Tag Archives: Musculoskeletal Disorders

Trigger finger

Alternative Name : Stenosing tenosynovitis, trigger thumb, or trigger digit,

Definition:
Trigger finger is a common disorder of later adulthood characterized by catching, snapping or locking of the involved finger flexor tendon, associated with dysfunction and pain. A disparity in size between the flexor tendon and the surrounding retinacular pulley system, most commonly at the level of the first annular (A1) pulley, results in difficulty flexing or extending the finger and the “triggering” phenomenon. The label of trigger finger is used because when the finger unlocks, it pops back suddenly, as if releasing a trigger on a gun.
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One of your fingers or your thumb gets stuck in a bent position and then straightens with a snap — like a trigger being pulled and released. If trigger finger is severe, the finger may become locked in a bent position.

Often painful, trigger finger is caused by a narrowing of the sheath that surrounds the tendon in the affected finger. People whose work or hobbies require repetitive gripping actions are more susceptible. Trigger finger is also more common in women and in anyone with diabetes.

Symptoms:
Signs and symptoms of trigger finger may get progressed from mild to severe and include:

*Finger stiffness, particularly in the morning

*A popping or clicking sensation as you move your finger

*Tenderness or a bump (nodule) at the base of the affected finger

*Finger catching or locking in a bent position, which suddenly pops straight

*Finger locked in a bent position, which you are unable to straighten

Trigger finger more commonly occurs in your dominant hand, and most often affects your thumb or your middle or ring finger. More than one finger may be affected at a time, and both hands might be involved. Triggering is usually more pronounced in the morning, while firmly grasping an object or when straightening your finger.

Trigger finger is not the same as Dupuytren’s contracture — a condition that causes thickening and shortening of the connective tissue in the palm of the hand — though it may occur in conjunction with this disorder.

Causes:
The cause of trigger finger is a narrowing of the sheath that surrounds the tendon in the affected finger. Tendons are fibrous cords that attach muscle to bone. Each tendon is surrounded by a protective sheath — which, in turn, is lined with a substance called tenosynovium. The tenosynovium releases lubricating fluid that allows the tendon to glide smoothly within its protective sheath as you bend and straighten your finger — like a cord through a lubricated pipe.

But if the tenosynovium becomes inflamed frequently or for long periods, the space within the tendon sheath can become narrow and constricting. The tendon can’t glide through the sheath easily, at times catching the finger in a bent position before popping straight. With each catch, the tendon itself becomes more irritated and inflamed, worsening the problem. With prolonged inflammation, scarring and thickening (fibrosis) can occur and bumps (nodules) can form.

More than one potential causes have been described but the etiology remains idiopathic. It has also been called stenosing tenosynovitis (specifically digital tenovaginitis stenosans), but this may be a misnomer, as inflammation is not a predominant feature.

It has been speculated that repetitive forceful use of a digit leads to narrowing of the fibrous digital sheath in which it runs, but there is little scientific data to support this theory. The relationship of trigger finger to work activities is debatable and scientific evidence for and against hand use as a cause exist.

Risk Factors:
Risk Factors  developing trigger finger include:

Repeated gripping. If one routinely grips an item — such as a power tool or musical instrument — for extended periods of time, one may be more prone to developing a trigger finger.

Certain health problems. One is also at greater risk if he or she has certain medical conditions, including rheumatoid arthritis, diabetes, hypothyroidism, amyloidosis and certain infections, such as tuberculosis.Your sex. Trigger finger is more common in women.

Diagnosis:
Diagnosis is made almost exclusively by history and physical examination alone. More than one finger may be affected at a time, though it usually affects the thumb, middle, or ring finger. The triggering is usually more pronounced in the morning, or while gripping an object firmly.

Treatment:
Injection of the tendon sheath with a corticosteroid is effective over weeks to months in more than half of patients.

 

When corticosteroid injection fails, the problem is predictably resolved by a relatively simple surgical procedure (usually outpatient, under local anesthesia). The surgeon will cut the sheath that is restricting the tendon.

