Niemann-Pick disease

Not billableICD-10-CM diagnosis code.

This code exists but cannot be submitted on its own. It groups the more specific codes beneath it.

A category header for its whole life, FY2016 onwards. It has never been valid for submission on its own.

Submit one of these 7 more specific codes instead:

E75.240 Niemann-Pick disease type A
E75.241 Niemann-Pick disease type B
E75.242 Niemann-Pick disease type C
E75.243 Niemann-Pick disease type D
E75.244 Niemann-Pick disease type A/B
E75.248 Other Niemann-Pick disease
E75.249 Niemann-Pick disease, unspecified

What has happened to this code

Every change the publisher made, from 2012 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.

FY2012 Added to the draft code set draft Niemann-Pick disease

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 E75 Disorders of sphingolipid metabolism and other lipid storage disorders

Excludes1 - never coded here

mucolipidosis, types I-III (E77.0-E77.1)
Refsum's disease (G60.1)

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.

State > confusional > acute
State > confusional > subacute
Disorder > major neurocognitive
Disorder > neurocognitive > major
Syndrome > brain > acute or subacute
Delirium, delirious > with > dementia
Reaction > organic, acute or subacute
Disorder > neurocognitive > major > in
Disorder > neurocognitive > major > in > with > violent behavior
Disorder > neurocognitive > major > in > with > combative behavior
Disorder > neurocognitive > major > in > with > aggressive behavior
Dementia > in > cerebral lipidoses
Dementia > in > cerebral lipidoses > with behavioral disturbance

Codes in the same group

Code	Description	Status
E75.21	Fabry (-Anderson) disease	Valid
E75.22	Gaucher disease	Valid
E75.23	Krabbe disease	Valid
E75.25	Metachromatic leukodystrophy	Valid
E75.26	Sulfatase deficiency	Valid
E75.27	Pelizaeus-Merzbacher disease	Valid
E75.28	Canavan disease	Valid
E75.29	Other sphingolipidosis	Valid