Case reports

In English literature, PAM has been reported from 51 countries where 40.6% are from Asia [2]. The mean age at presentation is 35 years [3]. There are two varieties described, familial and sporadic [4]. Autosomal recessive familial form has female preponderance. 50% of patients may be asymptomatic despite fluorid interstitial shadows on chest radiograph, or present with dyspnoea, prolonged cough, chest pain or haemoptysis [4]. In later stages, they may also present with features of corpulmonale [4]. Initially there may be no functional impairment, but later there may be restrictive type of defects [4]. Diagnosis is made by bronchoalveolar lavage or open lung biopsy. Typical sandstorm appearance in high resolution computed tomography scans also have a diagnostic value. The only curative treatment is bilateral sequential lung transplantation [4]. Other pharmacological agents which have no proven benefit include disodium etidronate 10 mg/ kg per day for 1 year [1]. Few cases of PAM during pregnancy have been published [5].