scholarly journals Successful Plasmapheresis and Immunoglobulin treatment for severe lipid storage myopathy: doing the right thing for the wrong reason

2021 ◽  
Author(s):  
Felix Kleefeld ◽  
Anja von Renesse ◽  
Carsten Dittmayer ◽  
Lutz Harms ◽  
Josefine Radke ◽  
...  
Keyword(s):  
Lipid Storage ◽  
Lipid Storage Myopathy ◽  
Wrong Reason ◽  
Immunoglobulin Treatment ◽  
The Right

Song pitch – habitat relationships in white-throated sparrows: cracks in acoustic windows?

1988 ◽  
Vol 66 (11) ◽  
pp. 2578-2581 ◽  
Author(s):  
Joseph R. Waas
Keyword(s):  
Boreal Forest ◽  
Inverse Relationship ◽  
Habitat Type ◽  
Zonotrichia Albicollis ◽  
Acoustic Window ◽  
Wrong Reason ◽  
Foliage Density ◽  
The Right ◽  
Forest Habitats

I compared the hypothesis that birds singing from song posts above 1–2 m should sing in as low a pitch as possible regardless of habitat type, with the traditional "acoustic window" hypothesis which argues that selection favours an inverse relationship between song pitch and foliage density. Songs of white-throated sparrows, Zonotrichia albicollis, were obtained from four habitats which could be arranged in a gradient of foliage density. The pitch of these songs was found to be inversely related to the density of foliage in the habitat occupied, consistent with the acoustic window hypothesis. Significant pitch variation occurred not only between open and forest habitats, but also between mixed and boreal forest habitats. I distinguish between hypotheses that could explain support for the traditional hypothesis despite recent data suggesting that low pitched sound travels best regardless of habitat at song post heights used by white-throated sparrows (5–10 m). I conclude that the acoustic window model may make the right prediction for the wrong reason in white-throated sparrows. I emphasize the need to define and test the assumptions of models before the models themselves are accepted on the basis of support for their predictions.

Doing the Right Thing for the Wrong Reason

2017 ◽  
Vol 99 (3) ◽  
pp. 596-597
Author(s):  
Irwin H. Lee ◽  
Avraham Eisbruch
Keyword(s):  
Wrong Reason ◽  
The Right

LACTIC ACIDEMIA, HYPERALANEMIA AND CARNITINE ABNORMALITIES IN DOGS WITH LIPID STORAGE MYOPATHY

1995 ◽  
Vol 54 (3) ◽  
pp. 457 ◽  
Author(s):  
G D Shelton ◽  
R H Hass ◽  
B Holmes ◽  
L Mitchell ◽  
R Broock ◽  
...  
Keyword(s):  
Lipid Storage ◽  
Lipid Storage Myopathy ◽  
Lactic Acidemia

scholarly journals Bias in CMIP6 models as compared to observed regional dimming and brightening

2020 ◽  
Vol 20 (24) ◽  
pp. 16023-16040
Author(s):  
Kine Onsum Moseid ◽  
Michael Schulz ◽  
Trude Storelvmo ◽  
Ingeborg Rian Julsrud ◽  
Dirk Olivié ◽  
...  
Keyword(s):  
Sulfur Dioxide ◽  
Surface Energy ◽  
Coupled Model ◽  
Shortwave Radiation ◽  
Anthropogenic Aerosol ◽  
Surface Energy Fluxes ◽  
Aerosol Emission ◽  
Wrong Reason ◽  
The Right ◽  
Aerosol Emissions

Abstract. Anthropogenic aerosol emissions have increased considerably over the last century, but climate effects and quantification of the emissions are highly uncertain as one goes back in time. This uncertainty is partly due to a lack of observations in the pre-satellite era, making the observations we do have before 1990 additionally valuable. Aerosols suspended in the atmosphere scatter and absorb incoming solar radiation and thereby alter the Earth's surface energy balance. Previous studies show that Earth system models (ESMs) do not adequately represent surface energy fluxes over the historical era. We investigated global and regional aerosol effects over the time period 1961–2014 by looking at surface downwelling shortwave radiation (SDSR). We used observations from ground stations as well as multiple experiments from eight ESMs participating in the Coupled Model Intercomparison Project Version 6 (CMIP6). Our results show that this subset of models reproduces the observed transient SDSR well in Europe but poorly in China. We suggest that this may be attributed to missing emissions of sulfur dioxide in China, sulfur dioxide being a precursor to sulfate, which is a highly reflective aerosol and responsible for more reflective clouds. The emissions of sulfur dioxide used in the models do not show a temporal pattern that could explain observed SDSR evolution over China. The results from various aerosol emission perturbation experiments from DAMIP, RFMIP and AerChemMIP show that only simulations containing anthropogenic aerosol emissions show dimming, even if the dimming is underestimated. Simulated clear-sky and all-sky SDSR do not differ greatly, suggesting that cloud cover changes are not a dominant cause of the biased SDSR evolution in the simulations. Therefore we suggest that the discrepancy between modeled and observed SDSR evolution is partly caused by erroneous aerosol and aerosol precursor emission inventories. This is an important finding as it may help interpret whether ESMs reproduce the historical climate evolution for the right or wrong reason.

