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

机译:用去铁酮治疗三只犀鸟的肝铁贮积病

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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. (J Am Vet Med Assoc 2012;240:75-81)
机译:病例描述:由于总体无精打采和羽毛光泽度下降,对3只犀鸟(2只巴布亚犀鸟[Aceros plicatus]和1只长尾犀鸟[Tockus albocristatus])进行了评估。临床发现-因为怀疑存在肝铁存储疾病,因此需要进行肝活检,并用福尔马林固定的肝样品进行组织学检查和定量图像分析(QIA)。另外的冷冻肝样品被提交用于化学分析。鸟类还在全身麻醉下接受了磁共振成像(MRI),以无创方式测量肝铁含量。还进行了血清生化分析和饲料分析。诊断测试结果表明,所有3只犀鸟都患有肝铁存储疾病。治疗和结果-给予铁螯合剂去铁酮(75 mg / kg [34.1 mg / lb],PO,每天一次,持续90天)。在治疗期间,定期采集肝脏活检样本进行QIA,并进行肝铁含量的化学分析和后续MRI。在所有3只犀鸟中,均观察到肝铁含量迅速且大量下降。定量肝铁含量的所有3种方法均能够验证肝铁含量的降低。临床相关性-口服给予的去铁酮被发现可有效降低这3例铁储存病犀鸟的肝铁含量。用于监测肝铁含量的所有3种方法(QIA,肝活检样品的化学分析和MRI)均具有相似的结果,表明在诊断铁储积病和监测肝铁含量时应考虑所有这些方法。治疗。 (J Am Vet Med Assoc 2012; 240:75-81)

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