Case 319: Multisystemic Smooth Muscle Dysfunction Syndrome.

HISTORY: A 7-year-old Chinese girl presented to a local hospital with a 5-day history of progressive right-sided hemiplegia, expressive aphasia, mild bulbar palsy, and reduced general responsiveness. At presentation, her Glasgow Coma Scale was 11/15 (E4 V1M6). Notably, she had two strokelike episodes approximately 7 and 3 months prior to the current episode, with headache, reduced movement, and numbness in the left hand. She also had an extensive medical history at a young age, including congenital mydriasis, patent ductus arteriosus with ligation, dysautonomia, low blood pressure, hypotonic bladder requiring intermittent catheterization, poor bowel transit, and gallstones. Her immunization record was up to date, and her development was otherwise unremarkable. Her parents and younger sibling were healthy. Her blood tests revealed a mildly increased venous lactate level at 2.3 mmol/L (normal range, 0.7-2.1 mmol/L), without acidosis. Results of a coagulopathy work-up (clotting profile and protein C, protein S, antithrombin 3, and fibrinogen levels) were normal. MRI and MR angiography of the brain were performed at presentation.

"And if my goal is never to leave Casey House?": The significance of place attachment for patients at a specialty HIV hospital in Toronto, Canada.

The current healthcare context prioritizes shorter hospital stays and fewer readmissions. However, these measures may not fully capture care experiences for people living with HIV, especially those experiencing medical, psychosocial, and economic complexity. As part of a larger study, we conducted seven focus groups with people living with HIV (n = 52), who were current/former patients at a Toronto-based specialty hospital, examining their desires/needs for hospital programs. Using a novel place attachment lens, we conducted a thematic analysis focusing on the emotional bond between person (patient) and place (hospital). Our findings show that participants wanted an ongoing connection to hospital to fulfill their need(s) for control, security, restoration and belonging. Indeed, continual attachment to hospital may be beneficial for patients with complex care needs. Our research has implications for care engagement and retention frameworks.

Case 319.

HISTORY: A 7-year-old Chinese girl presented to a local hospital with a 5-day history of progressive right-sided hemiplegia, expressive aphasia, mild bulbar palsy, and reduced general responsiveness. At presentation, her Glasgow Coma Scale was 11/15 (E4 V1M6). Notably, she had two strokelike episodes approximately 7 and 3 months prior to the current episode, with headache, reduced movement, and numbness in the left hand. She also had an extensive medical history at a young age, including congenital mydriasis, patent ductus arteriosus with ligation, dysautonomia, low blood pressure, hypotonic bladder requiring intermittent catheterization, poor bowel transit, and gallstones. Her immunization record was up to date, and her development was otherwise unremarkable. Her parents and younger sibling were healthy. Her blood tests revealed a mildly increased venous lactate level at 2.3 mmol/L (normal range, 0.7-2.1 mmol/L), without acidosis. Results of a coagulopathy work-up (clotting profile and protein C, protein S, antithrombin 3, and fibrinogen levels) were normal. MRI (Fig 1) and MR angiography of the brain (Fig 2) were performed at presentation.

How to Report: Hip MRI.

Writing a good magnetic resonance imaging (MRI) hip report requires a sound understanding of hip anatomy, imaging, and related pathologies. The structures of the hip most prone to pathology are the articular surfaces, labrum, subchondral bone, and gluteal tendons. Particular attention should be paid to abnormal hip morphology that is relatively common and can manifest as internal or external hip derangement. It is essential to appreciate and report the specific features of each pathology that carry clinical significance to aid patient management. This article is aimed at trainee radiologists and those less experienced with reporting hip MRI, focusing on the essential features to comment on and providing examples of terminology to use and MR images to illustrate these features.

Feasibility, acceptability, concerns, and challenges of implementing supervised injection services at a specialty HIV hospital in Toronto, Canada: perspectives of people living with HIV.

