Haemodialysis patients on warfarin should have very close monitor

Haemodialysis patients on warfarin should have very close monitoring of INR in dialysis units and the use of heparin for dialysis should be done very thoughtfully. “
“Aim:  Renal interstitial fibrosis is the final common pathway determining long-term prognosis of chronic kidney diseases, but its repair process is scarcely understood. Because recent reports indicate that M2 macrophages play important roles in the repair of various tissues, special attention was paid to the phenotypes of infiltrating macrophages in the present study when the histological changes occurring in mouse kidneys after the release of unilateral ureteral obstruction (UUO) inducing renal fibrosis were

analyzed. Methods:  The left ureter of male mice was obstructed for 10 days by using a vascular clamp, and that kidney was removed for analysis either on the day when the clamp was removed or Regorafenib after the kidney had been allowed to recover for 3, 7 or 21 days. Results:  Interstitial fibrosis assessed by picrosirius red staining decreased with time after the release, and this decrease was paralleled by a decrease in the interstitial area positive for α-smooth muscle actin. Macrophage

infiltration assessed by F4/80 staining also significantly decreased from day VX809 3. In contrast, real-time reverse transcription polymerase chain reaction revealed that the ratios of mRNA for the macrophage scavenger receptor (CD204) and the mannose receptor (CD206), both of which are preferentially expressed on M2 macrophages, to CD68 (a general macrophage marker) were significantly greater on day 7

than on day 0 in the UUO-released mice. Conclusion:  Although the total number of infiltrating myofibroblasts and macrophages decreased after UUO release, the ratios of macrophages expressing CD204 and CD206 increased, suggesting that M2 macrophages play an important role in the repair of renal fibrosis. “
“Aim:  Studies from the US have shown little effect of ethnicity on vascular calcification in dialysis patients. This has not been examined in the multi-ethnic population of South Africa where genetic and environmental OSBPL9 differences may exist. We assessed the extent and severity of vascular calcification in South African dialysis patients according to race and known risk factors. We further evaluated the association of abdominal aorta calcification with coronary artery calcification. Method:  Seventy-five CKD-5D patients and 20 healthy controls were enrolled consecutively. All subjects underwent chest computed tomography for coronary calcium score and abdominal X-ray for abdominal aorta calcium score. Ambulatory blood pressure monitoring was generated via radial artery applanation tonometry. Results:  Coronary calcification was present in 38.6% of patients and was associated with age and prior cardiovascular disease on multivariate analyses.

The native IgAN of the present case was undistinguished histologi

The native IgAN of the present case was undistinguished histologically; however, the clinical manifestations were significant. A few reports[16-18] show that crescent IgAN may lead to early graft failure. However, the fourth biopsy performed 195 days after kidney transplantation in our patient showed that cellular crescent was observed in only 1 of Small molecule library cell line 21 glomeruli (4.8%). In addition, the patient developed nephrotic-range proteinuria, had renal function deterioration, and showed refractory IgAN despite the

common IgAN histology. It is well known that high levels of serum soluble urokinase plasminogen activator receptor (suPAR) can be found as a circulating factor in some patients with primary focal segmental glomerulosclerosis. Such circulating factors have not yet been reported in IgAN patients. However, our case of early IgAN recurrence cast a new light on the possible existence of a circulating factor in IgAN patients. “
“Introduction: Clostridium difficile-associated diarrhoea (CDAD) is the most common cause of nosocomial diarrhoea in the USA. In this study, we sought Sirolimus datasheet to determine the association between chronic kidney disease (CKD) and CDAD. Methods:  A case–control study was designed to determine the association between CKD and CDAD in an urban hospital. Over a 2-year period, all patients diagnosed with CDAD (n = 188) were included

as cases and the prevalence of CKD was calculated. Age- and sex-matched patients without CDAD were considered as controls with a ratio of 2:1 controls to cases. The prevalence of different stages of advanced CKD (stages 3–5) was determined and compared between groups. Also the calculated odds ratios (OR) were adjusted for multiple possible confounding variables using logistic regression analysis. Results:  There was no significant difference in prevalence of advanced CKD between cases and controls (OR = 1.38, 95% confidence intervals (CI) = 0.90–2.12, P = 0.1365). PTK6 The association between CKD and CDAD remained insignificant in subjects with CKD stages 3–5 who were not on dialysis (OR = 1.07, 95% CI = 0.65–1.77), P = 0.7970).

