Department of Pediatrics, 'Infermi' Hospital, Rimini, Italy. lucvirgo75 @ hotmail.com
Horm Res Paediatr. 2012;78(4):256-60. doi: 10.1159/000337253. Epub 2012 Aug 14.
Infantile hepatic hemangioma can be associated to consumptive hypothyroidism due to overexpression of type 3 deiodinase in the endothelium of vascular tumor, which catalyzes the conversion of T4 to reverse T3 (rT3) and of T3 to T2, both of which are biologically inactive. Here, we report an infant with a massive biopsy-proven infantile hepatic hemangioma who developed thyroid dysfunction without a typical biochemical profile consistent with severe consumptive hypothyroidism, despite the large tumor burden. Our patient was treated with propranolol that rapidly resolved both hepatic hemangioma and thyroid dysfunction. We propose propranolol as a first-line therapy of thyroid dysfunction associated with infantile hepatic hemangioma, in order to avoid interference with neurological development caused by hypothyroidism in the first months of life.
婴儿肝脏血管瘤可因血管肿瘤内皮细胞中 3 型脱碘酶的过度表达而导致消耗性甲状腺功能减退症，该酶可催化 T4 向 rT3（反式 T3）转化，以及 T3 向 T2 转化，这两种转化产物均无生物学活性。在此，我们报告了一例经活检证实的巨大婴儿肝脏血管瘤患儿，其发生甲状腺功能障碍，但无典型的生化特征与严重消耗性甲状腺功能减退症一致，尽管肿瘤负荷较大。我们的患者接受了普萘洛尔治疗，该治疗迅速缓解了肝血管瘤和甲状腺功能障碍。我们建议将普萘洛尔作为治疗与婴儿肝脏血管瘤相关的甲状腺功能障碍的一线药物，以避免因甲状腺功能减退症在生命的最初几个月对神经发育造成干扰。
