Underestimated olfactory domains in Huntington's disease: odour discrimination and threshold.

BACKGROUND: Olfaction has recently found clinical value in prediction, discrimination and prognosis of some neurodegenerative disorders. However, data originating from standard tests on olfactory dysfunction in Huntington's disease are limited to odour identification, which is only one domain of olfactory perceptual space. METHOD: Twenty-five patients and 25 age- and gender-matched controls were evaluated by the Sniffin' Sticks test in three domains of odour threshold, odour discrimination, odour identification and the sum score of them. Patients' motor function was assessed based on the Unified Huntington's Disease Rating Scale. RESULTS: Compared with controls, patients' scores of all olfactory domains and their sum were significantly lower. Besides, our patients' odour threshold and odour discrimination impairments were more frequently impaired than odour identification impairment (86 per cent and 81 per cent vs 34 per cent, respectively). CONCLUSION: Olfactory impairment is a common finding in patients with Huntington's disease; it is not limited to odour identification but is more pronounced in odour discrimination and odour threshold.

COVID-19 pandemic-induced physical inactivity: the necessity of updating the Global Action Plan on Physical Activity 2018-2030.

To prevent and reduce inactivity, the World Health Organization (WHO) designed a global plan called Global Action Plan on Physical Activity 2018-2030 (GAPPA) in 2017. In this plan and according to the state of physical activity in 2016, actions and goals were set. However, the world is facing a COVID-19 pandemic, which has affected various aspects of lifestyle, including physical activity. Some studies have shown that physical activity reduced during the pandemic. For this reason, the WHO should review the GAPPA and update goals and actions according to the state of physical activity in 2020.

Extracting Mutual Interaction Rules Using Fuzzy Structured Agent-based Model of Tumor-Immune System Interactions.

BACKGROUND: There are many studies to investigate the effects of each interacting component of tumor-immune system interactions. In all these studies, the distinct effect of each component was investigated. As the interaction of tumor-immune system has feedback and is complex, the alternation of each component may affect other components indirectly. OBJECTIVE: Because of the complexities of tumor-immune system interactions, it is important to determine the mutual behavior of such components. We need a careful observation to extract these mutual interactions. Achieving these observations using experiments is costly and time-consuming. MATERIAL AND METHODS: In this experimental and based on mathematical modeling study, to achieve these observations, we presented a fuzzy structured agent-based model of tumor-immune system interactions. In this study, we consider the confronting of the effector cells of the adaptive immune system in the presence of the cytokines of interleukin-2 (IL-2) and transforming growth factor-beta (TGF-ß) as a fuzzy structured model. Using the experimental data of murine models of B16F10 cell line of melanoma cancer cells, we optimized the parameters of the model. RESULTS: Using the output of this model, we determined the rules which could occur. As we optimized the parameters of the model using escape state of the tumor and then the rules which we obtained, are the rules of tumor escape. CONCLUSION: The results showed that using fuzzy structured agent-based model, we are able to show different output of the tumor-immune system interactions, which are caused by the stochastic behavior of each cell. But different output of the model just follow the predetermined behavior, and using this behavior, we can achieve the rules of interactions.

Evaluation of patients with primary immunodeficiency associated with Bacille Calmette-Guerin (BCG)-vaccine-derived complications

BACKGROUND: Bacille Calmette-Guerin (BCG) vaccination has a great impact on the prevention of severe complications of tuberculosis. However, in patients with primary immunodeficiencies (PID), it can lead to severe complications such as severe combined immunodeficiency, chronic granulomatous disease, and Mendelian susceptibility to mycobacterial disease. This study highlights the demographics, clinical complications and laboratory parameters among PID patients associated with BCG vaccination side effects. METHODS: One hundred and thirty-seven PID patients with BCGosis were evaluated in this study, based on the complications following BCG vaccination. RESULTS: The mean age of the patients with BCG complications at the time of the first visit was five years. The within-group comparison of patients showed a highly significant incidence of pneumonia and hepatomegaly in severe combined immunodeficiency patients. Furthermore, the immunologic data showed an increase in the overall rates of lymphocytes such as CD3+, CD4+ and CD8 + T cells in Mendelian susceptibility to mycobacterial disease patients. The level of immunoglobulins has also increased in chronic granulomatous disease patients. CONCLUSION: The high rate of undiagnosed PIDs predisposes individuals to a high risk of severe side effects as a result of BCG vaccination, as well as infants that are less than one month of age. Therefore, there is a need for early screening and diagnosis of PIDs before exposing unknown PID status patients to BCG vaccination. The benefits of screening and early diagnosis of PID cannot be overemphasized, especially in patients with a previous family history of immunodeficiency

