Pulmonary manifestations of prolidase deficiency

Vered Nir, Anat Ilivitky, Fahed Hakim, Ronen Bar Yoseph, Michal Gur, Hana Mandel, Lea Bentur

Research output: Contribution to journalArticlepeer-review

14 Scopus citations


Background: Prolidase deficiency is a rare autosomal recessive disease, in which pulmonary manifestations have been sporadically reported. Aims: 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. Results: 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. Conclusions: 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.

Original languageEnglish
Pages (from-to)1229-1233
Number of pages5
JournalPediatric Pulmonology
Issue number11
StatePublished - 1 Nov 2016
Externally publishedYes

Bibliographical note

Publisher Copyright:
© 2016 Wiley Periodicals, Inc.


  • interstitial lung disease (ILD)
  • prolidase deficiency
  • pulmonary cysts


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