title: Long-term results of disodium etidronate treatment in pulmonary alveolar microlithiasis.
abstract: 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.
authors.affiliation: Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 06100 Sihhiye, Ankara, Turkey. uozcelik@hacettepe.edu.tr
authors.forename: Ugur
authors.initials: U
authors.lastname: Ozcelik
authors.forename: Ebru
authors.initials: E
authors.lastname: Yalcin
authors.forename: Macit
authors.initials: M
authors.lastname: Ariyurek
authors.forename: Deniz Dogru
authors.initials: DD
authors.lastname: Ersoz
authors.forename: Guzin
authors.initials: G
authors.lastname: Cinel
authors.forename: Bora
authors.initials: B
authors.lastname: Gulhan
authors.forename: Nural
authors.initials: N
authors.lastname: Kiper
mesh_terms: D002130:Calcium Phosphates / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D004164:Diphosphonates / Q000627:therapeutic use*; D012968:Etidronic Acid / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D020347:Lithiasis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D011650:Pulmonary Alveoli; D011859:Radiography; D050614:Sodium-Phosphate Cotransporter Proteins, Type IIb / Q000235:genetics
chemical_list: D002130:Calcium Phosphates; D004164:Diphosphonates; C493557:SLC34A2 protein, human; D050614:Sodium-Phosphate Cotransporter Proteins, Type IIb; D012968:Etidronic Acid