Klinische Padiatrie最新文献

Duchenne muscular dystrophy is the most common inherited neuromuscular disease in children. In addition to the progressive loss of motor skills and cardiac involvement, respiratory muscle weakness leads to a restrictive lung disease and cough insufficiency. Specific respiratory interventions have significantly improved survival and quality of life of the affected boys. The pediatric pulmonologist's tasks include monitoring of the lung function, polysomnography, starting and adjusting of non-invasive or invasive nocturnal ventilation and optimizing of secretion management. Providing of vaccinations, enhancing of nutrition, treatment of intercurrent infections, and provision of advice and training are important tasks for the pediatric pulmonologist. This article emphasizes on the tasks for the pediatric pulmonologist in the treatment of children with neuromuscular diseases i. e. Duchenne muscular dystrophy in interaction with the other specialist disciplines.

Artificial intelligence (AI) is already being used in most medical disciplines, including paediatric pneumology. This review describes current developments in AI-supported technologies and discusses their potential for the diagnosis and treatment of lung diseases in children and adolescents. The spectrum ranges from models for analysing respiratory sounds and the automated evaluation of medical imaging to systems for supporting clinical decisions. In particular, challenges in the adaptation of AI for paediatric populations are also described. Finally, open questions, such as the implementation of AI-based software in everyday clinical practice, will be discussed.

Premastication is a traditional feeding method whereby solids are pre-chewed by the mother or sometimes by other relatives and then given to the infant along with the oral microbiota. The aim of this study was to assess if premastication is known or performed among the German population as well as to evaluate the expectations regarding this feeding method, particularly in the context of allergy prevention. Two surveys about premastication were conducted. For the survey of the "general population (GP)" 10,000 participants (18 to 65 years old) were randomly selected from the official population register of Berlin. The survey of the "selected population (SP)" was conducted by the patient organization German Allergy and Asthma Association (DAAB). 526 people from the GP and 220 participants from the SP completed the questionnaire. 15.3% (n=114) of all respondents had previously heard of premastication, 1.5% (n=7/455) of the people with children reported that they have performed premastication for their child. 42.8% (n=225) of the GP as well as 36.4% (n=80) of the SP could imagine performing premastication for their future child or grandchild. 59.3% (n=312) of the GP stated that they at least partially think premastication may help to prevent the development of allergies.Premastication appears to be barely known and practiced among the German population. However, a considerable proportion of participants would consider giving pre-chewed food to their infant. A high rate of respondents, especially among the GP, believed that premastication may have a positive impact on the prevention of allergies.

Cystic fibrosis (CF) is a genetic disease that results from mutations in the CFTR gene. It primarily affects the lungs and digestive system. Recent advancements in the treatment of CF have been driven by highly effective therapies that modulate the function of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which target the underlying molecular defects in CFTR function. These modulators have been demonstrated to significantly improve lung function, weight gain, and quality of life for 90% of individuals with CF, particularly those with the F508del mutation. HEMT has also demonstrated potential benefits for pancreatic and liver function, and its early use in young children may delay or prevent disease progression. However, challenges remain in optimizing biomarkers and outcome measures for younger children, addressing side effects, and developing novel therapies for mutations not responsive to current treatments. This review focuses on the efficacy, safety, and future perspectives of HEMT in children under 12 years of age, emphasizing the importance of early intervention to improve long-term outcomes in CF patients.Mukoviszidose (CF) ist die häufigste genetisch bedingte Stoffwechselerkrankung durch Mutationen im CFTR-Gen. Sie betrifft insbesondere die Lunge und das Verdauungssystem. Die jüngsten Fortschritte in der Behandlung der CF sind das Resultat von hochwirksamen Therapien (HEMT), welche die Funktion des CFTR-Proteins (Cystic Fibrosis Transmembrane Conductance Regulator) modulieren und damit eine kausale Therapie ermöglichen. Diese führt nachweislich zu einer Verbesserung der Lungenfunktion und Gewichtszunahme, sowie einer Steigerung der Lebensqualität bei 90% der Menschen mit Mukoviszidose, insbesondere bei denen, die mindestens eine F508del-Mutation aufweisen. Zusätzlich besteht ein potenzieller Nutzen für die Funktion der Bauchspeicheldrüse und der Leber. Eine frühzeitige Anwendung von HEMT bei Kleinkindern kann das Fortschreiten der Krankheit voraussichtlich weiter verzögern oder gar verhindern. Herausforderungen bestehen in der Etablierung valider Biomarker - insbesondere für jüngere Kinder, unerwünschten Arzneimittelwirkungen und der Entwicklung neuartiger Therapien für Mutationen, die auf die HEMT nicht ansprechen. Die vorliegende Übersichtsarbeit beschreibt die Effektivität und Sicherheit bei Kindern unter 12 Jahren, sowie das zukünftige therapeutische Potential der HEMT. Ein besonderes Augenmerk wird dabei auf die Notwendigkeit eines frühzeitigen Therapiebeginns zur Verbesserung des langfristigen Verlaufs gelegt.

Seasonal respiratory syncytial virus (RSV) lower respiratory infections (RSV-LRI) and bronchiolitis in children remain a high burden to medical facilities. Studies evaluating the practical approach in outpatient settings are scarce. We conducted a survey to provide an insight into management in pediatric offices in Germany. Invitations for participation in an online survey were sent to 623 providers of pediatric primary care via email. Recipients were randomly chosen from national databanks based on zip codes. The response rate was 16,2%, n=92 (14,7%) questionnaires were fully answered and analyzed. 93,5% (n=86) are board certified pediatricians. In outpatient setting, every second patient with suspected or confirmed RSV-LRI is presented three times or more. 40,7% of our participants refer less than 10% of all patients to hospital, 83,7% refer less than one third (<30%). 33,7% of all participants never perform virological testing in suspected cases of RSV-LRI. More than 50% of all participants prescribe bronchodilators, hypertonic saline inhalation is prescribed by 17,4%. Antibiotics are prescribed in less than 10% of all cases of RSV-LRI. About 3/4 ask for a clearer, more conclusive guideline regarding prophylaxis. The diagnostic approach varies significantly. Although not recommended in international guidelines, bronchodilators are still frequently used. Considering emerging options for passive immunization, broader inclusion criteria into immunization regimes are requested by the participants. More conclusive guidelines and recommendations could further improve the management of outpatient RSV-LRI in children.