Bone Density Status in Bleeding Disorders: Where Are We and What Needs to Be Done?

Mansouritorghabeh, Hassan; Rezaieyazdi, Zahra

doi:10.11005/jbm.2017.24.4.201

Cited by 9 publications

(13 citation statements)

References 54 publications

(46 reference statements)

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“…Hence, they encounter unusual bleeding, either spontaneous or after trauma and surgery. The spectrum of bleeding manifestations varies from superficial ecchymosis to lethal hemorrhage in the central nervous system [ 97 , 98 ]. While the prevalence of hemophilia A varies among different countries, its prevalence has been estimated to be about 3 to 20 per 100,000 in the population.…”

Section: Hematological Rare Disodersmentioning

confidence: 99%

“…While the prevalence of hemophilia A varies among different countries, its prevalence has been estimated to be about 3 to 20 per 100,000 in the population. Hemophilia B is seen as 1 case among 5,000 male births in European countries and the United States [ 97 , 98 ]. Individuals with hemophilia A and B have respectively either reduced or absent levels of coagulation factor VIII and IX.…”

Section: Hematological Rare Disodersmentioning

confidence: 99%

“…Lack or reduced levels of coagulation factors VIII and IX result in impaired clot formation and tendency to bleeding episodes. These lifelong disorders are categorized as severe type (< 1%), medium type (1–5%), and mild type (5–30%) [ 98 ]. Replacement therapy using plasma derived or recombinant coagulation factor concentrates are the basis of treatment for bleeding episodes and prophylaxis regimens [ 99 ].…”

Section: Hematological Rare Disodersmentioning

confidence: 99%

“…Twenty-seven percent of hemophilic patients have osteoporosis and 43% osteopenia [ 92 ]. A large and growing body of literature has investigated osteopenia and osteoporosis both in children and in adults with hemophilia [ 98 , 100 , 101 ]. While physical activity is considered as a central indicator of bone resorption, patients with bleeding disorders are less likely to engage in high impact activities and weight-bearing exercises [ 98 ].…”

Section: Hematological Rare Disodersmentioning

confidence: 99%

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Bone fragility in patients affected by congenital diseases non skeletal in origin

Masi

Ferrari

Javaid

et al. 2021

Orphanet J Rare Dis

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Background Bone tissue represents a large systemic compartment of the human body, with an active metabolism, that controls mineral deposition and removal, and where several factors may play a role. For these reasons, several non-skeletal diseases may influence bone metabolism. It is of a crucial importance to classify these disorders in order to facilitate diagnosis and clinical management. This article reports a taxonomic classification of non-skeletal rare congenital disorders, which have an impact on bone metabolism Methods The International Osteoporosis Foundation (IOF) Skeletal Rare Diseases Working Group (SRD-WG), comprised of basic and clinical scientists, has decided to review the taxonomy of non-skeletal rare disorders that may alter bone physiology. Results The taxonomy of non-skeletal rare congenital disorders which impact bone comprises a total of 6 groups of disorders that may influence the activity of bone cells or the characteristics of bone matrix. Conclusions This paper provides the first comprehensive taxonomy of non-skeletal rare congenital disorders with impact on bone physiology.

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Section: Hematological Rare Disodersmentioning

confidence: 99%

Section: Hematological Rare Disodersmentioning

confidence: 99%

Section: Hematological Rare Disodersmentioning

confidence: 99%

Section: Hematological Rare Disodersmentioning

confidence: 99%

See 2 more Smart Citations

Bone fragility in patients affected by congenital diseases non skeletal in origin

Masi

Ferrari

Javaid

et al. 2021

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…There are discrepancies in dental health and oral hygiene in individuals with CBD [ 29 , 35 ]. The literature reports a frequent occurrence of osteoporosis in patients with hemophilia [ 36 , 37 ], as well as the possibility of complications related to hemorrhages into muscles and joints [ 38 ]. Complications include arthropathy, synovitis, contractures, or the formation of pseudo-tumors within the loco-motor system in the course of hemophilia.…”

