bluuebunny/crossref_metadata_2025_split huggingface dataset:
LYMPHOCYTES IN PERIPHERAL BLOOD AND THYROID TISSUE IN CHILDREN WITH GRAVES' DISEASE
Authors: Ben-Skowronek Iwona , Szewczyk Leszek , Sierocinska-Sawa Jadwiga , Korobowicz Elzbieta 
Published: January 2008

OBJECTIVE: Our goal was to analyze interactions of lymphocytes in peripheral blood and thyroid tissue in children with Graves' disease (GD).
METHODS: The prospective study concerned 15 children affected with GD and 15 healthy children. The levels of autoantibodies against thyrotropin receptor, thyroid peroxidase, and thyroglobulin were assayed. Monoclonal antibodies (Ortho Diagnostic Systems, Raritan, NJ) were used to define peripheral blood lymphocyte subsets and analyzed by using a flow cytometer. After thyroidectomy, thyroid specimens were stained with hematoxylin/eosin. T cells were detected by CD3+, CD4+, and CD8+ antibodies and the antigen-presenting dendritic cells with CD1a+ and CD35+ antibodies (DakoCytomation, Glostrup, Denmark).
RESULTS: Before treatment, all children with GD had increased thyroid autoantibody levels, an increased percentage of CD4+ helper cells, and decreased levels of CD8+ suppressor/cytotoxic T cells, which resulted in an elevated CD4/CD8 ratio. The percentage of CD19+Cd5+ B cells was increased, although the total population of CD19+ B cells did not differ from that of the control group. The number of lymphocytes in the thyroid was decreased in 10 patients after long-term thiamazole treatment. In 5 patients with short-term therapy (&lt;6 months after relapse of GD), the lymphocytes had formed lymphatic follicles: antigen-presenting dendritic cells CD1a+CD35+ in the germinal center and T-helper CD4+, T-suppressor CD8+, and B cells CD79+ on the edges.
CONCLUSIONS: The primary defect of immunoregulation in GD consists of an increase of T-helper lymphocytes with a simultaneous decrease in the number of T-cytotoxic/suppressor cells. Thiamazole therapy probably leads to reduction of the lymphocyte amount in the thyroid.
MILK-SPECIFIC IMMUNOGLOBULIN E/TOTAL IMMUNOGLOBULIN E RATIO AS A PREDICTOR OF POSITIVE ORAL FOOD CHALLENGES IN CHILDREN WITH ALLERGY TO COW'S MILK
Authors: Konstantinou George , Kalobatsou Alexandra , Koutli Maria , Xepapadaki Paraskevi , Douladiris Nikolaos , Saxoni-Papageorgiou Photini , Manoussakis Emmanuel , Papadopoulos Nikolaos G. 
Published: January 2008

INTRODUCTION: Skin-prick test wheal size and serum-specific immunoglobulin E (IgE) levels are able to predict, to an extent, the presence of allergy to certain foods. Nevertheless, the predictive value of these markers is not enough to substitute for oral food challenges, which suggests the need for improvement.
OBJECTIVE: The goal was to determine the prognostic value of specific IgE/total IgE (tIgE) ratio in patients with allergy to cow's milk by using receiver operating characteristic (ROC) analysis.
METHODS: Thirty-four open challenges were performed in children with a mean age of 18.4 months (range: 10.3–69.2 months) who had a previously diagnosed IgE-mediated allergy to cow's milk to reintroduce milk into their diet. Specific IgE levels, assessed by ImmunoCAP fluorescence enzyme immunoassay (Pharmacia Corp, Bridgewater, NJ), were obtained, and skin-prick tests were performed just before the challenge. The specific IgE (f2)/tIgE ratio was evaluated as a potential predictor of a positive challenge and compared with f2 alone by using ROC analysis.
RESULTS: Of 34 challenges, 6 (17.6%) results were positive. Prechallenge milk-specific IgE levels and the f2/tIgE ratio were significant predictors (Pf2 = .007; Pratio = .008) of a positive challenge outcome. After ROC analysis, f2 provided a discrimination (between positive and negative provocations) of 0.8601 (ROC area under the curve); however, the f2/tIgE ratio provided a significantly greater discrimination of 0.9464. Values of the f2/tIgE ratio that are &gt;0.1121 provide a 100% diagnostic accuracy (probability of a positive provocation result).
CONCLUSIONS: The f2/tIgE ratio may be a novel, promising predictor of positive oral food-challenge results and should be evaluated prospectively in a larger sample.
CLINICAL COURSE AND OUTCOME IN CHILDREN WITH RARE CONNECTIVE TISSUE DISEASES: A RETROSPECTIVE REVIEW OF A 17-YEAR EXPERIENCE
Authors: Dracou-Kakava Christina , Karakontinou Katerina , Galetselli Marianthi , Avgeri Maria , Drakonaki Sophia 
Published: January 2008

INTRODUCTION: Juvenile dermatomyositis/juvenile polymyositis (JDM/JPM), juvenile systemic scleroderma (JSCL-SYST), and juvenile mixed connective tissue disease (JMCTD) are rare in childhood.
OBJECTIVE: The objective of this study was to evaluate the prognosis of the rare connective tissue diseases (RCTDs) in children,
METHODS: We reviewed the medical charts of children with a diagnosis of RCTD since 1989 and a minimum follow-up of 5 years.
RESULTS: Twenty-four (16 female, 8 male) children with JDM/JPM, JSCL-SYST, and JMCTD were studied. The age at disease onset ranged from 4 to 13 years. The follow-up duration was 5 to 12 years. Sixteen children had JDM, and 2 had JPM. Four had JSCL-SYST, and 2 had JMCTD. Until now, 13 children have reached clinical remission, lasting &gt;3 years after stopping drug therapy. Twelve children had JDM/JPM, and 1 had JMCTD. Persistent disease activity was noted in 11 children: 4 with JSCL-SYST, 6 with JDM/JPM, and 1 with JMCTD. Severe pulmonary disease developed in 3 children: 2 with JSCL-SYST and 1 with JMCTD. None of the children with JDM had pulmonary disease. Pulmonary hypertension (PH) was found in 2 children with JMCTD or JSCL-SYST. The child with JSCL-SYST and PH died. Persistent scleroderma pattern by wide-field capillaroscopy was noted in 4 children who had JDM and had had skin ulcerations and have developed subcutaneous calcifications. One of them has also had marked muscle atrophy and severe contractures.
CONCLUSIONS: Persistent activity and/or severe pulmonary involvement may be present during the clinical course of RCTD. The presence of PH indicates very poor prognosis in JSCL-SYST/JMCTD cases. Capillaroscopy may identify children who have JDM and are candidates for aggressive therapy.
IMPAIRED DIURNAL BLOOD PRESSURE AND HEART RATE VARIATION AND THEIR RELATIONSHIP WITH LEFT-VENTRICULAR FUNCTION IN ADOLESCENTS WITH TYPE 1 DIABETES MELLITUS
Authors: Karavanaki Kyriaki , Kazianis George , Tsouvalas Emmanouil , Konstantopoulos Ilias , Karayianni Christina 
Published: January 2008

INTRODUCTION: Diabetic cardiomyopathy is a well-defined complication of diabetes that occurs in the absence of ischemic heart disease or hypertension and has been associated with autonomic dysfunction.
OBJECTIVE: Our aim was to evaluate diurnal blood pressure (BP) fluctuations and autonomic function and their possible association with left-ventricular function in adolescents with type 1 diabetes mellitus.
METHODS: In 48 normotensive, normoalbuminuric diabetic adolescents, with a mean (±SD) age of 17.3 (±4.1) years and diabetes duration of 8.5 (±3.3) years, 24-hour ambulatory BP and heart rate (HR) monitoring was performed. Left-ventricular end-diastolic and end-systolic diameters were estimated by echocardiography, and left-ventricular mass index (LVMI) was calculated.
