Articles: amyloidosis.
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Subtle cognitive decline represents a stage of cognitive deterioration in which pathological biomarkers may be present, including early cortical atrophy and amyloid deposition. Using individual items from the Montreal Cognitive Assessment and k-modes cluster analysis, we previously identified three clusters of individuals without overt cognitive impairment: (1) High Performing (no deficits in performance), (2) Memory Deficits (lower memory performance), and (3) Compound Deficits (lower memory and executive function performance). In this study, we sought to understand the relationships found in our clusters between cortical atrophy on MR and amyloid burden on PET. ⋯ The Compound Deficits cluster, which represents a group potentially at higher risk for decline, was observed to have significantly more cortical atrophy, particularly within the entorhinal cortex and hippocampus, associated with whole brain and frontal lobe amyloid burden. These findings point to a pattern of early pathological deterioration that may place these individuals at risk for future decline.
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Dtsch. Med. Wochenschr. · Nov 2022
[Cardiac amyloidosis - important differential diagnosis in heart failure].
The systemic disease amyloidosis is caused by deposits of misfolded proteins and can cause very different symptoms depending on the organ involvement. The two most common forms are AL amyloidosis and ATTR amyloidosis. The determination of light chains and bone scintigraphy have a high priority in the diagnostic algorithm. ⋯ The classic heart failure medication is often not well tolerated by patients with cardiac amyloidosis and must be adjusted in the presence of symptoms. This article shows which findings and symptom complexes should make you think of amyloidosis and how to arrive at the correct diagnosis in a targeted manner. Causal therapy options as well as special features of heart failure therapy in this patient collective are explained.
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Case Reports
Liver Biopsy-confirmed Primary Hepatic Amyloidosis with Only Jaundice as the Initial Symptom: An Autopsy Case Report.
Amyloidosis causes various symptoms in many organs of the body, but amyloidosis that presents with liver damage alone has never been reported. We treated an 83-year-old man with amyloidosis who presented with liver damage alone. ⋯ The administration of bortezomib and dexamethasone was not effective, so he rapidly died of liver failure. An aggressive liver biopsy should be considered when unexplained jaundice is observed.
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Objective High-dose melphalan and autologous stem cell transplantation (ASCT) therapy for AL amyloidosis are now associated with reduced mortality based on the application of strict criteria. However, there is no long-term evidence concerning the performance of induction therapy with newer agents, such as bortezomib or daratumumab. Concerns regarding long-term relapse despite treatment with ASCT exist, and missing the opportunity to perform ASCT might occur if induction proves to not be efficacious and cardiac amyloidosis progression deprives the patients of a chance to receive ASCT. ⋯ Although there was no significant difference in the survival rates between the two groups, the time to next treatment or death was significantly better in the VAD+ASCT group than in the the frontline ASCT group. Acute kidney injury was the most frequent reason for failure to receive two courses of VAD, and early mortality was mainly due to gastrointestinal complications. Conclusion Considering that only those who underwent 2 courses of VAD experienced a 10-year renal response, induction therapy was deemed to be directly related to the long-term control of AL amyloidosis.
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Internal medicine journal · Oct 2022
Efficacy of bortezomib, cyclophosphamide and dexamethasone in cardiac AL amyloidosis.
Cardiac light chain (AL) amyloidosis is a condition with a very poor prognosis. We report a retrospective analysis comparing the traditional melphalan and dexamethasone protocol with cyclophosphamide, bortezomib and dexamethasone in late-stage cardiac AL amyloidosis. The primary end points were overall survival and haematological response. Both regimens provided meaningful responses in this difficult to treat patient group.