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- Types of Osteogenesis imperfecta / OI : A Guide for Nurses
- Basic Facts Osteogenesis Imperfecta (OI): A Guide for Nurses
- Questions and Answers about Rosacea
| Types of Osteogenesis imperfecta / OI : A Guide for Nurses Posted: 12 Apr 2011 11:40 PM PDT The Types of Osteogenesis imperfecta / OIIn 1979, Sillence and others devised a classification scheme that divides OI into four types based on clinical, radiographic, and genetic distinctions. Features of OI vary not only between types but within each type as well. Patients with OI may present with some but not all of the clinical features. Children and adults with milder OI may have few obvious signs. Some patients appear to have characteristics of several types. Patients may walk unassisted; require the assistance of walkers, crutches, or braces; or be wheelchair-dependent. All types of OI may include dentinogenesis imperfecta and varying degrees of blue sclera. The frequency of fractures may decrease after puberty. An increase in fractures may be seen in women following menopause and in men in later life. While the Sillence classification is part of the commonly accepted language of OI, nurses are urged to look beyond type alone. The key to optimal care is to be aware of the patient’s specific symptoms and capabilities and to treat each patient individually. See Appendix 2: Sillence Classifications of OI for a summary of information about the types of OI. * Type I – Mildest form of the disorder. Manifests with relatively few fractures, minimal limb deformities, blue sclera, and high incidence of hearing loss. Stature may be average or slightly shorter than average for the unaffected family members. Hearing loss onset is primarily in young adulthood but may occur in early childhood. Some patients have few fractures or obvious signs of OI. Some patients experience multiple fractures of the long bones, compression fractures of the vertebrae, and have chronic pain. Dentinogenesis imperfecta may or may not be present. Life expectancy seems to be normal. Recently, researchers have reported additional types that do not involve a defect of type I collagen. Clinically, these patients are similar to Type IV OI. Additional radiographic or histologic data are required to diagnose Types V and VI. * Type V – Moderate in severity and similar to Type IV but also characterized by large hypertrophic calluses that develop at sites of fractures or surgical procedures. Calcification of the membrane between the radius and ulna restricts forearm rotation. Diagnosis Osteogenesis imperfecta / OI The diagnostic process may include: * physical exam The physical exam includes assessment for abnormalities in: * skull formation Some characteristics are age-dependent. Hearing loss may not be apparent in infancy or childhood. Bone malformation may not be present in an infant or young child with mild disorder. Pale blue sclerae are normal up to 18 months of age. Intense scleral hue and its presence past 2 years of age can suggest the need for further evaluation for OI. While tinted sclerae are a characteristic of OI, it is important to note that only some patients exhibit blue sclera. See Appendix 2: Sillence Classifications of OI for additional information. for further information Types of Osteogenesis imperfecta / OI
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| Basic Facts Osteogenesis Imperfecta (OI): A Guide for Nurses Posted: 12 Apr 2011 11:33 PM PDT Basic Facts Osteogenesis Imperfecta (OI) Osteogenesis imperfecta / OI , also known as brittle bone disease, is a genetic disorder of connective tissue characterized by bones that fracture easily, often from little or no apparent trauma. It is highly variable in severity from patient to patient, ranging from very mild to lethal. In addition to having fractures, people with OI often have muscle weakness, joint laxity, skeletal malformations, and other connective tissue problems. The prevalence of Osteogenesis Imperfecta The prevalence of OI is approximately 1 in 20,000, including patients diagnosed after birth. OI occurs with equal frequency among males and females and among all racial and ethnic groups. Patients with OI have the full range of intellectual capabilities as seen in the general population. There is nothing inherent in the disorder that affects cognitive abilities. Life expectancy varies according to the underlying severity of the disorder and ranges from very brief (Type II OI) to average. Medical treatment for OI is increasingly understood. Patients with osteogenesis imperfecta usually have a faulty gene that instructs their bodies to make too little type I collagen or poor quality type I collagen. Type I collagen is the protein “scaffolding” of bone and other connective tissues. Inheritance, in nearly all cases, follows an autosomal dominant pattern, although sporadic cases are common. When there is no family history of OI, the disease is caused by new dominant mutations. Patients are often knowledgeable about their health status and the problems associated with OI. Accordingly, the opinions, requests, and instructions of adult patients and parents of children with OI should be respected. Depending on the severity of Osteogenesis Imperfecta or OI, the following characteristics may be seen: * skeletal malformation source Basic Facts Osteogenesis Imperfecta (OI): A Guide for Nurses
