Management of Sickle Cell Disease: Recent Advances and Controversies

Castro, Oswaldo

doi:10.1046/j.1365-2141.1999.01565.x

Cited by 34 publications

(24 citation statements)

References 97 publications

(94 reference statements)

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“…Sickle-cell disease was another significant and independent risk factor for sepsis among patients with documented fever. This finding is due to the lack or reduction of spleen function, particularly during the first 6 years of life [27]. We should remember that sepsis may be the initial event in the presentation of asyet-undiagnosed sickle-cell disease (since the majority of such events occur before 3 years of age) [28,29].…”

Section: Discussionmentioning

confidence: 99%

Clinical and epidemiological characteristics of children admitted with fever in emergency department with or without sepsis

Sant'ana

Amoedo

Nascimento‐Carvalho

2017

J Infect Dev Ctries

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Introduction: Sepsis is a major cause of childhood death worldwide. In developing countries, epidemiological data about sepsis is scarce. This study describes and compares the frequency of etiological agents and initial sites of infection in children with or without sepsis, identifying risk factors and assessing outcomes. Methodology: Clinical and demographic data from patients < 13 years of age with reported fever in a pediatric emergency department were collected and registered in forms. Patients were classified as with or without sepsis according to Goldstein et al.'s criteria [6]. Results: Of 254 patients, 120 (47%) did and 134 (53%) did not meet the sepsis definition. Overall, the median age (IQR) was 1.7 (0.8-3.9) years, and 153 (60%) were boys. Patients with sepsis were older (2.8 [1.1-5.3] versus 1.3 [0.6-2.9] years; p < 0.001) and had sickle-cell disease more frequently (7.6% versus 0.8%; p = 0.007). By multiple logistic regression, age and sickle-cell disease were independently associated with sepsis. The most frequent initial infections were pneumonia (43.7%), diarrhea (17.3%) and cellulitis/adenitis (13.0%). The frequency of these did not differ when patients with or without sepsis were compared. Etiology was established in 57 (22.4%) patients, 32 (26.7%), and 25 (18.7%) with or without sepsis, respectively. Four (3.3%) patients died in the sepsis subgroup, whereas none died in the other subgroup. Conclusions: Children who met the 2005 international consensus definition of sepsis showed differences in age and comorbidities (sickle-cell disease) upon admission and were more likely to die.

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Section: Discussionmentioning

confidence: 99%

Clinical and epidemiological characteristics of children admitted with fever in emergency department with or without sepsis

Sant'ana

Amoedo

Nascimento‐Carvalho

2017

J Infect Dev Ctries

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“…This a permanent risk due to the lack or reduction of spleen function, 31 primarily during the first 6 years of life, 32 and it is the main cause of death among infants with sicklecell anemia. 18 We should remember that sepsis may be the initial event in the presentation of as-yet-undiagnosed sickle-cell anemia, 13,33 since a majority of such events occur before 3 years of age.…”

Section: Septicemiamentioning

confidence: 99%

“…5 Neonatal diagnosis, associated with an aggressive approach to febrile episodes in infants, was effective at preventing deaths by septicemia before the era of prophylaxis with penicillin. 32 One highly important point, essential to the adequate management of patients with sickle-cell anemia is rigorous outpatients follow-up, which should always be prioritized within health services.…”

Section: Prophylaxismentioning

confidence: 99%

“…After this age, a cost-benefit relationship has not been demonstrated that would justify continuing prophylaxis. 32,41 In some health care centers, the last dose of penicillin is given together with the last dose of the antipneumococcal vaccine, in contrast with other centers that firmly suggest the maintenance of this type of prophylaxis, particularly in those children with recurring episodes of sepsis by pneumococcus. The Buffalo Hospital in New York, for example, recommends such a continuity, maintaining a posological penicillin regime administered orally and rigorous outpatients follow-up: every two months until 6 months, every three months from 6 months until 2 years and, thereafter, twice-yearly.…”

Section: Antipneumococcalmentioning

confidence: 99%

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Anemia falciforme e infecções

Nuzzo¹,

Fonseca²

2004

J. Pediatr. (Rio J.)

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Objective: To discuss the high prevalence of sickle cell disease in our environment and the increased morbidity and mortality as a result of infection associated with this condition. Sources of data:Review of MEDLINE from 1986 to 2003. We found around 600 references about the subject. Thirty-five journal articles were reviewed, in addition to chapters in two text books. Summary of the findings:We discuss general information concerning sickle cell disease as well as a few topics about the most frequently observed infections in these patients. Drug prophylaxis and immunizations are also covered.Conclusions: This review hopes to provide the pediatric community with information concerning the association between sickle cell disease and infections, so as to minimize the occurrence of complications.

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“…O diagnóstico neonatal, associado a uma abordagem agressiva dos episó-dios febris em lactentes, foi efetivo na prevenção de mortes por septicemia antes da era da profilaxia com penicilina 32 .…”

Section: Profilaxiaunclassified

Sickle cell disease and infection

Nuzzo¹,

Fonseca²

2004

J Pediatr (Rio J)

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Este artigo é dedicado a Maria de Lourdes Santos Pardo -in memoriam.Artigo submetido em 16.01.04, aceito em 31.03.04. AbstractObjective: To discuss the high prevalence of sickle cell disease in our environment and the increased morbidity and mortality as a result of infection associated with this condition.Sources of data: Review of MEDLINE from 1986 to 2003. We found around 600 references about the subject. Thirty-five journal articles were reviewed, in addition to chapters in two text books. Summary of the findings:We discuss general information concerning sickle cell disease as well as a few topics about the most frequently observed infections in these patients. Drug prophylaxis and immunizations are also covered.Conclusions: This review hopes to provide the pediatric community with information concerning the association between sickle cell disease and infections, so as to minimize the occurrence of complications. ResumoObjetivo: A alta prevalência de anemia falciforme em nosso meio e a elevada morbimortalidade por infecções associada a esta condição estimularam a realização deste artigo de revisão. Síntese dos dados: Neste artigo, além de informações gerais a respeito da doença falciforme, são abordados alguns tópicos sobre as infecções mais freqüentemente observadas no paciente com anemia falciforme, assim como a profilaxia medicamentosa e imunizações disponíveis.Conclusões: Esta é uma revisão que visa fornecer à comunidade pediátrica informações sobre o binômio anemia falciforme e infecções, a fim de minimizar suas complicações nesta comunidade específica.

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Management of Sickle Cell Disease: Recent Advances and Controversies

Cited by 34 publications

References 97 publications

Clinical and epidemiological characteristics of children admitted with fever in emergency department with or without sepsis

Clinical and epidemiological characteristics of children admitted with fever in emergency department with or without sepsis

Anemia falciforme e infecções

Sickle cell disease and infection

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