Children — Open Access Journal

Pediatric transverse myelitis (TM) is an acquired, immune-mediated disorder that leads to injury of the spinal cord and often manifests as weakness, numbness, bowel dysfunction, and/or bladder dysfunction. Multiple etiologies for myelitis can result in a similar clinical presentation, including idiopathic transverse myelitis (TM), multiple sclerosis (MS), neuromyeltis optica spectrum disorder (NMOSD) associated with anti-aquaporin 4 antibodies, MOG antibody-associated disease, and acute flaccid myelitis (AFM). Diagnosis relies on clinical recognition of the syndrome and confirming inflammation through imaging and/or laboratory studies. Acute treatment is targeted at decreasing immune-mediated injury, and chronic preventative therapy may be indicated if TM is determined to be a manifestation of a relapsing disorder (i.e., NMOSD). Timely recognition and treatment of acute transverse myelitis is essential, as it can be associated with significant morbidity and long-term disability. Full article

Aortic valve stenosis in children is a congenital heart defect that causes fixed form of hemodynamically significant left ventricular outflow tract obstruction with progressive course. Neonates and young infants who have aortic valve stenosis, usually develop congestive heart failure. Children and adolescents who have aortic valve stenosis, are mostly asymptomatic, although they may carry a small but significant risk of sudden death. Transcatheter or surgical intervention is indicated for symptomatic patients or those with moderate to severe left ventricular outflow tract obstruction. Many may need reintervention. Full article

The Textbook of Neonatal Resuscitation, seventh edition, does not suggest routine endotracheal suctioning for non-vigorous infants born through meconium-stained amniotic fluid. We compared 301,150 infants at ≥35 weeks’ gestational age inborn at 311 Vermont Oxford Network member centers in the United States (U.S.) and admitted to neonatal intensive care units (NICU) who were born before (2013 to 2015) and after (2017) the guideline change. Logistic regression models adjusting for clustering of infants within centers were used to calculate risk ratios. NICU admissions for infants with a diagnosis of meconium aspiration syndrome (MAS) decreased from 1.8% to 1.5% (risk ratio: 0.82; 95% confidence interval: 0.68, 0.97) and delivery room endotracheal suctioning in this group decreased from 57.0% to 28.9% (0.51; 0.41, 0.62). Treatment with conventional or high frequency ventilation, inhaled nitric oxide, or extracorporeal membrane oxygenation remained unchanged 42.3% vs. 40.3% (0.95; 0.80, 1.13) among infants with MAS and 9.1% vs. 8.2% (0.91; 0.87, 0.95) among infants without MAS. The use of surfactant among infants with MAS increased from 24.6% to 30% (1.22; 1.02, 1.48). Mortality (2.6 to 2.9%, 1.12; 0.74, 1.69) and moderate/severe hypoxic-ischemic encephalopathy (5.4 to 6.8%, 1.24; 0.91, 1.69) increased slightly in 2017. Subgroup analyses of infants with 1 min Apgar scores of ≤3 found similar results. While NICU admissions for MAS and tracheal suctioning decreased after the introduction of the new guideline with no subsequent increase in severe respiratory distress among infants with and without a MAS diagnosis, limitations in our study preclude inferring that the new guideline is safe or effective. Full article

There is a lack of consensus regarding the preoperative pulmonary valve (PV) Z-score “cut-off” in tetralogy of Fallot (ToF) patients to attempt a successful valve sparing surgery (VSS). Therefore, the aim of this study was to review the available evidence regarding the association between preoperative PV Z-score and rate of re-intervention for residual right ventricular outflow tract (RVOT) obstruction, i.e. successful valve sparing surgery. A systematic search of studies reporting outcomes of VSS for ToF was performed utilizing PubMed, EMBASE, and Scopus databases. Patients with ToF variants such as pulmonary atresia, major aortopulmonary collaterals, absent pulmonary valve, associated atrioventricular septal defect, and discontinuous pulmonary arteries were excluded. Out of 712 screened publications, 15 studies met inclusion criteria. A total of 1091 patients had surgery at a median age and weight of 6.9 months and 7.2 kg, respectively. VSS was performed on the basis of intraoperative PV assessment in 14 out of 15 studies. The median preoperative PV Z-score was −1.7 (0 to −4.9) with a median re-intervention rate of 4.7% (0–36.8%) during a median follow-up of 2.83 years (1.4–15.8 years). Quantitatively, there was no correlation between decreasing preoperative PV Z-scores and increasing RVOT re-intervention rates with a correlation coefficient of −0.03 and an associated p-value of 0.91. In observational studies, VSS for ToF repair was based on intraoperative evaluation and sizing of the PV following complete relief of all levels of obstruction of the RVOT, rather than pre-operative echocardiography derived PV Z-scores. Full article

Compounding pharmacy has an important role to play in the field of pediatric medicine. These specialized pharmacies can offer solutions to the unique patient needs that arise in the pediatric population. Medication can be tailored to the child to allow better compliance in cases when the commercial product is unable to meet the needs of the patient. For example, a suspension, suppository, or lozenge formulation is sometimes needed when the manufactured products are only offered as solid oral dosage forms. Sensory processing disorder (SPD), patients with food allergies, and specific dietary needs can also be a big challenge for caregivers and practitioners who need alternatives to the commercially available forms. Three example cases are presented to help describe the process of collaboration between the pharmacist, patient, and doctor to solve the patient’s needs. Full article

Background: The prevalence and severity of pain, nausea/vomiting, and anxiety (PNVA) among hospitalized children is not well established. We describe the prevalence and severity of PNVA among hospitalized patients from oncology, general pediatrics, and cardiology services in a tertiary care center. Methods: Patients were recruited on admission and enrolled if their caregiver consented, spoke English, and were anticipated to stay 2–30 days. Symptoms were measured weekdays using age-validated tools. PNVA symptoms were described and compared. Results: We enrolled 496 (49.4%) patients of 1005 admitted. Patients were predominantly Caucasian (57.9%) on their first admission (53.6%). The average (SD) age was 8.6 years (5.9) in oncology, 4.2 (5.3) in general pediatrics and 2.6 (4.0) in cardiology. 325 (65.6%) patients reported anxiety, 275 (55.4%) reported nausea and 256 (52.0%) reported pain. Mean (SD) severity out of 10 was 3.7 (2.5) for anxiety, 3.2 (2.1) for nausea and 3.0 (1.5) for pain. Prevalence of PNVA was no different between clinical programs, but pain (p = 0.008) and nausea (p = 0.006) severity were. PNVA symptom co-occurrence was positively correlated (p < 0.001). Conclusions: Anxiety was the most common and severe symptom for hospitalized children. Patients in oncology demonstrated the least severe pain and nausea with no difference in anxiety between services. Full article

Preschool is one of the most important periods in a child’s life, and it can influence their social development. A lack of attention to this important life period can increase the risk of serious injuries to a child’s growth and development. The aim of this study was to examine the level of social skills in preschool children from teachers’ perspectives and investigate the relationship between social skills and the child’s environmental and cultural background. A cross-sectional study was conducted on 546 children studying in the preschool centers of Rasht city, Iran. They were selected using a multistage cluster sampling method. Data was gathered using the children’s and teachers’ demographic questionnaire and the Social Skills Rating System-Teachers (SSRS-T). Descriptive and inferential statistics were used for data analysis via SPSS software. It was found that the majority of children had moderate skills in terms of cooperation, assertion, self-control, and total social skills. Also, a statistically significant association was reported between total social skill scores and the mother’s age, mother’s education level, mother’s job, family’s income, teacher teaching experiences, and number of children in each classroom. The Iranian children were at a relatively low risk of problems with social skills. To improve children’s social skills, more attention should be paid to factors related to familial and socioeconomic status such as income, parents’ education level, maternal age, teacher’s selection for this age group, and number of children in each classroom. Full article

The prevalence of obesity continues to rise in adult and pediatric populations throughout the world. Obesity has a direct impact on all organ systems, including the reproductive system. This review summarizes current knowledge about the effects of obesity on the male reproductive system across age, highlighting the need for more data in children and adolescents. Male hypogonadism is commonly seen in patients with obesity and affects the onset, duration, and progression of puberty. Different pathophysiologic mechanisms include increased peripheral conversion of testosterone to estrone and increased inflammation due to increased fat, both of which lead to suppression of the hypothalamic-pituitary-gonadotropin (HPG) axis and delayed development of secondary sexual characteristics in adolescent males. Evaluation of the HPG axis in obesity includes a thorough history to exclude other causes of hypogonadism and syndromic associations. Evaluation should also include investigating the complications of low testosterone, including increased visceral fat, decreased bone density, cardiovascular disease risk, and impaired mood and cognition, among others. The mainstay of treatment is weight reduction, but medications such as testosterone and clomiphene citrate used in adults, remain scarcely used in adolescents. Male hypogonadism associated with obesity is common and providers who care for adolescents and young adults with obesity should be aware of its impact and management. Full article