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| Questions and Answers about Rosacea Posted: 12 Apr 2011 11:18 PM PDT About Rosacea Rosacea is a chronic (long-term) disease that affects the skin and sometimes the eyes. The disorder is characterized by redness, pimples, and, in advanced stages, thickened skin. Rosacea usually affects the face. Skin on other parts of the upper body is only rarely involved. Approximately 14 million people in the United States have rosacea. It most often affects adults between the ages of 30 and 60. Rosacea is more common in women (particularly during menopause) than men. Although rosacea can develop in people of any skin color, it tends to occur most frequently and is most apparent in people with fair skin. There are several symptoms and conditions associated with rosacea. These include frequent flushing, vascular rosacea, inflammatory rosacea, and several other conditions involving the skin, eyes, and nose. Frequent flushing of the center of the face, which may include the forehead, nose, cheeks, and chin, occurs in the earliest stage of rosacea. The flushing often is accompanied by a burning sensation, particularly when creams or cosmetics are applied to the face. Sometimes the face is swollen slightly. A condition called vascular rosacea causes persistent flushing and redness. Blood vessels under the skin of the face may dilate (enlarge), showing through the skin as small red lines. This is called telangiectasia (tel-AN-je-ek-tay-ze-ah). The affected skin may be swollen slightly and feel warm. A condition called inflammatory Rosacea causes persistent redness and papules (pink bumps) and pustules (bumps containing pus) on the skin. Eye inflammation and sensitivity as well as telangiectasia also may occur. In the most advanced stage of rosacea, the skin becomes a deep shade of red and inflammation of the eye is more apparent. Numerous telangiectases are often present, and nodules in the skin may become painful. A condition called rhinophyma also may develop in some men; it is rare in women. Rhinophyma is characterized by an enlarged, bulbous, and red nose resulting from enlargement of the sebaceous (oil-producing) glands beneath the surface of the skin on the nose. People who have rosacea also may develop a thickening of the skin on the forehead, chin, cheeks, or other areas. In addition to skin problems, up to 50 percent of people who have rosacea have eye problems caused by the condition. Typical symptoms include redness, dryness, itching, burning, tearing, and the sensation of having sand in the eye. The eyelids may become inflamed and swollen. Some people say their eyes are sensitive to light and their vision is blurred or otherwise impaired. Doctors do not know the exact cause of rosacea but believe that some people may inherit a tendency to develop the disorder. People who blush frequently may be more likely to develop rosacea. Some researchers believe that rosacea is a disorder where blood vessels dilate too easily, resulting in flushing and redness. Factors that cause rosacea to flare up in one person may have no effect on another person. Although the following factors have not been well-researched, some people claim that one or more of them have aggravated their rosacea: heat (including hot baths), strenuous exercise, sunlight, wind, very cold temperatures, hot or spicy foods and drinks, alcohol consumption, menopause, emotional stress, and long-term use of topical steroids on the face. Patients affected by pustules may assume they are caused by bacteria, but researchers have not established a link between rosacea and bacteria or other organisms on the skin, in the hair follicles, or elsewhere in the body. Although there is no cure for rosacea, it can be treated and controlled. A dermatologist (a medical doctor who specializes in diseases of the skin) usually treats rosacea. The goals of treatment are to control the condition and improve the appearance of the patient's skin. It may take several weeks or months of treatment before a person notices an improvement of the skin. Some doctors will prescribe a topical antibiotic, such as metronidazole, which is applied directly to the affected skin. For people with more severe cases, doctors often prescribe an oral (taken by mouth) antibiotic. Tetracycline, minocycline, erythromycin, and doxycycline are the most common antibiotics used to treat rosacea. The papules and pustules symptomatic of rosacea may respond quickly to treatment, but the redness and flushing are less likely to improve. Some people who have rosacea become depressed by the changes in the appearance of their skin. People who have rosacea may experience low self-esteem, feel embarrassed by their appearance, and claim their social and professional interactions with others are adversely affected. A doctor should be consulted if a person feels unusually sad or has other symptoms of depression, such as loss of appetite or trouble concentrating. Doctors usually treat the eye problems of rosacea with oral antibiotics, particularly tetracycline or doxycycline. People who develop infections of the eyelids must practice frequent eyelid hygiene. The doctor may recommend scrubbing the eyelids gently with diluted baby shampoo or an over-the-counter eyelid cleaner and applying warm (but not hot) compresses several times a day. When eyes are severely affected, doctors may prescribe steroid eye drops. Electrosurgery and laser surgery are treatment options if red lines caused by dilated blood vessels appear in the skin or if rhinophyma develops. For some patients, laser surgery may improve the skin's appearance with little scarring or damage. For patients with rhinophyma, surgical removal of the excess tissue to reduce the size of the nose usually will improve the patient's appearance. source Questions and Answers about Rosacea
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