Grand roundPersistent tachypnea and hypoxia in a 3-month-old term infant
Section snippets
Case report
A 3-month-old white male infant was transferred for evaluation of persistent tachypnea and hypoxemia. He was the product of a full-term uncomplicated pregnancy, born through spontaneous vaginal delivery without complications and with Apgar scores of 8 at 1 minute and 5 minutes. On the first day of life, he developed hypoxemia, necessitating intubation and was given a single dose of beractant (Survanta), 4 mg/kg, for respiratory distress syndrome. Chest x-ray revealed diffuse bilateral haziness.
Discussion
ILD encompasses a broad, heterogeneous group of rare disorders, with childhood ILD having emerged as distinct from adult ILD in outcome and etiology.1 A recent survey in the United Kingdom and Ireland revealed a prevalence of 3.6 cases per million in children from birth to age 16.2 ILD includes any entity that results in derangement of the alveolar capillary interface, often characterized clinically by tachypnea, crackles, hypoxia, and/or diffuse infiltrates. The onset is insidious in the first
Diagnostic discussion
Recently, a new classification of ILD for children under age 2 years was proposed. Entities specific to young children include diffuse developmental abnormalities, surfactant dysfunction disorders, lung growth–related abnormalities, and reactive or possibly reactive disorders (including NEHI and PIG).22 Reviews of previously unclassified and new cases are continually redefining these categories,23 with these 4 categories accounting for more than 50% of lung biopsy specimens from multiple
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Cited by (13)
57 - Lung Diseases Associated With Disruption of Pulmonary Surfactant Homeostasis
2019, Kendig's Disorders of the Respiratory Tract in ChildrenLung Diseases Associated with Disruption of Pulmonary Surfactant Homeostasis
2012, Kendig and Chernick's Disorders of the Respiratory Tract in ChildrenContemporary Perspectives on Pediatric Diffuse Lung Disease
2011, Radiologic Clinics of North AmericaCitation Excerpt :Some patients treated with corticosteroids or hydroxychloroquine show improvement and a few even become asymptomatic, but it is unclear whether this is attributable to the therapy or the natural history of the disease.5,57,61 Chest radiographs in young infants with disease-causing ABCA3 or SP-C mutations show diffuse or patchy hazy granular pulmonary opacities, while CT scans reveal diffuse ground-glass or consolidating opacity, septal thickening, and crazy paving (Fig. 7).59,62–65 In older infants and children, findings evolve to include ground-glass opacities that decrease in extent with age, and thin-walled parenchymal cysts that increase in number and size with age.
Genetics and Physiology of Surfactant Protein Deficiencies
2011, Fetal and Neonatal Physiology E-Book, Fourth EditionFatal Familial Lung Disease Caused by ABCA3 Deficiency without Identified ABCA3 Mutations
2010, Journal of Pediatrics
Supported by National Institutes of Health grants HL56387 (S.E.W., L.M.N., J.A.W.) and HL 54703 (L.M.N.).