However, the group with end-stage renal disease on dialysis showed a significant association (OR = 2.60, 95% CI = 1.25–5.41, P = 0.0165). Controlling for antibiotics as a possible confounding variable, yielded an OR that was not statistically significant (OR = 2.05, 95% CI = 0.94–4.47, P = 0.07), but still showing a trend towards increased risk. Conclusion:  End-stage renal disease may increase the risk of acquiring CDAD through unknown mechanisms. This suggests implementing better surveillance strategies for these patients and eliminating the known risk factors for CDAD. “
“Aim:  Children with steroid-dependent nephrotic syndrome (SDNS) need long-term steroid usage to maintain sustained remission.

It is well

known that the inflammatory response inhibits

It is well

known that the inflammatory response inhibits fibrinolysis, which contributes to the prothrombotic state seen in conditions such as sepsis [16], inflammatory bowel diseases [17] and rheumatoid arthritis [18]. However, to the best of our knowledge, no data are available concerning systemic fibrinolysis in BP patients, although it has been shown to be involved at local level BMS-777607 order in lesional skin in humans and experimental BP models [19-23]. With this background, we evaluated systemic fibrinolysis by measuring the plasma parameters of 20 patients with BP in an active phase and in clinical remission after systemic corticosteroid treatment, and correlated the results with coagulation

markers and the parameters of disease activity. We conducted an observational study enrolling 20 consecutive patients with previously untreated active BP (10 males and 10 females; mean age 76 years, range 53–99) who were admitted to our Dermatology Department from January 2010 to June 2011. The diagnosis of BP was established on the basis of clinical and immunopathological criteria. All the patients had a clinical picture of generalized BP without any mucous membrane involvement Everolimus cell line (mean disease duration: 1 month, range 0–2); the skin lesions (vesiculobullous and/or erythematous–oedematous lesions) covered a median 40% of total body area (range 20–60%). Direct immunofluorescence examinations of the perilesional skin revealed the linear deposition of IgG and/or C3 in the BMZ in all cases, Reverse transcriptase circulating anti-BP180 autoantibodies were detected by means of an ELISA. Concomitant neoplastic or inflammatory diseases were excluded on the basis of clinical and instrumental examinations. None of the patients had thyroid dysfunction or atrial fibrillation and were taking drugs affecting coagulation. Three of the 20 BP patients had type 2 diabetes and were receiving treatment with oral anti-diabetic drugs with an acceptable

disease control (haemoglobin A1c values 6·5, 6·7 and 7·0, respectively). After taking the blood samples, patients with active disease were treated with methylprednisolone at an initial dose of 0·5–0·75 mg/kg/day. When either new lesions or pruritic symptoms have not occurred for at least 2 weeks, the tapering of steroid was started until reaching the minimal dose of 0·05–0·1 mg/kg/day. All the patients were also studied during clinical remission, defined as the absence of any new BP lesions with the complete healing of the previous lesions for a minimum of 4 weeks. At the time of sampling, they were being treated with low-dose corticosteroids (methylprednisolone 4 mg daily). The control group consisted of 20 age- and sex-matched apparently healthy subjects with no history of thrombosis (10 males and 10 females; mean age 75 years, range 55–94).

13 Although incompletely documented, non-human primates appear to

13 Although incompletely documented, non-human primates appear to possess subpopulations of dendritic cells (DCs) and B cells that are similar

to those present in humans.14,15 Non-human primates are therefore valuable for studies aimed at investigating immune responses induced by human pathogens and vaccine components aimed for human use.16,17 Several reports indicate that TLR ligands show potency as vaccine adjuvants when tested in rhesus macaques18–20 or in human clinical trials.21–23 Subsets of human DCs and B cells express distinct repertoires of TLRs and they respond to TLR stimulation accordingly.2,24,25 Unlike rodents, rhesus macaques express a similar repertoire of TLRs on immune cells such as DCs and B cells as humans.26 Some differences between the human and rhesus macaque immune systems have been reported.17 An improved understanding about similarities selleckchem and disparities between human and non-human primate immune functions is therefore important and would provide valuable information for translating non-human

primate studies for the design of clinical trials aimed at testing new vaccine and treatment strategies. In this study, we performed a side-by side comparison of the phenotypes of human and rhesus DCs and B cells and we examined their responsiveness to well-defined ligands targeting TLR3, 7/8, and 9. We further asked if IFN-α comparably enhanced B-cell functions such as proliferation and differentiation into antibody-producing cells as Rapamycin nmr observed in culture systems of human cells. We found similar responses in human and rhesus primary cell cultures to TLR ligand stimulation in terms of B-cell proliferation and induction of IFN-α production by pDCs. In both species, B-cell proliferation to the TLR7/8 ligand (-L) and CpG class C showed a significant increase in the presence of IFN-α. Some phenotypic differences between human and rhesus B cells were observed as the cells differentiated

CHIR-99021 ic50 into antibody-producing cells, although in both species TLR stimulation promoted maturation of B cells into IgM-producing cells and this effect was enhanced in the presence of IFN-α. Untreated and healthy rhesus macaques of Chinese origin, 5–6 years old, were housed in the Astrid Fagraeus laboratory at the Swedish Institute for Infectious Disease Control. Housing and care procedures were in compliance with the provisions and general guidelines of the Swedish Animal Welfare Agency. All procedures were approved by the Local Ethical Committee on Animal Experiments. The animals were housed in pairs in 4-m3 cages and enriched daily. All blood samplings were performed under sedation with ketamine at 10 mg/kg (100 mg/ml Ketaminol; Intervet, Sollentuna, Sweden). All animals were confirmed negative for simian immunodeficiency virus, simian T-cell lymphotropic virus, and simian retrovirus type D.

aCL and

aCL and CAL-101 supplier aβ2-GPI ELISA kits were obtained from Diamedix (Miami, FL, USA). ELISA for aLBPA, anti-annexin II, anti-annexin V and anti-prothrombin were performed as described

previously [3,11–14]. IgG were isolated from sera of three SN-APS patients (Supplementary Table S1, patients 32, 34 and 35), from three APS patients and from three healthy donors by precipitation with 33% ammonium sulphate [15]. For in vitro studies, Eahy926, a human-derived endothelial cell line, was maintained in Dulbecco’s modified Eagle’s medium (high glucose), containing 10% fetal calf serum (FCS), hypoxanthine/aminopterin/thymidine (HAT supplement), 2 mM l-glutamine, 100 U/ml penicillin, 100 mg/ml streptomycin and 250 pg/ml ACP-196 Fungizone (Gibco, Grand Island, NY, USA) at 37°C in a humified 5% CO2 atmosphere. Experiments were performed in cells grown to 60–70% confluence. Eahy926 were incubated with IgG fraction from SN-APS patients (SN-APS IgG; 200 µg/ml), with IgG fraction from normal human serum (NHS-IgG; 200 µg/ml), IgG fraction from APS patients (APS IgG; 200 µg/ml), lipopolysaccharide (LPS) (100 ng/ml) or tumour necrosis factor (TNF)-α (20 ng/ml) as positive controls or with IgG fraction from SN-APS patients (SN-APS IgG; 200 µg/ml), preadsorbed with CL or LBPA, for different

