TY - JOUR AB - This report is a summary of the presentations given at the European Respiratory Society's Research Seminar on Asthma Prevention. The seminar reviewed both epidemiological and mechanistic studies and concluded that; (i) reducing exposure of pregnant women and children to air pollution will reduce incident asthma, (ii) there are promising data that both fish oil and a component of raw cow's milk prevent asthma, and (iii) modulating trained immunity by either mimicking helminth infection or oral and sublingual bacterial products is a promising area of research. AU - Grigg, J. AU - Barratt, B.J.* AU - Bønnelykke, K.* AU - Custovic, A.* AU - Ege, M.J. AU - Pasquali, C.* AU - Palomares, O.* AU - Shaheen, S.* AU - Sokolowska, M.* AU - Vercelli, D.* AU - Maizels, R.* AU - von Mutius, E. C1 - 72604 C2 - 56666 CY - 111 River St, Hoboken 07030-5774, Nj Usa TI - European respiratory society research seminar on preventing pediatric asthma. JO - Pediatr. Pulmonol. VL - 60 IS - 1 PB - Wiley PY - 2025 SN - 8755-6863 ER - TY - JOUR AB - BACKGROUND: Aim of the study was to investigate the association of IgE sensitizations in serum and self-reported allergic rhinitis (AR) and asthmatic symptoms. METHODS: Between 2015 and 2018, parents of 1190 children in Günzburg, Germany participated in the study by completing a questionnaire. Settings were the Health-Monitoring-Units (HMU) during the obligatory school entry examinations and a medical examination for all German children at the age of 60-64 months (U9). Random serum samples from 340 children were analyzed for IgE-sensitizations. For possible associations of sensitization and six health outcomes (symptoms of AR, dry cough at night, wheeze, asthma diagnosis, physician-diagnosed asthma, and physician-diagnosed AR), a regression analysis was performed. RESULTS: The parents of 1190 children completed the questionnaire. Parental asthma and physician-diagnosed asthma (OR 4.79; 95%-CI [2.16-10.65]) and underweight at birth/preterm delivery showed the highest associations with asthma based on the German ISAAC definition (OR 3.58; 95%-CI [1.77-7.22]). Out of 340 children blood samples 118 children were sensitized against at least one allergen (airborne pollen allergens, non-seasonal airborne allergens, or food allergens). Sensitized children reported more frequently from symptoms of AR, wheeze, and asthma based on the German ISAAC definition than children without sensitization. CONCLUSION: This observational study identified the prevalence of AR, asthma and existing sensitization among the participating children. Parental asthma and underweight at birth were identified as risk factors for allergic symptoms and asthma. AU - Kutzora, S.* AU - Mehrl, J.* AU - Huß, J.* AU - Buters, J.T.M. AU - Candeias, J. AU - Effner, R. AU - Hendrowarsito, L.* AU - Weinberger, A.* AU - Quartucci, C.* AU - Herr, C.* AU - Heinze, S.* C1 - 73596 C2 - 57129 TI - Survey health data on allergic rhinitis (AR) and asthmatic symptoms and blood samples (IgE Sensitizations) in preschool children: An observational study. JO - Pediatr. Pulmonol. VL - 60 IS - 3 PY - 2025 SN - 8755-6863 ER - TY - JOUR AB - BACKGROUND: Biallelic ATP-binding cassette subfamily A member 3 (ABCA3) variants can cause interstitial lung disease in children and adults, for which no proven treatments exist. Recent in vitro evidence suggested that cyclosporine A (CsA) could correct some ABCA3 variants, however for other variants this is unknown and no data in patients exist. METHODS: We retrieved the clinical data of two children aged 2 and 4 years carrying homozygous ABCA3 variants (G210C and Q1045R, respectively) and empiric CsA treatment from the Kids Lung Register database. In vitro experiments functionally characterized the two variants and explored the effects of CsA alone or combined with hydroxychloroquine (HCQ) in a human alveolar epithelial cell line (A549) derived from adenocarcinoma cells. RESULTS: Six weeks following the introduction of CsA, both children required a reduced O2 flow supply, which then remained stable on CsA. Later, when CsA was discontinued, the clinical status of the children remained unchanged. Of note, the children simultaneously received prednisolone, azithromycin, and HCQ. In vitro, both ABCA3 variants demonstrated defective lysosomal colocalization and impaired ABCA3+ vesicle size, with proteolytic cleavage impairment only in Q1045R. CsA alone corrected the trafficking impairment and ABCA3+ vesicle size of both variants with a variant-specific effect on phosphatidylcholine recycling in G210C. CsA combined with HCQ were additive for improving trafficking of ABCA3 in G210C, but not in Q1045R. CONCLUSIONS: CsA treatment might be helpful for certain patients with ABCA3 deficiency, however, currently strong clinical supporting evidence is lacking. Appropriate trials are necessary to overcome this unmet need. AU - Yang, X.