Decreased number of regulatory T lymphocytes is related to inflammationand number of CD8+ T cells expressing programmed cell death protein-1 in common variable immunodeficiency

Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder related to recurrent infections, as well as a range of non-infectious manifestations including autoimmune and inflammatory disorders. We hypothesized that patients with CVID and different clinical phenotypes would demonstrate alterations in lymphocyte T subsets, including T lymphocytes expressing programmed cell death protein 1 (PD-1), and regulatory T lymphocytes. We performed flow cytometry in two CVID groups: group 1 with infections only, and group 2 with infections and concomitant noninfectious manifestations. Patients were 18–59 years old (mean 35.8 years of age). Increased proportions of CD8+PD-1+ T cells and reduced regulatory T cells were associated with lymphadenopathy. Amount of regulatory T cells correlated with CD8+PD-1+ T lymphocytes (r = 0.54; p = 0.013), and with CRP (r = –0.64; p = 0.004). Forty percent of patients expressed manifestations in addition to infections (group 2), and they had reduction in number of regulatory T cells [8 (3-12) vs. 24 (11-26)/μl; p = 0.034), naive CD4+ T lymphocytes [36 (27-106) vs. 149 (81-283)/μl; p = 0.034], and elevated C-reactive protein (CRP) [5.33 (3.15-8.82) vs. 1 (1-2.16) mg/l; p = 0.003] in comparison to group 1. In conclusion, the amount of CD8+ T cells expressing PD-1 is associated with lymphadenopathy and number of regulatory T cells in patients with CVID. Patients with CVID and non-infectious complications have increased level of inflammation and alterations in regulatory T cells

Two siblings with an IL-12 and IFN-γ production disorder diagnosed with pulmonary mycobacteriosis caused by M. kansasii. Mendelian susceptibility to mycobacterial infection. An overview of literature

W pracy przedstawiono dwa przypadki wystąpienia mykobakteriozy płuc w przebiegu zakażenia M. kansasii u dotychczas zdrowego rodzeństwa. W wyniku przeprowadzonych badań u obojga chorych stwierdzono brak wydzielania interleukiny 12 (IL-12) i interferonu γ (IFN-γ) przez stymulowane limfocyty. Opisywany wcześniej defekt genetyczny warunkuje występowanie wrażliwości typu Mendla na zakażenie prątkami (MSMD). Pacjenci spełniali kryteria kliniczno-radiologiczne i bakteriologiczne rozpoznania mykobakteriozy opracowane przez American Thoracic Society w 2007 roku. Po 13 miesiącach typowego leczenia uzyskano ustąpienie dolegliwości, remisję radiologiczną oraz utrzymujące się przez 12 miesięcy odprątkowanie. Rokowanie pacjentów pozostaje niepewne. Genetycznie uwarunkowana skłonność do zakażeń prątkami może spowodować wznowę choroby lub być przyczyną nowego zachorowania na choroby wywołane przez prątki. Pacjenci będą wymagali systematycznych kontroli pulmonologicznych. Nie jest jasne, czy u potomstwa opisanych chorych istnieje możliwość uogólnionego zakażenia prątkami BCG (Baccillus Calmette-Guérin) w wyniku obowiązkowego szczepienia noworodków i czy ryzyko powikłań poszczepiennych jest większe niż ewentualne ryzyko zachorowania na gruźlicę, zwłaszcza krwiopochodną, w dzieciństwie. Pneumonol. Alergol. Pol. 2011; 79, 6: 428–436Two previously healthy siblings were diagnosed with pulmonary mycobacteriosis caused by M. kansasii. During examination both patients were diagnosed with an interleukin 12 (IL-12) and interferon γ (IFN-γ) production disorder of the stimulated lymphocytes. The given genetic defect conditions the occurrence of the Mendelian susceptibility to mycobacterial infection (MSMD). The patients fulfilled clinical, radiological, and bacteriological criteria for diagnosis of mycobacteriosis laid out by American Thoracic Society in 2007. After 13 months of standard treatment the ailments receded, and radiological remission, as well as a 12-month-lasting sputum negativity was achieved. The prognosis for the patients remains uncertain. The genetic conditioning to mycobacterial infections may cause disease recurrences or other mycobacterial illnesses. The patients will need to be checked systematically by pulmonologist. It is not known whether the offspring of the patients are exposed to general Baccillus Calmette-Guérin infection due to the compulsory vaccinations against tuberculosis, and whether the risk of complications is higher than the potential risk of coming down with hematogenous TB in childhood. Pneumonol. Alergol. Pol. 2011; 79, 6: 428–43

