Showing papers by "Primary Children's Hospital published in 2004"

The Role of Autonomy and Pubertal Status in Understanding Age Differences in Maternal Involvement in Diabetes Responsibility across Adolescence

Abstract: Objective To examine how autonomy and pubertal status explain age decreases in maternal involvement in type 1 diabetes management across adolescence, how they relate to metabolic control, and the reasons that guide declines in maternal involvement. Methods One hundred twenty-seven children ages 10–15 years with type 1 diabetes and their mothers participated. Data included maternal and child report of diabetes management, child report of autonomy level, maternal report of pubertal status, maternal reports of reasons for transfer of diabetes responsibility, and glycosylated hemoglobin (Hba1c) values. Results Autonomy and pubertal status partially mediated age effects on reports of maternal involvement. Mothers’ reasons for transferring responsibility included responding to the child’s competence, promoting competence and maturity in their child, and minimizing hassles and conflict. The transfer of diabetes responsibility from mother to child without sufficient autonomy and when pubertal status was low was related to higher Hba1c values. Conclusions The importance of chronological age for changes in maternal involvement suggests the need to examine mothers’ and adolescents’ developmental expectations for diabetes management. The reasons for transferring responsibility from mother to child suggest many avenues for intervention.

Adenoviral Infections in Children: The Impact of Rapid Diagnosis

Abstract: Background. Adenovirus (ADV) infections were difficult to diagnose in the past, and many infections were unrecognized. Direct fluorescent assay (DFA) for the rapid diagnosis of ADV infection, as part of a viral respiratory panel, became available at Primary Children’s Medical Center (Salt Lake City, UT) in December 2000. Objective. To describe children with ADV infection diagnosed by DFA and viral culture and document the impact of rapid ADV testing on patient care. Methods. DFA testing for respiratory viruses including ADV was performed on nasal wash specimens with parallel viral culture. Chart review was performed for all ADV-positive patients identified from microbiology records between December 2000 and May 2002. Results. Of 1901 patients positive for respiratory viruses, 143 (7.5%) were ADV-positive by DFA or culture. The mean age of ADV-positive children was 23 months; 90% were ≤60 months old. Eighty percent were previously healthy, and 56% required admission with a mean length of stay of 3.4 days. The most common diagnoses included fever (31%), bronchiolitis (24%), and pneumonia (14%). Other conditions included suspected Kawasaki disease (KD) and hepatitis. Forty-six percent of ADV-positive children were given antibiotics at presentation, but only 2 (1.4%) had documented bacterial infection (one had Escherichia coli urinary tract infection and one had Moraxella catarrhalis bacteremia). Thirty-six percent of children had a change in management based on positive ADV DFA. In children with suspected KD ( n = 5), 100% had positive ADV DFA, and immune globulin was withheld in 4. One immunocompetent patient with fulminant liver failure received cidofovir treatment after a positive ADV DFA and recovered before liver transplant. Conclusions. ADV is a common infection in young children and often results in admission and unnecessary antibiotic therapy. Identifying ADV as the cause of illness can favorably impact care and in some instances may be life-saving. DFA testing for ADV should be considered for infants and children requiring admission for fever, respiratory illness, suspected KD, and hepatitis.

Phase I Trial of Temozolomide and Protracted Irinotecan in Pediatric Patients with Refractory Solid Tumors

Abstract: Purpose The purpose is to estimate the maximum-tolerated dose (MTD) of temozolomide and irinotecan given on a protracted schedule in 28-day courses to pediatric patients with refractory solid tumors. Experimental design Twelve heavily pretreated patients received 56 courses of oral temozolomide at 100 mg/m(2)/day for 5 days combined with i.v. irinotecan given daily for 5 days for 2 consecutive weeks at either 10 mg/m(2)/day (n = 6) or 15 mg/m(2)/day (n = 6). We assessed toxicity, the pharmacokinetics of temozolomide and irinotecan, and the DNA repair phenotype in tumor samples. Results Two patients experienced dose-limiting toxicity (DLT) at the higher dose level; one had grade 4 diarrhea, whereas the other had bacteremia with grade 2 neutropenia. In contrast, no patient receiving temozolomide and 10 mg/m(2)/day irinotecan experienced DLT. Myelosuppression was minimal and noncumulative. No pharmacokinetic interaction was observed. Drug metabolite exposures at the MTD were similar to exposures previously associated with single-agent antitumor activity. One complete response, two partial responses, and one minor response were observed in Ewing's sarcoma and neuroblastoma patients previously treated with stem cell transplant. Responding patients had low or absent O(6)-methylguanine-DNA methyltransferase expression in tumor tissue. Conclusions The MTD using this schedule was temozolomide (100 mg/m(2)/day) and irinotecan (10 mg/m(2)/day), with DLT being diarrhea and infection. Drug clearance was similar to single-agent values, and clinically relevant SN-38 lactone and MTIC exposures were achieved at the MTD. As predicted by xenograft models, this combination and schedule appears to be tolerable and active in pediatric solid tumors. Evaluation of a 21-day schedule is planned.

