Dental follicle mesenchymal stem cells down-regulate Th2-mediated immune response in asthmatic patients mononuclear cells.

BACKGROUND: Asthma is a chronic inflammatory disease in which inflammatory responses have the polarisation of CD4+ T cells to Th2 cells. Dental follicle mesenchymal stem cells (DFSCs) have strong anti-inflammatory properties comparable to other mesenchymal stem cells. OBJECTIVE: We investigated the immunomodulatory effects of DFSCs on CD4+ T helper cell responses of asthmatic patients and compared the results with those obtained with asthmatic subjects on immunotherapy and with healthy individuals. METHOD: Peripheral blood mononuclear cells (PBMC) were isolated from immunotherapy naïve asthmatics, asthmatics on subcutaneous Der p1 immunotherapy and from healthy individuals. PBMC were pre-conditioned with anti-CD3/anti-CD28 mAbs, Der p1 or IFN-γ in the presence and absence of DFSCs and analysed for T cell viability and proliferation, CD4+ CD25+ FOXP3+ regulatory T cell frequencies, cytokine expression, and GATA3, T bet and FoxP3 expressions. Neutralisation of TGF-ß and blockade of IDO and PGE2 pathways were performed to determine suppressive signalling pathways of DFSCs. RESULTS: Dental follicle mesenchymal stem cells suppressed proliferative responses of CD4+ T lymphocytes and increased the frequency of Treg cells. DFSCs decreased effector and effector memory CD4+ T cell phenotypes in favour of naïve T cell markers. DFSCs decreased IL-4 and GATA3 expression and increased IFN-γ, T-bet and IL-10 expression in asthmatics. Costimulatory molecules were suppressed in monocytes with DFSCs in the cocultures. DFSCs down-regulated inflammatory responses via IDO and TGF-ß pathways in asthmatic patients. CONCLUSION: Dental follicle mesenchymal stem cells suppressed allergen-induced Th2-cell polarisation in favour of Th1 responses and attenuated antigen-presenting cell co-stimulatory activities. These studies suggest that DFSC-based cell therapy may provide pro-tolerogenic immunomodulation relevant to allergic diseases such as asthma.

Effect of leucine on NF-κB pathway in liver regeneration after partial hepatectomy in rats, as determined by miniarray analysis.

BACKGROUND: Although some amino acids are recognized to have favorable effects on the liver regeneration after partial hepatectomy (PH), molecular mechanisms underlying these effects are barely known. OBJECTIVE: Our study was aimed to investigate the effects of valine, glutamine, and leucine amino acids on PH-induced NF-κB signal pathway. The research team studied Leucine in a rat model in vivo. The study took place in the medical and surgical experimental research center at the Eskisehir Osmangazi University in Eskisehir, Turkey. The animals were Wistar albino male rats. RESULTS: Group I, the sham group, was administered phosphate buffered saline (PBS) after laparotomy. After 70 % PH procedure, group II, III, IV, and V received single intraperitoneal doses of PBS, valine, glutamine, and leucine amino acids, respectively. At hour 6 after PH, expressions of 88 genes involving in NF-κB signal pathway were examined by RT-PCR mini array method in the liver tissue specimen. Fold values below 0.5 and above 2 were regarded as significant. CONCLUSIONS: Our results suggested that valine, glutamine, and leucine amino acids may alter expressions of the genes of NF-κB signal pathway. In addition, among these amino acids, glutamine and valine proved to be much more effective on NF-κB signal pathway after the PH (Tab. 1, Ref. 41).

Polyethyleneimine brushes effectively inhibit encrustation on polyurethane ureteral stents both in dynamic bioreactor and in vivo.

