Essential Thrombocythemia in a Two-year-old Child, Responsive to Hydroxyurea but Not Aspirin

Essential thrombocythemia (ET) is a myeloproliferative neoplasm that occurs mostly in patients above the age of 50 years. Its incidence in children is very rare, with around 100 cases reported in the literature. High-risk patients are defined by previous life threatening major thrombotic or severe hemorrhagic complication or age > 60. Those patients probably benefit from cytoreductive therapy. On the other hand, antiplatelet drugs are recommended for patients with low risk group. Although rare, ET should be considered in the differential diagnosis of persistent thrombocytosis in children, even at a very young age. A constellation of clinical, pathologic ,and molecular testing are essential for diagnosis. Given the rarity of these cases, there is currently no consensus for treatment guidelines in children, especially in asymptomatic patients. We describe a case of a two-year old girl who presented with unexplained, isolated thrombocytosis which persisted for eight years. Bone marrow biopsy demonstrated typical features of ET. Over the course of the disease, hydroxyurea, but not aspirin, showed better control of symptoms and lowered the platelets level

Biocompatibility of biodentine™ ® with periodontal ligament stem cells: In vitro study

Biodentine™ is a tricalcium silicate-based cement material that has a great impact on different biological processes of dental stem cells, compared to other biomaterials. Therefore, we aimed to investigate the optimum biocompatible concentration of Biodentine™ with stem cells derived from periodontal ligament (hPDLSCs) by determining cell proliferation, cytotoxicity, migration, adhesion and mineralization potential. hPDLSCs were treated with Biodentine™ extract at different concentrations; 20, 2, 0.2 and 0.02 mg/mL. Cells cultured without Biodentine™ were used as a blank control. The proliferation potential of hPDLSCs was evaluated by MTT viability analysis for 6 days. Cytotoxicity assay was performed after 3 days by using AnnexinV/7AAD. Migration potential was investigated by wound healing and transwell migration assays at both cellular and molecular levels. The expression levels of chemokines CXCR4, MCP-1 and adhesion molecules FGF-2, FN, VCAM and ICAM-1 were measured by qPCR. The communication potentials of these cells were determined by adhesion assay. In addition, mineralization potential was evaluated by measuring the expression levels of osteogenic markers; ALP, OCN, OPN and Collagen type1 by qPCR. Our results showed significant increase in the proliferation of hPDLSCs at low concentrations of Biodentine™ (2, 0.2 and 0.02 mg/mL) while higher concentration (20 mg/mL) exhibited cytotoxic effect on the cells. Moreover, 2 mg/mL Biodentine™ showed a significant increase in the migration, adhesion and mineralization potentials of the derived cells among all concentrations and when compared to the blank control. Our findings suggest that 2 mg/mL of Biodentine™ is the most biocompatible concentration with hPDLSCs, showing a high stimulatory effect on the biological processes.Cell Therapy Center-The University of Jorda

PREVALENCE OF THROMBOPHILIC GENETIC FACTORS AMONG PATIENTS WITH RETINITIS PIGMENTOSA

Purpose:To determine the prevalence of thrombophilic factors in patients with retinitis pigmentosa (RP).Methods:Fifty consecutive patients with RP and 50 controls matched by age and gender were tested for the presence of the following mutations: factor II (GA20210), factor V Leiden (GA1691), methylenetetrahydrofolate reductase (CT677), factor XIIIa (Val(34)Leu), -fibrinogen (GA455), tumor necrosis factor receptor (TNFRII) (M196R), plasminogen activator inhibitor-1 (PAI-1) (4 G/5 G), and plasminogen activator inhibitor-1 (PAI-1) (GA844).Results:The following heterozygous mutations were found in patients/controls: factor V Leiden (12/14), factor XIIIa (20/30), methylenetetrahydrofolate reductase 677 TT (48/52), -fibrinogen GA455 (36/36), TNFRII (M196R) (40/42), PAI-1 4 G/5 G (40/48), and PAI-1 GA844 (50/52). The difference between patients with RP and the control group was not statistically significant for the prevalence of any of the studied factors (P > 0.05).Conclusion:In this study, thrombophilic mutations were not increased in patients with RP. Thrombophilic mutations do not seem to be risk factors for RP. Routine investigation of hereditary thrombophilia in these patients is not justified

Recent advances in using liposomes for delivery of nucleic acid-based therapeutics

Nucleic acid therapeutics are rapidly expanding because of recent advancements in production and purification. This class of therapeutics may change the field of disease treatment and personalized medicine since it can cure diseases. However, drug delivery systems are crucial for these therapeutics to fulfill their potential. Liposomes have long been considered ideal platforms for systemic drugs delivery. Considering the development in cancer therapeutics, this work emphases on the current advancements in liposomes’ Nano-formulations, functionalization, and design and how it has been applied to nucleic acid therapeutics. Accordingly, this review covers the literature that deals with liposomes in nucleic acid therapy

Development and validation of Ran as a prognostic marker in stage I and stage II primary breast cancer.

PURPOSE Existing prognostic biomarkers are inadequate for stratifying breast cancer patients with the highest risk of tumor progression at the time of diagnosis. Here, we demonstrate that the small GTPase Ran has predictive value for breast cancer (BC) patients as a whole, and for specific BC subtypes. PATIENTS AND METHODS Ran expression was quantified by immunohistochemistry in 263 patients with primary breast cancer diagnosed at the Breast Unit, Royal Liverpool Hospital. Additionally as an independent validation, we also analyzed the mRNA expressions of Ran, ER, PR, and Cerb-2, the triple-negative endocrine receptors, and their associations with patient survival in a combined patient cohorts of multiple public datasets (n = 1079). We analyzed the data with Spearman's rank correlation and Kaplan-Meier plots coupled with Wilcoxon-Gehan tests, respectively. All statistical tests were two-sided. RESULTS Ran nuclear, cytoplasmic, and total staining are substantially associated with poor survival, independent of conventional prognostic markers such as estrogen receptor (ER), human epidermal growth factor receptor 2 (HER2), and lymph node status. According to the datasets, Ran was significantly correlated with distant metastasis-free survival (DMFS) and relapse-free survival (RFS). CONCLUSION We found that Ran expression is a unique predictive biomarker for patient survival, metastasis, and tumor relapse. This biomarker could be used for diagnostic purposes, using formalin-fixed, paraffin-embedded tumor biopsy samples from breast cancer patients in the early stages