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• Abetalipoproteinemia (ABL) is a rare monogenic familial hypobetalipoproteinemia caused by bi-allelic variants in MTTP gene. • We identified four French-Canadian patients homozygous for the same MTTP variant. • The ABL carrier frequency in Saguenay-Lac-Saint-Jean is estimated at 1:203. • This represents the 2nd highest worldwide carrier estimates for ABL. • Screening for ABL should be considered in this population. Abetalipoproteinemia (ABL) is a rare recessive genetic disease caused by bi-allelic pathogenic variants in the microsomal triglyceride transfer protein (MTTP) gene. This disease is characterized by a deficiency in the secretion of apolipoprotein B-containing lipoproteins. Patients with ABL present with neurological, hematological, and gastrointestinal symptoms due to fat malabsorption and a deficiency in liposoluble vitamins. In this report, we present a total of four ABL cases, including three new cases, all originating from the same French-Canadian founder population in Saguenay-Lac-Saint-Jean, Québec, Canada. These individuals are homozygous for the same pathogenic variant in the MTTP gene (c.419dup, p.Asn140Lysfs*2). We found that this variant is more common than anticipated in this population, with an estimated carrier frequency of 1:203. Early diagnosis is essential to initiate treatment known to prevent complications associated with ABL. Population carrier screening or newborn screening for ABL should be considered in this French-Canadian founder population. [ABSTRACT FROM AUTHOR]

BACKGROUND: Adults with cystic fibrosis (CF) must continuously manage their condition, while working for a living, and want a normal life. Adherence rates to treatments/medications are less than optimal. Existing theory offers little to explain adherence rates. The purpose of this study was to develop a theory to further the understanding of how people with CF manage their condition in an adherence-driven health care system. METHODS: Constructivist Grounded Theory methodology was used to conduct 27 semistructured interviews with adults with CF, family members, and health care providers. Data collection and analysis were simultaneous, using constant comparative methods, initial and focused coding, and category identification and reduction to develop a theory. RESULTS: Doing what works to balance life and CF is the theory generated from this study. The main concern of participants was to be seen as normal. The theory depicts what participants with CF and their family members do about their concerns and involves 4 interrelated processes: working overtime, receiving support, passing as normal, and facing disease progression. CONCLUSION: Participants did not relate to the term nonadherent; rather they described working overtime to manage CF, to work, and to have a normal life. Health care provider and researcher perspectives on adherence differ from those of people with CF. Engaging adults with CF and health care providers in a dialogue in which expectations are shared may lead to individualized treatment regimens that work, because adults with CF will do what works. [ABSTRACT FROM AUTHOR]

Abstract: Changes to ecosystems caused by introduced herbivores can be predictable, stepwise transitions or unpredictable and even irreversible state changes. This study''s objectives were to explore effects on forest succession and soil development 5 years after moose (Alces alces L.) were fenced out of areas within and adjacent to a national park in Newfoundland, Canada. Study plots spanned a range of understorey broadleaf plant associations with regenerating balsam fir (Abies balsamea (L.) Mill.), an important winter forage plant for moose and a dominant canopy tree throughout Newfoundland. After 5 years, height–diameter ratios were significantly larger for larger basal diameters of understorey balsam fir in unfenced, but not in fenced subplots, suggesting that growth of the conifer is compromised within the exclosure. In contrast, for most broadleaf trees and shrubs, moose removal by fencing results in greater heights and basal diameters than in control subplots. The competitive advantage of broadleaf trees and shrubs over balsam fir in the short-term may be a result of past sustained heavy moose browsing benefiting plants that are better at investing resources into below-ground growth or benefiting plants that have broader leaf canopies. It is not clear how long the broadleaf transition state we document will continue. Restorative actions intended to mimic usual patterns of forest regeneration in this region of Newfoundland might best consider moose removal with site preparation and/or planting to historic densities. [Copyright &y& Elsevier]

The article offers information related to the Commonwealth Parliamentary Association in Canada in 2011. The 28th Canadian Presiding Officers' Conference was held in Nunavut from January 20-23. Gordie Gosse was the elected Speaker of the Nova Scotia Legislature on January 18. The 49th Canadian Regional Conference will be held in Prince Edward Island from July 13-19.