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1.

Background

The pathogenesis of biliary atresia (BA) is associated with an inflammatory process involving the biliary tree. This study aimed to investigate the association of T-helper cell cytokine levels with age in patients with BA.

Methods

Twenty-eight patients with BA were divided into three groups according to their age (<?2 months, 2–3 months, and?≥?3 months). All the patients underwent Kasai portoenterostomy. Blood samples were collected from the patients preoperatively, and the liver tissue specimens were obtained during surgery. We detected serum levels of interleukin (IL)-1β, IL-12p70, interferon (IFN)-γ, IL-6, IL-10, and transforming growth factor (TGF)-β1 and liver expression of IL-1β, IL-6, and TGF-β1.

Results

The serum levels of IL-1β, IL-12p70, IL-6, and IL-10 in patients aged?≥?3 months were significantly higher than those in patients aged?<?2 months. There were no significant age-related differences in the IL-1β, IL-6 and TGF-β1 expression levels in the liver tissue of patients with BA.

Conclusions

The serum levels of IL-1β, IL-6, IL-10 and IL-12p70 showed significant age-related differences in patients with BA. Interpretation of the role of cytokines in BA needs to take patient’s age into consideration.
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2.

Background

To evaluate the role of serum cytokines in the pathogenesis of respiratory syncytial virus (RSV) infection in infants with low birth weight (LBW).

Methods

A prospective observational study was performed, and hospitalized children with lower respiratory tract infection (LRTI) were recruited. Three hundred fifty-eight patients <?1 year met the inclusion criteria: 116 patients had only RSV infection (RSV group); 242 patients had no RSV or other specific pathogen (non-RSV group). Serum interleukin-2 (IL-2), IL-4, IL-6, IL-10, tumor necrosis factor-α (TNF-α), and interferon-γ (IFN-γ) were detected through flow cytometry.

Results

No significant differences in serum IL-2, 4, 6, 10, and IFN-γ levels were observed between the RSV and non-RSV groups. For RSV infected infants with or without wheezing, delivery mode had no obvious effect on the changes of serum cytokine levels. However, the level of IL-6 in the RSV-infected infants with LBW was significantly higher than that in infants with normal birth weight.

Conclusions

Serum IL-6 level was significantly increased in RSV infected infants with LBW. It is likely that the specific serum cytokine pattern will contribute to our understanding of the pathogenesis of RSV infections, especially in RSV-infected infants with LBW.
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3.

Objective

To assess the effect of placental transfusion by delayed cord clamping (DCC) of 60 s or cord milking (CM) on serum ferritin levels at hospital discharge and 3 mo of postmenstrual age (PMA) in preterm neonates of 30 to 33 wk gestation in comparison to early cord clamping (ECC) within 10 s.

Methods

This mixed longitudinal study was conducted in moderately preterm neonates of 30 to 33 wk gestation born in a level III unit in Northern India with the study sample nested within a randomized controlled trial on placental transfusion. Intervention was delayed cord clamping for 60 s or cord milking compared with early cord clamping (within 10 s). Primary outcome measure was serum ferritin levels at discharge. Secondary outcome measures were serum ferritin levels at 3 mo PMA, incidence of anemia, need for blood transfusion and incidence of iron deficiency by 3 mo PMA.

Results

Out of the 215 randomly chosen infants, serum ferritin levels were estimated at least at one time point (at discharge or at 3 mo PMA) in 197 neonates [placental transfusion – 107; early cord clamping – 90]. Amongst them, ferritin level was estimated at discharge in 141 neonates, at 3 mo PMA in 76 neonates and at both time points in 20 neonates. Median (IQR) serum ferritin (μg/L) at discharge was significantly higher in placental transfusion group in comparison to the ECC group [399 (309,600) (n = 79) vs. 254 (190,311) (n = 62); p < 0.001]. Median (IQR) ferritin level at 3 mo PMA was not different between the study groups [20 (14,57) (n = 39) vs. 24 (8,52) (n = 37); p = 0.2]. The incidence of anemia by 3 mo PMA was significantly lesser in the placental transfusion group. No difference was observed in anemia requiring blood transfusion and iron deficiency by 3 mo PMA between the groups.

Conclusions

In 30 to 33 wk preterm neonates, placental transfusion resulted in significantly higher serum ferritin at discharge in comparison to early cord clamping. However, this benefit did not persist till 3 mo PMA.
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4.

