OMIA 000642-9940 : Mesangiocapillary glomerulonephritis, type I in Ovis aries

Possibly relevant human trait(s) and/or gene(s) (MIM number): 305800

Mendelian trait/disorder: unknown

Considered a defect: yes

Cross-species summary: A chronic, slowly progressive inflammation of the capillary loops in the glomeruli of the kidney, in which the glomeruli are enlarged as a result of proliferation of mesangial cells and thickening of the capillary walls, which narrows the capillary lumina. The type I variant of this disorder is characterised by a double-contour appearance of the capillary walls. Affected animals die after a brief clinical episode which includes anorexia and signs of central nervous system distrubance. On post mortem, the kidneys are dramatically enlarged, with pale spotted cortices. Glomeruli show a thickening of the capillary walls, proliferation of mesangial cells and matrix, and formation of epithelial crescents. There may be focal haemorrhage and oedema evident on histopathological examination of the brain. The damage to the kidneys and brain are thought to be due to deposition of immune complexes (IgA, IgM, and IgG) and complement. [Researched and prepared by Philippa Beard]

Species-specific description: This disorder becomes clinically apparent when lambs are 1-3 months old. Affected lambs are born with a severe deficiency of the third component of complement (Gardiner, 1985), but the relevance of this is not known. (Not all lambs with this congenital complement deficiency develop the clinical disease.) Type I mesangiocapillary glomerulonephritis is strongly familial in Finnish Landrace purebred and crossbred flocks, but a single-locus pattern of inheritance has not yet been determined (Young et al., 1981; Angus, 1990). This ovine disorder appears to be a good model for the same disorder in humans (Angus, 1974; Frelier, 1990), for which X-linkage has been claimed, but not proven. [Researched and prepared by Philippa Beard]

References


Note: the references are listed in reverse chronological order (from the most recent year to the earliest year), and alphabetically by first author within a year.
1990 Angus, K.W. :
Nephropathy in Young Lambs Veterinary Record 126:525-528, 1990. Pubmed reference: 2195755.
Basrur, P.K., Yadav, B.R. :
Genetic Diseases of Sheep and Goats Veterinary Clinics of North America-Food Animal Practice 6:779-802, 1990. Pubmed reference: 2245374.
Frelier, P.F., Armstrong, D.L., Pritchard, J. :
Ovine mesangiocapillary glomerulonephritis type I and crescent formation Veterinary Pathology 27:26-34, 1990. Pubmed reference: 2309378.
1989 Henderson, J.M. :
Inherited renal disease in lambs Canadian Veterinary Journal 30:69 only, 1989.
1984 Frelier, P.F., Pritchard, J., Armstrong, D.L., Nagge, W.T., Lewis, R.M. :
Spontaneous mesangiocapillary glomerulonephritis in Finn cross lambs from Alberta Canadian Journal of Comparative Medicine 48:215-218, 1984. Pubmed reference: 6372972.
1983 Frelier, P.F., Pritchard, J. :
Mesangiocapillary glomerulonephritis in Finn cross-bred lambs Canadian Veterinary Journal 24:248 only, 1983.
1979 Angus, K.W., Gardiner, A.C. :
Mesangiocapillary glomerulonephritis in Dorset-Finnish landrace cross lambs Veterinary Record 105:471 only, 1979.
1977 Morgan, K.T., Gardiner, A.C., Angus, K.W. :
A choroid plexus lesion in lambs with mesangiocapillary glomerulonephritis Journal of Comparative Pathology 87:15-25, 1977. Pubmed reference: 838900.
1976 Gardiner, A.C. :
Mesangiocapillary glomerulonephritis in lambs. III. Quantitative and qualitative aspects of immunopathology Journal of Pathology 119:11-19, 1976. Pubmed reference: 932870. DOI: 10.1002/path.1711190104.
1974 Angus, K.W., Gardiner, A.C., Morgan, K.T., Gray, E.W., Thomson, D. :
Mesangiocapillary glomerulonephritis in lambs. II. Pathological findings and electron microscopy of the renal lesions Journal of Comparative Pathology 84:319-330, 1974. Pubmed reference: 4457563.
Angus, K.W., Sykes, A.R., Gardiner, A.C., Morgan, K.T., Thomson, D. :
Mesangiocapillary glomerulonephritis in lambs. I. Clinical and biochemical findings in a Finnish Landrace flock Journal of Comparative Pathology 84:309-317, 1974. Pubmed reference: 4457562.
1973 Thomson, D., Davison, A.M., Angus, K.W. :
Spontaneous glomerulonephritis in Finnish Landrace lambs Journal of Medical Microbiology 6:xvii only, 1973.

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  • Created by Frank Nicholas on 06 Sep 2005