BACKGROUND: Substance use significantly impacts health and healthcare of people living with HIV/AIDS (PLHIV), especially their ability to remain in hospital following admission. Supervised injection services (SIS) reduce overdoses and drug-related harms, but are not often provided within hospitals/outpatient programs. Leading us to question, what are PLHIV's perceptions of hospital-based SIS? METHODS: This mixed-methods study explored feasibility and acceptability of implementing SIS at Casey House, a Toronto-based specialty HIV hospital, from the perspective of its in/outpatient clients. We conducted a survey, examining clients' (n = 92) demand for, and acceptability of, hospital-based SIS. Following this, we hosted two focus groups (n = 14) and one-on-one interviews (n = 8) with clients which explored benefits/drawbacks of in-hospital SIS, wherein participants experienced guided tours of a demonstration SIS space and/or presentations of evidence about impacts of SIS. Data were analysed using descriptive statistics and thematic analysis. RESULTS: Among survey participants, 76.1% (n = 70) identified as cis-male and over half (n = 49;54.4%) had been a hospital client for 2 years or less. Nearly half (48.8%) knew about clients injecting in/near Casey House, while 23.6% witnessed it. Survey participants were more supportive of SIS for inpatients (76.1%) than for outpatients (68.5%); most (74.7%) reported SIS implementation would not impact their level of service use at Casey House, while some predicted coming more often (16.1%) and others less often (9.2%). Most focus group/interview participants, believed SIS would enhance safety by reducing health harms (e.g. overdose), increasing transparency between clients and clinicians about substance use, and helping retain clients in care. Debate arose about who (e.g., in/outpatients vs. non-clients) should have access to hospital-based SIS and how implementation may shift organizational priorities/resources away from services not specific to drug use. CONCLUSIONS: Our data showed widespread support of, and need for, hospital-based SIS among client stakeholders; however, attempts to reduce negative impacts on non-drug using clients need to be considered in the balance of implementation plans. Given the increased risks of morbidity and mortality for PLHIV who inject drugs as well as the problems in retaining them in care in a hospital setting, SIS is a key component of improving care for this marginalized group.

Possibilities and priorities for IJED in times of uncertainty: A 40th anniversary analysis.

This article reflects upon the history of the Journal, its evolving nature and rationale and upon possibilities and priorities for the future in what are uncertain times for all.

Challenges in glucoCEST MR body imaging at 3 Tesla.

BACKGROUND: The aim of this study was to translate dynamic glucose enhancement (DGE) body magnetic resonance imaging (MRI) based on the glucose chemical exchange saturation transfer (glucoCEST) signal to a 3 T clinical field strength. METHODS: An infusion protocol for intravenous (i.v.) glucose was optimised using a hyperglycaemic clamp to maximise the chances of detecting exchange-sensitive MRI signal. Numerical simulations were performed to define the optimum parameters for glucoCEST measurements with consideration to physiological conditions. DGE images were acquired for patients with lymphomas and prostate cancer injected i.v. with 20% glucose. RESULTS: The optimised hyperglycaemic clamp infusion based on the DeFronzo method demonstrated higher efficiency and stability of glucose delivery as compared to manual determination of glucose infusion rates. DGE signal sensitivity was found to be dependent on T2, B1 saturation power and integration range. Our results show that motion correction and B0 field inhomogeneity correction are crucial to avoid mistaking signal changes for a glucose response while field drift is a substantial contributor. However, after B0 field drift correction, no significant glucoCEST signal enhancement was observed in tumour regions of all patients in vivo. CONCLUSIONS: Based on our simulated and experimental results, we conclude that glucose-related signal remains elusive at 3 T in body regions, where physiological movements and strong effects of B1 + and B0 render the originally small glucoCEST signal difficult to detect.

Pars interarticularis injury in elite athletes - The role of imaging in diagnosis and management.

Injuries of the lumbar neural arch, in particular the pars interarticularis, are widely considered to be due to abnormal bone stress secondary to repetitive loading/shearing, and are a common pathology and a main cause of lower back pain in elite athletes across a range of sports. Medical imaging plays a pivotal role in the diagnosis, monitoring and prognostication of neural arch injury. Early detection is highly desirable in the young elite athlete, as early injuries have been shown to require shorter recovery time and have a higher potential of full healing, whilst accurate grading of injury allows appropriate rehabilitation planning. Various imaging modalities are used in the diagnosis and management of pars stress injury, each with their strengths and weaknesses. There is currently a lack of general consensus over the most appropriate imaging pathway for neural arch injury in this specific group of patients. In this review article, we present an overview of neural arch injury, the various imaging modalities used and their imaging appearances with a brief pictorial review, and a proposed imaging algorithm with special considerations in the young elite athletes.