* AU - Forstner, M.E.* AU - Rothenaigner, I. AU - Bullo, M.* AU - Şismanlar, T.E.* AU - Aslan, A.T.* AU - Latzin, P.* AU - Hadian, K. AU - Griese, M.* C1 - 71267 C2 - 55986 TI - Cyclosporine A in children with ABCA3 deficiency. JO - Pediatr. Pulmonol. PY - 2024 SN - 8755-6863 ER - TY - JOUR AB - Objective: Despite the routine use of antenatal steroids, exogenous surfactants, and different noninvasive ventilation methods, many extremely low gestational age neonates, preterm, and term infants eventually require invasive ventilation. In addition to prematurity, mechanical ventilation itself can induce ventilator-induced lung injury leading to lifelong pulmonary sequelae. Besides conventional mechanical ventilation, high-frequency oscillatory ventilation (HFOV) with tidal volumes below dead space and high ventilation frequencies is used either as a primary or rescue therapy in severe neonatal respiratory failure. Methods and Results: Applying a high-resolution computational lung modeling technique in a preterm infant, we studied three different high-frequency ventilation settings as well as conventional ventilation (CV) settings. Evaluating the computed oxygen delivery (OD) and lung mechanics (LM) we outline for the first time how changing ventilator settings from CV to HFOV lead to significant improvements in OD and LM. Conclusion: This personalized “digital twin” strategy advances our general knowledge of protective ventilation strategies in neonatal care and can support decisions on various modes of ventilatory therapy at high frequencies. AU - Förster, K. AU - Roth, C.J.* AU - Hilgendorff, A. AU - Ertl-Wagner, B.* AU - Flemmer, A.W.* AU - Wall, W.A.* C1 - 62854 C2 - 51108 CY - 111 River St, Hoboken 07030-5774, Nj Usa TI - In silico numerical simulation of ventilator settings during high-frequency ventilation in preterm infants. JO - Pediatr. Pulmonol. PB - Wiley PY - 2021 SN - 8755-6863 ER - TY - JOUR AB - Background Pulmonary alveolar proteinosis (PAP) is a heterogeneous condition with more than 100 different underlying disorders that need to be differentiated to target therapeutic options, which are generally limited. Methods The clinical course of two brothers with pathogenic variants in the methionyl-tRNA synthetase (MARS)1gene was compared to previously published patients. Functional studies in patient-derived fibroblasts were performed and therapeutic options evaluated. Results The younger brother was diagnosed with PAP at the age of 1 year. Exome sequencing revealed the homozygousMARS1variant p.(Arg598Cys), leading to interstitial lung and liver disease (ILLD). At 2 years of age, following surgery hypoglycemia was detected, the pulmonary condition deteriorated, and the patient developed multiorgan failure. Six therapeutic whole lung lavages (WLL) were necessary to improve respiratory insufficiency. Methionine supplementation was started and a high protein diet ensured, leading to complete respiratory recovery. The older brother, homozygous for the sameMARS1variant, had a long-known distinct eating preference of methionine-rich food and showed a less severe clinical phenotype. Decreased aminoacylation activity confirmed the pathogenicity of p.(Arg598Cys) in vitro. In agreement with our review of currently published ILLD patients, the presence of hepatopathy, developmental delay, muscular hypotonia, and anemia support the multisystemic character of the disease. Conclusions Catabolic events can provoke a severe deterioration of the pulmonary situation in ILLD with a need for repetitive WLL. Although the precise role of oral methionine supplementation and high protein intake are unknown, we observed an apparent treatment benefit, which needs to be evaluated systematically in controlled trials. AU - Lenz, D.* AU - Stahl, M.* AU - Seidl, E.* AU - Schöndorf, D.* AU - Brennenstuhl, H.* AU - Gesenhues, F.* AU - Heinzmann, T.* AU - Longerich, T.