T Lymphocytes and Natural Killer Cells in Pediatric Patients with Methylmalonic or Propionic Acidemia

Abstract

strong association has been postulated between organic acidemia and immunodeficiencies Compared to normal subjects, patients with organic acidemia have higher morbidity and mortality during infections. Good knowledge of probable defects in immune system can lead to administration of suitable broad spectrum antibiotics to reduce morbidity and mortality. We sought to assess T-lymphocytes and natural killer cells in pediatric patients with methylmalonic (MMA) or propionic acidemia (PA). For this purpose, a cross sectional study was conducted at the genetic unit of Children's hospital, Faculty of Medicine, Ain-Shams university where patients with diagnosed methylmalonic or propionic acidemia were enrolled consecutively. A detailed medical history was taken including, present, developmental and family history, history of recurrent infection, site and duration of infection, history of consanguinity or sibling deaths. All patients underwent general and local examination. Laboratory evaluation was done including: a complete blood picture and assessment of T-lymphocyte subsets (CD3+, CD4+, CD8+) and NK cells (CD56+) by flow-cytometry. Also, candida intradermal skin test was done in non lym