Barth syndrome

ValidICD-10-CM diagnosis code.

Valid for claims with a date of service in the current code year.

Valid FY2016 onwards.

What has happened to this code

Every change the publisher made, from 2007 to now. Entries marked draft are from the pre-implementation releases: ICD-10 took effect on 1 October 2015, so nothing before FY2016 was ever valid on a claim.

FY2007 Added to the draft code set draft Barth syndrome

Official coding rules

Published by CDC for FY2027. A rule attached to a category binds every code beneath it, so the rules inherited from this code's ancestors are shown with the code they come from.

Inherited from E78 Disorders of lipoprotein metabolism and other lipidemias

Excludes1 - never coded here

sphingolipidosis (E75.0-E75.3)

Inherited from the E70-E88 section Metabolic disorders (E70-E88)

Excludes1 - never coded here

androgen insensitivity syndrome (E34.5-)
congenital adrenal hyperplasia (E25.0)
hemolytic anemias attributable to enzyme disorders (D55.-)
Marfan syndrome (Q87.4-)
5-alpha-reductase deficiency (E29.1)

Excludes2 - not included here

Ehlers-Danlos syndromes (Q79.6-)

How CDC's index refers to this code

Wording from the official alphabetic index. Much of it appears nowhere in the code description, which is why a search for the everyday phrase so often finds nothing.

Barth syndrome
Syndrome > Barth
Disorder > bile acid and cholesterol metabolism > Barth syndrome
Disorder > cholesterol and bile acid metabolism > Barth syndrome

Codes in the same group

Code	Description	Status
E78.70	Disorder of bile acid and cholesterol metabolism, unspecified	Valid
E78.72	Smith-Lemli-Opitz syndrome	Valid
E78.79	Other disorders of bile acid and cholesterol metabolism	Valid