Kostmann disease--infantile genetic agranulocytosis: historical views and new aspects.
Zetterström. Rolf R
Key Findings
- Kostmann syndrome patients treated with colony stimulating factor still have low levels of cathelicidin LL‑37.
- LL‑37 is an antimicrobial peptide normally found in neutrophils and saliva.
- The deficiency of LL‑37 may explain why these patients continue to suffer from oral infections like chronic periodontitis.
Practical Outcomes
- For most biohackers this finding isn’t directly actionable because Kostmann syndrome is extremely rare. However, it highlights the role of LL‑37 in oral health, suggesting that supporting innate immunity—through good oral hygiene, possibly probiotic or peptide‑based mouth rinses—might help reduce mouth infections in people with compromised neutrophil function.
Summary
People with the rare Kostmann syndrome get a blood problem that is treated with a growth factor, but they still lack a natural antibiotic peptide called LL‑37 in their white blood cells and saliva, which may keep them prone to mouth infections.
Abstract
The results in the main reports on infantile genetic agranulocytosis or Kostmann Syndrome are summarized. New views on the pathogensis of the syndrome are given in a recent paper by Pütsep et al. Kostmann syndrome may cause early onset Group B streptococcal neonatal sepsis as reported in this issue of Acta Paediatrica (9). Patients with Kostmann Syndrome who are successfully treated for agranulocytosis with serum colony stimulating factor remain deficient in cathelin-LL-37, a peptide antibiotic, which is normally present in neutrophils and saliva. This deficiency may explain that patients who are successfully treated for agranulocytosis continue to suffer from oral infections such as chronic periodontitis.
Study Information
pubmed
2002