Elevated Asymmetric Dimethylarginine in Young Adult Survivors of Childhood Acute Lymphoblastic Leukemia: A Preliminary Report

TesisEn este trabajo se revisan algunos aspectos metodológicos, conceptuales y operativos relacionados con la calidad de servicio e influencia en la satisfacción de clientes en los restaurantes de la ciudad de Ayacucho. La importancia es la mejora de la calidad de sus actividades es uno de los retos más importantes que actualmente deben afrontar todas las empresas y en especial los responsables de su dirección y gestión, por lo cual el presente trabajo de investigación es de importancia porque me permitirá determinar la influencia de las dimensiones de la calidad de servicio en la satisfacción de los clientes de los restaurantes en la ciudad de Ayacucho. También se consigna los resultados, luego de haber procesado los datos se presentan en tablas y gráficos, habiéndose realizado el análisis de los mismos, contrastándose las hipótesis. Se incluye las conclusiones y recomendaciones del trabajo de manera somera

Efficacy and Safety of Human Intravenous Immunoglobulin 10% (Panzyga®) in Patients with Primary Immunodeficiency Diseases : a Two-Stage, Multicenter, Prospective, Open-Label Study

PurposeTo assess the efficacy and safety of panzyga® (intravenous immunoglobulin 10%) in preventing serious bacterial infections (SBIs) in patients with primary immunodeficiency diseases (PIDs), a prospective, open-label, multicenter, phase 3 study and an open-label extension study were undertaken.MethodsInitially, the study drug (infusion rate ≤0.08 mL/kg/min) was administered at intervals of 3 or 4 weeks for 12 months, followed by 3 months of panzyga® at infusion rates increasing from 0.08 to 0.14 mL/kg/min. The primary endpoint in the main study was the rate of SBIs per patient-year on treatment. Secondary outcomes included non-serious infections, work/school absence, episodes of fever, quality of life, and adverse events (AEs).ResultsThe main study enrolled 51 patients (35% female, mean age 26.8 years), with 21 participating in the extension study. The rate of SBIs per patient-year was 0.08 in the total population; there were four SBIs in the 4-weekly treatment group (2/30 patients) and none in the 3-weekly group (n = 21). Compared with 4-weekly treatment, 3-weekly treatment was associated with a higher rate of upper respiratory tract infections (RTIs), ear infections, and work/school absences, but a lower rate of lower RTIs and fever. Treatment was generally well tolerated; no AE led to treatment withdrawal or death.ConclusionsOverall, the use of panzyga® in patients with antibody-deficient PID was associated with a low rate of AEs and was effective in preventing SBIs, exceeding US FDA and European Medicines Agency recommendations for efficacy

Vaccination in autoimmune inflammatory rheumatic diseases patients

Pacjenci z autoimmunologicznymi zapalnymi chorobami reumatycznymi (AZCR) mają 2–4-krotnie zwiększone ryzyko infekcji w porównaniu z ogólną populacją. Zwiększona podatność na infekcje tej grupy związana jest z zaburzeniami systemu odpornościowego, które towarzyszą chorobie podstawowej oraz oddziaływaniem immunomodulującym leczenia (leki modyfikujące przebieg choroby niebiologiczne i biologiczne, glikokortykosteroidy). Niniejsza praca stanowi przegląd literatury światowej z lat 2000–2012, poruszającej problem bezpieczeństwa i skuteczności immunoprofilaktyki czynnej u dorosłych pacjentów z zapalnymi chorobami reumatycznymi.Patients with autoimmune inflammatory rheumatic diseases (AIIRD) have a two to four times greater risk of infection than the general population. The increased infection risk can be attributed to both the immunosupresive effect of AIIRD and the use of immunomodulatory treatment (biological and non-biological disease-modifying antirheumatic drugs; corticosteroids). The presented paper summarizes the results of international publications in the years 2000 and 2012 for safety and efficacy of vaccination in adult patients with autoimmune inflammatory rheumatic diseases