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Introduction: Traumatic brain injury (TBI) can disrupt the hypothalamo-pituitary axis, causing central neuroendocrine dysfunction and hormone abnormalities (HPAD). As one-third of children can develop post-traumatic HPAD, a longitudinal and thorough follow up may be required to exclude the transient or late-onset HPAD. Objective: To implement a protocol for standardized evaluation of children with TBI for the diagnosis of HPAD and to effectively establish a regular inpatient endocrine consultation and outpatient longitudinal follow up. Method: The study was divided into pre-QI (baseline phase) and QI phase (post-intervention phase). During the pre-QI phase, retrospective data were collected on children admitted with TBI at our institution for 1 year. The prevalence of HPAD and the percentage of children longitudinally followed in endocrine clinic were estimated. A consensus-based guidance protocol, detailing clinical and hormonal assay-based evaluation at presentation and during the follow up were formulated and implemented. Prospective data collection will be performed to estimate outcome measures (prevalence of HPAD, rate of initial endocrine consultation and endocrine outpatient follow up) and process measure (protocol adherence rate). Result: During the baseline phase (pre-QI), a total of 27 children, aged ≤19 years were admitted in the year for TBI management. The median (IQR) age at TBI diagnosis was 9 (3, 15) years. Motor vehicle accident was the predominant cause, accounting for 60%. In 85% of patients, the TBI was classified as severe based on GCS. Overall, only 8 children (30%) underwent limited (non-consultation based) endocrine evaluation (7 for central DI and 1 for central hypothyroidism) and 1 patient had complete evaluation (endocrinologist consulted). During the baseline period, the prevalence rate of transient central DI was diagnosed in 1 patient (4%). Implementation of protocol and post-intervention data collection are pending. Conclusion: The lower prevalence rate of HPAD in the current cohort of TBI patients may be due to under evaluation for endocrine dysfunction. QI initiative incorporating standardized evaluation using guiadance protocol will improve identification follow up rates of patients with endocrine dysfunction following TBI.

Background Retrievable inferior vena cava filters (rIVCF) reduce the short-term risk of pulmonary embolism without the filter and inferior vena cava (IVC) thrombosis that have been reported with the use of permanent filters. Studies have shown that most rIVCFs are not removed, leaving patients at risk for thrombotic complications of rIVCF retention. We hypothesize that the application of a systematic follow-up for rIVCF will improve filter removal rates, providing patients short-term prophylaxis from pulmonary embolism whereas avoiding complications of permanent filter retention. Methods The trauma registry of a Level I trauma center was queried to identify patients who underwent placement of IVCFs between January 1, 2003, and June 30, 2008. The medical records were reviewed and details of the patient's injuries, indications for filter placement, repositioning, and retrieval were collected. Radiographic images were reviewed to confirm ultimate filter retention or removal. Results Between January 1, 2003, and June 30, 2008, rIVCFs were placed in 118 patients, 44% had known venous thromboembolic event. Three patients died before rIVCFs could be considered for extraction, leaving 115 patients for evaluation. Filters were removed in 80 patients (70%) overall. Of the 35 patients in whom filters were not removed, 11 were lost to follow-up, 4 failed removal attempts, and 20 had indications for filter retention. The rIVCFs were retrieved in 75% of patients not lost to follow-up and 92% of patients who did not have contraindications for filter removal. Conclusion A dedicated system for following-up patients with rIVCFs markedly improves removal rates of retrievable filters.

Experimental and clinical evidence is accumulating that supports the assertion that the damaged human brain is capable of responding to sensory stimulation in a sufficient manner to result in sustainable and useful benefits. The intensity and duration of therapeutic maneuvers that elicit improvement are under active investigation. Recent studies in animals, adults, and children with hemiparesis have shown that constraint of the less involved upper limb coupled with a behavioral program that repetitively encourages graded unilateral movements can result in long-term “new” functional activities. Constraint-induced movement therapy (CIMT) is a promising approach for treatment of children with stroke-related hemiparesis from either prenatal or postnatal causes due to the enhanced neuroplasticity of the brain during early life.

Osteogenesis imperfecta (OI) is a group of connective tissue disorders that is associated with a variety of impairments. These include osteoporosis, fractures, hypermobility and hypomobility, changes in muscle physiology and bony deformity. Most of the literature and focused research to date has been on children with osteoporosis with fewer studies on adults. This chapter will use data from the OI Registry, case experience and the available literature to discuss the incidents of the types of impairments that are experienced by adults with OI, the factors associated with aging and injuries that occur in individuals with OI, and suggest therapeutic interventions for management.

Kennedy Krieger Institute, Baltimore, Maryland and Johns Hopkins University Purpose/Objective: Successful implementation of functional self-care skills depends upon adequate executive functioning; however, many scales assessing adaptive skills do not address the inherent executive burden of these tasks. This omission is especially relevant for individuals with spina bifida, for whom medical self-care tasks impose a significant burden requiring initiation and prospective memory. The Kennedy Krieger Independence Scales-Spina Bifida Version (KKIS-SB) is a caregiver-reported measure designed to address this gap; it assesses skills for managing both typical and spina bifida-related daily self-care demands, with a focus on the timely and independent initiation of adaptive skills. Research Method/Design: Parents of 100 youth and young adults with spina bifida completed the KKIS-SB. Exploratory factor analysis and Pearson's correlations were used to assess the factor structure, reliability, and construct validity of the KKIS-SB. Results: The scale demonstrates excellent internal consistency (Cronbach's alpha .891). Exploratory factor analysis yielded four factors, explaining 65.1% of the total variance. Two primary subscales were created, initiation of routines and prospective memory, which provide meaningful clinical information regarding management of a variety of typical (e.g., get up on time, complete daily hygiene routines on time) and spina bifida-specific self-care tasks (e.g., begin self-catheterization on time, perform self-examination for pressure sores). Conclusions/ Implications: Based upon internal consistency estimates and correlations with measures of similar constructs, initial data suggest good preliminary reliability and validity of the KKIS-SB.