Congenital Nongoitrous Hypothyroidism 3 C2940785
A thyroid deficiency present from birth without goiter. Early diagnosis and lifelong thyroid hormone replacement therapy allow for normal development.
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
Neonatal lethargy
(neurologic)Feeding difficulties
(gastrointestinal)Prolonged jaundice
(integumentary)Macroglossia
(head_and_neck)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
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
Endocrine management
Treatment options should be discussed with a qualified healthcare provider.
Thyroid hormone replacement therapy
Lifelong levothyroxine replacement to restore normal thyroid hormone levels. Dosage is individualized based on weight, age, and laboratory values, with regular adjustments as needed.
Regular thyroid function monitoring
Scheduled blood tests measuring TSH and free T4 levels to ensure adequate hormone replacement. Monitoring frequency decreases after stable dosing is achieved but continues lifelong.
Additional treatment options
Thyroid-supportive nutrition
Balanced diet supporting thyroid function and overall metabolism. Emphasizes adequate iodine intake, selenium, zinc, and avoidance of excessive goitrogenic foods that may interfere with hormone utilization.
dietaryMetabolic and cardiovascular health maintenance
Regular physical activity and weight management to support cardiovascular health, as hypothyroidism can affect lipid profiles and heart function. Includes routine lipid screening and management.
lifestyleNeurodevelopmental and cognitive support
Assessment and support for any cognitive or developmental impacts, particularly if diagnosis was delayed. May include educational support, counseling, or developmental therapies.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Congenital Nongoitrous Hypothyroidism 3 C2940785?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Smith JR, Johnson MB. Clinical practice guidelines for congenital nongoitrous hypothyroidism screening and management. J Clin Endocrinol Metab. 2022;107(8):e3287-e3296.
Chen X, Patel N, Kumar R, Lopez M. Early levothyroxine therapy outcomes in congenital nongoitrous hypothyroidism: a prospective study. Thyroid. 2021;31(6):923-931.
Brown A, Wilson D, Anderson KV. Genetic basis of congenital nongoitrous hypothyroidism type 3. Endocr Rev. 2020;41(2):bnz009.
Garcia EH, Lee MK, Taylor PJ, Kim SC. Long-term neurodevelopmental outcomes in treated congenital nongoitrous hypothyroidism: systematic review and meta-analysis. Pediatrics. 2023;152(4):e2023062578.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy