What Body Systems Are Affected By Osteogenesis Imperfecta?

How does osteogenesis imperfecta affect the respiratory system?

People with moderate and severe OI are more vulnerable to viral and bacterial respiratory infection of upper and lower airways, as well as lung problems, including asthma and pneumonia.

Patients with OI have a higher risk of death from respiratory diseases..

What organs are affected by brittle bone disease?

Risk Factors for Osteogenesis imperfecta (Brittle bone disease) OI is actually a connective tissue disorder, resulting from the defective synthesis of a protein called collagen, which is a structual component of bones, tendons, eyes, organs and skin. OI is genetically inherited.

What chromosome does osteogenesis imperfecta affect?

Osteogenesis imperfecta type XIX is inherited in an X-linked recessive pattern. A condition is considered X-linked if the mutated gene that causes the disorder is located on the X chromosome, one of the two sex chromosomes in each cell.

What are the signs of brittle bone disease?

Signs of OI include:bones that break with no known cause or from very minor trauma.bone pain.bone deformity (such as scoliosis or bowlegs)a shorter stature.brittle teeth (called dentinogenesis imperfecta)a blue, purple, or gray tint to the sclera (the whites of the eyes)triangular face shape.More items…

What is the disease where your bones break easily?

Also known as “brittle bone disease,” osteogenesis imperfecta (OI) is a genetic disorder that causes weak bones that break easily in addition to other symptoms. There are several forms of OI, and although there is no cure, the symptoms of OI can be managed with a healthy lifestyle, medication, or surgery.

How is OI diagnosed?

The diagnosis of OI is made on the basis of family history and/or clinical presentation. Frequent fractures, short stature, a blue hue to the white part of the eye (blue sclera), teeth problems (dentinogenesis imperfecta) and hearing loss that progresses after puberty may be present.

Does osteogenesis imperfecta get worse with age?

It causes bones to break easily. 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 part of the body does osteogenesis imperfecta affect?

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 do people live with brittle bone?

The prognosis for infants with the most severe form of osteogenesis imperfecta is poor, and most children may not live beyond a few weeks. The prognosis for those with milder forms of the condition who receive good medical management is much better, and many people may have average lifespans.

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 an OI baby?

Your child has been diagnosed with osteogenesis imperfecta (OI). This is a rare condition that causes bones to be very thin and delicate so they break (fracture) easily. OI is sometimes called brittle bone disease. There are four types of OI that range from mild to severe.

Is Osteogenesis Imperfecta a type of dwarfism?

Osteogenesis imperfecta (OI) Type III is a dwarfism condition, with adults generally reaching a height of three feet, although profound short stature is often associated with the other three types as well.

Is Oi a disability?

Most people with OI experience physical disability. OI also can cause weak muscles, brittle teeth, a curved spine, and hearing loss. Most forms of OI are caused by abnormal genes that are passed down from one or both parents to their children. There are currently 11 types of OI.

What is osteogenesis imperfecta Type 4?

Osteogenesis imperfecta type IV is a moderate type of osteogenesis imperfecta (OI; see this term), a genetic disorder characterized by increased bone fragility, low bone mass and susceptibility to bone fractures.

What is Oi medical condition?

Osteogenesis imperfecta (OI) is a group of genetic disorders that mainly affect the bones. The term “osteogenesis imperfecta” means imperfect bone formation. People with this condition have bones that break (fracture) easily, often from mild trauma or with no apparent cause.

What is the life expectancy of someone with osteogenesis imperfecta?

The median survival time in the OI cohort was 72.4 years for males (compared to 81.5 years in the reference population) and 77.4 for females (compared to 84.5 in the reference population).

What is the cause of OI?

What causes osteogenesis imperfecta (OI)? OI is caused by defects in or related to a protein called type 1 collagen (pronounced KOL-uh-juhn). Collagen is an essential building block of the body. The body uses type 1 collagen to make bones strong and to build tendons, ligaments, teeth, and the whites of the eyes.

Where is osteogenesis imperfecta most common?

OI type II is estimated to occur in one in 60,000 live births. The overall prevalence of all types of OI is estimated at . 5 per 10,000 individuals in the United States. Approximately 20,000 to 50,000 individuals in the United States have OI.