Primary carnitine deficiency: adult onset lipid storage myopathy with a mild clinical course

2004 ◽  
Vol 11 (8) ◽  
pp. 919-924 ◽  
Author(s):  
S. Vielhaber ◽  
H. Feistner ◽  
J. Weis ◽  
J. Kreuder ◽  
M. Sailer ◽  
...  
Keyword(s):  
Clinical Course ◽  
Carnitine Deficiency ◽  
Lipid Storage ◽  
Adult Onset ◽  
Lipid Storage Myopathy ◽  
Primary Carnitine Deficiency

LIPID STORAGE MYOPATHY ASSOCIATED WITH LOW ACYL-COA DEHYDROGENASE ACTIVITIES

Brain ◽  
1988 ◽  
Vol 111 (4) ◽  
pp. 815-828 ◽  
Author(s):  
D. M. TURNBULL ◽  
I. M. SHEPHERD ◽  
B. ASHWORTH ◽  
K. BARTLETT ◽  
M. A. JOHNSON ◽  
...  
Keyword(s):  
Lipid Storage ◽  
Lipid Storage Myopathy

Short-Chain Acyl-CoA Dehydrogenase Deficiency Associated with a Lipid-Storage Myopathy and Secondary Carnitine Deficiency

1984 ◽  
Vol 311 (19) ◽  
pp. 1232-1236 ◽  
Author(s):  
Douglass M. Turnbull ◽  
Kim Bartlett ◽  
David L. Stevens ◽  
K. George M. M. Alberti ◽  
G. John Gibson ◽  
...  
Keyword(s):  
Dehydrogenase Deficiency ◽  
Carnitine Deficiency ◽  
Lipid Storage ◽  
Short Chain ◽  
Lipid Storage Myopathy ◽  
Chain Acyl

scholarly journals Late-onset multiple acyl-CoA dehydrogenase deficiency mimicking myositis in an elderly patient: a case report

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Yiming Zheng ◽  
Yawen Zhao ◽  
Wei Zhang ◽  
Zhaoxia Wang ◽  
Yun Yuan
Keyword(s):  
Elderly Patient ◽  
Muscle Weakness ◽  
Dehydrogenase Deficiency ◽  
Late Onset ◽  
Lipid Storage ◽  
Exercise Intolerance ◽  
Lipid Storage Myopathy ◽  
Case Presentation ◽  
Acylcarnitine Profile ◽  
Progressive Weakness

Abstract Background Late-onset multiple acyl-CoA dehydrogenase deficiency (MADD) is a rare and treatable inherited lipid storage myopathy. Here, we report an elderly patient with MADD mimicking myositis. Case presentation An 80-year-old woman had progressive weakness in her limbs, exercise intolerance, and no muscle pain for 3 months. The patient’s serum creatine kinase level was slightly elevated. The initial diagnosis was myositis. However, muscle biopsy showed many cytoplasmic vacuoles stained with oil red O, indicating the presence of lipid storage myopathy. The plasma acylcarnitine profile showed increased medium-chain and long-chain acylcarnitine species, consistent with the diagnosis of MADD. Riboflavin treatment dramatically improved muscle weakness. Conclusions MADD should be considered when evaluating elderly patients with subacute muscle weakness.

Lipid Storage Myopathy in Multiple Acyl-CoA Dehydrogenase Deficiency: An Adult Case

1992 ◽  
Vol 32 (3) ◽  
pp. 170-176 ◽  
Author(s):  
T. Mongini ◽  
C. Doriguzzi ◽  
L. Palmucci ◽  
A. De Francesco ◽  
L. Bet ◽  
...  
Keyword(s):  
Dehydrogenase Deficiency ◽  
Lipid Storage ◽  
Adult Case ◽  
Lipid Storage Myopathy

A new lipid storage myopathy observed in individuals with the Ruvalcaba-Myhre-Smith syndrome

1984 ◽  
Vol 18 (1) ◽  
pp. 163-167 ◽  
Author(s):  
J. H. DiLiberti ◽  
A. N. D'Agostino ◽  
R. H. A. Ruvalcaba ◽  
J. R. Schimschock ◽  
John M. Opitz
Keyword(s):  
Lipid Storage ◽  
Lipid Storage Myopathy
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