Qualitative mucin disorders in patients with primary Sjögren's syndrome: a literature review.

BACKGROUND: It is a common opinion that Primary Sjögren Syndrome (pSS) damages the exocrine glands and determines the reduction of secreted saliva, some studies show that there are qualitative anomalies of the mucins produced in saliva, including MUC7, MUC5B, MUC1. The purpose of this study is to trace all the information useful to establish whether there is a qualitative or quantitative defect of the mucins in the pSS. MATERIAL AND METHODS: We reviewed the literature by looking for publications relevant to the topic in electronic databases. Sixteen articles met the search criteria. The studies were divided into two categories, those that studied the rheological characteristics of the saliva and those that studied the structural and / or metabolism modifications of the muciparous cells in the salivary glands. RESULTS: in Patients with pSS, xerostomia and the reduction of salivary spinnbarkeit are only partially related to the reduction of the unstimulated salivary flow. In pSS, pathological alterations of mucins' chemical-physical properties prevail as a cause of the clinical characteristics. Moreover, in pSS there are structural and metabolism changes in salivary glands' muciparous cells. CONCLUSIONS: There is much evidence that supports the presence of qualitative alterations in the saliva's rheological properties in Patients with pSS, and these are the main cause, more than the reduction of the unstimulated salivary flow, of the disease clinical characteristics - dry mouth and complications in the oral cavity. Therefore we propose to add to the classification criteria of pSS also a qualitative test of salivary glycoproteins.

Cancer stem cells in laryngeal cancer: what we know.

Cancer stem cells (CSCs) are a group of cells with eternal life or infinite self-renewal ability, which have high migrating, infiltrative, and metastatic abilities. Though CSCs only account for a small proportion in tumors, the high resistance to traditional therapy exempts them from therapy killing and thus they can reconstruct tumors. Previous studies found that heterogeneity of cancer cells extensively exists in head and neck cancers. Our current knowledge, about CSCs in the laryngeal cancer (LC), largely depends on head and neck studies. Given the lack of systematic data about CSCs in LC, we propose a review of the literature, reporting the evidences of CSCs in tumorigenesis of LC, with the purpose to provide new insights into the prevention and/or treatment of laryngeal carcinoma.

Evidence and role of autoantibodies in chronic rhinosinusitis with nasal polyps.

In this study, we review our current knowledge of the autoimmune etiopathogenesis of chronic rhinosinusitis with nasal polyps including bacterial infections, viral infections and immunomediated mechanisms and to discuss pathogenesis with relevance for pharmacotherapy. Relevant publications on the etiopathogenesis and treatment of chronic rhinosinusitis with nasal polyps (CRSwNP) from 1977 to 2013 were analyzed. The characteristic signs and symptoms include appearance of relapsing nasal polyps, with typical symptoms such as nasal obstruction, nasal discharge and, usually, loss of the sense of smell. The etiology and pathogenesis remain unknown. Proposed theories of causation include bacterial or viral infections and immunomediated mechanisms. The autoimmune aetiology of unknown origin or failure to respond to classic pharmacological treatments with nasal and oral steroids is now suspected. At present, the nature of the antigen trigger, the exact role played by B/T cells and anti-dsDNA autoantibodies in the pathogenesis of nasal polyposis remains unclear. Corticosteroids and surgery are the first line of treatment in CRSwNP. In the case of corticosteroid treatment failure, other drugs can be used such as rituximab, belimumab or omalizumab which have demonstrated clinical efficacy in the treatment of nasal polyposis with comorbid asthma. Immunosuppressive drugs such as methotrexate, and cyclophosphamide have also been used with varying degrees of success.

Carotid stenosis after adjuvant cervical radiotherapy in patients with head and neck cancers: a prospective controlled study.

