Children's Medical Center Dallas Research Articles

Multicenter comparison of head shape outcomes for three minimally invasive strip craniectomy techniques for sagittal craniosynostosis.

A literature gap exists comparing whole head shape outcome following correction of sagittal craniosynostosis. The objective of this multicenter study was to provide an analysis of long-term results following three different endoscopic strip craniectomy techniques for correction of sagittal craniosynostosis: 1) spring-assisted strip craniectomy, 2) wide-strip craniectomy with biparietal and bitemporal barrel-stave wedge osteotomies plus helmet orthosis, and 3) narrow-strip craniectomy plus orthosis without barrel staves. Pre- and postoperative 3D stereophotogrammetric images were collected from patients who underwent craniosynostosis surgery. Procedures were divided among institutions as follows: spring-assisted strip craniectomies were performed at Atrium Health Wake Forest Baptist Hospital; narrow-strip craniectomies were performed at St. Louis Children's Hospital by one craniofacial surgeon; and wide-vertex craniectomies were performed at St. Louis Children's Hospital prior to 2010, and then continued at Children's Medical Center Dallas. Pre- and postoperative 3D whole-head composite images were generated for each procedure to visually represent outcomes at final follow-up and compared with age-matched normal controls. Patients in the spring-assisted strip craniectomy group showed normalization of frontal bossing and skull height compared with age-matched controls, whereas patients undergoing wide-strip craniectomy showed greater correction of occipital protrusion. Patients in the narrow-strip craniectomy cohort had intermediate results between these outcomes. Nested aggregate head shapes showed good correction of head shapes from all techniques. This large, retrospective, multicenter study illustrated whole head shape outcomes from three different craniectomy procedures. Although each procedure showed some differences in loci of primary correction, all three surgical methods demonstrated good correction of primary scaphocephalic deformity.

Neighborhood Characteristics and the Burden of E-Cigarette, or Vaping, Product Use-Associated Lung Injury: An Ecological Comparison Study

Purpose: E-cigarette, or vaping, product use-associated lung injury (EVALI) is a severe consequence of vaping first described in 2019. Investigating associations between neighborhood-level characteristics and EVALI cases is an important step in identifying at-risk communities to implement future targeted prevention programs. Methods: We retrospectively identified 41 adolescents <19 years hospitalized for treatment for EVALI at Children's Medical Center Dallas from December 2018 to June 2021. Patient ZIP codes were extracted from the electronic medical record and were compared with Dallas area ZIP codes containing no EVALI cases. Socioeconomic status (SES) characteristics were obtained from the 2019 American Community Survey, and they were mapped for ZIP codes using ESRI ArcMap geospatial processing software. A parallel analysis was conducted utilizing data of adolescents hospitalized with appendicitis. Results: Ninety-five percent of our cohort used tetrahydrocannabinol-containing products, and 66% obtained their vaping products from informal sources. EVALI cases were less likely to reside in higher SES ZIP codes as measured by the proportion of the population with at least a high school education (odds ratio [OR]: 0.95, 95% confidence interval [CI]: 0.92-0.99), access to broadband access (OR: 0.95, 95% CI: 0.91-0.99), and private health insurance (OR: 0.97, 95% CI: 0.95-0.99). Alternatively, they were more likely to reside in lower SES ZIP codes as measured by proportion of the population without any health insurance (OR: 1.07, 95% CI: 1.01-1.12). No neighborhood level low SES characteristics were associated with appendicitis hospitalizations. Conclusions: Although small in magnitude, EVALI cases were associated with lower SES ZIP codes but not with vape shop density.

Neurosurgery at UT Southwestern Medical Center: 1956-2020.

The history of neurosurgery at UT Southwestern Medical Center in Dallas, Texas, is reviewed. Kemp Clark, MD, started the academic neurosurgical practice at Parkland Hospital in 1956. Clark developed a robust training program that required the resident to operate early. In 1972, the Dallas Veterans Affairs Hospital was added to the training program. Duke Samson, MD, became chair in 1988. He emphasized technical excellence and honest reporting of surgical outcomes. In 1989, Zale Lipshy University Hospital opened and became a center for neurosurgical care, and Hunt Batjer, MD, became chair in 2012. The program expanded significantly. Along with principles established by his predecessors, Batjer emphasized the need for all neurosurgeons to engage the community and to be active in policy leadership through local and national organizations. During his tenure, the pediatric neurosurgery group at Children's Medical Center Dallas was integrated with the department, and a multidisciplinary spine service was developed. In 2014, the Peter O'Donnell Jr. Brain Institute was established, and the William P. Clements Jr. University Hospital opened. For 64 years, UT Southwestern Medical Center has been fertile ground for academic neurosurgery, with a strong emphasis on excellence in patient care.

Invasive Fungal Infections in Pediatric Patients with High-Risk Acute Lymphoblastic Leukemia during Initial Phases of Therapy: A Retrospective Evaluation

BACKGROUND Patients with newly diagnosed acute lymphoblastic leukemia (ALL) are at increased risk of infection. While previously published guidelines recommend primary antifungal prophylaxis in patients with T-cell ALL, we sought to determine the pattern of invasive fungal disease (IFI) at our center so as to assess risk factors for IFI, beyond the diagnosis of T-ALL, with the administration of dexamethasone and an anthracycline during induction. The current practice at Children's Health Children's Medical Center Dallas is to provide primary antifungal prophylaxis with micafungin during induction therapy for hospitalized patients with T-cell ALL and those with Down Syndrome. Additionally, we recently decided to provide primary antifungal prophylaxis to patients with HR B-ALL with hyperglycemia who remain hospitalized during induction. The primary objective of this study was to capture the institution-specific five-year incidence of IFI prior to the start of delayed intensification (DI) phase chemotherapy among pediatric patients with ALL. Secondary objectives were to identify potential IFI risk factors specifically amongst pediatric patients with HR ALL. METHODS This retrospective chart review included patients younger than 21 years with newly diagnosed ALL between July 1, 2014 and June 30, 2019. Patients with secondary leukemia, infantile leukemia, or those receiving treatment for a fungal infection at presentation were excluded. The primary outcome was the development of probable or proven IFI, as defined by the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group criteria, prior to the start of the DI phase of therapy. Statistical methods included Chi-square test, t-test, and Wilcoxon Rank Sum test, as appropriate to the variable's level of measurement and distribution. Time series analyses were used to assess overall and seasonal trends of IFI incidence over the study period. RESULTS Of 220 included patients, there were 13 cases of IFI diagnosed during the induction and consolidation phases of therapy during the five-year period. IFI occurred in 15.3% of the HR group (11/72), 5.9% of the T-cell ALL group (1/17), and 0.8% of the standard risk (SR) group (1/131). Among individuals with HR ALL, the majority of cases occurred in the absence of primary antifungal prophylaxis (90.9%). The most common sites of IFI included the lungs (n=6) and sinuses (n=4). Implicated fungal pathogens included Aspergillus, Candida, Curvularia, Exserohilum, and Bipolaris. Univariate analysis of the potential IFI risk factors in HR ALL patients did not identify any significant differences between those patients that did or did not develop IFI, with respect to pre-existing comorbidities, body mass index, laboratory results at diagnosis, or hospital exposures during induction including length of stay, intensive care admissions, and receipt of systemic antibiotics (Table). Race and ethnicity was significantly different likely due to the skewed distribution of IFI among patients who identified as Asian (p = 0.03; 8/8 identified as Asian and did not develop IFI). With respect to seasonality of ALL diagnosis, the percent of patients in each group (winter, spring, summer, and fall) that developed IFI were 7.4%, 18.2%, 20%, and 31.2%, respectively. However, time series analysis did not show an association between seasonality of diagnosis and development of IFI (p=0.89). During the induction phase of therapy, hyperglycemia, defined as blood glucose 140-200 mg/dL for ≥ 2 days or &amp;gt;200 mg/dL for 1 day, was present in 100% of the HR patients that developed IFI and 74% of the HR patients that did not develop IFI (p=0.12). CONCLUSION In this pediatric population, patients with HR B-ALL developed more fungal infections during the early phases of therapy then those with SR disease (15.6% versus 0.8%). In analysis of potential risk factors, there were no significant differences between HR ALL groups that did or did not develop IFI. Larger-scale studies are needed in order to identify potential risk factors that will guide decisions on the routine use of primary antifungal prophylaxis in patients with HR ALL during induction therapy. Disclosures No relevant conflicts of interest to declare. OffLabel Disclosure: Micafungin use for primary antifungal prophylaxis during induction phase of therapy.

