Abstracts of staff publications
aSpring 1997
Dexamethasone in bronchiolitis: A randomised controlled trial
Genie Roosevelt, Karen Sheehan, Jacqueline Grupp-Phelan, Robert Tanz and Robert Listernick
From the Divisions of General Academic Pediatrics (RT, RL) and Pediatric Emergency Medicine (GR, KS, JG-P) at Children’s Memorial Hospital and the Department of Pediatrics, Northwestern University Medical School, Chicago.
Lancet 1996;348:292–295.
Background: Although corticosteroids are commonly prescribed in the treatment of bronchiolitis, there is no evidence on the efficacy of these drugs in this disorder. We designed a randomised, double-blind prospective study to assess the efficacy of dexamethasone in infants with bronchiolitis who require hospital management.
Methods: Infants younger than 12 months who had been admitted to hospital for an initial episode of wheezing were randomly allocated intramuscular dexamethasone (1 mg/kg daily) or placebo, every 24 h for three doses. We excluded infants who were younger than 4 weeks, who had required admission to the intensive care unit, or who had a history of congenital heart disease, mechanical ventilation, or supplemental oxygen use. We assessed infants on admission and every 12 h thereafter—vital signs were taken, severity of accessory muscle use and wheezing were measured by a clinical severity score, and pulse oximetry in room air was done. Our primary endpoints were the time to resolution of symptoms—defined as the number of assessments needed to reach oxygen saturation of more than 95% while receiving no supplemental oxygen, an accessory muscle score of 0, a wheeze score of 0 or 1, and resumption of normal feeding—and duration of oxygen therapy. Follow-up assessments were made 10 to 14 days after discharge by telephone. We used a proportional-hazards model for our survival analysis.
Findings: 197 infants presented with bronchiolitis that required inpatient management. 75 were not enrolled (31 no consent, 28 no approach made, 16 transferred elsewhere). Of the 122 enrolled, four were excluded (clinical deterioration, diagnosis of cystic fibrosis, previous intubation, did not receive all study treatment). There were no differences between the dexamethasone (n = 65) and placebo-treated infants in demographic factors, exposure to tobacco smoke, duration of illness, presence of respiratory syncytial virus (RSV) antigen, respiratory rate, or severity score. More dexamethasone-treated patients had an initial oxygen saturation of 95% or less (51 [79%] dexamethasone vs 31 [59%] placebo, p = 0.02). There were no differences in duration of oxygen therapy (p = 0.74) or time to resolution of symptoms (p = 0.22). Stratification for presence of RSV antigen or family history of atopy did not affect the results.
Interpretation: Our findings do not support the use of dexamethasone in the treatment of bronchiolitis in infants.
Relation of maternal race to the risk of preterm, non-low birth weight infants: A population study
James W. Collins, Jr., and Nancy Hammond
From the Division of Neonatology, Children’s Memorial Hospital, Northwestern University Medical School, Chicago
American Journal of Epidemiology 1996;143:333–337
The authors used 1982–1983 Illinois vital records and 1980 US Census income data to determine the contribution of maternal race to the risk of preterm (<260 days), non-low birth weight (>2,500 g) infants. This older cohort was chosen to avoid the confounding effect of cocaine associated with its increased local availability after 1985. In Chicago, the unadjusted preterm, non-low birth weight rate was 14% for African Americans (n = 43,059) compared with 9% for Mexican Americans (n = 10,397) and 7% for whites (n = 26,152) (odds ratio = 1.7, 95% confidence interval 1.6–1.8; odds ratio = 2.4, 95% confidence interval 2.3–2.5, respectively). In logistic models that included maternal sociodemographic and prenatal care variables, the adjusted odds ratio of preterm, non-low birth weight for African Americans compared with Mexican Americans and whites was 1.6 (95% confidence level 1.4–1.8) and 1.5 (95% confidence level 1.2–1.7) respectively. The authors conclude that some factor closely linked to the African American race, not underserved minority status per se, is a fundamental cause of preterm, non-low birth weight.
Eye manifestations of congenital toxoplasmosis
Marilyn Mets, Ellen Holfels, Kenneth Boyer, Charles Swisher, Nancy Roizen, Laszlo Stein, et al.
From the Divisions of Ophthalmology (MM), Neurology (CW), and Audiology (LS), Children’s Memorial Hospital and Northwestern University Medical School, Chicago; and staff members at Michael Reese Hospital, Rush-Presbyterian St. Luke’s, the University of Chicago, Illinois Institute of Technology, The Doctors Hospital of Chicago, University of Illinois at Chicago, Stanford University and the Palo Alto Research Institute, and Columbia University.
American Journal of Ophthalmology 1996;122;309–324
Purpose: To determine the natural history of treated and untreated congenital toxoplasmosis and impact of this infection on vision.
Methods: In this prospective, longitudinal study, 76 newborns were treated with pyrimethamine and sulfadiazine for approximately one year, and 18 individuals not treated during their first year of life entered the study after age 1 year (historical patients).
Results: Chorioretinal scars were the most common eye finding in all patients and were most common in the periphery (58% of treated and 82% of historical patients). Macular scars were present in 54% of the treated patients; 41% were bilateral. Macular scars were present in 76% of the historical patients; 23% were bilateral. Visual acuity in the presence of macular lesions ranged from 20/20 to 20/400. Of the patients followed up from the newborn period and treated, 29% had bilateral visual impairment, with visual acuity for the best eye of less than 20/40. Causes for this visual impairment in eyes with quiescent lesions included macular scars, dragging of the macula secondary to a peripheral lesion, retinal detachment, optic atrophy, cataract, amblyopia, and phthisis. There were recurrences in both treated (13%, 7/54) and previously untreated historical patients (44%, 8/18). The total, median, and range of years of follow-up during which recurrences were observed were, for treated patients, 189 years (total), five years (median), and three to ten years (range) and, for historical, untreated patients, 160 years (total), 11 years (median), and three to 24 years (range). News lesions occurred in previously normal retinas and also contiguous to older scars. Active lesions appeared to become quiescent within ten to 14 days after beginning pyrimethamine and sulfadiazine therapy.
Conclusion: Many children with congenital toxoplasmosis have substantial retinal damage at birth and consequent loss of vision. Nonetheless, vision may be remarkably good in the presence of large macular scars. Active lesions become quiescent with treatment.
Transmission of full-length echocardiographic images over ISDN for diagnosing congenital heart disease
Ernerio Alboliras, Kaliope Berdusis, Joel Fisher, Robert Harrison, D.Woodrow Benson, Jr., and Catherine Webb
From the Divisions of Cardiology (EA, KB, RH, DB, CW) and Neonatology (JF), Children’s Memorial Hospital and Northwestern University Medical School (EA, JF, DB, CW), Chicago; and Northwest Community Hospital, Arlington Heights (JF)
Telemedicine Journal 1996;2:251–258
Objectives: To study the feasibility of transmitting full-length diagnostic-quality echocardiograms with video conferencing systems linked by ISDN.
Methods: Forty-three previously video-recorded full-length echocardiograms (22 complete two-dimensional Doppler studies, 21 separate M-mode studies) were transmitted from a site 30 miles distant using desktop video conferencing systems linked with one ISDN line (128 kbps). Blinded interpretations of the original recorded and transmitted studies were made by two pediatric cardiologists (two-dimensional Doppler) and a pediatric sonographer (M-mode).
Results: Diagnoses of normal (N = 12) and abnormal (N = 10) conditions were made from the original tapes; the same interpretations were made from the transmitted studies. Twenty-one specific abnormalities in the 10 abnormal studies were seen on both original and transmitted videotapes. These abnormalities ranged from simple congenital heart defects, including ventricular septal defect, atrial septal defect, and patent ductus arteriosus, to complex ones, such as coronary artery fistula, double-outlet right ventricle, and complete atrioventricular canal. Qualitatively, there was mild degradation in gray scale, brightness, and contrast of the images. Six M-mode variables from the transmitted images had excellent concordance with the original tracings (P = 0.506 to 0.838; r = 0.86 to 0.97).
