- Email: [email protected]
Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children
INJR-334; No. of Pages 4 indian journal of rheumatology xxx (2016) xxx–xxx
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/injr
Original Article
Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children Vadood Javadi Parvaneh a,b, Reza Shiari a,b,* a
Department of Pediatric Rheumatology and bPediatrics Musculoskeletal Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
article info
abstract
Article history:
Objective: Benign joint hypermobility (BJH) is a clinical condition characterized by an in-
Received 23 May 2015
creased ability of joints during passive and dynamic movements. In this study a new
Accepted 22 March 2016
diagnostic criteria for generalized hypermobility in children has been proposed and com-
Available online xxx
pared with the Beighton criteria. Method: Two hundred children from 3 to 16 years of age were included. 100 children with BJH
Keywords:
(according to Beighton criteria) were compared with and equal number of age and sex
Benign joint hypermobility
matched controls. The children were separately examined by two qualified pediatric
Beighton criteria
rheumatologists and the Beighton score for hypermobility was calculated twice. Then
New criteria
examinations were performed based on the new criteria and the questionnaires were
Diagnosis
filled out.
Hypermobility
Results: Data 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. Conclusion: On the basis of pediatrics physiological and mechanical characteristics, it appears that the Shiari–Javadi criteria are able to detect hypermobile children more accurate. Also, the new criteria appears to be more practical, faster and easier. # 2016 Published by Elsevier B.V. on behalf of Indian Rheumatology Association.
1.
Introduction
The prevalence of benign joint hypermobility syndrome (BJHS) is different in various population.1–8 Although, there is no consensus about the prevalence of joint hypermobility in children, it has been estimated around 10–20% in community.9,10
Hypermobility is a clinical condition characterized by an increased ability of joints during passive and dynamic movements. It is more common in female gender, Asian and African descent.11 One of the most common criteria for the evaluation of generalized joint hypermobility is Beighton's criteria.12 These criteria have several disadvantages. For example, Beighton criteria were designed for all ages not specifically for children group (according to the children physiological and
* Corresponding author. E-mail addresses: [email protected], [email protected] (R. Shiari). http://dx.doi.org/10.1016/j.injr.2016.03.009 0973-3698/# 2016 Published by Elsevier B.V. on behalf of Indian Rheumatology Association.
Please cite this article in press as: Javadi Parvaneh V, Shiari R. Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children, Indian J Rheumatol. (2016), http://dx.doi.org/10.1016/j.injr.2016.03.009
INJR-334; No. of Pages 4
2
indian journal of rheumatology xxx (2016) xxx–xxx
growth characteristics).12 Inability to identify limited hypermobility,11 failure to differentiate generalized joint hypermobility from joint hypermobility syndrome and BJHS,10,13 and the restrictions on the use of epidemiological studies are further limitations.14 The revised Brighton 1998 criteria are another criteria applied. These criteria have competence in detection of mono or pauciarticular hypermobility and the ability to differentiate BJHS from generalized joint hypermobility.13,15 The need for clinically trained examiner make some difficulties in its application in epidemiological studies. The Del Mar Hospital criteria17 and the Rotès–Quérol criteria18 are the other criterias that are used less frequently. Other criteria that are self-reported questionnaire, the Hakim–Graham's five-item self-reported questionnaire14 and illustrated Del Mar Hospital's questionnaire.15 Screening individuals with localized or diffuse musculoskeletal complaints, without inflammatory or degenerative disease are the advantage of these questionnaires.14,15 In addition, these questionnaires, with acceptable sensitivity, have applicability in epidemiological studies with no need for examiners. However, it still suffers from inability to diagnose mono or pauciarticular hypermobility, and administrative problems persist. In addition, these criteria are not highly specific and their usage have been limited for screening and epidemiologic studies.14,15 Owing to the disadvantages of the existing criteria and the high prevalence of generalized joint hypermobility, we decided to propose further modifications to Beighton criteria for the diagnosis of joint hypermobility.