One recent study in the Journal of Hand Surgery suggests that the most cost-effective treatment is two trials of corticosteroid injection, followed by open release of the first annular pulley.  Choosing surgery immediately is the most expensive option and is often not necessary for resolution of symptoms.  More recently, a randomized controlled trial comparing corticosteroid injection with needle release and open release of the A1 pulley reported that only 57% of patients responded to corticosteroid injection (defined as being free of triggering symptoms for greater than 6 months). This is compared to a percutaneous needle release (100% success rate) and open release (100% success rate).  This is somewhat consistent with the most recent Cochrane Systematic Review of corticosteroid injection for trigger finger which found only 2 pseudo-randomized controlled trials for a total pooled success rate of only 37%.  However, this systematic review has not been updated since 2009.

There is a theoretical greater risk of nerve damage associated with the percutaneous needle release as the technique is performed without seeing the A1 pulley.

Investigative treatment options with limited scientific support include: non-steroidal anti-inflammatory drugs; occupational or physical therapy; steroid iontophoresis treatment; splinting; therapeutic ultrasound, phonophoresis (ultrasound with an anti-inflammatory dexamethasone cream); and Acupuncture.

Prognosis:
The natural history of disease for trigger finger remains uncertain.

There is some evidence that idiopathic trigger finger behaves differently in people with diabetes.

Recurrent triggering is unusual after successful injection and rare after successful surgery.

While difficulty extending the proximal interphalangeal joint may persist for months, it benefits from exercises to stretch the finger straighter.

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/Trigger_finger
http://www.mayoclinic.com/health/trigger-finger/DS00155
http://assets.sbnation.com/imported_assets/71765/trigger_finger_2.jpg
http://www.trigger-finger.com/
http://www.drmomeni.com/hand/trigger.html

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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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Torticollis

 

Alternative Names : Wry neck; Loxia

DEFINITION:
Torticollis is a twisted neck in which the head is tipped to one side, while the chin is turned to the other.It is a stiff neck associated with muscle spasm, classically causing lateral flexion contracture of the cervical spine musculature. The muscles affected are principally those supplied by the spinal accessory nerve.

CLICK  &  SEE THE PICTURES

Symptoms:
•Limited range of motion of the head
•Headache
•Head tremor
•Neck pain
•Shoulder is higher on one side of the body
•Stiffness of neck muscles
•Swelling of the neck muscles (possibly present at birth)

Types of Torticollis:

Temporary Torticollis: This type of wry neck usually disappears after one or two days. It can be caused by:

*swollen lymph nodes
*an ear infection
*a cold
*an injury to the head and neck that causes swelling

Fixed Torticollis:  Fixed torticollis is also called acute torticollis or permanent torticollis. It is usually due to a problem with muscle or bone structure.

Muscular Torticollis:
This is the most common type of fixed torticollis. It is caused by scarring or tight muscles on one side of the neck

Klippel-Feil Syndrome:
This is a congenital form of wry neck. It occurs when the bones in an infant’s neck have formed incorrectly. Children born with this condition may have difficulty with hearing and vision.

Cervical Dystonia:
This rare disorder is sometimes referred to as spasmodic torticollis. It causes neck muscles to contract in spasms. If you have cervical dystonia, your head twists or turns painfully to one side. It may also tilt forward or backward. Cervical dystonia sometimes goes away without treatment. However, there is a risk of recurrence.

This type of wry neck  or Torticollis can happen to anyone. However, it is most commonly diagnosed in middle age. It affects more women than men.

CAUSES:
Torticollis  can be inherited. It can also develop in the womb. This may happen if the fetus’ head is in the wrong position. It can also be caused by damage to the muscles or blood supply to the neck.

Anyone can develop wry neck after a muscle or nervous system injury. However, most of the time, the cause of wry neck is not known. This is called idiopathic torticollis.

DIAGNOSIS:
Evaluation of a child with torticollis begins with history taking to determine circumstances surrounding birth and any possibility of trauma or associated symptoms. Physical examination reveals decreased rotation and bending to the side opposite from the affected muscle. Some say that congenital cases more often involve the right side, but there is not complete agreement about this in published studies. Evaluation should include a thorough neurologic examination, and the possibility of associated conditions such as developmental dysplasia of the hip and clubfoot should be examined. Radiographs of the cervical spine should be obtained to rule out obvious bony abnormality, and MRI should be considered if there is concern about structural problems or other conditions.

Evaluation by an ophthalmologist should be considered in children to ensure that the torticollis is not caused by vision problems (IV cranial nerve palsy, nystagmus-associated “null position,” etc.). Most cases in infants respond well to physical therapy. Other causes should be treated as noted above.

TREATMENT:
Common treatments  might involve a multi-phase process:

1.Low-impact exercise to increase strong form neck stability
2.Manipulation of the neck by a chiropractor, physical therapist, or D.O.†
3.Extended heat application.
4.Repetitive shiatsu massage.

†An Osteopathic Physician (D.O.) may choose to use Cranial techniques to properly position the occipital condyles – thereby relieving compression of cranial nerve XI in children with Torticollis. This is an example of Osteopathic Manipulative Treatment.

Acquired torticollis:
Acquired torticollis occurs because of another problem and usually presents in previously normal children and adults…..

*A self-limiting spontaneously occurring form of torticollis with one or more painful neck muscles is by far the most common (‘stiff neck’) and will pass spontaneously in 1–4 weeks. Usually the sternocleidomastoid muscle or the trapezius muscle is involved. Sometimes draughts, colds or unusual postures are implicated; however in many cases no clear cause is found. These episodes are rarely seen by doctors other than a family physician.

*Trauma to the neck can cause atlantoaxial rotatory subluxation, in which the two vertebrae closest to the skull slide with respect to each other, tearing stabilizing ligaments; this condition is treated with traction to reduce the subluxation, followed by bracing or casting until the ligamentous injury heals.

*Tumors of the skull base (posterior fossa tumors) can compress the nerve supply to the neck and cause torticollis, and these problems must be treated surgically.

*Infections in the posterior pharynx can irritate the nerves supplying the neck muscles and cause torticollis, and these infections may be treated with antibiotics if they are not too severe, but could require surgical debridement in intractable cases.

*Ear infections and surgical removal of the adenoids can cause an entity known as Grisel’s syndrome, a subluxation of the upper cervical joints, mostly the atlantoaxial joint, due to inflammatory laxity of the ligaments caused by an infection. This bridge must either be broken through manipulation of the neck, or surgically resected.

*The use of certain drugs, such as antipsychotics, can cause torticollis.

*Antiemetics – Neuroleptic Class – Phenothiazines

There are many other rare causes of torticollis.

Spasmodic torticollis:
Torticollis with recurrent but transient contraction of the muscles of the neck and esp. of the sternocleidomastoid. “intermittent torticollis . “cervical dystonia”

TREATMENT: Botulinum toxin has been used to inhibit the spastic contractions of the affected muscles.

In animals:.CLICK & SEE
The condition can also occur in animals, usually as a result of an inner ear infection but sometimes as a result of an injury. It is seen largely in domestic rodents and rabbits, but may also appear in dogs and other different animals.

Possible ComplicationsComplications may include:

•Muscle swelling due to constant tension
•Neurological symptoms due to compressed nerve roots

Prognosis:
The condition may be easier to correct in infants and children. If the condition becomes chronic, numbness and tingling may develop as nerve roots become compressed in the neck.

The muscle itself may become large (hypertrophic) due to constant stimulation and exercise.

Botulinum toxin injections often provide substantial relief.

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/Torticollis
http://www.nlm.nih.gov/medlineplus/ency/article/000749.htm
http://www.umm.edu/imagepages/19090.htm

http://commons.wikimedia.org/wiki/File:Sternocleidomastoideus.png

http://www.healthline.com/health/torticollis#Causes2

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Juvenile Idiopathic Arthritis(JIA)

Definition:
Arthritis is an inflammation of the joints, with pain or stiffness. It may be acute or chronic. Acute arthritis is also called septic arthritis and may affect one or more joints.

Juvenile Idiopathic Arthritis (JIA) (once called Juvenile Rheumatoid Arthritis or JRA) is a type of arthritis that affects children who are under 16 years of age. This is an autoimmune disease that causes joints to swell and become stiff, sometimes hindering a child’s mobility. It can affect any joint, and in some cases it can affect internal organs and eyes as well. Symptoms can come and go, flare-up on occassion, while others have symptoms that never go away.

There are three types of JIA, which are diagnosed according to symptoms and blood tests:-
•Oligoarticular JIA – the most common kind of childhood arthritis, which often starts at the age of two or three. The problem is limited to four joints or fewer, which become swollen and painful. Sometimes the eyes are affected, too. It is also known as pauciarticular arthritis.

•Polyarticular JIA – affects five or more joints. It can start at any age, from a few months onwards, and usually spreads quite quickly from one joint to another. Children often feel generally unwell, sometimes with a fever.

•Systemic onset JIA – affects the whole body, and causes fever and rashes as well as inflamed and painful joints. It usually starts in children under five but can affect children of any age. It used to be called Still’s disease.

About one in 1,000 children has arthritis. In many cases, the inflammation stops in late childhood, but about one-third of children affected have problems that last into their adult life.

Juvenile idiopathic arthritis affects somewhere between 8 and 150 of every 100,000 children, depending on the analysis. Of these children, 50 percent have pauciarticular JIA, 40 percent have polyarticular JIA and 10 percent have systemic JIA.

Symptoms:
Symptoms depend on the type of arthritis and joints affected . They could include:
*Pain
*Fever
*Joint Pain
*Knee Pain
*Joint Swelling
*Ankle Pain
*Hip Pain
*Swollen Lymph Nodes
*Elbow Pain
*Wrist Pain
*Limited Range of Motion
*Morning Stiffness
*Migratory Joint Pain
*Hip Stiff
*Back Pain
*Rash
*Chronic pain
*Shoulder Pain
*Foot Pain
*Dry Mouth
*Joint Stiffness
*Eye Pain
*Arm Pain
*Inflamed Joint
*Double-Vision
*Visual Disturbance
*Joint Tenderness
*Enlarged Spleen
*Eye Redness
*Visual Impairment
*Joint Erythema
*Joints Warm
*Blurred vision
*Knee Stiff
*Feels Hot to Touch
*Light Hurts Eyes
*Uneven Limb Lengths

Causes:
So far the actual cause of JIA remains a mystery. However, the disorder is autoimmune   – meaning that the body’s own immune system starts to attack and destroy cells and tissues (particularly in the joints) for no apparent reason. It is believed that the immune system gets provoked by changes in the environment or perhaps there is an error in the gene. Experimental studies have shown that certain viruses that have mutated may be able to trigger JIA. JIA appears to be more common in young girls and the disease is most common in Caucasians.   Associated factors that may worsen or have been linked to rheumatoid arthritis include the following:

*genetic predisposition; it appears that when one family member has been diagnosed with rheumatoid arthritis, the chances are higher that other family members or
*siblings may also develop arthritis

*females are more likely to develop rheumatoid arthritis than males at all ages

*there is a strong belief that psychological stress may worsen the symptoms of rheumatoid arthritis. However, when the emotional stress is under control the arthritis symptoms do not always disappear suggesting that the association is not straightforward

*even though no distinct immune factor has been isolated as a cause of arthritis, there are some experts who believe that the triggering factor may be something like a virus which then disappears from the body after permanent damage is done

*because rheumatoid arthritis is more common in women, there is a belief that perhaps sex hormones may be playing a role in causing or modulating arthritis.

Unfortunately, neither sex hormone deficiency nor replacement has been shown to improve or worsen arthritis.
The cause of JIA, as the word idiopathic suggests, is unknown and currently an area of active research. Current understanding of JIA suggests that it arises in a genetically susceptible individual due to environmental factors.

Diagnosis:
Diagnosis of JIA is difficult because joint pain in children can be from many other causes. There is no single test that can confirm the diagnosis and most physicians use a combination of blood tests, x rays and the clinical presentation to make an initial diagnosis of JIA. The blood tests measure antibodies and the rheumatoid factor. Unfortunately, the rheumatoid factor is not present in all children with JIA. Moreover in most children the blood work is usually normal. X rays are obtained to ensure that the joint pain is not from a fracture, cancer, infection or a congenital abnormality.

In most cases, fluid from the joint is aspirated and analyzed. This test often helps in making a diagnosis of JIA by ruling out other causes of joint pain

Treatment :
The treatment of JIA is best undertaken by an experienced team of health professionals, including pediatric rheumatologists, nurse specialists, physiotherapists, and occupational therapists. Many others in the wider health and school communities also have valuable roles to play, such as ophthalmologists, dentists, orthopaedic surgeons, school nurses and teachers, careers advisors and, of course local general practitioners, paediatricians and rheumatologists. It is essential that every effort is made to involve the affected child and their family in disease education and balanced treatment decisions.

The major emphasis of treatment for JIA is to help the child regain normal level of physical and social activities. This is accomplished with the use of physical therapy, pain management strategies and social support.

There have been very beneficial advances in drug treatment over the last 20 years. Most children are treated with non-steroidal anti-inflammatory drugs and intra-articular corticosteroid injections. Methotrexate is a powerful drug which helps suppress joint inflammation in the majority of JIA patients with polyarthritis  (though less useful in systemic arthritis). Newer drugs have been developed recently, such as TNF alpha blockers, such as etanercept.  There is no controlled evidence to support the use of alternative remedies such as specific dietary exclusions, homeopathic treatment or acupuncture. However, an increased consumption of omega-3 fatty acids proved to be beneficial in two small studies.

Celecoxib has been found effective in one study.

Other aspects of managing JIA include physical and occupational therapy. Therapists can recommend the best exercise and also make protective equipment. Moreover, the child may require the use of special supports, ambulatory devices or splints to help them ambulate and function normally.

Surgery is only used to treat the most severe cases of JIA. In all cases, surgery is used to remove scars and improve joint function.

Home remedies that may help JIA includes getting regular exercises to increase muscle strength and joint flexibility. Swimming is perhaps the best activity for all children with JIA. Stiffness and swelling can also be reduced with application of cold packs but a nice warm bath or shower can also improve joint mobility

Complications:
JIA is a chronic disorder which if neglected can lead to serious complications. Proper follow up with health professionals can significantly reduce the chance of developing complications.

Eyes can be affected in some types of JIA. The inflamed eyes if left untreated can result in glaucoma, scars, cataracts and even blindness. Often the eye inflammation occurs without symptoms and thus it is important for all children to get regular eye checkups from an eye physician.

Growth retardation is common in children with JIA. Moreover, the medications (corticosteroids) used to treat JIA have potent side effects that can limit growth.

Children who delay treatment or do not participate in physical therapy can often develop joint deformities of the hand and fingers. Over time hand function is lost and almost impossible to recover.

Occupational therapy:
The best approach to treating a child with JIA involves a team of medical professionals including a rheumatologist, occupational therapist (OT), physical therapist, nurse and social worker.

The role of the OT is to help children participate as fully and independently as possible in their daily activities or “occupations”,  by preventing psychological and physical dependency. The aim is to maximize quality of life, and minimize disruption to the child’s and family’s life. OTs work with children, their families and schools, to come up with an individualized plan which is based on the child’s condition, limitations, strengths and goals. This is accomplished by ongoing assessments of a child’s abilities and social functioning. The plan may include the use of a variety of assistive devices, such as splints, that help a person perform tasks. The plan may also involve changes to the home, encouraging use of uninvolved joints, as well as providing the child and their family with support and education about the disease and strategies for managing it.  OT interventions will be changed depending on the progression and remission of JIA, in order to promote age-appropriate self-sufficiency. Early OT involvement is essential.

Self-care:
OTs can provide many strategies to assist children in their dressing routine. Clothes with easy openings and Velcro, as well as devices, such as buttonhooks and zipper pulls can be used. For children who have difficulty bending, a long handled reacher and sock aid is recommended. OTs may also show children how to sit during dressing so less strain is put on their joints.

OTs can help children maintain cleanliness through recommending assistive devices. For children who have trouble reaching all areas of their body, a long handled sponge with a soft grip can be provided. If children find it difficult to sit in a bath or stand in a shower, an OT can prescribe a bath bench or bath seat to be installed to help the child remain in a pain free position. If tooth brushing is challenging, a toothbrush with a larger, soft grip or an electric toothbrush may be recommended. For flossing, a flosser with an adapted handle may be provided.  Long handled hairbrushes may be used by children who have difficulty reaching the back of their head. Razors handles can be adapted for easier grip, or an electric razor may be used for shaving. The OT can also show girls wishing to use make-up, ways of increasing the sizes of the handles of make-up application tools for easier grip.

For children with pain in their hands and wrists, utensils and devices that are lightweight with large handles as well as other devices (such as angled knives, strap-on utensils, jar and bottle openers, turning handles, door knob extensions, etc.) can be provided to make the task easier, less painful and more enjoyable.[32] Tilted glasses can be used for children who have neck stiffness. Education can be provided about good eating habits that help control bone loss caused by inactivity and drug side effects. Occupational therapists provide a myriad of strategies to assist children with JIA in performing self-care tasks.

