RESUMEN
Hepatitis occurring in patients with congenital X-linked or common variable hypogammaglobulinemia has been reported to follow a usual pattern of rapid progression from acute hepatitis to either chronic active hepatitis or death. This article describes a 21-year-old black man with congenital X-linked hypogammaglobulinemia who has been known to be a hepatitis B-associated antigen carrier during a 9-year follow up period. Liver enzyme studies are normal. His immunologic studies demonstrate no impairment of cellular immunity. His brother, who has the same disease and lives in the same household, has remained negative for hepatitis B-associated antigen. This patient demonstrates that not all hypogammaglobulinemia patients invariably have a severe clinical course with hepatitis.
Asunto(s)
Agammaglobulinemia/genética , Hepatitis B/etiología , Adulto , Agammaglobulinemia/complicaciones , Agammaglobulinemia/congénito , Formación de Anticuerpos , Portador Sano/inmunología , Hepatitis B/inmunología , Hepatitis B/transmisión , Humanos , Inmunidad Celular , MasculinoRESUMEN
Although oropharyngeal dysfunction has been reported as a cause of dysphagia in newborns, to our knowledge oropharyngeal dysfunction and neonatal apnea have not been associated previously. We studied two infants in whom apnea and bradycardia developed primarily while feeding. The diagnosis was established by observation of the swallowing mechanism using cinefluoroscopy while the infant ingested a dilute barium meal. Thickened formula feedings and use of a special nipple resulted in complete cessation of apnea and bradycardia. Careful attention to the details that surround episodes of apnea is essential so that morbidity and mortality from treatable causes of apnea can be minimized.
Asunto(s)
Apnea/etiología , Enfermedades del Recién Nacido/fisiopatología , Enfermedades Nasofaríngeas/complicaciones , Bradicardia/etiología , Cinerradiografía , Humanos , Recién Nacido , MasculinoRESUMEN
A patient with congenital x-linked agammaglobulinemia, who had two separate episodes of an apparent bacterial purulent hepatic triaditis in the absence of any known local predisposing factors, is presented. These episodes may reflect the increased susceptibility of an immunodeficient patient to bacterial infections. This case demonstrates the need to consider hepatic involvement in the work-up of fevers of undetermined origin in immunodeficient patients, even in the absence of any radiologic or sonographic evidence of mechanical biliary tract obstruction.