Abdominopelvic splenosis--an unusual cause of tenesmus.

Splenosis is a rare condition defined as seeding and autotransplantation of splenic tissue, typically after blunt abdominal trauma (e.g. from road traffic collision). Sites of splenosis ranging from intrathoracic to intrapelvic have been reported, and symptoms vary greatly depending on the site and size of lesions. We present the use of Tc-99m sulphur colloid SPECT/CT in diagnosing a case of multiple abdominopelvic splenosis as the cause of new-onset tenesmus and constipation, which was initially thought to be due to colorectal malignancy, 47 years following the initial abdominal trauma.

Community engagement in health status instrument development: experience with the HIV disability questionnaire.

BACKGROUND: People with HIV face a variety of health-related challenges from HIV, concurrent health conditions, and side effects of treatment. These health-related challenges may be termed disability. Community involvement in conceptualizing and understanding disability is essential to developing appropriate measurement tools. OBJECTIVE: To describe a community-academic partnership in the development of a new self-administered health status instrument, the HIV Disability Questionnaire (HDQ), using a community-engaged approach. METHODS: With a Community Advisory Committee, we used categories from the Episodic Disability Framework, a conceptual framework developed in an earlier stage of research, as a foundation for item generation. RESULTS: The purpose of the 70-item HDQ is to characterize the presence, severity, and episodic nature of disability experienced by adults with HIV. The Community Advisory Committee suggested items beyond those in the Episodic Disability Framework, refined item phrasing, order, response options, and questionnaire instructions to enhance feasibility and relevance of the HDQ to adults with HIV, advised on how to structure the questionnaire to capture the episodic nature of disability, and suggested next steps for testing and implementation. CONCLUSIONS: The HDQ is the first HIV-specific questionnaire to describe disability experienced by adults with HIV. Engaging community research partners in the development of the HDQ enhanced the questionnaire's relevance, feasibility, and face and content validities. This approach may be considered by others interested in health status instrument development.

Dynamic contrast-enhanced MRI improves accuracy for detecting focal splenic involvement in children and adolescents with Hodgkin disease.

BACKGROUND: Accurate assessment of splenic disease is important for staging Hodgkin lymphoma. OBJECTIVE: The purpose of this study was to assess T2-weighted imaging with and without dynamic contrast-enhanced (DCE) MRI for evaluation of splenic Hodgkin disease. MATERIALS AND METHODS: Thirty-one children with Hodgkin lymphoma underwent whole-body T2-weighted MRI with supplementary DCE splenic imaging, and whole-body PET-CT before and following chemotherapy. Two experienced nuclear medicine physicians derived a PET-CT reference standard for splenic disease, augmented by follow-up imaging. Unaware of the PET-CT, two experienced radiologists independently evaluated MRI exercising a locked sequential read paradigm (T2-weighted then DCE review) and recorded the presence/absence of splenic disease at each stage. Performance of each radiologist was determined prior to and following review of DCE-MRI. Incorrect MRI findings were ascribed to reader (lesion present on MRI but missed by reader) or technical (lesion not present on MRI) error. RESULTS: Seven children had splenic disease. Sensitivity/specificity of both radiologists for the detection of splenic involvement using T2-weighted images alone was 57%/100% and increased to 100%/100% with DCE-MRI. There were three instances of technical error on T2-weighted imaging; all lesions were visible on DCE-MRI. CONCLUSIONS: T2-weighted imaging when complemented by DCE-MRI imaging may improve evaluation of Hodgkin disease splenic involvement.

Sensibility assessment of the HIV Disability Questionnaire.

