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Clin Auton Res. 2019 Aug 31. doi: 10.1007/s10286-019-00630-y. [Epub ahead of print]

Characteristics and natural history of autonomic involvement in hereditary ATTR amyloidosis: a systematic review.

Author information

1
Department of Neurology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Co. Belisario Domínguez, Sección XVI, Tlalpan, Mexico City, 14080, Mexico. gonzalezduarte@aol.com.
2
Department of Neurology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Co. Belisario Domínguez, Sección XVI, Tlalpan, Mexico City, 14080, Mexico.

Abstract

BACKGROUND:

Autonomic dysfunction is a hallmark feature of hereditary ATTR amyloidosis. The aim of this study was to summarize the characteristics and natural history of autonomic dysfunction in patients with hereditary ATTR amyloidosis.

METHODS:

A systematic review of the natural history and clinical trials of patients with ATTR amyloidosis was performed. Alternative surrogate markers of autonomic function were analyzed to understand the prevalence and outcome of autonomic dysfunction.

RESULTS:

Patients with early-onset disease displayed autonomic dysfunction more distinctively than those with late-onset disease. The nutritional status and some autonomic items in the quality-of-life questionnaires were used to assess the indirect progression of autonomic dysfunction in most studies. Gastrointestinal symptoms and orthostatic hypotension were resent earlier than urogenital complications. Once symptoms were present, their evolution was equivalent to the progression of the motor and sensory neuropathy impairment.

CONCLUSION:

The development of autonomic dysfunction impacts morbidity, disease progression, and mortality in patients with hereditary ATTR amyloidosis.

KEYWORDS:

ATTR amyloidosis; Autonomic dysfunction; Autonomic symptoms; Dysautonomia; Familial amyloid polyneuropathy; Orthostatic hypotension

PMID:
31473866
DOI:
10.1007/s10286-019-00630-y

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