OBJECTIVES: To determine the prevalence of radiation-induced carotid stenosis, in patients who were treated for head and neck malignancies, using colour-flow duplex scanning. DESIGN: Prospective controlled study at a single medical centre. PARTICIPANTS: We enrolled two groups of patients. The first (radiotherapy group) consisted of patients who received surgical treatment and adjuvant radiotherapy of the neck. The control group consisted of patients with head and neck malignancies who received only surgical treatment. MAIN OUTCOME MEASURES: All patients were evaluated with carotid artery ecoDoppler imaging 1 week before and 36 months after the surgical procedure. Intima-media thickness was measured bilaterally at the internal carotid artery and at the bifurcation. Carotid obstruction was classified as low (0-30%), moderate (31-49%) or severe (≥50%). RESULTS: The preoperative stenosis grade did not differ between groups. In 15/25 patients (60%) in the radiotherapy group, mild stenosis evolved to moderate stenosis, while only 6/37 (16%) of the controls did (P = 0.004). Additionally, 9/39 (23%) patients in the radiotherapy group progressed to severe stenosis compared with only 3/54 (6%) controls (P = 0.029). The overall evolution showed that stenosis worsened in 24/32 (62%) patients in the radiotherapy group and 9/54 (17%) patients in the control groups (P < 0.0001). CONCLUSIONS: These results highlight the need to study the long-term incidence of cerebrovascular events in these two different populations (radiation treated and surgically treated) to identify increased cerebrovascular morbidity.

The neutrophil/lymphocyte ratio as a prognostic factor in COVID-19 patients: a case-control study.

OBJECTIVE: SARS-CoV-2 (Severe Acute Respiratory Syndrome Coronavirus 2) has been identified in China as responsible for viral pneumonia, now called COVID-19 (Coronavirus Disease 2019). Patients infected can develop common symptoms like cough and sore throat, and, in severe cases, acute respiratory syndrome and even death. To optimize the available resources, it is necessary to identify in advance the subjects that will develop a more serious illness, therefore requiring intensive care.The neutrophil / lymphocyte ratio (NLR) parameter, resulting from the blood count, could be a significant marker for the diagnosis and management of risk stratification. PATIENTS AND METHODS: A retrospective, single-center case-control observational study was conducted. The differential cell count of leukocytes, the NLR and the clinical course of patients hospitalized in intensive care with COVID-19 were analyzed, comparing them with other patients (COVID-19 and non-COVID-19) and healthy individuals selected among workers of the Teaching Hospital Policlinico Umberto I in Rome. RESULTS: 370 patients (145 cases and 225 controls) were included in the case-control study, 211 males (57%) and 159 females (43%). The average age of the population was 63 years (SD 16.35). In the group of cases, out of 145 patients, 57 deaths and 88 survivors were recorded, with a lethality rate of 39.3%. The group of cases has an NLR of 7.83 (SD = 8.07), a much higher value than the control group where an NLR of 2.58 was recorded (SD = 1.93) (p <0.001). The Neutrophils / Lymphocytes ratio may prove to be a diagnostic factor for COVID-19, an NLR> 3.68 revealed an OR 10.84 (95% CI = 6.47 - 18.13) (p <0.005). CONCLUSIONS: The value of NLR considered together with the age variable allows a risk stratification and allows the development of diagnostic and treatment protocols for patients affected by COVID-19. A high neutrophil to lymphocyte ratio suggests worse survival. Risk stratification and management help alleviate the shortage of medical resources and reduce the mortality of critically ill patients.

Autologous serum skin test reactivity and basophil histamine release test in patients with nasal polyposis: preliminary results.