2.
Method
A case–control study was designed with 200 participants from 3 to 16 years of age with 100 children with BJH (according to Beighton criteria) in case group and 100 age-sex matched children as control group. Cases were selected from outpatients Clinic of Rheumatology and the control group was selected from the emergency department of hospital. The case group consisted of children who had musculoskeletal pain complaints or were suspected to be hypermobile. The Beighton criteria were used as the gold standard, and all of cases fulfilled the Beighton criteria. The exclusion criteria have been described in Table 1. In addition, the participants in the control group should be free of chronic disease or musculoskeletal complaints. For the determination of new criteria we performed literature review on the range of motion in different age and gender pediatric groups, studied the various existing criteria and their differences, and utilized our own clinical observations. Eight maneuvers were set for examination (Table 1). All were bilateral, except the neck hyperextension (Fig. 1). If hypermobility was present on both sides of the body, score of 1 was given. The total score was 8 (Fig. 1). The criteria and their scores are presented in Table 1.
3.
Results
There were 42 (42%) male children in each group. The mean age was 6.8 years. Table 2 compares the results of the new and Beighton criteria in both cases and controls. All cases were
Table 1 – The scores of the proposed new criteria (Shiari– Javadi) for the diagnosis of hypermobility in children. Number 1
2 3 4 5 6 7 8
Maneuvers
Score
Bilateral passive lateral neck rotation in which nose crosses imaginary line down the frontal appendage of acromio-clavicular joint Passive vertical shoulders hyperextension so that elbows touch together from behind Passive back hyperextension ≥ 308 Bilateral passive elbow hyperextension ≥ 108 Bilateral passive 2–5 MCPs hyperextension ≥ 908 Thumbs touch volar aspect of forearm passively Bilateral passive hip internal rotation ≥ 608 Bilateral passive knee hyperextension ≥ 108
1
Total scorea
1 1 1 1 1 1 1 8
a
The hypermobility score will have a maximum of eight-score if all are positive the criteria are fulfilled when 4 score or higher. b Exclusion criteria: lower than three years and over sixteen years of age, multiple fractures or a history of, dislocated optic lens, non-benign musculoskeletal pain and Marfan or Ehlers–Danlos syndrome.
hypermobile, and two of the 100 controls were hypermobile with the new criteria. Based on these results, new proposed criteria had the sensitivity, specificity, positive predictive value and negative predictive value of 100%, 98%, 100% and 98% respectively. Both the accuracy and the balanced accuracy were 99%. ROC curve in Figure shows the relationship between the new proposed criteria and Beighton criteria (Fig. 2). Area under the curve in this study for the new proposed modifications criteria was 99%.
4.
Discussion
BJHS is one of the most common causes of presentations to pediatric rheumatology. Its prevalence has been estimated around 10–20% in community.9,10 The female prevalence is three times more common than males and in Asians and Africans more common than Caucasian. It seems that the prevalence varies by age, gender and race.11 Despite its close relationship with various organ disorders, extensive morbidity and impact on quality of life, its significance and clinical effects are not well known. On the other hand, BJHS is often ignored by pediatricians, internists, orthopedists, and rheumatologists.11 Furthermore, to the best of our knowledge, specific diagnostic criteria have not been provided for children age, yet the criteria are still based on adults. Beighton criteria requires palms flat on the ground with the extended knee, which is a criterion for evaluating axial and hip joints, some children may not be able to perform this because of the difficulty of maneuver for young age. Unlike other maneuvers of criteria, the palm to ground examination should be performed actively and, it should be clearly explained first for children by the examiner. In addition, little attention is paid for the evaluation of the axial skeleton as a key element of the pediatric joints in the Beighton criteria due to the fact that the axial skeleton with hip joint has been just allocated to one point compared to the nine total points. In fact, in many cases
Please cite this article in press as: Javadi Parvaneh V, Shiari R. Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children, Indian J Rheumatol. (2016), http://dx.doi.org/10.1016/j.injr.2016.03.009
INJR-334; No. of Pages 4 indian journal of rheumatology xxx (2016) xxx–xxx
3
Fig. 1 – Eight maneuvers of proposed new criteria (Shiari–Javadi) for pediatric generalized hypermobility.
