Newborn Screening for Severe Combined Immunodeficiency.

The T-cell receptor excision circle (TREC) assay is an effective screening tool for severe combined immunodeficiency (SCID). The TREC assay was designed to detect typical SCID and leaky SCID, but any condition causing low naïve T-cell counts will also be detected. Newborn screening for SCID using the TREC assay has proven itself to be highly sensitive and cost-efficient. This review covers the history of SCID newborn screening, elaborates on the SCID subtypes and TREC assay limitations, and discusses diagnostic and management considerations for infants with a positive screen.

Elevated Glycated Hemoglobin Is Associated with Reduced Antibody Responses to Vaccinations in Children.

Background: Childhood obesity is a major health concern, and it is associated with an increased risk of infectious morbidity. Previous studies found a decrease in protective antibody titers in obese adults after hepatitis B, influenza, and tetanus vaccination. Objective: We aimed at determining whether obesity or abnormal hemoglobin A1C (HBA1C) levels are associated with altered antibody responses in children. Methods: Children (8-18 years) who have completed routine childhood immunization were recruited. Serum samples were tested by the enzyme-linked immunosorbent assay method for antibody levels to Diphtheria, Tetanus, Haemophilus influenzae type B (HIB), and Streptococcus pneumoniae, along with serum HBA1C levels. An electronic medical record review on the frequency of emergency visits for infection was conducted. Spearman rank correlation, Fisher-exact, and Pearson's Chi-squared tests were used for statistical analysis. Results: There was an overall negative correlation between body mass index (BMI) percentile and the majority of pneumococcal subtypes, Diphtheria and Tetanus titers, although not statistically significant. There was a statistically significant negative correlation between HBA1C level and the S. pneumoniae serotype P9N (P = 0.037), P4 (P = 0.017), P12 (P = 0.023), P19F (P = 0.050), and HIB (P = 0.001). On average, individuals with elevated HBA1C levels had more frequent emergency room visits for infection (P = 0.029) and more viral infections (P = 0.023) as compared with children with normal HBA1C. Conclusion: Children with higher HBA1C levels were more likely to have lower pneumococcal and HIB titers and increased rates of emergency room visits for infection in a prospective, population-based cohort study. Although not statistically significant, there was an overall negative correlation between BMI percentile and titers for routine childhood vaccines.

Newborn Screening for Severe Combined Immunodeficiency.

The T-cell receptor excision circle (TREC) assay is an effective screening tool for severe combined immunodeficiency (SCID). The TREC assay was designed to detect typical SCID and leaky SCID, but any condition causing low naïve T-cell counts will also be detected. Newborn screening for SCID using the TREC assay has proven itself to be highly sensitive and cost-efficient. This review covers the history of SCID newborn screening, elaborates on the SCID subtypes and TREC assay limitations, and discusses diagnostic and management considerations for infants with a positive screen.

Pediatric Nephrolithiasis: A Review.

The incidence of pediatric nephrolithiasis is on the rise. The composition of kidney stones in children is different than in adults, as most stones in children have a composition of calcium oxalate and calcium phosphate mixed with a small amount of uric acid. The symptoms of pediatric nephrolithiasis are nonspecific. Computed tomography (CT) is the gold standard for diagnosis; however, because of radiation exposure associated with a CT scan, ultrasonography is also an accepted modality for the diagnosis. Extensive metabolic evaluation is important to rule out an underlying metabolic disorder. Urinary decompression, medical expulsion therapy, and surgical interventions such as ureteroscopy and extracorporeal shockwave lithotripsy are some of the options available for treating pediatric nephrolithiasis. [Pediatr Ann. 2017;46(6):e242-e244.].

Current Understanding of the Health Effects of Electromagnetic Fields.

There has been an exponential increase in the use of electronic devices over the past few decades. This has led to increased exposure to electromagnetic fields (EMF). Electric fields result from differences in voltage, whereas magnetic fields result from the flow of electric current. Higher-frequency waves of EMF have more energy than lower-frequency waves, and thus generally tend to be more harmful. An EMF activates cellular stress response and also causes breaks in DNA strands. There are many methodological barriers to effectively measuring the associations of EMF and childhood cancers. The consensus from multiple studies is that there is no causal role of extremely low-frequency EMFs in childhood cancers, including brain cancer. A recent study showed a link between EMF radiation and the development of malignant tumors in rats. In light of that study, the American Academy of Pediatrics set out new recommendations to decrease the adverse effects of cellphone exposure on children. [Pediatr Ann. 2017;46(4):e172-e174.].

EKG Electrode as a Tactile Locator of Stoma after Decannulation: A Pilot Study.

OBJECTIVE: We aimed to evaluate the use of an electrocardiogram (EKG) electrode over decannulation dressings covering the stoma to improve speech intelligibility and volume and reduce air escape by facilitating identification of the "sweet spot" of the dressing. No objective data exist for patient outcomes with use of the EKG electrode dressing. METHODS: This prospective study included head and neck oncology patients at a tertiary hospital who received a tracheostomy. A standard tracheostomy decannulation dressing was placed followed by an EKG electrode. A speech pathologist evaluated speech volume via sound-level meter and captured speech intelligibility for random sentence-level speech. A blinded reviewer scored speech samples for intelligibility. Patients completed a 4-question satisfaction survey. RESULTS: Four patients completed the study. Based on the survey, the patients favored the button, with the lowest scores being 8.5 out of 10. Speech understanding was 48.5% without the button and 83% with the button. Normal speech volume was 73.75 dB without the button and 77.75 dB with the button. Loud speech volume was 80.75 dB without the button and 87 dB with the button. DISCUSSION: This pilot study shows objective benefits of the EKG button as well as improved patient satisfaction. Inexpensive and low maintenance, the EKG electrode provides better occlusion of stoma dressing with easier localization. IMPLICATIONS FOR PRACTICE: Dissemination of our results will aim to improve quality and patient outcomes following decannulation.

Curcumin is an early-acting stage-specific inducer of extended functional longevity in Drosophila.

Larval feeding with curcumin induces an extended health span with significantly increased median and maximum longevities in the adult fly. This phenotype is diet insensitive and shows no additive effect on longevity when combined with an adult dietary restriction (DR) diet, suggesting that curcumin and DR operate via the same or overlapping pathways for this trait. This treatment significantly slows the aging rate so that it is comparable with that of genetically selected long lived animals. The larval treatment also enhances the adult animal's geotactic activity in an additive manner with DR, suggesting that curcumin and DR may use different pathways for different traits. Feeding the drug to adults during only the health span also results in a significantly extended health span with increased median and maximum life span. This extended longevity phenotype is induced only during these stage-specific periods. Feeding adults with the drug over their whole life results in a weakly negative effect on median longevity with no increase in maximum life span. There are no negative effects on reproduction, although larval curcumin feeding increases development time, and also apparently accelerates the normal late-life neuromuscular degeneration seen in the legs. Gene expression data from curcumin-fed larvae shows that the TOR pathway is inhibited in the larvae and the young to midlife adults, although several other genes involved in longevity extension are also affected. These data support the hypothesis that curcumin acts as if it is a DR mimetic nutraceutical. These data also suggest that the search for DR mimetics may be enhanced by the use of stage-specific screening of candidate molecules.