Polyurethane (PU) ureteral stents have been widely used as biomedical devices to aid the flow of the urine. Due to the biofilm formation and encrustation complications it has been hindered their long term clinical usage. To overcome these complications, in this study, cationic polyethyleneimine (PEI) brushes grafted on PU stents and their performances were tested both in a dynamic biofilm reactor system (in vitro) and in a rat model (in vivo). Thus, we hypothesized that PEI brushes inhibit bacterial adhesion owing to the dynamic motion of brushes in liquid environment. In addition, cationic structure of PEI disrupts the membrane and so kills the bacteria on time of contact. Cationic PEI brushes decreased the biofilm formation up to 2 orders of magnitude and approximately 50% of encrustation amount in respect to unmodified PU, in vitro. In addition, according to Atomic Absorption Spectroscopy (AAS) results, approximately 90% of encrustation was inhibited on in vivo animal models. Decrease in encrustation was clearly observed on the stents obtained from rat model, by Scanning Electron Microscopy (SEM). Also, histological evaluations showed that; PEI brush grafting decreased host tissue inflammation in close relation to decrease in biofilm formation and encrustation. As a results; dual effect of anti-adhesive and contact-killing antibacterial strategy showed high efficiency on PEI brushes grafted PU stents both in vitro and in vivo.

Apoptosis induced by boric anhydrite (B2O3) after partial hepatectomy in rat liver.

Boron is one of the important elements that have a cell-growth suppressing effect. The apoptotic effects of B(2)O(3) that were investigated in rats on liver regeneration following 70 % partial hepatectomy (PH). Wistar albino male rats were used and divided into 4 groups (n = 7). The Saline control groups were given only a single dose of saline; the B(2)O(3)-treated groups that were only given a single dose of 1800 mg.kg(-1) B(2)O(3) by means of intraperitoneal injections following hepatectomy. Three and 6 hours after surgical procedures, all the groups were dissected and liver tissue samples were taken from the groups for NF-κB for caspase-3 gene and protein levels investigation by RT-PCR and TaqMan Protein Assay and histological analyses by TUNEL assay. B(2)O(3)-treated animals were examined and it was observed that NF-κB levels were decreased; however, caspase-3 gene expression and protein levels were increased significantly. This study demonstrated that B(2)O(3) induces caspase-3 and inhibits NF-κB at the early stage of liver regeneration (Fig. 4, Ref. 26).

JAGN1 Deficient Severe Congenital Neutropenia: Two Cases from the Same Family.

Recently autosomal recessively inherited mutations in the gene encoding Jagunal homolog 1 (JAGN1) was described as a novel disease-causing gene of severe congenital neutropenia (SCN) JAGN1-mutant neutrophils were characterized by abnormality in endoplasmic reticulum structure, absence of granules, abnormal N-glycosylation of proteins and susceptibility to apoptosis. These findings imply the role of JAGN1 in neutrophil survival. Here, we report two siblings with a homozygous mutation in JAGN1 gene, exhibiting multisystemic involvement.

Fluid intake from beverages across age groups: a systematic review.

BACKGROUND: Fluid intake, especially water, is essential for human life and also necessary for physical and mental function. The present study aimed to assess beverage consumption across age groups. METHODS: A systematic review was conducted. Original research in English language publications and available studies (or abstracts in English) from 2000 to 2013 was searched for by using the medical subheading (MeSH) terms: ('beverage' OR 'fluid' [Major]) AND ('consumption' [Mesh] OR 'drinking' [Mesh] OR 'intake' [Mesh]) AND ('child' [Mesh] OR 'adolescent' [Mesh] OR 'adult' [Mesh]). Article selection was restricted to those papers covering healthy populations of all age groups in a nationwide sample, or from a representative sample of the population of a city or cities, which examined the trends or patterns of beverage intake and the determinants of beverage intake. Sixty-five studies were identified with respect to beverage consumption across age groups. The papers were screened by thoroughly reading titles or abstracts. Full-text articles were assessed by three investigators. RESULTS: Total beverage intake varied between 0.6 and 3.5 L day(-1) among all age groups (males more than females). Plain water contributed up to 58%, 75% and 80% of the total beverage intake in children, adolescents and adults, respectively. Milk consumption was higher among children; consumption of soft drinks was higher among adolescents; and the consumption of tea, coffee and alcoholic beverages was higher among adults. CONCLUSIONS: Plain water is the main water source for all age groups and the consumption of other beverages varies according to age.

