Servicio de Anestesiología y Reanimación, Hospital Obispo Polanco, Teruel, Spain.
Servicio de Anestesiología y Reanimación, Hospital Obispo Polanco, Teruel, Spain.
Rev Esp Anestesiol Reanim (Engl Ed). 2022 Jun-Jul;69(6):360-363. doi: 10.1016/j.redare.2022.06.003. Epub 2022 Jun 24.
Familial hypokalaemic periodic paralysis (FHPP) is an uncommon genetic disease characterized by muscle weakness associated with hypokalaemia. Episodes are precipitated by drugs, stress, metabolic diseases, hypothermia or infection. We report the case of a 38-year-old pregnant women with FHPP who underwent epidural analgesia for labour. Pregnant women with FHPP require multidisciplinary management involving an anaesthesiologist, a gynaecologist and a paediatrician. It is important to maintain normothermia, prevent hyperventilation, monitor electrolytes, avoid glucose infusions and medications that cause hypokalaemia, and administer potassium supplements when required. Locoregional techniques should be preferred over general anaesthesia. Early epidural analgesia reduces the risk of pain that could trigger an episode of FHPP. In the case of general anaesthesia, drugs that can cause malignant hyperthermia should be avoided, and short-acting non-depolarizing neuromuscular blockers with blockade-depth monitoring should be used.
家族性低钾周期性瘫痪（FHPP）是一种罕见的遗传性疾病，其特征是低钾血症相关的肌肉无力。发作可由药物、应激、代谢疾病、低温或感染引发。我们报告了一例 FHPP 孕妇接受硬膜外分娩镇痛的病例。FHPP 孕妇需要多学科管理，包括麻醉师、妇科医生和儿科医生。保持正常体温、预防过度通气、监测电解质、避免输注葡萄糖和引起低钾血症的药物以及按需给予钾补充剂非常重要。应优先选择局部区域技术而不是全身麻醉。早期硬膜外镇痛可降低可能引发 FHPP 发作的疼痛风险。对于全身麻醉，应避免可能引起恶性高热的药物，并使用具有阻滞深度监测的短效非去极化神经肌肉阻滞剂。
