Citations

Antisynthetase syndrome is a rare multisystem autoimmune disorder which clinically manifests with myositis, arthritis, interstitial lung disease, Raynaud phenomenon, and skin hyperkeratosis. Lung involvement represents the most severe form of disease and has rarely been reported in pregnancy. We present the case of a 22-year-old woman with antisynthetase syndrome and severe restrictive pulmonary disease who experienced a successful pregnancy and delivery. We discuss anesthetic considerations and highlight the importance of a multidisciplinary team approach in caring for parturients with multifactorial medical conditions.

Abstract Background Foot and ankle soft tissue sarcomas (STS) are challenging for reconstruction due to limited surrounding soft tissue and weight‐bearing requirements. Currently, there is a paucity of data examining the outcome of flap reconstruction following resection of a foot and ankle sarcoma. Methods We reviewed 44 (31 female, 13 male) patients with STS arising from the ankle or foot which required coverage in the form of a free ( n = 21), pedicled ( n = 13), or rotational perforator flap ( n = 10). The mean tumor size was 6 ± 5 cm, with 39 (89%) patients receiving radiotherapy. The mean follow‐up was 10 ± 7 years. Results There were no cases of amputation due to flap failure, with a 10‐year limb salvage rate of 84%. Complications occurred in 19 (43%) patients, most commonly wound infections. There was no difference in the incidence of complications between patients undergoing a free flap and pedicled or perforator flap (odds ratio = 0.97, p = 1.0); with no difference in the mean Musculoskeletal Tumor Society (MSTS) score between patients with a free flap versus a pedicled/perforator flap (84% vs. 76%, p = 0.11). Conclusion Flap reconstruction is an essential part of limb salvage for foot and ankle STS. At final follow‐up nearly all the patients are ambulatory with an acceptable MSTS score.

Background: Spontaneous coronary artery dissection (SCAD) is the principal cause of peripartum myocardial infarction (MI). Clinical features remain incompletely understood. Methods: Medical records of all women enrolled in a SCAD registry at the Mayo Clinic were reviewed to identify those who were either pregnant or ≤12 weeks postpartum at time of initial event. Diagnosis of SCAD was confirmed with coronary angiography in all cases. Obstetrical outcomes and incidence of recurrent SCAD events were assessed. Results: In total, 38 of 363 women enrolled in the registry met criteria for peripartum MI due to SCAD. Mean age at time of SCAD was 36±3 (range 27-42) years; 3 patients were pregnant at the time of initial event, and all pregnancies except 2 resulted in a live birth. Ten (26%) were diagnosed with multivessel SCAD (MV-SCAD). Most (60%) postpartum events occurred within 30 days of delivery. Antecedent co-morbidities included gestational hypertension/preeclampsia (9), fibromuscular dysplasia (7), gestational diabetes (4), and mixed connective tissue disease (1). Compared to US population birth data, women with peripartum SCAD were more likely to be ≤ 30 years at the time of first delivery (60% vs 42%) and more often multiparous (80% vs 59%). On long-term follow-up (median 3.7 years, IQR 1.1-7.1), 5 (13%) women experienced recurrent SCAD, with one episode occurring following a subsequent pregnancy. MV-SCAD was not associated with increased risk of recurrence (p=0.44). Conclusion: Women with peripartum SCAD are both older than the general obstetrical population at the time of first pregnancy and frequently multiparous at time of initial SCAD event. The highest frequency of SCAD was encountered during the first postpartum month, suggesting variations in hormonal and hemodynamic factors may contribute to SCAD events in susceptible women.

