Oral Premalignant and Malignant Lesions in Fanconi Anemia Patients.

OBJECTIVE: There is a lack of data supporting cancer surveillance in pediatric Fanconi Anemia patients. We sought to describe the rates of upper aerodigestive lesions and malignancy in this population to augment current management guidelines. METHODS: A retrospective cohort study of patients with Fanconi Anemia from a quaternary referral center between 2007-2021 was completed for head and neck cancer risk. RESULTS: One hundred and five FA patients were reviewed. Average age at presentation was 11.3 years old and 90.5% of patients underwent hematopoietic stem cell transplant (HSCT). A total of 8.6% of patients had leukoplakia or erythroplakia and 3.8% developed malignancy. The standardized incidence ratio of head and neck malignancy was 483.8. Patients presented with leukoplakia and malignancy at an average age of 14.6 and 25.1 years old, respectively. Malignancies were aggressive and marked by recurrence. There were no premalignant or malignant lesions found on flexible laryngoscopy. This series represents the largest longitudinal series of pediatric FA head and neck lesions. CONCLUSIONS: Fanconi Anemia patients should begin screening for head and neck cancer at age 10 or after HSCT. LEVEL OF EVIDENCE: Level 4 Laryngoscope, 133:1745-1748, 2023.

Attitudes Among General Surgery Program Directors and Faculty Toward Openly Nonheterosexual Applicants.

OBJECTIVE: Historically, nonheterosexual physicians have experienced bias in medical training. While resident-focused studies have been conducted in general surgery, the views of program directors and faculty are less evident. In this study, we surveyed program directors and faculty in general surgery to assess their attitudes toward openly nonheterosexual residency applicants. DESIGN: A national, cross-sectional online survey. PARTICIPANTS: Program directors and faculty in general surgery listed on the Association of Program Directors in Surgery listserv. RESULTS: Of the 123 participants who returned the survey; 33% were faculty and 58% were program directors. The response rate was 28% for program directors and 13% for faculty. Of respondents, 68% reported having openly nonheterosexual residents in their program and 38% were aware the candidates were nonheterosexual when they ranked them. Most respondents (76%) would advise a nonheterosexual mentee to be honest about their sexuality if asked during the interview and application process for general surgery (Figure 1). Of respondents, 84% reported that knowing an applicant was nonheterosexual would not affect how they ranked them while 76% reported that their program's faculty would always feel comfortable if a nonheterosexual resident brought their partner to a residency social event. CONCLUSIONS: To our knowledge, this is the first survey of general surgery program directors regarding their attitudes toward nonheterosexual residency applicants. While a few individuals continue to hold biased beliefs, there appears to be substantial acceptance of nonheterosexual general surgery residency candidates amongst program directors and faculty who responded to our survey.

Outcomes of Patients Undergoing Interfacility Extracorporeal Membrane Oxygenation Transfer Based on Cannulation Location and Mode of Transport.

As the use of extracorporeal membrane oxygenation (ECMO) expands, so has the need for interfacility transfer to ECMO centers. However, the impact of these transfers has not been fully studied. This study evaluates complications and inhospital mortality in adult patients treated with venovenous (V-V) ECMO based on institutional location of cannulation and mode of transport. DESIGN: Retrospective cohort study. SETTING: Large midwestern ECMO center. PATIENTS: Adult patients receiving VV-ECMO. INTERVENTIONS: Need for transfer to ECMO center following VV-ECMO cannulation. MEASUREMENTS AND MAIN RESULTS: The study included 102 adult patients, 57% of which were cannulated at an outside institution prior to transfer. Of these, 60% were transported by ground, and the remainder were transported by air. Risk-adjusted logistic regression did not reveal any significant increase in odds for any complication or inhospital mortality between the groups based on location of cannulation or mode of transport. CONCLUSIONS: This study supports the practice of interfacility ECMO transfer with no difference in outcomes or inhospital mortality based on institutional location of cannulation or mode of transport.

Operationalizing Ethical Guidance for Ventilator Allocation in Minnesota: Saving the Most Lives or Exacerbating Health Disparities?

A statewide working group in Minnesota created a ventilator allocation scoring system in anticipation of functioning under a Crisis Standards of Care declaration. The scoring system was intended for patients with and without coronavirus disease 2019. There was disagreement about whether the scoring system might exacerbate health disparities and about whether the score should include age. We measured the relationship of ventilator scores to in-hospital and 3-month mortality. We analyzed our findings in the context of ethical and legal guidance for the triage of scarce resources. DESIGN: Retrospective cohort study. SETTING: Multihospital within a single healthcare system. PATIENTS: Five-hundred four patients emergently intubated and admitted to the ICU. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The Ventilator Allocation Score was positively associated with higher mortality (p < 0.0001). The 3-month mortality rate for patients with a score of 6 or higher was 96% (42/44 patients). Age was positively associated with mortality. The 3-month mortality rate for patients 80 and older with scores of 4 or greater was 93% (40/43 patients). Of patients assigned a score of 5, those with end stage renal disease had lower mortality than patients without end stage renal disease although the difference did not achieve statistical significance (n = 27; 25% vs 58%; p = 0.2). CONCLUSIONS: The Ventilator Allocation Score can accurately identify patients with high rates of short-term mortality. However, these high mortality patients only represent 27% of all the patients who died, limiting the utility of the score for allocation of scarce resources. The score may unfairly prioritize older patients and inadvertently exacerbate racial health disparities through the inclusion of specific comorbidities such as end stage renal disease. Triage frameworks that include age should be considered. Purposeful efforts must be taken to ensure that triage protocols do not perpetuate or exacerbate prevailing inequities. Further work on the allocation of scarce resources in critical care settings would benefit from consensus on the primary ethical objective.

Transcriptomic analysis of gene signatures associated with sickle pain.

Pain is a hallmark feature of sickle cell disease (SCD). Recurrent and unpredictable acute pain due to vaso-oclussive crises (VOC) is unique to SCD; and can be superimposed on chronic pain. To examine the mechanisms underlying pain in SCD, we performed RNA sequencing of dorsal root ganglion (DRG) of transgenic sickle mice and their age-matched control mice expressing normal human hemoglobin A, at 2 and 5 months of age. Sickle and control mice of both ages were equally divided into hypoxia/reoxygenation (to simulate VOC) and normoxia treatment, resulting in eight groups of mice. Each group had at least six mice. RNA isolated from the DRG was sequenced and paired-end 50 bp sequencing data were generated using Illumina's HiSeq 2000. This large dataset can serve as a resource for examining transcriptional changes in the DRG that are associated with age and hypoxia/reoxygenation associated signatures of nociceptive mechanisms underlying chronic and acute pain, respectively.