Leisure:
One of the best ways OTs can help children with JIA participate in activities with their friends is by helping them make their home exercise programs into play. Exercises are prescribed by both physiotherapists and OTs to increase the amount a child can move a joint and strengthen the joint to decrease pain and stiffness and prevent further limitations in their joint movements. OTs can provide children with age appropriate games and activities to allow the children to practice their exercises while playing and socializing with friends. Examples are crafts, swimming and non-competitive sports.

OTs will often prescribe custom made orthotics which are devices that support and correct body position and function. Orthotics help keep the child’s body in good alignment. Orthotics reduce discomfort in the legs and back when the child participates in physical activities such as sports. Splints can be used to support the joints during activity, to reduce the child’s pain and increase participation in their preferred leisure activities. Resting splints may be prescribed for children to wear during the night to reduce swelling and stiffness in joints, allowing children to have less pain and stiffness while participating in play activities.Furthermore, working splints are used to support the joint and relieve pain while working the with hands such as during crafts. A series of casts might be used to gradually extend shortened muscles allowing for increased participation in leisure activities.

OTs can help a child learn how to interact with their classmates and friends by collaboratively brainstorming strategies, role playing and modeling. OTs also help children see what activities they are good at and which ones give them difficulty. Furthermore, OTs can help children learn to communicate their pain to others. Benefits of OT treatment include: improved social interaction, improved self-confidence and a positive self-image. OTs can help children build friendships with other children suffering from similar diseases to help them feel less alone or less different from others. Many OTs run summer camps for children with similar diseases so children can get to know others with their disease. Education sessions on JIA and leisure, and activities such as swimming, canoeing and nature trails are common.

For children who find that cool or damp weather make it hard to play with friends outside, OTs can give ideas for clothing that will keep the child warm and dry without limiting movement. An example of this is biking gloves which allow children to move their fingers while still keeping their hands warm, as opposed to large winter gloves which limit hand function. Warm pajamas and electric blankets can reduce pain and improve sleep.

Prognosis:
With proper therapy, some children do improve with time and lead normal lives. However, severe cases of JIA which are not treated promptly can lead to poor growth and worsening of joint function. In the last two decades, significant improvements have been made in treatment of JIA and most children can lead a decent quality of life. The prognosis of JIA depends on prompt recognition and treatment. Finally, it is important for both the child and family member to be educated about the disorder. The more educated the person, the better the care you can receive. Chronic JIA is no longer the dreaded disease where one remains home bound. Many children with JIA have gone on to play professional sports and have a variety of successful careers

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://www.bbc.co.uk/health/physical_health/conditions/in_depth/arthritis/aboutarthritis_children.shtml
http://en.wikipedia.org/wiki/Juvenile_idiopathic_arthritis
http://www.healthline.com/channel/juvenile-rheumatoid-arthritis_symptoms
http://www.seattlecca.org/diseases/juvenileidiopathicarthritis-overview.cfm?gclid=CLCm7aSepakCFcO8Kgodl2kxyQ

http://apps.ashland.edu/index.php/File:Arthritic_joints.jpg

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Crouzon syndrome

Alternative Name :Branchial arch syndrome.

Definition:-
Crouzon syndrome is a genetic disorder of Chromosome 10.  Specifically, this syndrome affects the first branchial (or pharyngeal) arch, which is the precursor of the maxilla and mandible. Since the branchial arches are important developmental features in a growing embryo, disturbances in their development create lasting and widespread effects.

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This syndrome is named after Octave Crouzon, a French physician who first described this disorder. He noted the affected patients were a mother and her daughter, implying a genetic basis. First called “craniofacial dysostosis”, the disorder was characterized by a number of clinical features. This syndrome is caused by a mutation in the fibroblast growth factor receptor II, located on chromosome 10.

Breaking down the name, “craniofacial” refers to the skull and face, and “dysostosis” refers to malformation of bone.

Now known as Crouzon syndrome, the disease can be described by the rudimentary meanings of its former name. What occurs in the disease is that an infant’s skull and facial bones, while in development, fuse early or are unable to expand. Thus, normal bone growth cannot occur. Fusion of different sutures leads to different patterns of growth of the skull. Examples include: trigonocephaly (fusion of the metopic suture), brachycephaly (fusion of the coronal suture), dolichocephaly (fusion of the sagittal suture), plagiocephaly (unilateral premature closure of lambdoid and coronal sutures), oxycephaly (fusion of coronal and lambdoidal sutures), Kleeblattschaedel (premature closure of all sutures).

