Arkansas Children's Hospital Research Articles

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009—Arkansas Children's Hospital Health Care Camp Program

The shortage of nurses is estimated to reach 400,000 to 800,000 by 2020. Factors contributing to the shortage are many. One factor identified is that fewer people are choosing to pursue a career in nursing. Job shadowing experiences and personal contact with health care professionals can have a beneficial impact on students in deciding on or confirming their career choice in the health care field.

Cytogenetic Findings in Pediatric T-Lymphoblastic Lymphomas: One Institution's Experience and a Review of the Literature

Cytogenetic analyses of lymphomas commonly reveal nonrandom chromosomal abnormalities, but there are relatively few reports in childhood lymphoblastic lymphoma (LL). We retrospectively reviewed G-banded karyotypic analyses performed at Arkansas Children's Hospital between 1990 and 2004. Six children (2 to 20 years old) had LL that presented as mediastinal or cervical masses and had a T-cell immunophenotype and clonal abnormalities. The cytogenetic findings in these 6 patients were as follows: 46,XX,-7,inv(9)(p11q12),der (12)t(7;12)(q11.2;p13),t(16;18)(p13.1;q21),+22 in patient 1; 47,XX,+9,del(9)(q11q22)x2 in patient 2; 72-119, XY,+X,+1,+1, inv(2) (p11q13),-3,+5,+6,+7,+10,-12,-16, -21,-21,-22,+mar in patient 3; 48,XY,+5,+20,t(7;9) (q32;q34) in patient 4; 47 approximately 48,XX,der(10)t(10;14)(q23; q11.2),+12, del(12)(p12)x2, -14,del(16)(q22q22),+?add (19)(p13.3) in patient 5; and 48 approximately 49,XY,+7,+8,t(11;19) (q23;p?13.3),+der(19)t(11;19)[cp20] in patient 6. Eleven chromosome breakpoints in 6 of our patients (7q11, 12p13, 16p13, 18q21, 9q11, 2p11, 2q13, 7q32, and 7q23) have been reported in other patients with acute lymphoblastic leukemia or LL and involved regions containing TEL, ABL, E2A, MLL, and T-cell receptor-alpha genes. A review of the cytogenetic findings of these and other cases of LL reveals that clonal aberrations are common and most frequently involve T-cell receptor gene regions. The aberrations show some features similar to those of acute lymphoblastic leukemia and are not unique to LL, thus furnishing additional evidence of the equivalence of these two diseases. The cytogenetic features of LL may be helpful in the diagnosis of pediatric lymphomas and undifferentiated neoplasms.

A Review of Pediatric Patients Treated for Septic Arthritis at Arkansas Children's Hospital

A Review of Pediatric Patients Treated for Septic Arthritis at Arkansas Children's Hospital

Anesthetic Considerations for Fracture Management in the Outpatient Setting

Editor From the Arkansas Children's Hospital, Little Rock, Arkansas. The author received no financial support for this study. Reprints: R. Dale Blasier, MD, FRCS(C), Department of Orthopaedic Surgery, Arkansas Children's Hospital, Slot 839, 800 Marshall Street, Little Rock, AR 72202 (e-mail: [email protected]).

Control of Methicillin-Resistant Staphylococcus aureus in a Neonatal Intensive Care Unit by Modification of 2003 Society for Healthcare Epidemiology of America Guideline for Preventing Transmission of Resistant Pathogens

BACKGROUND: Invasive methicillin-resistant Staphylococcus aureus (MRSA) infection as a cause of death in hospitalized infants is rare and has not previously occurred in Arkansas Children's Hospital (ACH) neonatal intensive care unit (NICU). In August 2003, however two infants developed MRSA pneumonia and subsequently died. Anterior nares cultures during this outbreak found two additional infants colonized. In the first 7 months of 2003, the rate of MRSA among Staphylococcus aureus in ACH NICU was 75% (21/28). As part of control measures, we reviewed and modified the Society for Healthcare Epidemiology of America (SHEA) guideline for resistant pathogens published in 2003 for implementation. METHODS: After review of the guideline by the Hospital Infection Control Committee, the following interventions were selected: 1) cohorts of colonized/infected infants were established, 2) alcohol gel hand disinfectant was introduced, 3) admission surveillance cultures of the anterior nares were performed, 4) point prevalence surveys were conducted, and 5) an antibiotic susceptibility pattern, DNA sequence analysis of the staphylococcal protein A gene (SPA), and pulsed-field gel electrophoresis (PFGE) were reviewed to determine the similarity of MRSA isolates. RESULTS: Antibiotic Susceptibility pattern, SPA, and PFGE revealed that one pattern was responsible for this cluster of invasive disease in the initial group of infants. A point prevalence survey conducted in September of current high-risk NICU patients found four infants (hospitalized 25–197 days) colonized with MRSA; none matched the outbreak isolate. Because of the concern of unrecognized MRSA from other hospitals, admission cultures were collected between October and December. Three newly admitted infants were colonized with MRSA – two 25-week-premature newborns (day of life 58 and 10) and one 38-week-premature newborn (day of life 1) from separate facilities. Another point prevalence survey in December found two infants colonized (admission culture negative); none matched the outbreak isolate. The rate of MRSA among Staphylococcus aureus had declined to 45% (18/40) by January 2004. CONCLUSION: Even though it is labor-intensive and costly to implement (microbiology laboratory charges totaled $19,903.02), this SHEA guideline was a crucial component for control of MRSA in our NICU.

Optic radiation involvement in optic pathway gliomas in neurofibromatosis

PurposeOptic pathway gliomas (pilocytic astrocytomas) in neurofibromatosis type 1 (NF-1) typically involve some combination of the optic nerves, chiasm, or optic tracts. Involvement of the optic radiations is rare. DesignThis paper describes seven patients with NF-1 with gliomas involving the pregeniculate optic pathway in addition to the optic radiations. MethodsA retrospective database review was made of all patients with NF-1 and optic pathway gliomas seen by one of the authors (G.T.L.) at the Children's Hospital of Philadelphia from July 1993 to October 2001. Patients with involvement of pregeniculate optic pathway and the optic radiations were identified. From November 2001 to February 2003, patients were sought prospectively. Cases were also identified from the practice of another author (M.C.B.) at Arkansas Children's Hospital. ResultsFour patients from Children's Hospital of Philadelphia (three of 83 total NF-1/optic pathway gliomas from July 1993 to October 2001 and one prospectively) and three from Arkansas Children's Hospital were identified. Two had expanding mass lesions within the white matter of the temporal or parietal lobes, which were histopathologically demonstrated to be pilocytic astrocytomas. The other five had radiographic involvement of the optic radiations but did not undergo biopsy. In three of the cases the vision was 20/200 or worse in each eye. ConclusionsOptic pathway gliomas in NF-1 may rarely involve the optic radiations. Optic radiation involvement may signal a more aggressive optic pathway glioma in patients with neurofibromatosis-1.

Arterial oxygen fluctuation and retinopathy of prematurity in very-low-birth-weight infants.

To determine the influence of arterial oxygen fluctuation on development of threshold ROP. Retrospective study of 231 infants, < or =1500 g birth weight, who were admitted to Arkansas Children's Hospital NICU from January 1993 to June 1995. Fluctuation in partial pressure of dissolved arterial oxygen (PaO(2)) was expressed as coefficient of variation (CoV) for each infant. We investigated the relationship between CoV at three intervals and the risk of developing threshold ROP. The odds ratio (OR) of developing threshold ROP versus prethreshold ROP or less associated with a 10% increase in the CoV during the first 5 days of oxygen therapy was 1.44, and during the first 10 days was 1.51. When analysis was restricted to infants receiving 30 days of therapy, the OR during the first 5 days of therapy was 1.67, during the first 10 days was 1.82, and during days 11-30 was 1.68. Very-low-birth-weight infants experiencing fluctuating PaO(2) are at higher risk of threshold ROP.

Invasive disease due to nontypeable Haemophilus influenzae among children in Arkansas.

In this study, we reviewed cases of invasive disease due to nontypeable Haemophilus influenzae among children hospitalized at Arkansas Children's Hospital from 1993 to 2001. A total of 28 cases were examined, including 21 associated with bacteremia and 4 associated with meningitis. Of the patients examined, 86% were </=4 years of age, and 68% had underlying medical conditions. Characterization of the bacterial isolates by multilocus sequence type genotyping revealed significant overall genetic diversity, similar to the diversity in the general population structure for nontypeable H. influenzae. However, four separate pairs of isolates were closely related genetically, a relationship confirmed by pulsed-field gel electrophoresis and Southern hybridization studies using probes for the major H. influenzae adhesin genes. These results suggest that selected strains of nontypeable H. influenzae may have more invasive potential, especially in young children and patients with underlying medical conditions. At this point, the specific factors that contribute to enhanced virulence remain unclear.