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Physical activity during COVID-19 pandemic in the Iranian population: A brief report.

COVID-19 pandemic and restrictive public health measures due to it can have many effects on physical activity. Our study aimed to compare the levels of physical activity levels (expressed as MET-minute/week), among Qom city (Iran) adults in pre and during the COVID-19 pandemic (January 2020 and 20 to 29 May 2020, respectively). 670 adults were included in this study and grouped based on age and gender. The short-form International physical activity questionnaire (IPAQ, SF) was distributed to the participants through an online survey. The Wilcoxon signed-rank test was used for statistical analyses. The present study showed that the level of physical activity decreased significantly during COVID-19 compared to pre-COVID-19 in both sexes and age groups (p < 0.001). Also, this study found that a total of 78% of the participants did not meet the physical activity guidelines during COVID-19 in Iran. Our results indicate the necessity to consider an integrated and comprehensive approach to reduce the inactivity caused by COVID-19.

Evaluation of patients with primary immunodeficiency associated with Bacille Calmette-Guerin (BCG)-vaccine-derived complications.

BACKGROUND: Bacille Calmette-Guerin (BCG) vaccination has a great impact on the prevention of severe complications of tuberculosis. However, in patients with primary immunodeficiencies (PID), it can lead to severe complications such as severe combined immunodeficiency, chronic granulomatous disease, and Mendelian susceptibility to mycobacterial disease. This study highlights the demographics, clinical complications and laboratory parameters among PID patients associated with BCG vaccination side effects. METHODS: One hundred and thirty-seven PID patients with BCGosis were evaluated in this study, based on the complications following BCG vaccination. RESULTS: The mean age of the patients with BCG complications at the time of the first visit was five years. The within-group comparison of patients showed a highly significant incidence of pneumonia and hepatomegaly in severe combined immunodeficiency patients. Furthermore, the immunologic data showed an increase in the overall rates of lymphocytes such as CD3+, CD4+ and CD8 + T cells in Mendelian susceptibility to mycobacterial disease patients. The level of immunoglobulins has also increased in chronic granulomatous disease patients. CONCLUSION: The high rate of undiagnosed PIDs predisposes individuals to a high risk of severe side effects as a result of BCG vaccination, as well as infants that are less than one month of age. Therefore, there is a need for early screening and diagnosis of PIDs before exposing unknown PID status patients to BCG vaccination. The benefits of screening and early diagnosis of PID cannot be overemphasized, especially in patients with a previous family history of immunodeficiency.

Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management.

Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by hypogammaglobulinemia and increased susceptibility to recurrent bacterial infections. It is the most frequent symptomatic antibody deficiency, with a wide variety of infectious and noninfectious complications. Numerous studies have demonstrated that immunological and genetic defects are involved in the pathogenesis of CVID. However, in most cases, the genetic background of the disease remains unidentified. This review aims to discuss various aspects of CVID, including epidemiology, pathogenesis, symptoms, diagnosis, classification, and management.

