Advanced magnetic resonance imaging in benign hereditary chorea: Study of two familial cases

No brain abnormalities are usually detected on conventional magnetic resonance imaging (MRI) in benign hereditary chorea (BHC); there are currently no studies with advanced techniques in literature. We investigated whether conventional and advanced MRI techniques could depict regional brain abnormalities in two familial BHC patients and 24 healthy controls. No brain abnormalities on conventional scans were detectable; also, no significant differences in fractional anisotropy of the basal nuclei were observed. Volumetric analysis showed a decreased volume of the striatum bilaterally compared with controls, whereas spectroscopy demonstrated a significant increased myoinositol/creatine ratio bilaterally, a reduction of choline/creatine ratio bilaterally, and of N‐acetyl‐aspartate/creatine in the right putamen. With the limits of the small sample size in the patient group, these data show that, despite the absence of macroscopic changes on conventional MRI, volumetric and metabolic abnormalities are present in the basal nuclei of BHC patients. © 2010 Movement Disorder Society.     

Pathogenesis,immunology, and immune-targeted management of the multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multisystem syndrome (PIMS): EAACI Position Paper

Multisystem inflammatory syndrome in children (MIS-C) is a rare, but severe complication of coronavirus disease 2019 (COVID-19). It develops approximately 4 weeks after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and involves hyperinflammation with multisystem injury, commonly progressing to shock. The exact pathomechanism of MIS-C is not known, but immunological dysregulation leading to cytokine storm plays a central role. In response to the emergence of MIS-C, the European Academy of Allergy and Clinical Immunology (EAACI) established a task force (TF) within the Immunology Section in May 2021. With the use of an online Delphi process, TF formulated clinical statements regarding immunological background of MIS-C, diagnosis, treatment, follow-up, and the role of COVID-19 vaccinations. MIS-C case definition is broad, and diagnosis is made based on clinical presentation. The immunological mechanism leading to MIS-C is unclear and depends on activating multiple pathways leading to hyperinflammation. Current management of MIS-C relies on supportive care in combination with immunosuppressive and/or immunomodulatory agents. The most frequently used agents are systemic steroids and intravenous immunoglobulin. Despite good overall short-term outcome, MIS-C patients should be followed-up at regular intervals after discharge, focusing on cardiac disease, organ damage, and inflammatory activity. COVID-19 vaccination is a safe and effective measure to prevent MIS-C. In anticipation of further research, we propose a convenient and clinically practical algorithm for managing MIS-C developed by the Immunology Section of the EAACI.     

Impact of patient characteristic factors on the dynamics of liver glucose metabolism: Evaluation of multiparametric imaging with dynamic whole-body 18F-fluorodeoxyglucose-positron emission tomography

Aims

To assess the impact of various patient characteristics on the dynamics of liver glucose metabolism using automated multiparametric imaging with whole-body dynamic ¹⁸F-fluorodeoxyglucose (FDG)-positron emission tomography (PET).

Materials and Methods

We retrospectively enrolled 540 patients who underwent whole-body dynamic FDG-PET. Three quantitative indices representing hepatic glucose metabolism [mean standardized uptake value normalized by lean body mass (SULmean), metabolic glucose rate (kinetic index) and distribution volume (DV)] were measured from multiparametric PET images produced automatically based on the Patlak plot model. Patient characteristics including age, sex, body mass index, fasting time, blood glucose level, and the presence of diabetes mellitus (DM) or hepatic steatosis (HS) were collected. We examined the correlations between the characteristic factors and three quantitative indices using multiple regression analysis.

Results

The success rate of kinetic analysis using multiparametric PET imaging was 93.3% (504/540). Hepatic SULmean was significantly correlated with age (p < .001), sex (p < .001) and blood glucose level (p = .002). DV was significantly correlated with age (p = .033), sex (p < .001), body mass index (p = .002), fasting time (p = .043) and the presence of HS (p = .002). The kinetic index was significantly correlated with age (p < .001) and sex (p = .004). In the comparison of the healthy, DM and HS groups, patients with DM had a significantly increased SULmean, whereas patients with HS had a significantly decreased DV.

Conclusions

Our results showed that liver glucose metabolism was influenced by various patient characteristic factors. Multiparametric FDG-PET imaging can be used to analyse the kinetics of liver glucose metabolism in routine clinical practice.     

