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[卟啉病和抗利尿激素分泌失调综合征]

[Porphyria and inappropriate antidiuretic hormone syndrome].

作者信息

López Montes A, Lorenzo I, Pérez Martínez J

机构信息

Servicio de Nefrología, Hospital General Universitario de Albacete, Albacete.

出版信息

Nefrologia. 2004;24 Suppl 3:85-8.

Abstract

We report the case of a 37-years-old woman with inappropriate antidiuretic hormone syndrome due to an attack of acute porphyria. The patient was admitted to our hospital for abdominal pain, sleepiness and pink urine. Family and personal history were normal. Seven days before the admission the patient had a laparoscopy operation for endometriosis in her left ovary. The patient had had two normal pregnancies. The physical examination was normal, the skin turgor was good and no edema was present, the blood pressure was 140/90 mmHg. Her serum sodium was 114 mEq/L, serum osmolality 243 mOsm/kg, urine sodium 146 mEq/L and urine osmolality 457 mOsm/kg. Values from laboratory examination revealed a normal peripheral haematogram, a normal kidney function, normal liver, adrenal and thyroid function. The urine tested for amino-levulinic acid, coproporphyrin and uroporphyrin was strongly positive. These findings are compatible with Porphyria Variegata or Coproporphyria Hereditary. A diagnosis of Porphyria acute with SIADH was made, and water fluid restriction, i.v. hypertonic saline infusion and furosemide to correct the hyponatremia was begun. In 1966, lesions of the median eminence of the hypothalamus and both hypothalamic -hypophyseal tracts were described in a patient with Porphyria acute intermittent and SIADH. It was suggested that SIADH occurred because of damage to these areas of the brain from excessive exposure to porphyrins.

摘要

我们报告了一例37岁女性因急性卟啉病发作导致抗利尿激素分泌失调综合征的病例。患者因腹痛、嗜睡和粉红色尿液入院。家族史和个人史均正常。入院前7天,患者因左侧卵巢子宫内膜异位症接受了腹腔镜手术。患者曾有两次正常妊娠。体格检查正常,皮肤弹性良好,无水肿,血压为140/90 mmHg。她的血清钠为114 mEq/L,血清渗透压为243 mOsm/kg,尿钠为146 mEq/L,尿渗透压为457 mOsm/kg。实验室检查结果显示外周血细胞计数正常、肾功能正常、肝脏、肾上腺和甲状腺功能正常。检测尿中氨基乙酰丙酸、粪卟啉和尿卟啉呈强阳性。这些发现与杂色卟啉病或遗传性粪卟啉病相符。诊断为急性卟啉病合并抗利尿激素分泌失调综合征,开始限制水摄入、静脉输注高渗盐水和使用呋塞米以纠正低钠血症。1966年,在一名患有急性间歇性卟啉病和抗利尿激素分泌失调综合征的患者中,描述了下丘脑正中隆起和两条下丘脑 - 垂体束的病变。有人提出,抗利尿激素分泌失调综合征是由于过度暴露于卟啉而导致这些脑区受损所致。

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