* AU - Mendes, M.I.* AU - Prokisch, H. AU - Salomons, G.S.* AU - Schön, C.* AU - Smith, D.E.C.* AU - Sommerburg, O.* AU - Wagner, M. AU - Westhoff, J.H.* AU - Reiter, K.* AU - Staufner, C.* AU - Griese, M.* C1 - 59940 C2 - 49132 CY - 111 River St, Hoboken 07030-5774, Nj Usa SP - 3057-3066 TI - Rescue of respiratory failure in pulmonary alveolar proteinosis due to pathogenic MARS1 variants. JO - Pediatr. Pulmonol. VL - 55 IS - 11 PB - Wiley PY - 2020 SN - 8755-6863 ER - TY - JOUR AB - BACKGROUND: Rapid weight gain during infancy increases childhood asthma risk, which might be related to impaired lung function. This study investigated associations between peak weight velocity (PWV) during the first two years of life and spirometric lung function indices at 15 years of age. METHODS: Data from 1842 children participating in the GINIplus German birth cohort who underwent spirometry at age 15 were analysed. PWV was calculated from weight measurements obtained between birth and two years of age. Generalised additive models were fitted after adjustment for potential confounding factors (birth weight, height, and age at lung function testing). Results are presented per interquartile range increase (3.5 kg/year) in PWV. RESULTS: PWV was negatively associated with pre-bronchodilation flow rates after extensive adjustment for potential confounders including asthma: forced expiratory flow at 50% of forced vital capacity (FEF50 ) decreased by 141 ml/s (95%CI = [-225;-57]), FEF75 by 84 ml/s [-144;-24] and FEF25-75 by 118 ml/s [-192;-44]. FEV1 /FVC was also negatively associated with PWV (-0.750% [-1.273;-0.226]) whereas forced expiratory volume in 1s (FEV1 ) and forced vital capacity (FVC) were not. Similar results were found for measurements post-bronchodilation. CONCLUSION: Early life weight gain was negatively associated with flow indices in adolescence, suggesting structural changes in peripheral lungs. Pediatr Pulmonol. AU - Flexeder, C. AU - Thiering, E. AU - von Berg, A.* AU - Berdel, D.* AU - Hoffmann, B.* AU - Koletzko, S.* AU - Bauer, C.P.* AU - Koletzko, B.* AU - Heinrich, J. AU - Schulz, H. C1 - 45246 C2 - 37272 CY - Hoboken SP - 147-156 TI - Peak weight velocity in infancy is negatively associated with lung function in adolescence. JO - Pediatr. Pulmonol. VL - 51 IS - 2 PB - Wiley-blackwell PY - 2016 SN - 8755-6863 ER - TY - JOUR AB - BACKGROUND: Epidemiological studies and meta-analyses have shown an increased risk of childhood asthma for children born by Caesarean Section (C-Section). OBJECTIVE: To investigate the effect of delivery by C-Section on lung function and asthma in adolescence in a population-based prospective birth cohort of healthy full term newborns. METHODS: Questionnaire data on mode of delivery and asthma as well as spirometric measurements were available for 1850 adolescents at the age of 15 years, who participated in a follow-up examination of the GINIplus study. Linear regression models were used to examine associations between mode of delivery and lung function parameters. Two reference populations (Lunokid and GLI) were used to calculate the standardized z-scores of lung function parameters. RESULTS: The mean difference in lung function parameters for adolescents born by C-Section, compared to vaginal delivery was not statistically significant. The risk for developing asthma by the age of 15 years was not higher in children born by C-Section-OR: 0.87 (95% CI: 0.57, 1.33) adjusted for sex, age, study center, and parental education level. CONCLUSION: C-Section was not associated with impaired lung function or an increased risk of asthma at the age of 15 years in our birth cohort of healthy full term neonates. AU - Brüske, I. AU - Pei, Z. AU - Thiering, E. AU - Flexeder, C. AU - Berdel, D.* AU - von Berg, A.* AU - Koletzko, S.* AU - Bauer, C.P.* AU - Hoffmann, B.* AU - Heinrich, J. AU - Schulz, H. C1 - 44268 C2 - 36772 SP - 1262-1269 TI - Caesarean Section has no impact on lung function at the age of 15 years. JO - Pediatr. Pulmonol. VL - 50 IS - 12 PY - 2015 SN - 8755-6863 ER - TY - JOUR AU - Hartl, D.* AU - Belohradsky, B.H.* AU - Griese, M.* AU - Nicolai, T.* AU - Krauss-Etschmann, S. AU - Roos, D.* AU - Wintergerst, U.* C1 - 5482 C2 - 23298 SP - 344-348 TI - Celiac disease and pulmonary hemosiderosis in a patient with chronic granulomatous disease. JO - Pediatr. Pulmonol. VL - 38 PY - 2004 SN - 8755-6863 ER -