congenital nongoitrous hypothryoidism 5

ORPHA: 90673

Overview

congenital hypothyroidism that has material basis in heterozygous mutation in the NKX2-5 gene on chromosome 5q35

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 congenital nongoitrous hypothryoidism 5, sourced from HPO and Orphanet clinical annotations.

Impaired sensitivity to thyroid stimulating hormoneCongenital hypothyroidismElevated circulating thyroid-stimulating hormone concentrationProlonged neonatal jaundiceIncreased circulating thyroglobulin concentrationReduced radioactive iodine uptakeDecreased circulating T4 concentrationMacroglossiaLethargyHyporeflexiaUmbilical herniaBradycardiaConstipationNeonatal hyperbilirubinemiaLarge posterior fontanelleAbnormal epiphysis morphologyThyroid hypoplasiaCompensated hypothyroidismDelayed proximal femoral epiphyseal ossificationFeeding difficulties in infancyAbnormal cryIncreased radioactive iodine uptakeEdemaHypotoniaHypothermia

Classification & Codes

Orphanet Code

ORPHA:90673
congenital nongoitrous hypothryoidism 5
OrphanetORPHA:90673
Treatments0 drug(s)
Symptoms on record25 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO