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原发性和继发性黏液性水肿昏迷,表现不典型且肌酸激酶极度升高。

Myxedema coma of both primary and secondary origin, with non-classic presentation and extremely elevated creatine kinase.

作者信息

Benvenga S, Squadrito S, Saporito F, Cimino A, Arrigo F, Trimarchi F

机构信息

Cattedra e Divisione di Endocrinologia, University of Messina, School of Medicine, Italy.

出版信息

Horm Metab Res. 2000 Sep;32(9):364-6. doi: 10.1055/s-2007-978654.

DOI:10.1055/s-2007-978654
PMID:11014385
Abstract

Myxedema coma is a rare, often fatal endocrine emergency that concerns elderly patients with long-standing primary hypothyroidism; myxedema coma of central origin is exceedingly rare. Here, we report a 37-year-old woman in whom classical symptoms of hypothyroidism had been absent. Six years earlier, she had severe obstetric hemorrhage and, shortly after, two subsequent episodes of pericardial effusion. On the day of admission, pericardiocentesis was performed for the third episode of pericardial effusion. Because of the subsequent grave arrhythmias and unconsciousness, she was transferred to our ICU. Prior to the endocrine consultation, a silent myocardial infarction had been suspected, based on the extremely high serum levels of creatine kinase (CK) and isoenzyme CK-MB. However, based on thyroid sonography, pituitary computed tomography, elevated titers of antithyroid antibodies and pituitary stimulation tests, the final diagnosis was myxedema coma of dual origin: an atrophic variant of Hashimoto's thyroiditis and post-necrotic pituitary atrophy (Sheehan syndrome). Substitutive therapy caused a prompt clinical amelioration and normalization of CK levels. Our patient is the first case of myxedema coma of double etiology, and illustrates how its presentation deviates markedly from the one endocrinologists and physicians at ICU are prepared to encounter. In addition, cardiac problems as those of our patient should not discourage from substitutive treatment (using L-thyroxine and the gastrointestinal route of absorption), if the age is relatively low.

摘要

黏液性水肿昏迷是一种罕见且常致命的内分泌急症,多见于患有长期原发性甲状腺功能减退症的老年患者;中枢性黏液性水肿昏迷极为罕见。在此,我们报告一名37岁女性,她并无典型的甲状腺功能减退症状。六年前,她曾发生严重产科出血,此后不久又出现两次心包积液。入院当天,因第三次心包积液进行了心包穿刺术。由于随后出现严重心律失常和意识丧失,她被转入我们的重症监护病房。在内分泌会诊前,基于极高的血清肌酸激酶(CK)和同工酶CK-MB水平,怀疑发生了无症状心肌梗死。然而,基于甲状腺超声、垂体计算机断层扫描、抗甲状腺抗体滴度升高以及垂体刺激试验,最终诊断为双源性黏液性水肿昏迷:桥本甲状腺炎的萎缩变异型和坏死性垂体萎缩(席汉综合征)。替代治疗使临床症状迅速改善,CK水平恢复正常。我们的患者是首例双病因黏液性水肿昏迷病例,说明了其临床表现与内分泌科医生和重症监护病房医生所预期遇到的情况有明显差异。此外,如果患者年龄相对较轻,像我们患者这样的心脏问题不应妨碍进行替代治疗(使用左甲状腺素并采用胃肠道吸收途径)。

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