[Modern aspects of diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy in children]. / Sovremennye aspekty diagnostiki i lecheniya khronicheskoi vospalitel'noi demieliniziruyushchei polineiropatii u detei.

OBJECTIVE: Analysis of demographic, clinical, laboratory, electrophysiological and neuroimaging data and pathogenetic therapy of pediatric patients with chronic inflammatory demyelinating polyneuropathy (CIDP). MATERIAL AND METHODS: Patients (n=30) were observed in a separate structural unit of the Russian Children's Clinical Hospital of the Russian National Research Medical University named after. N.I. Pirogova Ministry of Health of the Russian Federation in the period from 2006 to 2023. The examination was carried out in accordance with the recommendations of the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society on the Management of CIDP (2021). All patients received immunotherapy, including intravenous immunoglobulin (IVIG) (n=1), IVIG and glucocorticosteroids (GCS) (n=17, 56.7%), IVIG+GCS+plasmapheresis (n=12, 40.0%). Alternative therapy included cyclophosphamide (n=1), cyclophosphamide followed by mycophenolate mofetil (n=1), rituximab (n=2, 6.6%), azathioprine (n=3), mycophenolate mofetil (n=2, 6.6%). RESULTS: In all patients, there was a significant difference between scores on the MRCss and INCAT functional scales before and after treatment. At the moment, 11/30 (36.6%) patients are in clinical remission and are not receiving pathogenetic therapy. The median duration of remission is 48 months (30-84). The longest remission (84 months) was observed in a patient with the onset of CIDP at the age of 1 year 7 months. CONCLUSION: Early diagnosis of CIDP is important, since the disease is potentially curable; early administration of pathogenetic therapy provides a long-term favorable prognosis.

Geophysical imaging of ophiolite structure in the United Arab Emirates.

The Oman-United Arab Emirates ophiolite has been used extensively to document the geological processes that form oceanic crust. The geometry of the ophiolite, its extension into the Gulf of Oman, and the nature of the crust that underlies it are, however, unknown. Here, we show the ophiolite forms a high velocity, high density, >15 km thick east-dipping body that during emplacement flexed down a previously rifted continental margin thereby contributing to subsidence of flanking sedimentary basins. The western limit of the ophiolite is defined onshore by the Semail thrust while the eastern limit extends several km offshore, where it is defined seismically by a ~40-45°, east-dipping, normal fault. The fault is interpreted as the southwestern margin of an incipient suture zone that separates the Arabian plate from in situ Gulf of Oman oceanic crust and mantle presently subducting northwards beneath the Eurasian plate along the Makran trench.

Evidence of micro-continent entrainment during crustal accretion.

Simple models involving the gradual outboard accretion of material along curvilinear subduction zones are often inconsistent with field-based evidence. A recent study using 3-D geodynamic modelling has shown that the entrainment of an exotic continental fragment within a simple subduction system can result in a complex phase of growth. Although kinematic models based on structural mapping and high-resolution gravity and magnetic maps indicate that the pre-Carboniferous Tasmanides in southeastern Australia may have been subjected to this process, to date there has been little corroboration from crustal scale geophysical imaging. Here, we apply Bayesian transdimensional tomography to ambient noise data recorded by the WOMBAT transportable seismic array to constrain a detailed (20 km resolution in some areas) 3-D shear velocity model of the crust beneath southeast Australia. We find that many of the velocity variations that emerge from our inversion support the recently developed geodynamic and kinematic models. In particular, the full thickness of the exotic continental block, responsible for orocline formation and the tectonic escape of the back arc region, is imaged here for the first time. Our seismic results provide the first direct evidence that exotic continental fragments may profoundly affect the development of an accretionary orogen.

Correlation between cortisol and components of the metabolic syndrome in obese children and adolescents.

BACKGROUND: In obese subjects it has been shown that cortisol (F) contributes to the reduction in insulin sensitivity, suggesting a role in the development of the metabolic syndrome (MS). AIM: The aim of this retrospective study was to evaluate the relationship between F and components of MS in 1,027 obese children and adolescents. SUBJECTS AND METHODS: Waist circumference, systolic and diastolic blood pressure (SP, DP), F, serum glucose (Glyc), cholesterol HDL, triglycerides and homeostatic model assessment (HOMA index) were evaluated in all subjects. MS was defined according to the International Diabetes Federation criteria. Accordingly, patients were subdivided into three age groups: 6-10, 10-16 and >16 years. RESULTS: In univariate regression analysis, F was correlated with Glyc, SP and HOMA in groups 1 and 2, with DP in Group 2. In multivariate regression analysis including age, sex, puberty, BMI-SDS and F as independent variables and one of the component of the MS as the dependent variable, F was a weak predictor of the variability when DP and Glyc were introduced as dependent variables in Group 2 and when SP was introduced as dependent variable both in groups 1 and 2. When patients were subdivided into subgroups according to the IDF criteria, in Group 2 patients with one or more components of the MS had higher F concentrations. CONCLUSIONS: In this cohort of obese children and adolescents, F was weakly associated with components of the MS. These findings do not support a major role for F in the development of MS.

Prevalence of Type 1 diabetes autoantibodies (GAD and IA2) in Sardinian children and adolescents with autoimmune thyroiditis.

AIMS: Type 1 diabetes and autoimmune thyroiditis are common autoimmune diseases characterized by the presence of autoantibodies against tissue-specific components. Non-thyroid-specific autoantibodies are frequent in patients with autoimmune thyroiditis. The prevalence of Type 1 diabetes autoantibodies in patients with autoimmune thyroiditis is unknown. METHODS: The prevalence of Type 1 diabetes autoantibodies (GAD and IA2) was analysed in 236 Sardinian children and adolescents with autoimmune thyroiditis. GAD and IA2 antibodies were measured at the time of the diagnosis of autoimmune thyroiditis and re-evaluated after 1 year in the children who were shown to be positive. Autoantibody prevalence was evaluated in 949 healthy age-matched controls. RESULTS: The prevalence of GAD and/or IA2 antibodies was 8% in the children and adolescents with autoimmune thyroiditis and 4.1% in control subjects (P = 0.017). When Type 1 diabetes autoantibodies were separately analysed, the difference remained significant for IA2 (3.39% in autoimmune thyroiditis vs. 1.16% in control subjects, P = 0.012), but not for GAD (5.1% in autoimmune thyroiditis vs. 3.79% in control subjects, P = 0.367). Seven of 10 children with autoimmune thyroiditis and detectable Type 1 diabetes autoantibodies at the diagnosis remained positive after 1 year. In the course of 2 years of follow-up, two patients who were positive for Type 1 diabetes autoantibodies at the time of diagnosis of autoimmune thyroiditis developed diabetes. CONCLUSIONS: This is the first study reporting the prevalence of Type 1 diabetes autoantibodies in a selected cohort of genetically homogeneous children and adolescents with autoimmune thyroiditis. The main finding was that the prevalence of Type 1 diabetes autoantibodies and of newly diagnosed Type 1 diabetes in patients with autoimmune thyroiditis was significantly higher than that observed in the general paediatric population, suggesting that children with autoimmune thyroiditis are at increased risk of developing Type 1 diabetes.

The effect of puberty on insulin resistance in obese children.

OBJECTIVE: Insulin resistance (IR) increases during puberty in normal children. IR is the first adverse metabolic event of obesity, and the marker of the metabolic syndrome. We aimed to study the effect of puberty on IR in obese and normal-weight children. DESIGN: Cross-sectional evaluation of fasting glucose, insulin concentrations, and homeostasis model assessment of IR (HOMA-IR) in obese and control children throughout puberty. PATIENTS AND METHODS: We recruited 424 obese children (207 pre-pubertal and 217 pubertal divided in Tanner stages 2-3, 4, and 5) and estimated IR using the HOMA-IR index. Data were compared to those obtained in 123 healthy normal-weight children (40 pre-pubertal and 83 pubertal divided in Tanner stages 2-3, 4, and 5). RESULTS: In the obese children mean HOMA-IR increased progressively across Tanner stages, and was significantly higher in all groups (pre-pubertal and Tanner stages 2-3, 4, and 5) of obese than in control children. HOMA-IR was significantly correlated with BMI. CONCLUSIONS: HOMA-IR in obese children increases at puberty more than in normal-weight children and does not return to pre-pubertal values at the end of puberty.

