raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:224

Neonatal diabetes mellitus

Synonyms: Congenital diabetes mellitus · Diabetes of infancy · Monogenic diabetes of infancy · NDM

Type
Clinical group
Group
Group of disorders
Inheritance
Autosomal dominant, Autosomal recessive, Not applicable
Age of onset
Infancy, Neonatal
Status
active

A group of rare diabetes mellitus characterized by neonatal diabetes mellitus presents with symptoms including hyperglycemia, failure to thrive and, in some cases, dehydration and ketoacidosis (which can be severe and lead coma), in a child within the first months of life. It encompasses several disorders in which diabetus mellitus may be transient or permanent. It may present as an isolated condition or as part of a syndrome.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enNeonatal diabetes mellitusCongenital diabetes mellitus · Diabetes of infancy · Monogenic diabetes of infancy · NDM
frDiabète néonatal
deDiabetes mellitus, neonatalerDiabetes im Säuglings- und Kleinkindalter · Diabetes mellitus, kongenitaler · Monogener Diabetes des Kindesalters · NDM
esDiabetes mellitus neonatalDiabetes de la infancia · Diabetes mellitus congénita · Diabetes monogénica de la infancia · NDM
itDiabete mellito neonataleDiabete infantile · Diabete mellito congenito · Diabete monogenico infantile · NDM
nlNeonatale diabetes mellitusCongenitale diabetes mellitus · Diabetes op zuigelingenleeftijd · Monogenische diabetes op zuigelingenleeftijd · NDM
ptDiabetes mellitus neonatalDiabetes da infância · Diabetes mellitus congénito · Diabetes monogénico da infância · NDM
plCukrzyca noworodkówNDM
csNovorozenecký diabetes mellitusNDM
ukНеонатальний цукровий діабетВроджений цукровий діабет · Дитячий діабет · Моногенний діабет немовлят · NDM
zh新生儿糖尿病NDM
trYenidoğan diabetes mellitusuNDM

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10P70.2Especificvalidated
ICD-11KB60.2Especificvalidated
MONDO0016391Evalidated
MONDO16391Evalidated
UMLSC0158981Evalidated
MedDRA10028933Evalidated

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 (2):

Children (5):

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

5 records
TypeClassValueAreaQualificationValidationSource
Prevalence at birth1-9 / 100 0001.1 / 100 000EuropeValue and classNot yet validated21953423[PMID]_19499210[PMID]_20546293[PMID]
Prevalence at birth1-9 / 100 0001.1 / 100 000ItalyValue and classValidated21953423[PMID]
Prevalence at birth1-9 / 100 0001.12 / 100 000GermanyValue and classValidated20546293[PMID]
Annual incidence1-9 / 1 000 0000.62 / 100 000AustriaValue and classValidated19496964[PMID]
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

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card