RESUMEN
OBJECTIVE: To evaluate the frequency of primary immunodeficiencies (PID) in juvenile systemic lupus erythematosus (JSLE) patients. METHODS: Some 72 JSLE patients were analyzed for levels of immunoglobulin classes and IgG subclasses and early components of the classical complement pathway. Determination of C4 gene copy number (GCN) and detection of type I C2 deficiency (D) were also performed. RESULTS: PID was identified in 16 patients (22%): C2D in three, C4D in three, C1qD in two, IgG2D (<20 mg/dl) in four, IgAD (<7 mg/dl) in three, and IgMD (<35 mg/dl) in three; one of these patients presented IgA, C2 and C4D. Two patients had low C4 GCN and two had type I C2D. Demographic data, family history of autoimmune disease and PID, JSLE clinical findings, occurrence of infections, disease activity and therapies were similar in patients with and without PID (p > 0.05). Remarkably, the median of Systemic Lupus International Collaborating Clinics/ACR-damage index (SLICC/ACR-DI) was significantly higher in JSLE patients with PID compared with patients without these abnormalities (p = 0.0033), likewise the high frequency of SLICC/ACR-DI > 1 (p = 0.023). CONCLUSIONS: A high frequency of PID was observed in JSLE patients, suggesting that these defects may contribute to lupus development. Our findings indicate that these two groups of PID should be investigated in severe pediatric lupus.
Asunto(s)
Proteínas del Sistema Complemento/deficiencia , Síndromes de Inmunodeficiencia/complicaciones , Síndromes de Inmunodeficiencia/inmunología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/inmunología , Adolescente , Anticuerpos Antinucleares/sangre , Autoanticuerpos/sangre , Secuencia de Bases , Niño , Preescolar , Complemento C1q/antagonistas & inhibidores , Complemento C1q/deficiencia , Complemento C1q/inmunología , Complemento C2/deficiencia , Complemento C2/genética , Complemento C4/deficiencia , Complemento C4/genética , Proteínas del Sistema Complemento/genética , Cartilla de ADN/genética , Femenino , Dosificación de Gen , Humanos , Inmunoglobulinas/sangre , Inmunoglobulinas/clasificación , Síndromes de Inmunodeficiencia/genética , Lactante , Lupus Eritematoso Sistémico/genética , MasculinoRESUMEN
We report here a comparison of serum endostatin levels in Down syndrome patients to normal control subjects. We analysed serum samples from 35 patients with Down syndrome and 54 normal control subjects and found that although serum levels of endostatin vary widely in a normal human population, serum endostatin levels are significantly elevated in patients with Down syndrome. This result may explain the relative decrease in incidence of various solid tissue tumours observed in Down syndrome, given the role of endostatin as a potent inhibitor of tumour-induced angiogenesis in both human and animal models. Based upon these data, we propose that an increase of about one-third of normal endostatin serum levels may represent an effective therapeutic dose to significantly inhibit many solid tumours.
Asunto(s)
Colágeno/sangre , Síndrome de Down/sangre , Fragmentos de Péptidos/sangre , Adolescente , Adulto , Niño , Preescolar , Síndrome de Down/genética , Síndrome de Down/prevención & control , Endostatinas , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , MasculinoRESUMEN
Genetic variation of 33 enteroinvasive Escherichia coli (EIEC), 12 non-EIEC and 39 Shigella strains (representing the 4 species of this genus) was analyzed using the random amplified polymorphic DNA (RAPD) technique. Reproducible polymorphisms were generated and the combined data allowed us to construct a dendrogram using Jaccard's distance. Two main groups were obtained: one for Shigella and the other for EIEC and non-EIEC strains. The first group contained four clusters, one for each Shigella species. The second group contained one cluster for EIEC and another for non-EIEC strains. The main clusters encompassed many small clusters corresponding to different serotypes. It was possible to characterize each one of the 84 strains under study as well as the boundaries among Shigella species and between this genus and EIEC strains.
Asunto(s)
Escherichia coli/genética , Polimorfismo Genético/genética , Shigella/genética , Técnicas de Tipificación Bacteriana , Dermatoglifia del ADN , ADN Bacteriano/genética , Electroforesis en Gel de Agar , Escherichia coli/clasificación , Variación Genética , Humanos , Técnica del ADN Polimorfo Amplificado Aleatorio , Shigella/clasificaciónRESUMEN
Genomic diversity among 34 strains of Escherichia coli belonging to different serotypes of the O26 serogroup -- encompassing strains from different geographical origins and Shiga toxin-negative Brazilian strains -- was evaluated through random amplified polymorphic DNA (RAPD) analysis. Our results indicate that Brazilian and non-Brazilian O26 strains fall under distinct but closely related differentiation clusters. RFLP-PCR analysis of the fliC gene sequence was done in order to identify the H(-) serotypes and served to confirm the clustering pattern obtained in the dendrogram generated from RAPD data. The epidemiological significance of these data is discussed.
Asunto(s)
Adhesinas Bacterianas , Proteínas Portadoras , Proteínas de Escherichia coli , Escherichia coli/clasificación , Escherichia coli/genética , Proteínas de la Membrana Bacteriana Externa/genética , Técnicas de Tipificación Bacteriana , Brasil , Escherichia coli/aislamiento & purificación , Escherichia coli/patogenicidad , Infecciones por Escherichia coli/microbiología , Flagelina/genética , Variación Genética , Humanos , Filogenia , Reacción en Cadena de la Polimerasa/métodos , Polimorfismo de Longitud del Fragmento de Restricción , Técnica del ADN Polimorfo Amplificado Aleatorio/métodos , Serotipificación , Toxina Shiga I/genética , Toxina Shiga II/genética , Toxinas ShigaRESUMEN
It is difficult to use tissue culture assays to investigate adherence and other properties of Edwardsiella tarda because the organism is invasive and produces a potent hemolysin. We therefore relied on polymerase chain reaction (PCR) to determine the occurrence of genes for enterotoxins (LT-I, EAST-1), Shiga toxin (Stx-1, Stx-2), cytotoxic necrotizing factors (CNF-1, CNF-2), aerobactin, invasion plasmid of enteroinvasive Escherichia coli, EPEC adherence factor (EAF), intimin (Eae), enterohemolysin (EntHly) and hemolysin (Hly) in 53 isolates of E. tarda from humans and fish from several countries. All isolates were negative for all genes investigated by PCR. Adhesion to and invasion of HeLa cells were determined by using the unusually short incubation time of 1h or 30 min. All isolates adhered and invaded in these tests. Finally, a random amplified polymorphic DNA (RAPD) test distinguished, with a few exceptions, isolates of human and fish origin.