Nephrogenic syndrome of inappropriate antidiuresis (NSIAD), first described in 2005, is a rare genetic X-linked disease, presenting with hyponatremia, hyposmolarity, euvolemia, inappropriately concentrated urine, increased natriuresis, and undetectable or very low arginine-vasopressine (AVP) circulating levels. It can occur in neonates, infants, or later in life. NSIAD must be early recognized and treated to prevent severe hyponatremia, which can show a dangerous impact on neonatal outcome. In fact, it potentially leads to death or, in case of survival, neurologic sequelae. This review is an update of NSIAD 12 years after the first description, focusing on reported cases of neonatal and infantile onset. The different molecular patterns affecting the AVP receptor 2 (V2R) and determining its gain of function are reported in detail; moreover, we also provide a comparison between the different triggers involved in the development of hyponatremia, the evolution of the symptoms, and modality and efficacy of the different treatments available.

中文翻译：

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侧重于不适当抗利尿剂的肾病综合征的新生儿和婴儿发作：12年后。

不适当抗利尿剂的肾源性综合征（NSIAD）于2005年首次被描述，是一种罕见的遗传X连锁疾病，表现为低钠血症，低渗性，尿毒症，尿液浓度不适当，利尿增多以及精氨酸-血管加压素（AVP）循环不足或非常低水平。它可以发生在新生儿，婴儿或以后的生活中。必须早期识别NSIAD并对其进行治疗，以防止严重的低钠血症，严重的低钠血症可能对新生儿结局产生危险影响。实际上，它有可能导致死亡，或者在生存时会导致神经系统后遗症。这篇综述是首次描述后12年对NSIAD的更新，重点是新生儿和婴儿发作的报道病例。详细报道了影响AVP受体2（V2R）并确定其功能获得的不同分子模式。此外，