Phoenix Children's Hospital Research Articles

Editorial introductions

Editorial introductions

Asthma treatment and outcomes for children in the emergency department and hospital

ABSTRACTObjective: To describe and compare the treatment of acute asthma exacerbations in children given in the emergency department (ED) and admitted to acute care floor in the hospital or intensive care unit (ICU). Methods: A retrospective chart review of visits for acute exacerbation of asthma treated at Phoenix Children's Hospital between January 1, 2014 and December 31, 2016. Results: A total of 287 asthma exacerbation cases were identified including 106 (37%) ED visits, 134 (47%) hospital floor and 47 (16%) ICU admissions. A history of a previous ED visit (ED 88%, Floor 60% and ICU 68%; p < 0.0001) and prior pulmonology inpatient consultation (ED 30%, Floor 19% and ICU 15%; p = 0.05) varied significantly. Pulmonology inpatient consultations were performed more frequently in the ICU than on the hospital floor (54% versus 8%; p < 0.0001). Although overall 145 (51%) of the cases were already on inhaled corticosteroids (ICS) at the time of visit with no differences across locations, ICS initiation/step-up was greater in the ICU (72%) than on the hospital floor (54%) and ED (2%) (p < 0.0001). A recommendation given to the family for follow-up with pulmonology was more frequent for patients who had been admitted to the ICU (68%) as compared to those only admitted to the floor (31%) or ED (4%) (p < 0.0001). Readmission rates were similar for patients previously admitted to the hospital (Floor 42%; ICU 40%), but significantly higher for previous ED visits (77%) (p < 0.0001). Conclusions: Physicians in the ED have an opportunity to provide preventative care in the acute care setting and should be encouraged to initiate treatment with ICS. Consideration should be given to develop a program or clinical pathway focused on long-term asthma management and maintenance to reduce readmissions and long hospital stays.

Clinical Presentation and Outcomes Associated with Different Treatment Modalities for Pediatric Bark Scorpion Envenomation.

Scorpion envenomation is potentially life-threatening and affects children in the Southwestern USA. An FDA-approved antivenom is available, but its high cost has led to use of off-label antivenom dosing or supportive care alone as alternatives to FDA-recommended dosing. This study sought to determine whether treatment approach influences outcomes in envenomated children. A retrospective cohort study of children with grade III or IV scorpion envenomation evaluated in Phoenix Children's Hospital ED between September 1, 2011, and March 31, 2014. Patients were grouped based on treatment: group 1, supportive care only; group 2, FDA-recommended dosing (3-vial initial dose); group 3, "off label" dosing (1-2 vial initial dose). Primary outcomes were ED length of stay and hospital admission. Secondary outcomes were mechanical ventilation and aspiration pneumonia. One hundred fifty-six patients were included with 58 patients in group 1, 16 patients in group 2, and 82 patients in group 3. Group 1 was significantly older than the antivenom groups (p<0.001), and group 2 was younger than group 3 (p=0.024). Envenomation grade was also different, with group 1 having fewer grade IV then groups 2 and 3 (p<0.001). Three percent of group 1, 56% of group 2, and 28% of group 3 had respiratory distress (p<0.001). ED LOS was not significantly different between groups. Hospital admission occurred in 3.4% group 1, no group 2, and 8.5% group 3 patients. Two intubations and two aspirations occurred in group 3. In this study, clinical presentation appeared to influence treatment. Groups that received antivenom had a higher envenomation grade than the group that received supportive care. The FDA-recommended dosing group was younger and had more respiratory distress than those treated with initial doses of 1-2 vials. Outcomes were not significantly different between groups. Prospective studies may identify the ideal population for each treatment approach.

Abstracts from The 34(th) Annual National Neurotrauma Symposium June 26-29, 2016 Lexington, Kentucky.

Abstracts from The 34(th) Annual National Neurotrauma Symposium June 26-29, 2016 Lexington, Kentucky.

