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

Zusammenfassung

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 Lungenfunktion, die Polysomnographie, der Beginn und die Anpassung der nichtinvasiven oder invasiven nächtlichen Beatmung und die Optimierung des Sekretmanagements. Aber auch die Komplettierung der Impfungen, die Ernährungstherapie, die Behandlung interkurrierender Atemwegsinfektionen sowie die Beratung und Schulung sind wichtige Aufgaben des Kinder-Pneumologen. In diesem Beitrag werden die Aspekte der pneumologischen Behandlung von Kindern mit neuromuskulären Erkrankungen am Beispiel der Muskeldystrophie Typ Duchenne im Zusammenspiel mit den anderen Fachdisziplinen aufgezeigt.

Abstract

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.

Schlüsselwörter

Muskeldystrophie Duchenne - Heimbeatmung - außerklinische Beatmung - Tracheostoma - Sekretmanagement

Keywords

Duchenne muscular dystrophy - home mechanical ventilation - tracheostoma - neuromuscular - lung function testing

Publication History

Article published online:
10 December 2024

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