Causes of death in sickle cell disease: an autopsy study

Manci, Elizabeth A.; Culberson, Donald E.; Yang, Yih‐Ming; Gardner, Todd M.; Powell, Randall W.; Haynes, Johnson; Shah, Arvind; Mankad, Vipul N.

doi:10.1046/j.1365-2141.2003.04594.x

Cited by 320 publications

(218 citation statements)

References 27 publications

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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%

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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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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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“…4 A mortalidade oscila entre 24% a 50%. Estudos em autópsias revelaram que o AVC é responsável por 9,8% das mortes nos EUA em pacientes do CSSCD -estudo multicêntrico norteamericano 5 ou Cooperative Study Sickle Cell Disease . No Brasil, estudo realizado no Hemocentro do Rio Grande do Norte encontrou causas cerebrais em 9% dos óbitos, semelhante às causas infecciosas, e inferior à síndrome torácica e seqüestro esplênico.…”

Section: Conclusõesunclassified

Acidente vascular cerebral e outras complicações do sistema nervoso central nas doenças falciformes

Ângulo¹

2007

Rev. Bras. Hematol. Hemoter.

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IntroduçãoA causa mais comum de acidente vascular cerebral (AVC) na infância é a anemia falciforme, o homozigoto da hemoglobina S ou Hb SS. A doença falciforme (Hb SS e interações com outras hemoglobinopatias e talassemias) é a principal enfermidade genética da população brasileira, com 13.500 pacientes catalogados em 2006, com prevalência populacional do gene S de 1/1.200 pessoas. 1 Manifesta-se com lesões em vários órgãos, causando elevada morbidade e mortalidade.Dentre essas lesões, a principal, na infância, é a das artérias cerebrais, mas após os 20 anos também pode se manifestar, como seqüela de lesões anteriores ou neoformadas. 2 A vasculopatia tem a maior importância no desenvolvimento da criança e na qualidade de vida. 3 Além do acidente vascular cerebral (AVC), completo ou incompleto ("silencioso"), existem outras complicações do sistema nervoso central, porém não exclusivas da doença falciforme, como convulsões, encefalopatia, hipertensão intracraniana, trauma, meningite, alterações mentais por distúrbios metabóli-cos e neuropatia periférica ou hipertensão intracraniana por oclusão venosa. 3 Com exceção do AVC, o diagnóstico da vasculopatia cerebral falciforme não pode ser feito clinicamente, necessitando auxílio neurorradiológico ou por outros meios. O tratamento se faz basicamente com transfusões de eritrócitos contendo hemoglobina A.O objetivo deste trabalho é rever a literatura médica, procurando selecionar a melhor conduta em diagnóstico e tratamento que seja possível empregar de imediato, para reduzir a morbidade e mortalidade da vasculopatia cerebral e elevar a qualidade de vida dos pacientes, principalmente na infância.

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“…Nargues A. Weir, 1 Rehan Saiyed, 2 Shoaib Alam, 1 Anna Conrey, 2 Himanshu D. Desai, 3 M. Patricia George, 4 Jennifer H. Keeley, 4 Elizabeth S. Klings, 5 Alem Mehari,6 James G. Taylor VI, 2 …”

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confidence: 99%

“…1 There are well-established therapies for pulmonary arterial hypertension (PAH), but few reports about their use in SCDPH. 2 We report the clinical course of 11 SCDPH patients on maximal supportive therapy including other pulmonary vasodilators, who received compassionate therapy with prostacyclin-analogs at four PH treatment centers.…”

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confidence: 99%