Cardiac amyloidosis pathophysiology: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
|||
Line 1: | Line 1: | ||
__NOTOC__ | |||
{{Cardiac amyloidosis}} | {{Cardiac amyloidosis}} | ||
{{CMG}}; '''Associate Editor:''' {{ | {{CMG}}; '''Associate Editor:''' {{LG}}; {{AN}}; {{RT}} | ||
==Pathophysiology== | ==Pathophysiology== |
Revision as of 17:49, 3 May 2013
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: Lakshmi Gopalakrishnan, M.B.B.S. [2]; Aarti Narayan, M.B.B.S [3]; Raviteja Guddeti, M.B.B.S. [4]
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
- Hereditary transthyretin (TTR)-related form
- Non-mutant TTR-related amyloidosis
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.
- caused by greater than 100 mutations of TTR
- the protein is synthesized by the liver
Familial amyloidotic cardiomyopathy
- defined as presence of TTR mutation that primarily affects the myocardium and without significant neuropathy.[6]
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) - ↑ Hodkinson HM, Pomerance A (1977). "The clinical significance of senile cardiac amyloidosis: a prospective clinico-pathological study". The Quarterly Journal of Medicine. 46 (183): 381–7. PMID 918253. Retrieved 2012-02-13. Unknown parameter
|month=
ignored (help) - ↑ Lie JT, Hammond PI (1988). "Pathology of the senescent heart: anatomic observations on 237 autopsy studies of patients 90 to 105 years old". Mayo Clinic Proceedings. Mayo Clinic. 63 (6): 552–64. PMID 3374172. Unknown parameter
|month=
ignored (help);|access-date=
requires|url=
(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.