Consensus clinical management guidelines for acid sphingomyelinase deficiency (Niemann–Pick disease types A, B and A/B)

Table 3 Classification of patients with ASMD

	Acute neurovisceral, ASMD type-A	Chronic neurovisceral, ASMD type-A/B	Chronic visceral; ASMD type-B
Hepatosplenomegaly	+	+	+
Proatherogenic lipid profile	+	+	+
Delayed growth and puberty	N/A	+	+
Thrombocytopenia	+	+	+
Interstitial lung disease	+	+	+
Skeletal involvement	+	+	+
Liver disease	+	+	+
Cherry red macula	+	Some patients	Some patients
Hypotonia	+	Some patient	Absent
Neurodegeneration	Rapidly progressive	Slowly progressive	Absent

ISSN: 1750-1172