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Paget’s Disease of Bone

Definition:
Paget’s disease of bone causes affected bones to become enlarged and misshapen. Our bones are living tissue, and our bodies are constantly breaking down old bone and replacing it with new bone. In Paget’s disease, however, old bone is broken down and replaced at a faster rate than normal. The new bone is larger and weaker than normal bone.

Click to see the picture


Click to see the picture

Paget’s disease can occur in any bone in the body, but it is most common in the pelvis, spine, skull, and leg bones. It may occur in just one bone or in several bones, but it does not affect the entire skeleton or spread from affected bones to normal bones. Common symptoms include pain, misshapen bones, and a greater chance of broken bones.

Decisions about treating Paget’s disease can be complicated because: 1) no two people are affected in exactly the same way by the disease, and 2) it is sometimes difficult to predict whether a person with Paget’s disease who shows no signs of the disorder will develop symptoms or complications, such as a bone fracture, at a later date. Although there is no cure for Paget’s disease, medications (bisphosphonates and calcitonin) can help control the disorder and lessen pain and other symptoms. Paget’s disease experts recommend that these medications be taken by people with Paget’s disease who: have bone pain, headache, back pain, or a nerve-related symptom (such as “shooting” pains in the leg) that is directly associated with the disease; have elevated levels of serum alkaline phosphatase (SAP) in their blood; display evidence that a bone fracture will occur; require pretreatment therapy for affected bones that require surgery; have active symptoms in the skull, long bones, or vertebrae (spine); have the disease in bones located next to major joints, placing them at risk of developing osteoarthritis; develop a rare condition called hypercalcemia that occurs when a person with several bones affected by Paget’s disease and a high SAP level is immobilized.

Today’s medications, especially when started before complications begin, are often successful in controlling the disorder. Paget’s disease is rarely diagnosed in people less than 40 years of age. Women are more commonly affected than men. Prevalence of Paget’s disease ranges from 1.5 to 8.0 percent, depending on age and country of residence. Prevalence of familial Paget’s disease (where more than one family member has the disease) ranges from 10 to 40 percent in different parts of the world.[citation needed] Because early diagnosis and treatment is important, after age 40, siblings and children of someone with Paget’s disease may wish to have an alkaline phosphatase blood test every two or three years. If the alkaline phosphatase level is above normal, other tests such as a bone-specific alkaline phosphatase test, bone scan, or X-ray can be performed.

Named for Sir James Paget (1814–1899)

Symptoms:
Most people who have Paget’s disease of bone experience no symptoms. When symptoms do occur, the most common complaint is bone pain. The disease may affect only one or two areas of your body, or may be widespread. Your signs and symptoms, if any, will depend on the part of your body that’s affected, including:

*Pelvis. Paget’s disease of bone in the pelvis can cause hip pain.

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*Skull. An overgrowth of bone in the skull can cause hearing loss or headaches.

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*Spine. If your spine is affected, nerve roots can become compressed. This can cause pain, tingling and numbness in an arm or leg.

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*Leg. As the bones weaken, they may bend — causing you to become bow-legged. Enlarged and misshapen bones in your legs can put extra stress on nearby joints, which may cause wear-and-tear arthritis in your knee or hip.

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 Click to see the pictures

Other common  Symptoms are:

•Bone and joint pain.
•Deformity of a bone such as bowing of the leg, skull enlargement or curvature of the spine.
•Muscle weakness due to bones pressing on nerves.
•Bone fractures resulting from minor injury (bone affected by Paget’s disease has a disorganised structure and is mechanically weak).
•Arthritis (when Paget’s affects bone around a joint).
•Deafness, vision disturbance, dizziness and tinnitus when enlargement of skull bones puts pressure on vital nerves.
•A “hot spot” over an area of affected bone due to high blood supply.
Causes:
Even after you’ve reached your full height, your bones don’t stop growing. Bone is living tissue engaged in a continual process of renewal. During this constant process called remodeling, old bone is removed and replaced by new bone. Paget’s disease of bone disrupts this process.

Early in the course of the disease, old bone starts breaking down faster than new bone can be built. Over time, your body responds by generating new bone at a faster than normal rate. This rapid remodeling produces bone that’s softer and weaker than normal bone, which can lead to bone pain, deformities and fractures.

Scientists haven’t identified a cause of Paget’s disease of bone, though they have discovered several genes that appear to be linked to the disorder.

Some scientists believe Paget’s is related to a viral infection in your bone cells that may be present for many years before problems appear. Hereditary factors seem to influence whether you’re susceptible to the disease.
Risk Factors:
*Age. People older than 40 are the most likely to develop Paget’s disease of bone.

*Sex. Men are more commonly affected than are women.

*National origin. Paget’s disease of bone is more common in people of Anglo-Saxon descent.

*Family history. If you have a close relative who has Paget’s disease of bone, you are much more likely to develop the condition yourself.

Complications:
In most cases, Paget’s disease of bone progresses slowly. The disease can be managed effectively in nearly all people. Possible complications include:

*Fractures. Bones affected by Paget’s disease are large and dense, but also weak and brittle. This makes them more prone to fractures. More blood vessels are created in these deformed bones, so they bleed more during repair surgeries.

*Osteoarthritis. Misshapen bones can increase the amount of stress on nearby joints, which can cause osteoarthritis.

*Heart failure. Unusually extensive Paget’s disease may force your heart to work harder to pump blood to the affected areas of your body. In people with pre-existing heart disease, this increased workload can lead to heart failure.Bone cancer. Bone cancer occurs in less than 1 percent of people with Paget’s disease.
Diagnosis:
DiagnosisPaget’s disease may be diagnosed using one or more of the following tests:

*Pagetic bone has a characteristic appearance on X-rays. A skeletal survey is therefore indicated.

*An elevated level of alkaline phosphatase in the blood in combination with normal calcium, phosphate, and aminotransferase levels in an elderly patient are suggestive of Paget’s disease.

*Bone scans are useful in determining the extent and activity of the condition. If a bone scan suggests Paget’s disease, the affected bone(s) should be X-rayed to confirm the diagnosis.
Associated medical conditions:
Paget’s disease may lead to other medical conditions, including:

*Arthritis may be caused by bowing of long bones in the leg, distorting alignment and increasing pressure on nearby joints. In addition, pagetic bone may enlarge, causing joint surfaces to undergo excessive wear. In these cases, pain may be due to a combination of Paget’s disease and osteoarthritis.

*Loss of hearing in one or both ears may occur when Paget’s disease affects the skull and the bone that surrounds the inner ear. Treating the Paget’s disease may slow or stop hearing loss. Hearing aids may also help.

*Cardiovascular disease can result from severe Paget’s disease (i.e. with more than 15% skeletal involvement). Arteriovenous connections can often form in the bone, and so the heart has to work harder (pump more blood) to ensure adequate oxygen supply to the tissues. This increase in cardiac output can lead to calcification of the aortic valve, and the resulting aortic stenosis causes left ventricular hypertrophy and eventually high-output congestive failure.

*Kidney stones are somewhat more common in patients with Paget’s disease.

*Pagetic bone can cause nervous system problems, such as pressure on the brain, spinal cord, or nerves, and reduced blood flow to the brain and spinal cord.

*Rarely, Paget’s disease is associated with the development of osteosarcoma (malignant tumor of bone).
*When there is a sudden onset or worsening of pain, sarcoma should be considered.

*When Paget’s disease affects the facial bones, the teeth may become loose. Disturbance in chewing may occur.

*Rarely, when the skull is involved, the nerves to the eye may be affected, causing some loss of vision.

*Angioid streaks may develop, possibly as a result of calcification of collagen or other pathological deposition.

Paget’s disease is not associated with osteoporosis. Although Paget’s disease and osteoporosis can occur in the same patient, they are different disorders. Despite their marked differences, several treatments for Paget’s disease are also used to treat osteoporosis.

Treatment:
Endocrinologists (internists who specialize in hormonal and metabolic disorders), rheumatologists (internists who specialize in joint and muscle disorders), orthopedic surgeons, neurologists, and otolaryngologists are generally knowledgeable about treating Paget’s disease, and may be called upon to evaluate specialized symptoms.

Drug therapy
The goal of treatment is to relieve bone pain and prevent the progression of the disease. The U.S. Food and Drug Administration has approved the following treatments for Paget’s disease:

Bisphosphonates
Five bisphosphonates are currently available. In general, the most commonly prescribed are: risedronic acid (Actonel), alendronic acid (Fosamax) and pamidronic acid (Aredia). Etidronic acid (Didronel) and other bisphosphonates may be appropriate therapies for selected patients, but are less commonly used. As a rule, bisphosphonate tablets should be taken with 200-250 mL (6–8 oz) of tap water (not from a source with high mineral content) on an empty stomach. None of these drugs should be used by people with severe kidney disease.

*Etidronate disodium (Didronel) in tablet form is available in 200–400 mg doses. The approved regimen is once daily for six months; the higher dose (400 mg) is more commonly used. No food, beverage, or medications should be consumed for two hours before and after taking. The course should not exceed six months, but repeat courses can be given after rest periods, preferably of three to six months duration.

*Pamidronate disodium (Aredia) in intravenous form: the approved regimen uses a 30 mg infusion over four hours on each of three consecutive days, but a more commonly used regimen is 60 mg over two to four hours for two or more consecutive or nonconsecutive days.

*Alendronate sodium (Fosamax) is given as tablets of 40 mg once daily for six months; patients should wait at least 30 minutes after taking before eating any food, drinking anything other than tap water, taking any medication, or lying down (patient may sit).

*Tiludronate disodium (Skelid) in two tablets of 200 mg are taken once daily for three months; they may be taken any time of day, as long as there is a period of two hours before and after resuming food, beverages, and medications.

*Risedronate sodium (Actonel) as a 30 mg tablet taken once daily for 2 months is the prescribed regimen; patients should wait at least 30 minutes after taking before eating any food, drinking anything other than tap water, taking any medication, or lying down (patient may sit).

*Zoledronic acid (Reclast, Aclasta) is given as an intravenous infusion; a single dose (5 mg over 15 minutes) is effective for two years.

Calcitonin
*Miacalcin is administered by injection; 50 to 100 units daily or three times per week for 6–18 months. Repeat courses can be given after brief rest periods. Miacalcin may be appropriate for certain patients, but is seldom used. The nasal spray form of this drug is not approved for the treatment of Paget’s disease.

Diet and regular exercise:
In general, patients with Paget’s disease should receive 1000–1500 mg of calcium, adequate sunshine, and at least 400 units of vitamin D daily. This is especially important in patients being treated with bisphosphonates; however, taking oral bisphosphonates should be separated from taking calcium by at least two hours, because the calcium can inhibit the absorption of the bisphosphonate. Patients with a history of kidney stones should discuss calcium and vitamin D intake with their physicians.

Exercise is  most important in maintaining skeletal health, avoiding weight gain, and maintaining joint mobility. Since undue stress on affected bones should be avoided, patients should discuss any exercise program with their physicians before beginning.
Prognosis:
The outlook is generally good, particularly if treatment is given before major changes in the affected bones have occurred. Any bone or bones can be affected, but Paget’s disease occurs most frequently in the spine, skull, pelvis, femur, and lower legs. In general, symptoms progress slowly, and the disease does not spread to normal bones. Treatment can control Paget’s disease and lessen symptoms, but is not a cure. Osteogenic sarcoma, a form of bone cancer, is an extremely rare complication that occurs in less than one percent of all patients.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://en.wikipedia.org/wiki/Paget’s_disease_of_bone
http://www.bbc.co.uk/health/physical_health/conditions/pagets1.shtml
http://www.endotext.org/parathyroid/parathyroid15/parathyroid15.html
http://www.mayoclinic.com/health/pagets-disease-of-bone/DS00485
http://www.surgeongeneral.gov/library/bonehealth/chapter_3.html
http://www.sciencephoto.com/media/260576/enlarge

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Cyst

Definition:
A cyst is a closed, saclike structure that contains fluid, gas, or semisolid material and is not a normal part of the tissue where it is located. Cysts are common and can occur anywhere in the body in people of any age. Cysts vary in size; they may be detectable only under a microscope or they can grow so large that they displace normal organs and tissues. The outer wall of a cyst is called the capsule.

Click to see the picture

Ganglion Cyst

Ganglion Cyst (Photo credit: Glenn E. Malone)

A collection of pus is called an abscess, not a cyst. Once formed, a cyst could go away on its own or may have to be removed through surgery.

Locations:
*Acne cyst – Pseudocysts associated with cystic acne. Actually an inflammatory nodule with or without an associated epidermoid inclusion cyst.
*Arachnoid cyst (between the surface of the brain and the cranial base or on the arachnoid membrane)
*Baker’s cyst or popliteal cyst (behind the knee joint)
*Bartholin’s cyst
*Breast cyst
*Buccal bifurcation cyst
*Calcifying odontogenic cyst
*Chalazion cyst (eyelid)
*Choroid plexus cyst (brain)
*Colloid cyst
*Cysticercal cyst (the larval stage of Taenia sp. (Crain’s backs))
*Dentigerous cyst (associated with the crowns of non-erupted teeth)
*Dermoid cyst (ovaries, testes, many other locations from head to tailbone)
*Epididymal cyst (found in the vessels attached to the testes)
*Ganglion cyst (hand/foot joints and tendons)
*Glandular odontogenic cyst
*Glial cyst (in the brain)
*Gartner’s duct cyst (vaginal or vulvar cyst of embryological origin)
*Hydatid cyst (larval stage of Echinococcus granulosus (tapeworm))
*Hydrocele (testicle)
*Keratocyst (in the jaws, these can appear solitary or associated with the Gorlin-Goltz or Nevoid basal cell carcinoma syndrome. *The latest World Health Organization classification considers Keratocysts as tumors rather than cysts)
*Liver cystic disease
*Meibomian cyst (eyelid)
*Mucoid cyst (ganglion cysts of the digits)
*Nabothian cyst (cervix)
*Ovarian cyst (ovaries, functional and pathological)
*Paradental cyst
*Paratubal cyst (fallopian tube)
*Periapical cyst (The periapical cyst, otherwise known as radicular cyst, is the most common odontogenic cyst.)
*Pericardial cyst
*Peritoneal cyst (lining of the abdominal cavity)
*Pilar cyst (cyst of the scalp)
*Pilonidal cyst (skin infection near tailbone)
*Renal cyst (kidneys)
*Polycystic ovary syndrome
*Pineal gland cyst
*Radicular cyst (associated with the roots of non-vital teeth, also known as Periapical cyst)
*Residual cyst
*Sebaceous cyst (sac below skin)
*Spermatocele (testicle)
*Tarlov cyst (spine)
*Trichilemmal cyst – Same as a pilar cyst. A familial cyst of the scalp.
*Vocal fold cyst

Cystic fibrosis:
Despite being described in 1938 as the microscopic appearance of cysts in the pancreas, cystic fibrosis is an example of a genetic disorder whose name is related to fibrosis of the cystic duct and does not involve actual cysts

Cystic neoplasm:
Most cysts in the body are benign (dysfunctional) tumors, the result of plugged ducts or other natural body outlets for secretions. However sometimes these masses are considered neoplasm:

*Dermoid cyst
*Keratocyst
*Calcifying odotogenic cyst

Symptoms:
Sometimes you can feel a cyst yourself when you feel an abnormal “lump.” For example, cysts of the skin or tissues beneath the skin are usually noticeable. Cysts in the mammary glands (breasts) also may be palpable (meaning that you can feel them when you examine the area with your fingers). Cysts of internal organs such as the kidneys or liver may not produce any symptoms or may not be detected by the affected individual.

