Musculoskeletal Disorders Part 3 Muscular Disorders

Maria Carmela L.
Domocmat, RN,MSN Instructor, Curative and Rehabilitative Nursing Care Management II School of Nursing Northern Luzon Adventist College Artacho, Sison, Pangasinan
Part 1: Degenerative & Metabolic bone disorders: Part 2: Bone infections Part 3: Muscular disorders
Muscular dystrophy Rhabdomyolysis
Part Part Part Part
4: Disorders of the hand 5: Spinal column deformities 6 : Disorders of foot 7: Sports Injuries
Maria Carmela L. Domocmat, RN, MSN 3/5/2012 2
Maria Carmela L. Domocmat, RN, MSN
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The word "dystrophy" comes originally from the Greek "dys," which means "difficult" or "faulty," and "trophe," meaning "nourishment." This word was chosen many years ago because it was at first believed that poor nourishment of the muscles was in some way to blame for muscular dystrophy. Today we know that muscle wasting in the disorder is caused by defective genes rather than poor nutrition.
http://www.humanillnesses.com/original/Men-Os/Muscular-Dystrophy.html
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refers to a group of more than 30 inherited diseases that cause muscle weakness and muscle loss. Some forms of MD appear in infancy or childhood, while others may not appear until middle age or later. The different muscular dystrophies vary in who they affect and the symptoms. All forms of MD grow worse as the person's muscles get weaker. Most people with MD eventually lose the ability to walk.
http://www.nlm.nih.gov/medlineplus/musculardystrophy.html
http://www.humanillnesses.com/original/images/hdc_0001_0002_0_i mg0181.jpg
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characterized by progressive weakness and degeneration of the skeletal muscles that control movement. Some forms seen in infancy or childhoodothers may not appear until middle age or later. differ in terms of the
distribution and extent of muscle weakness
(some forms of MD also affect cardiac muscle)
age of onset rate of progression, and pattern of inheritance

