PULMONARY ALVEOLAR MICROLITHIASIS IN PREGNANCY: A RARE CASE REPORT

被引:0
作者
Sethy, Hemanta Kumar [1 ]
Trilochan, Biswal Pradipta [1 ]
Panda, Geetanjali [1 ]
Misra, Milan [1 ]
机构
[1] SCB MCH, Dept Pulm Med, Cuttack, Orissa, India
来源
JOURNAL OF EVOLUTION OF MEDICAL AND DENTAL SCIENCES-JEMDS | 2015年 / 4卷 / 98期
关键词
PAM; Calcification; Familial; SLC34A2; Pregnancy; Cor Pulmonale;
D O I
10.14260/jemds/2015/2424
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Pulmonary Alveolar Microlithiasis (PAM) is a very rare diffuse lung disease, affecting both lungs by intra-alveolar deposition of calcium phosphates in form of microliths. There have been about 700 cases of PAM recorded in the medical literature. The etiopathogenesis of the disease is unknown. It is now thought to be an autosomal recessive disease caused by deletion of Na-Pi cotransporter (Npt2b) in alveolar epithelial cells due to defective DNA mutation in the gene SLC34A2. The disease is slowly progressive, usually detected in 3rd/4th decades of life and death is due to cardiorespiratory failure. The patients detected with PAM in pregnancy are exceptional. Very few cases were reported till date. We report a pregnant lady presenting with PAM delivering vaginally a healthy female child in spite of cardiomegally and severe PAH.
引用
收藏
页码:16387 / 16389
页数:3
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