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These guidelines are designed to assist the pediatrician in caring for the child in whom a diagnosis of Down syndrome has been confirmed by chromosome analysis. Although a pediatrician's initial contact with the child is usually during infancy, occasionally the pregnant woman who has been given a prenatal diagnosis of Down syndrome will be referred for review of the condition and the genetic counseling provided. Therefore, this report offers guidance for this situation as well.

Unlabelled: BACKGROUND; Delirium is a critical, costly, frequently reversible problem in older adults. Findings of previous studies indicate that delirium occurs in up to 65% of hospitalised older adults and up to 80% of terminally ill patients. Few studies address the frequency of delirium in community dwelling older adults and the extent to which delirium symptoms create distress for their family caregivers., Aims: To determine the frequency of delirium in older people attending two adult day centers (ADC) in the United States and identify the extent to which delirium symptoms were associated with family caregivers' mental health symptoms, and ways of coping with the older adults' care., Method: A descriptive, cross-sectional design was used. Thirty older adults and their family caregivers were randomly selected from the rosters of the ADC., Results: Only 6.7% of the older adults had a positive screen for delirium. The majority of family caregivers (96.6%) stated that they had no knowledge of delirium prior to participating in this study., Implications for Practice: Both older adults and their family caregivers need education about delirium symptoms and risks., (© 2010 Blackwell Publishing Ltd.)

Transporting children with special health care needs (CSHCN) may be complex and require a multidisciplinary approach to improve a child's comfort and safety. This review (adapted from the resource manual of the National Center for the Safe Transportation of Children with Special Health Care Needs.) will discuss the basic principles of child passenger safety for CSHCN, including types of child occupant restraints, the use of child occupant restraint devices (CRD) for selected CHSCN, and how to locate or, if needed, develop programs to train child passenger safety technicians (CPST) to help with safe transportation of CSHCN.

This study reviews trends, rear facing, top tether use, and seating position for children younger than 13y among motor vehicle passengers in Indiana. This is an observational, cross-sectional survey of drivers transporting children 15 years and younger and drivers collected at 25 convenience locations randomly selected in Indiana during summers 2005 through 2010. Observations were conducted by Certified Child Passenger Safety Technicians (CPST). As the driver completed a written survey collecting demographic data on the driver and children, the CPST recorded the vehicle seating location, the type of restraint, direction the car safety seat (CSS) was facing, and use of the CSS harness or safety belt as appropriate. Data was analyzed for infants younger than twelve months, children in forward facing CSS, and children < 13y. Between 2005 and 2010, 514 infants (age < 12m) were observed in motor vehicles. On average 83.5% (SD 4.8%) of the infants were rear facing. The percent of infants rear facing was 75.5% during 2005 and rose to 88.9% during 2010. Of the 442 vehicles observed with a forward facing car seat, 58% (SD 16.5%) had the top tether attached. In our sample, more than 88.7% (SD 0.8%) children < 13y were seated in a rear seat vehicle position. Driver variables affecting occupant protection are discussed. This information can be used by primary care providers and child passenger safety technicians and other child passenger safety advocates to develop counseling points and educational campaigns.

The relationship between the degree of GH deficiency and impaired bone integrity is not simple and may be influenced by related endocrine variables. To test the hypothesis that elevated adiposity and hyperleptinaemia are contributory factors, we quantified femoral trabecular organisation in two models of GH deficiency with divergent degrees of adiposity - the moderately GH-deficient/hyperleptinaemic transgenic growth retarded (Tgr) rat and the profoundly GH-deficient/hypoleptinaemic dw/dw rat. Trabecular density (bone volume/total volume) and surface were reduced by 16% in dw/dw males, with a more fragmented trabecular lattice. This impairment was more pronounced in Tgr rats, with trabecular number and density further reduced (by an additional 21%) and relative surface (bone surface/bone volume), trabecular convexity (structural modal index) and fragmentation (pattern factor) increased. To establish whether the presence of obesity/hyperleptinaemia exacerbates bone impairment in GH deficiency, trabecular structure was assessed in dw/dw rats following diet-induced obesity (DIO). DIO had minimal effect on trabecular architecture, the increased concavity of trabecular surfaces being the only observable effect. Similarly, infusion of leptin into the tibial bone marrow cavity had no effect on trabecular organisation or tibial growth in wild-type rats. However, while this procedure also failed to affect trabecular architecture or osteoclast number in dw/dw rats, distal osteoblast surface was increased by 23%, marrow adipocyte number and epiphyseal plate width being reduced (by 40 and 5% respectively), without increasing caspase-3 immunoreactivity. These findings suggest that while leptin may directly inhibit adipocyte differentiation and favour osteoblast production, hyperleptinaemia makes only a minimal contribution to the impairment of bone structure in GH deficiency.

Strategies for managing the nervous system are numerous while methods of evaluating the nervous system are limited. Given the physiological importance of neurotransmitters as signaling molecules in the nervous system, the measurement of neurotransmitters has significant potential as a clinical tool. Of all the biological fluids that can be utilized, urinary neurotransmitter testing, due to its stability, sensitivity, and non-invasiveness, is the desired method to analyze nervous system function. Increasing use of this technology in a clinical setting demands a review of its feasibility, utility, and clinical value. We review the current body of literature pertaining to the mechanism of neurotransmitter transport across the blood-brain barrier as well as neurotransmitter filtration and excretion by the kidneys. In addition, this review summarizes the historical use of urinary neurotransmitter assessment to diagnose pheochromocytoma. Early research also correlated urinary assessment of neurotransmitters to various clinical symptoms and treatments of which we present research only for depression, ADHD, and inflammation because of the abundant amount of research in these areas. Finally, we review the limitations and challenges of urinary neurotransmitter testing. Taken together, evidence suggests that neurotransmitters excreted in the urine may have a place in clinical practice as a biomarker of nervous system function to effectively assess disturbances and monitor treatment efficacy., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

Objective: We compared observed and reported practice among children with special health care needs transported in wheelchairs with the recommendations from the American National Standards Institute/Rehabilitation Engineering and Assistive Technology Society of North America Committee on Wheelchairs and Transportation voluntary standards for best practice for using wheelchairs in vehicles., Method: A convenience sample of vehicles exiting the garage of a children's hospital was observed. Certified child passenger safety technicians gathered driver demographics and the child's reported medical condition, weight, age, clinic visited, and relation to the driver. Technicians observed how the wheelchair and occupant were secured., Results: A sample of 20 vehicles showed that 90% used four-point tie-down systems to secure the wheelchairs. A total of 88% of drivers tied the wheelchairs down correctly; only 20% used a separate lap-shoulder belts to secure the occupants. Twenty-five percent used lap trays, which are not recommended. Fifteen participants traveled with medical equipment secured inappropriately., Conclusion: Many deviations from best practice were observed and highlight areas for increased awareness, education, and resources for caregivers.

Death receptor 3 is a proinflammatory member of the immunomodulatory tumor necrosis factor receptor superfamily, which has been implicated in several inflammatory diseases such as arthritis and inflammatory bowel disease. Intriguingly however, constitutive DR3 expression has been detected in the brains of mice, rats, and humans, although its neurological function remains unknown. By mapping the normal brain expression pattern of DR3, we found that DR3 is expressed specifically by cells of the neuron lineage in a developmentally regulated and region-specific pattern. Behavioral studies on DR3-deficient (DR3(ko)) mice showed that constitutive neuronal DR3 expression was required for stable motor control function in the aging adult. DR3(ko) mice progressively developed behavioral defects characterized by altered gait, dyskinesia, and hyperactivity, which were associated with elevated dopamine and lower serotonin levels in the striatum. Importantly, retrograde tracing showed that absence of DR3 expression led to the loss of corticostriatal innervation without significant neuronal loss in aged DR3(ko) mice. These studies indicate that DR3 plays a key nonredundant role in the retention of normal motor control function during aging in mice and implicate DR3 in progressive neurological disease.

Objective: We compare the use of the American Academy of Pediatrics (AAP) guidelines for the safe transportation of children with special health care needs (CSHCN) with reported and observed practices., Methods: This observational study was based on a convenience sample of vehicles exiting the garage of a tertiary children's hospital. Certified child passenger safety technicians with a health care background and specialized training in the transportation of CSHCN gathered the driver's demographic information and the child's reported medical condition, weight, age, clinic visited, and relation to the driver. The safety technicians observed the car safety seat (CSS) type, vehicle seating position, and if the child required postural support., Results: During the study, 275 drivers transporting 294 CSHCN were observed. Overall, most drivers complied with AAP recommendations by using a standard CSS seat (75.4%). Among the seats evaluated, 241 (82.0%) were the appropriate choice, but only 75 (26.8%) of 280 assessed had no misuses. Approximately 24% of the drivers modified the CSS, and 19.4% of the children would have benefited from additional body-positioning support. Only 8% of medical equipment was properly secured., Conclusions: Although most drivers seemed to choose the appropriate seat, many had at least 1 misuse. Drivers complied with most AAP recommendations; however, some deviated to facilitate care of the child during transport. Discussions with parents or caregivers about the proper transportation of CSHCN and referrals to child passenger safety technicians with special training may improve safety, care, and comfort in the vehicle.

Safe transportation of preterm and low birth weight infants requires special considerations. Both physiologic immaturity and low birth weight must be taken into account to properly position such infants. This clinical report provides guidelines for pediatricians and other caregivers who counsel parents of preterm and low birth weight infants about car safety seats.

Objective: Observe and report seat belt use among children transported in belt-positioning booster seats., Design: We conducted a cross-sectional, observational survey of children transported in motor vehicles between 2006 and 2007. While drivers completed a survey reporting the child's age, weight and gender, and the driver's age, gender, race, income, education, and relationship to the child; a child passenger safety technician recorded vehicle seating location, restraint type, and use of the car safety seat harness or seat belt as appropriate for the child., Setting: Twenty-five fast food restaurants and discount department stores throughout Indiana., Participants: A convenience sample of drivers transporting children younger than 16 years., Main Exposure: Seat belt use among children transported in belt-positioning booster seats., Outcome Measure: Seat belt misuse., Results: Overall, 1446 drivers participated, 2287 children were observed with 564 children in belt-positioning booster seats. At least one seat belt misuse was observed for 64.8% of the children transported. Common misuses were the shoulder belt being placed over the booster seat armrest (35.8%); shoulder belt not at mid-shoulder position (28.5%), seat belt was too loose (24.5%), and the shoulder belt was either behind the child's back (9.1%) or under their arm (10.0%)., Conclusion: There is a high frequency of seat belt misuses among children transported in booster seats. Advice to parents on appropriate car seat selection, and encouragement to parents to supervise seat belt use may decrease misuse.

Objectives: Special health care needs associated with behavioral conditions may influence a child's safety in motor vehicle crashes. The aim of this study was to describe and compare variation in restraint use, seating position, and injury risk in motor vehicle crashes among children with and without special health care needs likely to affect behavior., Patients and Methods: This study uses data collected between December 1, 1998, and November 30, 2002, in a cross-sectional study of children <16 years of age who were involved in crashes of State Farm-insured vehicles in 15 states. Parent reports via a validated telephone survey were used to define precrash special health care needs, restraint status, seating position, and the occurrence of clinically significant injuries by using a previously validated survey instrument., Results: Complete data were collected for 14654 children aged 4 to 15 years, representing 171633 children in crashes. Of these, 152 children were reported to have a special need likely to affect behavior, representing 1883 children. A greater proportion of children with special needs likely to affect behavior were appropriately restrained, particularly among children aged 4 to 8 years. Drivers of children with special needs likely to affect behavior were more often restrained and more often were the child passenger's parent. There were no differences in the rates of front-row seating. There was no significant association between the presence of a special need likely to affect behavior and risk of injury, after adjustment for child/driver characteristics and crash severity., Conclusions: Despite a greater proportion of children with special needs likely to affect behavior using proper vehicle restraint, their injury risk was similar to that of children without these special needs. Primary care pediatricians providing best practices for vehicle safety should consider the unique riding experience and risk of injury among children with special health care needs likely to affect behavior.

We report on a family with an apparently undescribed syndrome involving joint contractures, keloids, an increased optic cup-to-disc ratio, and renal stones. The propositus presented at 9 years of age with congenital contractures of his distal interphalangeal (DIP) joints, progressive stiffness of the shoulders and neck, a large optic cup-to-disc ratio, and mildly dysmorphic facies. He otherwise has been normal. The propositus's brother, uncle, great-uncle, a half great-uncle, and six male half-cousins once removed also have DIP joint stiffness. The propositus' mother and brother also have an increased optic cup-to-disc ratio. In addition, the great-grandfather, the uncle, the same six male half-cousins once removed, and an affected daughter of a half-cousin have keloids with the earliest age of onset of the keloids being 12 years. Further, three of the above affected males have had uric acid renal stones. Dupuytren contractures and subcutaneous nodules were also present in some affected males. Given the family history, the pattern of inheritance appears to be X-linked. An extensive literature search failed to reveal any previous reports associating these manifestations and this inheritance pattern. Therefore, we think the condition in this family comprises a new genetic syndrome., (Copyright (c) 2008 Wiley-Liss, Inc.)

Rheumatoid arthritis (RA) is a chronic inflammatory disease of synovial joints that is associated with cartilage and bone destruction. Death Receptor 3 (DR3), a tumor necrosis factor (TNF) receptor superfamily member, has recently been associated with the pathogenesis of RA. We demonstrate that absence of DR3 confers resistance to the development of adverse bone pathology in experimental antigen-induced arthritis (AIA). DR3(ko) mice exhibited a reduction in all histopathological hallmarks of AIA but, in particular, failed to develop subchondral bone erosions and were completely protected from this characteristic of AIA. In contrast, TNF-like protein 1A (TL1A), the ligand for DR3, exacerbated disease in a dose- and DR3-dependent fashion. Analysis of osteoclast number within AIA joint revealed a reduction in areas susceptible to bone erosion in DR3(ko) mice, whereas in vitro osteoclastogenesis assays showed that TL1A could directly promote osteoclastogenesis in mouse and man. Treatment with antagonistic anti-TL1A mAb protected animals in a systemic model of RA disease collagen-induced arthritis. We therefore conclude that the DR3-TL1A pathway regulates joint destruction in two murine models of arthritis and represents a potential novel target for therapeutic intervention in inflammatory joint disease.

Family caregivers provide more than 80% of the long-term care for elders in the United States and experience a variety of transitions that are intertwined with those of their elders. Previous research on health care transitions documents problems elders and family caregivers encountered following hospitalization, following nursing home admission, and in adjusting to home health care. Little is known about the transition to adult day health services (ADHS). The purpose of this study was (a) to examine family caregivers' perspectives on the transition to ADHS and (b) to develop a situation-specific theory to guide interventions for elders and their family caregivers during the transition. Semistructured interviews were conducted with 16 family caregivers 1 month and 3 months following the elder's enrollment in ADHS. The constant comparative method was used in analyzing the data. The findings indicate that family caregivers consistently sought what was best for elders throughout the transition.

Health disparities are a major public health problem in the United States. Doctorally prepared nurse scholars, who understand factors contributing to these disparities, possess research competence to study these factors, and pose strategies to be tested are essential to improve the health of these vulnerable populations. This article describes how a PhD curriculum was designed to prepare teacher-scholars to assume leadership roles in reducing health disparities.

This study describes the previously uncharacterized ontogeny and regulation of truncal adipose reserves in the profoundly GH-deficient dwarf (dw/dw) rat. We show that, despite normal proportionate food intake, dw/dw rats develop abdominal leanness and hypoleptinemia (circulating leptin halved in dw/dw males, P < 0.05) during puberty. This contrasts with the hyperleptinemia seen in moderately GH-deficient Tgr rats (circulating leptin doubled at 6 wk of age, P < 0.05) and in GH receptor-binding protein (GHR/BP)-null mice (circulating leptin doubled; P < 0.05). This lean/hypoleptinemic phenotype was not completely normalized by GH treatment, but dw/dw rats developed abdominal obesity in response to neonatal MSG treatment or maintenance on a high-fat diet. Unlike Tgr rats, dw/dw rats did not become obese with age; plasma leptin levels and fat pad weights became similar to those in wild-type rats. In contrast with truncal leanness, tibial marrow adiposity was normal in male and doubled in female dwarves (P < 0.01), this increase being attributable to increased adipocyte number (P < 0.01). Neonatal MSG treatment and high-fat feeding elevated marrow adiposity in dw/dw rats by inducing adipocyte enlargement (P < 0.05). These results demonstrate that, despite lipolytic influence of GH, severe GH deficiency in dw/dw rats is accompanied by a paradoxical leanness. This lean/hypoleptinemic phenotype is not solely attributable to reduced GH signaling and does not appear to result from a reduction in nutrient intake or the ability of dw/dw adipocytes to accumulate lipid. Disruption of preadipocyte differentiation or adipocyte proliferation in the dw/dw rat may lead to the development of this unusually lean/hypoleptinemic phenotype.

