Geriatric Care Boot Camp: An Interprofessional Education Program for Healthcare Professionals

In response to the need for interprofessional geriatrics education, a half‐day geriatric care boot camp for healthcare professionals was held that covered core concepts in geriatric medicine: delirium and dementia, medication management, palliative care, ethics, and a general overview of older adults. Aspects of the curriculum focused on interprofessional education, and the attendees and presenters were healthcare professionals from a wide variety of fields. Primary objectives were to determine changes in knowledge of core concepts in geriatrics and level of comfort in caring for older adults. Secondary objectives assessed whether participants found the interprofessional approach beneficial and whether they used or shared this information with others in their professional activities. Participants completed pre‐ and postassessment surveys. Changes in participant understanding of each core concept were statistically significant, as was the change in comfort level of participants in caring for older adults. Furthermore, attendees found the multidisciplinary perspective of the boot camp beneficial. A 3‐month follow‐up survey assessed whether attendees applied and shared information learned in their own professional activities. Half of the respondents who reported sharing universally shared core concepts. Delirium and dementia information was most frequently shared. Information was most frequently shared with students, nurses, and patients’ families. Attendees less frequently shared, or did not share, with physicians, physician assistants, social workers, physical and occupational therapists, nutritionists, and dentists. The healthcare professionals who may benefit greatly from future education programs are those with whom the boot camp information was least frequently shared; thus, they are appropriate targets for advertisements for future programs.     

Molecular outcomes of neuromyelitis optica (NMO)-IgG binding to aquaporin-4 in astrocytes

The astrocytic aquaporin-4 (AQP4) water channel is the target of pathogenic antibodies in a spectrum of relapsing autoimmune inflammatory central nervous system disorders of varying severity that is unified by detection of the serum biomarker neuromyelitis optica (NMO)-IgG. Neuromyelitis optica is the most severe of these disorders. The two major AQP4 isoforms, M1 and M23, have identical extracellular residues. This report identifies two novel properties of NMO-IgG as determinants of pathogenicity. First, the binding of NMO-IgG to the ectodomain of astrocytic AQP4 has isoform-specific outcomes. M1 is completely internalized, but M23 resists internalization and is aggregated into larger-order orthogonal arrays of particles that activate complement more effectively than M1 when bound by NMO-IgG. Second, NMO-IgG binding to either isoform impairs water flux directly, independently of antigen down-regulation. We identified, in nondestructive central nervous system lesions of two NMO patients, two previously unappreciated histopathological correlates supporting the clinical relevance of our in vitro findings: (i) reactive astrocytes with persistent foci of surface AQP4 and (ii) vacuolation in adjacent myelin consistent with edema. The multiple molecular outcomes identified as a consequence of NMO-IgG interaction with AQP4 plausibly account for the diverse pathological features of NMO: edema, inflammation, demyelination, and necrosis. Differences in the nature and anatomical distribution of NMO lesions, and in the clinical and imaging manifestations of disease documented in pediatric and adult patients, may be influenced by regional and maturational differences in the ratio of M1 to M23 proteins in astrocytic membranes.     

Gastrointestinal hypomotility with loss of enteric nicotinic acetylcholine receptors: active immunization model in mice

Background Autoimmune gastrointestinal dysmotility (AGID) is a limited form of dysautonomia. The only proven effector to date is IgG specific for ganglionic nicotinic‐acetylcholine receptors containing α3 subunits [α3*‐ nicotinic acetylcholine receptor (nAChR)]. Rabbits immunized with recombinant α3‐polypeptide produce α3*‐nAChR autoantibodies, and profound AGID ensues. Human and rabbit α3*‐nAChR‐specific‐IgGs induce transient hypomotility when injected into mice. Here, we describe success and problems encountered inducing gastrointestinal hypomotility in mice by active immunization. Methods We repeatedly injected young adult mice of seven different strains susceptible to autoimmunity (spontaneous diabetes or neural antigen immunization‐induced myasthenia gravis or encephalomyelitis) with: (i) α3‐polypeptide, intradermally or (ii) live α3*‐nAChR‐expressing xenogeneic cells, intraperitoneally. We measured serum α3*‐nAChR‐IgG twice monthly, and terminally assessed blue dye gastrointestinal transit, total small intestinal α3*‐nAChR content (radiochemically) and myenteric plexus neuron numbers (immunohistochemically, ileal–jejunal whole‐mount preparations). Key Results Standard cutaneous inoculation with α3‐polypeptide was minimally immunogenic, regardless of dose. Intraperitoneally injected live cells were potently immunogenic. Self‐reactive α3*‐nAChR‐IgG was induced only by rodent immunogen; small intestinal transit slowing and enteric α3*‐nAChR loss required high serum levels. Ganglionic neurons were not lost. Conclusions & Inferences Autoimmune gastrointestinal dysmotility is inducible in mice by active immunization. Accompanying enteric α3*‐nAChR reduction without neuronal death is consistent with an IgG‐mediated rather than T cell‐mediated pathogenesis, as is improvement of symptoms in patients receiving antibody‐depleting therapies.     

Maternal vitamin K deficient embryopathy: Association with hyperemesis gravidarum and Crohn disease

Chondrodysplasia punctata (CDP) is an etiologically heterogeneous disorder characterized by the radiographic finding of stippled epiphyses (punctate calcifications). It is often accompanied by a characteristic facial appearance, known as the Binder phenotype, which is attributed to hypoplasia of the nasal cartilages; abnormal distal phalanges (brachytelephalangy) are a common component manifestation as well. We report eight patients with a Binder phenotype with or without CDP who all shared a known or suspected maternal deficiency of vitamin K. We suspect that this phenotype is probably under recognized, and we hope to increase awareness about the maternal risk factors, especially hyperemesis gravidarum, which lead to nutritional deficiency. © 2013 Wiley Periodicals, Inc.