December 2017

SIGNALMENT (JPC Accession # 1483487):  Southdown ewe

HISTORY:  Tissue from a Southdown ewe that had been kept out of the sunlight for three years because of a photosensitivity that developed shortly after weaning.

HISTOPATHOLOGIC DESCRIPTION:  Kidney:  Diffusely, there is marked cortical and medullary interstitial fibrosis which widely separates, surrounds and replaces approximately 50% of the tubules.  Multifocally, remaining tubules are either shrunken and atrophic or dilated up to 300 um in diameter (tubular ectasia), lined by attenuated epithelium, and contain a variable amount of brightly eosinophilic, homogenous protein (tubular proteinosis) and occasionally, sloughed epithelial cells.  Diffusely, tubular epithelial cells exhibit one or more of the following changes:  Hypertrophy with indistinct cell borders and microvacuolated cytoplasm (degeneration); shrunken with bright eosinophilic cytoplasm and a pyknotic to absent nucleus (necrosis); or a high nuclear to cytoplasmic ratio with amphophilic cytoplasm and a vesiculate nucleus with rare mitotic figures (regeneration).  Multifocally, tubular epithelial cells occasionally contain granular to globular yellow-brown pigment (bilirubin).  Multifocally, there are moderate numbers of lymphocytes, plasma cells, occasional macrophages, and reactive fibroblasts which infiltrate the interstitium.  Multifocally, glomeruli are surrounded by dense fibrous connective tissue (periglomerular fibrosis) and Bowman’s space is often lined by cuboidal, hyperplastic and hypertrophied epithelial cells, which rarely are adhered to Bowman’s capsule (synechia).  Multifocally in less than 25% of glomeruli  there is either global or segmental increase in mesangial cell density, mesangial cells are mildy hypertrophied, and there is moderate thickening of the basement membrane of Bowman’s capsule.). 

MORPHOLOGIC DIAGNOSIS:  Kidney:  Fibrosis, interstitial, diffuse, marked, with tubular ectasia, degeneration, necrosis, loss, regeneration and pigmentation, with interstitial lymphoplasmacytic nephritis, Southdown, ovine.

ETIOLOGIC DIAGNOSIS:  Inherited hyperbilirubinemic nephropathy

CONDITION:  Gilbert (pronounced “zheel-BAIR”) syndrome; congenital hyperbilirubinemia









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