RESULTS: The patients were divided into 2 groups according to the absence of decrease (nondippers) or the decrease (dippers) of nocturnal diastolic BP. The nondippers presented, in comparison with the dippers, reduced mean HR during 24 hours (79.6 vs 84.0 beats/minute; P = .05) and also during daytime (81.3 vs 86.0 beats/minute; P = .05). The nondippers also presented greater end-systolic diameter (28.7 vs 25.9 mm; P = .001) and left-ventricular end-diastolic diameter (47.8 vs 45.1 mm; P = .040) and greater LVMI (90.2 vs 78.3 g/m2; P = .044) compared with the dippers. During stepwise multiple regression, the most important factors affecting LVMI were mean HR (day) (b = −0.40; P = .001), high-frequency variable of heart rate variability (b = 0.38; P = .016), and hemoglobin A1c: (b = 0.67; P = .001).
CONCLUSIONS: A group of normotensive diabetic adolescents with abnormal nocturnal BP reduction and impaired heart rate variation also had impaired left-ventricular function. Our findings suggest that an altered diurnal BP profile, as a result of autonomic dysfunction, may contribute to the development of left-ventricular hypertrophy in patients with type 1 diabetes mellitus.
PREVALENCE OF TUBERCULOSIS AMONG CHILDREN WHO HAD TYPE 1 DIABETES AND WERE ADMITTED TO ELMINIA UNIVERSITY HOSPITAL
Authors: Abdelmoez Basma , Abd-El-Nasser A. , Baheeg Mohamed G. , Sedky Ali A. 
Published: January 2008

INTRODUCTION: Tuberculosis has been a cause of significant morbidity and mortality for humans throughout history. There are 20 million cases of tuberculosis worldwide with 8 million new cases each year. Three million deaths annually are directly attributable to tuberculosis. Previous clinic-based studies in developed countries demonstrated an association between tuberculosis and diabetes but did not determine whether this is attributable an increase in recently transmitted or reactivated infection of tuberculosis.
OBJECTIVE: The objective of this study was to identify the epidemiologic relationship between tuberculosis and diabetes in children by using MycoDot, a simple, rapid, and reliable test.
METHODS: This study was a cross-sectional study of 2 groups. The first group was 110 children who had type 1 diabetes, were aged 5 to 10 years, and had a regular follow-up in the pediatric diabetes outpatient clinic in Elminia University Hospital. The second group consisted of 110 children (as a control group without diabetes) who were age and gender matched from the pediatric outpatient clinic in Elminia University Hospital. The children were subjected to tuberculin skin test and Ziehl Neelsen staining on sputum. The children with diabetes only were subjected to chest radiograph. The children's sera were subjected to MycoDot test.
RESULTS: Among the 110 children with diabetes, 6 (5.5%) were determined to have positive tuberculosis results using the MycoDot technique. Only 1 (0.9%) control patient was determined to have a positive tuberculosis result using the same test. Among the children with diabetes (110), 4 (3.8%) were found to have positive tuberculosis results by tuberculin skin test, whereas 2 (1.8%) were found to have positive tuberculosis results by Ziehl Neelsen staining on sputum.
CONCLUSIONS: Many studies have explored the association between diabetes and tuberculosis. In developed countries, studies dating to the first half of the 20th century demonstrated considerable increase in the frequency of tuberculosis among patients with diabetes, although the proportion with comorbidity ranged widely from 1.0% to 9.3%. Other studies have shown a higher frequency of diabetes among individuals with tuberculosis. In our results, 5.5% of children with diabetes had tuberculosis by MycoDot test, which is a simple and reliable test, whereas only 1 (0.9%) positive result was found in the group without diabetes by the same test. The former indicates that risk for tuberculosis increases among children with diabetes, which indicates that regular screening for the presence of active tuberculosis among children with diabetes should be conducted.
ADIPONECTIN AND PEROXISOME PROLIFERATOR-ACTIVATED RECEPTOR γ EXPRESSION IN SUBCUTANEOUS AND OMENTAL ADIPOSE TISSUE IN CHILDREN
Authors: Li Xiaonan , Lindquist Susanne , Angsten Gertrud , Myrnas Torbjorn , Yi Jun , Chen Ronghua , Hans Stenlund , Olsson Tommy , Hernell Olle 
Published: January 2008

INTRODUCTION: Adiponectin is an adipocyte-specific protein with insulin-sensitizing properties. Peroxisome proliferator-activated receptor γ (PPARγ) may be involved in its gene transcription.
