CONGENITAL HIP DYSPLASIA
Definition of Congenital hip dysplasia
A malformation of the hip joint that is present at birth. Genetic factors likely play a role in this disorder. Features include hip dislocation, asymmetry of leg positions, asymmetric fat folds, and diminished movement on the affected side. Some children will exhibit little or no features and must be diagnosed by physical examination of the hip joints.
Description of Congenital hip dysplasia
Congenital hip dysplasia is an abnormal formation of the hip joint in which the ball at the top of the thighbone (femoral head) is not stable in the socket (acetabulum). Also, the ligaments of the hip joint may be loose and stretched. The degree of instability or looseness varies. A baby born with DDH may have the ball of his or her hip loosely in the socket, the looseness may worsen as the child grows and becomes more active, or the ball may be completely dislocated at birth.
Left untreated, DDH or Congenital hip dysplasia leads to pain and osteoarthritis by early adulthood. It may cause legs of different lengths or a "duck-like" walk and decreased agility. DDH has a familial tendency. It usually affects the left hip and is predominant in:
Girls.
First born children.
Babies born in the breech position (especially with feet up by the shoulders). The American Academy of Pediatrics now recommends ultrasound screening of all female, breech babies.
Although Congenital hip dysplasia is usually noted in the newborn exam, treatment is easier and safer the earlier the diagnosis is made. Hips found normal at birth can be found abnormal later, but this is rare. Pediatricians screen for DDH at a newborn's first exam and at every well-baby checkup thereafter. Otherwise, the condition may not be noticed until a child begins to walk – by which time treatment is more complicated and uncertain.
Symptoms of Congenital hip dysplasia
Although some dislocated hips show no signs, contact a doctor if your baby has:
Legs of different lengths.
Uneven thigh skin folds.
Less mobility or flexibility on one side.
In children who have begun to walk, limping, toe walking and a waddling "duck-like" gait are also signs.
In addition to visual clues, doctors use careful physical examination tests to check for subtle signs of hip instability or dislocation in babies, such as listening and feeling for "clunks." Hip X-rays also may be helpful in older infants and children.
Treatment methods depend upon the child's age.
Causes of Congenital hip dysplasia
Clinical studies show a familial tendency toward Congenital hip dysplasia, with more females affected than males. This disorder is found in many cultures around the world. However, statistics show that the Native American population has a high incidence of hip dislocation. This has been documented to be due to the common practice of swaddling and using cradleboards for restraining the infants. This places the infant's hips into extreme adduction (brought together). The incidence of congenital Congenital hip dysplasia is also higher in infants born by caesarian and breech position births. Evidence also shows a greater chance of this hip abnormality in the first born compared to the second or third child. Hormonal changes within the mother during pregnancy, resulting in increased ligament laxity, is thought to possibly cross over to the placenta and cause the baby to have lax ligaments while still in the womb. Other symptoms of complete dislocation include a shortening of the leg and limited ability to abduct the leg.
Diagnosis of Congenital hip dysplasia
Because the abnormalities of this hip problem often vary, a thorough physical examination is necessary for an accurate diagnosis of congenital Congenital hip dysplasia. The hip disorder can be diagnosed by moving the hip to determine if the head of the femur is moving in and out of the hip joint. One specific method, called the Ortolani test, begins with each of the examiners hands around the infant's knees, with the second and third fingers pointing down the child's thigh. With the legs abducted (moved apart), the examiner may be able to discern a distinct clicking sound with motion. If symptoms are present with a noted increase in abduction, the test is considered positive for hip joint instability. It is important to note this test is only valid a few weeks after birth.
The Barlow method is another test performed with the infant's hip brought together with knees in full bent position. The examiner's middle finger is placed over the outside of the hipbone while the thumb is placed on the inner side of the knee. The hip is abducted to where it can be felt if the hip is sliding out and then back in the joint. In older babies, if there is a lack of range of motion in one hip or even both hips, it is possible that the movement is blocked because the hip has dislocated and the muscles have contracted in that position. Also in older infants, hip dislocation is evident if one leg looks shorter than the other.
X-ray films can be helpful in detecting abnormal findings of the hip joint. X rays may also be helpful in finding the proper positioning of the hip joint for treatments of casting. Ultrasound has been noted as a safe and effective tool for the diagnosis of congenital Congenital hip dysplasia. Ultrasound has advantages over x rays, as several positions are noted during the ultrasound procedure. This is in contrast to only one position observed during the x ray.
Treatment of Congenital hip dysplasia
The objective of treatment is to replace the head of the femur into the acetabulum and, by applying constant pressure, to enlarge and deepen the socket. In the past, stabilization was achieved by placing rolled cotton diapers or a pillow between the thighs, thereby keeping the knees in a frog like position. More recently the Pavlik harness and von Rosen splint are commonly used in infants up to the age of six months. A stiff shell cast may be used, which achieves the same purpose, spreading the legs apart and forcing the head of the femur into the acetabulum. In some cases, in older children between six to 18 months, surgery may be necessary to reposition the joint. Also at this age, the use of closed manipulation may be applied successfully, by moving the leg around manually to replace joint. Operations are not only performed to reduce the dislocation of the hip, but also to repair a defect in the acetabulum. A cast is applied after the operation to hold the head of the femur in the correct position. The use of a home traction program is now more common. However, after the age of eight years, surgical procedures are primarily done for pain reduction measures only. Total hip surgeries may be inevitable later in adulthood.
