Familial renal amyloidosis

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Familial renal amyloidosis
Classification and external resources
ICD-10 E85.0
ICD-9 277.3
OMIM 105200
DiseasesDB 33335
eMedicine med/3379

Familial renal amyloidosis (or familial visceral amyloidosis, or hereditary amyloid nephropathy) is a form of amyloidosis primarily presenting in the kidney.[1]

It is associated with fibrinogen alpha chain,[2] apolipoprotein A1,[3] and lysozyme.[4][5]

It is also known as "Ostertag" type, after B. Ostertag, who characterized it in 1932 and 1950.[6][7]

References

  1. ↑ "Amyloid". 
  2. ↑ Uemichi T, Liepnieks JJ, Gertz MA, Benson MD (September 1998). "Fibrinogen A alpha chain Leu 554: an African-American kindred with late onset renal amyloidosis". Amyloid 5 (3): 188–92. doi:10.3109/13506129809003844. PMID 9818055. 
  3. ↑ Soutar AK, Hawkins PN, Vigushin DM et al. (August 1992). "Apolipoprotein AI mutation Arg-60 causes autosomal dominant amyloidosis". Proc. Natl. Acad. Sci. U.S.A. 89 (16): 7389–93. doi:10.1073/pnas.89.16.7389. PMC 49715. PMID 1502149. 
  4. ↑ Granel B, Serratrice J, Disdier P et al. (March 2005). "Underdiagnosed amyloidosis: amyloidosis of lysozyme variant". Am. J. Med. 118 (3): 321–2. doi:10.1016/j.amjmed.2004.10.022. PMID 15745733. 
  5. ↑ Granel B, Valleix S, Serratrice J et al. (January 2006). "Lysozyme amyloidosis: report of 4 cases and a review of the literature". Medicine (Baltimore) 85 (1): 66–73. doi:10.1097/01.md.0000200467.51816.6d. PMID 16523055. 
  6. ↑ Ostertag B. (1932). "Demonstration einer eigenartigen familiaren paraamyloidose". Zentralbl Aug Pathol 56: 253–4. 
  7. ↑ Ostertag, B. (1950). "Familiaere Amyloid-erkrankung". Z. Menschl. Vererb. Konstitutionsl. 30: 105–115. 
Common amyloid forming proteins
Systemic amyloidosis
Organ-limited amyloidosis
Abdominal
Nephropathy/
(nephritis+
nephrosis)
Glomerulopathy/
glomerulitis/
(glomerulonephritis+
glomerulonephrosis)
Primarily
nephrotic
Non-proliferative
Proliferative
By condition
Primarily
nephritic,
RPG
Type I RPG/Type II hypersensitivity
Type II RPG/Type III hypersensitivity
Type III RPG/Pauci-immune
Tubulopathy/
tubulitis
Proximal
Thick ascending
Distal convoluted
Collecting duct
Renal papilla
Major calyx/pelvis
Any/all
Interstitium
Any/all
General syndromes
Vascular
Other
Ureter
Pelvic
Bladder
Urethra
Any/all

M: URI

anat/phys/devp/cell

noco/acba/cong/tumr, sysi/epon, urte

proc/itvp, drug (G4B), blte, urte

Inborn error of lipid metabolism: dyslipidemia (E78, 272.0–272.6)
Hyperlipidemia
Hypolipoproteinemia
Lipodystrophy
Other

M: MET

mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m

k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon

m (A16/C10), i (k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)


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