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ICD Hub / Endocrine, nutritional or metabolic diseases (E00–E89) / 5A00.04 - Congenital hypothyroidism due to iodine deficiency
ICD-11

ICD-11 Codes
for 5A00.04 - Congenital hypothyroidism due to iodine deficiency
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Understanding Congenital Hypothyroidism Due to Iodine Deficiency

This condition, identified by the ICD-11 code 5A00.04, refers to a state of thyroid hormone deficiency present from birth, specifically caused by insufficient iodine intake. Congenital hypothyroidism due to iodine deficiency impacts a newborn's physical and mental development. Iodine is essential for the synthesis of thyroid hormones, which are critical regulators of growth and neurological development. When iodine is deficient, the thyroid gland cannot produce adequate hormones, leading to a range of developmental issues. Iodine deficiency disorders (IDD) are recognised as a primary cause of preventable brain damage globally.

Causes and Risk Factors for Iodine-Deficiency Hypothyroidism

The primary cause of congenital hypothyroidism coded as 5A00.04 is a lack of iodine in the diet. This deficiency can affect populations, particularly in regions where soil iodine content is low, leading to iodine deficiency disorders (IDD). Maternal iodine status during pregnancy is a critical risk factor, as the developing fetus relies heavily on iodine for thyroid hormone production. Suboptimal iodine availability, especially during the first trimester, can significantly impact fetal brain development. Therefore, adequate iodine intake for pregnant individuals is paramount to prevent this condition in their offspring.

Diagnosing Congenital Hypothyroidism Linked to Iodine Deficiency

The diagnosis of congenital hypothyroidism due to iodine deficiency (ICD-11 code 5A00.04) typically begins with newborn screening (NBS) programs, which are standard in many countries. These screenings assess thyroid hormone levels in infants shortly after birth. If NBS results indicate potential hypothyroidism, a confirmatory venous blood test is performed to measure free T4 and TSH levels. Further investigations may include assessing urine iodine concentration to determine iodine status and evaluating maternal iodine levels during pregnancy. Early detection through these diagnostic pathways is crucial for timely intervention.

Management Strategies for Congenital Hypothyroidism from Iodine Deficiency

Prompt treatment is essential for congenital hypothyroidism due to iodine deficiency (5A00.04) to mitigate potential developmental impacts. Management typically involves lifelong thyroid hormone replacement therapy with levothyroxine (L-T4). Early initiation of L-T4, ideally within the first two weeks of life, is critical for optimising neurodevelopmental outcomes. For pregnant individuals in iodine-deficient areas, iodine supplementation is often recommended to ensure adequate intake for both mother and fetus. Public health initiatives like Universal Salt Iodization (USI) play a significant role in preventing iodine deficiency disorders on a population level.

Alternative Terms for Congenital Hypothyroidism Due to Iodine Deficiency

Congenital hypothyroidism due to iodine deficiency, identified by ICD-11 code 5A00.04, is also known by several other terms. These include congenital iodine deficiency syndrome, often abbreviated as CIDS, and fetal iodine deficiency syndrome. Historically, related conditions were sometimes referred to as endemic cretinism or neurological cretin, particularly when severe developmental impairments were present. Other descriptive terms include congenital iodine-deficiency hypothyroidism NOS and myxoedematous type congenital iodine-deficiency syndrome.

Coding and Documentation for ICD-11 Code 5A00.04

For accurate medical billing and record-keeping, the specific ICD-11 code for congenital hypothyroidism due to iodine deficiency is 5A00.04. This code precisely identifies the condition, distinguishing it from other forms of congenital hypothyroidism. Healthcare providers should ensure that documentation clearly supports the diagnosis, noting the link to iodine deficiency. Using the official condition name, "Congenital hypothyroidism due to iodine deficiency," is vital for precise coding. This ICD-11 code for congenital hypothyroidism due to iodine deficiency is reportable and billable when the condition is diagnosed.

Frequently asked questions

What is the ICD-11 classification meaning of 5A00.04 Congenital hypothyroidism due to iodine deficiency?
5A00.04 represents congenital hypothyroidism, a condition present at birth where the thyroid gland produces insufficient thyroid hormone, specifically due to iodine deficiency.
How does ICD-11 distinguish 5A00.04 Congenital hypothyroidism due to iodine deficiency from other congenital hypothyroidism codes?
ICD-11 uses the specific code 5A00.04 for congenital hypothyroidism caused by iodine deficiency, differentiating it from other related conditions such as transient congenital hypothyroidism (5A00.03).
Can additional coding details be applied to 5A00.04 Congenital hypothyroidism due to iodine deficiency in ICD-11?
Yes, ICD-11 allows for post-coordination with 5A00.04 by adding stem and/or extension codes to form a cluster code, providing more specific detail about the condition.
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