Assessing the Quality of Consumer Information Services Through Daily Log Analysis

The purpose of this study was to evaluate the resources and services of a pediatric consumer health library by analyzing data collected over a two-year period. Data gathered from May 1998 to May 2000 was analyzed under four major fields: number of requests, origin of requests, subjects of requests, sources of answers. There were a total of 359 reference questions asked. Some were initiated by patients or for patients by hospital staff (292); some were made by hospital employees (43), school nurses (11), insurance companies (8), and health agencies (5). Nurses made the most referrals to the library (78), followed by social workers and physicians (10). Birth defect questions were asked most often (88), followed by psychology (61), cancer (31), pulmonology (31), surgery (25), and others (123). Gale's Health Reference Center database was used 143 times, National Organization of Rare Diseases database was used 56 times, consumer books and videos were used 64 times, Internet was used 28 times, medical books and MEDLINE databases were used 47 times. There were seven questions that were not answered well, and seven others were not answered at all. Conclusion: Data gathered from the analysis demonstrated that the library collection was adequate in meeting the consumer health information needs in Arkansas Children's Hospital.

Parental Emotional and Time Costs Predict Compliance With Respiratory Syncytial Virus Prophylaxis

Two agents are effective in preventing respiratory syncytial virus (RSV) hospitalization in premature infants: RSV immune globulin (RSV-IG) and palivizumab. RSV-IG is associated with greater parental emotional and time costs, which may account for the more limited adherence to recommended monthly treatment with this agent. To compare the emotional distress and time costs associated with RSV-IG and palivizumab treatments and to determine the influence of these costs on treatment adherence. We surveyed parents of 82 infants who received RSV-IG (90% of eligible) and parents of 61 infants who received palivizumab (87% of eligible) at Arkansas Children's Hospital by telephone. We measured infant distress during treatment, parental distress, parental time costs, and adherence with recommended monthly prophylaxis. Half of parents of RSV-IG recipients witnessed their infant in distress during infusion, over half (61%) were upset by observing the needle stick, and 22% observed infusion in the scalp. Fewer than 5% of parents of palivizumab recipients observed their infant in distress or were themselves distressed during treatment. A quarter of parents took time off from work for RSV-IG or palivizumab treatment. RSV-IG recipients completed 62% of recommended monthly treatments compared with 86% completed by palivizumab recipients. Increased parental distress and time costs largely accounted for the reduction in adherence to monthly treatment among RSV-IG recipients. The emotional and time costs of RSV-IG treatment far exceed those of palivizumab and predict substantial differences in treatment adherence between the 2 agents. The impact of hidden costs on treatment adherence should be included in economic evaluations of medical procedures.

Should antibiotics be discontinued at 48 hours for negative late-onset sepsis evaluations in the neonatal intensive care unit?

To establish the appropriate length of antibiotic therapy for negative late-onset sepsis evaluations in the neonatal intensive care unit (NICU), based on time to detection of positive bacterial cultures. Culture results from late-onset sepsis evaluations between January 1, 1994 and June 30, 1998 from outborn neonates at the Arkansas Children's Hospital NICU were retrospectively reviewed. The time period from specimen collection to notification of NICU personnel was calculated for positive cultures. There were 2,783 blood, 724 urine, and 294 cerebrospinal fluid cultures obtained, of which 10.2%, 6.6%, and 5.4%, respectively, were positive for bacterial isolates. Of positive cultures, 98% had a time to detection < or = 48 hours. Of cultures that became positive > 48 hours, 7 of 8 grew coagulase-negative staphylococci; 4 were contaminants. Discontinuing antibiotic therapy for neonates with possible late-onset sepsis and negative cultures at 48 hours is appropriate and is now standard care in our NICU.

Meningococcal infections in children from Arkansas.

Infections with Neisseria meningitidis are an important cause of morbidity and mortality in children of all ages. With wide-spread use of the heptavalent pneumococcal conjugate vaccine, this organism might become the prominent pathogen for invasive disease in children. Retrospective reviews of medical and microbiologic records from Arkansas Children's Hospital were done to identify patients with invasive N. meningitidis infections from January, 1988, through December, 2000. Basic demographic and clinical data were gathered and reviewed. Data on invasive meningococcal infections were obtained from the Arkansas Department of Health. Three hundred ninety-four cases of invasive meningococcal infection were reported to the Arkansas Department of Health during the study period. Two hundred ninety-six cases were in patients <21 years of age. The estimated annual incidence of meningococcal disease for the State of Arkansas was calculated to be 1.2-cases/100000 population during the study period. The annual incidence of meningococcal disease in patients <21 years of age was estimated at 2.9 and 21.7 cases/100000 population for children < 1 year of age. One hundred fifty patients (51%) <21 years of age with 151 episodes of invasive meningococcal infections were treated at our institution. Eighty percent of the patients were Caucasian, 55% were male, 31% live in a rural area and the median age at presentation was 30 months (range, 2 weeks to 21 years). The most common signs and symptoms at admission included fever (95%), petechial/purpuric rash (62%), nuchal rigidity (41%) and hypotension (41%). Thirty-eight patients (26%) required both inotropic support and mechanical ventilation during hospitalization, 15 patients died and 18 patients had long term sequelae. Eighty-three of the isolates were serogrouped and included the following: A (2); B (38); C (33); and Y (10). Eighty-four of the index cases were treated with parenteral cephalosporin therapy alone and did not receive additional chemoprophylaxis. Many infections in the general pediatric population are a result of N. meningitidis. Although most patients do well and recover without sequelae, there are a significant number who experience major morbidity and mortality as a result of this infection.

