Risk factors associated with symptoms of temporomandibular disorders among women with hypermobile Ehlers-Danlos syndrome: Questionnaire-based study in Finland and Sweden.

BACKGROUND: Generalized joint hypermobility as a characteristic feature of Ehlers-Danlos syndromes (EDS) is among the factors contributing to temporomandibular disorders (TMD). OBJECTIVE: To evaluate the prevalence of TMD symptoms and their risk factors among women born in Sweden or Finland who were 27- to 78-year-olds with diagnosed hypermobile EDS (hEDS). METHODS: A cohort of women with confirmed hEDS (n = 185) was constructed from the members of the National EDS Associations in both countries. Based on questionnaire data, frequency of independent variables in terms of socio-demographic, general health and oral health-related factors, comorbid symptoms and psychological distress for self-reported TMD symptoms as the dependent variables, were calculated first. Prevalence ratios (PR) and their 95% confidence interval (95% CI) were estimated for the association between independent and dependent variables. RESULTS: Nearly all participants reported TMD symptoms (98%) with TMD pain (95%), TMJ clicking (90%) and jaw fatigue (80%) as the most common symptoms and TMJ crepitation (63%) and luxation (44%) as the least common symptoms. Risk factors for TMD among 27- to 50-year-olds participants were Finland as a country of birth, living alone and self-reported worst pain in the body (not the joints). The respective risk factors among the 51- to 78-year-olds were Finland as a country of birth, family history of EDS, tinnitus and regularly taking contraceptives. CONCLUSIONS: Among adult women with confirmed hEDS, socio-demographic and health-related factors and comorbid symptoms were significantly associated with TMD but with differences regarding age group. Therefore, management of TMD requires a multidisciplinary approach among the affected.

Generalized joint hypermobility and the risk of pregnancy-related pelvic girdle pain: Is body mass index of importance?-A prospective cohort study.

INTRODUCTION: Pelvic girdle pain (PGP) affects approximately 50% of pregnant women. The mechanisms are multifactorial but not fully understood. Women with generalized joint hypermobility (GJH) may be vulnerable to load in the pelvic joints during pregnancy. Our aim was to investigate if women with GJH had an increased risk of PGP and higher pain intensity during and after pregnancy, compared with women with normal joint mobility. We also studied if body mass index (BMI) in early pregnancy influenced that risk. MATERIAL AND METHODS: A prospective cohort study of 356 women, whose data were collected by self-reports and clinical examinations in early and in late pregnancy and 9 months after childbirth. GJH was present with ≥5/9 points on the Beighton score. PGP was defined by a pain drawing and ≥1 positive test. Pain intensity was measured with a visual analogue scale (0-100 mm). We adjusted for age and origin in logistic regression and ordinal logistic regression analysis. RESULTS: In early pregnancy, 47.1% of the women with GJH had PGP vs 32.6% of women with normal joint mobility (adjusted odds ratio [aOR] 1.76; 95% confidence interval [CI] 0.86-3.62) and had higher odds of reporting higher pain intensity (aOR 2.04; 95% CI 1.02-4.07). The odds of PGP were highest for women with GJH and BMI ≥25 kg/m2 (aOR 6.88; 95% CI 1.34-35.27) compared with women with normal joint mobility and BMI <25 kg/m2 . The estimated associations were weaker and not statistically significant in late pregnancy or after childbirth. CONCLUSIONS: Women with GJH did not have an increased risk of PGP during or after pregnancy but reported higher pain intensity in early pregnancy compared with women with normal joint mobility. Since women with combined GJH and BMI ≥25 kg/m2 had the highest odds of PGP in early pregnancy, our results may suggest that health care needs to pay attention to and develop methods to reduce the risk of PGP and delay the onset of pain during pregnancy in women with this combination.

The relationship between kinaesthesia, motor performance, physical fitness and joint mobility in children living in Nigeria.

