Elevated SM accumulation is defined as 4 to 6 times normal SM accumulation with normal enzyme activity. Niemaoh-Pick disease (NPD), also known as sphingomyelin lipidosis, is a congenital disorder of glycolipid metabolism. It is characterized by a large number of foamy cells containing neurosphingomyelin in all mononuclear macrophages and in the nervous system. It is less common than Gaucher’s disease. It is autosomal recessive and is more prevalent in Jews, with a prevalence of up to 1/25,000. there are at least five types. So how is elevated sm accumulation examined from clinical manifestations? Here is a brief introduction. Acute neurological type (type A or infantile): Initially, loss of appetite, vomiting, feeding difficulties, extreme wasting, dry waxy yellow skin, progressive mental and motor decompensation, hypotonia flaccid paralysis, eventually idiocy, half with cherryred spot of the fundus (cherryred spot), blindness, jaundice with hepatosplenomegaly. They are anemic and cachectic, and most die before the age of 4 due to infection. The skin often shows a small yellow verrucous rash with deafness. Neurospherin accumulation is 20-60 times higher than normal, and enzyme activity is 5-10% of normal, with a minimum of <1%. Non-neurological type (beta or visceral type): infantile or childhood onset of open door, slow progression of disease, prominent hepatosplenomegaly. Normal intelligence, no neurological symptoms. SM accumulation is 3-20 times higher than normal, enzyme activity is 5-20% of normal, low as in type A.