Background: Recently, the number of refugees in Germany has skyrocketed, leading to a marked increase in refugee children admitted to hospitals. This study describes the special characteristics encountered in pediatric surgical inpatient refugees compared to locally residing patients. Methods: Hospital records of minor refugees admitted to our department from 2005 up to and including 2015 were retrospectively reviewed. Demographic data, diagnoses, comorbidities, body mass indexes, hemoglobin values, and lengths of stay were extracted and statistically compared to local patients. Results: A total of 63 refugee children were analyzed and compared to 24,983 locally residing children. There was no difference in median body mass index (16.2 vs. 16.3, respectively, p = 0.26). However, refugee children had significantly lower hemoglobin values (11.95 vs. 12.79 g/dL, p < 0.0001) and were more likely to be colonized with methicillin-resistant Staphylococcus. aureus (8% vs. 0.04%, p < 0.01). Refugees were much more likely to present with burn injuries (16% versus 3% of admissions, p < 0.001), esophageal foreign bodies (4% vs. 0.5%, p < 0.001), as well as trauma, except for closed head injury. Conclusion: The cohort of refugee children in this study was found to be at a particular risk for suffering from burn injuries, trauma, foreign body aspirations, and anemia. Appropriate preventive measures and screening programs should be implemented accordingly. Full article

Pediatric cardiology imaging laboratories in the present day have several modalities for imaging of congenital and acquired cardiovascular disease. These modalities include echocardiography, cardiovascular magnetic resonance imaging, cardiac computed tomography and nuclear imaging. The utility and limitations of multimodal imaging is described herein along with a framework for establishing a cardiology-radiology interface. Full article

Premature and full-term infants are at high risk of morbidities such as intraventricular hemorrhage or hypoxic-ischemic encephalopathy. The sickest infants at birth are the most likely to die and or develop intraventricular hemorrhage. Delayed cord clamping has been shown to reduce these morbidities, but is currently not provided to those infants that need immediate resuscitation. This review will discuss recently published and ongoing or planned clinical trials involving neonatal resuscitation while the newborn is still attached to the umbilical cord. We will discuss the implications on neonatal management and delivery room care should this method become standard practice. We will review previous and ongoing trials that provided respiratory support compared to no support. Lastly, we will discuss the implications of implementing routine resuscitation support outside of a research setting. Full article

Delayed cord clamping (DCC) is endorsed by multiple professional organizations for both term and preterm infants. In preterm infants, DCC has been shown to reduce intraventricular hemorrhage, lower incidence of necrotizing enterocolitis, and reduce the need for transfusions. Furthermore, in preterm animal models, ventilation during DCC leads to improved hemodynamics. While providing ventilation and continuous positive airway pressure (CPAP) during DCC may benefit infants, the logistics of performing such a maneuver can be complicated. In this simulation-based study, we sought to explore attitudes of providers along with the safety and ergonomic challenges involved with safely resuscitating a newborn infant while attached to the placenta. Multidisciplinary workshops were held simulating vaginal and Caesarean deliveries, during which providers started positive pressure ventilation and transitioned to holding CPAP on a preterm manikin. Review of videos identified 5 themes of concerns: sterility, equipment, mobility, space and workflow, and communication. In this study, simulation was a key methodology for safe identification of various safety and ergonomic issues related to implementation of ventilation during DCC. Centers interested in implementing DCC with ventilation are encouraged to form multidisciplinary work groups and utilize simulations prior to performing care on infants. Full article

Children with special health care needs (CSHCN) use relatively high quantities of healthcare resources and have overall higher morbidity than the general pediatric population. Embedding clinical pharmacists into the Patient-Centered Medical Home (PCMH) to provide comprehensive medication management (CMM) through collaborative practice agreements (CPAs) for children, especially for CSHCN, can improve outcomes, enhance the experience of care for families, and reduce the cost of care. Potential network infrastructures for collaborative practice focused on CSHCN populations, common language and terminology for CMM, and clinical pharmacist workforce estimates are provided. Applying the results from the CMM in Primary Care grant, this paper outlines the following: (1) setting up collaborative practices for CMM between clinical pharmacists and pediatricians (primary care pediatricians and sub-specialties, such as pediatric clinical pharmacology); (2) proposing various models, organizational structures, design requirements, and shared electronic health record (EHR) needs; and (3) outlining consistent documentation of CMM by clinical pharmacists in CSHCN populations. Full article

Almost 30 million babies worldwide are born prematurely or become ill annually and need specialized care to survive. Formalized collaborative practice agreements (CPA) between clinical pharmacists and physicians have been put forward as a means for improving the overall medicating experience in many patient populations, including children. This report briefly describes opportunities for collaboration using examples from countries on each continent where CPA is established in professional governance documents and standards. It also provides resources in the form of a toolkit for countries and pharmacist–physician collaborators to authorize and form CPAs to provide comprehensive medication management (CMM) for children and youth with special health care needs (CSHCN). Full article

Neonatal mortality in Africa is among the highest in the world. In Liberia, providers face significant challenges due to lack of resources, and providers in referral centers need to be prepared to appropriately provide neonatal resuscitation. A team of American Heart Association health care providers taught a two-day neonatal resuscitation curriculum designed for low-resource settings at a regional hospital in Liberia. The goal of this study was to evaluate if the curriculum improved knowledge and comfort in participation. The curriculum included simulations and was based on the Neonatal Resuscitation Protocol (NRP). Students learned newborn airway management, quality chest compression skills, and resuscitation interventions through lectures and manikin-based simulation sessions. Seventy-five participants were trained. There was a 63% increase in knowledge scores post training (p < 0.00001). Prior cardiopulmonary resuscitation (CPR) training, age, occupation, and pre-intervention test score did not have a significant effect on post-intervention knowledge test scores. The median provider comfort score improved from a 4 to 5 (p < 0.00001). Factors such as age, sex, prior NRP education, occupation, and post-intervention test scores did not have a significant effect on the post-intervention comfort level score. A modified NRP and manikin simulation-based curriculum may be an effective way of teaching health care providers in resource-limited settings. Training of providers in limited-resource settings could potentially help decrease neonatal mortality in Liberia. Modification of protocols is sometimes necessary and an important part of providing context-specific training. The results of this study have no direct relation to decreasing neonatal mortality until proven. A general resuscitation curriculum with modified NRP training may be effective, and further work should focus on the effect of such interventions on neonatal mortality rates in the region. Full article

Immigrant families are known to be at higher risk of food insecurity compared to non-immigrant families. Documented immigrants in the U.S. <5 years are ineligible for the Supplemental Nutrition Assistance Program (SNAP). Immigration enforcement, anti-immigrant rhetoric, and policies negatively targeting immigrants have increased in recent years. Anecdotal reports suggest immigrant families forgo assistance, even if eligible, related to fear of deportation or future ineligibility for citizenship. In the period of January 2007–June 2018, 37,570 caregivers of young children (ages 0–4) were interviewed in emergency rooms and primary care clinics in Boston, Baltimore, Philadelphia, Minneapolis, and Little Rock. Food insecurity was measured using the U.S. Department of Agriculture’s Food Security Survey Module. Overall, 21.4% of mothers were immigrants, including 3.8% in the U.S. <5 years (“<5 years”) and 17.64% ≥ 5 years (“5+ years”). SNAP participation among <5 years families increased in the period of 2007–2017 to 43% and declined in the first half of 2018 to 34.8%. For 5+ years families, SNAP participation increased to 44.7% in 2017 and decreased to 42.7% in 2018. SNAP decreases occurred concurrently with rising child food insecurity. Employment increased 2016–2018 among U.S.-born families and was stable among immigrant families. After steady increases in the prior 10 years, SNAP participation decreased in all immigrant families in 2018, but most markedly in more recent immigrants, while employment rates were unchanged. Full article