Doppler velocimetry determined redistribution of fetal blood flow: Correlation with growth restriction in diamniotic monochorionic and dizygotic twins

OBJECTIVE: Our purpose was to study fetal growth and blood flow distribution in diamniotic monochorionic compared with dizygotic (diamniotic dichorionic) twins by use of Doppler velocimetry of the umbilical artery and middle cerebral artery. STUDY DESIGN: Study candidates were divided into group A, consisting of 33 pairs (66 fetuses) of diamniotic monochorionic twins, and group B, 50 pairs (100 fetuses) of diamniotic dichorionic twins. Diamniotic monochorionic placentation was confirmed by microscopic placental examination for group A. Diamniotic dichorionic placentation was ensured for group B by selecting only twins with different-sex pairs (dizygotic twins). Targeted ultrasonography with biometry was performed in each twin, and Doppler recordings of the umbilical artery and middle cerebral artery were obtained. Waveforms were analyzed and the systolic/diastolic ratio, the resistance index, and a measure of blood flow redistribution (brain-sparing effect), the cerebral/placental ratio, was calculated for each fetus. Growth status at birth was assessed by the number of small-for-gestational-age infants (≤10th percentile), low-birth-weight infants (≤25th percentile), and percent of growth discordance between twins. Intertwin differences were assessed by Δ values (value of larger twin minus value of smaller twin). RESULTS: Diamniotic monochorionic compared with dizygotic twins demonstrated a significantly greater probability of blood flow redistribution. For the study population as a whole, the brain-sparing effect was noted in 67% of small-for-gestational-age babies and only 7% of non-small-for-gestational-age infants (p ≤ 0.001). For the diamniotic monochorionic pregnancies blood flow redistribution occurred in 6 of 10 small-for-gestational-age infants (60%) and 6 of 46 non-small-for-gestational-age infants (13%). In the diamniotic monochorionic group small-for-gestational-age compared with non-small-for-gestational-age infants were more likely to show blood flow redistribution, which was the result of significantly decreased resistance in the middle cerebral artery and significantly increased resistance in the umbilical artery. Small-for-gestational-age infants (≤10th percentile) occurred much less frequently in the dizygotic group. Two of two small-for-gestational-age infants in the dizygotic group showed blood flow redistribution. Although the extremes of birth weight were more common in the diamniotic monochorionic group, both groups had relatively large numbers of small babies with birth weights in the lower 25th percentile (50.0% for diamniotic monochorionic and 44.0% for dizygotic twins, not significant). However, 42.3% (11/26) of diamniotic monochorionic twins who were in the low-birth-weight group showed blood flow redistribution compared with only 3.3% (1/30) whose birth weights were ≥25th percentile (p ≤ 0.001). In the dizygotic twins 10% of lower-birth-weight infants redistributed blood flow compared with 1% in the higher-birth-weight group, a nonsignificant difference. Diamniotic monochorionic compared with dizygotic twins were delivered earlier (32.9 weeks vs 34.8 weeks, p ≤ 0.001), were smaller (1832 gm vs 2304 gm, p ≤ 0.001), showed higher birth weight discordance (29.8% vs 14%, p ≤ 0.05), and had greater numbers (19.7% vs 2.3%, p ≤ 0.01) of infants at ≤10th percentile birth weight. CONCLUSIONS: Diamniotic monochorionic twins from the lower-birth-weight groups more often show blood flow redistribution compared with dizygotic twins of similar low birth weights. Placental vascular connections and the attendant hemodynamic changes in the fetuses of diamniotic monochorionic twins probably account for this difference. Brain-sparing events occur commonly without clinical twin transfusion syndrome in this group. These findings have implications for management. (Am J Obstet Gynecol 1998;178:1359-67.)     

An improved method for estimating the heart-to-mediastinum ratio from cardiac sympathetic nerve imaging with low-energy high-resolution collimators

Background

Septal penetration causes underestimation of the heart-to-mediastinum (H/M) ratio in cardiac ¹²³I-metaiodobenzylguanidine (MIBG) imaging with a low-energy high-resolution (LEHR) collimator. We aimed to improve the method of estimating the H/M ratios using the LEHR collimator.

