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Myelodysplastic neoplasms (MDS) with ring sideroblasts (RS) are diagnosed via bone marrow aspiration in the presence of either (i) ≥15% RS or (ii) 5–14% RS and an SF3B1 mutation. In the MEDALIST trial and in an interim analysis of the COMMANDS trial, lower-risk MDS-RS patients had decreased transfusion dependency with luspatercept treatment. A total of 6817 patients with suspected hematologic malignancies underwent molecular testing using a next-generation-sequencing-based genetic assay and 395 MDS patients, seen at our centre from 1 January 2018 to 31 May 2023, were reviewed. Of these, we identified 39 evaluable patients as having lower-risk MDS with SF3B1 mutations: there were 20 (51.3%) males and 19 (48.7%) females, with a median age of 77 years (range of 57 to 92). Nineteen (48.7%) patients had an isolated SF3B1 mutation with a mean variant allele frequency of 35.2% +/− 8.1%, ranging from 7.4% to 46.0%. There were 29 (74.4%) patients with ≥15% RS, 6 (15.4%) with 5 to 14% RS, one (2.6%) with 1% RS, and 3 (7.7%) with no RS. Our study suggests that a quarter of patients would be missed based on the morphologic criterion of only using RS greater than 15% and supports the revised 2022 definitions of the World Health Organization (WHO) and International Consensus Classification (ICC), which shift toward molecularly defined subtypes of MDS and appropriate testing.

Patient access to new oncology drugs in Canada is only possible after navigating multiple sequential systemic checkpoints for national regulatory approval, health technology assessment (HTA) and collective government price negotiation. These steps delay access and prevent health care providers from being able to prescribe optimal therapy. Eighteen Canadian oncology clinicians from the medicine, nursing and pharmacy professions met to develop consensus recommendations for defining reasonable government performance standards around process and timeliness to improve Canadian cancer patients’ access to best care. A modified Delphi methodology was used to identify consensus on 30 questions involving five themes: accountability, disparities, endpoints, timeliness, and cost-effectiveness. It was agreed that greater transparency is required across regulatory and HTA processes. Health professionals in oncology are frustrated for their patients because they are unable to deliver the modern guideline-supported therapies they want to provide due to delays in approval or funding. Canadian health care providers request improvements in timely access to life-saving therapeutics in line with other comparator countries. Clinicians expect urgent improvements in Canadian health systems to give our patients their best chance of survival.

ObjectiveTo evaluate new medical devices and drugs pertinent to otolaryngology-head and neck surgery that were approved by the Food and Drug Administration (FDA) in 2021.Data sourcesPublicly available FDA device and drug approvals from ENT (ear, nose, and throat), anesthesia, neurosurgery, plastic surgery, and general surgery FDA committees.Review methodsFDA device and therapeutic approvals were identified and reviewed by members of the American Academy of Otolaryngology-Head and Neck Surgery's Medical Devices and Drugs Committee. Two independent reviewers assessed the relevance of devices and drugs to otolaryngologists. Medical devices and drugs were then allocated to their respective subspecialty fields for critical review based on available scientific literature.ConclusionsThe Medical Devices and Drugs Committee reviewed 1153 devices and 52 novel drugs that received FDA approval in 2021 (67 ENT, 106 anesthesia, 618 general surgery and plastic surgery, 362 neurosurgery). Twenty-three devices and 1 therapeutic agent relevant to otolaryngology were included in the state of the art review. Advances spanned all subspecialties, including over-the-counter hearing aid options in otology, expanding treatment options for rhinitis in rhinology, innovative laser-safe endotracheal tubes in laryngology, novel facial rejuvenation and implant technology in facial plastic surgery, and advances in noninvasive and surgical treatment options for obstructive sleep apnea.Implications for practiceFDA approvals for new technology and pharmaceuticals present new opportunities across subspecialties in otolaryngology. Clinicians' nuanced understanding of the safety, advantages, and limitations of these innovations ensures ongoing progress in patient care.

