Salvatore Martellucci

Goodpasture's syndrome: A clinical update

Goodpastures syndrome (GS) is a rare and organ-specific autoimmune disease that is mediated by anti-glomerular basement membrane (anti-GBM) antibodies and has pathology characterized by crescentic glomerulonephritis with linear immunofluorescent staining for IgG on the GBM. It typically presents as acute renal failure caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary hemorrhage that may be life-threatening. It was first described as a distinctive syndrome by Pasture in 1919. Autoimmune Inner Ear Disease (AIED) may be associated. The etiology of GS is unknown. Researchers hypothesized a genetic predisposition HLA-associated. Complex immunological mechanisms are in the pathogenesis. The disease is caused by autoantibodies against the NC1 domain of the alpha 3 chain of type IV collagen. The limited presence of this molecule in the body explains the interest confined to specific target organs, such as the lung and kidney. It occurs when the immune system attacks the walls of the lungs and the tiny filtering units in the kidneys. Without prompt diagnosis and treatment, the disease can lead to bleeding in the lungs, kidney failure, and even death.

Kawasaki disease: An evolving paradigm

Kawasaki disease (KD) is a self-limited childhood systemic vasculitis that exhibits a specific predilection for the coronary arteries. KD predominantly affects young children between the ages of 6months and 4years. Incidence rates in Asians are up to 20 times higher than Caucasians. The aetiology of KD is not known. One reasonable open hypothesis is that KD is caused by an infectious agent that produces an autoimmune disease only in genetically predisposed individuals. The typical presentation of KD is a young child who has exhibited a high swinging fever for five or more days that persists despite antibiotic and/or antipyretic treatment. The lips are dry and cracked. There is a characteristic strawberry tongue, and a diffuse erythema of oropharyngeal mucosal surfaces. Lymphadenopathy is usually unilateral and confined to the anterior cervical triangle. Coronary aneurysms generally appear during the convalescence phase (beginning during the second week). The absence of any laboratory tests for KD means that the diagnosis is made by the presence of a constellation of clinical features. The aim of echocardiography is to assess the presence of coronary artery dilatation or aneurysm formation. Effective therapies exist for most patients with acute KD, but the exact mechanisms of action are not clear. Treatment with aspirin and intravenous immunoglobulins (IVIG) are first-line therapies. However, options are plentiful for the children who fail this treatment, but these treatments are not as beneficial. Some centres attempt to salvage resistant patients using intravenous pulsed doses of methylprednisolone. Other centres use infliximab or combinations of these approaches.

Total cricoidectomy in the treatment of laryngeal chondrosarcomas.

Our goal was to describe a total cricoidectomy, a laryngeal‐preserving procedure for the treatment of low‐grade chondrosarcomas of the larynx. These extremely rare cartilaginous tumors arise in the cricoid cartilage in most cases. Although these are slow‐growing and rarely metastasizing tumors, large chondrosarcomas of cricoid cartilage are generally treated with total laryngectomy. An oncologically radical but function‐preserving approach would therefore be preferable.

Cytologic and Functional Alterations of Nasal Mucosa in Smokers Temporary or Permanent Damage

Objective Tobacco smoke is a significant risk factor for respiratory diseases. The purpose of this study is to analyze the cytologic and functional features of nasal mucosa in smokers, nonsmokers, and ex-smokers to evaluate if nasal alterations in smokers are permanent or reversible conditions after smoking cessation. Study Design Case series with planned data collection. Setting University medical center. Subjects and Methods Ninety healthy volunteers recruited from the staff of Alfredo Fiorini Hospital, Sapienza University of Rome, were enrolled in this prospective study from October to November 2013. We divided the cases according to smoking habits (smokers, nonsmokers, ex-smokers). Each group was composed of 30 subjects. Cytologic features of nasal mucosa and effectiveness of nasal mucociliary clearance were studied, focusing on 4 parameters: (1) nasal mucociliary clearance, assessed by saccharin nasal transit time; (2) ratio between the number of ciliated cells and goblet cells, analyzed by microscopic observation of cytologic specimens of nasal mucosa that had undergone May Grunwald Giemsa staining; (3) evaluation of ciliary motility; and (4) time of ciliary movement of ciliated cells analyzed by phase-contrast microscopy. Results All parameters were significantly reduced in the smokers compared to the nonsmokers. There were no statistically significant differences between the nonsmoker and ex-smoker groups. Conclusion Cigarette smoking causes cytologic modifications of nasal mucosa that influence the effectiveness of mucociliary clearance. Our preliminary study suggests that these changes are not permanent and that nasal mucosa of ex-smokers recovers normal cytologic and functional features.

Ho:Yag Laser for Sialolithiasis of Wharton’s Duct

Objective To assess the feasibility of intracorporeal lithotripsy with holmium YAG laser under sialoendoscopic guidance for sialolithiasis of Wharton’s duct. Study Design Case series with planned data collection. Setting Tertiary referral university hospital. Methods This study was conducted on 16 patients with sialolithiasis of Wharton’s duct. Diagnosis was confirmed at ultrasound examination. Patients with stones ranging from 5 to 8 mm in diameter were enrolled in the study. The selected patients underwent intracorporeal lithotripsy with holmium Yag laser under endoscopic control. Debris was removed using sialoendoscopic forceps or a wire basket during the same procedure. After a 3-month follow-up, radiological tests were rerun. Results Stone fragmentation was possible in all cases. All patients experienced a regular postoperative course. Postoperative ultrasound examinations revealed residual stones in 3 patients, 1 of whom was asymptomatic. Three patients complained of residual symptoms after 3 months of follow-up. These patients were treated successfully during a second sialoendoscopic procedure. Conclusions In our experience, endoscopic laser lithotripsy was proved to be a feasible technique for Wharton’s duct lithiasis in clinical practice.

