scholarly journals The Beighton Score as a measure of generalised joint hypermobility

2021 ◽  
Author(s):  
Sabeeha Malek ◽  
Emma J. Reinhold ◽  
Gemma S. Pearce
Keyword(s):  
Scoring System ◽  
Joint Hypermobility ◽  
Clinical Tool ◽  
Beighton Score ◽  
Consistent Finding ◽  
Epidemiological Tool ◽  
Large Populations ◽  
Indirect Indicator ◽  
Clinical Thinking ◽  
Method Of Assessment

AbstractThe Beighton Score (BS) is a set of manoeuvres in a nine-point scoring system, used as the standard method of assessment for Generalised Joint Hypermobility (GJH). It was originally developed as an epidemiological tool used in screening large populations for GJH, but later adopted as a clinical tool for diagnostic purposes. Its ability to truly reflect GJH remains controversial, as joints within the scoring system are predominantly of the upper limb and disregard many of the major joints, preventing a direct identification of GJH. Furthermore, a consistent finding in the literature whereby the BS failed to identify hypermobility in joints outside the scoring system suggests its use as an indirect indicator of GJH is also not viable. As such, the collective findings of this review demonstrate a need for a change in clinical thinking. The BS should not be used as the principle tool to differentiate between localised and generalised hypermobility, nor used alone to exclude the presence of GJH. Greater emphasis should be placed on a clinician’s judgement to identify or exclude GJH, according to its full definition.

Simplified Fixation and Staining for Delineation of Nuclear Chromatin by the Barr Technique

PEDIATRICS ◽  
1959 ◽  
Vol 24 (5) ◽  
pp. 858-859
Author(s):  
A. ROBINSON
Keyword(s):  
Mucous Membrane ◽  
Somatic Cells ◽  
Screening Procedure ◽  
Nuclear Chromatin ◽  
Clinical Tool ◽  
Chromosomal Constitution ◽  
Large Populations ◽  
Buccal Mucous Membrane ◽  
Original Procedure

Examination of smears of the buccal mucous membrane for nuclear chromatin has become a useful clinical tool in the evaluation of conditions involving questions of chromosomal sex. Although actual delineation of the complete chromosomal constitution of the patient's somatic cells yields more information than the nuclear chromatin determination alone. the latter test is extremely useful because of its simplicity. This makes it readily available for use on any patient and applicable to large populations as a screening procedure for detection of certain kinds of anomalies. Hence, it seems worthwhile to call attention to the following modification of the Barr test for nuclear chromatin, which is more rapid than the original procedure, but appears to be every bit as reliable.

scholarly journals Ehlers-Danlos syndrome presenting with primary nocturnal enuresis

2020 ◽  
Vol 13 (2) ◽  
pp. e231977
Author(s):  
Margarida Cunha ◽  
Mafalda Matias ◽  
Inês Marques
Keyword(s):  
Genetic Testing ◽  
Nocturnal Enuresis ◽  
Bladder Dysfunction ◽  
Connective Tissue Disorder ◽  
Joint Hypermobility ◽  
Urinary Urgency ◽  
Beighton Score ◽  
Ehlers Danlos Syndrome ◽  
Primary Nocturnal Enuresis ◽  
Danlos Syndrome

Ehlers-Danlos syndrome (EDS), hypermobility type, is probably the most common EDS type, as well as the most common heritable connective tissue disorder. Bladder dysfunction is a rare clinical manifestation of EDS and manifests itself as primary nocturnal enuresis. We present a 10-year-old boy referred to the paediatrics nephrology consultation due to primary nocturnal enuresis and day time symptoms of urinary urgency. During the appointment, a tendency to joint hypermobility was noted. On evaluation the skin was hyperextensible and the Beighton score was positive. The genetic testing revealed a variant of the COL5A1 gene not yet described in the literature.

scholarly journals Dermal Ultrastructure in Low Beighton Score Members of 17 Families with Hypermobile-Type Ehlers-Danlos Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Trinh Hermanns-Lê ◽  
Marie-Annick Reginster ◽  
Claudine Piérard-Franchimont ◽  
Philippe Delvenne ◽  
Gérald E. Piérard ◽  
...  
Keyword(s):  
Clinical Parameter ◽  
Joint Hypermobility ◽  
Elastic Fibers ◽  
Hypermobility Syndrome ◽  
Beighton Score ◽  
Joint Hypermobility Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Transmission Electron ◽  
Asymptomatic Subjects ◽  
Danlos Syndrome

The distinction between the Ehlers-Danlos syndrome hypermobile type (EDSH) and the benign joint hypermobility syndrome (BJHS) is unclear. The aim of the present study was to compare skin ultrastructural abnormalities of EDSH and BJHS among different families. Skin of 23 EDSH, 27 BJHS, and 41 asymptomatic subjects from 17 families was examined using transmission electron microscopy. Similar ultrastructural abnormalities were found irrespective of the Beighton score. Flower-like collagen fibrils represented the key change and elastic fibers were altered as well. Beighton score is a clinical parameter rating joint mobility that appeared unrelated to quantitative and qualitative collagen ultrastructural alterations in the skin. Some EDSH family members fit with BJHS diagnosis. BJHS possibly represents a mild variant of EDSH.

A Novelin VivoSkin Extensibility Test for Joint Hypermobility

2010 ◽  
Vol 37 (7) ◽  
pp. 1513-1518 ◽  
Author(s):  
ADAM D. FARMER ◽  
HATTY DOUTHWAITE ◽  
SAM GARDINER ◽  
QASIM AZIZ ◽  
RODNEY GRAHAME
Keyword(s):  
Interobserver Variability ◽  
Group Versus ◽  
Strain Curve ◽  
Joint Hypermobility ◽  
Skin Thickness ◽  
Beighton Score ◽  
Joint Hypermobility Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Skin Fold Thickness ◽  
Dermal Collagen

Objective.The stress/strain curve derived from stretching skin is not linear, but follows a J-shaped curve. An initial generous yield is followed by a steep linear phase where considerable additional force is required to achieve modest increases in deformation. The former represents the taking up of slack resulting from the alignment of dermal collagen bundles in the line of force, while the gradient of the latter represents Young’s modulus for skin. Skin hyperextensibility in Ehlers-Danlos syndrome (EDS) is limited to the initial phase of taking up slack. Skin hyperextensibility and joint hypermobility (JHM) form part of the Revised 1998 Brighton diagnostic criteria for the benign joint hypermobility syndrome (BJHS), considered by many to be akin to EDS-hypermobility type. JHM may be screened for using the Beighton Score or a 5-point questionnaire. Our aim was to validate a novel method of measuring skin extensibility based on these observations in addition to revalidating the 5-point questionnaire.Methods.250 volunteers (131 female), median age 39 years (range 18–89 yrs), without BJHS, had their joint mobility evaluated using the Beighton Score, compared to the 5-point questionnaire. A Beighton score ≥ 4/9 was considered to represent JHM. Skin extensibility was determined by placing 2 dots on the dorsum of the right hand between the second and third metacarpals, approximately 10 mm apart, and was measured using an electronic caliper. Perpendicular to the metacarpals, a force was applied until the skin was fully taut and the increment was measured. Skin-fold thickness was measured using a Harpenden caliper. A corrected skin extensibility score (CSES) was calculated by dividing the percentage increment by skin thickness. Interobserver variability was measured in a further 50 healthy volunteers.Results.The prevalence of JHM was 17.6%. Revalidation of the 5-point questionnaire returned a sensitivity of 0.85 and specificity of 0.85. The mean CSES was 23.84%/mm in the hypermobile group versus 13.55%/mm in the normal mobility group (p < 0.0001). CSES sensitivity was 0.72, specificity 0.75. The κ value for interobserver variability was 0.83.Conclusion.The CSES is a useful and reproducible measure of skin extensibility in health. Further work is warranted to validate this test in patients with BJHS.

scholarly journals Generalized Joint Hypermobility among School-going children in Majmaah region, Saudi Arabia

2019 ◽  
Author(s):  
Mohamed Sherif Sirajudeen ◽  
Mohamed Waly ◽  
Mazen Alqahtani ◽  
Msaad Alzhrani ◽  
Hariraja Muthusamy ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
School Children ◽  
Personal Characteristics ◽  
Joint Hypermobility ◽  
Primary School Children ◽  
Cutoff Score ◽  
Score Distribution ◽  
Beighton Score ◽  
Cross Sectional ◽  
Generalized Joint Hypermobility

Abstract Background Generalized Joint Hypermobility (GJH) is common among schoolchildren and usually benign. However, progressively may lead to joint pain and developmental delay. Identifying the GJH at the school-age would facilitate to monitor early changes and to plan for early rehabilitative intervention. Hence, we aimed to determine the prevalence, pattern, and factors associated with the GJH among school-going children in the Majmaah region, Saudi Arabia. Methods The school children of both genders aged 8 to 14 years from the Majmaah region of Saudi Arabia participated in this cross-sectional study. The Beighton’s score was used to assess the joint hypermobility. Personal characteristics like age, height, weight, Body mass index (BMI), and handedness was also collected. Descriptive statistics were produced for personal characteristics, the point prevalence of hypermobility, frequency of Beighton score distribution, and prevalence of GJH. The association between specific factors and the presence of GJH was analyzed using Chi-square tests and logistic regression measures. Results The occurrence of GJH as defined by Beighton cutoff score ≥ 4 in the 303 participated primary school children was 15.2%. The prevalence of GJH was high among females (16.8%) compared to males (13.4%), but this difference is not statistically significant. Younger male children (age 8 to 11 years) were 16.7 times more probable to develop hypermobility (adjusted OR 0.06 [95% CI 0.01 – 0.56]) compared to older male children (age 12 to 14 years). No associations were observed among female children. Conclusion The prevalence rate reported in this study among school-going children was in the lower range in comparison with the rates reported across the globe. Beighton score of ≥4 was adequate for use in identifying GJH among children in Saudi Arabia.

