Activated phosphoinositide 3-kinase δ syndrome / 中华实用儿科临床杂志

Activated phosphoinositide 3-kinase δ (PIK3CD) syndrome is a combined immunodeficiency,caused by PIK3CD gain-of-function mutations,which encodes the catalytic subunit of PIK3CD.These patients presented with early onset sinopulmonary infections,lymphoproliferation,herpesvirus infections,autoinflammatory disease,lymphoma and mental retardation.Hyper immunoglobulin (Ig)M,IgG deficiency,CD4 lymphopenia were common immunologic features.Variable expression can lead to death and asymptom.Penetrance can be incomplete.Hematopoietie stem cell transplantation should be taken into account for severe cases.

Papulosis linfomatoide. Presentación de un caso / Lymphomatoid papulosis. Report of a case

La papulosis linfomatoide forma parte del espectro de los procesos linfoproliferativos cutáneos primarios de células T CD30+. Es una enfermedad rara de etiopatogenia incierta y compleja. El diagnóstico diferencial puede a veces resultar muy difícil. Se describió el caso de una mujer de 80 años con el diagnóstico, particularmente atípico desde la visión histopatológica, en el cual la correlación anatomoclínica ha sido un importante aspecto que lo hace interesante. El objetivo es comunicar un caso de presentación poco frecuente en la práctica médica (AU).

Lymphomatoid papulosis is part of the primary skin lymph proliferative processes of the T CD30+ cells. It is a rare disease of complex and uncertain etiopathogenesis. The differential diagnosis could be very difficult sometimes. The described case was the one of a female patient, aged 80 years with that diagnosis, particularly atypical from the histopathological point of view, where the anatomoclinical correlation has been an important aspect making it interesting. The objective is to inform a case of infrequent presentation in the medical practice (AU).

Obtenção de proteína recombinante baseada em antígenos do líquido vesicular de Taenia crassiceps: aplicação no imunodiagnóstico da neurocisticercose / Recombinant protein obtainment based on antigens from Taenia crassiceps vesicular fluid: application on immunodiagnostics of neurocisticercosis

A neurocisticercose (NC) é uma doença provocada por larvas de Taenia solium (Tso) no sistema nervoso central. Seu diagnóstico fundamenta-se em critérios clínicos, epidemiológicos e laboratoriais. A utilização de antígenos parasitários no imunodiagnóstico apresenta desvantagens como: necessidade de animais, ausência de homogeneidade entre lotes, baixo rendimento, e contaminação com proteínas suínas. Assim, os antígenos recombinantes podem otimizar o imunodiagnóstico da NC, pois são reagentes simples e reprodutíveis, sem requerer animais. Este estudo teve como objetivo a obtenção, caracterização e análise da reatividade de proteína recombinante baseada em antígenos de líquido vesicular de Taenia crassiceps (Tcra). Assim, o cDNA foi obtido por amplificação a partir de RNAm de cisticercos de Tcra. A proteína recombinante Tc14 foi produzida em Escherichia coli (DE3) BL21 utilizando-se o vetor de expressão pET-22b e purificada por cromatografia de afinidade. A caracterização antigênica deu-se por Imunoblot (IB) utilizando anticorpos monoclonais (AcMo). Houve reatividade com todos os AcMo utilizados (AcMo anti-antígeno de excreção/secreção de Tcra, AcMo anti-líquido vesicular de Tcra, AcMo antilíquido vesicular de Tso e AcMo anti-antígeno total de Tso), exceto com o AcMo anti-antígeno de escólex de Tso. Utilizando-se 22 amostras de soro e 19 de líquor (LCR) de pacientes com NC, 48 soros e 28 LCR do grupo controle negativo (GCN) e 17 soros de hidatidose do grupo outras parasitoses (OP) em Imunoblot foi observada reatividade na região de 14kDa, correspondente a Tc14, em todas as amostras NC, mas não nos GCN e OP. Em ELISA com Tc14 obteve-se sensibilidade (S) e especificidade (E) de 100% com LCR (29 amostras de NC e 35 do GCN) e S de 95,1% e E de 100% com soro (41 amostras de NC, 52 do GCN). Dentre 51 soros de OP, mostraram-se reagentes um de hidatidose e outro de estrongiloidíase. A análise comparativa entre diferentes antígenos e testes sorológicos apresentou índice...