Background: Suicide is an important public health concern. Firearms are the most common mechanism of suicide death. This study describes the epidemiology of fatal and nonfatal firearm suicide injuries (FSI) in one metropolitan area from 2002 through 2004 using a firearm injury surveillance system., Methods: Records were obtained of all victims of firearm injuries from hospitals, police, and the coroner. All injuries categorized as suicide were included., Results: Local age adjusted suicide rates were significantly higher than state or national rates for ages 15 to 24, and significantly higher than national rates for ages 25 to 44. Men were FSI victims more than five times as often as women were. There was no seasonal pattern identified. Handguns were used nearly three out of four times. Eighty-six percent of FSI victims died, two-thirds at the scene. Most wounds were in the head or chest. Mental illness or relationship problems were common. Most suicides occurred in a residence., Conclusions: Community level firearm injury surveillance effectively identifies local trends that may differ from national statistics. Collaboration among various groups is used to support injury prevention programs. These data can both complement and contribute to national statistics.

We report a case of local compression-induced transient femoral nerve palsy in a 46-year-old man. He had previously undergone surgical release of the soft tissues anterior to both hip joints because of contractures following spinal injury. An MRI scan confirmed a synovial cyst originating from the left hip joint, lying adjacent to the femoral nerve. The cyst expanded on standing, causing a transient femoral nerve palsy. The symptoms resolved after excision of the cyst.

Objective: The purpose of this work was to describe the epidemiology of nonfatal school bus-related injuries among children and teenagers aged < or = 19 years in the United States., Design/methods: Nationally representative data from the National Electronic Injury Surveillance System All-Injury Program operated by the US Consumer Product Safety Commission were analyzed. Case subjects included all of the patients in the National Electronic Injury Surveillance System All-Injury Program database who were treated in a hospital emergency department for a nonfatal school bus-related injury from 2001 to 2003., Results: There were an estimated 51,100 school bus-related injuries treated in US emergency departments from 2001 to 2003, for a national estimate of 17,000 injuries (rate: 21.0 per 100,000 population) annually. Ninety-seven percent of children were treated and released from the hospital. Children 10 to 14 years of age accounted for the greatest proportion of injuries (43.0%; rate: 34.7) compared with all other age groups. Motor vehicle crashes accounted for 42.3% of all injuries, followed by injuries that occurred as the child was boarding/alighting/approaching the bus (23.8%). Head injuries accounted for more than half (52.1%) of all injuries among children < 10 years of age, whereas lower extremity injuries predominated among children 10 to 19 years of age (25.5%). Strains and sprains accounted for the highest percentage of all injuries, followed by contusions and abrasions (28.3%) and lacerations (14.9%). More than three quarters (77.7%) of lacerations were to the head., Conclusions: This is the first study to describe nonfatal school bus-related injuries to US children and teenagers treated in US hospital emergency departments using a national sample. This study identified a much greater annual number of school bus-related injuries to children than reported previously.

Background: Studies demonstrate an increased prevalence of celiac disease in persons with Down syndrome, leading some organizations and authors to recommend universal screening of children with Down syndrome. However, many children with Down syndrome are asymptomatic, and the long-term implications of screening are unknown. The complication of celiac disease that leads to mortality in the general population is non-Hodgkin's lymphomas., Objectives: The purpose of this research in asymptomatic children with Down syndrome was to (1) calculate the number needed to screen to prevent a single case of lymphoma and (2) present a cost-effectiveness study of screening., Methods: We constructed a decision tree using probabilities derived from the published literature for Down syndrome or from the general population where Down syndrome-specific data were not available. Celiac disease was determined by serologic screening and confirmation with intestinal biopsy. Sensitivity analysis was used to alter probability estimates affecting the cost of preventing lymphoma., Results: Using our baseline values, the no-screen strategy is dominant; that is, screening not only costs more but also results in fewer quality-adjusted life-years. A screening strategy costs more than $500,000 per life-year gained. Screening all asymptomatic children with Down syndrome for celiac disease costs almost $5 million to prevent a single case of lymphoma., Conclusion: These analyses do not support the cost-effectiveness of screening, and more data are needed before recommendations to screen asymptomatic children with Down syndrome for celiac disease can be made.

