Retained primary teeth in STAT3 hyper-IgE syndrome: early intervention in childhood is essential.

ABSTRACT:

Background

STAT3 hyper-IgE syndrome (STAT3-HIES) is a rare primary immunodeficiency that clinically overlaps with atopic dermatitis. In addition to eczema, elevated serum-IgE, and recurrent infections, STAT3-HIES patients suffer from characteristic facies, midline defects, and retained primary teeth. To optimize dental management we assessed the development of dentition and the long-term outcomes of dental treatment in 13 molecularly defined STAT3-HIES patients using questionnaires, radiographs, and dental investigations.

Results

Primary tooth eruption was unremarkable in all STAT3-HIES patients evaluated. Primary tooth exfoliation and permanent tooth eruption was delayed in 83% of patients due to unresorbed tooth roots. A complex orthodontic treatment was needed for one pat

SUBMITTER: Meixner I 

PROVIDER: S-EPMC7488068 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature