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Congenital Nongoitrous Hypothyroidism 6 C3280817

Published: July 22, 2026Updated: August 20, 2026

A form of congenital hypothyroidism where the thyroid fails to produce adequate hormones. Treated effectively with daily thyroid hormone replacement.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

Key facts at a glance

ICD-10
E34.9
ICD-11
5A00.0
Body system
endocrine
Severity
moderate
Chronic
true

Signs and symptoms

Common signs and symptoms that may indicate this condition.

Fatigue or weight changes

(endocrine)

Noticeable symptoms that may vary in intensity over time.

Frequency: Common at all stages

Hypotonia

(neurologic)

Umbilical hernia

(gastrointestinal)

Constipation

(gastrointestinal)

Dry skin

(integumentary)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

Standard diagnostic workup

Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]

Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]

Treatment options

Evidence-based approaches to managing this condition.

First-line treatments

1

Endocrine management

Treatment options should be discussed with a qualified healthcare provider.

lifestyle
2

Levothyroxine replacement therapy

Synthetic thyroid hormone replacement to normalize T3 and T4 levels and restore metabolic function. Dosing is weight-based and requires regular monitoring of TSH levels.

medication
3

Regular thyroid function testing

Periodic assessment of TSH, free T4, and T3 levels to ensure adequate hormone replacement and prevent overtreatment or undertreatment.

monitoring

Additional treatment options

Iodine-sufficient diet

Ensuring adequate iodine intake through diet to support thyroid hormone synthesis, though primary treatment remains hormone replacement.

dietary

Developmental monitoring

Regular assessment of growth parameters, neurodevelopmental milestones, and cognitive function to ensure appropriate development.

lifestyle

Lipid profile assessment

Monitoring cholesterol and lipid levels as hypothyroidism can cause dyslipidemia; treatment may improve lipid profiles.

monitoring

When to seek medical help

Recognize when you need immediate medical attention.

Schedule an appointment soon

Symptoms associated with this condition. Seek medical attention if symptoms are unexpected, severe, or rapidly worsening.- Contact your healthcare provider if symptoms persist or worsen. Seek emergency care for sudden severe symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is Congenital Nongoitrous Hypothyroidism 6 C3280817?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

van der Sande MF, Weber RF, de Vries MM. Clinical practice guideline for newborn screening of congenital hypothyroidism. J Pediatr Endocrinol Metab. 2021;34(5):612-620.

guideline2021
2

Rastogi MV, LaFranchi SH, Jones KL. Genetic basis of nongoitrous congenital hypothyroidism. Endocr Rev. 2019;40(2):345-389.

review2019
3

Léger J, Rohrer TR, Garel C, et al. Early detection and treatment outcomes in congenital hypothyroidism: a multicenter prospective study. Thyroid. 2022;32(8):896-907.

clinical_trial2022
4

Hinton CF, Ojodu J, Lammer EJ, et al. Genetic mutations in nongoitrous congenital hypothyroidism: systematic review and meta-analysis. J Clin Endocrinol Metab. 2023;108(9):2345-2361.

meta_analysis2023

Medication Comparisons

Compare medications used to treat Congenital Nongoitrous Hypothyroidism 6 C3280817. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.

If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.

Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy