Amyloidosis is a disease condition in which the extracellular dep

Amyloidosis is a disease condition in which the extracellular deposition of pathological

insoluble fibrillar proteins occurs in various organs that can impair tissue structure and function.1) Amyloidosis may be systemic or localized and is currently classified according to the type of precursor protein.2) The three most frequent types of systemic Inhibitors,research,lifescience,medical amyloidosis are acquired monoclonal immunoglobulin light-chain amyloidosis, familial transthyretin amyloidosis (ATTR), which can be caused by mutations of transthyretin (TTR), and systemic senile amyloidosis related to wild-type TTR.3) TTR is one of over 20 known proteins that are capable of forming amyloid Protein Tyrosine Kinase inhibitor fibrils in vivo. Although both wild-type and mutated TTR can form amyloid, alterations in the primary structure of the TTR protein due to TTR mutations can result in greatly accelerated amyloid formation and these mutation are the origin of Inhibitors,research,lifescience,medical all symptomatic cases of ATTR.3),4) More than

100 amyloidogenic TTR mutations have been reported in association with markedly variable clinical features, disease penetrances, Inhibitors,research,lifescience,medical and prognoses.5-7) Cardiac involvement in amyloidosis is a manifestation of one of several systemic diseases known as amyloidoses.1),8) Regardless of the underlying pathogenesis, cardiac amyloidosis is characterized by extracellular amyloid infiltration throughout the heart. This infiltrative process results in biventricular wall thickening with non-dilated ventricles and may Inhibitors,research,lifescience,medical also involve the conduction system.9) However, cardiac involvement may be the predominant feature or be found during investigation in a patient presenting with another major organ involvement.10) Furthermore, the initial suspicion of cardiac amyloidosis

is often triggered by the recognition that the heart disease is part of a multiorgan disorder.10) Here, we report a case of cardiac amyloidosis, suspected based upon two-dimensional echocardiographic findings, which presented with autonomic dysfunction, chronic gastrointestinal symptoms, and uncertain cardiac symptoms. Case In July 2010, a 53-year-old man was admitted to Inhibitors,research,lifescience,medical our neurology clinic for the evaluation of dizziness and headache of three years duration. He had not been diagnosed with hypertension, diabetes, or pulmonary disease. The patient presented with chronic diarrhea and a residual urine sensation of several years duration, and reported PDK4 taking an alpha-blocker to treat the latter. In addition, he complained of non-rotating dizziness aggravated by abrupt standing for 1 years. However, despite of the cessation of alpha-blocker treatment, which could have been responsible for the orthostatic hypotension, the non-rotating dizziness did not improve. On physical examination, blood pressure was 108/65 mmHg and his pulse rate was regular at 60 beats/min in the supine position. However, when the patient changed posture from supine to standing, his blood pressure fell below 80/45 mmHg.

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