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2.1
ABri Amyloidosis
Familial Dementia Type British

Presentation

[…] episodes (without hemorrhage) 1,11 cerebral amyloidoma: mass-like lesions that have a varied presentation depending on the location of the amyloidoma Cerebral amyloid angiopathy is characterized by the deposition of amyloid in the tunica media and/or [radiopaedia.org]

[…] disorder Synonym(s): Familial dementia, British type Prevalence: - Inheritance: Autosomal dominant Age of onset: Adult ICD-10: E85.4+ I68.0* OMIM: 176500 UMLS: C1867773 MeSH: C538208 GARD: 8344 MedDRA: - The documents contained in this web site are presented [orpha.net]

A simplified overview is presented below. [amyloid.nl]

[medical citation needed] Treatment[edit] Treatment depends on the type of amyloidosis that is present. [en.wikipedia.org]

FBD is caused by a stop to Arg mutation in the BRI2 gene, generating de novo created amyloid molecule ABri which accumulates in FBD brains but is not present in the normal population. [ncbi.nlm.nih.gov]

Workup

Knowing this background helps us to understand how we use these tools developed over decades and apply them today for the diagnostic workup of this rather unusual and mystifying disease. Advertisement 4. [intechopen.com]

Treatment

Before taking any medication, always check with a qualified professional for healthcare information, treatment advice. [rarediseases.oscar.ncsu.edu]

For normal-sequence amyloidogenic TTR, the treatment is supportive care. 23. [slideshare.net]

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

[medical citation needed] Treatment[edit] Treatment depends on the type of amyloidosis that is present. [en.wikipedia.org]

However, the diagnosis requires a sample of an affected organ. [4] [7] Treatment The most effective treatment is autologous bone marrow transplants with stem cell rescues. [ipfs.io]

Prognosis

The prognosis does not appear to be related to the degree of the proteinuria. Treatment by peritoneal dialysis or hemodialysis or kidney transplantation improves the prognosis considerably. Hypertension is rare, except in long-standing amyloidosis. [perthhaematology.com.au]

[edit] Prognosis varies with the type of amyloidosis and the affected organ system. [en.wikipedia.org]

PROGNOSIS • Prognosis with generalized amyloidosis is poor. • those with AL amyloidosis have a median survival rate of 2 years after diagnosis. 60. [de.slideshare.net]

Cardiac involvement decides the prognosis. [revespcardiol.org]

[…] weak to tolerate this approach. [8] [9] Other treatments can involve application of chemotherapy similar to that used in multiple myeloma. [9] A combination of bortezomib and dexamethasone has been proposed, [10] as has melphalan and dexamethasone. [8] Prognosis [ipfs.io]

Etiology

Amyloid and Related Disorders presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis. [link.springer.com]

[…] not appreciated MRI: curvilinear regions of signal drop-out localized to one or more folia best seen on T2* sequences (GRE, echo-planar, SWI) 28 ischemia ischemic leukoencephalopathy chronic lesions, indistinguishable from leukoaraiosis due to other etiologies [radiopaedia.org]

Noninvasive etiologic diagnosis of cardiac amyloidosis using 99m Tc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. J Am Coll Cardiol. 2005;46:1076-84. Medline 66. [revespcardiol.org]

Epidemiology

New chapters on glomerulonephritis associated with complement disorders, interventional treatments for hypertension, renal disease and cancer, and epidemiology and prognostic impact of acute kidney injury. [books.google.de]

"Systemic AA amyloidosis: epidemiology, diagnosis, and management". Clinical Epidemiology. 6 : 369–77. doi : 10.2147/CLEP.S39981. PMC 4218891. PMID 25378951. ^ a b "AA (Inflammatory) Amyloidosis". Medscape Reference. ^ "AA Amyloidosis". [en.wikipedia.org]

[…] bortezomib and dexamethasone has been proposed, [10] as has melphalan and dexamethasone. [8] Prognosis Median survival for patients diagnosed with AL amyloidosis was 13 months in the early 1990s, but had improved to c. 40 months a decade later. [11] Epidemiology [ipfs.io]

Epidemiologic characteristics of primary amyloidosis, that is, amyloidosis without overt immunoproliferative disease, and myeloma are not significantly different. [abdominalkey.com]

For example, apolipoprotein E is reportedly a common constituent of amyloid deposits, and epidemiologic studies have shown an increased risk of Alzheimer disease among white persons carrying the 4 allele of apolipoprotein E. [humpath.com]

Pathophysiology

[…] random distribution fat embolism syndrome 'starfield' pattern of distribution lesions also show restricted diffusion on DWI and are likely visible on other sequences radiation-induced vasculopathy microhemorrhages have a very similar appearance (similar pathophysiology [radiopaedia.org]

"Amyloidosis and silicone synovitis: Updated classification, updated pathophysiology, and synovial articular abnormalities". Radiologic clinics of North America. 34 (2): 375–94, xi. [en.wikipedia.org]

The disease has no effective therapy and the mechanisms responsible for pathophysiology are not well understood. Mutations linked to familial ALS have been helpful in exploring the processes that cause the disease. [bitanlab.neurology.ucla.edu]

Despite their biochemical and clinical differences, the various amyloidoses share common pathophysiologic features: a precursor substance in appropriate concentration; appropriate host genetic background; abnormalities in proteolysis of fibril precursors [perthhaematology.com.au]

Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry. 2012;83(2):124–37. CrossRef PubMed Google Scholar Schneider JA. [link.springer.com]

Prevention

• Defibrillators may be a life-saving option for fatal arrhythmias, but there are not great data indicating they prevent mortality. • Even so, pacemakers may still have a role in preventing symptomatic bradycardia or conduction blocks. [slideshare.net]

[…] medicine Intensive care medicine Medical genetics Narcology Neurology Clinical neurophysiology Occupational medicine Ophthalmology Oral medicine Pain management Palliative care Pediatrics Neonatology Phlebology Physical medicine and rehabilitation (PM&R) Preventive [en.wikipedia.org]

Several of these small-molecule inhibitors efficiently prevent TTR amyloid fibril formation in vitro, and are being tested for their in vivo activity. [nature.com]

Bri23 peptide prevents aggregation of APP amyloid-beta protein 42 into toxic oligomers. [uniprot.org]

The structures produced in these studies will guide in the development of inhibitors to prevent amyloid formation in Alzheimer's disease. [case.edu]

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