JSACHD_Vol5_No3_Dec2016.book
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1 (2016 ) Fontan 1) 2) 1) 2) Fontan circulation, Fontan associated liver disease, Liver fibrosis, Liver tumor, Central venous Pressure 25% I. FALD Fontan Fontan ( CVP) Fontan associated liver disease ( FALD) 1981 Stanton Fontan 2010 FALD 1 1) FALD (Focal nodular hyperplasia FNH ) 2) 1 Fontan2010, Stanton RE, 1981 Fontan 21. Lemner JH,1987 van Nieuwenhuizen RC, 1999 Narkewics MR, 2003 Ghaferi AA, 2005 Kisewetter CH, 2005, 2007 Kendall TJ, 2007 Composilvan S, 2008 Friedrich-Rust M, 2008 Saliba T, 2010 Babaogu K, 2010 Baek JS, Fontan 5.. Fontan 20 γgtp Fontan , 2.. Fontan CPC, 5 CLN, 4, 1. Fontan 12 11/12 CT, 4/12, 7/12,. Fontan 16.. Fontan 18,, 14, 2.. Fontan 34,. Fontan 39 FibroScan elastrography, 37. Fontan 2. Fontan 7MRI. Fontan (41%). (26%), (7%), (21%), (3%). CPC: chronic passive congestion, CLN: centrilobular necrosis 1 12
2 ( ) FALDCVP Fontan (fenestration) CVP FALD Fontan 3) ( 2) 2010 CVP 6) II. FALD FALD Fontan 50% FALDPundi 195 FALD 40 (21%) 3) Lindsay Fontan (55%) 4) FALD FALD Kupffer Transforming growth factor-β (TGF-β) Matrix metalloproteikinase (MMP) TGF-β Disse ( ) () MMP MMP 2 Comparison of blood tests between non-lc and LC LC(-) n=33 LC(+) n=8 PT-INR n.s. TBil n.s. ALT n.s. Plt n.s. WBC n.s. BNP n.s. P-III-P n.s. IV Collagen n.s. Hyaluronic a n.s. (Tanaka Y, et al. Hepatic complications in adult patients after Fontan operation, 2009) 13
3 ( ) Simonetto (thrombosis) fibronection release 7) FALD FALD Fontan III. FALD AST ALT Fontan IV -III- (P-III-P) FALD (AFP) PIVKA-II K C Mac-2Binding Protein (M2BP) (M2BPGi) C FALD M2BPGi M2BPGi BNP M2BPGi ( ) IV. FALD hyperechoic spots (HES) ( 1) FibroScan MR elastography MRI CT hypervascular mass Zonal enhancement HCC FNH 2 FALD Fontan Fontan wedge V. FALD 3 14
4 日本成人先天性心疾患学会雑誌 (2016年12月) 図1 11 歳 女児 無脾症候群 単心室であり 3 回の心臓手術を経て 2 歳9 か月にFontan 術を受けた 精査入院した AST 27 IU/l ALT 18 IU/l GGT 127 IU/l ヒアルロン酸 P-III-Pは高値 %PTは 74%であった 超音波では右葉全体に Hyperechoic spots がみられた A. HE C. AM B. HE 肝組織１ D. Ret 図2 図 1 と同一症例であり で HESの見られた部分をエコーガイド下で狙撃肝臓針生検査をした 門脈域には炎症を伴わない線維化 A 中心静脈周囲の類洞の著明な拡張 B 架橋形成 C 偽小葉形成 D がみられ 肝硬変と診断した 15
5 ( ) 3 FALD Disse FALD (fenestration) QOL Fontan QOL FALD 1) et al, Fonan 29: , ) Ghaferi AG, et al, : Progression of liver pathology in patients undergoing the Fontan procedure : Chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thoracic and Cardiovascular Surgery 129: , ) et al, Fontan 1 111: 55-59, ) Kiesewetter CH, et al, :Hepatic changes in the failing Fontan circulation. Heart 93: , ) Pundi K, et al. : Liver disease in patients after the Fontan operation. Am J Cardiol 117: , ) Lindsay I, et al. : Impact of liver disease after the fontan operation. 115: , ) Simonetto DS, et al. Chronic venous congestion derives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces. Hepatology 61: , 2015 Fontan associated liver disease Tomoo Fujisawa 1), Yasuhiko Tanaka 2) 1) Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohamashi Tobu Hospital 2) Department of Pediatric Cardiology, Shizuoka Children s Hospital Abstract Recently, hepatic complications have been increasingly reported in patients who underwent Fontan surgery for serious complex congenital heart diseases, especially for single ventricle. Hepatic complications are those of liver fibrosis, liver cirrhosis, liver tumors, etc. The sinusoidal fibrosis is common feature in Fontan associated liver disease (FALD). A speculation of sinusoidal fibrosis related the mechanical expansion of liver sinusoid causes thrombosis in sinusoid, and stimulating fibronectin release from Ito cells (hepatic satellite cells). In FALD, hepatocytes are not destroyed, so the levels of transaminase heard to rise. Key words Fontan circulation, Fontan associated liver disease, Liver fibrosis, Liver tumor, Central venous Pressure 16
BUN, CRP K mg/ cm, 49.6 kg, BMI /72 mmhg, 92/ Hb 6.7 g/dl PT-INR CT 1 MRI 2a, b T1 T2 T1 MRI
20 2 2015 1 2 3 85 K CT Abstract A case of renal subcapsular hematoma resulting from trigger point injection under excessive effect of anticoagulant YAMANE Tateki, UMEDA Akira and SHIMAO Hitoshi An 85-year-old
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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
Table 1 Isolated venous thrombi in soleal muscle by ultrasonography US finding Acute Chronic Total No. Early stage non-retracted slightl
