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BACKGROUND Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited blistering skin disorder that is associated with significant esophageal strictures, resulting in dysphagia and nutritional failure. Although endoscopically guided balloon dilatation is a widely used treatment, the use of an endoscope carries the risk of oropharyngeal trauma. To(More)
OBJECTIVES/HYPOTHESIS To describe the clinical phenotype of pediatric patients with unilateral enlarged vestibular aqueduct (EVA) and then to compare the findings to two clinically related phenotypes: bilateral EVA and unilateral hearing loss without EVA. In view of clinical observations and previously published data, we hypothesized that patients with(More)
OBJECTIVE Mutations in the 12S rRNA gene have been associated with aminoglycoside-induced ototoxicity. Our objective was to study the relationship of these mutations in neonates, duration of aminoglycoside exposure, and other known risk factors to the presence of hearing loss. STUDY DESIGN Prospective case-cohort study. SETTING Three neonatal intensive(More)
Chronic pulmonary aspiration (CPA) in children is an important cause of recurrent pneumonia, progressive lung injury, respiratory disability and death. It is sporadic, intermittent and variable, and often occurs in children with complicated underlying medical conditions and syndromes that produce symptoms indistinguishable from CPA. For most types of(More)
OBJECTIVES To determine the prevalence of obstructive sleep apnea (OSA) in extremely overweight adolescents and to examine the effect of significant weight loss on OSA severity. RESEARCH METHODS AND PROCEDURES We reviewed the anthropometric and polysomnographic data on all extremely overweight adolescents who underwent laparoscopic Roux en Y gastric(More)
PURPOSE OF REVIEW To discuss the current status of endoscopic airway surgery in children. RECENT FINDINGS More refined endoscopic instruments have been introduced, including balloon dilators, powered debriders, lasers with more exact modes of delivery, and innovative suspension laryngoscopes. The use of balloon dilatation for primary management of(More)
During the first year of life, infants spend most of their time in the sleeping state. Assessment of sleep during infancy presents an opportunity to study the impact of sleep on the maturation of the central nervous system (CNS), overall functioning, and future cognitive, psychomotor, and temperament development. To assess what is currently known regarding(More)
BACKGROUND Although surgery has been the mainstay of treatment of chondrogladiolar pectus carinatum (PC), several authors have advocated the benefits of nonoperative approaches to induce chest wall remodeling. Based on our initial success with compression bracing, we have integrated this modality into our treatment algorithm. METHOD We reviewed the charts(More)
LEARNING OBJECTIVES The purpose of this review is to highlight fundamental aspects of obstructive sleep apnea (OSA), and to present an overview of the medical literature that pertains to the clinical interplay between various allergy-related disorders, nasal patency, and OSA. This should enable the reader to play a more proactive role in the diagnosis,(More)