raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:91359

Chronic pneumonitis of infancy

Synonyms: CPI

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Infancy, Neonatal
Status
active

Chronic pneumonitis of infancy is a rare pediatric form of interstitial lung disease (ILD).

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enChronic pneumonitis of infancyCPI
frPneumopathie chronique de l'enfance= en
dePneumonie, chronische, des Kindesalters= en
esNeumonitis crónica del lactante= en
itPolmonite cronica dell'infanzia= en
nlChronische pneumonitis op zuigelingenleeftijd= en
ptPneumonite crónica da infância
plPrzewlekłe dziecięce zapalenie płuc= en
csChronická pneumonitida kojenců= en
ukХронічний пневмоніт немовлят= en
zh婴儿期慢性肺炎= en
trSüt çocuğunda kronik pnömoni= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10J84.0NTBTattributedvalidated
ICD-11CB04.6Especificvalidated
MONDO0019621Evalidated
UMLSC3872848Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

13 annotations · validated 2021-10-13
HPOTerm (as annotated by Orphanet)Diagnostic
Frequent (79-30%)
HP:0012735Cough
HP:0002207Diffuse reticular or finely nodular infiltrations
HP:0001508Failure to thrive
HP:0025179Ground-glass opacification on pulmonary HRCT
HP:0002883Hyperventilation
HP:0012418Hypoxemia
HP:0032341Reduced forced vital capacity
Occasional (29-5%)
HP:0000961Cyanosis
HP:0001945Fever
HP:0030864Intercostal retractions
HP:0002098Respiratory distress
HP:0002789Tachypnea
Very rare (<4-1%)
HP:0100721Mediastinal lymphadenopathy

Annotation source: 30505512[PMID]_25755194[PMID]

7

Sources for this card