Nonvalvular Cardiovascular Device—Related Infections

Abstract: More than a century ago, Osler took numerous syndrome descriptions of cardiac valvular infection that were incomplete and confusing and categorized them into the cardiovascular infections known as infective endocarditis. Because he was both a clinician and a pathologist, he was able to provide a meaningful outline of this complex disease. Technical advances have allowed us to better subcategorize infective endocarditis on the basis of microbiological etiology. More recently, the syndromes of infective endocarditis and endarteritis have been expanded to include infections involving a variety of cardiovascular prostheses and devices that are used to replace or assist damaged or dysfunctional tissues (Table 1). Taken together, infections of these novel intracardiac, arterial, and venous devices are frequently seen in medical centers throughout the developed world. In response, the American Heart Association’s Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease wrote this review to assist and educate clinicians who care for an increasing number of patients with nonvalvular cardiovascular device–related infections. Because timely guidelines1,2 exist that address the prevention and management of intravascular catheter–related infections, these device-related infections are not discussed in the present Statement. View this table: TABLE 1. Nonvalvular Cardiovascular Device–Related Infections This review is divided into two broad sections. The first section examines general principles for the evaluation and management of infection that apply to all nonvalvular cardiovascular devices. Despite the marked variability in composition, structure, function, and frequency of infection among the various types of nonvalvular cardiovascular devices reviewed in this article, there are several areas of commonality for infection of these devices. These include clinical manifestations, microbiology, pathogenesis, diagnosis, treatment, and prevention. The second section addresses each device and describes unique clinical features of infection. Each device is placed into one of 3 categories—intracardiac, arterial, or venous—for discussion. ### Clinical Manifestations The specific signs and symptoms associated with an infection of a …

Surveillance of medical device-related hazards and adverse events in hospitalized patients.

Abstract: ContextAlthough adverse drug events have been extensively evaluated by computer-based surveillance, medical device errors have no comparable surveillance techniques.ObjectivesTo determine whether computer-based surveillance can reliably identify medical device–related hazards (no known harm to patient) and adverse medical device events (AMDEs; patient experienced harm) and to compare alternative methods of detection of device-related problems.Design, Setting, and ParticipantsThis descriptive study was conducted from January through September 2000 at a 520-bed tertiary teaching institution in the United States with experience in using computer tools to detect and prevent adverse drug events. All 20 441 regular and short-stay patients (excluding obstetric and newborn patients) were included.Main Outcome MeasuresMedical device events as detected by computer-based flags, telemetry problem checklists, International Classification of Diseases, Ninth Revision (ICD-9) discharge code (which could include AMDEs present at admission), clinical engineering work logs, and patient survey results were compared with each other and with routine voluntary incident reports to determine frequencies, proportions, positive predictive values, and incidence rates by each technique.ResultsOf the 7059 flags triggered, 552 (7.8%) indicate a device-related hazard or AMDE. The estimated 9-month incidence rates (number per 1000 admissions [95% confidence intervals]) for AMDEs were 1.6 (0.9-2.5) for incident reports, 27.7 (24.9-30.7) for computer flags, and 64.6 (60.4-69.1) for ICD-9 discharge codes. Few of these events were detected by more than 1 surveillance method, giving an overall incidence of AMDE detected by at least 1 of these methods of 83.7 per 1000 (95% confidence interval, 78.8-88.6) admissions. The positive predictive value of computer flags for detecting device-related hazards and AMDEs ranged from 0% to 38%.ConclusionsMore intensive surveillance methods yielded higher rates of medical device problems than found with traditional voluntary reporting, with little overlap between methods. Several detection methods had low efficiency in detecting AMDEs. The high rate of AMDEs suggests that AMDEs are an important patient safety issue, but additional research is necessary to identify optimal AMDE detection strategies.