Abstract Objective To review new drugs and devices relevant to otolaryngology approved by the Food and Drug Administration (FDA) in 2022. Data Sources Publicly available FDA data on drugs and devices approved in 2022. Review Methods A preliminary screen was conducted to identify drugs and devices relevant to otolaryngology. A secondary screen by members of the American Academy of Otolaryngology–Head and Neck Surgery's (AAO‐HNS) Medical Devices and Drugs Committee differentiated between minor updates and new approvals. The final list of drugs and devices was sent to members of each subspecialty for review and analysis. Conclusion A total of 1251 devices and 37 drugs were identified on preliminary screening. Of these, 329 devices and 5 drugs were sent to subspecialists for further review, from which 37 devices and 2 novel drugs were selected for further analysis. The newly approved devices spanned all subspecialties within otolaryngology. Many of the newly approved devices aimed to enhance patient experience, including over‐the‐counter hearing aids, sleep monitoring devices, and refined CPAP devices. Other advances aimed to improve surgical access, convenience, or comfort in the operating room and clinic. Implications for Practice Many new devices and drugs are approved each year to improve patient care and care delivery. By staying up to date with these advances, otolaryngologists can leverage new innovations to improve the safety and quality of care. Given the recent approval of these devices, further studies are needed to assess long‐term impact within the field of otolaryngology.

Background. Thrombocytosis is a common reason for referral to Hematology. Differentiating between secondary causes of thrombocytosis and essential thrombocythemia (ET) is often clinically challenging. A practical diagnostic approach to identify secondary thrombocytosis could reduce overinvestigation such as next generation sequencing (NGS) panel. Methods and Results. All adult patients with thrombocytosis (≥450 × 109/L) who underwent molecular testing at a single tertiary care centre between January 1, 2018 and May 31, 2021 were evaluated. Clinical and laboratory variables were compared between patients with secondary thrombocytosis vs. ET. Clinical variables included smoking, thrombosis, splenectomy, active malignancy, chronic inflammatory disease, and iron deficiency anemia. Laboratory variables included complete blood count (CBC), ferritin, and myeloid mutations detected by NGS. The overall yield of molecular testing was 52.4%; 92.1% of which were mutations in JAK2, CALR, and/or MPL. Clinical factors predictive of ET included history of arterial thrombosis (p

Abstract Objective Many facial plastic surgery procedures can be performed in an office‐based setting, ranging from simple chemical peels to reconstructive surgeries to assist with scar revision. The aim of this review is to summarize the current state of facial plastics in‐office surgical procedures, including scar revision, dermabrasion, lasers, and chemical peels. Methods A literature review was conducted with the PubMed search engine with the following keywords: facial plastics, scar revision, dermabrasion, lasers, chemical peels, face, office, and outpatient. The literature was surveyed for relevance, with a focus on realistically which procedures were performed in a facial plastics surgery outpatient clinic. These were then used to compile a review of the current state of this field. Results and Conclusions There are various scar revision procedures that can be performed in the office setting, including z‐plasty, w‐plasty, and geometric broken line closure techniques, as well as various nonmedical treatment options to improve the appearance of a scar. Dermabrasion can also be performed to assist with elevated scars. Various lasers are available to enhance cell turnover in the field of facial rejuvenation, scarring, and sun damage. Chemical peels are also available to assist with facial rejuvenation. With the appropriate counseling of the patient and medical staff, these can be safely performed in the office.

