PMID- 18344065
OWN - NLM
STAT- MEDLINE
DCOM- 20080411
LR  - 20161124
IS  - 0723-5003 (Print)
IS  - 0723-5003 (Linking)
VI  - 103
IP  - 3
DP  - 2008 Mar 15
TI  - [Cardiac amyloidosis].
PG  - 153-60
LID - 10.1007/s00063-008-1022-2 [doi]
AB  - Amyloidoses are a heterogeneous group of multisystem disorders, which are 
      characterized by an extracellular deposition of amyloid fibrils. Typically 
      affected are the heart, liver, kidneys, and nervous system. More than half of the 
      patients die due to cardiac involvement. Clinical signs of cardiac amyloidosis 
      are edema of the lower limbs, hepatomegaly, ascites and elevated jugular vein 
      pressure, frequently in combination with dyspnea. There can also be chest pain, 
      probably due to microvessel disease. Dysfunction of the autonomous nervous system 
      or arrhythmias may cause low blood pressure, dizziness, or recurrent syncope. The 
      AL amyloidosis caused by the deposition of immunoglobulin light chains is the 
      most common form. It can be performed by monoclonal gammopathy. The desirable 
      treatment therapy consists of high-dose melphalan therapy twice followed by 
      autologous stem cell transplantation. Due to the high peritransplantation 
      mortality, selection of appropriate patients is mandatory. The ATTR amyloidosis 
      is an autosomal dominant disorder caused by the amyloidogenic form of 
      transthyretin, a plasmaprotein that is synthesized in the liver. Therefore, liver 
      transplantation is the only curative therapy. The symptomatic treatment of 
      cardiac amyloidosis is based on the current guidelines for chronic heart failure 
      according to the patient's New York Heart Association (NYHA) state. Further types 
      of amyloidosis with possible cardiac involvement comprise the senile systemic 
      amyloidosis caused by the wild-type transthyretin, secondary amyloidosis after 
      chronic systemic inflammation, and the beta(2)-microglobulin amyloidosis after 
      long-term dialysis treatment.
FAU - Hoyer, Caroline
AU  - Hoyer C
AD  - Medizinische Klinik und Poliklinik I, Universitat Wurzburg, Klinikstrasse 6-8, 
      Wurzburg. Hoyer_C@klinik.uni-wuerzburg.de
FAU - Angermann, Christiane E
AU  - Angermann CE
FAU - Knop, Stefan
AU  - Knop S
FAU - Ertl, Georg
AU  - Ertl G
FAU - Stork, Stefan
AU  - Stork S
LA  - ger
PT  - Journal Article
PT  - Review
TT  - Kardiale Amyloidose.
PL  - Germany
TA  - Med Klin (Munich)
JT  - Medizinische Klinik (Munich, Germany : 1983)
JID - 8303501
RN  - 0 (Immunoglobulin Light Chains)
RN  - Q41OR9510P (Melphalan)
SB  - IM
MH  - Adult
MH  - Aged
MH  - *Amyloidosis/diagnosis/diagnostic imaging/drug 
      therapy/mortality/pathology/therapy
MH  - Amyloidosis, Familial/diagnosis/genetics
MH  - Biopsy
MH  - *Cardiomyopathies/diagnosis/diagnostic imaging/drug 
      therapy/mortality/pathology/therapy
MH  - Echocardiography
MH  - Female
MH  - Humans
MH  - Immunoglobulin Light Chains
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Melphalan/administration & dosage/therapeutic use
MH  - Myocardium/pathology
MH  - Practice Guidelines as Topic
MH  - Prognosis
MH  - Radionuclide Imaging
MH  - Stem Cell Transplantation
MH  - Transplantation, Autologous
RF  - 21
EDAT- 2008/03/18 09:00
MHDA- 2008/04/12 09:00
CRDT- 2008/03/18 09:00
PHST- 2008/03/18 09:00 [pubmed]
PHST- 2008/04/12 09:00 [medline]
PHST- 2008/03/18 09:00 [entrez]
AID - 10.1007/s00063-008-1022-2 [doi]
PST - ppublish
SO  - Med Klin (Munich). 2008 Mar 15;103(3):153-60. doi: 10.1007/s00063-008-1022-2.