The examination of protective effects of gallic acid against damage of oxidative stress during induced-experimental renal ischemia-reperfusion in experiment.

AIM: In this study, probable effects of gallic acid were investigated in experimentally induced renal I/R injury in rats. MATERIAL AND METHODS: For this purpose, each group consisted of 7 Spraque dawley male albino rats. Groups were defined as follows; Group I: control group; Group II: I/R group; Group III, IV and V: I/R+Gallic acid (50, 100 and 200 mg.kg-1 respectively-i.p.). Left kidney was removed by nephrectomy except for Group I. I/R was induced in the other kidney. Gallic acid was given 15 mins before ischemia induction. SOD, CAT and Gpx activities were determined by electrophoresis. MDA, MPO levels were determined spectrophotometrically. Histopathological investigations were also performed in kidney tissues. BUN and Creatinine levels in serum were determined. RESULTS: BUN, Creatinine and MDA levels were statistically significant but MPO level was not statistically significantly increased in Group II. For SOD, CAT, Gpx activities in Group II, an increase was determined with respect to Group I. Histopathological investigations revealed widespread hyperemia in glomerulus, expansion of the structure between tubules and cell disruptions in Group II. In Group V (200 mg.kg-1 gallic acid), in terms of biochemical parameters, in spite of the significant decrease in BUN, Creatinine and MDA levels; a decrease was determined in SOD, CAT and Gpx isoenzyme activities. Group V showed histologically that I/R injury had been prevented to a greater extent and appearances were close to the control. CONCLUSION: As a result, in terms of our study, evaluations regarding kidney functions and histopathology have shown that gallic acid has protective effects in renal I/R injury (Tab. 2, Fig. 5, Ref. 36).

Alteration in humoral immunity is common among family members of patients with common variable immunodeficiency.

BACKGROUND: The prevalence of primary immunodeficiency (PID) in the relatives of patients with common variable immunodeficiency (CVID) and IgA deficiency is high. Allergic disorders have been recorded in patients with humoral immunodeficiency. We aimed to determine the frequency of humoral immunodeficiency and atopy in the relatives of patients with CVID. METHODS: The study population comprised 20 CVID patients and their relatives. All relatives were screened using a questionnaire covering demographic characteristics, warning signs of PID (adults and children), and core questions on asthma, rhinitis, and eczema from the International Study of Asthma and Allergies in Childhood (ISAAC). We also recorded absolute neutrophil and lymphocyte counts, serum immunoglobulin levels, pulmonary function values, and skin prick test results. RESULTS: The study sample comprised 20 patients with CVID (15 males, 5 females; mean (SD] age, 16.4 (9] years) and 63 first-degree relatives (18 mothers, 16 fathers, 16 sisters, 10 brothers, and 3 offspring). The rate of parental consanguinity was 75%. Of 17 family members with positive PID warning signs, 6 had concomitant hypogammaglobulinemia (3 low IgM levels, 2 selective IgA deficiency, and 1 partial IgA deficiency). The ISAAC questionnaire revealed allergic rhinitis in 3 mothers, asthma in 2 fathers, and 1 sibling. Skin prick testing revealed sensitization to aeroallergens in 31.6% of cases in addition to 1 parent and 1 sibling. CONCLUSIONS: Almost half of the 20 families with a CVID patient had at least 1 additional member with hypogammaglobulinemia, leading us to recommend routine screening for relatives of CVID patients.

Vitamin D as an adjunct to subcutaneous allergen immunotherapy in asthmatic children sensitized to house dust mite.

