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While the management of childhood neutropenia associated with a modifiable factor should be appropriate for the primary cause, there are misconceptions regarding the management of severe congenital neutropenia, immune neutropenia and cases classified as "idiopathic". Antibiotic prophylaxis or granulocyte-colony stimulating factor (G-CSF) are prescribed by specialists in pediatric hematology or immunology, whereas immunization may be conducted by primary care physicians should clear recommendations by provided. There is a belief that severe neutropenia, as an immunodeficiency, is associated with compromised effectiveness and increased rate of complications of immunization. The immunization might be delayed or omitted, increasing the risk of unnecessary infection. We discuss the available data and recommendations regarding vaccination of children with chronic severe neutropenia. While there are virtually no studies addressing the safety and effectiveness of vaccination in neutropenia, expert opinions provide information on immunization policy in "phagocytic cells defects" or explicitly neutropenia. There are no contraindications for inactivated vaccines in neutropenia. Live bacterial vaccines are contraindicated. While in general the vaccination with live viral vaccines is encouraged, occasionally neutropenia might be associated with defects of adaptive immunity, which would preclude the administration of such vaccines. Although this should be easily phenotypically identified, we propose assessing immunoglobulin levels and performing a low-cost flow cytometry test for major lymphocyte subpopulations to exclude significant defects in adaptive immunity before administration of live viral vaccines to such patients. This can improve the adherence of patients' guardians and physicians to proposed vaccination policy and the professional and legal safety associated with the procedure.

Introduction Hydrocephalus is a common disorder of the central nervous system (CNS) in the pediatric population. Surgical treatment options involve ventriculoperitoneal shunt (VPS) placement. VPS infection is the most common complication of surgically treated hydrocephalus in pediatric patients [1, 2],which may lead to neuronal damage. Myelin basic protein (MBP) has been proposed as a marker of neuronal injury in a variety of contexts, and MBP levels in the cerebrospinal fluid (CSF) may be used to assess the severity of neuronal damage [1, 3, 4]. Therefore, the aim of this study was to evaluate the CSF level of myelin basic protein (MBP) in a group of pediatric patients with VPS infection. Material and methods Thirty CSF samples were collected from pediatric patients with VPS infection. CSF levels of MBP were measured at three time points, marked by contamination detection, obtention of the first sterile CSF culture, and VPS shunt implantation. The collected data were compared with those of the control group composed of children with active congenital hydrocephalus and valid CSF values. Results The MBP level in the study group was higher than the corresponding control values in the second and third measurements. The highest MBP level was reached in the study group in the second and third measurements. Conclusions The lack of normalization of MBP level in the CSF of children with shunt infection could be connected with ongoing brain damage. It takes longer than the normalization of CSF protein level and pleocytosis. The delay is associated with a prolonged reaction of the immunological system.

Recurrent respiratory tract infections in children are the most common cause of outpatient visits. Due to the necessity of treatment of chronic, frequent use of antibiotics and the dangers of complications are a major clinical problem. Big hopes are now with the possibility of the use of immunostimulation as prevention of these infections. The paper discusses the most important methods of stimulation of the immune system in children. In the light of previous reports were evaluated effects of this type of therapy.

Wstep Aktywacja nieswoistych mechanizmow ukladu odpornościowego stwierdzana u noworodkow i niemowląt z wrodzoną wadą serca predysponuje do czestszych zakazen i ich szybkiego uogolniania. W trakcie zabiegow kardiochirurgicznych z uzyciem krązenia pozaustrojowego, szczegolnie u najmlodszych dzieci, istnieje potrzeba wykonania cześciowej lub calkowitej tymektomii. Cel Celem pracy byla ocena wplywu zabiegu kardiochirurgicznego z wykorzystaniem krązenia pozaustrojowego wykonywanego w pierwszym roku zycia na dystrybucje populacji limfocytow krwi obwodowej. Material i metody Badaniami objeto 32 dzieci z wrodzonymi wadami serca operowanych w pierwszym roku zycia. Oceniano subpopulacje limfocytow krwi obwodowej za pomocą metody cytometrycznej i testu IMK-Plus. Ponadto u cześci dzieci wykonano badanie komorek pamieci immunologicznej. Oznaczenia wykonywano trzykrotnie: przed zabiegiem kardiochirurgicznym, we wczesnym oraz odleglym okresie pooperacyjnym. Wyniki W kolejnych badaniach wykazano obnizanie sie odsetka limfocytow CD3+ i CD4+ oraz wzrost odsetka limfocytow CD19+ w stosunku do wartości sprzed zabiegu. Istotne statystycznie roznice obserwowano porownując wartości uzyskane z drugiego i trzeciego oznaczenia (CD3+, CD4+) oraz wyjściowe z odleglymi (CD3+, CD4+, CD19+). Średni odsetek komorek CD45CD4RO+ w krotkim okresie pooperacyjnym byl nizszy w porownaniu z wartościami sprzed zabiegu, a w kolejnych miesiącach wzrastal istotnie statystycznie. Wnioski Wyniki dotychczasowych badan potwierdzają, ze zabieg kardiochirurgiczny z zastosowaniem krązenia pozaustrojowego powoduje zmiany odsetkow subpopulacji limfocytow krwi obwodowej oraz komorek pamieci immunologicznej w krotkim, a takze w odleglym okresie pooperacyjnym. Moze to wplywac na funkcjonowanie ukladu odpornościowego tych dzieci zarowno w okresie okolozabiegowym, jak i w dalszym zyciu. Konieczna jest kontynuacja badan w dluzszym okresie pozabiegowym oraz porownanie statusu immunologicznego dzieci operowanych z powodu wrodzonych wad serca z ich zdrowymi rowieśnikami.

