Internal Medicine Department and Research Unit for Study of Hydromineral Metabolism, Hôpital Universitaire Erasme, Bruxelles, Belgium.
Nephron Clin Pract. 2012;120(3):c168-72. doi: 10.1159/000338539. Epub 2012 Jun 19.
Nephrogenic syndrome of inappropriate antidiuresis (NSIAD) is a disorder of water balance linked to gain-of-function mutation of arginine vasopressin receptor type 2 (AVPR2) resulting in free water reabsorption and episodes of hyponatremia.
To review the long-term treatment of NSIAD.
In the first part of this paper, we report 3 cases of male patients presenting with hyponatremia due to NSIAD. The second part consists of a comprehensive review of all published case reports.
In our experience, long-term fluid restriction (FR) and treatment with low doses of urea are efficient and well tolerated. Episodic intake of urea seems sufficient in some patients. Treatment data were available for 13 of the 16 hyponatremic patients reported in the literature. Each of these 13 patients had regulated fluid intake. Six of the patients received urea with no reported failure to correct hyponatremia and 5 received NaCl supplementation with varying efficacy. The AVPR2 antagonists tolvaptan and satavaptan (prescribed before the diagnosis of NSIAD was made) showed no efficacy in 1 patient.
NSIAD is quite easy to treat with FR and urea in adults as well as in children, with good compliance and efficacy. Of note, FR is well tolerated, suggesting that NSIAD may differ from other causes of syndrome of inappropriate antidiuretic hormone secretion by reduction of thirst intensity due to lower levels of AVP (which stimulates thirst). In eventual refractory cases, furosemide (associated with NaCl supplementation) would represent a valuable therapeutic option by analogy of its efficacy in syndrome of inappropriate antidiuretic hormone secretion.
肾源性尿崩症（NSIAD）是一种水盐平衡紊乱，与精氨酸加压素受体 2（AVPR2）功能获得性突变有关，导致自由水重吸收和低钠血症发作。
回顾 NSIAD 的长期治疗。
本文第一部分报告了 3 例因 NSIAD 导致低钠血症的男性患者。第二部分对所有已发表的病例报告进行了全面回顾。
根据我们的经验，长期限制液体摄入（FR）和低剂量尿素治疗既有效又耐受良好。在某些患者中，间歇性摄入尿素似乎就足够了。文献中报道的 16 例低钠血症患者中有 13 例可获得治疗数据。这些患者均接受了液体摄入调节。其中 6 例患者接受了尿素治疗，没有报道低钠血症未得到纠正，5 例患者接受了不同疗效的氯化钠补充治疗。AVPR2 拮抗剂托伐普坦和沙他伐坦（在诊断为 NSIAD 之前开的）在 1 例患者中没有显示出疗效。
FR 和尿素治疗成人和儿童的 NSIAD 非常简单，具有良好的顺应性和疗效。值得注意的是，FR 耐受性良好，这表明 NSIAD 可能与其他抗利尿激素分泌不当综合征不同，因为 AVP（刺激口渴）水平较低会降低口渴强度。在最终难治的情况下，呋塞米（与氯化钠补充联合使用）将是一种有价值的治疗选择，因为它对抗利尿激素分泌不当综合征有效。