Conclusions: Although our sample size was small, this preliminary experience indicates that video conferencing equipment utilizing ISDN technology is a reliable method for transmitting full-length diagnostic-quality echocardiographic studies from remote sites. This technology may have a significant impact on the management of pediatric patients with suspected heart disease. Further evaluation is warranted.
Adrenal hypoplasia congenita with hypogonadotropic hypogonadism: Evidence that DAX–1 mutations lead to combined hypothalamic and pituitary defects in gonadotropin production
Reema Habiby, Paul Boepple, Lisa Nachtigall, Patrick Sluss, William Crowley, Jr., and J. Larry Jameson
From the Division of Endocrinology, Metabolism, and Molecular Medicine, Northwestern University Medical School, Chicago; and the Reproductive Endocrine Unit, Massachusetts General Hospital, Boston.
Journal of Clinical Investigation 1996;98:1055–62
Adrenal hypoplasia congenita (AHC) is an X-linked disorder that typically presents with adrenal insufficiency during infancy. Hypogonadotropic hypogonadism (HHG) has been identified as a component of this disorder in affected individuals who survive into childhood. Recently, AHC was shown to be caused by mutaions in DAX–1, a protein that is structurally similar in its carboxyterminal region to orphan nuclear receptors. We studied two kindreds with clinical features of AHC and HHG. DAX–1 mutations were identified in both families. In the JW kindred, a single base deletion at nucleotide 1219 was accompanied by an additional base substitution that resulted in a frameshift mutation at codon 329 followed by premature termination. In the MH kindred, a GGAT duplication at codon 418 caused a frameshift that also resulted in truncation of DAX–1. Baseline luteinizing hormone (LH), follicle-stimulating hormone (FSH), and free-a-subunit (FAS) levels were determined during 24 h of frequent (q10 min) venous sampling. In patient MH, baseline levels were low, but FAS levels were within the normal range. In constrast, in patient JW, the mean LH and FSH were within the normal range during baseline sampling, but LH secretion was erratic rather than showing typical pulses. FAS was apulsatile for much of the day, but a surge was seen over a 3–4 h period. Pulsatile gonadotropin releasing hormone (GnRH) (25 ng/kg) was administered every 2 h for 7 d to assess pituituary responsiveness to exogenous GnRH. MH did not exhibit a gonadotropin response to pulsatile GnRH. JW exhibited a normal response to the first pulse of GnRH, but there was no increase in FAS. In contrast to the priming effect of GnRH in GnRH-deficient patients with Kallmann syndrome, GnRH pulses caused minimal secretory responses of LH and no FAS responses in patient JW. The initial LH response in patient JW inplies a deficiency in hypothalamic GnRH. On the other hand, the failure to respond to pulsatile GnRH is consistent with a pituitary defect in gonadotropin production. These two cases exemplify the phenotypic heterogeneity of AHC/HHG, and suggest that DAX–1 mutations impair gonadotropin production by acting at both the hypothalamic and pituitary levels.
Do latex precautions in children with myelodysplasia reduce intraoperative allergic reactions?
Patrick Birmingham, Richard Dsida, John Grayhack, Jianping Han, Melissa Wheeler, Jacqueline Pongracic, Charles Coté, and Steven Hall
From the Department of Anesthesia (PB, RD, MW, CC) and the Divisions of Orthopaedic Surgery (JG, JH) and Allergy (JP), Children’s Memorial Hospital, Northwestern University Medical School, Chicago.
Journal of Pediatric Orthopaedics 1996;16:799–802
Children with myelodysplasia have an increased incidence of latex allergy, which can lead to severe intraoperative allergic reactions. Despite widespread recommendations to avoid intraoperative latex exposure, little evidence exists to support the efficacy of this practice. We examined the incidence of intraoperative allergic reactions in children with myelodysplasia who underwent 1,025 operations in a 36-month period before and after institution of a standardized latex-avoidance protocol. Risk factors for an intraoperative reaction were found to be a history of latex allergy (p = 0.001) and surgery performed before institution of the latex-avoidance protocol (p = 0.01) The estimate of increased risk for allergic reaction was 3.09 times higher in cases performed without latex avoidance. Recognized violation of the protocol after its institition led to severe allergic reactions in three patients. Our experience suggests that a latex-avoidance protocol reduces intraoperative allergic reactions in children with myelodysplasia. Development of severe allergic reactions with violation of the protocol reinforces the importance of vigilance on the part of all operating room personnel in its implementation.
