scholarly journals Modeling Manifest Huntington’s Disease Prevalence Using Diagnosed Incidence and Survival Time

2021 ◽  
pp. 1-8
Author(s):  
Valerie Crowell ◽  
Richard Houghton ◽  
Akanksha Tomar ◽  
Tricia Fernandes ◽  
Ferdinando Squitieri
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Burden ◽  
Epidemiological Data ◽  
Incidence Rates ◽  
Simulated Patients ◽  
Published Data ◽  
The Usa ◽  
Probability Of Death ◽  
The Uk

<b><i>Introduction:</i></b> Understanding the epidemiology of Huntington’s disease (HD) is key to assessing disease burden and the healthcare resources required to meet patients’ needs. We aimed to develop and validate a model to estimate the diagnosed prevalence of manifest HD by the Shoulson-Fahn stage. <b><i>Methods:</i></b> A literature review identified epidemiological data from Brazil, Canada, France, Germany, Italy, Spain, the UK, and the USA. Data on staging distribution at diagnosis, progression, and mortality were derived from Enroll-HD. Newly diagnosed patients with manifest HD were simulated by applying annual diagnosed incidence rates to the total population in each country, each year from 1950 onwards. The number of diagnosed prevalent patients from the previous year who remained in each stage was estimated in line with the probability of death or progression. Diagnosed prevalence in 2020 was estimated as the sum of simulated patients, from all the incident cohorts, still alive. <b><i>Results:</i></b> The model estimates that in 2020, there were 66,787 individuals diagnosed with HD in the 8 included countries, of whom 62–63% were in Shoulson-Fahn stages 1 and 2 (with less severely limited functional capacity than those in stages 3–5). Diagnosed prevalence is estimated to be 8.2–9.0 per 100,000 in the USA, Canada, and the 5 included European countries and 3.5 per 100,000 in Brazil. <b><i>Conclusion:</i></b> The modeled estimates generally accord with the previously published data. This analysis contributes to better understanding of the epidemiology of HD and highlights areas of uncertainty.

scholarly journals Country-Specific Prevalence and Incidence of Youth-Onset Type 2 Diabetes: A Narrative Literature Review

2020 ◽  
Vol 76 (5) ◽  
pp. 289-296 ◽  
Author(s):  
Jane L. Lynch ◽  
Margarita Barrientos-Pérez ◽  
Mona Hafez ◽  
Muhammad Yazid Jalaludin ◽  
Margarita Kovarenko ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Diagnostic Criteria ◽  
Incidence Rates ◽  
National Guidelines ◽  
Incidence Data ◽  
Epidemiology Data ◽  
The Usa ◽  
The Uk ◽  
Country Specific

<b><i>Background:</i></b> With increased awareness of type 2 diabetes (T2D) in children and adolescents, an overview of country-specific differences in epidemiology data is needed to develop a global picture of the disease development. <b><i>Summary:</i></b> This study examined country-specific prevalence and incidence data of youth-onset T2D published between 2008 and 2019, and searched for national guidelines to expand the understanding of country-specific similarities and differences. Of the 1,190 articles and 17 congress abstracts identified, 58 were included in this review. Our search found the highest reported prevalence rates of youth-onset T2D in China (520 cases/100,000 people) and the USA (212 cases/100,000) and lowest in Denmark (0.6 cases/100,000) and Ireland (1.2 cases/100,000). However, the highest incidence rates were reported in Taiwan (63 cases/100,000) and the UK (33.2 cases/100,000), with the lowest in Fiji (0.43 cases/100,000) and Austria (0.6 cases/100,000). These differences in epidemiology data may be partly explained by variations in the diagnostic criteria used within studies, screening recommendations within national guidelines and race/ethnicity within countries. <b><i>Key Messages:</i></b> Our study suggests that published country-specific epidemiology data for youth-onset T2D are varied and scant, and often with reporting inconsistencies. Finding optimal diagnostic criteria and screening strategies for this disease should be of high interest to every country. <b><i>Trial Registration:</i></b> Not applicable.

scholarly journals Predictive testing of minors for Huntington's disease: The UK and Netherlands experiences

