Hypermobility In Children Research Articles

Post-traumatic stress and joint hypermobility in children and adolescents of Nepal after exposure to an earthquake.

A substantial body of literature has traditionally addressed the connection between the exposure to catastrophic events and the development of Post-Traumatic Stress disorder (PTSD), especially in the vulnerable stratum of children and adolescents. However, little is known about their biological predisposing factors, and further research is needed, especially in the context of the recent earthquakes in Turkey and Syria. The data of this study was collected 4 months after the 2015 earthquakes in Nepal, with the objective of providing new evidence to the field and documenting the role of a new potential predisposing factor: the Joint Hypermobility Syndrome (JHS). 941 subjects from three different regions of the country, aged 8-18 years, were assessed in a school-based cross-sectional investigation. PTSD, as the main response variable, was assessed using the Child PTSD Symptom Scale (CPSS) questionnaire and analysed considering three sub-dimensions: the severity of symptoms, the severity of impairment, and both taken together. JHS was assessed using the Screening Questionnaire to detect Hypermobility (SQ-CH) questionnaire. The severity of symptoms was strongly predicted by the distance to the epicentre. Females showed more severe symptomatology, but a lower perturbation in the daily functioning. Younger children reported a greater functional impairment. JHS group showed more severe PTSD than non-JHS group. We observed variability in the severity of PTSD according to previously known risk factors such as the distance to the epicentre, sex, and age. We also found an association between PTSD and JHS, which is discussed in reference to the neuroconnective endophenotype. It might be useful to consider the role of each variable when planning a mass intervention after a disaster.

Variability of joint hypermobility in children: a meta-analytic approach to set cut-off scores

Current international consensus of the appropriate Beighton score cut-off to define if a child has generalised joint hypermobile or not is based upon expert opinion. Our aim was to determine the prevalence of Beighton scores of children worldwide to provide a recommendation for establishing the Beighton score cut-off to identify generalised joint hypermobility in children. We used AMED, OVID Medline, Embase and CINAHL to find published articles from inception to April 2024 describing Beighton scores of children up to and including 18 years from the general population. We extracted study demographics including country of publication, total number of participants, summary data about the age and sex of participant, Beighton scores and any cut-off used where authors deemed children hypermobile and how many children were rated at the corresponding Beighton scores. There were 37 articles reporting on the prevalence or incidence of hypermobility at cut-off scores from 28,868 participants. Using the cut-off of ≥ 6 resulted in a prevalence of 6% for studies reporting male data and 13% for studies reporting female data. Limited data reporting availability precluded further sub-analysis at a Beighton score of ≥ 7, age, pubertal status and ethnicity. Conclusion: The working threshold for identifying generalised joint hypermobility in children should be a Beighton score of 6 or more. Our analysis also suggests a Beighton score of 7 or greater may be appropriate in childhood, particularly for females.What is Known:• The working threshold for identifying generalised joint hypermobility in children previously was set based on expert opinion.What is New:• The threshold to identify hypermobility in children should be at a minimum of ≥ 6 on the Beighton score.

Relationship and New Prospectives in Joint Hypermobility in Children with Autism Spectrum Disorder: Preliminary Data.

Autism spectrum disorder (ASD) and joint hypermobility (JH) are considered two different etiological and clinical entities that most often appear in childhood. Despite growing increased research showing a co-occurrence for both conditions, a link between them is rarely established in clinical settings, and the relationship between ASD and JH has not so far been completely investigated in all age groups of ASD children. This preliminary study examined a cohort of 67 non-syndromic ASD children aged 2-18 years (sex ratio M:F = 12:1) showing different degrees of cognitive impairment and autism severity, using the Beighton scale and its revised version. A total of 63% of ASD patients aged 2-4 years and 73% of ASD patients aged ≥5 years presented significant scores of hypermobility. No significant correlation was found comparing total laxity score and cognitive assessments and severity of autistic symptomatology (p > 0.05). The results suggest that JH could be considered as a clinical characteristic of ASD patients and it needs to be assessed in order to schedule a better rehabilitation program.

Особенности скрининг-диагностики гипермобильности суставов у младших школьников

Joint hypermobility is a little-studied and relatively new risk factor for Russian school medicine, leading to impaired functions of the musculoskeletal system. The study involved 50 conditionally healthy children of primary school from 7 to 8.4 years. Main group consisted of 26 students of the 1st grade (12 boys, 14 girls) of the village school "Podosinki" (Moscow region). Сomparison group consisted of 24 female athletes (engaged in rhythmic gymnastics for at least one year) from 7.7 to 8.3 years, living in Moscow and Moscow region. All children underwent a detailed study of the volume of passive movements in the wrist, elbow, knee and hip joints, as well as in the small joints of the hand; Postural changes were determined by non-invasive methods. Comparison of the results in both groups showed that all respondents equally have risk factors for the development of orthopedic diseases and pathological conditions: posture disorders (kyphoscoliotic type), valgus installation of feet and flattening of their arches of the feet (mobile flat feet), hypermobility of the joints. The results of the study questioned the adequacy of the assessment of hypermobility on the Beighton scоre in children of this age group; According to studies, further research is needed to find a measure of joint hypermobility in children. The study was conducted with the support of the Ministry of Education of Russia. Key words: joint hypermobility, children, musculoskeletal system, risk factors

Determinants for Predicting Polyorganic Functional Changes of the Musculoskeletal System in Children with Various Degrees of Joints Hypermobility

Aim: The aim of the work was to study the presence of determinants for predicting the local structures involvement of the musculoskeletal system against the background of joint hypermobility in children. Materials and Methods: The study involved 446 children aged from four to six years. The study plan includes: copying the data of a comprehensive medical examination; determination of joint hypermobility by goniometry and evaluation according to the criteria of presence and degree of joint hypermobility by the method of C. Carter, J. Wilkinson – in the modification of P. Beighton; application of clinical and physiological methods (functional testing, genealogical analysis) and methods of mathematical data processing. Results: The significance hierarchy of morpho-functional features in relation to the further development of the pathological process in children with one of the main manifestations of undifferentiated connective tissue dysplasia – joint hypermobility – is predicted. Determinants of predicting local involvement of musculoskeletal structures (mobility of the spine in the sagittal plane, muscles endurance of the dorsal torso surface) were determined. Conclusions: Against the background of the study of a wide range of clinical features of several generations’ orthopaedic lesions, the fact of highly-progressing risk of scoliotic disease and flat foot was established.

Symptomatic hypermobility in children and young people: A scoping review of clinical characteristics using a developmental framework

OBJECTIVE: To evaluate the literature on children and young people with symptomatic hypermobility using a developmental framework. METHODS: A search was conducted in EMBASE, Medline, CINAHL, Web of Science and grey literature. Full text articles reporting children and young people (birth to 24 years) with a confirmed diagnosis of symptomatic hypermobility (including Hypermobility Spectrum Disorder or hypermobile Ehlers-Danlos syndrome) using internationally recognised criteria or equivalent diagnoses were included. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Extension for Scoping Reviews methodology was followed. RESULTS: Of the 1619 studies screened, 163 were included in the scoping review. Studies were published from 1967-2021, 30% were narrative reviews and 24% were case reports, from a range of journals. Musculoskeletal and cutaneous characteristics were common in all developmental stages. Cardiovascular characteristics including autonomic dysfunction, fatigue, and gastrointestinal issues were reported more frequently by adolescents. Young adult studies were scarce and included mostly case reports. Varied diagnostic criteria and assessment methods were identified. The Beighton Score was frequently used (93%), but rarely standardised (12%) and lacked consensus regarding the cut-off point of hypermobility. CONCLUSIONS: This novel systematic scoping review identifies the changing phenotype of symptomatic hypermobility from childhood to adolescence, and the unique stage of young adulthood. There is a lack of guidance regarding the utilisation of standardised tools to assess hypermobility in clinical practice and research. Numerous and inconsistent diagnostic criteria and assessment methods limit the data analysis. Case control and longitudinal studies using defined, bespoke criteria and assessments are needed.

P03 Utilizing a videography platform for the therapeutic management of chronic pain relating to Hypermobility in children and young people

Introduction/BackgroundMany patients present to Paediatric Rheumatology clinic, (53%) with chronic pain related to Hypermobility. Pre-Covid, an evidenced based hypermobility class was developed and run by the Allied Health Professional team for children and young people (C & YP), and their parents/carers. These 6 classes were group-based and included education and exercise therapy but were stopped due to the pandemic. Feedback gained was good from the class, although reported issues included reduced school attendance, accessibility due to location, parental time off work, financial and time implications of travel, and unsuitability of group interventions for CYP with social anxiety and autism disorders.Description/MethodA digital platform (Vimeo) was used to present Hypermobility education and exercise content created by the Paediatric Rheumatology team. The video aims to address these barriers, allowing more equality of access for all CYP; digital access allows CYP and parents to re-visit this information at any time, providing ongoing support for long term management of this condition. The digital platform is being developed to maximize the effectiveness of therapy resources and to improve the patients experience to promote self-management of their hypermobility.Funding was applied for via the Transformation service. Meeting with the hospital videographer enabled the AHP team to devise a template for the video including education on what is Hypermobility, on the role of exercise - targeted versus general, the benefits of exercise, supportive footwear, pain management, pacing, sleep hygiene, and hand function advice to manage hypermobility. The class exercise programme including hand therapy was videoed using a model.The video was filmed in half a day. It was filmed using an unscripted question- and- answer format. The Paediatric Rheumatology MDT were filmed in the video. The digital platform is currently with our videography team for further edit. Further signposting to established resources will be included in the Vimeo resource.Following a final edit, this resource will be used for this patient population group. Feedback from CYP and parents, along with other health professionals is planned.Discussion/ResultsOn receipt of the first edit, multi-source feedback from parents/carers, CYP and other health professionals is planned via Microsoft Teams. It is hoped to show the video via Teams and seek anonymized feedback on the Teams chat function. This feedback will be discussed by the Paediatric Rheumatology MDT and will inform the final edit of the Hypermobility video resource.Further data from Vimeo analytics will be gathered e.g. how many times video watched, how long video is watched for, and what devices are being used.It is hoped that the Hypermobility Video will help support CYP and families in the long-term management and pain resulting from this condition.Key learning points/ConclusionIt is hoped that the use of new technologies and innovative ways of delivering care can enable health professionals to implement clinical support, improve self-management of chronic pain related to hypermobility, whilst improving equitable patient access to therapy across the North-West.Multi-source feedback will help inform the final edit of the video resource. Ongoing evaluation and analysis of Vimeo data will inform whether the resource is a useful adjunct in long term management of pain arising from Hypermobility.

Developmental Coordination Disorder and Joint Hypermobility in Childhood: A Narrative Review.

Children with developmental coordination disorder (DCD) and joint hypermobility could present an overlap of symptoms and motor functional difficulties. The link between these two clinical conditions has not yet been clarified. Recent studies reported a high incidence (30–50%) of motor delay in children who are referred to hypermobility and of enhanced joint hypermobility in children with DCD. The aim of this study was to provide a critical review of the literature outlining the association between DCD or limited motor performance and joint hypermobility. Studies were eligible for inclusion if they were written in English and human-based. All the studies were first selected, looking for the presence of a clinical association between developmental coordination disorder or motor performance and hyperlaxity and reporting details of outcome. After a review of the full texts, 16 articles for a total of 1898 children met the inclusion criteria. In general, there was evidence of a higher incidence of motor delay or DCD in children who are referred to hypermobility and of enhanced joint hypermobility in children with DCD with similar range of functional difficulties. These results could influence the way to support children with rehabilitation and the type of intervention according to the prevalence of one of the two conditions.

Clinical characteristics of symptomatic hypermobility in children and young people: A scoping review protocol

INTRODUCTION: Joint hypermobility (JH) is a term used to define active or passive joint range of motion that is beyond normal range, accounting for age, sex, and ethnicity. Symptomatic hypermobility is a term that can be used when symptoms are thought to be associated with JH. Children and young people with symptomatic hypermobility complain of musculoskeletal symptoms but also may report symptoms from other domains including gastrointestinal, cardiovascular, psychological, and urogenital. Many of these symptoms are not included in formal diagnostic criteria yet may impact negatively on their quality of life. The pattern of these symptoms may change with age. PURPOSE: To map the literature on clinical characteristics of symptomatic hypermobility in an age and developmental context, to improve our understanding and assist in the clinical assessment of children and young people with symptomatic hypermobility. METHODS: This systematic scoping review will be conducted in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) methodology. Studies that include children and young people from birth to 24 years with a confirmed diagnosis of symptomatic hypermobility, HSD or hEDS using internationally recognised criteria or equivalent diagnoses will be included. Data extraction and analysis will be undertaken using an iterative process. DISCUSSION: Mapping and synthesis of the data will be carried out and gaps and limitations in the literature will be acknowledged. Results will be disseminated in a peer reviewed journal. The search strategy will be made available publicly for transparency.

Hypermobility among children with autism spectrum disorders and its correlation with anthropometric characteristics.

To determine the extent of hypermobility in children with Autism Spectrum Disorder and to determine the correlation between age, gender, height, weight, BMI and hypermobility. This cross-sectional study included 117 children with Autism Spectrum Disorder aged 2 to 17 years, of whom 91 were boys and 26 were girls. After obtaining the written informed consent from the parents of these children with Autism Spectrum Disorder, we assessed their level of hypermobility using the Beighton score. Out of 117 children, 47 (40.17%) were normal and 70 (59.83%) showed an abnormal increase in mobility. The average Beighton score was 5.33 ± 2.42. There is a moderate negative correlation between height, weight, and age with hypermobility. The extent of hypermobility among children with Autism Spectrum Disorder was 60%, that is, 70 out of 117 children had hypermobility. Our results also suggested that the age, height, weight, and BMI of the child had a moderate negative correlation with hypermobility.

Prevalence of generalized joint hypermobility in children with anxiety disorders

BackgroundConcerning the high prevalence of anxiety disorders and joint hypermobility in children and the lack of related studies in this age group, we aimed to assess the association of hypermobility with anxiety disorders in children.MethodsIn this case-control study, 93 children ages 8–15 years with anxiety disorders referring to the Child and Adolescent Psychiatry Clinic of Mofid Children’s Hospital, Tehran, Iran, during 2018, were enrolled. The control group consisted of 100 age and sex-matched children without anxiety disorders. Anxiety was evaluated using the Spence Children Anxiety Scale (SCAS). The diagnosis of generalized joint hypermobility was done based on Beighton and Shiari-Javadi criteria.ResultsBased on Beighton’s diagnostic criteria 52.7% of the children in the case group and 16% of the children in the control group had generalized joint hypermobility. Moreover, based on Shiari-Javadi criteria, 49.5 and 13% of the children in the case and control groups had generalized joint hypermobility, respectively. Moreover, the internal correlation between the two criteria was 0.91 showing almost complete compatibility between the two (P < 0.001). Age was a risk factor that could predict hypermobility in these children. Other variables such as sex, severity, and type of anxiety disorders, and ADHD, were not predictors of hypermobility syndrome.ConclusionThe prevalence of hypermobility was three times higher in children with anxiety disorders and only age was a predictor for the possibility to suffer from generalized joint hypermobility in these children.

The Prevalence of Hypermobility in Children with Irritable Bowel Syndrome and Functional Abdominal Pain Is Similar to that in Healthy Children

To test the hypothesis that the prevalence of joint hypermobility is greater in children with irritable bowel syndrome and functional abdominal pain than in healthy control children and is related to gastrointestinal symptoms and psychosocial distress (anxiety, depression, and somatization). Children (irritable bowel syndrome, n=109; functional abdominal pain, n=31; healthy control, n=69), 7-12years of age completed prospective 2-week pain and stooling diaries and child- and parent-reported measures of anxiety, depression, and somatization. Joint hypermobility was determined using Beighton criteria (score of ≥4 or 6). We also examined possible relationships between Beighton score, race, body mass index, gastrointestinal symptoms, and psychosocial distress. Beighton scores were similar between groups, as was the proportion with joint hypermobility. Scores were higher in girls (3.1±2.4) than boys (2.3±1.8; P=.004) and decreased with age (P<.001; r=-0.25). Race and body mass index did not impact joint hypermobility prevalence. Beighton scores were not related to abdominal pain or stooling characteristics. Participants with a score of ≥4 and≥6 had greater somatization and depression by child report (P=.017 and P=.048, respectively). No association was seen for anxiety. There was no significant association between joint hypermobility and psychosocial distress measures per parent report. Contrary to the adult literature, the prevalence of joint hypermobility does not differ among children with irritable bowel syndrome, functional abdominal pain, or healthy control children. The presence or severity of joint hypermobility does not correlate with abdominal pain or stooling characteristics. Somatization and depression by child report appear to have a relationship with joint hypermobility.

Joint hypermobility in children: a neglected sign needing more attention.

Joint hypermobility refers to the ability that a joint has to move beyond its normal range of motion. It is common in the general population, particularly in children. While many individuals manifesting joint hypermobility are healthy, this feature can accompany a wide range of symptoms and systemic disorders, whose management can be influenced by their prompt recognition. Given the increasing attention that joint hypermobility is attracting in various fields of medicine, many practitioners are asked to approach more carefully joint hypermobility, in order to avoid over- and under-diagnosis of related disorders. Among the most common conditions featuring joint hypermobility there are hypermobility spectrum disorders and hereditary connective tissue disorders, in particular, the Ehlers-Danlos syndromes. In children, joint hypermobility also accompany a variety of disorders affecting neurodevelopment. The nature of such an association is protean, as joint hypermobility may occur in selected congenital neuromuscular disorders, monogenic multiple malformation/intellectual disability syndromes, and well-known and emerging genomic syndromes. In addition, joint hypermobility seems strongly associated with developmental coordination disorders. This review offers an overview on definitions, assessment procedures, patterns of associated manifestations and disorders related to joint hypermobility, as well as treatment principles of associated musculoskeletal pain for practitioners that are not familial with this issue but encounter people featuring this physical attribute in their daily activity.

Functional Gastrointestinal Disorders, Autonomic Nervous System Dysfunction, and Joint Hypermobility in Children: Are They Related?

To evaluate the prevalence of orthostatic intolerance and joint hypermobility in schoolchildren with and without functional gastrointestinal disorders (FGIDs) and to assess autonomic nervous system dysfunction in children with FGIDs and joint hypermobility. Schoolchildren (10-18years) attending public schools from 3 Colombian cities (Cali, Palmira, and Bucaramanga) completed validated questionnaires for FGIDs and underwent testing for hypermobility and autonomic nervous system dysfunction. Heart rate and blood pressure were assessed in recumbency and upright position at regular intervals. The differences in characteristics between schoolchildren with and without FGIDs were compared with a t-test for continuous variables and with a Fisher exact test (2×2 contingency tables) for categorical variables. In total, 155 children with FGIDs were matched with 151 healthy controls. Children with FGIDs had historically significant greater frequency of 10 of 12 symptoms of orthostatic intolerance, no significant difference in any symptoms of orthostatic intolerance during recumbency, significantly greater frequency in 6 of 12 symptoms of orthostatic intolerance during orthostasis, trend toward statistical significance for orthostatic intolerance (P=.0509), and no significant difference in prevalence of orthostatic hypotension (OH) and postural orthostatic tachycardia syndrome (POTS). There was no significant difference in prevalence of orthostatic intolerance, OH, and POTS between those with joint hypermobility and those without. Children with FGIDs have a greater prevalence of symptoms of orthostatic intolerance but were not more likely to have OH and POTS as compared with children without FGIDs. Children with joint hypermobility did not have a greater prevalence of orthostatic intolerance, OH, and POTS.

Prevalence of joint hypermobility in children and adolescents: A systematic review and meta-analysis

Background:The inconsistent results of different studies regarding the prevalence of joint hypermobility (JH) or joint laxity in children and adolescents made us conduct a meta-analysis on the prevalence of JH in this age group.Materials and Methods:We searched electronic databases including Trip, Scopus, Medline, Embase, PubMed, and Google Scholar; some Iranian databases including Iran Medex and Magiran; and Scientific Information Database to find studies in which the prevalence of JH in children and adolescents had been reported since January 1990 to April 2017. In this process, two researchers evaluated the articles separately while they were not aware of each other's method, and they extracted and matched the information.Results:Necessary data of twenty studies (15,097 boys and 6048 girls) were entered into this meta-analysis. The age range in these studies was 3–19 years. According to the meta-analysis conducted on the twenty studies, it was determined that the total prevalence of JH among children and adolescents was 34.1% (95% confidence interval [CI]: 33.3%–34.8%). Based on the results obtained from the studies, a significant heterogeneity (I2 index equals to 99,415 and P ≤ 0.001) was shown, so we used random-effects model; moreover, the overall assessment of studies showed a statistically significant publication bias (P = 0.02). In total, the prevalence in girls was equal to 32.5% (95% CI: 31.4%–33.7%), and in boys, it was equal to 18.1% (95% CI: 17.2%–19.1%).Conclusion:According to this meta-analysis, studies showed high heterogeneity, and the prevalence of JH in children and adolescents around the world was equal to 34.1% (95% CI: 33.3%–34.8%) in total, whereas it was higher in girls and lower in older ages.

Joint hypermobility in children with and without functional constipation.

