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ItemNo Preview AvailableNeural pathways for colorectal control, relevance to spinal cord injury and treatment: a narrative review(NATURE PUBLISHING GROUP, 2018-03)STUDY DESIGN: Narrative review. OBJECTIVES: The purpose is to review the organisation of the nerve pathways that control defecation and to relate this knowledge to the deficits in colorectal function after SCI. METHODS: A literature review was conducted to identify salient features of defecation control pathways and the functional consequences of damage to these pathways in SCI. RESULTS: The control pathways for defecation have separate pontine centres under cortical control that influence defecation. The pontine centres connect, separately, with autonomic preganglionic neurons of the spinal defecation centres and somatic motor neurons of Onuf's nucleus in the sacral spinal cord. Organised propulsive motor patterns can be generated by stimulation of the spinal defecation centres. Activation of the somatic neurons contracts the external sphincter. The analysis aids in interpreting the consequences of SCI and predicts therapeutic strategies. CONCLUSIONS: Analysis of the bowel control circuits identifies sites at which bowel function may be modulated after SCI. Colokinetic drugs that elicit propulsive contractions of the colorectum may provide valuable augmentation of non-pharmacological bowel management procedures.
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ItemNo Preview AvailableThe Role of 68Ga-DOTA-Octreotate PET/CT in Follow-Up of SDH-Associated Pheochromocytoma and Paraganglioma(ENDOCRINE SOCIETY, 2019-11-01)Purpose: Germline succinate dehydrogenase (SDHx) mutation carriers, especially SDHB, are at increased risk for malignancy and require life-long surveillance. Current guidelines recommend periodic whole-body MRI imaging. We assessed the incremental value of 68Ga-DOTA-octreotate (GaTate) positron emission tomography (PET)/CT compared with conventional imaging in such patients. Methods: SDHx mutation carriers who had GaTate PET/CT were retrospectively reviewed. Detection of lesions were compared with MRI or CT on a per-patient and per-lesion basis. Proof of lesions were based on histopathology or clinical/imaging follow-up. Results: Twenty consecutive patients (median age, 46 years; 10 males) were reviewed. Fourteen patients had SDHB, four, SDHD, one SDHC, and one SDHA mutation. Fifteen had prior surgery and/or radiotherapy. Indications for PET/CT were as follows: 7 patients for surveillance for previously treated disease, 9 residual disease, 2 asymptomatic mutation carriers, and 2 for elevated catecholamines. Median time between modalities was 1.5 months. GaTate PET/CT had higher sensitivity and specificity than conventional imaging. On a per-patient basis: PET/CT sensitivity 100%, specificity 100%; MRI/CT 85% and 50%. Per-lesion basis: PET/CT sensitivity 100%, specificity 75%; MRI/CT 80% and 25%. PET/CT correctly identified additional small nodal and osseous lesions. MRI/CT had more false-positive findings. Change of management resulted in 40% (8/20 patients): 3 received localized treatment instead of observation, 1 changed to observation given extra disease detected, 4 with metastases had radionuclide therapy. Conclusions: GaTate PET/CT provided incremental diagnostic information with consequent management impact in SDHx-pheochromocytoma and paraganglioma. Incorporating this modality as part of a surveillance program seems prudent. Further research is needed to define the optimal surveillance strategy including use of MRI.
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ItemNo Preview AvailableDecrease in serum potassium levels post saline suppression test in primary aldosteronism: an under-recognised phenomenon?(NATURE PUBLISHING GROUP, 2016-11)Seventeen subjects with confirmed primary aldosteronism and stable serum potassium (K) levels ≥ 3.5 mmol l-1 underwent saline suppression testing. They were retrospectively evaluated for changes in serum K levels post test. We found that there was a significant decrease in serum K levels post saline suppression test (3.7 ± 0.05 vs 3.5 ± 0.08, P = 0.01). This effect of saline suppression testing on serum K levels is not well described. We conclude that a decrease in serum K is common post-saline suppression test, even in subjects who are normokalemic pretest. The factors which predispose to the decrease in serum K level post saline load remain unclear.
