The VIIIth International Symposium on Familial Amyloidotic Polyneuropathy and VIIth International Symposium on Liver Transplantation in Familial Amyloidotic Polyneuropathy was held in Kumamoto in Japan, from November 20 to 22, 2011. The two Symposiums focussed on recent news of amyloidogenesis of precursor proteins of FAP, pathogenesis of organ disorders in FAP, managements of FAP patients, and novel therapeutic approaches for FAP. November 23, the day after the Symposiums, the IInd International Symposium on Transthyretin was also held in Kumamoto.
The International Society of Amyloidosis (ISA) has a new website: www.amyloidosis.nl.
During the XIIth International Symposium on Amyloid and Amyloidosis in Rome in April 2010 the new Board of the International Society of Amyloidosis (ISA) has been elected. The new members of the Board are:
President: Martha Skinner, Boston, USA
President Elect: Merrill Benson, Indianapolis, USA
Past President: Giampaolo Merlini, Pavia, Italy
Secretary: Laura Obici, Pavia, Italy
Treasurer: Angela Dispenzieri, Rochester, USA
Representative of the Italian Society: Gina Gregorini, Brescia, Italy
Representative of the German Society: Chris Röcken, Kiel, Germany
Editor-in-Chief of AMYLOID: Per Westermark, Uppsala, Sweden
Chair of the Nomenclature Committee: Merrill Benson, Indianapolis, USA
Members at Large: Bouke Hazenberg, Groningen, The Netherlands; Giovanni Palladini, Pavia, Italy; David Seldin, Boston, USA
The XIIth International Symposium on Amyloidosis has been held in Rome from 18-21 April 2010.
At the end of January 2010 Prof. Dr. Enno Mandema passed away, 88-years-old. He was the chairman and organiser of the first International Symposium on Amyloidosis in Groningen in 1967 and remained interested since that time in the research on amyloidosis. He was one of the founding fathers of amyloid research, not only in Groningen, but also internationally. We will remember him as a good amyloid friend and, as remembered by his family, as a warm, interested and involved human being.
On Saturday 2 May 2009 the new German amyloid Centre was opened with a symposium. This is a very good development that may help to increase the quality of care for patients with amyloidosis within Europe and keep it on the highest level!
Greg Singer, a film maker, has recently made a 12-minute animated film to raise awareness among physicians about amyloidosis. Better awareness of the condition will help to increase proper diagnosis, early treatment and positive outcomes. The goal was reached in completing the film in time for Rare Disease Day (February 28) 2009.
The film, Amyloidosis Awareness, encourages understanding of the main types of amyloidosis, methods of diagnosis, treatment options, and resources for more information. While the film is primarily targeted to doctors, medical students and other health care providers, it will have a broad appeal such that the patient community can also benefit from its message.
Watch the full screen version of the movie or a smaller version of the movie with subtitles via Youtube.
From 3-5 September 2008 a symposium on hereditary ATTR amyloidosis took place in London. Two two-year studies were discussed in which diflunisal (dolocid™) and FoldRx™ are administered to patients with polyneuropathy caused by hereditary ATTR amyloidosis. The two trials started in 2006 and 2007 respectively in the USA, Japan, Sweden, Italy and Portugal and some other countries. Results are not known yet, but both drugs seem to be tolerated faily well by the patients.
In December 2006 a European research consortium (EURAMY) started in Uppsala with a three year collaboration of 12 institutes from 8 European countries, called: Systemic Amyloidosis in Europe. This research project was granted by the Eurpean Communion as part of the 6th Framework Programme and dealed with amyloidosis from basic science to clinical studies.
1st International EURAMY symposium and start meeting, Uppsala, 14 December 2006
2nd International EURAMY meeting, Rome, 20 and 22 September 2007
3rd International EURAMY symposium and meeting on “Systemic amyloidosis and related issues”, Berlin, 15 and 16 February 2008
4th International EURAMY midterm meeting, Paris, 14 and 15 May 2008
5th International EURAMY symposium and meeting on “Systemic amyloidosis and related issues”, Porto, 17 and 18 October 2008
6th International EURAMY symposium and meeting on “Systemic amyloidosis and related issues”, Amsterdam, 3 and 4 April 2009
7th International EURAMY final meeting, Paris, April 28-29, 2010
The co-ordinator of the Consortium is Per Westermark, a Swedish pathologist from Uppsala with outstanding expertise in the field of amyloidosis. The other participants are from Sweden ( Jin-Ping Li, Gunilla Westermark, Erik Lundgren, Ole Suhr), France (Michel Cogné, Gilles Grateau), United Kingdom (Chris Dobson, Philip Hawkins), Italy (Rino Esposito, Giampaolo Merlini), Germany (Chris Röcken), Portugal (Maria Saraiva, Ana Damas), Belgium (Mireille Dumoulin) and The Netherlands (Bouke Hazenberg).