scholarly journals Does the item ‘hands on floor’ add value to the Beighton score in identifying joint hypermobility?

2020 ◽  
Vol 7 (2) ◽  
pp. 79-83
Author(s):  
Lieselotte Corten ◽  
◽  
Gillian Ferguson ◽  
Bouwien Smits-Engelsman ◽  
◽  
...  
Keyword(s):  
Joint Hypermobility ◽  
Beighton Score ◽  
Hands On

The Relationship Between Beighton Score and Walking Age in Children

2021 ◽  
Vol 71 (1) ◽  
pp. 5-10
Author(s):  
Lavenia Kurniawati ◽  
Indrayuni Lukitra Wardhani ◽  
Nur Rochmah ◽  
Sulistiawati
Keyword(s):  
Joint Hypermobility ◽  
Beighton Score ◽  
Design Data ◽  
Cross Sectional ◽  
Walking Age ◽  
Physical Examinations ◽  
Score Method ◽  
Cross Sectional Design ◽  
The Relationship ◽  
Research Type

Introduction: Studies showed that the condition of joint hypermobility is quite common in children. In general, joint hypermobility asses through the Beighton score method. The objective of this research was to investigate the relationship between beighton score and walking age in children.Method: This research was conducted at a kindergarten in Surabaya using an observational analytic research type and a cross-sectional design. Data collected through interviews and physical examinations. A joint hypermobility examination performed using the Beighton score criteria. Meanwhile data was analyzed using the crosstabulation and Spearman test.Result: It was found that 84% of children had ≥4/9 score on the Beighton score and there were 92% with positive Beighton score in the knee joint. In this study, as many as 20 children experienced the first independent walks over 18 months when 18 of them who has Beighton score ≥4 points. However, there is no correlation found on the Beighton score and the walking age [sig. (2-tailed) = 0.053].Conclusion: Children who have ≥4 points of Beighton score are mostly found in this study. The relationship between the Beighton score and walking age was not statistically significant.

Prevalence of Suspected Collagen Disorders In the Adult Bleeding Disorder Clinic.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1399-1399
Author(s):  
Shannon Jackson ◽  
Leah Odiaman ◽  
Morna Brown ◽  
Carol Spitzer ◽  
Robert T. Card ◽  
...  
Keyword(s):  
Bleeding Time ◽  
Joint Hypermobility ◽  
Bleeding Tendency ◽  
Beighton Score ◽  
Female Population ◽  
Primary Hemostasis ◽  
History Of ◽  
Bleeding Score ◽  
Von Willebrand ◽  
Predominately Female

