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.

Odontoid synchondrosis fractures in children.

Abstract: Object. Odontoid synchondrosis fractures, although rare in the overall incidence of spinal trauma, are one of the more common fractures in young children. The goal of this study was to evaluate the demographic data, incidence of neurological deficits, treatment strategies, and outcomes in a combined series of odontoid synchondrosis fractures treated at the authors’ institution and reported in other series. Methods. In a retrospective chart review, the authors identified four odontoid synchondrosis fractures treated at their hospital since January 2000; these were combined with cases reported in six other series in the literature, yielding a total of 55 patients. Data regarding the patients’ age, sex, delayed diagnosis, odontoid displacement, neurological deficits, treatment, and fusion status were collected. The patients’ ages ranged from 9 months to 7 years (mean 2.8 years), with neither sex predominating. Diagnosis was delayed in eight cases. The orientation of the odontoid fracture was reported for 36 patients, with 94% experiencing anterior displacement. Spinal cord injury (SCI) was noted in 15 patients, including 11 with complete injuries and eight with SCI at the cervicothoracic junction. Forty-two (93%) of 45 patients with fractures initially treated with external immobilization attained fusion. Eight patients were treated with surgery; four initially, with no attempt at conservative therapy, three after failed halo immobilization, and one after nonunion because of delayed diagnosis. Conclusions. Odontoid synchondrosis fractures can be difficult to diagnose. In children younger than 7 years of age who present with neck pain or neurological deficits attributable to SCI, this fracture should be suspected. Given the high rate of fusion attained with conservative therapy, it is recommended for most synchondrosis fractures, although surgery may be warranted for individual cases.

Validation of the Pittsburgh Infant Brain Injury Score for abusive head trauma

Abstract: BACKGROUND: Abusive head trauma is the leading cause of death from physical abuse. Misdiagnosis of abusive head trauma as well as other types of brain abnormalities in infants is common and contributes to increased morbidity and mortality. We previously derived the Pittsburgh Infant Brain Injury Score (PIBIS), a clinical prediction rule to assist physicians deciding which high-risk infants should undergo computed tomography of the head. METHODS: Well-appearing infants 30 to 364 days of age with temperature RESULTS: A total of 1040 infants were enrolled: 214 cases and 826 controls. The 5-point PIBIS included abnormality on dermatologic examination (2 points), age ≥3.0 months (1 point), head circumference >85th percentile (1 point), and serum hemoglobin CONCLUSIONS: Our data suggest that the PIBIS accurately identifies infants who would benefit from neuroimaging to evaluate for brain injury. An implementation analysis is needed before the PIBIS can be integrated into clinical practice.

Spinal muscular atrophy type 1: are proactive respiratory interventions associated with longer survival?

Abstract: Context:Spinal muscular atrophy type 1, an autosomal recessive motor neuron disease, is a leading genetic cause of death in infancy and early childhood.Objective:To determine whether the early initiation of noninvasive respiratory interventions is associated with longer survival.Design:Single-instit

Sulfated glycosaminoglycans and low-density lipoprotein receptor contribute to Clostridium difficile toxin A entry into cells

Abstract: Clostridium difficile toxin A (TcdA) is a major exotoxin contributing to disruption of the colonic epithelium during C. difficile infection. TcdA contains a carbohydrate-binding combined repetitive oligopeptides (CROPs) domain that mediates its attachment to cell surfaces, but recent data suggest the existence of CROPs-independent receptors. Here, we carried out genome-wide clustered regularly interspaced short palindromic repeats (CRISPR)-CRISPR-associated protein 9 (Cas9)-mediated screens using a truncated TcdA lacking the CROPs, and identified sulfated glycosaminoglycans (sGAGs) and low-density lipoprotein receptor (LDLR) as host factors contributing to binding and entry of TcdA. TcdA recognizes the sulfation group in sGAGs. Blocking sulfation and glycosaminoglycan synthesis reduces TcdA binding and entry into cells. Binding of TcdA to the colonic epithelium can be reduced by surfen, a small molecule that masks sGAGs, by GM-1111, a sulfated heparan sulfate analogue, and by sulfated cyclodextrin, a sulfated small molecule. Cells lacking LDLR also show reduced sensitivity to TcdA, although binding between LDLR and TcdA are not detected, suggesting that LDLR may facilitate endocytosis of TcdA. Finally, GM-1111 reduces TcdA-induced fluid accumulation and tissue damage in the colon in a mouse model in which TcdA is injected into the caecum. These data demonstrate in vivo and pathological relevance of TcdA-sGAGs interactions, and reveal a potential therapeutic approach of protecting colonic tissues by blocking these interactions.