Lee Mi Ji, Kim Byung Young, Ma Jae Sook, Choi Young Earl, Kim Young Ok, Cho Hwa Jin, Kim Chan Jong
Department of Pediatrics, Chonnam National University Hospital, Gwangju, Korea.
Korean J Pediatr. 2016 Nov;59(Suppl 1):S112-S115. doi: 10.3345/kjp.2016.59.11.S112. Epub 2016 Nov 30.
Noonan syndrome is an autosomal dominant, multisystem disorder. Autoimmune thyroiditis with hypothyroidism is an infrequent feature in patients with Noonan syndrome. A 16-year-old boy was admitted because of chest discomfort and dyspnea; an echocardiogram revealed pericardial effusion. Additional investigations led to a diagnosis of severe hypothyroidism due to Hashimoto thyroiditis. The patient was treated with L-thyroxine at 0.15 mg daily. However, during admission, he developed symptoms of cardiac tamponade. Closed pericardiostomy was performed, after which the patient's chest discomfort improved, and his vital signs stabilized. Herein, we report a case of an adolescent with Noonan syndrome, who was diagnosed with Hashimoto thyroiditis with an unusual presentation of cardiac tamponade.
努南综合征是一种常染色体显性多系统疾病。伴有甲状腺功能减退的自身免疫性甲状腺炎在努南综合征患者中并不常见。一名16岁男孩因胸部不适和呼吸困难入院；超声心动图显示心包积液。进一步检查诊断为桥本甲状腺炎导致的严重甲状腺功能减退。患者接受每天0.15毫克左甲状腺素治疗。然而，住院期间，他出现了心脏压塞症状。进行了心包闭式造口术，术后患者胸部不适改善，生命体征稳定。在此，我们报告一例患有努南综合征的青少年病例，该患者被诊断为桥本甲状腺炎并伴有心脏压塞这一不寻常表现。