Online publication October 8, 2010 原 著 第 50 回総会座長推薦論文 孤立性ひらめ筋内静脈血栓と抗核抗体との関連 1 2 要旨 : 89 116 D 3 30 70 63 26 100 J Jpn Coll Angiol, 2010, 50: 417 422 Key words: isolated soleal venous thrombi, recurrent
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Special Issue 1Sudden cardiac death symposium in Tokushima Special Issue 2How to do for your health? Reviews Original Epidemiologic problems of sudden death of school children in Tokushima Prefecture
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2015; 21: 5866 Yoichi Goto: Current status and future perspective of cardiac rehabilitation in Japan. J Jpn Coron Assoc 2015; 21: 5866 I 1 1 3 1 2 3 QOL 2 Fig. 1 1 196070 AMI AMI 1980 AMI CABG comprehensive
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症例報告 37 急性大動脈解離に対する上行大動脈置換術後の大動脈基部拡張 大動脈弁閉鎖不全に対する自己弁温存基部置換術 (remodeling 手術 ) 概要 : mm remodeling 索引用語 : remodeling Remodeling technique for aneurysm of aortic root and moderate aortic valve regurgitation
Table 1 Laboratory data on admission. Fig. 1 US shows a hyperechoic large tumor. Fig. 2 CT shows a large hepatic tumor. Central necrosis and dilatatio
Table 1 Laboratory data on admission. Fig. 1 US shows a hyperechoic large tumor. Fig. 2 CT shows a large hepatic tumor. Central necrosis and dilatation of the intrahepatic bile duct can be found. Fig.
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Vol.1_No.2_Dec.2012.book
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18 61 1 2011 症例報告 2010 5 13 2010 12 20 A Case of Varicella with Severe Back Pain in a Patient with Systemic Lupus Erythematosus RIE SAITO, KIORI YANO, FUMIKO KATSUSHIMA, HARUYO IWADATE, HIROKO KOBAYASHI,
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Arts and Sciences 2 Diagnostic significance of contrast-enhanced ultrasonography for hepatocellular adenoma: experiences of two cases 41080 Key words: Abdominal ultrasonography Contrast-enhanced ultrasonography
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134 Vol. 52, No. 23 Liver transplantation in Japan. Registry by the Japanese Liver Transplantation Society The Japanese Liver Transplantation Society Summary As of December 31, 2016, a total of 8,825 liver
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156 Vol. 50, No. 23 Liver Transplantation in Japan Registry by the Japanese Liver Transplantation Society The Japanese Liver Transplantation Society Summary As of December 31, 2014, a total of 7,937 liver
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原著・報告・記録（４４行）／Ｐ２６１～２７４＿報告 肝移植症例登録
261 Liver Transplantation in Japan Registry by the Japanese Liver Transplantation Society The Japanese Liver Transplantation Society Summary As of December 31, 2013, a total of 7,474 liver transplants
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特別寄稿 PEDIATRIC CARDIOLOGY and CARDIAC SURGERY VOL. 26 NO. 4 ( ) Prospect of Pediatric Cardiac Surgery 50 年を振り返って Key word: prospect of pediatric
特別寄稿 PEDIATRIC CARDIOLOGY and CARDIAC SURGERY VOL. 26 NO. 4 (274 279) Prospect of Pediatric Cardiac Surgery 50 年を振り返って Key word: prospect of pediatric cardiac surgery, tetralogy of Fallot, single ventricle,
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原著・報告・記録（４４行）／Ｐ１４５～１５９＿報告 肝移植症例登録
145 Liver Transplantation in Japan Registry by the Japanese Liver Transplantation Society The Japanese Liver Transplantation Society Summary As of December 31, 2015, a total of 8,387 liver transplants
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2014Vol.63No.4p.383393 Thermal environment for health and safety Hirohumi HAYAMA Masaya SAITO Haruka MIKAMI Faculty of Engineering, Hokkaido University School of Design, Sapporo City University Graduate
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社会学部紀要 １１４号☆／２２．松村
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1) Garner J. S.: The Hospital Infection Control Practices Advisory Committee, CDC, Guidelines for Isolation Precautions in Hospitals, 1996. http://wonder.cdc.gov/wonder/prevguid/ p0000419/p0000419.asp.
1996 papilloma virus 2001 Bowen AIHA PSL1mg/kg BMA PRCA parvovirus B19 PVB19 DNA PCR PV IgM 4 PVB19 PRCA MAP PVB19 DNA DNA PR
1996 papilloma virus 2001 Bowen2002 12 2003 2 2002 9 10 AIHA PSL1mg/kg BMAPRCA parvovirus B19 PVB19 DNA PCR PV IgM 4PVB19 PRCA MAPPVB19DNA DNA PRCA IVIG;20 50g/ IVIG PVB19AIHA steroid 15 20mg/day 2003
2 W.T T.S. 70 2 1 238 WBC7400/µl, RBC 386 10 4 /µl, Hb 11.6 g/dl, Ht 36.2%, PLT 25 10 4 /µl AST 19 IU/l, ALT 11 IU/l, LDH 182 IU/l, BUN 15 mg/dl, Cr 0.54 mg/dl, CRP6.83 mg/dl, RF 353.9IU/ml, CCP 28.0IU/ml