Strensiq

Strensiq Uses, Dosage, Side Effects, Food Interaction and all others data.

Strensiq is a first-in-class bone-targeted enzyme replacement therapy designed to address the underlying cause of hypophosphatasia (HPP)—deficient alkaline phosphatase (ALP). Hypophosphatasia is almost always fatal when severe skeletal disease is obvious at birth. By replacing deficient ALP, treatment with Asfotase Alfa aims to improve the elevated enzyme substrate levels and improve the body's ability to mineralize bone, thereby preventing serious skeletal and systemic patient morbidity and premature death. Strensiq was first approved by Pharmaceuticals and Medicals Devices Agency of Japan (PMDA) on July 3, 2015, then approved by the European Medicine Agency (EMA) on August 28, 2015, and was approved by the U.S. Food and Drug Administration (FDA) on October 23, 2015. Asfotase Alfa is marketed under the brand name Strensiq® by Alexion Pharmaceuticals, Inc. The annual average price of Asfotase Alfa treatment is $285,000.

Perinatal/infantile- and juvenile-onset HPP patients treated with Strensiq had reductions in plasma TNSALP (tissue non-specific alkaline phosphatase) substrates, PPi and pyridoxal 5'-phosphate (PLP) within 6 to 12 weeks of treatment. Reductions in plasma PPi and PLP levels did not correlate with clinical outcomes. Bone biopsy data from perinatal/infantile-onset and juvenile-onset HPP patients treated with Strensiq demonstrated decreases in osteoid volume and thickness indicating improved bone mineralization.

Strensiq
Uses
Dosage
Side Effect
Precautions
Interactions
Uses during Pregnancy
Uses during Breastfeeding
Accute Overdose
Food Interaction
Half Life
Volume of Distribution
Clearance
Interaction With other Medicine
Contradiction
Storage

Trade Name	Strensiq
Generic	Asfotase alfa
Asfotase alfa Other Names	Asfotase alfa
Weight	18mg/0.45ml, 28mg/0.7ml, 40mg/ml, 80mg/0.8ml,
Type	Injection, Subcutaneous Solution
Formula	C₇₁₀₈H₁₁₀₀₈N₁₉₆₈O₂₂₀₆S₅₆
Weight	180000.0 Da (Approximate)
Groups	Approved, Investigational
Therapeutic Class
Manufacturer	Alexion Pharma UK Ltd
Available Country	Australia, United Kingdom, United States,
Last Updated:	September 19, 2023 at 7:00 am

Uses

Strensiq is an enzyme replacement therapy used for the treatment of perinatal/infantile and juvenile onset hypophosphatasia (HPP).

Indicated for the treatment of patients with perinatal/infantile and juvenile onset hypophosphatasia (HPP).

Strensiq is also used to associated treatment for these conditions: Infantile-onset Hypophosphatasia, Juvenile-onset Hypophosphatasia, Perinatal-onset Hypophosphatasia

How Strensiq works

HPP is caused by a deficiency in TNSALP (tissue non-specific alkaline phosphatase) enzyme activity, which leads to elevations in several TNSALP substrates, including inorganic pyrophosphate (PPi). Elevated extracellular levels of PPi block hydroxyapatite crystal growth which inhibits bone mineralization and causes an accumulation of unmineralized bone matrix which manifests as rickets and bone deformation in infants and children and as osteomalacia (softening of bones) once growth plates close, along with muscle weakness. Replacement of the TNSALP enzyme upon Strensiq treatment reduces the enzyme substrate levels.

Toxicity

There are no available human data on Asfotase Alfa use in pregnant women to inform a drug associated risk. In animal reproduction studies, Asfotase Alfa administered intravenously to pregnant rats and rabbits during the period of organogenesis showed no evidence of fetotoxicity, embryolethality or teratogenicity at doses causing plasma exposures up to 21 and 24 times, respectively, the exposure at the recommended human dose.