incubation times at 37°C [16–18]. All in vitro experiments were performed using purified IgG from three patients and three controls. We preliminarily determined the optimal IgG concentration and incubation time on the basis of a time–IgG concentration curve, but all the experiments were shown at the best concentration and incubation time. In order to investigate the specificity of the assay, adsorption tests of purified IgG with both CL and LBPA were performed according to the technique described elsewhere [3]. All the materials contained less the 0·00025 ng endotoxin/mg protein,

as detected by the Limulus amebocyte lysate (LAL) test, performed at Associates of Cape Cod (Falmouth, MA, USA). Equal amounts of whole or nuclear extracts proteins [19] (from unstimulated or stimulated Eahy926 with SN-APS IgG fraction, NHS-IgG fraction, LPS, APS IgG fraction or SN-APS IgG fraction preadsorbed Palbociclib with CL or LBPA for 45 min at 37°C, 5% CO2) were separated in 7·5 sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE). The proteins were transferred electrophoretically to nitrocellulose membrane (Bio-Rad Laboratories, Richmond, CA, USA) and then, after blocking with PBS, containing 1% albumin, probed with polyclonal rabbit anti-phospho-IRAK (Cell Signaling, Inc., Danvers, MA, USA) or polyclonal rabbit anti-phospho-NF-κB p65 (Cell Signaling, Inc.), as reported previously [18]. Indirect immunofluorescence was performed to analyse VCAM-1 expression on the cell plasma membrane of Eahy926 cells.

Catestatin reportedly inhibits catecholamine release via nAChRs s

Catestatin reportedly inhibits catecholamine release via nAChRs so these receptors were chosen as candidates for our investigation of possible catestatin receptors in human mast cells.6 Among nAChRs examined, we only found the α7 subunit to be expressed in human mast cells, and unexpectedly this receptor was not likely to be used by catestatin peptides because neither α7 nAChR gene silencing nor the α7 nAChR antagonist α-bungarotoxin inhibited buy Dabrafenib catestatin-induced activation of mast cells. This was not consistent with the studies by Kageyama-Yahara et al.39 reporting the expression of α4, α7 and β2 nAChRs in mouse bone-marrow-derived

mast cells, and by Mishra et al.40 demonstrating the expression of α7, α9 and α10 nAChRs in a rat mast/basophil Z VAD FMK cell line (RBL-2H3). However, as there are important functional differences between rodent and human mast cells,41 and because there is a marked heterogeneity in mast

cell responses both between species and from different tissues within the same species,42 one could not conclude that the presence of the α7 subunit in human mast cells in our study was irrelevant. The αnAChR has also been detected in another human mast cell line (HMC-1), in basophils, macrophages, epithelial cells and endothelial cells;43–45 however, the role of the α7 receptor in inflammation is not yet known. Although the presence of non-functional α7 receptor in human mast cells does not exclude the existence of other still Nintedanib (BIBF 1120) unidentified catestatin receptors, it is noteworthy that as catestatin is a cationic peptide, it might act either at some non-selective membrane receptors or might directly bind to and activate G proteins sensitive to pertussis toxin and coupled to PLC, as has been shown for most basic secretagogues of mast cells.46 This is supported by a previous report that catestatin probably elicits its histamine releasing activity from rat mast cells via a receptor-independent activation of the pertussis toxin-sensitive pathway.23 In the course of evaluating the downstream cellular

mechanisms involved in mast cell activation by catestatin, we focused on MAPK cascades, which participate in different activities such as cell survival and proliferation, and expression of pro-inflammatory cytokines and chemokines.47,48 Catestatin peptides induced the phosphorylation of ERK and JNK, but not p38. Given that the ERK-specific inhibitor U0126 showed an almost complete inhibition of catestatin-stimulated cytokine and chemokine production, we concluded that only ERK was involved in catestatin-mediated mast cell activation. Notably, although JNK phosphorylation was increased by catestatin peptides, the inhibition of JNK did not affect the ability of catestatin to stimulate mast cells, implying that the JNK pathway might not be required for mast cell activation by wild-type catestatin and its variants. Neuropeptides and the neuroendocrine system have previously been thought to be regulators of cutaneous immunity.