AbstractIntroductionProximal femur replacement (PFR) in the setting of tumor resection is associated with a high rate of postoperative complication. The online American College of Surgeons‐National Surgical Quality Improvement Program (ACS‐NSQIP) surgical risk calculator is approved by the Center of Medicare and Medicaid services to estimate 30‐day postoperative complications. This study was to determine if the ACS‐NSQIP can predict postoperative complications following PFR.MethodsWe reviewed 103 (61 male and 42 female) patients undergoing PFR using the Current Procedural Terminology (CPT) codes available in the calculator: 27125 (hemiarthroplasty), 27130 (total hip), 27132 (conversion to total hip), 27134 (revision total hip), 27137 (revision acetabulum), 27138 (revision femur), and 27365 (excision tumor hip). The predicted rates of complications were compared with the observed rates.ResultsComplications occurred in 54 (52%) of patients, with the predicted risk based on CPT codes: 27125 (21.5%); 27130 (7.8%); 27132 (16.6%), 27134 (17.8%), 27137 (14.4%), 274138 (22.7%), and 27365 (16.2%). The calculator was a poor predictor of complications (27125, area under the curve [AUC] 0.576); (27130, AUC 0.489); (27132, AUC 0.490); (27134, AUC 00.489); (27137, AUC 0.489); (27138, AUC 0.471); and (27365, AUC 0.538).ConclusionOncologic PFR is known for complications. The ACS‐NSQIP does not adequately predict the incidence of complications, and therefore cannot reliably be used in their shared decision‐making process preoperative.

Multiple myeloma, a malignant neoplasm of plasma cells that accumulate in bone marrow, accounts for approximately 18% of hematologic malignancies in the United States. Patients are often treated with triplet therapy and may undergo stem cell transplantation. Despite effective therapies, multiple myeloma remains incurable. Patients often require maintenance therapy, and many will progress or relapsed following upfront treatment. Selection of treatment in the relapse/refractory setting is complex due numerous active therapeutic agents and combinations. Treatment is often tailored to prior exposure and duration. In 2020, three novel pharmacological agents were approved in the relapsed setting. We highlight the clinical safety and efficacy of selinexor, isatuximab-irfc, and belantamab mafodotin for patients with multiple myeloma.

In these studies, we examined the effect of estrogen on renal inorganic phosphate (Pi) transport and the roles of its receptor isoforms (ERα and ERβ) and Klotho/FGF23 pathway in this effect in mice. Mice with null mutations (KO) of ERα or ERβ or Klotho and their wild‐type (WT) were placed in metabolic cages and injected daily with 17β‐estradiol or vehicle for 3 days. Food intake, urine volume and Pi excretion were measured daily. The results indicate that estrogen‐treated WT mice exhibited a significant increase in renal Pi wasting despite a reduction in food intake. Molecular studies showed a significant downregulation of NaPi‐IIa and NaPi‐IIc proteins with no change in their mRNA expression levels in the kidney. Estrogen‐induced reduction in food intake, phosphaturia and downregulation of NaPi‐IIa and NaPi‐IIc proteins were observed in ERβ KO but not in ERα KO mice. Further, Klotho KO mice exhibited a significant downregulation of NaPi‐IIa and hypophosphatemia in response to estrogen. In a cell line stably co‐expressing both ERα and ERβ, 24h of estrogen downregulated NaPi‐IIa protein, when cells are transiently transfected with a plasmid containing ORF‐3'UTR but not 5'UTR‐ORF of the mouse NaPi‐IIa transcript. In conclusion, estrogen causes phosphaturia in mice through the downregulation NaPi‐IIa and NaPi‐IIc protein abundance in the kidney. This effect is independent of Klotho/FGF23 pathway and is mediated through the activation of ERα. 3'UTR region of mouse NaPi‐IIa mRNA likely plays an important role in the inhibition of its translation by estrogen.