B-type natriuretic peptide levels in congenital heart disease.

Abstract: The objective of this study was to evaluate the potential role of B-type natriuretic peptide (BNP) levels in children with congenital heart disease undergoing cardiac catheterization. Measurement of plasma BNP concentration has been shown to be useful in the diagnosis, risk stratification, and management of adult patients with congestive heart failure, but little is known about the role of BNP in children with structural congenital heart disease. We measured plasma BNP levels using the Triage BNP test in patients with congenital heart disease referred for diagnostic or interventional cardiac catheterization. Plasma BNP concentration was measured in 96 children and 11 adults ≥19 years old (7.9 ± 8.3 years) undergoing heart catheterization for underlying congenital heart disease. BNP levels ranged from 1300 pg/ml, with a median BNP concentration of 19.0 pg/ml. Baseline BNP concentrations were >100.0 pg/ml on 19 occasions in 17 patients. The pressure difference between the left ventricle and ascending aorta was 10–110 mmHg in 21 patients. BNP concentrations for this cohort ranged from <5.0 to 1060.0 pg/ml and correlated with the degree of left ventricular outflow obstruction (correlation coefficient, 0.661; p = 0.001). This study suggests that with additional research, BNP concentration may prove to be a useful clinical tool in managing children and adults with congenital heart disease.

The volume of lung parenchyma as a function of age: a review of 1050 normal CT scans of the chest with three-dimensional volumetric reconstruction of the pulmonary system.

Abstract: Study design An Institutional Review Board-approved retrospective review of 3400 sequential CT scans of the thorax obtained at a single institution over a 3-year period from 2000 to 2003 was performed. Objectives We determined values for the volume of the right lung, left lung, and total lung volume and plot these data as a function of age and sex. Summary of background data To our knowledge, no normative data on CT determined lung volume as a function of age have been published. Methods All examinations with a report of a normal CT scan of the chest (1050 examinations) were identified. The volume of lung parenchyma in each normal examination was determined by performing a three-dimensional reconstruction of the pulmonary system. Results Predicted increases in pulmonary volume with age for the third to 97th percentiles of male and female children were calculated. Conclusions Normal values for the volume of lung parenchyma as a function of age and sex increase the clinical utility of a standard CT scan of the thorax in evaluating children with complex spinal deformities. They are a useful adjunct to pulmonary function testing. These data can be used in the pre- and postoperative evaluation of patients who are at risk of thoracic insufficiency syndrome, particularly in patients younger than 5 years of age, when standard pulmonary function testing cannot be accomplished. The effects of nonoperative treatment, early spinal fusion, and new techniques for the fusionless management of spinal deformity on lung volume can be quantified and compared to normal values.

Care provider assessment of intrathecal baclofen in children

Abstract: Intrathecal baclofen is used increasingly to manage severe spasticity in children. Before implanting the baclofen pump, care providers typically ask how it will benefit their child. The purpose of this study was to describe the perceptions of care providers about intrathecal baclofen for management of spasticity in 80 individuals (52 males, 28 females). The individuals were younger than 22 years at the time of implantation (mean age 11 years, SD 5 years; range 3 to 21 years). Participants had been implanted with the pump for a minimum of one year at the time of evaluation. The most common diagnoses were quadriplegic and diplegic cerebral palsy and traumatic brain injury. Most participants were at level IV and V on the Gross Motor Function Classification System. After pump implantation most participants had tone reduction on the Ashworth scale of 1 to 1.9 in the lower extremities and 0 to 0.9 in the upper extremities. Lower extremity range of motion was maintained in 43 of 51 individuals (84%) and lost in 8 participants (16%). Complications requiring surgery occurred in 63 of a larger group of 152 patients (incidence per patient-year of follow-up was 0.19). Thirty-one of the 80 children had orthopedic procedures after pump placement. Only one of these was unexpected and none had rapid progression of scoliosis. Most treatment goals were achieved. Goals most commonly chosen (decreased pain, prevention of worsening of deformity, and improved ease of care) were improved in 91%, 91%, and 88% of participants respectively. Ninety-five per cent of care providers agreed that they would have this procedure performed again (81% strongly agreed, 14% slightly agreed). All care providers reported improvement in scores on the Caregiver Questionnaire. This information has been helpful to families considering intrathecal baclofen therapy.