Antibiotic-Induced Depletion of Anti-inflammatory Clostridia Is Associated with the Development of Graft-versus-Host Disease in Pediatric Stem Cell Transplantation Patients

Adult stem cell transplantation (SCT) patients with graft-versus-host-disease (GVHD) exhibit significant disruptions in gut microbial communities. These changes are associated with higher overall mortality and appear to be driven by specific antibiotic therapies. It is unclear whether pediatric SCT patients who develop GVHD exhibit similar antibiotic-induced gut microbiota community changes. Here, we show that pediatric SCT patients (from Children's Medical Center Dallas, n = 8, and Cincinnati Children's Hospital, n = 7) who developed GVHD showed a significant decline, up to 10-log fold, in gut anti-inflammatory Clostridia (AIC) compared with those without GVHD. In fact, the development of GVHD is significantly associated with this AIC decline and with cumulative antibiotic exposure, particularly antibiotics effective against anaerobic bacteria (P = .003, Firth logistic regression analysis). Using metagenomic shotgun sequencing analysis, we were able to identify specific commensal bacterial species, including AIC, that were significantly depleted in GVHD patients. We then used a preclinical GVHD model to verify our clinical observations. Clindamycin depleted AIC and exacerbated GVHD in mice, whereas oral AIC supplementation increased gut AIC levels and mitigated GVHD in mice. Together, these data suggest that an antibiotic-induced AIC depletion in the gut microbiota is associated with the development of GVHD in pediatric SCT patients.

The Effect of 13-Valent Pneumococcal Conjugate Vaccine on the Serotype Distribution and Antibiotic Resistance Profiles in Children With Invasive Pneumococcal Disease.

BackgroundInvasive pneumococcal disease (IPD) continues to be a significant burden in children despite the implementation of two generations of conjugate vaccines. Serotype replacement by nonvaccine serotypes is reported in multiple areas around the world. This study is a continuation of previous studies and describes the incidence, serotype distribution, and antibiotic resistance pattern of Streptococcus pneumoniae serotypes causing IPD at Children's Medical Center Dallas after introduction of 13-valent pneumococcal conjugate vaccine (PCV13).Methods Streptococcus pneumoniae isolates from normally sterile sites were collected from January 1, 1999 to June 30, 2014. Demographic and clinical information was extracted for analysis. Incidence of IPD was calculated using inpatient and emergency center admissions to Children's Medical Center of Dallas as the denominator. Isolates were serotyped and penicillin/cefotaxime susceptibilities were determined. Selected nontypeable isolates were further characterized by multilocus sequence typing. A χ2 test and the Cochran–Armitage Trend Test for trend analysis were used to evaluate change in serotype and antibiotic susceptibility patterns over time.ResultsComparison of the different study periods showed a significant reduction in the incidence of IPD in PCV13 era compared with prevaccine era and PCV7 era (P < .05). Children younger than 24 months showed the largest reduction of disease incidence. More than 40% of patients with IPD had a documented comorbidity. Cases of pneumonia continued to decrease in the PCV13 era (P < .002). The most common non-PCV13 serotypes after vaccine introduction were as follows: 23B, 6C, 23A, 9N/L, and 12. Penicillin resistance by meningitis breakpoint decreased significantly in the PCV13 era.ConclusionsAfter introduction of PCV13 in Dallas, incidence of IPD caused by strains contained in the vaccine and penicillin resistance continued to decrease. Serotype replacement phenomena and persistence of PCV7 serotypes were documented. Patients with comorbidities represented a large percentage of patients with IPD. Concerns for geographic variation in serotype replacement phenomena arise from the present study.

Tolerance of a high-protein baked-egg product in egg-allergic children

BackgroundEgg allergy is one of the most common immunoglobulin E (IgE)–mediated food allergies. Extensively heating egg has been found to decrease its allergenicity and 64% to 84% of children allergic to egg have been found to tolerate baked-egg products. Because there is no reliable method for predicting baked-egg tolerance, oral food challenges remain the gold standard. Prior studies have reported on baked-egg challenges using up to 2.2 g of egg white (EW) protein. ObjectiveTo establish whether children with egg allergy would pass a baked-egg challenge to a larger amount of egg protein and the potential criteria for predicting the likelihood of baked-egg tolerance. MethodsA chart review was conducted of all patients 6 months to 18 years of age with egg allergy who underwent oral baked-egg challenges at Children's Medical Center Dallas over a 2-year period. Challenges were conducted in the clinic with a 3.8-g baked-egg product. ResultsFifty-nine of 94 patients (63%) tolerated the 3.8-g baked-egg product. The presence of asthma (P < .01), EW skin prick test (SPT; P < .01) reactive wheal, and EW-specific IgE level (P = .02) correlated with baked-egg reactivity, whereas ovomucoid-specific IgE level did not. The positive predictive value approached 66% at an EW SPT reactive wheal of 10 mm and 60% for an EW-specific IgE level of 8 kUA/L. ConclusionMost subjects with egg allergy tolerated baked egg. This study is the first to use 3.8 g of EW protein for the challenges. The EW SPT wheal diameter and EW-specific IgE levels were the best predictors of baked-egg tolerance.

