title: Severe respiratory distress syndrome unresponsive to intensive care treatment--diagnostic and therapeutic considerations.
abstract: Respiratory Distress Syndrome (RDS) is a common complication in preterm neonates. If RDS is not responding to conventional treatment modalities (surfactant therapy, ventilatory support, etc.), an underlying pathology (pulmonary lymphangiectasia, capillary alveolar dysplasia, alpha-1 antitrypsin deficiency, etc.) other then prematurity should be taken into consideration.Here, we report on a preterm neonate with the unusual simultaneous occurrence of pulmonary and systemic lymphangiectasia and homozygous alpha-1 antitrypsin deficiency who developed severe RDS that was refractory to conventional treatment. The diagnostic and therapeutic approach in this patient is presented.
authors.affiliation: Department of Pediatric Cardiology, University Hospital of Saarland, Kirrbergerstrasse, Homburg, Germany.
authors.forename: H-G
authors.initials: HG
authors.lastname: Limbach
authors.forename: A
authors.initials: A
authors.lastname: Hasenfus
authors.forename: R M
authors.initials: RM
authors.lastname: Bohle
authors.forename: A
authors.initials: A
authors.lastname: Baghai
authors.forename: G
authors.initials: G
authors.lastname: Löffler
authors.forename: L
authors.initials: L
authors.lastname: Gortner
authors.forename: P
authors.initials: P
authors.lastname: Fries
authors.forename: S
authors.initials: S
authors.lastname: Meyer
mesh_terms: D015148:Cardiotocography; D015897:Comorbidity; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005865:Gestational Age; D006720:Homozygote; D006801:Humans; D052577:Infant, Extremely Low Birth Weight; D007231:Infant, Newborn; D015931:Intensive Care, Neonatal; D007668:Kidney / Q000473:pathology; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D008200:Lymphangiectasis / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D042601:Lymphatic Vessels / Q000473:pathology; D008297:Male; D009206:Myocardium / Q000473:pathology; D011247:Pregnancy; D012127:Respiratory Distress Syndrome, Newborn / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D012131:Respiratory Insufficiency / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D017211:Treatment Failure; D019896:alpha 1-Antitrypsin Deficiency / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*