An eosinophilic inflammatory process is generally observed in patients suffering from nasal polyposis (NP), however its onset has not yet been defined. It has been suggested that immune activation of inflammatory cells may be the cause. The aim of this study is to verify whether autoantibodies and/or histamine-releasing factors are present in the serum of patients suffering from NP. In fact, we assume that autoantibodies and/or histamine-releasing factors, as already demonstrated in chronic idiopathic urticaria and asthma, may be involved in the pathogenesis of NP. In this case-control analytical study 40 patients with NP and 27 control subjects underwent the in vivo autologous serum skin test (ASST). The sera from 6 patients suffering from NP and 9 control group subjects, who had all been previously studied and randomly selected, underwent basophil histamine release assay from normal donor as a pilot study. The ASST showed positive results in 55% of patients suffering from NP versus 8% of the control group (p= .00006), the basophil histamine release test (BHRT) turned out positive in all patients tested and in 11% of the control group. We found a weak positive correlation between the percentage of histamine release and the wheal diameter. ASST reactivity is very frequent in patients suffering from NP, thus suggesting the presence of histamine-releasing factors in the blood stream. The BHRT was positive in the serum of all patients, thus suggesting the presence of anti-FcepsilonRI, anti-IgE autoantibodies and/or other histamine-releasing factors, the presence of which can play a role in triggering and maintaining the eosinophilic inflammatory process in NP.

[Phlegmons of the neck: our experience with 7 cases]. / Flemmoni del collo: nostra esperienza su 7 casi clinici.

Phlegmons of the neck originate from infectious diseases of head and neck. Odontogeneous inflammation of the oral cavity is most frquently primary lesion, followed by sinusitis, otitis, as well as radiation therapy and surgical procedures. Phlegmons of head and neck can drain into the spaces among the muscles, aponevrosis, organs inside the neck like sublingual space, lateral pharyngeal space, retro-pharyngeal space or pre-vertebral space. We hereby report our experience with 7 patients treated from 2001 to 2005.

Radiotherapy alone for local tumour control in esthesioneuroblastoma.

Esthesioneuroblastoma is an uncommon tumour. Due to its low incidence, this neoplasm is difficult to evaluate and its treatment remains a matter of debate. Although the role of post-operative radiation is relatively well-defined, little is reported regarding the role of radiotherapy as the only treatment modality. A retrospective analysis of the literature has been conducted. With reference to the treatment of esthesioneuroblastoma, 55 patients submitted only to radiotherapy have been selected from publications of internationally indexed literature between 1979 and 2006. According to the Kadish classification, 6 patients were in stage A, 12 in stage B, and 37 in stage C. Response to therapy for each stage was assessed. There was no evidence of disease in: 6/6 stage A patients with a median follow-up period of 103.6 months, 7/12 stage B patients with a median followup period of 120 months, and 7/37 stage C patients with a median follow-up period of 77.3 months. A total of 27 patients died due to tumour-related causes and 5 due to intercurrent disease, while 3 patients were alive with disease (local recurrence and cervical lymph node metastasis). In conclusion, esthesioneuroblastoma is a malignant tumour which grows both locoregionally and distantly. For this reason, despite the satisfying results regarding response to radiotherapy alone in stage A patients, irradiation should be used only in early lesions arising below the cribriform plate, whereas all other cases require aggressive and multimodal therapy.

Quality of life measurements for patients with chronic suppurative otitis media: Italian adaptation of "Chronic Ear Survey".

The chronic ear survey (CES) is a sensitive and disease specific quality of life (QoL) measurement tool in patients with chronic suppurative otitis media (CSOM). It is a 13-item survey that evaluates the frequency, duration and severity of problems associated with this disease. It is composed of three subscales that describe activity restrictions, symptoms and medical resource utilisation. Based on patient's answers, it is possible to obtain a score resulting in a scale ranging from 0 to 100; the highest indicates the best health, while the lowest denotes poor health. The questionnaire was originally created in English. The aim of this study is to validate the CES questionnaire in Italian (CES-I). Translation was made following international guidelines. The application follows the stages of translation from English to Italian and linguistic adaptation, and grammatical and idiomatic equivalence review. The CES-I and the Short Form Health Survey 36 (SF-36) questionnaires were administered to 54 patients with CSOM. A cross-sectional design was used to examine the internal consistency (Cronbach's alpha) and concurrent validity (Pearson's product moment correlation). To confirm the external validity of CES-I, Pearson correlation coefficient, considering the total score and single subscales of CES and the 8 scales of the SF-36, was calculated. Cronbach's alpha coefficient for internal consistency was 0.737. The intraclass correlation coefficient, measured through mixed effects, was 0.737 (95% CI: 0.600-0.835, p < 0.001) for average measures and 0.412 (95%CI: 0.273-0.559, p < 0.001) for individual measures. According to our results, CES-I is a reliable tool for evaluation of QoL in patients with CSOM among the Italian-speaking population.

