Aggressive periodontitis associated with Kindler syndrome in a large Kindler syndrome pedigree

Turk J Pediatr. 2017;59(1):56-61. doi: 10.24953/turkjped.2017.01.009.

Abstract

Talo-Yıldırım T, Acun-Kaya F, Taşkesen M, Dündar S, Bozoğlan A, Tekin GG, Akdeniz S. Aggressive periodontitis associated with Kindler syndrome in a large Kindler syndrome pedigree. Turk J Pediatr 2017; 59: 56-61. Kindler syndrome (KS) is a rare genetic disorder. The clinical features include aggressive periodontal disease and severe desquamative gingivitis. Five individuals with KS were assessed by oral examination, radiographic analysis and periodontal measurements. All the patients' indexes were recorded prior to periodontal treatment and at the end of the 1th, 3th , 6th, 9th and 12th month respectively. All the patients had improvement of periodontal status and enhancement in index scores. The affected individuals were previously screened for FERMT1 mutations. KS patients' periodontal disease activity could be taken under control with regular follow-up.

Keywords: FERMT-1; growth retardation; periodontal disease; periodontal treatment.

MeSH terms

  • Adolescent
  • Aggressive Periodontitis / complications*
  • Blister / complications*
  • Child
  • Epidermolysis Bullosa / complications*
  • Female
  • Humans
  • Male
  • Membrane Proteins / genetics*
  • Mutation
  • Neoplasm Proteins / genetics*
  • Pedigree
  • Periodontal Diseases / complications*
  • Photosensitivity Disorders / complications*

Substances

  • FERMT1 protein, human
  • Membrane Proteins
  • Neoplasm Proteins

Supplementary concepts

  • Poikiloderma of Kindler