Development of an International SMA Bulbar Assessment for Inter-professional Administration

BACKGROUND: Progressive weakness can affect bulbar muscles in individuals with moderate to severe forms of spinal muscular atrophy (SMA). The paucity of standardized, valid bulbar assessments capturing clinically significant deficits in SMA impedes the ability to monitor function, facilitate intervention, or detect treatment response. OBJECTIVE: To fill this void, an international multidisciplinary team gathered to develop an agreed upon consensus-derived assessment of bulbar function in SMA for inter-professional administration to enhance our ability to monitor disease progression, support clinical management, and evaluate treatment effects. METHODS: Fifty-six international clinicians experienced in SMA were invited and engaged using the Delphi method over multiple rounds of web-based surveys to establish consensus. RESULTS: Serial virtual meetings occurred with 42 clinicians (21 speech and language therapists, 11 physical therapists, 5 neurologists, 4 occupational therapists, and 1 dentist). Seventy-two validated assessments of bulbar function were identified for potential relevance to individuals with SMA (32 accessible objective, 11 inaccessible objective, 29 patient-reported outcomes). Delphi survey rounds (n = 11, 15, 15) achieved consensus on individual items with relevance and wording discussed. Key aspects of bulbar function identified included: oral intake status, oral facial structure and motor strength, swallowing physiology, voice & speech, and fatigability. CONCLUSIONS: Multidisciplinary clinicians with expertise in bulbar function and SMA used Delphi methodology to reach consensus on assessments/items considered relevant for SMA across all age groups. Future steps include piloting the new scale moving towards validation/reliability. This work supports the advancement of assessing bulbar function in children and adults with SMA by a variety of professionals

Research priorities to improve the health of children and adults with dysphagia: a National Institute of Health Research and Royal College of Speech and Language Therapists research priority setting partnership

Objective To conduct the first UK-wide research priority setting project informing researchers and funders of critical knowledge gaps requiring investigation to improve the health and well-being of patients with eating, drinking and swallowing disorders (dysphagia) and their carers. Design A priority setting partnership between the National Institute of Health Research (NIHR) and the Royal College of Speech and Language Therapists using a modified nominal group technique. A steering group and NIHR representatives oversaw four project phases: (1) survey gathering research suggestions, (2) verification and aggregation of suggestions with systematic review research recommendations, (3) multistakeholder workshop to develop research questions, (4) interim priority setting via an online ranking survey and (5) final priority setting. Setting UK health services and community. Participants Patients with dysphagia, carers and professionals who work with children and adults with dysphagia from the UK. Results One hundred and fifty-six speech and language therapists submitted 332 research suggestions related to dysphagia. These were mapped to 88 research recommendations from systematic reviews to form 24 ‘uncertainty topics’ (knowledge gaps that are answerable by research). Four patients, 1 carer and 30 healthcare professionals collaboratively produced 77 research questions in relation to these topics. Thereafter, 387 patients, carers and professionals with experience of dysphagia prioritised 10 research questions using an interim prioritisation survey. Votes and feedback for each question were collated and reviewed by the steering and dysphagia reference groups. Nine further questions were added to the long-list and top 10 lists of priority questions were agreed. Conclusion Three top 10 lists of topics grouped as adults, neonates and children, and all ages, and a further long list of questions were identified by patients, carers and healthcare professionals as research priorities to improve the lives of those with dysphagia

Columbite-tantalite mineral chemistry from rare-element granitic pegmatites: Separation Lakeh area, N.W. Ontario, Canada

The Separation Lake area is host to the most important rare-element pegmatites in Ontario, Canada. They include the Big Whopper and Big Mack petalite pegmatite systems which potentially represent the world's second largest lithium deposit of this type. The pegmatites occur in two distinct clusters adjacent to the Separation Rapids pluton which is thought to be the source of the rare-elements. Beryl-type and complex-, petalite-subtype pegmatites are the most common and a few pegmatites have characteristics similar to the lepidolite-subtype. This study reveals that columbite-tantalite in the pegmatites has an extremely wide range of composition from primitive ferrocolumbite to evolved, almost end-member manganotantalite. Evidence is provided that melt evolution resulted in increased fluorine activity (as seen in microlite compositions) and that in situ fractionation of magma within individual pegmatites often led to the crystallization of rare-element-enriched, Li mica-fluorapatite-cleavelandite pods. Zonation patterns seen in backscattered electron images show primary compositions of columbite-tantalite were modified by secondary processes related to extreme fractionation and involving the late stage development of albitic units in individual pegmatites. This alteration led to recrystallization of columbite-tantalite and produced compositions with lower Ta contents, but with little change in Mn content

Rare-element mineralization of the Separation Lake area, northwest Ontario: characteristics of a new discovery of complex-type, petalite-subtype, Li-Rb-Cs-Ta pegmatite

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Project Unit 04-039. Rare-element granitic pegmatites of the Fort Hope Field, North-Central Ontario

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New discovery of rare-element pegmatite mineralization, Separation Lake area, northwestern Ontario

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Rare-metal exploration potential of the Separation Lake area: an emerging target for Bikita-type mineralization in the Superior Province of Ontario

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Project Unit 93-11. Granite-related mineralization in Northwestern Ontario: IV. New Rare-Element Pegmatite Discoveries in the Separation Lake Area

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Project Unit 93-11. Granite-related mineralization in Northwestern Ontario: II. Detailed examination of the Separation Rapids (English River) rare-element pegmatite group

Abstract not available

Homobasidiomycete transformation : agrobacterium methodologies and marker gene development in Agaricus and Coprinus

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