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Open Access Publications from the University of California

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This series is automatically populated with publications deposited by UC San Diego School of Medicine Department of Medicine researchers in accordance with the University of California’s open access policies. For more information see Open Access Policy Deposits and the UC Publication Management System.

Cover page of Anakinra-responsive lichen planus in a woman with Erdheim-Chester disease: a therapeutic enigma

Anakinra-responsive lichen planus in a woman with Erdheim-Chester disease: a therapeutic enigma

(2014)

Background:  Anakinra is a recombinant form of interleukin-1 receptor antagonist.  It is the drug of choice for Schnitzler syndrome and cryopyrin-associated periodic syndromes.  It has also recently been demonstrated to have activity in the treatment of the non-Langerhans cell histiocytosis known as Erdheim-Chester disease.

Purpose:  To describe the activity of anakinra in a patient with co-existing lichen planus and Erdheim-Chester disease.

Methods:  A 43-year-old woman with progressive Erdheim-Chester disease presented for management of her night sweats and chills, systemic skeletal bone pain, and neurologic (diabetes insipidus) manifestations.  She also had widespread cutaneous lichen planus.  Anakinra, 100 mg subcutaneously daily, was initiated for the treatment of her Erdheim-Chester disease.

Results:  Within 2 days of starting anakinra, there was prompt resolution of her Erdheim-Chester disease-related symptoms.  Subsequently, her bone pain resolved and her diabetes insipidus improved.  Also, the lichen planus-associated pruritus rapidly ceased and most of the skin lesions improved.

Conclusions:  Our experience confirms the efficacy of anakinra for the treatment of Erdheim-Chester disease.  The concomitant improvement of her lichen planus on anakinra suggests that this agent warrants additional study in this disorder.

Cover page of Imatinib-induced postoperative periorbital purpura: GASP (Gleevec-Associated Surgical Purpura) in a woman with imatinib-treated chronic myelogenous leukemia

Imatinib-induced postoperative periorbital purpura: GASP (Gleevec-Associated Surgical Purpura) in a woman with imatinib-treated chronic myelogenous leukemia

(2014)

Background:  Imatinib mesylate is a selective tyrosine kinase inhibitor used in the treatment of chronic myelogenous leukemia.  Ocular side effects of imatinib include periorbital edema, which may become so severe as to obstruct the visual field.

Purpose: The purpose of this case study is to describe the clinical characteristics of imatinib- induced postoperative periorbital purpura.

Materials and methods: We retrospectively reviewed the medical literature using PubMed, searching the terms edema, Gleevec, imatinib, periorbital, postoperative and purpura.  Patient reports and previous reviews of the subject were critically assessed and the salient features are presented.

Results: Three patients have undergone surgery to reduce the imatinib-induced periorbital edema; two of these individuals have developed imatinib-induced postoperative periorbital purpura.

Conclusion:  We recommend discontinuing imatinib usage one week prior to periorbital surgery and not resuming therapy until the eighth postoperative day.

Cover page of Disparities Exist in the Application of Low Tidal-volume Ventilation in the Emergency Department

Disparities Exist in the Application of Low Tidal-volume Ventilation in the Emergency Department

(2023)

Introduction: Low tidal-volume ventilation (LTVV), defined as a maximum tidal volume of 8 milliliters per kilogram (mL/kg) of ideal body weight, is a key component of lung protective ventilation. Although emergency department (ED) initiation of LTVV has been associated with improved outcomes, disparities in LTVV application exist. In this study our aim was to evaluate whether rates of LTVV are associated with demographic and physical characteristics in the ED.

Methods: We conducted a retrospective observational cohort study using a dataset of patients who underwent mechanical ventilation at three EDs in two health systems from January 2016–June 2019. Demographic, mechanical ventilation, and outcome data including mortality and hospital-free days were abstracted by automatic query. A LTVV approach was defined as a tidal volume ≤8 mL/kg ideal body weight. We performed descriptive statistics and univariate analysis as indicated, and created a multivariate logistic regression model.

Results: Of 1,029 patients included in the study, 79.5% received LTVV. Tidal volumes of 400-500 mL were used in 81.9% of patients. Approximately 18% of patients had tidal volumes changed in the ED. Female gender (adjusted odds ratio [aOR] 4.17, P< 0.001), obesity (aOR 2.27, P< 0.001), and first-quartile height (aOR 12.2, P < 0.001) were associated with receiving non-LTVV in multivariate regression analysis. Hispanic ethnicity and female gender were associated with first quartile height (68.5%, 43.7%, P < 0.001 for all). Hispanic ethnicity was associated with receiving non-LTVV in univariate analysis (40.8% vs 23.0%, P < 0.001). This relationship did not persist in sensitivity analysis controlling for height, weight, gender, and body mass index. Patients who received LTVV in the ED had 2.1 more hospital-free days compared to those who did not (P = 0.040). No difference in mortality was observed.

Conclusion: Emergency physicians use a narrow range of initial tidal volumes that may not meet lung-protective ventilation goals, with few corrections. Female gender, obesity, and first-quartile height are independently associated with receiving non-LTVV in the ED. Using LTVV in the ED was associated with 2.1 fewer hospital-free days. If confirmed in future studies, these findings have important implications for achieving quality improvement and health equality.