Irradiated splenocytes that were used as a source of APCs in our

Irradiated splenocytes that were used as a source of APCs in our experiments could

be treated with Ficoll–Hypaque and separated from the CD4+ T cells only after 1 day in cultures. In preparation for later experiments, Fig. 1(c) was included, showing that anergy could be demonstrated using beads instead of antigen to stimulate secondary cultures. In addition to proliferative unresponsiveness, Th1 cells stimulated with antigen in the presence of n-butyrate demonstrated a 37–77% decrease in IL-2 and a 26–55% decrease in interferon-γ secretion when stimulated in secondary culture with three different stimulation indices (Fig. 1d). Hence, n-butyrate-induced anergy selleck chemicals llc was demonstrated by a loss of both antigen-induced proliferation and cytokine production. It has

been reported previously that n-butyrate increased p21Cip1expression in antigen-stimulated Th1 cells.8 However, p21Cip1 is also induced in antigen-stimulated Th1 cells in the absence of n-butyrate. Consequently, the kinetics of p21Cip1 up-regulation was studied in antigen-stimulated Th1 cells in the presence and absence of n-butyrate during the 6-day primary cultures to compare the two groups for NVP-BKM120 cost any possible difference in p21Cip1 expression. When antigen was added in the initiation of the primary culture (day 0), p21Cip1 was up-regulated in control Th1 cells by day 1, remained high on day 2, but decreased significantly by day 3 and was back to resting levels by day 5 (Fig. 2a). In contrast, when antigen was added on day 0 and n-butyrate was added on day 1, the p21Cip1 levels remained

elevated in anergic Th1 cells during the entire 6-day primary culture. p27Kip1 is another cdk inhibitor thought to play a role in T-cell anergy. As expected, p27Kip1 was high in resting Th1 cells. Its level decreased with the antigen stimulation and was later restored to resting levels in control Th1 cells by day 5 of the primary cultures. In contrast, p27Kip1 levels failed to be completely restored in Th1 cells incubated with antigen and n-butyrate in 6-day primary cultures (Fig. 2b). Hence, because p21Cip1 rather than p27Kip1 was high in the anergic Th1 cells at the end of the 6-day primary cultures, subsequent experiments MTMR9 were focused on the role of p21Cip1 in maintaining proliferative unresponsiveness. The kinetics of other cell cycle proteins was also studied to assess their possible involvement in n-butyrate-induced T-cell anergy. No significant differences between the antigen-stimulated control and anergic Th1 cells were observed in the expression of cdk2, cdk4, cdk6, cyclin D2, cyclin D3 and cyclin E (Fig. 2b). In summary, the kinetics studies on cell cycle proteins revealed that the most detectable difference between anergic and control Th1 cells was the high level of p21Cip1 maintained throughout the primary cultures in the anergic Th1 cells. Localization of proteins such as p21Cip1 in the cell can have important functional consequences.

In the present study, interestingly, we found that the proteinuri

In the present study, interestingly, we found that the proteinuria level was not consistent with GalNAc exposure. The level of proteinuria is higher in the less GalNAc exposure group. It is tempting to speculate that patients with lower GalNAc exposure will reach a remission of disease not long after immunosupressive treatment even with heavy proteinuria. For the first time, we herein investigated the GalNAc exposure of serum IgA1 in IgAN patients, and explored its associations with clinical parameters and histological manifestations. Our results indicated that patients of IgAN with higher GalNAc exposure rate have lower proteinuria. However, the GalNAc

selleckchem exposure rate of more than 40% was a risk factor of glomerular sclerosis and tubulointerstitial injury. The GalNAc exposure rate may be used to predict prognosis of IgA nephropathy. Our study had several limitations that should be noted. First, it is only a cross-section study. Second, Chinese patients were the only ethnic group to be studied and finally, it was a single-centre study. Therefore, further prospective and multicenter studies are needed to confirm our results. Meanwhile, whether GalNAc exposure will change along with prognosis of disease will also need further BAY 80-6946 clarification. This work was supported by the fund of National Nature

