A rare cause of acute abdominal pain in a patient with Primary ciliary dyskinesia with situs inversus totalis


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Ciki K., Turer Ö., Hizal M., Tugcu G. D. , EMİRALİOĞLU N., Yalcin E., ...More

TURKISH JOURNAL OF PEDIATRICS, vol.62, no.1, pp.156-159, 2020 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 62 Issue: 1
  • Publication Date: 2020
  • Doi Number: 10.24953/turkjped.2020.01.024
  • Journal Name: TURKISH JOURNAL OF PEDIATRICS
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.156-159
  • Keywords: Kartagener syndrome, primary ciliary dyskinesia, spleen torsion
  • Hacettepe University Affiliated: Yes

Abstract

Primary ciliary dyskinesia (PCD) is a rare, genetic disease characterized by ciliary dysfunction. Patients may present with respiratory distress during neonatal period; chronic sinopulmonary disease, bronchiectasis, recurrent otitis media, sinusitis and infertility in later periods. About 50% of PCD patients have situs inversus totalis and 6-12% have situs ambiguous known as heterotaxy syndromes. Herein, we present a case of PCD and accompanying situs inversus who had acute abdominal pain and was diagnosed with torsion of one of the multiple spleens. Evaluation of acute abdominal pain in these patients has great importance since the internal organs are not at their typical locations.