Assuntos
Citometria de Fluxo/estatística & dados numéricos , Leucemia/diagnóstico , Programas Médicos Regionais/organização & administração , América Central , Criança , Citometria de Fluxo/economia , Citometria de Fluxo/métodos , Humanos , Imunofenotipagem , Leucemia/classificação , Leucemia/patologia , Desenvolvimento de Programas , Encaminhamento e Consulta/estatística & dados numéricos , Programas Médicos Regionais/economia , Medição de RiscoRESUMO
PURPOSE: To investigate the relationship between survival and malnutrition at the time of diagnosis among children treated for cancer in two developing countries. PATIENTS AND METHODS: We studied 443 children treated for cancer between 1995 and 1998 at two centers in San Salvador, El Salvador, and Recife, Brazil. Median age at diagnosis was 4.9 years; 283 children had leukemia and 160 had solid tumors. Z-scores were calculated for weight for age (WAZ), height for age (HAZ), and weight for height (WHZ) at diagnosis. Z scores <-2 indicated malnutrition. Patients were also stratified by low-risk disease (solid tumors: stage I, stage II, or localized; acute lymphocytic leukemia: white blood cell count <25,000/microL, no central nervous system involvement, no mediastinal mass and age >1 and <10 yrs) and high-risk disease (all other patients, including those with acute or chronic myelocytic leukemia). RESULTS: Z-scores indicated malnutrition in 23.5% (WAZ), 22.8% (HAZ), and 15.7% (WHZ) of patients. Z-score was not significantly related to overall survival rates, to survival rates analyzed by type of malignancy or risk status, or to survival rates at the end of the first month of treatment. CONCLUSIONS: We found no relationship between nutritional status and survival in these patients. This implies that future protocols for use in developing countries can be designed to provide optimal treatment intensity despite the high incidence of malnutrition.
Assuntos
Neoplasias/complicações , Distúrbios Nutricionais/complicações , Estado Nutricional , Brasil , Criança , Pré-Escolar , El Salvador , Humanos , Leucemia/complicações , Leucemia/epidemiologia , Leucemia/mortalidade , Leucemia/terapia , Neoplasias/epidemiologia , Neoplasias/mortalidade , Neoplasias/terapia , Distúrbios Nutricionais/epidemiologia , Distúrbios Nutricionais/mortalidade , Fatores de Risco , Taxa de Sobrevida , Resultado do TratamentoRESUMO
OBJECTIVE: Osteonecrosis (ON) is a debilitating complication of cancer treatment in children and is usually associated with systemic steroid therapy. Defects of coagulation may be important in the pathogenesis of ON. This study evaluated the prevalence of factor V Leiden (FVL, 1691G-->A), the most common inherited thrombophilic state, the prothrombin 20210G-->A polymorphism, and the thermolabile methylene tetrahydrofolate reductase (MTHFR, 677C-->T) variant in a group of children in whom ON developed during or after treatment for cancer. STUDY DESIGN: Children in whom ON developed during cancer treatment at St Jude Children's Research Hospital were studied (n = 24). Genomic DNA was isolated, and polymerase chain reaction was performed to identify the FVL, prothrombin 20210, and thermolabile MTHFR mutations. RESULTS: Sixteen of 24 patients had acute lymphoblastic leukemia. The mean age at ON diagnosis was 14.4 +/- 3. 7 years. The mean interval between cancer diagnosis and ON diagnosis was 27 +/- 21 months. Twenty-two patients had received steroids for a mean duration of 24 +/- 15 weeks before having development of ON. No patient had a history of thrombosis. Five (21%) patients had a family history of thrombosis. Genetic analysis revealed 0 (0%) of 24 FVL, 1 (4.5%) of 22 prothrombin 20210, and 3 (13.6%) of 22 thermolabile MTHFR. None of these mutation frequencies was significantly different from our control frequencies or published values. CONCLUSIONS: Although procoagulant abnormalities in general and FVL in particular have been detected in a significant number of patients with ON of the jaw and Legg-Perthes disease, we did not identify an increased prevalence of FVL or other hypercoagulable state mutations in a cohort of children with ON that developed during or after treatment for a variety of cancers.