http://www.ninds.nih.gov/disorders/md/md.htm Maria Carmela L. Domocmat, RN, MSN
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are a group of inherited conditions caused by incorrect or missing genetic information that prevents the body from making the proteins needed to build and maintain healthy muscles. Many cases of MD occur from spontaneous mutations that are not found in the genes of either parent, and this defect can be passed to the next generation.
http://pathologyproject.wordpress.com/2011/04/24/muscular-dystrophy/
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is a general term for a group of inherited diseases involving a defective gene. Each form of muscular dystrophy is caused by a genetic mutation that's particular to that type of the disease. The most common types of muscular dystrophy appear to be due to a genetic deficiency of the muscle protein dystrophin
http://www.mayoclinic.com/health/musculardystrophy/DS00200/DSECTION=symptoms
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Duchenne's and Becker's muscular dystrophies - passed from mother to son through one of the mother's genes in a pattern called X-linked recessive inheritance. Boys inherit an X chromosome from their mothers and a Y chromosome from their fathers. The X-Y combination makes them male. Girls inherit two X chromosomes, one from their mothers and one from their fathers. The X-X combination determines that they are female.
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The defective gene that causes Duchenne's and Becker's muscular dystrophies is located on the X-chromosome. Women who have only one X-chromosome with the defective gene that causes these muscular dystrophies are carriers and sometimes develop heart muscle problems (cardiomyopathy) and mild muscle weakness.
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The disease can skip a generation until another son inherits the defective gene on the X-chromosome. In some cases of Duchenne's and Becker's muscular dystrophies, the disease arises from a new mutation in a gene rather than from an inherited defective gene.
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Women can pass down Xlinked recessive disorders such as Duchenne's muscular dystrophy. A woman who is a carrier of an X-linked recessive disorder has a 25 percent chance of having an unaffected son, a 25 percent chance of having an affected son, a 25 percent chance of having an unaffected daughter and a 25 percent chance of having a daughter who also is a carrier.
http://www.mayoclinic.com/health/medical/IM02723 Maria Carmela L. Domocmat, RN, MSN
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Myotonic dystrophy and most MFMs -passed along in a pattern called autosomal dominant inheritance. inheritance If either parent carries the defective gene for myotonic dystrophy, there's a 50 percent chance the disorder will be passed along to a child.
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Some of the less common types of muscular dystrophy are passed along in the same inheritance pattern that marks Duchenne's and Becker's muscular dystrophies. Other types of muscular dystrophy can be passed on from generation to generation and affect males and females equally. Still others require a defective gene from both parents.
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In an autosomal dominant disorder, the mutated gene is a dominant gene located on one of the nonsex chromosomes (autosomes). You need only one mutated gene to be affected by this type of disorder. A person with an autosomal dominant disorder in this case, the father has a 50 percent chance of having an affected child with one mutated gene (dominant gene) and a 50 percent chance of having an unaffected child with two normal genes (recessive genes).
http://www.mayoclinic.com/health/medical/IM00991
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most common form of MD primarily affects boys. caused by the absence of dystrophin, aprotein in volved in maintaining the integrity of muscle. Onset is between 3 and 5 years Progresses rapidly.
Most boys are unable to walk by age 12, and later need a respirator to breathe.
Girls in these families have a 50percent chance of inheriting and passing the defective gene to their children.
http://www.ninds.nih.gov/disorders/md/md.htm Maria Carmela L. Domocmat, RN, MSN 3/5/2012 17
a group of genetic disorders that affect the use of muscles in the body. inherited as an X-linked disorder. This is why Duchenne MD primarily affects boys. Girls can inherit the gene for DMD but not have any symptoms of the disease. affects approximately 1 in every 3,500 live male births. There are thousands of new cases every year. affects children of all ethnic backgrounds. causes an absence of dystrophin, a protein that helps keep muscle cells intact.
This means that muscle cells are easily damaged and become weak over time.
http://www.checkorphan.org/grid/iwishes/duchenne-musculardystrophy Maria Carmela L. Domocmat, RN, MSN 3/5/2012 18
http://www.checkorphan.org/grid/iwishes/duchenne-musculardystrophy Maria Carmela L. Domocmat, RN, MSN 3/5/2012 19
http://trialx.com/curetalk/wpcontent/blogs.dir/7/files/2011/05/diseases/Muscular_Dystrophy_Duc henne-3.jpg Maria Carmela L. Domocmat, RN, MSN
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similar to Duchenne but the symptoms are milder and can appear till 25 years of age. Boys with Becker MD have faulty or not enou gh dystrophi Usually the affected people can live and enjoy life and are also able to walk but they have some heart problems and is present only in males.
http://healthmedcare.com/muscular-dystrophy-types-symptoms-of-each-form/
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usually begins in the teenage years. causes progressive weakness in muscles of th e face, arms, legs, and around the shoulders an d chest. affects both males and females progresses slowly and can vary in symptoms f rom mild to disabling. about half of the sufferers are able to walk throughout their life and almost all the patients live a normal life span.
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Also known as Landouzy-Dejerine dystrophy Landouzyinvolves progressive muscle weakness involving:

Face Shoulders Abdomen Feet Upper arms Pelvic area Lower arms
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When someone with facioscapulohumeral MD raises his or her arms, the shoulder blades may stick out like wings. Progression of this form is slow, with some spurts of rapidly increasing weakness. Onset usually occurs during the teen to early adult years.
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Aka: Steinerts disease the disorder's most common adult form produces stiffness of muscles and an inability to relax muscles at will (myotonia), as well as the muscle weakness of the other forms of muscular dystrophy. typified by prolonged muscle spasms, cataracts, cardiac abnormalities, and endocrine disturbance s. Individuals with myotonic MD have
long, thin faces drooping eyelids a swan-like neck
http://www.ninds.nih.gov/disorders/md/md.htm RN, Maria Carmela L. Domocmat, MSN
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Cause
A repeated section of DNA on either chromosome 19 or chromosome 3.
Onset
Congenital form appears at birth. More common form may begin in teen or adult years.
http://www.mda.org/disease/dm.html
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http://www.websters-onlinedictionary.org/images/wiki/wikipedia/commons/thumb/e/e1/Autodominant.jpg/180pxMaria Carmela L. Domocmat, RN, Autodominant.jpg
http://www.websters-onlinedictionary.org/images/wiki/wikipedia/commons/thumb/e/e1/Autodominant.jpg/180px -Autodominant.jpg
MSN
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Symptoms
Generalized weakness and muscle wasting first affecting the face, lower legs, forearms, hands and neck, with delayed relaxation of muscles after contraction common. Other symptoms involve the gastrointestinal system, vision, heart or respiration. Learning disabilities occur in some cases. Congenital myotonic dystrophy is the more severe form. Men with myotonic muscular dystrophy have baldness on their foreheads.
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Progression
Progression is slow, sometimes spanning 50 to 60 years.
Inheritance
Autosomal dominant; the disease may be inherited through either the father or the mother.
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http://blog.thirdeyehealth.com/images/muscular-dystrophy-1.jpg
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The other major types of muscular dystrophy include:

Limb-girdle muscular dystrophy Congenital muscular dystrophy Oculopharyngeal muscular dystrophy Distal muscular dystrophy Emery-Dreifuss muscular dystrophy Myofibrillar myopathies
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the type which affects from teenage years to adulthood is present in both males and females. As the name indicates, in this type the problem starts from hip (pelvic girdle) region and then reaches to the shoulders ( pectoral girdle ) and later legs and arms are also affected, sufferers are unable to walk and most patients live past mid adulthood.
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the form which is present at the time of birth rare cause the loss of muscles The term "congenital muscular dystrophy" refers to a group of inherited muscular dystrophies. Signs of these disorders may include:
General muscle weakness Joint deformities
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is apparent at birth or becomes evident before age 2. The course of this disorder varies significantly depending on the type.
Some forms of congenital MD progress slowly and cause only mild disability, while others progress rapidly and cause severe impairment.
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Signs in infants may include:

Severe muscle weakness Difficulty sucking and swallowing Difficulty breathing Cognitive impairment
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affects primarily the muscles of eyes and throat which occur around 40s to onward ages symptoms include the weakness of eyes and facial muscles which could later cause dysphagia - predisposes the patients to pneumonia and choking.
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The first sign of this type of muscular dystrophy is usually drooping of the eyelids followed by weakness of the muscles of the eye, face and throat, resulting in difficulty swallowing. Progression is slow. Signs and symptoms first appear in adulthood, usually in a person's 40s or 50s.
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very rare mildest affects the muscles of fore arms to hands and muscles of lower legs to feet very slowly progressing not very severe
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This group involves the muscles farthest away from the center of the body (distal muscles) those of the hands, forearms, feet and lower legs. The severity is generally less than for other forms of MD, and this form tends to progress slowly. Distal MD generally begins in adulthood between the ages of 40 and 60.
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the type which affects from childhood to teen years present only in the males affects the muscles of pectoral region to upper arms and lower parts of legs and along with that patients have extreme heart problems that are usually fatal. This is the type which also affects the carriers (females) but the symptoms are not very severe.
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This form of muscular dystrophy usually begins in the muscles of the:

