Background Mycosis fungoides (MF) in young patients is rare and may have atypical presentations. There are limited data in these patients. Objective Determine the clinical outcome and prognosis of young patients with MF. Methods A search of our institutional cancer registry database was conducted for patients diagnosed with MF at ≤ 30 years of age. Results Our study includes 74 patients (median age at diagnosis= 25.5 years). Most patients (n=65, 88%) presented with early stage disease and variants of MF (n=44, 59%) leading to a median delay in diagnosis of 2.5 years. Hypopigmented MF (n=27, 36.5%) was the most common variant affecting predominantly African-American (44.4% vs.19%, p= 0.02) and younger age group (20 vs. 26 years, p<0.001). All patients with hypopigmented MF presented with early stage disease and were less likely to develop progressive disease (PD) compared to other variants (11% vs. 34%, p= 0.03). Overall, nineteen patients (26%) developed PD during median follow-up of 3.5 years which was associated with advanced stage disease (89% vs. 17%, p<0.0001), older age (>20 years) [31% vs. 13%, p=0.08 (NS)], African-American race (52.6% vs. 20%, p= 0.0090) and poikilodermatous presentation (p<0.01). The overall survival was favorable (97.2% and 95.9% at 5 and 10 years) despite delay in diagnosis and atypical presentation. Conclusions PD is associated with older age, African-American race, the poikilodermatous variant, and advanced-stage disease. The hypopigmented variant is a common presentation in young patients and has an indolent disease course. Our study confirms an overall favorable prognosis in young patients with MF.
IMPORTANCE Children with epidermolysis bullosa (EB) comprise a rare population with high morbidity and mortality. An improved understanding of the clinical trajectory of patients with EB, including age at time of clinical diagnosis and major clinical events, is needed to refine best practices and improve quality of life and clinical outcomes for patients with EB. OBJECTIVES To describe demographics, clinical characteristics, milestone diagnostic and clinical events (such as initial esophageal dilation), and outcomes in patients with EB using the Epidermolysis Bullosa Clinical Characterization and Outcomes Database and to determine what characteristics may be associated with overall EB severity and/or disease progression. DESIGN, SETTING, AND PARTICIPANTS This cohort study included data on patients with EB who were enrolled in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database from January 1, 2011, to June 30, 2017; 17 participating EB centers in the United States and Canada contributed data to this study. EXPOSURES Type of EB, including recessive dystrophic epidermolysis bullosa (RDEB), junctional epidermolysis bullosa (JEB), dominant dystrophic epidermolysis bullosa (DDEB), and epidermolysis bullosa simplex (EBS). MAIN OUTCOMES AND MEASURES Demographic information, clinical characteristics (including age at onset of signs of EB and subsequent clinical diagnosis), types of diagnostic testing performed, and milestone clinical events for patients with RDEB. RESULTS Of 644 enrolled patients from 17 sites included in this study, 323 were male (50.2%), with a mean (SD) age of 14.4 (11.7) years; 283 (43.9%) had RDEB, 194 (30.1%) had EBS, 104 (16.2%) had DDEB, and 63 (9.8%) had JEB. Signs of disease were present at birth in 202 patients with RDEB (71.4%), 39 with JEB (61.9%), 60 with DDEB (57.7%), and 74 with EBS (38.1%). For those with signs of disease at birth, a clinical diagnosis was made at the time of birth in 135 patients with RDEB (67.0%), 31 with DDEB (52.6%), 35 with EBS, (47.3%) and 18 with JEB (46.2%). Patients with JEB had the highest rate of any confirmatory testing (51 of 63 [81.0%]), followed by RDEB (218 of 283 [77.0%]), DDEB (71 of 104 [68.3%]), and EBS (100 of 194 [51.5%]). For all types of EB, both electron microscopy and immunofluorescence microscopy were performed at younger ages than genetic analysis. Among 283 patients with RDEB, 157 (55.5%) had esophageal dilation, 104 (36.7%) had gastrostomy tube placement, 62 (21.9%) had hand surgery, 18 (6.4%) developed squamous cell carcinoma, and 19 (6.7%) died. CONCLUSIONS AND RELEVANCE The findings suggest that diagnostic testing for EB is more common for patients with severe phenotypes. Earlier diagnostic testing may enable improved characterizations of patients so that appropriate counseling and clinical care may be offered, especially pertaining to milestone events for those with RDEB.
Background/Objectives: Many patients with epidermolysis bullosa (EB) require
Introduction Initial reports show an increase in youth onset type 2 diabetes during the COVID‐19 pandemic. We aim to expand on existing evidence by analyzing trends over a longer period. Objectives Our study aims to describe change in the amount, severity, and demographics of youth onset type 2 diabetes diagnoses during the COVID‐19 pandemic compared to the five years before. Methods We performed a retrospective cross‐sectional review of youth (age ≤ 21) diagnosed with type 2 diabetes during the COVID‐19 pandemic (1 May 2020–30 April 2021) and the five years before (1 May 2015–30 April 2020) at a tertiary care center. Children were identified by International Classification of Diseases codes. Charts were reviewed to confirm diagnosis. Chi‐square, t tests, and Fisher's exact tests were used for analyses. Results In the prepandemic era annual diagnoses of type 2 diabetes ranged from 41–69 (mean = 54.2), whereas during the pandemic period 159 children were diagnosed, an increase of 293%. The increase resulted in a higher incidence rate ratio during the pandemic than before, 2.77 versus 1.07 ( p = .006). New diagnoses increased most, by 490%, in Non‐Hispanic Black patients. The average HbA 1c at presentation was higher during the pandemic (9.5% ± 2.6) (79.9 mmol/mol ± 28.2) than before (8.7%±2.1) (72.1 mmol/mol ± 23.1) ( p = .003). Of those diagnosed during the pandemic, 59% were tested for COVID‐19 and three tested positive. Conclusions New diagnoses of type 2 diabetes increased during the pandemic, most notably in Non‐Hispanic Black youth. There was not a significant correlation found with clinical or biochemical COVID‐19 infection in those tested.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.