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Journal of Personalized Medicine Jun 2024Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are inflammatory polyneuropathies with an autoimmune etiology. These diseases...
INTRODUCTION
Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are inflammatory polyneuropathies with an autoimmune etiology. These diseases differ mainly in the timing of their course but also in certain clinical differences. Electroneurography and electromyography are crucial for fulfilling the primary (for CIDP) and secondary (for GBS) diagnostic criteria. High-resolution ultrasound (HRUS) is recognized as a complementary method in the diagnosis of CIDP and GBS.
AIM
The aim of this study was to present the neurophysiological and ultrasound findings of patients with clinically diagnosed inflammatory neuropathies (GBS and CIDP).
MATERIAL AND METHODS
We collected data from clinically confirmed patients with GBS (3 persons) and CIDP (6 persons). The neurography and high-resolution ultrasound examinations according to the UPSS scale were performed.
RESULTS
The neurography tests of GBS and CIDP patients showed mainly demyelinating lesions of the examined nerves, often with abnormal F-wave recordings. Examination using HRUS in GBS patients showed mild and regional nerve swelling with hypoechoic bundles with a predilection for proximal segments and cervical spinal nerve roots. In contrast, CIDP patients had diffused nerve swelling with hypoechoic bundles of greater severity and extent than those with GBS.
CONCLUSION
Neurophysiological tests and HRUS of peripheral nerves, plexi, and roots performed together can be very valuable, complementary diagnostic methods for the early diagnosis and effective treatment of inflammatory polyneuropathies.
PubMed: 38929824
DOI: 10.3390/jpm14060603 -
Life (Basel, Switzerland) Jun 2024Costello syndrome (CS) is a rare genetic syndrome in which, due to the occurrence of a mutation in the HRAS gene on chromosome 11 that causes the manifestation, a set of...
Costello syndrome (CS) is a rare genetic syndrome in which, due to the occurrence of a mutation in the HRAS gene on chromosome 11 that causes the manifestation, a set of features such as a characteristic appearance, many congenital defects, intellectual disability and a genetic predisposition to cancer, friendly personality, and others can be identified. CS is very rare, with an incidence of ~1/300,000, but it belongs to one of the largest groups of congenital syndromes, called RASopathies, occurring with an incidence of 1/1000 people. Scoliosis and kyphosis, as well as other spinal defects, are common, in 63% and 58% of patients, respectively, and a study conducted among adult patients showed the presence of scoliosis in 75% of patients; there may be excessive lordosis of the lumbar section and inverted curvatures of the spine (lordosis in the thoracic section and kyphosis in the lumbar section). The aim of our study is to present a case report of treatment of severe scoliosis of 130 degrees in a 14-year-old patient with Costello syndrome, with coexisting Chiari II syndrome and syrinx in the absence of skeletal maturity. This patient underwent foramen magnum decompression 3 months before planned surgical correction for severe scoliosis. The patient was qualified for surgical treatment using magnetically controlled growing rods (MCGR). After spine surgery using MCGR, we gradually performed MCGR distraction over the next 2 years; we performed the final surgery, conversion to posterior spinal fusion (PSF) with simultaneous multi-level Ponte osteotomy, which gave a very good and satisfactory surgical result. In the perioperative period, two serious complications occurred: pneumothorax caused by central catheter and gastrointestinal bleeding due to previously undiagnosed gastrointestinal varices. This case shows that the treatment of severe and neglected scoliosis is complicated and requires special preparation and a surgical plan with other cooperating specialists. The scoliosis was corrected from 130 degrees to approximately 48 degrees, sagittal balance was significantly improved, and the surgical outcome was very pleasing, significantly improving quality of life and function for the patient.
PubMed: 38929723
DOI: 10.3390/life14060740 -
Brain Sciences Jun 2024Alcohol use disorder (AUD) is related to mental and somatic disorders that result in alcohol withdrawal syndrome (AWS), with 30% of AWS cases leading to life-threatening... (Review)
Review
BACKGROUND
Alcohol use disorder (AUD) is related to mental and somatic disorders that result in alcohol withdrawal syndrome (AWS), with 30% of AWS cases leading to life-threatening delirium tremens (DTs). Currently, studies do not support using any one biomarker in DTs. Neurotrophins affect neuromodulation, playing a role in the pathogenesis of AUD, AWS, and DTs.