Accuracy of Administrative Coding for Sickle Cell Disease

BackgroundAdministrative data are increasingly used in sickle cell disease (SCD) research to study large numbers of patients at low cost. However, the validity of research with these data depends on the accuracy of administrative coding, which has been understudied in SCD. In particular, the validity of ICD-9CM coding for SCD's clinical hallmark, vaso-occlusive crisis (VOC), has not been reported. Therefore, we aim to describe the accuracy of ICD-9CM coding for VOC, acute chest syndrome (ACS), and acute splenic sequestration crisis (ASSC) in addition to SCD genotypes.MethodsAdministrative coding for all acute care visits (emergency department [ED], inpatient observation, and inpatient hospitalization) in SCD patients was captured for the 2013 calendar year at Children's Medical Center Dallas (CMCD) by query of administrative records. SCD visits were identified using the ICD-9CM codes 282.4x and 282.6x in the primary or any of 14 secondary code positions. From the administrative data, VOC was defined by the use of the "in crisis" codes (282.42, 282.62, 282.64, or 282.69). ACS was defined by 517.3 and ASSC by 289.52. Genotypes were defined as HbSS - 282.61 or 282.62; HbSC - 282.63 or 282.64; and HbS-BetaThal - 282.41 or 282.42.For the chart review, all visits were independently evaluated by two reviewers with the senior author settling disagreements. Previously-published consensus definitions were used for the complications of interest: VOC was defined as new onset of pain lasting at last 4 hours for which there is no other explanation; ACS was defined by new pulmonary infiltrate on chest X-ray with associated fever or respiratory symptoms; and ASSC was defined as splenic enlargement leading to anemia (hemoglobin >2g/dL under baseline), typically with thrombocytopenia. The primary reviewers and senior author evaluated 10 cases for the purpose of consensus-building before beginning data abstraction.Data capture included demographics and cause of the visit. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR+), and negative likelihood ratio (LR-) of ICD-9CM coding for VOC, ACS, ASSC, and the SCD genotype were calculated. The LR+ and LR- were prevalence-weighted.ResultsFrom 448 patients, 1107 acute care visits were identified at CMCD in 2013. Patients had a median age of 8.2 years (range 0.1 - 33.9 years). Females accounted for 53% of visits. Inpatient hospitalization accounted for 374 visits (34%) whereas ED-only visits accounted for 681(61%) and inpatient observation 52 (5%). The prevalence of SCD genotypes included 69% HbSS, 19% HbSC, and 7% HbS-BetaThal. VOC, ACS, and ASSC were the "true cause" for 36% (n=402), 6% (n=68), and 1% (n=13) of acute care visits, respectively.The results of the primary analyses are displayed in the table. Agreement between reviewers for VOC and ACS was 91% (kappa = 0.80) and 99% (kappa = 0.92), respectively. When VOC was falsely coded (n=176), the most common true diagnoses were ACS (26%), fever (24%), chronic pain exacerbation (14%), and abdominal pain (10%). When ACS was falsely coded (n=21), VOC was the most common true diagnosis (71%). Of 25 (2.2%) patients incorrectly coded for SCD, 14 had sickle trait.ConclusionsICD-9CM coding for VOC demonstrated poor accuracy; however, coding for ACS and ASSC was remarkably sensitive and specific. Genotype coding lacked sensitivity with otherwise variable results. Unfortunately, coding for SCD in ICD-10 differs minimally from ICD-9CM. Therefore, these data provide an impetus to restructure ICD coding for SCD.Table 1Sensitivity (95% CI)Specificity (95% CI)PPV(95% CI)NPV(95% CI)Postive Likelihood Ratio(95% CI)Negative Likelihood Ratio(95% CI)VOC91.8(88.6-94.2)75.0(71.6-78.2)67.7(63.6-71.6)94.1(91.8-95.9)2.1(1.8-2.4)0.06(0.04-0.08)ACS92.6(83.0-97.3)98.0(96.9-98.7)75.0(64.1-83.5)99.5(98.8-99.8)3.0(2.0-4.4)0.004(0.002-0.01)ASSC92.3(62.1-99.6)99.8(99.3-100.0)85.7(56.2-97.5)99.9(99.4-100.0)6.0(1.6-22.0)0.0009(0.0001-0.006)HbSS genotype74.2(70.9-77.2)67.7(62.5-72.6)83.8(80.7-86.4)53.8(49.0-58.6)5.1 (4.2-6.1)0.9(0.8-1.0)HbSC genotype31.0(24.9-37.7)99.6(98.8-99.9)94.3(85.3-98.2)85.8(83.5-87.9)16.5(6.4-42.8)0.16(0.14-0.19)HbS-BetaThalgenotype57.1(45.4-68.2)99.6(98.9-99.9)91.7(79.1-97.3)96.9(95.6-97.8)11(4.3-28.2)0.03(0.02-0.05) DisclosuresMcCavit:Pfizer: Research Funding; Novartis: Speakers Bureau; Novartis: Speakers Bureau; Gensavis LLC: Research Funding; Pfizer: Research Funding.

Leading change: evidence-based transition.

The purpose of this article was to provide a framework for evidence-based transition of patient populations within an acute care pediatric institution. Transition within a hospital is foreseeable, given the ever-changing needs of the patients within an evolving healthcare system. These changes include moving patient populations because of expansion, renovation, or cohorting similar patient diagnoses to provide care across a continuum. Over the past 1 to 2 years, Children's Health Children's Medical Center Dallas has experienced a wide variety of transition. To provide a smooth transition for patients and families into new care areas resulting in a healthy work environment for all team members. The planning phase for patient population moves, and transition should address key aspects to include physical location and care flow, supplies and equipment, staffing model and human resources (HR), education and orientation, change process and integrating teams, and family preparation. It is imperative to consider these aspects in order for transitions within a healthcare system to be successful. During a time of such transitions, the clinical nurse specialist (CNS) is a highly valuable team member offering a unique perspective and methodological approach, which is central to the new initiative's overall success. The themes addressed in this article on evidence-based transition are organized according to the CNS spheres of influence: system/organization, patient/family, and nursing. An evidence-based transition plan was developed and implemented successfully with the support from the CNS for 3 patient populations. Organizational leadership gained an increased awareness of the CNS role at the conclusion of each successful transition. The CNS plays a pivotal role as clinical experts and proponents of evidence-based practice and effects change in the system/organization, nursing, and patient/family spheres of influence. While transitions can be a source of stress for leaders and bedside staff, it is also a time that allows for growth and new opportunities for staff and may result in development of a healthier work environment. The CNS is able to provide leadership while working collaboratively to oversee the moves with a forward-thinking approach. There are key components to consider during times of transition. These include (1) organize, plan, and improve work efficiencies during a construction build; (2) identify the key elements for improvement in nurse and patient satisfaction; (3) develop or maintain healthy work environment standards; (4) establish adequate staffing levels and staff education to successfully care for patient populations following transition; and (5) support the staff and patients during transition.

School Competence and Fluent Academic Performance: Informing Assessment of Educational Outcomes in Survivors of Pediatric Medulloblastoma

Academic difficulties are widely acknowledged but not adequately studied in survivors of pediatric medulloblastoma. Although most survivors require special education services and are significantly less likely than healthy peers to finish high school, measured academic skills are typically average. This study sought to identify potential factors associated with academic difficulties in this population and focused on school competence and fluent academic performance. Thirty-six patients (ages 7–18 years old) were recruited through the Departments of Neurosurgery and Neuro-Oncology at Children's Medical Center Dallas and Cook Children's Medical Center in Fort Worth, TX. Participants completed a neuropsychological screening battery including selected Woodcock-Johnson III Tests of Achievement subtests. Parents completed the Child Behavior Checklist. School competence was significantly correlated with measured academic skills and fluency. Basic academic skill development was broadly average, in contrast to significantly worse fluent academic performance. School competence may have utility as a measure estimating levels of educational success in this population. Additionally, academic difficulties experienced by childhood medulloblastoma survivors may be better captured by measuring deficits in fluent academic performance rather than skills. Identification of these potential factors associated with educational outcomes of pediatric medulloblastoma survivors has significant implications for research, clinical assessment, and academic services/interventions.

