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SHR Neuro Krebs Kardio Lipid

Reiterer, F; Grossauer, K; Morris, N; Uhrig, S; Resch, B.
Congenital pulmonary lymphangiectasis.
Paediatr Respir Rev. 2014; 15(3):275-280
Web of Science PubMed FullText FullText_MUG

 

Autor/innen der Med Uni Graz:
Grossauer Karin
Morris Nicholas
Reiterer Friedrich
Resch Bernhard
Uhrig Sabine
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Abstract:
Congenital pulmonary lymphangiectasis (CPL) is a rare vascular malformation causing dilated lymph vessels and disturbed drainage of lymph fluid. Based on the pathogenesis and clinical phenotype it can be classified as primary or secondary CPL. Associated genetic syndromes with or without lymphedema, familial occurrence and gene mutations have been described. In utero, it may present as non-immune hydrops with pleural effusions. At birth neonates may have respiratory failure due to chylothorax and pulmonary hypoplasia, causing very high short term mortality rates. Other cases may become symptomatic any time later in childhood or even during adult life. CPL is usually diagnosed based on the combination of clinical signs, imaging and histological findings. Open-lung biopsy is considered the gold standard for the diagnosis of CPL. Treatment is primarily supportive featuring aggressive mechanical ventilation and the management of problems associated with congenital chylothorax including chest-drainage, medium-chain triglycerides (MCT) diet, and octreotide. Copyright © 2014 Elsevier Ltd. All rights reserved.

Find related publications in this database (Keywords)
Congenital pulmonary lymphangiectasis
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Classification
Diagnosis
Therapeutic options
Genetic counselling
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