6 research outputs found
Hip Morphology in Mucolipidosis Type II
Mucolipidosis type II (MLII) is a rare lysosomal storage disorder caused by defective
trafficking of lysosomal enzymes. Severe skeletal manifestations are a hallmark of the disease
including hip dysplasia. This study aims to describe hip morphology and the natural course of
hip pathologies in MLII by systematic evaluation of plain radiographs, ultrasounds and magnetic
resonance imaging (MRI). An international two-centered study was performed by retrospective
chart review. All MLII patients with at least one pelvic radiograph were included. A total of 16
patients were followed over a mean of 3.5 years (range 0.2–10.7 years). Typical age-dependent
radiographic signs identified were femoral cloaking (7/16), rickets/hyperparathyroidism-like changes
(6/16) and constrictions of the supra-acetabular part of the os ilium (16/16) and the femoral neck (7/16).
The course of acetabular and migration indexes (AI, MI) significantly increased in female patients.
However, in the overall group, there was no relevant progression of acetabular dysplasia with a
mean AI of 23.0 (range 5◦–41◦
) and 23.7â—¦
(range 5◦–40◦
) at the first and last assessments, respectively.
Better knowledge on hip morphology in MLII could lead to earlier diagnosis, improved clinical
management and enables assessment of effects of upcoming therapies on the skeletal system
Architecture, heterogeneity, and origin of late Miocene fluvial deposits hosting the most important aquifer in the Great Plains, USA
Changes in Activities of Respiratory Enzymes in Lungs of Guinea-pigs Exposed to Silica Dust: II. Comparison of the Effects of Quartz Dust and Lampblack on the Succinate Oxidase System
Exploration of prostanoid receptor subtype regulating estradiol and prostaglandin E2 induction of spinophilin in developing preoptic area neurons
Characterization of the LIGO detectors during their sixth science run
In 2009-2010, the Laser Interferometer Gravitational-Wave Observatory (LIGO) operated together with international partners Virgo and GEO600 as a network to search for gravitational waves (GWs) of astrophysical origin. The sensitivity of these detectors was limited by a combination of noise sources inherent to the instrumental design and its environment, often localized in time or frequency, that couple into the GW readout. Here we review the performance of the LIGO instruments during this epoch, the work done to characterize the detectors and their data, and the effect that transient and continuous noise artefacts have on the sensitivity of LIGO to a variety of astrophysical sources