Abstract Abstract 1399 Background: Disorders of collagen may be associated with a mild bleeding tendency because of the interaction of collagen with von Willebrand factor (VWF) and platelets required during primary hemostasis and generalized soft tissue fragility. Therefore, collagen disorders may be unrecognized contributors to existing disorders of primary hemostasis. Synergism between these entities could produce a more significant bleeding tendency than predicted based on laboratory abnormalities alone. Symptomatic joint hypermobility (SJH) is found in over 90% of collagen disorders and can be objectively measured as a surrogate marker of a potential collagen disorder. Objective: To determine the prevalence of suspected collagen disorders in a cohort of adult subjects with mucocutaneous inherited bleeding disorders in comparison to healthy controls using SJH as a marker for a potential collagen disorder. Methods: Fifty-five consecutive subjects, ≥ 16 years of age, attending the Adult Bleeding Disorders (BD) Clinic with a history of von Willebrand disease, platelet function disorder or undefined bleeding disorder and 60 controls (age and sex-matched) were enrolled between June 2008 and February 2010. All subjects were assessed for: i) Generalized joint hypermobility, defined using the Beighton score whereby a score of ≥ 4 out of 9 is considered positive; ii) SJH, defined using the major Brighton criteria which is a Beighton score of ≥ 4 AND arthralgia for longer than 3 months in 4 or more joints; and, iii) Bleeding score (BS), using the Condensed MCMDM1-VWD, with a positive score defined as ≥ 4 for both sexes. CBC, blood group, VWF:Ag, VWF:RCo, FVIII:C and closure times were collected prospectively in the control group and retrospectively in the clinic subjects in addition to bleeding time and platelet aggregation studies. Results: The prevalence of SJH with a positive bleeding score was 24% in the BD clinic and 1.6% in controls (see table). Seventy-seven percent of SJH subjects (10/13) in the BD clinic had a prior personal or family history of Ehlers-Danlos Syndrome, other Joint Hypermobility Syndrome or Osteogenesis Imperfecta (OI). Only the OI subject had prior clinical documentation of a collagen disorder. There was no statistical difference in laboratory measures of hemostasis or bleeding score in the BD clinic subjects with or without SJH. Of interest, mean bleeding time in the BD clinic subjects (available in 38 subjects) was 10.5 minutes in the SJH group and 10 minutes in the non-SJH group suggesting that bleeding time is not a good screening test for the presence of abnormal collagen. Isolated joint hypermobility (Beighton score of ≥ 4) not associated with arthralgia was common in both groups (40% and 30% respectively) in this predominately female population of subjects with inherited mucocutaneous bleeding. Conclusions: SJH suggesting a collagen disorder is common and often unrecognized in the BD clinic as a potential contributor to the bleeding symptoms. Asymptomatic joint hypermobility is common in this predominately female population and not an adequate screening tool for a potential collagen disorder. A prolonged bleeding time did not predict the presence of SJH in this cohort. Further study is required to confirm the nature of collagen disorders in the BD clinic population and the relationship to bleeding manifestations. Disclosures: No relevant conflicts of interest to declare.

scholarly journals An innovative risk-scoring system of dental procedures and safety protocols in the COVID-19 era

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
M. E. Bizzoca ◽  
G. Campisi ◽  
Lorenzo Lo Muzio
Keyword(s):  
Scoring System ◽  
Direct Contact ◽  
Infection Risk ◽  
Risk Scores ◽  
Risk Level ◽  
Clinical Tool ◽  
Dental Procedures ◽  
Risk Scoring ◽  
General Dentists ◽  
Risk Scoring System

Abstract Background The aim of this paper is to assess an innovative risk score for common dental procedures, based on the most recent contaminant SARS-CoV-2. After scoring the level of infection risk, safety procedures, advice and personal protective equipment (PPE) are recommended for the dental team in each dental practice. Methods The authors of this research analysed 42 common dental procedures on the basis of known transmission risks. In increasing order, many consider the parameters leading to different risk scores for the dental team and patients for each procedure to be: direct contact with saliva (score 1), direct contact with blood (score 2), production of low levels of spray/aerosol via air–water syringes (score 3), the production of high levels of spray/aerosol from rotating, ultrasound and piezoelectric tools (score 4); and the duration of the procedure, which may increase the risk of procedures producing droplets and aerosols. Results Using this innovative risk-scoring system, the authors classified the different dental procedures according to low, medium or high risk: low (1–3), medium (4–5), high (≥ 6). A safety protocol for each procedure was thereafter matched with the calculated risk level. Conclusions The innovative risk-scoring system presented in this research permits the reclassification of dental procedures according to the infection risk level. Consequently, specific procedures, previously considered as entry level, will now merit revision. This paper also highlighted an effective and routine clinical tool for general dentists and oral medicine practitioners.

Cervical Spine Joint Hypermobility: A Possible Predisposing Factor for New Daily Persistent Headache

Cephalalgia ◽  
2006 ◽  
Vol 26 (10) ◽  
pp. 1182-1185 ◽  
Author(s):  
TD Rozen ◽  
JM Roth ◽  
N Denenberg
Keyword(s):  
Cervical Spine ◽  
Range Of Motion ◽  
Physical Therapists ◽  
Joint Hypermobility ◽  
New Daily Persistent Headache ◽  
Beighton Score ◽  
Predisposing Factor ◽  
Cervical Range Of Motion ◽  
Persistent Headache

The objective of this study was to suggest that joint hypermobility (specifically of the cervical spine) is a predisposing factor for the development of new daily persistent headache (NDPH). Twelve individuals (10 female, 2 male) with primary NDPH were evaluated by one of two physical therapists. Each patient was tested for active cervical range of motion and for the presence of excessive intersegmental vertebral motion in the cervical spine. All patients were screened utilizing the Beighton score, which determines degree of systemic hypermobility. Eleven of the 12 NDPH patients were found to have cervical spine joint hypermobility. Ten of the 12 NDPH patients had evidence of widespread joint hypermobility with the Beighton score. Based on our findings we suggest that joint hypermobility, specifically of the cervical spine, may be a predisposing factor for the development of NDPH.

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