BACKGROUND: We aimed to investigate the efficacy, safety, and T regulatory cell response of vitamin D as an adjunct to allergen-specific immunotherapy (IT). METHODS: Fifty children with asthma and receiving pharmacotherapy were randomized into three groups as: subcutaneous IT (SCIT) along with vitamin D supplementation (650 U/day; n: 17), SCIT alone (n: 15), and pharmacotherapy alone (n: 18). All patients were evaluated at baseline, 6th and 12th months for scorings of symptoms and medication, skin prick testing, total IgE, specific IgE, and Der p 1-specific IgG4. In addition, D. pteronyssinus-induced CD4(+) CD25(+) FOXP3(+) T regulatory cell percentage, intracellular Foxp3 expression, and peripheral blood mononuclear cell IL-10 and TGF-ß responses were assessed. RESULTS: In the SCIT + vitamin D and SCIT alone groups, total asthma symptom score (TASS), total symptom score (TSS), and total medication scores (TMS) were significantly lower than pharmacotherapy group at the end of 1 year. While the comparison of delta values (Δ 6th and Δ 12th month - baseline) of those scores revealed no significant differences between the two IT groups, TASS at the 6th month was lower in the SCIT + vitamin D group compared with others. There was a significant and positive trend in the levels of Der p 1-specific IgG4 in both IT groups throughout the study period. Whereas the levels of Der p 1-induced IL-10 and TGF-ß were similar between IT groups, the mean fluorescence intensity of Foxp3 was highest in the SCIT + vitamin D group compared with others at the 12th month. The rate of discontinuation of inhaled corticosteroid (ICS) was 6/17 in SCIT + vitamin D, 3/15 in SCIT, and 0/18 in the pharmacotherapy group (P = 0.02). CONCLUSION: Both SCIT groups fared better than pharmacotherapy alone at the end of 1 year. Although the clinical and immunologic outcomes were mostly similar between the two IT groups, some favorable outcomes of vitamin D warrant further investigation in more selected populations with varying doses as adjunct to IT.

Subfoveal choroidal thickness measurements with enhanced depth imaging optical coherence tomography in patients with nanophthalmos.

PURPOSE: To compare subfoveal choroidal thickness (SFCT) between patients with nanophthalmos and age-matched controls. METHODS: This prospective, cross-sectional and comparative study included 31 eyes from 31 patients with nanophthalmos (study group) and 31 eyes from 31 healthy subjects (control group). SFCT and central macular thickness (CMT) were measured with spectral domain optical coherence tomography. All participants underwent a standardised ocular examination including best corrected visual acuity (BCVA), central corneal thickness (CCT), anterior chamber depth (ACD) and refractive error (RE) measurements. RESULTS: Mean SFCT in the nanophthalmic eyes and in the control eyes were 551.3 ± 87 µm and 330.5 ± 46 µm, respectively (p<0.001). Mean BCVA, RE, CMT, axial length (AL), ACD, CCT values were 0.4 ± 0.28 logMAR Unit, +10.6 (3.06), 331.9 ± 78 µm, 18.8 ± 1.5 mm, 2.42 ± 0.4 mm and 577.2 ± 32 µm, respectively, in nanophthalmic eyes and there was a statistically significant difference between groups (p<0.001 for each). There were negative correlations between SFCT with AL (r=-0.836, p<0.001) and ACD (r=-0.597, p<0.001) for the entire study population. SFCT was significantly correlated with CCT (r=0.471, p<0.001) and CMT (r=0.585, p<0.001), RE (r=0.836, p<0.001). CONCLUSIONS: SFCT was significantly higher in nanophthalmic eyes. Further studies are needed to evaluate the role of SFCT to explain the pathophysiology of nanophthalmos.

