Acute Splenic Sequestration Crises (ASSC) in Young Children with Sickle Cell Anemia

Seeler, Ruth Andrea; Shwiaki, M. Ziad

doi:10.1177/000992287201101214

Cited by 57 publications

(18 citation statements)

References 6 publications

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“…O tratamento eficaz consiste no diagnóstico precoce, suporte clí-nico e transfusão de concentrado de hemácias. Existem relatos que demonstram elevada taxa de recorrência e de mortalidade 4,[6][7][8][12][13][14][15][16][17][18][19] . O manejo subsequente das crises ainda é motivo de discussão.…”

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“…Alguns autores destacaram uma possível associação com processos infecciosos agudos, mas sem conseguirem estabelecer uma relação direta de causa-efeito 9,15,26 .…”

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“…A maioria dos autores, entretanto, recomenda a realização de esplenectomia após o primeiro 4,6,21 ou, no máximo, após o segundo episódio de SEA 5,8,11,15 , o que denota uma conduta conservadora por parte dos médicos assistentes na presente casuística, talvez influenciada pelas dificuldades socioeconômicas e de compreensão das famílias atendidas e pelos obstáculos do acesso a serviços capacitados para o procedimento cirúrgico. Os dados sobre o tempo entre a indicação da esplenectomia e a realização do procedimento são preocupantes.…”

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Acute splenic sequestration in a cohort of children with sickle cell anemia

Rezende¹,

Viana²,

Murao³

et al. 2009

J Pediatr (Rio J)

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ResumoObjetivo: Analisar o sequestro esplênico agudo (SEA) em crianças com anemia falciforme, provindas da triagem neonatal de Minas Gerais e acompanhadas pelo Hemominas de Belo Horizonte (MG). Conclusões: O SEA é um evento comum na anemia falciforme, principalmente nos 2 primeiros anos de vida, com recidiva em mais da metade dos casos. Predominou conduta conservadora na indicação da esplenectomia. Embora a letalidade tenha sido baixa, o SEA representou a segunda causa de óbito. Isso aponta para fragilidades estruturais do sistema de saúde de MG e para a necessidade de melhor capacitação profissional na abordagem do problema. Métodos J Pediatr (Rio J) AbstractObjective: 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. Case-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. MethodsConclusions: 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. Artigo submetido em 15.12.08, aceito em 04.02.09.

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“…Alguns autores destacaram uma possível associação com processos infecciosos agudos, mas sem conseguirem estabelecer uma relação direta de causa-efeito 9,15,26 .…”

unclassified

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Acute splenic sequestration in a cohort of children with sickle cell anemia

Rezende¹,

Viana²,

Murao³

et al. 2009

J Pediatr (Rio J)

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“…Our findings are similar to the data published by some authors. 12,15,16,18,19,25 Among national data, the study by Fernandes 22 socioeconomic difficulties, lack of safe and fast transportation of the affected children and lack of resources to immediately perform blood transfusion and basic hematologic tests.…”

Section: Discussionmentioning

confidence: 99%

“…Some reports demonstrate high recurrence and mortality rates. 4,[6][7][8][12][13][14][15][16][17][18][19] There is still controversy concerning subsequent management. Options include: careful clinical observation, periodic packed red blood cell transfusion regimens and splenectomy.…”

Section: Introductionmentioning

confidence: 99%

Sequestro esplênico agudo em coorte de crianças com anemia falciforme

Rezende¹,

Viana²,

Murao³

et al. 2009

J. Pediatr. (Rio J.)

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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.

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