Tolerability and Safety Study of Recombinant Human Acid Sphingomyelinase in Acid Sphingomyelinase Deficiency Patients

Inclusion Criteria: * Patients with documented non-neuronopathic acid sphingomyelinase deficiency * The patient has a diffusing capacity of carbon monoxide (DLco) \>20% and ≤80% of the predicted normal value. * The patient has a spleen volume ≥6 multiples of normal(MN). A partial splenectomy will be permitted if performed ≥1 year prior to Screening/Baseline and residual spleen volume is ≥6 MN. * The patient who is receiving lipid lowering therapy should be on a stable dose and regimen of lipid-lowering therapy(ies) for at least 12 weeks prior to Screening/Baseline, with the patient expected to remain on the same dose and regimen throughout the 26-week treatment period. * The patient who is female and of childbearing potential must have a negative serum pregnancy test for β-HCG. Exclusion Criteria: * The patient is female and pregnant or lactating. * The patient has a Body Mass Index(BMI)\>30. * The patient has received an investigational drug within 30 days prior to study enrollment * The patient has a medical condition or any extenuating circumstance that may significantly interfere with study compliance, including all prescribed evaluations and follow-up activities. * The patient has had a major organ transplant * ALT or AST \>250 IU/L or total bilirubin \>1.5 mg/dL. * The patient is unwilling or unable to abstain from the use of alcohol for 1 day prior to and 3 days after each rhASM infusion for the duration of the study. * The patient requires medications that may decr…