Section: Introductionmentioning

confidence: 99%

Practical Recommendations for Treatment of Dental Patients with Congenital Bleeding Disorders during the Covid-19 Pandemic: A Narrative Review

Czajkowska

Rupa‐Matysek

Gil

et al. 2020

IJERPH

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At the end of 2019, a new disease—COVID-19—was identified, and a few months later, the World Health Organization announced a pandemic. It is now known that SARS-CoV-2 is highly contagious and most confirmed infections are mild to moderate. The situation is particularly difficult for dentists due to the high risk of virus transmission in the dental surgery. Complications including, but not limited to, problems with the respiratory and cardiovascular systems have been reported in patients with SARS-CoV-2. Several changes in the coagulation system, such as lower platelet numbers or increased prothrombin time, as well as increased D-dimer and fibrinogen, were observed. This review is intended to systematize the knowledge on the treatment of patients with congenital bleeding disorders (CBD) during the SARS-CoV-2 pandemic. Extensive literature research was conducted into COVID-19 and the general medical and dental treatment of patients with CBD. Case studies, research results and recommendations of international societies were used in the study. The results of this research are presented in the form of recommendations for the treatment of patients with coagulopathies. It should be remembered that the impact of COVID-19 on the health condition of patients with CBD is unknown to date.

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Characterization of Extracellular Vesicles in Osteoporotic Patients Compared to Osteopenic and Healthy Controls

Pepe

Rossi

Battafarano

et al. 2020

Journal of Bone and Mineral Research

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Extracellular vesicles (EVs) are mediators of a range of pathological conditions. However, their role in bone loss disease has not been well understood. In this study we characterized plasma EVs of 54 osteoporotic (OP) postmenopausal women compared to 48 osteopenic (OPN) and 44 healthy controls (CN), and we investigated their effects on osteoclasts and osteoblasts. We found no differences between the three groups in terms of anthropometric measurements and biochemical evaluation of serum calcium, phosphate, creatinine, PTH, 25‐hydroxy vitamin D and bone biomarkers, except for an increase of CTX level in OP group. FACS analysis revealed that OP patients presented a significantly increased number of EVs and RANKL+ EVs compared with both CN and OPN subjects. Total EVs are negatively associated with the lumbar spine T‐score and femoral neck T‐score. Only in the OPN patients we observed a positive association between the total number of EVs and RANKL+ EVs with the serum RANKL. In vitro studies revealed that OP EVs supported osteoclastogenesis of healthy donor peripheral blood mononuclear cells at the same level observed following RANKL and M‐CSF treatment, reduced the ability of mesenchymal stem cells to differentiate into osteoblasts, while inducing an increase of OSTERIX and RANKL expression in mature osteoblasts. The analysis of miRNome revealed that miR‐1246 and miR‐1224‐5p were the most upregulated and downregulated in OP EVs; the modulated EV‐miRNAs in OP and OPN compared to CN are related to osteoclast differentiation, interleukin‐13 production and regulation of canonical WNT pathway. A proteomic comparison between OPN and CN EVs evidenced a decrease in fibrinogen, vitronectin, and clusterin and an increase in coagulation factors and apolipoprotein, which was also upregulated in OP EVs. Interestingly, an increase in RANKL+ EVs and exosomal miR‐1246 was also observed in samples from patients affected by Gorham‐Stout disease, suggesting that EVs could be good candidate as bone loss disease biomarkers. © 2022 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).

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Bone Density Status in Bleeding Disorders: Where Are We and What Needs to Be Done?

Cited by 9 publications

References 54 publications

Bone fragility in patients affected by congenital diseases non skeletal in origin

Bone fragility in patients affected by congenital diseases non skeletal in origin

Practical Recommendations for Treatment of Dental Patients with Congenital Bleeding Disorders during the Covid-19 Pandemic: A Narrative Review

Characterization of Extracellular Vesicles in Osteoporotic Patients Compared to Osteopenic and Healthy Controls

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