Common variable immunodeficiency: epidemiology, pathogenesis, clinical manifestations, diagnosis, classification, and management

Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by hypogammaglobulinemia and increased susceptibility to recurrent bacterial infections. It is the most frequent symptomatic antibody deficiency, with a wide variety of infectious and noninfectious complications. Numerous studies have demonstrated that immunological and genetic defects are involved in the pathogenesis of CVID. However, in most cases, the genetic background of the disease remains unidentified. This review aims to discuss various aspects of CVID, including epidemiology, pathogenesis, symptoms, diagnosis, classification, and management

La inmunodeficiencia variable común (CVID) es un trastorno heterogéneo caracterizado por una hipogammaglobulinemia y por una mayor susceptibilidad a infecciones bacterianas recurrentes. Se trata de la inmunodeficiencia humoral sintomática más frecuente y cursa con una extensa variedad de complicaciones infecciosas y no infecciosas. En la patogenia de la CVID están involucrados diferentes defectos inmunológicos y genéticos. Sin embargo, en la mayoría de los casos, el fondo genético de la enfermedad permanece sin identificar. Esta revisión tiene como objetivo discutir diferentes aspectos de la CVID, incluyendo epidemiología, patogenia, síntomas, diagnóstico, clasificaciones y tratamiento de la enfermedad

Two faces of LRBA deficiency in siblings: hypogammaglobulinemia and normal immunoglobulin levels

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G protein-coupled estrogen receptor and estrogen receptor ligands regulate colonic motility and visceral pain.

BACKGROUND: Diarrhea-predominant irritable bowel syndrome (IBS-D) is a functional gastrointestinal (GI) disorder, which occurs more frequently in women than men. The aim of our study was to determine the role of activation of classical estrogen receptors (ER) and novel membrane receptor, G protein-coupled estrogen receptor (GPER) in human and mouse tissue and to assess the possible cross talk between these receptors in the GI tract. METHODS: Immunohistochemistry was used to determine the expression of GPER in human and mouse intestines. The effect of G-1, a GPER selective agonist, and estradiol, a non-selective ER agonist, on muscle contractility was characterized in isolated preparations of the human and mouse colon. To characterize the effect of G-1 and estradiol in vivo, colonic bead expulsion test was performed. G-1 and estradiol activity on the visceral pain signaling was assessed in the mustard oil-induced abdominal pain model. KEY RESULTS: GPER is expressed in the human colon and in the mouse colon and ileum. G-1 and estradiol inhibited muscle contractility in vitro in human and mouse colon. G-1 or estradiol administered intravenously at the dose of 20 mg/kg significantly prolonged the time to bead expulsion in females. Moreover, G-1 prolonged the time to bead expulsion and inhibited GI hypermotility in both genders. The injection of G-1 or estradiol resulted in a significant reduction in the number of pain-induced behaviors in mice. CONCLUSIONS AND INFERENCES: GPER and ER receptors are involved in the regulation of GI motility and visceral pain. Both may thus constitute an important pharmacological target in the IBS-D therapy.

Nailfold capillaroscopic changes in patients with systemic lupus erythematosus: correlations with disease activity, skin manifestation and nephritis.

Introduction The clinical expression of systemic lupus erythematosus (SLE) is the consequence of endothelial cell damage leading to serious multiple organ dysfunction. The aim of this study was to assess the association between nailfold capillaroscopic changes and disease activity, skin and renal involvement in patients with SLE. Methods Demographic variables, clinical manifestations and laboratory data of 108 patients with SLE were investigated. Nailfold capillaroscopy (NFC) was performed in all patients. Result Morphological changes in NFC were observed in 102 out of 108 (94.4%) SLE patients. Minor changes were found in 33 (30.6%) and major changes in 69 (63.9%) cases. The disease activity was significantly higher in the patients with major changes ( p < 0.002). A higher incidence of microhaemorrhages was seen in patients with active SLE disease ( p < 0.04). In SLE patients with active skin involvement, the disturbed distribution ( p < 0.004) was more frequent and subtle changes ( p < 0.009) were less frequently observed as compared with patients without active skin involvement. In the group of SLE patients with renal involvement, no correlation was found between the capillary abnormalities and the presence of renal involvement ( p > 0.05), except for the elongated capillary loops, which were seen more often in patients with renal involvement than in patients without it ( p < 0.03). Conclusion The results of the study showed that capillary changes (abnormal capillaroscopy) were very common in patients with SLE, although there were no specific patterns like the ones in scleroderma patients, and some changes may be associated with disease activity, especially in patients with active skin involvement.

Glucantime efficacy in the treatment of zoonotic cutaneous leishmaniasis.