Background

Children with Kawasaki disease (KD) and pyuria have been misdiagnosed with urinary tract infection (UTI). We compared clinical and laboratory features at admission between two groups of infants under 6?months of age who showed initial pyuria, to identify the initial clues suggestive of KD.

Methods

We retrospectively reviewed the medical records of children with fever who were under 6?months of age with pyuria, over a 10-year period (2007–2017). We included infants with sterile pyuria who were finally diagnosed with KD and those with UTI.

Results

During the period investigated, 12 (9.9%) KD patients with sterile pyuria and 378 infants with UTI were included in this study. Older age (P?<?0.01), a longer duration of fever; total and before admission (P?<?0.01), more negative nitrite test (P?<?0.01), higher platelet count (P?=?0.04), increased C-reactive protein (CRP) (P?<?0.01) and erythrocyte sedimentation rate (ESR) (P?<?0.01), were identified as initial features of infants finally diagnosed with KD. In the receiver operating characteristic analysis, optimal cut-off values of 509?k/μL for platelet count, 60?mg/L for CRP, and 68?mm/H for ESR were selected. Patients with ESR?>?68?mm/hr had a ninefold higher odds of KD compared to those with lower ESR levels (odds ratio: 8.963, 95% confidence intervals: 1.936–41.493, P?=?0.005), whereas CRP and platelet count could not significantly increase in the odds of KD at a cut-off point.

Conclusion

Persistent fever, elevated ESR, and negative urine nitrite test can serve as early clues to suspect KD in febrile infants with pyuria.
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5.

Objectives

To compare levels of Interleukin-6 (IL-6) in children with febrile seizures and febrile controls.

Methods

Study conducted in a tertiary-care hospital in Northern India from November 2013 to April 2015, enrolling 160 children (80 each with febrile seizures and febrile controls), aged 6–60 months. Serum IL-6 estimated by ELISA method. Iron study done as per standard technique. All the cases of febrile seizure were followed up at 1 week, 3 months and 6 months for recurrence of seizures.

Results

The mean serum IL-6 levels in children with febrile seizures was 62.0 (63.9) pg/mL and febrile controls was 86.9 (70.6) pg/mL (P=0.025).

Conclusion

Serum IL-6 levels were significantly lower in children with febrile seizures as compared to febrile controls.
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6.

Background

Kawasaki disease (KD) is sometimes confused with urinary tract infection (UTI) because both can present with pyuria and C-reactive protein (CRP) elevation. The present study investigated the clinical and laboratory findings that can differentiate KD from UTI in febrile children with pyuria and CRP elevation.

Methods

Medical records were retrospectively reviewed for children with KD and those with UTI. The clinical and laboratory findings between the KD with pyuria group (n?=?48) and the UTI group (n?=?118) were compared.

Results

The KD with pyuria group had older age (P?<?0.001) and longer duration of fever (P?<?0.001) than the UTI group. In blood tests, both groups showed increased CRP level, but the value of CRP was higher in the KD with pyuria group than in the UTI group (P?<?0.001). The KD with pyuria group also showed higher values for liver enzymes than the UTI group (P?<?0.001); >?70.0% of children in the KD with pyuria group, but <?20.0% of children in the UTI group possessed elevated liver enzymes (P?<?0.001). On urinalysis, 40.7% of the UTI group had a positive nitrite test, but 0.0% of the KD with pyuria group had a positive nitrite test (P?<?0.001).

Conclusions

Elevated liver enzymes are more specific to KD than to UTI, whereas a positive nitrite test is more specific to UTI than to KD. Our findings can be used as diagnostic clues to differentiate KD from UTI in febrile children with pyuria and CRP elevation.
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7.

Background

Viral pneumonia is the main type of community-acquired pneumonia (CAP) in children. YKL-40, a chitinase-like protein, is regarded as a biomarker of the degree of inflammation.

Methods

Children who were diagnosed with CAP, including viral pneumonia, bacterial pneumonia, and dual infection, were included in the cohort study. The pathogenic diagnosis depended on PCR and immunoassay test. YKL-40 levels were examined twice by enzyme-linked immunoassay (ELISA).

Results

Serum YKL-40 levels were higher in patients with pneumonia than in healthy controls. The admission levels of YKL-40 in serum and Bronchoalveolar lavage (BALFs) indicated a positive correlation with the serum levels of C-reactive protein and other inflammatory cytokines (IL-6 and TNF-α). The disease severity have no correlation with the admission serum levels of YKL-40. Meanwhile, reductions in YKL-40 levels from initial admission levels to day 5 post-admission were correlated with disease severity. The multiple logistic analysis indicated the decreased extent of serum YKL-40 level as an independent prognostic predictor of severe cases in patients with viral pneumonia.

