DEND syndrome

MeSH: C565253ORPHA: 79134

Overview

DEND syndrome is a very rare, generally severe form of neonatal diabetes mellitus (NDM, see this term) characterized by a triad of developmental delay, epilepsy, and neonatal diabetes

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with DEND syndrome, sourced from HPO and Orphanet clinical annotations.

HyperglycemiaElevated hemoglobin A1cSeizureMuscle weaknessAxial hypotoniaMild global developmental delayLong philtrumAnteverted naresBilateral ptosisDehydrationVomitingHypsarrhythmiaDownturned corners of mouthShort noseProminent metopic ridgePeripheral neuropathyThickened earsClinodactyly of the 4th finger

Classification & Codes

MeSH Code

C565253

Orphanet Code

ORPHA:79134
DEND syndrome
MeSHC565253
OrphanetORPHA:79134
Treatments0 drug(s)
Symptoms on record18 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO