A Panoramic X-ray as a Supportive Diagnostic Tool for the Screening of Osteoporosis in Patients with Hemophilia A and B.

BACKGROUND: Hemophilia is associated with an increased risk of developing osteoporosis and osteopenia. The aim of the study was to interpret the usefulness of fractal analysis of the trabecular bone of the mandible (FD) and selected radiomorphic indices (the antegonial index (AI) and Klemetti index (KI)) to assess the risk of reduced bone mineral density (BMD) in patients with hemophilia A/B. METHODS: The study group consisted of 50 patients with type A/B hemophilia. The control group consisted of 25 males without congenital bleeding disorders. The patients had a panoramic radiograph taken with the same X-ray machine (Vistapano S, Durr Dental, Bietigheim-Bissingen, Germany). The AudaXCeph software (Audax d.o.o., Ljubljana, Slovenia) was used to evaluate the AI index, and the ImageJ, software with a specially prepared script, was used to evaluate the FD. The mandibular cortex was assessed distal to the mental foramen using the Klemetti index. RESULTS: There were no statistically significant differences between the AI, KI and FD values between the study group and the control group. CONCLUSIONS: This study indicated the lack of usefulness of AI, KI and fractal analysis in identifying patients with hemophilia at risk of reduced bone mass.

Assessment of Oral Health and Healthy Habits in Adult Patients with Congenital Hemophilia.

OBJECTIVES: The objective of our study was to assess the current knowledge, the oral health status, and the pro-health needs of patients with hemophilia. MATERIALS AND METHODS: The study included 77 patients with known hemophilia. The study included the assessment of dental indicators related to caries incidence (decayed, missing, and filled teeth [DMFT] and decayed, missing, and filled permanent teeth or surfaces [DMFS]), caries treatment (treatment index), oral hygiene status (Approximal Plaque Index [API] and simplified oral hygiene index [OHI-S]), and periodontal status (bleeding on probing [BoP]). A questionnaire addressed symptoms of hemorrhagic diathesis and health-promoting habits. The influence of routine management ("on-demand" vs. prophylactic therapy), type of hemophilia (A, B), and severity of clotting factor deficiency (mild, moderate, or severe) on oral health was assessed. STATISTICAL ANALYSIS: The tests used in the study were Shapiro-Wilk, Mann-Whitney, Kruskal-Wallis, and Dunn's tests. The level of significance was set at p < 0.05. RESULTS: Patients with hemophilia showed a higher prevalence of caries compared with patients without hemorrhagic diathesis (DMFT 14 and 9, and DMFS 30 and 15, respectively), and they also presented a higher bleeding index value during probing and worse effectiveness of hygienic interdental procedures. The type of therapy and secondary prophylaxis used and the type and severity of hemophilia did not affect the oral health status. CONCLUSIONS: Patients with hemophilia have an increased risk of developing dental caries. Undertaking educational activities related to the prevention of oral diseases is necessary to improve oral health.

Transfusion of fibrinogen concentrate before dental extractions in patients with afibrinogenemia: a narrative review supported by a case report with a proposed treatment protocol.

Afibrinogenemia is a coagulation disorder that occurs with a frequency of 1-2 cases/1,000,000 population and is characterized by a lack of capacity to synthesize fibrinogen. The predominant symptoms related to fibrinogen deficiency are mucocutaneous bleeding, bleeding from the gastrointestinal tract, genital tract or other vascularized tissues as well as excessive bleeding after minor injuries or accidental cuts. Thromboembolic complications and impaired wound healing may also occur. Due to the rarity of the disease, there are no recommendations about fibrinogen substitution before dental procedures (including dental surgery). The purpose of this review is to discuss the indications for the transfusion of a coagulation factor in the preparation of a patient with afibrinogenemia for dental extraction. The article is a narrative review with a proposed management protocol for the dental procedure. The authors have included information from previously published case reports, research studies, and review papers as well as their own case report.

The Profile of Markers of Bone Turnover, Inflammation and Extracellular Neutrophil Traps on Bone Mass in Haemophilia and the Development of Haemophilic Arthropathy.