Alternative treatment of Congenital hip dysplasia
Nonsurgical treatments include exercise programs, orthosis (a force system, often involving braces), and medications. A physical therapist may develop a program that includes strengthening, range-of-motion exercises, pain control, and functional activities. Chiropractic medicine may be helpful, especially the procedures of closed manipulations, to reduce the dislocated hip joint.
Prognosis
Unless corrected soon after birth, abnormal stresses cause malformation of the developing femur, with a characteristic limp or waddling gait. If cases of congenital Congenital hip dysplasia go untreated, the child will have difficulty walking , which could result in life-long pain. In addition, if this condition goes untreated, the abnormal hip positioning will force the acetabulum to locate to another position to accommodate the displaced femur.
Prevention
Prevention includes proper prenatal care to determine the position of the baby in the womb. This may be helpful in preparing for possible breech births associated with hip problems. Avoiding excessive and prolonged infant hip adduction may help prevent strain on the hip joints. Early diagnosis remains an important part of prevention of congenital Congenital hip dysplasia.
Definition of Congenital Congenital hip dysplasia
A malformation of the hip joint that is present at birth. Genetic factors likely play a role in this disorder. Features include hip dislocation, asymmetry of leg positions, asymmetric fat folds, and diminished movement on the affected side. Some children will exhibit little or no features and must be diagnosed by physical examination of the hip joints.
Descriptionof Congenital Congenital hip dysplasia
Congenital Congenital hip dysplasia is a disorder in children that is either present at birth or shortly thereafter. During gestation, the infant's hip should be developing with the head of the thigh bone (femur) sitting perfectly centered in its shallow socket (acetabulum). The acetabulum should cover the head of the femur as if it were a ball sitting inside of a cup. In the event of congenital Congenital hip dysplasia, the development of the acetabulum in an infant allows the femoral head to ride upward out of the joint socket, especially when weight bearing begins.
Causes & symptoms of Congenital Congenital hip dysplasia
Clinical studies show a familial tendency toward Congenital hip dysplasia, with more females affected than males. This disorder is found in many cultures around the world. However, statistics show that the Native American population has a high incidence of hip dislocation. This has been documented to be due to the common practice of swaddling and using cradleboards for restraining the infants. This places the infant's hips into extreme adduction (brought together). The incidence of congenital Congenital hip dysplasia is also higher in infants born by caesarian and breech position births. Evidence also shows a greater chance of this hip abnormality in the first born compared to the second or third child. Hormonal changes within the mother during pregnancy, resulting in increased ligament laxity, is thought to possibly cross over to the placenta and cause the baby to have lax ligaments while still in the womb. Other symptoms of complete dislocation include a shortening of the leg and limited ability to abduct the leg.
Diagnosis of Congenital Congenital hip dysplasia
Because the abnormalities of this hip problem often vary, a thorough physical examination is necessary for an accurate diagnosis of congenital Congenital hip dysplasia. The hip disorder can be diagnosed by moving the hip to determine if the head of the femur is moving in and out of the hip joint. One specific method, called the Ortolani test, begins with each of the examiners hands around the infant's knees, with the second and third fingers pointing down the child's thigh. With the legs abducted (moved apart), the examiner may be able to discern a distinct clicking sound with motion. If symptoms are present with a noted increase in abduction, the test is considered positive for hip joint instability. It is important to note this test is only valid a few weeks after birth.
The Barlow method is another test performed with the infant's hip brought together with knees in full bent position. The examiner's middle finger is placed over the outside of the hipbone while the thumb is placed on the inner side of the knee. The hip is abducted to where it can be felt if the hip is sliding out and then back in the joint. In older babies, if there is a lack of range of motion in one hip or even both hips, it is possible that the movement is blocked because the hip has dislocated and the muscles have contracted in that position. Also in older infants, hip dislocation is evident if one leg looks shorter than the other.
X-ray films can be helpful in detecting abnormal findings of the hip joint. X rays may also be helpful in finding the proper positioning of the hip joint for treatments of casting. Ultrasound has been noted as a safe and effective tool for the diagnosis of congenital Congenital hip dysplasia. Ultrasound has advantages over x rays, as several positions are noted during the ultrasound procedure. This is in contrast to only one position observed during the x ray.
Treatment of Congenital Congenital hip dysplasia
The objective of treatment is to replace the head of the femur into the acetabulum and, by applying constant pressure, to enlarge and deepen the socket. In the past, stabilization was achieved by placing rolled cotton diapers or a pillow between the thighs, thereby keeping the knees in a frog like position. More recently the Pavlik harness and von Rosen splint are commonly used in infants up to the age of six months. A stiff shell cast may be used, which achieves the same purpose, spreading the legs apart and forcing the head of the femur into the acetabulum. In some cases, in older children between six to 18 months, surgery may be necessary to reposition the joint. Also at this age, the use of closed manipulation may be applied successfully, by moving the leg around manually to replace joint. Operations are not only performed to reduce the dislocation of the hip, but also to repair a defect in the acetabulum. A cast is applied after the operation to hold the head of the femur in the correct position. The use of a home traction program is now more common. However, after the age of eight years, surgical procedures are primarily done for pain reduction measures only. Total hip surgeries may be inevitable later in adulthood.