PURPOSE: Our purpose was to assess the sensibility of the human immunodeficiency virus (HIV) Disability Questionnaire (HDQ), the first HIV-specific disability questionnaire. METHODS: We administered the HDQ, a sensibility questionnaire and a structured qualitative interview to 22 adults living with HIV and five experienced clinicians. We considered the HDQ sensible if median scores on the sensibility questionnaire were ≥5 for adults living with HIV and ≥4 for clinicians for at least 80% of the items. We analyzed the interview data using directed qualitative content analytical techniques. RESULTS: Questionnaire scores were ≥5 for 88% (15/17) of the items and ≥4 for 100% (17/17) of the items for adults living with HIV and clinicians, respectively. The interview analysis indicated participants felt the HDQ possessed face and content validity in all disability dimensions, had adequate response options, was easy to complete, and adequately captured the episodic nature of disability. Participants had mixed responses about the questionnaire title and provided recommendations to refine item wording and response options. CONCLUSIONS: The HDQ appears sensible for use with adults living with HIV. Next steps include further measurement property assessment. The HDQ may be used by rehabilitation clinicians and researchers to assess disability experienced by adults living with HIV. IMPLICATIONS FOR REHABILITATION: • As people with HIV infection live longer, individuals may face a range of health-related challenges due to the disease, concurrent health conditions and the potential adverse effects of treatment. Together, these health-related challenges may be termed disability. • The HIV Disability Questionnaire (HDQ) is the first HIV-specific instrument developed to describe the presence, severity and episodic nature of the disability experienced by adults living with HIV. The HDQ is comprised of four domains including symptoms and impairments, uncertainty about future health, difficulties carrying out day-to-day activities, and challenges to social inclusion. • The HDQ appears sensible for use with adults living with HIV, possessing face and content validity and ease of use in all four domains as well as describing the daily episodic nature of disability. • The HDQ may be used by rehabilitation clinicians and researchers to assess disability experienced by adults living with HIV.

Bone marrow mononuclear cells reduce myocardial reperfusion injury by activating the PI3K/Akt survival pathway.

OBJECTIVE: Adult bone marrow mononuclear cells (BMMNCs) can restore cardiac function following myocardial necrosis. Protocols used to date have administered cells relatively late after ischaemia/reperfusion injury, but there is the opportunity with elective procedures to infuse cells shortly after restoration of blood flow, for example after angioplasty. Our aim was therefore to try and quantify protection from myocardial injury by early infusion of BMMNCs in a rat ischaemia reperfusion (I/R) model. METHODS AND RESULTS: Male Wistar rats underwent 25 min of ischaemia followed by 2 h reperfusion of the left anterior descending coronary artery. Ten million BMMNCs were injected i.v. at reperfusion. We found BMMNCs caused a significant reduction in infarct size at 2 h when assessed by staining the area at risk with p-nitro blue tetrazolium (42% reduction, P<0.01). Apoptosis and necrosis of isolated cardiomyocytes was significantly reduced in the area at risk. Functional assessment at 7 days using echocardiography and left ventricular catheterisation showed improved systolic and diastolic function in the BMMNC treatment group (LVEF: BMMNC 71 ± 3% vs. PBS 48 ± 4%, P<0.0001). In functional studies BMMNC injected animals showed increased activation of Akt, inhibition of GSK-3ß, amelioration of p38 MAP kinase phosphorylation and NF-κB activity compared to control myocardium. Inhibition of PI3K with LY294002 abolished all beneficial effects of BMMNC treatment. Proteomic analysis also demonstrated that BMMNC treatment induced alterations in proteins within known cardioprotective pathways, e.g., heat shock proteins, stress-70 protein as well as the chaperone protein 14-3-3 epsilon. CONCLUSIONS: Early BMMNC injection during reperfusion preserves the myocardium, with evidence of reduced apoptosis, necrosis, and activation of survival pathways.

How do existing HIV-specific instruments measure up? Evaluating the ability of instruments to describe disability experienced by adults living with HIV.

BACKGROUND: Despite the multitude of health challenges faced by adults living with HIV, we know of no HIV-specific instrument developed for the purpose of describing the health-related consequences of HIV, a concept known as disability. In a previous phase of research, adults living with HIV conceptualized disability as symptoms/impairments, difficulties carrying out day-to-day activities, challenges to social inclusion, and uncertainty that may fluctuate on a daily basis and over the course of living with HIV. In this paper, we describe the extent to which existing HIV-specific health-status instruments capture the experience of disability for adults living with HIV. METHODS: We searched databases from 1980 to 2006 for English language, HIV-specific, self-reported questionnaires consisting of at least two items that were tested for reliability and validity. We then conducted a content analysis to assess how well existing questionnaires describe disability as defined by the Episodic Disability Framework, a framework that conceptualizes this experience from the perspective of adults living with HIV. We matched items of the instruments with categories of the framework to evaluate the extent to which the instruments capture major dimensions of disability in the framework. RESULTS: We reviewed 4274 abstracts, of which 30 instruments met the inclusion criteria and were retrieved. Of the four major dimensions of disability, symptoms/impairments were included in all 30 instruments, difficulties with day-to-day activities in 16, challenges to social inclusion in 16, and uncertainty in 9. Seven instruments contained at least 1 item from all 4 dimensions of disability (breadth) however, the comprehensiveness with which the dimensions were represented (depth) varied among the instruments. CONCLUSIONS: In general, symptoms/impairments and difficulties carrying out day-to-day activities were the disability dimensions characterized in greatest depth while uncertainty and challenges to social inclusion were less well represented. Although none of the instruments described the full breadth and depth of disability as conceptualized by the Episodic Disability Framework, they provide a foundation from which to build a measure of disability for adults living with HIV.