 

Cover page of Anti-N-Methyl-D-Aspartate Receptor Encephalitis, an Underappreciated Disease in the Emergency Department

Anti-N-Methyl-D-Aspartate Receptor Encephalitis, an Underappreciated Disease in the Emergency Department

(2016)

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis is a novel disease discovered within the past 10 years. Antibodies directed at the NMDAR cause the patient to develop a characteristic syndrome of neuropsychiatric symptoms. Patients go on to develop autonomic dysregulation and often have prolonged hospitalizations and intensive care unit stays. There is little literature in the emergency medicine community regarding this disease process, so we report on a case we encountered in our emergency department to help raise awareness of this disease process.

Cover page of Telephone CPR Instructions in Emergency Dispatch  Systems: Qualitative Survey of 911 Call Centers

Telephone CPR Instructions in Emergency Dispatch Systems: Qualitative Survey of 911 Call Centers

(2015)

Introduction: Out-of-hospital cardiac arrest (OHCA) is a leading cause of death. The 2010 American Heart Association Emergency Cardiovascular Care (ECC) Guidelines recognize emergency dispatch as an integral component of emergency medical service response to OHCA and call for all dispatchers to be trained to provide telephone cardiopulmonary resuscitation (T-CPR) pre-arrival instructions. To begin to measure and improve this critical intervention, this study describes a nationwide survey of public safety answering points (PSAPs) focusing on the current practices and resources available to provide T-CPR to callers with the overall goal of improving survival from OHCA.

Methods: We conducted this survey in 2010, identifying 5,686 PSAPs; 3,555 had valid e-mail addresses and were contacted. Each received a preliminary e-mail announcing the survey, an e-mail with a link to the survey, and up to three follow-up e-mails for non-responders. The survey contained 23 primary questions with sub-questions depending on the response selected.

Results: Of the 5,686 identified PSAPs in the United States, 3,555 (63%) received the survey, with 1,924/3,555 (54%) responding. Nearly all were public agencies (n=1,888, 98%). Eight hundred seventy-eight (46%) responding agencies reported that they provide no instructions for medical emergencies, and 273 (14%) reported that they are unable to transfer callers to another facility to provide T-CPR. Of the 1,924 respondents, 975 (51%) reported that they provide pre-arrival instructions for OHCA: 67 (3%) provide compression-only CPR instructions, 699 (36%) reported traditional CPR instructions (chest compressions with rescue breathing), 166 (9%) reported some other instructions incorporating ventilations and compressions, and 92 (5%) did not specify the type of instructions provided. A validation follow up showed no substantial difference in the provision of instructions for OHCA by non-responders to the survey.

Conclusion: This is the first large-scale, nationwide assessment of the practices of PSAPs in the United States regarding T-CPR for OHCA. These data showing that nearly half of the nation’s PSAPs do not provide T-CPR for OHCA, and very few PSAPs provide compression-only instructions, suggest that there is significant potential to improve the implementation of this critical link in the chain of survival for OHCA.

 

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Cover page of Lessons Learned From the Liver About the Undergraduate to Graduate Medical Education Transition.

Lessons Learned From the Liver About the Undergraduate to Graduate Medical Education Transition.

(2025)

The burden of cirrhosis and chronic liver disease is growing, yet there is a projected worsening deficit in hepatology providers. As such, cirrhosis and liver disease have been important inclusions within the core curricula of Internal Medicine. Formal assessments of provider preparedness resulting from the curriculum are lacking though. Prior studies have demonstrated that exposure to cirrhosis in undergraduate medical education is insufficient, as are learner comfort and self-reported knowledge levels. These findings are further corroborated by a multicenter survey of incoming Internal Medicine interns showing that subjective comfort with and objective knowledge of various liver disease topics are lacking compared to other common Internal Medicine topics. This paper also demonstrates how similar surveys may be used to identify additional topics that may require adjustments for curricular improvement.

Cover page of Lipoprotein(a), high-sensitivity c-reactive protein, homocysteine and cardiovascular disease in the Multi-Ethnic Study of Atherosclerosis.

Lipoprotein(a), high-sensitivity c-reactive protein, homocysteine and cardiovascular disease in the Multi-Ethnic Study of Atherosclerosis.