Predictors of atopic dermatitis phenotypes and severity: Roles of serum immunoglobulins and filaggrin gene mutation R501X

Background: Atopic dermatitis (AD), the most common chronic relapsing skin condition of infancy and childhood, is a complex multifactorial disease, which arises from the interaction between strong genetic and environmental factors. Objective: To investigate the roles of several factors on the severity of AD including FLG R501X gene mutation, serum immunoglobulin (Ig) levels, atopy and accompanying allergic disorders. Method: Children were genotyped for the mutation in FLG R501X gene. Serum levels of major Ig isotypes, atopy and accompanying allergic disorders were assessed. Results: Study group consisted of 49 patients (M: 26, F: 23) with a mean age of 4.9±3.6 years and control group consisted of 50 children (M: 30, F: 20) with a mean age of 3.8±2.8 years. Genotyping of R501X mutation revealed risk alleles in none of the children in study group or control group. IgG z-scores were significantly higher in patients with AD compared to controls (−0.97±1.13 vs 1.48±1.02, p=0.026). There was a positive trend in IgG z-scores and a negative trend in IgA z-scores across the severity of AD. History of recurrent infections was significantly associated with asthma and/or AR (47.8% in patients with asthma/AR vs 3.8% in those without). Children with low IgG or IgA levels presented at an earlier age with lower rates of atopy and mild type AD. Conclusion: In a sample of Turkish children, FLG R501X genotyping revealed no risk alleles in variable severities of AD or healthy controls. Our data suggest that IgG and IgA levels might have a role in phenotypic features of AD in terms of severity and atopic sensitisation(AU)

Immunohistochemical expression of nitric oxide synthase enzymes (iNOS, eNOS, nNOS) in the estrual and luteal phases of the sexual cycle in the cow oviduct.

This study was aimed to determine staining intensity, cellular localization and distribution of the nitric oxide synthase (NOS) enzymes during the sexual cycle in the cow oviduct. Oviduct samples belonging to 20 cows, 10 of which were in the estrual phase and 10 in the luteal phase of the sexual cycle, were examined by an immunohistochemical procedure to determine the presence of the NOS enzymes. In the epithelial cells of the isthmus, endothelial NOS (eNOS) expression showed a strong positive reaction during the estrual phase and a weak positive reaction during the luteal phase in the endothelium and smooth muscle of the blood vessels found in the serosa and lamina propria. eNOS expression was not observed in the epithelium of either the ampulla or the fimbria in the two particular phases of the sexual cycle. The eNOS reactions observed in the blood vessel wall in these regions were stronger during the estrual phase. eNOS activity was not observed in the tunica muscularis in any of the regions of the oviduct. During the estrual phase, it was observed that inducible NOS expression showed a stronger positive reaction in the epithelium and muscle layer of the isthmus and ampulla and in the epithelium of the fimbria, compared to the luteal phase. Neuronal NOS immunoreactivity was observed in the epithelial cells of all oviduct regions and in the muscle layer of the isthmus and ampulla and did not display any significant difference between the estrual and luteal phases.

Predictors of atopic dermatitis phenotypes and severity: roles of serum immunoglobulins and filaggrin gene mutation R501X.

BACKGROUND: Atopic dermatitis (AD), the most common chronic relapsing skin condition of infancy and childhood, is a complex multifactorial disease, which arises from the interaction between strong genetic and environmental factors. OBJECTIVE: To investigate the roles of several factors on the severity of AD including FLG R501X gene mutation, serum immunoglobulin (Ig) levels, atopy and accompanying allergic disorders. METHOD: Children were genotyped for the mutation in FLG R501X gene. Serum levels of major Ig isotypes, atopy and accompanying allergic disorders were assessed. RESULTS: Study group consisted of 49 patients (M: 26, F: 23) with a mean age of 4.9±3.6 years and control group consisted of 50 children (M: 30, F: 20) with a mean age of 3.8±2.8 years. Genotyping of R501X mutation revealed risk alleles in none of the children in study group or control group. IgG z-scores were significantly higher in patients with AD compared to controls (-0.97±1.13 vs 1.48±1.02, p=0.026). There was a positive trend in IgG z-scores and a negative trend in IgA z-scores across the severity of AD. History of recurrent infections was significantly associated with asthma and/or AR (47.8% in patients with asthma/AR vs 3.8% in those without). Children with low IgG or IgA levels presented at an earlier age with lower rates of atopy and mild type AD. CONCLUSION: In a sample of Turkish children, FLG R501X genotyping revealed no risk alleles in variable severities of AD or healthy controls. Our data suggest that IgG and IgA levels might have a role in phenotypic features of AD in terms of severity and atopic sensitisation.

Pretreatment parameters obtained from peripheral blood sample predicts invasiveness of bladder carcinoma.

PURPOSE: To predict the invasiveness of urothelial bladder carcinoma using a logistic regression model on preoperative peripheral blood samples. PATIENTS AND METHODS: Hospital data of patients operated for urothelial carcinoma were reviewed retrospectively. Preoperative blood samples were collected before the first cystoscopic examination. Any kind of infection or inflammation was an exclusion criterion. Patients were grouped as having a non-muscle-invasive or muscle-invasive urothelial carcinoma. The mean age was 69 years and was determined as the cut-off value. According to receiver operating characteristic curves, threshold points were determined for lymphocytes, neutrophil count, neutrophil-to-lymphocyte ratio (NLR), thrombocytes and mean platelet volume. Demographic specialties, parameters obtained from blood samples, tumor size and multiplicity were evaluated and significant parameters were put into a logistic regression model. RESULTS: The study group consisted of 80 non-muscle-invasive and 102 muscle-invasive patients. Age (≤69 vs. >69), female gender, NLR (2.57), mean platelet volume (7.9/fl) and platelet count (400,000/µl) were significant parameters and put in a model. Using odds ratios, the probability of tumor invasiveness was calculated by a formula. CONCLUSION: Age, female gender, NLR and platelet count were found to be the predictors of invasiveness of urothelial carcinoma.

Can radiation-induced chronic oxidative stress in kidney and liver be prevented by dimethylsulfoxide? Biochemical determination by serum and tissue markers.

PURPOSE: To investigate the protective effects of dimethylsulfoxide (DMSO) on chronic oxidative stress in the liver, kidney and serum with biochemical parameters such as malondialdehyde (MDA), advanced oxidation protein product (AOPP), catalase, glutathione (GSH), and free-thiols (F-SH). METHODS: Thirty Wistar albino female rats were randomly divided into 3 groups: group I (control, n=10), group II (irradiation-alone group, n=10) and group III (DMSO and irradiation group, n=10). Rats in groups II and III were irradiated with a single dose of 6 Gy to the entire liver and right kidney. Group III received DMSO 4.5 g/kg by intraperitoneal injection 30 min before irradiation. At the end of the 24th week, the rats were sacrificed and their trunk blood, kidney and liver tissues were collected. RESULTS: Group II rats showed increased levels of lipid peroxidation and protein oxidation, with decreased GSH, FSH and catalase levels in all specimens when compared with group I. Serum and kidney MDA and AOPP levels were significantly lower in group III when compared with group II. However, serum and kidney GSH and F-SH levels were significantly higher in group III when compared with group II. The additive effect on catalase was seen only in the serum. CONCLUSION: DMSO is a protective agent on chronic oxidative stress in the serum and kidney tissue. No oxidant or antioxidant effect of DMSO in the liver was seen.

Impact of early weight loss on growth of Caesarean delivered babies: how long does it last?

