RESUMO
OBJECTIVE: To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications. METHODS: A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up. FINDINGS: Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P =.01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P <.01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P =.01). Febrile events did not differ between the groups or between the indications for splenectomy. CONCLUSION: Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons.
Assuntos
Anemia Falciforme/complicações , Homozigoto , Esplenectomia , Adolescente , Adulto , Anemia Falciforme/mortalidade , Anemia Falciforme/cirurgia , Antibioticoprofilaxia , Bacteriemia/epidemiologia , Bacteriemia/prevenção & controle , Estudos de Casos e Controles , Causas de Morte , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Jamaica/epidemiologia , Modelos Logísticos , Masculino , Penicilinas/uso terapêutico , Estudos Retrospectivos , Fatores de Risco , Esplenectomia/estatística & dados numéricosRESUMO
OBJECTIVE: To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications. METHODS: A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up. FINDINGS: Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P = .01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P < .01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P = .01). Febrile events did not differ between the groups or between the indications for splenectomy. CONCLUSION: Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons (Au)
Assuntos
Adulto , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Lactente , Estudo Comparativo , Anemia Falciforme/complicações , Homozigoto , Esplenectomia/normas , Antibioticoprofilaxia , Bacteriemia/epidemiologia , Bacteriemia/prevenção & controle , Estudos de Casos e Controles , Causas de Morte , Distribuição de Qui-Quadrado , Seguimentos , Jamaica/epidemiologia , Modelos Logísticos , Penicilinas/uso terapêutico , Estudos Retrospectivos , Fatores de Risco , Anemia Falciforme/mortalidade , Anemia Falciforme/cirurgiaRESUMO
The aim of this study was to confirm reports of low protein C (PC) and S (PS) concentrations in steady-state patients with homozygous sickle cell (SS) disease when compared to a racially matched normal haemoglobin (AA) control group and to examine the mechanisms of this reduction with respect to hepatic function, coagulation activation and haematological indices. In 36 SS patients and 35 AA race-matched controls PC (functional and immunoreactive), PS (free and total) were measured. C4B binding protein (C4B) was assessed by immunoelectrophoresis and D-dimer by ELISA. Hepatic function was assessed by prothrombin (PT) time (49 SS, 64 AA), factor V (34 SS, 36 AA) and factor VII concentrations (28 SS, 29 AA). Proteins induced in vitamin K absence or antagonism (PIVKA) were sought in 12 SS's. The relationship between PC, PS and total bilirubin, haemoglobin (Hb) F and reticulocyte count was also assessed. PC, PS and C4B were lower in SS disease. SS patients had longer PT times, and lower factor V and VII concentrations in comparison to AA controls. PC (functional and immuno-reactive) and free PS correlated with PT. Within SS genotype PT correlated negatively with factor V and factor VII. Factor V and VII were positively correlated. PIVKAs were not detected. There was no correlation between PC, PS and D-dimer, haemolytic rate or Hb F concentration. Prolongation of PT time, low factor V and VII suggest that hepatic dysfunction, rather than coagulation activation or haemolytic rate, accounts for the reduced concentrations of PC and PS in steady-state SS disease. The absence of PIVKAs suggests a hepatocellular problem.
Assuntos
Anemia Falciforme/etiologia , Hepatopatias/fisiopatologia , Deficiência de Proteína C , Deficiência de Proteína S/complicações , Anemia Falciforme/sangue , Anemia Falciforme/genética , Fator V/análise , Fator VII/análise , Genótipo , Hemoglobinas/análise , Humanos , Hepatopatias/complicações , Estudos Longitudinais , Tempo de ProtrombinaRESUMO
The activated protein C resistance (APC-R) ratios in 50 patients with steady state homozygous sickle cell (SS) disease and 59 healthy AA controls was measured. There was a significant reduction in median APC-R ratio in sickle cell disease compared to controls. This reduction in APC-R ratio was not explained by (1) the presence of the factor V Leiden, found in only one of 165 patients with SS disease including those tested for APC-R, or (2) the presence of lupus anticoagulants. However, the raised levels of factor VIIIC in SS patients in this study may be contributing to increased resistance to APC, which in turn may contribute to the vaso-occlusive complications of SS disease.
Assuntos
Anemia Falciforme/sangue , Transtornos das Proteínas Sanguíneas/sangue , Proteína C , Adolescente , Adulto , Anemia Falciforme/complicações , Anemia Falciforme/genética , Transtornos da Coagulação Sanguínea/sangue , Transtornos da Coagulação Sanguínea/complicações , Transtornos das Proteínas Sanguíneas/complicações , Feminino , Homozigoto , Humanos , Masculino , Tempo de ProtrombinaRESUMO
The activated protein C resistance (APC-R) ratios in 50 patients with steady state homozygous sickle cell (SS) disease and 59 healthy AA controls was measured. There was a significant reduction in median APC-R ratio in sickle cell disease compared to controls. This reduction in APC-R ratio was not explained by (1) the presence of the factor V Leiden, found in only one of 165 patients with SS disease including those tested for APC-R, or (2) the presence of lupus anticoagulants. However, the raised levels of factor VIIIC in SS patients in this study may be contributing to the increased resistance to APC, which in turn may contribute to the vaso-occlusive complication of SS disease.(AU)
Assuntos
Adolescente , Adulto , Feminino , Humanos , Masculino , Anemia Falciforme/sangue , Transtornos das Proteínas Sanguíneas/sangue , Proteína C , Anemia Falciforme/complicações , Anemia Falciforme/genética , Transtornos da Coagulação Sanguínea/sangue , Transtornos da Coagulação Sanguínea/complicações , Transtornos das Proteínas Sanguíneas/complicações , Homozigoto , Tempo de ProtrombinaRESUMO
The association of Salmonella osteomyelitis with sickle-cell disease is well described. There are few data on the frequency, morbidity or mortality of the salmonella septicaemia which precedes the development of focal bone infection. We have reviewed 34 cases (19 male, 15 female, 29 SS, 3 SB thalassaemia, 1 SC, 1 SO Arab) of salmonella septicaemia without clinical evidence of osteomyelitis. Clinical presentation: 44 percent occurred in children between 4 and 7 years old; 17 had fever > 38.5§C associated with painful crisis, 8 had increased jaundice and hepatomegaly, 6 hepatomegaly alone, 3 increased jaundice alone, 6 acute chest syndrome, 6 a diarrhoeal illness and 4 splenic enlargement. Laboratory findings:- 16 had leucocytosis and neutrophilia, 8 erythroid hypoplasia and 3 erythroid aplasia. A variety of salmonella species were isolated. Outcome:- 18 recovered without sequelae. Osteomyelitis developed in 11 (between 5 days and 2 months from positive blood culture). There were 5 fatalities, their illness being characterised by its rapid onset and overwhelming nature. There was no predominant organism responsible for progression to osteomyelitis or death. A review of positive blood culture results (1973 to date) in this clinic shows salmonella species present in 22 percent (this figure excludes patients with salmonella osteomyelitis who subsequently had a positive blood culture). Salmonella septicaemia without progression to osteomyelitis seems more common than previously assumed and we suggest that in dealing with the febrile sick child, in whom blood culture results are not yet available, this association should be borne in mind when selecting antimicrobial therapy (AU)