Pediatric pneumological aspects in the care of children with neuromuscular diseases with focus on Duchenne muscular dystrophy

被引：0

作者：

Fuchs, Hans ^{[1
]}

Gunst, Lennart ^{[1
]}

Wendt, Anke ^{[2
]}

Becker, Sebastian ^{[3
]}

Grychtol, Ruth Margarethe ^{[4
]}

Vlajnic, Dejan ^{[5
]}

Aschmann-Muehlhans, Dorit ^{[6
]}

Wuerfel, Christiane ^{[7
,8
]}

Steindor, Mathis ^{[9
]}

Stehling, Florian ^{[9
]}

机构：

[1] Albert Ludwigs Univ Freiburg, Med Fak, Zentrum Kinder & Jugendmed, Univ Klinikum Freiburg,Sekt Neonatol & Pad Pad Int, Freiburg, Germany

[2] Charite Univ Med Berlin, Klin Padiat Schwerpunkt Pneumol Immunol & Intens M, Padiatr Intens Med, Berlin, Germany

[3] Darmstadter Kinderkliniken Prinzessin Margaret, Kinderpneumol, Darmstadt, Germany

[4] Hannover Med Sch, Klin Padiat Pneumol Allergol & Neonatol, Padiat Pneumol, Hannover, Germany

[5] Univ Witten Herdecke, Kinderpalliat Zentrum, Vest Kinder & Jugendklin Datteln, Datteln, Germany

[6] Kinderkrankenhaus St Marien, Padiat Pneumol, Landshut, Germany

[7] Tech Univ Dresden, Med Fak, Klin & Poliklin Kinder & Jugendmed, Padiat Pneumol, Dresden, Germany

[8] Tech Univ Dresden, Univ Klinikum Carl Gustav Carus, Dresden, Germany

[9] Univ Duisburg Essen, Univ Klinikum Essen, Klin Kinderheilkunde 3, Padiat Pneumol, Essen, Germany

来源：

KLINISCHE PADIATRIE | 2025年 / 237卷 / 02期

关键词：

Duchenne muscular dystrophy; home mechanical ventilation; tracheostoma; neuromuscular; lung function testing; NONINVASIVE VENTILATION; DECLINE;

D O I：

10.1055/a-2464-6210

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：59 / 65

页数：7

共 28 条

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