Formative Evaluation of CLABSI Adoption and Sustainment Interventions in a Pediatric Intensive Care Unit.

Background: Pediatric patients require central venous catheters to maintain adequate hydration, nutritional status, and delivery of life-saving medications in the pediatric intensive care unit. Although central venous catheters provide critical medical therapies, their use increases the risk of severe infection, morbidity, and mortality. Adopting an evidence-based central line-associated bloodstream infection (CLABSI) bundle to guide nursing practice can decrease and sustain low CLABSI rates, but reliable and consistent implementation is challenging. This study aimed to conduct a mixed-methods formative evaluation to explore CLABSI bundle implementation strategies in a PICU. Methods: The team used The Consolidated Framework for Implementation Research to develop the interview guide and data analysis plan. Results: Facilitators and barriers for the CLABSI bundle occurred in four domains: inner setting, process, characteristics of individuals, and innovation characteristics in each cycle that led to recommended implementation strategy opportunities. The champion role was a major implementation strategy that facilitated the adoption and sustainment of the CLABSI bundle. Conclusions: Implementation Science Frameworks, such as Consolidated Framework for Implementation Research (CFIR), can be a beneficial framework to guide quality improvement efforts for evidence-based practices such as the CLABSI bundle. Using a champion role in the critical care setting may be an important implementation strategy for CLABSI bundle adoption and sustainment efforts.

Autonomic dysreflexia during urodynamics in children and adolescents with spinal cord injury or severe neurologic disease.

INTRODUCTION: Autonomic dysreflexia (AD) is a well-established association of high spinal cord injury (SCI), particularly in those occurring above T6. When a noxious stimulus is encountered, the body responds by stimulating an increase in blood pressure, which is then countered by vasodilation. In patients with autonomic dysreflexia, the patient is unable to vasodilate below the level of spinal injury due to interruption of the autonomic innervation below the injury. This then leads to persistently elevated blood pressure causing uncoordinated autonomic responses such as headache, flushing, sweating, and even hypertensive crisis. The noxious stimulus most commonly reported is bladder or bowel distention [1]. This potential trigger is especially important since many patients with SCI require catheterization and repeated urodynamic testing, both of which predispose them to bladder distention. In response to an incident during which a patient developed severe hypertension during UDS, institutional concern was raised about the potential risk of AD in other patients with SCI ≥ T8 and other severe neurological disease undergoing urodynamic testing, and a protocol was initiated in 2007 for monitoring for AD during UDS. Although no long-term complication was encountered in this incident, the need for improvement in our understanding of the detection and treatment of AD during urodynamic testing was highlighted. However, due to the potential of UDS to trigger AD and possible subsequent severe cardiovascular crisis, a protocol was established at our institution. Because of reports documenting episodes of AD for patients with severe, non-SCI neurologic disease and the unknown risk, these patients also were historically monitored at our institution as well. OBJECTIVE: Autonomic dysreflexia (AD) is an uncoordinated autonomic response seen in patients with spinal cord injury (SCI). AD is often triggered by bladder distention, which may occur during urodynamic studies (UDS), and has potentially life-threatening consequences. Our purpose is to determine the prevalence and associated factors of AD in children undergoing UDS with either SCI or other neurological disease. METHODS: We identified 13 pediatric patients with SCI at the eighth thoracic vertebrae or above (SCI ≥ T8) or other severe neurological disorder with urodynamic evaluations between 2007 and 2011 at our institution. We retrospectively reviewed these patients for age, gender, bladder volume, bladder compliance, detrusor instability, symptoms of AD, blood pressure, and urinary infection. RESULTS: There were a total of 13 patients with SCI ≥ T8 (9), transverse myelitis (2), and encephalomyelitis (2). There were a total of 41 urodynamic studies with an average of 3.2 studies per patient. One adolescent with C1/2 injury and a prepubertal child with T2/3 injury developed AD. AD was not observed in non-SCI patients. The patients who developed AD had multiple subsequent episodes with follow up UDS. No statistical associations were found for the variables evaluated. No major complications occurred, and AD was successfully managed conservatively. CONCLUSIONS: With appropriate monitoring and education, AD is easily recognized and managed conservatively. We found an increased risk of patients developing subsequent AD episodes after an initial episode. Patients who did not have autonomic dysreflexia during initial UDS did not experience autonomic dysreflexia on subsequent UDS. We did not observe autonomic dysreflexia occurring in children with transverse myelitis or encephalomyelitis.

