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BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (MPN) are most prevalent in the older population (median age at the diagnosis is above 60 years) and rarely diagnosed in pediatrics. Thus, our knowledge about the clinical presentation, mutational status, and complications of MPNs in pediatrics is limited. METHODS: The literature in English (PubMed, SCOPUS, and Google Scholar) was searched for studies, reviews, case series, and case reports of patients with Philadelphia-negative MPNs (including essential thrombocythemia, polycythemia vera, primary myelofibrosis, and profibrotic myelofibrosis) in the pediatrics age group (less than 18 years). Only studies that fulfilled WHO 2008 or 2016 criteria for MPNs were included. We aimed to describe the clinical characteristics, vascular and long-term complications, types of driver mutations, and treatment approaches in pediatric patients with MPNs. RESULTS: We reviewed 33 articles of available published literature from 2008 to 2022 and collected data from a total of 196 patients of the pediatric population. Among the cohort of patients, 139 had essential thrombocythemia (ET), 20 had polycythemia vera (PV), and 37 had primary myelofibrosis (PMF). The median age at the time of diagnosis for each disease varied, with 8.8 years for ET, 10 years for PV, and 3.6 years for MF. There was a slight difference in gender prevalence between both gender groups and all three diseases. The presenting symptoms were not mentioned in more than 50% of studies. We found that JAK2 was the most prevalent among all mutations. Both bleeding and thrombosis were present equally in ET, with 9% of cases complicated by bleeding and 9% complicated by thrombosis. Hemorrhagic events did not occur in patients with PV; thrombosis in children with MF was also not found. The progression into AML occurred in two patients with PV and one with ET. CONCLUSION: Given the rarity of MPNs in pediatrics and their different characteristics compared with adults, we believe there is a need for unique diagnostic criteria to match the different molecular statuses in pediatrics. Based on our review, the incidence of MPN complications in pediatrics, including thrombotic events, hemorrhage, and leukemic transformation, differs from that in adults.
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Background and Aim: The coronavirus-19 is an ongoing global pandemic resulting in millions of deaths worldwide. For a patient population at higher risk of infection, telemedicine is a promising means of providing safe and alternative care routes while minimizing their risk of exposure. This study gives insight into patients' experiences and satisfaction with telemedicine during this pandemic. Methods: We conducted a cross-sectional study on 297 patients (RR: 85%) at the National Center for Cancer Care and Research (NCCCR), Qatar. Data was collected through electronic medical records of the eligibe patient population, and phone calls were made whereby the physician read a standard introductory script followed by a survey questionnaire. We focused on patients' experience with telemedicine services amid the pandemic. This was done using a six-point Likert scoring system of seven questions that were scaled from 1 to 6. Results: More than 80% of patients somewhat to strongly agreed that telemedicine met their healthcare needs, improved their confidence in their healthcare system, and were generally satisfied with the quality of care provided. Nearly all patients (90%) understood their physicians' recommendations over the phone. In addition, more than half of the patients (89%) felt they could freely communicate their concerns. Patients also showed an inclination towards face-to-face consultations at 68%; however, 90% were willing to participate in future teleconsultations. Conclusion: Our study indicates an overall positive experience among patients towards the use of telemedicine. Telemedicine is a safe, futuristic approach toward patient care management and, thus, provides healthcare professionals a platform to implement further patient and physician education. Even though our data also showed that patients liked in-person visits to some degree, this needs to be looked into more in future studies.
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BACKGROUND Lemierre's syndrome is a potential life-threatening disease commonly occurring in young, healthy individuals. It is often preceded by an oropharyngeal infection causing bacteremia. This may rapidly progress into thrombophlebitis of the internal jugular venous system, its branches, and septic embolization and often fulminant organ failure. CASE REPORT A previously healthy 31-year-old male with recent history of facial herpes zoster infection, presented with 1-week history of increasingly painful nasal, and periorbital swelling. Imaging confirmed superior ophthalmic vein thrombosis. Staphylococcus aureus was isolated in blood cultures and had an uncomplicated hospital course with full recovery. CONCLUSIONS Early recognition of Lemierre's syndrome contributes significantly in reducing morbidity and mortality associated with it. Staphylococcus aureus skin infection is a very rare cause of Lemierre's syndrome, and its association with superior ophthalmic vein thrombosis has not yet been reported in literature.