Glycogen Storage Disease Type Ia in Two Littermate Maltese Puppies
作者： A. E. Brix , E. W. Howerth , A. McConkie-Rosell , D. Peterson , D. Egnor
DOI: 10.1177/030098589503200502
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摘要: Glycogen storage disease type Ia (GSD-Ia) (von Gierke's disease) was identified in two 47-day-old littermate Maltese puppies. The puppies were presented for necropsy with a history of failure to thrive, mental depression, and poor body condition. Gross findings included small size emaciation (212 246 g versus 595 normal littermate), severely enlarged pale livers (48 61 g), kidneys. Histologically, there marked diffuse vacuolation hepatocytes large amounts glycogen lipid. Renal tubular epithelium mildly moderately vacuolated. Soft tissue mineralization present renal tubules pulmonary alveolar septa. Biochemical analysis showed that levels glucose-6-phosphatase markedly reduced liver (0.3 0.4 μM/minute/g 4.7 ± 1.5 controls) kidney (0.45 4.1 content increased (9.4% 9.4...
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