Quick and easy: Indirect fabrication of composite veneers.

Composite veneers can be fabricated directly on a vinyl polysiloxane (VPS) die. Using the following technique, a dentist (or their trained auxiliary) can quickly fabricate an esthetic composite veneer with an acceptable marginal fit. This article describes such a technique.

Oral health outcomes in an adult dental population: the impact of payment systems.

This study compared the effect of payment systems on recall visits and oral health outcomes for four patient payer groups. The authors reviewed recall audit data obtained over a 4-year period. Dental payer groups in the study population differed with respect to dental outcomes. Patients who had recall visits at 1 year or longer had worse outcomes than patients seen more frequently. A Medicaid subgroup with the greatest number of risk factors displayed improved oral health outcomes when seen on a more frequent recall schedule. Study findings suggest that, given the increased risks to maintaining oral health, patients being supported by public-funded programs would benefit from more frequent recalls. Such an emphasis would appear to provide the opportunity to improve outcomes in disadvantaged populations and increase the quality of care offered.

Variations of L*a*b* values among Vitapan Classical Shade Guides.

PURPOSE: To measure the variations in L*a*b* values of a group of 25 guides and to assess whether shade guides are indeed interchangeable. MATERIALS AND METHODS: The L*a*b* values of individual shade tabs were measured with a LabScan(tm) XE scanning spectrocolorimeter (SSC) with a special attachment for reproducibly positioning shade tabs. Each shade guide (Vitapan Classical, Vident) contained 16 shade tabs. Absolute calibration of the SSC was performed with color tiles traceable to NIST. One shade guide was used to determine the reproducibility of the experimental method by measuring and then removing each shade tab of the standard 10 times. This assessed the variations in observed values induced by the measurement method and the geometry of the specimens. The entire sample consisted of 25 shade guides. The tabs of each of the 25 shade guides were read five times in the SSC, without moving the specimen. Measurements and calculations of E, L*, a*, and b* were performed using Universal Software V4.10 (Hunter Associates Laboratory). The mean, standard deviation, and range were determined of the E, L*, a*, and b* values for each one of the 16 shades in the shade guides. Differences in color are expressed as DeltaE in color science. The standard deviation of E (E(sd)) and the range of E (E(r)) for each of the shades were used as a DeltaE value to assess color differences. RESULTS: For the reproducibility measurements, the shades had values for E(r) varying from 0.08 to 0.69, and E(sd) between 0.02 and 0.22. This established the detection limit for our method for each of the shades. For the group of 25 shade guides, E(r) varied from 0.75 to 3.05, and E(sd) from 0.22 to 0.54. The difference in value of E(r) obtained from the reproducibility test and the E(r) of the group of 25 guides is significant at p < 0.05. The same was found for E(sd). The largest reproducible E(r) observed was 3.05 for shade C1. CONCLUSIONS: The differences observed between shade guides are larger than the variations induced by the experimental method. The difference in E values for the shades C1 and C2 is 2.19; hence the observed E(r) of 3.05 between shade tabs of the same shade (C1) is larger than the changes in color between shades. This large a variation in what are claimed as identical shade tabs is deemed of clinical importance, and therefore, the shade guides should not be considered interchangeable.

Retrofitting technique for a crown or abutment following coronal fracture.

The fracture of tooth structure and/or restorative material within a crown or fixed partial denture abutment poses a restorative challenge. Depending upon the severity of the fracture and health of the tooth or teeth involved, the restoration often can be re-cemented, restoring the tooth to a serviceable condition. This article describes a technique that provides a safe and efficient means of restoring the fractured tooth, thereby overcoming a major obstacle to the conventional recementation process. An application of the technique also is described.