Morbimortalidade em doença falciforme

Abstract

The high morbidity-mortality rates in sickle cell disease (SCD) led us to study the epidemiological profile and respective clinical complications of patients seen at a Regional Blood Center (HR) and a University Clinical Hospital (HC-U) between 1998 and 2007. In a retrospective study, 151 patients were evaluated regarding age, gender, skin color, origin, diagnosis, reason of visit to HR or reason and length of stay in the HC-U, and cause and age in cases of death. Simple descriptive statistical analysis was performed. The mean age was 17.7 years, 52.4% of the patients were female, 58.2% were from the city and no information regarding skin color was available in 92.2% of the records. Sickle cell anemia was present in 82.5% of cases. The most frequent reason for the 910 visits to HR and 589 hospitalizations in HC-U were afebrile pain episodes (61.9% and 25.3%, respectively). The mean age of the 11 patients who died was 33.5 years with one patient being younger than 10 years old, and multiple organ failure was the most frequent cause. The epidemiological profile shows a predominance of children and young adults, women, and the SS genotype. The rates of hospitalization in HC-U and of visits to HR and the low mean age at death confirm the high morbidity and mortality related to SCD. However, a large number of children do not present with complications or require hospitalization which reflects the efficacy of preventive measures provided by the early diagnosis implemented over the last 10 years.

Anti-Toxoplasma gondii antibodies in patients with beta-hemoglobinopathies: the first report in the Americas

“…However, there is a risk of infections by viruses, bacteria and parasites with transfusions once serological screening is not compulsory for all microorganisms [10, 17, 18, 30–32]. Despite the lack of data on blood transfusions performed in the individuals analyzed in the current study, we should consider this risk, and more detailed studies should be performed to verify the possible transmission of pathological agents in blood transfusions.…”

Musculoskeletal pain, profile and quality of life of individuals with sickle cell disease

“…The results demonstrated that 100% of the subjects experienced pain on at least one body region in the previous 12 months and 81.5% in the previous seven days. A previous study also conducted in the same recruitment site (HRU) 25 showed that the most prevalent cause of health care consultation (64.4%) were pain crises.…”

Anemia Falciforme: Caracterização Dos Pacientes Atendidos Em Um Ambulatório De Referência

“…Destaca-se ainda que a procedência da clientela das regiões NorteNoroeste (38%) do Estado do Paraná, pode ser explicada historicamente devido ao maior grau de miscigenação africana existente nessas regiões (7,9) . A grande maioria dos pacientes foi acompanhada periodicamente no ambulatório por três anos ou mais, o que pressupõe acompanhamento regular, orientações preventivas, contribuindo para melhor qualidade de vida e, por conseguinte menor índice de mortalidade, principalmente por problemas infecciosos em crianças menores de cinco anos, como evidenciado em outras pesquisas (9,20) . A crise álgica como evento clínico mais dramático da AF, causada pela oclusão microvascular com subsequente lesão tecidual, em resposta ao afoiçamento das hemácias, representou a complicação clínica mais frequente neste estudo e também a principal causa de internações hospitalares.…”

“…Ocorre geralmente em decorrência da isquemia tecidual e liberação de pirógenos endógenos; secundária às crises de anemia hemolítica ou como sinal de infecção. As infecções recorrentes, como a segunda maior complicação clínica, se justifica pela disfunção esplênica, resultante da perda da função retículo-endotelial do baço, em consequência dos recorrentes infartos esplênicos, favorecidos pela microvascularização deste órgão, precipitando o processo de falcização, e com isso a hipóxia tecidual (10,20) . Alguns achados clínicos presentes na clientela assistida -êmese, diarréia, linfonodomegalia, dispnéia, tosse e coriza hialina -podem reafirmar a recorrência desses processos infecciosos.…”