AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2018 Issue 4; New/Revised ICD-10-CM Codes

Multiple Sulfatase Deficiency

Code E75.26, Sulfatase deficiency, was created for multiple sulfatase deficiency (MSD), a rare inherited metabolic disorder that is caused by the deficiency of the formylglycine-generating enzyme (FGE) that is responsible for activating various sulfatase enzymes. Deficiency in the FGE results in defective functioning of sulfatase enzymes. The impairment causes signs and symptoms in the brain, skeleton and skin. While there is a wide spectrum in the effects of MSD, the age of onset distinguishes the three types of MSD. The most severe form, neonatal MSD, develops soon after birth. There may be loss of motor skills, epilepsy, spasticity...

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