Background: Cleidocranial dysplasia (CCD) is an autosomal-dominant skeletal dysplasia syndrome that is characterized by widely patent calvarial sutures, clavicular hypoplasia, supernumerary teeth, and short stature. CCD is caused by mutations in the transcription factor RUNX2, which is known to function as a major regulator of bone differentiation. Despite the characterization of 67 unique mutations in 97 individual cases, and the availability of animal models, no obvious genotype-phenotype correlation has emerged., Methods: We describe 3 new cases that were ascertained on the basis of a severe calvarial phenotype, that were associated with 3 novel mutations in the C-terminal region of RUNX2 distal to the DNA-binding runt domain. In addition, a review of all previously described cases was undertaken in an effort to standardize mutation nomenclature, characterize the position of known mutations relative to the runt domain, and explore the hypothesis that C-terminal mutations that preserve the runt domain may lead to more-severe craniofacial phenotypes., Results: Upon mutational analysis of RUNX2, we identified either frameshift or splice-site mutations that affect the C-terminal region of the resultant protein distal to the runt domain., Conclusions: In the context of previously described mutations, these cases suggest that C-terminal mutations that preserve the DNA-binding runt domain while disrupting the SMAD 1,2,3,5 binding domain and the nuclear matrix targeting signal may be responsible for the severe phenotype observed., (Birth Defects Research (Part A) Birth Defects Research (Part A), 2006. (c) 2006 Wiley-Liss, Inc.)

The Marshall-Smith syndrome (MSS) is a distinct malformation syndrome characterized by accelerated skeletal maturation, relative failure to thrive, respiratory difficulties, mental retardation, and unusual facies, including prominent forehead, shallow orbits, blue sclerae, depressed nasal bridge, and micrognathia. At least 33 cases have been reported in the literature, mostly as single case reports or small series. The purpose of the present study is to report on the clinical findings and natural history of MSS in five children and to review the features of three others previously reported, with particular attention to the skeletal and connective tissue findings. Our study demonstrates an increased rate of nontraumatic fractures and other bony and connective tissue abnormalities that support the hypothesis that MSS should be considered an osteochondrodysplasia. In addition, long-term survival beyond infancy is possible if respiratory problems are expectantly and aggressively managed., ((c) 2005 Wiley-Liss, Inc.)

Objective: The role of multiplanar image reconstruction (MPR) in staging lung cancer was investigated using multislice helical computed tomography (CT), which allows high-quality volumetric imaging., Methods: Forty-one consecutive patients with lung cancer (mean age = 71 years) underwent multislice CT of the thorax. The scans were acquired using contiguous 4-mm x 2.5-mm slices from the lung apex to the diaphragm in a single breath hold after injection of 100 mL intravenous contrast media. Contiguous axial, coronal, and sagittal images (5-mm slice thickness) were reconstructed in the lung and mediastinal windows. The axial images with and without multiplanar reformatted images were reviewed on a workstation on 2 separate occasions (a minimum of 6 weeks apart) by 2 experienced chest radiologists. The films were assessed for features relating to the primary lesion (size; location; and invasion of the chest wall, mediastinum, diaphragm, and/or fissures) and secondary features (mediastinal lymphadenopathy and lung metastases). The diagnostic confidence of each feature was expressed on a 4-point scale., Results: A significant increase in confidence was seen on the part of both observers when diagnosing features relating to the primary lesion. The mean confidence score increased from 1.68 to 2.08 (P = 0.038) for observer A and from 1.50 to 1.80 (P = 0.020) for observer B. Confidence in assessing invasion of fissures was increased from 1.70 to 2.30 (P = 0.022) for observer A and from 1.67 to 2.27 (P = 0.006) for observer B. Improvement in interobserver agreement (kappa-value from 0.61 to 0.75) was observed with multiplanar reconstruction (MPR) in the assessment of tumor location. No statistical difference was demonstrated in the diagnosis of mediastinal lymphadenopathy or lung secondaries., Conclusion: Multiplanar imaging of the thorax is a useful supplementary tool in the staging of lung cancer, particularly in delineating the relation of the primary lesion to fissures and the diaphragm.

We examined 23 consecutive patients (11 males and 12 females with mean age of 56 years) with possible airway diseases to assess the impact of multiplanar image reconstruction (MPR) on the degree of confidence and accuracy in diagnosing bronchial abnormalities and emphysema. The thorax was scanned contiguously at 1 mm slice thickness using Siemens Volume Zoom Multislice CT scanner. Images were reconstructed at 1 mm slice thickness (lung windows L-600HU W-1600HU utilizing high spatial frequency algorithm) in the axial (10 mm apart), sagittal (4 images per lung) and coronal (6 images) plane. Paddle wheel image reconstructions were also performed in the assessment of bronchiectasis. Axial images were assessed with and without the help of MPR by three chest radiologists at two separate occasions (at least 4 weeks apart). The presence of bronchiectasis, emphysema and bronchiolitis in each lobe was documented on a confidence scale of 0 to 3. The overall mean confidence for each observer with and without MPR was compared. Consensus diagnosis was used as the gold standard for the assessment of the diagnostic accuracy of each observer. A confidence score of 2 or more for any lobe was considered diagnostic of the particular airway disease. The diagnostic accuracy for each observer with and without MPR was compared. Consensus reporting diagnosed bronchiectasis in 7 patients (30.4%), bronchiolitis in 5 patients (21.7%) and emphysema in 12 patients (52%). MPR did not increase the confidence of assessing the different abnormalities for all observers but improvement in diagnosing bronchiectasis was noted in two observers. The improvement did not reach statistical significance. However, agreement between observers in the diagnosis of bronchiectasis and emphysema was improved when the MPR images were used in conjunction with standard axial imaging (Kappa statistic improved from 0.29 to 0.54 for bronchiectasis and from 0.7 to 0.81 for emphysema). Agreement on the diagnosis of bronchiolitis was not improved by MPR for all observers. Our results suggest that MPR seems to improve the confidence in diagnosing bronchiectasis and emphysema.

