Aim
To examine deprivation measured by the Scottish index of multiple deprivation (SIMD) and its relation to urgent suspicion of head and neck cancer referrals. A secondary aim was to examine the ...symptomatology generating urgent suspicion of cancer (USOC) referrals by SIMD category.
Methods
All “urgent suspicion of cancer” referrals to the GGC ENT department over a one‐year period, between 2015 and 2016, were reviewed. Information was recorded anonymously and included demographics and red flag referral symptoms.
Results
A total of 1998 patients were assessed, 43.4% (n = 867) were male. A total of 171 (8.6%) patients had primary head and neck cancer. A total of 61 patients had other types of cancer, giving an all cause cancer rate of 11.6%. About 71.3% of primary patients with head and neck cancer (HNC) were male. The most common SIMD category observed was SIMD1, the most common SIMD category yielding a primary head and neck cancer diagnosis was SIMD1. Neck lump was the commonest symptom amongst all SIMD categories.
Conclusion
A link between deprivation and USOC referrals has been established. A difference in gender distribution between referrals and HNC was observed, more females are referred but a significantly higher number of patients with HNC are males. Neck lump is a very strong referral indicator for HNC and intermittent hoarseness is not. The findings from this analysis could be used to refine local referral patterns and priority of referral.
Abstract The relationship between body composition and function in spinal muscular atrophy (SMA) is poorly understood. 53 subjects with SMA were stratified by type and Hammersmith functional motor ...scale, expanded score into three cohorts: low-functioning non-ambulatory (type 2 with Hammersmith score <12, n = 19), high-functioning non-ambulatory (type 2 with Hammersmith score ⩾ 12 or non-ambulatory type 3, n = 17), and Ambulatory ( n = 17). Lean and fat mass was estimated using dual-energy X-ray absorptiometry. Anthropometric data was incorporated to measure fat-free (lean mass in kg/stature in m2 ) and fat (fat mass in kg/stature in m2 ) mass indices, the latter compared to published age and sex norms. Feeding dysfunction among type 2 subjects was assessed by questionnaire. Fat mass index was increased in the high-functioning non-ambulatory cohort (10.4 ± 4.5) compared with both the ambulatory (7.2 ± 2.1, P = 0.013) and low-functioning non-ambulatory (7.6 ± 3.1, P = 0.040) cohorts. 12 of 17 subjects (71%) in the high-functioning non-ambulatory cohort had fat mass index >85th percentile for age and gender (connoting “at risk of overweight”) versus 9 of 19 subjects (47%) in the low-functioning non-ambulatory cohort and 8 of 17 ambulatory subjects (47%). Despite differences in clinical function, a similar proportion of low functioning (7/18, 39%) and high functioning (2/7, 29%) type 2 subjects reported swallowing or feeding dysfunction. Non-ambulatory patients with relatively high clinical function may be at particular risk of excess adiposity, perhaps reflecting access to excess calories despite relative immobility, emphasizing the importance of individualized nutritional management in SMA.
Obstetric venous thromboembolism is a leading cause of severe maternal morbidity and mortality. Maternal death from thromboembolism is amenable to prevention, and thromboprophylaxis is the most ...readily implementable means of systematically reducing the maternal death rate. Observational data support the benefit of risk‐factor‐based prophylaxis in reducing obstetric thromboembolism. This bundle, developed by a multidisciplinary working group and published by the National Partnership for Maternal Safety under the guidance of the Council on Patient Safety in Women's Health Care, supports routine thromboembolism risk assessment for obstetric patients, with appropriate use of pharmacologic and mechanical thromboprophylaxis. Safety bundles outline critical clinical practices that should be implemented in every maternity unit. The safety bundle is organized into 4 domains: Readiness, Recognition, Response, and Reporting and Systems Learning. Although the bundle components may be adapted to meet the resources available in individual facilities, standardization within an institution is strongly encouraged.
No treatment is currently available for mucopolysaccharidosis (MPS) IIIB, a neuropathic lysosomal storage disease due to defect in α-N-acetylglucosaminidase (NAGLU). In preparation for a clinical ...trial, we performed an IND-enabling GLP-toxicology study to assess systemic rAAV9-CMV-hNAGLU gene delivery in WT C57BL/6 mice at 1 × 10(14) vg/kg and 2 × 10(14) vg/kg (n = 30/group, M:F = 1:1), and non-GLP testing in MPS IIIB mice at 2 × 10(14) vg/kg. Importantly, no adverse clinical signs or chronic toxicity were observed through the 6 month study duration. The rAAV9-mediated rNAGLU expression was rapid and persistent in virtually all tested CNS and somatic tissues. However, acute liver toxicity occurred in 33% (5/15) WT males in the 2 × 10(14) vg/kg cohort, which was dose-dependent, sex-associated, and genotype-specific, likely due to hepatic rNAGLU overexpression. Interestingly, a significant dose response was observed only in the brain and spinal cord, whereas in the liver at 24 weeks postinfection (pi), NAGLU activity was reduced to endogenous levels in the high dose cohort but remained at supranormal levels in the low dose group. The possibility of rAAV9 germline transmission appears to be minimal. The vector delivery resulted in transient T-cell responses and characteristic acute antibody responses to both AAV9 and rNAGLU in all rAAV9-treated animals, with no detectable impacts on tissue transgene expression. This study demonstrates a generally safe and effective profile, and may have identified the upper dosing limit of rAAV9-CMV-hNAGLU via systemic delivery for the treatment of MPS IIIB.