Sialendoscopic management of autoimmune sialadenitis: a review of literature.

Autoimmune diseases of major salivary glands include Sjögren's syndrome and a complex of disorders classified as immunoglobulin G4-related diseases. These pathologies are characterised by an autoimmune reaction mediated by T-helper lymphocytes that targets the ducts of exocrine glands in Sjögren's syndrome and glandular parenchyma in immunoglobulin G4-related diseases. Immunoglobulin G4-related diseases represent recently introduced multi-organ diseases that also involve the salivary glands. However, the morbid conditions once known as Mikulicz's disease and Kuttner's tumour were recently considered as two variants of immunoglobulin G4-related diseases affecting the major salivary glands ( immunoglobulin G4-related sialadenitis). This review briefly summarises the pathogenesis and clinical features of autoimmune diseases of the major salivary glands, focusing on the diagnostic and therapeutic role of sialendoscopy.

Pathological and cytological changes of the nasal mucosa in acute rhinosinusitis: the role of hyaluronic acid as supportive therapy.

OBJECTIVE: The aim of this study was to evaluate the reparative role of hyaluronic acid in acute rhinosinusitis (ARS). PATIENTS AND METHODS: 48 patients affected by ARS were submitted to nasal endoscopy, nasal cytology, mucociliary transport evaluation (MCTt) and visual analogue scale questionnaire (VAS) at T0, after 14-18 days (T1) and after 30-35 days (T2). The patients were randomized into two groups, A and B, and received Levofloxacin and Prednisone. Moreover, using a nebulizer ampoule for nasal douche, Group A received high molecular weight Sodium Hyaluronate (3%) plus saline solution (NaCl 0.9%) twice a day for 30 days; Group B received saline solution twice a day for 30 days. RESULTS: At T0 only the VAS score showed differences regarding nasal discharge and post-nasal drip. At T1, in Group A MCTt and the number of bacteria were significantly lower than in Group B. The VAS score showed improvement in Group A. At T2 in Group A, MCTt and number of neutrophils were significantly lower than in Group B. The VAS score showed statistically significant differences between the two groups regarding nasal discharge. CONCLUSIONS: In ARS patients sodium hyaluronate plus saline solution significantly improved symptoms, MCT time and reduced neutrophil count on nasal cytology.

Clinic manifestations in granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility.In Europe, the prevalence of GPA is five cases per 100,000 population, with greater incidence in Northern Europe. GPA can occur in all racial groups but predominantly affects Caucasians. Both sexes are affected equally. GPA affects a wide age range (age range, 8-99 years).Granulomatosis with polyangiitis is characterised by necrotising granulomatous lesions of the respiratory tract, vasculitis and glomerulonephritis. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose and throat (ENT); lungs; and kidneys. Because the upper respiratory tract is involved in 70-100% of cases of GPA, classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease. The nasal cavity and the paranasal sinuses are the most common sites of involvement in the head and neck area (85-100%), whereas otological disease is found in approximately 35% (range, 19-61%) of cases.Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis. The 10-year survival rate is estimated to be 40% when the kidneys are involved and 60-70% when there is no kidney involvement.The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide. In young patients, cyclophosphamide should be switched to azathioprine in the maintenance phase.A multidisciplinary approach, involving otorhinolaryngologists, oral and maxillofacial surgeons, oral physicians, rheumatologists, renal and respiratory physicians, and ophthalmologists, is necessary for the diagnosis and therapeutic treatment of GPA. ENT physicians have a determining role in recognising the early onset of the disease and starting an appropriate therapy.

