Adrenal Crisis

Glucocorticoid replacement therapy, available since the 1950s, has prolonged the survival of patients with adrenal insufficiency. However, adrenal crises, which are life-threatening medical emergencies, still develop in many affected patients. Adrenal crisis appears to be increasing in frequency, despite the availability of effective preventive strategies. This review examines the definitions, pathophysiology, epidemiology, and treatment of adrenal crises

Pre-hospital management of acute Addison’s Disease – Audit of patients attending a referral hospital in a regional area

Context: Adrenal crises (AC) cause morbidity and mortality in patients with Addison’s disease [primary adrenal insufficiency (PAI)]. Patient-initiated oral stress dosing, with parenteral hydrocortisone, is recommended to avert ACs. While these should be effective, the continued incidence of ACs remains largely unexplained. Methods: Audit of all attendances between 2000 and 2017 by adult patients with treated PAI to one large regional referral centre in New South Wales, Australia. Measurements were those taken on arrival at hospital. Results: There were 252 attendances by 56 patients with treated PAI during the study period. Women comprised 60.7% (n=34) of the patients. The mean age of attendees was 53.7 (19.6) years. Nearly half (45.2%, n=114) the patients had an infection. There were 61 (24.2%) ACs diagnosed by the treating clinician. Only 17.9% (n=45) of the hospital presentations followed any form of stress dosing. IM hydrocortisone was used before 7 (2.8%) attendances only. Among patients with a clinician diagnosed AC, only 32.8% (n=20) had used stress dosing before presentation. Vomiting was reported by 47.6% (n=120) of the patients but only 33 (27.5%) of these attempted stress dosing and 5 patients with vomiting used IM hydrocortisone. The number of prior presentations was a significant independent predictor of use of stress doses [1.05 (1.01,1.09)]. Conclusion: Dose escalation strategies are not used universally or correctly by unwell patients with PAI, many patients do not use IM or SC hydrocortisone injections. Previous hospital treatment increases the likelihood of stress dosing and offers the opportunity for reinforcement of prevention strategies

Adrenal crises: perspectives and research directions

Adrenal crises (AC) are life-threatening complications of adrenal insufficiency (AI). These events have an estimated incidence of between 5 and 10 ACs/100 patient years (PY) and are responsible for some of the increased morbidity and excess mortality experienced by patients with AI. Treatment involves urgent administration of IV/IM hydrocortisone and IV fluids. Patient education regarding preventive measures, such as increasing the dose of replacement therapy (“stress dosing”) when sick, using parenteral hydrocortisone as necessary and accessing medical assistance promptly, is still considered the best approach to averting the onset of an AC at times of physiological stress, most commonly an infection. However, recent evidence has demonstrated that patient education does not prevent many AC events and the reasons for this are not fully understood. Furthermore, there is no widely accepted definition of AC. Without a validated AC definition it is difficult to interpret variations in the incidence of AC and determine the effectiveness of preventive measures. This article aims to review the clinical aspects of AC events; to explore the epidemiology; and to offer a definition for an AC and to offer a perspective on future directions for research into AC prevention

Adrenal insufficiency due to bilateral adrenal metastases - A systematic review and meta-analysis

Objective: Bilateral adrenal metastases may cause adrenal insufficiency (AI) but it is unclear if screening for AI in patients with bilateral adrenal metastases is justified, despite the potential for adrenal crises. Method: A search using PubMed/Medline, ScienceDirect and Cochrane Reviews was performed to collect all original research articles and all case reports from the past 50 years that describe AI in bilateral adrenal metastases. Results: Twenty studies were included with 6 original research articles, 13 case reports and one case series. The quality was generally poor. The prevalence of AI was 3–8%. Of all cases of AI (n ¼ 25) the mean pooled baseline cortisol was 318 237 nmol/L and stimulated 423 238 nmol/L. Hypotension was present in 69%, hyponatremia in 9% and hyperkalemia in 100%. Lung cancer was the cause in 35%, colorectal 20%, breast cancer 15% and lymphoma 10%. The size of the adrenal metastases was 5.5 2.8 cm (left) and 5.5 3.1 cm (right), respectively. There was no correlation between basal cortisol, stimulated cortisol concentration or ACTH with the size of adrenal metastases. The median time to death was 5.0 months (IQR 0.6–6.5). However, two cases were alive after 12–24 months. Conclusion: The prevalence of AI in patients with bilateral adrenal metastases was low. Prognosis was very poor. Due to the low prevalence of AI, screening is likely only indicated in patients with symptoms and signs suggestive of hypocortisolism

A survey of the aeromycota of Sydney and its correspondence with environmental conditions: grass as a component of urban forestry could be a major determinant

© 2015, Springer Science+Business Media Dordrecht. A comprehensive survey of airborne fungi has been lacking for the Sydney region. This study determined the diversity and abundance of outdoor airborne fungal concentrations in urban Sydney. Monthly air samples were taken from 11 sites in central Sydney, and culturable fungi identified and quantified. The genus Cladosporium was the most frequently isolated fungal genus, with a frequency of 78 % and a mean density of 335 CFU m−3. The next most frequently encountered genus was Alternaria, occurring in 53 % of samples with a mean of 124 CFU m−3. Other frequently identified fungi, in decreasing occurrence, were as follows: Penicillium, Fusarium, Epicoccum, Phoma, Acremonium and Aureobasidium. Additionally, seasonal and spatial trends of airborne fungi were assessed, with increases in total culturable fungal concentrations experienced in the summer months. The correspondence between a range of key environmental variables and the phenology of airborne fungal propagules was also examined, with temperature, wind speed and proximal greenspace having the largest influence on fungal propagule density. If the greenspace was comprised of grass, stronger associations with fungal behaviour were observed

