Congenital iodine-deficiency syndrome (E00)
Clinical Information
Congenital Hypothyroidism - A condition in infancy or early childhood due to an in-utero deficiency of THYROID HORMONES that can be caused by genetic or environmental factors, such as thyroid dysgenesis or HYPOTHYROIDISM in infants of mothers treated with THIOURACIL during pregnancy. Endemic cretinism is the result of iodine deficiency. Clinical symptoms include severe MENTAL RETARDATION, impaired skeletal development, short stature, and MYXEDEMA.
Myxedema - A condition characterized by a dry, waxy type of swelling (EDEMA) with abnormal deposits of MUCOPOLYSACCHARIDES in the SKIN and other tissues. It is caused by a deficiency of THYROID HORMONES. The skin becomes puffy around the eyes and on the cheeks. The face is dull and expressionless with thickened nose and lips.
Scleromyxedema - A connective tissue disorder characterized by widespread thickening of SKIN with a cobblestone-like appearance. It is caused by proliferation of FIBROBLASTS and deposition of MUCIN in the DERMIS in the absence of thyroid disease. Most scleromyxedema cases are associated with a MONOCLONAL GAMMOPATHY, immunoglobulin IgG-lambda.
Instructional Notations
Use Additional Code
The “use additional code” indicates that a secondary code could be used to further specify the patient’s condition. This note is not mandatory and is only used if enough information is available to assign an additional code.
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- subclinical iodine-deficiency hypothyroidism E02
Endocrine, nutritional and metabolic diseases (E00–E89)
Disorders of thyroid gland (E00-E07)
E00 Congenital iodine-deficiency syndrome
- E00.0 Congenital iodine-deficiency syndrome, neurological type
- E00.1 Congenital iodine-deficiency syndrome, myxedematous type
- E00.2 Congenital iodine-deficiency syndrome, mixed type
- E00.9 Congenital iodine-deficiency syndrome, unspecified
Congenital iodine-deficiency syndrome (E00)