In this review management of the most common cyanotic congenital heart defects (CHDs) was discussed; the management of acyanotic CHD was reviewed in Part I of this series. While the need for intervention in acyanotic CHD is by and large determined by the severity of the lesion, most cyanotic CHDs require intervention, mostly by surgery. Different types of tetralogy of Fallot require different types of total surgical corrective procedures, and some may require initial palliation, mainly by modified Blalock–Taussig shunts. Babies with transposition of the great arteries with an intact ventricular septum as well as those with ventricular septal defects (VSD) need an arterial switch (Jatene) procedure while those with both VSD and pulmonary stenosis should be addressed by Rastelli procedure. These procedures may need to be preceded by prostaglandin infusion and/or balloon atrial septostomy in some babies. Infants with tricuspid atresia require initial palliation either with a modified Blalock–Taussig shunt or banding of the pulmonary artery and subsequent staged Fontan (bidirectional Glenn and fenestrated Fontan with extra-cardiac conduit). Neonates with total anomalous pulmonary venous connection are managed by anastomosis of the common pulmonary vein with the left atrium either electively in non-obstructed types or as an emergency procedure in the obstructed types. Babies with truncus arteriosus are treated by surgical closure of VSD along with right ventricle to pulmonary artery conduit. The other defects, namely, hypoplastic left heart syndrome, pulmonary atresia with intact ventricular septum, double-outlet right ventricle, double-inlet left ventricle and univentricular hearts largely require multistage surgical correction. The currently existing medical, trans-catheter and surgical techniques to manage cyanotic CHD are safe and effective and can be performed at a relatively low risk. Full article

Introduction: Peritoneal dialysis and continuous renal replacement therapy (CRRT) are the most frequently used treatment modalities for acute kidney injury. CRRT is currently being used for the treatment of several non-renal indications, such as congenital metabolic diseases. CRRT can efficiently remove toxic metabolites and reverse the neurological symptoms quickly. However, there is not enough data for CRRT in children with metabolic diseases. Therefore, we aimed a retrospective study to describe the use of CRRT in metabolic diseases and its associated efficacy, complications, and outcomes. Materials and Methods: We performed a retrospective analysis of the records of all patients admitted in the pediatric intensive care unit (PICU) for CRRT treatment. Results: Between December 2014 and November 2018, 97 patients were eligible for the present study. The age distribution was between 2 days and 17 years, with a mean of 3.77 ± 4.71 years. There were 13 (36.1%) newborn with metabolic diseases. The patients were divided into two groups: CRRT for metabolic diseases and others. There was a significant relationship between the groups, including age (p ≤ 0.001), weight (p = 0.028), blood flow rate (p ≤ 0.001); dialysate rate (p ≤ 0.001), and replacement rate (p ≤ 0.001). The leucine reduction rate was 3.88 ± 3.65 (% per hour). The ammonia reduction rate was 4.94 ± 5.05 in the urea cycle disorder group and 5.02 ± 4.54 in the organic acidemia group. The overall survival rate was 88.9% in metabolic diseases with CRRT. Conclusion: In particularly hemodynamically unstable patients, CRRT can effectively and quickly reduce plasma ammonia and leucine. Full article

The current guidelines recommend the use of 100% O₂ during resuscitation of a neonate requiring chest compressions (CC). Studies comparing 21% and 100% O₂ during CC were conducted in postnatal models and have not shown a difference in incidence or timing of return of spontaneous circulation (ROSC). The objective of this study is to evaluate systemic oxygenation and oxygen delivery to the brain during CC in an ovine model of perinatal asphyxial arrest induced by umbilical cord occlusion. Pulseless cardiac arrest was induced by umbilical cord occlusion in 22 lambs. After 5 min of asystole, lambs were resuscitated with 21% O₂ as per Neonatal Resuscitation Program (NRP) guidelines. At the onset of CC, inspired O₂ was either increased to 100% O₂ (n = 25) or continued at 21% (n = 9). Lambs were ventilated for 30 min post ROSC and FiO₂ was gradually titrated to achieve preductal SpO₂ of 85–95%. All lambs achieved ROSC. During CC, PaO₂ was 21.6 ± 1.6 mm Hg with 21% and 23.9 ± 6.8 mm Hg with 100% O₂ (p = 0.16). Carotid flow was significantly lower during CC (1.2 ± 1.6 mL/kg/min in 21% and 3.2 ± 3.4 mL/kg/min in 100% oxygen) compared to baseline fetal levels (27 ± 9 mL/kg/min). Oxygen delivery to the brain was 0.05 ± 0.06 mL/kg/min in the 21% group and 0.11 ± 0.09 mL/kg/min in the 100% group and was significantly lower than fetal levels (2.1 ± 0.3 mL/kg/min). Immediately after ROSC, lambs ventilated with 100% O₂ had higher PaO₂ and pulmonary flow. It was concluded that carotid blood flow, systemic PaO₂, and oxygen delivery to the brain are very low during chest compressions for cardiac arrest irrespective of 21% or 100% inspired oxygen use during resuscitation. Full article