Long-term results of disodium etidronate treatment in pulmonary alveolar microlithiasis.
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.
Ozcelik
Ugur
U
Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 06100 Sihhiye, Ankara, Turkey. uozcelik@hacettepe.edu.tr
Yalcin
Ebru
E
Ariyurek
Macit
M
Ersoz
Deniz Dogru
DD
Cinel
Guzin
G
Gulhan
Bora
B
Kiper
Nural
N
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
D002130:Calcium Phosphates; D004164:Diphosphonates; C493557:SLC34A2 protein, human; D050614:Sodium-Phosphate Cotransporter Proteins, Type IIb; D012968:Etidronic Acid