The aim of the study was to investigate the incidence of thyroid abnormalities in neck irradiated lymphoma patients. Of the 298 patients who had irradiation to the neck for lymphoma between 1966-1988, 174 were found to be alive and free of disease. These patients were invited to participate in the study. From the 174, 93 were able to participate (group 1). Two control groups were recruited; both were sex and aged matched. One group (group 2) consisted of lymphoma patients who were treated with chemotherapy (n=39) or irradiation to areas other than the neck (n=16). The other group (group 3) consisting of healthy volunteers (n=35) recruited from hospital staff and minor surgery attendees, had never had lymphoma or radiotherapy. All participants were required to complete a past medical history and thyroid symptom questionnaire, had blood taken for assays of thyroid stimulating hormone (TSH), thyroglobulin antibodies, thyroid peroxidase antibodies, sodium iodide symporter antibodies and TSH receptor antibodies and underwent ultrasound and clinical examination of the neck. A significant percentage of patients who had been irradiated in the neck had abnormalities on ultrasound, compared to groups 2 and 3 (77% vs 42% vs 24%). Abnormal TSH levels were found to be significantly more common in neck irradiated patients compared to the other groups (50% vs 9% vs 5%). There is a clear difference between neck irradiated patients and control groups. The importance of screening irradiated patients for thyroid abnormalities is re-emphasised.

Lawn mower-related injuries to children are relatively common and can result in severe injury or death. Many amputations during childhood are caused by power mowers. Pediatricians have an important role as advocates and educators to promote the prevention of these injuries.

HLA-B27 is strongly linked with a group of human diseases called spondyloarthropathies. Even though HLA-B27 as an MHC class I molecule would be expected to present endogenously processed peptides such as cytosolic or viral proteins, many of the B27-linked diseases begin after an infection with an enterobacteria, an exogenous antigen. In our previous studies, we have described development of spontaneous inflammatory disease in HLA-B27 transgenic mice expressing beta(2)m free heavy chains on the cell surface. In order to address the role of endogenous versus exogenous antigens and a role for Tap genes in the development of spontaneous diseases, mice lacking Tap-1 (knockout) were mated to HLA-B27/human beta(2)m transgenic mice. B27(+)/human beta(2)m(+) double-transgenic mice (without mouse beta(2)m) lacking the Tap-1 gene developed spontaneous inflammatory disease similar to wild-type Tap-1 gene-expressing counterparts. Our data demonstrate that peptide transporters (Tap) were not involved in the development of spontaneous inflammatory disease in B27(+)/human beta(2)m transgenic animals., (Copyright 2001 Academic Press.)

The objectives of the study were to determine the most effective and reliable means of restraining children on an ambulance cot and to develop recommended field procedures for emergency medical service providers. A series of crash tests at 48 km/h were conducted using convertible child restraints, car beds, and harness systems tested with 3-year, infant, and 6-year size dummies. Belt configuration and backrest position were varied. A new cot and fastener system significantly improved restraint performance over older systems previously tested. A two-belt attachment with elevated cot backrest was found to be the method with the least performance variability for securing either a convertible child restraint or a car bed. It was concluded that children who weight up to 18 kg, fit in a convertible child restraint, and can tolerate a semi-upright seated position can be restrained in a convertible child restraint secured with two belts to an ambulance cot. Infants who must lie flat can be restrained in a car bed modified for two seatbelt paths and secured to a cot. In each case, the cot backrest must be elevated, and the cot and anchor system must be crashworthy. None of the harness configurations tested proved to be satisfactory, but an effective system could be developed by following accepted restraint design principles.

Family caregivers play vital roles in assisting elders after they are released from the hospital. Although health care professionals advocate involving family caregivers in discharge planning for elders, little is known about the extent to which this involvement benefits or jeopardizes the caregiver's health and their perceptions of the caregiving experience. The purpose of this study was to determine whether the level of family caregiver involvement in discharge planning for an elder made a difference in caregiver health, discharge planning satisfaction, perception of care continuity, preparedness to assist the elder, and acceptance of the caregiving role 2 weeks and 2 months postdischarge. The sample consisted of 130 family caregivers for elders hospitalized with heart failure. Telephone interviews were conducted 2 weeks and 2 months postdischarge. The findings indicated that family caregivers who reported more involvement in discharge planning had significantly higher scores on satisfaction, feelings of preparedness, and perception of care continuity 2 weeks following the elder's hospitalization than those who reported little or no involvement in planning. Caregivers who reported more involvement in planning also were more accepting of the caregiving role. At 2 months postdischarge, caregivers who reported more involvement in discharge planning reported better health and more acceptance of the caregiving role than those who had little or no involvement in planning.

Client satisfaction is considered an important outcome measure in a managed care environment faced with escalating health care costs, shortened lengths of hospital stay, and competition among acute care hospitals. With shortened lengths of stay in acute care hospitals, discharge planning has assumed increased importance, particularly for elders who have chronic conditions, such as heart failure, that require follow-up care. Consequently, understanding the predictors of client satisfaction with discharge planning can help hospitals and their nursing staff to tailor services to meet client needs. Previous studies have focused on patient satisfaction with hospital care, with little attention given specifically to satisfaction with discharge planning and to family caregiver satisfaction with discharge planning. The purpose of this study was to determine whether there is a difference between elder and family member satisfaction with discharge planning 2 weeks after hospitalization and what factors predict satisfaction with discharge planning 2 weeks after hospitalization for elders hospitalized with heart failure and their family caregivers. Telephone interviews were conducted with 134 elder/family caregiver dyads 2 weeks after hospitalization. The results indicated that there were no statistically significant differences in discharge planning satisfaction of elders and their family caregivers. Continuity of care and extent to which they felt prepared to manage care following hospitalization were the best predictors of elder's and family caregiver's satisfaction with discharge planning.

