Conjunctival melanoma is a rare and potentially deadly tumor. Therefore, adequate oncological resection is essential, commonly leading to total orbital exenteration, which causes patients' extensive functional and cosmetic impairment. As a result, it is essential to reconstruct the orbital region post-exenteration to obliterate the cavity, provide adequate and pliable cutaneous covering, and restore a stable vascularized tissue that can withstand adjuvant radiotherapy.
View Article and Find Full Text PDFJuvenile hyaline fibromatosis (JHF) is a rare, hereditary disease characterized by abnormal hyaline deposits within the skin, soft tissues, joints, and bones. The condition itself is often debilitating, with no curative treatment available. A definitive diagnosis is established by genetic testing.
View Article and Find Full Text PDFJ Allergy Clin Immunol
May 2022
Background: Asthma epidemics associated with thunderstorms have had catastrophic effects on individuals and emergency services. Seasonal allergic rhinitis (SAR) is present in the vast majority of people who develop thunderstorm asthma (TA), but there is little evidence regarding risk factors for TA among the SAR population.
Objective: We sought to identify risk factors for a history of TA and hospital presentation in a cohort of individuals with SAR.
Epidemic thunderstorm asthma (ETSA) occurs following a thunderstorm due to the interaction of environmental and immunologic factors. Whilst first reported in the 1980s, the world's largest event in Melbourne, Australia, on November 21, 2016 has led to a wealth of clinical literature seeking to identify its mechanisms, susceptibility risk factors, and management approaches. Thunderstorm asthma (TA) typically presents during an aeroallergen season in individuals sensitized to perennial rye grass pollen (RGP) in Australia, or fungus in the United Kingdom, in combination with meteorological factors such as thunderstorms and lightning activity.
View Article and Find Full Text PDFArthritis Care Res (Hoboken)
January 2019
Objective: To investigate all-cause and cause-specific mortality in patients with newly diagnosed granulomatosis with polyangiitis (GPA) between 2 calendar time periods, 1997-2004 and 2005-2012.
Methods: Using an administrative health database, we compared all patients with incident GPA with non-GPA controls matched for sex, age, and time of entry into the study. The study cohorts were divided into 2 subgroups based on the year of diagnosis ("early cohort [1997-2004] and "late cohort" [2005-2012]).
Objective: To determine the magnitude of all-cause mortality risk in patients with antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) compared with the general population through a meta-analysis of observational studies.
Methods: We searched Medline and Embase databases from their inception to April 2015. Observational studies that met the following criteria were assessed by two researchers: (1) clearly defined AAV identified by either the American College of Rheumatology 1990 classification criteria or the 2012 Chapel Hill Consensus Conference disease definitions, and (2) reported standardised mortality ratios (SMR) and 95% CI.
Objective: To assess the relative risk of myocardial infarction (MI) and ischemic stroke in patients with newly diagnosed granulomatosis with polyangiitis (Wegener's) (GPA) compared with that in controls from the general population.
Methods: Using a population-based database from the province of British Columbia, Canada, we conducted a matched cohort study in which each patient with incident GPA was matched for age, sex, and entry time with up to 10 individuals from the general population. Patients in the GPA cohort were required to have received at least 1 prescription for oral glucocorticoids, methotrexate, cyclophosphamide, leflunomide, azathioprine, cyclosporine, mycophenolate mofetil, or rituximab within 1 month before or 6 months after the index date.
J Allergy Clin Immunol Pract
January 2015
Myopathy associated with anti-signal recognition particle (SRP) antibodies is a rare form of myopathy, which is thought to be distinct from classic polymyositis. We sought to determine the demographic, clinical and histopathological features of patients with anti-SRP antibodies. Hence we undertook an audit of patients with histologically-confirmed myositis who had anti-SRP antibodies.
View Article and Find Full Text PDFAim: To describe the epidemiology of biopsy-proven idiopathic inflammatory myopathies (IIM) in South Australia (SA).
Methods: Cases of IIM were ascertained by review of all muscle biopsy reports from the Neuropathology Laboratory, Hanson Institute (wherein all adult muscle biopsies in SA are reported) from 1980 to 2009. Clinical correlation of these patients by review of medical records was undertaken.
Introduction: Necrotizing myopathy (NM) is distinguished from idiopathic inflammatory myositis (IIM) by dominance of myofiber necrosis, lack of mononuclear inflammatory infiltrates, and presence of antibodies to signal recognition particle (SRP).
Methods: The clinical features of 64 cases of NM were determined. Measurement of autoantibodies was undertaken on stored sera from 23 patients with NM.