In-line skate and rollerskate injuries in childhood
Elizabeth Powell and Robert Tanz
From the Divisions of Pediatric Emergency Medicine (EP) and General Academic Pediatrics (RT), Children’s Memorial Hospital, Northwestern University Medical School, Chicago
Pediatric Emergency Care 1996;8:259–262
Objective: To describe the estimated frequency and types of injuries associated with in-line skates in U.S. children and to compare in-line skating injuries to rollerskating injuries.
Design: National case series.
Setting: Emergency departments of hospitals participating in the United States Consumer Product Safety Commission (USCPSC) National Electronic Injury Surveillance System.
Patients: Persons with injuries associated with the use of in-line skates or rollerskates reported to the USCPSC in 1992 and 1993.
Results: There were an estimated 66,465 injuries associated with in-line skates; the incidence of injury was highest in children 11 and 12 years old. An estimated 40,730 in-line skate injuries involved children <20 years old. The mean age of injured children was 11.8 years (median, 12 years); 68% were boys. Fractures (45%) were the most common injury; 66% of fractures involved the distal forearm. Five percent had head injuries. Two and one-half percent required hospital admission; 90% of children admitted had a fracture and 11% had a head or face injury. There were an estimated 147,928 rollerskating injuries among children <20 years old; the mean age was 10.5 years (median 10 years) (P <0.001 vs in-line skates). Thirty-two percent were to boys (P <0.001 vs in-line skates). Fractures were the most common injury; forearm fractures accounted for 72% (P<0.001 vs in-line skates). Five percent had head injuries. One and one-half percent were admitted to the hospital (P <0.001 vs in-line skates). In 1993, the injury rate among children for in-line skates was 31/100,000, and the injury rate for rollerskates was 95/100,000.
Conclusions: Injuries associated with in-line skates are highest among preadolescents. Injuries associated with in-line skate use are less common than injuries associated with rollerskate use. Distal forearm fractures are the most common injuries related to both in-line skate and rollerskate use. Exposure data and analysis of the efficacy of protective gear, including wrist guards and helmets, are needed.
Growth of Chicago-area infants, 1985 through 1987: Not what the reference curves predict
Helen Binns, Yvonne Senturia, Susan LeBailly, Mark Donovan, and Katherine Kaufer Christoffel
From Children’s Memorial Hospital and Northwestern University Medical School, Chicago.
Archives of Pediatrics & Adolescent Medicine 1996;150;842–849
Objective: To determine if the National Center for Health Statistics (NCHS) growth curves (including 867 white infants, born between 1929 and 1975, in the Yellow Springs, Ohio, area) reflect contemporary infant growth in pediatric practices.
Design: Observational cohort study of healthy term infants. Office personnel obtained standardized measurements at health maintenance visits.
Setting: Ten pediatric community practices that were members of the Chicago, Ill., area Pediatric Practice Research Group (PPRG).
Methods: Measurements of 1,574 PPRG infants, seen on at least 5 occasions between 2 and 54 weeks of age, generated sex-specific growth curves using a 3-parameter mathematical model fitted to the serial data for each infant. Values from the computed curves were compared with NCHS growth references at 1, 3, 6, 9 and 12 months. The birth weights of PPRG and NCHS cohorts were compared.
Results: Mean birth weight of PPRG infants was significantly greater than a similar NCHS measure. Compared with the NCHS-reference curve, PPRG infants were heavier at 1, 3, and 6 months, longer on all comparisons, and had greater head circumferences, particularly in the early months of age.
Conclusions: The NCHS growth curves do not accurately reflect infant growth in this cohort. Nationally representative data are needed to revise the NSHS growth curves.
