Kostmann disease--infantile genetic agranulocytosis: historical views and new aspects.

UNLABELLED: 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).

CONCLUSION

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.