title: Pulmonary manifestations of prolidase deficiency.
abstract: Prolidase deficiency is a rare autosomal recessive disease, in which pulmonary manifestations have been sporadically reported.
  
  
  
  We have encountered two patients who presented with severe pulmonary cystic lesions leading to respiratory failure. This led us to retrospectively evaluate pulmonary involvement in patients with prolidase deficiency treated in our hospital.
  
  
  
  Of 21 patients (including the 2 mentioned above), 12 had a history of recurrent pulmonary infections and 10 were diagnosed as having chronic lung disease. Of seven chest CT scans performed, four patients had subpleural cysts, two patients had bronchiectatic changes, and one had diffused ground glass attenuation and minor linear atelectasis. Three patients died, with all deaths being attributed to respiratory insufficiency.
  
  
  
  Prolidase deficiency is frequently associated with various pulmonary manifestations, including extensive cystic changes that may be life endangering. The differential diagnosis of bilateral cystic changes should include prolidase deficiency, and pulmonary evaluation should be performed in patients with prolidase deficiency. Pediatr Pulmonol. 2016;51:1229-1233. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Division of Pediatric Pulmonology, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Vered
authors.initials: V
authors.lastname: Nir
authors.affiliation: Pediatric Radiology, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Anat
authors.initials: A
authors.lastname: Ilivitky
authors.affiliation: Division of Pediatric Pulmonology, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel.|Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
authors.forename: Fahed
authors.initials: F
authors.lastname: Hakim
authors.affiliation: Division of Pediatric Pulmonology, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Ronen Bar
authors.initials: RB
authors.lastname: Yoseph
authors.affiliation: Division of Pediatric Pulmonology, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Michal
authors.initials: M
authors.lastname: Gur
authors.affiliation: Hana Mandel, Metabolic Unit, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Hana
authors.initials: H
authors.lastname: Mandel
authors.affiliation: Division of Pediatric Pulmonology, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel. l_bentur@rambam.health.gov.il.|Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. l_bentur@rambam.health.gov.il.
authors.forename: Lea
authors.initials: L
authors.lastname: Bentur
mesh_terms: D000328:Adult; D001987:Bronchiectasis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D056732:Prolidase Deficiency / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D012131:Respiratory Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
keywords: interstitial lung disease (ILD); prolidase deficiency; pulmonary cysts