Four performed scintigraphy with 99mTc-DPD which was negative.

Of these, 3 had probable fibrinogen A alpha-chain (AFib) amyloidosis, after a heterozygous mutation FGA p.Glu545Val was detected, 1, who did not have IHC performed, was assumed as having AA amyloidosis due to a history of serious recurrent infections and 1 is still under study. Among them, 2 had AL amyloidosis with light chain restriction by IF, 1 had AA amyloidosis with intense IHC stain and 5 patients had non-AL and non-AA forms of amyloidosis. Mean creatinine at presentation was 3.2☒.3mg/dL. Mean age was 63.8☙.2 years of age, 5 were female, 6 were referred for nephrotic syndrome and 2 for what seemed like acute kidney injury. Among these, 8 (4.1%) revealed amyloidosis. We aimed at describing cases of kidney amyloidosis identified by biopsy during a 4-year interval and discuss possible implication for future diagnosis.įrom January 2016 until December 2019, 194 kidney biopsies were performed at the Hospital of Braga in the Portuguese province of Minho. Since the cause of amyloidosis vary among regions, analyzing local patterns can help establish a diagnostic procedure. Other options include electron microscopy with immunogold staining and complementary exams such as scintigraphy with 99mTc-DPD to detect transthyretin-related amyloidosis. The gold standard has become proteomics but laser microdissection and mass spectroscopy are not routinely available. Immunohistochemistry (IHC) and immunofluorescence (IF) studies use a limited number of antibodies to detect specific epitopes and may be difficult to interpret. After identifying amyloidosis by its suggestive pale pink amorphous appearance in optic microscopy (OM) and Congo red positivity, a correct diagnosis of the amyloidogenic precursor protein is determinant to establish prognosis and treatment. Renal amyloidosis include amyloid A (AA) and light chain (AL) as well as amyloidogenic leukocyte chemotactic factor 2 (ALECT2) and numerous hereditary forms.

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