Magnetic resonance diagnosis of congenital hypopituitarism in children with optic nerve hypoplasia

Purpose: To determine whether structural abnormalities of the neurohypophysis, as detected by magnetic resonance imaging (MRI), can be used to diagnose hypopituitarism in children with optic nerve hypoplasia. Methods: Retrospective analysis of 67 children with optic nerve hypoplasia who had MRI and pediatric endocrinologic evaluation at Arkansas Children's Hospital from 1989 to 1999. Results: A total of 26 children with optic nerve hypoplasia had pituitary hormone deficiency. MRI disclosed posterior pituitary ectopia in 16 of these cases, absence of the pituitary infundibulum and posterior pituitary bright spot in 7 of these cases, and a normal neurohypophysis in 3 of these cases. All 41 children with optic nerve hypoplasia and normal endocrinologic function had a normal neurohypophysis. Conclusion: MRI of the neurohypophysis can predict when congenital hypopituitarism will be associated with optic nerve hypoplasia. (J AAPOS 2001;5:275-80)

Randomized, Double-Blind, Placebo-Controlled, Food Allergy Challenge to Olestra Snacks

Following approval of the fat replacer olestra for use in preparing savory snacks, Procter & Gamble implemented a postmarketing surveillance program to monitor marketplace introduction. Three and one-half percent of all health effects reported by consumers to the surveillance toll-free number were allergy-type symptoms (e.g., rash, itching, edema, hives, dyspnea). Because of these reports, we investigated whether olestra or some component of olestra snacks was a likely allergen in some subset of the population. A single center, randomized, double-blind, placebo-controlled, within-subject crossover food challenge study was conducted to confirm or refute the allergenicity of olestra snacks. Of the 65 subjects who reported symptoms consistent with immediate hypersensitivity to olestra's postmarketing surveillance program, 14 men and women traveled to the Arkansas Children's Hospital Research Institute to participate in this study. Each subject underwent a standard skin prick test at the beginning of the study, to help determine what component, if any, of the olestra product was allergenic. Following the skin prick test, subjects ate in random order, olestra-containing potato chips and regular fat-containing potato chips. The dose of potato chips consumed at each challenge was at least the amount alleged to have caused the symptoms that prompted the consumer to phone the postmarketing surveillance toll-free number. No subject experienced an allergic reaction after consuming the olestra-containing chips. Nor did any subject elicit a positive response to olestra following the skin prick testing. Two subjects had positive reactions consistent with immediate hypersensitivity after consuming the regular-fat, placebo potato chips. The results of this study confirm that olestra is unlikely to have an allergenic potential.

Experience with an anesthesiologist interventional model for endoscopy in a pediatric hospital.

Endoscopy is now a routine part of the work-up for many patients with gastrointestinal symptoms. Adults tolerate these procedures well, with either no sedation or a relatively light level. In contrast, children often require deep sedation or a general anesthetic to successfully perform these procedures. Therefore, pediatric endoscopies may require more time, personnel, and monitoring equipment to provide optimal conditions for the patient. The goals of this retrospective case series were to describe the anesthesia times and recovery duration of the different procedures, the types and amounts of medications commonly used, and the types and rates of complications experienced. Patients (2,306) who underwent endoscopy in the Arkansas Children's Hospital endoscopy suite during a 4-year period were identified. A random sample of 720 charts was reviewed retrospectively. Patients ranged in age from younger than 1 year to 29 years. Patients most often had abdominal pain or multiple gastrointestinal symptoms. Sixty-eight percent of patients underwent esophagogastroduodenoscopies; 30% colonoscopy or a combination of the two. Ninety-five percent of patients received a propofol-based anesthetic. Midazolam, fentanyl, and alfentanil were frequently used as supplemental agents. Complications occurred infrequently and were airway related. All complications were easily treated, with no adverse sequelae. This model of anesthesiologist-provided sedation/anesthesia for gastrointestinal endoscopy procedures has been extremely successful in the Arkansas Children's Hospital and has served to heighten awareness of many issues surrounding sedation and anesthesia outside of the operating room, while ensuring a high level of care is provided.

