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Use of deferiprone for the treatment of hepatic iron storage disease in three hornbills

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Abstract
Case Description-3 hornbills (2 Papua hornbills [Aceros plicatus] and 1 longtailed hornbill [Tockus albocristatus]) were evaluated because of general listlessness and loss of feather glossiness. Clinical Findings-Because hepatic iron storage disease was suspected, liver biopsy was performed and formalin-fixed liver samples were submitted for histologic examination and quantitative image analysis (QIA). Additional frozen liver samples were submitted for chemical analysis. Birds also underwent magnetic resonance imaging (MRI) under general anesthesia for noninvasive measurement of liver iron content. Serum biochemical analysis and analysis of feed were also performed. Results of diagnostic testing indicated that all 3 hornbills were affected with hepatic iron storage disease. Treatment and Outcome-The iron chelator deferiprone was administered (75 mg/kg [34.1 mg/lb], PO, once daily for 90 days). During the treatment period, liver biopsy samples were obtained at regular intervals for QIA and chemical analysis of the liver iron content and follow-up MRI was performed. In all 3 hornbills, a rapid and large decrease in liver iron content was observed. All 3 methods for quantifying the liver iron content were able to verify the decrease in liver iron content. Clinical Relevance-Orally administered deferiprone was found to effectively reduce the liver iron content in these 3 hornbills with iron storage disease. All 3 methods used to monitor the liver iron content (QIA, chemical analysis of liver biopsy samples, and MRI) had similar results, indicating that all of these methods should be considered for the diagnosis of iron storage disease and monitoring of liver iron content during treatment.
Keywords
CHELATOR DEFERIPRONE, IMAGE-ANALYSIS, STARLING STURNUS-VULGARIS, PHARMACOKINETIC DISPOSITION, DIETARY IRON, OVERLOAD, BIRDS, LIVER, HEMOCHROMATOSIS, TOUCAN

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Chicago
Sandmeier, Peter, Marcus Clauss, Olivio F Donati, Koen Chiers, Ellen Kienzle, and Jean-Michel Hatt. 2012. “Use of Deferiprone for the Treatment of Hepatic Iron Storage Disease in Three Hornbills.” Javma-journal of the American Veterinary Medical Association 240 (1): 75–81.
APA
Sandmeier, P., Clauss, M., Donati, O. F., Chiers, K., Kienzle, E., & Hatt, J.-M. (2012). Use of deferiprone for the treatment of hepatic iron storage disease in three hornbills. JAVMA-JOURNAL OF THE AMERICAN VETERINARY MEDICAL ASSOCIATION, 240(1), 75–81.
Vancouver
1.
Sandmeier P, Clauss M, Donati OF, Chiers K, Kienzle E, Hatt J-M. Use of deferiprone for the treatment of hepatic iron storage disease in three hornbills. JAVMA-JOURNAL OF THE AMERICAN VETERINARY MEDICAL ASSOCIATION. 2012;240(1):75–81.
MLA
Sandmeier, Peter, Marcus Clauss, Olivio F Donati, et al. “Use of Deferiprone for the Treatment of Hepatic Iron Storage Disease in Three Hornbills.” JAVMA-JOURNAL OF THE AMERICAN VETERINARY MEDICAL ASSOCIATION 240.1 (2012): 75–81. Print.
@article{3124590,
  abstract     = {Case Description-3 hornbills (2 Papua hornbills [Aceros plicatus] and 1 longtailed hornbill [Tockus albocristatus]) were evaluated because of general listlessness and loss of feather glossiness. 
Clinical Findings-Because hepatic iron storage disease was suspected, liver biopsy was performed and formalin-fixed liver samples were submitted for histologic examination and quantitative image analysis (QIA). Additional frozen liver samples were submitted for chemical analysis. Birds also underwent magnetic resonance imaging (MRI) under general anesthesia for noninvasive measurement of liver iron content. Serum biochemical analysis and analysis of feed were also performed. Results of diagnostic testing indicated that all 3 hornbills were affected with hepatic iron storage disease. 
Treatment and Outcome-The iron chelator deferiprone was administered (75 mg/kg [34.1 mg/lb], PO, once daily for 90 days). During the treatment period, liver biopsy samples were obtained at regular intervals for QIA and chemical analysis of the liver iron content and follow-up MRI was performed. In all 3 hornbills, a rapid and large decrease in liver iron content was observed. All 3 methods for quantifying the liver iron content were able to verify the decrease in liver iron content. 
Clinical Relevance-Orally administered deferiprone was found to effectively reduce the liver iron content in these 3 hornbills with iron storage disease. All 3 methods used to monitor the liver iron content (QIA, chemical analysis of liver biopsy samples, and MRI) had similar results, indicating that all of these methods should be considered for the diagnosis of iron storage disease and monitoring of liver iron content during treatment.},
  author       = {Sandmeier, Peter and Clauss, Marcus and Donati, Olivio F and Chiers, Koen and Kienzle, Ellen and Hatt, Jean-Michel},
  issn         = {0003-1488},
  journal      = {JAVMA-JOURNAL OF THE AMERICAN VETERINARY MEDICAL ASSOCIATION},
  keyword      = {CHELATOR DEFERIPRONE,IMAGE-ANALYSIS,STARLING STURNUS-VULGARIS,PHARMACOKINETIC DISPOSITION,DIETARY IRON,OVERLOAD,BIRDS,LIVER,HEMOCHROMATOSIS,TOUCAN},
  language     = {eng},
  number       = {1},
  pages        = {75--81},
  title        = {Use of deferiprone for the treatment of hepatic iron storage disease in three hornbills},
  url          = {http://dx.doi.org/10.2460/javma.240.1.75},
  volume       = {240},
  year         = {2012},
}

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