The First Department of Medicine, Wakayama Medical University, Wakayama, Japan.
Thyroid. 2012 Jul;22(7):661-79. doi: 10.1089/thy.2011.0334. Epub 2012 Jun 12.
Thyroid storm (TS) is life threatening. Its incidence is poorly defined, few series are available, and population-based diagnostic criteria have not been established. We surveyed TS in Japan, defined its characteristics, and formulated diagnostic criteria, FINAL-CRITERIA1 and FINAL-CRITERIA2, for two grades of TS, TS1, and TS2 respectively.
We first developed diagnostic criteria based on 99 patients in the literature and 7 of our patients (LIT-CRITERIA1 for TS1 and LIT-CRITERIA2 for TS2). Thyrotoxicosis was a prerequisite for TS1 and TS2 as well as for combinations of the central nervous system manifestations, fever, tachycardia, congestive heart failure (CHF), and gastrointestinal (GI)/hepatic disturbances. We then conducted initial and follow-up surveys from 2004 through 2008, targeting all hospitals in Japan, with an eight-layered random extraction selection process to obtain and verify information on patients who met LIT-CRITERIA1 and LIT-CRITERIA2.
We identified 282 patients with TS1 and 74 patients with TS2. Based on these data and information from the Ministry of Health, Labor, and Welfare of Japan, we estimated the incidence of TS in hospitalized patients in Japan to be 0.20 per 100,000 per year. Serum-free thyroxine and free triiodothyroine concentrations were similar among patients with TS in the literature, Japanese patients with TS1 or TS2, and a group of patients with thyrotoxicosis without TS (Tox-NoTS). The mortality rate was 11.0% in TS1, 9.5% in TS2, and 0% in Tox-NoTS patients. Multiple organ failure was the most common cause of death in TS1 and TS2, followed by CHF, respiratory failure, arrhythmia, disseminated intravascular coagulation, GI perforation, hypoxic brain syndrome, and sepsis. Glasgow Coma Scale results and blood urea nitrogen (BUN) were associated with irreversible damages in 22 survivors. The only change in our final diagnostic criteria for TS as compared with our initial criteria related to serum bilirubin concentration >3 mg/dL.
TS is still a life-threatening disorder with more than 10% mortality in Japan. We present newly formulated diagnostic criteria for TS and clarify its clinical features, prognosis, and incidence based on nationwide surveys in Japan. This information will help diagnose TS and in understanding the factors contributing to mortality and irreversible complications.
甲状腺危象（TS）是危及生命的疾病。其发病率定义不佳，很少有系列报道，也没有建立基于人群的诊断标准。我们调查了日本的 TS，定义了其特征，并制定了诊断标准 FINAL-CRITERIA1 和 FINAL-CRITERIA2，分别用于 TS1 和 TS2 两个等级。
我们首先根据文献中的 99 例患者和我们的 7 例患者制定了诊断标准（LIT-CRITERIA1 用于 TS1，LIT-CRITERIA2 用于 TS2）。甲状腺毒症是 TS1 和 TS2 以及中枢神经系统表现、发热、心动过速、充血性心力衰竭（CHF）和胃肠道（GI）/肝紊乱的前提条件。然后，我们从 2004 年到 2008 年进行了初步和随访调查，针对日本的所有医院，采用八层随机抽样选择过程，以获取和验证符合 LIT-CRITERIA1 和 LIT-CRITERIA2 的患者信息。
我们确定了 282 例 TS1 患者和 74 例 TS2 患者。根据这些数据和日本厚生劳动省的信息，我们估计日本住院患者 TS 的发病率为每年每 100,000 人 0.20 例。文献中的 TS 患者、日本 TS1 或 TS2 患者以及一组无 TS 的甲状腺毒症患者（Tox-NoTS）的血清游离甲状腺素和游离三碘甲状腺原氨酸浓度相似。TS1 患者的死亡率为 11.0%，TS2 患者的死亡率为 9.5%，Tox-NoTS 患者的死亡率为 0%。多器官衰竭是 TS1 和 TS2 患者死亡的最常见原因，其次是 CHF、呼吸衰竭、心律失常、弥漫性血管内凝血、GI 穿孔、缺氧性脑综合征和败血症。格拉斯哥昏迷量表（Glasgow Coma Scale，GCS）结果和血尿素氮（BUN）与 22 名幸存者的不可逆转损伤有关。与我们最初的标准相比，我们对 TS 最终诊断标准的唯一改变与血清胆红素浓度>3mg/dL 有关。
TS 仍然是一种危及生命的疾病，在日本的死亡率超过 10%。我们提出了新的 TS 诊断标准，并根据日本的全国性调查阐明了其临床特征、预后和发病率。这些信息将有助于诊断 TS，并了解导致死亡率和不可逆转并发症的因素。