e18561 Background: Young women with breast cancer (BC) have unique issues, including fertility, genetic, and emotional health concerns; these may sometimes be inadequately addressed by providers. Given documented disparities in BC care and outcomes, we sought to explore whether provider attention to these issues differs by race, as well as to examine racial differences in satisfaction with care among young patients with BC. Methods: The Young & Strong Study was a cluster randomized trial of an educational and supportive care intervention at 14 academic + 40 community oncology practices across the US enrolling women with newly diagnosed BC at age ≤45 and their providers. Patients completed surveys at baseline, 3, 6, and 12 months after enrollment. Race was self-reported at baseline. Provider attention to fertility, genetics, and emotional health was evaluated by medical record review. The proportions of patients with attention to these concerns by 3 months was compared by race (white, Black, Asian, multi-racial/other/unknown) using Fisher’s exact tests. Satisfaction with care was assessed with the Patient Satisfaction Questionnaire-18 (PSQ-18) at 3 months. Median scores for each of 7 PSQ-18 subscales (1-5 scale, higher scores=more satisfaction) were compared by race with the Kruskal-Wallis test. Results: 465/467 (99.6%) of enrolled patients had evaluable data. 77% were white, 12% Black, 4% Asian, and 7% multiracial/other/unk. Median age at diagnosis was 40 (range: 22-45) years. Provider attention to genetics (≥85%) and emotional health (≥90%) was high across groups, and there were no differences by race in attention to fertility, genetics, or emotional health (Table). Among 359/465 women (77%) who completed the PSQ-18, median subscale scores ranged from 3.5-4.5, indicating high levels of satisfaction with care. For the PSQ-18 technical quality domain, median scores were lower (p=.03), indicating less satisfaction, in Black and Asian women (4.0) and higher in white women (4.5). Median scores for other PSQ-18 domains (general satisfaction, interpersonal manner, communication, financial, time spent with doctor, accessibility) did not differ by race. Conclusions: Satisfaction with care was high and issues related to fertility, genetics and emotional health were addressed by providers in the majority of young women in our study, with minimal differences by race. These patients were all enrolled in a clinical trial and had access to high-quality care, limiting the generalizability of our findings. Larger, population-based studies in more diverse settings are warranted. Clinical trial information: NCT01647607. Attention to psychosocial concerns by race. [Table: see text]

Introduction Antipsychotics are a treatment option for delirium in the intensive care unit. Atypical antipsychotics are preferred over first-generation antipsychotics because of their lower incidence of extrapyramidal adverse effects. The most common such effect is akathisia or restlessness. This report describes a case of atypical antipsychotic–induced akathisia and addresses the clinical distinction between extrapyramidal movements and movements due to intensive care unit delirium. Clinical Findings A 56-year-old man who had a prolonged hospital stay after orthotopic liver transplant complicated by multisystem organ failure, primary graft failure requiring a second transplant, and enterocutaneous fistula developed agitated delirium on hospital day 28. Initial treatment included intravenous haloperidol and scheduled sublingual olanzapine (5 mg daily). His delirium and insomnia persisted, requiring dexmedetomidine infusion. Olanzapine dosing was increased to 10 mg daily on hospital day 34 and 15 mg daily on hospital day 45. The following day, his mentation improved; however, he exhibited asynchronous, nonrhythmic, involuntary rolling motions of his hands and choreiform gait. Diagnosis and Outcomes Antipsychotics were immediately discontinued owing to acute akathisia. All symptoms resolved within 2 days, and the patient was transferred out of the intensive care unit on hospital day 52. Conclusion Although extrapyramidal adverse effects are less common with olanzapine than with typical antipsychotics, they sometimes occur and can mimic manifestations of delirium. Restlessness should alert the nurse to assess for possible extrapyramidal adverse effects. If they are suspected, antipsychotic medications should be reduced or discontinued to prevent progression to functional disability.

INTRODUCTION: Using standard anterior-only or anterior then posterior approaches can make an R0 resection difficult to achieve in patients with pelvic sidewall recurrences because of confined working spaces and poor visibility. TECHNIQUE: Given the limitations of standard approaches, we have used a novel posterior-first then anterior 2-stage approach allowing us to widely expose and secure deep margins and control vessels under direct visualization. RESULTS: We present a technical note describing this approach in patients with recurrent rectal cancer involving the pelvic sidewall with extrapelvic extension. CONCLUSION: The posterior-first approach may assist in achieving a higher number of R0 resections in patients with locally recurrent rectal cancer involving the pelvic sidewall.