BACKGROUND: We hypothesized that Caesarean delivered babies might experience excessive weight loss during neonatal period. AIM: To investigate amount of weight loss among Caesarean delivered babies with exclusive breastfeeding and to identify role of relative weight change (RWC) in early postnatal period on the growth of infants in subsequent months. METHODS: We studied healthy infants born ≥36 completed weeks, by Caesarean section between September 2008 and August 2009, with Apgar scores >7. Weight measurements were performed at birth, at 24 h postpartum and at days 2, 4, 7, 14, 30 and monthly up to 6 months. Predictive roles of RWC at each assessment on detecting excessive weight loss and poor weight gain in 1st month, as well as bodyweight z scores in subsequent months were assessed. RESULTS: We studied 160 infants (88 female, 72 male) with a mean gestational age of 38.01 ± 1 weeks and birthweight of 3239 ± 421 g. Overall, 22.5% of infants lost >10% of birthweight; 11.9% at day 2, 16.9% at day 4 and 6.6% at day 7. Degree of weight loss was not related with parity, gestational age, birthweight or type of anaesthesia. Predictive values of RWCs in detecting poor weight gain in 0-30 days were: 2nd day; limit =-9.4%, specificity = 91.5%, 4th day; limit =-10.2%, specificity = 89.4%, 7th day; limit =-6.3%, specificity = 85%, sensitivity = 78%, 14th day; limit =+0.8%, specificity = 91.5%, sensitivity = 83.3%. Adjusting for birthweight z scores, RWC at 14th day had a significant influence on 1st and 2nd month bodyweight z scores. Bodyweight z scores were significantly correlated with the z scores of bodyweight in 1-5 months but not with that of 6 months. CONCLUSION: Incidence of excessive weight loss is very high among Caesarean delivered babies. RWC at earlier measurements proves satisfactory in predicting poor infant growth. Management strategies based on identifying infants at risk could prevent excessive weight loss and improve future growth of those infants.

Acute radiation effect in the brain in infant rats: Neurocognitive and histopathological examination.

PURPOSE: The aim of the present study was to evaluate the radiation-induced cognitive dysfunction and the radioprotective effect of amifostine (AMI) in the brain of infantile rats. METHODS: Thirty 2-week-old rats were randomly assigned into 3 groups of 10 rats each. Group 1: control (CONT), group 2: radiation alone (RT), and group 3: AMI before radiation (AMI+RT). The rats in the RT and AMI+RT groups were irradiated individually with a single dose of 20 Gy. All animals were evaluated by using the Morris water maze test to evaluate of their cognitive functions. Histopathological analyses of the hippocampus were also carried out after euthanasia. RESULTS: The study showed that the place navigational function and the spatial probe test were not significantly different between the groups. CONCLUSION: It can be said that it is very important to determine when the radiation-induced brain injury is formed. From a clinical perspective, the patients can be intervened before irreversible functional deficits are formed and may be amenable to treatment.

Efficacy of intravenous immunoglobulin treatment in children with common variable immunodeficiency.

BACKGROUND: Children with common variable immunodeficiency (CVID) have increased susceptibility to infections. OBJECTIVE: We evaluated the role of intravenous immunoglobulin (IVIG) replacement therapy on the clinical outcome of patients with CVID. METHODS: We studied children diagnosed with CVID and treated with IVIG (500 mg/kg every 3 weeks). RESULTS: The study population comprised 29 children with CVID (mean [SD] age, 11.8 [6.1] years) with at least 1 year of follow-up before IVIG replacement therapy. Mean follow-up duration was 5.6 (3.5) years (range, 15 months-14 years). During therapy, median serum IgG levels increased from 410 to 900 mg/dL. The mean number of respiratory infections per patient per year decreased significantly from 10.2 to 2.5. The annual number and length of hospital stays decreased significantly from 1.36 to 0.21 and 16.35 to 6.33 days per patient, respectively. The mean annual number of antibiotics used decreased significantly from 8.27 to 2.50 per patient. Twelve patients had developed bronchiectasis before initiation of IVIG; 3 patients were cured of this condition. Age at diagnosis, diagnostic delay, number of respiratory tract infections, and number of antibiotics were found to be significantly higher in patients with bronchiectasis, as was lower B-cell percentage. However, gastrointestinal involvement due to noninfectious causes did not improve significantly after IVIG replacement therapy. CONCLUSION: CVID patients treated with IVIG (500 mg/kg every 3 weeks) had satisfactory serum IgG levels, fewer respiratory tract infections, fewer and shorter hospital stays, and reduced antibiotic usage. However, no effect on gastrointestinal involvement was observed. Early IVIG replacement therapy is important in preventing bronchiectasis.