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Learn more about Strensiq® (asfotase alfa) and HPP.

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Strensiq® has been evaluated in patients 1 day to 58 years of age with perinatal/infantile- and juvenile-onset hypophosphatasia (HPP).1,a

Most patients were treated with Strensiq® for more than 2 years (range: 1 day up to 6 years for some patients).1

Study 1
(ENB-002-08/ENB-003-08)1

Study Design

  • Prospective, single-arm trial and extension (N=11 patients with severe perinatal/infantile-onset hypophosphatasia)
  • Patients who completed trial ENB-002-08 were enrolled in the extension (ENB-003-08)
  • Inclusion Criteria: Onset of hypophosphatasia symptoms <6 months of age,7 with biochemical and radiographic evidence and a medical history of hypophosphatasia as well as the presence of any of the following: rachitic chest deformity, vitamin B6-dependent seizures, or failure to thrive

Patient Characteristics

  • Age: 3 weeks to 3.3 years at baseline1

Efficacy Endpoints

  • Survival and Ventilation-Free Survival compared with historical controls1
  • Change in hypophosphatasia-related rickets (as measured by the RGI-C scale)1
  • Growth (as measured by height and weight z scores)1
Study 2
(ENB-010-10)1

Study Design

    • Prospective, open-label trial (N=59 patients with perinatal/infantile-onset hypophosphatasia)
    • Inclusion criteria: Onset of hypophosphatasia symptoms <6 months of age, with biochemical and radiologic evidence and a medical history of hypophosphatasia8

Patient Characteristics

  • Age: 1 day to 6.5 years at baseline1

Efficacy Endpoints

  • Survival and Ventilation-Free Survival compared with historical controls1
  • Change in hypophosphatasia-related rickets (as measured by the RGI-C scale)1
  • Growth (as measured by height and weight z scores)1
Study 3
(ENB-006-09/ENB-008-10)1

Study Design

  • Prospective, open-label trial and extension (N=13; 5 patients with perinatal/infantile-onset hypophosphatasia and 8 patients with juvenile-onset hypophosphatasia)
  • All patients with juvenile-onset hypophosphatasia entered the extension study (ENB-008-10) and were treated for ≥48 months

Patient Characteristics

Age: 6 to 12 years of age at baseline1

  • 38% (5/13) of patients had onset of symptoms <6 months of age1
  • 62% (8/13) of patients had onset of symptoms ≥6 months of age1

Efficacy Endpoints

Change in:

  • Hypophosphatasia-related rickets (as measured by the RGI-C scale) compared with historical controls
  • Growth (as measured by height and weight z scores) compared with historical controls
  • Mobility (as measured by the 6-minute walk test [6MWT]b)
  • Gait (as measured by the Modified Tinetti Performance-Oriented Mobility-Gait [MPOMA-G] scale)
  • Running speed and agility (BOT-2)8
  • Bone mineralization8
Study 4
(ENB-009-10)8

Study Design

  • Randomized, open-label, dose-ranging trial and concurrent control study and extension (N=19; including 10 adult patients with perinatal/infantile- or juvenile-onset hypophosphatasia)

Patient Characteristics

10 adult patients with perinatal/infantile- and juvenile-onset hypophosphatasia were 26 to 58 years at baseline

  • 10% (1/10) of patients had onset of symptoms <6 months of age
  • 90% (9/10) of patients had onset of symptoms ≥6 months and ≤18 years of age

Efficacy Endpoints

Change in:

  • Alkaline phosphatase substrate levels (inorganic pyrophosphate [PPi] and pyridoxal-5’-phosphate [PLP])
  • Mobility (as measured by 6MWTc)
  • Bone mineralization

Indication

STRENSIQ® is indicated for the treatment of patients with perinatal/infantile- and juvenile-onset hypophosphatasia (HPP)1

Important Safety Information

  • Hypersensitivity reactions, including anaphylaxis, have been reported in STRENSIQ-treated patients. Signs and symptoms consistent with anaphylaxis included difficulty breathing, choking sensation, nausea, periorbital edema, and dizziness. These reactions have occurred within minutes after subcutaneous administration of STRENSIQ and can occur in patients on treatment for more than one year. Other hypersensitivity reactions have also been reported in STRENSIQ-treated patients, including vomiting, fever, headache, flushing, irritability, chills, skin erythema, rash, pruritus and oral hypoesthesia. If a severe hypersensitivity reaction occurs, discontinue STRENSIQ treatment and initiate appropriate medical treatment. Consider the risks and benefits of re-administering STRENSIQ to individual patients following a severe reaction. If the decision is made to re-administer the product, monitor patients for a reoccurrence of signs and symptoms of a severe hypersensitivity reaction.
  • Localized lipodystrophy, including lipoatrophy and lipohypertrophy, has been reported at injection sites after several months in patients treated with STRENSIQ. Advise patients to follow proper injection technique and to rotate injection sites.
  • Patients with HPP are at increased risk for developing ectopic calcifications. In clinical trials, 14 cases (14%) of ectopic calcification of the eye including the cornea and conjunctiva, and the kidneys (nephrocalcinosis) were reported. There was insufficient information to determine whether or not the reported events were consistent with the disease or due to STRENSIQ. No visual changes or changes in renal function were reported. Ophthalmology examinations and renal ultrasounds are recommended at baseline and periodically during treatment with STRENSIQ to monitor for signs and symptoms of ophthalmic and renal ectopic calcifications and for changes in vision or renal function.
  • The most common adverse reactions (≥ 10%) are injection site reactions, lipodystrophy, ectopic calcifications and hypersensitivity reactions.