raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:90051

Sepsis in premature infants

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

A rare systemic condition affecting neonates born at less than 37 weeks gestational age and characterized by life-threatening organ dysfunction caused by a dysregulated host response to an infection, which may have been acquired shortly before or during birth (resulting in early-onset neonatal sepsis during the first 72 hours of life), or after birth (leading to late-onset neonatal sepsis between 72 hours and three months). Prematurity constitutes one of the primary risk factors for neonatal sepsis. The clinical picture may develop gradually with signs and symptoms like irritability, lethargy, or poor feeding, or progress rapidly to respiratory distress, fever, hypothermia, hypotension, shock, and multiple organ failure.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enSepsis in premature infants
frSepsis chez le nouveau-né prématuré
deSepsis bei Frühgeborenen
esSepsis en neonatos prematuros
itSepsi nei neonati prematuri
nlSepsis bij premature zuigeling
ptSépsis de apresentação tardia em lactentes prematuros
plSepsa u wcześniaków
csSepse u nedonošených
ukСепсис у недоношених дітей
zh早产儿败血症
trPrematüre bebeklerde sepsis

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10P36.0NTBTattributedvalidated
ICD-10P36.1NTBTattributedvalidated
ICD-10P36.2NTBTattributedvalidated
ICD-10P36.3NTBTattributedvalidated
ICD-10P36.4NTBTattributedvalidated
ICD-10P36.5NTBTattributedvalidated
ICD-10P36.8NTBTattributedvalidated
ICD-10P36.9NTBTattributedvalidated
ICD-11KA60NTBTattributedvalidated
UMLSC5681350Evalidated

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 prevalence1-5 / 10 00032 / 100 000EuropeValue and classValidatedEuropean Medicines Agency[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.

6

HPO phenotypes

51 annotations · validated 2020-02-14
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0010978Abnormality of immune system physiology
HP:0040187Neonatal sepsis
HP:0001622Premature birth
HP:0001518Small for gestational age
HP:0001649Tachycardia
Frequent (79-30%)
HP:0003270Abdominal distention
HP:0031602Abnormal mucociliary clearance
HP:0011410Caesarian section
HP:0004325Decreased body weight
HP:0001875Decreased total neutrophil count
HP:0002094Dyspnea
HP:0011227Elevated circulating C-reactive protein concentration
HP:0001945Fever
HP:0012719Functional abnormality of the gastrointestinal tract
HP:0002579Gastrointestinal dysmotility
HP:0002045Hypothermia
HP:0030783Increased circulating interleukin 6 concentration
HP:0001974Increased total leukocyte count
HP:0001942Metabolic acidosis
HP:0001319Neonatal hypotonia
HP:0000980Pallor
HP:0032169Severe infection
Occasional (29-5%)
HP:0001892Abnormal bleeding
HP:0002795Abnormal respiratory system physiology
HP:0000236Abnormality of the anterior fontanelle
HP:0001903Anemia
HP:0001662Bradycardia
HP:0000961Cyanosis
HP:0001410Decreased liver function
HP:0002014Diarrhea
HP:0000969Edema
HP:0004387Enterocolitis
HP:0002240Hepatomegaly
HP:0001265Hyporeflexia
HP:0002615Hypotension
HP:0000952Jaundice
HP:0001287Meningitis
HP:0030863Nasal flaring
HP:0100520Oliguria
HP:0000967Petechiae
HP:0002686Prenatal maternal abnormality
HP:0000979Purpura
HP:0004713Reversible renal failure
HP:0001250Seizure
HP:0001744Splenomegaly
HP:0005968Temperature instability
HP:0001873Thrombocytopenia
HP:0002013Vomiting
Very rare (<4-1%)
HP:0011880Acute disseminated intravascular coagulation
HP:0031696Disseminated viral infection
HP:0020101Invasive fungal infection

Run the differential on the 5 obligate / very frequent terms

Annotation source: 28709162[PMID]_27589549[PMID]_30207217[PMID]_28434651[PMID]_29382648[PMID]

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Sources for this card