Amyloid and Related Disorders: Surgical Pathology and - download pdf or read online

By Maria M. Picken MD PhD FASN, Ahmet Dogan, Guillermo A. Herrera

ISBN-10: 3319192930

ISBN-13: 9783319192932

ISBN-10: 3319192949

ISBN-13: 9783319192949

The moment variation of this article provides an outline of the newest advancements during this zone together with medical presentation, etiology, pathogenesis, and differential prognosis. the explanation for varied treatments, together with transplantation, is mentioned and tissue prognosis (its pitfalls and techniques for warding off them) and laboratory help are integrated. The involvement of all significant organ platforms together with renal/genitourinary, cardiac, gastrointestinal, pulmonary, peripheral nerve/central worried approach, delicate tissue, epidermis, lymph node/spleen and bone marrow pathology is usually covered.

Amyloid and similar problems, moment Edition may be precious to really expert and normal pathologists in addition to cytopathologists. different doctors can also make the most of this concise replace at the systemic amyloidoses.

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Additional info for Amyloid and Related Disorders: Surgical Pathology and Clinical Correlations

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Electron microscopic observations on a fibrous component in amyloid of diverse origins. Nature. 1959;183:1202–3. 24 46. Eanes ED, Glenner GG. X-ray diffraction studies on amyloid filaments. J Histochem Cytochem. 1968; 16:673–7. 47. Termine JD, Eanes ED, Ein D, Glenner GG. Infrared spectroscopy of human amyloid fibrils and immunoglobulin proteins. Biopolymers. 1972;11:1103–13. 48. Petkova AT, et al. A structural model for Alzheimer’s beta-amyloid fibrils based on experimental constraints from solid state NMR.

When it does occur it leads to hyposplenism [207] and, anecdotally, to splenic rupture [208]. The involvement of the peripheral/autonomous nervous system is seen in about 14 % of patients [174] in the form of a predominantly sensitive, axonal, symmetrical and progressive neuropathy [209]. When peripheral neuropathy is the dominant syndrome, a differential diagnosis between AL and hereditary amyloidosis becomes mandatory. The presence of amyloid autonomic neuropathy manifests as postural hypotension, erectile dysfunction and gastrointestinal symptoms (constipation, diarrhoea or an alternation thereof).

Intriguingly, the propensity of wild-type transthyretin to form amyloid is also enhanced by exposure to a mutant disease-related form of the protein. In individuals heterozygous for one of the pathogenic mutation, both the mutated and the wild-type transthyretin are found in deposits [27–30], and this phenomenon explains why cardiac amyloid deposits can further progress in patients for whom liver transplantation has minimized the production of the mutant protein [27–29]. 11 The inherent amyloidogenicity of a specific protein, per se, is not sufficient to explain the likelihood that amyloid deposition finally occurs in vivo.

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Amyloid and Related Disorders: Surgical Pathology and Clinical Correlations by Maria M. Picken MD PhD FASN, Ahmet Dogan, Guillermo A. Herrera


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