Causes:
Cysts can arise through a variety of processes in the body, including

#”wear and tear” or simple obstructions to the flow of fluid,

#infections,

#tumors,

#chronic inflammatory conditions,

#genetic (inherited) conditions,

#defects in developing organs in the embryo.

Most cysts arise due to the types of conditions listed above and are only preventable to the extent that the underlying cause is preventable.

Diagnosis:
Cysts of internal organs such as the kidneys or liver may not produce any symptoms or may not be detected by the affected individual. These cysts often are first discovered by imaging studies (X-ray, ultrasound, computerized tomography or CAT scan, and magnetic resonance imaging or MRI). Cysts may or may not produce symptoms, depending upon their size and location.

Treatment:
The treatment for a cyst depends upon the cause of the cyst along with its location. Cysts that are very large and result in symptoms due to their size may be surgically removed. Sometimes the fluid contained within a cyst can be drained, or aspirated, by inserting a needle or catheter into the cyst cavity, resulting in collapse of the cyst. Radiologic imaging may be used for guidance in draining (aspirating) cyst contents if the cyst is not easily accessible. Drainage or removal of a cyst at home is not advised.

Surgical removal of a cyst is sometimes necessary. If there is any suspicion that a cyst is cancerous, the cyst is generally removed by surgery or a biopsy is taken of the cyst wall (capsule) to rule out malignancy. In certain cases, aspirated fluid from a cyst is examined under a microscope to determine if cancer cells are present in the cyst.

If a cyst arises as part of a chronic medical condition (for example, in polycystic ovary syndrome or fibrocystic breast disease), treatment is generally directed at the underlying medical condition.

Prognosis:
The majority of cysts are benign conditions and do not result in long-term or serious complications. However, cysts that are associated with malignancy or serious infections can have a poor prognosis.

Prevention:
Prevention of cyst formation is only possible to the extent to which prevention of the underlying cause of the cyst is possible. Most kinds of cysts are not preventable.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://en.wikipedia.org/wiki/Cyst
http://www.medicinenet.com/cysts/article.htm

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When ‘Baby Fat’ is Good for Health

Want to shed your baby fat? Wait, don’t do it just yet, for a new study has revealed that such a fat is good – as long as it is calorie-burning -”Brown Fat”.

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Brown fat burns off calories and generates heat in babies and small mammals.

Most of the body fat is white fat, which also provides insulation but stores calories. It becomes “bad” fat when an individual have too much. The “good” fat-brown fat-was considered essentially nonexistent in human adults.

The new study has found that adults have much more of this type of fat than previously believed.

“We now know that it is present and functional in adults,” said the study’s lead author, Dr Aaron Cypess, MMSc, of the Joslin Diabetes Centre in Boston.

“Three ounces of brown fat can burn several hundred calories a day,” he added.

In the new study involving 1,970 study participants, researchers measured the patches of brown adipose tissue-brown fat-in people with the help of high-tech imaging method that combines positron emission tomography and computed tomography, called PET/CT.

By evaluating biopsy tissue of what appeared to be brown fat, the authors confirmed that they were, indeed, looking at stores of brown fat.

The researchers found that brown fat was located in an area extending from the front of the neck to the chest.

Of the subjects who had detectable brown fat, about 6 percent had 3 ounces or more of the fat.

“We believe that this percentage greatly underestimates the number of adults in the population who have a large amount of brown fat,” said Cypess.

They also discovered that brown fat is most abundant in young women and least frequent in older, overweight men. In fact, women were more than twice as likely as men to have substantial amounts of brown fat.

“One theory for this is that women may have less muscle mass overall, so they need more brown fat to generate heat and keep warm,” Cypess said.

Source: The study appears in New England Journal of Medicine.

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