Document Detail

Oesophageal dysfunction in familial amyloidosis with polyneuropathy.
MedLine Citation:
PMID:  8435977     Owner:  NLM     Status:  MEDLINE    
Sixteen patients with familial amyloidosis and polyneuropathy (FAP) and 14 health subjects underwent oesophageal manometry. Six of the patients had a severe oesophageal dysmotility with almost completely abolished propulsive pressure waves on swallowing in the lower 2/3 of the oesophagus. Ten patients had moderate dysfunction with reduced propulsive pressure wave amplitudes. Neostigmine increased the pressure wave amplitudes in healthy subjects but less so in the FAP patients. Scopolamine (Scopyl)-terbutaline (Bricanyl) almost abolished the propulsive pressure waves in healthy subjects and all patients in the lower 2/3 of the esophagus. Oesophageal distensibility, tested by inflating a rubber balloon in the oesophagus, was similar in FAP patients and healthy subjects. Thus, it is unlikely that amyloid deposits in the mucosal wall increased the oesophageal stiffness. An autonomic, predominantly vagal, denervation probably explains the disturbed function.
P Bjerle; B Ek; H Linderholm; L Steen
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical physiology (Oxford, England)     Volume:  13     ISSN:  0144-5979     ISO Abbreviation:  Clin Physiol     Publication Date:  1993 Jan 
Date Detail:
Created Date:  1993-03-19     Completed Date:  1993-03-19     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8309768     Medline TA:  Clin Physiol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  57-69     Citation Subset:  IM    
Department of Clinical Physiology, University of Umeå, Sweden.
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MeSH Terms
Amyloidosis / complications*,  genetics
Esophageal Diseases / etiology*,  physiopathology
Gastrointestinal Motility
Middle Aged
Muscle Relaxation / drug effects,  physiology
Neostigmine / pharmacology
Nervous System Diseases / complications*
Scopolamine / pharmacology
Terbutaline / pharmacology
Reg. No./Substance:
23031-25-6/Terbutaline; 51-34-3/Scopolamine; 59-99-4/Neostigmine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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