2,250 results match your criteria: "Dr von Hauner Children's Hospital[Affiliation]"

European Respiratory Society Research Seminar on Preventing Pediatric Asthma.

Pediatr Pulmonol

January 2025

Institute of Asthma and Allergy Prevention, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, Germany.

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.

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Article Synopsis
  • Flavivirus infections are widespread and pose significant public health risks, necessitating the urgent development of a safe and effective vaccine, with the self-assembled pan-epitope vaccine being a promising approach.* -
  • The study created a nanovaccine called TBT-CpG NaVs, which was found to be stable and effectively taken up by immune cells, enhancing their ability to present antigens and trigger an immune response.* -
  • In tests, TBT-CpG NaVs increased specific antibody levels and showed protective benefits against Dengue and Zika viruses, with a good safety profile and no detected organ damage in vaccinated mice, indicating its potential for future vaccine development.*
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  • * A study involving 2,727 children showed that ICH occurred in 2.2% of cases, mostly before the age of one, with a significantly higher incidence in hemophilia A compared to hemophilia B.
  • * Early diagnosis of hemophilia is crucial, as 40% of ICH cases happened before the diagnosis, and preventive treatment could potentially lower the incidence of ICH if initiated promptly.
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Identification of Biochemical Determinants for Diagnosis and Prediction of Severity in 5q Spinal Muscular Atrophy Using H-Nuclear Magnetic Resonance Metabolic Profiling in Patient-Derived Biofluids.

Int J Mol Sci

November 2024

Division of Child Neurology and Metabolic Medicine, Department of Pediatrics I, Center for Pediatrics and Adolescent Medicine, Medical Faculty Heidelberg, University Hospital Heidelberg, Heidelberg University, 69120 Heidelberg, Germany.

This study explores the potential of H-NMR spectroscopy-based metabolic profiling in various biofluids as a diagnostic and predictive modality to assess disease severity in individuals with 5q spinal muscular atrophy. A total of 213 biosamples (urine, plasma, and CSF) from 153 treatment-naïve patients with SMA across five German centers were analyzed using H-NMR spectroscopy. Prediction models were developed using machine learning algorithms which enabled the patients with SMA to be grouped according to disease severity.

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Enrolling in a clinical trial or study requires informed consent. Furthermore, it is crucial to ensure proper consent when storing samples in biobanks for future research, as these samples may be used in studies beyond their initial purpose. For pediatric studies, consent must be obtained from both the child and their legal guardians, requiring the recording of multiple consents at once.

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Individuals with monoallelic pathogenic variants in the histone lysine methyltransferase DOT1L display global developmental delay and varying congenital anomalies. However, the impact of monoallelic loss of remains unclear. Here, we present a largely female cohort of 11 individuals with variants with developmental delays and dysmorphic facial features.

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Exploring the genetics of airflow limitation in lung function across the lifespan - a polygenic risk score study.

EClinicalMedicine

September 2024

Department of Clinical Science and Education, Södersjukhuset, Karolinska Institutet, Sjukhusbacken 10, 118 83, Stockholm, Sweden.

Article Synopsis
  • Chronic obstructive pulmonary disease (COPD) can be influenced by genetic factors and may stem from reduced lung growth during childhood, leading to lower lung function throughout life.
  • A polygenic risk score (PRS) was calculated using data from a large genome-wide association study and tested for its correlation with lung function in individuals aged 4-50 from multiple research cohorts.
  • Results indicated that higher PRS scores were associated with significantly lower lung function, measured by key indicators, starting from childhood and continuing into adulthood, regardless of smoking, sex, or asthma diagnosis.
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Dupilumab in a 9-week-old with Netherton Syndrome Leads to Deep Symptom Control.

J Clin Immunol

November 2024

Department of Pediatrics, Dr von Hauner Children's Hospital, Ludwig-Maximilians-University Munich, Lindwurmstraße 4 , Munich, European Union (EU), D-80337 , Germany.

Purpose: Netherton syndrome (NS) is a rare inborn error of immunity (IEI) with an incidence of approximately 1:200,000 and the phenotypic triad of trichorrhexis invaginate (bamboo hair), congenital ichthyosiform erythroderma, and multiple atopic manifestations. Treatment options especially in infants are scarce and generally not licensed.

Methods: Case report of a 9-week-old infant with NS treated with dupilumab off-label.

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The main monogenic causes of pulmonary fibrosis in adults are mutations in telomere-related genes. These mutations may be associated with extrapulmonary signs (hepatic, haematological and dermatological) and typically present radiologically as usual interstitial pneumonia or unclassifiable fibrosis. In children, the monogenic causes of pulmonary fibrosis are dominated by mutations in surfactant-related genes.

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Diagnosis of fetal alcohol spectrum disorders: German guideline version 2024.

Eur J Paediatr Neurol

November 2024

Department of Paediatric Neurology and Developmental Medicine, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University of Munich, Germany.

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Prophylaxis is the standard of care for patients with severe hemophilia, patients with moderate hemophilia, or those with another congenital bleeding disorder that is associated with a severe bleeding phenotype and/or a high risk of spontaneous life-threatening bleeding. Patients with nonsevere hemophilia (factor VIII [FVIII] ≥ 1%) may also have a bleeding phenotype that requires prophylaxis. To date, however, there are no clear criteria as to when prophylaxis is indicated in these patients.

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Drug prioritization identifies panobinostat as a tailored treatment element for patients with metastatic hepatoblastoma.

J Exp Clin Cancer Res

November 2024

Department of Pediatric Surgery, Dr. von Hauner Children's Hospital, University Hospital, LMU Munich, Lindwurmstreet 2a, Munich, 80337, Germany.