Pentavalent antimony (SbV) compounds are still considered the first line of treatment for all forms of leishmaniasis. There have been reports of drug resistance and unresponsiveness to treatment with these drugs. We investigated the clinical response to treatment of cutaneous leishmaniasis with glucantime, the drug of choice for all forms of leishmaniasis in Iran. All individuals suspected of cutaneous leishmaniasis from October 2007 to March 2008 were included in the study if met specific criteria. After laboratory diagnosis and parasite identification by PCR, 43 patients agreed to participate and complete the protocol for treatment. Meglumine antimoniate (glucantime) was given at a dose of 20 mg/kg/day for 20 days (two 10-day periods) according to a World Health Organization (WHO) recommended protocol. Response to treatment was evaluated 6 weeks after initiation of treatment. Fifteen patients (34.9%) were clinically unresponsive to glucantime treatment while the remaining 28 patients (65.1%) responded to treatment. There were no statistically significant differences by occupation, gender, chronicity of the disease before starting treatment, number of lesions, or age between the glucantime sensitive and resistant patients. Our study showed a significant level of unresponsiveness to glucantime among patients with cutaneous leishmaniasis caused by Leishmania major in Iran. These findings highlight the need for new treatment regimens.

Post-traumatic capillary hemangiomas: a rare cause of hand nodules.

Capillary hemangiomas are hamartomatous proliferation of vascular endothelial cells, usually presenting in infancy or are age-related (senile). These tend to affect the head and neck regions. Herein, we report a rare cause of hand nodules: post-traumatic capillary hemangioma.

Double-blind, randomized feedback control fails to improve the hypocholesterolemic effect of a plant-based low-fat diet in patients with moderately elevated total cholesterol levels.

OBJECTIVE: To determine whether the cholesterol-lowering effect of a plant-based low-fat diet can be improved by a flexible control design that controls the extent of fat reduction based on the individual response of blood cholesterol. DESIGN: Randomized, double-blind intervention study. SETTING: A hotel in Prerow, Germany. SUBJECTS: A total of 32 participants (21 female and 11 male participants) with total cholesterol level > 5.7 mmol/l. INTERVENTION: The control group consumed a plant-based low-fat diet with constantly 20% of energy as fat; the intervention group received a diet with either 20 or 15% of energy as fat, depending on the serum cholesterol response of the preceding week. A flexible control design based on the individual cholesterol response during a run-in period of 1 week was used within a low-fat intervention. RESULTS: During the run-in period, the consumption of a plant-based low-fat diet led to a reduction in total cholesterol by 18+/-6 mmol/l (P < 0.001), in LDL cholesterol by 19+/-9 mmol/l (P < 0.001) and triglycerides by 13+/-3 mmol/l (P < 0.001). During the feedback control period, an additional reduction in total cholesterol by 13+/-8 (P < 0.001) and in LDL cholesterol by 17+/-11 (P < 0.001) was observed compared to 15+/-15 and 7+/-18 in the control group. The effect of an additional feedback control was only marginal and not statistically significant compared to the effect of the low-fat diet alone. CONCLUSIONS: On a level of fat intake already reduced to 20% of energy, the use of a feedback control to adapt the fat content of the diet depending on the individual serum cholesterol response was not more effective in reducing blood cholesterol levels than a plant-based low-fat diet alone.

Assessment of sedation, analgesia, and neuromuscular blockade in the perioperative period.

Identification of adequate pain relief, sedation, and neuromuscular blockade in the perioperative period (be it for monitored anesthesia care, conscious sedation, ICU analgesia or sedation, or during intraoperative care) continues to be a challenge. Current subjective and objective techniques have been reviewed. The combination of clinical judgement, physiological response, and selected monitoring tools provide the current standard of care used to optimize patient care and comfort. Future directions in assessing pain control, adequacy of sedation, and degree of neuromuscular blockade include: (1) simplified scoring scales, (2) computerized analysis of the EEG, (3) computerized evoked potentials, (4) portable accelerography of electromyography for determination of neuromuscular blockade, and (5) closed loop system for delivery of analgesics, sedatives, and paralytics.