Disease-specific electrophysiological findings in adult ceroid-lipofuscinosis (Kufs disease)

Two adults with mild dementia and a history of memory loss and disequilibrium were seen in the eye clinic following complaints of acuity loss in the 20/30–20/70 (Snellen) range. Results from the fundus examination of one patient were entirely normal; the other showed minimal vascular attenuation and optic atrophy. Electrophysiology was remarkable: (1) Photopic ERG b-waves were reduced, delayed, and showed pronounced oscillations. (2) EOG light-rise potentials were absent or very small. (3) Binocular pattern-VER signals showed addition of the monocular signal. Scotopic ERG signals were normal. Brain biopsy and microscopy showed intercellular, autofluorescent ceroid deposits which provided a clear diagnosis of Kufs disease. Histology of model animal retinal cells show ceroid deposits in cell classes implicated by the human retinal signals. The cluster of electrophysiological results point toward early changes in the pigment epithelium and inner plexiform layer cells as a means of non-invasive diagnosis.     

The effects of diazepam on "fear" reactions in rats are modulated by environmental constraints on the rat's defensive repertoire

The simultaneous effects of diazepam on three shock-induced reactions in rats were studied in order to determine the reliability of these behaviors as indices of anxiolytic drug action. Rats were injected with 1 mg/kg of diazepam or vehicle, placed in a 2-compartment chamber containing bedding material, and shocked with 1, 2, or 6 mA when they first touched a wire-wrapped prod attached to one end of the chamber. Diazepam-treated animals displayed significantly less burying behavior, but paradoxically, they also displayed more passive avoidance behavior and fewer exploratory side-transitions than vehicle-injected controls. Defensive burying behavior tended to be negatively correlated with passive avoidance behavior and positively correlated with exploratory side transitions. When the "competitive" relationship between defensive burying and passive avoidance was eliminated by testing rats in a 2-compartment chamber not containing bedding material, diazepam produced a significant suppression of passive avoidance and a significant increase in exploratory side-transitions, compared to control. Taken together, these results suggested that the validity of any single behavioral model of anxiolytic drug action might vary as a function of environmental constraints on the subjects' defensive repertoire.     

Failure of the rabbit tibial growth plate to respond to the long-term application of a capacitively-coupled electrical field

A continuous 5-V peak-to-peak, 60 kHz capacitively-coupled sine wave signal was applied to the proximal tibial growth plate in fifteen 9-week-old male New Zealand white rabbits for 6 weeks. A pair of flexible stainless steel "injectrodes" was held in place medially and laterally on the surface of the proximal hindlimb in each rabbit by means of tape wrappings. The electrodes were connected to a 9-V battery-operated power unit carried in a dorsal pouch in a body vest worn by each rabbit. Control animals wore the identical apparatus, only the power unit was inactive. Small Tantalum markers were inserted into the anteromedial aspect of the proximal tibial metaphysis 1 cm distal to the proximal tibial growth plate in all of the animals, control and experimental, 2 weeks prior to the onset of electrical stimulation. The distance between the proximal lateral tibial spine and the Tantalum marker, between the Tantalum marker and the apex of the distal tibial intercondylar notch, and between the proximal tibial spine and the distal notch was measured from roentgenograms made at the time of bone marker insertion, at the time of electrode application to the limb, and at the end of the stimulation period. Results indicate that there was no significant difference in tibial lengths between the stimulated and control groups. There was significantly less total body weight gain in both the experimental and control animals than that which occurred in paired normal animals during the same period of time. This failure to thrive may be responsible for the resultant lack of longitudinal growth stimulation of the capacitive coupling.(ABSTRACT TRUNCATED AT 250 WORDS)     

Health-related quality of life,uncertainty and coping strategies in solid organ transplant recipients during shielding for the COVID-19 pandemic

Strict isolation of vulnerable individuals has been a strategy implemented by authorities to protect people from COVID-19. Our objective was to investigate health-related quality of life (HRQoL), uncertainty and coping behaviours in solid organ transplant (SOT) recipients during the COVID-19 pandemic. A cross-sectional survey of adult SOT recipients undergoing follow-up at our institution was performed. Perceived health status, uncertainty and coping strategies were assessed using the EQ-5D-5L, Short-form Mishel Uncertainty in Illness Scale (SF-MUIS) and Brief Cope, respectively. Interactions with COVID-19 risk perception, access to health care, demographic and clinical variables were assessed. The survey was completed by 826 of 3839 (21.5%) invited participants. Overall, low levels of uncertainty in illness were reported, and acceptance was the major coping strategy (92%). Coping by acceptance, feeling protected, self-perceived susceptibility to COVID-19 were associated with lower levels of uncertainty. Health status index scores were significantly lower for those with mental health illness, compromised access to health care, a perceived high risk of severe COVID-19 infection and higher levels of uncertainty. A history of mental health illness, risk perceptions, restricted healthcare access, uncertainty and coping strategies was associated with poorer HRQoL in SOT recipients during strict isolation. These findings may allow identification of strategies to improve HRQoL in SOT recipients during the pandemic.     

Remote intervention engagement and outcomes in the Clinical Trials in Organ Transplantation in Children consortium multisite trial

Remote interventions are increasingly used in transplant medicine but have rarely been rigorously evaluated. We investigated a remote intervention targeting immunosuppressant management in pediatric lung transplant recipients. Patients were recruited from a larger multisite trial if they had a Medication Level Variability Index (MLVI) ≥2.0, indicating worrisome tacrolimus level fluctuation. The manualized intervention included three weekly phone calls and regular follow-up calls. A comparison group included patients who met enrollment criteria after the subprotocol ended. Outcomes were defined before the intent-to-treat analysis. Feasibility was defined as ≥50% of participants completing the weekly calls. MLVI was compared pre- and 180 days postenrollment and between intervention and comparison groups. Of 18 eligible patients, 15 enrolled. Seven additional patients served as the comparison. Seventy-five percent of participants completed ≥3 weekly calls; average time on protocol was 257.7 days. Average intervention group MLVI was significantly lower (indicating improved blood level stability) at 180 days postenrollment (2.9 ± 1.29) compared with pre-enrollment (4.6 ± 2.10), p = .02. At 180 days, MLVI decreased by 1.6 points in the intervention group but increased by 0.6 in the comparison group (p = .054). Participants successfully engaged in a long-term remote intervention, and their medication blood levels stabilized. NCT02266888.     

Dental Care Utilization of Hospitalized Persons Living with HIV and Substance Use

Journal of Community Health - People living with HIV (PLWH) who use drugs experience worse health outcomes than their non-using counterparts. Little is known about how often they seek dental care...     

Wound microbiology and associated approaches to wound management

The majority of dermal wounds are colonized with aerobic and anaerobic microorganisms that originate predominantly from mucosal surfaces such as those of the oral cavity and gut. The role and significance of microorganisms in wound healing has been debated for many years. While some experts consider the microbial density to be critical in predicting wound healing and infection, others consider the types of microorganisms to be of greater importance. However, these and other factors such as microbial synergy, the host immune response, and the quality of tissue must be considered collectively in assessing the probability of infection. Debate also exists regarding the value of wound sampling, the types of wounds that should be sampled, and the sampling technique required to generate the most meaningful data. In the laboratory, consideration must be given to the relevance of culturing polymicrobial specimens, the value in identifying one or more microorganisms, and the microorganisms that should be assayed for antibiotic susceptibility. Although appropriate systemic antibiotics are essential for the treatment of deteriorating, clinically infected wounds, debate exists regarding the relevance and use of antibiotics (systemic or topical) and antiseptics (topical) in the treatment of nonhealing wounds that have no clinical signs of infection. In providing a detailed analysis of wound microbiology, together with current opinion and controversies regarding wound assessment and treatment, this review has attempted to capture and address microbiological aspects that are critical to the successful management of microorganisms in wounds.     

Differential expression of FMR1, FXR1 and FXR2 proteins in human brain and testis

Lack of expression of the fragile X mental retardation protein (FMRP) results in mental retardation and macroorchidism, seen as the major pathological symptoms in fragile X patients. FMRP is a cytoplasmic RNA- binding protein which cosediments with the 60S ribosomal subunit. Recently, two proteins homologous to FMRP were discovered: FXR1 and FXR2. These novel proteins interact with FMRP and with each other and they are also associated with the 60S ribosomal subunit. Here, we studied the expression pattern of the three proteins in brain and testis by immunohistochemistry. In adult brain, FMR1, FXR1 and FXR2 proteins are coexpressed in the cytoplasm of specific differentiated neurons only. However, we observed a different expression pattern in fetal brain as well as in adult and fetal testis, suggesting independent functions for the three proteins in those tissues during embryonic development and adult life.      

Cervical spine injuries in children

Cardiorespiratory arrest occurring immediately after multiple injuries is usually assumed to be due to severe cerebral injury, acute hemorrhage, or airway obstruction. We have identified a group of 19 children (mean age, 6.3 years) who presented with absent vital signs (VSA) or severe hypotension, unexplained by blood loss, where these findings were caused by injury to the high cervical spine and cord, demonstrated either by X-ray or postmortem examination. Fourteen had radiologic evidence of injury to the spine between C1 and C3. In two patients the bony injury was at the C6-7 level, while in two patients the cervical spine X-ray was normal. Eighteen of the 19 children were initially resuscitated but died from a combination of hypoxic/ischemic encephalopathy and cerebral injury. Sixteen patients underwent postmortem examination and in 13 there was evidence of cord laceration, up to and including cord transection. These findings demonstrate a distinct pattern of "juvenile" cervical spine injury involving the high spine and cord which results in either apnea and cardiorespiratory arrest, or severe hypotension. This previously unrecognized cause of cardiorespiratory arrest should be considered in all children presenting with VSA after multiple trauma, even when there is no apparent radiologic abnormality of the cervical spine.