Frame navigation guided percutaneous balloon compression for intractable trigeminal neuralgia secondary to multiple sclerosis.

Background: Patients with multiple sclerosis (MS) are more likely to develop trigeminal neuralgia (TN) compared to the regular population, due to scarring of the nerve and development of a demyelination plaque. Despite treatment, approximately 10% of MS patients treated for TN experience symptom recurrence, including the development of MS-like symptoms such as optic neuritis and bilateral facial pain. Methods: A computed tomography (CT) scan was performed preoperatively on two patients diagnosed with multiple sclerosis (MS) who experienced secondary trigeminal neuralgia (TN). A precise reference frame was strapped firmly to the patient's forehead during the intraoperative procedure. Preliminary CT images were registered using the navigation system and the bony landmarks were set. Case description: Two patients diagnosed with multiple sclerosis (MS) who experienced refractory trigeminal neuralgia (TN) underwent percutaneous balloon compression. Initial conservative treatment and one dosage of Gamma Knife Radiosurgery (GKR) resulted in symptom control for a few weeks. Both patients had an acute recurrence of pain; thus, percutaneous retrogasserian balloon compression was performed. During follow-up, the patients reported a 70% decrease in pain after the procedure, with minimal recurrence of shooting episodes. Conclusion: Management of trigeminal neuralgia secondary to drug-resistant multiple sclerosis presents a persistent challenge. The percutaneous technique for retrogasserian balloon compression may offer a solution for some patients, but it presents unique challenges for neurosurgeons. Given the complexity of the pathogenesis, target identification, and the potential absence of neurovascular conflict, microvascular decompression remains a debated approach for this patient population. While stereotactic radiosurgery may be a promising alternative.

Anisakis simplex and urticaria. What we know about its real incidence and management in dermatological settings?

Acute or chronic infections have been described among causes of chronic urticaria (CU). Anisakidosis is a human disease caused by the ingestion of larval nematodes of the family Anisakidae. The infestation is acquired by eating raw seafood or undercooked fish and squid. There are considerable variations in the frequency of underlying causes in the different studies and in different countries, such as differences in diets and the prevalence of infections. Anisakis simplex has been recognized as a trigger of both acute and CU manifestations. However, there is still a lack of evidence about its management and treatment in dermatology. We, therefore, reviewed some biologic properties of Anisakis simplex in order to understand the relationship between its biology and the mechanism it uses to establish chronic dermatological conditions such as urticaria and cause late complications. In addition, we herein report some concerns about the effectiveness of systemic treatment in preventing complications and management in dermatological settings.

Prevalence of Autoimmune and Autoinflammatory Diseases in Chronic Urticaria: Pathogenetic, Diagnostic and Therapeutic Implications.

Chronic spontaneous urticaria (CSU) is defined as the almost daily occurrence of widespread wheals, angioedema, or both, for more than 6 weeks. It affects 1-2% of the general population, with a higher prevalence in female patients, and is more frequent patients over 20 years of age. More than half of all cases of chronic idiopathic urticaria are thought to occur due to an autoimmune mechanism, specifically the production of autoantibodies against the high-affinity immunoglobulin E (IgE) receptor (FcεRI). The quality of life in these patients is often greatly compromised, also due to the onset of comorbidities represented by other autoimmune diseases, such as thyroid disease, rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, celiac disease, and type 1 diabetes, among others. This review aimed to analyze the close correlation between CSU and some autoimmune and autoinflammatory diseases, in order to encourage a multidisciplinary and multimorbid approach to the patient affected by CSU, which allows not only control of the natural course of the disease, but also any associated comorbidities.

Acquired Hemophilia A Treated with Recombinant Porcine Factor VIII: Case Report and Literature Review on Its Efficacy.

Acquired hemophilia A (AHA) is a bleeding disorder due to the presence of neutralizing autoantibodies named inhibitors in patients with a previously normal hemostasis. Recent international recommendations suggest the use of bypassing agents or substitutive therapy as the first-line treatment, usually preferring the former. The adequate hemostatic therapy needs an accurate balance between bleeding and thrombotic risks. We report a clinical case of acquired hemophilia A successfully treated with recombinant porcine factor VIII (Susoctocog alfa) as the first-line treatment. Despite the patient having a high-risk thrombotic score and a history of recent myocardial infarction, our experience showed the absence of thrombotic complications related to the use of Susoctocog alfa and a complete restoration of hemostatic parameters. Limited literature is present on the use of recombinant porcine factor VIII as a first-line treatment, and our report supports its use, especially when the thrombotic risk is high.

5-Aminolevulinic Acid-A Biomarker for Worse Prognosis in IDH-Wildtype II Tumors? Evolution of a Fluorescence-Positive Diffuse Astrocytoma: A Case Report.

Introduction In 2017, the U.S. Food and Drug Administration (FDA) approved 5-aminolevulinic acid (5-ALA) as an intraoperative optical imaging agent in patients with suspected high-grade gliomas (HGGs). However, the application of 5-ALA for low-grade gliomas is still less accepted. Astrocytoma, isocitrate dehydrogenase (IDH) mutant tumors are diffuse infiltrating astrocytic tumors where there is no identifiable border between the tumor and normal brain tissue, even though the borders may appear relatively well-marginated on imaging. Generally, it is considered that 5-ALA cannot pass through a normal blood-brain barrier (BBB). Thus, 5-ALA fluorescence may mean disruption of BBB in grade II glioma. Case Report A 74-year-old male patient was diagnosed with a right parietal lesion suggestive of a low-grade brain tumor in a surgical resection using 5-ALA, which led to the detection of tiny fluorescence spots during the surgery. The frozen section was consistent with diffuse astrocytoma, IDH-wildtype (World Health Organization [WHO] grade II). The patient's postoperative magnetic resonance imaging (MRI) showed complete resection. Eight months after surgery, he began experiencing symptoms again and was admitted with a brain MRI finding consistent with recurrent infiltrating astrocytomas. This required reoperation of the brain tumor resection with 5-ALA. Unlike the first surgery, they observed a high fluorescence intensity; the pathological finding was glioblastoma, IDH-wildtype (WHO grade IV). Postsurgical brain MRI showed total resection of the tumor. The patient was discharged 4 weeks after surgery and continued with specialized clinical follow-up. Conclusion The use of 5-ALA continues to be a great contributor to the improvement in complete resection of primary brain tumors, especially HGG. Besides, fluorescence is increasingly approaching its use as a prognostic tool for aggressive clinical course, regardless of the initial grade of the tumor. This case report is an effort to expand knowledge for potentially using 5-ALA to help prognosticate brain tumors. Nevertheless, more clinical prospective studies must be conducted.

Involvement of Il-33 in the Pathogenesis and Prognosis of Major Respiratory Viral Infections: Future Perspectives for Personalized Therapy.

Interleukin (IL)-33 is a key cytokine involved in type-2 immunity and allergic airway disease. At the level of lung epithelial cells, where it is clearly expressed, IL-33 plays an important role in both innate and adaptive immune responses in mucosal organs. It has been widely demonstrated that in the course of respiratory virus infections, the release of IL-33 increases, with consequent pro-inflammatory effects and consequent exacerbation of the clinical symptoms of chronic respiratory diseases. In our work, we analyzed the pathogenetic and prognostic involvement of IL-33 during the main respiratory viral infections, with particular interest in the recent SARS-CoV-2virus pandemic and the aim of determining a possible connection point on which to act with a targeted therapy that is able to improve the clinical outcome of patients.

Sjögren's syndrome: a systemic autoimmune disease.

Sjögren's syndrome is a chronic autoimmune disease characterized by ocular and oral dryness resulting from lacrimal and salivary gland dysfunction. Besides, a variety of systemic manifestations may occur, involving virtually any organ system. As a result, the disease is characterized by pleomorphic clinical manifestations whose characteristics and severity may vary greatly from one patient to another. Sjögren's syndrome can be defined as primary or secondary, depending on whether it occurs alone or in association with other systemic autoimmune diseases, respectively. The pathogenesis of Sjögren's syndrome is still elusive, nevertheless, different, not mutually exclusive, models involving genetic and environmental factors have been proposed to explain its development. Anyhow, the emergence of aberrant autoreactive B-lymphocytes, conducting to autoantibody production and immune complex formation, seems to be crucial in the development of the disease. The diagnosis of Sjögren's syndrome is based on characteristic clinical signs and symptoms, as well as on specific tests including salivary gland histopathology and autoantibodies. Recently, new classification criteria and disease activity scores have been developed primarily for research purposes and they can also be useful tools in everyday clinical practice. Treatment of Sjögren's syndrome ranges from local and symptomatic therapies aimed to control dryness to systemic medications, including disease-modifying agents and biological drugs. The objective of this review paper is to summarize the recent literature on Sjögren's syndrome, starting from its pathogenesis to current therapeutic options.

