Concurrent congenital hemophilia B and acquired hemophilia A: a unique case report.

Congenital hemophilia B is a rare X-linked recessive bleeding disorder caused by factor IX deficiency. Acquired hemophilia A is a rare, acquired bleeding disorder that presents with new-onset bleeding, especially in older adults, due to the development of auto-antibodies against factor VIII (FVIII). This case report presents the medical management of a patient with congenital hemophilia B and acquired hemophilia A. We highlight the limitations of maintaining factor levels with factor replacement therapy alone, particularly in hemophilia patients who have developed factor inhibitors. In addition, we draw attention to the need for dose escalation, the cost, and the need for immune-tolerance induction therapy. This case illustrates that when the current diagnosis does not explain the full clinical picture and laboratory data are inadequate, it is important to continue to seek alternative diagnoses and cost-effective treatment.

Modulatory role ulexit against thiamethoxam-induced hematotoxicity/hepatotoxicity oxidative stress and immunotoxicity in Oncorhynchusmykiss.

Contamination of the aquatic environment with different insecticides is a major concern in the aquatic ecosystem today. For this reason, in the designed study, Thiamethoxam (TMX) for which there is limited information on its negative effects on Oncorhynchus mykiss was investigated, its effects on hematotoxicity, oxidative status, cytotoxicity, DNA damage and apoptotic status indicators in blood/liver tissue. However, the antitoxic potential of ulexite (UX) supplementation in the elimination of TMX-mediated toxicity has been determined. LC50-96h value determined for TMX 0.73 mg/L has been determined. As a result of hematology profile, TMX application, RBC, Hgb and Hct values showed a temporal decrease compared to the control group, while increases were determined in MCV, MCH and MCHC values. It was determined that the inhibition/induction of hematological parameters was slowed down by adding UX to the medium. During the trial (48th and 96th hours), it was noted that TMX induced cortisol level, while UX supplementation slowed this induction at 48th hour. Antioxidant enzyme activities were significantly inhibited by TMX application, and MDA and MPO values increased as a result of the stimulation of ROS. It was determined that UX added to the medium showed activity in favor of antioxidants and tried to inhibit MDA and MPO levels. When Nrf-2, one of the inflammation parameters, was compared with the administration and control groups, it was determined that it inhibited depending on time, TNF-α, IL-6, DNA damage and apoptosis were induced, and UX suppressed this situation. The results obtained were evaluated as statistically meaningful. Briefly, it was determined that TMX induced oxidative damage in all tissues at 48th - 96th hours, whereas UX mitigated this situation. The results provide possible in vivo evidence that UX supplements can reduce TMX-mediated oxidative stress and tissues damage in O. mykiss blood and liver tissues.

Knowledge and Attitudes of a Group of Dental Students in Turkey About Molar Incisor Hypomineralization.

BACKGROUND This study aimed to evaluate the knowledge and attitudes of 4th- and 5th-year students of Dicle University Faculty of Dentistry (Diyarbakir) about molar incisor hypomineralization (MIH). MATERIAL AND METHODS The questionnaires previously used in other countries were modified and the questionnaire forms consisting of 23 questions were distributed to the students online. The results of the analysis are presented as frequencies. The significance level was set as P<0.05. RESULTS A total of 111 4th-year students (51%) and 107 5th-year students (49%) participated in our study. A statistically significant difference was found between the 4th-year students (68.5%) and the 5th-year students (98.1%) in terms of having heard of the term MIH before (P<0.001). While most students reported knowing the clinical features of MIH, only 26.1% were confident in their diagnosis. A statistically significant difference was found between the 4th- and 5th-year students who thought that MIH was a clinical problem, in the distribution of those who had problems both in terms of aesthetics, and in providing adequate restoration (P=0.012). CONCLUSIONS Although most of the students were theoretically familiar with MIH, they were not confident in their diagnosis and found it difficult to differentiate MIH from other dental anomalies. However, the awareness of 5th-year dental students was better than that of the 4th-year students.

Risk factors for early onset acute kidney injury after allogeneic haematopoietic stem cell transplantation and the role of drug-drug interactions.