Indoor air pollutants in occupational buildings in a sub-tropical climate: Comparison among ventilation types

© 2016 Elsevier Ltd. Few studies have concurrently assessed both abiotic and biotic air pollutants in the built environment in sub-tropical areas. The investigation comprised a field study of air pollutants in eleven indoor environments in Sydney throughout one year, to elucidate Indoor/Outdoor ratios of carbon dioxide, carbon monoxide, total volatile organic compounds, nitric oxide, nitrogen dioxide, sulfur dioxide, total suspended particulate matter, suspended particles <10 μm in diameter (PM10) and particulate matter <2.5 μm (PM2.5). Further, a concurrent assessment of airborne fungi was conducted along with the other air pollutants to determine their diversity and abundance for urban Sydney and to establish baseline Indoor/Outdoor ratios of airborne fungi. Building ventilation types were identified as natural, mechanical and mixed-type ventilation, to assess whether building ventilation type has an impact on prevalence and concentrations of indoor air pollutants. We found that generally the indoor air quality of a typical Australian office building is relatively good. The ventilation type of the buildings did affect indoor air quality; however not to the extent that occupant health was at risk in any case. Low concentrations of airborne fungi were encountered in samples, across all buildings and months, with naturally ventilated buildings having higher concentrations. Buildings with high airborne fungal concentrations also supported higher diversity of fungal species. Few organisms of concern to public health were identified. Significant differences were observed when comparing the structure of airborne fungal communities across building types, with buildings with centralised mechanical (air conditioning) systems harbouring different communities to the other ventilation types

Correspondence Between Urban Bird Roosts and the Presence of Aerosolised Fungal Pathogens

© 2016, Springer Science+Business Media Dordrecht. Habitat fragmentation in urban environments concentrates bird populations that have managed to adapt to these newly developed areas. Consequently, the roosts of these birds are potentially creating environments conducive to fungal growth and dissemination. Airborne fungi derived from these environments are relatively unstudied, as is the potential health risk arising from these fungi. This study documented the diversity of culturable airborne fungal propagules associated with forty urban bird roosts. Environmental variables from each site were recorded to allow us to analyse the correspondence between different bird species, the substrate they occupy and airborne fungal propagules. Associations were established between Rhodotorula and Pacific black ducks, wood ducks, myna birds and miner birds when in the presence of bare soil as a substrate. Further associations were established between Penicillium, Scopulariopsis and Cunninghamella and pigeons, sparrows and swallows living in areas with hard surfaces such as bitumen and rocks

Using pot plants to clean indoor air

Polluted indoor air, air contaminated by Volatile Organic Compounds (VOCs), are a major cause of headaches, nausea, concentration loss and other `building-related illnesses. Previous laboratory research by the Plants and Environmental Quality Group at the University of Technology, Sydney (UTS) has shown that the `pot plant system (plants-and-potting-mix combination) can daily eliminate several times the Australian maximum exposure concentrations of the common VOCs benzene and n-hexane

Case report of whole genome sequencing in the XY female: identification of a novel SRY mutation and revision of a misdiagnosis of androgen insensitivity syndrome

Background: The 46,XY female is characterised by a male karyotype and female phenotype arising due to any interruption in the sexual development pathways in utero. The cause is usually genetic and various genes are implicated. Case presentation: Herein we describe a 46,XY woman who was first diagnosed with androgen insensitivity syndrome (testicular feminisation) at 18 years; however, this was later questioned due to the presence of intact Müllerian structures. The clinical phenotype suggested several susceptibility genes including SRY, DHH, NR5A1, NR0B1, AR, AMH, and AMHR2. To study candidate genes simultaneously, we performed whole genome sequencing. This revealed a novel and likely pathogenic missense variant (p.Arg130Pro, c.389G>C) in SRY, one of the major genes implicated in complete gonadal dysgenesis, hence securing this condition over androgen insensitivity syndrome as the cause of the patient’s disorder of sexual development. Conclusion: This case highlights the emerging clinical utility of whole genome sequencing as a tool in differentiating disorders of sexual development.Sunita M. C. De Sousa, Karin S. Kassahn, Liam C. McIntyre, Chan-Eng Chong, Hamish S. Scott and David J. Torp

Hospital admission patterns in children with CAH: admission rates and adrenal crises decline with age

Objective: To examine patterns of hospitalisation for acute medical conditions in children with congenital adrenal hyperplasia (CAH). Design: A retrospective study of hospitalisation using administrative data. Setting. All hospitals in NSW, Australia. Patients: All patients admitted with CAH and a random sample of admissions in patients aged 0 to 18 years without adrenal insufficiency (AI). Main Outcome Measures: Admissions and comorbidities by age and sex. Results: Of 573 admissions for medical problems in CAH children, 286 (49.9%) were in males, and 236 (41.2%) had a principal diagnosis of CAH or had an adrenal crisis (AC). 37 (6.5%) ACs were recorded. An infection was found in 43.5% ( = 249) of the CAH patient admissions and 51.7% ( = 1613) of the non-AI group, \u3c 0.001. Children aged up to one year had the highest number of admissions ( = 149) and six ACs (four in males).There were 21 ACs recorded for children aged 1–5 years. Older CAH children had fewer admissions and fewer ACs. No in-hospital deaths were recorded. Conclusions. Admission for medical problems in CAH children declines with age. An AC was recorded in 6.5% of the admissions, with the majority of ACs occurring in the 1 to 5 years age group and there were no deaths