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Background: There is a need to prognosticate the severity of cystic fibrosis (CF) detected by newborn screening (NBS) by early assessment of CF Transmembrane Conductance Regulator protein function (CFTR). We introduce novel instrumentation and protocol for evaluating CFTR activity as reflected by β-adrenergically stimulated sweat secretion. Methods: A pixilated Image-Sensor detects sweat rates. Compounds necessary for maximum sweat gland stimulation are applied by Iontophoresis, replacing intradermal injections. Results are compared to a validated β-adrenergic assay that measures sweat secretion by evaporation (Evaporimetry). Users may view, print, copy, and download text and data-mine the content in such documents, for the purposes of academic research, subject always to the full Conditions of use:

Autoimmune hepatitis (AIH), post-transplant recurrent AIH (rAIH), and plasma cell-rich rejection (PCR) are clinical diagnoses with the shared histopathologic hallmark of plasma cell hepatitis (PCH). As these histologically and serologically indistinguishable diagnoses are differentiated by clinical context, it remains uncertain whether they represent distinct immunologic phenomena. Improved understanding of immunoglobulin subclass 4-producing plasma cells (IgG4-PC) has brought attention to IgG4 as an immunophenotypic biomarker. To date, degree and clinical significance of IgG4-PC infiltration in PCH remain elusive. This retrospective, single-center study assessed IgG4-PC infiltration in AIH, rAIH, and PCR via standardized immunohistochemistry analysis. Identified cases from 2005 to 2020 (n = 47) included AIH (treatment-naïve AIH (tnAIH): n = 15 and AIH-flare on treatment (fAIH); n = 10), rAIH (n = 8), and PCR (n = 14) were analyzed and correlated with clinical characteristics. IgG4-Positivity (# IgG4-PC/# pan-IgG-expressing cells) distribution was heterogenous and overlapping [tnAIH: 0.060 (IQR 0.040–0.079), fAIH: 0.000 (0.000–0.033), rAIH: 0.000 (0.000–0.035), PCR: 0.228 (0.039–0.558)]. IgG4-Positivity was inversely correlated with corticosteroid use (p < 0.001). IgG4-Positivity ≥0.500 was associated with rapid AST improvement (p = 0.03). The variable IgG4-Positivity of AIH, rAIH and PCR suggests diverse and overlapping immunopathologic mechanisms and that current diagnostic schemes inadequately capture PCH immunopathology. We propose incorporation of IgG4-Positivity to refine current PCH classification and treatment strategies.

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INTRODUCTION: There are many variables whose role remains undefined in predicting esophagitis severity. This is particularly true for female and non-white populations for whom existing data is limited. Our aim was to determine clinical and endoscopic characteristics of reflux esophagitis in the predominantly Hispanic population of a large, safety-net hospital. METHODS: We performed a retrospective electronic medical record review of patients identified with mild (Los Angeles Grade A/B) and severe (Los Angeles Grade C/D) esophagitis between January 2017 to December 2018. Demographic, clinical, and endoscopic data were recorded. Continuous and categorical data was analyzed using a two tailed T-test and/or chi-square test or Fisher’s Exact test. RESULTS: Reflux esophagitis was identified in 382 patients; 56.5% (n = 216) males, 79.8% (n = 305) Hispanic, 8.4% (n = 32) Caucasian, 5.8% (n = 22) African American, and 6.0% (n = 23) other. Among the cohort, 59% (n = 225) had mild esophagitis and 41% (n = 157) had severe esophagitis with median ages 56 and 58 years, respectively (P = 0.05). Patients with mild esophagitis had a higher body mass index (BMI) as compared to those with severe esophagitis [median 28.4 (25.5, 33.7) vs. 26.3 (21.9, 29.6); P < 0.01], were more frequently female (P = 0.03), diagnosed in the outpatient setting vs. inpatient (P < 0.01), and had higher rates of cirrhosis (P = 0.03). Gastroparesis (P = 0.02) and immunosuppression (P = 0.01) were more common in severe vs. mild esophagitis. Hiatal hernia (P < 0.01) and esophageal strictures (P = 0.01) were more frequent in severe esophagitis. Gastrointestinal bleed (P < 0.01), dysphagia (P < 0.01) and nausea/vomiting (P = 0.02) were more frequently the indication for endoscopy in severe vs. mild esophagitis. Esophagitis severity was not associated with tobacco or alcohol use. CONCLUSION: In this predominantly Hispanic cohort, patients with severe esophagitis were more frequently male, hospitalized, and had lower BMI compared to those with mild esophagitis. Ongoing multivariate analysis is underway to determine whether these variables are independent risk factors for severity of esophagitis in this unique population after adjustment for age, BMI, and hospitalization status.