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.

Flow cytometric vs morphologic assessment of remission in childhood acute lymphoblastic leukemia: a report from the children’s oncology group (cog)

Abstract: Flow cytometric vs morphologic assessment of remission in childhood acute lymphoblastic leukemia: A report from the Children’s Oncology Group (COG)

A Dual-center Review of Compressive Osseointegration for Fixation of Massive Endoprosthetics: 2- to 9-year Followup

Abstract: Background Aseptic failure of massive endoprostheses used in the reconstruction of major skeletal defects remains a major clinical problem. Fixation using compressive osseointegration was developed as an alternative to cemented and traditional press-fit fixation in an effort to decrease aseptic failure rates.

Outcomes of Childhood Cholestasis in Alagille Syndrome: Results of a Multicenter Observational Study.

Abstract: Alagille syndrome (ALGS) is an autosomal dominant multisystem disorder with cholestasis as a defining clinical feature. We sought to characterize hepatic outcomes in a molecularly defined cohort of children with ALGS-related cholestasis. Two hundred and ninety-three participants with ALGS with native liver were enrolled. Participants entered the study at different ages and data were collected retrospectively prior to enrollment, and prospectively during the study course. Genetic analysis in 206 revealed JAGGED1 mutations in 91% and NOTCH2 mutations in 4%. Growth was impaired with mean height and weight z-scores of <-1.0 at all ages. Regression analysis revealed that every 10 mg/dL increase in total bilirubin was associated with a decrease in height z-score by 0.10 (P = 0.03) and weight z-score by 0.15 (P = 0.007). Total bilirubin was higher for younger participants (P = 0.03) with a median of 6.9 mg/dL for those less than 1 year old compared with a median of 1.3 mg/dL for participants 13 years or older. The median gamma glutamyl transferase also dropped from 612 to 268 in the same age groups. After adjusting for age, there was substantial within-individual variation of alanine aminotransferase. By 20 years of age, 40% of participants had developed definite portal hypertension. Estimated liver transplant-free survival at the age of 18.5 years was 24%. Conclusions: This is the largest multicenter natural history study of cholestasis in ALGS, demonstrating a previously underappreciated burden of liver disease with early profound cholestasis, a second wave of portal hypertension later in childhood, and less than 25% of patients reaching young adulthood with their native liver. These findings will promote optimization of ALGS management and development of clinically relevant endpoints for future therapeutic trials.

Neurocognitive outcomes in pediatric and adolescent patients with central nervous system germinoma treated with a strategy of chemotherapy followed by reduced-dose and volume irradiation.

Abstract: Background Evaluation of neurocognitive outcomes after a treatment strategy of chemotherapy followed by reduced-dose and volume irradiation for primary central nervous system (CNS) germinoma. Methods Neuropsychological evaluations including measures of verbal and nonverbal intellectual functioning, working memory, processing speed, verbal and nonverbal memory, executive functioning, depression, anxiety, and social functioning were analyzed for all patients (N = 20) with histologically diagnosed CNS germinoma diagnosed at our institution between 2003 and 2009. All 20 patients underwent at least one neuropsychological evaluation, 14 patients had at least two evaluations, 7 patients had at least three, and 2 had four, generating a total of 43 test batteries at a mean of 3.0 years from diagnosis. Results The 20 patients performed as a group in the average range on all neurocognitive measures administered when compared to the nonmedical normative group. No decline over time was indicated on any of the neurocognitive measures. An improvement over time was indicated for nonverbal reasoning, nonverbal memory, processing speed, working memory, response inhibition, and cognitive flexibility. No significant differences were found by tumor location, age at diagnosis, or hydrocephalus at presentation. Conclusions For pediatric and adolescent patients with newly diagnosed CNS germinoma, modest chemotherapy followed by reduced dose ventricular field irradiation with simultaneous integrated boost to the primary site, is associated with preservation of neurocognitive, social and emotional functioning. Pediatr Blood Cancer 2011; 57: 669–673. © 2011 Wiley-Liss, Inc.