METHODS
This review aims to summarize experimental and clinical data related to neurotrophins and S100B in neuroplasticity, as well as neurodegeneration in the context of AUD, AWS, and DTs. This work used publications that were selected based on the protocol consistent with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) statement.
RESULTS
The BDNF level could be a good candidate biomarker for relapse susceptibility, as it is significantly reduced during consumption and gradually increases during abstinence. GDNF influences AUD through its integral role in the function of dopaminergic neurons and ablates the return to alcohol-drinking behavior. NGF protects neurons from ethanol-induced cytotoxic damage and affects recovery from cognitive deficits after brain damage. The NT-3 level is decreased after alcohol exposure and is involved in compensatory mechanisms for cognitive decline in AUD. NT-4 affects oxidative stress, which is associated with chronic alcohol consumption. S100B is used as a biomarker of brain damage, with elevated levels in serum in AUD, and can protect 5-HT neurons from the damage caused by alcohol.
CONCLUSIONS
BDNF, GDNF, NT-3, NT-4, NGF, and S100B may be valuable markers for withdrawal syndrome. In particular, the most relevant is their association with the development of delirium complications. However, there are few data concerning some neurotrophins in AWS and DTs, suggesting the need for further research.
PubMed: 38928583
DOI: 10.3390/brainsci14060583 -
International Journal of Molecular... Jun 2024The role of adipose mesenchymal stem cells (Ad-MSCs) in metabolic syndrome remains unclear. We aimed to assess the expression of selected microRNAs in Ad-MSCs of...
The role of adipose mesenchymal stem cells (Ad-MSCs) in metabolic syndrome remains unclear. We aimed to assess the expression of selected microRNAs in Ad-MSCs of non-diabetic adults in relation to Ad-MSC secretion of protein regulators and basic metabolic parameters. Ten obese, eight overweight, and five normal weight subjects were enrolled: 19 females and 4 males; aged 43.0 ± 8.9 years. Ad-MSCs were harvested from abdominal subcutaneous fat. Ad-MSC cellular expressions of four microRNAs (2 values) and concentrations of IL-6, IL-10, VEGF, and IGF-1 in the Ad-MSC-conditioned medium were assessed. The expressions of miR-21, miR-122, or miR-192 did not correlate with clinical parameters (age, sex, BMI, visceral fat, HOMA-IR, fasting glycemia, HbA1c, serum lipids, CRP, and eGFR). Conversely, the expression of miR-155 was lowest in obese subjects (3.69 ± 2.67 × 10 vs. 7.07 ± 4.42 × 10 in overweight and 10.25 ± 7.05 × 10 in normal weight ones, = 0.04). The expression of miR-155 correlated inversely with BMI (sex-adjusted r = -0.64; < 0.01), visceral adiposity (r = -0.49; = 0.03), and serum CRP (r = -0.63; < 0.01), whereas it correlated positively with serum HDL cholesterol (r = 0.51; = 0.02). Moreover, miR-155 synthesis was associated marginally negatively with Ad-MSC secretion of IGF-1 (r = -0.42; = 0.05), and positively with that of IL-10 (r = 0.40; = 0.06). Ad-MSC expression of miR-155 appears blunted in visceral obesity, which correlates with Ad-MSC IGF-1 hypersecretion and IL-10 hyposecretion, systemic microinflammation, and HDL dyslipidemia. Ad-MSC studies in metabolic syndrome should focus on miR-155.
Topics: Humans; MicroRNAs; Female; Male; Metabolic Syndrome; Mesenchymal Stem Cells; Adult; Middle Aged; Adipose Tissue; Insulin-Like Growth Factor I; Obesity; Interleukin-10; Gene Expression Regulation; Vascular Endothelial Growth Factor A
PubMed: 38928349
DOI: 10.3390/ijms25126644 -
Biomedicines Jun 2024The consequences of COVID-19 constitute a significant burden to healthcare systems worldwide. Conducting an HRQoL assessment is an important aspect of the evaluation of...
