32 research outputs found
Should patients with Phosphomannomutase 2-CDG (PMM2-CDG) be screened for adrenal insufficiency?
PMM2-CDG is the most common congenital disorder of glycosylation (CDG) accounting for almost 65% of known CDG cases affecting N-glycosylation. Abnormalities in N-glycosylation could have a negative impact on many endocrine axes. There is very little known on the effect of impaired N-glycosylation on the hypothalamic-pituitary-adrenal axis function and whether CDG patients are at risk of secondary adrenal insufficiency and decreased adrenal cortisol production. Cortisol and ACTH concentrations were simultaneously measured between 7:44 am to 1 pm in forty-three subjects (20 female, median age 12.8 years, range 0.1 to 48.6 years) participating in an ongoing international, multi-center Natural History study for PMM2-CDG (ClinicalTrials.gov Identifier: NCT03173300). Of the 43 subjects, 11 (25.6%) had cortisol below 5 μg/dl and low to normal ACTH levels, suggestive of secondary adrenal insufficiency. Two of the 11 subjects have confirmed central adrenal insufficiency and are on hydrocortisone replacement and/or stress dosing during illness; 3 had normal and 1 had subnormal cortisol response to ACTH low-dose stimulation test but has not yet been started on therapy; the remaining 5 have upcoming stimulation testing planned. Our findings suggest that patients with PMM2-CDG may be at risk for adrenal insufficiency. Monitoring of morning cortisol and ACTH levels should be part of the standard care in patients with PMM2-CDG.Glycomine, Inc. was the sponsor of this study, and was involved in the study design and in the and interpretation of data; in the writing of the report; and in the decision to submit the article for publication.
Several authors of this publication are members of the European Reference Network for Rare Hereditary Metabolic Disorders (MetabERN) - Project ID No 739543.info:eu-repo/semantics/publishedVersio
Interchange Slip-Running Reconnection and Sweeping SEP Beams
We present a new model to explain how particles (solar energetic particles;
SEPs), accelerated at a reconnection site that is not magnetically connected to
the Earth, could eventually propagate along the well-connected open flux tube.
Our model is based on the results of a low-beta resistive magnetohydrodynamics
simulation of a three-dimensional line-tied and initially current-free bipole,
that is embedded in a non-uniform open potential field. The topology of this
configuration is that of an asymmetric coronal null-point, with a closed fan
surface and an open outer spine. When driven by slow photospheric shearing
motions, field lines, initially fully anchored below the fan dome, reconnect at
the null point, and jump to the open magnetic domain. This is the standard
interchange mode as sketched and calculated in 2D. The key result in 3D is
that, reconnected open field lines located in the vicinity of the outer spine,
keep reconnecting continuously, across an open quasi-separatrix layer, as
previously identified for non-open-null-point reconnection. The apparent
slipping motion of these field lines leads to form an extended narrow magnetic
flux tube at high altitude. Because of the slip-running reconnection, we
conjecture that if energetic particles would be traveling through, or be
accelerated inside, the diffusion region, they would be successively injected
along continuously reconnecting field lines that are connected farther and
farther from the spine. At the scale of the full Sun, owing to the super-radial
expansion of field lines below 3 solar radii, such energetic particles could
easily be injected in field lines slipping over significant distances, and
could eventually reach the distant flux tube that is well-connected to the
Earth
Sulfonated Styrene-(ethylene-co-butylene)-styrene/Montmorillonite Clay Nanocomposites: Synthesis, Morphology, and Properties
Sulfonated styrene-(ethylene-butylene)-styrene triblock copolymer (SSEBS) was synthesized by reaction of acetyl sulfate with SEBS. SSESB-clay nanocomposites were then prepared from hydrophilic Na-montmorillonite (MT) and organically (quaternary amine) modified hydrophobic nanoclay (OMT) at very low loading. SEBS did not show improvement in properties with MT-based nanocomposites. On sulfonation (3 and 6 weight%) of SEBS, hydrophilic MT clay-based nanocomposites exhibited better mechanical, dynamic mechanical, and thermal properties, and also controlled water–methanol mixture uptake and permeation and AC resistance. Microstructure determined by X-ray diffraction, atomic force microscopy, and transmission electron microscopy due to better dispersion of MT nanoclay particles and interaction of MT with SSEBS matrix was responsible for this effect. The resulting nanocomposites have potential as proton transfer membranes for Fuel Cell applications
Anatomical and radiographic appearance of the capuchin monkey thoracic cavity (Cebus apella)
Why are flare ribbons associated with the spines of magnetic null points generically elongated?
Coronal magnetic null points exist in abundance as demonstrated by
extrapolations of the coronal field, and have been inferred to be important for
a broad range of energetic events. These null points and their associated
separatrix and spine field lines represent discontinuities of the field line
mapping, making them preferential locations for reconnection. This field line
mapping also exhibits strong gradients adjacent to the separatrix (fan) and
spine field lines, that can be analysed using the `squashing factor', . In
this paper we make a detailed analysis of the distribution of in the
presence of magnetic nulls. While is formally infinite on both the spine
and fan of the null, the decay of away from these structures is shown in
general to depend strongly on the null-point structure. For the generic case of
a non-radially-symmetric null, decays most slowly away from the spine/fan
in the direction in which increases most slowly. In particular,
this demonstrates that the extended, elliptical high- halo around the spine
footpoints observed by Masson et al. (Astrophys. J., 700, 559, 2009) is a
generic feature. This extension of the halos around the spine/fan
footpoints is important for diagnosing the regions of the photosphere that are
magnetically connected to any current layer that forms at the null. In light of
this, we discuss how our results can be used to interpret the geometry of
observed flare ribbons in `circular ribbon flares', in which typically a
coronal null is implicated. We conclude that both the physics in the vicinity
of the null and how this is related to the extension of away from the
spine/fan can be used in tandem to understand observational signatures of
reconnection at coronal null points.Comment: Pre-print version of article accepted for publication in Solar
Physic