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Cited by 22Amyloidosis: Diagnosis, Treatment, OutcomesGastrointestinal motility and disordersAlzheimer's disease research and treatments

Abnormal small bowel motility in patients with hereditary transthyretin amyloidosis

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Wixner, J., Törnblom, H., Karling, P., Anan, I., Lindberg, G.·2018·Neurogastroenterology & Motility
DOI 10.1111/nmo.13354
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Abstract

BACKGROUND: Gastrointestinal complications are common in hereditary transthyretin amyloid (ATTRm) amyloidosis. The underlying mechanisms have not been fully elucidated, and the patients' small bowel function remains largely unexplored. The aim of the present study was to compare the small bowel motility in ATTRm amyloidosis patients with that in non-amyloidosis patient controls. METHODS: ATTRm amyloidosis patients undergoing evaluation for liver transplantation were consecutively investigated with 24-hour duodenojejunal manometry (n = 19). The somatostatin analogue octreotide was used to induce fasting motility. Patients with age at onset of ≥50 years were defined as late-onset cases. For each patient, three age- and sex-matched patient controls (n = 57) were selected from the total pool of investigated patients. KEY RESULTS: Manometry was judged as abnormal in 58% of the patients and in 26% of the patient controls (P = .01). Patients displayed significantly more daytime phase III migrating motor complexes than patient controls (median 4 vs 2, P < .01), and had a higher frequency of low-amplitude complexes (16% vs 4%; however, this difference did not reach statistical significance, P = .10). Furthermore, late-onset patients showed a delay in octreotide response (5.4 vs 3.8 minutes, P < .01), but this was not observed for early-onset patients or within the control group. CONCLUSIONS AND INFERENCES: Patients with ATTRm amyloidosis displayed abnormalities in their small bowel motility more frequently than non-amyloidosis patient controls, and the manometric pattern was probably best consistent with a combined neuromyopathic disorder. The delayed octreotide response in late-onset patients warrants further investigation.

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Cited by

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Authors
Wixner, J., Törnblom, H., Karling, P., Anan, I., & Lindberg, G.
Affiliations
  • Umeå University (SE)
  • University of Gothenburg (SE)
  • Karolinska University Hospital (SE)
Corresponding author
Wixner, J.
ORCID
Venue
Neurogastroenterology & Motility
Publisher
Wiley
Published
2018-04-14
Volume
30
Issue
9
Pages
e13354-e13354
ISSN
  • 1350-1925 (ISSN-L)
  • 1365-2982
DOI
10.1111/nmo.13354
OpenAlex
W2797950244
Cited by
22
References
1
Citations by year
2026: 5 · 2025: 3 · 2024: 2 · 2022: 3 · 2021: 2 · 2020: 4 · 2019: 3
FWCI
0.80
Type
journal article
Language
en
Open access
Licence
Locations
  • Neurogastroenterology & Motility (publishedVersion, cc-by-nc)
  • PubMed (publishedVersion)
  • DiVA at Umeå University (Umeå University) (submittedVersion, other-oa)
Funders
  • Swedish Patients’ Organizations FAMY Norrbotten and Västerbotten
  • AMYL Foundation
  • Swedish Gastroenterology Fund (Mag-tarmfonden)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
  • Health Sciences › Medicine › Gastroenterology › Gastrointestinal motility and disorders
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
Keywords
hereditary transthyretin amyloidosis, small intestine motility, migrating motor complex, gastrointestinal complications, neuromyopathy, liver transplantation
MeSH terms
Adult, Aged, Female, Gastrointestinal Diseases, Gastrointestinal Motility, Humans, Intestine, Small, Male, Manometry, Middle Aged, Amyloid Neuropathies, Familial

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