A Comparison of Accidental and Nonaccidental Trauma: It Is Worse Than You Think

BackgroundChild abuse, or nonaccidental trauma (NAT), is a major cause of pediatric morbidity and mortality, and is often unrecognized. Our hypothesis was that injuries due to accidental trauma (AT) and NAT are significantly different in incidence, injury, severity, and outcome, and are often unrecognized. ObjectiveOur aim was to carry out an examination of the differences between pediatric injuries due to AT and NAT regarding incidence, demographics, injury severity, and outcomes. MethodsA 4-year retrospective review of the Trauma Registry at Children's Medical Center Dallas, a large Level I pediatric trauma center, comparing incidence, age, race, trauma activation, intensive care unit (ICU) need, Injury Severity Score (ISS), and mortality between AT and NAT patients was carried out. ResultsThere were 5948 admissions, 92.5% were AT and 7.5% were NAT victims. The NAT patients were younger (1.8 ± 3.3 years vs. 6.8 ± 4.2 years for AT patients; p < 0.01), more often required an ICU stay (NAT 36.5% vs. 13.8% for AT patients; p < 0.0001), and had a higher ISS 14.0 ± 9.7 vs. 7.5 ± 7.2; p < 0.0001). The mortality rate in NAT was 8.9% vs. 1.4% for AT (p < 0.001). Of the 40 NAT patients who ultimately died, 17.5% were not initially diagnosed as NAT. ConclusionsNAT victims differ significantly from the AT patients, with a greater severity of injury and a 6-fold higher mortality rate. Delayed recognition of NAT occurred in almost 20% of the cases. It is generally accepted that NAT is underestimated. Its increased mortality rate and severity of injury are also not well recognized compared to the typical pediatric trauma child.

Hepatopulmonary Syndrome in the Presence of Abernethy Malformation: A Pediatric Case Report

Duke University Kimberly Saulters, Pediatric Nurse Practitioner, Pediatric Intensive Care Unit, Children's Medical Center Dallas, Dallas, TX. Kristin Hittle, Pediatric Nurse Practitioner, Pediatric Intensive Care Unit, Children's Medical Center Dallas, Dallas, TX.

Transverse Myelitis Plus Syndrome and Acute Disseminated Encephalomyelitis Plus Syndrome

Classically, transverse myelitis and acute disseminated encephalomyelitis are considered central nervous system demyelinating conditions. In both conditions, the spinal cord is involved to varying degrees, and there is a variety of presentations, usually involving some degree of progressive paralysis of the upper and/or lower extremities. Treatment usually consists of high-dose intravenous steroids in addition to plasma exchange and/or intravenous immunoglobulin. In some cases, immunosuppressive medications, such as intravenous cyclophosphamide, have been used with variable success. Cases with atypical features on examination, imaging, or with neurophysiological studies may be helpful in shedding light on the etiology and/or pathophysiology because many of these patients have permanent disabilities despite appropriate treatment. This case series presents 5 pediatric cases observed from 2009-2012 at our medical center, Children's Medical Center Dallas. These cases were notable because they provided evidence of autoimmune events affecting the central nervous system but with additional peripheral axonal pathology. We describe these cases with respect to findings that suggest a variant of these conditions that have concomitant nerve-root involvement. These patients had worse outcomes than typical patients with transverse myelitis/acute disseminated encephalomyelitis, and these observations build on previous work by other investigators that highlighted persistent flaccid paralysis and electrophysiological evidence of axonal loss portending a poorer prognosis. Furthermore, these cases suggest a potential role for approaching how we classify subtypes of transverse myelitis and acute disseminated encephalomyelitis.

Late and very late onset group B Streptococcus sepsis: one and the same?

This study aimed to describe the clinical characteristics of group B Streptococcus (GBS) sepsis in infants aged 4-90 days [late onset (LO)] compared to infants >90 days of age [very late onset (VLO)]. Microbiology records at Children's Medical Center Dallas were screened. Demographic, clinical, and outcome data were collected for infants with GBS recovered from blood or cerebrospinal fluid culture from January 1, 2006 to July 1, 2012. Totally 48 infants were identified (42 LO, 6 VLO). Infants with VLO sepsis had lower median gestational age (28.5 vs. 39 weeks gestation, P<0.001) and longer median nursery admissions (8.8 vs. 0.5 weeks, P=0.004). When gestational age was controlled for, there were no differences in clinical presentation, intensive care unit admission, length of stay, neurodevelopmental outcome, and mortality. Infants with VLO sepsis were more likely to receive vancomycin (83% vs. 33%, P=0.02) or third-generation cephalosporins (83% vs. 24%, P=0.009), and more likely to continue on those agents even after GBS was identified. Infants with VLO sepsis had lower gestational ages and longer nursery stays than infants with LO sepsis. Beyond age at presentation, there were no significant differences in clinical presentations, hospital course, frequency of neurodevelopmental sequelae, and mortality in infants presenting with LO vs. VLO GBS sepsis. Infants with VLO sepsis were more likely to receive empiric broad spectrum antimicrobials and more likely to continue receiving broad therapy even following GBS identification.