Cerebral sinovenous thrombosis in children: another reason to treat iron deficiency anemia.

Abstract: Iron deficiency anemia is a rare cause of cerebral sinovenous thrombosis in children. We report three cases of cerebral sinovenous thrombosis and iron deficiency anemia treated at Primary Children's Medical Center in Salt Lake City, Utah, between 1998 and 2001. The children were 9, 19, and 27 months old at the time of admission. Hemoglobin levels ranged from 6.6 to 7.0 g/dL, mean corpuscular volume levels from 45 to 56 fL, and platelet counts from 248,000 to 586,000/microL. Magnetic resonance imaging and magnetic resonance venography revealed thrombosis of the straight sinus and internal cerebral veins in all three children, with the addition of the vein of Galen, left transverse and sigmoid sinuses, and upper left internal jugular vein in one child. Recovery ranged from excellent to poor in 3 months to 3 years of follow-up. Four additional cases, ages 6 to 22 months, were found in the English-language literature. Evaluation for prothrombotic disorders was negative in all children, including the current cases. Treatments have included thrombectomy, corticosteroids, mannitol, heparin, low-molecular-weight heparin, warfarin, aspirin, blood transfusion, and iron supplementation, but there is no consensus regarding therapy, other than to correct the anemia and treat iron deficiency. Iron deficiency anemia, a preventable cause of cerebral sinovenous thrombosis, deserves consideration when cerebral sinovenous thrombosis is detected in young children.

Detailed clinical models for sharable, executable guidelines.

Abstract: The goal of shareable, executable clinical guidelines is both worthwhile and challenging. One of the largest hurdles is that of representing the necessary clinical information in a precise and shareable manner. Standard terminologies and common information models, such as the HL7 RIM, are necessary, they are not sufficient. In addition, common detailed clinical models are needed to give precise semantics and to make the task of mapping between models manageable. We discuss the experience of the SAGE project related to detailed clinical models.

Adjustment and malfunction of a programmable valve after exposure to toy magnets. Case report.

Abstract: Inadvertent adjustments and malfunctions of programmable valves have been reported in cases in which patients have encountered powerful electromagnetic fields such as those involved in magnetic resonance imaging, but the effects of small magnetic fields are not well known. The authors present a case in which a child playing with a collection of commercially available toy magnets altered the pressure setting of an implanted valve and may have caused its permanent malfunction.

High-Throughput Detection of Glutathione S-Transferase Polymorphic Alleles in a Pediatric Cancer Population

Abstract: Polymorphisms of glutathione S-transferase (GST) enzymes have been correlated with altered risk of several cancers, as well as altered response and toxicity from cancer chemotherapy. We report a low cost, highly reproducible and specific PCR-based high-throughput assay for genotyping different GSTs designed for use in large clinical trials. In comparison to an alternative genotyping method (single nucleotide extension), the sensitivity and specificity of the high throughput assay was shown to be 92 and 97%, respectively, depending on the source of genomic DNA. Using the high-throughput assay, we demonstrate by multivariate analysis an increased risk of acute lymphoblastic leukemia, glial brain tumors, and osteosarcoma for patients carrying nonnull alleles of GSTM1 and/or GSTT1.

Determining lung volume with three-dimensional reconstructions of CT scan data: A pilot study to evaluate the effects of expansion thoracoplasty on children with severe spinal deformities.

Abstract: A retrospective review of a cohort of five patients who had been treated with a new technique for expansion thoracoplasty was performed. This study was designed to demonstrate that measurement of the volume of the lungs by three-dimensional reconstruction of CT scan data from children with complex spinal and thoracic deformities is a useful method for determining lung volumes in children who are too young to cooperate with pulmonary function testing. The authors' results indicate that lung volume by analysis of CT scan data is also a means of evaluating and quantifying the effects of expansion thoracoplasty. The authors were able to show that expansion thoracoplasty and stabilization with a titanium rib was able to increase the volume of lung parenchyma in the five patients in the study group by approximately 25% to 90% after surgical intervention.

Juvenile pilocytic astrocytoma of the brainstem in children.