2017 ◽  
Vol 177 (1) ◽  
pp. 35-39 ◽  
Author(s):  
Oliver W. Quarrell ◽  
Angus J. Clarke ◽  
Cecilia Compton ◽  
Christine E.M. de Die-Smulders ◽  
Alan Fryer ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Predictive Testing ◽  
The Uk

scholarly journals 22 Years of predictive testing for Huntington’s disease: the experience of the UK Huntington’s Prediction Consortium

2016 ◽  
Vol 24 (10) ◽  
pp. 1396-1402 ◽  
Author(s):  
Sheharyar S Baig ◽  
◽  
Mark Strong ◽  
Elisabeth Rosser ◽  
Nicola V Taverner ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Predictive Testing ◽  
The Uk

J1 Predictive testing for huntington’s disease (HD) in the UK 1994 – 2014: changes in age distribution and outcome of results

2016 ◽  
Vol 87 (Suppl 1) ◽  
pp. A75.3-A75
Author(s):  
Oliver Quarrell ◽  
Sheharyar Baig ◽  
Mark Strong ◽  
Elisabeth Rosser ◽  
Nicola Taverner ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Age Distribution ◽  
Predictive Testing ◽  
The Uk

scholarly journals Incidence of adult Huntington's disease in the UK: a UK-based primary care study and a systematic review

BMJ Open ◽  
2016 ◽  
Vol 6 (2) ◽  
pp. e009070 ◽  
Author(s):  
Nancy S Wexler ◽  
Laura Collett ◽  
Alice R Wexler ◽  
Michael D Rawlins ◽  
Sarah J Tabrizi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Primary Care ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
The Uk ◽  
Care Study

Towards a Protocol for the Preparation and Delivery of Striatal Tissue for Clinical Trials of Transplantation in Huntington's Disease

2000 ◽  
Vol 9 (2) ◽  
pp. 223-234 ◽  
Author(s):  
Colin Watts ◽  
Stephen B. Dunnett
Keyword(s):  
Clinical Trials ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Scientific Evidence ◽  
Tissue Preparation ◽  
Phase I Clinical Trials ◽  
Clinical Transplantation ◽  
The Uk ◽  
Near Future

There is a growing body of scientific evidence contributing to the development of clinical transplantation programs in patients with Huntington's disease. Phase I clinical trials have already commenced in France and North America and are starting in the near future in Sweden and the UK. Protocols for patient selection, surgical implantation, and pre- and postoperative follow-up are well defined. However, considerable variability exists with respect to the harvesting, preparation, and timing of implantation of the donor material. In this article we review the scientific evidence on which a rational protocol for donor tissue preparation and delivery may be based. Strategies aimed at minimizing the variability of tissue preparation should reduce the variability of functional outcome of striatal transplantation observed in animal models of Huntington's disease.

scholarly journals Huntington's disease research and practice: reflections on the journey made and lessons learned

2011 ◽  
Vol 23 (6) ◽  
pp. 851-857 ◽  
Author(s):  
Anita M.Y. Goh ◽  
Edmond Chiu
Keyword(s):  
Neurodegenerative Disease ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuropsychiatric Symptoms ◽  
Current Treatment ◽  
Lessons Learned ◽  
Monogenic Disorder ◽  
European Origin ◽  
Common Causes ◽  
The Usa

Knowledge about some of the rarer causes of dementia is now quite advanced (Lautenschlager and Martins, 2005), which can in turn inform other more common causes of dementia. Such is the case with the monogenic disorder of Huntington's disease (HD) when compared to, say, Alzheimer's disease (AD). HD is an autosomal dominant hereditary neurodegenerative disease, which involves the basal ganglia, its connections to the frontal lobe and related neural circuits. The onset of HD is typically in mid-life (but onset can range from childhood to old age), with motor, cognitive and neuropsychiatric symptoms. There is currently no cure for this devastating and inevitably fatal neurodegenerative disease, with current treatment approaches being solely symptomatic. The highest frequencies of HD are found in Europe and in those countries whose populations are of predominately European origin such as the USA and Australia (approximately 1 case per 10,000 people).

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