Methods and Results

4 hours after ¹²³I-MIBG injection, 40 patients were imaged successively with the medium-energy (ME) and LEHR collimators using gamma cameras having 3/8-inch crystals. Severe underestimation of the H/M ratios was observed with the LEHR collimator when compared to the ME collimator. Narrowing the energy window width did not reduce the underestimation. Application of ¹²³I-dual-window (IDW) correction using a narrow or wide subwindow reduced the underestimation substantially but not entirely. The H/M ratios estimated from the LEHR images with or without IDW correction were corrected based on their correlations with the ratios estimated from the ME images. This empiric correction removed systematic underestimation, and residual errors were reduced when the H/M ratios after IDW correction were converted using the empiric equation. The conversion equation was successfully applied to the correction of the H/M ratios determined in another 40 patients using a 5/8-inch crystal.

Conclusions

In estimating the H/M ratios using an LEHR collimator, empiric correction combined with IDW correction improves concordance with ME-based values in comparison with empiric correction alone.     

Impact of intensive care unit admission during morning bedside rounds and mortality: a multi-center retrospective cohort study

ABSTRACT: INTRODUCTION: Recent data have suggested that patient admission during intensive care unit (ICU) morning bedside rounds is associated with less favorable outcome. We undertook the present study to explore the association between morning round-time ICU admissions and hospital mortality in a large Canadian health region. METHODS: A multi-center retrospective cohort study was performed at five hospitals in Edmonton, Canada, between July 2002 and December 2009. Round-time ICU admission was defined as occurring between 8 and 11:59 a.m. Multivariable logistic regression analysis was used to explore the association between round-time admission and outcome. RESULTS: Of 18,857 unique ICU admissions, 2,055 (10.9%) occurred during round time. Round-time admissions were more frequent in community hospitals compared with tertiary hospitals (12.0% vs. 10.5%; odds ratio [OR] 1.16; 95% CI, 1.05-1.29, P < 0.004) and from the ward compared with the emergency department (ED) or operating theater (17.5% vs. 9.2%; OR 2.1; 95% CI, 1.9-2.3, P < 0.0001). Round-time admissions were more often medical than surgical (12.6% vs. 6.6%; OR 2.06; 95% CI, 1.83-2.31, P < 0.0001), had more comorbid illness (11.9% vs. 10.5%; OR 1.15; 95% CI, 1.04-1.27, P < 0.008) and higher APACHE II score (22.2 vs. 21.3, P < 0.001), and were more likely to have a primary diagnosis of respiratory failure (37.0% vs. 31.3%, P < 0.001) or sepsis (11.1% vs. 9.0%, P = 0.002). Crude ICU mortality (15.3% vs. 11.6%; OR 1.38; 95% CI, 1.21-1.57, P < 0.0001) and hospital mortality (23.9% vs. 20.6%; OR 1.21; 95% CI, 1.09-1.35, P < 0.001) were higher for round-time compared with non-round-time admissions. In multi-variable analysis, round-time admission was associated with increased ICU mortality (OR 1.19, 95% CI, 1.03-1.38, P = 0.017) but was not significantly associated with hospital mortality (OR 1.02; 95% CI, 0.90-1.16, P = 0.700). In the subgroup admitted from the ED, round-time admission showed significantly higher ICU mortality (OR 1.54; 95% CI, 1.21-1.95; P < 0.001) and a trend for higher hospital mortality (OR 1.22; 95% CI, 0.99-1.51, P = 0.057). CONCLUSIONS: Approximately 1 in 10 patients is admitted during morning rounds. These patients are more commonly admitted from the ward and are burdened by comorbidities, are non-operative, and have higher illness severity. These patients admitted during morning rounds have higher observed ICU mortality but no difference in hospital mortality.     

Geographical information system and predictive risk maps of urinary schistosomiasis in Ogun State,Nigeria

Background  

The control of urinary schistosomiasis in Ogun State, Nigeria remains inert due to lack of reliable data on the geographical distribution of the disease and the population at risk. To help in developing a control programme, delineating areas of risk, geographical information system and remotely sensed environmental images were used to developed predictive risk maps of the probability of occurrence of the disease and quantify the risk for infection in Ogun State, Nigeria.