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Die Zeitschrift Klinische Pädiatrie erscheint 7-mal jährlich und veröffentlicht Beiträge aus dem Gebiet der (klinischen) Pädiatrie und deren Grenzgebiete in deutscher und englischer Sprache. Die Zeitschrift wird in allen wichtigen internationalen Zitationsdiensten geführt.  

DOI 10.1055/s-00000034

Klinische Pädiatrie

In dieser Ausgabe:

Notfallpläne zum Management von Notfällen bei Kindern mit außerklinischer Beatmung

Die chronisch respiratorische Insuffizienz wird auch in der Pädiatrie zunehmend ambulant mit außerklinischer Beatmung behandelt. Die oft komplex, erkrankten Kinder werden in der Häuslichkeit durch pflegerische und ärztliche Teams unterschiedlichster Zusammensetzung betreut. Strukturierte Behandlungsprozesse, speziell Notfallpläne zum Management des respiratorischen Notfalls von außerklinisch beatmeten Kindern fehlen. Dieser Beitrag ist ein Vorschlag zum Notfallmanagement bei Atemwegsinfekten, No...

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Ten-year experience of whole lung lavage in pediatric Pulmonary Alveolar Proteinosis

Background Pulmonary Alveolar Proteinosis (PAP) is extremely rare and can be caused by hereditary dysfunction of the granulocyte macrophage colony-stimulating factor receptor (GM-CSF) receptor, autoantibodies against GM-CSF, or other diseases leading to alveolar macrophage (AM) dysfunction. This leads to protein accumulation in the lung and severe dyspnea and hypoxemia. Whole lung lavage (WLL) is the first line treatment strategy.

Methods Here, we present data from more than ten years of WLL practi...

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Impact of Reanalysis of Nitrogen Multiple-Breath Washout on its Relationship with Chest Magnetic Resonance Imaging Findings in Clinically Stable and Pulmonary Exacerbated Children with Cystic Fibrosis

Rationale Multiple-breath washout (MBW)-derived lung clearance index (LCI) detects lung disease in children with cystic fibrosis (CF). Correction of a cross-talk error in the software of the MBW device Exhalyzer D in a new software version has generated significant interest regarding its impact on previous MBW findings. Since LCI and chest magnetic resonance imaging (MRI) correlated before in CF children, this study aims to reassess previous MBW data after correction.

Patients/Methods Reanalysis of...

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A Mild Case of Jeune Syndrome Associated with a Recurrent Missense Variant in DYNC2H1 : Confirmation of a Genotype-Phenotype Correlation

Asphyxiating thoracic dystrophy type Jeune is a rare, potentially fatal, autosomal recessive skeletal dysplasia characterized by a small and narrow thorax, lung hypoplasia, limb shortness, and facultative congenital abnormalities (e. g. polydactyly and ocular, hepatic, or renal complications) (de Vries et al., Eur J Pediatr 2010; 169: 77–88). Despite the identification of at least 17 associated genes since its first description (Jeune et al., Arch Fr Pediatr 1955; 12: 886–891), prenatal diagnosi...

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Pulmonary Alveolar Proteinosis and new therapeutic concepts

Pulmonary alveolar proteinosis (PAP) is an umbrella term used to refer to a pulmonary syndrome which is characterized by excessive accumulation of surfactant in the lungs of affected individuals. In general, PAP is a rare lung disease affecting children and adults, although its prevalence and incidence is variable among different countries. Even though PAP is a rare disease, it is a prime example on how modern medicine can lead to new therapeutic concepts, changing ways and techniques of (geneti...

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Relapsing Polychondritis with Tracheobronchial Involvement: A Detailed Description of Two Pediatric Cases and Review of the Literature

Relapsing polychondritis (RP) is a rare immune-mediated disease that primarily affects the cartilaginous structures of the ears, nose and airways. The clinical spectrum ranges from mild to severe disease characterized by progressive destruction of cartilage in the tracheobronchial tree leading to airway obstruction and acute respiratory failure. Early diagnosis is crucial to prevent irreversible airway damage and life-threatening complications. Due to its rarity and variability of symptoms, the ...

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