Amyloidoses are a group of heterogeneous diseases classified according to the nature of their causative amyloid proteins. Commonly, paraffin-embedded tissue is used for the typing of amyloid by immunohistochemistry. DNA analysis should always be considered if hereditary amyloidosis is suspected. Since the kidneys are one of the organs that are most commonly involved in amyloid deposition in systemic amyloidoses...
Pathogenic mutations in genes COL4A3/COL4A4 are responsible for autosomal Alport syndrome (AS) and thin basement membrane nephropathy (TBMN). We used Sanger sequencing to analyze all exons and splice site regions of COL4A3/COL4A4, in 40 unrelated Portuguese probands with clinical suspicion of AS/TBMN. To assess genotype-phenotype correlations, we compared clinically relevant phenotypes/outcomes between homozygo...
This article discusses the overall situation of cultural studies in Portugal. It starts by analysing some of the courses and graduate programmes currently on offer. The results suggest that cultural studies is experiencing a fast academic expansion. While this seems to be entangled with top-down institutional changes, in the wake of the Bologna process and the turn to the cultural/ creative industries and as pa...
Aproveitando a publicação, em 2009, de The Sage Handbook of Tourism Studies e de The Sociology of Tourism: European Origins and Developments – duas extensas antologias sobre o estudo do turismo nas ciências sociais –, o artigo discute as tradições aca- démicas que cada um destes volumes representa e defende, nomeadamente, uma tradição anglófona transatlântica e uma tradição europeia continental. Identificam- -s...
Orthotopic liver transplantation has become the treatment of choice for familial amyloidotic polyneuropathy. The aims of this study were to evaluate the renal complications post orthotopic liver transplantation in familial amyloidotic polyneuropathy and their impact. We retrospectively studied 185 recipients who underwent 217 orthotopic liver transplants. Mean age 36.8±9.5 years, 59% males, 14.3% with renal dys...
O presente estudo registrou as verbalizações inapropriadas de um esquizofrênico adulto e do sexo masculino. Os comportamentos verbais inapropriados foram observados durante breves períodos de exposição a quatro condições: atenção, atenção não contingente, demanda e sozinho. Os resultados indicaram que as condições afetaram os comportamentos verbais inapropriados diferentemente. Esses resultados são discutidos e...
Acute renal failure (ARF) is common after orthotopic liver transplantation (OLT). The aim of this study was to evaluate the prognostic value of RIFLE classification in the development of CKD, hemodialysis requirement, and mortality. Patients were categorized as risk (R), injury (I) or failure (F) according to renal function at day 1, 7 and 21. Final renal function was classified according to K/DIGO guidelines. ...
Renal dysfunction often complicates the course of orthotopic liver transplant recipients and is associated with increased morbid -mortality. The aims of this study were to determine the incidence of chronic renal disease and its impact on patient survival. Clinical data included age, gender and weight,aetiology of hepatic failure, presence of diabetes,hypertension, hepatitis B and C infection, renal dysfunction...
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