Patterns of mortality in sickle cell disease in the United Kingdom.

Gray, Andrew; Anionwu, Elizabeth; Davies, Sally C.; Brozović, Milica

doi:10.1136/jcp.44.6.459

Cited by 124 publications

(60 citation statements)

References 16 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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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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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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“…Relatively few studies have analyzed deaths in adults with SCD [2][3][4][5][6], and only one study apparently focused on deaths since 1990 [5]. The current study was carried out at a single institution, Howard University Hospital, where we analyzed deaths in 141 adult patients with SCD who expired between 1976 and 2001.…”

Section: Introductionmentioning

confidence: 99%

Circumstances of death in adult sickle cell disease patients

et al. 2006

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The purpose of the study was to analyze clinical and/or autopsy findings at the time of death among adults with sickle cell disease (SCD) at Howard University in Washington, DC over a 25-year period. A single physician recorded circumstances of death among 141 adult SCD patients he treated and knew well from 1976 to 2001. These findings were determined by autopsy report and/or clinical assessment. In a subset of 31 patients, autopsy records were reviewed for reports of iron deposition in liver and heart and of organ pathology. One hundred and fourteen (80.9%) of the patients had SS phenotype and 66 (46.8%) were female. The mean ± SD age at death was 36 ± 11 years. Leading circumstances of death included pulmonary hypertension (PHT) (26.2%), sudden death (23.4%), renal failure (22.6%), infection (18.4%), thromboembolism (14.9%), cardiac diagnoses (12.0%), cirrhosis (11.3%), pneumonia or acute chest syndrome (9.9%), bleeding (7.8%), and iron overload (7.0%). When circumstances of deaths that occurred after 1991 (n = 69) were compared to those that occurred in 1991 or earlier (n = 72), PHT (36.2% vs. 16.6%; P < 0.01) was significantly more common in 1992 or later. Significant associations were found between PHT and thromboembolism and between cirrhosis and iron overload. In this proportional mortality study of adults with SCD, PHT was the leading finding at the time of death. Thromboembolism was associated with PHT, and iron overload was associated with cirrhosis. Am. J. Hematol. 81:858-863, 2006. V V C 2006 Wiley-Liss, Inc.

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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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Patterns of mortality in sickle cell disease in the United Kingdom.

Cited by 124 publications

References 16 publications

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

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

Circumstances of death in adult sickle cell disease patients

Sequestro esplênico agudo em coorte de crianças com anemia falciforme

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