Initial Clinical Presentation Research Articles

Is the Proof in the Pain? Association between Head and Neck Pain and Vessel Pathology at Follow-Up in Cervical Artery Dissection: A Retrospective Data Analysis

Unilateral head and neck pain is a hallmark of cervical artery dissection (CAD). While pain is conceived as an alarming sign for patients and often leads to discovery of the dissection, it is not known if persistence of pain is associated with the course of CAD. Potentially, pain could indicate persisting vessel pathology and thus guide treatment decisions aimed at reducing risk of ischemic stroke in CAD. We performed a retrospective analysis of data from patients with CAD treated at the University Hospital Zurich (USZ). Only patients with information about the presence of pain, independence after CAD according to the modified Rankin scale (mRS), and imaging-based information on vessel status were included. Patients were grouped according to presence/absence of head and/or neck pain on admission and at a three-month follow-up. We used descriptive statistics and logistic regression to reveal a potential association between pain on admission and pain at follow-up with status of the dissected vessel at follow-up (open vs. stenosed or occluded). We screened 139 patients with CAD between 2014 and 2019 and included 68. Fifty-nine patients (86.8%) had pain on admission, which was resolved in 46 (68%) at follow-up. Our post hoc analysis revealed that more patients with headache or neck pain on admission had a migraine diagnosis in medical history (n = 7 (10.4%) vs. n = 0 (0%), p = 0.029) and that NIHSS on admission was higher in patients with no pain at presentation (group B NIHSS = 3, IQR 8 vs. group A NIHSS = 2, IQR 5, group C NIHSS = 0, IQR 2, p = 0.041). There were no other differences between the three patient groups in the descriptive analysis. Logistic regression analysis for vessel status at follow-up did not show an association with pain on admission or at follow-up. In our cohort of patients with CAD, headache was a common initial clinical presentation, which rarely persisted for three months. Headache on admission or at follow-up did not predict persisting vessel pathology in patients with CAD.

Adjunctive hemoperfusion with Resin Hemoadsorption (HA) 330 cartridges improves outcomes in patients sustaining multiple Blunt Trauma: a prospective, quasi-experimental study

BackgroundMulti-organ dysfunction syndrome and multi-organ failure are the leading causes of late death in patients sustaining severe blunt trauma. So far, there is no established protocol to mitigate these sequelae. This study assessed the effect of hemoperfusion using resin-hemoadsorption 330 (HA330) cartridges on mortality and complications such as acute respiratory distress syndrome (ARDS) and systemic inflammatory response syndrome (SIRS) among such patients.MethodsThis quasi-experimental study recruited patients ≥ 15 years of age with blunt trauma, injury severity score (ISS) ≥ 15, or initial clinical presentation consistent with SIRS. They were divided into two groups: the Control group received only conventional acute care, while the case group received adjunctive hemoperfusion. P-values less than 0.05 were statistically significant.ResultsTwenty-five patients were included (Control and Case groups: 13 and 12 patients). The presenting vital signs, demographic and injury-related features (except for thoracic injury severity) were similar (p > 0.05). The Case group experienced significantly more severe thoracic injuries than the Control group (Thoracic AIS, median [IQR]: 3 [2–4] vs. 2 [0–2], p = 0.01). Eleven and twelve patients in the Case group had ARDS and SIRS before the hemoperfusion, respectively, and these complications were decreased considerably after hemoperfusion. Meanwhile, the frequency of ARDS and SIRS did not decrease in the Control group. Hemoperfusion significantly reduced the mortality rate in the Case group compared to the Control group (three vs. nine patients, p = 0.027).ConclusionsAdjunctive Hemoperfusion using an HA330 cartridge decreases morbidity and improves outcomes in patients suffering from severe blunt trauma.