Conclusions

Reductions in serum YKL-40 levels on day 5 after receiving therapy is a possible prognostic biomarker for children with viral pneumonia.
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8.

Background

Leptin may induce inflammation in asthma by activation of Th2 cells. It has also been demonstrated that leptin expression increases upon inflammation and that asthmatic patients show increased serum leptin levels. We hypothesized that the polymorphism in leptin (LEP) and leptin receptor (LEPR) genes is associated with childhood asthma and may affect their serum level. To our knowledge, there are no reports analyzing LEP and LEPR polymorphisms in association with their serum levels in childhood asthma.

Methods

We analyzed 35 subjects: 25 asthmatic pediatric patients and 10 healthy children aged from 6 to 18. The diagnosis of allergic asthma was based on clinical manifestation, lung function, positive skin prick tests and increased immunoglobulin E levels. The polymorphisms were genotyped with use of polymerase chain reaction-restriction fragment length polymorphism method. Serum levels of leptin and leptin receptor were determined using BioVendor enzyme-linked immunosorbent assay kits. Statistical analysis was done with Statistica v.12.

Results

We observed that leptin levels were increased in asthmatic subjects as compared to healthy controls and were significantly higher during exacerbation than in the asymptomatic period (P?=?0.025). We observed that LEP polymorphism (rs13228377) was associated with higher serum leptin levels in asthma and these two variables had high predictive value for asthma risk (P?=?0.007, odds ratio 17.5, predictive accuracy 83.9%). LEPR polymorphisms did not show association with its serum level and asthma risk.

Conclusion

LEP polymorphism may increase asthma risk via influence on its serum level.
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9.

Objective

To evaluate the post treatment changes in disease activity and inflammatory markers over time in longitudinal follow-up involving different subtypes of juvenile idiopathic arthritis (JIA) patients.

Methods

This prospective longitudinal study, carried out over a period of 2 y, included JIA patients, both old and new, with high disease activity. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), ferritin, CHAQ (Childhood Health Assessment Questionnaire) score and JADAS27 (Juvenile Arthritis Disease Activity score with 27 active joint counts) were estimated at the initial visit, 6 mo, 12 mo and 18 mo of follow-up.

Results

Out of 40 patients, 10 had persistent oligoarthritis, 11 had rheumatoid factor (RF) positive polyarthritis, 8 had RF negative polyarthritis and 11 had systemic JIA. Twenty-one of them were females. Serum ferritin was highly elevated in systemic JIA patients with a range of 750–7712 ng/ml at the initial visit. All three inflammatory markers with disease activity score decreased significantly over 18-mo-period in all four subtypes. At any visit, all these parameters had largest value in systemic arthritis and least in oligoarthritis variety. At 18 mo, all oligoarthritis and polyarthritis cases had low or inactive disease while none of the systemic JIA patients achieved inactive disease. Elevated ESR and serum ferritin was found in all at 18 mo. CRP normalized in some with low or moderate disease activity.

Conclusions

Inflammatory markers and disease activity decreased in all subtypes of JIA with treatment without biologics. Acute phase markers often remain elevated in inactive disease state. Similarly, normal level of an inflammatory marker does not necessarily indicate absence of active disease.
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10.

Objectives

To evaluate the Quality of life (QoL) and the factors affecting it in transfusion-dependent thalassemic children.

Methods

Current study was conducted at Department of Pediatrics, Dr. SNMC, Jodhpur. Ninety-three transfusion-dependent thalassemic children aged between 8 and 18 y were enrolled. Quality of life was assessed using Pediatric Quality of Life (PedsQL) measurement model in thalassemic children.

Results

Mean age of children was 11.8?±?3.2 y. Lowest mean score was observed in School functioning (57.4) while in Physical functioning, Emotional functioning and Social functioning it was 73.9, 82.2 and 83.49 respectively. Mean PedsQL score was higher in 8-12 y age group as compared to 13-18 y (p?=?0.024). PedsQL score was higher in children who required transfusion once in a month (p?=?0.028) and children on combined chelation therapy as compared to children who were on injectable chelator (p?=?0.039). Health Related QoL was not affected with gender, education status, family history of thalassemia, ferritin level, splenectomy or consanguinity.