Basophils and Mast Cells in COVID-19 Pathogenesis.

Basophils and mast cells are among the principal inducers of Th2 responses and have a crucial role in allergic and anti-parasitic protective immunity. Basophils can function as antigen-presenting cells that bind antigens on their surface and boost humoral immune responses, inducing Th2 cell differentiation. Their depletion results in lower humoral memory activation and greater infection susceptibility. Basophils seem to have an active role upon immune response to SARS-CoV-2. In fact, a coordinate adaptive immune response to SARS-CoV-2 is magnified by basophils. It has been observed that basophil amount is lower during acute disease with respect to the recovery phase and that the grade of this depletion is an important determinant of the antibody response to the virus. Moreover, mast cells, present in a great quantity in the nasal epithelial and lung cells, participate in the first immune response to SARS-CoV-2. Their activation results in a hyperinflammatory syndrome through the release of inflammatory molecules, participating to the "cytokine storm" and, in a longer period, inducing pulmonary fibrosis. The literature data suggest that basophil counts may be a useful prognostic tool for COVID-19, since their reduction is associated with a worse prognosis. Mast cells, on the other hand, represent a possible therapeutic target for reducing the airway inflammation characteristic of the hyperacute phase of the disease.

Improvement in Health-Related Quality of Life in Food-Allergic Patients: A Meta-Analysis.

BACKGROUND: Food allergy (FA) is a growing global problem that can affect patients' health-related quality of life (HRQoL) owing to increased anxiety as well as social and economic restrictions. Interventions such as oral food challenges (OFCs) and oral immunotherapy (OIT) have been shown to improve HRQoL. However, meta-analyses and systematic synthesis of these data are lacking. OBJECTIVE: To review and quantitatively synthesize potential benefits of interventions (OIT and OFC) systematically to address FA to a variety of foods. METHODS: We conducted a systematic search through PubMed and Cochrane Medical Library databases and performed a meta-analysis focusing on studies assessing changes in HRQoL after OIT and/or OFCs in FA participants and caregivers from 2010 to July 2020. Random effects model and I2 statistics were used to assess overall intervention effects and heterogeneity across studies. RESULTS: We included 13 publications in this meta-analysis (OIT = 7; OFCs = 6). Mean change in HRQoL scores after OIT and OFCs was -1.25 (P < .001) and -0.78 (P = .052), with a significant I2 of 87% (P < .001) and 90% (P < .001), respectively. Five OIT studies found significant improvements in HRQoL in the OIT group compared with the placebo group, with an overall standardized mean difference of -0.56 (P = .007; I2 = 42%, P = .099). CONCLUSIONS: This meta-analysis showed that in FA patients, both OIT and OFCs are associated with an improvement in HRQoL. Well-designed and long-term HRQoL studies are necessary to ascertain sustained benefits of OIT and OFCs.

Hygiene hypothesis and autoimmune diseases: A narrative review of clinical evidences and mechanisms.

Since the start of the "modern era", characterized by the increase in urbanization, a progressive attention to hygiene and autoimmune conditions has considerably grown. Although these diseases are often multifactorial, it was demonstrated that environment factors such as pollution, diet and lifestyles may play a crucial role together with genetic signature. Our research, based on the newest and most significant literature of this topic, highlights that the progressive depletion of microbes and parasites due to increased socioeconomic improvement, may lead to a derangement of immunoregulatory mechanisms. Moreover, special attention was given to the complex interplay between microbial agents, as gut microbiome, diet and vitamin D supplementation with the aim of identifying promising future therapeutic options. In conclusion, autoimmunity cannot be limited to hygiene-hypothesis, but from the point of view of precision medicine, this theory represents a fundamental element together with the study of genomics, the microbiome and proteomics, in order to understand the complex functioning of the immune system.

