Kazuya Kawazoe

Ileal carcinoid tumor complicating carcinoid heart disease and secondary retroperitoneal fibrosis

A 70‐year‐old man with metastatic liver tumors showed carcinoid syndrome with clinical symptoms of facial flushing, palpitation, dyspnea, and an itching sensation. Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine excretion of serotonin metabolites were confirmed. Autopsy confirmed a serotonin‐immunoreactive 1 cm ileal carcinoid tumor with metastasis to the liver, bone and peritoneum. The tumor cells were argyrophilic but not argentaffin, and showed erythrophagocytosis in the primary lesion. All the four heart valves and bilateral atrial endocardium showed fibromyxoid thickening, indicating the association of carcinoid heart disease. Desmoplastic reaction with deposition of sulfated acid mucopolysaccharides was also observed in the retroperitoneal space (secondary retroperitoneal fibrosis). Stenosis of the bilateral ureters and inferior mesenteric artery provoked hydronephrosis and lethal ischemic necrosis of the left‐sided colon, respectively. Rarity of functioning ileal carcinoid tumor and pathogenesis of systemic fibroplasia are discussed.

Mechanisms of Hepatic Microcirculatory Disturbances Induced by Acute Ethanol Administration in Rats, With Special Reference to Alterations of Sinusoidal Endothelial Fenestrae

Elucidation of the hepatic hemodynamics in acute ethanol administration is an issue of clinical importance for better understanding of alcoholic liver diseases. The purpose of this study is to clarify the mechanism of hepatic microcirculatory disturbances after acute ethanol administration, especially regarding the effects of ethanol on alterations of sinusoidal endothelial fenestrae (SEF) and the involvement of endothelin-l (ET-1) in the mechanism of portal hypertension induced by ethanol. Ethanol was administrated into the portal vein via the mesenteric vein branch of rats as a continuous infusion (4 and 8 mg/min of ethanol) for 60 min. Hepatic tissue blood flow measured with a laser Doppler blood flowmeter was found to be remarkably decreased with time, whereas portal pressure began to increase at 10 min and showed a significant increase by ˜1.5 cm H2 O at 60 min. Ethanol concentrations in blood at 60 min after 4 and 8 mg/min of ethanol infusion were 0.75 mg/ml and 1.77 mg/ml, respectively. At this point, scanning electron microscopy revealed significant decreases in number and diameter of SEF both in zone 1 and zone 3, with the increase in ethanol level. These findings suggested that decreases in number and diameter of SEF, whether primary or secondary, may lead to the impairment of the transport of plasma substances from sinusoids to hepatocytes in acute ethanol administration. Furthermore, the pretreatment of BQ-123 inhibited a decrease in hepatic tissue blood flow and an increase in portal pressure caused by ethanol, indicating that ET-1 may be involved in the mechanism of hepatic circulatory disturbances in acute ethanol administration.

A Case of Budd-Chiari Syndrome Associated with Polycythemia Vera.

症例は43歳, 女性. 1993年9月頃より腹水の貯留, 黄疸が出現し, 1994年1月入院. 血液検査では全血球系の著増とともに著明な肝障害が認められた. 血液所見および骨髄生検より真性多曲症ならびに急性肝不全と診断した. また腹部超音波検査にて右・中・左肝静脈は血栓で充満し面流は認められず, 血栓による肝静脈の閉塞が考えられた. 入院後急速に肝不全が進行し, 1カ月の経過でDIC, 多臓器不全のため死亡. 病理解剖では肝静脈内に器質化した血栓が認められ, 肝細胞はzone 3を中心に広範な変性, 壊死を示していた. 本邦におけるBudd-Chiari症候群の多くは膜様閉塞に伴うものであり, 肝静脈血栓によるBudd-Chiari症候群の報告は少ない. 特に真性多血症に伴う肝静脈閉塞は本例を含め2例のみで極めて稀な症例と考えられる.