Thigh muscle volume was assessed using magnetic resonance imaging in 16 subjects with spinal muscular atrophy. Scans were successful for 14 of 16 subjects (1 type 1, 6 type 2, and 7 type 3) as young ...as 5.7 years. Muscle volume with normal and abnormal signal was measured using blinded, semiautomated analysis of reconstructed data. Results were compared with segmental lean mass estimated by dual-energy X-ray absorptiometry and correlated with clinical and electrophysiological measures of disease severity. Muscle volume was reduced with abnormal signal quality. Test-retest reliability (r = .99) and correlation with dual-energy X-ray absorptiometry (r = .91) were excellent. Type 2 subjects had lower volume (3.5 ± 1.6 vs 6.3 ± 2.8 mL/cm height; P = .06) and higher percentage of muscle with abnormal signal (68% ± 20% vs 47% ± 27%; P = .14) than type 3. Reproducibility, tolerability, and strong correlation with clinical measures make magnetic resonance imaging a candidate biomarker for clinical research.
Guaroa virus (GROV) was first isolated from humans in Colombia in 1959. Subsequent isolates of the virus have been recovered from febrile patients and mosquitoes in Brazil, Colombia, and Panama; ...however, association of the virus with human disease has been unclear. As part of a study on the etiology of febrile illnesses in Peru and Bolivia, 14 GROV strains were isolated from patients with febrile illnesses, and 3 additional cases were confirmed by IgM seroconversion. The prevalence rate of GROV antibodies among Iquitos residents was 13%; the highest rates were among persons with occupations such as woodcutters, fisherman, and oil-field workers. Genetic characterization of representative GROV isolates indicated that strains from Peru and Bolivia form a monophyletic group that can be distinguished from strains isolated earlier in Brazil and Colombia. This study confirms GROV as a cause of febrile illness in tropical regions of Central and South America.
Spinal Muscular Atrophy Type III Dunaway, Sally; Montes, Jacqueline; Ryan, Patricia A. ...
Journal of Child Neurology,
06/2012, Letnik:
27, Številka:
6
Book Review, Journal Article
Recenzirano
Spinal muscular atrophy is a relatively stable chronic disease. Patients may gradually experience declines in muscle strength and motor function over time. However, functional progression is ...difficult to document, and the mechanism remains poorly understood. An 11-year-old girl was diagnosed at 19 months and took a few steps without assistance at 25 months. She was evaluated for 54 months in a prospective multicenter natural history study. Outcome measures were performed serially. From 6 to 7.5 years, motor function improved. From 7.5 to 11 years, motor function declined with increasing growth. Manual muscle testing scores minimally decreased. Motor unit number estimation studies gradually increased over 4.5 years. Compared to the published natural history of spinal muscular atrophy type III, our patient lost motor function over time. However, she walked with assistance 2 years longer than expected. Our report highlights possible precipitating factors that could affect the natural history of spinal muscular atrophy type III.
Accurate, noninvasive measures of body composition are needed for management of patients with spinal muscular atrophy. Fat mass index (fat mass/height2 in kg/m2) was measured in 16 subjects with ...spinal muscular atrophy using 5 bioelectrical impedance analysis equations and compared with a reference method, dual-energy x-ray absorptiometry. The machine default equation, validated by Cordain, was the primary analysis. Fat mass index calculated by impedance measures differed by between -2.5 kg/m2 and 1.7 kg/m2 from the reference mean (8.3 ± 5.0 kg/m2). The Cordain equation provided the smallest difference (-0.4 ± 2.0 kg/m2), with correlation coefficient of 0.92. The Cordain equation showed high sensitivity (85.7%) and specificity (100%) for prediction of ‘‘at risk for overweight’’ (fat mass index > 85th percentile for age and gender). Although insufficiently accurate for use as a research tool, bioelectrical impedance can have application as a well-tolerated, noninvasive, easily used screening tool for excess adiposity in patients with spinal muscular atrophy.
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