ORPHA:217557
Pulmonary interstitial glycogenosis
Synonyms: Infantile cellular interstitial pneumonitis · PIG
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Infancy, Neonatal
- Status
- active
Pulmonary interstitial glycogenosis (PIG) is a rare non-lethal pediatric form of interstitial lung disease (ILD).
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pulmonary interstitial glycogenosis | Infantile cellular interstitial pneumonitis · PIG |
| fr | Glycogénose interstitielle pulmonaire | Pneumonite interstitielle cellulaire infantile |
| de | Glykogenose, interstitielle pulmonale | PIG · Pneumonitis , infantile interstitielle zelluläre |
| es | Glucogenosis pulmonar intersticial | Neumonitis intersticial celular del lactante |
| it | Glicogenosi interstiziale polmonare | PIG · Polmonite interstiziale cellulare infantile |
| nl | Pulmonale interstitiële glycogenose | Infantiele cellulaire interstitiële pneumonitis · PIG |
| pt | Glicogenose intersticial pulmonar | |
| pl | Płucna glikogenoza śródmiąższowa | Dziecięce śródmiąższowe komórkowe zapalenie płuc · PIG |
| cs | Plicní intersticiální glykogenóza | PIG · Infantilní buněčná intersticiální pneumonitida |
| uk | Легеневий інтерстиціальний глікогеноз | Інфантильний клітинний інтерстиціальний пневмоніт · PIG |
| zh | 肺间质糖原病 | 婴儿细胞间质性肺炎 |
| tr | Pulmoner interstisyel glikojenoz | PIG · İnfantil hücresel interstisyel pnömoni |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | P22.8 | NTBT | attributed | validated |
| ICD-11 | CB04.Y | NTBT | index | validated |
| MONDO | 0016321 | E | validated | |
| UMLS | C3161106 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:264694Interstitial lung disease specific to infancy
rare respiratory diseases; rare transplant-related disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/217557
- This record as JSON: /data/d/217557.json