개에서 발생한 구리 축적성 간경화에 D-penicillamine, SAMe, DBB로 병용 치료하여 장기간 생존한 1례 Long-term Management of Copper-associated Hepatic Cirrhosis with D-penicillamine, SAMe, and DBB in a Dog원문보기
4살령의 아메리칸 코가 스파니엘이 식욕부진, 몸떨림 그리고 복부 팽만증세로 내원하였다. 혈청학적 검사, 방사선 검사, 초음파 검사 그리고 간생검을 통한 조직병리학적 검사를 통해 경화로 진행된 만성 간염으로 진단할 수 있었으며, 특수 염색법을 통해 대부분의 간세포내에 구리가 축적되어 있는 것을 확인할 수 있었으며, 구리 농도는 1460 ppm으로 매우 높음을 알 수 있었다. 이러한 검사 결과를 바탕으로 구리 축적으로 인한 간염이 간경화까지 진행된 말기 병변으로 진단하고 D-penicillamine, s-adenosymethionine, biphenyl-dimethyl-dicarboxylate과 대증 치료로 본 환자를 치료 하였으며, 본 환자는 진단 후 35개월을 생존하는 것에 성공하였다.
4살령의 아메리칸 코가 스파니엘이 식욕부진, 몸떨림 그리고 복부 팽만증세로 내원하였다. 혈청학적 검사, 방사선 검사, 초음파 검사 그리고 간생검을 통한 조직병리학적 검사를 통해 경화로 진행된 만성 간염으로 진단할 수 있었으며, 특수 염색법을 통해 대부분의 간세포내에 구리가 축적되어 있는 것을 확인할 수 있었으며, 구리 농도는 1460 ppm으로 매우 높음을 알 수 있었다. 이러한 검사 결과를 바탕으로 구리 축적으로 인한 간염이 간경화까지 진행된 말기 병변으로 진단하고 D-penicillamine, s-adenosymethionine, biphenyl-dimethyl-dicarboxylate과 대증 치료로 본 환자를 치료 하였으며, 본 환자는 진단 후 35개월을 생존하는 것에 성공하였다.
A 4-year-old intact female American Cocker Spaniel presented with lack of appetite, shivering, and abdominal distension. It was initially diagnosed with chronic hepatitis with cirrhosis, by serum chemistry, radiography, ultrasonography, and histopathologic examination following liver biopsy. Abundan...
A 4-year-old intact female American Cocker Spaniel presented with lack of appetite, shivering, and abdominal distension. It was initially diagnosed with chronic hepatitis with cirrhosis, by serum chemistry, radiography, ultrasonography, and histopathologic examination following liver biopsy. Abundant copper granules were detected in most hepatocytes with rhodanine stain, with hepatic copper concentration at 1460 ppm (reference range: <400 ppm). Based on these findings, copper-associated hepatitis with cirrhosis was diagnosed and successfully managed with long-term D-penicillamine, s-adenosylmethionine, biphenyl-dimethyl-dicarboxylate and supportive care. The spaniel died 35 months after diagnosis.
A 4-year-old intact female American Cocker Spaniel presented with lack of appetite, shivering, and abdominal distension. It was initially diagnosed with chronic hepatitis with cirrhosis, by serum chemistry, radiography, ultrasonography, and histopathologic examination following liver biopsy. Abundant copper granules were detected in most hepatocytes with rhodanine stain, with hepatic copper concentration at 1460 ppm (reference range: <400 ppm). Based on these findings, copper-associated hepatitis with cirrhosis was diagnosed and successfully managed with long-term D-penicillamine, s-adenosylmethionine, biphenyl-dimethyl-dicarboxylate and supportive care. The spaniel died 35 months after diagnosis.
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제안 방법
The patient managed well with D-penicillamine; SAMe, and BDD twice a day until it died 35 months after diagnosis. At necropsy the liver tissue was submitted to Antech Diagnostics, Inc. (Memphis, USA) for histologic evaluation and copper dialysis. Necropsy revealed gross macronodule cirrho sis (Fig IB).
3333px;">clinical signs, including anorexia, shivering, and ascites, gradually diminished, although changes in serum chemistry profiles were not significant. The patient managed well with D-penicillamine; SAMe, and BDD twice a day until it died 35 months after diagnosis. At necropsy the liver tissue was submitted to Antech Diagnostics, Inc.
D-penicillamine (10 ㎎/㎏, PO, bid) was prescribed as a copper chelator and was administered 1 hr before feeding. The patient was examined bi-weekly, and the owner was asked to report adverse effects such as vomiting, lethargy, nausea, fever, and skin problems. The
대상 데이터
This study was supported by the Brain Korea 21 Program for Veterinary Science, and Research Institute of VeterinMy Science, College of Veterinary Medicine, Seoul National University.
성능/효과
7℃), mildly dehydrated and the abdomen was distended. Complete blood cell count results were within the reference range, while abnormal serum chemistry results included elevated ALT (466 U/L; reference range: 3-50 U/L), AST (64 U/L; reference range: 10-43 U/L), ALP (186 U/L; reference range: 8-100 U/L), and GGT (22 U/L; reference range: 0-8 U/L) activity, and low BUN (5 mg/dl; reference range: 8-30 mg/dl), glucose (75 mg/dl; reference range: 80-120 mg/dl), albumin (2 g/dl; reference range: 2.8-4.5 g/dl), and total protein (4.9 g/dl; reference range: 5.0-7.5 g/dl). Cystocentesis and abdominocentesis were performed to collect urine and ascites.
While liver cirrhosis did not improve, and necropsy showed macronodular cirrhosis, staining did not reveal a significant amount of hepatocellular copper. The results of semi-quantitative staining for copper concentrations suggest that the long-term treatment with D-penicillamine, SAMe, and BDD was successful in eliminating copper from the hepatocytes.
Supportive care included whole blood transfusion (120 ml), fluid therapy, and Vitamin (1 ㎎/㎏ SC, bid). Three days later, RBC count had increased (4.38 x 106cells/gl) and PCV was slightly increased (25%).
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