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Background Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course. Methods A convenience cohort of 339 affected subjects with papillomas positive for only HPV6 or HPV11 and clinical course data available for 1 year or more, from a large multicenter international study were included. Exploratory data analysis was conducted followed by inferential analyses with frequentist and Bayesian statistics. Results We examined 339 subjects: 82% were diagnosed prior to the age of 18 years, 65% were infected with HPV6, and 69% had an aggressive clinical course. When comparing age at diagnosis with clinical course, the probability of aggressiveness is high for children under five years of age then drops rapidly. For patients diagnosed after the age of 10 years, an indolent course is more common. After accounting for confounding between HPV11 and young age, HPV type was minimally associated with aggressiveness. Fast and Frugal Trees (FFTs) were utilized to determine which algorithms yield the highest accuracy to classify patients as having an indolent or aggressive clinical course and consistently created a branch for diagnostic age at ~5 years old. There was no reliable strong association between clinical course and socioeconomic or parental factors. Conclusion In the largest cohort of its type, we have identified a critical age at diagnosis which demarcates a more aggressive from less aggressive clinical course.

By means of differential display reverse-transcriptase polymerase chain reaction, increased expression of the mRNA encoding the anti-apoptosis gene IEX-1L was found in respiratory epithelial cells infected with respiratory syncytial virus (RSV). IEX-1L mRNA expression increased 5-7-fold in RSV-infected cells at 72 h after infection but remained unchanged in cells exposed to irradiated, replication-incompetent RSV. Because IEX-1L is reported to protect cells from apoptosis induced by tumor necrosis factor (TNF)-alpha, the effect of TNF-alpha on epithelial cell apoptosis in the context of RSV infection was determined. Epithelial cells were exposed to vehicle, RSV, or irradiated RSV for 72 h, and then TNF-alpha was added to appropriate cultures. Cytochemical staining of cellular DNA with 4,6-diamidino-2-phenylindole demonstrated TNF-alpha-induced apoptosis in 23.4% of control cells but only 5% of RSV-infected cells. These data show that RSV infection protects epithelial cells from TNF-alpha-induced apoptosis and that this effect is temporally associated with IEX-1L gene expression.

Experience with laryngotracheal reconstruction (LTR) has resulted in and continues to yield modifications and refinements in approach and technique with the goal to restore and maintain total laryngeal function. In addition to airway obstruction, the laryngeal functions of phonation and swallowing also may be affected by the underlying injury as well as by procedures designed to enlarge the airway. This paper discusses various problems encountered with phonation and swallowing in pediatric patients who underwent LTR and postoperative patients who were seen during the year July 1, 1990, through June 30, 1991. Phonation problems became apparent as long-term difficulties that persisted after tracheotomy decannulation. Swallowing was frequently a short-term perioperative problem while a stent was in place following LTR. The approaches and techniques that have been employed to treat, minimize, and prevent these problems are discussed.

Hemangiomas are the most common tumor of infancy, and the vast majority occur in the head and neck region. In children, laryngeal hemangiomas typically occur below the level of the true vocal folds, in the region of the subglottis, and other sites are exceedingly rare. We present four cases of hemangiomas located in the postcricoid region of the hypopharynx. Because of the location of these lesions, children may present with obstructive symptoms such as dysphagia, intermittent aspiration, hypersalivation, or recurrent respiratory infections. Clinical diagnosis is relatively easily made with flexible laryngoscopy, as the lesions have a propensity to enlarge with crying or straining. When these patients are examined under general anesthesia in a relaxed state, however, the lesions are typically much smaller, and can even go unnoticed. Unlike other reported cases, the postcricoid hemangiomas in our patients were not causing any symptoms and were simply incidental findings. Thus, we believe that the true incidence of postcricoid hemangiomas is likely higher than reports suggest. To our knowledge, we report the longest follow-up (6 years) of a patient with a postcricoid hemangioma and are the first to describe the natural course of such a lesion.