An atypical pulmonary fibrosis is associated with co-inheritance of mutations in the calcium binding protein genes and .

Background: Pulmonary fibrosis is one of the leading indications for lung transplantation. The disease, which is of unknown aetiology, can be progressive, resulting in distortion of the extracellular matrix (ECM), inflammation, fibrosis and eventual death.

Methods: 13 patients born to consanguineous parents from two unrelated families presenting with interstitial lung disease were clinically investigated.

Pancreaticopleural fistula: an overlooked entity.

Pleuropulmonary complications of pancreatitis are well known. Less commonly encountered is pancreaticopleural fistula. We describe the case of a 15-year-old boy with a presumed episode of pancreatitis, complicated by pseudocyst and development of a pancreaticopleural fistula.