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.
© Georg Thieme Verlag KG Stuttgart · New York.
MeSH terms
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Cardiotocography
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Comorbidity
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Disease Progression
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Fatal Outcome
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Female
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Gestational Age
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Homozygote
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Humans
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Infant, Extremely Low Birth Weight
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Infant, Newborn
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Intensive Care, Neonatal*
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Kidney / pathology
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Lung / pathology
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Lung Diseases / congenital*
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Lung Diseases / diagnosis
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Lung Diseases / pathology
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Lung Diseases / therapy
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Lymphangiectasis / congenital*
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Lymphangiectasis / diagnosis
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Lymphangiectasis / pathology
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Lymphangiectasis / therapy
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Lymphatic Vessels / pathology
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Male
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Myocardium / pathology
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Pregnancy
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Respiratory Distress Syndrome, Newborn / diagnosis*
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Respiratory Distress Syndrome, Newborn / etiology
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Respiratory Distress Syndrome, Newborn / pathology
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Respiratory Distress Syndrome, Newborn / therapy*
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Respiratory Insufficiency / diagnosis
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Respiratory Insufficiency / etiology
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Respiratory Insufficiency / therapy
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Treatment Failure
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alpha 1-Antitrypsin Deficiency / diagnosis*
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alpha 1-Antitrypsin Deficiency / pathology
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alpha 1-Antitrypsin Deficiency / therapy*
Supplementary concepts
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Lymphangiectasia, pulmonary, congenital