Alternative treatment of Congenital Congenital hip dysplasia
Nonsurgical treatments include exercise programs, orthosis (a force system, often involving braces), and medications. A physical therapist may develop a program that includes strengthening, range-of-motion exercises, pain control, and functional activities. Chiropractic medicine may be helpful, especially the procedures of closed manipulations, to reduce the dislocated hip joint.
Prognosis of Congenital Congenital hip dysplasia
Unless corrected soon after birth, abnormal stresses cause malformation of the developing femur, with a characteristic limp or waddling gait. If cases of congenital Congenital hip dysplasia go untreated, the child will have difficulty walking , which could result in life-long pain. In addition, if this condition goes untreated, the abnormal hip positioning will force the acetabulum to locate to another position to accommodate the displaced femur.
Prevention of Congenital Congenital hip dysplasia
Prevention includes proper prenatal care to determine the position of the baby in the womb. This may be helpful in preparing for possible breech births associated with hip problems. Avoiding excessive and prolonged infant hip adduction may help prevent strain on the hip joints. Early diagnosis remains an important part of prevention of congenital Congenital hip dysplasia.
NOTE
Cervical dysplasia refers to a medical condition of the cervix in which squamous cells on the surface of the cervix undergo a premalignant transformation. This abnormal growth (dysplasia) may lead to the development of cervical cancer if left untreated. Cervical dysplasia can be diagnosed by a biopsy of the cervix. An abnormal Pap smear may lead to a recommendation for colposcopy of the cervix during which the cervix is examined under magnification. A biopsy of the cervix is taken of any abnormal appearing areas.
Fibrous Dysplasia
Fibrous dysplasia is a disease that causes growths or lesions in one or more bones of the human body. These lesions are tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Especially when involving the skull or facial bones, the lesions can cause externally visible deformities. The skull is often, but not necessarily, affected, and any other bone(s) can be involved. Many patients have lesions localized in only one bone (monostotic, affecting 70-80%), but others have them in many bones (polyostotic)
Vulva Dysplasia
The vulva is the external female genitalia, including the labia, clitoris, and vaginal opening. Vulva Dysplasia is any abnormal change in the vulva. Precancerous changes of the vulva (vulva dysplasia) may appear as white red or brown patches on the vulva and are often accompanied by itching
DEGENERATIVE JOINT DISEASE ... ARTHRITIS
Degenerative Joint Disease (degenerative arthritis) is a non-infectious, progressive disorder of the weight bearing joints. The normal articular joint cartilage is smooth, white and translucent. It is composed of cartilage cells (chondrocyte) imbedded in a sponge-like middle, or matrix, made of collagen, protein polysaccharides and water. With early, primary degenerative arthritis, the cartilage
becomes yellow and opaque with localized areas of softening and roughening of the surfaces. As the degeneration progresses, the soft areas become cracked and worn exposing bone under the cartilage, which begins to remodel and increase in density while any remaining cartilage begins to fray. Eventually, osteophytes (spurs of new bone), covered by cartilage, form at the edge of the joint. Also, as mechanical wear increases, the cartilage needs repairing. The cartilage cells are unable to produce enough of the sponge-like matrix and therefore the damaged cartilage cannot repair itself. In fact, it has no blood supply to enhance healing.
The majority of degenerative joint disease is the result of mechanical instabilities or aging changes within the joint. This includes old age degenerative arthritis and, in younger animals, may be the result of injuries, bruises, abnormal joint configuration, (i.e. hip dysplasia), or mechanical wear from anterior cruciate ligament rupture (see Volume V/5 of The Mastiff Reporter), patellar luxation, or osteochondritis dissecans.
SYMPTOMS of Degenerative Joint Disease
In most cases, degenerative joint disease is seen in dogs midway through their life expectancy, with an increasing incidence in the older animal. The early signs are mild and include a slight decrease in physical activity with a history that the dog is stiff, "not as playful", and is reluctant to go "on long walks". As the condition progresses, the animal, depending on the joint or joints involved, may stand up slowly after lying down for a period of time, may walk up and down stairs more slowly or may be reluctant to jump up as usual upon household furniture. At this stage, the individual's stiffness will diminish as he/she moves about. This is referred to as warming out of the lameness. Eventually, he/she may be asymptomatic at some time during the course of the day, only to have the whole episode repeat itself the next time he/she gets up from a rest. As the disease progresses, the above-mentioned signs increase in severity to a point where the dog refuses to stand and walk. Cold, damp weather accentuates the clinical signs in most cases, not only because of the cold temperature, but also because of barometric changes.
RADIOGRAPHS (x-rays)
Because cartilage does not show on radiographs (x-rays), early degenerative joint disease may show only a narrowing of the joint space. As the disease progresses, bone spurs, or osteophytes, form and the bone under the cartilage becomes very hard.