H2-antagonist in IgE-mediated type I hypersensitivity reactions: what literature says so far?

Histamine is a monoamine synthesized from the amino acid histidine that is well-known for its role in IgE-mediated anaphylaxis but has shown pleiotropic effects on the immune system, especially in order to promote inflammatory responses. H1-receptor antagonist are common drugs used in mild/moderate allergic reactions whereas H2-receptor antagonist are commonly administered in gastric ulcer but showed some properties in allergy too. The EAACI guidelines for diagnosis and treatment of anaphylactic reactions recommend their use as third-line therapy in adjunct to H1-antagonists. The purpose of this article is to produce a complete summary of findings and evidence known so far about the usefulness of H2-receptor antagonist in allergic reactons.

Gamma knife stereotactic radiosurgery as an effective tool in primary CNS lymphoma: Evaluation of stereotactic radiosurgery and methotrexate treatment in a prospective and observational clinical research study.

OBJECTIVE: The purpose of this study was to compare the progression of Primary Central Nervous System Lymphoma (PCNSL) in patients treated with methotrexate (MTX) versus those treated with a combination of Stereotactic Radiosurgery (SRS) and MTX. Progression was measured via brain lesion count and tumor volume. METHODS: This observational and prospective cohort study evaluated the outcome of SRS treatment of PCNSL in one hundred twenty-eight subjects. We analyzed baseline, prospective, and retrospective data of patients enrolled in the brain tumor registry between June 2010 and August 2017. Seventy-three patients were treated exclusively with MTX while the remaining fifty-five patients received a combination of SRS and MTX. Strict inclusion and exclusion criteria were established. RESULTS: Mean survival rate for patients receiving combined SRS and MTX treatment was significantly higher (52.6 months) compared to the MTX group (19.8 months); p = 0.0029. At the 36 months follow-up, patients treated with SRS and MTX also had a lower rate of tumor progression (32.7 %) than the MTX group (95.9 %); p = 0.00192. Local tumor control was achieved in all patients treated with SRS. No clinical toxicity was observed in this group. CONCLUSIONS: Clinical results obtained from this observational study highlight the potential effectiveness of SRS in the treatment of PCNSL. Although treatment outcomes have improved in the past years, additional evidence in the clinical design of randomized trials is needed to evaluate the strength of this treatment in specific situations.

A rare hepatic mass in an Italian resident.

BACKGROUND: Amebiasis is a rare condition in developed countries but epidemiologically growing. Clinical manifestation may range from asymptomatic to invasive disease, amoebic liver abscess being the most common manifestation. We report a peculiar case of left hepatic amoebic liver abscess in a patient without a well-known source of infection and presenting with left portal vein thrombosis. CASE PRESENTATION: Patient, working as longshoreman, presented with complaints of remittent-intermittent fever lasting from 2 weeks. Physical examination was normal. Blood tests showed mild anemia, neutrophilic leukocytosis and elevated inflammation markers. Chest x-rays was normal. Abdominal ultrasound showed multiple hypoechoic liver masses. CT-scan of abdomen showed enlarged left liver lobe due to the presence of large abscess cavity along with thrombosis of left portal vein. The indirect hemagglutination test for the detection of antibodies to Entamoeba histolytica (Eh) was positive. Ultrasound-guided percutaneous drainage revealed "anchovy sauce" pus. Metronidazole and a follow up imaging at 3 months showed resolution of abscess cavity. CONCLUSION: This case shows that amoebic liver abscess is possible even in first world country patients without travel history. Left sided abscess and portal vein thrombosis are rare and hence reported.

New Developments in Non-allergen-specific Therapy for the Treatment of Food Allergy.

PURPOSE OF REVIEW: The prevalence of food allergy is increasing. At the current time, there are no approved treatments for food allergy. Major limitations of immunotherapy are long treatment periods (months or years), frequent clinic visits, high costs, increased risk of adverse events during treatment, and lack of durability of desensitization. Additionally, it is allergen-specific, and in those allergic to multiple allergens, the length and cost of treatment are further increased. In this review, we summarize recent developments in novel non-allergen-specific treatments for food allergy. RECENT FINDINGS: A number of monoclonal antibodies that block IgE or specific pro-allergenic cytokines or their receptors have shown promise in clinical trials for food allergy. The insight we have gained through the use of one drug for the treatment of an atopic disease is quickly being translated to other atopic diseases, including food allergy. The future for food allergy treatment with biologics looks bright.

