ABSTRACT
Ciliary dyskinesia syndrome or immotile cilia syndrome, is a congenital defect in the ultrastructure of cilia, which result in a clinical expression diverse: recurrent respiratory infections, recurrent otitis media, infertility in the adult male, and half of those situs inversus. A case of ciliary dyskinesia syndrome is presented, who suffered of recurrent pneumonia and relapsing secretory otitis media.
Subject(s)
Ciliary Motility Disorders/complications , Otitis Media with Effusion/etiology , Adenoidectomy , Child , Cilia/ultrastructure , Ciliary Motility Disorders/diagnosis , Ciliary Motility Disorders/pathology , Dyneins/deficiency , Hearing Loss, Bilateral/etiology , Hearing Loss, Conductive/etiology , Humans , Male , Middle Ear Ventilation , Otitis Media with Effusion/surgery , Pulmonary Fibrosis/etiology , Recurrence , Respiratory Tract Infections/etiologyABSTRACT
The paramount features of the multiple nevoid basal Carcinoma, the so-called Gorlin's syndrome, are the presence of multiple maxillary cysts (odontogenic keratocysts), several nevoid basal cells carcinomata and some skeletal anomalies. Since its first description, due to Jarish (1894), at least about 250 cases have been published. The odontogenic keratocysts present themselves with ENT symptoms, specially when arising on the upper maxillary bone or on the homonymous sinus. Owing to this reason, the AA. of the paper, recall the clinic aspects of the condition.