Conclusions

Factors related with low QoL in current study were higher age, increased transfusion frequency and injectable mode of chelation. In order to improve the QoL in thalassemic children appropriate programmes and interventions should be started targeting above domains.
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11.

Objective

The aim of our study was to assess serum Adiponectin, Interleukin-6 (IL-6) and high-sensitivity C-reactive protein (hsCRP) levels and their correlation with conventional risk factors for cardiovascular disease and diabetes in overweight/obese Indian children.

Methods

Body mass index (BMI), waist circumference, blood pressure, fasting serum adiponectin, IL-6, hsCRP, blood glucose, triglycerides, and total and high density lipoprotein cholesterol were measured in children aged 7–15 years with BMI >85th centile.

Results

84 overweight/obese children (48 boys) with mean (SD) age 10.2 (1.9) years were enrolled. Mean (SD) adiponectin, hsCRP and median (IQR) IL-6 levels were 6.0 (3.1) μg/mL, 3.4 (2.4) mg/L and 12.7 (5.0–90.0) pg/mL, respectively. Low adiponectin, high hsCRP and high IL-6 were noted in 16.5%, 49.4% and 54.4% participants, respectively. Adiponectin was inversely correlated with waist circumference, and IL-6 positively with BMI and blood glucose.

Conclusions

Inflammatory mediators, hsCRP and IL-6 were elevated in half of the overweight children. Adiponectin and IL-6 correlated well with traditional risk markers.
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12.

Background

Vitamin D plays an important role in etiology of Autism Spectrum Disorders (ASDs). We aimed to evaluate the serum 25 - hydroxyl vitamin D level among children with ASDs in Ahvaz city, Iran.

Methods

It was a cross-sectional study which had conducted on 62 subjects in two groups: a case group (n?=?31) consisted of ASD children who study in especial schools; and a control group (n?=?31) of healthy children who were selected by simple random sampling from regular schools in Ahvaz city, Iran during 2016. Maching between two groups has done regarding Socioeconomic status, type and amount of food intake, place of living and age. The levels of serum 25 - hydroxyl vitamin D were assessed in early morning means fasted state and also measured using ELISA method. Data were analyzed using Statistical Package for Social Sciences (SPSS) version 20. The significant level was considered < 0.05.

Results

In ASD children, the average serum 25-hydroxyvitamine D level was 9.03?±?4.14?ng/mg. In ASD group, 96.8% (30 subjects) had vitamin D deficiency. In healthy children group, average serum 25-hydroxyvitamine D level was 15.25?±?7.89?ng/mg. Average serum 25-hydroxyvitamine D level in intervention group was significantly lower than the control group (P?>?0.001). Although the parents of patients in control group reported longer exposure to sun (27.42?m per day against 33.06?m per day), no significant difference was observed between these groups in terms of exposure to sun (P?< 0.05).

Conclusions

A significant difference was observed between serum 25-hydroxyvitamine D levels between the healthy and ASD children. It is recommended to use vitamin D supplement in children with ASDs under medical care.
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13.

Purpose

Lgr5+ intestinal epithelial stem cells (ISCs) crucial for intestinal epithelial regeneration are impaired during necrotizing enterocolitis. This study aims to investigate the influence of different stressors on intestinal epithelial injury and regeneration in vitro.

Methods

Intestinal epithelial cells (IEC-18) were exposed to stressors such as lipopolysaccharide, hydrogen peroxide, and serum. Cell viability was assessed using MTT assay at 18 and 24 h. IL-6 and Lgr5 gene expressions were measured using qPCR.

Results

IEC-18 cell viability decreased 18 h following administration of lipopolysaccharide, hydrogen peroxide, and low serum concentration. However, after 24 h, the decrease in cell viability was observed only in higher, but not in lower concentrations of lipopolysaccharide and hydrogen peroxide. IL-6 expression increased in all groups compared to control. Lgr5 expression was up-regulated in cells exposed to a single stressor, but down-regulated when multiple stressors were administered.

Conclusion

Lipopolysaccharide, hydrogen peroxide, or low serum induced IEC-18 injury. The upregulation of Lgr5 expression after exposure to a single stressor suggests that minor injury to IEC-18 induces Lgr5+ ISCs to stimulate repair. Conversely, when IEC-18 cells were exposed to multiple stressors, Lgr5 expression was reduced. We speculate that this finding is similar to what happens in NEC when multiple stressors cause impairment of intestinal epithelium regeneration.
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14.

