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

• Published in: Children (Basel) Vol. 11; no. 3; p. 344
• Main Authors: Lamari, Mateus Marino, Lamari, Neuseli Marino, de Medeiros, Michael Peres, Giacomini, Matheus Gomes, Santos, Adriana Barbosa, de Araújo Filho, Gerardo Maria, Goloni-Bertollo, Eny Maria, Pavarino, Érika Cristina
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 01-03-2024; MDPI
• Subjects: Age; Analysis; child; Children & youth; Connective tissue; Consortia; Ehlers–Danlos syndrome; Ethnicity; hypermobility spectrum disorders; joint hypermobility; Medical records; Pediatrics; Range of motion; Statistical analysis; Teenagers; Variables; Brazil; Ehlers–Danlos syndrome; joint hypermobility; hypermobility spectrum disorders; pediatrics; child
• ISSN: 2227-9067; 2227-9067
• DOI: 10.3390/children11030344

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