Renal infarction is associated with acute kidney injury in patients with cardiac amyloidosis
(Elsevier) Systemic amyloidosis is a major cause of renal injury, mostly due to direct kidney damage caused by deposits of abnormal protein called amyloid, in the kidney parenchyma. In patients with cardiac amyloidosis, renal infarction is associated with acute kidney injury according to a new study in Mayo Clinic Proceedings, published by Elsevier. Investigators recommend that a diagnosis of renal infarction should be systematically considered in patients with unexplained acute kidney injury in the context of cardiac amyloidosis.
Abstract Misfolding and aggregation of immunoglobulin light chains (LCs) leads to the degeneration of post-mitotic tissue in the disease immunoglobulin LC amyloidosis (AL). We previously reported the discovery of small molecule kinetic stabilizers of the native dimeric structure of full-length LCs, which slow or stop the LC aggregation cascade at the outset. A predominant structural category of kinetic stabilizers emerging from the high-throughput screen are coumarins substituted at the 7-position, which bind at the interface between the two variable domains of the light chain dimer. Here, we report the binding mo...
Condition: Cardiac Amyloidosis Intervention: Procedure: diagnostic algorithm Sponsors: University Hospital Center of Martinique; Pfizer Not yet recruiting
Editor's Summary by Howard Bauchner, MD, Editor in Chief of JAMA, the Journal of the American Medical Association, for the July 7, 2020 issue
This systematic review summarizes the characteristic presentations, diagnosis, and management of immunoglobulin light chain (AL) and amyloid transthyretin (ATTR) amyloidosis.
Publication date: Available online 4 July 2020Source: Revista Española de Cardiología (English Edition)Author(s): María Bastos Fernández, Diego López Otero, Javier López Pais, Virginia Pubul Núñez, Carmen Neiro Rey, José R. González-Juanatey
Authors: Romero-Caballero MD, Pozo-Lorenzo FJ, Berrio-Campos R, Quilez-Franco P, Pastor-Quirante FA Abstract Primary conjunctival amyloidosis is a rare disease of unknown origin, secondary to the deposit of amyloid material within the conjunctiva itself, producing a tumour. In the case presented, the concurrence of allergic conjunctivitis and chronic eye scratching could be triggers of the excessive production of immunoglobulins. The deposit of insoluble immunoglobulin light chains located within the conjunctiva itself causes this conjunctival mass. PMID: 32616371 [PubMed - as supplied by publisher]
Authors: Yamamoto A, Fujii N, Obika M, Yamashita T, Otsuka F Abstract The prognosis of systemic amyloid light chain (AL) amyloidosis is generally poor, hence requiring chemotherapy or hematopoietic stem cell transplantation, while the prognosis of localized AL amyloidosis without an abnormal monoclonal immunoglobulin light chain is good. Localized AL amyloidosis has been previously reported to be observed in pulmonary, urinary tract, gastrointestinal, oropharyngeal, and laryngeal sites. However, only a few cases of localized lymph node AL amyloidosis have so far been reported. We herein present a case of localized ...
Early diagnosis of wild-type transthyretin cardiac amyloidosis remains clinically challenging but is now of highest importance given the recently-reported favorable prognostic impact of tafamidis . Ladefoged and colleagues demonstrated that the diagnostic delay in cardiac amyloidosis was substantial, and prolonged diagnostic delay was associated with a higher clinical disease burden .
Condition: Amyloidosis Intervention: Procedure: Shear wave elastography Sponsors: Fábio Fernandes; Pfizer Recruiting