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颅咽管瘤致抗利尿激素分泌不当综合征(SIADH)及肾上腺功能不全:一例报告

Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by rathke's cleft cyst: a case report.

作者信息

Iwai H, Ohno Y, Hoshiro M, Fujimoto M, Nishimura A, Kishitani Y, Aoki N

机构信息

Second Department of Medicine, Kinki University School of Medicine, Osaka-Sayama, Japan.

出版信息

Endocr J. 2000 Aug;47(4):393-9. doi: 10.1507/endocrj.47.393.

Abstract

We report a case of a seventy-year-old woman with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by Rathke's cleft cyst. She experienced nausea, vomiting, diarrhea, and headache and disturbance of consciousness induced by hyponatremia at a serum sodium level of 100 mEq/l. In spite of severe hyponatremia, urinary sodium excretion was not suppressed and serum osmolality (270 mOsm/kg) was lower than urine osmolality (304 mOsm/kg), and arginine vasopressin (AVP) remained within normal range. SIADH was diagnosed because she was free from other diseases known to cause hyponatremia such as dehydration, cardiac dysfunction, liver dysfunction, renal dysfunction, hypothyroidism, and adrenal insufficiency. Cranial computed tomographic (CT) scan and cranial magnetic resonance (MR) imaging showed a cystic lesion of approximately 2 cm in diameter in the pituitary gland. These images suggested that the cystic lesion was a Rathke's cleft cyst, which was the cause of SIADH. Water restriction therapy normalized her serum sodium concentration and improved her symptoms. After one year, she suffered from general fatigue, appetite loss, fever, and body weight loss (5 kg/2 months). She had neither hypotension nor hypoglycemia, but her serum sodium level was low and serum cortisol, ACTH, and urine free cortisol were very low. Therefore, secondary adrenal insufficiency was suspected and diagnosed by stimulation tests. After start of hydrocortisone replacement therapy (10 mg/day), her symptoms disappeared. In conclusion, Rathke's cleft cyst should be kept in mind as a potential cause in a patient with SIADH, hypopituitarism, and/or adrenal insufficiency.

摘要

我们报告一例70岁女性,因拉克氏裂囊肿导致抗利尿激素分泌不当综合征(SIADH)和肾上腺功能不全。她出现恶心、呕吐、腹泻、头痛,血清钠水平为100 mEq/l时因低钠血症导致意识障碍。尽管存在严重低钠血症,但尿钠排泄未受抑制,血清渗透压(270 mOsm/kg)低于尿渗透压(304 mOsm/kg),且精氨酸加压素(AVP)仍在正常范围内。由于她没有已知可导致低钠血症的其他疾病,如脱水、心脏功能障碍、肝功能障碍、肾功能障碍、甲状腺功能减退和肾上腺功能不全,故诊断为SIADH。头颅计算机断层扫描(CT)和头颅磁共振成像(MR)显示垂体有一个直径约2 cm的囊性病变。这些图像提示该囊性病变为拉克氏裂囊肿,是SIADH的病因。限水疗法使她的血清钠浓度恢复正常并改善了症状。一年后,她出现全身乏力、食欲减退、发热和体重减轻(2个月内减轻5 kg)。她既无低血压也无低血糖,但血清钠水平低,血清皮质醇、促肾上腺皮质激素(ACTH)和尿游离皮质醇极低。因此,怀疑为继发性肾上腺功能不全,并通过刺激试验确诊。开始氢化可的松替代治疗(10 mg/天)后,她的症状消失。总之,对于患有SIADH、垂体功能减退和/或肾上腺功能不全的患者,应将拉克氏裂囊肿视为潜在病因。

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