Despite efforts to improve the discharge planning process and subsequent outcomes, existing mechanisms fail to accurately identify elders' needs for follow-up care. Studies report rehospitalization rates ranging from 12 to 50%. The two aims of this study were to (1) examine the difference in outcomes for elders hospitalized with heart failure and caregivers who participated in a professional-patient partnership model of discharge planning compared to those who received the usual discharge planning and (2) examine differences in costs associated with hospital readmission and use of the emergency room following hospital discharge. A before-and-after nonequivalent control group design was used for this study. Data were collected from the control and the intervention cohorts before discharge and at 2 weeks and 2 months postdischarge. One hundred and fifty-eight patient-caregiver dyads completed both the predischarge and 2-weeks postdischarge interviews; 140 also completed a 2-month follow up. The average age of elders was 73.7 years; the average age of the caregivers was 58.5 years. The findings indicated that elders in the intervention cohort felt more prepared to manage care, reported more continuity of information about care management and services, felt they were in better health, and when readmitted spent fewer days in the hospital than the control cohort. Caregivers in the intervention cohort also reported receiving more information about care management and having a more positive reaction to caregiving 2 weeks postdischarge than the control cohort.

The NHS and Community Care Act 1990 emphasized the importance of discharge planning for elders and recognized the role of community health nurses in promoting continuity of care. Given the emphasis on discharge planning for vulnerable populations at policy level, it is timely for nurses to examine the research literature for evidence that may guide practice. This article reviews the key findings of research related specifically to discharge planning for older people and suggests areas in which additional research is needed.

Ten patients with clinically suspected neuromas following amputation were submitted for ultrasound examination. Neuromas in seven of the ten patients were identified as the cause of pain with ultrasound guided infiltration of local anaesthetic. Pre operative localisation, using breast localisation wires allowed the neuromas to be surgically excised with reduced dissection. This small study demonstrates that ultrasound is an effective method for identifying and localising neuromas in amputation stumps. This leads to reduced dissection and may lead to a better outcome in these patients.

Human spondyloarthropathies are strongly associated with a major histocompatibility complex (MHC) class I allele, HLA-B27. HLA-B27 transgenic mice and rats demonstrate many features of these diseases further confirming the role of HLA-B27 in disease. Yet the exact role of this molecule in disease pathogenesis is not clearly understood. We have previously reported spontaneous arthritis and nail disease in HLA-B27 transgenic mice lacking beta2-microglobulin (B27+beta2m(o)). These observations along with binding studies of B27 derived peptides to HLA-B27 molecule itself led to two hypotheses: (i) HLA-B27 derived peptide as a source of autoantigen; and (ii) HLA-B27 functions as an antigen presenting molecule. In this report, we confirm spontaneous disease in transgenic mice expressing a hybrid B27 molecule with alpha1alpha2 domain of B27 and alpha3 domain of mouse H-2Kd. These mice developed spontaneous arthritis and nail disease when transferred from specific pathogen free barrier facility to the conventional area. Other control mice with MHC class I transgene (e.g., HLA-B7, HLA-Cw3, and H2-Dd) did not develop such disease. In a MHC reassembly assay, binding of similar peptides to both wild type and hybrid B27 molecules was observed. In addition, the hybrid B27 molecule lacks at least one of the 3 proposed peptides from the third hypervariable (HV3) region of HLA-B27. These data strongly suggest that HLA-B27 molecule is an antigen presenting molecule rather than a peptide donor in the disease pathogenesis.

Introduction: This research describes temperament characteristics of infants with Down syndrome who are 1 to 4 months old. In the literature children with Down syndrome have been stereotyped. It was hypothesized that temperament characteristics may be evident and unique in newborns with Down syndrome., Method: A standardized questionnaire, The Early Infancy Temperament Questionnaire, a cover letter, informed consent forms, and a brief explanation of the project were given to families with a newborn infant with Down syndrome. Thirty-two (families with infants with Down syndrome completed the study., Results: Mean scores indicate that infants with Down syndrome are rated more active, less intense, more distractible, and have a tendency to demonstrate more approach behaviors compared with normally developing infants. Data suggest infants with Down syndrome have some unique characteristics but are more like their normally developing peers than different., Discussion: Integration and mainstreaming of infants and children with Down syndrome emphasizes the need for nurse practitioners to appreciate the temperament characteristics of these infants.

We describe 3 sibs (2 males and 1 female) with multiple congenital anomalies, poor growth, seizures, and progressive central nervous system (CNS) degeneration leading to death in infancy. Radiographic changes in all 3 were similar, and included moderate shortness of long bones, platyspondyly, and hypoplastic pelvis. Autopsies showed diffuse encephalomyelopathy and enlargement of the lateral and third ventricles. Lysosomal enzyme activities were normal. Collagen type II analysis on 2 of the sibs indicated normal collagen. Chromosomes appeared normal. Even though the radiographic and chondroosseous morphologic findings in these sibs have a certain similarity to Dyggve-Melchior-Clausen syndrome, their clinical course does not fit this condition. These infants appear to represent a new syndrome of bone dysplasia and CNS degeneration inherited as an autosomal recessive trait.

