Citation: Molecular and Cellular Pediatrics 2020 7:18
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Impact of early-life diet on long-term renal health
In the last years, great advances have been made in the effort to understand how nutritional influences can affect long-term renal health. Evidence has accumulated that maternal nutrition before and during pre...
Citation: Molecular and Cellular Pediatrics 2020 7:17
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Evolving pituitary hormone deficits in primarily isolated GHD: a review and experts’ consensus
Isolated growth hormone deficiency (GHD) is defined by growth failure in combination with retarded bone age, low serum insulin-like growth factor-1, and insufficient GH peaks in two independent GH stimulation ...
Citation: Molecular and Cellular Pediatrics 2020 7:16
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Diagnostic and therapeutic odyssey of two patients with compound heterozygous leptin receptor deficiency
Rare genetic variations in the leptin-melanocortin signalling pathway can severely impair appetite regulation and cause extreme obesity in early childhood.
Citation: Molecular and Cellular Pediatrics 2020 7:15
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A case of recurrent herpes simplex 2 encephalitis, VZV reactivations, and dominant partial interferon-gamma-receptor-1 deficiency supports relevance of IFNgamma for antiviral defense in humans
Unlike infections with mycobacteria, reports of unusual viral infections in interferon-gamma-receptor (IFNγR) deficient patients are scarce. Therefore, discussion about increased susceptibility to viral infect...
Citation: Molecular and Cellular Pediatrics 2020 7:14
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A role for the alpha-8 integrin chain (itga8) in glomerular homeostasis of the kidney
Glomerulonephritis results in a dysregulation of glomerular cells and may end up in chronic alterations and subsequent loss of renal function. Therefore, understanding mechanisms, which contribute to maintain ...
Citation: Molecular and Cellular Pediatrics 2020 7:13
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Mutations of uncertain significance in heterozygous variants as a possible cause of severe short stature: a case report
Linear bone growth is achieved by the division of chondrocytes at the growth plate and is regulated by endocrine and paracrine factors such as growth hormone. Mutations that negatively affect chondrogenesis ca...
Citation: Molecular and Cellular Pediatrics 2020 7:11
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Early clinical course after hematopoietic stem cell transplantation in children with juvenile metachromatic leukodystrophy
Long-term outcomes of hematopoietic stem cell transplantation (HSCT) in children with juvenile metachromatic leukodystrophy (MLD) have been investigated systematically, while short-term effects of HSCT on the ...
Citation: Molecular and Cellular Pediatrics 2020 7:12
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Nup133 and ERα mediate the differential effects of hyperoxia-induced damage in male and female OPCs
Hyperoxia is a well-known cause of cerebral white matter injury in preterm infants with male sex being an independent and critical risk factor for poor neurodevelopmental outcome. Sex is therefore being widely...
Citation: Molecular and Cellular Pediatrics 2020 7:10
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Osteogenesis imperfecta—pathophysiology and therapeutic options
Osteogenesis imperfecta (OI) is a rare congenital disease with a wide spectrum of severity characterized by skeletal deformity and increased bone fragility as well as additional, variable extraskeletal symptom...
Citation: Molecular and Cellular Pediatrics 2020 7:9
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Genotype-phenotype correlations in children and adolescents with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency is caused by mutations in the active 21-hydroxylase gene (CYP21A2). The clinical symptoms can vary greatly. To date, no systematic s...
Citation: Molecular and Cellular Pediatrics 2020 7:8
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DNA methylation biomarkers of future health outcomes in children
Biomarkers which predict future health outcomes are key to the goals of precision health. Such biomarkers do not have to be involved in the causal pathway of a disease, and their performance is best assessed u...
Citation: Molecular and Cellular Pediatrics 2020 7:7
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Infant formula with cow’s milk fat and prebiotics affects intestinal flora, but not the incidence of infections during infancy in a double-blind randomized controlled trial
The postnatal intestinal colonization of human milk-fed and formula-fed infants differs substantially, as does the susceptibility to infectious diseases during infancy. Specific ingredients in human milk, such...
Citation: Molecular and Cellular Pediatrics 2020 7:6
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Pro-inflammatory cytokine ratios determine the clinical course of febrile neutropenia in children receiving chemotherapy
Febrile neutropenia is a common and serious complication during treatment of childhood cancer. Empirical broad-spectrum antibiotics are usually administered until neutrophil cell count recovery. It was the aim...
Citation: Molecular and Cellular Pediatrics 2020 7:5
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Modulation of CYP2E1 metabolic activity in a cohort of confirmed caffeine ingesting pregnant women with preterm offspring
To ascertain interactions of caffeine ingestion, food, medications, and environmental exposures during preterm human gestation, under informed consent, we studied a cohort of Mexican women with further preterm...
Citation: Molecular and Cellular Pediatrics 2020 7:4
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Shedding light on pediatric diseases: multispectral optoacoustic tomography at the doorway to clinical applications
Optoacoustic imaging (OAI), or photoacoustic imaging (PAI), has fundamentally influenced basic science by providing high-resolution visualization of biological mechanisms. With the introduction of multispectra...
Citation: Molecular and Cellular Pediatrics 2020 7:3
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Random X chromosome inactivation in patients with Klinefelter syndrome
X chromosome inactivation (XCI) is an indispensable process in the development of human female embryos. Reportedly, XCI occurs when a blastocyst contains 10–12 embryonic progenitor cells. To date, it remains u...
Citation: Molecular and Cellular Pediatrics 2020 7:1
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Shaping of the nephron – a complex, vulnerable, and poorly explored backdrop for noxae impairing nephrogenesis in the fetal human kidney
The impairment of nephrogenesis is caused by noxae, all of which are significantly different in molecular composition. These can cause an early termination of nephron development in preterm and low birth weigh...
Citation: Molecular and Cellular Pediatrics 2020 7:2
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Functional analysis of granulocyte and monocyte subpopulations in neonates
Neonate immune cell functions lack full protection against pathogens. This could be either defect or protective mechanism against overshooting proinflammatory immune responses.
Citation: Molecular and Cellular Pediatrics 2019 6:5
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Vegetarian diets in childhood and adolescence
In Western countries, vegetarian diets are associated with lower intakes of energy, saturated fatty acids and animal protein and higher intakes of fibre and phytochemicals, compared to omnivorous diets. Whethe...
Citation: Molecular and Cellular Pediatrics 2019 6:4
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Vitamin D supplementation after the second year of life: joint position of the Committee on Nutrition, German Society for Pediatric and Adolescent Medicine (DGKJ e.V.), and the German Society for Pediatric Endocrinology and Diabetology (DGKED e.V.)
Low vitamin D serum concentrations have been associated with rickets and other disorders in observational studies. Since vitamin D serum concentrations in children and adolescents are frequently below referenc...
Citation: Molecular and Cellular Pediatrics 2019 6:3
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Complementary foods in baby food pouches: position statement from the Nutrition Commission of the German Society for Pediatrics and Adolescent Medicine (DGKJ, e.V.)
Pureed complementary feeding products packed in squeezable plastic pouches, usually with a spout and a screw cap, have been increasingly marketed. The Committee on Nutrition recommends that infants and young c...
Citation: Molecular and Cellular Pediatrics 2019 6:2
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KUNO-Kids birth cohort study: rationale, design, and cohort description
Birth cohort studies can contribute substantially to the understanding of health and disease — in childhood and over the life course. The KUNO-Kids birth cohort study was established to investigate various asp...
Citation: Molecular and Cellular Pediatrics 2019 6:1
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Gene correction of HBB mutations in CD34+ hematopoietic stem cells using Cas9 mRNA and ssODN donors
β-Thalassemia is an inherited hematological disorder caused by mutations in the human hemoglobin beta (HBB) gene that reduce or abrogate β-globin expression. Although lentiviral-mediated expression of β-globin an...
Citation: Molecular and Cellular Pediatrics 2018 5:9
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Chemotherapy and the pediatric brain
Survival rates of children with cancer are steadily increasing. This urges our attention to neurocognitive and psychiatric outcomes, as these can markedly influence the quality of life of these children. Neuro...
