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肝肺症候群と門脈圧亢進症に伴う肺動脈性肺高血圧症の臨床病態の差異

出典
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1: International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension.
著者: Michael J Krowka, Michael B Fallon, Steven M Kawut, Valentin Fuhrmann, Julie K Heimbach, Michael A E Ramsay, Olivier Sitbon, Ronald J Sokol
雑誌名: Transplantation. 2016 Jul;100(7):1440-52. doi: 10.1097/TP.0000000000001229.
Abstract/Text: Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Society Task Force on Pulmonary-Hepatic Disorders convened in 2002 to standardize the diagnosis and guide management of these disorders. These International Liver Transplant Society diagnostic and management guidelines are based on that task force consensus and should continue to evolve as clinical experience dictates. Based on a review of over 1000 published HPS and POPH articles identified via a MEDLINE search (1985-2015), clinical guidelines were based on, selected single care reports, small series, registries, databases, and expert opinion. The paucity of randomized, controlled trials in either of these disorders was noted. Guidelines are presented in 5 parts; I. Definitions/Diagnostic criteria; II. Hepatopulmonary syndrome; III. Portopulmonary hypertension; IV. Implications for liver transplantation; and V. Suggestions for future clinical research.
Transplantation. 2016 Jul;100(7):1440-52. doi: 10.1097/TP.000000000000...

肝肺症候群における低酸素血症、AaDO2開大、肺内シャントの説明

前毛細血管の拡張により肺内シャントが増加してPaO2の低下、AaDO2開大が生じる。
出典
imgimg
1: Hepatopulmonary syndrome--a liver-induced lung vascular disorder.
N Engl J Med. 2008 May 29;358(22):2378-87. doi: 10.1056/NEJMra0707185.

99mTc-MAAによる肺血流シンチグラフィーによる脳への99mTc-MAAの集積

Panel Aは肺の前面から、Panel Bは肺の背面から(腎臓にuptakeがみられる)、Panel CおよびDは右および左脳(正常は<6%であるが、提示した例は62%の取り込みがある)、肺内シャントを99mTc-MAAが通過して脳血管で捉えられたことを示している。
出典
imgimg
1: Hepatopulmonary syndrome--a liver-induced lung vascular disorder.
N Engl J Med. 2008 May 29;358(22):2378-87. doi: 10.1056/NEJMra0707185.

コントラスト心エコー検査

RV(右心室)、RA(右心房)でのマイクロバブルに5心拍くらい遅れてLV(左室)、LA(左房)にマイクロバブルが現れた場合に肺内シャントの存在が示唆される。
出典
imgimg
1: Hepatopulmonary syndrome--a liver-induced lung vascular disorder.
N Engl J Med. 2008 May 29;358(22):2378-87. doi: 10.1056/NEJMra0707185.

肝肺症候群と門脈圧亢進症に伴う肺動脈性肺高血圧症の臨床病態の差異

出典
imgimg
1: International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension.
著者: Michael J Krowka, Michael B Fallon, Steven M Kawut, Valentin Fuhrmann, Julie K Heimbach, Michael A E Ramsay, Olivier Sitbon, Ronald J Sokol
雑誌名: Transplantation. 2016 Jul;100(7):1440-52. doi: 10.1097/TP.0000000000001229.
Abstract/Text: Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Society Task Force on Pulmonary-Hepatic Disorders convened in 2002 to standardize the diagnosis and guide management of these disorders. These International Liver Transplant Society diagnostic and management guidelines are based on that task force consensus and should continue to evolve as clinical experience dictates. Based on a review of over 1000 published HPS and POPH articles identified via a MEDLINE search (1985-2015), clinical guidelines were based on, selected single care reports, small series, registries, databases, and expert opinion. The paucity of randomized, controlled trials in either of these disorders was noted. Guidelines are presented in 5 parts; I. Definitions/Diagnostic criteria; II. Hepatopulmonary syndrome; III. Portopulmonary hypertension; IV. Implications for liver transplantation; and V. Suggestions for future clinical research.
Transplantation. 2016 Jul;100(7):1440-52. doi: 10.1097/TP.000000000000...

肝肺症候群における低酸素血症、AaDO2開大、肺内シャントの説明

前毛細血管の拡張により肺内シャントが増加してPaO2の低下、AaDO2開大が生じる。
出典
imgimg
1: Hepatopulmonary syndrome--a liver-induced lung vascular disorder.
N Engl J Med. 2008 May 29;358(22):2378-87. doi: 10.1056/NEJMra0707185.