Clinical characteristics of palmoplantar discoid lupus erythematosus: A multicenter retrospective review of 43 patients

AbstractBackgroundPalmoplantar discoid lupus erythematosus (ppDLE) is a rare manifestation of chronic cutaneous lupus erythematosus that can result in painful, hyperkeratotic, and/or erosive lesions on the palms and soles. Despite this penchant to cause significant morbidity, the natural history of ppDLE and its response to conventional treatment approaches remain poorly understood.ObjectivesTo characterize the initial presentation, treatment response, and association with systemic lupus erythematosus (SLE) of ppDLE.MethodsWe performed an International Classification of Diseases (ICD‐9 and ICD‐10) code and natural‐language query for medical records from Brigham and Women's Hospital and Massachusetts General Hospital to identify all cases of ppDLE based on biopsy and/or expert opinion from January 2000 through October 2022. Demographics, clinical features, and treatment data were analysed.ResultsForty‐three patients (median age, 29.5 years [range, 8−70 years]; 36 female [83.7%]) with ppDLE were identified. Mean time from initial clinical presentation of ppDLE symptoms to diagnosis was 14.5 months (n = 34). Of the 43 patients, 74.4% developed coexistent SLE. A mean of 5.9 (standard deviation, 2.7) lines of therapy were required to achieve control of ppDLE, with the majority of patients requiring the initiation of at least two concomitant systemic medications.ConclusionsHerein, we present the largest cohort of patients with ppDLE in the literature to date. Despite a high incidence of coexistent SLE, patients experienced lengthy diagnostic delays. Even with appropriate diagnosis, an overwhelming trend towards treatment‐refractory disease was observed.

Impact of the COVID-19 Pandemic on the Number, Clinical Characteristics, Surgical Types, and Anatomical Outcome of Patients with Primary Rhegmatogenous Retinal Detachment during and after COVID-19 Lockdown in Thailand

Background: During lockdown in 2020 from the outbreak of coronavirus disease 2019 (COVID-19), rhegmatogenous retinal detachment (RRD) has been affected in several aspects including prevalence alteration, delayed presentations, and poorer treatment outcomes. These effects are unknown after lockdown by comparing with the preceding year. Objective: To determine the impact of the COVID-19 pandemic on the number, clinical characteristics, type of surgical procedure, and anatomical outcome of primary RRD during and after lockdown compared with those parameters in the same periods of the previous year. Materials and Methods: In the present retrospective cohort study, the medical records of patients with primary RRD underwent retinal surgery at Mettapracharak Hospital during and after the first lockdown in 2020 and the corresponding period in 2019 were reviewed. These four periods had an equal number of days as the first lockdown period. The following data were analyzed, baseline demographics, initial clinical presentations, type of surgical procedure, and outcome. Results: Four hundred fifty-five patients, for 455 eyes, underwent surgery for primary RRD. One hundred seven patients were treated during lockdown, 106 patients after lockdown, whereas 117 patients and 125 patients were treated in identical periods in the previous year, respectively. A decrease of 8.5% of RRD cases during lockdown and of 15.2% of RRD cases post-lockdown were documented. No significant differences were found with respect to demographic features, clinical characteristics, type of surgical procedure, or the anatomical single surgery success rate of RRD patients among all time periods. However, a significantly lower prevalence of right-eye involvement and shorter waiting time for surgery after lockdown were documented. Conclusion: The authors revealed the impact of the COVID-19 pandemic on the reduction in the number of surgical procedures for primary RRD during and after lockdown. Our findings could aid redefinition of a strategic plan for RRD management after the COVID-19 pandemic had subsided. Keywords: Rhegmatogenous retinal detachment; COVID-19; Pandemic; Lockdown; Tertiary hospital; Eye

Fatal gastric volvulus: forensic pathology considerations and postmortem CT findings

Gastric volvulus is a rare cause of gastric obstruction, due to the rotation of the stomach by more than 180°. It is a rare but life-threatening medical emergency that is considered difficult to diagnose at the initial clinical presentation. Forensic pathologists may be presented with gastric volvulus in several ways, for instance, as a cause of sudden and unexpected death or in the context of suspected clinical errors. The post-mortem examination of gastric volvulus may be challenging, due to the specific technical issues it presents and the various mechanisms by which volvulus may cause death. We therefore present five cases of gastric volvulus that in combination represent almost the entire spectrum of presentations and post-mortem findings, to discuss how gastric volvulus may come to the attention of a forensic pathologist, the approach and findings at post-mortem examination (including post-mortem CT), and the variety of mechanisms by which gastric volvulus may result in death.

Multimodal AI for prediction of distant metastasis in carcinoma patients.

