RESUMO
We describe a case in which nodular macroglossia, a very rare type of tongue involvement, was associated with the co-deposition of lambda light chain and beta-2 microglobulin fibrils in the tongue. The combined presence of two different amyloid fibrils did not lead to a more unfavourable clinical outcome. We believe that both these features often remain underdiagnosed and are in fact more frequent than reported. A careful clinical examination of the tongue together with serum immunofixation should be routine in all patients with dialysis-related amyloidosis in order to investigate the prevalence and type of tongue involvement and to rule out other types of amyloidosis. In all cases of suspected mixed amyloidosis, immunohistochemical characterization of fibrils should be carried out by electron microscopy.
Assuntos
Amiloidose/patologia , Glomerulonefrite/terapia , Macroglossia/patologia , Diálise Renal/efeitos adversos , Microglobulina beta-2/metabolismo , Amiloidose/etiologia , Doença Crônica , Seguimentos , Glomerulonefrite/diagnóstico , Humanos , Imuno-Histoquímica , Assistência de Longa Duração , Macroglossia/etiologia , Masculino , Microscopia Eletrônica , Pessoa de Meia-Idade , Diálise Renal/métodos , Medição de Risco , Microglobulina beta-2/análiseRESUMO
A series of 121 consecutive cases with accelerated hypertension is presented. Forty-seven patients had essential hypertension. This diagnosis was confirmed during the follow-up, which lasted from 3 to 255 months (mean 63.1 +/- 58.1). The relationship between the effects of pharmacological treatment on high blood pressure (BP) and disease course were analyzed in accelerated essential hypertensive patients only, to avoid the imponderable influence of basic disease in secondary hypertension. In most cases, therapeutical effect on BP was monitored by multiple daily BP self-measurements. Since 1974, when this study was initiated, plasma creatinine concentration at admission showed a clear-cut decrease, especially after the introduction in the eighties of new hypotensive drugs. A sustained BP decrease assured a long-lasting preservation of renal function, if the initial functional loss was limited (plasma creatinine concentration of 2 mg/dl (176.8 mumol/l) or less). Renal function was preserved even if the BP decrease was not optimal and signs of relapsing malignant retinopathy were observed in the course of disease. Although the whole series showed a 12-year survival rate of about 69%, patients enrolled after 1980 had a 100% survival rate.