E75.240

Applicable To

• Acid sphingomyelinase deficiency type A ASMD type A)

• Infantile neurovisceral acid sphingomyelinase deficiency

Approximate Synonyms

• Niemann pick disease type a

• Niemann-pick disease, type a

Clinical Information

• The classic infantile form of niemann-pick disease, caused by mutation in sphingomyelin phosphodiesterase. It is characterized by accumulation of sphingomyelins in the cells of the mononuclear phagocyte system and other cell throughout the body leading to cell death. Clinical signs include jaundice, hepatosplenomegaly, and severe brain damage.

Details

• E75.240 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM E75.240 became effective on October 1, 2022.

• This is the American ICD-10-CM version of E75.240 – other international versions of ICD-10 E75.240 may differ.