In the United States it is estimated to affect 1 per 60,000 live births.It is responsible for approximately 4.8% of all cases of craniosynostosis. Crouzon syndrome affects individuals of all ethnic backgrounds. It may be inherited or it may occur spontaneously.

Incidence of Crouzon syndrome is currently estimated to occur in 1 out of every 25,000 people out of the general population. There is a greater frequency in families with a history of the disorder, but that doesn’t mean that everyone in the family is affected (as referred to above).

Symptoms:-
Crouzon syndrome is usually diagnosed in infancy because of its particular face and skull deformities, which are:

•Early fusion of the bones of the skull (craniosynostosis), causing a misshapen head
•The skull problems may push the brain down (tonsillar herniation), and may obstruct the flow of cerebrospinal fluid (hydrocephalus)
•The nose and upper jaw appear sunken in because of poor bone growth in the face (midface hypoplasia)
•The eyes may appear to pop out (exophthalmos or proptosis) for the same reason (midface hypoplasia)


There may be other internal problems with the face and head such as narrow or absent ear canals, problems with the teeth and palate, and problems with the nose and sinuses. In some individuals with Crouzon syndrome (about 18%), two or more bones of the neck may be fused together.

Some individuals with the syndrome (about 5%) may also have a skin disorder called acanthosis nigricans, in which lesions of darkened, thickened skin are present.

Causes:
Associations with mutations in the genes of FGFR2 and FGFR3 have been identified as cause  of Crouzon syndrome. This FGFR2 gene provides instructions for making a protein called fibroblast growth factor receptor 2. Among its multiple functions, this protein signals immature cells to become bone cells during embryonic development. Mutations in the FGFR2 gene probably overstimulate signaling by the FGFR2 protein, which causes the bones of the skull to fuse prematurely.

The condition is inherited in an autosomal dominant way, so that each child of a person with Crouzon syndrome has a 50 per cent chance of inheriting the condition. However, in about half of all cases the syndrome has resulted from a new mutation (that is, neither parents were affected).

Like Apert syndrome, Crouzon syndrome may be more common among children born to older fathers.

Diagnosis:
Diagnosis of Crouzon syndrome usually can occur at birth by assessing the signs and symptoms of the baby. Further analysis, including radiographs, magnetic resonance imaging (MRI) scans, genetic testing, X-rays and CT scans can be used to confirm the diagnosis of Crouzon syndrome.

Treatment:
Like other genetic conditions, Crouzon’s cannot be ‘cured’. But with the right help and care, most children lead a relatively normal life.

Surgery is typically used to prevent the closure of sutures of the skull from damaging the brain’s development. Without surgery, blindness and mental retardation are typical outcomes. Craniofacial surgery is a discipline of plastic surgery. To move the orbits forward, plastic surgeons work with neurosurgeons to expose the skull and orbits and reshape the bone. To treat the midface deficiency, plastic surgeons can move the lower orbit and midface bones forward (this does not need neurosurgical assistance). For jaw surgery, either plastic surgeons have experience to perform these operations. It is rare to wear a custom-fitted helmet (or cranial band) for several months after surgery as that is only for single-suture “strip craniectomy” repair. Crouzon patients tend to have multiple sutures involved, most specifically bilateral coronal craniosynostoses, therefore an open operation is used rather than the strip craniectomy with helmeting.

Excessive fluid around the brain (hydrocephalus) may need to be drained by inserting a tube called a shunt. Other specialist help, for example, to treat dental, eye or ear, nose and throat problems, is often needed.

Long-term supportive treatments such as speech therapy, psychological and educational help, and genetic counselling for the family are also important in helping the child to reach their potential.

Once treated for the cranial vault symptoms, Crouzon patients generally go on to live a normal lifespan.

You may click to learn more  if you  have specific questions about Crouzon syndrome.

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://www.bbc.co.uk/health/physical_health/conditions/crouzon2.shtml
http://en.wikipedia.org/wiki/Crouzon_syndrome
http://emedicine.medscape.com/article/942989-overview
http://rarediseases.about.com/cs/crouzonsyndrome/a/011804.htm

http://emedicine.medscape.com/article/1280034-overview

http://ufacts.blogspot.com/2007/12/real-egg-head-boy.html

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