Shoulders Upper arms Shins
Cardiac arrhythmias, stiffness of the spine and muscle contractures are other features of Emery-Dreifuss MD. Emery-Dreifuss MD usually begins in the childhood to early teen years and progresses slowly.
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Though in some cases the MFMs affect only the muscles closest to the center of the body (proximal muscles) such as the shoulder and hip muscles the distal muscles also are usually involved. This group of muscle disorders also is commonly associated with:
Stiffness of the spine Muscle contractures Nerve damage (peripheral neuropathy) Thickening and stiffening of the heart muscle (cardiomyopathy)
http://healthmedcare.com/muscular-dystrophy-types-symptoms-of-eachform/ Maria Carmela L. Domocmat, RN,
MSN
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Progressive muscular weakness Delayed meeting of motor milestones Waddling gait Walking on toes Frequent falls Gowers sign Hyperthrophied calf muscles Poor balance Scoliosis of the spine Fracture of long bones Inability to walk (late stages)
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Waddling gait, a distinctive ducklike walk, is an important sign of muscular dystrophy, spinal muscle atrophy or, rarely, congenital hip displacement. The gait results from deterioration of the pelvic girdle musclesprimarily the gluteus medius, hip flexors, and hip extensors. Weakness in these muscles hinders stabilization of the weightbearing hip during walking, causing the opposite hip to drop and the trunk to lean toward that side in an attempt to maintain balance.
http://www.wrongdiagnosis.com/bookimages/8/2591.1.png
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Typically, the legs assume a wide stance and the trunk is thrown back to further improve stability, exaggerating lordosis and abdominal protrusion. In severe cases, leg and foot muscle contractures may cause equinovarus deformity of the foot combined with circumduction or bowing of the legs.
http://www.wrongdiagnosis.com/bookimages/8/2591.1.png
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With Duchenne's muscular dystrophy, waddling gait becomes clinically evident between ages 3 and 5. The gait worsens as the disease progresses, until the child loses the ability to walk and requires the use of a wheelchair, usually between ages 10 and 12. Early signs are usually subtle: a delay in learning to walk, frequent falls, gait or posture abnormalities, and intermittent calf pain
http://www.wrongdiagnosis.com/symptoms/walking_symptoms/book-causes-16g.htm
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With Becker's muscular dystrophy, waddling gait typically becomes apparent in late adolescence, slowly worsens during the third decade, and culminates in total loss of ambulation. Muscle weakness first appears in the pelvic and upper arm muscles. Progressive wasting with selected muscle hypertrophy produces lordosis with abdominal protrusion, poor balance, a positive Gowers'sign and, possibly, mental retardation.
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With facioscapulohumeral muscular dystrophy, which usually occurs late in childhood and during adolescence, waddling gait appears after muscle wasting has spread downward from the face and shoulder girdle to the pelvic girdle and legs. Earlier effects include progressive weakness and atrophy of facial, shoulder, and arm muscles; slight lordosis; and pelvic instability.
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to stand, affected children press their hands against their ankles, knees and thighs
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Morbidity and mortality rates depend on the type of congenital muscular dystrophy. Its incidence varies, as some forms are more common than others. Duchenne muscular dystrophy affects one in 3300 live male births.
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Myotonic muscular dystrophy is the most frequent form of muscular dystrophy among adults. Its prevalence is estimated at one case for every 10,000 people in most countries. However, this frequency is 20 times higher in the Charlevoix and Saguenay-Lac-St-Jean regions, where one person out of 500 is estimated to carry the disease.
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Careful review of family's history of muscle disease medical history review and physical examination Blood tests
Damaged muscles release enzymes, such as creatine kinase (CK), into blood. High blood levels of CK suggest a muscle disease, such as muscular dystrophy.
Electromyography Ultrasonography Muscle biopsy

http://www.mayoclinic.com/health/musculardystrophy/DS00200/DSECTION=treatments-and-drugs
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Genetic testing.
Blood samples are examined for mutations in some of the genes that cause different types of muscular dystrophy. For Duchenne's and Becker's muscular dystrophies, standard tests examine just the portions of the dystrophin gene responsible for most cases of these types of MD. These tests identify deletions or duplications on the dystrophin gene in more than twothirds of people with Duchenne's and Becker's MDs. The genetic defects responsible for Duchenne's and Becker's muscular dystrophies are harder to identify in other cases of those affected, but new tests that examine the entire dystrophin gene are making it possible to pinpoint tiny, less common mutations.
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There is no cure for muscular dystrophy dystrophy. Current treatment is designed to help prevent or reduce deformities in the joints and the spine and to allow people with MD to remain mobile as long as possible.
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Treatments include
Physical and speech therapy Orthopedic devices Surgery Medications
Some people with muscular dystrophy have mild cases that worsen slowly. Other cases are disabling and severe. Research into gene therapy may eventually provide treatment to stop the progression of some types of muscular dystrophy.
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As muscular dystrophy progresses and muscles weaken, fixations (contractures) can develop in joints. Tendons can shorten, restricting the flexibility and mobility of joints. Contractures are uncomfortable and may affect the joints of your hands, feet, elbows, knees and hips. One goal of physical therapy is to provide regular range-of-motion exercises to keep joints as flexible as possible, delaying the progression of contractures, and reducing or delaying curvature of your spine. Using hot baths (hydrotherapy) also can help maintain range of motion in joints.
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In some cases, doctors may prescribe medications to slow the progression and manage signs and symptoms of muscular dystrophy:
Muscle spasms, stiffness and weakness myotonia). ).Medications that may be used to help (myotonia). manage myotonia associated with MD include mexiletine (Mexitil), phenytoin (Dilantin, Phenytek), baclofen (Lioresal), dantrolene (Dantrium) and carbamazepine (Tegretol, Carbatrol).
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Muscle deterioration. The anti-inflammatory corticosteroid medication prednisone may help improve muscle strength and delay the progression of Duchenne's MD. The immunosuppressive drugs cyclosporin and azathioprine also are sometimes prescribed to delay some damage to dying muscle cells.
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Assistive devices Braces can both provide support for weakened muscles of your hands and lower legs and help keep muscles and tendons stretched and flexible, slowing the progression of contractures. Other devices, such as canes, walkers and wheelchairs, can help maintain mobility and independence. If respiratory muscles become weakened, using a ventilator may become necessary.
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Surgery To release the contractures that may develop and that can position joints in painful ways, doctors can perform a tendon release surgery. This may be done to relieve tendons of your hip and knee and on the Achilles tendon at the back of your foot. Surgery may also be needed to correct curvature of the spine.
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Other treatments Because respiratory infections may become a problem in later stages of muscular dystrophy, it's important to be vaccinated for pneumonia and to keep up to date with influenza shots.
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Perform passive and active muscle-stretching exercises to the arms and legs. Encourage the patient to walk at least 3 hours each day (with leg braces, if necessary) to maintain muscle strength, reduce contractures, and delay further gait deterioration, if possible.
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Stay near the patient during ambulation, to provide support if necessary. Provide a balanced diet to maintain energy levels and prevent obesity. Because of the grim prognosis associated with muscular dystrophy and spinal muscle atrophy, provide emotional support for the patient and his family.
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Caution the patient about long, unbroken periods of bed rest, which accelerate muscle deterioration. Refer the patient to a local Muscular Dystrophy Association chapter, as indicated. Suggest genetic counseling for parents, if they're considering having more children.
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On the cover: Andy Vladimir, of Coconut Grove, Fla., had MMD and used a wheelchair, but that barely slowed him down. A successful businessman, textbook author, world traveler and travel writer, including for MDA's Quest magazine, Andy lived to age 76.
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http://www.mdausa.org/ http://www.mayoclinic.com/health/muscular dystrophy/DS00200/DSECTION=treatmentsand-drugs
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a condition that may occur when muscle tissue is damaged due to an injury in which muscle in the body is damaged rhabdomyo=skeletal muscle + lysis= rapid breakdown
http://www.emedicinehealth.com/rhabdomyolysis/page2_ em.htm
There are a three of types of muscle in the body, including:

skeletal muscles that move the body; cardiac muscle located in the heart; and smooth muscle that lines blood vessels, gastrointestinal tract, bronchi in the lung, and the bladder and uterus. This type of muscle is not under conscious control.
Rhabdomyolysis occurs when there is damage to the skeletal muscle.

http://www.emedicinehealth.com/rhabdomyolysis/page2_ em.htm
is the breakdown of muscle fibers with leakage of potentially toxic cellular contents into the systemic circulation. The final common pathway of rhabdomyolysis may be a disturbance in myocyte calcium homeostasis
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The injured muscle cell leaks myoglobin (a protein) into the blood stream. Myoglobin can be directly toxic to kidney cells, and it can impair and clog the filtration system of the kidney. Both mechanisms can lead to kidney failure (the major complication of rhabdomyolysis).
http://www.emedicinehealth.com/rhabdomyolysis/page2_ Maria Carmela L. Domocmat, RN, em.htm MSN
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Significant muscle injury can cause fluid and electrolyte shifts from the bloodstream into the damaged muscle cells, and in the other direction (from the damaged muscle cells into the bloodstream). As a result, dehydration may occur. Elevated levels of potassium in the bloodstream (hyperkalemia) may be associated with heart rhythm disturbances and sudden cardiac death due to ventricular tachycardia and ventricular fibrillation.
http://www.emedicinehealth.com/rhabdomyolysis/page2_ Maria Carmela L. Domocmat, RN, em.htm MSN
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When muscles are damaged, especially due to a crush injury, swelling within the muscle can occur, causing compartment syndrome. If this occurs in an area where the muscle is bound by fascia (a tough fibrous tissue membrane), the pressure inside the muscle compartment can increase to the point at which blood supply to the muscle is compromised and muscle cells begin to die.
Medscpae
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Rhabdomyolysis was first appreciated as a significant complication from crush and blast injuries sustained in a volcano eruption in Italy, in 1908. Victims of the blast injuries during the first and second World Wars help further understand the relationship between massive muscle damage and kidney failure.
Medscpae
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United States
Rhabdomyolysis accounts for an estimated 8-15% of cases of acute renal failure.
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When muscle is damaged

