Background. The Hypermobyle syndrome (HS) has an incidence of 9%-16%. The collagen defect produces fragile skin (and so it causes atrophic scars), hypermobile joints (and so they cause luxations) and mitral prolapse (and so it causes rigurgitation). HS is divided into three stages with progressive clinical importance. Aim. This study reserches and desccribes the diagnostic findings useful to stage the clinical importance of HS from chidhood to adulthood. Methods Skin, joints and mitral valve of 48 subjects, aged 6-9 years, with HS (24 males and 24 females) were studied by the macrophotography, the Beighton scoring system (Bss), the range of motion (ROM) and the echocardigraphy. Results Scars appered in 54,16% of cases, luxations in 22,91% of cases and prolapse in 34,41% of cases. The I stage of the HS arose in 52,08333 %, the II stage in 33,3333 % and the III stage in 14,5833% of patients. Spearman test between Bss and ROM values showed high sognificance (< 0,0001%). Discussion and conclusions. The diagnosis of the HS in children can employ the Bss and the ROM tests (major criteria) and the appearance of the scars, luxations and of the mitral prolapse (minor criteria).
La sindroma di Ehlers-Danlos tipo III (sindrome ipermobile): i danni alla cute, alle articolazioni ed al cuore in uno studio su 48 bambini
IURASSICH, Stefano;
2008
Abstract
Background. The Hypermobyle syndrome (HS) has an incidence of 9%-16%. The collagen defect produces fragile skin (and so it causes atrophic scars), hypermobile joints (and so they cause luxations) and mitral prolapse (and so it causes rigurgitation). HS is divided into three stages with progressive clinical importance. Aim. This study reserches and desccribes the diagnostic findings useful to stage the clinical importance of HS from chidhood to adulthood. Methods Skin, joints and mitral valve of 48 subjects, aged 6-9 years, with HS (24 males and 24 females) were studied by the macrophotography, the Beighton scoring system (Bss), the range of motion (ROM) and the echocardigraphy. Results Scars appered in 54,16% of cases, luxations in 22,91% of cases and prolapse in 34,41% of cases. The I stage of the HS arose in 52,08333 %, the II stage in 33,3333 % and the III stage in 14,5833% of patients. Spearman test between Bss and ROM values showed high sognificance (< 0,0001%). Discussion and conclusions. The diagnosis of the HS in children can employ the Bss and the ROM tests (major criteria) and the appearance of the scars, luxations and of the mitral prolapse (minor criteria).I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
