Paget’s disease
Paget’s disease (PD), also known as osteitis deformans, is considered to be an osteometabolic disorder. It can be described as a focal disorder of accelerated and disorganized skeletal remodeling that may affect multiple bones in the body, giving rise to progressive enlargement and deformity of the bones and joints.1 PD is prevalent across both the sexes with incidence ranging from 1.5% to 8%.2 It is more common in individuals over 50 years of age.
Pathology
PD is described by a confined, disproportionate, and uncontrolled osteoclastic bone resorption. This results in subsequent compensatory augmented osteoblastic activity, ultimately resulting in an unstable, unstructured fibroblastic and biomechanical less stable bone.3 However, the precise pathophysiology of PDB still remains to be established. It is believed that PD may result from genetic alterations that could account for its presence in multiple family members.4 Other studies also hypothesize that paramyxovirus infection is the etiologic agent of the disorder.5
The disease process consists of the following three phases:
Phase I: An osteolytic phase characterized by noticeable and significant bone resorption and hyper vascularization.
Phase II: A sclerotic phase, which indicates a previous increase in bone formation but currently decreased cellular activity and vascularity.
Phase III: A phase comprising a mixture of both, active bone resorption, which is compensated by bone formation. This phase is characterized by a disordered skeletal structure, making the bone sponge-like, weak, and deformed.1
A typical course of Paget's disease is often asymptomatic. However, the clinical presentation of a symptomatic patient differs significantly. T...
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...f bone. J of Bone Miner R. 13(7):1061-10.
6) Josse RG, Hanley DA, Kendler D, et al. Diagnosis and treatment of Paget’s disease of bone. Clin Invest Med. 2007; 30(5): E210-E223.
7) Singer FR. Evolution of the clinical presentation of Paget’s disease of bone. Clin Rev Bone Miner Metab. 2002; 1(2): 95-98.
8) Roux C, Dougados M. Treatment of patients with Paget’s disease of Bone. Drugs. 1999 Nov; 58 (5): 823-830.
9) Selby PL, Davie MWJ, Ralston H, et al. Guidelines on the management of Paget’s disease of bone. Bone. 2002; 31: 10-9.
10) Patel S, Pearson D, Hosking DJ. Quantitative bone scintigraphy in the management of monostotic Paget’s disease of bone. Arthritis Rheum. 1995; 38: 1506-12.
11) Drake WM, Kendler DL, Brown JP. Consensus statement on the modern therapy of Paget’s disease of bone from a Western Osteoporosis Alliance Symposium. Clin Ther. 2001; 23: 620-6.
Fibrodysplasia ossificans progressiva also known as FOP is a one of the rarest, most disabling genetic bone conditions known to medicine. FOP causes muscles, tendons, ligaments, and other connective tissues to turn in to bone. Movement becomes limited in the affected areas of the body. People with FOP typically have malformed toes at birth, meaning the big toe is typically shorter than normal and abnormally turned outward in a position called a valgus deviation. Symptoms of FOP start to show up in early childhood. Most people with FOP develop painful tumor-like swellings also known as fibrous nodules. The fibrous nodules are visible on the neck, shoulders, and back.
Ooi, C., & Fraser, W. (1997). Paget's disease of bone. The Fellowship of Postgraduate Medicine, 73, 69-74. Retrieved June 12, 2010, from the PubMed database.
Hypophosphatasia is a rare genetic bone disorder characterized by osteoblast hyperactivity and bone remodeling with loss of, or incomplete, mineral deposition. It is comparable to osteomalacia and rickets, but maintains a unique set of characteristic identifiers (Mornet 2008; Brickley and Ives 2008). Also called, Rathbun’s Syndrome, hypophosphatasia can be autosomal dominant or autosomal recessive depending on the individual. Severe forms are usually transmitted as autosomal recessive with a recurrence risk of 25 percent, while milder forms can be transmitted as either autosomal dominant or autosomal recessive, with between 25-50 percent recurrence rates (Mornet 2008). Hypophosphatasia has an incidence rate of 1 in 100,000 live births (Brickley and Ives 2008; Wendling et al. 2001). In half of all cases, the condition is fatal.
Osteogenesis Imperfecta (OI), also called fragile bone ailment or Lobstein disorder, is an inherent bone issue portrayed by weak bones that are inclined to break effortlessly with practically zero cause. A arrangement of various sorts of OI is regularly used to depict how seriously a man with OI is affected.OI is brought on by hereditary deformities that influence the body's capacity to make solid bones. In predominant established OI, a man has too little sort I collagen or a low quality of sort I collagen because of a transformation in one of the sort I collagen qualities which makes the bones
Norvell, J. G. (2013, June 11). Tibia and Fibula Fracture Clinical Presentation. Retrieved from http://emedicine.medscape.com/article/826304-clinical
Introduction : Mrs Dorothy Beecham has been admitted to hospital with community –acquired pneumonia and query DVT. She is currently on waiting list of total knee replacement. Her past medical history including cardiovascular disease, COPD, osteoporosis, varicose vein and recurrent DVT in the past two years. A blood test has been done and result is available. DVT on her right calf is also confirmed by Doppler ultrasound.