Road traffic injuries in the province of Grosseto.

INTRODUCTION: Road traffic injuries constitute a major public health issue. The Province of Grosseto is one of the territories most affected in the Region of Tuscany. The objective of the study, part of the Road Safety Provincial Council's project, is to describe the epidemiology of the road accidents in order to contribute to the reduction of the burden of deaths and injuries. METHODS: The data relative to road accidents occurring in the Province were drawn from the various sources available: Death Certificates (1991-2005), Police Reports (1991-2003), Hospital Discharge Records (1996-2005), Emergency Room visits (2004-2005). RESULTS: On average, each year road accidents cause 30 deaths, at least 530 hospitalizations, and approximately 3,300 Emergency Room visits. The standardized mortality rate (2003-2005, males: 20.6; females: 6.0), the mortality ratio (2003:34.6 deaths for every 1,000 accidents), and the severity ratio (2003: 1,432 injured for every 1,000 accidents) are higher than regional figures. DISCUSSION: The greater relative number of fatalities, casualties and crashes can be explained by various physical and social environmental factors such as vast flatland, few greater urban settlements, deprived area. The territory specifically demonstrates an accentuated seasonality in August, a month in which a peak in both the number of accidents and their severity is reported, brought about by the intense volume of commuter and transit traffic, and highlighted by the fact that in that same month approximately half of Emergency Room visits concern non-residents. CONCLUSION: The complexity of the issue, the number of determinant factors involved, and the disproportionately greater impact on the more disadvantaged and vulnerable segments of society require the development of inter-sectoral strategies and the sharing of responsibility among individuals, groups and communities.

[Laboratory diagnosis: correlated health and environmental risks]. / La diagnostica di laboratorio: rischi sanitari e ambientali correlati.

Method description and initial results of a study to assess risks to health sector workers and environment due to chemical agents used and waste products generated in diagnostic clinical chemical laboratories, and image diagnostic testing. A survey was conducted of the methods and agents used and their toxicological classification, the number of workers exposed and an analytical profile of the waste produced. The assessment of risk to workers was based on cytogenetic tests (chromosome aberrations and micronuclei); the assessment of environmental risk from waste disposal was based on tests on plant systems.

Expression of HLA class I-specific inhibitory natural killer cell receptors in HIV-specific cytolytic T lymphocytes: impairment of specific cytolytic functions.

Human T lymphocytes have been shown to express inhibitory natural killer cell receptors (NKR), which can down-regulate T cell antigen receptor-mediated T cell function, including cytolytic activity. In the present study, we demonstrate that CD3+NKR+ cells can be identified in HIV-infected patients. HIV-specific cytolytic activity was analyzed in five patients in whom autologous lymphoblastoid B cell lines could be derived as a source of autologous target cells. Phytohemagglutinin-activated T cell populations that had been cultured in interleukin 2 displayed HIV-specific cytotoxic T lymphocyte (CTL) activity against HIV env, gag, pol, and nef in 3 of 5 patients. Addition of anti-NKR mAb of IgM isotype could increase the specific CTL activity. Moreover, in one additional patient, HIV-specific CTL activity was undetectable; however, after addition of anti-NKR mAb such CTL activity appeared de novo. Similar results were obtained by analysis of CD3+NKR+ clones derived from two patients. These data provide direct evidence that CD3+NKR+ cells may include antigen (HIV)-specific CTLs and that mAb-mediated masking of inhibitory NKR may revert the down-regulation of CTL function.