Cardiac amyloidosis pathophysiology: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
|||
Line 18: | Line 18: | ||
*All three forms frequently involve the myocardium.<ref name="pmid9302305">{{cite journal |author=Falk RH, Comenzo RL, Skinner M |title=The systemic amyloidoses |journal=[[The New England Journal of Medicine]] |volume=337 |issue=13 |pages=898–909 |year=1997 |month=September |pmid=9302305 |doi=10.1056/NEJM199709253371306 |url=http://www.nejm.org/doi/abs/10.1056/NEJM199709253371306?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed |accessdate=2012-02-13}}</ref><ref name="pmid10319082">{{cite journal |author=Gertz MA, Lacy MQ, Dispenzieri A |title=Amyloidosis: recognition, confirmation, prognosis, and therapy |journal=[[Mayo Clinic Proceedings. Mayo Clinic]] |volume=74 |issue=5 |pages=490–4 |year=1999 |month=May |pmid=10319082 |doi=10.4065/74.5.490 |url=http://linkinghub.elsevier.com/retrieve/pii/S0025-6196(11)65130-6 |accessdate=2012-02-13}}</ref> | *All three forms frequently involve the myocardium.<ref name="pmid9302305">{{cite journal |author=Falk RH, Comenzo RL, Skinner M |title=The systemic amyloidoses |journal=[[The New England Journal of Medicine]] |volume=337 |issue=13 |pages=898–909 |year=1997 |month=September |pmid=9302305 |doi=10.1056/NEJM199709253371306 |url=http://www.nejm.org/doi/abs/10.1056/NEJM199709253371306?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed |accessdate=2012-02-13}}</ref><ref name="pmid10319082">{{cite journal |author=Gertz MA, Lacy MQ, Dispenzieri A |title=Amyloidosis: recognition, confirmation, prognosis, and therapy |journal=[[Mayo Clinic Proceedings. Mayo Clinic]] |volume=74 |issue=5 |pages=490–4 |year=1999 |month=May |pmid=10319082 |doi=10.4065/74.5.490 |url=http://linkinghub.elsevier.com/retrieve/pii/S0025-6196(11)65130-6 |accessdate=2012-02-13}}</ref> | ||
*'''Familial amyloidotic cardiomyopathy''' is defined as presence of TTR mutation that primarily affects the myocardium and without significant neuropathy.<ref name="pmid10774481">{{cite journal |author=Buxbaum JN, Tagoe CE |title=The genetics of the amyloidoses |journal=[[Annual Review of Medicine]] |volume=51 |issue= |pages=543–69 |year=2000 |pmid=10774481 |doi=10.1146/annurev.med.51.1.543 |url=http://arjournals.annualreviews.org/doi/full/10.1146/annurev.med.51.1.543?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed |accessdate=2012-02-13}}</ref> | |||
==Histopathological Findings== | ==Histopathological Findings== |
Revision as of 15:53, 13 February 2012
Cardiac amyloidosis Microchapters |
Diagnosis |
---|
Treatment |
Case Studies |
Cardiac amyloidosis pathophysiology On the Web |
American Roentgen Ray Society Images of Cardiac amyloidosis pathophysiology |
Risk calculators and risk factors for Cardiac amyloidosis pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor: Cafer Zorkun, M.D., Ph.D. [2]; Lakshmi Gopalakrishnan, M.B.B.S. [3]
Pathophysiology
- Amyloidosis is characterized by the deposition and extracellular accumulation of fibrillary proteins, leading to the loss of normal tissue architecture.[1]
- Most frequent types are:
- Acquired monoclonal immunoglobulin light-chain amyloidosis (AL)
- the immunoglobulin lights chains of fibrillary deposits are produced by the clonal plasma cells in the bone marrow.
- Hereditary transthyretin (TTR)-related form (ATTRm)
- caused by greater than 100 mutations of TTR
- the protein is synthesized by the liver
- Non-mutant TTR-related amyloidosis (ATTRwt)
- also know as systemic senile amyloidosis
- elderly male prepondrance
- Familial amyloidotic cardiomyopathy is defined as presence of TTR mutation that primarily affects the myocardium and without significant neuropathy.[4]
Histopathological Findings
-
Amyloidosis Lesion In Left Atrium: Gross natural color view of a diagnostic lesion
-
Amyloidosis Lesion In Left Atrium: Gross natural color close-up
-
Heart: Perivascular amyloid, amyloidosis, congo red showing birefringence
-
Heart: Perivascular amyloid, amyloidosis (Hematoxylin and eosin staining)
References
- ↑ Merlini G, Bellotti V (2003). "Molecular mechanisms of amyloidosis". The New England Journal of Medicine. 349 (6): 583–96. doi:10.1056/NEJMra023144. PMID 12904524. Retrieved 2012-02-13. Unknown parameter
|month=
ignored (help) - ↑ Falk RH, Comenzo RL, Skinner M (1997). "The systemic amyloidoses". The New England Journal of Medicine. 337 (13): 898–909. doi:10.1056/NEJM199709253371306. PMID 9302305. Retrieved 2012-02-13. Unknown parameter
|month=
ignored (help) - ↑ Gertz MA, Lacy MQ, Dispenzieri A (1999). "Amyloidosis: recognition, confirmation, prognosis, and therapy". Mayo Clinic Proceedings. Mayo Clinic. 74 (5): 490–4. doi:10.4065/74.5.490. PMID 10319082. Retrieved 2012-02-13. Unknown parameter
|month=
ignored (help) - ↑ Buxbaum JN, Tagoe CE (2000). "The genetics of the amyloidoses". Annual Review of Medicine. 51: 543–69. doi:10.1146/annurev.med.51.1.543. PMID 10774481. Retrieved 2012-02-13.