PURPOSE: This study aimed to determine the relationship between kinaesthesia, motor performance, fitness, and joint mobility in children. METHODS: A descriptive cross-sectional study was conducted involving children from two primary schools in the South-Eastern part of Nigeria. The Beighton criteria were used to measure joint mobility. Motor performance, fitness, and kinaesthesia were measured in all the children. Spearman's rank correlation was used to evaluate the relationship between the outcomes. RESULTS: A total of 91 children (51.6% girls) participated in the study. The mean age of the children was 8.20 ± 1.98 years. Using a Beighton score of ≥ 6, Generalized Joint Hypermobility (GJH) was identified in a total of 35 (38.46%) children and was more prevalent in females (60.0%). Joint mobility had significant correlations with most fitness and motor performance items, but not kinaesthesia. Agility & power, and motor performance seem to be reduced if mobility is larger. Kinaesthesia was correlated with most fitness and motor performance items, indicating that better fitness and better motor performance cooccur with better kinaesthesia or vice versa. CONCLUSION: Joint mobility may have a significant influence on fitness and motor performance in children. Hence, it may be useful for future studies to investigate how fitness and motor performance modulate the onset and progression of musculoskeletal symptoms in GJH.

Balance training benefits chronic ankle instability with generalized joint hypermobility: a prospective cohort study.

BACKGROUND: Balance training is the first choice of treatment for chronic ankle instability (CAI). However, there is a lack of research on the effects of balance training in CAI with generalized joint hypermobility (GJH). This study is to compare the outcomes of balance training in CAI patients with and without GJH. METHODS: Forty CAI patients were assigned into the GJH group (Beighton ≥ 4, 20) and non-GJH group (Beighton < 4, 20) and they received same 3-month supervised balance training. Repeated measure ANOVA and independent t test were used to analyze self-reported questionnaires (Foot and ankle ability measure, FAAM), the number of patients experiencing ankle sprain, isokinetic muscle strength and postural control tests (Star excursion balance test, SEBT and Balance errors system, BES) before training, post-training immediately, and post-training 3 months, respectively. RESULTS: At baseline, no differences were found between groups with except for GJH group having poorer SEBT in the posteromedial direction (83.6 ± 10.1 vs 92.8 ± 12.3, %) and in the posterolateral direction (84.7 ± 11.7 vs 95.7 ± 8.7, %). Following the balance training, GJH group demonstrated lower re-sprain ratio (immediately after training, 11.1% vs 23.5%, 3 month after training, 16.7% vs 29.4%) than non-GJH group, as well as greater FAAM-S score, plantarflexion strength and dorsiflexion strength at post-training immediately and 3 months, and both groups improved similarly in the FAAM-A score, muscle strength and balance control (SEBT in the posterior-lateral and posterior-medial directions, and BES scores) compared with baseline. CONCLUSIONS: CAI patients with GJH gained equally even better postural stability and muscle strength after the balance training than the non-GJH patients. Balance training could still be an effective treatment for CAI patients with GJH before considering surgery. TRIAL REGISTRATION: ChiCTR1900023999, June 21st, 2019.

Generalized joint hypermobility does not influence 1-year patient satisfaction or functional outcome after ACL reconstruction.

PURPOSE: The purpose of this study was to evaluate whether generalized joint hypermobility (GJH) influences postoperative results, including return to sport, patientreported outcomes, functional performance (hop tests), muscular strength, and the occurrence of ACL re-injury, in patients 1 year after anterior cruciate ligament (ACL) reconstruction. METHODS: Data was extracted from a regional rehabilitation-specific registry containing information on patients with ACL injury. Patients between the ages of 16-50 years previously undergoing ACL reconstruction with available 1 year follow-up data were eligible for inclusion. Generalized joint hypermobility was assessed using the Beighton score (BS). Patients were examined one year postoperatively in terms of return to sport, patient-reported outcome, hop tests, muscular strength and the occurrence of reinjury. For purpose of analysis, patients were allocated into two groups, depending on the existence of GJH. The KOOS subscale of sports and recreation was considered the primary outcome. Analyses were performed both dichotomously and by using adjusted logistic regression, to consider potential confounders. RESULTS: A total of 356 patients (41% males) were included, of which 76 (24% male) were categorized as having GJH. Patients with GJH had an inferior limb symmetry index preoperatively in terms of knee extension (mean 81.6 [SD 16.4] vs. 91.4 [SD 15.9], p = 0.02) and flexion strength (mean 91.9 vs. 99.1, p = 0.047) compared to patients without GJH. There was no difference between the groups in terms of the primary outcome, nor in any of the other postoperative outcomes. Nine patients (11.8%) in the group with GJH suffered ACL re-injury, compared with 13 patients (4.6%) in the control group (n.s.). CONCLUSION: One year after ACL reconstruction the existence of GJH did not affect postoperative patient satisfaction, strength or functional outcome. No conclusive statements can be made regarding the influence of GJH on the risk of ACL re-injury in this particular study. LEVEL OF EVIDENCE: Level II.