Symptoms of cobalamin activation disorders in relation to ethnicity and Native American tribal affiliation: An exploratory study

The Cobalamin activation disorders (CblA‐CblG) are deleterious inborn errors of Vitamin B12 activation, resulting in severe intellectual disability, vision impairment, and seizures. Although cobalamin activation disorders are exceedingly rare, typically occurring 1 in every 200,000 births, Phoenix Children's Hospital has an unusually high number of patients (n=21). We conducted a retrospective, exploratory examination of medical records to identify symptom trends in individuals clinically diagnosed with CblC (n=16) in relation to race, ethnicity, and tribal affiliation. This study is unique due to our direct access to a large population of children with this condition, the majority of whom are Native American (n=6) or Hispanic (n=8), while the remaining 2 are of Caucasian descent. We also examined the symptoms of two Native American children believed to have a novel mutation related to cobalamin metabolism. Of the 16 individuals clinically diagnosed with CblC, those with the most severe eye abnormalities tended to be of Athabascan descent. In addition, growth delays were present in 5 children, with no apparent connection to race or ethnicity. Finally, two children believed to exhibit a novel mutation related to cobalamin activation, who are both of the same Native American tribal (non‐Athabascan) descent, display no ophthalmological symptoms. Findings from this study will expand the clinical understanding of this disease and will shed light on the impact of these conditions in minority populations. In addition, these results lay the foundation for future research examining associations between symptoms, symptom severity, and genetic analysis data. The ultimate goal of this research is to contribute to the Cobalamin activation section of a gene chip that addresses genetic disorders in Native Americans.Support or Funding InformationPhoenix Children's Hospital

Impact of Conditioning Regimen in Allogeneic Stem Cell Transplant for Acute Myeloid Leukemia: The Phoenix Children's Hospital Experience

Impact of Conditioning Regimen in Allogeneic Stem Cell Transplant for Acute Myeloid Leukemia: The Phoenix Children's Hospital Experience

Outcomes of thoracoscopic thymectomy in patients with juvenile myasthenia gravis

IntroductionMyasthenia gravis (MG) is an autoimmune disorder of the postsynaptic neuromuscular junction resulting in fatigability of voluntary muscles. There has been increasing evidence supporting thymectomy for MG in adults, and evidence for the role of surgery in pediatric age groups is increasing. The purpose of this study is to describe the outcomes of our patients with juvenile MG undergoing thoracoscopic thymectomy. Material and methodsAll patients with juvenile MG who underwent thoracoscopic thymectomy at Phoenix Children's Hospital between 1999 and 2014 were included. Patients were diagnosed by their treating neurologist. An Osserman and Genkins criterion was used to classify the severity of the disease and DeFilippi classification was used to assess remission. ResultsTwelve patients underwent thoracoscopic thymectomy for juvenile MG during the time frame studied. Nine (75%) patients had an Osserman stage of IIB, with only two patients with ocular disease. There were no conversions to an open procedure. Seven (59%) patients had normal thymic histology, 4 (33%) had evidence of follicular hyperplasia and one (8%) had involutional changes. The median length of hospital stay was 2days (range 1–5days). There was no 30-day postoperative morbidity, reoperations or mortality. The median length of follow-up was 31months (range, 4–91months) and at the time of their last follow-up; all 12 (100%) patients had a DeFilippi Classification of 3 or better. ConclusionSurgery for MG in children is indicated for antibody-receptor-positive patients with moderate to severe disease. Thoracoscopic thymectomy is a safe and acceptable treatment for juvenile MG with good disease control. The low morbidity and shorter hospital duration make it an excellent option for consideration.

Review of Prevention for Pediatric and Adolescent Migraine.

Authors from the Barrow Neurological Institute at Phoenix Children's Hospital present a narrative overview of preventive treatment for pediatric and adolescent migraine.

Establishing Measurement Invariance

Registered nurses and nurse researchers often use questionnaires to measure patient outcomes. When questionnaires or other multiple-item instruments have been developed using a relatively homogeneous sample, the suitability of even a psychometrically well-developed instrument for the new population comes into question. Bias or lack of equivalence can be introduced into instruments through differences in perceptions of the meaning of the measured items, constructs, or both in the two groups. To explain measurement invariance and illustrate how it can be tested using the English and Spanish versions of the Paediatric Asthma Quality of Life Questionnaire (PAQLQ). A sample of 607 children from the Phoenix Children's Hospital Breathmobile was selected for this analysis. The children were of ages 6-18 years; 61.2% completed the PAQLQ in Spanish. Testing measurement invariance using multiple-group confirmatory factor analysis, a series of hierarchical nested models, is demonstrated. In assessing the adequacy of the fit of each model at each stage, both χ2 tests and goodness-of-fit indexes were used. The test of measurement invariance for the one-factor model showed that the English and Spanish versions of the scale met the criteria for measurement invariance. The level of strict invariance (equal factor loadings, intercepts, and residual variances between groups) was achieved. Confirmatory factor analysis is used to evaluate the structural integrity of a measurement instrument; multiple confirmatory factor analyses are used to assess measurement invariance across different groups and to stamp the data as valid or invalid. The PAQLQ, a widely used instrument having evidence to support reliability and validity was used separately in English- and Spanish-speaking groups. Traditional methods for evaluating measurement instruments have been less than thorough, and this article demonstrates a well-developed approach, allowing for confident comparisons between populations.

