PMID- 22277512
OWN - NLM
STAT- MEDLINE
DCOM- 20120918
LR  - 20190918
IS  - 1882-0654 (Electronic)
IS  - 0009-918X (Linking)
VI  - 51
IP  - 11
DP  - 2011 Nov
TI  - [Metabolic turn over of amyloid fibrils and post-treatment regression of amyloid 
      deposits in systemic amyloidosis with polyneuropathy].
PG  - 1143-5
AB  - Systemic amyloidosis that includes familial transthyretin (TTR)-related amyloid 
      polyneuropathy (FAP) and primary systemic immunoglobulin light chain (AL)-derived 
      amyloidosis was long considered to be an incurable disease, but effective 
      therapeutic approaches developed during 20 years ago: liver transplantation for 
      FAP and high dose melphalan with autologous peripheral blood stem cell 
      transplantation (Auto-PBSCT). Systemic amyloidosis is characterized by the 
      presence of an amyloid precursor protein in serum and both treatments can cease 
      the production of amyloid precursor proteins in serum. After transplantation the 
      progression of FAP symptoms certainly stopped, and the patients who were in an 
      early stage of the disease and underwent successful operation showed considerable 
      improvement in their quality of life. Similar clinical recovery was seen in 
      primary systemic AL amyloidosis patients with polyneuropathy after high dose 
      melphalan with auto-PBSCT. Histopathological regression of amyloid deposits on 
      aspirated abdominal fat tissues and/or gastroduodenal mucosa was demonstrated in 
      the patients with long post-treatment courses, indicating that an amyloid 
      precursor protein is dynamically turned over in the lesions with amyloid 
      deposits.
FAU - Ikeda, Shu-Ichi
AU  - Ikeda S
AD  - Department of Medicine (Neurology and Rheumatology), Shinshu University School of 
      Medicine.
LA  - jpn
PT  - English Abstract
PT  - Journal Article
PL  - Japan
TA  - Rinsho Shinkeigaku
JT  - Rinsho shinkeigaku = Clinical neurology
JID - 0417466
RN  - 0 (Amyloid)
SB  - IM
MH  - Amyloid/*metabolism
MH  - Amyloid Neuropathies, Familial/*metabolism/therapy
MH  - Amyloidosis/*metabolism/therapy
MH  - Humans
EDAT- 2012/01/27 06:00
MHDA- 2012/09/19 06:00
CRDT- 2012/01/27 06:00
PHST- 2012/01/27 06:00 [entrez]
PHST- 2012/01/27 06:00 [pubmed]
PHST- 2012/09/19 06:00 [medline]
AID - JST.JSTAGE/clinicalneurol/51.1143 [pii]
AID - 10.5692/clinicalneurol.51.1143 [doi]
PST - ppublish
SO  - Rinsho Shinkeigaku. 2011 Nov;51(11):1143-5. doi: 10.5692/clinicalneurol.51.1143.