Multisystem Inflammatory Syndrome in Children.

Multisystem inflammatory syndrome in children (MIS-C) is an uncommon but emerging syndrome related to SARS-CoV-2 infection. While the presentation of MIS-C is generally delayed after exposure to the virus that causes coronavirus 2019, both MIS-C and Kawasaki disease (KD) share similar clinical features. Multisystem inflammatory syndrome in children poses a diagnostic and therapeutic challenge given the lack of definitive diagnostic tests and a paucity of evidence regarding treatment modalities. We review the clinical presentation, diagnostic evaluations, and management of MIS-C and compare its clinical features to those of KD.

Delirium in the NICU: A Point Prevalence Study.

OBJECTIVES: Delirium is a well-described complication of critical illness, with occurrence rates of >25% in the PICU, and associated morbidity. Infants in the NICU are likely at risk. There have been no previous screening studies to quantify delirium rates in the neonatal population. We hypothesized that delirium was prevalent in term neonates in the NICU. In this pilot study, our objective was to estimate prevalence using a validated pediatric delirium screening tool, which has not yet been tested in NICUs. METHODS: In this point prevalence study, all term or term-corrected infants admitted to the NICU on designated study days were screened for delirium using the Cornell Assessment of Pediatric Delirium. RESULTS: A total of 149 infants were eligible for screening over 8 study days. A total of 147 (98.6%) were successfully screened with the Cornell Assessment of Pediatric Delirium. Overall, 22.4% (n = 33) screened positive for delirium. Delirium was more commonly detected in children on invasive mechanical ventilation (67% vs 17%, P < .01) and those with underlying neurologic disorders (64% vs 13%, P < .01). A multivariate logistic regression revealed that neurologic disability and mechanical ventilation were both independently associated with a positive delirium screen (aOR: 12.3, CI: 4.5-33.6 and aOR: 9.3, CI: 2.5-34.6, respectively). CONCLUSIONS: Our results indicate that delirium likely occurs frequently in term-equivalent infants in the NICU. Further research is necessary to establish feasibility, validity, and interrater reliability of delirium screening in this population.

Pediatric Maxillofacial Trauma: A Review of 156 Patients.

PURPOSE: To review the epidemiology and management of facial fractures in a pediatric population. MATERIALS AND METHODS: This study was a retrospective review of patients younger than 18 years who presented to a pediatric emergency department during a 5-year period in an urban, academic, level 1 designated trauma center. RESULTS: Of the 156 patients identified, most were boys (87%) and the mean age was 13.5 years (standard deviation, 4.9 yr; interquartile range, 12 to 17 yr). The most common mechanism of injury was assault (48.1%). Mandibular fractures (40.7%) were most common. Multiple fractures occurred in 26.9% of patients. Concomitant injuries occurred in 73.7% of patients, most commonly concussions (39.1%). Intracranial hemorrhages were associated with panfacial (P = .005), frontal (P = .001), and orbital (P = .04) fractures. Most patients (91.7%) were admitted, and nonoperative repair was undertaken in 57.1%. There was an independent association of surgical intervention with age older than 14 years and with mandibular fractures (P < .01). CONCLUSIONS: Assault was the most common mechanism of injury and mandibular fracture was the most commonly encountered. Concomitant nonfacial injuries occurred in most patients. Patients sustaining panfacial, frontal, and orbital fractures should provoke an evaluation for other intracranial injuries. Children older than 14 years and those with mandibular fractures should prompt mobilization of resources for operative repair.

Accuracy of weight estimates in pediatric patients by prehospital Emergency Medical Services personnel.

OBJECTIVES: Most medications administered to children are weight-based, and inaccurate weight estimation may contribute to medical errors. Previous studies have been limited to hypothetical patients and those in cardiopulmonary arrest. We aim to determine the accuracy of weight estimates by Emergency Medical Services (EMS) personnel of children receiving medications and to identify factors associated with accuracy. METHODS: EMS records of children <18 years old receiving weight-based medications were merged with EMS staffing data and hospital records. The rate of accurate weight estimates, defined as a value within 20% of the actual weight, was evaluated as the primary outcome. Factors associated with patients and prehospital personnel were also evaluated. RESULTS: 29233 transports occurring during the study period were reviewed, and 199 transports of 179 children were analyzed. The average experience of EMS personnel was 35.8 months (SD ±30.7). EMS personnel accurately estimated weights in 164/199 (82.4%) patients; estimated weights were within 10.8% (SD ±10.5) of the actual weights. Underestimated weights were associated with receiving doses outside of the therapeutic range. Inaccurate weight estimates were associated with age less than 10 years or cardiopulmonary arrest. There was a trend toward inaccurate weight estimates among children who presented with seizures. CONCLUSIONS: EMS personnel are generally accurate in estimating weights of children. There was an association between underestimated weights and inaccurate medication dosing. Younger children or those presenting with seizure or cardiopulmonary arrest were more likely to have inaccurate weight estimates.

Baclofen withdrawal presenting as irritability in a developmentally delayed child.

Irritability in children has a broad differential diagnosis, ranging from benign processes to life-threatening emergencies. In children with comorbid conditions and developmental delay, the diagnostic process becomes more challenging. This case report describes a developmentally delayed 14-year-old boy who presented with pain and crying caused by a malfunction of a surgically implanted baclofen pump. We describe recommendations concerning the diagnostic evaluation, medical management, and surgical repair.

The zebrafish moonshine gene encodes transcriptional intermediary factor 1gamma, an essential regulator of hematopoiesis.

Hematopoiesis is precisely orchestrated by lineage-specific DNA-binding proteins that regulate transcription in concert with coactivators and corepressors. Mutations in the zebrafish moonshine (mon) gene specifically disrupt both embryonic and adult hematopoiesis, resulting in severe red blood cell aplasia. We report that mon encodes the zebrafish ortholog of mammalian transcriptional intermediary factor 1gamma (TIF1gamma) (or TRIM33), a member of the TIF1 family of coactivators and corepressors. During development, hematopoietic progenitor cells in mon mutants fail to express normal levels of hematopoietic transcription factors, including gata1, and undergo apoptosis. Three different mon mutant alleles each encode premature stop codons, and enforced expression of wild-type tif1gamma mRNA rescues embryonic hematopoiesis in homozygous mon mutants. Surprisingly, a high level of zygotic tif1gamma mRNA expression delineates ventral mesoderm during hematopoietic stem cell and progenitor formation prior to gata1 expression. Transplantation studies reveal that tif1gamma functions in a cell-autonomous manner during the differentiation of erythroid precursors. Studies in murine erythroid cell lines demonstrate that Tif1gamma protein is localized within novel nuclear foci, and expression decreases during erythroid cell maturation. Our results establish a major role for this transcriptional intermediary factor in the differentiation of hematopoietic cells in vertebrates.