Macroglossia as initial clinical manifestation of primary amyloidosis

Amyloidosis is a rare disease caused by pathological deposit of an amyloid extracellular proteinaceus material. We report a case of a 50-year-old man with history of lips and tongue swelling associated to periorbital ecchimosys for 6 months. At the physical examination an important macroglossia was observed. Complementary tests were accomplished, and the pathological examination of the tongue biopsy confirmed the diagnosis. The patient was treated with prednisone and melphalan with an unsatisfactory response. Along the follow up the patient developed heart failure, and died 7 months after diagnosis of pulmonary infection and sepsis.A amiloidose é uma doença rara causada pelo depósito patológico de substância amilóide no meio extracelular. Relatamos o caso de um paciente de 50 anos de idade, masculino, com história de edema de lábios e língua associado à equimose periorbitária bilateral há 6 meses. Ao exame físico, observamos importante macroglossia. Foram realizados exames complementares, e o diagnóstico foi confirmado pela análise histopatológica da biópsia de língua. O paciente recebeu tratamento com prednisona e melfalan com resposta insatisfatória. Evoluiu com insuficiência cardíaca congestiva e infecções urinárias e respiratórias de repetição. Após 7 meses do diagnóstico, o paciente foi internado com infecção pulmonar complicada com sepse e evoluiu a óbito.Universidade Metropolitana de SantosCentro Universitário LusíadaUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de MedicinaSociedade Brasileira de ReumatologiaUNIFESP-EPMHospital Ana Costa Clínica MédicaUNIFESP, EPMSciEL

In comparison to the myocardial perfusion scintigraphy, a treadmill stress test is a viable, efficient and cost effective option to predict cardiovascular events in elderly patients

OBJETIVO: Definir o valor prognóstico e a custo-efetividade do teste ergométrico (TE) em comparação à cintilografia de perfusão miocárdica com dipiridamol (DIP) em indivíduos com > 75 anos de idade. MÉTODOS: Foram avaliados, consecutiva e prospectivamente, 66 pacientes (40% homens), com média de idade de 81 ± 5 anos. Desses pacientes, 57% eram hipertensos, 38% eram dislipidêmicos e 28%, diabéticos. O protocolo de Bruce para rampa foi adaptado, obtendo-se o valor prognóstico do TE pelo escore de Duke. RESULTADOS: A duração do TE, o porcentual da freqüência cardíaca máxima preconizada e o duplo produto no pico do exercício foram, respectivamente, de 7 ± 3 minutos, 95 ± 9% e 24.946 ± 4.576 (bpm x mmHg). O TE e a DIP apresentaram resultados positivos para isquemia miocárdica similares (21% vs 15%, respectivamente). A concordância entre os testes foi de 88% (Kappa 0,63, p 75 years of age. METHODS: Consecutive and prospective assessment of 66 patients (40% male) aged 81 ± 5 years of which 57% were hypertensive, 38% had dyslipidemia and 28% were diabetics. The Bruce protocol was adapted for a tilt treadmill and the TST prognostic value was obtained using the Duke treadmill score. RESULTS: The TST duration, recommended maximum heart rate percentage and double product at peak exercise were respectively: 7 ± 3 minutes, 95 ± 9% and 24,946 ± 4,576 (bpm x mmHg). The TST and DIP presented similar positive results for myocardial ischemia (21% vs 15%, respectively). The correlation between the tests was 88% (Kappa 0.63, p<0.01). During 685 ± 120 days of follow-up, nine major events occurred: 6 deaths, 2 acute coronary syndromes and 1 myocardial revascularization. The variables associated with the major events were: age (83 ± 6 vs 80 ± 4 years; p=0.048), male gender (78% vs 33%; p=0.02), ST segment depression (1 ± 1 mm vs 0.25 ± 0.6 mm; p= 0.01), high or intermediate risk determined by the Duke treadmill score - combined in one group (44% vs 2%; p=0.001) and abnormal DIP (44% vs 10%, p= 0.02). CONCLUSION: For this elderly population, the TST was an efficient and viable option with a similar diagnostic value in comparison to the DIP. However, the TST was more accurate in the prediction of major events and offers a lower cost