TREATMENT of Degenerative Joint Disease
Treatment is limited to reducing the degree of pain present in the joint capsule and surrounding ligaments (the joint cartilage has no nerve endings) so that activity can continue and muscular support can be increased. The most important consideration in the long term treatment of arthritis is the Maintenance of Muscular Support.
As the arthritis progresses and pain worsens, aspirin (Ascriptin), or more potent drugs such as Phenylbutazone, may be required. Corticosteroids can be given, but because of potential side effects, should be used only after other drugs have been tried and found to be unsuccessful. (Ed. Note: New non-steroidal anti-inflammatory drugs have been developed by Pfizer Labs and Fort Dodge are potential candidates for treatment of these problems -- ask your vet if your pet would be a good candidate for use of Rimidyl (Pfizer) or Ecto-Gesic (Fort Dodge) for pain and arthritis). These drugs should allow one to maintain the animal on normal exercise levels of walking, jogging or swimming. Swimming is by far the best activity, as it allows maximum motion with minimal weight bearing. Strenuous exercise is contraindicated. Obese dogs should be put on a reducing diet. If surgery is performed on the affected joint, passive flexion and extension exercises will begin soon after bandage removal to "work out the stiffness". This will be followed by very light activity as the long road toward complete rehabilitation is begun.
Another treatment involves drugs that actually help heal the matrix of the damaged cartilage. These innovative drugs have revolutionized the treatment of arthritis when treatment begins before irreversible damage has been sustained.
INFECTIOUS ARTHRITIS
Infectious arthritis is the result of the penetration of living microorganisms into the synovial membrane (joint lining), or into the synovial membrane and joint cavity. The most common type of organism causing this problem is bacteria, usually Staphylococcus or Streptococcus.
Three basic causes of bacterial arthritis are:
a penetrating wound of a joint,
the presence of a bacteremia (blood stream infection) with localization in the joint,
the spread of infection from an adjacent osteomyelitis (bone infection).
Once bacteria have entered the joint, the cartilage matrix begins to degenerate. Sometimes, after the patient has been treated and the joint rendered sterile, the cartilage destruction continues. It is thought that this is due to the inability of the uninjured chondrocyte to replace depleted matrix.
The clinical signs are those of the basic inflammatory process: swelling, heat, and pain in the joint(s), and occasional redness of the overlying skin. One or more joints may be involved. The body temperature is often elevated, and the patient is lethargic, occasionally anorectic, resists movement of the joint, and is lame in the affected limb(s). The area should be examined closely for a penetrating wound.
A definitive diagnosis of infectious arthritis can be made only by isolating the infecting organism from
the joint fluid or synovial tissue. Clinical history and signs, synovial fluid analysis (other than culture), and radiographic findings can help only to limit the possible causes of a painful joint.
DISCITIS
Discitis, or disc space infection, is an inflammatory lesion of the intervertebral disc that occurs in adults but more commonly in children. Its cause has been the subject of debate, although most authors believe it to be infectious. The infection probably begins in one of the continguous end plates, and the disc is infected secondarily. Severe back pain that begins insidiously is characteristic of the disease.
Discitis in Children
Although most children will continue to walk in spite of the pain, young children may refuse to ambulate. The characteristic finding is extension of the spine and the child's complete refusal to flex the spine. Children with discitis usually are not systemically ill. They rarely have an elevated temperature and their white blood cell count is frequently normal. However the erythrocyte sedimentation rate is usually increased. Lateral radiographs of the spine usually will reveal disc space narrowing with erosion of the vertebral end plates of the contiguous vertebrae. bone scanning may be helpful in localizing a lesion that is difficult to diagnose clinically. Some bone scans are falsely negative, so the diagnosis of disc space infection should not be excluded simply because the bone scan is normal. Magnetic resonance imaging (MRI) seems to be helpful in identifying a disc space infection.
The appropriate treatment of these lesions has been the subject of controversy. Most authors recommend plaster cast immobilization, a treatment that seems to be effective by itself in many cases. Some authors think that antibiotics also should be given because the condition most likely is an infection of the disc (the organism involved is frequently Staphylococcus aureus). In treating the lesion in children, a biopsy is not usually necessary. A biopsy may be indicated in adolescents or adults, especially if drug abuse is suspected, because of the possibility of organisms other than Staphylococcus aureus being present.
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Infective discitis
disc infection due to blood-borne bacteria, usually Staphylococcus aureus, or a low grade viral infection. Older children clinically present with back pain, similar to adults. The infective focus is in the thoracic or lumbar spine.
Younger children and infants may present with nonspecific abdominal pain, features of meningism, alteration in posture, or limp. The child may be febrile and ill. Because of this varied clinical presentation the diagnosis may be delayed. Upper respiratory tract infection or diarrhoea may precede the illness. The affected disc is in the lumbar spine. Treatment is bed rest, pain relief and antibiotics, and sometimes immobilization with plaster jacket. Failure to respond should suggest tuberculosis.
Radiologically, there is disc space narrowing, which may progress to end plate destruction. Scintigraphy shows generalised increased uptake in the adjacent vertebral bodies. On MRI, there is increased signal on T2-weighted images and gadolinium enhancement
DISCOID LUPUS ERYTHEMATOSUS
Discoid lupus erythematosus (DLE) is a disease in which coin-shaped (discoid) red bumps appear on the skin.
Description of discoid lupus erythematosus
The disease called discoid lupus erythematosus only affects the skin, although similar discoid skin lesions can occur in the serious disease called systemic lupus erythematosus (SLE). Only about 10% of all patients with DLE will go on to develop the multi-organ disease SLE.
The tendency to develop DLE seems to run in families. Although men or women of any age can develop DLE, it occurs in women three times more frequently than in men. The typical DLE patient is a woman in her 30s.
Causes and symptoms of discoid lupus erythematosus
The cause of DLE is unknown. It is thought that DLE (like SLE) may be an autoimmune disorder. Autoimmune disorders are those that occur when cells of the immune system are misdirected against the body. Normally, immune cells work to recognize and help destroy foreign invaders like bacteria, viruses, and fungi. In autoimmune disorders, these cells mistakenly recognize various tissues of the body as foreign invaders, and attack and destroy these tissues. In SLE, the misdirected immune cells are antibodies. In DLE, the damaging cells are believed to be a type of white blood cell called a T lymphocyte. The injury to the skin results in inflammation and the characteristic discoid lesions.
In DLE, the characteristic skin lesion is circular and raised. The reddish rash is about 5-10 mm in diameter, with the center often somewhat scaly and lighter in color than the darker outer ring. The surface of these lesions is sometimes described as "warty." There is rarely any itching or pain associated with discoid lesions. They tend to appear on the face, ears, neck, scalp, chest, back, and arms. As DLE lesions heal, they leave thickened, scarred areas of skin. When the scalp is severely affected, there may be associated hair loss (alopecia).
People with DLE tend to be quite sensitive to the sun. They are more likely to get a sunburn, and the sun is likely to worsen their discoid lesions.
Diagnosis of discoid lupus erythematosus
Diagnosis of DLE usually requires a skin biopsy. A small sample of a discoid lesion is removed, specially prepared, and examined under a microscope. Usually, the lesion has certain microscopic characteristics that allow it to be identified as a DLE lesion. Blood tests will not reveal the type of antibodies present in SLE, and physical examination usually does not reveal anything other than the skin lesions. If antibodies exist in the blood, or if other symptoms or physical signs are found, it is possible that the discoid lesions are a sign of SLE rather than DLE.
Treatment of discoid lupus erythematosus
Treatment of DLE primarily involves the use of a variety of skin creams. Sunscreens are used for protection. Steroid creams can be applied to decrease inflammation. Occasionally, small amounts of a steroid preparation will be injected with a needle into a specific lesion. Because of their long list of side effects, steroid preparations taken by mouth are avoided. Sometimes, short-term treatment with oral steroids will be used for particularly severe DLE outbreaks. Medications used to treat the infectious disease malaria are often used to treat DLE.
Alternative treatment of discoid lupus erythematosus
Alternative treatments for DLE include eating a healthy diet, low in red meat and dairy products and high in fish containing omega-3 fatty acids. These types of fish include mackerel, sardines, and salmon. Following a healthy diet is thought to decrease inflammation. Dietary supplements believed to be helpful include vitamins B, C, E, and selenium. Vitamin A is also recommended to improve DLE lesions. Constitutional homeopathic treatment can help heal DLE as well as help prevent it developing into SLE.
Prognosis of discoid lupus erythematosus
For the most part, the prognosis for people with DLE is excellent. While the lesions may be cosmetically unsightly, they are not life threatening and usually do not cause a patient to change his or her lifestyle. Only about 10% of patients with DLE will go on to develop SLE.
Prevention of discoid lupus erythematosus
DLE cannot be prevented. Recommendations to prevent flares of DLE in patients with the disease include avoiding exposure to sun and consistently using sunscreen.
ELEPHANTIASIS
Definition of Elephantiasis
Lymphatic filariasis, also known as elephantiasis, is best known from dramatic photos of people with grossly enlarged or swollen arms and legs. The disease is caused by parasitic worms, including Wuchereria bancrofti, Brugia malayi, and B. timori, all transmitted by mosquitoes. Lymphatic filariasis currently affects 120 million people worldwide, and 40 million of these people have serious disease.
When an infected female mosquito bites a person, she may inject the worm larvae, called microfilariae, into the blood. The microfilariae reproduce and spread throughout the bloodstream, where they can live for many years. Often disease symptoms do not appear until years after infection. As the parasites accumulate in the blood vessels, they can restrict circulation and cause fluid to build up in surrounding tissues. The most common, visible signs of infection are excessively enlarged arms, legs, genitalia, and breasts.
Medicines to treat lymphatic filariasis are most effective when used soon after infection, but they do have some toxic side effects. In addition, the disease is difficult to detect early. Therefore, improved treatments and laboratory tests are needed. A vaccine is not yet available.
Description of Elephantiasis
True elephantiasis is the result of a parasitic infection caused by three specific kinds of round worms. The long, threadlike worms block the body's lymphatic system--a network of channels, lymph nodes, and organs that helps maintain proper fluid levels in the body by draining lymph from tissues into the bloodstream. This blockage causes fluids to collect in the tissues, which can lead to great swelling, called "lymphedema." Limbs can swell so enormously that they resemble an elephant's foreleg in size, texture, and color. This is the severely disfiguring and disabling condition of elephantiasis.
There are a few different causes of elephantiasis, but the agents responsible for most of the elephantiasis in the world are filarial worms: white, slender round worms found in most tropical and subtropical places. They are transmitted by particular kinds (species) of mosquitoes, that is, bloodsucking insects. Infection with these worms is called "lymphatic filariasis" and over a long period of time can cause elephantiasis.
Lymphatic filariasis is a disease of underdeveloped regions found in South America, Central Africa, Asia, the Pacific Islands, and the Caribbean. It is a disease of the poor that has been present for centuries, as ancient Persian and Indian writings clearly described elephant-like swellings of the arms, legs, and genitals. It is estimated that 120 million people in the world have lymphatic filariasis, as of 1997. The disease appears to be spreading, in spite of decades of research in this area.
Other terms for elephantiasis are Barbados leg, elephant leg, morbus herculeus, mal de Cayenne, and myelolymphangioma.
Other situations that can lead to elephantiasis are:
A protozoan disease called leishmaniasis.
A repeated streptococcal infection.
The surgical removal of lymph nodes (usually to prevent the spread of cancer).
A hereditary birth defect.
Causes & symptoms of Elephantiasis
Three kinds of round worms cause elephantiasis filariasis: Wuchereria bancrofti, Brugia malayi, and Brugia timori. Of these three, W. bancrofti makes up about 90% of the cases. Man is the only known host of W. bancrofti.
Culex, Aedes, and Anopheles mosquitoes are the carriers of W. bancrofti. Anopheles and Mansonia mosquitoes are the carriers of B. malayi. In addition Anopheles mosquitoes are the carriers of B. timori.
Infected female mosquitoes take a blood meal from a human, and, in doing so, introduce larval forms of the particular parasite they carry to the person. These larvae migrate toward a lymphatic channel, then travel to various places within the lymphatic system, usually positioning themselves in or near lymph nodes throughout the body. During this time, they mature into more developed larvae and eventually into adult worms. Depending upon the species of round worm, this development can take a few months or more than a year. The adult worms grow to about 1 in (3.5 cm) to 4 in (10 cm) long.
The adult worms can live from about 3-8 years. Some have been known to live to 20 years, and in one case 40 years. The adult worms begin reproducing numerous live embryos, called microfilariae. The microfilariae travel to the bloodstream, where they can be ingested by a mosquito when it takes a blood meal from the infected person. If they are not ingested by a mosquito, the microfilariae die within about 12 months. If they are ingested by a mosquito, they continue to mature. They are totally dependent on their specific species of mosquito to develop further. The cycle continues when the mosquito takes another blood meal.
Most of the symptoms an infected person experiences are due to the blockage of the lymphatic system by the adult worms and due to the substances (excretions and secretions) produced by the worms.
The body's allergic reactions may include repeated episodes of fever, shaking chills, sweating, headaches, vomiting, and pain. Enlarged lymph nodes, swelling of the affected area, skin ulcers, bone and joint pain, tiredness, and red streaks along the arm or leg also may occur. Abscesses can form in lymph nodes or in the lymphatic vessels. They may appear at the surface of the skin as well.
Long-term infection with lymphatic filariasis can lead to lymphedema, hydrocele (a buildup of fluid in any saclike cavity or duct) in the scrotum, and elephantiasis of the legs, scrotum, arms, penis, breasts, and vulvae. The most common site of elephantiasis is the leg. It typically begins in the ankle and progresses to the foot and leg. At first the swollen leg may feel soft to the touch but eventually becomes hard and thick. The skin may appear darkened or warty and may even crack, allowing bacteria to infect the leg and complicate the disease. The microfilariae usually don't cause injury. In some instances, they cause "eosinophilia," an increased number of eosinophils (a type of white blood cells) in the blood.
This disease is more intense in people who never have been exposed to lymphatic filariasis than it is in the native people of tropical areas where the disease occurs. This is because many of the native people often are immunologically tolerant.
Diagnosis
The only sure way to diagnose lymphatic filariasis is by detecting the parasite itself, either the adult worms or the microfilariae.
Microscopic examination of the person's blood may reveal microfilariae. But many times, people who have been infected for a long time do not have microfilariae in their bloodstream. The absence of them, therefore, does not mean necessarily that the person is not infected. In these cases, examining the urine or hydrocele fluid or performing other clinical tests is necessary.
Collecting blood from the individual for microscopic examination should be done during the night when the microfilariae are more numerous in the bloodstream. (Interestingly, this is when mosquitoes bite most frequently.) During the day microfilariae migrate to deeper blood vessels in the body, especially in the lung. If it is decided to perform the blood test during the day, the infected individual may be given a "provocative" dose of medication to provoke the microfilariae to enter the bloodstream. Blood then can be collected an hour later for examination.
Detecting the adult worms can be difficult because they are deep within the lymphatic system and difficult to get to. Biopsies usually are not performed because they usually don't reveal much information.
Treatment
The drug of choice in treating lymphatic filariasis is diethylcarbamazine (DEC). The trade name in the United States is Hetrazan.
