Łamliwość kości w EDS6a

Niestety pacjenci z typem VI jak zwykle są bardziej narażeni na wszystko, także na złamania. To wyjaśnia, dlaczego po latach znajduje się w jakimś przypadkowym badaniu złamania, o których nie mieliśmy pojęcia. 

"Recent findings: Low bone mineral density and fractures seem to be frequent in some of the rare EDS types (kyphoscoliotic, arthrochalasia, spondylodysplastic, and classic-like EDS). For the more prevalent hypermobile and classic EDS types, some case-control studies found mildly decreased bone mineral density, but it was not clear that fracture rates were increased. Nevertheless, abnormalities in vertebral shape seem to be common in classical and hypermobile EDS types. In a cohort of individuals with EDS followed since birth, no fractures were observed during infancy. Bone mineral density varies widely among the different types of EDS, and vertebral abnormalities seem to be common in classical and hypermobile EDS. It might be justified to perform spine radiographs and bone mineral density assessments in newly diagnosed EDS".

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Prosthetic Dislocation After Total Hip Arthroplasty

Abstract

Introduction:

Ehlers-Danlos syndromes (EDS) are genetic connective tissue disorders affecting multiple organ systems that frequently result in connective tissue hyperlaxity and early osteoarthritis. Short- and long-term outcomes after total hip arthroplasty in this cohort remain poorly characterized.
Methods:

Data were abstracted from the 2010 to 2018 PearlDiver Mariner administrative database. Patients with EDS undergoing total hip arthroplasty for osteoarthritis were identified and matched 1:10 to patients without a diagnosis of EDS. Ninety-day incidence of postoperative complications and hospital readmission were identified and compared. Odds of 90-day adverse events were compared using multivariate logistic regression. Finally, 5-year implant survival was compared using a log-rank test. Significance was set at P < 0.005 after Bonferroni correction.
Results:

Matching was done for 354 patients with EDS to 3,518 patients without EDS. Patients with EDS had markedly higher rates of periprosthetic dislocation within 90 days of surgery (4.2% dislocation rate in EDS vs. 1.7% in matched control subjects, P = 0.001). On multivariate analysis, patients with EDS continued to have increased odds of dislocation in the 90-day postoperative period (Odds Ratio=2.64, P = 0.001), whereas the rates of the other studied adverse events were not markedly different between groups. At 5 years, 92.7% of patients with EDS and 96.1% of the matched control subjects remained unrevised (significant log rank difference, P = 0.004).
Conclusion:

Compared with patients without EDS, patients with EDS were found to have a higher rate of perioperative dislocation and markedly lower implant survival at 5 years. These findings are consistent with the increased connective tissue laxity associated with EDS.

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