PMC6477140_01
Family pedigree. I.-IV. - generations,  - affected male,  - affected female,  proband,  - deceased, probably affected (not genetically confirmed).
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F1
III consists of 8 individuals (21-33 years) with 5 of them affected. (Figure 1, Table 1). In 4 cases MWS was confirmed, one additional symptomatic family member has not yet become interested in specialized care (III/3).
PMC6477140_02
Family pedigree. I.-IV. - generations,  - affected male,  - affected female,  proband,  - deceased, probably affected (not genetically confirmed).
fimmu-10-00802-g0001.jpg
PMC6477140_02_fimmu-10-00802-g0001.jpg
F1
III consists of 8 individuals (21-33 years) with 5 of them affected. (Figure 1, Table 1). In 4 cases MWS was confirmed, one additional symptomatic family member has not yet become interested in specialized care (III/3).
PMC6477140_03
Patient II/2 renal biopsy. Congo red staining showing amyloid deposits in the glomeruli, arterioles and also in the interstitium around the peritubular capillaries. (Courtesy of Assoc. Prof. E. Honsova Ph.D, Clinical and Transplant Pathology Centre, Institute for Clinical and Experimental medicine, Prague, Czech Republic).
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F2
It confirmed presence of widespread AA amyloidosis (Figure 2).
Skin rash in patient II/2.
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F3
Being the oldest living affected family member he explained how the symptoms of fevers, rash (Figure 3), eye and joint problems have been so common in the family that people have become used to them.
PMC6477140_04