OBJECTIVE: The aim of this study was to compare the expression levels of the genes that encode adiponectin and PPARγ in subcutaneous and omental adipose tissue in children in relation to age and anthropometric variables.
METHODS: Paired biopsies (from subcutaneous and omental adipose tissue) were obtained from 53 children (age: 0.2–14.0 years; BMI: 12.5–25.8 kg/m2). Messenger RNA (mRNA) levels of adiponectin and PPARγ were measured by using reverse-transcription and quantitative real-time polymerase chain reaction.
RESULTS: Adiponectin mRNA levels in adipose tissue were positively associated with PPARγ mRNA levels in children (subcutaneous adipose: r = 0.73, P &lt; .001; omental adipose: r = 0.78, P &lt; .001). In overweight children, the median adiponectin mRNA level was lower in omental adipose tissue (odds ratio: 0.51 [95% confidence interval: 0.1–2.17]) compared with subcutaneous adipose tissue (odds ratio: 1.29 [95% confidence interval: 0.16–5.08]) (P = .032) but not in normal-weight children (P = .54), and the difference remained significant after adjustment for age (P = .045).
CONCLUSIONS: The close association between adiponectin and PPARγ expression supports the hypothesis that PPARγ is involved in adiponectin gene regulation. The fact that adiponectin expression was decreased in omental adipose tissue relative to subcutaneous adipose tissue in overweight children suggests that a risk of insulin resistance may be present in childhood, which allows such resistance to develop after a relatively short duration of overweight.
IDENTIFICATION OF 7 NOVEL TRANSFORMING GROWTH FACTOR β RECEPTOR 2 MUTATIONS IN CHINESE PATIENTS WITH MARFAN SYNDROME
Authors: Chung Brian Hon-Yin , Li Susanna , Lam Stephen Tak-Sum , Yang Wanling , Lun Kin-Shing , Lau Yu-Lung 
Published: January 2008

INTRODUCTION: Marfan syndrome (MFS) (Online Mendelian Inheritance in Man [OMIM] No. 154700) is an autosomal-dominant connective tissue disorder that affects multiple systems including the cardiovascular, ocular, and musculoskeletal systems. Fibrillin 1 (FBN1) (OMIM No. 134797) mutations are causative in &gt;90% of the cases, and recent studies have shown that transforming growth factor β receptor 2 (TGFBR2) (OMIM No. 190182) mutations could be identified in ∼10% of non-FBN1 probands (Mátyás G, Arnold E, Carrel T, et al. Hum Mutat. 2006;27:760–769).
OBJECTIVE: Our objective was to examine the mutation spectrum of TGFBR2 in non-FBN1 Chinese patients with MFS and related phenotypes.
METHODS: All Chinese probands who were referred for evaluation of MFS and tested negative for FBN1 mutations were included. Mutational screening was performed by denaturing high-pressure liquid chromatography (Kosaki K, Udaka T, Okuyama T. Mol Genet Metab. 2005;86:117–123). Amplicons with an abnormal elution pattern were selected for direct sequencing.
RESULTS: Seven novel mutations were identified in 7 of 41 probands. All of them had prominent cardioskeletal phenotypes without ocular or dural involvement, which confirmed previous findings (Disabella E, Grasso M, Marziliano N, et al: Eur J Hum Genet. 2006;14;34–38). Six mutations were missense (R190H, D247V, T325P, G357R, I510N, and T530I), and 1 was frameshift (P501fsX17). Except for R190H, all were found in the functionally important kinase domain. Bioinformatic analyses showed that (1) all mutations occurred in conserved positions by cross-species comparison between 6 orthologs, and (2) R190H, T325P, T530I, and G357R were also found in conserved positions among 3 paralogs (TGFBR1 and activin receptors AVR2A and AVR2B) in the TGFBR superfamily. None of the 7 were found in 50 unaffected individuals (100 normal alleles). With the TGFBR2 mutations, 4 additional probands would fulfill the diagnostic criteria of MFS.
