RESUMO
OBJECTIVE: To determine the response to treatment of pediatric patients diagnosed with autoimmune inner ear disease. PATIENTS: Seven children who presented with sensorineural hearing loss and were diagnosed with autoimmune inner ear disease. INTERVENTION: Diagnosis through blood testing. Treatment with steroids and/or cytotoxic medication. MAIN OUTCOME MEASURES: Improvement in pure-tone average and speech discrimination scores on audiometric testing. RESULTS: Six of the 7 children (85.7%) improved with treatment, and the remaining patient had no measurable progression of disease. CONCLUSION: Children with autoimmune inner ear disease seem to benefit from treatment with steroids and/or cytotoxic medication. Although such medications must be used with caution in the pediatric population, they should not be withheld simply because of young age.
Assuntos
Doenças Autoimunes/imunologia , Perda Auditiva Neurossensorial/imunologia , Doenças do Labirinto/imunologia , Adolescente , Anti-Inflamatórios/uso terapêutico , Antimetabólitos Antineoplásicos/uso terapêutico , Antineoplásicos/uso terapêutico , Antineoplásicos Alquilantes/uso terapêutico , Limiar Auditivo , Western Blotting , Condução Óssea , Criança , Pré-Escolar , Ciclofosfamida/uso terapêutico , Feminino , Antígeno HLA-DR4/imunologia , Testes Hematológicos , Humanos , Masculino , Metotrexato/uso terapêutico , Prednisona/uso terapêutico , Estudos Retrospectivos , Percepção da Fala , Esteroides/uso terapêuticoRESUMO
Intracellular Hsp70 provides cytoprotection against a variety of stressful stimuli, and an effective means of increasing intracellular Hsp70 levels could prove beneficial in the prevention and treatment of a variety of human diseases. A novel protein transduction domain consisting of the single chain Fv fragment of an anti-DNA antibody known to penetrate into living cells and tissues, mAb 3E10, has recently been used to deliver functional proteins to cells. The ability of the single chain Fv fragment to deliver Hsp70 into living cells was tested by generating an Fv-Hsp70 fusion protein. Fv-Hsp70 was produced as a secreted protein in both COS-7 cells and the methylotropic yeast strain Pichia pastoris and was shown capable of penetrating into COS-7 cells and primary rat cortical neurons. Pre-treatment with Fv-Hsp70 protected both COS-7 cells and primary rat cortical neurons against subsequent exposure to hydrogen peroxide. These results provide the first evidence that the Fv fragment of mAb 3E10 is capable of delivering proteins to neurons and indicate its potential in the development of Hsp70 protein therapy.