Sutton F D, Zwillich C W, Creagh C E, Pierson D J, Weil J V
Ann Intern Med. 1975 Oct;83(4):476-9. doi: 10.7326/0003-4819-83-4-476.
Ten patients with the Pickwickian syndrome, characterized by obesity, hypoxemia, hypercapnia, polycythemia, and cor pulmonale, underwent long-term treatment as outpatients with medroxyprogesterone acetate. Although there was no significant weight change in the group, PaO2 rose 12.6 +/- 2.7 mm Hg (SEM) from 49 +/- 2.6 mm Hg to 62 +/- 2.3 mm Hg (P less than 0.001), while PaCO2 fell 13 +/- 2.6 mm Hg from 51 +/- 1.9 mm Hg to 38 +/- 1.2 mm Hg (P less than 0.001). Hematocrit fell from 56 +/- 2.5% to 50 +/- 1.2%, a mean fall of 6% (P less than 0.01), during medroxyprogesterone acetate therapy. In the 2 patients who had cardiac catheterization before and during medroxyprogesterone acetate therapy, mean pulmonary arterial pressure fell 13 and 19 mm Hg. There were no recurrences of cor pulmonale during treatment. These effects on arterial blood gas values and clinical state were sustained during therapy. On withdrawal of medroxyprogesterone acetate during 1-month period, arterial oxygen and carbon dioxide tensions deteriorated to their previous pretreatment values. Reinstitution of medroxyprogesterone acetate caused improvement in both the oxygen and carbon dioxide tensions. We conclude that sublingual medroxyprogesterone acetate therapy is useful in the management of the Pickwickian syndrome.
10例患有匹克威克综合征的患者,其特征为肥胖、低氧血症、高碳酸血症、红细胞增多症和肺源性心脏病,作为门诊患者接受了醋酸甲羟孕酮的长期治疗。尽管该组患者体重无显著变化,但动脉血氧分压(PaO2)从49±2.6 mmHg升至62±2.3 mmHg,升高了12.6±2.7 mmHg(标准误)(P<0.001),而动脉血二氧化碳分压(PaCO2)从51±1.9 mmHg降至38±1.2 mmHg,下降了13±2.6 mmHg(P<0.001)。在醋酸甲羟孕酮治疗期间,血细胞比容从56±2.5%降至50±1.2%,平均下降6%(P<0.01)。在接受醋酸甲羟孕酮治疗前后均进行心导管检查的2例患者中,平均肺动脉压分别下降了13 mmHg和19 mmHg。治疗期间未出现肺源性心脏病复发。这些对动脉血气值和临床状态的影响在治疗期间持续存在。在1个月的时间内停用醋酸甲羟孕酮后,动脉血氧和二氧化碳分压恶化至治疗前的水平。重新使用醋酸甲羟孕酮可使血氧和二氧化碳分压均得到改善。我们得出结论,舌下含服醋酸甲羟孕酮治疗对匹克威克综合征的管理是有用的。