INTRODUCTION: Acute kidney injury (AKI) is an important and life-threatening complication following allogeneic haematopoietic stem cell transplantation (allo-HSCT). This is therefore an active research area with studies aiming to understand the factors that cause this complication. MATERIALS AND METHODS: We conducted a retrospective study to identify the factors that caused AKI in 100 patients who underwent allo-HSCT in the first 100 days after transplantation using logistic regression analysis. RESULTS: The mean time of onset of AKI was 45.58 days (range 13-97) and the mean±SD maximum serum creatinine value was 1.53±0.78 mg/dL. In 47 patients, level 1 or higher AKI occurred in the first month of transplantation and 38 of these patients were diagnosed with a higher level of AKI 31-100 days after transplantation. According to multivariate analysis, use of cyclophosphamide (adjusted odds ratio (AOR) 4.01, p=0.012), mean ciclosporin blood levels ≥250 ng/mL (AOR 2.81, p=0.022) and ciclosporin blood levels ≥450 ng/mL in the first month of transplantation (AOR 3.30, p=0.007) were found to be potential factors for early onset AKI. Ciclosporin blood levels exceeded 450 ng/mL in 35% of those using posaconazole and voriconazole during administration route change of ciclosporin. Use of ≥2 nephrotoxic anti-infective drugs (AOR 3, p=0.026) and developing AKI in the first month of transplantation (AOR 4.14, p=0.002) were found to be potential factors in the development of advanced AKI. CONCLUSION: Nephrotoxic drugs, cyclophosphamide use and ciclosporin blood levels are factors to be considered to prevent the development of AKI in patients undergoing allo-HSCT.

Comparison of Antibacterial Activity, Cytotoxicity, and Fluoride Release of Glass Ionomer Restorative Dental Cements in Dentistry.

BACKGROUND This study aimed to compare the antibacterial activity, cytotoxicity, and fluoride release of 4 different glass ionomer cements (GIC). MATERIAL AND METHODS A total of 200 samples were prepared: Riva Silver, a silver-reinforced GIC; Equia Forte HT, a glass hybrid GIC; ChemFil Rock, a zinc-added GIC; and Ketac™ Molar Easymix. Using the agar diffusion test for antibacterial activity, 30 samples from each group were analyzed for Streptococcus mutans, Lactobacillus acidophilus, and Actinomyces naeslundii. The areas around the samples where no bacterial growth occurred were digitally measured and recorded. For cytotoxicity analysis, the WST-1 test was performed on 10 samples from each group using the L929 mouse fibroblast cell line. The fluoride release property was evaluated using an ion-selective electrode method on 10 samples from each group. RESULTS The group that used Ketac™ Molar Easymix showed the lowest antibacterial activity against S. mutans, L. acidophilus, and A. naeslundii. In all 3 days of cytotoxicity testing, the group that used Riva Silver was found to be the least toxic material, while the group that used ChemFil Rock did not have viable cells after the 1st day. In all 4 materials, fluoride release values gradually increased since the first day, with Ketac™ Molar Easymix having the highest fluoride release. CONCLUSIONS Of all the GICs tested, Ketac™ Molar Easymix demonstrated the least antibacterial activity despite having the highest fluoride release, while Riva Silver was found to be the least cytotoxic material.

A comparison of four drug-drug interaction databases for patients undergoing haematopoietic stem cell transplantation.

WHAT IS KNOWN AND OBJECTIVE: Patients who have undergone haematopoietic stem cell transplantation are prone to drug-drug interactions due to polypharmacy. Drug-drug interaction databases are essential tools for identifying interactions in this patient group. However, drug-drug interaction checkers, which help manage interactions, may have disagreements about assessing the existence or severance of the interactions. The study aimed to determine differences among popular drug-drug interaction databases from several angles for patients who underwent haematopoietic stem cell transplantation. METHODS: The 21-day treatment sheets of one hundred patients who underwent haematopoietic stem cell transplantation were examined in two subscription-based (Uptodate and Micromedex) and two open-access databases (Drugs.com and Epocrates) in terms of several categories two years in a row. Statistical analysis was utilized to understand the compatibility of databases in terms of severity scores, evidence levels, given references, and word counts in interaction reports. Fleiss' and Cohen's kappa statistics were used to analyse the databases' agreement levels. RESULTS AND DISCUSSION: A total of 1393 and 1382 different drug-drug interactions were detected in subsequent versions of the databases, namely the 2021 and 2022 versions. The Fleiss kappa overall agreement among databases was slight. Uptodate and Micromedex showed fair agreement, and other database pairs showed slight agreement in severity ratings. CONCLUSION: There was a poor agreement among databases for interactions seen in bone marrow transplantation patients. Therefore, it would be safer to use more than one database in daily practice. Further work needs to be done to understand the agreement level of databases for different types of interactions.