Science Foundation of China (81100511) and the NSFC of Guangdong province (845100800400162). We are deeply grateful to all the patients who donated blood. “
“Aim:  Minimal-change nephrotic syndrome (MCNS) is characterized by a good response to corticosteroid, but a high incidence of relapse. We compared

the effect of intravenous methylprednisolone pulse plus oral prednisolone therapy (pulse group) with that of conventional oral prednisolone alone therapy (oral group) on the responsiveness and relapse in the first attack of adult-onset MCNS patients. Methods:  Eighty-one adult patients with biopsy-proven MCNS, who were previously untreated and admitted to our hospital with their first attack of nephrotic syndrome, were analyzed retrospectively. They were arbitrarily assigned to either pulse group Edoxaban (n = 29, 1000 mg of methylprednisolone intravenously for 3 days, and then oral prednisolone 30 to 40 mg daily for 4 to 8 weeks) or oral group (n = 52, oral prednisolone 1 mg/kg daily for 4 to 8 weeks). We compared the time to response and relapse between the two groups. Results:  Time to steroid response was significantly shorter in the pulse group compared with the oral group (15.2 ± 10.2 vs 26.7 ± 17.6 days, P = 0.03). In 74 patients who reached remission within 12 weeks (pulse vs oral groups; 86.2% vs 96.2%, ns), the time to relapse was not different between two groups but the relapse rate was significantly higher in the pulse group (pulse vs oral groups; 60% vs 35%, P = 0.038).

1), similar to other NOD mouse lines congenic for a resistant Idd

1), similar to other NOD mouse lines congenic for a resistant Idd3 locus 37–39. Consistent with previous findings 38 naïve CD4+ T cells

isolated from the spleen of NOD.B6Idd3 mice exhibited increased IL-2 secretion upon in vitro stimulation relative to NOD CD4+ T cells (Supporting Information Fig. 1). To determine the influence of Idd3 on FoxP3+Tregs, the frequency and number of gated CD4+CD3+ T cells expressing FoxP3 and CD25 (Fig. 2A) were assessed in the thymus, spleen, PaLN, and islets of age-matched NOD and NOD.B6Idd3 female mice via FACS. No difference in the frequency of FoxP3+Tregs was detected in the thymus of NOD and NOD.B6Idd3 mice suggesting that thymic development of FoxP3+Tregs is unaffected by IL-2 expression this website levels. On the other hand, an increased frequency and number of FoxP3+Tregs was detected in the PaLN and spleen of older NOD.B6Idd3 mice relative to age-matched NOD mice (Fig. 2A–C). In addition, the frequency of FoxP3+Tregs was significantly increased in the islets of Selleck RXDX-106 10- and 16-wk-old NOD.B6Idd3 versus NOD female mice (Fig. 2B). Notably, however, a greater number of FoxP3+Tregs were detected in the islets of older NOD mice (Fig. 2C) reflecting increased T-cell infiltration of the islets relative to age-matched NOD.B6Idd3

mice. These data demonstrate that the frequency of FoxP3+Tregs is increased in the PaLN and islets of NOD.B6Idd3 mice compared with NOD mice. We and others have shown that those CD62Lhi- versus CD62Llo-expressing FoxP3+Tregs exhibit increased suppressor activity 7, 19. Accordingly, CD62Lhi- and CD62Llo-expressing FoxP3+Tregs were examined