Genotype/phenotype correlation in 325 individuals referred for a diagnosis of tuberous sclerosis complex in the United States.

Tuberous sclerosis complex is an autosomal dominant neurocutaneous disorder marked by hamartoma growth in multiple organ systems. We performed mutational analyses on 325 individuals with definite tuberous sclerosis complex diagnostic status. We identified mutations in 72% (199/257) of de novo and 77% (53/68) of familial cases, with 17% of mutations in the TSC1 gene and 50% in the TSC2 gene. There were 4% unclassified variants and 29% with no mutation identified. Genotype/phenotype analyses of all observed tuberous sclerosis complex findings in probands were performed, including several clinical features not analyzed in two previous large studies. We showed that patients with TSC2 mutations have significantly more hypomelanotic macules and learning disability in contrast to those with TSC1 mutations, findings not noted in previous studies. We also observed results consistent with two similar studies suggesting that individuals with mutations in TSC2 have more severe symptoms. On performing meta-analyses of our data and the other two largest studies in the literature, we found significant correlations for several features that individual studies did not have sufficient power to conclude. Male patients showed more frequent neurologic and eye symptoms, renal cysts, and ungual fibromas. Correlating genotypes with phenotypes should facilitate the disease management of tuberous sclerosis complex.

Long-term outcome of transcatheter embolization of renal angiomyolipomas due to tuberous sclerosis complex.

PURPOSE: Complications from renal angiomyolipomas (AMLs) are common in patients with tuberous sclerosis complex (TSC) and tumors greater than 4 cm are more likely to cause symptoms. AMLs are the most common cause of death in adults with TSC. We present our long-term experience with transcatheter tumor embolization as a definitive treatment for AMLs due to TSC. MATERIALS AND METHODS: A total of 16 patients with TSC between 7.5 and 47.2 years old with symptomatic or large (4 to 21 cm) AMLs underwent embolization. Followup consisted of periodic physician visits or telephone contacts and renal imaging. RESULTS: The 16 patients underwent 18 treatment sessions to embolize 27 tumors. There were no intraoperative complications. The post-embolization syndrome occurred in 11 individuals but all responded to medical management. Two individuals had an arterial aneurysm within a tumor. The AML size decreased in the 13 patients who were imaged 3 months after treatment, and the 7 patients who were imaged 3 to 9 years after treatment have shown no tumor regrowth. No renal failure or hemorrhage has developed in patients following embolization. CONCLUSIONS: Transcatheter embolization of symptomatic or large AMLs due to TSC prevents hemorrhage and renal loss. The treatment is minimally invasive, preserves renal function, and can be performed multiple times. All of the patients who underwent followup renal imaging after embolization showed decreased AML size, and none of the 16 patients has developed renal loss or renal insufficiency in these individuals. Embolization should be considered the initial treatment of choice for large or symptomatic AMLs.

Seizure remission and antiepileptic drug discontinuation in children with tuberous sclerosis complex.

BACKGROUND: Epilepsy is a common neurologic complication of tuberous sclerosis complex (TSC) and it is often refractory to treatment. Therefore, treating physicians are often reluctant to discontinue antiepileptic drugs (AEDs) in individuals with TSC who have attained seizure remission. To our knowledge, seizure remission and AED discontinuation in children with TSC has not been studied. OBJECTIVE: To characterize seizure remission and AED discontinuation in children with TSC. METHODS: Retrospective medical record and neuroimaging analysis of 15 children with TSC and epilepsy who had seizure remission, with a subsequent trial of discontinuation of AED treatment. RESULTS: The seizure remission rate for the group of patients with TSC and epilepsy was 14.2%. From the group of 15 patients who had a remission, the absolute relapse rate was 26.7% after a mean follow-up of 5 years 7 months. Patients with sustained remission were more likely to have normal intelligence and only a few cortical or subcortical lesions on neuroimaging. CONCLUSIONS: The proportion of children with TSC and epilepsy who achieve seizure remission is small. Nevertheless, some do attain seizure remission, and AEDs may be successfully discontinued. Mild cerebral involvement is a general clinical marker for seizure remission. The relapse rate in those who have undergone a trial of discontinuation of AED therapy is comparable with the rate in the general pediatric population with epilepsy.