Molecular and cellular pediatrics最新文献_第5页

How peritoneal dialysis transforms the peritoneum and vasculature in children with chronic kidney disease—what can we learn for future treatment? 腹膜透析如何改变儿童慢性肾脏疾病的腹膜和血管系统——我们对未来的治疗有什么了解?

Molecular and cellular pediatrics Pub Date : 2022-05-05 DOI: 10.1186/s40348-022-00141-3

M. Bartosova, S. Zarogiannis, C. Schmitt, Klaus Gema Aysun K. Rainer Salim Rimante Dorota Sahar Gü Arbeiter Ariceta Bayazit Büscher Caliskan Cerkausk, K. Arbeiter, G. Ariceta, A. Bayazıt, R. Büscher, S. Çalışkan, R. Čerkauskienė, D. Drożdż, S. Fathallah-Shaykh, G. Klaus, R. Krmar, J. Oh, V. Peters, U. Querfeld, B. Ranchin, P. Sallay, B. Schaefer, C. Taylan, S. Testa, J. Vandewalle, E. Verrina, K. Vondrák, B. Warady, Y. Yap, A. Zaloszyc

引用次数: 2

When inflammation meets lung development—an update on the pathogenesis of bronchopulmonary dysplasia 当炎症与肺发育相结合——支气管肺发育不良发病机制的最新进展

Molecular and cellular pediatrics Pub Date : 2022-04-20 DOI: 10.1186/s40348-022-00137-z

Lena Holzfurtner, T. Shahzad, Ying Dong, Lisa Rekers, Ariane Selting, B. Staude, Tina Lauer, A. Schmidt, S. Rivetti, K. Zimmer, Judith Behnke, S. Bellusci, H. Ehrhardt

引用次数: 18

Uni-ventricular palliation vs. bi-ventricular repair: differential inflammatory response 单心室缓解vs双心室修复:不同的炎症反应

Molecular and cellular pediatrics Pub Date : 2022-03-20 DOI: 10.1186/s40348-022-00138-y

M. Sigler, H. Rouatbi, J. Vázquez-Jiménez, M. Seghaye

引用次数: 0

Correction to: Precision medicine in pediatric oncology 更正：儿科肿瘤学中的精准医学

Molecular and cellular pediatrics Pub Date : 2022-03-10 DOI: 10.1186/s40348-022-00140-4

S. Burdach, M. Westhoff, M. Steinhauser, K. Debatin

引用次数: 0

DMBT1 is upregulated in cystic fibrosis, affects ciliary motility, and is reduced by acetylcysteine DMBT1在囊性纤维化中上调，影响纤毛运动，并被乙酰半胱氨酸降低

Molecular and cellular pediatrics Pub Date : 2022-03-05 DOI: 10.1186/s40348-022-00136-0

Alexander Kiefer, Erika Plattner, R. Ruppel, C. Weiss, Z. Zhou-Suckow, M. Mall, M. Renner, H. Müller

引用次数: 2

Perinatal origins of chronic lung disease: mechanisms-prevention-therapy-sphingolipid metabolism and the genetic and perinatal origins of childhood asthma. 慢性肺病的围产期起源:机制-预防-治疗-鞘脂代谢以及儿童哮喘的遗传和围产期起源。

Molecular and cellular pediatrics Pub Date : 2021-12-20 DOI: 10.1186/s40348-021-00130-y