hypermobility in axial skeleton may be ignored. On the other hand, in the large joints, Beighton criteria scores only knees. Another point worth mentioning is that the Beighton criteria cannot determine whether the child has hypermobility in small, medium, large or axial joints. Finally, the Beighton criteria are not suitable for epidemiological studies due to a need for skilled examiner.14 Due to the problems mentioned in the Beighton criteria and the lack of specific criteria for children, we proposed modifications criteria on the basis of reviewing the literature. In this criterion, we considered the range of motion in different genders and age groups of children, and use our clinical observations to reduce the possible disadvantages of Beighton criteria.19–24 We categorized the joints into three large, medium-small, and axial joints to cover generalized hypermobility. Unlike the Beighton criteria, increased range of motion on both sides of the body is considered as positive hypermobility to take one score. In this scale, in large joints group, all the major joints of the body including shoulders, hips, and knees were taken and each was assessed in a
separate score, while the Beighton criteria have paid little attention to shoulder and hip independently. Special attention was given to the axial joints in the new criteria since high prevalence of its hypermobility in this study and also easily examination due to anatomical, height and body condition in pediatrics. Also the neck and back have been separately rated in new criteria. In the case of small and medium-sized joints, unlike Beighton criteria, hyperextension of all second to fifth fingers on both sides were considered which led to greater joint
Table 2 – The result of the proposed new criteria (Shiari– Javadi) in both cases and control groups. According to the new criteria is hypermobile
According to the new criteria is not hypermobile
Total
Case group Control group
100 (100%) 2 (2%)
0 98 (98%)
100 100
Total
102
98
200
Fig. 2 – ROC curve of the relationship between the proposed new criteria (Shiari–Javadi) and the Beighton criteria.
Please cite this article in press as: Javadi Parvaneh V, Shiari R. Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children, Indian J Rheumatol. (2016), http://dx.doi.org/10.1016/j.injr.2016.03.009
INJR-334; No. of Pages 4
4
indian journal of rheumatology xxx (2016) xxx–xxx
examination and a more generalized perception. Like most of the other criteria, the examination of toe joints was omitted due to difficulty of examination. Also, there are considerable differences between normal range of motion of the fingers and apart from the first finger, the examination of the other fingers should be performed separately which does not fit the scope of the examiner and scoring; and the range of motion reference's for children is limited.19–24 In Beighton criteria in terms of the number of joints, a total of 21 joint are examined, while in this proposed criteria, 35 joints are evaluated. Another interesting point in the new criteria is that all maneuvers were done passively and therefore the examination is easy for examiner and the children. The criteria have limitation in epidemiological studies because there are still the need for training medical interns and residents. The proposed modifications criteria for hypermobility in children have a high diagnostic power (area under the ROC curve: 0.990) with a sensitivity, specificity, positive predictive value and negative predictive value 100%, 98%, 100% and 98%, respectively, in the population under investigation. The accuracy was calculated as 99.5% and the balanced accuracy was 99.5%. For determining the new criteria's cut-off point, the point with the highest sensitivity (100%) and a very high specificity (98%) was 4, compared with the Beighton criteria. Among the 100 patients in the control group, two patients were diagnosed hypermobile with the proposed criteria, while they were not recognized by Beighton criteria. It means that there are cases of hypermobility which Beighton criteria fail to determine.
5.
Conclusion
The new proposed criteria (Shiari–Javadi criteria) appears to be useful for detecting hyper mobility. In addition, they have also overcome many of the disadvantages of the Beighton criteria, and are easier, more practical and more comprehensive to be used in children. However, further studies are required to validate this proposed modification of the Beighton criteria.
