1,139 research outputs found
Fatherhood in males with cystic fibrosis: modality of conception and impact on clinical status
Common Skin Lesions in Baby Pigs
Greasy pig disease and parakeratosis focused considerable attention on the skin of the pig and pointed out how critical cutaneous disease can be. The importance of other skin lesions in baby pigs is often overlooked and considerable mortality may occur before their significance is recognized
Rapid desensitization for non-immediate reactions in patients with cystic fibrosis
AbstractNon-immediate hypersensitivity reactions to antibiotics in patients with CF represent a real-life challenge for clinicians. Desensitization is often performed in patients who have exhausted all therapeutic options. Whilst desensitization is an established procedure for immediate reactions we assessed the outcomes and safety of desensitization for non-immediate reactions.We retrospectively reviewed 275 desensitization procedures in 42 patients with a range of non-immediate reactions to six commonly used antibiotics. Desensitization was performed using a 7-step rapid intravenous protocol on a normal medical ward.250 (91%) of overall desensitization procedures were successful; however, this figure incorporates certain individuals having multiple successful procedures. Individual patient success ranged from 55% with tazocin through to 88% with tobramycin. In the 25 patients who failed desensitization the reactions were mild and the majority occurred within 48h of starting treatment. Prophylactic anti-histamines and steroids did not reduce the risk of reaction.Whilst the mechanisms remain uncertain we can confirm that rapid desensitization is a safe and effective way of re-introducing an antibiotic to a patient with a non-immediate reaction
Impact of aggressive nutritional intervention on early and late acquisition of CFRD in an adult population?
270 New nebulisers and colistin-resistant Pseudomonas aeuginosa (crPA) in patients with cystic fibrosis (CF)
Geometry of River Networks II: Distributions of Component Size and Number
The structure of a river network may be seen as a discrete set of nested
sub-networks built out of individual stream segments. These network components
are assigned an integral stream order via a hierarchical and discrete ordering
method. Exponential relationships, known as Horton's laws, between stream order
and ensemble-averaged quantities pertaining to network components are observed.
We extend these observations to incorporate fluctuations and all higher moments
by developing functional relationships between distributions. The relationships
determined are drawn from a combination of theoretical analysis, analysis of
real river networks including the Mississippi, Amazon and Nile, and numerical
simulations on a model of directed, random networks. Underlying distributions
of stream segment lengths are identified as exponential. Combinations of these
distributions form single-humped distributions with exponential tails, the sums
of which are in turn shown to give power law distributions of stream lengths.
Distributions of basin area and stream segment frequency are also addressed.
The calculations identify a single length-scale as a measure of size
fluctuations in network components. This article is the second in a series of
three addressing the geometry of river networks.Comment: 16 pages, 13 figures, 4 tables, Revtex4, submitted to PR
Excessive ENaC-mediated sodium influx drives NLRP3 inflammasome-dependent autoinflammation in cystic fibrosis
Cystic Fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and results in defective CFTR-mediated chloride transport, dysregulation of epithelial sodium channels (ENaC) and exaggerated innate immune responses. We tested the hypothesis that upregulation of ENaC drives autoinflammation in this complex monogenic disease.We show that monocytes from patients with CF exhibit a systemic proinflammatory cytokine signature, with associated anti-inflammatory M2-type macrophage deficiency. Cells harboring CF mutations are hyperresponsive to NLRP3 stimulation, as evidenced by increased IL-18, IL-1β, ASC-specks levels in serum and caspase-1 activity in monocytes, and by increased IL-18 production and caspase-1 activity in human bronchial epithelial cells (HBECs). In both cell types there is an associated shift to glycolytic metabolism with succinate release, in response to increased energy requirements. Inhibition of amiloride-sensitive sodium channels partially reverses the NLRP3-dependent inflammation and metabolic shift in these cells. Overexpression of β-ENaC, in the absence of CFTR dysfunction, increases NLRP3-dependent inflammation, indicating a CFTR-independent ENaC axis in CF pathophysiology. Sodium channel modulation provides an important therapeutic strategy to combat lung inflammation in CF.</jats:p
Healthcare choice: Discourses, perceptions, experiences and practices
Policy discourse shaped by neoliberal ideology, with its emphasis on marketisation and competition, has highlighted the importance of choice in the context of healthcare and health systems globally. Yet, evidence about how so-called consumers perceive and experience healthcare choice is in short supply and limited to specific healthcare systems, primarily in the Global North. This special issue aims to explore how choice is perceived and utilised in the context of different systems of healthcare throughout the world, where choice, at least in policy and organisational terms, has been embedded for some time. The articles are divided into those emphasising: embodiment and the meaning of choice; social processes associated with choice; the uncertainties, risks and trust involved in making choices; and issues of access and inequality associated with enacting choice. These sociological studies reveal complexities not always captured in policy discourse and suggest that the commodification of healthcare is particularly problematic
- …