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Guideline of transthyretin-related hereditary amyloidosis for clinicians

Transthyretin amyloidosis is a progressive and eventually fatal disease primarily characterized by sensory, motor, and autonomic neuropathy and/or cardiomyopathy. Given its phenotypic unpredictability and variability, transthyretin amyloidosis can be difficult to recognize and manage. Misdiagnosis is common, and patients may wait several years before accurate diagnosis, risking additional significant irreversib...

Utilização do diagnóstico genético pré-implantação por portadores de paramiloidose

Variations of the soft tissue thicknesses external to the ribs in Pectus Excava...

BACKGROUND: Surgical repair of pectus excavatum (PE) has become more popular due to improvements in the minimally invasive Nuss procedure. The pre-surgical assessment of PE patients requires Computerized Tomography (CT), as the malformation characteristics vary from patient to patient. OBJECTIVE: This work aims to characterize soft tissue thickness (STT) external to the ribs among PE patients. It also presents ...

Variations of the soft tissue thicknesses external to the ribs in Pectus Excava...

BACKGROUND: Surgical repair of pectus excavatum (PE) has become more popular due to improvements in the minimally invasive Nuss procedure. The pre-surgical assessment of PE patients requires Computerized Tomography (CT), as the malformation characteristics vary from patient to patient. OBJECTIVE: This work aims to characterize soft tissue thickness (STT) external to the ribs among PE patients. It also presents ...

Long-term effects of tafamidis for the treatment of transthyretin familial amyl...

Tafamidis, a transthyretin (TTR) kinetic stabilizer, delayed neuropathic progression in patients with Val30Met TTR familial amyloid polyneuropathy (TTR-FAP) in an 18-month randomized controlled trial (study Fx-005). This 12-month, open-label extension study evaluated the long-term safety, tolerability, and efficacy of tafamidis 20 mg once daily in 86 patients who earlier received blinded treatment with tafamidi...

Avaliação económica da implementação de um programa de nutrição parenteral domi...

Neuregulin1/ErbB system: importance in the control of cardiovascular function

The family of Neuregulins (NRG), growth factors like epidermal growth factor, is known to induce growth and differentiation of epithelial, glial, neuronal, and skeletal muscle cells. This family comprises four members, being NRG1 the most largely studied, particularly at the cardiovascular level. The biological effects of NRG1 in the adult heart are mediated by the tyrosine kinase receptors ErbB. In the adult h...

Moderate exercise training provides left ventricular tolerance to acute pressur...

The present study evaluated the impact of moderate exercise training on the cardiac tolerance to acute pressure overload. Male Wistar rats were randomly submitted to exercise training or sedentary lifestyle for 14 wk. At the end of this period, the animals were anaesthetized, mechanically ventilated, and submitted to hemodynamic evaluation with biventricular tip pressure manometers. Acute pressure overload was ...

Current pathophysiological concepts and management of pulmonary hypertension

Pulmonary hypertension (PH), increasingly recognized as a major health burden, remains underdiagnosed due mainly to the unspecific symptoms. Pulmonary arterial hypertension (PAH) has been extensively investigated. Pathophysiological knowledge derives mostly from experimental models. Paradoxically, common non-PAH PH forms remain largely unexplored. Drugs targeting lung vascular tonus became available during the ...

Comparison of different methods of heart rate entropy analysis during acute ano...

Acute life-threatening situations are particularly critical when superimposed on chronic diseases. The objective of this study was the assessment of heart rate (HR) dynamics during episodes of acute anoxia superimposed on a rat model of chronic pulmonary hypertension. In 10 adult Wistar rats, five weeks after pulmonary hypertension induction with Monocrotaline, we analysed eight 1-min HR segments, during episod...