Publications by authors named "A Alavi"

1,615 Publications

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International Dermatology Outcome Measures (IDEOM): Report from the 2020 Annual Meeting.

Dermatology 2021 Sep 17:1-8. Epub 2021 Sep 17.

Department of Dermatology, Harvard Medical School, Brigham and Women's Hospital, Boston, Massachusetts, USA.

Background: The International Dermatology Outcome Measures (IDEOM) initiative is a non-profit organization that aims to develop evidence-based outcome measurements to evaluate the impact of treatments for patients with dermatological disease. IDEOM includes all key stakeholders in dermatology (patient, physician, industry, insurer, and government) during the process of developing such outcome measurements.

Summary: Here, we provide an update of IDEOM activities that were presented at the 2020 IDEOM Virtual Annual Meeting (October 23-24, 2020). During the meeting, multiple IDEOM workgroups (psoriasis, psoriatic arthritis, hidradenitis suppurativa, acne, pyoderma gangrenosum, and actinic keratosis) shared their progress to date, as well as future directions in developing and validating Patient-Reported Outcome Measures. Updates on demonstrating efficacy in clinicals trials by the US Food and Drug Administration are also summarized. Key Messages: In this report, we summarize the work presented by each IDEOM workgroup (psoriasis, psoriatic arthritis, hidradenitis suppurativa, acne, pyoderma gangrenosum, and actinic keratosis) at the 2020 IDEOM Virtual Annual Meeting.
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http://dx.doi.org/10.1159/000518966DOI Listing
September 2021

Clinical and genetic spectrum of a large cohort of patients with δ-sarcoglycan muscular dystrophy.

Brain 2021 Sep 13. Epub 2021 Sep 13.

Neuromuscular Diseases Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Departament of Medicine, Barcelona, 08041, Spain.

Sarcoglycanopathies include four subtypes of autosomal recessive limb-girdle muscular dystrophies (LGMDR3, LGMDR4, LGMDR5 and LGMDR6) that are caused, respectively, by mutations in the SGCA, SGCB, SGCG and SGCD genes. Delta-sarcoglycanopathy (LGMDR6) is the least frequent and is considered an ultra-rare disease. Our aim was to characterize the clinical and genetic spectrum of a large international cohort of LGMDR6 patients and to investigate whether or not genetic or protein expression data could predict diseasés severity. This is a retrospective study collecting demographic, genetic, clinical and histological data of patients with genetically confirmed LGMDR6 including protein expression data from muscle biopsies. We contacted 128 pediatric and adult neuromuscular units around the world that reviewed genetic data of patients with a clinical diagnosis of a neuromuscular disorder. We identified 30 patients with a confirmed diagnosis of LGMDR6 of which 23 patients were included in this study. Eighty seven percent of the patients had consanguineous parents. Ninety one percent of the patients were symptomatic at the time of the analysis. Proximal muscle weakness of the upper and lower limbs was the most common presenting symptom. Distal muscle weakness was observed early over the course of the disease in 56.5% of the patients. Cardiac involvement was reported in 5 patients (21.7%) and 4 patients (17.4%) required non-invasive ventilation. Sixty percent of patients were wheelchair-bound since early teens (median age of 12.0 years old). Patients with absent expression of the sarcoglycan complex on muscle biopsy had a significant earlier onset of symptoms and an earlier age of loss of ambulation compared to patients with residual protein expression. This study confirmed that delta-sarcoglycanopathy is an ultra-rare neuromuscular condition and described the clinical and molecular characteristics of the largest yet-reported collected cohort of patients. Our results showed that this is a very severe and quickly progressive disease characterized by generalized muscle weakness affecting predominantly proximal and distal muscles of the limbs. Similar to other forms of sarcoglycanopathies, the severity and rate of progressive weakness correlates inversely with the abundance of protein on muscle biopsy.
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http://dx.doi.org/10.1093/brain/awab301DOI Listing
September 2021

Utility of FDG PET/CT in assessing bowel inflammation.

Am J Nucl Med Mol Imaging 2021 15;11(4):271-279. Epub 2021 Aug 15.

Department of Radiology, University of Pennsylvania Philadelphia, PA, USA.

Purpose: To develop a methodology for the quantification of gastrointestinal (GI) inflammation as indicated by 2-deoxy-2-(F)fluoro-D-glucose (FDG) uptake on positron-emissions tomography/computed tomography (PET/CT) imaging. This is intended to investigate the feasibility of using standard uptake value (SUV) levels to assess levels of GI inflammation in humans.

Methods: 131 participants were injected with a weight-controlled dose of FDG 180 minutes prior to PET/CT scanning. Operator-guided software was used to segment the GI tract and perform (SUV) calculations. Regions of interest (ROIs) were created using CT images and stacked to create three dimensional volumes of interest (VOIs). These VOIs defined 6 sections of the GI tract: esophagus, stomach, descending colon, ascending and transverse colon, bowel below the ilium and small bowel above the ilium.

Results: This study found a significant correlation between age and average FDG uptake (avg-SUV) of the GI tract (P=.0003) with the esophagus showing the highest significance. Correlations were found between avg-SUV of the sigmoid segment and the group average (P<.0001), and between the descending colon VOI and the group (P<.0001). Intra-operator reproducibility over 3 trials showed a coefficient of variation (CV) of .63%. Inter-operator CV over 5 randomly selected patients was 5.6% over the entire GI tract.

Conclusion: This study shows that FDG-PET/CT imaging is a promising technique for quantifying bowel inflammation, despite the fact that age related inflammation may not be of clinical utility. The fact that we were able to detect these subtle changes indicates this as an avenue for potential future investigation.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8414397PMC
August 2021

Multifunctional Meta-Tribomaterial Nanogenerators for Energy Harvesting and Active Sensing.

Nano Energy 2021 Aug 16;86. Epub 2021 Apr 16.

Department of Civil and Environmental Engineering, University of Pittsburgh, Pittsburgh, PA, USA.

Discovering novel multifunctional metamaterials with energy harvesting and sensing functionalities is likely to be the next technological evolution of the metamaterial science. Here, we introduce a novel concept called self-aware composite mechanical metamaterial (SCMM) that can transform mechanical metamaterials into nanogenerators and active sensing mediums. In pursuit of this goal, we examine new paradigms where finely tailored and seamlessly integrated self-recovering snapping microstructures composed of topologically different triboelectric materials can form self-powering and self-sensing meta-tribomaterial systems. We explore various deformation mechanisms required to induce contact electrification between these snapping microstructures under periodic deformations. The multifunctional meta-tribomaterial systems created under the SCMM concept will act as triboelectric nanogenerators capable of generating electrical signals in response to the applied mechanical excitations. The generated electrical signal can be used for active sensing of the applied force and can be stored for empowering sensors and embedded electronics. We conduct theoretical and experimental studies to understand the mechanical and electrical behavior of the multifunctional SCMM systems. The broad application of the proposed SCMM concept for designing artificial materials with novel properties and functionalities is highlighted via prototyping self-powering and self-sensing blood vessel stents and shock absorbers.
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http://dx.doi.org/10.1016/j.nanoen.2021.106074DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8423374PMC
August 2021

Comparison the efficacy of vaginal progesterone 17-alpha-hydroxyprogesterone caproate to prevent preterm birth in high-risk pregnant women undergo cerclage: a randomized clinical trial.

J Matern Fetal Neonatal Med 2021 Sep 1:1-7. Epub 2021 Sep 1.

Community Medicine Department, Hormozgan University of Medical Sciences, Bandar Abbas, Iran.

Objective: The aim of this study was to compare the effect of vaginal progesterone with 17-alpha-hydroxyprogesterone caproate (17OHP-C) in prevention of preterm birth in high-risk pregnant women undergo cerclage.

Materials And Methods: This prospective randomized clinical trial registered in the Iranian Registry of Clinical Trials (IRCT20181107041585N4), was performed from May 2017 to August 2018 in Bandar Abbas, Iran. Fifty-eight eligible women who were scheduled for cervical cerclage due to a history of two or more previous preterm birth <28 weeks or a cervical length less than 25 mm with at least one previous preterm birth <34 weeks were randomly divided into two groups. The first group received 200 mg of vaginal progesterone suppository daily and the second one received 250 mg of 17OHP-C intramuscular weekly after cerclage procedure until the end of 36 weeks. Patients were followed up to the end of delivery and the newborn until the first 28 d after delivery.

Results: Gestational age at the time of birth in 17OHP-C group was significantly higher than vaginal progesterone group (=.021). However, the incidence of preterm birth in both groups was not statistically significant (20.7% 24.1%). Apgar scores, newborn birthweight, admission to neonatal intensive care unit (NICU), incidence of respiratory distress syndrome (RDS), sepsis, necrotizing enterocolitis (NEC), and, intraventricular hemorrhage (IVH), was similar in both groups. Adverse events were reported in 48.3% of patients in 17-OHP-C group, and 27.6% of patients in the vaginal progesterone group (= .014).

Conclusions: Vaginal progesterone and 17OHP-C had similar results in terms of prevention of preterm birth and neonatal outcome. However, the adverse events associated with 17-OHP-C were higher than vaginal progesterone.
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http://dx.doi.org/10.1080/14767058.2021.1949451DOI Listing
September 2021
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