Prevalence, frontal plane knee alignment, and lower limb joint pain and injury in generalized joint hypermobility in Thai physical therapy students.

Objectives: The primary aim was to study the prevalence of generalized joint hypermobility (GJH) among Thai physical therapy (PT) students. The secondary aims were to compare the lower limb alignments and lower limb joint pain and injury between GJH and non-GJH individuals. Furthermore, the association between GJH, lower limb alignment, and joint pain and injury were also evaluated. Material and methods: Generalized joint hypermobility was assessed using the Beighton score with a cut-off of 4/9 in 255 PT students. The lower limb alignments measured in the study included pelvic tilt angle, tibiofemoral angle, quadriceps angle (QA), and navicular drop. Tibiofemoral angle and QA were measured with and without quadriceps contraction. The history of lower limb joint pain and injury was recorded with a simple questionnaire. Lastly, logistic regression analysis was used to study the association between GJH, lower limb alignment, and joint pain and injury. Results: The prevalence of GJH was 21.18% among the studied population. Quadriceps angle during quadriceps relaxation of the non-dominant leg of the GJH group was the only lower limb alignment found greater than those of the non-GJH group. The rate of lower limb joint pain and injury was not different between the two groups. Furthermore, no significant association between GJH, lower limb alignment, and lower limb joint pain and injury was found. Conclusions: GJH is not uncommon among Thai PT students. Only the non-dominant QA was found different between groups. Generalized joint hypermobility neither increase risk nor is it associated with lower limb joint pain and injury among Thai PT students.

No difference in knee muscle activation and kinematics during treadmill walking between adolescent girls with and without asymptomatic Generalised Joint Hypermobility.

BACKGROUND: Altered knee muscle activity in children with asymptomatic Generalized Joint Hypermobility (GJH) is reported during isometric contraction, static and dynamic balance tasks and jumping, but has not been studied during gait. Therefore, the aim was to investigate group differences in knee muscle activity simultaneously with knee joint kinematics during treadmill walking between children with and without GJH. METHODS: Girls 14-15 years of age with GJH (inclusion criteria: Beighton score ≥6 of 9 and positive hyperextension ≥10° (one/both knees)) and a matched control group without GJH (inclusion criteria: Beighton score ≤5 and no knee hyperextension ≥10° ) were recruited. In total 16 participants with GJH and 10 non-GJH participants were included in the study. Surface electromyography (sEMG) was measured from the quadriceps, hamstrings and gastrocnemius muscles of the dominant leg during treadmill walking. Maximal voluntary isometric contractions while sitting were used for normalisation of sEMG to % of Maximum Voluntary EMG (%MVE). Knee joint angles during treadmill walking were measured by electrogoniometer. Furthermore, co-contraction index (CCI) was calculated, and presented for muscle groups of hamstrings-quadriceps (HQ) and gastrocnemius-quadriceps (GQ). CCI of medial and lateral sides of the knee, including ratio of the medial and lateral CCI for HQ and GQ were calculated. RESULTS: No group differences were found in demographics, muscle activation level, nor CCI and CCI ratios. However, participants with GJH displayed significantly decreased knee joint angle, mean (153º vs. 156º; p =0.03) and minimum (105º vs. 111º; p=0.01), during treadmill walking compared with controls. CONCLUSION: Muscle activity during gait was not different between participants with GJH and non-GJH participants. However, participants with GJH displayed minor but statistically significant increased knee flexion during gait. Since the clinical consequences of increased knee joint flexion during gait are unknown, future studies should follow a larger cohort longitudinally during overground walking for development of clinical complications in this group.

Generalized joint hypermobility, scoliosis, patellofemoral pain, and physical abilities in young dancers.