Testing of an intrathoracic artificial lung in a pig model
Keith Cook, Anthony Makarewicz, Carl Backer, Lyle Mockros, H. J. Przybylo, Susan Crawford, José Hernandez, Ronald Leonard, and Constantine Mavroudis
From the Department of Biomedical Engineering, Northwestern University, Evanston (KC, LM); the Division of Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, Chicago (KC, AM, CB, HJB, SC, JH, CM); and Sarns/3M Healthcare, Ann Arbor, Michigan (RL)
American Society for Artificial Internal Organs Journal 1996;42:M604–M609
A low input impedance, intrathoracic artificial lung is being developed for use in acute respiratory failure or as a bridge to transplantation. The device uses microporous, hollow fibers in a 0.74 void fraction, 1.83.m2
surface area bundle. The bundle is placed within a thermoformed polyethylene terephthalate glucose modified housing with a gross volume of 800 cm3
. The blood inlet and outlet are 18 mm inner diameter vascular grafts. Between the inlet graft and the device is a 1-inch inner diameter, thin-walled, latex tubing compliance chamber. These devices were implanted in Yorkshire pigs via median sternotomy with an end-to-side anastomosis to the pulmonary artery and left atrium. The distal pulmonary artery was occluded to divert the right ventricular output to the device. Pigs 1 and 2 were supported fully for 24 hrs and then killed. Pig 3 was supported partially for 20 hrs and died from bleeding complications. The first implant, in a 55 kg male pig, transferred an average of 176 ml/min ±42.4 of O2 and 190 ml/min ±39.7 of CO2 with an average blood flow rate of 2.7 L/min ±0.46. The normalized average right ventricular output power, Pn, was 0.062 W/(L/min) ±0.0082, and the average device resistance, R, was 3.5 mmHg/(L/min) ±0.62. The second implant, in a 60 kg male pig, transferred an average of 204 ml/min ±22.5 of O2 and 242 ml/min ±17.2 of CO2 with an average blood flow rate of 3.7 L/min ±0.45, Pn of 0.064 W/(L/min) ±0.0067, and R of 4.3 mmHg/(L/min) ±0.89. The third implant, in an 89 kg male pig, transferred an average of 156 ml/min ±39.6 of O2 and 187 ml/min ±21.4 of CO2 with an average blood flow rate of 2.5 L/min ±0.49, Pn of 0.052 W/(L/min) ±0.0067 and R of 3.4 mmHg/(L/min) ±0.74. These experiments suggest that such an artificial lung can temporarily support the gas transfer requirements of adult humans without overloading the right ventricle.
Firearm violence among youth: Public health strategies for prevention
Elizabeth Powell, Karen Sheehan, Katherine Kaufer Christoffel
From the Divisions of Pediatric Emergency Medicine (EP, KS) and General Academic Pediatrics (KKC) and the Violent Injury Prevention Center (KKC), Children’s Memorial Hospital, and Northwestern University Medical School, Chicago.
Annals of Emergency Medicine 1996;28:204–212.
Firearm violence is a serious threat to the health of our children: an American child dies of gunshot wounds every 11
/2 hours, and every 2 days 30 children—the equivalent of a school classroom—lose their lives to guns. Injured children and adolescents are cared for in emergency departments and trauma centers, and in some urban areas the increasing incidence of firearm injuries threatens to overwhelm the trauma care delivery system. Because of the prevalence and enormous cost of firearm violence it has been identified as an epidemic and a public health emergency. In this article we discuss the burden of firearm injury and its effect on children and young adults, and we outline a public health approach to firearm injury prevention.
The genetic basis of hereditary blistering disorders
Amy Paller
From the Division of Dermatology, Children’s Memorial Hospital, Chicago.
Current Opinion in Pediatrics 1996;8:367–371
Inherited blistering disorders have been classified by inheritance pattern, ultrastructural morphology and level of skin cleavage, and immunofluorescence detection of cutaneous antigens. During the past 5 years, investigators have discovered the molecular bases for the major inherited blistering disorders, epidermolytic hyperkeratosis, epidermolysis bullosa simplex, epidermolytic palmoplantar keratoderma, junctional epidermolysis bullosa, and dystrophic forms of epidermolysis bullosa. In addition to providing answers to patients about the underlying mechanisms of these devastating blistering disorders, the discoveries have provided insight into the function of several cutaneous proteins and have led to the ability to diagnose prenatally these lifelong conditions through genetic analysis in affected families.