The consequences of COVID-19 constitute a significant burden to healthcare systems worldwide. Conducting an HRQoL assessment is an important aspect of the evaluation of the impact of the disease. The aim of this study was to investigate the prevalence of persistent symptoms and their impact on HRQoL and health status in COVID-19 convalescents. The study group consists of 46 patients who required hospitalization due to respiratory failure and who were subsequently evaluated 3 and 9 months after hospital discharge. At the follow-up visits, the patients were asked to assess their HRQoL using the EQ-5D-5L questionnaire. The results of chest CT, 6MWT, as well as the severity of the course of COVID-19 were also considered in the analysis. The obtained results have identified fatigue as the most common persistent symptom. The majority of the convalescents reported an impairment of HRQoL in at least one domain (80% and 82% after 3 and 9 months, respectively), of which the most common was that of pain/discomfort. The presence of ongoing symptoms may affect HRQoL in particular domains. The 6MWT outcome correlates with HRQoL 3 months after hospital discharge. Therefore, it may be useful in identifying patients with reduced HRQoL, allowing early interventions aimed at its improvement.
PubMed: 38927489
DOI: 10.3390/biomedicines12061282 -
Biomedicines Jun 2024This report focuses on the treatment histories of 21 patients diagnosed with Dravet syndrome (DRVT) under the care of the Mother and Child Institute in Warsaw. This...
BACKGROUND
This report focuses on the treatment histories of 21 patients diagnosed with Dravet syndrome (DRVT) under the care of the Mother and Child Institute in Warsaw. This paper aims to present typical treatment schemes for patients with drug-resistant epilepsy, as well as to highlight the influence of genetic diagnosis on pharmacotherapeutic management and to present an economic analysis of hospitalization costs. This paper will also summarize the effectiveness of the latest drugs used in DRVT.
METHODS
Clinical data were collected retrospectively from available medical records. The effectiveness of anticonvulsant treatment was assessed based on epileptic seizure diaries and observations by caregivers and pediatric neurologists.
RESULTS
The study group ( = 21) consisted of patients aged 3-26 years. Orphan drugs dedicated to Dravet syndrome were introduced in all patients due to the genetic diagnosis, which significantly improved the patients' clinical conditions. The breakthrough drugs were stiripentol (in 16/21) and fenfluramine (in 3/21).
CONCLUSIONS
In recent years, molecular genetics has rapidly developed in Poland, along with a steady increase in knowledge of Dravet syndrome among the medical profession. Early and precise diagnosis provides the opportunity to target treatment with drugs dedicated to Dravet syndrome with high efficacy.
PubMed: 38927456
DOI: 10.3390/biomedicines12061249 -
Biomedicines Jun 2024Despite the fact that the global COVID-19 pandemic has officially ended, we continue to feel its effects and discover new correlations between SARS-CoV-2 infection and... (Review)
Review
Despite the fact that the global COVID-19 pandemic has officially ended, we continue to feel its effects and discover new correlations between SARS-CoV-2 infection and changes in the organism that have occurred in patients. It has been shown that the disease can be associated with a variety of complications, including disorders of the nervous system such as a characteristic loss of smell and taste, as well as less commonly reported incidents such as cranial polyneuropathy or neuromuscular disorders. Nervous system diseases that are suspected to be related to COVID-19 include Guillain-Barré syndrome, which is frequently caused by viruses. During the course of the disease, autoimmunity destroys peripheral nerves, which despite its rare occurrence, can lead to serious consequences, such as symmetrical muscle weakness and deep reflexes, or even their complete abolition. Since the beginning of the pandemic, case reports suggesting a relationship between these two disease entities have been published, and in some countries, the increasing number of Guillain-Barré syndrome cases have also been reported. This suggests that previous contact with SARS-CoV-2 may have had an impact on their occurrence. This article is a review and summary of the literature that raises awareness of the neurological symptoms' prevalence, including Guillain-Barré syndrome, which may be impacted by the commonly occurring COVID-19 disease or vaccination against it. The aim of this review was to better understand the mechanisms of the virus's action on the nervous system, allowing for better detection and the prevention of its complications.
PubMed: 38927455
DOI: 10.3390/biomedicines12061248 -
Plant Physiology and Biochemistry : PPB Jun 2024Cytokinins are growth-regulating plant hormones that are considered to adjust plant development under environmental stresses. During sole ammonium nutrition, a condition...
