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Somatic symptoms, pain, catastrophizing and the association with disability among children with heritable connective
Lisanne E de Koning1,2, Jessica Warnink-Kavelaars2,3, Marion A van Rossum4,5
1Centre of Expertise Urban Vitality, Faculty of Health, Amsterdam University of Applied Sciences, Amsterdam, The Netherlands.
Insights
Children with Heritable Connective Tissue Disorders (HCTD) experience significant somatic symptoms and pain, which are linked to disability. Comprehensive assessment is crucial for effective, tailored treatment plans for these conditions.
Area of Science:
- Pediatric Rheumatology
- Genetics
- Pain Medicine
Background:
- Heritable Connective Tissue Disorders (HCTD) encompass a group of genetic conditions affecting connective tissues.
- These disorders, including Marfan syndrome and Ehlers-Danlos syndromes, can lead to diverse clinical manifestations.
- Understanding the impact of somatic symptoms and pain in affected children is essential for holistic care.
Purpose of the Study:
- To investigate the prevalence and nature of nonspecific somatic symptoms, pain, and pain catastrophizing in children with HCTD.
- To determine the association between these symptoms and functional disability in this pediatric population.
- To compare symptom reporting in HCTD patients with normative data.
Main Methods:
- An observational, multicenter study involving 127 children (aged 4-18 years) diagnosed with MFS, LDS, EDS, or hEDS.
- Utilized validated questionnaires including the Children's Somatization Inventory (CSI), Pain Visual-Analogue Scale (VAS), and Childhood Health Assessment Questionnaire (CHAQ-30).
- Assessed pain catastrophizing using the Pain Catastrophizing Scale (PCS) and pain distribution via a body diagram.
Main Results:
- Children with HCTD aged ≥8 years reported significantly higher somatization, pain intensity, and disability compared to normative data.
- Pain was prevalent in 59% of older children, affecting a median of 4 pain areas.
- Somatic symptom intensity and pain explained 45% of the variance in disability in older children; functional disability was moderately to highly correlated with pain and somatization in younger children.
Conclusions:
- Nonspecific somatic symptoms and pain are significant issues for children with HCTD, contributing substantially to disability.
- The findings highlight the need for thorough assessment of these symptoms in pediatric HCTD cases.
- Tailored treatment strategies addressing somatic symptoms, pain, and disability are recommended for improved outcomes in children with HCTD.
Abstract:
The aim of the present study was to investigate the nature and prevalence of nonspecific somatic symptoms, pain and catastrophizing in children with Heritable Connective Tissue Disorders (HCTD), and to determine their association with disability. This observational, multicenter study included 127 children, aged 4-18 years, with Marfan syndrome (MFS) (59%), Loeys-Dietz syndrome (LDS) (8%), Ehlers-Danlos syndromes (EDS) (12%) and hypermobile Ehlers-Danlos syndrome (hEDS) (23%). The assessments included the Children's Somatization Inventory or parent proxy (CSI, PCSI), pain visual-analogue scale (VAS), SUPERKIDZ body diagram, Pain Catastrophizing Scale Child or parent proxy (PCS-C, PCS-P) and Childhood Health Assessment Questionnaire (CHAQ-30). Data from children aged ≥8 years were compared to normative data. In children ≥ 8 years (n = 90), pain was present in 59%, with a median of 4 (IQR = 3-9) pain areas. Compared to normative data, the HCTD group reported significantly higher on the CSI (p ≤ 0.001, d = 0.85), VAS pain intensity (p ≤ 0.001, d = 1.22) and CHAQ-30 (p ≤ 0.001, d = 1.16) and lower on the PCS-C (p = 0.017, d = -0.82) and PCS-P (p ≤ 0.001, d = -0.49). The intensity of nonspecific somatic symptoms and pain explained 45% of the variance in disability (r2 = 0.45 F(2,48) = 19.70, p ≤ 0.001). In children ≤ 7 years (n = 37), pain was present in 35% with a median of 5(IQR = 1-13) pain areas. The mean(SD) VAS scores for pain intensity was 1.5(2.9). Functional disability was moderately correlated to the number of pain areas (r = 0.56, p ≤ 0.001), intensity of nonspecific somatic symptoms (r = 0.63, p ≤ 0.001) and pain (r = 0.83, p ≤ 0.001). In conclusion, this study supports the need for comprehensive assessment of nonspecific somatic symptoms, pain, and disability in children with HCTD to allow tailored treatment.
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