Infantile hemangiomas treated with propranolol: Description of the Phoenix Children's Hospital experience

Infantile hemangiomas treated with propranolol: Description of the Phoenix Children's Hospital experience

It is what it was*

Phoenix Children's Hospital; University of Arizona School of Medicine; Phoeniz, AZ The author has not disclosed any potential conflicts of interest.

Transition of Adolescents to Young Adulthood for Vulnerable Populations

1. Tressia M. Shaw, MD* 2. David E. DeLaet, MD, MPH† 1. *Director, Teaming Up for Transition, Phoenix Children's Hospital; Clinical Assistant Professor of Pediatrics, University of Arizona College of Medicine, Phoenix, Ariz. 2. †Assistant Professor of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, NY. After completing this article, readers should be able to: 1. Define the transition process and the populations for whom it is most critical. 2. Identify barriers to transition for the practitioner, patient, and family and how to address them best. 3. Review existing legislation and local support services available to aid transition in the domains of education, vocation, and independent living for vulnerable populations. 4. Describe the central role played by physicians in the transition process. Transition is defined as the movement from adolescence to adulthood in all areas, including home, health care, education, and community. Transition should be a process rather than an event. Optimally, a child, his or her family, and the practitioner should be preparing for transition throughout childhood and adolescence. Although all adolescents make this transition, this article focuses on the transition process for vulnerable populations, including those who have chronic medical conditions, intellectual disabilities, and mental health conditions. With advances in technology and medical care, many children who have serious chronic medical conditions now are living into adulthood. It is estimated that more than 500,000 youth in the United States who have special health-care needs are entering adulthood every year, with more than 90% born in the 1990s surviving into adulthood. In addition, an estimated 2.4 million noninstitutionalized adults ages 18 to 26 years are living with serious mental illness. In 2002, a consensus statement from the American Academy of Pediatrics, American Academy of Family Physicians, American College of Physicians, and American Society of Internal Medicine called for critical first steps to improve the transition process to adult-oriented health care (Table 1). (1) However, since that time, relatively little …

0016—Adventures in Endocrine Electronic Medical Records: The Phoenix Children's Hospital Experience

0016—Adventures in Endocrine Electronic Medical Records: The Phoenix Children's Hospital Experience

Surgical management of coccidioidomycosis in children

Background/Purpose: Operations for Coccidioides immitis infection in children have received little study. The authors perceived an increase in the number of patients requiring operations for coccidioidomycosis at Phoenix Children's Hospital. The authors therefore reviewed the surgical management of children with C immitis infection in Phoenix, Arizona. Methods: The medical records of all children hospitalized from 1994 to 1999 with a confirmed laboratory diagnosis of coccidioidomycosis were reviewed. Age, gender, race, immune status, laboratory data, operations, and outcome were recorded. Results: Of 39 children with proven coccidioidomycosis, 21 patients (54%) underwent a total of 49 operations. Boys represented 72% of all children reviewed. Sixteen patients (29% of the operative cohort) were immunocompromised. Thoracic procedures (19 cases) were most frequent. Conclusions: Over half the children hospitalized for coccidioidomycosis required at least 1 operation. Most were immunocompetent boys with thoracic disease. Pediatric surgeons, who provide the bulk of operative care, should expect to encounter coccidioidomycosis in endemic areas, and increasingly in nonendemic areas. J Pediatr Surg 35:1633-1634. Copyright © 2000 by W.B. Saunders Company.

Nonsurgical, nonorthotic treatment of occipital plagiocephaly: what is the natural history of the misshapen neonatal head?