temporally in age-matched NOD.B6Idd3 and NOD female mice. Interestingly, age-dependent differences in the frequency and number of CD62Lhi- and CD62Llo-expressing FoxP3+Tregs were detected in the PaLN and islets of the respective groups of mice. NOD female mice exhibited a temporal decrease in the frequency of CD62LhiFoxP3+Tregs and a concomitant increase in CD62LloFoxP3+Tregs in PaLN (Fig. 3B). Although the number of CD62LhiFoxP3+Tregs progressively increased in the PaLN of NOD female mice (5.2×104 (4 wk) versus 9.0×104 (16 wk)), a greater increase in CD62LloFoxP3+Tregs numbers was detected (6.3×104 (4 wk) versus 14.9×104 (16 wk)) (Fig. 3C). In the PaLN of NOD.B6Idd3 mice, however, the frequency and number of CD62LhiFoxP3+Tregs showed no marked change with age, which were increased relative to age-matched NOD females (Fig. 3B and C). A similar scenario was observed in the islets of NOD and NOD.B6Idd3 female mice. A temporal increase in the frequency of CD62LloFoxP3+Tregs was detected in the islets of NOD female mice which was due to elevated numbers relative to CD62LhiFoxP3+Tregs (Fig. 3D and E). Despite a progressive decline, the frequency of CD62LhiFoxP3+Tregs in the islets of NOD.B6Idd3 female mice was elevated relative to age-matched NOD female mice (Fig. 3D and E).

It is difficult to establish reliable numbers

It is difficult to establish reliable numbers LY294002 on the disease burden of PID, as there are very different approaches to accessing the incidence and prevalence of PID, including telephone surveys [2] and geographically limited cohort studies [3]. However, patient registries

represent the most common approach, and literature provides a large range of results from these registries that have been organized mainly at the national level [4–6]. Patient registries can work as a powerful tool that fulfils a range of purposes, such as describing the natural history of a disease, determining clinical and/or cost-effectiveness of treatment, assessing safety or harm and measuring or improving quality of care [7,8]. Since 2004, the European Society for Immunodeficiencies (ESID; http://www.esid.org) is running a pan-European registry for primary Cobimetinib concentration immunodeficiencies (the ESID database).

The aim of this database is long-term compilation of PID patient data to answer challenging epidemiological questions as outlined above. In addition, the ESID database serves as a basis for outcome-related research questions and to generate research hypotheses that can be tested further in dedicated (clinical) studies. Using the database, researchers have the possibility of identifying patient cohorts for genetic screening and multi-centre trials. Data sets can be extended flexibly for studies on subgroups of patients using the database as a platform for their reporting forms [9,10]. Current

studies include a study on hypogammaglobulinaemia in children (PedPAD; by Esther de Vries, ‘s-Hertogenbosch), a survey on dedicator of cytokinesis 8 (DOCK8)-deficient patients (Michael Albert, Munich) and a survey on chest computed tomography (CT) findings in antibody-deficient patients (Ulrich Baumann, Hanover; http://www.chest-ct-group.eu). Some of the diseases present in the ESID database are also the subject of other rare disease registries. These include registries for autoinflammatory syndromes [11,12]), severe neutropenia [13] and a registry for stem cell transplants in PID [14]. The ESID database co-operates with these registries to ensure a high level of completeness and data quality. ESID provide updates regularly on the development of the database project; this is the third update in this very series. First analyses on the data collected from 2006 and 2008 have been published previously in this journal [15,16]. The ESID online database is a secure, internet-based patient registry which combines both clinical and laboratory data of PID patients. Patients are grouped into nine main categories. These are predominantly T cell deficiencies, antibody disorders, phagocytic disorders, complement deficiencies, other well-defined PIDs, autoimmune and immunedysregulation syndromes, autoinflammatory syndromes, defects in innate immunity and unclassified immunodeficiencies.