Selmaier M, Cidlinsky K, Ell C, Hahn E G
Medizinische Klinik I mit Poliklinik, Universität Erlangen-Nürnberg.
Dtsch Med Wochenschr. 1993 Jul 16;118(27-28):1015-9. doi: 10.1055/s-2008-1059420.
Heart failure occurred in a 50-year-old woman as a result of calcified haemangiomatosis of the liver with a high shunt volume. In the subsequent years there were several bleedings from peptic ulcers. Ultrasonography revealed an increase in liver size and the previously diagnosed calcification. 14 years later the patient was again hospitalized because of increasing weakness and stress dyspnoea; the haemoglobin level was 5.5 g/dl. In addition to the florid gastric and duodenal ulcers, angiodysplasias were for the first time demonstrated in the stomach, duodenum and sigmoid colon, as were telangiectasias in the face and echo-dense round foci in the spleen. After transfusion of red cell concentrates and healing of the peptic ulcers under a 14-day treatment with omeprazole (20 mg two times daily by mouth) and amoxycillin (750 mg three times daily by mouth) the haemoglobin level increased at first (10 g/dl), then tarry stools were once again noted. The condition stabilized after laser coagulation of all accessible gastrointestinal angiodysplasias. The isolated calcified hepatic haemangiomatosis, diagnosed 14 years previously, is most likely a rare variant of hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease).
一名50岁女性因肝脏钙化性血管瘤病伴高分流体积而发生心力衰竭。在随后的几年里，出现了几次消化性溃疡出血。超声检查显示肝脏大小增加以及先前诊断出的钙化。14年后，患者因日益虚弱和劳力性呼吸困难再次住院；血红蛋白水平为5.5 g/dl。除了明显的胃和十二指肠溃疡外，首次在胃、十二指肠和乙状结肠发现血管发育异常，面部出现毛细血管扩张，脾脏出现回声致密的圆形病灶。输注红细胞浓缩液并在使用奥美拉唑（每日口服2次，每次20 mg）和阿莫西林（每日口服3次，每次750 mg）进行14天治疗后消化性溃疡愈合，血红蛋白水平起初升高（10 g/dl），随后再次出现柏油样便。对所有可触及的胃肠道血管发育异常进行激光凝固后病情稳定。14年前诊断出的孤立性钙化性肝血管瘤病很可能是遗传性出血性毛细血管扩张症（奥斯勒-韦伯-伦杜病）的一种罕见变体。
