Document Detail


99mTc-DPD scintigraphy in transthyretin-related familial amyloidotic polyneuropathy.
MedLine Citation:
PMID:  12002714     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Familial amyloidotic polyneuropathy (FAP) caused by amyloidogenic transthyretin (ATTR) mutations is the most common form of hereditary amyloidosis. We investigated the diagnostic value of the bone scanning agent technetium-99m 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) in this disease. Eight patients (four males, four females; age 54.4+/-8.3 years, range 43-67 years) with ATTR-FAP proven by immunohistochemistry and molecular analysis and a control group comprising ten oncological out-patients (five males, five females; age 53.4+/-8.5 years, range 34-66 years) without evidence of bony metastases were studied using 99mTc-DPD. Whole body tracer retention was 80.1%+/-10.3% (range 65.1%-94.8%) in FAP patients and 55.7%+/-8.1% (range 40.2%-66.7%) in controls at 3 h p.i. (P<0.001), and cardiac uptake was 7.3%+/-2.2% (range 4.2%-10.1%) in FAP patients and 3.1%+/-0.5% (range 2.3%-4.0%) in controls (P<0.001). The heart/whole body uptake ratio was 8.9%+/-1.7% (range 6.5%-11.0%) in FAP patients and 5.6%+/-0.5% (range 5.1%-6.8%) in controls (P<0.001). The three FAP patients with the highest cardiac tracer uptake had cardiomyopathy or arrhythmia. 99mTc-DPD scintigraphy is proposed as a simple and valuable diagnostic aid to evaluate the severity of the disease and the risk of concomitant heart problems.
Authors:
Max Puille; Klaus Altland; Reinhold P Linke; Mary K Steen-Müller; Rigobert Kiett; Dagmar Steiner; Richard Bauer
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Publication Detail:
Type:  Clinical Trial; Controlled Clinical Trial; Journal Article    
Journal Detail:
Title:  European journal of nuclear medicine and molecular imaging     Volume:  29     ISSN:  1619-7070     ISO Abbreviation:  Eur. J. Nucl. Med. Mol. Imaging     Publication Date:  2002 Mar 
Date Detail:
Created Date:  2002-05-10     Completed Date:  2002-11-13     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  101140988     Medline TA:  Eur J Nucl Med Mol Imaging     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  376-9     Citation Subset:  IM    
Affiliation:
Department of Nuclear Medicine, Justus-Liebig-University, Giessen, Germany. Max.F.Puille@radiol.med.uni-giessen.de
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Amyloid Neuropathies, Familial / metabolism,  pathology,  radionuclide imaging*
Biopsy
Diphosphonates / diagnostic use*,  pharmacokinetics
Female
Heart / radionuclide imaging*
Humans
Male
Middle Aged
Organotechnetium Compounds / diagnostic use*,  pharmacokinetics
Radiopharmaceuticals / diagnostic use,  pharmacokinetics
Whole-Body Counting
Chemical
Reg. No./Substance:
0/Diphosphonates; 0/Organotechnetium Compounds; 0/Radiopharmaceuticals; 0/technetium Tc 99m 1,1-diphosphonopropane-2,3-dicarboxylic acid

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