Management of neonates with nonsynostotic occipital plagiocephaly has been controversial, and there has been a lack of uniformity concerning its treatment. Patients with nonsynostotic occipital plagiocephaly have been treated surgically or with cranial remodeling orthotic devices and have shown improvement in asymmetry. The cost of orthotic treatment has risen, and its validity has been contested by many third-party insurance payers. The effectiveness of orthotic treatment has not been adequately compared to the natural history of nonsynostotic occipital plagiocephaly. A nonsurgical, nonorthotic treatment study was initiated in June 1995 at Phoenix Children's Hospital. All new patients referred with a diagnosis of nonsynostotic occipital plagiocephaly were categorized into two groups: those with mild-to-moderate asymmetry and those with moderate-to-severe asymmetry. Categories were determined by cephalic measurements. The patients with moderate-to-severe asymmetry were offered orthotic treatment with a cranial remodeling band. Those patients with mild-to-moderate asymmetry were treated with physiotherapy, repositioning of the head, and repeated notation of cephalic measurements without orthotic devices or surgery. Seventy-two neonates, seen consecutively, with mild-to-moderate, nonsynostotic occipital plagiocephaly were evaluated by noting cephalic measurements. The parents of six of these patients elected treatment with a cranial remodeling band and results in these patients were excluded from our data. The remaining 66, treated without orthotic devices, showed improvement in average cranial vault asymmetry (CVA) from 9.2 to 4.7 mm over an average treatment period of 4.5 months that commenced when the average age of the patient was 6.4 months. A comparison of the present data with data published in 1994 for neonates treated with a headband indicates that neonates with mild-to-moderate asymmetry who are treated aggressively with physiotherapy and repositioning have similar improvement in CVA.

Nonsurgical, nonorthotic treatment of occipital plagiocephaly: what is the natural history of the misshapen neonatal head?

Plagiocephaly has recently received increased attention in pediatric neurosurgical circles and its treatment has been controversial. Lack of uniformity persists in the treatment of neonates with occipital plagiocephaly. Plagiocephaly resulting from synostosis is rare. Many patients with nonsynostotic plagiocephaly have been treated surgically. Cranial remodeling orthotic devices (such as headbands and helmets) have also been reported to correct nonsynostotic plagiocephaly. The cost of orthotic treatment has risen and its validity has been contested by many third-party insurance payers. The effectiveness of orthotic treatment has not been adequately compared to the natural history of nonsynostotic plagiocephaly. The first phase of a natural history study was initiated in June 1995 at Phoenix Children's Hospital. All new patients referred with a diagnosis of plagiocephaly were categorized into two groups: those with mild-to-moderate asymmetry and those with moderate-to-severe asymmetry. Categories were determined by cephalic measurements. The patients with moderate-to-severe asymmetry were offered orthotic treatment with a cranial remodeling band. Those patients with mild-to-moderate asymmetry were observed with repeated measurements. Data in patients left untreated and followed for natural history were compared to our data published in 1994 for those treated with external cranial orthosis. Our published data in 47 patients treated with a headband during the period from 1988 to 1993 indicated that cranial vault asymmetry improved from a mean of 8.9 mm to 4 mm over an average treatment period of 4.3 months beginning when the average age of the patient was 5.9 months. Seventy-two neonates treated with neck stretching exercises and repositioning of the head without surgery or orthotic devices showed improvement in cranial vault asymmetry from 10.6 mm to 5.5 mm over an average treatment period of 4.5 months beginning when the average age of the patient was 4.5 months. This study indicates that neonates with mild-to-moderate asymmetry who are less than 6 months of age and who are treated with physiotherapy and repositioning have similar improvement in cranial vault asymmetry as those treated with orthotic devices. The second phase of our study deals with neonates who have moderate-to-severe plagiocephaly.

Through the eyes of a child: Teresa L. Boeger, MS, CCLS/CTRS, Child Life Manager, Phoenix Children's Hospital, Phoenix, Arizona

Through the eyes of a child: Teresa L. Boeger, MS, CCLS/CTRS, Child Life Manager, Phoenix Children's Hospital, Phoenix, Arizona

CAUSALITY OF PEDIATRIC SUBMERSION INJURIES

Division of Critical Care, Phoenix Children's Hospital, 1111 East McDowell Road, Phoenix, Arizona 85006

MEASUREMENT OF REGIONAL CEREBRAL BLOOD FLOW (rCBF) IN PEDIATRIC HEAD INJURY

Department of Pediatric Intensive Care, Phoenix Children's Hospital, 1111 East McDowell Road, Phoenix, Arizona 85006.

THE USE OF STATIC COMPLIANCE (Csta) TO PREDICT VENTILATOR DEPENDENCE IN STATUS POST OPEN HEART SURGERY IN INFANTS WITH DOWNʼS SYNDROME

Department of Pediatric Critical Care, Phoenix Children's Hospital, 1111 East McDowell Road, Phoenix, Arizona 85006.