Thyroid dyshormonogenesis

ICD-10: E07.1MeSH: C564766ORPHA: 95716

Overview

Familial thyroid dyshormonogenesis is a type of primary congenital hypothyroidism (see this term), a permanent thyroid hormone deficiency that is present from birth, which results from inborn errors of thyroid hormone synthesis

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 Thyroid dyshormonogenesis, sourced from HPO and Orphanet clinical annotations.

Neurodevelopmental delayMacroglossiaFacial edemaIntellectual disabilityHypotoniaLethargyHyporeflexiaBradycardiaHypothermiaNeonatal hyperbilirubinemiaDepressed nasal bridgePositive perchlorate discharge testAbnormal circulating thyroglobulin concentrationReduced radioactive iodine uptakeIncreased radioactive iodine uptakeSensorineural hearing impairmentElevated circulating thyroid-stimulating hormone concentrationDecreased circulating T4 concentrationDelayed cranial suture closureCongenital hypothyroidismGoiterUmbilical herniaConstipationLarge posterior fontanelleAbnormal epiphysis morphologyProlonged neonatal jaundiceThyroid defect in oxidation and organification of iodideDelayed proximal femoral epiphyseal ossificationFeeding difficulties in infancy

Classification & Codes

ICD-10 Code

E07.1

MeSH Code

C564766

Orphanet Code

ORPHA:95716
Thyroid dyshormonogenesis
ICD-10E07.1
MeSHC564766
OrphanetORPHA:95716
Treatments0 drug(s)
Symptoms on record29 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
Thyroid dyshormonogenesis | OrphanDrug