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Transient erythropoietic protoporphyria associated with chronic hepatitis and cirrhosis in a cohort of German shepherd dogs
  1. E. J. B. Veldhuis Kroeze, DVM1,
  2. J. Zentek, DVM, PhD3,
  3. A. Edixhoven-Bosdijk4,
  4. J. Rothuizen, DVM, PhD, DipECVIM2 and
  5. T. S. G. A. M. van den Ingh, DVM, PhD, DipECVP1
  1. 1Pathology Division, Department of Pathobiology
  2. 2Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Yalelaan 8, 3584 CM Utrecht, The Netherlands
  3. 3Institute of Animal Nutrition, School of Veterinary Medicine Hannover, Bischofsholer Damm 15, D-30173 Hannover, Germany
  4. 4Department of Internal Medicine, Erasmus MC, University Medical Centre Rotterdam, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands

Abstract

Over the course of one year, slight jaundice and ascites suggestive of chronic liver disease occurred in 17 German shepherd dogs from one breeding colony. Blood analyses, performed twice with a six-month interval, revealed elevated serum activities of liver enzymes in 13 dogs. In addition, four young adult German shepherd dogs that showed severe ascites, slight jaundice and increased serum liver enzyme activities were referred for further evaluation. Because of their poor prognosis these four dogs were euthanased. There were no signs of photosensitivity. Postmortem examinations revealed macronodular darkened livers, which were characterised histopathologically by cirrhosis associated with aggregates of brown pigments showing a striking orange birefringence in polarised light. Ultrastructurally, the crystalline pigments were typical of protoporphyrins. High-performance liquid chromatographic analysis of liver samples revealed very high levels of protoporphyrins (mean 9550 nmol/g wet liver, reference value 0·41 nmol/g wet liver) and low activities of ferrochelatase (mean 0·274 mmol/mg protein/hour, reference value 0·684 nmol/mg protein/hour). Twenty-six months after the onset of the hepatopathies, the clinical condition of the 13 surviving dogs had improved and their serum liver enzyme activities were normal. The clinical histories and pedigree analyses were not in concordance with an inherited form of protoporphyria. There was no known history of exposure to toxic substances or drugs. The findings are in accordance with a transient erythropoietic protoporphyria associated with hepatic complications, presumably caused by exposure to a porphyrinogenic, ferrochelatase-inhibitory substance of unknown origin.

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      Footnotes

      • Dr Zentek’s present address is University of Veterinary Medicine Vienna, Veterinärplatz 1, A-1210 Vienna, Austria

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