Unknown

Dataset Information

0

High prevalence of symptomatic spinal stenosis in Norwegian adults with achondroplasia: a population-based study.


ABSTRACT:

Background

Symptomatic spinal stenosis (SSS) is a well-known medical complication in achondroplasia. The reported prevalence of SSS is 10 to 30%, an estimate based on small studies or selected populations. No population-based studies exist currently. Furthermore, the relationship between SSS and physical functioning has not been investigated in detail. The aims of this study were to describe the prevalence of SSS in Norwegian adults with achondroplasia, and to explore the impact of SSS on physical functioning.

Methods

This was a population-based study on Norwegian community-dwelling adults with genetically confirmed achondroplasia. Prevalence of SSS was defined by clinical symptoms, and confirmed by imaging or surgical reports. Physical functioning was assessed by walking capacity (6-min walk test), hand strength (Grippit), and activities of daily living (the Health Assessment Questionnaire, HAQ). Pain was assessed by pain site locations and intensity (Numeric Rating Scale, NRS).

Results

In total, 50 participants were included (27 males, 23 females). Median age was 41?years (range 16 to 87?years), 34 (68%) had SSS. The estimated median age at first symptom onset was 33?years (95% confidence interval (CI) 29 to 43?years), range 10 to 67?years. The majority had multiple spinal levels affected. The walking distance was 110?m shorter in the SSS group (95% CI -?172 to -?40?m) as compared with the non-SSS group (p?ConclusionsSSS was highly prevalent in Norwegian adults with achondroplasia, with symptom onset at young age, and multiple spinal levels affected. The presence of SSS was associated with reduced walking distance, activity limitations, and more pain. The findings underline the importance of thorough assessment and monitoring of SSS in achondroplasia, including a formal assessment of physical functioning.

SUBMITTER: Fredwall SO 

PROVIDER: S-EPMC7249360 | biostudies-literature | 2020 May

REPOSITORIES: biostudies-literature

altmetric image

Publications

High prevalence of symptomatic spinal stenosis in Norwegian adults with achondroplasia: a population-based study.

Fredwall Svein O SO   Steen Unni U   de Vries Olga O   Rustad Cecilie F CF   Eggesbø Heidi Beate HB   Weedon-Fekjær Harald H   Lidal Ingeborg B IB   Savarirayan Ravi R   Månum Grethe G  

Orphanet journal of rare diseases 20200525 1


<h4>Background</h4>Symptomatic spinal stenosis (SSS) is a well-known medical complication in achondroplasia. The reported prevalence of SSS is 10 to 30%, an estimate based on small studies or selected populations. No population-based studies exist currently. Furthermore, the relationship between SSS and physical functioning has not been investigated in detail. The aims of this study were to describe the prevalence of SSS in Norwegian adults with achondroplasia, and to explore the impact of SSS o  ...[more]

Similar Datasets

| S-EPMC8570016 | biostudies-literature
| S-EPMC7116132 | biostudies-literature
| S-EPMC10624983 | biostudies-literature
| S-EPMC4671898 | biostudies-other
| S-EPMC10102780 | biostudies-literature
| S-EPMC9231541 | biostudies-literature
| S-EPMC6102665 | biostudies-other
| S-EPMC9715794 | biostudies-literature
| S-EPMC7960346 | biostudies-literature
| S-EPMC8820012 | biostudies-literature