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DOI 10.1055/s-00000034

Klinische Pädiatrie

In dieser Ausgabe:

Kinderpneumologische Aspekte bei der Betreuung von Kindern mit neuromuskulären Erkrankungen am Beispiel der Muskeldystrophie Typ Duchenne

Die Muskeldystrophie Typ Duchenne ist die häufigste erbliche Muskelerkrankung im Kindesalter. Neben der progredienten Einschränkung der Motorik und der kardialen Beteiligung stehen die aus der Muskelschwäche resultierende restriktive Lungenerkrankung und die Hustenschwäche im Vordergrund. Die spezifische kinderpneumologische Therapie hat das Überleben und die Lebensqualität der betroffenen Jungen maßgeblich verbessert. Zu den Aufgaben des Kinder-Pneumologen gehören das Monitoring der Lungenfunkt...

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Pre-chewing of infant food – expectations towards an old, traditional feeding habit for potential allergy prevention

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 ...

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Management of RSV Bronchiolitis in Outpatient Setting

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 base...

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Severe Manifestation of Granulomatosis with Polyangiitis in an Adolescent with Respiratory, Hemorrhagic and Vascular Occlusive Complications

For two weeks, the 17-year-old female adolescent suffered from productive and hemorrhagic coughing, sneezing, and fever up to 39.7°C (103.5°F) which did not respond adequately to antipyretic medication. Over the next few days, shortness of breath and multiple episodes of epistaxis occurred. A perforation of the nasal septum was suspected and treated with antibiotics. Previous history revealed no serious infectious diseases, asthma or suspected autoimmune disorders, but recurrent epistaxis for ab...

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Severe Neonatal Episodic Laryngospasm (SNEL) due to Mutation in the SCN4A Gene as a Rare Differential Diagnosis in Paroxysmal Inspiratory Stridor with Cyanosis in Infancy

Episodic stridor with cyanosis during infancy can occur due to various underlying pathologies. Laryngomalacia is the most common cause, mostly resolving spontaneously within the first year of life. However, cardiac, respiratory, neurological, and gastrointestinal diseases as well as malformations have also to be considered as differentials for cyanosis. Laryngospasm is a rare cause of stridor and is usually related to intubation anaesthesia. Moreover, it can be difficult to differentiate laryngo...

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CFTR Modulator Treatment in Children<12 Years of Age – Status Quo and Challenges

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 qualit...

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