Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis
The differentiated assessment of functional parameters besides morphological changes is essential for the evaluation of prognosis in systemic immunoglobulin light chain (AL) amyloidosis.
Publication date: Available online 8 April 2020Source: Respiratory Medicine Case ReportsAuthor(s): Thiyaphat Laohawetwanit, Kei Tanaka, Yoshiaki Zaizen, Kazuhiro Tabata, Kouji Ando, Hiroshi Ishimoto, Hiroshi Mukae, Yasushi Miyazaki, Andrey Bychkov, Junya Fukuoka
No abstract available
Light chain deposition disease (LCDD) is commonly associated with monoclonal gammopathy of renal significance (MGRS). Venetoclax is a Bcl-2 inhibitor approved for the treatment of chronic lymphocytic leukemia with activity against multiple myeloma and AL amyloidosis clones that harbor the t(11;14) abnormality. Here, we report the first use of venetoclax in a patient with LCDD secondary to MGRS after kidney transplantation. A 43 year-old male presented with 17.9 g/d of proteinuria and creatinine of 3.7 mg/dl.
No concerted investigation has been conducted to explore overlapping and distinct pathobiological mechanisms between repetitive mild traumatic brain injury (r-mTBI) and tau/amyloid proteinopathies considering the long history of association between TBI...
AbstractCerebral amyloid angiopathy-related inflammation is a syndrome of reversible encephalopathy with cerebral amyloid angiopathy, however the pathology is not well understood. We clear a part of the pathology through the first case of an 80-year-old man with cerebral amyloid angiopathy-related inflammation induced by relapsing polychondritis (RP) analysis. An 80-year-old man was diagnosed with RP by auricular cartilage biopsy. Almost no abnormality including intracranial microbleeding was detected by cranial magnetic resonance image (MRI) at diagnosis. However, he developed a headache and hallucination after five month...
SO Abstract Autoinflammatory syndromes (AIS) are characterized by uniform attacks often with febrile episodes, exanthema, abdominal pain, muscle and joint pain. Patients show markedly elevated levels of the inflammatory serum parameters C‑reactive protein (CRP) and systemic amyloid A (SAA) during an attack. The origin of the family of the patient and the duration of the attacks are helpful to find the appropriate diagnosis. Molecular genetic tests are used to confirm the clinical diagnosis of an AIS. Colchicine can prevent attacks of familial Mediterranean fever but not the other forms of AIS. In refractory cas...
We present the case of a young female patient diagnosed through kidney biopsy with amyloid A (AA) amyloidosis after 30 years of evolution of FMF and we review the present knowledge regarding the pathogenesis and management of this rare genetic disease. PMID: 32239108 [PubMed - in process]
Publication date: April 2020Source: Journal of Comparative Pathology, Volume 176Author(s): C. Bianco, P.J. Sánchez-Cordón, R. Verin, A. Godinho, U. Weyer, S. Lesellier, J. Spiropoulos, T. Floyd, D. Everest, A. Núñez
Elucidation of the biologic mechanisms underlying the pathogenesis of transthyretin (TTR) amyloidosis has led to the approval of therapies to treat both inherited (hATTR) and age-related or wild-type (wtATTR) forms of transthyretin amyloidosis manifesting as neuropathy and/or cardiomyopathy1-3. These developments have been hailed as a model of scientific and medical progress emanating from precision medicine, which can arrest or slow progression of fatal disorders and provide a potential pathway for progress in other complex clinical syndromes such as heart failure with preserved ejection fraction by endo-phenotyping.
(Shinshu University) Small gauge vitrectomy for vitreous amyloidosis and subsequent management of secondary glaucoma in patients with hereditary transthyretin amyloidosis.