TY  - JOUR
AB  - In the neonatal lung, exposure to both prenatal and early postnatal risk factors converge into the development of injury and ultimately chronic disease, also known as bronchopulmonary dysplasia (BPD). The focus of many studies has been the characteristic inflammatory responses provoked by these exposures. Here, we review the relationship between immaturity and prenatal conditions, as well as postnatal exposure to mechanical ventilation and oxygen toxicity, with the imbalance of pro- and anti-inflammatory regulatory networks. In these conditions, cytokine release, protease activity, and sustained presence of innate immune cells in the lung result in pathologic processes contributing to lung injury. We highlight the recruitment and function of myeloid innate immune cells, in particular, neutrophils and monocyte/macrophages in the BPD lung in human patients and animal models. We also discuss dissimilarities between the infant and adult immune system as a basis for the development of novel therapeutic strategies.
AU  - Heydarian, M.
AU  - Schulz, C.*
AU  - Stöger, T.
AU  - Hilgendorff, A.
C1  - 65842
C2  - 52928
CY  - Campus, 4 Crinan St, London, N1 9xw, England
TI  - Association of immune cell recruitment and BPD development.
JO  - Mol. Cell. Pediatr.
VL  - 9
IS  - 1
PB  - Springernature
PY  - 2022
SN  - 2194-7791
ER  - 

TY  - JOUR
AB  - The development of neonatal chronic lung disease (nCLD), i.e., bronchopulmonary dysplasia (BPD) in preterm infants, significantly determines long-term outcome in this patient population. Risk factors include mechanical ventilation and oxygen toxicity impacting on the immature lung resulting in impaired alveolarization and vascularization. Disease development is characterized by inflammation, extracellular matrix remodeling, and apoptosis, closely intertwined with the dysregulation of growth factor signaling. This review focuses on the causes and consequences of altered signaling in central pathways like transforming growth factor (TGF), platelet-derived growth factor (PDGF), and vascular endothelial growth factor (VEGF) driving these above indicated processes, i.e., inflammation, matrix remodeling, and vascular development. We emphasize the shared and distinct role of these pathways as well as their interconnection in disease initiation and progression, generating important knowledge for the development of future treatment strategies.
AU  - Oak, P.
AU  - Hilgendorff, A.
C1  - 52295
C2  - 43897
TI  - The BPD trio? Interaction of dysregulated PDGF, VEGF, and TGF signaling in neonatal chronic lung disease.
JO  - Mol. Cell. Pediatr.
VL  - 4
IS  - 1
PY  - 2017
SN  - 2194-7791
ER  - 

TY  - JOUR
AU  - van den Bruck, R.*
AU  - Weil, P.P.*
AU  - Ziegenhals, T.*
AU  - Schreiner, P.J.*
AU  - Juranek, S.*
AU  - Godde, D.*
AU  - Vogel, S.*
AU  - Schuster, F.*
AU  - Orth, V.*
AU  - Dörner, J.*
AU  - Pembaur, D.*
AU  - Röper, M.*
AU  - Störkel, S.*
AU  - Zirngibl, H.*
AU  - Wirth, S.*
AU  - Jenke, A.C.W.*
AU  - Postberg, J.*
AU  - Boy, N.*
AU  - Heringer, J.*
AU  - Haege, G.*
AU  - Glahn, E.M.*
AU  - Hoffmann, G.F.*
AU  - Garbade, S.F.*
AU  - Burgard, P.*
AU  - Kölker, S.*
AU  - Chao, C.M.*
AU  - Yahya, F.*
AU  - Moiseenko, A.*
AU  - Shrestha, A.*
AU  - Ahmadvand, N.*
AU  - Quantius, J.*
AU  - Wilhelm, J.*
AU  - El-Agha, E.*
AU  - Zimmer, K.P.*
AU  - Bellusci, S.*
AU  - Staufner, C.*
AU  - Prokisch, H.
AU  - Seeliger, S.*
AU  - Müller, M.*
AU  - Hippe, A.*
AU  - Steinkraus, H.*
AU  - Wauer, R.*
AU  - Lachmann, B.*
AU  - Hofmann, S.R.*
AU  - Hedrich, C.M.*
AU  - Zierk, J.*
AU  - Arzideh, F.*
AU  - Haeckel, R.*
AU  - Rascher, W.*
AU  - Rauh, M.*
AU  - Metzler, M.*
AU  - Thieme, S.*
AU  - Bandoła, J.*
AU  - Richter, C.*
AU  - Ryser, M.*
AU  - Jamal, A.*
AU  - Ashton, M.P.*
AU  - von Bonin, M.*
AU  - Kuhn, M.*
AU  - Hedrich, C.M.*
AU  - Bonifacio, E.*
AU  - Berner, R.*
AU  - Brenner, S.*
AU  - Hammersen, J.*
AU  - Has, C.*
AU  - Naumann-Bartsch, N.*
AU  - Stachel, D.*
AU  - Kiritsi, D.*
AU  - Söder, S.*
AU  - Tardieu, M.*
AU  - Bruckner-Tuderman, L.*
AU  - Schneider, H.*
AU  - Bohne, F.*
AU  - Langer, D.*
AU  - Cencic, R.*
AU  - Eggermann, T.*
AU  - Zechner, U.*
AU  - Pelletier, J.*
AU  - Zepp, F.*
AU  - Enklaar, T.*
AU  - Prawitt, D.*
AU  - Pech, M.*
AU  - Weckmann, M.*
AU  - Heinsen, F.A.*
AU  - Franke, A.*
AU  - Happle, C.*
AU  - Dittrich, A.M.*
AU  - Hansen, G.*
AU  - Fuchs, O.*
AU  - von Mutius, E.
AU  - Oliver, B.G.*
AU  - Kopp, M.V.*
AU  - Paret, C.*
AU  - Russo, A.*
AU  - Theruvath, J.*
AU  - Keller, B.*
AU  - El Malki, K.*
AU  - Lehmann, N.*
AU  - Wingerter, A.*
AU  - Neu, M.A.*
AU  - Aslihan, G.A.*
AU  - Wagner, W.*
AU  - Sommer, C.*
AU  - Pietsch, T.*
AU  - Seidmann, L.*
AU  - Faber, J.H.*
AU  - Schreiner, F.*
AU  - Ackermann, M.*
AU  - Michalik, M.*
AU  - Rother, E.*
AU  - Bilkei-Gorzo, A.*
AU  - Rácz, I.*
AU  - Bindila, L.*
AU  - Lutz, B.*
AU  - Dötsch, J.*
AU  - Zimmer, A.*
AU  - Woelfle, J.*
AU  - Fischer, H.S.*
AU  - Ullrich, T.L.*
AU  - Bührer, C.*
AU  - Czernik, C.*
AU  - Schmalisch, G.*
AU  - Stein, R.*
AU  - Hagenbuchner, J.*
AU  - Kiechl-Kohlendorfer, U.*
AU  - Obexer, P.*
AU  - Ausserlechner, M.J.*
AU  - Loges, N.T.*
AU  - Frommer, A.T.*
AU  - Wallmeier, J.*
AU  - Omran, H.*
AU  - Öner-Sieben, S.*
AU  - Gimpfl, M.*
AU  - Rozman, J.
AU  - Irmler, M.
AU  - Beckers, J.
AU  - Hrabě de Angelis, M.
AU  - Roscher, A.*
AU  - Wolf, E.*
AU  - Ensenauer, R.*
AU  - Nemes, K.*
AU  - Frühwald, M.C.*
AU  - Hasselblatt, M.*
AU  - Siebert, R.*
AU  - Kordes, U.*
AU  - Kool, M.*
AU  - Wang, H.*
AU  - Hardy, H.*
AU  - Refai, O.*
AU  - Barwick, K.E.S.*
AU  - Zimmerman, H.H.*
AU  - Weis, J.*
AU  - Baple, E.L.*
AU  - Crosby, A.H.*
AU  - Cirak, S.*
AU  - Hellmuth, C.*
AU  - Uhl, O.*
AU  - Standl, M.
AU  - Heinrich, J.
AU  - Thiering, E.
AU  - Koletzko, B.*
AU  - Blümel, L.*
AU  - Kerl, K.*
AU  - Picard, D.*
AU  - Frühwald, M.C.*
AU  - Liebau, M.C.*
AU  - Reifenberger, G.*
AU  - Borkhardt, A.*
AU  - Hasselblatt, M.*
AU  - Remke, M.*
AU  - Tews, D.*
AU  - Wabitsch, M.*
AU  - Fischer-Posovszky, P.*
AU  - Westhoff, M.A.*
AU  - Nonnenmacher, L.*
AU  - Langhans, J.*
AU  - Schneele, L.*
AU  - Trenkler, N.*
AU  - Debatin, K.M.*
C1  - 51148
C2  - 42876
TI  - Abstracts of the 52nd Workshop for Pediatric Research : Frankfurt, Germany. 27-28 October 2016.
JO  - Mol. Cell. Pediatr.
VL  - 4
PY  - 2017
SN  - 2194-7791
ER  - 

