Katerina Haidopoulou

Bronchiectasis secondary to primary immunodeficiency in children: longitudinal changes in structure and function

Primary immunodeficiency is a common cause of bronchiectasis in children. The term bronchiectasis suggests an irreversible process; however, disease progression following treatment is controversial. The aim of this study was to evaluate the progression of bronchiectasis in children with primary immunodeficiency after institution of treatment.

Alveolar LCI vs. standard LCI in detecting early CF lung disease.

Multiple breath washout (MBW) is a sensitive technique that detects early airways disease. However in very young children, large equipment and physiological dead space relative to lung volumes may result in a higher Lung Clearance Index (LCI). We investigated whether alveolar LCI (aLCI) is a more sensitive index than standard LCI in children. MBW data-sets from children aged 0.1-10.7 years [97 healthy controls and 93 with cystic fibrosis (CF)] were analysed. LCI is traditionally calculated by dividing the cumulative expired volume (CEV) by functional residual capacity (FRC) after correcting for equipment dead space. aLCI was calculated similarly, but after correcting the CEV and FRC for Langleys physiological dead space. There was a significant correlation between LCI and aLCI in health (r(2): 0.993; p<0.0001) and disease (r(2): 0.984; p<0.0001). Sensitivity of both LCI and aLCI in detecting abnormal lung function in CF was 39% during infancy, which increased to 77% and 83% respectively in older children. However, the difference in sensitivity (aLCI vs. LCI) was not significant (p=0.36). We conclude that LCI is minimally affected by airway deadspace, or relative equipment deadspace, and is an appropriate measure of lung function in infancy.

Ketogenic diet in a patient with Angelman syndrome

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The role of TLR4 and CD14 polymorphisms in the pathogenesis of respiratory syncytial virus bronchiolitis in greek infants.

Clinical manifestations of respiratory syncytial virus (RSV) infection vary from minimal disease to severe acute bronchiolitis. The structural complex of TLR4/CD14 participates in the virus recognition as a component of natural immune response. Genetic variations of TLR4/CD14 may explain great variations in disease severity. The aim of this study was to investigate the possible role of polymorphisms of TLR4, Asp299Gly and Thr399Ile and CD14, C-159T and C-550T in the development of RSV bronchiolitis. Our study included two groups of Greek infants and young children (A and B). Group A consisted of 50 infants ≤2 years of age hospitalised with bronchiolitis and group B of 99 previously healthy children aged 4–14 years (control group) with a free past medical history. RSV was indentified by PCR of genetic material that was extracted from nasopharyngeal samples collected from all patients. Blood samples were used to extract DNA and by using the PCR-RFLP method we performed TLR4 and CD14 genotyping. We found no association between TLR4 polymorphisms (Asp299Gly and Thr399Ile) and the development of acute bronchiolitis. For CD14 polymorphisms, a positive association was found between the C-159T and the development of bronchiolitis (p=0.05) but not for the other loci. There were no differences detected in the frequencies of the four polymorphisms studied among infants with RSV and non-RSV bronchiolitis. It is concluded that protein CD14 may have a functional role in the viral conjunction to the structural complex TLR4/CD14. The association between the polymorphism C-159T and the manifestation of disease found in our study points out that the severity in the development of acute bronchiolitis is not specified exclusively by the pathogen, but the immune response of the host also plays a significant role. More extensive multicentric studies need to take place, in order to lead to safer conclusions.

Sleep apneas and epilepsy comorbidity in childhood: a systematic review of the literature

PurposeOur aim is to review studies which assess the prevalence of sleep apneas in children with epilepsy and discuss possible mechanisms linking these two conditions, as well as the impact of sleep apneas on the prognosis of these children.MethodsPubMed was used as the medical database source, and articles were selected and classified according to their originality, level of evidence, and relevance to the broad scope of the review.ResultsChildren with epilepsy have a higher prevalence of sleep breathing disorders in comparison to healthy children, but this prevalence varies widely depending on the methodology of each study. Major risk factors for sleep apneas in childhood epilepsy include mainly poor seizure control and antiepileptic drug polytherapy. Indeed, epilepsy can trigger sleep apneas, as abnormal electrical discharge amplifies sleep-induced breathing instability, antiepileptic drugs disturb muscle tone, and vagus nerve stimulation modulates neurotransmission to airway muscles. On the other hand, sleep apneas enhance sleep fragmentation, thus reducing the threshold for the appearance of seizures. Moreover, they have a negative effect on the neurocognitive profile of these children, as they disturb neuroplasticity mechanisms and also have a probable association with sudden unexpected death in epilepsy. The surgical treatment of sleep apneas has been found to reduce seizure frequency, and this can offer new therapeutic choices.ConclusionsBetween sleep apneas and childhood epilepsy, there is a complex relationship with reciprocal interactions. The presence of sleep apneas should be taken into account when designing the management of these children, as it creates therapeutic opportunities and limitations.

Human bocavirus infections in hospitalized Greek children.

Introduction The epidemiology of human bocavirus (HBoV) infections has not been described in Greece, a south-eastern European country. To define the epidemiological profile and the clinical characteristics associated with HBoV infection in a population of children hospitalized with respiratory tract infection. Material and methods During a one-year period throat swab samples were collected from 370 previously healthy children, aged 14 days to 13 years, admitted to two different paediatric wards because of respiratory tract infection. Samples were tested for HBoV by PCR amplifying a part of the NS1 gene. Results Human bocavirus was detected in 12 children (3.2%). Four of the 12 cases were co-infections, 3 of them with influenza A and 1 with coronavirus OC43. Cases were observed only during the cold months. The mean age of children was 1.8 years (range 2 months to 4 years). The most common symptoms were fever, cough and various degrees of respiratory distress. All children were clinically diagnosed as having lower respiratory tract infections, mainly pneumonia and acute laryngotracheobronchitis, and recovered uneventfully. Conclusions HBoV infections occur in Greece mostly among very young children. They accounted for 3.2% of children hospitalized with acute respiratory disease. Cases were observed only in late autumn to early spring.

Acute laryngotracheobronchitis and associated transient hyperphosphatasemia: A new case of transient hyperphosphatasemia in early childhood

We report the case of a 20‐month old boy with markedly elevated serum alkaline phosphatase (ALP) levels, documented during an episode of acute laryngotracheobronchitis. Biochemical investigations and imaging studies revealed no evidence of bone or liver disease. Transient hyperphosphatasemia (TH) was confirmed when serum ALP levels normalized within 2 months. Several theories were suggested for TH pathophysiology, viral infections among them; the exact causes, however, remain unclear. It is important to recognize TH and avoid misdiagnosis and unnecessary investigations.

Reduced exercise capacity in Greek children with mild to moderate obstructive sleep apnea syndrome

Obstructive sleep apnea syndrome (OSAS) is a common disease that is increasingly recognized among pediatric population. The exercise capacity of adults with OSAS has been demonstrated to be impaired, but there are no data about pediatric exercise response.

Impaired glucose metabolism and bronchial hyperresponsiveness in obese prepubertal asthmatic children

Introduction: The prevalence of asthma and obesity has risen in parallel over the last decades, but the exact mechanisms linking these two diseases still remain unclear. The aim of the present study was to investigate the associations between bronchial hyperresponsiveness (BHR), impaired glucose metabolism, obesity, and asthma in prepubertal children. Methods: A total of 71 prepubertal children were included in the study and divided in four groups according to the presence of asthma and their Body Mass Index (BMI): Group 1‐Healthy Controls (HC), Group 2‐Non Obese Asthmatics (NOA), Group 3‐Obese Non Asthmatics (ONA), Group 4‐Obese Asthmatics (OA) Αll children underwent spirometry and bronchial hyperresponsiveness testing by using the cumulative Provoking Dose of mannitol (PD15, primary study variable); homeostasis model assessment‐estimated insulin resistance (HOMA‐IR) index was calculated in order to evaluate insulin resistance. Obese children also underwent an oral glucose tolerance testing (OGTT). Results: A statistically significant difference in bronchial hyperreactivity (mean ± SD) was detected in the group of obese asthmatic children who had lower values of PD15, (174.16 ± 126.42) as compared to normal weight asthmatic children (453.93 ± 110.27), (P < 0.001). Moreover, obese asthmatic children with confirmed insulin resistance (HOMA‐IR ≥2.5), had significantly lower PD15 values (89.05 ± 42.75) as compared to those with HOMA‐IR <2.5 (259.27 ± 125.75), (P = 0.006). Finally, obese asthmatic children with impaired OGTT had likewise significantly lower PD15 (81.02 ± 42.16) measurements as compared to children with normal OGTT (267.3 ± 112.62), (P = 0.001). Conclusion: Our findings suggest that obesity per se does not correlate to airway hyperreactivity unless it is accompanied by glucose intolerance and insulin resistance. Pediatr Pulmonol. 2017;52:160–166.

Sleep respiratory parameters in children with idiopathic epilepsy: A cross-sectional study.

BACKGROUND The aim of this study is to explore and compare through polysomnography respiratory sleep parameters between children with idiopathic epilepsy and healthy children. METHODS Our cross-sectional study included 40 children with idiopathic epilepsy and 27 healthy children, who underwent overnight polysomnography. Data about sleep respiratory parameters were obtained and statistically analyzed. The level of statistical significance was set at 0.05. RESULTS The prevalence of Obstructive Sleep Apnea Syndrome was significantly higher in the epilepsy group (35% vs 7.4%, p<0.01). Moreover, the odds ratio of an obstructive apnea index ≥1 in the epilepsy group was 10.6 (95% Confidence Intervals: 3.08-37.08) in comparison to the control group. The mean value of the obstructive apnea-hypopnea index was significantly higher in children with epilepsy compared to healthy children (2.46±1.22 vs 1.21±0.83, p=0.027). The mean values of central apnea index and desaturation index were comparable between these two groups. Longest apnea duration was significantly higher in the group of poor seizure control. All other sleep respiratory variables did not differ significantly between children with poor and good seizure control and between children with generalized and focal epilepsy. CONCLUSIONS Children with epilepsy seem to present more prominent sleep breathing instability in comparison to healthy children, which mainly includes a predisposition to obstructive respiratory events. More studies are needed to investigate the relationship between sleep apneas and seizure control.