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ItemNo Preview AvailableDilemmas in metastatic differentiated thyroid cancer: To irradiate, medicate, or palliate?(Jaypee Brothers Medical Publishing, 2016-05-01)Aims: To explore the challenges in the management of metastatic differentiated thyroid cancer. Introduction: Differentiated thyroid cancer (DTC) is the most common form of thyroid cancer. The initial diagnosis of thyroid carcinoma and the distinction between benign and neoplastic disease can be challenging. Radioiodine-refractory metastatic DTC also presents a therapeutic dilemma. Novel targeted agents for advanced radioiodine-refractory metastatic thyroid cancer, such as tyrosine kinase inhibitors (TKIs), are being increasingly used with clinical success, broadening current available therapeutic options. Case report: We present the case of a 61-year-old woman with radioiodine-refractory metastatic follicular thyroid carcinoma, which was initially misdiagnosed as benign Hurthle cell adenoma. We focus on the challenges in both the initial diagnosis and the subsequent management of her advanced disease with skeletal dominant metastases. Conclusion: The advent of targeted systemic therapies as emerging frontline and salvage therapy is a novel addition to the management of radioiodine-refractory advanced DTC. Further studies to expand the role of sequential and redifferentiation therapy for advanced disease and strategies to reduce skeletalrelated events are still required.
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ItemNo Preview AvailableHyponatraemia and hypopituitarism: an easily missed entity(AUSTRALASIAN MED PUBL CO LTD, 2017-10-02)
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ItemNo Preview AvailableNeuropredictors of oromotor feeding impairment in 12 month old children (vol 128, pg 49, 2017)(ELSEVIER IRELAND LTD, 2019-01)The publisher regrets that the abstract is incomplete in the published manuscript. Please find below the complete abstract., 1, Background, Feeding impairment is prevalent in children with neurodevelopmental issues. Neuroimaging and neurobehavioral outcomes at term are predictive of later neuromotor impairment, but it is unknown whether they predict feeding impairment., 2, Aims, To determine whether neurobehavior and brain magnetic resonance imaging (MRI) at term predict oromotor feeding at 12 months in preterm and term-born children., 3, Study design, Prospective cohort study., 4, Subjects, 248 infants (97 born <30 weeks and 151 born at term) recruited at birth., 5, Outcome measures, Neurobehavioral assessments (General Movements (GMA), Hammersmith Neonatal Neurological Examination (HNNE), Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS)); and brain MRI were administered at term-equivalent age. Oromotor feeding was assessed at 12 months corrected age using the Schedule for Oral Motor Assessment., 6, Results, 49/227 children had oromotor feeding impairment. Neurobehavior associated with later feeding impairment was: suboptimal NNNS stress (odds ratio [OR] 2.68; 95% confidence interval [CI] 1.20–6.01), non-optimal reflexes (OR 3.33; 95% CI 1.37–8.11) and arousal scales (OR 2.54; 95% CI 1.03–6.27); suboptimal HNNE total (OR 4.69; 95% CI 2.20–10.00), reflexes (OR 2.62; 95% CI 1.06–6.49), and tone scores (OR 3.87; 95% CI 1.45–10.35); and abnormal GMA (OR 2.60; 95% CI 1.21–5.57). Smaller biparietal diameter also predicted feeding impairment (OR 0.88; 95% CI 0.79–0.97). There was little evidence that relationships differed between birth groups., 7, Conclusions, Neurobehavior and biparietal diameter at term are associated with oromotor feeding at 12 months. These results may identify children at greatest risk of oromotor feeding impairment. The publisher would like to apologise for any inconvenience caused.
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ItemIs there an inherent conflict in managing fire for people and conservation?(CSIRO PUBLISHING, 2017)Wildfires are a natural disturbance in many ecosystems, creating challenges for land management agencies who need to simultaneously reduce risk to people and maintain ecological values. Here we use the PHOENIX RapidFire fire behaviour simulator to compare fuel treatment strategies that meet the twin objectives of reducing wildfire risk to human settlements and a fire sensitive endangered species, the koala (Phascolarctos cinereus) in south-eastern Australia. The local koala population is in decline and a conservation management plan is being prepared to exclude wildfire for a 10-year period to assist with population recovery. Twelve scenarios developed by the land management agencies were compared using four indicators: wildfire size; burn probability; impact from exposure to fire; and treatment cost. Compared with the current risk setting, three treatment scenarios were found to reduce wildfire size and burn probability concurrently to both people and koalas. These strategies worked by increasing the landscape area treated, which came with increased financial cost. However, the impact from exposure to fire for both property and koala habitat remains high. Additional complementary strategies beyond landscape fuel reductions are needed to reduce impact from exposure in the event of a wildfire.
