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1.
J Pediatr Hematol Oncol ; 44(2): e493-e495, 2022 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-33560079

RESUMO

Paroxysmal nocturnal hemoglobinuria is a clonal hematopoietic stem cell disorder resulting in complement-mediated hemolysis. Eculizumab, a monoclonal antibody against complement protein C5, has been shown to reduce both intravascular hemolysis and risk for thrombosis, and thereby improve the quality of life in these patients. While the infection risk from Neisseria meningitidis due to terminal complement blockade can be mitigated with appropriate immunizations and prophylactic antibiotics, these patients remain vulnerable to infections from Neisseria gonorrhoeae. Physicians and families should be aware of disseminated and severe gonococcal infections in patients receiving complement blockade, especially in this era of emerging cephalosporin and azithromycin resistance.


Assuntos
Hemoglobinúria Paroxística , Qualidade de Vida , Complemento C5 , Proteínas do Sistema Complemento , Hemoglobinúria Paroxística/tratamento farmacológico , Hemólise , Humanos
2.
Pediatr Blood Cancer ; 66(6): e27642, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30724001

RESUMO

BACKGROUND: Children with sickle cell disease (SCD) are at increased risk for invasive infection with encapsulated bacteria. Antibiotic prophylaxis and immunizations against Streptococcus pneumoniae and Haemophilus influenzae type b (Hib) have decreased the overall incidence of invasive infections and have shifted distribution of serotypes causing disease toward those not covered by immunizations. We sought to determine the current incidence of invasive H. influenzae infections in children with SCD and to describe the clinical features and management of these infections. METHODS: Microbiology reports of a large pediatric tertiary care center were reviewed to identify all isolates of H. influenzae detected in sterile body fluid cultures from January 1, 2010 to December 31, 2017. Results were compared with the center's comprehensive clinical database of all children with SCD to identify all cases of children ages 0 to18 years with SCD with invasive H. influenzae disease for the same time period. RESULTS: We captured 2444 patients with SCD, with 14,336 person-years. There were eight episodes of H. influenzae bacteremia in seven children with SCD (five type f, two non-typable, one type a). Most episodes (7 of 8) were in children < 5 years. The incidence rate of invasive H. influenzae in SCD was 0.58/1000 person-years for ages 0 to 18 years and 1.60/1000 person-years for children age < 5 years. There were no deaths from H. influenzae infection. CONCLUSIONS: In the era of universal antibiotic prophylaxis and immunization against Hib, invasive H. influenzae disease due to nonvaccine serotypes remains a risk for children with SCD, particularly those under five years of age.


Assuntos
Anemia Falciforme/complicações , Infecções por Haemophilus/epidemiologia , Haemophilus influenzae/isolamento & purificação , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Georgia/epidemiologia , Infecções por Haemophilus/complicações , Infecções por Haemophilus/microbiologia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Prognóstico
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