The treatment schedule is typically 2 mg/kg per day, three times a day, for three weeks. The drug is taken in tablet form.
DEC kills the microfilariae quickly and injures or kills the adult worms slowly, if at all. If all the adult worms are not killed, remaining paired males and females may continue to produce more larvae. Therefore, several courses of DEC treatment over a long time period may be necessary to rid the individual of the parasites.
DEC has been shown to reduce the size of enlarged lymph nodes and, when taken long-term, to reduce elephantiasis. In India, DEC has been given in the form of a medicated salt, which helps prevent spread of the disease.
The side effects of DEC almost all are due to the body's natural allergic reactions to the dying parasites rather than to the DEC itself. For this reason, DEC must be given carefully to reduce the danger to the individual. Side effects may include fever, chills, headache, dizziness, nausea and vomiting, itching, and joint pain. These side effects usually occur within the first few days of treatment. These side effects usually subside as the individual continues taking the drug.
There is an alternate treatment plan for the use of DEC. This plan is designed to kill the parasites slowly (to reduce allergic reactions to the dead microfilariae and dying adult worms within the body). Lower doses of DEC are taken for the first few days, followed by the higher dose of 2 mg/kg per day for the remaining three weeks. In addition, steroids may be prescribed to prevent the individual's body from reacting severely to the dead worms.
Another drug used is Ivermectin. Early research studies of Ivermectin show that it is excellent in killing microfilariae, but the effects of this drug on the adult worms are still being investigated. It is probable that patients will need to continue using DEC to kill the adult worms. Mild side effects of Ivermectin include headache, fever, and myalgia.
Other means of managing lymphatic filariasis are pressure bandages to wrap the swollen limb and elastic stockings to help reduce the pressure. Exercising and elevating a bandaged limb also can help reduce its size.
Surgery can be performed to reduce elephantiasis by removing excess fatty and fibrous tissue, draining the swelled area, and removing the dead worms.
Prognosis
With DEC treatment, the prognosis is good for early and mild cases of lymphatic filariasis. The prognosis is poor, however, for heavy parasitic infestations.
Prevention
The two main ways to control this disease are to take DEC preventively, which has shown to be effective, and to reduce the number of carrier insects in a particular area.
Avoiding mosquito bites with insecticides and insect repellents is helpful, as is wearing protective clothing and using bed netting.
Much effort has been made in cleaning the breeding sites (stagnant water) of mosquitoes near people's homes in areas where filariasis is found.
Before visiting countries where lymphatic filariasis is found, it would be wise to consult a travel physician to learn about current preventative measures.
FUNGAL ARTHRITIS
An inflammation of a joint caused by a fungal infection.
Causes of Fungal Arthritis
Fungal or mycotic arthritis is a very rare condition and may be caused by any of the invasive fungi, including coccidioidomycosis, histoplasmosis, blastomycosis, cryptococcosis, candidiasis, and sporotrichosis. These organisms may affect bone or joint tissue. One or more joints may be affected.
The large weight-bearing joints, especially the knee, are most commonly affected. The infection usually occurs as a result of an infection in another organ, frequently the lungs, and tends to progress very slowly.
Symptoms of Fungal Arthritis
joint swelling joint stiffness joint pain arthritis ankle, feet, and leg swelling Signs and tests of Fungal Arthritis
A culture of joint fluid that grows fungus
A joint X-ray showing joint changes
A synovial biopsy showing fungus
Positive antibody test (serology) for fungal disease
Skin tests
Treatment of Fungal Arthritis
The objective of treatment is to cure the infection with anti-fungal medication. Amphotericin B or medications in the azole family (fluconazole, ketoconazole, or itraconazole) are frequently used anti-fungal medications.
Chronic or advanced bone or joint infection may require surgical removal (debridement) of infected tissue.
Complications of Fungal Arthritis
Joint damage can occur if the infection is not treated.
Prevention of Fungal Arthritis
Thorough treatment of fungal infections elsewhere may help prevent fungal arthritis.
GUILLAIN-BARRÉ SYNDROME
Definition of Guillain-Barré syndrome
Guillain-Barrè (ghee-yan bah-ray) syndrome is a disorder in which the body's immune system attacks part of the peripheral nervous system. The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs. In many instances the weakness and abnormal sensations spread to the arms and upper body. These symptoms can increase in intensity until certain muscles cannot be used at all and, when severe, the patient is almost totally paralyzed. In these cases the disorder is life threatening – potentially interfering with breathing and, at times, with blood pressure or heart rate – and is considered a medical emergency. Such a patient is often put on a respirator to assist with breathing and is watched closely for problems such as an abnormal heart beat, infections, blood clots, and high or low blood pressure. Most patients, however, recover from even the most severe cases of Guillain-Barrè syndrome, although some continue to have a certain degree of weakness.
Guillain-Barrè syndrome can affect anybody. It can strike at any age and both sexes are equally prone to the disorder. The syndrome is rare, however, afflicting only about one person in 100,000. Usually Guillain-Barrè occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection. Occasionally surgery or vaccinations will trigger the syndrome. The disorder can develop over the course of hours or days, or it may take up to 3 to 4 weeks. Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear, and by the third week of the illness 90 percent of all patients are at their weakest.