Effect of Surface Wetting Resin on the Color Stability and Microhardness of Esthetic Composites / Efecto de la resina humectante de superficies sobre la estabilidad del color y la microdureza de resinas compuestas estéticas

ABSTRACT: Objective: The aim of this study was to evaluate and compare the effects of the superficial application of two different modeling resins on the surface microhardness and discoloration of composite resins. Material and Methods: The present study used two different composites and modeling resins. The composites were placed in plastic molds. Subsequently, the modeling resins were applied on the surface of the two composite groups. The microhardness and color pertaining to all the groups were evaluated. The current study used the One-Way ANOVA and Kruskal Wallis tests, in order to perform the statistical evaluation (p<0.05). Results: The present study compared the microhardness pertaining to the groups and the highest microhardness values were observed in the Estelite Asteria group (none), whereas the lowest values were observed in the GC Essentia group (Ultradent). Moreover, the current study evaluated the color stability and the greatest discoloration was observed in the control group of the GC Essentia group, whereas the least discoloration was observed in the Estelite Asteria group that included the samples prepared using Ultradent Wetting Resin. Conclusions: The results of the present study demonstrated that the use of modeling resins, which facilitate the placement of composite resins, reduced the microhardness and discoloration of composite resins. The aforementioned effect can be attributed to the variations in the structure of the filling. However, further studies are warranted to support and verify the results of the current study.

RESUMEN: Objetivo: El objetivo del presente estudio fue evaluar y comparar los efectos de la aplicación superficial de dos resinas humectantes sobre la microdureza superficial y la decoloración de las resinas compuestas. Material y métodos: El presente estudio utilizó dos resinas humectantes y resinas de modelado diferentes. Las resinas compuestas se colocaron en moldes de plástico. Posteriormente, se aplicaron las resinas humectantes sobre la superficie de los dos grupos. Se evaluó la microdureza y el color de todos los grupos. El presente estudio utilizó las pruebas One-Way ANOVA y Kruskal Wallis, para realizar la evaluación estadística (p<0.05). Resultados: Los valores más altos de microdureza se observaron en el grupo Estelite Asteria, mientras que los menores valores se observaron en el grupo GC Essentia (Ultradent). La mayor decoloración se observó en el grupo control o el grupo GC Essentia, mientras que la menor decoloración se observó en el grupo Estelite Asteria con Ultradent Wetting Resin. Conclusiones: Los resultados del presente estudio demostraron que el uso de resinas humectantes, que facilitan la colocación de resinas compuestas, redujo la microdureza y decoloración de las resinas compuestas. El efecto mencionado anteriormente se puede atribuir a las variaciones en la estructura del relleno. Sin embargo, se necesitan más estudios para respaldar y verificar los resultados del presente estudio.

An evaluation of clinical, radiological and three-dimensional dental tomography findings in ectodermal dysplasia cases.

BACKGROUND: This study aimed to review the results related to head and jaw disorders in cases of ectodermal dysplasia. The evaluation of ectodermal dysplasia cases was made by clinical examination and examination of the jaw and facial areas radiologically and on cone-beam 3-dimensional dental tomography (CBCT) images. MATERIAL AND METHODS: In the 36 cases evaluated in the study, typical clinical findings of pure hypohidrotic ectodermal displasia (HED) were seen, such as missing teeth, dry skin, hair and nail disorders. CBCT images were obtained from 12 of the 36 cases, aged 1.5- 45 years, and orthodontic analyses were made on these images. RESULTS: The clinical and radiological evaluations determined, hypodontia or oligodontia, breathing problems, sweating problems, a history of fever, sparse hair, saddle nose, skin peeling, hypopigmentation, hyperpigmentation, finger and nail deformities, conical teeth anomalies, abnormal tooth root formation, tooth resorption in the root, gingivitis, history of epilepsy, absent lachrymal canals and vision problems in the cases which included to the study. CONCLUSIONS: Ectodermal dysplasia cases have a particular place in dentistry and require a professional, multi-disciplinary approach in respect of the chewing function, orthognathic problems, growth, oral and dental health. It has been understood that with data obtained from modern technologies such as three-dimensional dental tomography and the treatments applied, the quality of life of these cases can be improved.

A case of cleidocranial dysplasia with peculiar dental features: pathogenetic role of the RUNX2 mutation and long term follow-up.

This report deals with a case of Cleidocranial Dysplasia (CCD) associated to a rare mutation of the RUNX2 gene and a peculiar dental phenotype, namely no supernumerary teeth. The aim consists in evaluating the long-term follow-up after treatment and discussing the pathogenetic mechanism of the mutation. We have carried out a clinical evaluation after treatment and attempted to analyze the potential pathogenetic effect of the mutation, based upon the available experimental structure of RUNX family domain and the highly conserved homology of RUNX1-3. Clinically the treatment has led to tooth development in crowns an roots, correction of cross-bite and eruption of the central maxillary incisor. The structural analysis has pointed out impairment in the DNA binding capability of the mutant protein. The described mutation, c.391C>T (p.R131C) appears to influence both structure and function of the protein by hampering the interaction of RUNX2 with DNA. The impaired function could explain the peculiar reported CCD phenotype. The dental condition of our patient has largely improved after treatment.