Neuroendocrine cell hyperplasia of infancy (NEHI) Ground Glass opacity primarily affecting the middle and lingular lobes
NEHI• Another typical example of right middle lobe ,and left lingular GGO.
Surfactant Metabolism Dysfunction• Surfactant is a complex mixture of phospholipidsand proteins (SP-A, -B, -C and -D)& ABCA3.• ABCA3 an ATP-binding transporter Of lipids. Diffuse GG opacity with variable Intelobular septal thickening (chILD) due to ABCA3 gene mutations
Nonspecific interstitial pneumonitisBilateral scattered middle zonal GGOBi basilar consolidations.Bronchial dilatation. HRCT shows a mosaic perfusion pattern and multiple bilateral linear densities
Lesson learnedMost HRCT features are non-specific,but when related to the clinical findings,they can suggest the proper diagnosis andobviate biopsy.
A new classification system for pediatricinterstitial lung disease evolved out of the recognitionthat clinical setting is an important considerationin the diagnosis of pediatric ILD and thatcombined clinical, imaging, and pathological correlationis a more powerful diagnostic tool, thanany one single component. This new pediatric interstitial lung disease classification system was validated for infants and very young children in a retrospective review of 186 lung biopsies done between 1999 and 2004 with accompanying clinical histories and images from children under age 2 contributed by 11 pediatric institutions in North America. Based on this new classification system, ChILD is classified into three main groups: (1) disorders of infancy; (2) other categories (not specific to infancy); and (3)unclassifiable.