During the 11th International Symposium on Amyloid and Amyloidosis in Woods Hole in November 2006 Alan Cohen has been honored with an Outstanding Achievement Award. Dr Cohen has played a very important role in the field of amyloid research of the last 50 years, so he deserves this award without any doubt.
During the 11th International Symposium on Amyloid and Amyloidosis in Woods Hole in November 2006 a new Board of the International Society of Amyloidosis (ISA) was elected. The elected members of the Board were:
President: Giampaolo Merlini, Pavia, Italy
President Elect: Martha Skinner, Boston, USA
Past President: Robert Kyle, Rochester, USA
Secretary: Bouke Hazenberg, Groningen, The Netherlands
Treasurer: Morie Gertz, Rochester, USA
Representative of the Italian Society: Gina Gregorini, Brescia, Italy
Representative of the German Society: Hartmut Schmidt, Münster, Germany
Editor-in-Chief of AMYLOID: Alan Cohen, Boston, USA
Chair of the Nomenclature Committee: Per Westermark, Uppsala, Sweden
Members at Large: Merrill Benson, Indianapolis, USA; Robert Kisilevsky, Kingston, Canada; Jeffrey Kelly La Jolla, USA
The fat aspiration instruction video is now available as file that can be downloaded (about 6MB) for windows media player. You can download the file here, for more details about the procedure see the part of this website about investigations.
The 11th international Symposium on Amyloid and Amyloidosis has been held in the Marine Biological Laboratories in Woods Hole (Cape Cod), Massachusetts, USA from 5 until 9 November 2006. The next Symposium will be held in Rome in Italy from 18-21 April 2010.
|Semi-quantitative assessment of amyloid in Congo red-stained subcutaneous abdominal fat tissue in systemic amyloidosis and disease severity (download as pdf file)|
|Laryngeal amyloidosis in patients with apolipoprotein AI mutations L174S and L178P (download as pdf file)|
|Serum amyloid P component (SAP) concentration in abdominal fat aspirates of patients with amyloidosis (download as pdf file)|
|Diagnostic accuracy and prognostic value of 123I-labeled serum amyloid P component body retention in patients with amyloidosis (download as pdf file)|
A symposium about familial ATTR amyloidosis has been held in La Jolla (Californië) from 24 until 26 August 2005. One development is the announcement of the start of a two-year trial of diflunisal in patients with familial ATTR amyloidotic polyneuropathy probably in the beginning of 2006 in the USA, Japan, Sweden, Italy, and Portugal. The next ATTR symposium will probably be held in London in 2008.
From 18-22 April 2004 the 10th International Symposium on Amyloid and Amyloidosis has been held in Tours in France. It was a successful and informative symposium with about 300 participants.
|Scintigraphy at different time intervals after administration of 123I labelled serum amyloid P component (SAP) in patients with amyloidosis (download as pdf file)|
|Laryngeal amyloidosis: localized versus systemic disease and update on diagnosis and therapy (download as pdf file)|
|Familial amyloidotic polyneuropathy with severe renal involvement in association with transthyretin Gly47Glu in Dutch, British and American families (download as pdf file)|
|Serum levels of free kappa and lambda light chains in patients with systemic AL, AA, and ATTR amyloidosis (download as pdf file)|
|Diagnostic value of free kappa and lambda light chains in fat tissue of patients with systemic AL amyloidosis (download as pdf file)|
|Fat tissue analysis by Congo red method or by amyloid A protein quantification in clinical AA amyloidosis (download as pdf file)|
An international study to investigate effectiveness of eprodisate (Kiacta™), a drug that possibly can inhibit deposition of AA amyloid in tissue, has been performed in about 150 patients with AA amyloidosis. In december 2004 the last patients have completed the two years of the study. In these two study years Kiacta™ is compared with placebo in a double blind way. The patients have already completed the two years of the study at the end of 2003 and they have been offered the opportunity to enter an open follow-up study of two years in which all participants get the active drug Kiacta™. The first analysis in 2006 has shown that the primary endpoint of the study has been reached (p=0.03). The results have been published as an article in the prestigious medical journal The New England Medical Journal in 2007. However, the drug has not been registered by the regulatory authorities are FDA and EMEA as an anti-amyloid orphan drug (Kiacta™). Both regulatory authoroties demand a second trial before they allow this drug to be registered on the market.
A recent development is the proposal of a number of leading groups involved in the clinincal management of patients with AL amyloidosis to define criteria for organ involvement and response or progression after therapy. The use of common criteria will make it possible to compare studies of different centres. The article in which these criteria are described by Morie Gertz has been published in The American Journal of Hematology in 2005.
Recent biochemical developments are the measurement of free light chains in blood and the measurement of NT-proBMP.
Free light chains are helpful to diagnose AL amyloidosis and to monitor the effect of therapy in patients with this disease. NT-proBNP might be a good marker of cardiac dysfunction in amyloidosis.
Last update 04-07-2012