BACKGROUND: The aim of the study is to evaluate selected biomarkers of bone turnover, inflammation, neutrophil trap and factors predisposing haemophiliacs to bone loss, and to analyse their relationship with clinical features, treatment and complications. METHODS: The levels of interleukin 6 (IL-6); citrullinated histone (CH3); osteocalcin (BGLAP); bone alkaline phosphatase (BALP); N-terminal procollagen type I propeptide (P1NP); and C-terminal collagen type I telopeptide (C1CP) were examined in 60 patients with haemophilia. RESULTS: The cut-off value for BGLAP is 26.41 ng/mL, and 929.7 pg/mL for CH3. There is a statistically significant difference between BGLAP, BALP, C1CP and CH3 concentrations, depending on the prophylaxis used. The median concentration of BGLAP in patients taking the factor on demand is 28.0 ng/mL, BALP 322.5 U/L, C1CP 191.2 ng/mL and CH3 1114.4 pg/mL. In patients taking recombinant coagulation factor VIII/IX as prophylaxis of bleeding, the median BGLAP concentrations are 35.9 ng/mL, BALP 280.9 U/L, C1CP 161.6 ng/mL and CH3 952.5 pg/mL. BGLAP and BALP concentrations are dependent on the development of haemophilic arthropathic. CONCLUSIONS: The concentrations of selected markers of bone turnover and NETs may help to identify patients at particular risk of developing haemophilic arthropathy and bone metabolic turnover abnormalities.

Potential Biochemical Markers and Radiomorphometric Indices as Predictors of Reduced Bone Mass in Patients with Congenital Hemophilia.

Background: The study aimed to evaluate radiomorphometric indices derived from panoramic X-rays and selected blood markers of bone turnover and neutrophil extracellular traps, with a view to identifying hemophilic patients at risk of developing osteoporosis. Methods: The study consisted of 50 adult men with hemophilia A and B (mild, moderate, and severe). The control group consisted of 25 healthy adult men. In both groups, blood samples were collected to determine concentrations of citrullinated histone H3 (CH3) and osteocalcin (BGLAP) with ELISA tests, and panoramic X-rays were obtained. Images were imported into AudaXCeph software to calculate two radiomorphometric indices: mental index (MI) and panoramic mandibular index (PMI). Concentrations of BGLAP and CH3 were compared with MI and PMI values in patients with and without hemophilia. Results: There were statistically significant differences in BGLAP, CH3, and PMI between the study and the control group (p < 0.05). Multivariate logistic regression analysis showed a predictive value for PMI, BGLAP, and CH3.The ROC curve with cutoff point (Youden index) at 0.40­PMI was calculated. No correlation was observed for the PMI index in any particular subgroup of patients. No correlation between MI and BGLAP/CH3 was observed. Conclusions: Simultaneous use of PMI value and BGLAP and CH3 levels may allow the identification of patients with hemophilia who requirea detailed diagnosis of osteoporosis with DXA.

Preventing the suspension of dental clinics by minimizing the risk of SARS­CoV­2 transmission during dental treatment.

Due to a high risk of the transmission of severe acute respiratory syndrome coronavirus 2 (SARS­CoV­2) during dental work, the coronavirus disease 2019 (COVID­19) pandemic has had a considerable influence on the functioning of dental clinics. The elevated risk of transmission is related to the production of aerosol containing secretions from the upper respiratory tract that is produced during dental procedures.The purpose of this narrative review was to present the current knowledge concerning COVID­19 and to propose methods for reducing the spread of the virus. Dental staff should follow the current guidelines and ensure safety at work through the use of personal protective equipment (PPE), including FFP2/FFP3 filter masks, the implementation of the screening protocols and telephone consultations as well as the appropriate preparation of dental practices and patient waiting areas. In addition, it is essential to reduce the number of people simultaneously occupying one building by effectively planning visiting times or discouraging patients from accompanied visits. Procedures that may contribute to production of the aerosol should also be limited.

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

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.