Osteoporosis is a condition, which advances with age, resulting in fragile, weak bones due to a decrease in bone mass. Externally osteoporotic bone is shaped like normal bone, however it’s internal appearance differs. Internally the bone becomes porous due to a loss in essential minerals, including phosphate and calcium. The minerals are loss more quickly than they can be replaced and in turn cause the bones to become less dense and weak. The bones become prone to fracture, due to their weakness. Therefore the awareness of the disease tends to occur after a fracture has been sustained. The bones most commonly affected are the ribs, wrist, pelvis and the vertebrae.
J.P. was positive for dyspnea and a productive cough. She also was positive for dysuria and hematuria, but negative for flank pain. After close examination of her integumentary and musculoskeletal system, the examiner discovered a shiny firm shin on the right lower extremity with +2 edema complemented by severe pain. A set of baseline vitals were also performed, revealing a blood pressure of 124/80, pulse of 87 beats per minute, oxygen saturation of 99%, temperature of 97.3 degrees Fahrenheit, and respiration of 12 breaths per minute. The blood and metabolic panel exposed to several abnormal labs.
Osteoporosis is a disease in which the bones become so weak and brittle that even a cough can cause enough stress on the bone that it will cause the bone to facture. The most commonly broken bones are the hip, wrist, and the spine. Although it affects men and women of all races, post-menopausal Caucasian and Asian women are more commonly affected than those of other ethnicities and sexes. In fact, thirty percent of all post-menopausal women in the US and Europe will be diagnosed with Osteoporosis and at least 40 percent of those will suffer from a fracture in their lifetime.
Type IV of OI is much rarer, comprising only 5% of cases. The bones may appear normal at the time of the first...
The syndrome is caused because of Genetic mutation that replaces connective tissues (muscles) with bones when someone gets injured instead of getting cured. This results in a new skeletal structure. Unfortunately this syndrome does not have any cure and the patients are advised to always be careful and not to fall or have any kind of traumas. They can’t engage in any sports in order to prevent any injuries. Surgery for removal of extra bones is not an option because removal of bones will lead to ingrowth of more bones. From previous cases it is seen that most of the patients suffering from this condition do not live more than 40 years and they die of respiratory
The skeleton helps to support, move and protect the human body and its health is necessary for normal functioning. Because of the skeleton’s importance, diseases of the skeletal system can be debilitating. For example, a group of genetic disorders called osteogenesis imperfecta affects about 50 000 people in the United States alone Ref. The body of a person with osteogenesis imperfecta (OI), or ‘brittle bone disease’ cannot properly form bones due to a mutation preventing them from producing a healthy amount of collagen, causing bones to fracture easily. This paper provides an overview of osteogenesis imperfecta’s symptoms, genetic causes, diagnosis, and its development. treatment and effect on a patient’s life.
Osteoporosis comes from the latin word meaning “porous bone”. If you were to look closely at a bone you could see there are these small spaces on the bone which is good, now if you look at someone who suffers from osteoporosis you will see these spaces are much larger. As these spaces become larger it takes away from the strength and integrity of the bone making it grow weak and thin. Osteoporosis is most common in women over the age of 50 and gives them a higher risk of fractures and or breaks especially common in the hips. While we know osteoporosis comes from a number of things it can be broken down to age, the hormonal changes most commonly seen in menopause and a lower intake of Vitamin D and Calcium. Age is the unpreventable factor that doctors or you cannot change. Hormonal changes can be fixed with supplements or hormone therapy along with ones intake of Vitamin D and Calcium. Hormone therapy, estrogen alone or the combination of estrogen and progestin have been proven to prevent and aide in the treatment of osteoporosis in
The purpose the study is the presentation of successful use of Signafuse Bone Graft Putty in vivo. Fusion Products are commonly used in the field of Orthopaedics. They serve an important role as far as relieving pain and treating morbidities.
Paget’s disease persistent condition by the normal bone remodeling process. The regular bone has a balance that makes it lay down new bone and take up the old bone. We are suppose to have a normal calcium level in our blood with the bone remodeling. Paget’s disease is affected mostly in older bones of adults. Don’t get me wrong Paget’s disease can be found in children which is called juvenile Paget’s disease. Another name for Paget’s disease is osteitis deformans.