Blood pressure abnormalities in children with sickle cell anemia

Kidney disease is an important cause of morbidity and mortality in patients with sickle cell anemia (SCA). The factors that affect progression of renal disease are unknown, especially in children and adolescents. Alterations in blood pressure, including hypertension and lack of the normal nocturnal dip in blood pressure, are important determinants of diabetic nephropathy and other renal diseases and may play a role in sickle cell nephropathy. Our primary hypothesis was that children with SCA who have microalbuminuria will demonstrate less nocturnal dipping of blood pressure compared to patients without microalbuminuria. We also investigated other potential factors associated with microalbuminuria. This prospective study of 52 adolescents with SCA followed in the Children's Medical Center Dallas Comprehensive Sickle Cell Center characterized 24-hour ambulatory blood pressure profiles and presence of microalbuminuria. Stepwise logistic regression was performed to identify significant independent factors that are associated with microalbuminuria. Thirty-five percent of patients were identified as having previously unrecognized hypertension, and 17% had pre-hypertension (blood pressure greater than the 90th percentile but less than the 95th percentile). Fifty-six percent of patients lacked the normal nocturnal dip in blood pressure. In addition, 21% had microalbuminuria, and their percent nocturnal dip was significantly less than those without microalbuminuria (P = 0.01). Blood pressure abnormalities are common in adolescents with SCA and are a possible modifiable risk factor in the progression of sickle cell nephropathy.

Healthcare Epidemiology Practicum Rotation for Postgraduate Physician Trainees in Medicine–Infectious Diseases

Practicum education in healthcare epidemiology and infection control (HEIC) for postgraduate physician trainees in infectious diseases is necessary to prepare them to be future participants and leaders in patient safety. Voss et al suggested that training in HEIC should be offered as a “common trunk” for physicians being trained in clinical microbiology or infectious diseases. A 1-month rotation has been recommended previously. A survey by Joiner et al indicated that only 50% of infectious diseases fellows found the infection control training adequate. The objective of this article is to report our 2-year experience with a 1-month practicum rotation we designed and implemented at our institution.The setting is the Adult Infectious Diseases fellowship program at the University of Texas Southwestern Medical Center (UTSW), Dallas, Texas. The fellows have clinical rotations at the Parkland Health and Hospital System, UTSW University hospitals, North Texas Veterans Affairs Health Care System, and Children's Medical Center Dallas. The 2-year program recruits 7 fellows every 2 years. The 1-month core rotation was established in July 2011 and is ongoing. Fellows who completed the rotation during the period July 2011 to April 2013 are included in this study.

Association of hospital construction with the development of healthcare associated environmental mold infections (HAEMI) in pediatric patients with leukemia

Healthcare associated mold infections (HAEMI) increase morbidity and mortality in children with leukemia. Excavation adjacent to Children's Medical Center Dallas (CMCD) April 2006-February 2007 provided an opportunity to determine if excavation adjacent to a hospital building is associated with increased risk of developing HAEMI in children receiving intensive chemotherapy for acute leukemia. Children who began receiving intensive chemotherapy for acute leukemia at CMCD from 2004 to 2008 were identified (n = 275). Exposures to the CMCD campus during intensive chemotherapy and duration of neutropenia per exposure were recorded. Proven, probable, or possible invasive fungal disease (IFD) was classified using EORTC/MSG guidelines. Institutional guidelines categorized mold infections as definite or possible HAEMI. A bivariate time-to-event model compared the association of excavation with HAEMI and yeast infections, controlling for neutropenia. There were 7,454 CMCD exposures, 1,007 (13.5%) during excavation. Of 50 cases of IFD, 31 were HAEMI. By time-to-event analysis exposure to the CMCD campus during the excavation period was significantly associated with HAEMI (HR = 2.8, P = 0.01) but not yeast infections (HR = 0.75, P = 0.75). Neutropenia was significantly associated with both HAEMI and yeast infections (P < 0.001). Voriconazole prophylaxis did not prevent HAEMI in 42% of the 14 patients with AML who had been receiving this agent. This study is the first to demonstrate an association between exposure to hospital construction that includes excavation and HAEMI in pediatric oncology patients. Since neutropenic patients need protection from aerosolized fungal spores during visits to expanding medical centers, preventive strategies with adherence monitoring need additional study.

