Classification of Osteogenesis Imperfecta Syndromes
Osteogenesis Imperfecta is a daunting condition which has a number of very difficult symptoms to deal with, which can even result in death. Congenital bone condition, where the individual suffers from brittle bones that are much more likely to break or fracture compared to normal bone tissue. It is "commonly known as brittle bone disease" (9). It can range in symptom type and severity dramatically. Some cases show very slight symptoms, while others can prove fatal. The most obvious and common symptoms relate to bone strength being compromised. In fact, "the hallmark feature of OI is fragile bones that fracture easily" (9). As such, bones become brittle and weak, being able to break and fracture much easier than typical bone tissue. However, the condition does not stop at affecting only bone tissue. It can impact other bodily systems as well. For example, "in some people, height, hearing, skin, blood vessels, muscles, tendons, and teeth may also be affected" (10). As such, it can be an incredibly dangerous condition, where treatment is only just now being able to show signs of potential relief for patients suffering from the syndrome.
The disorder is very diverse, and includes eight different known types of specific patterns of symptoms, and which vary from slight to potentially life threatening. There is actually a specific classification system which has eight specific types. Type I Osteogenesis Imperfecta syndrome has symptoms that are often found within the other types as well, even though the other types often prove much more severe and with even more potentially dangerous symptoms. According to the clinical research, Type I symptoms include very brittle bones which fracture easily, as well as a curved spine. Another major bone-related symptom is the presence of incredibly loose joints. Bones are not the only part of the body that is affected, however. In fact, muscle and skin tissue as well as vital organs can also be affected within this condition. There is also a potential for Type I to show signs of discoloration of the whites of the eyes and hearing loss, which can threaten perception. From Type I, the classification types show more severe and specified symptoms. Unfortunately, Type II cases often have short life expectancies because the bone structure is unable to facilitate breathing and underdevelopment of lungs and organ tissues. Thus, there are a multitude of respiratory and organ difficulties that accompany the disease at Type II. Next, Type III conditions show a lack of collagen or a disruption in collagen production. This ultimately causes bone deformity and increased risk of fracture, even before birth. Individuals with Type II Osteogenesis Imperfecta often are very short and can have facial deformities. Additionally, Type IV is concentrated in symptoms similar to Type I occurring before puberty. These cases also have the short height, barrel-shaped ribs and curved spines. Type V cases show mesh-like bone structures, where the bones have clear gaps within the surface of the bones. These cases often show abnormal amounts of repair tissue surrounding injuries, illustrating the ineffective nature of the repair system of the bone structures. Type VI shows the bone structure reminiscent of a fish scale appearance. Finally, Type VII is incredibly severe most of the time and is often found mostly in Native American populations. Clearly, there are great specifications which demand clarity in symptom documentation in order to be able to distinguish exactly what type of Osteogenesis Imperfecta has.
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