Protein C Levels in Patients With Legg-Calve-Perthes Disease

Mehta, J. S.; Conybeare, M. E.; Hinves, B. L.; Winter, Jb

doi:10.1097/01.bpo.0000194698.21645.37

Cited by 9 publications

(5 citation statements)

References 30 publications

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“…No significant differences between patients and controls were found in baseline coagulation tests, antithrombin activity, protein C activity, free protein S antigen levels and antiphospholid antibodies. 4,[6][7][8]38,41,[44][45][46][47][48] In the systematic review of the literature summarised in Table II, it was shown that the prevalence of thrombophilic polymorphisms 677T-MTHFR and FIIG20210A were equally distributed among the patients and control groups. Of a total of 475 patients tested for FVL in the cited studies, 31 were heterozygous and five were homozygous for FVL (36 patients; 7.5%) compared with 23 heterozygous FVL cases of 953 tested controls (2.4%) (Mantel-Haenszel test; p = 0.00029).…”

Section: Discussionmentioning

confidence: 99%

Perthes’ disease and the search for genetic associations

Kenet

Ezra²,

Wientroub³

et al. 2008

The Journal of Bone and Joint Surgery. British volume

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The role of heritable thrombophilic risk factors in the pathogenesis of the Perthes' disease is controversial. The clinical and radiological findings of Perthes' disease may be indistinguishable from those of Gaucher's disease, and the most common Jewish N370S Gaucher mutation is threefold greater in patients with Perthes' disease. Familial osteonecrosis of the femoral head is associated with variant mutations of collagen type II (COL2A1 mutations). We therefore studied the potential role of genetic thrombophilia and the Gaucher and COL2A1 mutations in children with Perthes' disease. Genomic DNA of 119 children with radiologically-confirmed Perthes' disease diagnosed between 1986 and 2005 was analysed for the thrombophilic polymorphisms Factor V Leiden, 677T-MTHFR and FIIG20210A. The results were compared with those of a group of 276 children without Perthes' disease. DNA was also analysed for the Gaucher mutations N370S, G insertion (84GG), L444P, Intron 2 (IVS2+1G>A) and R496H. Enzymic assays confirmed the Gaucher disease status. Collagen (COL2A1) mutations of the 12q13 gene were also analysed. The prevalence of thrombophilic markers was similar among the 119 patients with Perthes' disease and the 276 control subjects. The prevalence of the Gaucher mutation was consistent with Israeli population carriership data and did not confirm an earlier-claimed association with Perthes' disease. All 199 patients were negative for the studied COL2A1 mutations. We found no genetic association between Perthes' disease and either Gaucher's disease or COL2A1 mutations or increased genetic thrombophilia among our patients compared with the control group. A systematic review of case-control studies suggested that there was a positive association between Perthes' disease and Factor V Leiden. The impact of this association upon the disease, although not consistent across the studies, remains unclear.

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Section: Discussionmentioning

confidence: 99%

Perthes’ disease and the search for genetic associations

Kenet

Ezra²,

Wientroub³

et al. 2008

The Journal of Bone and Joint Surgery. British volume

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“…Other authors have found reduced protein C activity in non-traumatic ON (Zalavras et al 2000). In Perthes' disease, discordant results have been reported for protein C: some authors have not found any changes (Almeida Matos 2008) while others have found reduced levels (Mehta et al 2006). …”

Section: Discussionmentioning

confidence: 92%

Idiopathic and secondary osteonecrosis of the femoral head show different thrombophilic changes and normal or higher levels of platelet growth factors

et al. 2011

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Background and purpose Thrombophilia represents a risk factor both for idiopathic and secondary osteonecrosis (ON). We evaluated whether clotting changes in idiopathic ON were different from corticosteroid-associated ON. As platelet-rich plasma has been proposed as an adjuvant in surgery, we also assessed whether platelet and serum growth factors were similar to those in healthy subjects.Methods 18 patients with idiopathic ON and 18 with corticosteroid-associated ON were compared with 44 controls for acquired and inherited thrombophilia. Platelet factor 4 (PF4), transforming growth factor-β1, platelet-derived growth factor-BB (PDGF-BB), and vascular endothelial growth factor were assayed in the supernatants of thrombin-activated platelets, in platelet lysates, and in serum from 14 ON patients and 10 controls.Results Idiopathic ON patients had higher plasminogen levels (median 118%) than controls (101%) (p = 0.02). Those with corticosteroid-associated ON had significantly higher D-dimer (333 ng/mL) and lower protein C levels (129%) than controls (164 ng/mL, p = 0.004; 160%, p = 0.02). The frequency of inherited thrombophilia was not different from the controls. No statistically significant differences were found between idiopathic and corticosteroid-associated ON. 20 of the 36 ON patients were smokers. (The controls were selected from smokers because nicotine favors hypercoagulability). ON patients had significantly higher serum PF4 levels (7,383 IU/mL) and PDGF-BB levels (3.1 ng/mL) than controls (4,697 IU/mL, p = 0.005; 2.2 ng/mL, p = 0.02).Interpretation Acquired hypercoagulability was common in both ON types, but the specific changes varied. The release of GF from platelets was not affected, providing a biological basis for platelet-rich plasma being used as an adjuvant in surgical treatment.

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“…The tendency to thrombosis is one of the possible causes of Perthes disease that disputes mainly focus on, and some studies have demonstrated that the disease is associated with abnormalities in blood coagulation factors [2][3][4][5][6][7][8][9][10], whereas others do not [11][12][13][14][15][16]. The deficiency of protein C has been considered a possible etiological factor of Perthes disease [17,18]. However, the meta-analysis of Matos M.A.…”

Section: Introductionmentioning

confidence: 99%

Can large doses of glucocorticoids lead to Perthes? a case report and review of the literature

Chen

Zhang

et al. 2021

BMC Pediatr

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Background Perthes disease (Legg-Calvé-Perthes, LCP) is a self-limited and non-systemic disease occurring in the femoral heads of children, which is mainly manifested as an ischemic necrosis of the femoral head epiphysis, leading to subchondral ossification injury of the femoral head. Case presentation Here we report a case of 11-year-old child with long-term use of high-dose glucocorticoids. With MRI examination finding the epiphyseal necrosis of right humeral head, femur and tibia, and X-ray examination finding bilateral femoral head necrosis, the child was diagnosed as Perthes disease based on his clinical and imaging data. Conclusions Long-term and high-dose glucocorticoids may be one of the causes of Perthes disease.

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Protein C Levels in Patients With Legg-Calve-Perthes Disease

Cited by 9 publications

References 30 publications

Perthes’ disease and the search for genetic associations

Perthes’ disease and the search for genetic associations

Idiopathic and secondary osteonecrosis of the femoral head show different thrombophilic changes and normal or higher levels of platelet growth factors

Can large doses of glucocorticoids lead to Perthes? a case report and review of the literature

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