Although association of HLA-B27 with human spondyloarthropathies has been known for several years, its role in disease pathogenesis is not understood. Recently, a few investigators have proposed that presentation of B27-derived peptides by MHC class II molecules may be the underlying mechanism. HLA-B27 transgenic rat and mouse models have provided a new tool for understanding the exact role of B27 in disease pathogenesis. HLA-B27 mice lacking endogenous beta2-microglobulin (B27+ beta2m(o)) develop disease after they are transferred from the barrier facility to the conventional colony. This model was utilized to test the hypothesis that B27-derived peptide presented by MHC class II molecules is the cause of the disease. The MHC class II knockout gene, A beta(o), was bred into our B27+ beta2m(o) mice, and disease manifestation was monitored. These mice develop spontaneous disease, demonstrating that MHC class II molecules do not play a major role in B27-related disease. Thus, the disease is not manifested by presentation of B27-derived peptides by class II molecules, since these mice are devoid of H2-A and H2-E molecules. Furthermore, in vivo treatment with mAb against the heavy chain of B27 reduced the incidence of disease in B27+ beta2m(o) mice. Our results clearly demonstrate that B27 heavy chains are directly involved in the disease process.

Livedo reticularis is a vascular abnormality of the skin resulting in an erythematous reticular rash. The combination of livedo reticularis and stroke-like episodes in adults is known as Sneddon syndrome [Sneddon, IB (1965). Br J Dermatol 77:180-188]. A similar combination of stroke-like episodes and livedo reticularis has been reported to occur in children [Baxter P et al. (1993). Dev Med Child Neuro 35:917-926]. We present here a 7-year-old male with congenital livedo reticularis, obesity, developmental delay, stroke-like episode, hypertension and cystic kidneys. We summarize our patient's findings and family history, and compare his disorder to other possibly related conditions.

We present the findings of 13 additional cases of the urorectal septum malformation (URSM) sequence, and review the literature. The URSM sequence consists of ambiguous genitalia concurrent with absence of perineal and anal openings. The sex ratio of the 13 new cases was 7 males to 6 females and from the literature 21 males and 28 females. In addition, 11 of the 13 new cases had anorectal atresia with 5 of the cases also having partial agenesis of the colon. Bilateral renal agenesis was present in 3 of the 13 cases, unilateral renal agenesis occurred in 6, and dysplastic kidneys were found in 10. The URSM sequence is a lethal condition with long-term survival reported in only 3 of a total of 62 literature and new cases. Recurrence of this condition has not been reported.

The purpose of this study was to examine the effect of family caregivers' perceptions of elders' behaviors on caregiver burden and health while controlling for other factors often associated with burden. The model tested included the following concepts: elders' health, caregivers' perceptions of elder behaviors, caregiving involvement, coping, discrepancy in social support, caregiver response and caregiver health. Data for the analyses were obtained from a longitudinal study of 346 elder/caregiver dyads in which the elders had been hospitalized for acute episodes of chronic conditions. The sample for these analyses consisted of a subset of 118 dyads from the longitudinal project. A structural equation approach using the computer software Linear Structural Relations (LISREL) was used in analyzing the data. The findings indicated that the data fit reasonably well with some of the general patterns hypothesized in the model; the Goodness of Fit Index was 0.85 and the Tucker-Lewis Fit index, .88. More important than overall fit were specific relationships. Caregivers' perception of elders' behaviors was directly linked with caregiving response two weeks postdischarge; caregiver response directly linked with caregiver health at two weeks postdischarge but not at two months postdischarge. The findings provide support for Lazarus and Folkman's theory that appraisal of the situation influences response.

We describe 13 unrelated children with abnormalities of somatic growth, face, brain, and connective tissue including vasculature. Although the condition in these children falls under the general group of disorders known as cutis marmorata telangiectatica congenita (CMTC), the constellation of abnormalities appears to constitute a distinct and easily recognizable phenotype within this general group. In contrast to most children reported with CMTC, children in this subgroup have a high risk for neurologic abnormalities, including developmental delay, mental retardation, megalencephaly, and hydrocephalus. Early recognition of this condition is important for appropriate surveillance for known complications and parental counseling.

Objective: Dysphagia aortica describes swallowing difficulty caused by external compression from a tortuous or aneurysmal aorta. We present 5 cases in which dysphagia to solids accompanied a localised high pressure barrier (HPB) on manometry suggestive of dysphagia aortica, and explore other investigation modalities useful to confirm the diagnosis., Methods: Four females and 1 male with a median age of 56 years (range 47-58) were investigated. All underwent investigation with endoscopy, chest radiography, CT scanning, barium swallow, and video solid bolus swallow in addition to oesophageal manometry and 24 h ambulatory pH monitoring., Results: Median basal pressure rise at the mid oesophageal HPB was 45 mmHg (range 40-80). In addition to the HPB, 4 patients had manometric abnormalities of swallow activity and 2 patients had significant gastroesophageal reflux disease (GORD). Contrast enhanced computed tomography and barium swallow were normal in all cases. Video bolus swallow showed pronounced obstruction to transit at the aortic arch in 2 cases and excluded significant dysphagia aortica in 2 others., Conclusions: Dysphagia aortica commonly coexists with motility disorders and GORD. Video solid bolus swallow allowed us to determine the clinical significance of a manometric HPB in 4 out of 5 patients suspected on dysphagia aortica where standard evaluation would have failed. We recommend its use in those patients with a manometric HPB suggestive of dysphagia aortica in whom standard barium swallow is normal.