RESUMO
Retrospective analysis of eight patients suffering from infantile cortical hyperostosis shows clearly autosomic dominant inheritance of the disease, the strong variation in the clinical expressivity, and therefore the need for an in deep work-up of the patient and his relatives in the presence of any suspicious symptom.
Assuntos
Hiperostose Cortical Congênita/genética , Adulto , Feminino , Humanos , Hiperostose Cortical Congênita/diagnóstico por imagem , Hiperostose Cortical Congênita/transmissão , Lactente , Masculino , Linhagem , Radiografia , Estudos RetrospectivosAssuntos
Coma/etiologia , Vasculite por IgA/complicações , Convulsões/etiologia , Criança , Coma/terapia , Humanos , Masculino , Convulsões/terapiaRESUMO
We review our experience on IgA nephropathy (Berger's disease), defined as the IgA mesangial deposit in the absence of systemic disease. Following these criteria ten patients have been diagnosed which amount to 10% of all biopsied glomerulonephritis in our centre during 1977-1985. These ten patients have been controlled for periods of time ranging from 4 months to 7 years (means = 3 years). After that time, 2 patients have chronic renal failure, in contrast with other pediatric series in which the prognosis is less severe.