BACKGROUND: Many young girls with generalized joint hypermobility (GJH) choose to participate in dance because their bodies are suited for this activity. Scoliosis tends to occur often in thin girls, who also are more likely to choose dance. Both anomalies (GJH and scoliosis) may be related to reduced abilities such as diminished strength and insufficient postural balance, with increased risk for musculoskeletal conditions. The main objectives of the present study were to determine the prevalence of dancers with GJH, the prevalence of dancers with scoliosis, and the prevalence of dancers with these two anomalies; and, to determine differences in physical abilities and the presence of patellofemoral pain (PFP) between young female dancers with and without such anomalies. METHODS: One hundred thirty-two female dancers, aged 12-14 years, were assessed for anthropometric parameters, GJH, scoliosis, knee muscle strength, postural balance, proprioception ability, and PFP. RESULTS: GJH was identified in 54 dancers (40.9%) and scoliosis in 38 dancers (28.8%). Significant differences were found in the proportion of dancers with no anomalies (74 dancers, 56.1%) and dancers with both anomalies (34 dancers, 25.8%) (p < .001). Dancers with both anomalies had reduced dynamic postural balance in the anterior direction (p = .023), reduced proprioception ability (p < .001), and weaker knee extensors (p = .036) and flexors (p = .040) compared with dancers with no anomalies. Among dancers with both anomalies, 73.5% suffered bilateral PFP, 17.6% suffered unilateral PFP, and 8.8% had no PFP (p < .001). CONCLUSIONS: A high prevalence of young girls participating in dance classes had GJH, as the increased joint flexibility probably provides them with some esthetic advantages. The high prevalence of scoliosis found in these young dancers might be attributed to their relatively low body mass, their delayed maturation, and the selection process of dancers. Dancers with both GJH and scoliosis had decreased muscle strength, reduced postural balance, reduced proprioception, with higher risk of PFP. The main clinical implications are the need to reduce the risk of PFP among dancers by developing appropriate strength and stabilizing exercises combined with proprioceptive and postural balance training, to improve the correct alignment of the hyperextended and hypermobile joints, and to improve their supporting muscle strength.

Evaluation of the uninjured anterior talofibular ligament by ultrasound for assessing generalized joint hypermobility.

BACKGROUND: Most clinicians use the Beighton score to assess generalized joint hypermobility (GJH) when deciding on the treatment of chronic lateral ankle instability (CLAI). The purpose of the study was to evaluate anterior talofibular ligament (ATFL) status by ultrasound and correlate these values with Beighton scores and the manual anterior drawer test (ADT). METHODS: The participants were divided into two groups, those without GJH (24 ankles) and with GJH (20 ankles). For the investigation of ATFL, resting and stress ultrasonography was performed to assess the length, height (degree of loosening) and thickness. Beighton scores, manual ADT grades and ultrasound parameters of participants with and without GJH were compared. The correlation coefficients among those values were analyzed. RESULTS: The mean ATFL length, resting height, stress height and mean difference in height between resting and stress ATFL were all significantly different between the two groups (P < .05). The resting and stress ATFL length, height, and difference in height between resting and stress ATFL showed a positive linear relationship with Beighton scores and manual ADT grades (P < .05). CONCLUSIONS: The ATFL stress ultrasound parameters showed significant differences between participants with high and low Beighton scores and were correlated with Beighton scores and manual ADT grades. LEVEL OF EVIDENCE: Cross-sectional cohort study; Level of evidence IV.

Urogenital and pelvic complications in the Ehlers-Danlos syndromes and associated hypermobility spectrum disorders: A scoping review.

The Ehlers-Danlos syndromes (EDS) and associated hypermobility spectrum disorders (HSD) are a heterogenous group of connective tissue disorders associated with significant morbidity. The urogenital aspects of these disorders are understudied and there is little guidance on the prevalence, types, or outcomes of urogenital complications in EDS/HSD. Our objective was to perform a scoping review to characterize and synthesize the literature reporting urogenital and pelvic complications in EDS/HSD patients. We performed a systematic search of three databases (Medline, CINAHL, Embase) to January 2019. English language, full-text articles reporting on urogenital or pelvic complications in EDS/HSD were included. A total of 105 studies were included (62 case reports/series, 43 observational) involving patients with hypermobile (23%), vascular (20%), classical (12%) EDS, and HSD (24%). Some studies looked at multiple subtypes (11%) or did not report subtype (33%). Reported complications included urinary (41%), gynecological (36%), obstetrical (25%), renal (9%), and men's health problems (7%), with some studies reporting on multiple areas. Urinary and gynecological complications were most prevalent in patients with HSD, while a broad range of complications were reported in EDS. While further research is required, results suggest a higher index of suspicion for urogenital problems is probably warranted in this population.