The polymorphonuclear neutrophils (PMN) possess sufficient potential to affect both immune response and inflammation, however it has not been yet described in the course of multiple sclerosis (MS). We have studied binding of fluorescein isothiocyanate (FITC)- stained TNF-α by PMN, the expression of CD11a, CD11b, and CD18 molecules of β 2-integrines and the expression of CD10 (neutral endopeptidaseNEP) and of CD13 (aminopeptidase N; APN) antigens on PMN in three different groups of MS patients. The control group included neurological patients (OND) with noninflammatory diseases. The obtained results have proved that during MS exacerbation and in the course of chronic progressive MS, PMN reveal several forms of preactivation, including significantly higher stained-TNF-α binding, higher expression of CD11b and CD18, as well as CD10 and CD13 antigens, in comparison with MS remission or OND. We suggest that the increased expression of these molecules on PMN of MS patients in exacerbation of the disease and to a lower degree in the course of CP-MS is a result of PMN priming, and directly prove the PMN involvement in the disease pathogenesis.

We estimated adherence, aggregation and chemiluminescence of neutrophils as well as concentrations of C3c, C4 and C5 complement components and complement haemolytic activity (CH50) in 27 patients with unstable angina pectoris subjected to percutaneous transluminal coronary angioplasty (PTCA). The control group consisted of 12 patients with unstable angina pectoris, in whom coronary angiography was performed but PTCA was decided against for various reasons. Blood samples for examination were taken from coronary sinus and peripheral vein just before, 1 min and 20 min after PTCA or coronary angiography. We observed enhancement of neutrophil adherence, aggregation and chemiluminescence, and decrease in concentrations of C3c, C5 and complement haemolytic activity (CH50) after PTCA procedure. In conclusion we think that ischemia resulting from PTCA causes complement activation in an alternative pathway which seems to be connected with neutrophil activation.

Cardiac surgery with cardiopulmonary bypass (CPB) in children with congenital heart disease induces a systemic inflammatory response. This inflammatory response is thought to be produced by exposing patients to proinflammatory factors.To explore the role of cytokines and proteolytic enzymes in inflammatory complications after cardiac surgery in children.We investigated the dynamics of concentrations of IL-6, IL-8 and IL-10, and metalloproteinases (MMPs) MMP-2 and MMP-9, and their inhibitors - tissue inhibitors of metalloproteinases (TIMPs) TIMP-1 and TIMP-2. These investigations were carried out in 28 children, aged 4-34 months, who underwent a cardiac operation with CPB. Serum concentrations of proteins were sequentially measured before induction of anaesthesia, at the initiation of CPB, after 30 minutes of CPB, at the end of CPB, and 4 and 48 hours after CPB.The serum levels of IL-6 increased dramatically 4 hours after CPB compared with the level before anaesthesia (141.83+/-25.49 vs. 10.68+/-5.01 ng/ml, p=0.00004) and correlated with duration of CPB (r=0.74, p=0.00028). The serum levels of IL-8 increased 4 hours after CPB compared with the level before anaesthesia (267.1+/-41.3 vs. 8.5+/-6.3 ng/ml, p=0.00002). A significant increase of IL-10 concentration at the end of surgery and 4 hours after CPB was detected (95.12+/-23.57 vs. 10.34+/-6.45 ng/ml, p=0.000004 and 59.41+/-21.4 vs. 10.34+/-6.45 ng/ml, p=0.00004, respectively ). The MMP-9 concentration increased at the end of CPB and remained elevated for a period of 48 hours (44.40+/-13.95 vs. 19.53+/-7.58, p=0.000004 and 38.97+/-10.76 vs. 19.53+/-7.58, p=0.00004, respectively). The concentration of MMP-9 detected at the end of CPB positively correlated with duration of CPB (r=0.68, p=0.0045). The TIMP-1 concentration decreased significantly after 30 minutes of CPB, and remained lowered to the end of CPB (respectively 52.68+/-17.72 vs. 83.29+/-17.06 ng/ml, p=0.000006 and 34.94+/-10.58 vs. 83.29+/-17.06 ng/ml, p=0.00004,respectively).Cardiac surgery causes an increase of IL-6 and IL-8 concentrations in peripheral blood 4 hours after CPB termination. The concentration of anti-inflammatory IL-10 cytokine increases immediately after the end of CPB. We showed an increase of the MMP-2 and MMP-9 concentrations during and after CPB and simultaneous decrease of TIMP-1 inhibitor. We demonstrated a link between CPB duration and IL-6 and MMP-9 concentrations.

