Haemophilia is a hereditary bleeding disorder that poses challenges in dental and medical care due to excessive bleeding risk during procedures. Patients often have higher rates of dental caries and periodontal disease due to poor oral hygiene, underscoring the importance of regular cleanings and preventive treatments. Managing these patients in dental settings requires collaboration between dentists and hematologists for meticulous planning and effective clotting factor replacement therapy. Establishing standardized protocols and raising awareness are necessary for optimizing care. This review enhances understanding of haemophilia by exploring its types, genetic basis, pathophysiology, clinical presentation, and management strategies to improve patient outcomes. Advances include extended half-life agents, non-replacement therapies, gene and cellular therapies, and supportive measures such as dental lasers, all aimed at reducing treatment burden and improving quality of life.
Introduction This study aimed to investigate the role and mechanism of Guanylate Cyclase Activator 1C (GUCA1C) in osteoclast (OC) differentiation within the context of bone tuberculosis (TB), focusing on the RANKL/RANK/OPG pathway. Methods RNA sequencing was performed on peripheral blood samples from 10 treatment-naïve patients with isolated skeletal TB, 10 with pulmonary TB, and 10 healthy volunteers. Bioinformatics analyses (GO, KEGG, PPI network) were employed to identify differentially expressed genes (DEGs). In vitro validation was conducted using THP-1-derived macrophages. Cells were transfected with GUCA1C-overexpression or -knockdown lentivirus, followed by osteoclastogenic induction with RANKL and M-CSF. Osteoclast formation and function were assessed by TRAP staining, Western blot (for RANK, RANKL, OPG proteins), and qPCR for key osteoclast markers (NFATc1, TRAP, CTSK, RANK, RANKL, OPG). Results Bioinformatics screening identified 210 unique DEGs in the bone TB group. GUCA1C, enriched in the phototransduction pathway, was highlighted as a key candidate. In vitro, GUCA1C overexpression significantly promoted the formation of TRAP-positive multinucleated osteoclasts and upregulated the mRNA and protein levels of RANK, RANKL, and the transcription factor NFATc1 and its downstream effectors (TRAP, CTSK). While both GUCA1C overexpression and knockdown increased OPG expression compared to the control, the knockdown group exhibited higher OPG levels than the overexpression group. Correspondingly, GUCA1C knockdown attenuated the pro-osteoclastic effects observed in the overexpression group. Discussion This study provides the first evidence linking GUCA1C, which encodes the calcium-sensor GCAP3, to bone pathology. We propose that GUCA1C, beyond its known retinal function, may act as a novel calcium-signaling modulator in osteoclast precursors. It appears to preferentially enhance pro-osteoclastic signals (RANKL/RANK) while exerting a comparatively weaker upregulatory effect on the decoy receptor OPG. This differential regulation disrupts the RANKL/RANK/OPG axis balance towards enhanced osteoclastogenesis, potentially contributing to inflammatory bone destruction in bone TB. Thus, GUCA1C represents a potential therapeutic target for mitigating pathological bone loss. Conclusion GUCA1C promotes osteoclast differentiation by modulating the RANKL/RANK/OPG signaling axis, identifying it as a potential candidate therapeutic target for bone tuberculosis.
Eosinophilic myocarditis is a rare and aggressive form of infiltrative cardiomyopathy associated with significant morbidity and mortality, especially when diagnosis is delayed. Symptoms are often vague and overlap with other cardiac conditions, making recognition challenging. Positron emission tomography combined with computed tomography has become more relevant as a supplementary diagnostic tool, although its effectiveness in follow-up remains underexplored. This report discusses a likely paraneoplastic case in classical Hodgkin’s lymphoma, emphasizing that metabolic cardiac activity on serial imaging closely mirrored clinical progression. A 75-year-old woman with classical Hodgkin’s lymphoma initially responded well to chemotherapy and maintained stability for over eighteen months. She later developed progressive heart failure with restrictive physiology, biventricular involvement, severe pulmonary hypertension, and an apical intraventricular thrombus. Laboratory tests indicated intermittent eosinophilia and varying inflammatory markers. Multimodal imaging revealed diffuse myocardial edema and extensive endocardial fibrosis, indicating eosinophilic involvement. Across five oncological scans, myocardial metabolic activity increased alongside the patient's deterioration. Despite thorough investigations, including biopsies and ruling out infectious, autoimmune, infiltrative, and coronary causes, histological confirmation remained elusive. Given her advanced cardiac condition, comorbidities, and complications from treatment, immunosuppression and invasive interventions were not viable options, and the patient continued to decline. This case illustrates the diagnostic challenges of eosinophilic myocarditis in unusual paraneoplastic contexts and underscores the value of multimodal imaging and interdisciplinary collaboration. The metabolic changes observed across sequential scans support this approach for early detection and follow-up, though additional validation is required.
To investigate the dosimetric correlation between radiation dose to the cervical site and radiation-induced dermatitis in patients with advanced nasopharyngeal carcinoma undergoing concurrent chemoradiotherapy. Data from 109 patients treated with concurrent chemoradiotherapy between November 2018 and June 2020 were analyzed. The study focuses on identifying clinical and dosimetric factors associated with moderate to severe radiation dermatitis and determining the optimal radiation dose to the cervical site. Univariate analysis revealed that nodal stage, tumor node metastasis stage, volume to gross tumor volume of the neck, and mean dose to the cervical site were associated with an increased risk of grade 3/4 radiation dermatitis. Multivariate analysis identified cervical site of mean dose (odds ratio=1.003; 95%confidence Interval=1.001-1.005; P =0.003) as an independent predictor. The optimal cut-off value of cervical site as was 5206 centigray (area under curve =0.733%; P <0.01;95% confidence Interval =0.637-0.829). While prior studies have established an association between cervical radiation dose and dermatitis severity, detailed radiotherapy dose-specific data regarding radiation dermatitis remain scarce. This study aims to clarify in detail the toxicity levels of such skin damage across different radiation doses and provides precise dosimetric insights. Moderate or severe radiation dermatitis is significantly correlated with the mean dose to the cervical site, and keeping the cervical dose below 5200 centigray may decrease the incidence of severe skin toxicity. We recommend patients with mean dose over 5200 centigray to consider topical treatment during the early stages of radiotherapy and advanced techniques to reduce radiation volumes and doses to the cervical site may be useful.
A cardiovascular implantable electronic device infection (CIEDI) is a rare but serious complication, often difficult to diagnose. We present a unique case of CIEDI involving a long-standing right atrial pseudoaneurysm with pericardial abscess related to an old pacemaker system. A 51-year-old woman with a dual-chamber pacemaker presented with prolonged fever and lead dysfunction. Multimodal imaging revealed lead perforation into the pericardial sac, resulting in an abscess. A hybrid approach combining open surgery, percutaneous extraction, and targeted antibiotic therapy led to full recovery. Accurate diagnosis through advanced imaging and multidisciplinary care is essential for the management of complex CIEDI and to improve outcomes.
