maturity-onset diabetes of the young type 9

MeSH: C567393ORPHA: 552

Overview

maturity-onset diabetes of the young that has material basis in heterozygous mutation in the PAX4 gene on chromosome 7q32.1

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 maturity-onset diabetes of the young type 9, sourced from HPO and Orphanet clinical annotations.

Glucose intoleranceHyperglycemiaGlycosuriaAbnormal oral glucose toleranceAbnormal circulating C-peptide concentrationAbnormal circulating insulin concentrationHypoinsulinemiaElevated hemoglobin A1cNephropathyRetinopathyHyperinsulinemic hypoglycemiaInsulin-resistant diabetes mellitusIntrauterine growth retardationLarge for gestational ageNeonatal hypoglycemiaTransient neonatal diabetes mellitusOverweightAbnormality of the kidneyRenal cystAbnormality of the genitourinary systemObesityExocrine pancreatic insufficiencyPancreatic hypoplasiaHepatocellular adenoma

Classification & Codes

MeSH Code

C567393

Orphanet Code

ORPHA:552
maturity-onset diabetes of the young type 9
MeSHC567393
OrphanetORPHA:552
Treatments0 drug(s)
Symptoms on record24 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO