveness analyses to guide policy around AHI screening are lacking; particularly after more sensitive third-generation antibody screening and rapid testing.Methods and Findings
We conducted a cost-effectiveness analysis of pooled NAAT screening that assessed the prevention benefits of identification and notification of persons with AHI and cases averted compared with repeat antibody testing at different intervals. Effectiveness data were derived from a Centers for Disease Control and Prevention AHI study conducted in three settings: municipal sexually transmitted disease (STD) clinics, a community clinic serving a population of men who have sex with men, and HIV counseling and testing sites. Our analysis included a micro-costing study of NAAT and a mathematical model of HIV transmission. Cost-effectiveness ratios are reported as costs per quality-adjusted life year (QALY) gained in US dollars from the societal perspective. Sensitivity analyses were conducted on key variables, including AHI positivity rates, antibody testing frequency, symptomatic detection of AHI, and costs. Pooled NAAT for AHI screening following annual antibody testing had cost-effectiveness ratios exceeding US$200,000 per QALY gained for the municipal STD clinics and HIV counseling and testing sites and was cost saving for the community clinic. Cost-effectiveness ratios increased substantially if the antibody testing interval decreased to every 6 months and decreased to cost-saving if the testing interval increased to every 5 years. NAAT was cost saving in the community clinic in all situations. Results were particularly sensitive to AHI screening yield.Conclusions
Pooled NAAT screening for AHI following negative third-generation antibody or rapid tests is not cost-effective at recommended antibody testing intervals for high-risk persons except in very high-incidence settings. Please see later in the article for the Editors'' Summary 相似文献19.
Herman Branson 《Bulletin of mathematical biology》1946,8(4):159-165
An integral equation is established applicable to many types of metabolizing systems. The equation is applied to first order chemical reactions and to some biological systems. 相似文献
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