myoglobin (protein pigment) - released into the bloodstream and filtered out of the body by the kidneys. Myoglobin breaks down into potentially harmful compounds. It may block the structures of the kidney, causing damage such as acute tubular necrosis or kidney failure.
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Dead muscle tissue may cause a large amount of fluid to move from the blood into the muscle, reducing the fluid volume of the body and leading to shock and reduced blood flow to the kidneys. The disorder may be caused by any condition that results in damage to skeletal muscle, especially trauma.
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The etiologies may be subdivided into

Traumatic
exercise induced toxicologic
Environmental Metabolic Infectious Immunologic inherited classifications
http://emedicine.medscape.com/article/827738clinical#showall
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Major blunt trauma and crush injury Electrocution Lightening strikes Major burns Excessive exercise, for example, running a marathon or excessive weight lifting Patients in status epilepticus, in which the seizure lasts for a prolonged period of time and muscles involuntarily contract
http://www.emedicinehealth.com/rhabdomyolysis/page3_em.htm
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Prolonged immobilization
patients who have been lying in one position for a prolonged period of time due to a debilitating stroke, alcohol or drug overdose, or those who have remained unconscious for a prolonged period of time for other reasons The weight of the body is enough to crush the muscles that are pushed up against a hard surface such as the floor.
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Dystonic reactions cause muscles to spasm, and if left untreated can damage muscle Cholesterol lowering medications [for example, statins prescribed to treat high cholesterol (particularly when combined with other cholesterol lowering medications such as fibrates) Antidepressant medications
[for example selective serotonin reuptake inhibitors (SSRIs) antidepressants may cause a serotonin syndrome characterized by agitation, fever, and muscle spasm]
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Some anesthetics can cause malignant hyperthermia syndrome with high fever and muscle rigidity A variety of drugs of abuse [for example, cocaine, heroin, phencyclidine (PCP), and amphetamines] Hyperthermia and hypothermia (high and low body temperature, respectively)
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Complications from a variety of infections caused by bacteria, viruses, and fungi Association with other diseases such as sickle cell disease, polymyositis, and dermatomyositis Complications from the venom from snake bites and black widow spider bites
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Alcoholism (with subsequent muscle tremors) Certain inherited or genetic syndromes Crush Injuries Heat intolerance Heatstroke Ischemia or necrosis of the muscles (as may occur with arterial occlusion, deep venous thrombosis, or other conditions)
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Low phosphate levels Seizures Severe exertion such as marathon running or calisthenics Shaking chills Trauma Use or overdose of drugs, especially cocaine, amphetamines, statins, heroin, or PCP
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Melli et al reviewed 475 patients with rhabdomyolysis hospitalized at Johns Hopkins Hospital and found that the most common risk factors were exogenous toxins toxins, with
illicit drugs, alcohol, and prescription medications responsible in 46% of patients.
http://emedicine.medscape.com/article/827738-clinical#showall
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Abnormal urine color (dark, red, or cola colored) General weakness Muscle stiffness or aching (myalgia) Muscle tenderness Weakness of the affected muscles
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Additional symptoms that may be associated with this disease include the following:
Fatigue Joint pain Seizures Weight gain (unintentional)
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An examination reveals tender or damaged skeletal muscles.

CPK is very high. Serum myoglobin test is positive. Serum potassium may be very high Urinalysis may reveal casts and be positive for hemoglobin without evidence of red blood cells on microscopic examination. Urine myoglobin test is positive.
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This disease may also alter the results of the following tests:
CPK isoenzymes Urine creatinine Serum creatinine
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Vigorous hydration with isotonic crystalloid

the cornerstone of therapy for rhabdomyolysis. Support of the intravascular volume increases the glomerular filtration rate (GFR) and oxygen delivery and dilutes myoglobin and other renal tubular toxins. Immediately obtain intravenous access with a largebore catheter. Administer isotonic crystalloid 500 mL/h and then titrate to maintain a urine output of 200-300 mL/h.
http://emedicine.medscape.com/article/827738clinical#showall Maria Carmela L. Domocmat, RN,