Endoscopic treatment of benign and malignant strictures of the cervical esophagus and hypopharynx.

Objectives: We evaluated the efficacy of endoscopic techniques employed in the management of cervical esophageal and hypopharyngeal strictures. Methods: A series of 45 patients with cervical esophageal (35) and/or hypopharyngeal strictures (10) were included. Twenty-five patients (55.6%) with neoplastic strictures were treated for palliation alone. The stenosis was related to radiotherapy in 11 patients (24.4%) and to postsurgical complications in 9 (20%). A group of 23 patients was treated with dilation alone (group 1). A second group included 22 patients treated with insertion of a self-expandable stent after failure of dilation treatment (group 2). The swallowing test data, clinical notes, and surgical reports were reviewed. Results: All of the patients showed some degree of relief of dysphagia. In group 1, 19 of the 23 patients required multiple dilation treatments to maintain normal deglutition. In group 2, 7 of the 22 patients recovered regular oral feeding after stent placement, 10 patients reported pain and foreign body sensation, 2 patients reported pain so severe that stent removal was required, and 3 patients experienced stent migration. All but 3 of the 25 patients with inoperable tumors died during follow-up, but no patients with benign stenosis died. Conclusions: The two groups showed comparable functional results. Dilation often requires multiple procedures, but is usually well tolerated. Placement of self-expandable stents is effective, but is generally less well tolerated.

Features of Residual Dizziness after Canalith Repositioning Procedures for Benign Paroxysmal Positional Vertigo

Objectives To assess factors related to residual dizziness (RD) in patients who underwent successful canalith repositioning procedures (CRPs) for benign paroxysmal positional vertigo (BPPV). Study Design Prospective cohort study. Setting Academic center. Subjects and Methods Ninety-seven consecutive patients with BPPV of the posterior semicircular canal were initially enrolled. Diagnosis was assessed according to clinical history and bedside evaluation. All patients were treated with CRPs until nystagmus disappeared. Three days after the successful treatment, presence of RD was investigated. If RD was present, patients were monitored every 3 days until the symptoms disappeared. Subjects who required ≥4 CRPs or who failed to meet the follow-up visit were excluded. The Dizziness Handicap Inventory (DHI) was obtained from patients at the time of diagnosis and at every subsequent visit. Results At the end of selection, 86 patients were included; 33 (38.36%) reported RD after successful treatment. A significant difference in the incidence of RD was observed in consideration of the age of the subjects (P = .0003) and the DHI score at the time of diagnosis (P < .001). A logistic regression analysis showed that the probability of RD occurrence increased with the increase of the emotional subdomain score of the DHI questionnaire. Conclusion RD is a common self-limited disorder, more frequent in the elderly, which may occur after the physical treatment for BPPV. The DHI score at the time of BPPV diagnosis represents a useful tool to quantify the impact of this vestibular disorder on the quality of life and to estimate the risk of RD after CRPs.

Wharton’s Duct Repair after Combined Sialolithectomy Is Ductoplasty Necessary?

The aim of this prospective study is to establish whether ductoplasty is necessary to repair the Wharton’s duct after a combined endoscopic and external approach to remove large submandibular gland stones or if simple suturing of the oral floor mucosa after stent placement may be sufficient to achieve proper duct healing. Ten patients were enrolled. Ductoplasty was performed in 6 cases, whereas in 4 cases, only the mucosa of the oral floor was sutured. The stent was removed about 3 weeks after surgery. Sialoliths were completely removed in all cases. During follow-up, patient symptom evaluation, ultrasound, and physical examination showed no signs of recurrence or complications. In our limited experience, ductoplasty after the combined approach was not necessary. After stent placement, the mere suture of the oral mucosa overlying the damaged ductal wall allowed a correct recanalization of the ductal lumen as commonly observed after careful ductoplasty.

Role of Human Papillomavirus in the Pathogenesis of Laryngeal Dysplasia

Objective A synergistic effect between smoking and alcohol intake is the major cause of premalignant and malignant lesions of the larynx, but the risk factors and pathogenesis of the neoplastic transformation in nonsmokers remain poorly defined. The aim of this retrospective study is to establish the relationship between smoking habits and human papillomavirus (HPV) infection in laryngeal dysplasia. Study Design Cross-sectional study. Setting Academic university hospital. Subjects and Methods HPV DNA was amplified from 30 paraffin-embedded laryngeal dysplasia tissue specimens by the polymerase chain reaction using 2 groups of different consensus primers (MYO9/MY11 and LCRF1-4, E7R1-4). Fifteen samples were taken from smokers and 15 from nonsmokers. Results The present investigation failed to demonstrate the HPV genome in all samples of laryngeal precancerous lesions, whereas HPV was detected in 4 laryngeal papilloma samples used as control to confirm the reliability of our method on paraffin-embedded samples. Conclusions Although the small number of cases in our series limits the power of our statistical analysis, the absence of viral genomes in the specimens analyzed in this study suggests the lack of a relationship between HPV infection and laryngeal dysplasia in smokers as well as in nonsmokers.

Risultati della scialoendoscopia interventistica nelle patologie ostruttive delle ghiandole salivari: Uno studio multicentrico Italiano

SUMMARY 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.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.