Metastasis of cancer is directly related to death in almost all cases, however a lot is yet to be understood about this process. Despite advancements in the available radiological investigation techniques, not all cases of Distant Metastasis (DM) are diagnosed at initial clinical presentation. Also, there are currently no standard biomarkers of metastasis. Early, accurate diagnosis of DM is however crucial for clinical decision making, and planning of appropriate management strategies. Previous works have achieved little success in attempts to predict DM from either clinical, genomic, radiology, or histopathology data. In this work we attempt a multimodal approach to predict the presence of DM in cancer patients by combining gene expression data, clinical data and histopathology images. We tested a novel combination of Random Forest (RF) algorithm with an optimization technique for gene selection, and investigated if gene expression pattern in the primary tissues of three cancer types (Bladder Carcinoma, Pancreatic Adenocarcinoma, and Head and Neck Squamous Carcinoma) with DM are similar or different. Gene expression biomarkers of DM identified by our proposed method outperformed Differentially Expressed Genes (DEGs) identified by the DESeq2 software package in the task of predicting presence or absence of DM. Genes involved in DM tend to be more cancer type specific rather than general across all cancers. Our results also indicate that multimodal data is more predictive of metastasis than either of the three unimodal data tested, and genomic data provides the highest contribution by a wide margin. The results re-emphasize the importance for availability of sufficient image data when a weakly supervised training technique is used. Code is made available at: https://github.com/rit-cui-lab/Multimodal-AI-for-Prediction-of-Distant-Metastasis-in-Carcinoma-Patients.

Orbital abscess with white eye in patients treated with oral antibiotics for orbital cellulitis

ABSTRACT Purpose To report a case series of seven patients with late presentation of orbital/subperiosteal abscess following oral treatment of orbital cellulitis. Methods A retrospective case series of all patients presented with orbital abscess following oral treatment of orbital cellulitis from two tertiary-care eye centres in Riyadh, Saudi Arabia, was conducted. Demographic profiles, risk factors, initial clinical presentation, management regimens, and final outcome were analysed. Results Patients presented mainly with proptosis and limitation of extraocular motility without external ophthalmic inflammatory signs. Most patients needed surgical evacuation despite the appropriate initiation of intravenous antibiotics following presentation to our hospitals. Conclusion Treating orbital cellulitis with oral antibiotics may lead to delayed presentation of orbital abscess without external ophthalmic inflammatory signs.

External validation of the ORBI risk score for cardiogenic shock prediction in patients with ST-segment elevation myocardial infarction

Abstract Funding Acknowledgements Type of funding sources: None. Background Cardiogenic shock (CS) complicates 5 to 15 % of ST-segment elevation myocardial infarction (STEMI) cases. With a high-short term fatality rate estimated between 40 to 50 %, it is considered as the first cause of mortality in myocardial infarction. Early detection of patients in the state of "pre-CS" may improve prognosis as it makes it possible to adapt the monitoring and to establish an appropriate treatment at an earlier stage. The ORBI risk score (ORS) based on 11 variables was created in order to identify patients at high-risk of in-hospital STEMI-related CS but external validation is lacking. Aim The primary objective of this study was to evaluate the external validity of the ORBI Risk Score in a Tertiary Hospital Centre in southern France. Methods Consecutive STEMI patients managed by primary percutaneous coronary intervention (< 24 h after chest-pain onset) at our tertiary hospital in 2019 were retrospectively included. Patients with CS at admission, with previous fibrinolysis or without attempted p-PCI were excluded. Data necessary for the calculation of the ORBI risk score were collected (age, prior stroke/transient ischaemic attack, cardiac arrest upon admission, type of STEMI, first medical contact-to-pPCI delay, initial clinical presentation (Killip class, heart rate, blood pressure, glycaemia, culprit lesion, and post-pPCI TIMI-flow grade and the occurrence of in-hospital CS). The score's calibration was assessed by a graphical method and its discriminatory performance by calculating the C-statistic (ROC curve). Results 287 patients were included (mean age 64 y.o, 81.2% male, anterior STEMI 40.8%, LAD culprit lesion 47%, median first medical contact-to-PCI delay 112 min). In-hospital CS occurred in 18 patients (6.3%) with all-cause in-hospital mortality of 4.2%. The observed in-hospital CS rates were respectively 1.8%, 2.9%, 41.7%, and 50 % in the four risk categories, ranging from low ( ORS 0–7) to low- to-intermediate (ORS 8–10), and from intermediate-to-high (ORS 11–12) to high (ORS≥13). The score showed satisfactory discrimination (c-statistic of 0.90 (CI 95% 0.83–0.97), p<0.001) with 93 % correctly classified patients at a cut-off ≥ 10. However, in term of calibration, the score tends to under-estimate the occurrence of CS, mostly in high-risk patients. Conclusion The ORBI risk score demonstrated good discrimination, but it lacked calibration in the case of this validation cohort. Notwithstanding this drawback, it may facilitate the identification of STEMI patients at high risk of CS and thus increase their enrolment in CS dedicated clinical trials. Further validation studies are needed before scaling up its routinely clinical use.

