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Increased risk of pyogenic liver abscess in children with Papillon-Lefevre syndrome
P Oğuzkurt1, F C Tanyel, N Büyükpamukçu
1Department of Pediatric Surgery, Hacettepe University School of Medicine, Ankara, Turkey.
Insights
Children with Papillon-Lefevre syndrome may face a higher risk of pyogenic liver abscess. This risk might stem from bacteremia originating in the gums and a potentially weakened immune system.
Area of Science:
- Hepatology
- Genetics
- Pediatrics
Background:
- Pyogenic liver abscess (PLA) is a serious infection.
- Papillon-Lefevre syndrome (PLS) is a rare genetic disorder characterized by periodontitis and palmoplantar hyperkeratosis.
Observation:
- Two pediatric cases of PLA were identified in patients with PLS.
- These patients had PLA alongside the typical manifestations of PLS.
Findings:
- Bacteremia originating from periodontal tissues is a potential source of PLA in PLS patients.
- An impaired immune response in PLS may contribute to the increased susceptibility to PLA.
Implications:
- This suggests a potential link between PLS and an increased risk of PLA in children.
- Early recognition and management of periodontal health may be crucial for preventing PLA in individuals with PLS.
- Further research is warranted to elucidate the mechanisms underlying this association.
Abstract:
Among 16 with pyogenic liver abscess, two were found to have Papillon-Lefevre syndrome. The two cases are reported herein. Bacteremia from involved periodontal tissues and a possible impaired immune response could indicate an increased risk of pyogenic liver abscess among children with Papillon-Lefevre syndrome.
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