Hiperoxalúria primária com insuficiência renal crônica terminal em lactente

Objetivo: descrever caso clínico de um lactente com insuficiência renal crônica terminal, causada por hiperoxalúria primária.Método: após revisão da literatura, verifica-se a raridade da doença; na França, a prevalência é de 1,05/milhão, com taxa de incidência de 0,12/milhão/ano. Pesquisa abordando centros especializados mundiais detectou, em 1999, 78 casos em lactentes; destes, em 14% o quadro inicial foi de uremia. A gravidade e a raridade da doença sugerem o relato deste caso.Resultados: criança de sexo feminino, com quadro de vômitos e baixo ganho de peso desde os primeiros meses de vida, desenvolveu insuficiência renal terminal aos 6 meses de idade, sendo mantida em tratamento dialítico desde então. Aos 8 meses, foi encaminhada para esclarecimento diagnóstico, apresentando déficit pôndero-estatural grave e os seguintes exames laboratoriais: uréia= 69 mg/dl, creatinina=2,2 mg/dl e clearance de creatinina= 12,5 ml/min/1.73m²SC. O exame de urina foi normal, a ultra-sonografia renal revelou tamanho normal e hiperecogenicidade de ambos os rins. A dosagem de oxalato urinário foi de 9,2mg/kg/dia ou 0,55 mmol/1.73m²SC, e a relação oxalato:creatinina, de 0,42. A biópsia renal diagnosticou presença de grande quantidade de depósitos de cristais de oxalato de cálcio no parênquima renal. A radiografia de ossos longos evidenciou sinais sugestivos de osteopatia oxalótica, e a fundoscopia indireta, sinais de retinopatia por oxalato. A criança foi mantida em diálise peritoneal ambulatorial contínua, tendo sido iniciado tratamento com piridoxina.Conclusões: a hiperoxalúria primária deve ser considerada como um dos diagnósticos diferenciais de insuficiência renal crônica em lactentes, especialmente na ausência de história sugestiva de outras patologias.

Systemic racism: the hidden barrier to educational success for Indigenous school students

Explanations for poor educational experiences and results for Australian Indigenous school students have, to a great extent, focused on intended or conscious acts or omissions. This paper adopts an analysis based on the legislation prohibiting indirect racial discrimination. Using the elements of the legislation and case law it argues that apparently benign and race-neutral policies and practices may unwittingly be having an adverse impact on Indigenous students' education. These practices or policies include the building blocks of learning, a Eurocentric school culture. Standard English as the language of assessment, legislation to limit schools' legal liability, and teachers' promotions.

In Pursuit of Excellence : A Policy Driven Systemic Initiative to Embed Gifted Education

Embedding gifted education practices requires major professional development strategies supported by transparent, credible and enforceable policy. This paper describes an analysis of a state-wide initiative involving the establishment of a series of schools tasked to develop and disseminate gifted education principles. The authors have been involved with this initiative at a number of levels over a ten-year period. Their involvement culminated in a commissioned review of the program. Extensive qualitative data were purposively collected from all stakeholders and the effectiveness of the initiative is examined from a theoretical framework of policy development and excellence. The findings summarised in this proposal, indicate the achievement of excellence at a systemic level was constrained by lack of vision, leadership and commitment to long term achievements of excellence. At a local level evidence exists that excellence can be manifested when there is synchronicity of vision, purpose, decisions, and actions.

The failure of Australian legislation on indirect discrimination to detect the systemic racism which prevents Aboriginal people from fully participating in the workforce

Government figures put the current indigenous unemployment rate at around 23%, 3 times the unemployment rate for other Australians. This thesis aims to assess whether Australian indirect discrimination legislation can provide a remedy for one of the causes of indigenous unemployment - the systemic discrimination which can result from the mere operation of established procedures of recruitment and hiring. The impact of those practices on indigenous people is examined in the context of an analysis of anti-discrimination legislation and cases from all Australian jurisdictions from the time of the passing of the Racial Discrimination Act by the Commonwealth in 1975 to the present. The thesis finds a number of reasons why the legislation fails to provide equality of opportunity for indigenous people seeking to enter the workforce. In nearly all jurisdictions it is obscurely drafted, used mainly by educated middle class white women, and provides remedies which tend to be compensatory damages rather than change to recruitment policy. White dominance of the legal process has produced legislative and judicial definitions of "race" and "Aboriginality" which focus on biology rather than cultural difference. In the commissions and tribunals complaints of racial discrimination are often rejected on the grounds of being "vexatious" or "frivolous", not reaching the required standard of proof, or not showing a causal connection between race and the conduct complained of. In all jurisdictions the cornerstone of liability is whether a particular employment term, condition or practice is reasonable. The thesis evaluates the approaches taken by appellate courts, including the High Court, and concludes that there is a trend towards an interpretation of reasonableness which favours employer arguments such as economic rationalism, the maintenance of good industrial relations, managerial prerogative to hire and fire, and the protection of majority rights. The thesis recommends that separate, clearly drafted legislation should be passed to address indigenous disadvantage and that indigenous people should be involved in all stages of the process.

Mild systemic hypoxia and photopic visual field sensitivity

Purpose: Flickering stimuli increase the metabolic demand of the retina,making it a sensitive perimetric stimulus to the early onset of retinal disease. We determine whether flickering stimuli are a sensitive indicator of vision deficits resulting from to acute, mild systemic hypoxia when compared to standard static perimetry. Methods: Static and flicker visual perimetry were performed in 14 healthy young participants while breathing 12% oxygen (hypoxia) under photopic illumination. The hypoxia visual field data were compared with the field data measured during normoxia. Absolute sensitivities (in dB) were analysed in seven concentric rings at 1°, 3°, 6°, 10°, 15°, 22° and 30° eccentricities as well as mean defect (MD) and pattern defect (PD) were calculated. Preliminary data are reported for mesopic light levels. Results: Under photopic illumination, flicker and static visual field sensitivities at all eccentricities were not significantly different between hypoxia and normoxia conditions. The mean defect and pattern defect were not significantly different for either test between the two oxygenation conditions. Conclusion: Although flicker stimulation increases cellular metabolism, flicker photopic visual field impairment is not detected during mild hypoxia. These findings contrast with electrophysiological flicker tests in young participants that show impairment at photopic illumination during the same levels of mild hypoxia. Potential mechanisms contributing to the difference between the visual fields and electrophysiological flicker tests including variability in perimetric data, neuronal adaptation and vascular autoregulation, are considered. The data have implications for the use of visual perimetry in the detection of ischaemic/hypoxic retinal disorders under photopic and mesopic light levels.

Systemic inflammatory responses to maximal versus submaximal lengthening contractions of the elbow flexors

We compared changes in markers of muscle damage and systemic inflammation after submaximal and maximal lengthening muscle contractions of the elbow flexors. Using a cross-over design, 10 healthy young men not involved in resistance training completed a submaximal trial (10 sets of 60 lengthening contractions at 10% maximum isometric strength, 1 min rest between sets), followed by a maximal trial (10 sets of three lengthening contractions at 100% maximum isometric strength, 3 min rest between sets). Lengthening contractions were performed on an isokinetic dynamometer. Opposite arms were used for the submaximal and maximal trials, and the trials were separated by a minimum of two weeks. Blood was sampled before, immediately after, 1 h, 3 h, and 1-4 d after each trial. Total leukocyte and neutrophil numbers, and the serum concentration of soluble tumor necrosis factor-alpha receptor 1 were elevated after both trials (P < 0.01), but there were no differences between the trials. Serum IL-6 concentration was elevated 3 h after the submaximal contractions (P < 0.01). The concentrations of serum tumor necrosis factor-alpha, IL-1 receptor antagonist, IL-10, granulocyte-colony stimulating factor and plasma C-reactive protein remained unchanged following both trials. Maximum isometric strength and range of motion decreased significantly (P < 0.001) after both trials, and were lower from 1-4 days after the maximal contractions compared to the submaximal contractions. Plasma myoglobin concentration and creatine kinase activity, muscle soreness and upper arm circumference all increased after both trials (P < 0.01), but were not significantly different between the trials. Therefore, there were no differences in markers of systemic inflammation, despite evidence of greater muscle damage following maximal versus submaximal lengthening contractions of the elbow flexors.

Does venture opportunity variation matter? Investigating systemic process differences between innovative and imitative new ventures

The central thesis in the article is that the venture creation process is different for innovative versus imitative ventures. This holds up; the pace of the process differs by type of venture as do, in line with theory-based hypotheses, the effects of certain human capital (HC) and social capital (SC) predictors. Importantly, and somewhat unexpectedly, the theoretically derived models using HC, SC, and certain controls are relatively successful explaining progress in the creation process for the minority of innovative ventures, but achieve very limited success for the imitative majority. This may be due to a rationalistic bias in conventional theorizing and suggests that there is need for considerable theoretical development regarding the important phenomenon of new venture creation processes. Another important result is that the building up of instrumental social capital, which we assess comprehensively and as a time variant construct, is important for making progress with both types of ventures, and increasingly, so as the process progresses. This result corroborates with stronger operationalization and more appropriate analysis method what previously published research has only been able to hint at.