Cardiac surgery with cardiopulmonary bypass (CPB) can lead to postoperative complications like systemic inflammatory response syndrome (SIRS). These changes are associated with a risk of postoperative organ dysfunction and increased morbidity and mortality in the postoperative period. Its etiology is not yet clarified in details, but this thought to be of immunologic origin. The exact knowledge of these reactions is crucial for the selection of treatment strategies. Cardiac surgery induces neutrophil activation, degranulation and endothelial dysfunction. The nature of the participation of the neutrophil and its time course in the post CPB inflammatory response remains controversial. The aim of this work is to review state-of-the-art knowledge about neutrophil and the role of this cell as a regulator of immune response after cardiovascular surgery.

The 22q11.2 deletion syndrome occurs in approximately 1 of 3000-5000 children. This is a congenital disorder characterized by facial dysmorphic features, cardiac defects, thymic hypoplasia, cleft palate, hypoparathyroidism, and psychiatric disorders. Patients generally exhibit a mild to moderate decrement in T-cell numbers with preservation of T-cell function. We describe advances in understanding the genetic basis of this syndrome, its clinical manifestations, and new information on immunodeficiences in this syndrome.

Cardiac surgery (CS) induces a systemic inflammatory response that may be implicated in postoperative organ dysfunction. The exposure of blood cells and plasma to artificial membranes and the activation of several cell types in the setting of ischemia and reperfusion are believed to play an important role in the development of this reaction. Various mediators have been reported to be involved in the CS-induced reaction, which can lead to postoperative organ dysfunction. Understanding the role of these mediators in systemic inflammatory response may lead to better prognostic assessment and therapeutic modalities. Interleukin 10 (IL-10) is a pleiotropic cytokine produced by both T cells and macrophages and possesses anti-inflammatory properties. IL-10 circulates in the blood of patients undergoing CS with cardiopulmonary bypass (CPB), and increased concentrations of IL-10 have been associated with an adverse clinical outcome. The aim of this work is to review current know-ledge about the role of IL-10 as a regulator of immune response after CS.

Cardiac surgery induces systemic inflammatory response that may have been implicated the postoperative organ dysfunction. This inflammatory response is thought to be produced by exposing patients to proinflammatory factors. The aim of our study was to investigate alterations in procalcitonin (PCT) concentration in peripheral blood in children as the potential early indicator of complications occurring during and after surgery in extracorporeal circulation. Additionally, we evaluated the perioperative time course of IL-6.The investigations were carried out in 21 children undergoing cardiac operation with cardiopulmonary bypass (CPB). Serum concentrations of PCT and IL-6 were sequentially measured before induction of anesthesia, at the initiation of CPB, at the end of CPB, and 24 hours, and 72 hours after CPB.There was no significant PCT-elevation at all 5 times of measurement. Levels of IL-6 increased significantly after surgery, and remained elevated for up to 1 day. Peak values correlated with the duration of CPB (r=0.68, p=0.0006).We conclude, that cardiac surgery with CPB did not have any influence on the secretion of PCT in children. These results suggest that IL-6 was more effective than PCT to monitor patients with a favorable outcome.

Cardiopulmonary bypass (CPB) is known to induce several pathogenic responses in cardiovascular surgery. Children suffering from cyanotic congenital heart disease (CCHD) have been assumed to have a deficiency in their defence mechanisms. The preoperative clinical condition (hypoxemia and circulation disorders) could initiate post CPB inflammatory response. The nature of the participation of neutrophils and its time course in the post CPB inflammatory response, however, is less clear. THE AIM of our study was to investigate alterations in expression of adhesion molecules and L-selectin on neutrophils, and soluble adhesion molecules levels in CCHD children.23 infants and children with congenital heart disease, scheduled for primary corrective surgical repair; were enrolled. Expression of CD11b. CD18, and CD62L on neutrophils was determined by cytometry. Additionally, serum concentrations of soluble ELAM-1, L-selectin were measured using enzyme-linked immunoassay. Blood samples of children with CCHD were collected at the following times: before anaesthesia, at the initiation of CPB, after 30 minutes of CPB, at the end of CPB, and 24 hours and 72 hours after surgery.The expression of CD11b molecule on neutrophils was increased significantly at the start of CPB and returned to the baseline after 24 hrs. In all patients, preoperative CD11b expression on neutrophils correlated with hypoxemia. Reduced CD62L expression on neutrophils and soluble L-selectin concentration were observed at the end of CPB.We conclude, that cardiac surgery with CPB has a suppressive effect on adhesion molecules expression in children with CCHD, These findings also suggest that the type of neutrophil response to cardiac surgery appears to depend on preoperative clinical condition.