Abstract: Object. In reports involving the operative treatment of brainstem tumors, multiple histological types are often grouped together. To determine prognosis after resection, histology-specific data may be helpful. Methods. Twenty-eight patients with juvenile pilocytic astrocytoma (JPA) of the brainstem (six in the midbrain, four in the pons, and 18 in the medulla) were identified from the medical records. Initial treatment was resection in 25 and biopsy sampling in three. Postoperative imaging revealed gross-total resection (GTR) or resection with linear enhancement (RLE) in 12 of 25 patients and solid residual tumor in the other 13. In 10 of the 13 patients harboring solid residual tumor, observation was undertaken; the residual lesion disappeared in one, was stable in four, and progressed in five. Of the 12 patients with complete excision or RLE only, seven underwent no further treatment, with tumor progression occurring in one. All patients were alive at last follow-up examination (range 0.3–20.4 years, me...

Treatment of CML in pediatric patients: Should imatinib mesylate (STI-571, Gleevec) or allogeneic hematopoietic cell transplant be front-line therapy?

Abstract: Background Long-term survival of pediatric patients with chronic myelogenous leukemia (CML) receiving myeloablative hematopoietic stem cell transplantation from fully-matched related and unrelated donors has been reported between 60 and 75%, but is associated with significant morbidity. Imatinib mesylate (STI-571, Gleevec) and reduced intensity conditioning stem cell transplantation (RIC) are two promising new tools that offer potential for decreasing therapy associated morbidity for patients with CML. Results Large trials have shown significant responses in chronic phase patients treated with imatinib and reasonable but short-lived responses in advanced phase CML. Data from adult studies is beginning to define populations likely to progress or have prolonged responses to imatinib, and some adult treatment paradigms are moving toward reserving transplantation until patients are at risk of failure with imatinib. Early trials of RIC transplantation in CML show decreased transplant related morbidity with efficacy similar to conventional transplantation, but the approach has yet to be verified in phase III studies. Data in pediatric patients with imatinib and RIC transplantation is limited. Conclusions Studies with imatinib are underway in pediatrics, but whether pediatric dosing schemes will lead to outcomes similar to adults is unknown. Because HLA-matched myeloablative transplantation offers a high rate of cure in the pediatric population, clinical studies assessing the role of imatinib mesylate and RIC transplantation should be planned carefully in order to avoid sub-optimal outcomes. © 2004 Wiley-Liss, Inc.

Understanding shaken baby syndrome.

Abstract: Health care professionals involved in the care of infants are in an ideal position to identify and to educate families, the public, and other health care professionals about the risk factors, dangers, and consequences of infant shaking. The purpose of this article is to review the incidence, biomechanics, risk factors, clinical presentation, diagnosis, and prognosis of shaken baby syndrome (SBS), as well as to encourage involvement in SBS prevention through the use of a family teaching tool. Education is essential to decrease the incidence, morbidity, and mortality of SBS.

Pediatric chronic rhinosinusitis

Abstract: Purpose of review Pediatric sinusitis is prevalent, and the debate continues regarding how best to care for these children. Although acute sinusitis is commonly associated with an upper respiratory infection, the focus of this paper is on chronic rhinosinusitis in children. Research is often more difficult in children than adults, so many times one can learn from the adult literature and determine whether there can be application to the childhood population. Recent findings This paper looks at both medical and surgical treatment of chronic rhinosinusitis. "Maximal medical management" is often cited in the literature, but what this should consist of has never been clearly proved in the literature. Alternative medicine approaches as well as irrigation as an adjunct to care are discussed. Biomaterials are also be discussed. Recent outcome data are put in perspective. Summary Hopefully the reader will find the presentation stimulating. The paper does not promote surgery as a "cure all," and in the end, analysis will hopefully leave the reader more cautious but with a better understanding of this complex disease.

Integrating detailed clinical models into application development tools.

Abstract: Several groups are currently working on defining detailed clinical models (also called templates or archetypes) that are refinements of abstract medical models like the HL7 (Health Level Seven) Reference Information Model. At IHC, we have created over 3,000 detailed clinical models in the last five years. These models have become an essential part of the architecture of our electronic medical record (EMR) system. As a result, we have created an increasingly sophisticated set of tools that allow the models to be searched, viewed, and ultimately incorporated into medical applications. These browsers have some commonality with terminology browsers, but are distinct in that the explicit structure of the information models must be accommodated. In this paper we report our experience in making browsers for detailed clinical models that are integrated with application authoring tools.