Are bisphosphonates approved for osteogenesis imperfecta?
Oral and intravenous (IV) bisphosphonates are currently the most promising pharmacologic therapy and are routinely used for OI, since clinical trials of these agents have consistently shown improvements in bone mineral density (BMD) in people with OI.
What are the four types of osteogenesis imperfecta?
The OI types are as follows:
- Type I. Mildest and most common type.
- Type II. Most severe type.
- Type III. Most severe type in babies who don’t die as newborns.
- Type IV. Symptoms are between mild and severe.
- Type V. Similar to type IV.
- Type VI. Very rare.
- Type VII. May be like type IV or type II.
- Type VIII.
What is the treatment or management for osteogenesis imperfecta?
To date, there is no known treatment, medicine, or surgery that will cure osteogenesis imperfecta (OI). The goal of treatment is to prevent deformities and fractures and allow the child to function as independently as possible. Treatments for preventing or correcting symptoms may include: Care of fractures.
What is the primary treatment for osteogenesis imperfecta?
Because osteogenesis imperfecta (OI) is a genetic condition, it has no cure. For many years, surgical correction of deformities, physiotherapy, and the use of orthotic support and devices to assist mobility (eg, wheelchairs) were the primary means of treatment.
Is Osteogenesis Imperfecta painful?
Conclusions: Pain is a common occurrence for children with OI and is both acute and chronic in nature, interfering with children’s daily living activities. OI pain may not be optimally treated because many children experienced moderate to severe pain despite use of analgesics and/or coping strategies.
What is oral bisphosphonate?
Oral bisphosphonates are commonly prescribed to prevent or treat osteoporosis in postmenopausal women. Common brand names of medications in this class include Fosamax, Actonel, Boniva, and Reclast.
What organs are affected by osteogenesis imperfecta?
In more severe forms of osteogenesis imperfecta, there may be bone deformities, poor lung development and lung problems, a barrel-shaped chest, poor muscle development in the arms and legs. Osteogenesis imperfecta is caused by a faulty gene that affects the body’s ability to produce collagen.
How long can a child live with osteogenesis imperfecta?
What is the life expectancy of someone with osteogenesis imperfecta (OI)? Life expectancy varies greatly depending on OI type. Babies with Type II often die soon after birth. Children with Type III may live longer, but often only until around age 10.
Does osteogenesis imperfecta get worse with age?
In type 3 OI, your child’s body produces enough collagen but it’s poor quality. Your child’s bones can even begin to break before birth. Bone deformities are common and may get worse as your child gets older.
What are the two types of osteogenesis?
Static and dynamic osteogenesis: two different types of bone formation.
What is the most severe form of osteogenesis imperfecta?
OI type II is the most severe type of osteogenesis imperfecta. Affected infants often experience life-threatening complications at birth or shortly after. Infants with OI type II have low birth weight, abnormally short arms and legs and blue sclera.
What is wrong with Byron the Baxter boy?
When you listen to 3-year-old Byron Baxter laugh and talk, you’d never know anything was wrong with him. But he was born with a rare condition called osteogenesis imperfecta, or brittle bone disease.
How are bisphosphonates used to treat Osteogenesis Imperfecta?
Background: Osteogenesis imperfecta is caused by a genetic defect resulting in an abnormal type I collagen bone matrix which typically results in multiple fractures with little or no trauma. Bisphosphonates are used in an attempt to increase bone mineral density and reduce these fractures in people with osteogenesis imperfecta.
Are there any randomized trials for osteogenesis imperfecta?
Date of the most recent search of the Cochrane Cystic Fibrosis and Genetic Disorders Group’s Inborn Errors of Metabolism Register: 28 April 2016. Randomised and quasi-randomised controlled trials comparing bisphosphonates to placebo, no treatment, or comparator interventions in all types of osteogenesis imperfecta.
What are the long-term benefits of bisphosphonate therapy?
Given their current widespread and expected continued use, the optimal method, duration of therapy and long-term safety of bisphosphonate therapy require further investigation. In addition, attention should be given to long-term fracture reduction and improvement in quality of life indicators.
How is osteogenesis imperfecta passed from parent to child?
Osteogenesis imperfecta is also known as brittle bone disease. It is a genetic condition which can be passed on from a parent to child or occur in the child without any other family history. An affected person is at risk for frequent breaks of the long bones or collapse of the bones of the spine.