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Systemic light-chain amyloidosis presenting with rapid progressive heart failure.
Ther Adv Cardiovasc Dis. 2013 Aug; 7(4):224-7.TA

Abstract

Systemic amyloid light-chain (AL) amyloidosis is caused by deposition of immunoglobulin light-chain proteins. AL amyloidosis is a rapidly progressive disease that affects multiple organs. Cardiac involvement is frequent (50%) and the median survival is 5 months following diagnosis. Although transthoracic echocardiography can show characteristics findings, further diagnostic modalities are required for diagnosis. The gold standard of the diagnosis is presence of the amyloid in a tissue biopsy specimen. We present here a case of AL amyloidosis with presenting rapid progressive heart failure. Despite improvements in diagnostic and therapeutic modalities, diagnosis is difficult and prognosis is still poor in patients with cardiac involvement as in the present case.

Authors+Show Affiliations

Marmara University Faculty of Medicine, Department of Cardiology, Kadıköy, İstanbul, Turkey. drsunbul@yahoo.com.trNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

23868674

Citation

Sunbul, Murat, et al. "Systemic Light-chain Amyloidosis Presenting With Rapid Progressive Heart Failure." Therapeutic Advances in Cardiovascular Disease, vol. 7, no. 4, 2013, pp. 224-7.
Sunbul M, Durmus E, Kivrak T, et al. Systemic light-chain amyloidosis presenting with rapid progressive heart failure. Ther Adv Cardiovasc Dis. 2013;7(4):224-7.
Sunbul, M., Durmus, E., Kivrak, T., Besiroglu, F., Gerin, F., Sari, I., & Mutlu, B. (2013). Systemic light-chain amyloidosis presenting with rapid progressive heart failure. Therapeutic Advances in Cardiovascular Disease, 7(4), 224-7. https://doi.org/10.1177/1753944713493468
Sunbul M, et al. Systemic Light-chain Amyloidosis Presenting With Rapid Progressive Heart Failure. Ther Adv Cardiovasc Dis. 2013;7(4):224-7. PubMed PMID: 23868674.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Systemic light-chain amyloidosis presenting with rapid progressive heart failure. AU - Sunbul,Murat, AU - Durmus,Erdal, AU - Kivrak,Tarik, AU - Besiroglu,Fatih, AU - Gerin,Fatma, AU - Sari,Ibrahim, AU - Mutlu,Bulent, PY - 2013/7/23/entrez PY - 2013/7/23/pubmed PY - 2014/2/11/medline KW - heart failure KW - restrictive cardiomyopathy KW - systemic amyloidosis SP - 224 EP - 7 JF - Therapeutic advances in cardiovascular disease JO - Ther Adv Cardiovasc Dis VL - 7 IS - 4 N2 - Systemic amyloid light-chain (AL) amyloidosis is caused by deposition of immunoglobulin light-chain proteins. AL amyloidosis is a rapidly progressive disease that affects multiple organs. Cardiac involvement is frequent (50%) and the median survival is 5 months following diagnosis. Although transthoracic echocardiography can show characteristics findings, further diagnostic modalities are required for diagnosis. The gold standard of the diagnosis is presence of the amyloid in a tissue biopsy specimen. We present here a case of AL amyloidosis with presenting rapid progressive heart failure. Despite improvements in diagnostic and therapeutic modalities, diagnosis is difficult and prognosis is still poor in patients with cardiac involvement as in the present case. SN - 1753-9455 UR - https://www.unboundmedicine.com/medline/citation/23868674/Systemic_light_chain_amyloidosis_presenting_with_rapid_progressive_heart_failure_ L2 - http://journals.sagepub.com/doi/full/10.1177/1753944713493468?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -