Self-Reported Executive Functioning in Young Adult Survivors of Childhood Bacterial Meningitis.

OBJECTIVE: This study investigated executive functions (EFs) in young adult survivors of childhood bacterial meningitis (BM). These skills are important for normal development, and their potential vulnerability in early years suggests that childhood BM could affect executive functions in the longer term. METHOD: The adult self-report Behavior Rating Inventory of Executive Function was administered to 474 young adult survivors of childhood BM who participated in the 20|30 Dutch Postmeningitis study. Average scores were compared to population-norm group scores. Subgroup scores were compared according to causative pathogen and age at onset. RESULTS: Young adult survivors of childhood BM scored lower on overall metacognition than the age-matched population norm group. Young adult survivors of childhood BM caused by Streptococcus pneumoniae, S. agalactiae, or Escherichia coli had lower scores than cases caused by Neisseria meningitidis. Survivors with age-at-onset below 12 months had a higher (worse) overall EF score than survivors with age-at-onset above 12 months. CONCLUSIONS: Young adult survivors of childhood BM experience difficulties in EF. However, most of the self-reported EF scores were within the norm. Future studies need to additionally assess EF in adult survivors of childhood BM using performance-based tests.

Genetic predisposition to infection in a case of atypical hemolytic uremic syndrome.

Most cases of hemolytic uremic syndrome (HUS) are caused by infection with enterohemorrhagic Escherichia coli (EHEC). Genetic defects causing uncontrolled complement activation are associated with the more severe atypical HUS (aHUS). Non-EHEC infections can trigger the disease, however, complement defects predisposing to such infections have not yet been studied. We describe a 2-month-old patient infected with different Gram-negative bacterial species resulting in aHUS. Serum analysis revealed slow complement activation kinetics. Rare variant R229C was found in complement inhibitor vitronectin. Recombinant mutated vitronectin showed enhanced complement inhibition in vitro and may have been a predisposing factor for infection. Our work indicates that genetic changes in aHUS can not only result in uncontrolled complement activation but also increase vulnerability to infections contributing to aHUS.