Splenectomy in Children With “Mild” Hereditary Spherocytosis

Splenectomy is considered the treatment of choice for patients with "symptomatic" hereditary spherocytosis (HS). Published guidelines offering recommendations for splenectomy in HS categorize patients primarily based on hemoglobin concentration. We performed a retrospective review of 64 children having splenectomy for HS at Children's Medical Center Dallas. On the basis of hemoglobin concentration alone, 16 children (25%) had mild, 38 (59%) moderate, and 10 (16%) severe HS. However, when reticulocyte count was used to categorize disease severity, only 3 patients (5%) having splenectomy would be considered mild, 17 (27%) moderate, and 42 (66%) severe. Despite otherwise having "mild" disease defined by near-normal hemoglobin levels, many children with nontraditional or subjective signs and symptoms related to hemolytic rate received a splenectomy, and, therefore, reticulocyte count might be a more reliable laboratory marker suggesting consideration of splenectomy. A validated assessment tool incorporating quality of life indicators in addition to the traditional medical indications for splenectomy in HS would be valuable in assessing indications for the procedure.

Low Morbidity and Mortality in Children with Diabetic Ketoacidosis Treated with Isotonic Fluids

To assess current rates of complications of diabetic ketoacidosis (DKA), particularly cerebral edema, in a large tertiary-care pediatric hospital with a consistent management protocol. We report our single-center retrospective experience with 3712 admissions with DKA in 1999-2011. Our DKA protocol features a "3-bag" system using 2 bags of rehydration fluids, identical except for the presence in 1 bag of 10% dextrose, to allow rapid adjustment of glucose infusion rate. The third bag contains insulin. Fluids are administered at a total rate of 2-2.5 times "maintenance" fluid requirements. Total electrolyte concentration is kept approximately isotonic. Billing and medical records databases at Children's Medical Center Dallas were examined for cases of DKA, cerebral edema, other morbidities, and death. We ascertained 20 cases of cerebral edema (0.5%). Most presented early (median duration of treatment 2 hours). Only 10 of 20 computed tomography scans were graded as moderate edema or worse. Only 10 patients received treatment other than routine DKA management. There was 1 death in a patient with sickle cell trait who developed intravascular sickling. Two patients had neurologic sequelae at hospital discharge but both recovered fully. Compared with data in recent consensus statements, the Dallas protocol is associated with extremely low rates of death and disability (0.08% vs 0.3%) from DKA.

Tolvaptan Increases Serum Sodium in Pediatric Patients With Heart Failure

This study aimed to evaluate the use of tolvaptan in a consecutive series of pediatric patients with heart failure. Patients 18 years of age or younger with heart failure prescribed tolvaptan between January 2009 and October 2011 were retrospectively identified at Children's Medical Center Dallas. Laboratory parameters, urine output, fluid balance, and concurrent medications were recorded at baseline and at specified intervals after a single dose of tolvaptan. The 28 patients in the study had a median age of 2 years (range 1 month-18 years). The median tolvaptan dose administered was 0.3 mg/kg (range 0.1-1.3 mg/kg). The study patients had a median baseline serum sodium concentration of 127 mmol/L, and the increases in sodium were 2.5 mmol/L at 12 h, 5 mmol/L at 24 h, 4 mmol/L at 48 h, and 5 mmol/L at 72 h (all p < 0.001). Urine output was increased at 24 h (p < 0.001) and 48 h (p = 0.03), and fluid balance changes were significantly different at 24 h (p = 0.004). The changes in potassium, blood urea nitrogen, and serum creatinine were not significant at any interval. When controlling for traditional diuretic therapy, increases in serum sodium concentration and urine output remained statistically significant. A single dose of tolvaptan increased serum sodium concentrations for the majority in this small series of pediatric patients with heart failure. These results suggest that tolvaptan can be safely and effectively administered to pediatric patients. Prospective, randomized controlled trials are needed to evaluate the safety and efficacy of its use further.