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ItemAntiepileptic Medications Increase Osteoporosis Risk in Male Fabry Patients: Bone Mineral Density in an Australian Cohort(SPRINGER-VERLAG BERLIN, 2014)BACKGROUND: Fabry disease (FD) is an inherited X-linked lysosomal storage disease with widespread clinical manifestations. Small prospective studies have shown increased osteopenia and osteoporosis in male FD patients. Limited information however exists about bone metabolism and osteoporosis risk factors within this group. We reviewed osteoporosis risk factors within our cohort. METHODS: A retrospective analysis of bone mineral density (BMD) results and fracture incidence in 44 patients (22 males and 22 females) was undertaken. Dual X-ray absorptiometry scans were performed at the lumbar spine, hip and femoral neck. The impact of risk factors including renal function, antiepileptic drug (AED), analgesia and vitamin D levels were assessed. RESULTS: Male FD patients had low T scores at all sites (spine -1.2 ± 1.06, hip -1.6 ± 0.9, femoral neck -2.23 ± 1.01). Female T scores showed more typical distribution (spine -0.07 ± 1.47, hip 0.02 ± 1.14, femoral neck -0.49 ± 1.31). A higher incidence of osteopenia and/or osteoporosis occurred in males versus females (spine 46.9% versus 31.8%, hip 75.5% versus 18.2% and femoral neck 86.4% versus 45.5%). Multiple regression analysis showed a 50.8% (p < 0.001) reduction in femoral neck BMD with AED usage, after adjustment for age, gender and renal function. Non-traumatic fractures occurred in 27.3% males over 205 patient-years versus 4.6% in females over 149 patient-years, p = 0.095. CONCLUSIONS: Low bone density was highly prevalent in male patients with increased incidence of non-traumatic fractures. AED usage significantly reduces BMD. Treatment to prevent BMD deterioration will depend on determining the bone turnover status.
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ItemEffect of Reduced Agalsidase Beta Dosage in Fabry Patients: The Australian Experience(SPRINGER-VERLAG BERLIN, 2012)BACKGROUND: In Australia, enzyme replacement therapy (ERT) for Fabry Disease (FD), both Agalsidase alfa (Replagal, Shire HGT) and beta (Fabrazyme, Genzyme), is funded and monitored through a specific government program. Agalsidase beta supply has been rationed by Genzyme since 2009 due to manufacturing issues. Consequently, the Australian Fabry Disease Advisory Committee has treated patients on Agalsidase beta at 50% of their usual dose from mid-2009, with a further reduction to 30% for some patients from late 2009. AIM: To determine the clinical effect of Agalsidase beta dose reduction in the Australian FD patient cohort. METHODS: A questionnaire assessing FD symptoms was administered to 40 patients on long-term ERT. Clinical data from The Fabry Registry for patients receiving Agalsidase alfa or beta, for at least 2 years prior to the time of enforced Agalsidase beta dose reduction, were reviewed. Disease burden and quality of life (QOL) were graded using the Disease Severity Scoring System, Mainz Severity Score Index, Brief Pain Inventory and Short Form 36 Health Survey at 2 years before dose reduction, at the time of dose reduction and at the most recent clinical review following dose reduction. RESULTS: Disease severity and QOL scores did not change between the ERT groups. Males on Agalsidase beta reported lower energy levels after dose reduction, while no change was reported by females on either product or by males on a stable dose of Agalsidase alfa. CONCLUSION: This study suggests that energy levels in male patients worsen after dose reduction of Agalsidase beta.
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ItemSevere Infusion Reactions to Fabry Enzyme Replacement Therapy: Rechallenge After Tracheostomy(SPRINGER-VERLAG BERLIN, 2012)A 34-year-old male patient with Fabry disease (OMIM 301500) commenced enzyme replacement therapy (ERT) with Agalsidase alfa, with positive clinical response. Infusion reactions, initially mild and easily managed, commenced during his 13th infusion, and continued over the next 3 years. Severity of reactions subsequently increased despite very slow infusion, extended prophylactic medication and attempted desensitisation, requiring regular intensive care unit (ICU) admissions. Facial oedema and flushing, throat tightness, headache and joint pain typically occurred 4-36 h after completion of most infusions, responding rapidly to subcutaneous adrenaline. Low titre specific IgG seroconversion was noted at 12 months, with subsequent reversion to negative after 5 years, despite persistence of infusion reactions. Specific IgE and skin testing was negative. Trial of ERT product switch to Agalsidase-beta resulted in no improvement in reactions. At 5 years, ERT was ceased in the face of recurrent ICU readmissions. In the face of progressive clinical deterioration, he underwent tracheostomy to allow recommencement of ERT. Two years later, he has clinically improved on regular attenuated dose Agalsidase-beta, administered by slow infusion in a local hospital setting.