Conflicts of interest The authors have none to declare.
references
1. Al-Rawi ZS, Al-Rawi ZT. Joint hypermobility in women with genital prolapse. Lancet. 1982;1:1439–1441.
2. Birrell FN, Adebajo AO, Hazleman BL, Silman AJ. High prevalence of joint laxity in West Africans. Br J Rheumatol. 1994;33:56–59. 3. Klemp P, Williams SM, Stansfield SA. Articular mobility in Maori and European New Zealanders. Rheumatology. 2002;41:554–557. 4. Bravo JF, Wolff C. Clinical study of hereditary disorders of connective tissues in a Chilean population: joint hypermobility syndrome and vascular Ehlers–Danlos syndrome. Arthritis Rheum. 2006;54:515–523. 5. Grahame R, Hakim AJ. High prevalence of joint hypermobility syndrome in clinic referrals to a north London community hospital [abstract]. Rheumatology. 2004;43:91. 6. Pountain G. Musculoskeletal pain in Omanis, and the relationship to joint mobility and body mass index. Br J Rheumatol. 1992;31:81–85. 7. Al Rawi ZS, Al Aszawi AJ, Al Chalabi T. Joint mobility among university students in Iraq. Br J Rheumatol. 1985;24:326–331. 8. Clinch J, Deere K, Tobias J, Clarke E. Epidemiology of generalised joint laxity in 14 year old children from the UK: a population based evaluation. Pediatric Rheumatol. 2012;10: A107. 9. Beighton PH, Grahame R, Bird HA, eds. In: Hypermobility of Joints 4th ed. Berlin: Springer; 2012. 10. Ross J, Grahame R. Easily missed? Joint hypermobility syndrome. BMJ. 2011;342:7167. 11. Grahame R, Hakim A. Joint hypermobility. Best Pract Res Clin Rheumatol. 2003;17:989–1004. 12. Beighton P, Solomon L, Soskolnet L. Articular mobility in an African population. Ann Rheum Dis. 1973;32:413–418. 13. Grahame R, Bird HA, Child A, et al. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol. 2000;27:1777–1779. 14. Hakim AJ, Grahame R. A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain. Int J Clin Pract. 2003;57:163–166. 15. Bulbena A, Mallorquí-Bagué N, Pailhez G, et al. Self-reported screening questionnaire for the assessment of Joint Hypermobility Syndrome (SQ-CH): a collagen condition, in Spanish population. Eur J Psychiatr. 2014;28:17–26. 17. Bulbena A, Duro JC, Mateo A, Porta M, Vallejo J. Anxiety disorders in the joint hypermobility syndrome. Lancet. 1988;2:694. 18. Rotes-Querol J. Articular laxity considered as factor of changes of the locomotor apparatus. Rev Rhum Mal Osteoartic. 1957;24:535–539. 19. Cynthia C, Norkin D, Joyce W. Measurement of Joint Motion, A Guide to Goniometry. 4th ed. FA Davis Company; 2009. 20. www.Cdc.gov/ncbddd/jointrom. 21. American Academy of Orthopedic Surgeons. Joint Motion, Method of Measuring and Recording. Chicago: AAOS; 1965. 22. Cocchiarella L, Andersson GBI. American Medical Association: Guides to the Evaluation of Permanent Impairment. 5th ed. AMA: Chicago; 2001. 23. http://web.mit.edu. Stretching & Flexibility. 24. In: www.assh.org/Public/Hand Anatomy/Anatomy/Pages/ Normal-Range–Motion.aspx.
