Right and left ventricular function and myocardial scarring in adult patients with sickle cell disease: a comprehensive magnetic resonance assessment of hepatic and myocardial iron overload

Background: Patients with Sickle cell disease (SCD) who receive regular transfusions are at risk for developing cardiac toxicity from iron overload. The aim of this study was to assess right and left cardiac volumes and function, late gadolinium enhancement (LGE) and iron deposits in patients with SCD using CMR, correlating these values with transfusion burden, ferritin and hemoglobin levels. Methods: Thirty patients with SCD older than 20 years of age were studied in a 1.5 T scanner and compared to age- and sex-matched normal controls. Patients underwent analysis of biventricular volumes and function, LGE and T2* assessment of the liver and heart. Results: When compared to controls, patients with SCD presented higher left ventricular (LV) volumes with decreased ejection fraction (EF) with an increase in stroke volume (SV) and LV hypertrophy. The right ventricle (RV) also presented with a decreased EF and hypertrophy, with an increased end-systolic volume. Although twenty-six patients had increased liver iron concentrations (median liver iron concentration value was 11.83 +/- 9.66 mg/g), only one patient demonstrated an abnormal heart T2* < 20 msec. Only four patients (13%) LGE, with only one patient with an ischemic pattern. Conclusions: Abnormal heart iron levels and myocardial scars are not a common finding in SCD despite increased liver iron overload. The significantly different ventricular function seen in SCD compared to normal suggests the changes in RV and LV function may not be due to the anemia alone. Future studies are necessary to confirm this association.1

Epidemiologia de internações por doença falciforme no Brasil Epidemiology of sickle cell disease hospital admissions in Brazil

OBJETIVO: A doença falciforme é uma enfermidade hereditária que afeta principalmente a população negra. O estudo teve por objetivo analisar as internações devido às complicações da doença, com enfoque nos aspectos epidemiológicos e clínicos. MÉTODOS: A população estudada foi constituída de 9.349 pacientes com diagnóstico de doença falciforme internados em hospitais da Bahia, Rio de Janeiro e São Paulo, no período de 2000 a 2002. Utilizaram-se os dados do Sistema de Internações Hospitalares do Sistema Único de Saúde. As variáveis respostas foram o óbito e o tempo médio de permanência hospitalar. As covariáveis foram o sexo, a idade, o tipo de admissão e a natureza jurídica do hospital. As proporções foram comparadas utilizando-se o teste qui-quadrado ou de Fischer; e para as variáveis contínuas, foi utilizado o teste Mann-Whitney ou Kruskall-Wallis. RESULTADOS: A mediana de idade variou de 11,0 a 12,0 anos e cerca de 70% das internações foram abaixo dos 20 anos. A mediana de dias de permanência hospitalar variou com a idade e o tipo de admissão. O tipo de admissão mais freqüente foi pela emergência (65,6 a 90,8%). Foi observada maior letalidade hospitalar entre adultos. A mediana da idade do óbito foi baixa (26,5 a 31,5 anos). CONCLUSÕES: Os resultados confirmaram a alta morbidade na população jovem e evidenciaram predominância de óbitos entre adultos jovens.<br>OBJECTIVE: Sickle cell disease is a hereditary disease, which affects mainly the black population. The aim of the present study was to analyze hospital admissions due to acute events resulting from sickle cell disease, at the epidemiological and clinical levels. METHODS: The study population included 9,349 patients with sickle cell disease admitted to hospitals in Bahia, Rio de Janeiro, and Sao Paulo, between 2000 and 2002. The national hospital database of the Brazilian Healthcare System was used. Response variables were death and mean duration of hospital admission. Covariables included sex, age, type of admission, and hospital legal status. Proportions were compared using Chi-square or Fischer tests; for continuous variables, the Mann-Whitney or Kruskall-Wallis tests were used. RESULTS: Median age ranged between 11.0 and 12.0 years, and 70% of admissions were of patients below age 20 years. Length of stay varied with age and type of admission. Emergency rooms were the most frequent form of admission (65.6 to 90.8%). Mortality was higher among adults. Median age of death was low (26.5 to 31.5 years). CONCLUSIONS: Our results confirm the high morbidity among youngsters and show a predominance of death among young adults