Qualitative mucin disorders in patients with primary Sjögren's syndrome: a literature review

BACKGROUND: It is a common opinion that Primary Sjögren Syndrome (pSS) damages the exocrine glands and determines the reduction of secreted saliva, some studies show that there are qualitative anomalies of the mucins produced in saliva, including MUC7, MUC5B, MUC1. The purpose of this study is to trace all the information useful to establish whether there is a qualitative or quantitative defect of the mucins in the pSS. MATERIAL AND METHODS: We reviewed the literature by looking for publications relevant to the topic in electronic databases. Sixteen articles met the search criteria. The studies were divided into two categories, those that studied the rheological characteristics of the saliva and those that studied the structural and / or metabolism modifications of the muciparous cells in the salivary glands. RESULTS: in Patients with pSS, xerostomia and the reduction of salivary spinnbarkeit are only partially related to the reduction of the unstimulated salivary flow. In pSS, pathological alterations of mucins' chemical-physical properties prevail as a cause of the clinical characteristics. Moreover, in pSS there are structural and metabolism changes in salivary glands' muciparous cells. CONCLUSIONS: There is much evidence that supports the presence of qualitative alterations in the saliva's rheological properties in Patients with pSS, and these are the main cause, more than the reduction of the unstimulated salivary flow, of the disease clinical characteristics - dry mouth and complications in the oral cavity. Therefore we propose to add to the classification criteria of pSS also a qualitative test of salivary glycoproteins

No disponible

Outcomes of interventional sialendoscopy for obstructive salivary gland disorders: an Italian multicentre study.

Interventional sialendoscopy has become the predominant therapeutic procedure for the management of obstructive salivary disorders, but only a few multicentre studies of large series of patients with a long-term follow-up have been published. This Italian multicentre study involved 1152 patients (553 females; mean age 50 years) who, after at least a clinical and ultrasonographic evaluation, underwent a total of 1342 diagnostic and interventional sialendoscopies, 44.6% of which involved the parotid gland. 12% (n = 138) of patients underwent multiple treatments. The procedure was successful in 1309 cases. In 33 cases (2.4%) the procedure could not be concluded mainly because of complete duct stenosis (21 cases). Salivary stones were the main cause of obstruction (55%), followed by ductal stenosis and anomalies (16%), mucous plugs (14.5%) and sialodochitis (4.7%). Complete therapeutic success was obtained in 92.5% of patients after one or more procedures, and was ineffective in < 8%. Untoward effects (peri and postoperative complications) were observed in 5.4% of cases. Sialendoscopy proved to be an effective, valid and safe procedure in the diagnostic and therapeutic management of non-neoplastic obstructive salivary gland diseases.

Anti-cyclic citrullinated peptide antibodies in type 1 autoimmune hepatitis.

BACKGROUND: Besides the autoantibodies included in the diagnostic criteria of type 1 autoimmune hepatitis, many other autoantibodies have been described in this condition. Recently, antibodies against cyclic citrullinated peptide have been validated as specific diagnostic and prognostic markers of rheumatoid arthritis. AIM: To assess whether these antibodies are part of the autoantibody repertoire of type 1 autoimmune hepatitis and correlate with rheumatological manifestations. METHODS: Antibodies against cyclic citrullinated peptide were tested by a commercially available enzyme-linked immunosorbent assay. RESULTS: The antibodies were found in 12 of 133 (9%) type 1 autoimmune hepatitis, two of 49 (4%) with primary biliary cirrhosis, one of 80 (1%) with hepatitis C virus-related chronic liver disease and 53 of 89 (60%) with rheumatoid arthritis serum samples. High titres were found only in rheumatoid arthritis and type 1 autoimmune hepatitis. No clinical (in particular rheumatological manifestations), biochemical or immunoserological differences were detectable between antibodies against cyclic citrullinated peptide positive and negative type 1 autoimmune hepatitis sera, with the exception of rheumatoid factor, always negative in the positive ones. CONCLUSIONS: Antibodies against cyclic citrullinated peptide can be detected in a subgroup of patients with type 1 autoimmune hepatitis. They might be part of the wide range of autoantibody production characteristic of this condition and/or, less probably, be predictive of future rheumatoid arthritis development.

