Pulmonary involvement in a child with ligneous conjunctivitis and homozygous type I plasminogen deficiency

ÖZÇELİK H. U., Akcoren Z., Anadol D., Kiper N., Orhon M., Gocmen A., ...More

PEDIATRIC PULMONOLOGY, vol.32, no.2, pp.179-183, 2001 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 32 Issue: 2
  • Publication Date: 2001
  • Doi Number: 10.1002/ppul.1105
  • Journal Name: PEDIATRIC PULMONOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.179-183
  • Keywords: ligneous conjunctivitis, alveolitis, plasminogen deficiency, tracheitis, lower respiratory tract infection, OBSTRUCTION, MUTATIONS, GENE
  • Hacettepe University Affiliated: Yes

Abstract

Ligneous conjunctivitis (LC) is a rare disorder characterized by a chronic course of recurrent membranous lesions at conjunctivae. Pseudomembranes of other mucous membranes have been reported in patients with LO, but to the best of our knowledge, no case with alveolar involvement has been described. Here, we report a 2.5-year-old girt with LC who had tracheoaveolar involvement and homozygous type I plasminogen deficiency. Tracheal involvement was diagnosed by bronchoscopic biopsy and alveolar involvement with postmortem biopsy. She was shown to be homozygous for frameshift mutation in plasminogen exon 14 (Gly565ins-G) with molecular genetic examination of DNA which was obtained from parafin embedded postmortem lung tissue. Ligneous inflammation of the upper and lower respiratory tract must be considered in children with LC and recurrent respiratory tract problems. (C) 2001 Wiley-Liss, Inc.