2012; 18: 200 207 Necropsy 1 1 1 2 65 11 autoimmune hepatitisaih 14 21 1 5 13 Necropsy CHF1 A1 Kupffer cell hemosiderin Berlin blue KEY WORDS: hemochromatosis, autoimmune hepatitis, liver cirrhosis, necropsy Ayada M 1, Hotta N 1, Kurokawa T 2 : A case of autoimmune hepatitis diagnosed with iron overload disease in the necropsy. JJPH 2012; 18: 200 207 I. autoimmune hepatitisaih 11 necropsy 1 AIH II. 65 11 AIH 14 Child- Pugh Grade B AIH 1 453-0016 35-28 2 453-0016 35-28 1 Department of Hepato-Gastroenterology, Masuko Memorial Hospital, 35-28 Takebashi Nakamura-ku Nagoya-shi, Aichi, 453-8566 Japan 2 Department of Surgery, Division of Hepato-Gastroenterology, Masuko Memorial Hospital, 35-28 Takebashi Nakamura-ku Nagoyashi, Aichi, 453-8566 Japan 18 2011 2012 2 2 2012 7 31 300 mg 40 mg 25 mg 20 12 80 mg 50 mg 120 mg 21 1 18 8 Red Cells ConcentratesRCC5 149 cm 44 Kg 36.2 100 82 mmhg 86 SATO 2 96 200
1 Blood chemistry Peripheral blood Viral marker TP 5.4 g dl WBC 4.310 3 ml Syphilis TPAb Alb 2.7 g dl RBC30210 4 ml HBsAg T. bil 2.3 mg dl Hb 11 g dl HBsAb GOT 52 IU l Ht 32.2 HCVAb GPT 41 IU l Plt 4.210 4 ml Blood gas analysis LDH 414 IU l Differential white blood count ph 7.506 ALP 440 IU l Neutro 79.2 PO2 70.2 mmhg g-gtp 13 IU l Eosino 0 PCO2 32.2 mmhg AMY 98 IU l Lymph 11.8 HCO3 24.9 mmoll BUN 50 mg dl Mono 8.1 SATO2 97.8 Cr 1.03 mg dl Baso 0.9 Na 131 meq l Coagulation K 4.3 meq l PT 49 Cl 99 meq l HPT 56 glu 112 mg dl Tumor marker UA 6.1 mg dl AFP 3.7 ng mlnormal range0.0 16.5 T. cho 150 mg dl PIVKA 19 mau mlreference value 40 CRP 0.1 mg dl Autoantibody ANA 2560 timesreference value 40 AMA negative 1 X-P 1Hb 11 g dl Plt 4.210 4 ml Alb 2.7 g dl T. bil 2.3 mg dl, AST 52 mg dl, ALT 41 mg dl, LDH 414 mg dl, ALP 440 mg dlg-gpt 13 mg dl 49 56 2560 40 room airph7.506, 2012; 18: 200 207 201
2 CT CT 70 HUHounsfield unit 3 CT 202 2012; 18: 200 207
Lm, F1, Cb, RC 0 Lg PHG 4 PO270.2 mmhg, PCO232.2 mmhg, HCO324.9 mmol l, SATO297.8 X-P 1 CT 2 CT 3 CT 70 HUHounsfield unit 4 Lm, F1, Cb, RC 0 Lg PHG 5 4 necropsy 6 8Hematoxylin-eosin Kupffer hemosiderin Azan CHF1 A1Berlin blue Kupffer bridging fibrosis AIH III. AIH 1 3 NASH GIgG 2012; 18: 200 207 203
4 necropsy 5 1 3 AIH IgG AIH 1999 13 2008 4 AIH 1996 2560 5 3 AIH CT AIH 4 6 4 2 7, 8 204 2012; 18: 200 207
AHematoxylin-eosin stain BAzan stainchf1 CBerlin blue stain Berlin blue 640 A100CHA1 B200 hemosiderin 7Hematoxylin-eosin stain 7, 13 C NASH 4, 9 13 AIH Kupffer 2012; 18: 200 207 205
A100 Berlin blue B, C200 Kupffer 8Berlin blue stain 2, 14 hepcidin 4 6, 15 C hepcidin hepcidin 16 18 necropsy Kupffer Kupffer C hepcidin 2 RCC 5 10 13, 18 CT 19 CT 22 72 HU 72 HU 20 CT 70 HU 2, 21, 22 21 adenosine triphosphate ATP 3 2 2 Fenton 206 2012; 18: 200 207
2, 21 23 AIH AIH bridging fibrosis 24 24 AIH necropsy hemosiderin F1 A1 AIH 1 necropsy AIH 25, 26 necropsy AIH AIH IV. necropsy 1 1 2010; 105: 986991 2 1 2006; 47: 341346 3 2009; 50: 618625 4 2011; 100: 24122424 5 2007; 48: 3645 6 hepcidin. BIO Clinica 2006; 21: 350354 7 1998; 20: 493494 8 Zone 1 2007; 55: 8391 9 1 2008; 49: 574580 10 MDS PBC 1 2007; 44: 148151 11 refractory anemia with ringed siderblasts (RARS 1 1993; 71: 363366 12 myelodysplastic syndrome 1 1986; 75: 14381442 13 1986; 44: 18791891 14 Kyosuke Kaji, Yasuni Nakamura, Motoko Sasaki, et al: Hemosiderin Deposition in Portal Endotherial Cells. Human Pathology 1995; 26: 10801085 15 2005; 214: 11: 945948 16 Naoki Fujita, Ryosuke Sugimoto, Masaki Takeo, et al: Hepcidin expression in the liver: relatively low level in patients with chronic hepatitis C. Mol Med 2007; 13: 97 104 17 Naoki Fujita, Ryosuke Sugimoto, Satoshi Motonishi, et al: Patients with chronic hepatitis C achieving a sustained virological response to peginterferon and ribavirin therapy recover from impaired hepcidin secretion. J Hepatol 2008; 49: 702710 18 2009; 39: 418425 19 1979; 20: 1435 1439 20 Howard JM, Ghent CN, Carey LS, et al: Diagnostic efficacy of hepatic computed tomography in the detection of body iron overload. Gastroenterology 1983; 84: 209215 21 2006; 52: 875885 22 2003; 47: 489495 23 Reddy MB, Clark L: Iron, oxidative stress, and disease risk. Nutrition Reviews 2004; 62: 120124 24 BIO Clinica 2010; 25: 264268 25 2011; 108: 18231836 26 2005; 102: 1724 2012; 18: 200 207 207