RESUMEN
Although tonsillectomy are used as therapeutic options to prevent chronic renal failure in IgA nephropathy (IgAN) patients, the relationship between IgAN and tonsils is not fully proved by basic research. Recently, circulating CX3CR1-positive cells were reportedly involved in promoting hematuria in patients with IgAN. In this study, we focused on the expression of CX3CR1 in tonsillar mononuclear cells in IgAN patients. Immunohistological analysis revealed greater distribution of CX3CR1-positive cells in the inter-follicular area of tonsils in IgAN patients than in non-IgAN patients. CX3CR1-positive cells were also found in the affected renal glomerulus of IgAN patients. Flow cytometric analysis revealed the expression of CX3CR1 on tonsillar CD8-positive cells to be significantly higher in IgAN patients. CpG-oligodeoxynucleotides enhanced the expression in IgAN patients. The chemotactic response of tonsillar mononuclear cells to fractalkine was significantly higher in IgAN patients. Expression of CX3CR1 on peripheral blood CD8-positive cells in IgAN patients was significantly higher, and decreased after tonsillectomy, along with the disappearance of hematuria. These results suggest that hyper-immune response to microbial DNA enhanced the expression of CX3CR1 on tonsillar CD8-positive cells in IgAN patients, followed by the migration of the cells to renal lesions via blood circulation, resulting in the development of hematuria.
Asunto(s)
Linfocitos T CD8-positivos/fisiología , Glomerulonefritis por IGA/diagnóstico , Hematuria/diagnóstico , Tonsila Palatina/patología , Receptores de Quimiocina/metabolismo , Adolescente , Adulto , Receptor 1 de Quimiocinas CX3C , Quimiocina CX3CL1/inmunología , Femenino , Glomerulonefritis por IGA/inmunología , Hematuria/inmunología , Humanos , Masculino , Persona de Mediana Edad , Receptores de Quimiocina/genética , Tonsilectomía , Regulación hacia Arriba , Adulto JovenRESUMEN
Congenital cholesteatoma may originate at various sites in the temporal bone. Congenital cholesteatoma of the mastoid origin shows a variable clinical presentation, although the least common site is the mastoid process. We report an extremely rare case of congenital cholesteatoma isolated to the mastoid presenting as stricture of the external auditory canal. A 10-year-old boy presented with stricture of the left-sided external auditory canal caused by bulging of the posterior wall of the external auditory canal. Computed tomography showed destruction of the posterior wall of the external auditory canal by a lesion of soft tissue density in the left mastoid cells. At surgery, cholesteatoma was observed in the mastoid cavity. Although destruction of the posterior wall of the external auditory canal was identified, the external auditory canal skin and tympanic membrane were intact, and the aditus ad antrum mucosa was normal. Congenital cholesteatoma isolated to the mastoid was diagnosed. Diagnosis of congenital cholesteatoma isolated to the mastoid should be based on clinical examination, radiological evaluation, and surgical findings. In addition, the possibility of congenital cholesteatoma isolated to the mastoid should be considered in patients with stricture of the external auditory canal.