Background

Kawasaki disease (KD) has now become the leading cause of acquired heart disease among children in developed countries. This study investigated whether patients with KD have an increased risk of atherosclerosis.

Methods

Electronic databases, including PubMed, Embase and Springer link, were searched through June 1, 2015, for eligible studies. Studies were included when they met the following criteria: 1) an observational study focusing on evaluating the risk factors for atherosclerosis in patients with KD; 2) KD was diagnosed clinically according to the Japan Kawasaki Disease Research Committee or American Heart Association’s diagnostic criteria; 3) the study subjects were KD patients without coronary heart disease or related cardiovascular disease (KD group) and non-KD patients as control (control group), and 4) investigation of important atherosclerosis risk factors, total cholesterol (TC), low-density lipoprotein cholesterol (LDL), triglycerides (TG), systolic blood pressure (SBP), and flowmediated dilatation (FMD). The methodological quality of the included studies was evaluated using the Newcastle- Ottawa Scale. Mean difference (MD) and relative risk (RR) and corresponding 95% confidence intervals (CI) were used to calculate the pooled results.

Results

Sixteen studies were included with a total of 870 patients, including 421 KD patients and 449 non-KD controls. Differences in TG and SBP between KD patients and controls were not significant; in contrast, TC and LDL levels were significantly higher in KD patients than the controls, whereas FMD in the KD patients was significantly lower.

Conclusions

KD patients may have an increased risk of developing atherosclerosis.
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15.

Objectives

To identify the expression of IL-33 during SLIT (Sublingual immunotherapy) in AR (Allergic rhinitis) children.

Methods

Thirty children received house dust mite (HDM) allergen extract for SLIT and thirty children received placebo in this study. Serum and nasal lavage samples of cases and controls were collected at different time points during SLIT. Interleukin (IL)-33 and other cytokines were estimated in these samples by enzyme-linked immuno sorbent assay (ELISA). Peripheral blood mononuclear cells (PBMC) were prepared and stimulated with rhIL-33 (with or without other stimulators) at different time points during SLIT.

Results

The present results showed that both serum and nasal lavage of IL-33 levels decreased significantly after 12 mo treatment and this trend maintained at least until 24 mo. The decreased nasal IL-33 level was positively correlated to local Th2 cytokines and increased IL-10 expression at 2 y post SLIT treatment. In vitro experiments showed that IL-33 promotes IL-4 and IL-5 and inhibits IL-10 expression by peripheral blood mononuclear cells (PBMCs) in AR.

Conclusions

Decreased IL-33 expression during SLIT may contribute to low Th2 response and enhanced Regulatory T cell cytokines expression. Thus, IL-33 maybe an important predictor during SLIT.
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16.

Objective

To study the association between asthma control and serum 25OH Vitamin D levels in children with moderate persistent asthma on preventer therapy.

Methods

Children aged 6–18 years, with moderate persistent asthma, on preventer therapy for ≥2 months were included. Control was categorized as good, partial or poor as per GINA guidelines. Serum 25 (OH) Vitamin D levels were measured and their relationship with the level of control was studied.

Results

Out of 50 children enrolled, 22 had well-controlled asthma, and 21 had partially controlled asthma. Vitamin D was deficient in 30 children and insufficient in 18 children. Children with vitamin D deficiency had significantly less wellcontrolled asthma as compared to those with insufficient or sufficient levels of 25 (OH) vitamin D (13.3% vs 88.9 % vs 100%).

Conclusion

Vitamin D deficiency is associated with suboptimal asthma control.
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17.

Background

Recently, studies suggesting that vitamin D deficiency correlates with the severity and frequency of Type 1 (insulin-dependent) diabetes mellitus (T1DM) and that vitamin D supplementation reduces the risk of developing T1DM have been reported.

Objective

In this study, we aimed to assess vitamin D status in Egyptian children and adolescents with T1DM.

Methods

This was a case–control study including 80 T1DM diagnosed cases aged 6 to 16 years and 40 healthy children with comparable age and gender as the control group. For all subjects, serum 25 (OH) D levels were measured by ELISA, Serum parathyroid hormone (PTH) and serum insulin were measured by an electrochemiluminesce immunoassay. Serum glucose, Glycosylated hemoglobin (HbA1c) levels and homeostasis model assessment of insulin resistance (HOMA-IR) were also assessed.