Please cite this article in press as: Javadi Parvaneh V, Shiari R. Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children, Indian J Rheumatol. (2016), http://dx.doi.org/10.1016/j.injr.2016.03.009
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/injr
Original Article
Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children Vadood Javadi Parvaneh a,b, Reza Shiari a,b,* a
Department of Pediatric Rheumatology and bPediatrics Musculoskeletal Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
article info
abstract
Article history:
Objective: Benign joint hypermobility (BJH) is a clinical condition characterized by an in-
Received 23 May 2015
creased ability of joints during passive and dynamic movements. In this study a new
Accepted 22 March 2016
diagnostic criteria for generalized hypermobility in children has been proposed and com-
Available online xxx
pared with the Beighton criteria. Method: Two hundred children from 3 to 16 years of age were included. 100 children with BJH
Keywords:
(according to Beighton criteria) were compared with and equal number of age and sex
Benign joint hypermobility
matched controls. The children were separately examined by two qualified pediatric
Beighton criteria
rheumatologists and the Beighton score for hypermobility was calculated twice. Then
New criteria
examinations were performed based on the new criteria and the questionnaires were
Diagnosis
filled out.
Hypermobility
Results: Data 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. Conclusion: On the basis of pediatrics physiological and mechanical characteristics, it appears that the Shiari–Javadi criteria are able to detect hypermobile children more accurate. Also, the new criteria appears to be more practical, faster and easier. # 2016 Published by Elsevier B.V. on behalf of Indian Rheumatology Association.
1.
Introduction
The prevalence of benign joint hypermobility syndrome (BJHS) is different in various population.1–8 Although, there is no consensus about the prevalence of joint hypermobility in children, it has been estimated around 10–20% in community.9,10
Hypermobility is a clinical condition characterized by an increased ability of joints during passive and dynamic movements. It is more common in female gender, Asian and African descent.11 One of the most common criteria for the evaluation of generalized joint hypermobility is Beighton's criteria.12 These criteria have several disadvantages. For example, Beighton criteria were designed for all ages not specifically for children group (according to the children physiological and
* Corresponding author. E-mail addresses: [email protected], [email protected] (R. Shiari). http://dx.doi.org/10.1016/j.injr.2016.03.009 0973-3698/# 2016 Published by Elsevier B.V. on behalf of Indian Rheumatology Association.
Please cite this article in press as: Javadi Parvaneh V, Shiari R. Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children, Indian J Rheumatol. (2016), http://dx.doi.org/10.1016/j.injr.2016.03.009
INJR-334; No. of Pages 4
2
indian journal of rheumatology xxx (2016) xxx–xxx
growth characteristics).12 Inability to identify limited hypermobility,11 failure to differentiate generalized joint hypermobility from joint hypermobility syndrome and BJHS,10,13 and the restrictions on the use of epidemiological studies are further limitations.14 The revised Brighton 1998 criteria are another criteria applied. These criteria have competence in detection of mono or pauciarticular hypermobility and the ability to differentiate BJHS from generalized joint hypermobility.13,15 The need for clinically trained examiner make some difficulties in its application in epidemiological studies. The Del Mar Hospital criteria17 and the Rotès–Quérol criteria18 are the other criterias that are used less frequently. Other criteria that are self-reported questionnaire, the Hakim–Graham's five-item self-reported questionnaire14 and illustrated Del Mar Hospital's questionnaire.15 Screening individuals with localized or diffuse musculoskeletal complaints, without inflammatory or degenerative disease are the advantage of these questionnaires.14,15 In addition, these questionnaires, with acceptable sensitivity, have applicability in epidemiological studies with no need for examiners. However, it still suffers from inability to diagnose mono or pauciarticular hypermobility, and administrative problems persist. In addition, these criteria are not highly specific and their usage have been limited for screening and epidemiologic studies.14,15 Owing to the disadvantages of the existing criteria and the high prevalence of generalized joint hypermobility, we decided to propose further modifications to Beighton criteria for the diagnosis of joint hypermobility.
2.
