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Keywords:

  • amyloidosis;
  • autonomic nervous system disorders;
  • gastric emptying;
  • hereditary;
  • nutrition status;
  • transthyretin

Abstract

Background  Gastrointestinal (GI) complications are common in hereditary transthyretin amyloidosis and an autonomic dysfunction has been considered to explain these symptoms. The aim of this study was to investigate the impact of autonomic neuropathy on gastric emptying in hereditary transthyretin amyloidosis and to relate these findings to nutritional status, GI symptoms, gender, and age at disease onset.

Methods  Gastric emptying was evaluated with gastric emptying scintigraphy. Spectral analysis of the heart rate variability and cardiovascular responses after tilt test were used to assess the autonomic function. The nutritional status was evaluated with the modified body mass index (s-albumine × BMI).

Key Results  Gastric retention was found in about one-third of the patients. A weak correlation was found between the scintigraphic gastric emptying rate and both the sympathetic (rs = −0.397, P < 0.001) and parasympathetic function (rs = −0.282, P = 0.002). The gastric emptying rate was slower in those with lower or both upper and lower GI symptoms compared with those without symptoms (median T50 123 vs 113 min, P = 0.042 and 192 vs 113 min, P = 0.003, respectively). Multiple logistic regression analysis showed that age of onset (OR 0.10, CI 0.02–0.52) and sympathetic dysfunction (OR 0.23, CI 0.10–0.51), but not gender (OR 0.76, CI 0.31–1.84) and parasympathetic dysfunction (OR 1.81, CI 0.72–4.56), contributed to gastric retention.

Conclusions and Inferences  Gastric retention is common in hereditary transthyretin amyloidosis early after onset. Autonomic neuropathy only weakly correlates with gastric retention and therefore additional factors must be involved.