MSN 3/5/2012 93
Because injured myocytes can sequester large volumes of extracellular fluid, crystalloid requirements may be surprisingly large.
http://emedicine.medscape.com/article/827738clinical#showall Maria Carmela L. Domocmat, RN,

MSN 3/5/2012 94
Assess ABCs and support as needed. Treat any underlying conditions, such as trauma, infection, or toxins. General recommendations for the treatment of rhabdomyolysis include
fluid resuscitation prevention of end-organ complications
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Patients with CK elevation in excess of 2-3 times the reference range, appropriate clinical history, and risk factors should be suspected of having rhabdomyolysis. Administer isotonic crystalloid 500 mL/h and titrate to maintain a urine output of 200-300 mL/h. Consider central venous pressures or Swan-Ganz catheterization in patients with cardiac or renal disease.
These invasive studies can assist in the assessment of the intravascular volume.
Repeat CK assay every 6-12 hours in order to determine peak CK level.

Acute renal failure develops in 30-40% of patients with rhabdomyolysis. Suggested mechanisms include
precipitation of myoglobin and uric acid crystals within renal tubules, decreased glomerular perfusion, and the nephrotoxic effect of ferrihemate (formed upon dissociation of myoglobin in the acidic environment of the renal parenchyma). I To prevent renal failure, many authorities advocate urine alkalinization, mannitol, and loop diuretics alkalinization, diuretics.
http://emedicine.medscape.com/article/827738Maria Carmela L. Domocmat, RN, clinical#showall MSN
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Urinary alkalinization
recommended for patients with rhabdomyolysis and CK levels in excess of 6000 IU/L. should be considered earlier in patients with acidemia, dehydration, or underlying renal disease. 0.5 isotonic sodium chloride solution with one ampule of sodium bicarbonate administered at 100 mL/h and titrated to a urine pH higher than 7. After establishing an adequate intravascular volume, mannitol may be administered to enhance renal perfusion. Loop diuretics may be used to enhance urinary output in oliguric patients, despite adequate intravascular volume.
http://emedicine.medscape.com/article/827738Maria Carmela L. Domocmat, RN, clinical#showall
MSN 3/5/2012 98
Treatment of hyperkalemia consists of

intravenous sodium bicarbonate, glucose, and insulin; oral or rectal sodium polystyrene sulfonate (Kayexalate); and hemodialysis. Administer intravenous calcium chloride for patients who are hemodynamically compromised and hyperkalemic.
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Hypocalcemia is noted early in the course of rhabdomyolysis and generally is not of clinical significance.
Calcium supplementation is not recommended.
Compartment syndrome
requires immediate orthopedic consultation for fasciotomy.
DIC
fresh frozen plasma, cryoprecipitate, and platelet transfusions.
Hyperuricemia and hyperphosphatemia

rarely are of clinical significance and rarely require treatment. http://emedicine.medscape.com/article/827738clinical#showall
Maria Carmela L. Domocmat, RN, MSN 10 0 3/5/2012
Medical therapy for rhabdomyolysis focuses on

restoring adequate intravascular volume using isotonic crystalloid. Adjunctive measures that may decrease the incidence of acute myoglobinuric renal failure include urinary alkalinization and osmotic and loop diuresis.
Maria Carmela L. Domocmat, RN, MSN 10 1
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Class Summary
Sodium bicarbonate is administered IV to alkalinize urine in patients with rhabdomyolysis. This may prevent toxicity caused by the presence of myoglobin in acidic urine and crystallization of uric acid.
(Neut Neut) Sodium bicarbonate (Neut)

Useful in alkalization of urine to prevent acute myoglobinuric renal failure. Titrate dose to increase pH to >7.
3/5/2012
Class Summary
These agents increase osmolarity of glomerular filtrate and induce diuresis. They hinder tubular reabsorption of water, causing sodium and chloride excretion to increase.
(Osmitrol Osmitrol) Mannitol (Osmitrol)