Emily Wasserman, Stefan Worgall

{"title":"Perinatal origins of chronic lung disease: mechanisms-prevention-therapy-sphingolipid metabolism and the genetic and perinatal origins of childhood asthma.","authors":"Emily Wasserman, Stefan Worgall","doi":"10.1186/s40348-021-00130-y","DOIUrl":"https://doi.org/10.1186/s40348-021-00130-y","url":null,"abstract":"Childhood asthma derives from complex host-environment interactions occurring in the perinatal and infant period, a critical time for lung development. Sphingolipids are bioactive molecules consistently implicated in the pathogenesis of childhood asthma. Genome wide association studies (GWAS) initially identified a link between alleles within the 17q21 asthma-susceptibility locus, childhood asthma, and overexpression of the ORMDL sphingolipid biosynthesis regulator 3 (ORMDL3), an inhibitor of de novo sphingolipid synthesis. Subsequent studies of pediatric asthma offer strong evidence that these asthma-risk alleles correlate with early-life aberrancies of sphingolipid homeostasis and asthma. Relationships between sphingolipid metabolism and asthma-related risk factors, including maternal obesity and respiratory viral infections, are currently under investigation. This review will summarize how these perinatal and early life exposures can synergize with 17q21 asthma risk alleles to exacerbate disruptions of sphingolipid homeostasis and drive asthma pathogenesis.","PeriodicalId":74215,"journal":{"name":"Molecular and cellular pediatrics","volume":"8 1","pages":"22"},"PeriodicalIF":0.0,"publicationDate":"2021-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8688659/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39743102","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2

Patho-mechanisms of the origins of bronchopulmonary dysplasia. 支气管肺发育不良起源的病理机制。

Molecular and cellular pediatrics Pub Date : 2021-12-11 DOI: 10.1186/s40348-021-00129-5

Mitali Sahni, Vineet Bhandari

引用次数: 9

Translational research approaches to study pediatric polycystic kidney disease. 儿童多囊肾病的转化研究方法

Molecular and cellular pediatrics Pub Date : 2021-12-09 DOI: 10.1186/s40348-021-00131-x

Max Christoph Liebau, Djalila Mekahli

{"title":"Translational research approaches to study pediatric polycystic kidney disease.","authors":"Max Christoph Liebau, Djalila Mekahli","doi":"10.1186/s40348-021-00131-x","DOIUrl":"https://doi.org/10.1186/s40348-021-00131-x","url":null,"abstract":"Polycystic kidney diseases (PKD) are severe forms of genetic kidney disorders. The two main types of PKD are autosomal recessive and autosomal dominant PKD (ARPKD, ADPKD). While ARPKD typically is a disorder of early childhood, patients with ADPKD often remain pauci-symptomatic until adulthood even though formation of cysts in the kidney already begins in children. There is clinical and genetic overlap between both entities with very variable clinical courses. Subgroups of very early onset ADPKD may for example clinically resemble ARPKD. The basis of the clinical variability in both forms of PKD is not well understood and there are also limited prediction markers for disease progression for daily clinical life or surrogate endpoints for clinical trials in ARPKD or early ADPKD.As targeted therapeutic approaches to slow disease progression in PKD are emerging, it is becoming more important to reliably identify patients at risk for rapid progression as they might benefit from early therapy. Over the past years regional, national and international data collections to jointly analyze the clinical courses of PKD patients have been set up. The clinical observations are complemented by genetic studies and biorepositories as well as basic science approaches to elucidate the underlying molecular mechanisms in the PKD field. These approaches may serve as a basis for the development of novel therapeutic interventions in specific subgroups of patients. In this article we summarize some of the recent developments in the field with a focus on kidney involvement in PKD during childhood and adolescence and findings obtained in pediatric cohorts.","PeriodicalId":74215,"journal":{"name":"Molecular and cellular pediatrics","volume":"8 1","pages":"20"},"PeriodicalIF":0.0,"publicationDate":"2021-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8660924/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39959120","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Imaging of peripheral vascular malformations - current concepts and future perspectives. 外周血管畸形的影像学-目前的概念和未来的展望。

Molecular and cellular pediatrics Pub Date : 2021-12-07 DOI: 10.1186/s40348-021-00132-w

Vanessa F Schmidt, Max Masthoff, Michael Czihal, Beatrix Cucuruz, Beate Häberle, Richard Brill, Walter A Wohlgemuth, Moritz Wildgruber