Sialoendoscopy: state of the art, challenges and further perspectives. Round Table, 101(st) SIO National Congress, Catania 2014.

This draft of the Official Round Table held during the 101(st) SIO National Congress is an updated review on sialoendoscopy, a technique used for diagnosis and treatment of obstructive pathologies of salivary glands in a minimally invasive fashion. This review treats many aspects of salivary gland endoscopy, starting from anatomy to deal with the more advanced surgical techniques and analyses the main decisional algorithms proposed in the literature. In addition, particular attention was directed to the current limitations of this technique and to the potential developments that sialoendoscopy could have in the near future.

Counterimmunoelectrophoresis (CIE) for the detection of anti-liver-kidney microsome (LKM) antibodies in the sera of patients with chronic liver disease.

A counterimmunoelectrophoresis (CIE) test for the detection of liver-kidney microsome specific antibodies in human sera is described. By testing different subcellular preparations the LKM antigen was found in the membranes of the smooth endoplasmic reticulum subfraction. The antigen was sensitive to trypsin digestion and behaved as an anionic protein in the experimental conditions used in the test. All sera positive for LKM in immunofluorescence gave a precipitin line of identity while none of the control sera gave a positive reaction. The CIE titers ranged between neat and 1/4096. A significant correlation was observed between the LKM titers obtained in immunofluorescence and those obtained in CIE. Moreover, by absorption experiments, it was concluded that the antigen preparation reactive in CIE was able to abolish the immunofluorescence pattern of LKM positive sera on rat liver and kidney sections. The LKM target antigen, although previously considered a structural protein of microsomal membranes, was shown to solubilize spontaneously during the isolation of microsomal membranes. Counterimmunoelectrophoresis appears to be an appropriate test for anti-LKM antibodies in human sera.

Anti-actin antibodies: a new test for an old problem.

Smooth muscle antibodies with anti-actin specificity are commonly regarded as markers of autoimmune liver disease. However, there are interpretational problems because different techniques have been used for their identification and therefore the results are difficult to compare. The present paper reports the results of a new method for the identification of anti-actin antibodies (indirect immunofluorescence on cryostat sections of liver from rats chronically injected with phalloidin). The results have been compared with those obtained by four other techniques: demonstration by immunofluorescence of kidney peritubular reactivity (SMAT), of anti-microfilament antibodies (on HEp-2 cells and vinblastine-treated peripheral blood mononuclear cells) and counterimmunoelectrophoresis with purified muscle actin as antigen. The new method proved to be the most sensitive and specific. Furthermore, its reproducibility was found to be high, the interpretation easy and the cost low. The clinical significance of anti-actin antibodies in patients with chronic liver disease is also discussed.

Ultrasonography detection of early bone erosions in the metacarpophalangeal joints of patients with rheumatoid arthritis.

OBJECTIVE: To compare ultrasonography (US) and magnetic resonance imaging (MRI) in their capability to detect bone erosions in early-advanced rheumatoid arthritis, where no erosion was evident on conventional radiography (X-ray). METHODS: Metacarpophalangeal (MCP), radiocarpal and ulnocarpal joints of 13 patients with rheumatoid arthritis, with bone erosion that was not detected by conventional X-ray, were examined by US and MRI. Ten controls underwent examination of the same joints by US. RESULTS: None of the controls showed bone erosions at US examination. No significant difference between US and MRI in detecting bone erosion was observed in wrist joints, whereas a significantly higher number of erosions was detected by US in MCP joints. CONCLUSION: US is at least as sensitive as MRI in detecting bone erosions in MCP and wrist joints. Since US examination is a more easily available and less expensive procedure than MRI, our findings justify its use as a diagnostic tool for early arthritis. In addition US may also be utilized in the follow up of patients with an established diagnosis of inflammatory arthritis.