CONCLUSIONS: TGFBR2 mutation was identified in 17% of our non-FBN1 probands. It should be considered in the evaluation for MFS after FBN1 screening, especially if there are compatible clinical features.
N-TERMINAL PRO–BRAIN NATRIURETIC PEPTIDE AND PATENT DUCTUS ARTERIOSUS IN PRETERM INFANTS
Authors: Nuntnarumit Pracha , Thanomsingh Pichaya , Khositseth Anant 
Published: January 2008

INTRODUCTION: N-terminal pro–brain natriuretic peptide (NT-proBNP) in adults has been reported to be a diagnostic marker of ventricular enlargement and volume overload. However, clinical studies using NT-proBNP in premature infants have been very limited.
OBJECTIVE: We sought to determine whether plasma NT-proBNP in premature infants can identify hemodynamically significant patent ductus arteriosus (hsPDA) and determine the correlation between serial plasma NT-proBNP level and echocardiographic assessment of PDA.
METHODS: Thirty-nine preterm infants underwent clinical and echocardiographic examinations for PDA on days-of-life 2, 4, and 7 and simultaneous blood sampling to measure plasma NT-proBNP concentrations. When ≥2 clinical features of PDA were detected along with left-to-right ductal shunting demonstrated by echocardiogram, hsPDA was diagnosed and the patient treated with indomethacin or ibuprofen.
RESULTS: On day 2, the mean NT-proBNP concentration in the hsPDA group (n = 12) was significantly higher than that in the non-hsPDA group (n = 23) (3160.3 ± 3104.9 vs 618.1 ± 490.7 pmol/L; P ≤ .05). Eight infants (72%) in the hsPDA group became asymptomatic after an initial course of indomethacin or ibuprofen, and their NT-proBNP levels concomitantly declined. NT-proBNP concentrations were significantly correlated with the magnitudes of the ductal shunt, such as left-atrium/aorta ratio and left-atrium volume index (r = 0.753 and 0.596, respectively). The cutoff of NT-proBNP concentration at 1204 pmol/L on day 2 gave the best predictive values for hsPDA with 100% sensitivity, 91% specificity, 86.7% positive predictive value, 100% negative predictive value, and a likelihood ratio of 11.5.
CONCLUSIONS: The plasma NT-proBNP level on day-of-life 2 is a sensitive marker for predicting hsPDA in preterm infants. Successful closure of PDA corresponds with a decline in plasma levels of NT-proBNP.
EFFECT OF BODY WEIGHT ON BONE AGE AND HORMONAL PARAMETERS IN CHILDREN WITH PREMATURE ADRENARCHE
Authors: Papathanasiou Asteroula , Lekka Garyfallia , Evangelopoulou Catherine , Petrou Vasilios , Petros Papachilleos , Nikolopoulos Panagiotis , Paraskaki Irini , Hadjiathanasiou Charalambos 
Published: January 2008

OBJECTIVE: Our goal was to investigate the effect of body weight on bone age (BA) and hormonal features in children with benign premature adrenarche.
METHODS: We studied 221 children (175 girls, 46 boys) with premature adrenarche (pubic and/or axillary hair at &lt;8 years of age in girls and &lt;9 years of age in boys) and mean chronological age (CA) at presentation of 7.0 years (girls) and 7.8 years (boys). Anthropometric features and laboratory data (dehydroepiandrosterone sulfate [DHEA-S], 17-hydroxyprogesterone, Δ4-androstenedione [Δ4-A], testosterone, estradiol, insulin-like growth factor I [IGF-I], cholesterol, triglycerides, high- and low-density lipoprotein cholesterol, and BA) were recorded. The population was divided into 3 groups according to BMI: (1) normal weight, (2) overweight (BMI: 85th–95th percentile), and (3) obese (BMI: &gt;95th percentile). Children with late-onset congenital adrenal hyperplasia were excluded from study.