University of Arkansas for Medical Sciences College of Medicine

University of Arkansas for Medical Sciences College of Medicine

Relationship of pediatric overall performance category and pediatric cerebral performance category scores at pediatric intensive care unit discharge with outcome measures collected at hospital discharge and 1- and 6-month follow-up assessments.

Given the current focus on outcomes, there is a crucial need for easily utilized measures that can effectively quantify morbidity or disability after a child's critical illness or injury. The purpose of this study is to significantly extend the research on two such promising measures: the Pediatric Overall Performance Category (POPC) and the Pediatric Cerebral Performance Category (PCPC). Cross-sectional analysis of a sample of pediatric intensive care unit (PICU) discharges and a prospective follow-up of this cohort of children. Arkansas Children's Hospital. Two hundred children (ranging in age from birth to 21 yrs) discharged from a PICU. None. Data were collected at PICU discharge, hospital discharge, and 1- and 6-month follow-up assessments after hospital discharge. Measures utilized included the POPC (at PICU discharge), PCPC (at PICU discharge), Stanford-Binet Intelligence Scale, fourth edition (at hospital discharge), Bayley Scales of Infant Development, second edition (at hospital discharge), and the Vineland Adaptive Behavior Scales (at 1 and 6 months after discharge). Stanford-Binet Intelligence Quotients and Bayley Mental Developmental Index scores were significantly different across PCPC categories (p < .0001). Bayley Psychomotor Developmental Index scores and Vineland Adaptive Behavior Scales scores varied significantly across POPC categories (p < .0001). The test for linear trend was also significant for each of the comparisons. The results of this study offer additional support for the use of the PCPC and POPC. These brief and easily completed measures can provide useful information regarding probable outcomes for pediatric intensive care patients when more extensive psychometric testing is not feasible or desirable.

Pain assessment and treatment in children with cognitive impairment: a survey of nurses' and physicians' beliefs.

To gather information about healthcare providers' beliefs regarding pain and its treatment in children with cognitive impairment. A survey consisting of two acute pain vignettes (a child undergoing surgery and a child undergoing an outpatient medical procedure) was completed by 440 nurses and 146 physicians at Arkansas Children's Hospital. Respondents completed one of four different surveys that systematically varied the child's level of cognitive impairment (none, mild, moderate, severe) in both vignettes. Questions addressed expected pain, pain assessment methods, pharmacologic regimens, and nonpharmacologic interventions. In general, provider discipline and level of cognitive impairment did not significantly influence responses about pain experience and treatment. Potent analgesia, regularly scheduled dosing, and nonpainful administration were selected most frequently. Self-report of pain, patient-controlled analgesia, and behavioral interventions requiring higher cognitive skills were selected less frequently for children with more severe cognitive impairment. The presence of cognitive impairment appeared to influence provider decisions regarding the appropriateness of specific pain assessment and treatment methods requiring skills on the part of the child. Overall, healthcare provider views regarding analgesia and sedation were similar for all children, regardless of impairment. Whether these beliefs are consistent with clinical practice is yet to be documented.

Endoscopic management of hydrocephalus secondary to tumors of the posterior third ventricle

Management of the obstructive hydrocephalus that accompanies tumors located in the third ventricle has traditionally involved either urgent tumor resection, with resultant ventricular decompression, or cerebrospinal fluid diversion that requires either ventriculostomy or shunt placement prior to tumor removal. Although this approach has worked well for the better part of a century, it has both short- and long-term sequelae that can possibly be avoided. Beacause a number of lesions in this area are benign or are amenable to radiotherapy, a less invasive approach to their treatment is desirable. The advances in both instrumentation and techniques of endoscopic surgery have established alternatives to the traditional treatment of third ventricular tumors and resultant hydrocephalus. The authors review the treatment of 19 patients with posterior third ventricular tumors who presented to Arkansas Children's Hospital over a 5-year period (September 1993-July 1999). In 11 patients signs and/or symptoms of hydrocephalus were demonstrated and were treated with endoscopic third ventriculostomy, additionally, a biopsy procedure, resection, or fenestration of the tumor was performed in a number of patients. Endoscopy was believed to have been of benefit in all patients, despite the eventual failure of the ventriculostomy in one patient. There were no complications in this series. The algorithm thus developed by the authors provides both a diagnostic and therapeutic pathway that may ultimately reduce the morbidity associated with the treatment of patients with posterior third ventricular lesions.