External validation of the ORBI risk score for cardiogenic shock prediction in patients with ST elevation myocardial infarction

Abstract Funding Acknowledgements Type of funding sources: Public hospital(s). Main funding source(s): CHU de Toulouse. Background Cardiogenic shock (CS) complicates 5 to 15 % of ST-segment elevation myocardial infarction (STEMI) and is associated with high short-term mortality (up to 40-50%). The ORBI risk score (ORS) based on 11 variables was created in order to identify patients at high-risk of in-hospital STEMI-related CS but external validation is lacking. Purpose To externally validate the ORBI risk score. Methods Consecutive STEMI patients managed by percutaneous coronary intervention (< 24 h after chest-pain onset) in our tertiary hospital in 2019 were retrospectively included. Patients with CS at admission, with previous fibrinolysis or without PCI attempted were excluded. Data necessary for the calculation of the ORBI risk score were collected (age, prior stroke/transient ischaemic attack, cardiac arrest upon admission, type of STEMI, first medical contact-to-pPCI delay, initial clinical presentation, Killip class, heart rate, blood pressure, glycaemia, culprit lesion, and post-pPCI TIMI-flow grade) and the occurrence of in-hospital CS. Then we have tested the discrimination and the calibration of the score based of ROC curves and C-statistic analysis. Results 287 patients were included (age 64 yo, 81.2% male, anterior STEMI 40.8%, LAD culprit lesion 47%, median first medical contact-to-PCI delay 112 min). The median ORBI-risk score was 4 (IQR 2-7) with 78% patients considered at low (ORS 0-7), 12.2% intermediate-low (ORS 8-10), 4.1% intermediate-high (11-12) and 5.6% high (ORS ≥13) risk. In-hospital CS occurred in 18 patients (6.3%) as compared with the predicted CS likelihood of 4.3 % signing a possible CS occurrence under-estimation. The AUC-ROC for CS prediction was 0.91 (CI95 %: 0.83-0.97) in our population. The best cut off was ≥ 10 (Se 77.7% and Spe 92%) allowing good classification of 93 % of patients. All-cause in-hospital mortality was 4.2%. Conclusions Our study confirmed the good discrimination of the ORBI-risk score to predict CS occurrence in STEMI patients. The score is simple to use based on simple clinical and biological data available at patient admission and may help to adapt initial patient monitoring and management. Future dedicated studies are needed to test this hypothesis.

Analysis of novel RAC2 variants associated with neutrophil dysfunction, lymphopenia, and primary immunodeficiency

Abstract RAC2 is a member of Rho-family GTPases, abundant in neutrophils serving roles in NADPH oxidase activation and cytoskeleton dynamics. Patients with dominant, activating mutations in RAC2 (E62K, N92T, Q61R) are associated with neutrophil dysfunction, lymphopenia and immunodeficiency. We now characterize additional disease-related RAC2 variants with in-vitro analysis of RAC2 effector functions including NOX2-based superoxide production, regulation of PAK1-PDB binding, activation of AKT, protein stability, subcellular localization, and F-actin formation. We identified both active and inactive variants associated with lymphopenia and immunodeficiency. Patients with dominant, active RAC2 variants with high protein stability (Q61R, Q61K) presented in the first days of life with severe combined immunodeficiency and absent lymphocytes. Activating variants with lower protein stability (I21S, E62K, N92K, N92S) presented as combined variable immunodeficiency with lymphopenia, detectable by newborn screening but clinically presenting later with recurrent sinopulmonary infections, reduced B-cells and hypogammaglobulinemia. A third class of mutations with unstable transcript or protein were phenotypic only in homozygosity (W56*, R68W). Severity of phenotype and initial clinical presentation was correlated with protein stability, with increased stability leading to neonatal presentation and decreased stability of dominant mutations having later clinical onset. Thus, heterologous expression and analysis of RAC2 variants is useful for deciphering the genetic and molecular mechanistic basis for the common underlying clinical phenotype related to lymphopenia and immunodeficiency.