Results

Compared to the control group, serum vitamin D levels were not significantly lower in diabetic subjects (24.7?±?5.6 vs 26.5?±?4.8 ng/ml; P?>?0.05). Among diabetic cases 44(55%) were vitamin D deficient; meanwhile 36(45%) cases had normal vitamin D level (P?<?0.01). In addition, 26(32.5%) diabetic cases had 2ry hyperparathyroidism and 54(67.5%) cases had normal parathyroid hormone level; meanwhile, none of the control group had 2ry hyperparathyroidism (P?<?0.01). Furthermore, we found a significant difference between vitamin D deficient diabetic cases and those with normal vitamin D level as regards HOMA-IR and diabetes duration (P?<?0.01).

Conclusion

Public health message on the importance of vitamin D status; especially in diabetic children and adolescents, should be disseminated to the public.
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18.

Objective

To study the association between serum vitamin D levels and levels of asthma control in children aged 5-15 years.

Methods

Children with physician-diagnosed asthma who were under follow-up for at least 6 months were enrolled. Participants were categorized into three asthma control groups as per standard guidelines, and their serum 25-hydroxy vitamin D levels and pulmonary function tests were compared.

Results

Out of 105 children with asthma enrolled in the study, 50 (47.6%) were controlled, 32 (30.5%) were partly controlled and 23 (21.9%) were uncontrolled. Median (IQR) serum vitamin D levels in these three groups were 9.0 (6.75, 15) ng/mL, 10 (6.25, 14.75) ng/mL and 8 (5, 10) ng/mL (P=0.24), respectively.

Conclusion

We did not observe any association of serum 25-hydroxy vitamin D levels with the level of control of childhood asthma.
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19.

Objective

To analyze the association of IL-10 gene and its polymorphisms with preterm birth (PTB).

Methods

Five hundred and fifty nine women with term birth and 559 with preterm birth were recruited from Lucknow, India. Genetic association analysis was conducted between cases and controls. Subjects recruited as cases were women (aged between 18–40 y) with singleton delivery before 37 wk of gestation and controls were with delivery after or on 37 wk. The genotyping was performed for rs1800871, rs1800872 and rs1800896 for assessing the allelic distribution, haplotypic association and linkage disequilibrium analysis. IL-10mRNA levels were evaluated by real time quantitative polymerase chain reaction (PCR) method.

Results

The risk of PTB was found significant in women carrying IL-10 (?1082) GA genotype [OR=1.72(1.7–2.5), p=0.006]. The haplotypic analysis of studied polymorphisms for rs1800871, rs1800872 and rs1800896 depicted the association of ATA (p=0.02) and ATC (p=0.01) haplotypes with PTB. The IL-10 mRNA levels were significantly lower in cases (p=0.05).

Conclusions

IL-10 marks a protective impact in the inflammatory pathway of PTB.
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20.

Objectives

To determine the association of ocular manifestations in beta-thalassemia with the patient’s age, blood transfusion requirements, average serum ferritin and dose and duration of iron chelation therapy.

Methods

Sixty multi-transfused beta thalassemia patients of 12 to 18 y of age on chelation therapy were included in this cross-sectional analysis. Structural and functional evaluation of the retina was done using Optical coherence tomography (OCT) and Electroretinography (ERG), including flash ERG and Pattern ERG (PERG). Routine ophthalmic examination and B scan of the eye was also done. Flash ERG a-waves and b-waves were recorded, however only a-wave amplitude was evaluated. Pattern ERG n35, n95 and p50 waves were recorded and p50 wave amplitude was evaluated. The a-wave on flash and p50 on pattern waves represent retinal photoreceptor epithelium (RPE) photoreceptor response, which is mainly affected in beta-thalassemia.

Results

Ocular changes were detected in 38.3% and a significant correlation was noted with increase in age (p = 0.045) but not with serum ferritin, transfusion requirements or chelation therapy. Refractive errors were found in 14 cases (23%), such as myopia with astigmatism in 13 (21.7%) and only myopia in 6 subjects (10%). OCT abnormality was noted in 1 patient (1.7%) who had thinning of central retina; right eye 132 μm and left eye 146 μm (n > 200 μm). Abnormalities were noted in a-wave amplitude on flash ERG in 20% of cases, while reduced p50 amplitude on PERG was noted in 15%.

Conclusions

A significant correlation was noted between ocular findings and increase in age, but not with serum ferritin, transfusion requirements or chelation therapy. ERG appears to be a promising tool for screening patients with beta-thalassemia and can serve as a follow-up test for evaluating retinal function.
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