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 v
erified and 62 suspected episodes of sepsis. These accounted for half
of all infections recorded. Forty percent of bacteremias were caused b
y coagulase-negative staphylococci, 16% were caused by Staphylococcus
aureus, 17% were streptococcal, and 24% were caused by Gram-negative b
acteria. One infant died of pleural empyema, but otherwise the outcome
of infections was good. The use of central venous lines tended to inc
rease the rate of staphylococcal bacteremias but had no significant ef
fect on the overall incidence of infections. Prophylactic use of antib
iotics did not reduce the incidence of septic or other infections. Inf
ants with CNF had low levels of serum IgG, but prophylactic immunoglob
ulin infusions (0.5 - 1.0 g once or twice a week) did not reduce the f
requency of infections, probably because the infused Ige was quickly l
ost into the urine. The results indicate that infants with CNF often s
uffer from septic infections associated with the invasive treatment mo
dalities. Parenteral antibiotics covering the hospital strains of bact
eria (especially staphylococci) should be started without delay when a
nephrotic patient is not doing well.