Description of Guillain-Barré syndrome
The classic scenario in GBS involves a patient who has just recovered from a typical, seemingly uncomplicated viral infection. Symptoms of muscle weakness appear one to four weeks later. The most common preceding infections are cytomegalovirus, herpes, Epstein-Barr virus, and viral hepatitis. A gastrointestinal infection with the bacteria Campylobacter jejuni is also common and may cause a severe type of GBS from which it is particularly difficult to recover. About 5% of GBS patients have a surgical procedure as a preceding event. Patients with lymphoma, systemic lupus erythematosus, or AIDS have a higher than normal risk of GBS. Other GBS patients have recently received an immunization, while still others have no known preceding event. In 1976-77, there was a vastly increased number of GBS cases among people who had been recently vaccinated against the Swine flu. The reason for this phenomenon has never been identified, and no other flu vaccine has caused such an increase in GBS cases.
Causes & symptoms of Guillain-Barré syndrome
The cause of the weakness and paralysis of GBS is the loss of myelin, which is the material that coats nerve cells (the loss of myelin is called demyelination). Myelin is an insulating substance which is wrapped around nerves in the body, serving to speed conduction of nerve impulses. Without myelin, nerve conduction slows or stops. GBS has a short, severe course. It causes inflammation and destruction of the myelin sheath, and it disturbs multiple nerves. Therefore, it is considered an acute inflammatory demyelinating polyneuropathy.
The reason for the destruction of myelin in GBS is unknown, although it is thought that the underlying problem is autoimmune in nature. An autoimmune disorder is one in which the body's immune system, trained to fight against such foreign invaders as viruses and bacteria, somehow becomes improperly programmed. The immune system becomes confused, and is not able to distinguish between foreign invaders and the body itself. Elements of the immune system are unleashed against areas of the body, resulting in damage and destruction. For some reason, in the case of GBS, the myelin sheath appears to become a target for the body's own immune system.
The first symptoms of GBS consist of muscle weakness (legs first, then arms, then face), accompanied by prickly, tingling sensations (paresthesias). Symptoms affect both sides of the body simultaneously, a characteristic that helps distinguish GBS from other causes of weakness and paresthesias. Normal reflexes are first diminished, then lost. The weakness eventually affects all the voluntary muscles, resulting in paralysis. When those muscles necessary for breathing become paralyzed, the patient must be placed on a mechanical ventilator which takes over the function of breathing. This occurs about 30% of the time. Very severely ill GBS patients may have complications stemming from other nervous system abnormalities which can result in problems with fluid balance in the body, severely fluctuating blood pressure, and heart rhythm irregularities.
Diagnosis of Guillain-Barré syndrome
Diagnosis of GBS is made by looking for a particular cluster of symptoms (progressively worse muscle weakness and then paralysis), and by examining the fluid that bathes the brain and spinal canal through cerebrospinal fluid (CSF) analysis. This fluid is obtained by inserting a needle into the lower back (lumbar region). When examined in a laboratory, the CSF of a GBS patient will reveal a greater-than-normal quantity of protein, with normal numbers of white blood cells and a normal amount of sugar. Electrodiagnostic studies may show slowing or block of conduction in nerve endings in parts of the body other than the brain. Minor abnormalities will be present in 90% of patients.
Treatment
There is no direct treatment for GBS. Instead, treatments are used that support the patient with the disabilities caused by the disease. The progress of paralysis must be carefully monitored, in order to provide mechanical assistance for breathing if it becomes necessary. Careful attention must also be paid to the amount of fluid the patient is taking in by drinking and eliminating by urinating. Blood pressure, heart rate, and heart rhythm also must be monitored.
A procedure called plasmapheresis, performed early in the course of GBS, has been shown to shorten the course and severity of GBS. Plasmapheresis consists of withdrawing the patient's blood, passing it through an instrument that separates the different types of blood cells, and returning all the cellular components (red and white blood cells and platelets) along with either donor plasma or a manufactured replacement solution. This is thought to rid the blood of the substances that are attacking the patient's myelin.
It has also been shown that the use of high doses of immunoglobulin given intravenously (by drip through a needle in a vein) may be just as helpful as plasmapheresis. Immunoglobulin is a substance naturally manufactured by the body's immune system in response to various threats. It is interesting to note that corticosteroid medications (such as prednisone), often the mainstay of anti-autoimmune disease treatment, are not only unhelpful, but may in fact be harmful to patients with GBS.
Prognosis
About 85% of GBS patients make reasonably good recoveries. However, 30% of adult patients, and a greater percentage of children, never fully regain their previous level of muscle strength. Some of these patients suffer from residual weakness, others from permanent paralysis. About 10% of GBS patients begin to improve, then suffer a relapse. These patients suffer chronic GBS symptoms. About 5% of all GBS patients die, most from cardiac rhythm disturbances.
Patients with certain characteristics tend to have a worse outcome. These include people of older age, those who required breathing support with a mechanical ventilator, and those who had their worst symptoms within the first seven days.
Prevention
Because so little is known about what causes GBS to develop, there are no known methods of prevention.
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