raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:137577

Neonatal hypoxic and ischemic brain injury

Synonyms: HIE · Hypoxic and ischemic brain injury in the newborn · Hypoxic-ischemic encephalopathy · Perinatal hypoxia

Type
Particular clinical situation in a disease or syndrome
Group
Disorder
Inheritance
Not applicable
Age of onset
Infancy, Neonatal
Status
active

A rare neonatal encephalopathy characterized by alterations in mental status ranging from irritability and decreased responsiveness to coma, as well as abnormal primitive reflexes, hypotonia, seizures, and abnormalities in feeding and respiration, with an onset within the first hours of life. The condition is associated with high mortality. Long-term sequelae include a spectrum of signs and symptoms including behavioral deficits, developmental delay, learning disabilities, cognitive impairment, seizures, visual and auditory dysfunction, and cerebral palsy.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enNeonatal hypoxic and ischemic brain injuryHIE · Hypoxic and ischemic brain injury in the newborn · Hypoxic-ischemic encephalopathy · Perinatal hypoxia
frLésion cérébrale ischémique et hypoxique néonataleEncéphalopathie hypoxique-ischémique · Hypoxie périnatale · Lésion cérébrale ischémique et hypoxique du nouveau-né
deHypoxische und ischämische Hirnverletzung des NeugeborenenHIE · Hypoxisch-ischämische Enzephalopathie · Hypoxische und ischämische Hirnschäden bei Neugeborenen · Perinatale Hypoxie
esLesión hipóxico-isquémica cerebral neonatalEHI · Encefalopatía hipóxico-isquémica · Hipoxia perinatal
itDanno cerebrale neonatale ipossico e ischemicoDanno cerebrale ipossico e ischemico nel neonato · Encefalopatia ipossico-ischemica · HIE · Ipossia perinatale
nlNeonataal hypoxisch en ischemisch hersenletselHIE · Hypoxisch en ischemisch hersenletsel bij neonaat · Hypoxisch-ischemische encefalopathie · Perinatale hypoxie
ptLesão cerebral hipóxico-isquémica neonatalEncefalopatia hipóxico-isquémica · HIE · Hipóxia perinatal · Lesão cerebral hipóxico-isquémica do recém-nascido
plUszkodzenie mózgu noworodka z powodu niedotlenienia i niedokrwieniaEncefalopatia niedotleniowo-niedokrwienna · HIE · Hipoksja okołoporodowa · Niedotlenienie okołoporodowe · Niedotlenieniowe i niedokrwienne uszkodzenie mózgu noworodków · Noworodkowe uszkodzenie mózgu wskutek niedotlenienia i niedokrwienia
csHypoxicko-ischemická encefalopatieHIE · Novorozenecká hypoxie a ischemické poškození mozku · Hypoxicko-ischemické postižení mozku u novorozence
ukНеонатальна гіпоксична та ішемічна травма головного мозкуHIE · Гіпоксичне та ішемічне ураження головного мозку у новонароджених · Гіпоксично-ішемічна енцефалопатія · Перинатальна гіпоксія
zh新生儿缺氧缺血性脑损伤新生儿缺血缺氧性脑病
trYenidoğan hipoksik ve iskemik beyin hasarıHIE · Perinatal hipoksi · Perinatal asfiksi · Hipoksik iskemik ensefalopati · Yenidoğanda hipoksik ve iskemik beyin hasarı

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10P91.6Especificvalidated
ICD-11KB04Especificvalidated
UMLSC5575064Evalidated
GARD19875Evalidated

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):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalence1-9 / 100 000—EuropeClass onlyValidatedEuropean Medicines Agency 2015[OTHER]

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