Background:Previous studies report an association between joint hypermobility (JH), as a hallmark of connective tissue disorder, and autonomic dysfunction, digestive problems, and irritable bowel syndrome. However, its association with functional constipation (FC) has not been evaluated. This study is run and implemented to justify this theme/topic.Materials and Methods:In this case–control study among 200 subjects, 100 were of FC according to the ROME III Criteria (case group) and each child was matched for age and gender with a healthy control that did not meet criteria for FC (control group). The demographic information and JH were assessed and compared in both groups, through a physical examination according to the Beighton score.Results:A total of 200 children with a mean age of 6.2 ± 2.2 years constituted the statistical population. The prevalence of JH was assessed to establish the Beighton score (≥4 was considered JH). There was no significant difference in JH between children with and without FC, odds ratio (OR) 1.13 (95% confidence interval [CI]: 0.65–1.98, P = 0.669). There was no significant difference in terms of gender and age between the two groups (P = 0.887, P = 0.396, respectively). JH was not significantly associated with gender (P = 0.445) while significantly associated with age (P = 0.041). Furthermore, there was no significant association between JH and FC (P = 0.669). Following multivariate logistic regression analysis between the presence of JH as the dependent variable and the measured variables as the independent variables, only age had significant independent predictive values in the development of JH (P = 0.041, OR =0.88 [0.77–1]). The obtained adjusted OR in this study indicated that at each year age increase the JH risk decreased by 12%.Conclusion:Here, it is revealed that the relative frequency of JH in this age range, with and without FC, is not significantly different, and it is not significantly associated with gender while significantly associated with age.

Children with vesicoureteric reflux have joint hypermobility and occasional tenascin XB sequence variants.

To consider alternative mechanisms that give rise to a refluxing ureterovesical junction (UVJ), we hypothesized that children with a common heritable urinary tract defect, vesicoureteric reflux (VUR), may have a defect in the extracellular matrix composition of the UVJ and other tissues that would be revealed by assessment of the peripheral joints. Hypermobile joints can arise from defects in the extracellular matrix within the joint capsule that affect proteins, including tenascin XB (TNXB). We performed an observational study of children with familial and non-familial VUR to determine the prevalence of joint hypermobility, renal scarring, and DNA sequence variants in TNXB. Most children (27/44) exhibited joint hypermobility using the Beighton scoring system. This included 15/26 girls (57.7%) and 12/18 boys (66.7%), which is a significantly higher prevalence for both sexes when compared to population controls (p<0.005). We found no association between joint hypermobility and renal scarring. Seven of 49 children harbored rare pathogenic sequence variants in TNXB, and two also exhibited joint hypermobility. No sequence variants in TNXB were identified in 25/27 children with VUR and joint hypermobility. Due to the observational design of the study, there was missing data for joint hypermobility scores in six children and for dimercaptosuccinic acid (DMSA) scans in 17 children. We observed a high prevalence of VUR and joint hypermobility in children followed within a tertiary care pediatric urology clinic. While mutations in TNXB have been reported in families with VUR and joint hypermobility, we identified only two children with these phenotypes and pathogenic variants in TNXB. We, therefore, speculate that VUR and joint hypermobility may be due to mutations in other extracellular matrix genes.

Comparison of Beighton score and Brighton Criterion in order to diagnosis of joint hypermobility in children

Comparison of Beighton score and Brighton Criterion in order to diagnosis of joint hypermobility in children

Hypermobility in children

Abstract Hypermobility can be a normal variant in children but it is also found in a group of children presenting with musculoskeletal pain. A smaller group of children will have hypermobility associated with a more concerning syndrome. The clinical challenge is to differentiate those children who have isolated hypermobility with no other associations from those with syndromes that have potentially life limiting complications. In this review we will discuss the assessment of and provide a framework for classifying those children who are found to be hypermobile. We review the components of connective tissue and describe where known the genetic basis for particular phenotypes. In 2017 the international classification of Ehlers Danlos (EDS) was published which supersedes the Villefranche classification. This review describes the diagnostic criteria for the common subtypes of EDS, provides information on further differentials and discusses the management options.

Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children

ObjectiveBenign joint hypermobility (BJH) is a clinical condition characterized by an increased ability of joints during passive and dynamic movements. In this study a new diagnostic criteria for generalized hypermobility in children has been proposed and compared with the Beighton criteria. MethodTwo hundred children from 3 to 16 years of age were included. 100 children with BJH (according to Beighton criteria) were compared with and equal number of age and sex matched controls. The children were separately examined by two qualified pediatric rheumatologists and the Beighton score for hypermobility was calculated twice. Then examinations were performed based on the new criteria and the questionnaires were filled out. ResultsData analysis revealed significant correlation between Beighton and the new criteria in which sensitivity, specificity, positive and negative predictive values were 100%, 98%, 100%, 98%, respectively. Accuracy was 99% and balanced accuracy was 99%. The area under the ROC curve was 0.99. ConclusionOn the basis of pediatrics physiological and mechanical characteristics, it appears that the Shiari–Javadi criteria are able to detect hypermobile children more accurately. Also, the new criteria appears to be more practical, faster and easier.