Giulio Pagliuca

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.

Swallowing ability and chronic aspiration after supracricoid partial laryngectomy

Objective: Sporadic episodes of aspiration may occur after supracricoid partial laryngectomy. To prevent risks of pulmonary consequences, a limitation of oral intake of food or a “nothing by mouth” regimen has been suggested. The objective of this study was to evaluate the long-term swallowing and pulmonary status of patients after supracricoid partial laryngectomy. Study Design: Case series with chart review. Setting: Tertiary University Hospital Policlinico “Umberto I” and Santa Lucia Foundation, Rome, Italy. Subjects and Methods: The swallowing status of a selected group of 116 patients who recovered functional deglutition after supracricoid partial laryngectomy was analyzed during follow-up consultation. Swallowing evaluation included clinical observation, fiberoptic endoscopic evaluation of swallowing, and a videofluoroscopy. When aspiration was confirmed by videofluoroscopy, a high-resolution computed tomography (CT) of the chest was performed in order to assess the radiological manifestations of aspiration. A group of 45 patients with chronic obstructive pulmonary disease and normal deglutition was used as a control. Results: Seventy-nine patients out of 116 showed a fiberoptic endoscopic evaluation of swallowing suggestive for aspiration; only 45 patients had aspiration confirmed by videofluoroscopy. No significant differences in radiological findings were noted in the patients affected by postoperative chronic aspiration compared to the control group. Conclusion: Patients with functional deglutition after supracricoid partial laryngectomy show a mild and well-tolerated degree of chronic aspiration and do not require a limitation of oral intake of food.

Detection of Human Papillomavirus and Adenovirus in Benign and Malignant Lesions of the Larynx

OBJECTIVE: To investigate the role of human papillomavirus (HPV) and adenovirus (AdV) infections in the oncogenesis of squamous cell carcinomas of the larynx and of laryngeal dysplasia. STUDY DESIGN: Cross-sectional study with planned data collection. SETTING: Department of Otorhinolaryngology G. Ferreri and Department of Experimental Medicine and Pathology-Section of Virology, Sapienza University of Rome. SUBJECTS AND METHODS: Biopsy samples were taken from 68 patients with benign and malignant lesions of the larynx. All tissue samples were analyzed by means of polymerase chain reaction with two groups of primers for HPV and with a pair of primers for AdV. RESULTS: All cases of carcinomas and dysplasia as well as all control cases were negative for both viruses. Four of the five cases of laryngeal papillomas were positive for only HPV, confirming the role of these viral types in the origin of papillomas. CONCLUSION: The absence of viral genomes in laryngeal carcinomas as in the other cases studied suggests the existence of other factors that play a more important role than viral infection in the carcinogenesis of these lesions.

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.

Oncologic results of the surgical salvage of recurrent laryngeal squamous cell carcinoma in a multicentric retrospective series: Emerging role of supracricoid partial laryngectomy

Several studies in the last decade evaluated conservative surgical procedures and, in particular, supracricoid operations as an alternative to total laryngectomy for the salvage of recurrences of laryngeal squamous cell carcinoma (SCC) after a first attempt of organ preservation.

Olfactory identification deficits and associated response inhibition in obsessive-compulsive disorder: On the scent of the orbitofronto-striatal model

Olfactory identification ability implicates the integrity of the orbitofrontal cortex (OFC). The fronto-striatal circuits including the OFC have been involved in the neuropathology of Obsessive Compulsive Disorder (OCD). However, only a few studies have examined olfactory function in patients with OCD. The Brief Smell Identification Test (B-SIT) and tests from the Cambridge Neuropsychological Automated Battery (CANTAB) were administered to 25 patients with OCD and to 21 healthy matched controls. OCD patients showed a significant impairment in olfactory identification ability as well as widely distributed cognitive deficits in visual memory, executive functions, attention, and response inhibition. The degree of behavioural impairment on motor impulsivity (prolonged response inhibition Stop-Signal Reaction Time) strongly correlated with the B-SIT score. Our study is the first to indicate a shared OFC pathological neural substrate underlying olfactory identification impairment, impulsivity, and OCD. Deficits in visual memory, executive functions and attention further indicate that regions outside of the orbitofronto-striatal loop may be involved in this disorder. Such results may help delineate the clinical complexity of OCD and support more targeted investigations and interventions. In this regard, research on the potential diagnostic utility of olfactory identification deficits in the assessment of OCD would certainly be useful.

Oncological results of the surgical salvage of recurrent laryngeal SCC in a multicentric retrospective series. The emerging role of supracricoid partial laryngectomy

Several studies in the last decade evaluated conservative surgical procedures and, in particular, supracricoid operations as an alternative to total laryngectomy for the salvage of recurrences of laryngeal squamous cell carcinoma (SCC) after a first attempt of organ preservation.

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

Clin. Otolaryngol. 2012, 37, 376–381

Severe obstructive sleep apnoea syndrome and erectile dysfunction: a prospective randomised study to compare sildenafil vs. nasal continuous positive airway pressure

A high incidence of erectile dysfunction (ED) among patients with obstructive sleep apnoea syndrome (OSAS) has been reported, with a strong correlation between obstructive sleep apnoea, ED, and quality of life (QOL), and it has been estimated that 10–60% of patients with OSAS suffer from ED. In this prospective randomised controlled trial, we investigated 82 men with ED consecutively who were referred to the outpatient clinic for sleep disorders and had severe OSAS (AHI> 30 events/h) without any other comorbidities as a possible cause of ED. The aim of this study was to evaluate and compare the efficacy of sildenafil vs. continuous positive airway pressure (CPAP) in men with ED and severe OSAS.