Majewski Osteodysplastic Primordial Dwarfism, Type II (MOPD II) is a rare, autosomal recessive disorder. Features include severe intrauterine growth retardation (IUGR), poor postnatal growth (adult stature approximately 100 cm), severe microcephaly, skeletal dysplasia, characteristic facial features, and normal or near normal intelligence. An Institutional Review Board (IRB) approved registry was created and currently follows 25 patients with a diagnosis of MOPD II. Based on previous studies, a neurovascular screening program was implemented and 13 (52%) of these patients have been found to have cerebral neurovascular abnormalities including moyamoya angiopathy and/or intracranial aneurysms. The typical moyamoya pathogenesis begins with vessel narrowing in the supraclinoid internal carotid artery, anterior cerebral (A1) or middle cerebral (M1) artery segments. The narrowing may predominate initially on one side, progresses to bilateral stenosis, with subsequent occlusion of the vessels and collateral formation. We present four patients who, on neurovascular screening, were found to have cerebrovascular changes. Two were asymptomatic, one presented with a severe headache and projectile vomiting related to a ruptured aneurysm, and one presented after an apparent decline in cognitive functioning. Analysis of the registry suggests screening for moyamoya disease be performed at the time of MOPD II diagnosis and at least every 12-18 months using MRA or computerized tomographic angiography (CTA). We believe this is imperative. If diagnosed early enough, re-vascularization and aneurysm treatment in skilled hands can be performed safely and prevent or minimize long-term sequelae in this population. Emergent evaluation is also needed when other neurologic or cardiac symptoms are present.

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As hospital-acquired infections increase, it is essential that infection control practitioners and hospital administrators have an understanding of the perceptions and knowledge of health care providers as they relate to infection control practices. This article describes the use of the Health Belief Model, a theory-based model used to predict health-related behaviors, in assessing hospital clinical professionals' perceptions and knowledge of infection control practices and summarizes findings from an exploratory study conducted in a community hospital. A total of 130 providers within a hospital setting completed a 51-item survey instrument. The scores for the 6 Health Belief Model constructs show variation, with perceived severity, perceived benefits, and self-efficacy rated higher than perceived susceptibility and cues to action. Knowledge on hand hygiene practices was limited. Providers did not identify any perceived barriers or possible cues to action to increase the likelihood of engaging in proper infection control practices. The constructs of perceived susceptibility and cues to action show a need for improvement by determining appropriate cues for this workforce and addressing susceptibility of workers. These findings can be used by administrators and infection control practitioners to develop and disseminate educational and other interventions to increase compliance with infection control protocols.

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