Cytokinins are growth-regulating plant hormones that are considered to adjust plant development under environmental stresses. During sole ammonium nutrition, a condition known to induce growth retardation of plants, altered cytokinin content can contribute to the characteristic ammonium toxicity syndrome. To understand the metabolic changes in cytokinin pools, cytokinin biosynthesis and degradation were analyzed in the leaves and roots of mature Arabidopsis plants. We found that in leaves of ammonium-grown plants, despite induction of biosynthesis on the expression level, there was no active cytokinin build-up because they were effectively routed toward their downstream catabolites. In roots, cytokinin conjugation was also induced, together with low expression of major synthetic enzymes, resulting in a decreased content of the trans-zeatin form under ammonium conditions. Based on these results, we hypothesized that in leaves and roots, cytokinin turnover is the major regulator of the cytokinin pool and does not allow active cytokinins to accumulate. A potent negative-regulator of root development is trans-zeatin, therefore its low level in mature root tissues of ammonium-grown plants may be responsible for occurrence of a wide root system. Additionally, specific cytokinin enhancement in apical root tips may evoke a short root phenotype in plants under ammonium conditions. The ability to flexibly regulate cytokinin metabolism and distribution in root and shoot tissues can contribute to adjusting plant development in response to ammonium stress.
PubMed: 38924907
DOI: 10.1016/j.plaphy.2024.108858 -
Journal of Cardiovascular Pharmacology Apr 2024Current guidelines recommend that direct anticoagulants should not be used in prevention of recurrent thrombosis in patients with antiphospholipid syndrome (APS)....
Current guidelines recommend that direct anticoagulants should not be used in prevention of recurrent thrombosis in patients with antiphospholipid syndrome (APS). However, except for triple-positive APS and rivaroxaban use, little evidence supports such recommendation. In a real-life cohort study, we evaluated the risk of thromboembolism and bleeding in APS patients on apixaban versus vitamin K antagonists (VKA). We enrolled 152 APS patients (aged 44 [interquartile range 36-56], 83% women), including 66 patients treated with apixaban 5 mg bid and 86 with warfarin (target INR [international normalized ratio] 2-3). During a median follow-up of 53 months, we recorded venous thromboembolism (VTE), ischemic stroke or myocardial infarction, along with major bleeding. We observed 4 (6.1%, 3 VTE and 1 ischemic stroke) thrombotic events in patients on apixaban and 12 events (14%, 9 VTE, 2 ischemic strokes and 1 myocardial infarction) in VKA patients. APS patients on apixaban had similar risk of recurrent thromboembolism compared to those on warfarin (HR=0.327, 95% CI: 0.104-1.035). Thromboembolic events occurred less commonly in statin users (8% vs 50%, p=0.01) and more frequently in triple-positive APS (50% vs 22.1%, p=0.028) and in subjects with higher D-dimer at baseline (p=0.023); the latter difference was present in the apixaban group (p=0.02). Patients on apixaban had similar risk of major bleeding compared to warfarin (HR=0.54, 95% CI: 0.201-1.448). In real-life APS patients apixaban appears to be similar to VKA for the prevention of thromboembolism and risk of bleeding, which might suggest that some APS patients could be treated with apixaban.
PubMed: 38922590
DOI: 10.1097/FJC.0000000000001578 -
Metabolites May 2024Cardiovascular diseases accompanying metabolic syndrome comprise one of the leading causes of death worldwide. The medical community undertakes attempts to improve... (Review)
Review
Cardiovascular diseases accompanying metabolic syndrome comprise one of the leading causes of death worldwide. The medical community undertakes attempts to improve treatment options and minimize cardiovascular diseases' numerous consequences and exacerbations. In parallel with pharmacotherapies provided by physicians, nutritionists are developing strategies for diet therapy and prevention based on lifestyle changes, with high success rates. Consumption of specified food compounds included in various products with proven protective properties can be helpful in this regard. Due to the wide possibilities of diet in metabolic health promotion, it seems necessary to systematize information about the metabolically protective and cardioprotective properties of fiber, probiotic bacteria, plant sterols, folic acid, vitamins B12, C, and E, PUFAs, lycopene, polyphenols, arginine, CoQ10, and allicin. The aim of this review was to present the food compounds with potential use in cardiometabolic prevention and diet therapy based on the latest available literature.
PubMed: 38921431
DOI: 10.3390/metabo14060296