Adherence to the Mediterranean diet and inflammatory markers in children with asthma.

INTRODUCTION: There is accumulated evidence supporting a beneficial role of Mediterranean diet (MD) in the control of asthma symptoms. The aim of this study was to investigate the relationships between adherence to MD and serum levels of certain cytokines namely, interleukin (IL)-4, and IL-17 known to have a pathogenetic role in the airway changes associated with asthma. METHODS: We measured serum IL-4, IL-33, and IL-17, in 44 asthmatic and 26 healthy children, 5-15 years old. Their adherence to MD was estimated with the Mediterranean Diet Quality Index for children and adolescents (KIDMED) score. RESULTS: KIDMED score did not differ between the two groups (P=0.59) and was not correlated with any of the three measured cytokines. However, when the analysis was restricted only to asthmatic children, the KIDMED score was correlated with IL-4, IL-33, and IL-17 (Beta: -0.56, P=0.007; Beta: 0.57, P=0.010; Beta: -0.62, P=0.017, respectively). CONCLUSION: Our results indicate that MD can modulate the production of some of the main inflammatory mediators of asthma, in asthmatic children.

Anemia and autoimmunity markers in an adolescent with Castleman disease.

We describe herein the case of an adolescent girl with anemia non-responsive to oral iron, associated with low-grade fever, diminished appetite and fatigue. A palpable mass below the xiphoid was noted. Laboratory findings were consistent with anemia of inflammation. Direct antiglobulin test was positive without any other evidence of autoimmune anemia. Other autoantibodies, such as anti-thyroid and anti-nuclear antibodies, were also positive. After thorough investigation, Castleman disease was the most likely diagnosis on the basis of high serum interleukin (IL)-6 and the magnetic resonance imaging findings. (18)F-FDG positron emission tomography-computed tomography showed a localized hypermetabolic mass, which was resected. Castleman disease of plasma type was identified on histology. Hemogloblin and IL-6 gradually returned to normal, whereas positive autoantibodies became negative. This case emphasizes the need to investigate thoroughly for the underlying cause of anemia of inflammation and to include Castleman disease in the differential diagnosis, on the measurement of IL-6.

Acalculous cholecystitis or biliary dyskinesia for Epstein-Barr virus gallbladder involvement?

We present two patients with Epstein-Barr virus (EBV) infection related to gallbladder involvement. Such an association is already known as EBV induced acalculous cholecystitis, diagnosed on the basis of ultrasonographic findings. In our patients, radioisotopic cholescintigraphy was also performed and it showed that gallbladder was visualized in both patients in contrast to that what can be observed in cases of cholecystitis. However, the value of ejection fraction was compatible with biliary dyskinesia. We, therefore, consider that impaired gallbladder contractility in EBV infection cases may actually represent biliary dyskinesia and not acalculous cholecystitis taking into account the radioisotopic findings and the self limited course of the disorder.

Gilbert syndrome.

UNLABELLED: Gilbert syndrome is a common autosomal dominant hereditary condition with incomplete penetrance and characterized by intermittent unconjugated hyperbilirubinemia in the absence of hepatocellular disease or hemolysis. In patients with Gilbert syndrome, uridine diphosphate-glucuronyl transferase activity is reduced to 30% of the normal, resulting in indirect hyperbilirubinemia. In its typical form, hyperbilirubinemia is first noticed as intermittent mild jaundice in adolescence. However, Gilbert syndrome in combination with other prevailing conditions such as breast feeding, G-6-PD deficiency, thalassemia, spherocytosis, or cystic fibrosis may potentiate severe hyperbilirubinemia and/or cholelithiasis. It may also reduce plasma oxidation, and it may also affect drug metabolism. Although in general the diagnosis of the syndrome is one of exclusion, molecular genetic tests can now be performed when there is a diagnostic problem. The most common genotype of Gilbert syndrome is the homozygous polymorphism A(TA)7TAA in the promoter of the gene for UDP-glucuronosyltransferase 1A1 (UGT1A1), which is a TA insertion into the promoter designated UGT1A1*28. No specific management is necessary as Gilbert syndrome is a benign condition. CONCLUSION: Gilbert genotype should be kept in the clinician's mind, at least as a contributor factor, in cases with unexplained indirect hyperbilirubinemia.

Severe jaundice in two children with Kawasaki disease: a possible association with Gilbert syndrome.

Kawasaki disease is a systemic vasculitis, mainly encountered in children. It may affect any organ. Acute cholestasis and severe obstructive jaundice is an atypical manifestation of the disease. We herein present two children with Kawasaki disease and severe direct hypebilibirunemia who also were homozygous and heterozygous respectively for the (TA)(7) promoter polymorphism of Gilbert syndrome. Intravenous immunoglobulin was administered to both patients at the acute phase of the disease and the fever remitted within 24 hr following the immunoglobulin administration. Furthermore oral aspirin at a dose of 80-100 mg/kg/24 hr was also given. The first child did not develop any coronary ectasia or aneurysm, whereas dilation of the right coronary artery was identified in the second child, one month after the disease onset. We discuss the possible contribution of Gilbert syndrome to the development of jaundice in our patients.

MRSA blistering distal dactylitis and review of reported cases.

We describe a 6-month-old infant with blistering distal dactylitis. Bacterial culture from the skin lesion grew methicillin resistant Staphylococcus aureus. No carriage of this bacterial agent was identified in her family. She responded to vancomycin administration and incision and drainage of the lesion. This is the first reported case of methicillin resistant Staphylococcus aureus-associated blistering distal dactylitis in an infant.

Correlation of computed tomography findings and lung function in children and adolescents with cystic fibrosis.

BACKGROUND: The timely and appropriate monitoring of pulmonary status is of utmost importance for patients with cystic fibrosis (CF). Computed tomography (CT) has been used in clinical and research settings for tracking lung involvement in CF patients. However, as CT delivers a considerable amount of radiation, its sequential use in CF patients remains a concern. The application of CT, therefore, should take into account its potential risks. This review aims to understand whether and to what extent the CT findings correlate with the findings from other monitoring tools in CF lung disease. DATA SOURCES: PubMed was searched for articles about the correlation of chest CT findings with spirometric indices and with lung clearance index in children and adolescents with CF. The most relevant articles were reviewed and are presented herein. RESULTS: Most studies have shown that forced expiratory volume in the first second (FEV1) and other spirometric indices correlate moderately with CT structural lung damage. However, at the individual level, there were patients with FEV1 within the normal range and abnormal CT and vice versa. Furthermore, longitudinal studies have indicated that the deterioration of structural lung damage does not occur in parallel with the progression of lung function. Lung clearance index is a better predictor of CT findings. CONCLUSIONS: In general, the existing studies do not support the use of lung function tests as surrogates of chest CT.

Observations in febrile children with round air space opacities.

BACKGROUND: Community-acquired pneumonia in children is rarely depicted as round opacity. The aim of the present study was therefore to describe the clinical and laboratory characteristics of round pneumonia in children. METHODS: The clinical series consisted of 30 children aged 1-7 years (mean age, 3.4 +/- 1.8 years) who were compared to an equal number of children with segmental or lobar pneumonia aged 3-11 years (mean age, 5.5 +/- 2.7 years). RESULTS: Round pneumonia was localized more often in the lower lobe (17/30), and the right side was more commonly affected (20/30). The same, however, was the case for the control group. The two groups were not different regarding the severity of the disease but cough was much more common in the control patients. The white blood cells were considerably higher in round pneumonia, whereas the other inflammatory indices were not significantly different. All round consolidations responded promptly to antibiotics and resolved radiographically in 8 weeks. CONCLUSION: Round consolidations in febrile children associated with elevated inflammatory indices seem to be attributed to round pneumonia, which responds promptly to antibiotics without complications.

Papular-purpuric gloves and socks syndrome in children and adolescents.

We describe the case of a 12-year-old boy with gloves and socks syndrome caused by coinfection with HHV-6 and PVB19, and review the published cases from 5 to 18 years of age to profile the disease in this age group. The review of the literature yielded 25 cases of gloves and socks syndrome. Most patients were febrile and had acute PVB19 infection.

Endocrine-immune interactions in adrenal function of asthmatic children on inhaled corticosteroids.

The present review highlights adrenal function in the context of endocrine-immune interactions and hypothalamic-pituitary-adrenal (HPA) axis activity in asthmatic children on long-term treatment with inhaled corticosteroids (ICS). Activation of the HPA axis by specific cytokines increases the release of cortisol, which in turn feeds back and suppresses the immune reaction. Reduced responsiveness of the HPA axis in patients with various chronic allergic inflammatory disorders and a blunted HPA axis response of poorly controlled asthmatics before long-term treatment with ICS have been reported. It appears that pro- and anti-inflammatory cytokines may be involved in the attenuation of cortisol and ACTH responses to stress in these patients. ICS as anti-inflammatory agents may have favorable effects in asthmatics with baseline subnormal adrenal responses, thus improving adrenal function during successful long-term treatment; on the other hand, few patients on conventional doses may experience further deterioration of adrenal function, a phenomenon that most likely is genetically determined. When ICS are administered at high doses, secondary adrenal insufficiency due to the excessive exogenous corticosteroid certainly may become manifest.

Clinical impact of altered immunoglobulin levels in Henoch-Schönlein purpura.

BACKGROUND: The aim of the present study was the identification of immunological features, present at the time of diagnosis, that would predict the severity of Henoch-Schönlein purpura and its outcome. METHODS: A cohort study was carried out in a tertiary pediatric hospital of 69 children with Henoch-Schönlein purpura, in whom serum complement components C3, C4 and IgA, IgM, IgG were repeatedly determined. RESULTS: During the acute phase of the disease in 54/69 patients (78.3%) immunological imbalances were observed. In 24/54 cases (44.4%) certain complications involving the kidneys and the gastrointestinal tract were noted as opposed to in 3/15 children (20%) without immunologic abnormalities. In 50/69 children (72.5%), elevated serum IgA was detected and 16 of them (32%) developed renal involvement while only 1/19 children (5.3%) with normal IgA concentration had renal involvement. Considering separately the group of 9/69 children (13%) with increased IgM and those with normal IgM levels (53/69; 76.8%), irrespective of IgA and IgG concentration, we found a comparable percentage of children who had both renal and intestinal involvement without, however, developing severe complications, which were exclusively seen in patients with increased IgA (5/7 children) and reduced IgM levels. Serum C3 fraction was elevated in 26 children (37.7%) and in 73% of cases it was associated with increased serum IgA values. CONCLUSION: Renal involvement was seen in 32% of children with increased IgA values. Most importantly, elevated IgA concentration along with reduced IgM levels was associated with higher prevalence of severe complications.

Long-term follow-up of a multifocal hepatic mesenchymal hamartoma producing a-fetoprotein.

We here present a 6-month-old girl with cystic hepatic mesenchymal hamartoma and elevated a-fetoprotein (aFP). Following hepatectomy of the left lobe and partial right lobectomy, decline of the serum aFP was observed. The child has been well for 20 years and it is one among a few cases with such a long observational period.

Meningococcal group A sepsis associated with rare manifestations and complicated by Kawasaki-like disease.

Neisseria meningitidis serogroup A septicemia and the development of Kawasaki disease in a 10-month-old male infant are described. The patient also experienced a number of primary/septic manifestations as consequences of the direct infectious assault and secondary/hypersensitivity immune-mediated complications. The present observation indicates an etiological relationship between invasive meningococcal infection group A and Kawasaki disease, an association in favor of superantigen-induced theory for the latter occurrence.

Applications of lung clearance index in monitoring children with cystic fibrosis.

A sensitive, reproducible and feasible measure of lung function for monitoring the respiratory health is a prerequisite for the optimization of management of the patients with cystic fibrosis (CF). Spirometry has been considered the method of choice, although it is applicable only in children older than 6 years of age, as good cooperation is necessary for its proper performance. However, over the last 15 years, scientific interest in gas dilution techniques and particularly in multiple breath wash out (MBW) method has been revived. The most commonly reported index of MBW is lung clearance index (LCI). The aim of this review is to present the most recent developments in the application of LCI as a monitoring index of respiratory status of CF patients. LCI is a sensitive and reproducible marker of ventilation inhomogeneity. It is more sensitive than spirometry and, unlike spirometry; it can be performed across the whole pediatric age range. Since it is dependent on body size, until at least the age of 6 years, the relative and not the absolute changes are more appropriate for providing clinically meaningful conclusion on ventilation inhomogeneity. Until now, MBW has been mainly used as a research tool. Based on the currently available data LCI cannot safely predict high-resolution computed tomography findings in children with CF, especially in infants. It can be used as an end-point measure for the assessment of beneficial effect of interventions. However, its utility as an outcome measure for the efficacy of therapeutic interventions seems to be dependent on the pathophysiologic mechanisms that underlie each intervention. It seems that more studies, especially longitudinal ones, are required in order to fully clarify the clinical usefulness of LCI, not only in the research setting, but also in every day practice of CF clinic.

Asthma symptoms and bronchial reactivity in school children sensitized to food allergens in infancy.

Food allergy in infancy usually disappears but is followed primarily by respiratory allergy. We hypothesized that children allergic to common food allergens in infancy are at increased risk of wheezing illness and bronchial hyperresponsiveness during school age. In a case-control study 69 children 7.2 to 13.3 years of age allergic to egg (N = 60) and/or fish (N = 29) in early life (first 3 years) who attended our allergy outpatient clinic were recruited. They received follow-up for 1 year and were evaluated by parental questionnaire, skin prick testing, spirometry, and metacholine bronchial challenge. Another 154 children (70 sensitized to inhaled allergens) recruited selectively from a general population sample with no history of food allergy during their first 3 years served as control subjects. Twenty-three children (38.3%) maintained their sensitization to egg and 19 (65.5%) to fish; the prevalence of sensitization to > or = 1 inhaled allergen(s) increased from 59.4% to 71% during childhood. Current asthma symptoms were reported more frequently in the study group than in either control groups, sensitized to inhaled allergens and non-sensitized. Children of the study group showed a significantly increased frequency of positive response to metacholine bronchial challenge compared to the control group as a whole; the difference was statistically indicative when study groups separately were compared to the sensitized control subjects. Multivariate logistic regression analysis showed that bronchial hyperresponsiveness, as well as reported current asthma symptoms were associated with early wheezing and early sensitization to inhaled allergens but not with atopic dermatitis in infancy or persistence of egg or fish allergy. Children allergic to egg or fish in infancy are at increased risk for wheezing illness and hyperactive airways in school age; asthma and bronchial hyperresponsiveness development is mostly determined by wheezing and senzitization to inhaled allergens in early life regardless of atopic dermatitis in infancy or retention of food allergy.

Okamoto syndrome in a girl of Caucasian origin.

We report the clinical and genetic evaluation of a 2-year-old Greek female with striking phenotypic similarities to the three previously published cases of Okamoto syndrome. The main features were characteristic facies, cleft palate, generalized hypotonia, severe developmental delay, congenital hydronephrosis, and congenital heart defects. Routine chromosome testing and whole-genome high-resolution comparative genetic hybridization analysis were negative for any gross numerical or structural chromosome aberrations and for microdeletions/duplications of more than 3 million base pairs respectively. Fluorescence in situ hybridization analysis for 22q11.2 deletion and DNA analysis of the protein tyrosine phosphatase, non-receptor type II gene were normal, thus excluding DiGeorge and Noonan syndromes. Our patient did not show most of the cardinal features of Schinzel-Giedion, otopalatodigital, and C-trigonocephaly syndromes. Moreover, in our patient some new malformations were identified: unilateral kidney hypoplasia and severe anal stenosis. The latter was considered as pertinent and is described here to establish a wider clinical spectrum of Okamoto syndrome. At the age of 3 years 6 months the child continues to show severe growth failure and significant global developmental delay. For the practising paediatrician it is prudent to bear Okamoto syndrome in mind, especially in children with learning disability and a pattern of dysmorphic features.

Breakfast cereal is associated with a lower prevalence of obesity among 10-12-year-old children: the PANACEA study.

BACKGROUND AND AIM: Eating behaviours and obesity status among children have already been evaluated in several studies, with conflicting results. The aim of this study is to assess the correlation of breakfast cereal with childhood obesity. METHODS AND RESULTS: A representative sample of 700 children (323 male) selected from 18 schools located in Athens greater area were enrolled. Children and their parents completed questionnaires that evaluated dietary habits and physical activity. We also retrieved information about the type of breakfast most frequently consumed. Height and weight of the children was measured and body mass index (BMI) was calculated. Simple and multiple logistic regression methods were used in order to determine the relationship between cereal intake for breakfast and obesity. Some boys (8.6%) and girls (9.0%) were obese, whereas 33.9% of boys and 22.1% of girls were overweight. For boys, the adjusted odds ratio for breakfast cereal intake for being overweight or obese was 0.54 (95% confidence interval (CI): 0.45-1.29), while for girls it was 0.41 (95% CI: 0.21-0.79). Moreover, the odds ratio of overweight/obesity for boys who ate daily breakfast was 0.51 (95% CI: 0.25-1.05), and for girls was 0.27 (95% CI: 0.12-0.64), adjusted for physical activity and other potential confounders. CONCLUSION: These data provide evidence that breakfast cereal as a most frequent choice, and daily consumption of breakfast, are inversely associated with the prevalence of overweight or obesity in 10-12-year-old children.