Citation: Molecular and Cellular Pediatrics 2018 5:8
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The role of S100 proteins in the pathogenesis and monitoring of autoinflammatory diseases
S100A8/A9 and S100A12 are released from activated monocytes and granulocytes and act as proinflammatory endogenous toll-like receptor (TLR)4-ligands. S100 serum concentrations correlate with disease activity, ...
Citation: Molecular and Cellular Pediatrics 2018 5:7
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Precision medicine in pediatric oncology
Outcome in treatment of childhood cancers has improved dramatically since the 1970s. This success was largely achieved by the implementation of cooperative clinical research trial groups that standardized and ...
Citation: Molecular and Cellular Pediatrics 2018 5:6
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Anti-inflammatory monocytes—interplay of innate and adaptive immunity
Monocytes are central to our health as they contribute to both hemispheres of our immune system, the innate and the adaptive arm. Sensing signals from the outside world, monocytes govern the innate immunity by...
Citation: Molecular and Cellular Pediatrics 2018 5:5
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Intrauterine growth restriction - impact on cardiovascular diseases later in life
Intrauterine growth restriction (IUGR) is a fetal pathology which leads to increased risk for certain neonatal complications. Furthermore, clinical and experimental studies revealed that IUGR is associated wit...
Citation: Molecular and Cellular Pediatrics 2018 5:4
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The potential of antisense oligonucleotide therapies for inherited childhood lung diseases
Antisense oligonucleotides are an emerging therapeutic option to treat diseases with known genetic origin. In the age of personalised medicines, antisense oligonucleotides can sometimes be designed to target a...
Citation: Molecular and Cellular Pediatrics 2018 5:3
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CISH promoter polymorphism effects on T cell cytokine receptor signaling and type 1 diabetes susceptibility
Impaired regulatory T cell immunity plays a central role in the development of type 1 diabetes (T1D). Interleukin-2 receptor (IL-2R) signaling is essential for regulatory T cells (TREG), and cytokine-inducible SH...
Citation: Molecular and Cellular Pediatrics 2018 5:2
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Preserved in vitro immunoreactivity in children receiving long-term immunosuppressive therapy due to inflammatory bowel disease or autoimmune hepatitis
Children with inflammatory bowel disease (IBD) or autoimmune hepatitis (AIH) are at risk for severe infections. This is partially a result of their chronic disease condition but, moreover, a side effect of the...
Citation: Molecular and Cellular Pediatrics 2018 5:1
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Concepts for a therapeutic prolongation of nephrogenesis in preterm and low-birth-weight babies must correspond to structural-functional properties in the nephrogenic zone
Numerous investigations are dealing with anlage of the mammalian kidney and primary development of nephrons. However, only few information is available about the last steps in kidney development leading at bir...
Citation: Molecular and Cellular Pediatrics 2017 4:12
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The BPD trio? Interaction of dysregulated PDGF, VEGF, and TGF signaling in neonatal chronic lung disease
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 m...
Citation: Molecular and Cellular Pediatrics 2017 4:11
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Estimated prevalence of potentially damaging variants in the leptin gene
Mutations in the leptin gene (LEP) can alter the secretion or interaction of leptin with its receptor, leading to extreme early-onset obesity. The purpose of this work was to estimate the prevalence of heteroz...
Citation: Molecular and Cellular Pediatrics 2017 4:10
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Transcatheter atrial septal defect closure in an infant (body weight 6.4 kg) using the GORE CARDIOFORM septal occluder (GCSO)
Transcatheter closure has become the treatment of choice for secundum atrial septal defects (ASD II), but particularly in small children, there is concern regarding procedure-related complications.
Citation: Molecular and Cellular Pediatrics 2017 4:9
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Autism spectrum disorder and Li-Fraumeni syndrome: purely coincidental or mechanistically associated?
Autism spectrum disorders (ASDs) are neurodevelopmental disorders with impaired social interactions and communication and restrictive, repetitive patterns of behaviors, interests, and activities. A recent epid...
