Orbital Exenteration and Reconstruction Using a Free Radial Forearm Flap in Conjunctival Melanoma: Old but Gold.

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. In recent years, the techniques used for orbital reconstruction have included the transorbital temporoparietal fascial flap, the anterolateral thigh flap, and local flaps, such as the paramedian forehead flap. A free radial forearm flap is currently not commonly used for orbital reconstruction due to potential donor site morbidity and cosmetic issues. In our case, we report a free radial forearm fasciocutaneous flap that has been utilized with promising surgical outcomes to reconstruct the orbital region following orbital exenteration.

Atypical Presentation of Lip Nodules in Clinically Diagnosed Juvenile Hyaline Fibromatosis.

Juvenile 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. However, the hallmarks of gingival hypertrophy, subcutaneous scalp nodules, and joint contractures can be used as a clinical guide when genetic testing is unavailable. Here, we report an unusual case of a five-year-old child clinically diagnosed with juvenile hyaline fibromatosis with atypical nodules exclusively confined to the perioral region.

Thunderstorm asthma in seasonal allergic rhinitis: The TAISAR study.

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. METHODS: This multicenter study recruited adults from Melbourne, Australia, with a past diagnosis of TA and/or self-reported SAR. Clinical information, spirometry results, white blood cell count, ryegrass pollen-specific (RGP-sp) IgE concentration, and fractional exhaled nitric oxide were measured to identify risk factors for a history of TA in individuals with SAR. RESULTS: From a total of 228 individuals with SAR, 35% (80 of 228) reported SAR only (the I-SAR group), 37% (84 of 228) reported TA symptoms but had not attended hospital for treatment (the O-TA group), and 28% (64 of 228) had presented to the hospital for TA (the H-TA group). All patients in the H-TA group reported a previous asthma diagnosis. Logistic regression analysis of factors associated with O-TA and H-TA indicated that lower FEV1 value and an Asthma Control Questionnaire score higher than 1.5 were associated with H-TA. Higher blood RGP-sp IgE concentration, eosinophil counts, and fractional exhaled nitric oxide level were significantly associated with both O-TA and H-TA. Receiver operating curve analysis showed an RGP-sp IgE concentration higher than 10.1 kU/L and a prebronchodilator FEV1 value of 90% or lower to be biomarkers of increased H-TA risk. CONCLUSION: Clinical tests can identify risk of a history of TA in individuals with SAR and thereby inform patient-specific treatment recommendations.

Managing Exacerbations in Thunderstorm Asthma: Current Insights.

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. It is now well recognized that large pollen grains, which usually lodge in the upper airway causing seasonal allergic rhinitis (SAR), are ruptured during these events, leading to sub-pollen particles respirable to the lower respiratory tract causing acute asthma. The identified risk factors of aeroallergen sensitization, specifically to RGP in Australians with a history of SAR, and individuals born in Australia of South-East Asian descent as a risk factor for TA has been key in selecting appropriate patients for preventative management. Moreover, severity-determining risk factors for ETSA-related asthma admission or mortality, including pre-existing asthma or prior hospitalization, poor inhaled corticosteroid adherence, and outdoor location at the time of the storm are important in identifying those who may require more aggressive treatment approaches. Basic treatments include optimizing asthma control and adherence to inhaled corticosteroid therapy, treatment of SAR, and education regarding TA to increase recognition of at-risk days. Precision treatment approaches may be more beneficial in select individuals, including the use of allergen immunotherapy and even biologic treatment to mitigate asthma severity. Finally, we discuss the importance of environmental health literacy in the context of concerns surrounding the increased frequency of ETSA due to climate change and its implications for the frequency and severity of future events.

All-Cause and Cause-Specific Mortality in Patients With Granulomatosis With Polyangiitis: A Population-Based Study.

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]). The outcome was death (all-cause, cardiovascular disease [CVD]-related cancer-related, renal disease-related, and infection-related) during the follow-up period. Hazard ratios (HR) were estimated using Cox proportional hazards models, first adjusted for age, sex, and time of entry and then adjusted for selected covariates based on a purposeful selection algorithm. RESULTS: Three hundred seventy patients with GPA and 3,700 non-GPA controls were included in this study, contributing 1,624.8 and 1,8671.3 person-years of follow-up, respectively. Sixty-eight deaths occurred in the GPA cohort, and 310 deaths occurred in the non-GPA cohort. Overall, the age-, sex-, and entry time-adjusted all-cause mortality HR in the GPA cohort was 3.12 (95% confidence interval CI 2.35-4.14). There was excess mortality due to CVD-related causes, but not cancer, in the GPA cohort. Reports of death due to infection or renal disease was not permitted, because the numbers of death were insufficient (<6 deaths for each outcome). All-cause mortality significantly improved between the early cohort and late cohort time periods (HR 5.61 and 2.33, respectively; P for interaction = 0.017). CONCLUSION: This population-based study showed increased all-cause and CVD-related mortality risks in patients with GPA. There was significant improvement in the all-cause mortality risk over time, but the risk remained increased compared with that in the general population.

Mortality in ANCA-associated vasculitis: ameta-analysis of observational studies.

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. We calculated weighted-pooled summary estimates of SMRs (meta-SMRs) for all-cause mortality using random-effects model, tested for publication bias and heterogeneity. RESULTS: Ten studies met the inclusion criteria, comprising 3338 patients with AAV enrolled from 1966 to 2009, and a total of 1091 observed deaths. Overall, we found a 2.7-fold increased risk of death in patients with AAV when compared with the general population (meta-SMR: 2.71 (95% CI 2.26 to 3.24)). Analysis on studies that included only granulomatosis with polyangiitis cases also indicated a similar mortality risk (meta-SMR: 2.63 (95% CI 2.02 to 3.43)). There was no significant publication bias or small-study effect. Subgroup analyses showed that mortality risks were higher in older cohorts, with a trend towards improvement over time (ie, those with their midpoint of enrolment periods that were between 1980-1993 and 1994-1999, vs 2000-2005). CONCLUSION: Published data indicate there is a 2.7-fold increase in mortality among patients with AAV compared with the general population.

Inflammatory myopathy with anti-SRP antibodies: case series of a South Australian cohort.

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. Of 144 patients with inflammatory myositis tested for myositis-specific and myositis-associated antibodies between 2007 and 2011 inclusive, five with anti-SRP antibodies were identified. All five were male, four had severe proximal weakness, one was asymptomatic and three had dysphagia. None had cardiovascular involvement. All patients showed isolated anti-SRP positivity and absence of antinuclear antibodies. Muscle histopathology showed variable myofibre necrosis, and most had an inflammatory infiltrate. Majority showed a favorable response to combination immunosuppressive therapy. Myopathy associated with anti-SRP antibodies is clinically heterogeneous in presentation. Muscle histopathology shows a mixture of necrotic and inflammatory features.

Incidence and prevalence of idiopathic inflammatory myopathies in South Australia: a 30-year epidemiologic study of histology-proven cases.

AIM: 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. SA population denominator numbers were obtained from the Australian Bureau of Statistics. RESULTS: Three hundred and fifty-two biopsy-proven cases of IIM were identified between 1980 and 2009. The overall annual incidence of IIM appeared to be rising with a mean incidence of eight cases per million population (95% CI: 7.2-8.9). This corresponded with an increasing annual incidence of inclusion body myositis (IBM) (prevalence of 50.5 cases per million population in 2009, 95% CI: 40.2-62.7). A female preponderance was noted in both dermatomyositis (DM) (F : M = 2.75 : 1.00) and polymyositis (PM) (F : M = 1.55 : 1.00) but gender distribution was almost equal in IBM (F : M = 1.1 : 1.0). Mean age at diagnosis for IBM (67.5 years) was higher than for DM (55.1 years) and PM (59.0 years). A higher proportion of DM patients reported living in urban dwellings and DM patients tended to be predominantly professionals. CONCLUSIONS: In SA there is an increasing incidence of IBM and the prevalence is one of the highest reported to date. This may reflect an increase in the number of biopsies performed, improved histological techniques or a genuine increase in incidence.