Heritable Connective Tissue Disorders (HCTD) show an overlap in the physical features that can evolve in childhood. It is unclear to what extent children with HCTD experience burden of disease. This study aims to quantify fatigue, pain, disability and general health with standardized validated questionnaires.METHODS: This observational, multicenter study included 107 children, aged 4-18 years, with Marfan syndrome (MFS), 58%; Loeys-Dietz syndrome (LDS), 7%; Ehlers-Danlos syndromes (EDS), 8%; and hypermobile Ehlers-Danlos syndrome (hEDS), 27%. The assessments included PROMIS Fatigue Parent-Proxy and Pediatric self-report, pain and general health Visual-Analogue-Scales (VAS) and a Childhood Health Assessment Questionnaire (CHAQ).RESULTS: Compared to normative data, the total HCTD-group showed significantly higher parent-rated fatigue T-scores (M = 53 (SD = 12), p = 0.004, d = 0.3), pain VAS scores (M = 2.8 (SD = 3.1), p < 0.001, d = 1.27), general health VAS scores (M = 2.5 (SD = 1.8), p < 0.001, d = 2.04) and CHAQ disability index scores (M = 0.9 (SD = 0.7), p < 0.001, d = 1.23). HCTD-subgroups showed similar results. The most adverse sequels were reported in children with hEDS, whereas the least were reported in those with MFS. Disability showed significant relationships with fatigue (p < 0.001, rs = 0.68), pain (p < 0.001, rs = 0.64) and general health (p < 0.001, rs = 0.59).CONCLUSIONS: Compared to normative data, children and adolescents with HCTD reported increased fatigue, pain, disability and decreased general health, with most differences translating into very large-sized effects. This new knowledge calls for systematic monitoring with standardized validated questionnaires, physical assessments and tailored interventions in clinical care.
Background: Experienced assessors show good intra-rater reproducibility (within-session and between-session agreement and reliability) when using an algometer to determine pressure pain thresholds (PPT). However, it is unknown whether novice assessors perform equally well. This study aimed to determine within and between-session agreement and reliability of PPT measurements performed by novice assessors and explored whether these parameters differed per assessor and algometer type.Methods: Ten novice assessors measured PPTs over four test locations (tibialis anterior muscle, rectus femoris muscle, extensor carpi radialis brevis muscle and paraspinal muscles C5-C6) in 178 healthy participants, using either a Somedic Type II digital algometer (10 raters; 88 participants) or a Wagner Force Ten FDX 25 digital algometer (nine raters; 90 participants). Prior to the experiment, the novice assessors practiced PPTs for 3 h per algometer. Each assessor measured a different subsample of ~9 participants. For both the individual assessor and for all assessors combined (i.e., the group representing novice assessors), the standard error of measurement (SEM) and coefficient of variation (CV) were calculated to reflect within and between-session agreement. Reliability was assessed using intraclass correlation coefficients (ICC1,1).Results: Within-session agreement expressed as SEM ranged from 42 to 74 kPa, depending on the test location and device. Between-session agreement, expressed as SEM, ranged from 36 to 76 kPa and the CV ranged from 9-16% per body location. Individual assessors differed from the mean group results, ranging from -55 to +32 kPa or from -9.5 to +6.6 percentage points. Reliability was good to excellent (ICC1,1: 0.87 to 0.95). Results were similar for both types of algometers.Conclusions: Following 3 h of algometer practice, there were slight differences between assessors, but reproducibility in determining PPTs was overall good.
Background: Physical therapy is regarded an effective treatment for temporomandibular disorders (TMD). Patients with TMD often report concomitant headache. There is, however, no overview of the effect of physical therapy for TMD on concomitant headache complaints. Objectives: The aim of this study is to systematically evaluate the literature on the effectiveness of physical therapy on concomitant headache pain intensity in patients with TMD. Data sources: PubMed, Cochrane and PEDro were searched. Study eligibility criteria: Randomized or controlled clinical trials studying physical therapy interventions were included. Participants: Patients with TMD and headache. Appraisal: The Cochrane risk of bias tool was used to assess risk of bias. Synthesis methods: Individual and pooled between-group effect sizes were calculated according to the standardized mean difference (SMD) and the quality of the evidence was rated using the GRADE approach. Results: and manual therapy on both orofacial region and cervical spine. There is a very low level of certainty that TMD-treatment is effective on headache pain intensity, downgraded by high risk of bias, inconsistency and imprecision. Limitations: The methodological quality of most included articles was poor, and the interventions included were very different. Conclusions: Physical therapy interventions presented small effect on reducing headache pain intensity on subjects with TMD, with low level of certainty. More studies of higher methodological quality are needed so better conclusions could be taken.