The Association of Self-Reported Generalized Joint Hypermobility with pelvic girdle pain during pregnancy: a retrospective cohort study.

BACKGROUND: Pelvic girdle pain (PGP) is common during pregnancy but the causes remain poorly understood. Generalized joint hypermobility (GJH) is an inherited trait, with joint mobility beyond normal limits and is assumed to be related with PGP. The aim of this project was to study the association between self-reported GJH and the presence of PGP during pregnancy. METHODS: In this cohort study, 4884 Swedish-speaking women were consecutively recruited at their first visit for registration in the national antenatal screening programme in Sweden. We used the five-part questionnaire (5PQ) to assess GJH and pain drawings to identify PGP. Our primary outcome was the presence of PGP during the entire pregnancy and secondary outcomes were PGP in each trimesters. We tested the associations with logistic regression analysis, and adjusted for age and ethnicity. RESULTS: In all, 2455 (50.3%) women responded to both questionnaires. The prevalence of self-reported GJH was 28.7%. A higher proportion of women with GJH than women without GJH reported PGP during the entire pregnancy (47.9% vs. 41.0%), particularly in trimester 1 (31.6% vs. 22.0%). Thus, women with GJH also had higher odds of PGP during the entire pregnancy (adjusted odds ratio (aOR) 1.27: 95% CI 1.11-1.47) and in trimester 1 (aOR 1.54: 95% CI 1.20-1.96), but the associations were not statistically significant in trimester 2 (aOR 1.24: 95% CI 0.82-1.88) or trimester 3 (aOR 1.20: 95% CI 0.99-1.45). The odds of PGP in pregnancy increased with increasing numbers of positive answers to the 5PQ (p for linear trend < 0.001) for the entire pregnancy and in trimester 1 (p for linear trend < 0.001), but not in trimesters 2 or 3 (p = 0.13 and p = 0.06, respectively). CONCLUSIONS: Compared to women with normal joint mobility, women with GJH had higher odds of reporting PGP during pregnancy and the odds increased with number of positive responses to the 5PQ. The associations were present in trimester 1 but did not reach statistical significance in trimester 2 and 3.

An update on the new classification of Ehlers-Danlos syndrome and review of the causes of bleeding in this population.

It has long been hypothesized that bleeding symptoms in people with hypermobility occur as a result of abnormalities in the collagen of the vessel wall or the connective tissues. The bleeding symptoms, particularly in the skin, have been attributed to the fragility of skin and blood vessels caused by "defective collagen wickerwork" of the reticular layer of the skin. Collagen, which forms the framework of vessel walls, is altered in many patients with Ehlers-Danlos syndrome (EDS) leading to weakening of the vessel wall or the supporting tissues. Another important function of subendothelial collagen is its interaction with platelets and von Willebrand factor, which results in the propagation of a platelet plug. Thus, abnormalities in subendothelial collagen may alter its interaction with platelets and VWF. More recently, hypermobile-EDS (hEDS) has been associated with mast cell disorders, a condition independently associated with bleeding symptoms. It has also been observed that patients with mild bleeding disorders have a more severe bleeding phenotype when they have co-existing joint hypermobility.

Generalized joint hypermobility does not influence clinical outcomes following isolated MPFL reconstruction for patellofemoral instability.