Sharook A Alhawaj,1 Alia Saeed Almuhanna,1 Fatimah Saeed Alabbad,1 Hadeel Hisham Almomattin,1 Ragad S Alsultan,1 Zahra Abdulla Shaiban,1 Chittibabu Vatte,2 Cyril Cyrus2 1College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia; 2College of medicine, Department of Biochemistry, Imam Abdulrahman Bin Faisal University, Dammam, Saudi ArabiaCorrespondence: Sharook A Alhawaj, College of Medicine, Imam Abdulrahman bin Faisal university, Dammam, 34212, Saudi Arabia, Tel +966595292920, Email shroog7676@gmail.comBackground: COVID‐19 vaccines were developed to control the pandemic spread as they have been proven to be efficient and safe. However, the likelihood of such postvaccination effects as poor glycemic control and adverse events has been noted in several studies.Objective: To determine the effect of COVID-19 vaccines on the glycemic control and the development of hyperglycemic emergencies among type 1 and 2 diabetes patients.Methods: A cross-sectional study was conducted on 409 participants aged 18 years and above with type 1 or 2 diabetes who had received at least a single dose of COVID-19 vaccine.Results: Among the 409 diabetes patients, a majority reported general mild postvaccination symptoms regardless of diabetes duration or type. After vaccination, severe diabetic emergencies were mostly reported in long-standing diabetes patients. Diabetes-related complications and emergencies were more profound among those who had received the Pfizer vaccine. Nonetheless, occurrence of adverse events could possibly be due to various factors, including the duration of diabetes and COVID-19 infection status.Conclusion: COVID-19 vaccinations have the potential to influence diabetic patients in regard to acute glycemic complications. However, vaccine efficiency and benefits are superior to the side effects of COVID-19 vaccines, as these adverse events only affect a small number of individuals. A need for postvaccination monitoring of diabetes patients is suggested.Keywords: diabetes, vaccine, COVID-19, hyperglycemic emergencies, diabetes complications

Key Clinical Message Additional investigations for systemic involvement should be initiated once the diagnosis of cutaneous mastocytosis has been established in an adult patient. A serum tryptase can serve as a screening test for systemic mastocytosis, and persistent elevations should prompt further investigations, such as bone marrow studies. Abstract Urticaria pigmentosa (UP) is the most common form of cutaneous mastocytosis, presenting as a wide variety of macroscopic appearances. Cutaneous mastocytosis in pediatric patients usually does not present with systemic involvement, but more than half of adult patients with cutaneous mastocytosis demonstrate systemic involvement. Currently, there is no guidance surrounding systemic testing in patients with UP. A 50‐year‐old Caucasian male was referred to the Clinical Immunology and Allergy clinic with a history of a rash. He initially presented to hospital 12 years prior with group A beta hemolytic streptococcus bacteremia treated with multiple different antibiotics. One week following discharge, he developed erythematous brown spots on his right leg which were flat, non‐pruritic, and not painful. The rash later expanded to his trunk and extremities. A skin biopsy performed 2 years prior to referral to our clinic demonstrated urticaria pigmentosa. The CD117 immunohistochemical stain showed increased perivascular and interstitial mast cells in the superficial dermis. Darier's sign was negative on physical examination, and venom testing was also negative. Although he had no symptoms of systemic involvement, his serum tryptase was elevated at 47.6 ng/mL in the context of normal kidney and liver function. A skeletal survey was normal, and an abdominal ultrasound ruled out splenomegaly. Bone marrow biopsy demonstrated a mild increase in paratrabecular and perivascular atypical mast cells, in keeping with systemic mastocytosis. Adult patients with cutaneous mastocytosis have a high likelihood of having an underlying systemic mast cell disorder. Therefore, any patient presenting with characteristic skin findings should be investigated as having a cutaneous manifestation of systemic mastocytosis. This case demonstrates the utility of serum tryptase and its role in triggering additional investigations and guiding appropriate therapy.

Extramedullary hematopoiesis (EMH) is a rare complication of solid tumor malignancies. We describe the first case of a patient who developed EMH in the pericardium secondary to metastatic gastrointestinal or pancreaticobiliary cancer. A 58-year-old man presented with recurrent episodes of fatigue and shortness of breath and was treated with thoracocentesis and pericardiocentesis for pleural and pericardial effusions, respectively. Owing to a markedly elevated alkaline phosphatase, a bone scan was performed and demonstrated diffuse sclerotic lesions. Evaluation of pleural effusion diagnosed metastatic adenocarcinoma, and cytospin morphology of the pericardial fluid demonstrated EMH. While EMH secondary to solid tumors is commonly suggested to be due to cytokine signaling, we propose the mechanism of EMH in this patient was due to extensive disruption of bone marrow hematopoiesis, similar to what is seen in myeloproliferative neoplasms.

