Sequestro esplênico agudo em coorte de crianças com anemia falciforme

Abstract

Objective: To analyze acute splenic sequestration (ASS) in children with sickle cell anemia diagnosed through a newborn screening program in the state of Minas Gerais, Brazil, and followed up at the hematology center in the city of Belo Horizonte, Minas Gerais, Brazil. Results: A total of 89 patients had 173 episodes of ASS (10.2 first episodes per 100 patient-years); 75% of the first episodes occurred before 2 years of age. The estimated probability of occurrence of the first episode of ASS during the study period was 40%. Recurrence rate reached 57.3%. After the first episode, splenectomy was indicated in only 12.4% of the cases; after the second, in 60.4% of the cases. After the third episode, 41.7% of the patients remained under clinical observation. The median time between indication for splenectomy and the actual surgical procedure was 2 months. During the intervening period, 37.2% of the children suffered a new episode of ASS and one child died. MethodsCase-fatality rate was 1.1% for the first episode and 7.8% for the subsequent episodes. Among a total of 255 children, 19 died: 36.8% due to infections and 26.3% after ASS. Conclusions:ASS is relatively common in sickle cell anemia, mainly in the first 2 years of life; relapse occurs in more than half of the cases. Conservative management instead of immediate splenectomy was the method of choice.Although the case-fatality rate was low, ASS was the second most common cause of death. These results disclose some fragilities of the health system in the state of Minas Gerais and the need for better professional education to approach ASS crises.J Pediatr (Rio J). 2009;85(2):163-169: Sickle cell anemia, acute splenic sequestration, children, splenectomy, newborn screening, mortality.

A Assistência De Enfermagem Ao Paciente Vítima De Sequestro Esplênico Na Anemia Falciforme

“…Eventos recorrentes de vaso-oclusão no baço são antecessores do sequestro esplênico, característico pelo aumento súbito do baço, diminuição da concentração de hemoglobina maior ou igual a 2 g/dl comparada ao valor basal do paciente, reticulocitose, piora da palidez e plaquetopenia, podendo haver insuficiência cardíaca e, não raramente, evoluindo para choque hipovolêmico. (Bruniera, 2007;Ladeia, et al, 2020;Rezende et.al., 2007;Fernandes, et al, 2010;Brasil, 2014;Carvalho, et al, 2016) Estudos apontam que o SEA acomete cerca de 7,5% a 30% dos pacientes, com a taxa de mortalidade chegando a 12%. O risco de recorrência é maior quando o primeiro episódio acontece antes dos 2 anos de idade, sendo então indicado como forma de prevenção a esplenectomia, aumentando o risco de infecção.…”