PURPOSE: Generalized joint hypermobility (GJH) has frequently been described as a risk factor for patellofemoral instability; however, only a few primary research studies have demonstrated any evidence of a relationship. The primary purpose of this study was to determine if isolated proximal soft tissue stabilization for patellofemoral instability is as successful in patients with GJH compared those without, as measured by disease-specific quality-of-life (QOL), symptom scores and functional outcomes. The secondary purpose was to determine if clinical and patient-reported outcomes were influenced by patient demographic factors in the presence of joint hypermobility. METHODS: Between 2009 and 2014, data were collected on 174/193 knees (92% follow-up) following an isolated medial patellofemoral ligament reconstruction (MPFL-R). Patients with a Beighton score of 4 or greater were classified as positive for GJH, and any score of 3 or less was classified as negative. Pre- and post-operative Banff Patella Instability Instrument (BPII) scores were compared using a two-sample t test to determine the influence of GJH on QOL. The relationship between the Beighton score, and demographic and clinical factors was explored using correlational analysis. Functional testing including balance and single-leg hop testing was conducted 1 and 2 years post-operatively. Limb symmetry and mean limb-to-limb performance differences were calculated. RESULTS: In this cohort of isolated MPFL reconstructions, 55.1% had a positive Beighton score. There were seven surgical failures (3.6%). There was no evidence of a relationship between a positive Beighton score and pre-operative BPII scores, post-operative BPII scores or post-operative symptom scores. GJH did not correlate significantly with any post-operative functional testing measures. A positive Beighton score was inversely related to patient age and more prevalent in females. CONCLUSIONS: This study determined that the presence of GJH did not influence disease-specific quality of life, physical symptom score or functional outcomes following MPFL reconstruction. LEVEL OF EVIDENCE: III.

Inter- and intra-rater reliability for measurement of range of motion in joints included in three hypermobility assessment methods.

BACKGROUND: Comparisons across studies of generalized joint hypermobility are often difficult since there are several classification methods and methodological differences in the performance exist. The Beighton score is most commonly used and has been tested for inter- and intra-rater reliability. The Contompasis score and the Hospital del Mar criteria have not yet been evaluated for reliability. The aim of this study was to investigate the inter- and intra-rater reliability for measurements of range of motion in joints included in these three hypermobility assessment methods using a structured protocol. METHODS: The study was planned in accordance with guidelines for reporting reliability studies. Healthy adults were consecutively recruited (49 for inter- and 29 for intra-rater assessments). Intra-class correlations, two-way random effects model, (ICC 2.1) with 95% confidence intervals, standard error of measurement, percentage of agreement, Cohen's Kappa (κ) and prevalence-adjusted bias-adjusted kappa were calculated for single-joint measured in degrees and for total scores. RESULTS: The inter- and intra-rater reliability in total scores were ICC 2.1: 0.72-0.82 and 0.76-0.86 and for single-joint measurements in degrees 0.44-0.91 and 0.44-0.90, respectively. The difference between ratings was within 5 degrees in all but one joint. Standard error of measurement ranged from 1.0 to 6.9 degrees. The inter- and intra-rater reliability for prevalence of positive hypermobility findings the Cohen's κ for total scores were 0.54-0.78 and 0.27-0.78 and in single joints 0.21-1.00 and 0.19-1.00, respectively. The prevalence- and bias adjusted Cohen's κ, increased all but two values. CONCLUSIONS: Following a structured protocol, the inter- and intra-rater reliability was good-to-excellent for total scores and in all but two single joints, measured in degrees. The inter- and intra-rater reliability for prevalence of positive hypermobility findings was fair-to-almost perfect for total scores and slight-to-almost-perfect in single joints. By using a structured protocol, we attempted to standardize the assessment of range of motion in clinical and in research settings. This standardization could be helpful in the first part of the process of standardizing the tests thus avoiding that assessment of GJH is based on chance.

Contralateral knee hyperextension is associated with increased anterior tibial translation and fewer meniscal injuries in the anterior cruciate ligament-injured knee.

