Recent Zika virus (ZIKV) outbreaks have been associated with an increased incidence of neonatal microcephaly. Subsequently, tropism for the brain was established in human fetal brain tissue. We present the first congenital ZIKV infection in the United States, confirmed by high ZIKV immunoglobulin M antibody titers in serum and cerebrospinal fluid. The phenotypic characteristics of the patient fall within fetal brain disruption sequence, suggesting impaired brain development in the second half of gestation. Brain imaging revealed an almost agyric brain with diffuse parenchymal calcifications, hydrocephalus ex vacuo, and cerebellar hypoplasia. Ophthalmologic examination revealed macular pigment stippling and optic nerve atrophy. Liver, lungs, heart, and bone marrow were not affected. The patient had progressive neurologic deterioration in the first month of life. The discovery of ZIKV infection in human fetal brain tissue along with serologic confirmation proves the vertical transmission of ZIKV. Therefore, ZIKV has joined the group of congenital infections.

In a Hawaii Hereditary Anemia Screening Project, 4,984 participants were tested for glucose-6-phosphate dehydrogenase (G6PD) deficiency by a filter paper blood spot fluorescence test. Abnormal samples and suspected heterozygotes were checked by quantitative G6PD assay (normal 4.5 to 14 units/g Hb). G6PD was deficient (< 1.5 units/g Hb) in 188 of 2,155 males; 7 other males had low activity (1.5 to 2.8 units/g Hb). The gene frequency, estimated from males after excluding referred and related cases, was 0.037 for Chinese, 0.134 for Filipinos, and 0.203 for Laotians. Among 2,829 females tested, family data showed 111 females were obliged to be at least heterozygous, regardless of G6PD activity, and 43 others had low G6PD activity. Most heterozygotes probably remained undetected by G6PD screening. In 28 females, activity was under 10%; in another 9 females, activity was < 1.5 units/g Hb. Since only 25 homozygotes would be predicted, this apparent excess of females with deficient activity could be due to unequal X-inactivation in some heterozygotes. DNA analysis by polymerase chain reaction amplification and special analytic procedures revealed 10 different missense mutations in 75 males. The nucleotide 835 A-->T and 1360 C-->T transitions were first detected in this Hawaiian Project; we found that the nucleotide 1360 mutation was the most common cause of G6PD deficiency in Filipinos. This is the first report of G6PD screening and analysis of molecular G6PD mutations in Filipino and Laotian populations.

HIV-1-infected children and adolescents had modest LPA responses to tetanus following booster, similar to HIV-1-infected adults. However, the majority of patients developed protective TAb levels after booster and maintained the response. Shorter intervals may need to be considered for TT immunization boosters in HIV-1-infected pediatric patients, as only 38% had protective TAb at baseline.

Fever in a neutropenic oncology patient requires rapid initiation of effective empiric antibiotics to prevent mortality. We evaluated the appropriateness of our current empiric antibiotic regimen by assessing local antibiotic-susceptibility patterns in our pediatric oncology patients, and comparing them to the general pediatric patterns in our hospital. All blood culture isolates from pediatric oncology patients were reviewed over a 3-year period. Gram-negative and Gram-positive organisms were reviewed separately, with antibiotic susceptibilities for all unique isolates evaluated, and antibiograms generated and compared with general pediatric patients via the Fisher exact test. A total of 84% of Gram negatives were susceptible to meropenem; all resistant organisms were Pseudomonas aeruginosa, with 50% meropenem susceptibility. A total of 91% of Gram negatives were susceptible to cefepime, including 90% of P. aeruginosa and 80% of Escherichia coli. In total, 96% of Gram positives were vancomycin-susceptible; the only resistant organism was a single enterococcal isolate. In comparison with the general pediatric population, significantly fewer pseudomonal isolates were sensitive to meropenem among the oncology population (50% vs. 89%, P=0.0034). As such, in our population, meropenem does not provide adequate monotherapy against Pseudomonas. Ongoing surveillance of antibiotic resistance in this high-risk population is warranted, to ensure appropriate empiric antibiotic usage.

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