J84.841

Billable

Neuroendocrine cell hyperplasia of infancy

HCC Category Mapping

V24HCC 112 — Fibrosis of Lung and Other Chronic Lung Disorders

0.268

ESRDHCC 112 — Fibrosis of Lung and Other Chronic Lung Disorders

0.000

RxHCCHCC 227 — Sarcoidosis, Pulmonary Alveolar Disorders

0.000

What This Code Means

An abnormal increase in hormone-producing cells in the lungs of infants, which can cause breathing problems and other complications.

Coding Tips

•Verify this is documented in an infant or young child, as this is a pediatric condition
•Document any associated respiratory distress or need for supplemental oxygen

Clinical Significance

Neuroendocrine cell hyperplasia of infancy is a rare pediatric interstitial lung disease presenting in the first two years of life with tachypnea, retractions, and hypoxemia. While it generally has a favorable prognosis with gradual improvement, affected infants often require supplemental oxygen and close monitoring. Accurate diagnosis prevents unnecessary invasive procedures and inappropriate aggressive treatments.

Documentation Requirements

✓Documentation of onset in infancy (typically first year of life)
✓Chest computed tomography showing geographic ground-glass opacities and air trapping
✓Lung biopsy showing neuroendocrine cell hyperplasia with bombesin-positive cells if performed
✓Clinical presentation including tachypnea, retractions, crackles, and hypoxemia
✓Exclusion of other causes of infant respiratory distress (infection, congenital heart disease, cystic fibrosis)

Commonly Confused Codes

J84.842 — Pulmonary interstitial glycogenosis is a different pediatric ILD with glycogen accumulation J84.843 — Alveolar capillary dysplasia with vein misalignment is a fatal neonatal condition with very different prognosis J84.848 — Other interstitial lung diseases of childhood should not be used when NEHI is specifically diagnosed P22.0 — Respiratory distress syndrome of newborn is an acute neonatal condition, not a chronic ILD

Code Hierarchy

└J84Other interstitial pulmonary diseases └J84.8Other specified interstitial pulmonary diseases └J84.84Other interstitial lung diseases of childhood └J84.841Neuroendocrine cell hyperplasia of infancy

└J84.841Neuroendocrine cell hyperplasia of infancy