Assuntos
Fator V/análise , Neoplasias/sangue , Osteonecrose/sangue , Mutação Puntual/genética , Trombofilia/sangue , Adolescente , Criança , Intervalos de Confiança , DNA/sangue , DNA/genética , Fator V/genética , Feminino , Humanos , Masculino , Neoplasias/complicações , Neoplasias/tratamento farmacológico , Neoplasias/genética , Razão de Chances , Osteonecrose/epidemiologia , Osteonecrose/etiologia , Osteonecrose/genética , Reação em Cadeia da Polimerase , Prevalência , Trombofilia/epidemiologia , Trombofilia/etiologia , Trombofilia/genéticaRESUMO
OBJECTIVE: To determine whether abnormalities of the CNS are present in very young children with sickle cell anemia. STUDY DESIGN: Thirty-nine children with hemoglobin SS between the ages of 7 and 48 months were examined with magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). No child had a history of clinical stroke, although 3 had a history of seizures (2 neonatal). Twenty-one patients underwent developmental testing with the Bayley or McCarthy Scales. RESULTS: The overall prevalence of CNS abnormalities in asymptomatic children was 4 of 36 (11%, confidence interval 3, 26%). One patient had a silent infarct observed on MRI and a stenotic lesion on MRA; 3 other patients had stenotic lesions on MRA. The 3 patients who had a history of seizures all had lesions consistent with infarcts on MRI. Of the asymptomatic patients who had psychometric testing, 1 of 18 was developmentally delayed. One of 3 with a history of seizures had mild developmental delay. CONCLUSIONS: Very young children with sickle cell anemia (and no history of clinical stroke) have infarction in the brain and/or stenosis of major cerebral arteries, similar to those reported in older children. These findings indicate a need for larger studies to define the incidence of CNS lesions in this age group and to determine the need for early therapeutic intervention to prevent CNS sequelae of sickle cell disease.
Assuntos
Anemia Falciforme/complicações , Encéfalo/patologia , Doenças do Sistema Nervoso Central/etiologia , Doenças do Sistema Nervoso Central/diagnóstico , Doenças Arteriais Cerebrais/diagnóstico , Doenças Arteriais Cerebrais/etiologia , Infarto Cerebral/diagnóstico , Infarto Cerebral/etiologia , Comportamento Infantil , Pré-Escolar , Feminino , Humanos , Lactente , Comportamento do Lactente , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética , Masculino , Testes Neuropsicológicos , Testes PsicológicosRESUMO
Streptococcus pneumoniae sepsis is the most common invasive infection among patients with sickle cell disease. The risk of a recurrent episode of sepsis and subsequent death in those patients who have had a previous septic event is much higher. Patients with sickle disease who have had pneumococcal sepsis should continue penicillin prophylaxis indefinitely and should not be candidates for out-patient management of febrile episodes.
Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/tratamento farmacológico , Penicilinas/uso terapêutico , Infecções Pneumocócicas/etiologia , Sepse/etiologia , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Cooperação do Paciente , Infecções Pneumocócicas/mortalidade , Infecções Pneumocócicas/prevenção & controle , Recidiva , Fatores de Risco , Sepse/mortalidade , Sepse/prevenção & controle , Resultado do TratamentoRESUMO
OBJECTIVE: We investigated the possibility that antimicrobial-resistant pneumococci were causing invasive disease in children with sickle-cell disease (SCD). STUDY DESIGN: Records of all children with SCD observed at the Mid-South Sickle Cell Center (MSSCC) at LeBonheur Children's Medical Center were reviewed from January 1990 to June 1994. Children with SCD and pneumococcal sepsis were identified. The Streptococcus pneumoniae isolates from these children were examined for serotype and antimicrobial susceptibilities. Two additional children not observed in the MSSCC had pneumococcal sepsis caused by penicillin-resistant isolates and were also included. RESULTS: Antimicrobial susceptibility testing of the six penicillin-resistant isolates revealed that four were resistant to trimethoprim-sulfamethoxazole, two to erythromycin, and one to clindamycin. The two isolates that were resistant to ceftriaxone also were multiply resistant. From the MSSCC, 26 children had pneumococcal sepsis during the 4 1/2-year period studied. Five of these children (19%) died. Four (15%), including one who died, were infected with penicillin-resistant strains. CONCLUSION: Pneumococcal sepsis, meningitis, and infections of other foci in children with SCD may be caused by S. pneumoniae that is resistant to one or more antimicrobial agents, including penicillin. The addition of vancomycin to the antibiotics currently used for initial management should be considered in areas where the antibiotic resistance of S. pneumoniae is prevalent.