The neurocisticercosis (NC) disease is caused by the presence of Taenia solium (Tso) larvae in the central nervous system. Its diagnosis is based on clinical criteria, epidemiological studies and laboratorial exams. Nevertheless, the use of parasite antigenic extracts into the immunodiagnosis presents some disadvantages: it requires animals, lacks of homogeneity between lots, low yield and may become contaminated with swine proteins. Consequently, the utilization of recombinant antigens could optimize the immunodiagnostic of NC, as they are simple and reproducible reagents that do not require animals. This study aimed the capture, characterization and reactivity analysis of the recombinant protein based on antigens of the vesicular fluid of Taenia crassiceps (Tcra). In order to do so, the cDNA was obtained through the amplification deriving from RNAm of cysticerci of Tcra. The recombinant protein Tc14 was produced in Escherichia coli (DE3) BL21 using the expression vector pET-22b and purified by affinity chromatography (nickel resin). The antigenic characterization was performed by immunoblotting (IB) using monoclonal antibodies (MoAb). The recombinant protein presented reactivity with all the MoAb used (Anti-secretion/excretion antigens from Tcra MoAb, anti-vesicular fluid from Tcra MoAb, anti-vesicular fluid from Tso MoAb and anti- total antigen from Tso MoAb), except with the anti-antigen from Tso scolex MoAb. The immunoblot was performed using 22 serum samples and 19 cerebrospinal fluid (CSF) from patients with NC, 48 serum and 28 CSF from the negative control group (GCN) and 17 hydatidosis serum from other parasitosis' group (OP). It showed reactivity in the 14kDa region, correlated to Tc14, in all NC samples, but not presented on GCN and OP. In ELISA with Tc14, the sensibility (S) and specificity (E) of 100% was obtained with CSF (29 NC samples and 35 GCN samples) and 95.1% of S and 100% of E with serum (41 NC samples, 52 GCN samples)...

Effect of Echinacea purpurea (Asteraceae) aqueous extract on antibody response to Bothrops asper venom and immune cell response

The effect of aqueous extract of Echinacea purpurea roots on the murine antibody response to Bothrops asper snake venom in vivo was studied. Three groups were used. Group #1, baseline control, was treated with snake venom plus PBS. Group #2 was treated with snake venom plus sodium alginate as adjuvant (routine method used at Instituto Clodomiro Picado), and group #3 or experimental group, was treated with snake venom plus aqueous extract ofE. purpurea root as adjuvant. In all groups, the first inoculation was done with Freund's complete adjuvant (FCA). By the time of the second bleeding, mice in group #3 showed a remarkable increment in the level of anti-venom antibodies compared with those in groups #1 or #2. In vitro immune cell proliferation as a response to aqueous extract of E. purpurea root was studied using human lymphocytes activated with different lectins (Con A, PHA and PWM). In all cases, increase in percentage of lymphoproliferation was greater when E. purpurea root extract was used in addition to individual lectins.

Impairment of lymphoproliferative response to human herpesvirus-6 in oral squamous cell carcinoma patients / 基础医学与临床

Objective To study the lymphoproliferative responses to HHV-6(Human Herpesvirus-6,HHV-6)among patients with oral squamous cell carcinoma,and to discuss the role of HHV-6 in the pathogenesis of oral squamous cell carcinoma.Methods IgG antibody to HHV-6 in plasma was identified by indirect immunofluorescence(IIF).Immuno-magnetic beads were used to prepare CD4+ T cells and CD8+ T cells.The proliferation of CD4+ T cells,CD8+ T cells and PBMCs stimulated with HHV-6 or anti-CD3 antibodies was measured by 3H-thymidine uptake.The percentage of CD4+ C25+ Treg cells(Regulatory T cell,Treg)within the peripheral blood CD4+ T cell compartment was analyzed by flow cytometry.Results Significantly higher proportion of patients with oral squamous cell carcinoma had IgG antibody to HHV-6(8/8) in plasma as compared with those in control subjects(12/20);The proliferative responses of PBMCs and CD4+ T cells from patients with oral squamous cell carcinoma to HHV-6 were significantly decreased as compared to those from HHV-6-infected and healthy individuals(P

Inhibition of mouse and rat lymphoproliferation by gangliosides

Our previous study have demonstrated that Glycosphingolipids (GSLs) have an immunosuppressive effect on murine lymphoproliferation and IL-2 production. In the present study we examined the effect of a pool of Gangliosides (Gang) on spleen lymphocyte proliferation from either isogeneic strains of Wistar rats or BALB/c mice. Two hundred-fifty grams adult female isogeneic Wistar rats and 8-week-old BALB/c mice were used. The animals were sacrificed and the spleen harvested aseptically for cellular assays. Spleen cells suspensions were obtained by homogenization in RPMI 1640 with a loose tissue grinder. After washing, the cells were suspended in RPMI 1640 supplemented. Cell viability was measured by Trypan blue exclusion. Cells were cultured in triplicate using increasing concentrations of Gang (1; 2; 5; 10; 15; 20 mug/well) and in the presence of Concanavalin A. The cells were incubated for 48 hours and were pulsed with [³H] thymidine 18 hours prior to harvesting on glass fiber paper for counting in a beta-counter. Data were presented as rate of inhibition, as previously described. At concentrations 1 and 2 mug/well, Gang stimulated lymphoproliferation (30% and 50%, rats and mice respectively), while at concentration from 5 to 20 mug/well an increasing inhibition was observed for spleen cells from both mouse and rat (from 40% up to 80%). In preliminary studies we observed inhibition of mixed lymphocyte reaction on spleen cells from rats treated with Gang for 10 days (data not shown). Our data suggest that Gang may be investigated as a immunosuppressive drug in organ transplantation.