Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 06100 Sihhiye, Ankara, Turkey.
Pediatr Pulmonol. 2010 May;45(5):514-7. doi: 10.1002/ppul.21209.
Pulmonary alveolar microlithiasis (PAM) is a rare disease with alveolar microliths mainly composed of calcium phosphate. The gene responsible for the disease is SLC34A2, which encodes a type-IIb sodium phosphate cotransporter, has been described recently. Treatment of this disease is not clearly defined. Disodium etidronate is a member of bisphonates and it has been administered in these patients due to its inhibitory effect on the precipitation of hydroxyapatite microcrystals. Here, clinical and radiological improvement of two patients with PAM who were treated with disodium etidronate for 9 and 11 years, respectively, are presented. The pathogenetic mechanism of this treatment on the genetic basis of disease is discussed.
肺肺泡微结石症（PAM）是一种罕见的疾病，其肺泡微结石主要由磷酸钙组成。最近描述了导致该疾病的基因是 SLC34A2，它编码一种 IIb 型钠磷酸盐协同转运蛋白。这种疾病的治疗方法尚未明确。依替膦酸二钠是双膦酸盐的一种成员，由于其对羟磷灰石微晶沉淀的抑制作用，已在这些患者中使用。本文介绍了两名分别接受依替膦酸二钠治疗 9 年和 11 年的 PAM 患者的临床和影像学改善情况。讨论了这种治疗方法在疾病遗传基础上的发病机制。
