11. NEHI
⢠Another
typical
example of
right middle
lobe ,and left
lingular GGO.
12. Surfactant Metabolism Dysfunction
⢠Surfactant is a complex mixture of phospholipids
and 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
13. Nonspecific interstitial
pneumonitis
Bilateral scattered middle zonal GGO
Bi basilar consolidations.
Bronchial dilatation.
HRCT shows a mosaic
perfusion pattern and multiple bilateral linear densities
23. Langerhans cell histiocytosis
Bizarre
thick- and thin-walled cysts; shaped
few micronodules also seen
pulmonary cystic lesions, some located
subpleurally, and
bilateral pneumothoraces
24. Lympngiomatosis
Consider vascular/lymphatic cause
Prominent diffuse smooth septal thickening, bronchovascular
bundles and ground-glass attenuation
25. Lesson learned
Most HRCT features are non-specific,
but when related to the clinical findings,
they can suggest the proper diagnosis and
obviate biopsy.
26. A new classification system for pediatric
interstitial lung disease evolved out of the recognition
that clinical setting is an important consideration
in the diagnosis of pediatric ILD and that
combined clinical, imaging, and pathological correlation
is a more powerful diagnostic tool, than
any 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.