JPC SYSTEMIC PATHOLOGY
URINARY SYSTEM

January 2018
U-M21 (NP)

Signalment (JPC # 1945143):  Rat

HISTORY: Tissue from a rat fed a leguminous plant of the genus Swainsona.

MICROSCOPIC DESCRIPTION (U-M21a):  Kidney:  Diffusely within the cortex proximal tubular epithelial cells are swollen and contain abundant clear cytoplasmic vacuoles that are 2-50 um in diameter, often peripheralize the nucleus, and obscure or occlude tubular lumina.  Tubules occasionally contain intraluminal eosinophilic amorphous material (protein cast). Within the medulla, epithelial cells of tubules and collecting ducts are mildly to moderately vacuolated.  Occasional cells in glomerular tufts are distended by cytoplasmic vacuolation.  Multifocally, there are low numbers of lymphocytes and plasma cells in the interstitium.

MORPHOLOGIC DIAGNOSIS (U-M21a):  Kidney, tubular epithelium:  Vacuolation, cytoplasmic, diffuse, severe, rat, rodent.

CAUSE:  Swainsonine toxicity (toxic inhibition of alpha-mannosidase)

CONDITION:  Acquired alpha-mannosidosis

Signalment (JPC # 1640-4):  Calf

HISTORY (U-M21b):  Tissue from a neonatal Salers calf.

HISTOPATHOLOGICAL DESCRIPTION (U-M21b):  Kidney:  Diffusely, tubular epithelial cells are swollen, severely vacuolated, and often obscure or occlude tubule lumina.  Vacuoles are 2-40 um in diameter and peripheralize nuclei.  Multifocally there are occasional lymphocytes and plasma cells in the interstitium.

Spinal cord:  Multifocally, neurons have numerous intracytoplasmic vacuoles that are 1-10 um in diameter and often obscure or displace Nissl substance.

MORPHOLOGIC DIAGNOSIS (U-M21b):  Kidney, tubular epithelium:  Vacuolation, cytoplasmic, diffuse, severe, Salers, bovine.

Spinal cord, neurons:  Vacuolation, cytoplasmic, multifocal, moderate.

CONDITION:  Autosomal recessive hereditary beta-mannosidosis

GENERAL DISCUSSION:

 PATHOGENESIS:

Congenital alpha-mannosidosis:

Acquired alpha-mannosidosis:

 TYPICAL CLINICAL FINDINGS:

TYPICAL GROSS FINDINGS:

TYPICAL LIGHT MICROSCOPIC FINDINGS:

ULTRASTRUCTURAL FINDINGS: 

ADDITIONAL DIAGNOSTIC TESTS: 

Comparative List of lysosomal storage Diseases

 

Disease Storage Product Deficient enzyme Species Breed

GM1 gangliosidosis

GM1 ganglioside

b-galactosidase

Bovine

Canine

 

 

Feline

Ovine

Holstein-Friesian

Beagle, English Springer spaniel, Portuguese water dog

Siamese, DSH

Suffolk, Coopworth-Romney

GM2 gangliosidosis

GM2 ganglioside

b-hexosaminidase

Canine

 

 

Feline

Porcine

German shorthair pointer, Japanese

Spaniel

DSH, Korat

Yorkshire

Globoid-cell

Leukodystrophy

(krabbe’s-like disease)

Galactosylceramide

(galactocerebroside)

Galactosylsphingosine

(psychosine)

Galactoslceramidase

(galactocerebroside

b-galactosidase)

Canine

 

 

 

 

Feline

Ovine

 

West Highland terrier, Cairn terrier, Miniature poodle, blue tick hound, beagle, Pomeranian

DSH

Polled Dorset

a-mannosidosis

Mannose-containing

oligosaccharide

a-mannosidase

Bovine

 

Feline

Angus, Murray Gray, Galloway

Persian, DSH

b-mannosidosis

Mannose-containing

oligosaccharide

b-mannosidase

Caprine

Bovine

Nubian

Salers

Mucopolysaccharidosis

Different glycosaminoglycans

Several different enzyme deficiencies

Canine

 

 

Feline

Caprine

Plott hound (type I, Hurler’s disease) miniature pinscher (type VI, Maroteaux-Lamy disease)

DSH(type I, Type VII sly disease)

Nubian (type III, sanfilippo’s disease)

Ceroid-lipofuscinosis

Subunit c of mitochondrial ATPase

Prelysosomal defect?

Canine

 

Ovine

Bovine

English setter, border collie, Tibetan terrier

South Hampshire

Devon

 

Sphingolipid

Activating proteins A & D

 

Palmitoyl protein thioesterase

Canine

Ovine

Miniature schnauzer 

Swedish Landrace                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                  

 

REFERENCES:

  1. Armien AG, Tokarnia CH, Vargas Peixoto P, Frese K. Spontaneous and Experimental Glycoprotein Storage Disease of Goats Induced by Ipomoea carnea subsp fistulosa (Convolvulaceae). Vet Pathol 2007;44:170-184.
  2. Cantile C, Youssef S. Nervous system. In: Maxie MG, ed. Jubb, Kennedy, and Palmer’s Pathology of Domestic Animals. 6th ed., vol. 1. St. Louis, MO: Elsevier Limited; 2016:288, 292.
  3. Cianciolo RE, Mohr FC. Urinary system. In: Maxie MG, ed. Jubb, Kennedy, and Palmer's Pathology of Domestic Animals. 6th ed. Vol 2. Philadelphia, PA: Elsevier Saunders;2016:429.
  4. Haskins M, Giger U: Lysosomal storage diseases. In: Kaneko JJ, Harvey JW, Bruss ML, eds. Clinical Biochemistry of Domestic Animals., 5th ed. San Diego, CA: Academic Press; 1997: 741-760.
  5. Jolly RD, Walkley SU. Lysosomal storage diseases in animals: An essay in comparative pathology. Vet Pathol 1997;34:527-548.
  6. Laurent S, Sabot A, Colle M, Nicolier A. Lysosomal storage disease in two presumed-related springboks (Antidorcas marsupialis). Journal of Zoo and Wildlife Medicine. 2010;41(1):104-110.
  7. Mendonca FS, Albuquerque RF, Evencio-Neto J, et al. Alpha-mannosidosis in goats caused by the swainsonine-containing plant Ipomoea verbascoidea. JVDI. 2012;24(1):90-95.
  8. Skelly BJ, Franklin RJM. Recognition and diagnosis of lysosomal storage diseases in the cat and dog. J Vet Intern Med 2002;16:133-141.
  9. Stegelmeier BL, Molyneux RJ, Elbein AD, James LF. The lesions of locoweed (Astragalus mollissimus), swainsonine, and castanospermine in rats. Vet Pathol 1995;32:289-98.
  10. Warren CD, Alroy J. Morphological, biochemical and molecular biology approaches for the diagnosis of lysosomal storage diseases. J Vet Diagn Invest 2000;12:482-496.


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