Congenital tuftsin deficiency

Ann N Y Acad Sci. 1983:419:214-9. doi: 10.1111/j.1749-6632.1983.tb37106.x.

Abstract

The serum tuftsin activity in four different pediatric groups has been studied: (a) 20 premature infants, (b) 20 full-term infants, (c) 20 normal children (controls), and (d) 5 patients with recurrent and severe infections of the respiratory system, skin, and lymph nodes. The normal children as well as the premature and full-term infants had normal tuftsin activity. Tuftsin activity in the serum of the 5 patients in group (d) was absent. Cellular and humoral immunity in these patients was normal. All patients have been shown to have a mutant peptide that is strongly inhibitory to the normal tetrapeptide tuftsin. The clinical response of these patients to gamma-globulin was excellent.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Infant, Newborn*
  • Infant, Premature*
  • Infections / etiology
  • Lymph Nodes
  • Male
  • Respiratory Tract Infections / etiology
  • Skin Diseases, Infectious
  • Tuftsin / antagonists & inhibitors
  • Tuftsin / blood
  • Tuftsin / deficiency*
  • gamma-Globulins / therapeutic use

Substances

  • gamma-Globulins
  • Tuftsin