This research project investigated the orthotic prescription habits of podiatric physicians in Australia and New Zealand. A 23-item questionnaire was distributed to all members of the Australian Podiatry Association and the New Zealand Society of Podiatrists. When asked what type of foot orthoses they prescribe most often, 72% of respondents reported functional foot orthoses; the next most common response was prefabricated orthoses (12%). A typical prescription for functional foot orthoses consisted of a modified Root style orthosis, balanced to the neutral calcaneal stance position, with the shell made from polypropylene and an ethyl vinyl acetate (EVA) rearfoot post applied. The majority of podiatric physicians surveyed used a commercial orthotic laboratory to fabricate their orthoses. However, New Zealand respondents were three times more likely to prescribe prefabricated foot orthoses, and males were twice as likely as females to manufacture the orthoses themselves rather than use a commercial orthotic laboratory.
Patients with PD are more likely to suffer serious health problems, including delirium, adverse drug reactions, syncope, falls and fractures than controls. These findings highlight PD as a multisystem neuropsychiatric disorder in which motor and non-motor features contribute to morbidity. Increased awareness of the added risk PD poses in acute hospitalised patients can be used to inform strategies to improve patient outcomes.
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 = 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.
Context
P450 oxidoreductase deficiency (PORD) is a rare genetic disorder that is associated with significant morbidity. However there has been limited analysis of reported PORD cases.
Objective
To determine, based on the cohort of reported PORD cases, genotype-phenotype relationships for skeletal malformations, maternal virilisation in pregnancy, adrenal insufficiency, and disorders of sexual development (DSD).
Data Sources
PubMed and Web of Science from January 2004 to February 2018.
Study Selection
Published case reports/series of patients with PORD. Eligible patients were unique, had biallelic mutations, and their clinical features were reported.
Data Extraction
Patient data were manually extracted from the text of case reports/series. A malformation score, representing the severity of skeletal malformations, was calculated for each patient.
Data Synthesis
Of the 211 patients published in the literature, 90 were eligible for inclusion. More than 60 unique mutations were identified in this cohort. Four groups of mutations were identified, through regression modeling, as having significantly different skeletal malformation scores. Maternal virilization in pregnancy, reported for 21% of patients, was most common for R457H mutations. Adrenal insufficiency occurred for the majority of patients (78%) and was typically mild, with homozygous R457H mutations being the least deficient. DSD affected most patients (72%), but were less common for males (46XY) with homozygous R457H mutations.
Conclusions
PORD is a complex disorder with many possible mutations affecting a large number of enzymes. By analyzing the cohort of reported PORD cases, this study identified clear relationships between genotype and several important phenotypic features.
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