PURPOSE: To investigate the influence of hyperextension of the contralateral healthy knee on anterior tibial translation (ATT) and the presence of associated injuries in the anterior cruciate ligament (ACL)-injured knee. METHODS: A local patient data register containing the surgical and clinical data of patients undergoing ACL reconstruction was analyzed. Patients were divided into groups according to the degree of hyperextension of the contralateral knee: normal (Group A ≤ 0°), mild (Group B 1°-5°), moderate (Group C 6°-10°), and severe (Group D > 10°). The ATT was measured in both knees preoperatively and 6 months postoperatively using the KT-1000 arthrometer. The presence of associated meniscal and cartilage injuries was noted. Using multivariate analysis, Groups B, C, and D were compared with Group A, using this group as a reference. RESULTS: A total of 10,957 patients were available in the register and 8502 (Group A n = 4335, Group B n = 3331, Group C n = 771, Group D n = 65) were included in the final analysis. Groups B (10.3 mm; 95% CI 0.06-0.042, p < 0.0001) and C (10.6 mm; 95% CI 0.23-0.89, p = 0.006) showed significantly greater preoperative ATT in the injured knee compared with the control group (10.1 mm). Moreover, at the 6-month follow-up, greater ATT was observed for Groups B (8.5 mm; 95% CI 0.13-0.45, p < 0.0001), C (8.5 mm; 95% CI 0.02-0.60, p = 0.035), and D (9.1 mm; 95% CI - 0.08-1.77, p = 0.082) compared with Group A (8.2 mm). Meniscal injuries were less frequent in patients with contralateral hyperextension [Group B 903 (27.1%) p < 0.0001, Group C 208 (27.0%) p = 0.0003, and Group D 12 (18.5%), 0.012] compared with the control group [Group A 1479 (34.1%)]. CONCLUSION: Contralateral knee hyperextension is associated with greater pre- and postoperative ATT in the ACL-injured knee. In patients with contralateral knee hyperextension, concomitant injuries to the menisci are less frequent. Surgeons should consider grafts with superior properties regarding postoperative anteroposterior laxity to patients with contralateral knee hyperextension. LEVEL OF EVIDENCE: Retrospective cohort study, Level IV.

Correlation between quantitative pivot shift and generalized joint laxity: a prospective multicenter study of ACL ruptures.

PURPOSE: To investigate whether an increased magnitude of quantitative rotatory knee laxity is associated with a greater level of generalized joint laxity in ACL-injured and contralateral knees. METHODS: A total of 103 patients were enrolled across four international centers to undergo anatomic ACL reconstruction. Rotatory knee laxity was evaluated preoperatively, both in the awake state and under anesthesia, using the standardized pivot shift test. Two devices were used to quantify rotatory knee laxity; an inertial sensor, measuring the joint acceleration, and an image analysis system, measuring the lateral compartment translation of the tibia. The presence of generalized joint laxity was determined using the Beighton Hypermobility Score. The correlation between the level of generalized joint laxity and the magnitude of rotatory knee laxity was calculated for both the involved knee and the non-involved knee. Further, patients were dichotomized into low (0-4) or high (5-9) Beighton Score groups. Alpha was set at < 0.05. RESULTS: Ninety-six patients had complete datasets, 83 and 13 in the low and high Beighton Score groups respectively. In anesthetized patients, there was a significant correlation between the degree of Beighton Score and quantitative pivot shift when analyzing the non-involved knee using the image analysis system (r = 0.235, p < 0.05). When analyzing the same knee, multivariate analysis adjusted for meniscal injury, age and gender revealed an increased odds ratio for patients with increased lateral compartment translation to be part of the high Beighton Score group (OR 1.86, 95% CI 1.10-3.17, p < 0.05). No other correlation was significant. When analyzing the dichotomized subgroups, no significant correlations could be established. CONCLUSION: The findings in this study suggest that there is a weak correlation between generalized joint laxity and the contralateral healthy knee, indicating increased rotatory knee laxity in these patients. Generalized joint laxity does not appear to correlate with rotatory knee laxity in ACL-injured knees. LEVEL OF EVIDENCE: Prospective cohort study; level of evidence, 2.

Beighton scores and cut-offs across the lifespan: cross-sectional study of an Australian population.

Objectives: The primary aim of this study was to evaluate generalized joint hypermobility (GJH) according to the Beighton scoring system in an Australian population. Secondary aims were to identify whether the commonly used Beighton score cut-off of ⩾4 is appropriate, and to suggest age- and sex-specific Beighton score cut-offs across the lifespan. Methods: A thousand individuals aged 3-101 years were assessed for GJH with the Beighton scoring system. Differences between age, sex and ethnicity were investigated. The appropriateness of the ⩾4 cut-off was investigated with use of a binary logistic regression. Each Beighton score cut-off was established as the nearest Beighton score that delineated the uppermost 5% of the population. Results: Overall, females and non-Caucasians had higher Beighton scores across the lifespan (P < 0.001). Based on a binary logistic regression model, if a cut-off of ⩾4 was utilized, the Beighton scoring system demonstrated a sensitivity of 0.8% and a specificity of 99.3% (P < 0.001). A cut-off of ⩾4 was only found to be appropriate for females aged 40-59 years and males aged 8-39 years. Conclusion: Beighton scores varied across the lifespan and were significantly influenced by age, sex and ethnicity. Assessing GJH using the Beighton scoring system required age- and sex-specific cut-off scores based on the uppermost 5% values. This was confirmed by the low sensitivity, high specificity and 60% false-positive rate if a cut-off of ⩾4 was used for both sexes across the lifespan. To lower the risk of a false-positive diagnosis of GJH, further tests of hypermobility need to be utilized.