The nature of the participation of neutrophils in the post-cardiopulmonary bypass (CPB) inflammatory response is not very clear. The aim of our study was to investigate alterations in neutrophil phagocytic activity and adhesion molecule expression on these cells in children during and after CPB.Twenty-one children aged 6-33 months with congenital heart disease, scheduled for pri mary corrective surgery, were enrolled. The expressions of CD11b adhesion molecules and Fc? receptor on neutrophils and their phagocytic activity were evaluated. The studied markers were sequentially measured before, at the initiation of, and after CPB.During the course of the operation, CD11b molecule expression on neutrophils showed a slight elevation at the start of CPB (876.5+/-104.8 mean fluorescence intensity, MFI, vs. 768.1+/-178.2; p = 0.0047), followed by a significant decrease to 689.01+/-166.7 MFI after completion of the procedure. The expression of CD11b molecule on neutrophils measured at the end of CPB inversely correlated with the duration of CPB (r = -0.68, p = 0.00059). The expression of CD16 antigen dropped significantly at the start of CPB (1164.6+/-307.3 MFI vs. 1327.4+/-345.3 MFI; p = 0.0007) and remained decreased until the end of CPB (814.0+/-198.1 MFI).These findings suggest that the characteristics of the neutrophil response to cardiac surgery appear to depend on many factors. We demonstrated a link between the duration of CPB and adhesion molecule expression on neutrophils.

Cardiovascular surgery is associated with systemic inflammatory response syndrome (SIRS), and these patients are recognized to be at increased risk for delayed infectious complications. There is a need for effective and accurate biological and biochemical tests to support, or exclude, the diagnosis of infection. Inflammatory response involves the release of a wide array of mediators, which has led to the suggestion that some of these mediators could be used as markers of infection or sepsis severity. Recently, plasma procalcitonin (PCT) concentration has been proposed as an indicator of the presence of infection. The aim of this work is to review state-of-the-art knowledge about PCT and the role of this peptide as a regulator of immune response after cardiovascular surgery.

Infants and small children who undergo cardiac surgery due to congenital heart disorders, are at risk of developing inflammatory complications and multi-organ failure.To assess the changes in the expression of adhesive molecules on neutrophils and in peripheral blood plasma in children undergoing cardiac surgery with or without the use of cardiopulmonary bypass (CPB).In 18 children who underwent surgery with the use of CPB and in 7 children who underwent surgery without CPB, the expression of CD11b, CD11c and CD62L molecules on neutrophils and soluble adhesive molecules - L-selectin and ICAM-1, was assessed before, during and after cardiac surgery.A significant increase in the expression of CD11b on neutrophils at the time of the initiation of CPB was observed. The most pronounced decrease in the expression of CD11c was detected at the end of surgical procedure. There was also a negative correlation between L-selectin concentration measured at the end of CPB and CPB duration as well as between ICAM-1 concentration and duration of hypothermia.Cardiac surgery influences the expression of adhesive molecules on neutrophils and in blood plasma. These changes are more pronounced in children who undergo CPB, and depend on CPB duration as well as the use of hypothermia.

CD13 AG and CD11a, CD11b, CD18 molecule expression on peripheral blood mononuclear cells (PBMC) were studied as these cells’ adherent or transendothelial migration properties in three different multiple sclerosis (MS) patients groups (total 38): with clinically active MS (acute exacerbation of MS and primary chronic progressive MS (CP-MS)) and with MS remission. The control group consisted of patients, suffering from other non-inflammatory neurological diseases (OND). The results of our study suggest that CD11a/CD18 molecules expression on PB lymphocytes, although higher on these cells’ surface in the course of MS as compared to OND, does not differentiate clinical forms of MS. CD11a molecule expression on monocytes did not differ significantly in all tested MS patient groups in comparison to OND. Although the expression of CD11b/CD18 molecules on monocytes’ surface shows their activation in the course of MS, it does not differentiate them either. However, CD13 Ag of APN expression on PBMC surface may be an immunological marker of MS clinical form. CD13 Ag expression may also be a sensitive marker of these cells’ transendothelial migration properties.