RESUMO
We describe 5 children from 2 families with mutations in the CD40 ligand (CD40L) gene leading to absent expression of CD40L on activated CD4 cells. All subjects presented with interstitial pneumonia with low serum IgG and normal serum IgM. One child had normal and one child had elevated serum IgA. Four had confirmed Pneumocystis carinii pneumonia. In spite of intravenous immunoglobulin treatment yielding therapeutic serum immunoglobulin levels, 3 children had enteroviral encephalitis. When assessed by flow cytometry, the 3 surviving affected male children had absent CD40L expression on activated CD4(+) T cells. The affected children from both families were shown to have the same single nucleotide insertion (codon 131) resulting in frameshift and early termination within exon 4 (extracellular domain). This observation demonstrates that persistent enteroviral infection is not only observed in X-linked agammaglobulinemia but may also occur in patients with X-linked hyper IgM syndrome.
Assuntos
Linfócitos T CD4-Positivos/imunologia , Antígenos CD40/imunologia , Infecções por Enterovirus/etiologia , Hipergamaglobulinemia/complicações , Hipergamaglobulinemia/genética , Imunoglobulina M/sangue , Glicoproteínas de Membrana/genética , Meningoencefalite/etiologia , Mutação , Ligante de CD40 , Análise Mutacional de DNA , Citometria de Fluxo , Ligação Genética , Humanos , Hipergamaglobulinemia/terapia , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Imunoglobulinas Intravenosas/uso terapêutico , Lactente , Ligantes , Ativação Linfocitária , Masculino , Linhagem , Pneumonia por Pneumocystis/complicações , Reação em Cadeia da Polimerase , Síndrome , Cromossomo XRESUMO
To investigate the causes and clinical characteristics of acute pharyngitis among school-aged children (4 to 18 years), we obtained throat cultures for respiratory viruses, Mycoplasma pneumoniae, group A streptococcus, and Chlamydia trachomatis from 320 patients with sore throat and 308 controls without respiratory complaints. The study was conducted from January to April 1985 in a private pediatric practice in central New York State. Sixty percent of the patients and 26% of the control subjects had positive cultures for at least one organism. Forty percent of patients had positive cultures for group A streptococcus, compared with 11.9% of the controls. Fifty (16%) patients had positive viral cultures, compared with eight (2.6%) controls; the predominant viral isolate was influenza A Philippines. Patients infected with influenza A were significantly more likely to complain of cough and hoarseness, and were less likely to have pharyngeal exudate or tender cervical adenopathy, than were patients who had positive cultures for group A streptococcus. Although 49 (15.8%) patients with acute pharyngitis had cultures positive for M. pneumoniae, 53 (17.6%) asymptomatic controls were also had M. pneumoniae-positive cultures. Thus detection of M. pneumoniae in the throat of school-aged children with pharyngitis may not be sufficient to establish a diagnosis of disease caused by this organism. C. trachomatis was not isolated from any patient or control.
Assuntos
Infecções por Chlamydia , Faringite/etiologia , Pneumonia por Mycoplasma/complicações , Infecções Estreptocócicas , Viroses , Doença Aguda , Adolescente , Criança , Pré-Escolar , Chlamydia trachomatis/isolamento & purificação , Tosse/etiologia , Feminino , Rouquidão/etiologia , Humanos , Vírus da Influenza A/isolamento & purificação , Influenza Humana/complicações , Masculino , New York , Faringite/microbiologia , Faringe/microbiologia , Estações do Ano , Fatores Socioeconômicos , Streptococcus pyogenes/isolamento & purificaçãoRESUMO
In a May, 1975, outbreak, 147 adolescents, ages 12 to 19 years, were identified as having measles by a physician or school nurse. One junior high school, with an enrollment of 1,122, contributed 131 of the cases. Of the 147 students, 54 were seen by physicians who also supplied their immunization records; 19 of 54 (35%) had received live measles virus vaccine without measles immune globulin, after age one year. The remaining 35 received: killed virus vaccine only (1), K + L (4), L + MIG (4), L at less than 1 year of age (4), L + ? MIG (4), immune serum globulin only, for exposure (6), no vaccine but history of measles previously (9); history uncertain (3). Hemagglutination-inhibition antibody titers were consistent with the diagnosis of acute measles in 11 children. No index case was identified and no secondary cases occured within the families of the 54 cases. This measles outbreak among seemingly immunized adolescents raises a serious question as to the duration of such protection.
Assuntos
Surtos de Doenças , Sarampo/epidemiologia , Adolescente , Adulto , Criança , Estudos de Avaliação como Assunto , Testes de Hemaglutinação , Humanos , Imunização , Sarampo/diagnóstico , Sarampo/prevenção & controle , Vacina contra Sarampo/uso terapêutico , New YorkRESUMO
Eight patients developed elevations of hepatic enzymes while receiving oxacillin intravenously. In all instances the patients were asymptomatic and anicteric. Peripheral eosinophilia was present in five of eight patients. In each patient change of therapy to a different pencillinase-resistant penicillin or to penicillin G was associated with a rapid decrease in liver function abnormalities and evenutal return of the enzymatic values to normal. Change of medication to an alternative penicillinase-resistant penicillin or to penicillin G is suggested as a safe procedure for completion of antistaphylococcal therapy in patient who develop oxacillin-related hepatotoxicity.