Primary Children's Hospital

About: Primary Children's Hospital is a healthcare organization based out in Salt Lake City, Utah, United States. It is known for research contribution in the topics: Population & Health care. The organization has 1770 authors who have published 2594 publications receiving 107857 citations. The organization is also known as: Intermountain Primary Children's Medical Center & Intermountain Primary Children's Hospital.

Dose-Limiting Toxicity After Hypofractionated Dose-Escalated Radiotherapy in Non–Small-Cell Lung Cancer

Abstract: PurposeLocal failure rates after radiation therapy (RT) for locally advanced non–small-cell lung cancer (NSCLC) remain high. Consequently, RT dose intensification strategies continue to be explored, including hypofractionation, which allows for RT acceleration that could potentially improve outcomes. The maximum-tolerated dose (MTD) with dose-escalated hypofractionation has not been adequately defined.Patients and MethodsSeventy-nine patients with NSCLC were enrolled on a prospective single-institution phase I trial of dose-escalated hypofractionated RT without concurrent chemotherapy. Escalation of dose per fraction was performed according to patients' stratified risk for radiation pneumonitis with total RT doses ranging from 57 to 85.5 Gy in 25 daily fractions over 5 weeks using intensity-modulated radiotherapy. The MTD was defined as the maximum dose with ≤ 20% risk of severe toxicity.ResultsNo grade 3 pneumonitis was observed and an MTD for acute toxicity was not identified during patient accrual. How...

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.

Real-time image-guided direct convective perfusion of intrinsic brainstem lesions. Technical note.

Abstract: Recent preclinical studies have demonstrated that convection-enhanced delivery (CED) can be used to perfuse the brain and brainstem with therapeutic agents while simultaneously tracking their distribution using coinfusion of a surrogate magnetic resonance (MR) imaging tracer. The authors describe a technique for the successful clinical application of this drug delivery and monitoring paradigm to the brainstem. Two patients with progressive intrinsic brainstem lesions (one with Type 2 Gaucher disease and one with a diffuse pontine glioma) were treated with CED of putative therapeutic agents mixed with Gd-diethylenetriamene pentaacetic acid (DTPA). Both patients underwent frameless stereotactic placement of MR imaging-compatible outer guide-inner infusion cannulae. Using intraoperative MR imaging, accurate cannula placement was confirmed and real-time imaging during infusion clearly demonstrated progressive filling of the targeted region with the drug and Gd-DTPA infusate. Neither patient had clinical or imaging evidence of short- or long-term infusate-related toxicity. Using this technique, CED can be used to safely perfuse targeted regions of diseased brainstem with therapeutic agents. Coinfused imaging surrogate tracers can be used to monitor and control the distribution of therapeutic agents in vivo. Patients with a variety of intrinsic brainstem and other central nervous system disorders may benefit from a similar treatment paradigm.

The burden of inherited leukodystrophies in children.

Abstract: Objectives: Leukodystrophies are diseases of the white matter for which data concerning clinical characteristics, incidence, disease burden, and description of outcomes are sparse. The purpose of our study was to determine the incidence and most common types of inherited leukodystrophies in a population, the mortality and time course of deaths, common neurologic features in patients, and health care costs associated with leukodystrophies. Methods: We conducted a retrospective, hospital- and clinic-based surveillance of inherited leukodystrophies among children younger than 18 years presenting to a regional children9s hospital. We enrolled children evaluated from January 1, 1999, through December 31, 2007; clinical information was obtained from medical records. We calculated incidence based on state birth rates. Results: A total of 122 children with an inherited leukodystrophy were identified; 542 patients were excluded. A total of 49% had epilepsy, 43% required a gastrostomy tube, and 32% had a history of developmental regression. Mortality was 34%; average age at death was 8.2 years. No final diagnosis was reported in 51% of patients. The most common diagnoses were metachromatic leukodystrophy (8.2%), Pelizaeus-Merzbacher disease (7.4%), mitochondrial diseases (4.9%), and adrenoleukodystrophy (4.1%). Endocrine abnormalities and hypoplastic cerebellum were noted in significant portions of patients (15% and 14%). Average yearly per-patient medical costs were $22,579. Population incidence was 1 in 7,663 live births. Conclusions: Inherited leukodystrophies are associated with substantial morbidity and mortality in children. Overall population incidence is higher than generally appreciated (1 in 7,663 live births). Most leukodystrophies remain undiagnosed, but a logical algorithm based on prevalence could aid testing.