HEPATOPULMONARY SYNDROME - CLINICAL OBSERVATIONS AND LACK OF THERAPEUTIC RESPONSE TO SOMATOSTATIN ANALOG
HEPATOPULMONARY SYNDROME - CLINICAL OBSERVATIONS AND LACK OF THERAPEUTIC RESPONSE TO SOMATOSTATIN ANALOG
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DOI：
10.1378/chest.104.2.515
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发表时间：
1993-08-01
期刊：
影响因子：
9.6
通讯作者：
CORTESE, DA
中科院分区：
文献类型：
--
作者：
KROWKA, MJ;DICKSON, ER;CORTESE, DA
我们回顾性地研究了1984年至1991年在马约医学中心评估的22例肝病综合征（HPS）患者。所有患者均患有肝硬化并伴有门静脉高压症的临床证据; 13例（59%）患者在仰卧位呼吸室内空气时出现严重低氧血症（PaO 2 < 60 mm Hg），16例患者中有14例（88%）患者呼吸室内空气时出现直立性低氧血症。根据血管造影观察，我们将HPS的肺血管闭塞分为1型和2型。对100%氧气的反应和治疗方案可能在血管造影模式上有所不同。在连续动脉血气检测研究的7例患者中，有5例记录了与临床稳定肝功能障碍相关的PaO 2显著恶化; 4例随后在48个月内死亡。总死亡率为41%，平均发生在诊断后2.5年。在22例患者中的7例中，我们前瞻性地研究了连续4天皮下注射生长抑素类似物的效果。当呼吸室内空气或100%氧气时，没有记录到PaO 2的显著改善（p < 0.05）。我们的结论是，在临床上稳定的肝功能障碍和氧合恶化的患者中，预后很差。我们的数据结合最近的手术报告表明，肝移植可能是HPS和氧合恶化患者的治疗选择。
We retrospectively studied 22 patients with hepatopulmonary syndrome (HPS) evaluated at the Mayo Medical Center from 1984 to 1991. All patients had hepatic cirrhosis with clinical evidence of portal hypertension; 13 (59 percent) had severe hypoxemia while breathing room air in the supine position (PaO2 < 60 mm Hg), and 14 of 16 (88 percent) had orthodeoxia breathing room air. On the basis of angiographic observations, we defined type 1 and type 2 patterns of pulmonary vascular ahnormalities in HPS. Response to 100 percent oxygen and therapeutic regimens may differ in the angiographic patterns. Substantial deterioration in PaO2 associated with clinically stable hepatic dysfunction was documented in five of seven patients studied with sequential arterial blood gas testing; four subsequently died within 48 months. Overall mortality was 41 percent, occurring a mean of 2.5 years after diagnosis. In 7 of the 22 patients, we prospectively studied the effect of somatostatin analogue given subcutaneously for 4 consecutive days. No significant improvement in PaO2 was documented while breathing room air or 100 percent oxygen (p < 0.05). We conclude that in selected patients with clinically stable hepatic dysfunction and deteriorating oxygenation, the prognosis is poor. Our data in combination with recent surgical reports suggest that liver transplantation may be the treatment of choice in patients with HPS and worsening oxygenation.