Citation: Molecular and Cellular Pediatrics 2017 4:8
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The molecular pathophysiology of chronic non-bacterial osteomyelitis (CNO)—a systematic review
Chronic non-bacterial osteomyelitis (CNO) belongs to the growing spectrum of autoinflammatory diseases and primarily affects the skeletal system. Peak onset ranges between 7 and 12 years of age. The clinical s...
Citation: Molecular and Cellular Pediatrics 2017 4:7
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Pediatric precursor B acute lymphoblastic leukemia: are T helper cells the missing link in the infectious etiology theory?
Precursor B acute lymphoblastic leukemia (BCP-ALL), the most common childhood malignancy, arises from an expansion of malignant B cell precursors in the bone marrow. Epidemiological studies suggest that infect...
Citation: Molecular and Cellular Pediatrics 2017 4:6
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Citation: Molecular and Cellular Pediatrics 2017 4(Suppl 1):5
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Human perinatal immunity in physiological conditions and during infection
The intrauterine environment was long considered sterile. However, several infectious threats are already present during fetal life. This review focuses on the postnatal immunological consequences of prenatal ...
Citation: Molecular and Cellular Pediatrics 2017 4:4
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Role of second-hand smoke (SHS)-induced proteostasis/autophagy impairment in pediatric lung diseases
Exposure to second-hand tobacco smoke (SHS) is one of the prime risk factors for chronic lung disease development. Smoking during pregnancy may lead to birth defects in the newborn that include pulmonary dysfu...
Citation: Molecular and Cellular Pediatrics 2017 4:3
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Renal consequences of preterm birth
The developmental origin of health and disease concept identifies the brain, cardiovascular, liver, and kidney systems as targets of fetal adverse programming with adult consequences. As the limits of viabilit...
Citation: Molecular and Cellular Pediatrics 2017 4:2
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Acquisition and adaptation of the airway microbiota in the early life of cystic fibrosis patients
Cystic fibrosis (CF) is a genetic disease in which bacterial infections of the airways play a major role in the long-term clinical outcome. In recent years, a number of next-generation sequencing (NGS)-based s...
Citation: Molecular and Cellular Pediatrics 2017 4:1
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Neutrophil plasticity enables the development of pathological microenvironments: implications for cystic fibrosis airway disease
The pathological course of several chronic inflammatory diseases, including cystic fibrosis, chronic obstructive pulmonary disease, and rheumatoid arthritis, features an aberrant innate immune response dominat...
Citation: Molecular and Cellular Pediatrics 2016 3:38
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Severe changes in colon epithelium in the Mecp2-null mouse model of Rett syndrome
Rett syndrome is best known due to its severe and devastating symptoms in the central nervous system. It is produced by mutations affecting the Mecp2 gene that codes for a transcription factor. Nevertheless, evid...
Citation: Molecular and Cellular Pediatrics 2016 3:37
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Wilms’ tumor susceptibility: possible involvement of FOXP3 and CXCL12 genes
Wilms’ tumor is an embryonal neoplasm of the kidney that accounts for approximately 6 % of all childhood tumors. The chemokine CXCL12 (C-X-C chemokine ligand 12) and its ligand CXCR4 (C-X-C chemokine receptor ...
Citation: Molecular and Cellular Pediatrics 2016 3:36
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Leukocyte recruitment in preterm and term infants
Impaired cellular innate immune defense accounts for susceptibility to sepsis and its high morbidity and mortality in preterm infants. Leukocyte recruitment is an integral part of the cellular immune response ...
Citation: Molecular and Cellular Pediatrics 2016 3:35
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Linking bronchopulmonary dysplasia to adult chronic lung diseases: role of WNT signaling
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...
Citation: Molecular and Cellular Pediatrics 2016 3:34
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Transient spontaneous remission in congenital MLL-AF10 rearranged acute myeloid leukemia presenting with cardiorespiratory failure and meconium ileus
Neonatal leukemia is a rare disease with an estimated prevalence of about one to five in a million neonates. The majority being acute myeloid leukemia (AML), neonatal leukemia can present with a variety of sym...
Citation: Molecular and Cellular Pediatrics 2016 3:30
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