Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan

Aim The present study aimed to map and summarise the research on children, aged 0‐18 years, with Marfan syndrome, identify research gaps and point to research agendas. Methods A scoping review was systematically performed by searching multiple databases from January 1996 to April 2019. Primary studies presenting results on at least six individuals aged 0‐18 years with Marfan syndrome, diagnosed according to the Ghent nosology, were selected. Results From 2341 de‐duplicated records, 92 papers were included, mapped and described. Their topics were diagnostics (12%), cardiovascular matters (50%), skeletal matters (22%), ocular matters (9%), other medical aspects (5%) and psychosocial perspectives (2%). Most studies were from Europe and North America and published between 1999 and 2019 in subject‐specific or paediatric journals, while a few were published in genetics journals. All studies had quantitative designs, and very few were multicentre studies. Each study had six to 608 subjects for a total of approximately 5809. Conclusion A wide range of research topics on adolescent and paediatric Marfan syndrome was found, but qualitative studies and a focus on psychosocial matters were lacking. Future investigations addressing noncardiovascular consequences and patient experiences are needed, as well as studies reaffirming or replicating existing intervention study results.

show abstract

Halogravity traction in the preoperative treatment of scoliosis in twins with Marfan syndrome

Čimić

Crnogaća

Vrdoljak

et al. 2015

BMJ Case Reports

View full text Add to dashboard Cite

We report on the influence of the duration of halogravity traction for achieving curve correction in monozygotic twins with Marfan syndrome who underwent posterior spinal fusion. Review of the medical charts and standard radiograph analysis of twin girls treated at our department was performed. Halogravity traction with a four-pin skull construct was applied for 3 weeks in twin A and for 2 weeks in twin B with a maximum of 20% body weight used. Both were on a 24-hours-day halogravity traction regime. Achieved thoracic curve correction after halogravity traction was 31% in twin A and 18% in twin B. Although less curve correction after traction was achieved in twin B, this had no significant implications on final postoperative curve correction. Halogravity traction can be a useful tool in the preoperative treatment of scoliosis in patients with Marfan syndrome if applied for 3 weeks. In order to avoid complications, we propose that lower weights be used with a starting weight of 1.5 kg increased by 1 kg daily until 20% body weight is reached.

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

hi@scite.ai

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan

Cited by 6 publications

References 25 publications

Arm span as a predictor of the six-minute walk test in healthy children

Arm span as a predictor of the six-minute walk test in healthy children

A scoping review presenting a wide variety of research on paediatric and adolescent patients with Marfan syndrome

Halogravity traction in the preoperative treatment of scoliosis in twins with Marfan syndrome

Contact Info

Product

Resources

About