Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
J Korean Med Sci. 2020 Jun 8;35(22):e180. doi: 10.3346/jkms.2020.35.e180.
Consumptive hypothyroidism is a rare paraneoplastic syndrome characterized by excessive inactivation of the thyroid hormones due to increased type 3 iodothyronine deiodinase activity of tumors. We report the case of severe consumptive hypothyroidism in a 1-month-old boy with infantile hepatic hemangiomas who presented with cardiac failure and cholestasis. Diffuse infiltration of hepatic hemangiomas was detected on abdominal imaging studies, and thyroid function screening test revealed severe hypothyroidism, which necessitated the administration of higher-than-usual doses of levothyroxine for the normalization of thyroid function. The patient was successfully treated with propranolol, prednisolone, and levothyroxine, and he showed normal thyroid function at 3 months of age and normal neurodevelopment at 9 months of age. This case highlights the importance of early recognition and prompt management of consumptive hypothyroidism in patients with infantile hepatic hemangiomas.
消耗性甲状腺功能减退症是一种罕见的副肿瘤综合征,其特征是由于肿瘤中 3 型碘甲状腺原氨酸脱碘酶活性增加导致甲状腺激素过度失活。我们报告了一例 1 个月大的婴儿患婴儿性肝血管瘤,表现为心力衰竭和胆汁淤积性消耗性甲状腺功能减退症的病例。腹部影像学研究发现弥漫性肝血管瘤浸润,甲状腺功能筛查试验显示严重的甲状腺功能减退症,需要给予高于常规剂量的左甲状腺素以恢复甲状腺功能正常。该患者使用普萘洛尔、泼尼松龙和左甲状腺素成功治疗,3 个月时甲状腺功能正常,9 个月时神经发育正常。本例强调了在婴儿性肝血管瘤患者中早期识别和及时治疗消耗性甲状腺功能减退症的重要性。