RAC2 mutations and immune deficiency – functional spectrum of an international cohort

Mutations in RAC2 were first identified in neonates carrying RAC2 dominant negative p.D57N, who presented with severe neutrophil defects and T-cell lymphopenia. Subsequently, dominant-activating mutations were reported in patients with combined immunodeficiency/immunodysregulation (CIID) or severe combined immunodeficiency (SCID). We assembled an international cohort of 48 published and unpublished patients with RAC2 mutations to characterize the spectrum of disease.Clinical and immunologic phenotypes of 48 germline mutant RAC2 patients were obtained from referring physicians and literature reports. All patients had significant T lymphopenia with several patients detected by newborn screening, most patients exhibited B and NK lymphopenia as well. Respiratory infections were common, affecting 90% of patients while 56% had significant viral infections and 15% had malignancies. Heterologous RAC2 expression assays including superoxide production, PAK1 binding, and AKT activation were used to characterize RAC2 variant effects on downstream signaling. Protein stability was determined by Western blot. Patient presentation varied by mutation: five patients presented as SCID with reticular dysgenesis, five as neonatal neutrophil deficiency (D57N), and 38 as CIID. Patients with dominant, active RAC2 variants with high protein stability (Q61R, Q61K) presented in the first days of life with SCID and absent lymphocytes. Activating variants with lower stability (C18W, G30R, E62K, N92T, R174) presented as CIID with lymphopenia, detectable by newborn screening but presenting later with recurrent sinopulmonary and viral infections. A third class of mutations with unstable transcript or protein were phenotypic only in homozygosity (W56*, R68W). Consistent with the role of RAC2 in the NADPH oxidase complex, patients with mutations causing reduced superoxide production exhibited bacterial infections. Severity of phenotype and initial clinical presentation were correlated with protein stability such that high stability led to neonatal presentation whereas dominant mutations with decreased stability had later clinical onset.Clinical presentation of RAC2 mutation-bearing patients is determined by a combination of protein activity and stability. Heterologous expression together with RAC2 functional analysis is useful for deciphering the molecular basis of the clinical phenotype related to these immunodeficiencies.

Evaluating the Efficacy and Safety of EZC Pak, a 5-Day Combination Echinacea-Zinc-Vitamin C Dose Pack with or without Vitamin D, in the Management of Outpatient Upper Respiratory Infections.

Growing antibiotic resistance is among the most serious threats to public health, with antibiotic misuse considered a leading driver of the problem. One of the largest areas of misuse is in outpatient upper respiratory infections (URIs). The purpose of this research is to evaluate the efficacy of EZC Pak, a combination Echinacea-Zinc-Vitamin C dose pack with or without Vitamin D, on the duration of illness and symptom severity of non-specific URIs as an alternative to antibiotics when none are deemed clinically necessary. A secondary analysis was carried out on patient satisfaction. A total of 360 patients across the United States were enrolled and randomized in a double-blind manner across two intervention groups, EZC Pak, EZC Pak+Vitamin D, and one placebo group. The study utilized a smartphone-based app to capture data. Once a participant reported the first URI symptom, they were instructed to take the intervention as directed and complete the daily symptom survey score until their symptoms resolved. The average EZC Pak participant recovered 1.39 days (90% CI 1.05 to 1.73) faster than the average placebo participant (p=0.017). The average EZC Pak participant reported a 17.43% (90% CI 17.1 to 17.8) lower symptom severity score versus placebo (p=0.029). EZC Pak users reported 2.9 times higher patient satisfaction versus placebo users (p=0.012). The addition of Vitamin D neither benefited nor harmed illness duration or symptom severity. The findings support the potential use of EZC Pak as an alternative to patient request for antibiotics when none are deemed clinically necessary at the time of initial clinical presentation. The decision to replete vitamin D in the acute phase of URI is an individualized decision left to the patient and their clinician. EZC Pak may play a critical role in improving outpatient URI management and antibiotic stewardship (ClinicalTrials.gov number, NCT04943575).