Method
A case–control study was designed with 200 participants from 3 to 16 years of age with 100 children with BJH (according to Beighton criteria) in case group and 100 age-sex matched children as control group. Cases were selected from outpatients Clinic of Rheumatology and the control group was selected from the emergency department of hospital. The case group consisted of children who had musculoskeletal pain complaints or were suspected to be hypermobile. The Beighton criteria were used as the gold standard, and all of cases fulfilled the Beighton criteria. The exclusion criteria have been described in Table 1. In addition, the participants in the control group should be free of chronic disease or musculoskeletal complaints. For the determination of new criteria we performed literature review on the range of motion in different age and gender pediatric groups, studied the various existing criteria and their differences, and utilized our own clinical observations. Eight maneuvers were set for examination (Table 1). All were bilateral, except the neck hyperextension (Fig. 1). If hypermobility was present on both sides of the body, score of 1 was given. The total score was 8 (Fig. 1). The criteria and their scores are presented in Table 1.
3.
Results
There were 42 (42%) male children in each group. The mean age was 6.8 years. Table 2 compares the results of the new and Beighton criteria in both cases and controls. All cases were
Table 1 – The scores of the proposed new criteria (Shiari– Javadi) for the diagnosis of hypermobility in children. Number 1
2 3 4 5 6 7 8
Maneuvers
Score
Bilateral passive lateral neck rotation in which nose crosses imaginary line down the frontal appendage of acromio-clavicular joint Passive vertical shoulders hyperextension so that elbows touch together from behind Passive back hyperextension ≥ 308 Bilateral passive elbow hyperextension ≥ 108 Bilateral passive 2–5 MCPs hyperextension ≥ 908 Thumbs touch volar aspect of forearm passively Bilateral passive hip internal rotation ≥ 608 Bilateral passive knee hyperextension ≥ 108
1
Total scorea
1 1 1 1 1 1 1 8
a
The hypermobility score will have a maximum of eight-score if all are positive the criteria are fulfilled when 4 score or higher. b Exclusion criteria: lower than three years and over sixteen years of age, multiple fractures or a history of, dislocated optic lens, non-benign musculoskeletal pain and Marfan or Ehlers–Danlos syndrome.
hypermobile, and two of the 100 controls were hypermobile with the new criteria. Based on these results, new proposed criteria had the sensitivity, specificity, positive predictive value and negative predictive value of 100%, 98%, 100% and 98% respectively. Both the accuracy and the balanced accuracy were 99%. ROC curve in Figure shows the relationship between the new proposed criteria and Beighton criteria (Fig. 2). Area under the curve in this study for the new proposed modifications criteria was 99%.
4.
Discussion
BJHS is one of the most common causes of presentations to pediatric rheumatology. Its prevalence has been estimated around 10–20% in community.9,10 The female prevalence is three times more common than males and in Asians and Africans more common than Caucasian. It seems that the prevalence varies by age, gender and race.11 Despite its close relationship with various organ disorders, extensive morbidity and impact on quality of life, its significance and clinical effects are not well known. On the other hand, BJHS is often ignored by pediatricians, internists, orthopedists, and rheumatologists.11 Furthermore, to the best of our knowledge, specific diagnostic criteria have not been provided for children age, yet the criteria are still based on adults. Beighton criteria requires palms flat on the ground with the extended knee, which is a criterion for evaluating axial and hip joints, some children may not be able to perform this because of the difficulty of maneuver for young age. Unlike other maneuvers of criteria, the palm to ground examination should be performed actively and, it should be clearly explained first for children by the examiner. In addition, little attention is paid for the evaluation of the axial skeleton as a key element of the pediatric joints in the Beighton criteria due to the fact that the axial skeleton with hip joint has been just allocated to one point compared to the nine total points. In fact, in many cases
Please cite this article in press as: Javadi Parvaneh V, Shiari R. Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children, Indian J Rheumatol. (2016), http://dx.doi.org/10.1016/j.injr.2016.03.009
INJR-334; No. of Pages 4 indian journal of rheumatology xxx (2016) xxx–xxx
3
Fig. 1 – Eight maneuvers of proposed new criteria (Shiari–Javadi) for pediatric generalized hypermobility.