IL-33/IL-31 Axis in Immune-Mediated and Allergic Diseases.

Several allergic and immunologic diseases including asthma, food allergy (FA), chronic spontaneous urticaria (CSU), atopic dermatitis (AD), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), rheumatoid arthritis (RA), and Behçet's disease (BD) are characterized by the involvement of Th2 immunity. Several mediators lead to immunoglobulin (Ig)E production, thus including key cytokines such as interleukin (IL)-4, IL-5, and IL-13. Among them, IL-31 and IL-33 have been recently studied as novel biomarkers and future therapeutic targets for allergic and immunological disorders. IL-31 is a proinflammatory cytokine-it regulates cell proliferation and is involved in tissue remodeling. IL-33, acting through its receptor suppression of tumorigenity (ST2L), is an alarmin cytokine from the IL-1 family, whose expression is mediated by tissue damage. The latter has a pleiotropic effect, as it may modulate specific and innate immune cells functions. To date, several researchers have investigated the involvement of IL-31 and IL-33 in several allergic and immune-mediated diseases. Further studies are needed to understand the future applications of these molecules as novel therapeutic agents. This paper aims to give the readers a complete and updated review of IL-31 and IL-33 involvement among the most common autoimmune and allergic disorders.

Effects of AntagomiRs on Different Lung Diseases in Human, Cellular, and Animal Models.

INTRODUCTION: MiRNAs have been shown to play a crucial role among lung cancer, pulmonary fibrosis, tuberculosis (TBC) infection, and bronchial hypersensitivity, thus including chronic obstructive pulmonary disease (COPD) and asthma. The oncogenic effect of several miRNAs has been recently ruled out. In order to act on miRNAs turnover, antagomiRs have been developed. MATERIALS AND METHODS: The systematic review was conducted under the PRISMA guidelines (registration number is: CRD42019134173). The PubMed database was searched between 1 January 2000 and 30 April 2019 under the following search strategy: (((antagomiR) OR (mirna antagonists) OR (mirna antagonist)) AND ((lung[MeSH Terms]) OR ("lung diseases"[MeSH Terms]))). We included original articles, published in English, whereas exclusion criteria included reviews, meta-analyses, single case reports, and studies published in a language other than English. RESULTS AND CONCLUSIONS: A total of 68 articles matching the inclusion criteria were retrieved. Overall, the use of antagomiR was seen to be efficient in downregulating the specific miRNA they are conceived for. The usefulness of antagomiRs was demonstrated in humans, animal models, and cell lines. To our best knowledge, this is the first article to encompass evidence regarding miRNAs and their respective antagomiRs in the lung, in order to provide readers a comprehensive review upon major lung disorders.

Emerging role of vitamin D in autoimmune diseases: An update on evidence and therapeutic implications.

Vitamin D plays a key role in in calcium homeostasis and, thus, provides an important support in bone growth by aiding in the mineralization of the collagen matrix. However, vitamin D performs various immunomodulatory, anti-inflammatory, antioxidant and anti-fibrotic actions. Autoimmune diseases result from an aberrant activation of the immune system, whereby the immune response is directed against harmless self-antigens. Does vitamin D play a role in the pathophysiology of autoimmune diseases? And, if so, what is its role? In the last decade, researchers' interest in vitamin D and its correlations with autoimmune diseases has considerably increased. We conducted a literature review, covering the period January 1, 2009 through March 30, 2019, in PubMed. We analyzed more than 130 studies in order to find a correlation between vitamin D levels and its effect upon several autoimmune diseases. The analysis demonstrated an inverse association between vitamin D and the development of several autoimmune diseases, such as SLE, thyrotoxicosis, type 1 DM, MS, iridocyclitis, Crohn's disease, ulcerative colitis, psoriasis vulgaris, seropositive RA, polymyalgia rheumatica. International multicenter study could allow us to confirm the data already present in the literature in the single clinical studies and to evaluate when to effectively supplement vitamin D in patients who do not take corticosteroids.