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. 2024 Mar 14;11(3):344.
doi: 10.3390/children11030344.

Generalized Joint Hypermobility: A Statistical Analysis Identifies Non-Axial Involvement in Most Cases

Mateus Marino Lamari  1 , Neuseli Marino Lamari  2 , Michael Peres de Medeiros  2 , Matheus Gomes Giacomini  3 , Adriana Barbosa Santos  4 , Gerardo Maria de Araújo Filho  2 , Eny Maria Goloni-Bertollo  5 , Érika Cristina Pavarino  5
Affiliations

Generalized Joint Hypermobility: A Statistical Analysis Identifies Non-Axial Involvement in Most Cases

Mateus Marino Lamari et al. Children (Basel). .

Abstract

Context: Joint hypermobility (JH) represents the extreme of the normal range of motion or a condition for a group of genetically determined connective tissue disorders. Generalized joint hypermobility (GJH) is suspected when present in all four limbs and the axial skeleton, scored in prepubescent children and adolescents by a Beighton Score (BS) ≥ 6. Parameters are also used to identify GJH in hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSDs). The purpose of this study is to characterize children with JH based on the location of variables in the BS ≥ 6 and identify children with JH in the axial skeleton, upper limbs (ULs), and lower limbs (LLs) simultaneously.

Methods: We analyzed 124 medical records of one- to nine-year-old children with JH by BS.

Results: The characterization of GJH by combinations of the axial skeleton, ULs, and LLs simultaneously totaled 25.7%. BS = 6 and BS = 8 consisted of variables located in ULs and LLs. BS = 7 included the axial skeleton, ULs, and LLs. BS ≥ 6 represents the majority of the sample and predominantly girls.

Conclusions: BS ≥ 6 represents the majority of the sample and predominantly girls. Most characterized children with GJH present BS = 6 and BS = 8 with variables located only in ULs and LLs, a condition that does not imply the feature is generalized. In children, BS = 7 and BS = 9 characterize GJH by including the axial skeleton, ULs, and LLs. These results draw attention to the implications for defining the diagnosis of hEDS and HSDs.

Keywords: Ehlers–Danlos syndrome; child; hypermobility spectrum disorders; joint hypermobility; pediatrics.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Illustration of five variables for assessment of GJH: 1. 5th finger hyperextension is defined by passive extension with an angle > 90°; 2. wrist hyperflexion is defined by passive thumb touch to the forearm flexor region; 3. elbow hyperextension is defined by active extension with an angle > 10°; 4. knee hyperextension is defined by active extension with an angle > 10°; 5. anterior spinal column hyperflexion is defined by palms touching the ground with extended lower limbs.
Figure 2
Figure 2
Percentage distribution of Beighton variables A, F, E, K, and ATF in overall sample.
Figure 3
Figure 3
Absolute and percentage distribution BS and variables.
See this image and copyright information in PMC

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