Assuntos
Anemia Falciforme/complicações , Antibacterianos/uso terapêutico , Cefalosporinas/uso terapêutico , Resistência Microbiana a Medicamentos , Meningites Bacterianas/etiologia , Penicilinas/uso terapêutico , Sepse/etiologia , Infecções Estreptocócicas/complicações , Infecções Estreptocócicas/tratamento farmacológico , Streptococcus pneumoniae/isolamento & purificação , Streptococcus pneumoniae/patogenicidade , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Salmonella/isolamento & purificação , SorotipagemRESUMO
We retrospectively examined the medical and autopsy records of seven previously unpublished cases of fatal pneumococcal septicemia in children with hemoglobin SC disease. The earliest death occurred in a 1-year-old child who had congenital heart disease with cyanosis; the other children were aged 3 1/2 to 15 years. Only one child had received pneumococcal vaccine or prophylactic penicillin therapy. All seven children had an acute febrile illness and rapid clinical deterioration despite parenterally administered antibiotic therapy and intensive medical support. Erythrocyte pit counts in two patients were 40.3% and 41.7%, respectively (normal, < or = 3.6%). Autopsy data from five cases showed marked splenic congestion without infarction in five, splenomegaly in four, and bilateral adrenal hemorrhage in three. These cases illustrate that functional asplenia predisposes some children with hemoglobin SC disease to the development of fatal septicemia after the age of 3 years. We conclude that pneumococcal vaccine should be administered to all children with hemoglobin SC disease and that acute febrile illnesses should be investigated promptly for the possibility of septicemia. The routine use of prophylactic penicillin therapy in infants and children with hemoglobin SC disease remains controversial.
Assuntos
Doença da Hemoglobina SC/complicações , Infecções Pneumocócicas/etiologia , Sepse/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
Although long-term transfusion therapy is at least 90% effective in preventing recurrent strokes after an initial cerebrovascular accident in patients with sickle cell disease, it is unknown how long transfusion therapy should be continued. To address this question, we prospectively discontinued transfusions in 10 patients with sickle cell disease whose median duration of transfusion therapy after an initial stroke was 9 1/2 years (range 5 to 12 years). Before the transfusions were discontinued, patients were examined by cerebral angiography, magnetic resonance imaging of the head, neuropsychologic testing, electroencephalography, and a complete neurologic examination. Within 12 months after transfusion therapy was stopped, 5 of 10 patients had had an ischemic event. Three events caused relatively mild deficits in the same areas as those originally affected. Two were associated with massive intracranial hemorrhage, including one on the contralateral side of original involvement. An additional patient died suddenly of unknown causes. Of the four remaining patients, three declined to resume transfusion and are relatively well at greater than or equal to 18 months after therapy was stopped. The studies performed before transfusions were stopped were not predictive of recurrent stroke. The risk of recurrent cerebrovascular accident in this group was significantly greater than the estimated risk of 10% in patients who are receiving long-term transfusion therapy (p = 0.002). This adverse outcome suggests that patients with sickle cell disease who have had a stroke must receive long-term transfusion indefinitely or a suitable therapeutic alternative must be devised.