{"title":"Imaging of peripheral vascular malformations - current concepts and future perspectives.","authors":"Vanessa F Schmidt, Max Masthoff, Michael Czihal, Beatrix Cucuruz, Beate Häberle, Richard Brill, Walter A Wohlgemuth, Moritz Wildgruber","doi":"10.1186/s40348-021-00132-w","DOIUrl":"https://doi.org/10.1186/s40348-021-00132-w","url":null,"abstract":"Vascular Malformations belong to the spectrum of orphan diseases and can involve all segments of the vascular tree: arteries, capillaries, and veins, and similarly the lymphatic vasculature. The classification according to the International Society for the Study of Vascular Anomalies (ISSVA) is of major importance to guide proper treatment. Imaging plays a crucial role to classify vascular malformations according to their dominant vessel type, anatomical extension, and flow pattern. Several imaging concepts including color-coded Duplex ultrasound/contrast-enhanced ultrasound (CDUS/CEUS), 4D computed tomography angiography (CTA), magnetic resonance imaging (MRI) including dynamic contrast-enhanced MR-angiography (DCE-MRA), and conventional arterial and venous angiography are established in the current clinical routine. Besides the very heterogenous phenotypes of vascular malformations, molecular and genetic profiling has recently offered an advanced understanding of the pathogenesis and progression of these lesions. As distinct molecular subtypes may be suitable for targeted therapies, capturing certain patterns by means of molecular imaging could enhance non-invasive diagnostics of vascular malformations. This review provides an overview of subtype-specific imaging and established imaging modalities, as well as future perspectives of novel functional and molecular imaging approaches. We highlight recent pioneering imaging studies including thermography, positron emission tomography (PET), and multispectral optoacoustic tomography (MSOT), which have successfully targeted specific biomarkers of vascular malformations.","PeriodicalId":74215,"journal":{"name":"Molecular and cellular pediatrics","volume":"8 1","pages":"19"},"PeriodicalIF":0.0,"publicationDate":"2021-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8651875/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39576736","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 12

The role of the immune system in idiopathic nephrotic syndrome. 免疫系统在特发性肾病综合征中的作用。

Molecular and cellular pediatrics Pub Date : 2021-11-18 DOI: 10.1186/s40348-021-00128-6

Agnes Hackl, Seif El Din Abo Zed, Paul Diefenhardt, Julia Binz-Lotter, Rasmus Ehren, Lutz Thorsten Weber

{"title":"The role of the immune system in idiopathic nephrotic syndrome.","authors":"Agnes Hackl, Seif El Din Abo Zed, Paul Diefenhardt, Julia Binz-Lotter, Rasmus Ehren, Lutz Thorsten Weber","doi":"10.1186/s40348-021-00128-6","DOIUrl":"https://doi.org/10.1186/s40348-021-00128-6","url":null,"abstract":"Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia and usually responds well to steroids. However, relapses are frequent, which can require multi-drug therapy with deleterious long-term side effects. In the last decades, different hypotheses on molecular mechanisms underlying INS have been proposed and several lines of evidences strongly indicate a crucial role of the immune system in the pathogenesis of non-genetic INS. INS is traditionally considered a T-cell-mediated disorder triggered by a circulating factor, which causes the impairment of the glomerular filtration barrier and subsequent proteinuria. Additionally, the imbalance between Th17/Tregs as well as Th2/Th1 has been implicated in the pathomechanism of INS. Interestingly, B-cells have gained attention, since rituximab, an anti-CD20 antibody demonstrated a good therapeutic response in the treatment of INS. Finally, recent findings indicate that even podocytes can act as antigen-presenting cells under inflammatory stimuli and play a direct role in activating cellular pathways that cause proteinuria. Even though our knowledge on the underlying mechanisms of INS is still incomplete, it became clear that instead of a traditionally implicated cell subset or one particular molecule as a causative factor for INS, a multi-step control system including soluble factors, immune cells, and podocytes is necessary to prevent the occurrence of INS. This present review aims to provide an overview of the current knowledge on this topic, since advances in our understanding of the immunopathogenesis of INS may help drive new tailored therapeutic approaches forward.","PeriodicalId":74215,"journal":{"name":"Molecular and cellular pediatrics","volume":"8 1","pages":"18"},"PeriodicalIF":0.0,"publicationDate":"2021-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8600105/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39902466","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 7