[Correlation between generalized joint laxity and symptoms of temporomandibular disorders]. / Zwiazek miedzy wystepowaniem uogólnionej wiotkosci stawowej oraz zaburzen czynnosciowych ukladu ruchowego narzadu zucia.

UNLABELLED: Generalized joint hypermobility (GJH) is an increase of joints mobility over accepted norms. Many authors include GJH to etiologic factors of temporomandibular disorders (TMD). AIM: The aim of the study was to verify if there is an association between GJH and TMD. MATERIAL AND METHODS: Study was performed on 126 subjects (95 females and 31 males) aged 12-46 years. In order to find signs and symptoms of TMD Polish version of RDC/TMD questionnaire was used. GJH was assessed using Beighton's scale. Using r-Pearson's correlation an association between GHJ and TMD signs and symptoms was verified. RESULTS: No statistically significant association between TMD sings and symptoms and GJH was found. CONCLUSIONS: Presence of GJH is not associated with TMD signs and symptoms (in group of young adults).

Knowledge, assessment, and management of adults with joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type among Flemish physiotherapists.

Physiotherapy plays a fundamental role in managing adults with the joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type (JHS/EDS-HT). However, it is a challenge for both the patient and the physiotherapist as the condition is poorly understood and treatment for JHS/EDS-HT is currently undefined. Insight into current practice is, therefore, necessary in order to establish baseline knowledge in this area and in the long term to improve the standard of patient care. Therefore, the purpose of this study was to evaluate current physiotherapists' knowledge of JHS/EDS-HT and to gain insight into current physiotherapy practice with emphasis on assessment, management, and treatment efficacy. Three hundred twenty-five Flemish physiotherapists participated in the study by filling out electronically a modified version of the "Hypermobility and Hypermobility Syndrome Questionnaire" (HHQ), which covered theoretical constructs such as general knowledge, assessment, management, and learning in relation to generalized joint hypermobility and JHS/EDS-HT. The results show that physiotherapists report a low level of confidence with regard to assessment and management of JHS/EDS-HT. Knowledge of hypermobility and JHS/EDS-HT is weak, especially regarding the features associated with JHS/EDS-HT. Many treatment approaches are used by physiotherapists with the majority showing preference for education, reassurance, muscle strengthening, proprioceptive and core stability training. Almost all approaches were perceived as being clinically effective by the physiotherapists, highlighting a lack of consensus. In conclusion, this study in Flemish physiotherapists confirms that JHS/EDS-HT is under-recognized, not well known and deemed difficult to treat. Further education is required and sought by the physiotherapists surveyed, and future research is needed.

Phenotypic Expansion of Autosomal Dominant LZTR1-Related Disorders with Special Emphasis on Adult-Onset Features.

Leucine zipper-like transcription regulator 1 (LZTR1) acts as a negative factor that suppresses RAS function and MAPK signaling; mutations in this protein may dysregulate RAS ubiquitination and lead to impaired degradation of RAS superfamily proteins. Germline LZTR1 variants are reported in Noonan syndrome, either autosomal dominant or autosomal recessive, and in susceptibility to schwannomatosis. This article explores the genetic and phenotypic diversity of the autosomal dominant LZTR1-related disorders, compiling a cohort of previously published patients (51 with the Noonan phenotype and 123 with schwannomatosis) and presenting two additional adult-onset cases: a male with schwannomatosis and Parkinson's disease and a female with Noonan syndrome, generalized joint hypermobility, and breast cancer. This review confirms that autosomal dominant LZTR1-related disorders exhibit an extreme phenotypic variability, ranging from relatively mild manifestations to severe and multi-systemic involvement, and offers updated frequences of each clinical feature. The aim is to precisely define the clinical spectrum of LZTR1-related diseases, using also two new emblematic clinical cases. Gaining insight into the mechanisms underneath this variability is crucial to achieve precision diagnostics and the development of therapeutic interventions.