Wilson's Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Wilson's Disease, including details on treatment, causes, symptoms, medication.

Interferon Response Factors 3 and 7 Protect against Chikungunya Virus Hemorrhagic Fever and Shock.

Rudd PA, Wilson J, Gardner J, Larcher T, Babarit C, Le TT, Anraku I, Kumagai Y, Loo YM, Gale M, Akira S, Khromykh AA, Suhrbier A

Queensland Institute of Medical Research, Brisbane, Qld. 4029, Australia.

Chikungunya virus (CHIKV) infections can produce severe disease and mortality. Here we show that CHIKV infection of adult mice deficient in interferon response factors 3 and 7 (IRF3/7(-/-)) was lethal. Mortality was associated with undetectable serum IFNα/β, ≈50 and ≈10 fold increases in IFNγ and TNF, respectively, increased virus replication, edema, vasculitis, hemorrhage, fever followed by hypothermia, oliguria, thrombocytopenia, and raised hematocrits. These features are consistent with hemorrhagic shock and were also evident in infected IFNα/β-receptor deficient mice. In situ hybridization suggested CHIKV infection of endothelium, fibroblasts, skeletal muscle, mononuclear cells, chondrocytes and keratinocytes in IRF3/7(-/-) mice; all but the latter two stained positive in wild-type mice. Vaccination protected IRF3/7(-/-) mice, suggesting defective antibody responses were not responsible for mortality. IPS-1- and TRIF-dependent pathways were primarily responsible for IFNα/β induction, with IRF7 up-regulated >100 fold in infected wild-type mice. These studies suggest that inadequate IFNα/β responses following virus infection can be sufficient to induce hemorrhagic fever and shock, a finding with implications for understanding severe CHIKV disease, and dengue hemorrhagic fever/dengue shock syndrome.

Published 4 July 2012 in J Virol.
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Articles on Wilson's Disease published 4 July 2012:

A descriptive analysis of notifiable gastrointestinal illness in the Northwest Territories, Canada, 1991-2008.   BMJ Open, 2(4).

[Abstract] [Full-text]

Lycopene inhibits lymphocyte proliferation through mechanisms dependent on early cell activation.   Mol Nutr Food Res, 56(7): 1034-42.

[Abstract] [Full-text]

The laparoscopic approach is the default for colorectal resections.   Colorectal Dis.

We read with interest the article 'The majority of colorectal resections require an open approach' [1]. This has not been our experience. After a conscious decision was made to adopt a laparoscopic or laparoscopic-assisted approach as the default for primary conventional colorectal resection, as our experience grew, we found 100% (127/127) of cases were suitable for a laparoscopic approach with a 4% conversion rate. © 2012 The Authors Colorectal Disease © 2012 The Association of ... [Abstract] [Full-text]

Abstracts.   Pharmacoepidemiol Drug Saf, 21(7): 788-798.

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Articles on Wilson's Disease published 3 July 2012:

Daytime alertness in Parkinson's disease: Potentially dose-dependent, divergent effects by drug class.   Mov Disord.

Many patients with idiopathic Parkinson's disease experience difficulties maintaining daytime alertness. Controversy exists regarding whether this reflects effects of antiparkinsonian medications, the disease itself, or other factors such as nocturnal sleep disturbances. In this study we examined the phenomenon by evaluating medicated and unmedicated Parkinson's patients with objective polysomnographic measurements of nocturnal sleep and daytime alertness. Patients (n = 63) underwent a 48-hour ... [Abstract] [Full-text]

Cardiac Transplantation in Friedreich Ataxia.   J Child Neurol.

In this article, we describe a 14-year-old boy with a confirmed diagnosis of Friedreich ataxia who underwent cardiac transplantation for left ventricular failure secondary to dilated cardiomyopathy with restrictive physiology. His neurological status prior to transplantation reflected early signs of neurological disease, with evidence of dysarthria, weakness, mild gait impairment, and limb ataxia. We review the ethical issues considered during the process leading to the decision to offer ... [Abstract] [Full-text]

Tau protein kinases: Involvement in Alzheimer's disease.   Ageing Res Rev.

Tau phosphorylation is regulated by a balance between tau kinase and phosphatase activities. Disruption of this equilibrium was suggested to be at the origin of abnormal tau phosphorylation and thereby might contribute to tau aggregation. Thus, understanding the regulation modes of tau phosphorylation is of high interest in determining the possible causes at the origin of the formation of tau aggregates in order to elaborate protection strategies to cope with these lesions in AD. Among the ... [Abstract] [Full-text]

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A deeper understanding of the composition and organization of extracellular matrix molecules in native, healthy meniscus tissue is required to fully appreciate the degeneration that occurs in joint disease and the intricate environment in which an engineered meniscal graft would need to function. In this study, regional variations in the tissue-level and pericellular distributions of collagen types I, II and VI and the proteoglycans aggrecan, biglycan and decorin were examined in the juvenile ... [Abstract] [Full-text]

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