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Phase 2 N=11 Treatment

Safety and Efficacy Study of Asfotase Alfa in Severely Affected Infants With Hypophosphatasia (HPP)

Hypophosphatasia (HPP)

Enrolled (actual)
11
Serious AEs
63.6%
Results posted
Sep 2011
Primary outcome: Primary: Change in Rickets Severity From Baseline to Week 24, Based on Assessment of Skeletal Radiographs Using Radiologic Global Impression of Change (RGI-C) — 2.00 Units on a scale — p=0.0039

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
asfotase alfa (Biological)
Age
Pediatric
Sex
All
Sponsor
Alexion Pharmaceuticals, Inc.
Primary completion
May 2010

Outcome Measures

OutcomeResultp-value
PRIMARY
Change in Rickets Severity From Baseline to Week 24, Based on Assessment of Skeletal Radiographs Using Radiologic Global Impression of Change (RGI-C)
2.00 0.0039 sig
SECONDARY
Maximum Serum Concentration of Asfotase Alfa (Cmax)
2230; 376; 897
SECONDARY
Time at Maximum Serum Concentration of Asfotase Alfa (Tmax)
4.3; 29.7; 12.0
SECONDARY
Area Under Serum Concentration-time Curve to Last Measurable Concentration of Asfotase Alfa (AUCt)
79800; 14700; 33700

Summary

This clinical trial studies the safety and efficacy of asfotase alfa in infants and young children with infantile onset HPP.

Eligibility Criteria

Inclusion Criteria

  • Legal guardian(s) must provide informed consent prior to any study procedures
  • Documented diagnosis of severe HPP as indicated by:
  • Total serum alkaline phosphatase at least 3 standard deviations (SD) below the mean for age
  • Plasma pyridoxal 5'-phosphate (PLP) at least 4 times the upper limit of normal
  • Radiographic evidence of HPP (hypophosphatasia), characterized by:
  • Flared and frayed metaphyses
  • Severe, generalized osteopenia
  • Widened growth plates
  • One or more HPP-related findings:
  • History or presence of:
  • Non-traumatic post-natal fracture
  • Delayed fracture healing
  • History of elevated serum calcium
  • Functional craniosynostosis with decreased head circumference growth
  • Nephrocalcinosis
  • Respiratory compromise
  • Rachitic chest deformity and/or vitamin B6 dependent seizures
  • Failure to thrive
  • Onset of symptoms prior to 6 months of age
  • Age ≤ 36 months
  • Otherwise medically stable (patient may be on ventilatory support)
  • Legal guardian(s) must be willing to comply with the study

Exclusion Criteria

  • History of sensitivity to any of the constituents of the study drug
  • Current or prior clinically significant cardiovascular, endocrinologic, hematologic, hepatic, immunologic, metabolic, infectious, urologic, pulmonary, neurologic, dermatologic, renal condition and/or other major disease which, in the opinion of the investigator, precludes study participation
  • Treatment with an investigational drug within 1 month prior to the start of study drug administration
  • Current enrollment in any other study involving an investigational new drug, device or treatment for HPP (e.g., bone marrow transplantation)
  • Low serum calcium, phosphate or 25(OH) vitamin D
  • Current evidence of a treatable form of rickets
  • Prior treatment with bisphosphonate
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00744042). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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