There are various neurological manifestations of coronavirus disease 2019 (COVID-19). Recent data suggest a connection between hemifacial paralysis, or Bell’s palsy, and COVID-19. Although the etiology of Bell’s palsy is unknown, the leading proposed etiology is viral in nature. Since the onset of the pandemic, numerous studies have investigated the relationship between Bell’s palsy, COVID-19 infection, and COVID-19 vaccination. The researchers studied the current literature on the topic of COVID-19 as it relates to Bell’s palsy.

Background: Since the identification of JAK2 V617F and exon 12 mutations as driver mutations in polycythemia vera (PV) in 2005, molecular testing of these mutations for patients with erythrocytosis has become a routine clinical practice. However, the incidence of myeloid mutations other than the common JAK2 V617F mutation in unselected patients referred for elevated hemoglobin is not well studied. This study aimed to characterize the mutational landscape in a real-world population of patients referred for erythrocytosis using a targeted next-generation sequencing (NGS)-based assay. Method: A total of 529 patients (hemoglobin levels >160 g/L in females or >165 g/L in males) were assessed between January 2018 and May 2021 for genetic variants using the Oncomine Myeloid Research Assay (ThermoFisher Scientific, Waltham, MA, USA) targeting 40 key genes with diagnostic and prognostic implications in hematological conditions (17 full genes and 23 genes with clinically relevant “hotspot” regions) and a panel of 29 fusion driver genes (>600 fusion partners). Results: JAK2 mutations were detected in 10.9% (58/529) of patients, with 57 patients positive for JAK2 V617F, while one patient had a JAK2 exon 12 mutation. Additional mutations were detected in 34.5% (20/58) of JAK2-positive patients: TET2 (11; 19%), DNMT3A (2;3.4%), ASXL1 (2; 3.4%), SRSF2 (2; 3.4%), BCOR (1; 1.7%), TP53 (1; 1.7%), and ZRSR2 (1; 1.7%). Diagnosis of PV was suspected in 2 JAK2-negative patients based on the 2016 World Health Organization (WHO) diagnostic criteria. Notably, one patient carried mutations in the SRSF2 and TET2 genes, and the other patient carried mutations in the SRSF2, IDH2, and ASXL1 genes. Three JAK2-negative patients with elevated hemoglobin who tested positive for BCR/ABL1 fusion were diagnosed with chronic myeloid leukemia (CML) and excluded from further analysis. The remaining 466 JAK2-negative patients were diagnosed with secondary erythrocytosis and mutations were found in 6% (28/466) of these cases. Conclusion: Mutations other than JAK2 mutations were frequently identified in patients referred for erythrocytosis, with mutations in the TET2, DNMT3A, and ASXL1 genes being detected in 34.5% of JAK2-positive PV patients. The presence of additional mutations, such as ASXL1 mutations, in this population has implications for prognosis. Both the incidence and mutation type identified in patients with secondary erythrocytosis likely reflects incidental, age-associated clonal hematopoiesis of indeterminate potential (CHIP).

Abstract Background Family history (FamH) of type 2 diabetes might indicate shared genotypes, environments, and/or behaviors. We hypothesize that FamH interacts with unhealthy behaviors to increase the risk of early onset of diabetes and poor cardiometabolic control. Methods In a cross-sectional analysis of the prospective Joint Asia Diabetes Evaluation Register including patients from 427 clinics in 11 Asian countries/regions in 2007–2021, we defined positive FamH as affected parents/siblings and self-management as (1) healthy lifestyles (balanced diet, non-use of alcohol and tobacco, regular physical activity) and (2) regular self-monitoring of blood glucose (SMBG). Results Among 86,931 patients with type 2 diabetes (mean±SD age: 56.6±11.6 years; age at diagnosis of diabetes: 49.8±10.5 years), the prevalence of FamH ranged from 39.1% to 85.3% in different areas with FamH affecting mother being most common (32.5%). The FamH group (n=51,705; 59.5%) was diagnosed 4.6 years earlier than the non-FamH group [mean (95% CI): 47.9 (47.8–48.0) vs. 52.5 (52.4–52.6), logrank p

Cyclopropanols are versatile starting materials which can undergo various ring opening reactions due to their intrisic ring strain. Herein, we report two transition metal-catalyzed α-hydroxycyclopropanol ring opening cyclizations to divergently transform the same α-hydroxycyclopropanol substrate into two different products of enhanced value. One is a palladium-catalyzed α-hydroxycyclopropanol ring opening carbonylative lactonization to synthesize δ-valerolactones. The other one is a copper-catalyzed α-hydroxycyclopropanol ring opening cyclization to access furanones. [ABSTRACT FROM AUTHOR]

Objective To evaluate new medical devices and drugs pertinent to otolaryngology–head and neck surgery that were approved by the Food and Drug Administration (FDA) in 2021. Data Sources Publicly available FDA device and drug approvals from ENT (ear, nose, and throat), anesthesia, neurosurgery, plastic surgery, and general surgery FDA committees. Review Methods FDA device and therapeutic approvals were identified and reviewed by members of the American Academy of Otolaryngology–Head and Neck Surgery’s Medical Devices and Drugs Committee. Two independent reviewers assessed the relevance of devices and drugs to otolaryngologists. Medical devices and drugs were then allocated to their respective subspecialty fields for critical review based on available scientific literature. Conclusions The Medical Devices and Drugs Committee reviewed 1153 devices and 52 novel drugs that received FDA approval in 2021 (67 ENT, 106 anesthesia, 618 general surgery and plastic surgery, 362 neurosurgery). Twenty-three devices and 1 therapeutic agent relevant to otolaryngology were included in the state of the art review. Advances spanned all subspecialties, including over-the-counter hearing aid options in otology, expanding treatment options for rhinitis in rhinology, innovative laser-safe endotracheal tubes in laryngology, novel facial rejuvenation and implant technology in facial plastic surgery, and advances in noninvasive and surgical treatment options for obstructive sleep apnea. Implications for Practice FDA approvals for new technology and pharmaceuticals present new opportunities across subspecialties in otolaryngology. Clinicians’ nuanced understanding of the safety, advantages, and limitations of these innovations ensures ongoing progress in patient care.

Background Surgery is often avoided in the setting of pediatric orbital complications from acute sinusitis unless necessitated by alarming ophthalmological signs. Criteria for surgical intervention are not well-defined. Objective We aim to review our experiences, management practices and patient outcomes over a ten-year period for Chandler III patients. Methods A retrospective review was performed from January 1, 2007 through December 31, 2016 of patients treated for orbital symptoms secondary to acute sinusitis at a free-standing tertiary-care pediatric hospital. Results Of the 186 patients reviewed, 42 Chandler III patients were included. Average age was 82.6 months (SD 50.6) with a slight male predominance (M to F, 1.8 to 1). 27 patients (64.3%) underwent intervention including endoscopic sinus surgery (ESS) with or without orbitotomy. Late surgical intervention (>48hrs from admission) demonstrated significant increase in overall length of stay (LOS) when compared with early surgical intervention and/or medical management (median, 6.9 vs 3.6 vs 3.7 days; p

Myelodysplastic neoplasms (MDS) with ring sideroblasts (RS) are diagnosed via bone marrow aspiration in the presence of either (i) ≥15% RS or (ii) 5–14% RS and an SF3B1 mutation. In the MEDALIST trial and in an interim analysis of the COMMANDS trial, lower-risk MDS-RS patients had decreased transfusion dependency with luspatercept treatment. A total of 6817 patients with suspected hematologic malignancies underwent molecular testing using a next-generation-sequencing-based genetic assay and 395 MDS patients, seen at our centre from 1 January 2018 to 31 May 2023, were reviewed. Of these, we identified 39 evaluable patients as having lower-risk MDS with SF3B1 mutations: there were 20 (51.3%) males and 19 (48.7%) females, with a median age of 77 years (range of 57 to 92). Nineteen (48.7%) patients had an isolated SF3B1 mutation with a mean variant allele frequency of 35.2% +/− 8.1%, ranging from 7.4% to 46.0%. There were 29 (74.4%) patients with ≥15% RS, 6 (15.4%) with 5 to 14% RS, one (2.6%) with 1% RS, and 3 (7.7%) with no RS. Our study suggests that a quarter of patients would be missed based on the morphologic criterion of only using RS greater than 15% and supports the revised 2022 definitions of the World Health Organization (WHO) and International Consensus Classification (ICC), which shift toward molecularly defined subtypes of MDS and appropriate testing. [ABSTRACT FROM AUTHOR]

Abstract Objective To evaluate the association of weather, seasons, months and holidays on the frequency and pattern of pediatric facial fractures. Methods Retrospective review of pediatric patients treated for facial fractures at two Level I trauma centers in a midsize Midwestern US city over a 5‐year period. Patients were included only if presentation was within 3 hours of inciting trauma, transfers from other facilities were excluded. Demographic characteristics, fracture patterns, operative interventions, weather data, and local public school schedules were acquired and associations were analyzed with unpaired t tests, χ2, multivariate and binomial regression model analyses. Results Two hundred and sixty patients were included. The average age (SD) was 11.8 (5.0) years, with 173 males and 87 females. The highest distribution of presentations occurred in the summer season (35.0%), on weekends and holidays (58.1%), and when the weather was described as clear (48.5%). The most common mechanisms of injury were motor vehicle collisions (25.8%), followed by sports—(21.5%) and assault—(16.5%) related injuries. Mechanisms were significantly associated with certain fracture patterns. Older age was associated with fewer orbital fractures (P

Fostamatinib is a small molecule spleen tyrosine kinase (Syk) inhibitor that was approved for the treatment of adult patients with immune thrombocytopenia (ITP) in second-line therapy. Syk inhibition prevents cytoskeletal rearrangements during phagocytosis, allowing platelet survival in ITP. However, fostamatinib treatment in elderly patients with ITP has not been well established. We performed a retrospective review of all elderly patients (age greater than or equal to 65 years) who had started on fostamatinib for the treatment of ITP at a single tertiary care centre to evaluate its efficacy and safety. Seven patients, median age 80 years (range 78–94), four women and three men, all of Caucasian background, with various comorbidities, started fostamatinib 100 mg orally twice daily as second or subsequent line therapy. Patients had a diagnosis of ITP for a median of 6 years (range approximately 6 months–30 years), had six comorbidities (range 2–14), and experienced 2 unique prior lines of ITP therapy (range 1 to 6). Over 1290 days of fostamatinib exposure, two patients required dose escalation to 150 mg orally twice daily, while five patients remained on the initial starting dose of 100 mg twice daily. The median platelet count at the time of initiating fostamatinib was 25 × 109/L (range less than 10–193). The median time to response (defined as any first platelet count greater than or equal to 30 × 109/L) was 19 days (range 0–181 days), with two patients responding rapidly (5 days and 19 days). Two patients required dose escalation and rescue therapy, and these same two patients discontinued fostamatinib after 175 days and 216 days of treatment. Treatment was tolerated in all patients with no thromboembolic events observed. One death was noted and unrelated to treatment. Overall, fostamatinib was effective and safe for the majority of these very elderly patients with ITP.

Abstract Patients undergoing head and neck free flap reconstruction should be evaluated for radiation‐induced venous stenosis and presence of central venous port as a potential risk for flap failure.