Role of Surface Electrocardiography in Differentiation between Obstructive and Non-Obstructive Hypertrophic Cardiomyopathy.

Electrocardiography (ECG), as an easily accessible modality, is usually helpful in hypertrophic cardiomyopathy (HCM) diagnosis. The purpose of this study was to evaluate the role of ECG in differentiating between obstructive (OHCM) and non-obstructive (NOHCM) HCM. The present study is a cross-sectional analysis of HCM patients referred to our center between 2008 and 2017. The study variables included age, sex, clinical presentation, medications, and ECG characteristics including PR interval, QRS width, QTc duration, Tpeak-Tend interval, QRS axis, QRS transition, ventricular hypertrophies, atrial abnormalities, ST-T abnormalities, and abnormal Q waves. The HCM sample consisted of 200 patients (55% males; age 45.60±15.50 y) from our HCM database. We compared the clinical and ECG characteristics of 143 NOHCM patients with those of 57 OHCM patients. The OHCM group was significantly younger than the NOHCM group (age =41.7 vs 47.0 y; P=0.016). The initial clinical presentation was similar between the 2 forms (P>0.05), and palpitations were the dominant symptom. Baseline ECG intervals, including PR (155.6 vs 157.9 ms), QRS (82.5 vs 82.0 ms), and QTc (430.5 vs 433.0 ms), were similar (all Ps>0.050). There were no differences regarding baseline rhythm, atrial abnormalities, QRS transition, ventricular hypertrophies, axis changes, ST-T changes, and abnormal Q waves between the HCM groups (all Ps>0.05). The present study showed that standard 12-lead ECG had no role in distinguishing patients with the obstructive and non-obstructive forms of HCM.

Woman with agonal breathing.

A 42-year-old healthy woman with cerebral palsy, oriented consciousness, and partial dependence in daily activities at baseline reported gradual decrease in muscle strength in all her limbs for 6 months. The patient became comatose with agonal breathing unexpectedly during the night hours without any warning. Initial clinical presentation showed a very slow respiratory rate of 8 times/min and SpO2 = 92% under non-rebreathing mask covering. Atrial blood gas test revealed hypercapnic respiratory acidosis (pH, 6.9; PCO2 > 200 mmHg). Intubation was performed immediately. Chest radiography showed no active lesions (Figure 1). Her consciousness returned to baseline a few hours after intubation, but she developed quadriplegia. Brain computed tomography showed no obvious lesions. Brain magnetic resonance imaging (MRI) revealed no obvious lesion but incidentally revealed C2 odontoid process fracture with cord indentation (Figure 2). Cervical MRI showed displaced odontoid process fracture with cord compression, causing myelopathy at C2–C4 levels (Figure 3). It is challenging to diagnose spinal cord injury (SCI) in the absence of trauma history.1 Non-traumatic SCI is a rare neurological emergency.2 There are few case reports of spontaneous atraumatic cervical fracture due to tuberculosis or rheumatoid arthritis.3, 4 High-level SCI causes impairment of respiratory muscles and reduces vital capacity and chest wall compliance. It also influences the respiratory drive, leading to hypercapnia.5, 6 SCI is an often forgotten but important cause of central nervous system depression in patients with hypercapnic respiratory failure in emergency settings. We present a case of atraumatic odontoid fracture, resulting in myelopathy and hypercapnic respiratory failure. The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Low-grade Spindle Cell Sarcoma of the Tonsil: A Rare Entity

Background: Spindle cell sarcoma of the tonsil is an extremely rare malignancy in the head and neck region. The initial clinical presentation can be an asymptomatic mass lesion, and the biopsy of this growing mass should be conducted to exclude a malignant process. In this case report, we described a patient with spindle cell sarcoma of the tonsil with good clinical outcome after surgery alone. Case presentation: A 38-year-old male presented with foreign body sensation of the throat and clinical examination revealed asymmetrical enlargement of the right tonsil. Tonsillectomy was performed, and histopathological examination and immunohistochemistry analysis revealed a low-grade spindle cell sarcoma of the tonsil. He developed local recurrence at twelve months and underwent wide local excision. The patient remained well without any local recurrences and metastases eight years after diagnosis. Conclusion: We reported this case study in an effort to increase the awareness of spindle cell sarcoma of the tonsil and to emphasize the challenges in the diagnosis of this sarcoma. Surgical extirpation with a clear margin appeared to be an adequate treatment for the low-grade spindle cell sarcoma of the tonsil.

