5A00.01

ICD-11 MMS 2026-01

Permanent congenital hypothyroidism without goitre

Description

This is a permanent congenital state in which the thyroid gland does not make enough thyroid hormone. This diagnosis is without swelling of the thyroid gland.

Index terms

  • Permanent congenital hypothyroidism without goitre
  • Congenital atrophy of thyroid
  • aplasia of thyroid
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  • congenital hypothyroidism without goitre
  • congenital subthyroidism
  • congenital thyroid aplasia
  • congenital thyroid atrophy
  • congenital thyroid gland insufficiency
  • congenital thyroid insufficiency
  • thyroid atrophy with cretinism
  • Primary congenital hypothyroidism due to a developmental anomaly
  • Hypothyroidism due to agenesis or dysgenesis of the thyroid gland
  • Thyroid dysgenesis
  • Thyroid ectopia
  • Thyroid agenesis
  • congenital absence of thyroid
  • congenital absence of thyroid gland
  • congenital athyreosis
  • Thyroid hemiagenesis
  • Thyroid hypoplasia
  • hypoplastic thyroid gland
  • Primary congenital hypothyroidism due to TSH receptor mutations
  • Resistance to TSH binding or signalling
  • Congenital thyroid atrophy with myxoedema
  • aplasia of thyroid with myxoedema

Exclusions from above levels

  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5A00.00 – Permanent congenital hypothyroidism with diffuse goitre
  • 5A00.02 – Pendred syndrome
  • 5A00.03 – Transient congenital hypothyroidism
  • 5A00.04 – Congenital hypothyroidism due to iodine deficiency
  • 5A00.0Y – Other specified congenital hypothyroidism
  • 5A00.0Z – Congenital hypothyroidism, unspecified

Foundation entity

Permanent congenital hypothyroidism without goitre – http://id.who.int/icd/entity/1756727158

Part of: 5A00.0: Congenital hypothyroidism › 5A00: Hypothyroidism › 05: Endocrine, nutritional or metabolic diseases