Kobayashi Takashi, Suzuki Kenji
Department of Anesthesiology, School of Medicine, Iwate Medical University, 19-1 Uchimaru, Morioka-shi, Iwate, 020-8505, Japan.
JA Clin Rep. 2019 Oct 30;5(1):69. doi: 10.1186/s40981-019-0293-3.
Hemoglobin Kansas (Hb Kansas) is a rare disease with cyanosis. We report a case of anesthetic management for a patient with an acute aortic dissection complicated by Hb Kansas.
We encountered a 62-year-old male, surgical patient with an acute aortic dissection complicated by postoperative Hb Kansas. During anesthesia, his arterial oxygen saturation was low, while the partial pressure of arterial oxygen was within the normal range. The patient underwent ascending aortic replacement under hypothermic circulation arrest with a bladder temperature of 22 °C after introducing cardiopulmonary bypass. The patient was then referred to the hematology department for detailed examination and was diagnosed as having Hb Kansas through genetic analysis at 2 months after surgery.
Except for apparent cyanosis, Hb Kansas causes no clinical problems because the delivery of oxygen to peripheral tissues may be enhanced for such patients. When we perform anesthetic management for cyanosis patients with unknown causes, it is necessary to consider the oxygen supply-demand balance.
堪萨斯血红蛋白病（Hb Kansas）是一种罕见的伴有发绀的疾病。我们报告一例急性主动脉夹层并发Hb Kansas患者的麻醉管理情况。
我们遇到一名62岁男性外科患者，患有急性主动脉夹层并术后并发Hb Kansas。麻醉期间，他的动脉血氧饱和度较低，而动脉血氧分压在正常范围内。在建立体外循环后，患者在膀胱温度为22°C的低温循环停跳下接受了升主动脉置换术。术后2个月，患者被转诊至血液科进行详细检查，并通过基因分析被诊断为患有Hb Kansas。
除明显发绀外，Hb Kansas不会引起临床问题，因为此类患者向周围组织的氧输送可能会增强。当我们对病因不明的发绀患者进行麻醉管理时，有必要考虑氧供需平衡。