Systematic hybrid laparoscopic and endovascular treatment of median arcuate ligament syndrome: A single-center experience.

Median arcuate ligament syndrome (MALS) is caused by celiac trunk (CT) compression by the median arcuate ligament. Clinically, this pathology varies from postprandial pain (Dunbar syndrome) to a life-threatening hemorrhage because of a rupture of a gastroduodenal artery aneurysm (GAA). Due to the low prevalence of this disease, there is no standard management for MALS. This was a single-center, retrospective study of 13 patients. Two groups were identified on the basis of the initial clinical presentation: those operated for a GAA rupture (bleeding group-BG) and those operated electively for Dunbar syndrome (Dunbar syndrome group-DG). The primary endpoint was 30-day postoperative complications of a systematic laparoscopic release of the median arcuate ligament and stenting during the same procedure. Seven patients (54%) underwent elective surgery. Six patients (46%) underwent semiurgent repair under elective conditions post-embolization for GAA bleeding. The total operative time was longer in the BG (p = 0.06). Two patients in the BG suffered early major complications and needed reintervention, and those in the DG had a lower comprehensive complication index. No mortality was reported at 30 days. Overall median length of stay was 5 days (IQR: 3.5-15.3). Patients in the DG had a significantly shorter length of stay (p = 0.02). At 6 months, the primary and secondary CT stent patencies were 82% and 100%, respectively. There were no high-flow GAA recurrences. A combined approach of laparoscopic release of the median arcuate ligament and stenting during the same procedure is feasible and safe, and this approach must be systematically discussed in symptomatic patients.

Delineation of clinical course, outcomes, and prognostic factors in patients with T-cell prolymphocytic leukemia.

T-cell prolymphocytic leukemia (T-PLL) is a rare, post-thymic T-cell neoplasm with a diverse clinical course. T-PLL is typically associated with a poor prognosis; however, a subset of patients have inactive disease on initial presentation. There is a lack of accurate delineation of the disease based on initial clinical presentation and pathological assessment, hindering clinical decision-making. To characterize and delineate disease subtypes based on initial clinical presentation and pathologic assessment, we retrospectively reviewed 81 patients with T-PLL treated at our institution. We compared patients with T-PLL who initially presented with a relatively indolent or stable disease course to those with an aggressive disease course. Clinicopathologic characteristics, overall survival (OS), and prognostic factors were analyzed. Patients with inactive disease had a significantly longer OS than patients with active disease. At diagnosis, presence of B symptoms, low hemoglobin, low platelet count, lymphocyte doubling time of fewer than 3 months, and abnormal cytogenetics were associated with shorter OS. Cell morphology, immunophenotype, absolute lymphocyte count, lactate dehydrogenase levels, involvement of liver, spleen, skin or central nervous system, presence of TCL1 rearrangement or inv (14)/t(14;14), presence of chromosome 8 abnormalities, and presence of deletion of 11q were not associated with significant OS difference among the patients. Receiving alemtuzumab as first-line treatment and consolidation with allogeneic hematopoietic stem cell transplant were associated with better outcomes. T-PLL inactive and active disease subtypes can exhibit overlapping yet different clinical and pathological features. We describe several prognostic factors at diagnosis that can be used for risk stratification and aid in guiding treatment decisions.

181 Evaluating the Association of Age with Acuity and Severity of Illness at Initial Clinical Presentation in Patients with Leukemia and Lymphoma