RESULTS: Mean CA of adrenarche was 6.3 years (girls) and 7.1 years (boys). The percentages of overweight and obese children was significantly higher than those reported in the general population of children in Greece. Obese children had significantly more advanced BA compared with overweight and normal-weight children. Higher levels of DHEA-S and Δ4-A were observed in overweight and obese girls compared with normal-weight girls, whereas higher levels of DHEA-S, testosterone, and IFG-I were observed in overweight and obese boys (Table 1). No statistically significant difference was observed between the 3 groups in the levels of 17-hydroxyprogesterone, estradiol, cholesterol, triglycerides, or high- and low-density lipoprotein cholesterol.
CONCLUSIONS: A higher frequency of obesity and advanced BA was observed in children with benign premature adrenarche, with a strong correlation between BA and degree of obesity. Furthermore, obese children were characterized by higher levels of adrenal androgens compared with normal-weight children.
TABLE 1.Levels of Adrenal Androgens in Normal-Weight and Obese ChildrenNormal Weight Mean (Range)Overweight Mean (Range)Obese Mean (Range) P                 a Girls    Testosterone, ng/mL0.11 (0.06–0.20)0.14 (0.10–0.21)0.16 (0.09–0.24).112    DHEAS, μg/mL0.71 (0.41–1.10)0.85 (0.61–1.15)0.88 (0.57–1.21)b.081    Δ 4-A, ng/mL0.4 (0.2–0.8)b0.6 (0.4–1.1)b0.6 (0.4–0.8)b&lt;.05b    IGF-1, ng/mL199 (140–318)236 (180–283)200 (190–266).681Boys    Testosterone, ng/mL0.08 (0.04–0.15)b0.17 (0.06–0.26)b0.28 (0.09–0.39)b&lt;.05b    DHEAS, μg/mL0.51 (0.21–0.92)1.10 (0.82–1.24)b1.12 (0.20–2.12).056    Δ 4-A, ng/mL0.4 (0.2–0.7)0.5 (0.3–0.6)0.6 (0.4–1.0).524    IGF-1, ng/mL200 (89–257)b170 (116–207)b288 (267–369)b&lt;.05baKruskal-Wallis test.bResults were significant.
PREDICTIVE VALUE OF AMPLITUDE-INTEGRATED ELECTROENCEPHALOGRAPHY ON OUTCOME IN NEONATAL EXTRACORPOREAL MEMBRANE OXYGENATION
Authors: Pappas Athina , Shankaran Seetha , Stockmann Paul 
Published: January 2008

INTRODUCTION: The early and accurate assessment of cerebral function in neonates who undergo extracorporeal membrane oxygenation (ECMO) may identify high-risk infants who are amenable to neuroprotective strategies or, at least, in need of more detailed neuroimaging and neurodevelopmental follow-up.
OBJECTIVE: The specific aims of this study were to assess the clinical utility and long-term predictive value of amplitude-integrated electroencephalography (aEEG) in neonatal ECMO.
METHODS: Thirty-four infants who required ECMO for respiratory failure were enrolled in the study prospectively. Serial aEEGs were recorded before, during, and after ECMO and classified by 2 independent interpreters on the basis of background pattern and amplitude criteria. Surviving infants were followed up to 18 to 22 months and assessed with a structured neurologic examination and formal developmental testing by using the Bayley Scales of Infant Development II. The primary outcome was death or neurodevelopmental impairment (defined as moderate-to-severe cerebral palsy and/or a Mental Developmental Index or Psychomotor Development Index score of &lt;70).
RESULTS: Preliminary data analysis on the first 20 patients was performed. Thirteen patients survived, 3 died while on ECMO, and 4 died before discharge. All surviving infants were evaluated at follow-up. A severely abnormal aEEG predicted death or moderate-to-severe neurodevelopmental impairment with a sensitivity of 0.85 (95% confidence interval [CI]: 0.70–0.95), a specificity of 0.57 (95% CI: 0.3–0.76), a positive predictive value of 0.79 (95% CI: 0.65–0.88), and a negative predictive value of 0.67 (95% CI: 0.35–0.88).
CONCLUSIONS: aEEG is a useful neuromonitoring tool during neonatal ECMO.