TY  - JOUR
AB  - Chronic lung disease of the newborn, also known as bronchopulmonary dysplasia (BPD), is the most common chronic lung disease in early infancy and results in an increased risk for long-lasting pulmonary impairment in the adult. BPD develops upon injury of the immature lung by oxygen toxicity, mechanical ventilation, and infections which trigger sustained inflammatory immune responses and extensive remodeling of the extracellular matrix together with dysregulated growth factor signaling. Histopathologically, BPD is characterized by impaired alveolarization, disrupted vascular development, and saccular wall fibrosis. Here, we explore the hypothesis that development of BPD involves disturbance of conserved pathways of molecular aging that may contribute to premature aging of the lung and an increased susceptibility to chronic lung diseases in adulthood.
AU  - Meiners, S.
AU  - Hilgendorff, A.
C1  - 49080
C2  - 41594
TI  - Early injury of the neonatal lung contributes to premature lung aging: A hypothesis.
JO  - Mol. Cell. Pediatr.
VL  - 3
IS  - 1
PY  - 2016
SN  - 2194-7791
ER  - 

TY  - JOUR
AB  - Bronchopulmonary dysplasia (BPD) is one of the most common chronic lung diseases in infants caused by pre- and/or postnatal lung injury. BPD is characterized by arrested alveolarization and vascularization due to extracellular matrix remodeling, inflammation, and impaired growth factor signaling. WNT signaling is a critical pathway for normal lung development, and its altered signaling has been shown to be involved in the onset and progression of incurable chronic lung diseases in adulthood, such as chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF). In this review, we summarize the impact of WNT signaling on different stages of lung development and its potential contribution to developmental lung diseases, especially BPD, and chronic lung diseases in adulthood.
AU  - Ota, C.
AU  - Baarsma, H.A.
AU  - Wagner, D.E.
AU  - Hilgendorff, A.
AU  - Königshoff, M.
C1  - 49695
C2  - 40879
SP  - 34
TI  - Linking bronchopulmonary dysplasia to adult chronic lung diseases: Role of WNT signaling.
JO  - Mol. Cell. Pediatr.
VL  - 3
PY  - 2016
SN  - 2194-7791
ER  - 

TY  - JOUR
AB  - Neonatal chronic lung disease in the preterm infant, i.e. bronchopulmonary dysplasia (BPD) is characterized by impaired pulmonary development with its effects persisting into adulthood. Triggered in the immature lung by infectious complications, oxygen toxicity and the impact of mechanical ventilation, a sustained inflammatory response, extensive remodeling of the extracellular matrix, increased apoptosis as well as altered growth factor signaling characterize the disease. The current review focuses on selected pathophysiologic processes and their interplay in disease development. Furthermore, the potential of both, acute and long-term changes to the pulmonary scaffold and the cellular interface in concert with dysregulated growth factor signaling to affect aging and repair processes in the adult lung is discussed.
AU  - Niedermaier, S.
AU  - Hilgendorff, A.
C1  - 47443
C2  - 39329
SP  - 1-7
TI  - Bronchopulmonary dysplasia - an overview about pathophysiologic concepts.
JO  - Mol. Cell. Pediatr.
VL  - 2
IS  - 1
PY  - 2015
SN  - 2194-7791
ER  - 

TY  - JOUR
AB  - Protein homeostasis describes the tight supervision of protein synthesis, correct protein maturation and folding, as well as the timely disposal of unwanted and damaged proteins by the ubiquitin-proteasome pathway or the lysosome-autophagy route. The cellular processes involved in preservation of protein homeostasis are collectively called proteostasis. Dysregulation of proteostasis is an emerging common pathomechanism for chronic lung diseases in the adult and aged patient. There is also rising evidence that impairment of protein homeostasis contributes to early sporadic disease onset in pediatric lung diseases beyond the well-known hereditary proteostasis disorders such as cystic fibrosis and alpha-1 antitrypsin (AAT) deficiency. Identifying the pathways that contribute to impaired proteostasis will provide new avenues for therapeutic interference with the pathogenesis of chronic lung diseases in the young and adult. Here, we introduce the concept of proteostasis and summarize available evidence on dysregulation of proteostasis pathways in pediatric and adult chronic lung diseases.
AU  - Meiners, S.
AU  - Ballweg, K.
C1  - 47341
C2  - 40581
TI  - Proteostasis in pediatric pulmonary pathology.
JO  - Mol. Cell. Pediatr.
VL  - 1
PY  - 2014
SN  - 2194-7791
ER  -