hypermobility in axial skeleton may be ignored. On the other hand, in the large joints, Beighton criteria scores only knees. Another point worth mentioning is that the Beighton criteria cannot determine whether the child has hypermobility in small, medium, large or axial joints. Finally, the Beighton criteria are not suitable for epidemiological studies due to a need for skilled examiner.14 Due to the problems mentioned in the Beighton criteria and the lack of specific criteria for children, we proposed modifications criteria on the basis of reviewing the literature. In this criterion, we considered the range of motion in different genders and age groups of children, and use our clinical observations to reduce the possible disadvantages of Beighton criteria.19–24 We categorized the joints into three large, medium-small, and axial joints to cover generalized hypermobility. Unlike the Beighton criteria, increased range of motion on both sides of the body is considered as positive hypermobility to take one score. In this scale, in large joints group, all the major joints of the body including shoulders, hips, and knees were taken and each was assessed in a
separate score, while the Beighton criteria have paid little attention to shoulder and hip independently. Special attention was given to the axial joints in the new criteria since high prevalence of its hypermobility in this study and also easily examination due to anatomical, height and body condition in pediatrics. Also the neck and back have been separately rated in new criteria. In the case of small and medium-sized joints, unlike Beighton criteria, hyperextension of all second to fifth fingers on both sides were considered which led to greater joint
Table 2 – The result of the proposed new criteria (Shiari– Javadi) in both cases and control groups. According to the new criteria is hypermobile
According to the new criteria is not hypermobile
Total
Case group Control group
100 (100%) 2 (2%)
0 98 (98%)
100 100
Total
102
98
200
Fig. 2 – ROC curve of the relationship between the proposed new criteria (Shiari–Javadi) and the Beighton criteria.
Please cite this article in press as: Javadi Parvaneh V, Shiari R. Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children, Indian J Rheumatol. (2016), http://dx.doi.org/10.1016/j.injr.2016.03.009
INJR-334; No. of Pages 4
4
indian journal of rheumatology xxx (2016) xxx–xxx
examination and a more generalized perception. Like most of the other criteria, the examination of toe joints was omitted due to difficulty of examination. Also, there are considerable differences between normal range of motion of the fingers and apart from the first finger, the examination of the other fingers should be performed separately which does not fit the scope of the examiner and scoring; and the range of motion reference's for children is limited.19–24 In Beighton criteria in terms of the number of joints, a total of 21 joint are examined, while in this proposed criteria, 35 joints are evaluated. Another interesting point in the new criteria is that all maneuvers were done passively and therefore the examination is easy for examiner and the children. The criteria have limitation in epidemiological studies because there are still the need for training medical interns and residents. The proposed modifications criteria for hypermobility in children have a high diagnostic power (area under the ROC curve: 0.990) with a sensitivity, specificity, positive predictive value and negative predictive value 100%, 98%, 100% and 98%, respectively, in the population under investigation. The accuracy was calculated as 99.5% and the balanced accuracy was 99.5%. For determining the new criteria's cut-off point, the point with the highest sensitivity (100%) and a very high specificity (98%) was 4, compared with the Beighton criteria. Among the 100 patients in the control group, two patients were diagnosed hypermobile with the proposed criteria, while they were not recognized by Beighton criteria. It means that there are cases of hypermobility which Beighton criteria fail to determine.
5.
Conclusion
The new proposed criteria (Shiari–Javadi criteria) appears to be useful for detecting hyper mobility. In addition, they have also overcome many of the disadvantages of the Beighton criteria, and are easier, more practical and more comprehensive to be used in children. However, further studies are required to validate this proposed modification of the Beighton criteria.
Conflicts of interest The authors have none to declare.
references
1. Al-Rawi ZS, Al-Rawi ZT. Joint hypermobility in women with genital prolapse. Lancet. 1982;1:1439–1441.
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Please cite this article in press as: Javadi Parvaneh V, Shiari R. Proposed modifications to Beighton criteria for the diagnosis of joint hypermobility in children, Indian J Rheumatol. (2016), http://dx.doi.org/10.1016/j.injr.2016.03.009