Infections in infants with congenital nephrosis of the Finnish type

The incidence and type of infections were retrospectively analyzed in 21 infants with congenital nephrosis of the Finnish type (CNF). During the median follow-up time of 1.1 years the infants suffered from 63 verified and 62 suspected episodes of sepsis. These accounted for half of all infections recorded. Forty percent of bacteremias were caused by coagulase-negative staphylococci, 16% were caused by Staphylococcus aureus, 17% were streptococcal, and 24% were caused by Gram-negative bacteria. One infant died of pleural empyema, but otherwise the outcome of infections was good. The use of central venous lines tended to increase the rate of staphylococcal bacteremias but had no significant effect on the overall incidence of infections. Prophylactic use of antibiotics did not reduce the incidence of septic or other infections. Infants with CNF had low levels of serum IgG, but prophylactic immunoglobulin infusions (0.5 - 1.0 g once or twice a week) did not reduce the frequency of infections, probably because the infused Ige was quickly lost into the urine. The results indicate that infants with CNF often suffer from septic infections associated with the invasive treatment modalities. Parenteral antibiotics covering the hospital strains of bacteria (especially staphylococci) should be started without delay when a nephrotic patient is not doing well.