Supine posture changes lung volumes and increases ventilation heterogeneity in cystic fibrosis

INTRODUCTION: Lung Clearance Index (LCI) is recognised as an early marker of cystic fibrosis (CF) lung disease. The effect of posture on LCI however is important when considering longitudinal measurements from infancy and when comparing LCI to imaging studies. METHODS: 35 children with CF and 28 healthy controls (HC) were assessed. Multiple breath washout (MBW) was performed both sitting and supine in triplicate and analysed for LCI, Scond, Sacin, and lung volumes. These values were also corrected for the Fowler dead-space to create 'alveolar' indices. RESULTS: From sitting to supine there was a significant increase in LCI and a significant decrease in FRC for both CF and HC (p<0.01). LCI, when adjusted to estimate 'alveolar' LCI (LCIalv), increased the magnitude of change with posture for both LCIalv and FRCalv in both groups, with a greater effect of change in lung volume in HC compared with children with CF. The % change in LCIalv for all subjects correlated significantly with lung volume % changes, most notably tidal volume/functional residual capacity (Vtalv/FRCalv (r = 0.54,p<0.001)). CONCLUSION: There is a significant increase in LCI from sitting to supine, which we believe to be in part due to changes in lung volume and also increasing ventilation heterogeneity related to posture. This may have implications in longitudinal measurements from infancy to older childhood and for studies comparing supine imaging methods to LCI

The effectiveness of local corticosteroids therapy in the management of mild to moderate viral croup

Aim. The purpose of this study was to determine whether local anti-inflammatory therapy with inhaled beclomethasone dipropionate is effective in the outpatient management of acute viral croup. Methods. Children six months to five years of age, presenting to the Emergency Department (ED) with a croup score of at least 2 participated in the study. All children were assigned in a randomised double-blind fashion to receive either nebulized L-epinephrine (LE), a single intramuscular injection of dexamethasone (D) 0.6 mg/kg, or inhaled beclomethasone dipropionate (BD) 200 mg, via aerochamber. Croup score (CS), heart rate (HR), blood pressure, respiratory rate (RR) and oxygen saturation were recorded at study entry and at 15, 30, 60, 90 and 120 minutes after treatment. Results. Sixty-four patients were enrolled into the study. Significant improvement of the croup score was noticed at the end of observation time in all groups. The LE group showed significant improvements of CS, HR and RR in comparison to the Other two groups. Inhaled BD was as effective as intramuscular D in the treatment of mild to moderate croup in the ED. Conclusion. The use of inhaled beclomethasone in the outpatient management of croup was associated with a significant reduction in the severity of illness within 24 h after treatment

Procalcitonin and Inflammatory Cytokines in Children with Asthma

Asthma is currently defined as a chronic inflammatory disorder of the airway mucosa. The resulting inflammation of the airway mucosa shows signs of an acute as well as a more chronic type of inflammation. Cytokine-mediated interactions among the inflammatory cells may play a role in the pathogenesis of bronchial asthma. The aim of this study is to assess inflammatory agents as markers of chronic inflammation in childhood asthma and as indicators for determining the state of the disease. This study included 3 groups of children. Group A consisted of 35 children with asthma and FEV1<80% of predicted values, 24 boys, 11 girls, aged (mean age ± SE) 9.3 ± 0.4 years, Group B of 70 children with asthma and FEV1≥80% of predicted values, 44 boys, 26 girls, aged 8.65 ± 0.36 years and Group C of 48 healthy children, 33 boys, 15 girls, aged 10.73 ± 0.4 years. In serum, levels of PCT were determined by immunoluminescence, CRP by nephelometry and cytokines IL-1β, IL-6, IL-4 and IL-5 by ELISA. Our results show that the mean concentration of CRP and PCT were not significantly different between groups A, B and C. The mean values of IL-1β were significantly different between groups A, B and C. The mean values of IL-6 were higher in group A than those of Groups B and C, although the difference between the groups was not statistically significant. The mean values of Th2 cytokines IL-4 and IL-5 were significantly different between groups A, B and C. In conclusion, CRP and PCT levels did not play any role in airway allergic inflammation, while strong indications were found that sera levels of inflammatory cytokines associated mainly with Th2 responses play a key role in airway allergic inflammation

Acute respiratory tract infections in SARS-CoV-2-negative children during the COVID-19 pandemic

&lt;p&gt;During the early stage of coronavirus disease 2019 (COVID-19) pandemic, detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was a priority; however, influenza viruses and RSV continued to cause seasonal epidemics complicating the diagnostic strategies. In the present study we estimated the proportion of SARS-CoV-2-negative pediatric cases attributed to respiratory syncytial virus (RSV) and influenza viruses during a 3-month period after the identification of the first COVID-19 case in Greece. Ninety SARS-CoV-2-negative children hospitalized with acute respiratory tract infection were included in the study. Following a SARS-CoV-2 negative result, the samples were tested by molecular methods for detection of RSV and influenza viruses. The positive samples were further tested for identification of the subtype of the viruses.&lt;/p&gt;&lt;p&gt;We detected RSV or influenza viruses in 22 (24.4%) samples. Influenza virus was detected in 13 (14.4%) patients (two of type A and 11 of type B), and RSV (all RSV-A) was detected in 9 (10%) patients.&lt;/p&gt;&lt;p&gt;In conclusion, a syndromic approach for simultaneous detection of at least influenza virus, SARS-CoV-2 and RSV will be beneficial for the prompt implementation of appropriate hospital management including antiviral treatment and isolation measures.&lt;/p&gt

Evidence for treatable inborn errors of metabolism in a cohort of 187 Greek patients with autism spectrum disorder (ASD)

We screened for the presence of inborn errors of metabolism (IEM) in 187 children (105 males; 82 females, ages 4-14 years old) who presented with confirmed features of autism spectrum disorder (ASD). Twelve patients (7%) manifested increased 3-hydroxyisovaleric acid (3-OH-IVA) excretion in urine, and minor to significant improvement in autistic features was observed in seven patients following supplementation with biotin. Five diagnoses included: Lesch Nyhan syndrome (2), succinic semialdehyde dehydrogenase (SSADH) deficiency (2), and phenylketonuria (1) (2.7%). Additional metabolic disturbances suggestive of IEMs included two patients whose increased urine 3-OH-IVA was accompanied by elevated methylcitrate and lactate in sera, and 30 patients that showed abnormal glucose-loading tests. In the latter group, 16/30 patients manifested increased sera beta hydroxybutyrate (b-OH-b) production and 18/30 had a paradoxical increase of sera lactate. Six patients with elevated b-OH-b in sera showed improved autistic features following implementation of a ketogenic diet (KD). Five patients showed decreased serum ketone body production with glucose loading. Twelve of 187 patients demonstrated non-specific MRI pathology, while 25/187 had abnormal electroencephalogram (EEG) findings. Finally, family history was positive for 22/187 patients (1st or 2nd degree relative with comparable symptomatology) and consanguinity was documented for 12/187 patients. Our data provide evidence for a new biomarker (3-OH-IVA) and novel treatment approaches in ASD patients. Concise 1 sentence take-home message: Detailed metabolic screening in a Greek cohort of ASD patients revealed biomarkers (urine 3-hydroxyisovaleric acid and serum b-OH-b) in 7% (13/187) of patients for whom biotin supplementation or institution of a KD resulted in mild to significant clinical improvement in autistic features

Evidence for treatable inborn errors of metabolism in a cohort of 187 Greek patients with autism spectrum disorder (ASD)

We screened for the presence of inborn errors of metabolism (IEM) in 187 children (105 males; 82 females, ages 4–14 years old) who presented with confirmed features of autism spectrum disorder (ASD). Twelve patients (7%) manifested increased 3-hydroxyisovaleric acid (3-OH-IVA) excretion in urine, and minor to significant improvement in autistic features was observed in seven patients following supplementation with biotin. Five diagnoses included: Lesch Nyhan syndrome (2), succinic semialdehyde dehydrogenase (SSADH) deficiency (2), and phenylketonuria (1) (2.7%). Additional metabolic disturbances suggestive of IEMs included two patients whose increased urine 3-OH-IVA was accompanied by elevated methylcitrate and lactate in sera, and 30 patients that showed abnormal glucose-loading tests. In the latter group, 16/30 patients manifested increased sera beta hydroxybutyrate (b-OH-b) production and 18/30 had a paradoxical increase of sera lactate. Six patients with elevated b-OH-b in sera showed improved autistic features following implementation of a ketogenic diet (KD). Five patients showed decreased serum ketone body production with glucose loading. Twelve of 187 patients demonstrated non-specific MRI pathology, while 25/187 had abnormal electroencephalogram (EEG) findings. Finally, family history was positive for 22/187 patients (1st or 2nd degree relative with comparable symptomatology) and consanguinity was documented for 12/187 patients. Our data provide evidence for a new biomarker (3-OH-IVA) and novel treatment approaches in ASD patients. Concise 1 sentence take-home message: Detailed metabolic screening in a Greek cohort of ASD patients revealed biomarkers (urine 3-hydroxyisovaleric acid and serum b-OH-b) in 7% (13/187) of patients for whom biotin supplementation or institution of a KD resulted in mild to significant clinical improvement in autistic features