(2025)

BACKGROUND AND AIMS: Elevated lipoprotein(a) [Lp(a)], high-sensitivity C-Reactive Protein (hs-CRP), and total homocysteine (tHcy) are associated with atherosclerotic cardiovascular disease (ASCVD) risk. This study investigated the individual and joint associations of Lp(a), hs-CRP and tHcy with coronary heart disease (CHD) and stroke. METHODS: This study was conducted in the Multi-Ethnic Study of Atherosclerosis (MESA) cohort (2000-2017) (CHD analytic N = 6,676; stroke analytic N = 6,674 men and women). Associations between Lp(a) (<50 vs. ≥50 mg/dL), hs-CRP (<2 vs. ≥2 mg/L) and tHcy (<12 vs. ≥12 µmol/L) and CHD and stroke incidence were evaluated individually and jointly using Cox proportional hazards regression. RESULTS: Individually, elevated tHcy was associated with CHD and stroke incidence, Lp(a) with CHD only and hs-CRP with stroke only. In combined analyses, CHD risk was higher when multiple biomarkers were elevated [hs-CRP+Lp(a), hazard ratio (HR)=1.39, 95 % confidence interval (CI): 1.06, 1.82; hs-CRP+ tHcy, HR = 1.34, 95 % CI: 1.02, 1.75; Lp(a)+ tHcy HR = 1.58, 95 % CI: 1.08, 2.30; hs-CRP+Lp(a)+ tHcy HR = 2.02, 95 % CI: 1.26, 3.24]. Stroke risk was elevated when hs-CRP and either Lp(a) (HR = 1.51, 95 % CI: 1.02, 2.23) or tHcy (HR = 2.10, 95 % CI: 1.44, 3.06) was also high, when all three biomarkers were elevated (HR = 2.99, 95 % CI: 1.61, 5.58), or when hs-CRP and tHcy (HR = 1.79, 95 % CI: 1.16, 2.76) were both high. CONCLUSIONS: Risk of ASCVD was highest with concomitant elevation of tHcy, hs-CRP and Lp(a). Inclusion of tHcy and consideration of biomarker combination rather than individual biomarker levels may help better identify individuals at greatest risk for ASCVD events.

Genetic analysis of elevated levels of creatinine and cystatin C biomarkers reveals novel genetic loci associated with kidney function

(2025)

The rising prevalence of chronic kidney disease (CKD), affecting an estimated 37 million adults in the United States, presents a significant global health challenge. CKD is typically assessed using estimated Glomerular Filtration Rate (eGFR), which incorporates serum levels of biomarkers such as creatinine and cystatin C. However, these biomarkers do not directly measure kidney function; their elevation in CKD results from diminished glomerular filtration. Genome-wide association studies (GWAS) based on eGFR formulas using creatinine (eGFRcre) or cystatin C (eGFRcys) have identified distinct non-overlapping loci, raising questions about whether these loci govern kidney function or biomarker metabolism. In this study, we show that GWAS on creatinine and cystatin C levels in healthy individuals reveal both nonoverlapping genetic loci impacting their metabolism as well as overlapping genetic loci associated with kidney function; whereas GWAS on elevated levels of these biomarkers uncover novel loci primarily associated with kidney function in CKD patients.

Cover page of Distributed cross-learning for equitable federated models - privacy-preserving prediction on data from five California hospitals.

Distributed cross-learning for equitable federated models - privacy-preserving prediction on data from five California hospitals.

(2025)

Quality improvement, clinical research, and patient care can be supported by medical predictive analytics. Predictive models can be improved by integrating more patient records from different healthcare centers (horizontal) or integrating parts of information of a patient from different centers (vertical). We introduce Distributed Cross-Learning for Equitable Federated models (D-CLEF), which incorporates horizontally- or vertically-partitioned data without disseminating patient-level records, to protect patients privacy. We compared D-CLEF with centralized/siloed/federated learning in horizontal or vertical scenarios. Using data of more than 15,000 patients with COVID-19 from five University of California (UC) Health medical centers, surgical data from UC San Diego, and heart disease data from Edinburgh, UK, D-CLEF performed close to the centralized solution, outperforming the siloed ones, and equivalent to the federated learning counterparts, but with increased synchronization time. Here, we show that D-CLEF presents a promising accelerator for healthcare systems to collaborate without submitting their patient data outside their own systems.

Cover page of Compassion and equity-focused clinical genomics training for health professional learners.

Compassion and equity-focused clinical genomics training for health professional learners.

(2025)

There remains an urgent need for expanded genomics training in undergraduate medical education, especially as genetic and genomic assessments become increasingly important in primary care and routine clinical practice across specialties. Physician trainees continue to report feeling poorly prepared to provide effective consultation or interpretation of genomic test results. Here we report on the development, pilot implementation, and evaluation of an elective offering for pre-clinical medical students called the Sanford Precision Health Scholars Immersive Learning Experience (PHS), which was designed leveraging genetic counseling expertise as one means to address this need. This 9-week course, piloted in Fall 2021 at UC San Diego, afforded students the opportunity to build technical skills and competencies in clinical genomics while identifying, addressing, and engaging with pervasive health disparities in genomics. Interactive exercises focused students learning on strategies for empathic and compassionate patient interactions while supporting the application of concepts and knowledge to future practice. Upon completion of the course, participants reported increases in confidence related to skills required for clinical genomics practice. Drawing on learnings from this pilot implementation, recommendations for refining the program include deepening pedagogical engagement with ethical issues, expanding the offering to trainees across health professions, including pharmacy students, and incorporating an optional experiential learning component. Educational offerings, like PHS, that are designed with the input of genetic counseling expertise may ease pressures on the genetic counseling profession by building a more genomic-literate healthcare workforce that can better support efforts to expand access for patients.