Birthweight, breast-feeding, parental weight and prevalence of obesity in schoolchildren aged 10-12 years, in Greece; the Physical Activity, Nutrition and Allergies in Children Examined in Athens (PANACEA) study.

BACKGROUND: The purpose of the present study was to determine the prevalence of overweight and obesity in a sample of Greek children aged 10-12 years, and to evaluate these rates in relation to parental weight and birthweight. METHODS: During the 2005-2006 school period, 700 schoolchildren (323 boys, 377 girls) were randomly recruited from 18 schools, in Athens. Height and weight were measured and body mass index (BMI) was calculated. Cut-off points for BMI defining obesity and overweight for gender and age were calculated in accordance with international standards. RESULTS: Overall, 8.6% of boys and 9.0% of girls were obese, and 33.9% of boys and 22.1% of girls were overweight. Having an obese parent increased the odds of having an overweight or obese child (P < 0.01). Compared to non-breast-fed, boys who were breast-fed for >3 months had 70% lower likelihood of being overweight or obese (P < 0.01) and breast-fed girls had 80% lower odds (P < 0.01). Excessive birthweight (>3500 g) increased by 2.5-fold the likelihood of being overweight or obese only in girls (P < 0.05). CONCLUSIONS: Parental weight, lack of breast-feeding and excess birthweight (in girls) were significant predictors of overweight or obesity in Greek children aged 10-12 years.

Henoch-Schönlein purpura: a long-term prospective study in Greek children.

OBJECTIVE: Henoch-Schönlein purpura is a common vasculitis of childhood. The present study, comprising 74 children enrolled during a 9-year period, aimed to delineate the clinical spectrum and the long term follow-up of the disease. METHODS: Patients were diagnosed strictly on the criteria established by the American College of Rheumatology. The diagnosis was further strengthened by a skin biopsy performed in more than half of cases (42/74), which revealed leukocytoclastic vasculitis in all patients, whereas IgA deposits were found in 37/42 children. RESULTS: There was a predominance of boys (41 males), and the mean age of study population was 5.2 +/- 2.54 years. Palpable purpura was identified in 73/74 children, whereas transient arthritis manifested in 68/74 children. Gastrointestinal blood loss was elicited in 30 children, 11 of whom had also renal involvement. The latter was seen in 19/74 (25.7%) children, and in the long term follow-up (extended to 2004, from 4-12 years, mean duration 7.3 years), 2 children remained with persistent occult hematuria. Relapses occurred in 49 children (66%), and half of them experienced more than 1 recurrence. Arthritis was less common in the relapsed episodes, and this difference was statistically significant (P < 0.001). Relapses tended to be more common in children with renal involvement and colicky abdominal pain without however reaching statistical significance. CONCLUSION: Despite common relapses, this disease is benign in the long term even if severe renal involvement can occur during its active period. Recurrences often differ to some extent from the initial episode with arthritis being less common with relapses.

Is obesity related to the lung function of non-asthmatic children?

Overweight and obesity are highly prevalent in developed and developing countries among children and adolescents. During the last two decades, it became evident that excess weight is adversely related to respiratory health in childhood and adolescence mainly in terms of asthma occurrence. Additionally, there is a mounting body of evidence that overweight/obesity may also affect lung function in non-asthmatic subjects. The aim of this review was to present and discuss the studies that investigated this issue in non-asthmatic children and adolescents. Only a few studies have evaluated the impact of excess weight on static volumes and their results point towards an inverse relationship between overweight/obesity and functional residual capacity. More studies have been conducted on the impact of excess weight on dynamic lung volumes with inconsistent, however, results. Nevertheless, a relatively consistent finding was that the ratio of forced expiratory volume in 1 s/forced vital capacity was significantly lower among overweight/obese children compared to their counterparts with normal weight. The underlying mechanisms of these observations have not been adequately elucidated but it is believed to result from complex interaction of mechanical, developmental, and metabolic causes. There is a need for more well-designed studies in order to clarify the impact of excess weight on lung function in non-asthmatic subjects, as well as to explore the contribution of factors such as duration and degree of obesity, and fat distribution. Despite the absence of conclusive data, there are still convincing evidence to be communicated to the children and their families as part of the arguments to encourage them to adopt a healthier lifestyle.