Background: Patients with metastatic hepatoblastoma are treated with severely toxic first-line chemotherapies in combination with surgery. Yet, inadequate response of lung metastases to neo-adjuvant chemotherapy still compromises patient outcomes making new treatment strategies, tailored to more efficient lung clearance, mandatory.

Methods: We harnessed a comprehensive patient-derived xenograft platform and a variety of in vitro and in vivo assays to establish the preclinical and biological rationale for a new drug for patients with metastatic hepatoblastoma.

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Background: There is a lack of studies evaluating longitudinal changes in adiposity indicators and the association with 24-hour movement behavior guidelines in preschoolers. Therefore, this study aimed to investigate associations between changes in Body Mass Index (BMI) z-scores, waist circumference and waist-to-height ratio, and compliance with the 24-hour movement behavior guidelines in three- to six-year-old children from a European sample.

Methods: In total, data from a European sample of 719 preschoolers (50.

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Article Synopsis
  • Integrin alpha V is crucial for cell adhesion and signaling during development, and mutations in its gene (ITGAV) can lead to serious health issues.
  • In three families, biallelic variants were found that caused either dysfunctional protein production or the integrin being misplaced, resulting in severe developmental problems like eye and brain abnormalities, inflammatory bowel disease, and immune issues.
  • Studies in patient cells and zebrafish models confirmed these mutations resulted in impaired immune signaling and developmental defects, linking the ITGAV variants to a newly identified human disease.
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Background: Humans are subjected to various environmental stressors (bacteria, viruses, pollution) throughout life. As such, an inherent relationship exists between the effect of these exposures with age. The impact of these environmental stressors can manifest through DNA methylation (DNAm).

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Background: Esophageal strictures following esophageal atresia repair are a source of significant morbidity. To test new therapeutic approaches, we designed a piglet model of esophageal stricture by resecting variable lengths of esophagus with subsequent re-anastomosis. This study describes the model and validates its physiologic impact by blinded analysis of the weight gains of the piglets.

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Aim: Enterostomy takedown is common in neonates with Hirschsprung's disease, anorectal malformations, or necrotizing enterocolitis. Stapled bowel anastomosis has become routine in adults, but size of up to 12 mm diameter precludes performing enterostomy takedown in young infants using regular intestinal staplers. After the introduction of miniature (5 mm diameter) staplers, we increasingly used them for enterostomy takedown.

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Congenital Anomalies of the Kidney and Urinary Tract: A Continuum of Care.

Semin Nephrol

October 2023

Division of Pediatric Nephrology, Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany.

Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of kidney failure in children and adolescents. CAKUT describes a wide spectrum of structural disorders with a prenatal origin. The etiology of CAKUT is multifactorial, including environmental, nongenetic, and genetic causes that impact kidney development as well as upper and lower urinary tract development.

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Magnets in Paediatric Surgery.

J Pediatr Surg

October 2024

Department of Paediatric Surgery, Dr. von Hauner Children's Hospital, LMU University Hospital, Lindwurmstrasse 4, 80337 Munich, Germany. Electronic address:

Magnetism, recognized in ancient Greece and China, is a fundamental physical force influencing numerous domains, including medicine and surgery. Historically, the medical use of magnets dates back over two millennia. As proof, the ancient Sanskrit medical textbook Sushruta Samhita describes the removal of a metallic arrow lodged in the flesh with a magnet.

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Season of birth, viral infections, HLA haplogenotypes and non-HLA variants are implicated in the development of celiac disease and celiac disease autoimmunity, suggesting a combined role of genes and environmental exposures. The aim of the study was to further decipher the biological pathways conveying the season of birth effect in celiac disease autoimmunity to gain novel insights into the early pathogenesis of celiac disease. Interactions between season of birth, genetics, and early-life environmental factors on the risk of celiac autoimmunity were investigated in the multicenter TEDDY birth cohort study.

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Exclusive enteral nutrition initiates individual protective microbiome changes to induce remission in pediatric Crohn's disease.

Cell Host Microbe

November 2024

Chair of Nutrition and Immunology, TUM School of Life Sciences, Technische Universität München, 85354 Freising, Germany; TUMCREATE, 1 CREATE way, #10-02 CREATE Tower, Singapore 138602, Singapore; ZIEL Institute for Food & Health, Technische Universität München, 85354 Freising, Germany. Electronic address:

Article Synopsis
  • Exclusive enteral nutrition (EEN) is a crucial treatment for pediatric Crohn's disease, but the exact protective mechanisms of how it works are not fully understood.
  • A research study analyzed the fecal microbiota and metabolites in treatment-naive Crohn’s patients, identifying key protective features like Lachnospiraceae and medium-chain fatty acids.
  • The findings demonstrated that EEN leads to distinct changes in gut microbiome compositions that vary by individual, potentially influencing the development or prevention of inflammatory bowel disease through these microbiome dynamics.
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Editorial on Asymptomatic Rib Fractures and Their Relationship to Child Abuse.

Children (Basel)

September 2024

Department of Child and Adolescent Psychiatry, Saarland University Medical Center, 66421 Homburg, Germany.

Article Synopsis
  • A recent Special Issue has been released that highlights the serious impacts of child abuse and neglect on children's development and well-being.
  • The issue emphasizes these forms of abuse as significant adverse childhood experiences (ACEs) that can lead to long-term psychological and physical health challenges.
  • It aims to raise awareness and promote research on prevention and intervention strategies to support affected children and families.
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  • Social inequalities in child mental health are a significant public health issue, and this study aims to examine these inequalities over time across various countries.
  • Using longitudinal data from eight birth cohorts in twelve countries, the research tracks children's socio-economic circumstances and mental health outcomes from ages two to eighteen.
  • Results indicate that children from poorer socio-economic backgrounds generally show higher levels of internalising and externalising problems, although some cohorts exhibit minimal inequalities in certain age groups.
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