OBJECTIVES/GOALS: Adolescent and young adult (AYA) patients, diagnosed with cancer between 15 and 39 years of age, often have worse outcomes compared to younger children and older adults. We will characterize age differences in acuity and severity of illness at initial presentation, as a measure for health outcomes, in patients with new diagnoses of leukemia or lymphoma. METHODS/STUDY POPULATION: We will perform a single institution-based, retrospective analysis of a cohort of pediatric oncology patients at Children’s Healthcare of Atlanta (CHOA) who were diagnosed with leukemia or lymphoma from 2010 to 2018. Data will be abstracted from the CHOA medical record and cancer registry. We will construct a severity score, with one point for 1) admission to the Intensive Care Unit (ICU), 2) ICU-level requirements at admission, 3) stage IV or metastatic disease, and 4) white blood cell count over 100,000/microliter. We will associate this score with age at presentation using logistic regression models among patients overall and stratified by disease type. All regression models will adjust for confounders (e.g., health insurance, race and ethnicity, and local-area social vulnerability level). RESULTS/ANTICIPATED RESULTS: Previous literature has shown increased cancer incidence and mortality in AYAs compared to younger children. Therefore, we hypothesize that AYA patients with a new diagnosis of leukemia or lymphoma will have a higher acuity and severity of illness at presentation than younger patients. We conducted a pilot study analyzing acuity and severity of illness by age, race, ethnicity, and insurance status. Bivariate comparisons suggested patients aged 10 and older were as likely as those younger to have a severity score of more than 0 at diagnosis (OR=0.661 , 95% CI 0.43-1.01). However, this result was collapsed across all hematologic malignancies, did not encompass the entire cohort, and did not address possible confounders; we anticipate our estimates will be different taking these factors into account. DISCUSSION/SIGNIFICANCE: Our work will be the first steps in creating a validated tool to understand disease acuity and severity. By using this tool to characterize presentation by disease type and age, we will identify unmet needs prior to an initial diagnosis of cancer. Our findings inform strategies toward narrowing age disparities in outcomes for AYA hematologic cancers.

Secondary Cutaneous Mucormycosis - Retrospective Analysis From Tertiary Care Hospitall.

Background: Covid-19 infection increases the risk of opportunistic infections like mucormycosis. Cutaneous mucormycosis can occur primarily by direct inoculation or secondary to involvement of the underlying structures. Cutaneous manifestations include tender, erythematous, indurated lesions and necrotic plaques. As the disease evolves, cutaneous features manifest progressively. Objectives: To study the manifestations of the cutaneous signs of sinonasal mucormycosis and management of such cases based on severity of involvement. Materials and methods: A retrospective analysis of 21 patients with diagnosis of cutaneous mucormycosis secondary to sinonasal involvement was done with assessment of their skin lesion, area involved and their clinical stage being noted at the time of admission and after 24h. Treatment consisted of combination of surgical debridement, daily dressing and liposomal amphotericin B. Observations and Results: Out of total 21 patients, there were 10 males and 11 females. Among risk factors, 14 cases had history of covid 19 infection, 5 had history of steroid intake, 6 had history of ICU stay and all had deranged blood sugar levels. Among disease prognosis, excellent outcomes appeared in stage I and stage III showed worst outcome. Conclusion: Since initial clinical presentation is similar to cellulitis and other soft-tissue infections, early recognition is difficult. In this cohort, the prognosis of secondary cutaneous mucormycosis remained poor, especially in ICU patients and those with numerous predisposing factors. Such patients presented in late stages of the disease and mortality rate was very high in such group.

Development and Utility of Practical Indicators of Critical Outcomes in Dengue Patients Presenting to Hospital: A Retrospective Cross-Sectional Study

Global travel and climate change have drastically increased the number of countries with endemic or epidemic dengue. The largest dengue outbreak in Taiwan, with 43,419 cases and 228 deaths, occurred in 2015. Practical and cost-effective tools for early prediction of clinical outcomes in dengue patients, especially the elderly, are limited. This study identified the clinical profile and prognostic indicators of critical outcomes in dengue patients on the basis of clinical parameters and comorbidities. A retrospective cross-sectional study was conducted in a tertiary hospital from 1 July 2015 to 30 November 2015. Patients diagnosed with dengue were enrolled, and the initial clinical presentations, diagnostic laboratory data, details of the underlying comorbidities, and initial management recommendations based on 2009 World Health Organization (WHO) guidelines were used to evaluate prognostic indicators of critical outcomes in dengue patients. Dengue patients from another regional hospital were used to evaluate accuracy. A group B (4 points) classification, temperature < 38.5 °C (1 point), lower diastolic blood pressure (1 point), prolonged activated partial thromboplastin time (aPTT) (2 points), and elevated liver enzymes (1 point) were included in the scoring system. The area under the receiver operating characteristic curve of the clinical model was 0.933 (95% confidence interval [CI]: 0.905-0.960). The tool had good predictive value and clinical applicability for identifying patients with critical outcomes.