RESUMO
Recurrent respiratory papillomatosis is the most common neoplasm of the larynx in childhood. Extension into lung parenchyma occurs in less than 1% of patients and has a low risk of malignant transformation. Treatment options for intrapulmonary spread have shown limited success. We describe a case of recurrent respiratory papillomatosis with extensive parenchymal involvement and adenosquamous carcinoma in a 14-year-old girl.
Assuntos
Carcinoma Adenoescamoso/patologia , Transformação Celular Neoplásica , Neoplasias Laríngeas/patologia , Neoplasias Pulmonares/patologia , Recidiva Local de Neoplasia/patologia , Papiloma/patologia , Adolescente , Biópsia por Agulha , Carcinoma Adenoescamoso/diagnóstico por imagem , Carcinoma Adenoescamoso/terapia , Transformação Celular Neoplásica/patologia , Terapia Combinada , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Neoplasias Laríngeas/diagnóstico por imagem , Neoplasias Laríngeas/terapia , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/terapia , Papiloma/diagnóstico por imagem , Papiloma/terapia , Radiografia Torácica , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVE: To evaluate the relations among glucose intolerance, genotype, and exocrine pancreatic status in patients with cystic fibrosis (CF). STUDY DESIGN: Data on 335 patients <18 years of age were from the Toronto CF database. A modified oral glucose tolerance test was given to 94 patients 10 to 18 years of age without recognized CF-related diabetes. CF transmembrane conductance regulator mutations and exocrine pancreatic status were determined for all patients. RESULTS: CF-related diabetes was clinically recognized in 9 of 335 (2.7%) patients <18 years of age, all of whom were pancreatic insufficient, and 8 of 9 had severe (classes I through III) mutations on both alleles. The ninth patient had unidentified mutations. Although all patients given the oral glucose tolerance test were asymptomatic and had normal fasting blood glucose, 16 of 94 (17%) had impaired glucose tolerance and 4 of 94 (4.3%) had CF-related diabetes without fasting hyperglycemia. Abnormal glucose tolerance was associated exclusively with severe mutations and exocrine pancreatic insufficiency. Glycosylated hemoglobin (HbA(1)C) levels did not correlate with glucose tolerance results. CONCLUSIONS: Screening of pancreatic-insufficient, adolescent patients with CF identified more with abnormal oral glucose tolerance than was suspected clinically and is recommended as a routine practice. HbA(1)C was not useful in screening for CF-related glucose intolerance.