Alternative diuretic used when urine output is inadequate despite aggressive fluid therapy. Initially assess for adequate renal function in adults by administering a test dose of 200 mg/kg IV over 3-5 min. Should produce a urine flow of at least 30-50 mL/h over 2-3 h. In children, assess for adequate renal function by administering a test dose of 200 mg/kg IV over 3-5 min. It should produce a urine flow of at least 1 mL/h over 13 h. http://emedicine.medscape.com/article/827738clinical#showall
Class Summary
These agents elicit a loss of free water, increasing diuresis.
Furosemide (Lasix)
Increases water excretion by interfering with the chloride-binding cotransport system, resulting in inhibition of sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule. Individualize doses. Depending on response, administer at increments of 20-40 mg q6-8h until desired diuresis occurs. When treating infants, titrate with 1-mg/kg/dose increments until a satisfactory effect is achieved http://emedicine.medscape.com/article/827738clinical#showall
continued volume support and urinary alkalinization Obtain serial CK measurements to verify that values have peaked and are returning to reference range. Serial physical examinations and laboratory studies are indicated to monitor for
compartment syndrome Hyperkalemia acute oliguric or nonoliguric renal failure DIC

MSN 3/5/2012
10 5
In patients with no apparent precipitating factors for rhabdomyolysis

consider inherited disorders of carbohydrate or lipid metabolism and myopathies.

MSN 3/5/2012
10 6
Death from hyperkalemia or renal failure Compartment syndrome Disseminated intravascular coagulation (DIC) Hepatic insufficiency Hypovolemia (sequestration of plasma water within injured myocytes)
3/5/2012
Hyperkalemia (release of cellular potassium into the systemic circulation) Metabolic acidosis (release of cellular phosphate and sulfate) Acute tubular necrosis (ATN) Acute renal failure (nephrotoxic effects of liberated myocyte components)
3/5/2012
The prognosis depends on the underlying etiology and any existing comorbidities. Acute kidney failure occurs in many patients. Treatment soon after rhabdomyolysis begins will reduce the risk of chronic kidney damage. People with milder cases may return to normal activity within a few weeks to a month or more. However, some continue to have problems with fatigue and muscle pain. http://emedicine.medscape.com/article/827738clinical#showall
Advise patients with rhabdomyolysis caused by hyperthermia and/or inordinate exertion to exercise in moderation with careful attention to hydration and external methods of cooling. Advise patients with rhabdomyolysis related to ethanol, recreational drugs, or prescription medications to discontinue use of the offending agent and refer them to a rehabilitation program.
3/5/2012
Drink plenty of fluids after strenuous exercise to dilute the urine and flush the myoglobin out of the kidney. Proper hydration is also necessary after any condition or event that may involve damage to skeletal muscle.
3/5/2012
11 1
In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 114. http://www.nlm.nih.gov/medlineplus/ency/a rticle/000473.htm http://emedicine.medscape.com/article/827 738-clinical#showall
3/5/2012
11 2

Musculoskeletal Disorders Part 3 Muscular Disorders

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Musculoskeletal Disorders Part 3 Muscular Disorders

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Maria Carmela L.

Part Part Part Part

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

age of onset rate of progression, and pattern of inheritance

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

http://www.checkorphan.org/grid/iwishes/duchenne-musculardystrophy Maria Carmela L. Domocmat, RN, MSN 3/5/2012 19

http://trialx.com/curetalk/wpcontent/blogs.dir/7/files/2011/05/diseases/Muscular_Dystrophy_Duc henne-3.jpg Maria Carmela L. Domocmat, RN, MSN

http://www.ninds.nih.gov/disorders/md/md.htm Maria Carmela L. Domocmat, RN, MSN

http://www.ninds.nih.gov/disorders/md/md.htm Maria Carmela L. Domocmat, RN, MSN

Also known as Landouzy-Dejerine dystrophy Landouzyinvolves progressive muscle weakness involving:

http://www.websters-onlinedictionary.org/images/wiki/wikipedia/commons/thumb/e/e1/Autodominant.jpg/180pxMaria Carmela L. Domocmat, RN, Autodominant.jpg

Maria Carmela L. Domocmat, RN, MSN

The other major types of muscular dystrophy include:

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Signs in infants may include:

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

This form of muscular dystrophy usually begins in the muscles of the:

http://healthmedcare.com/muscular-dystrophy-types-symptoms-of-eachform/ Maria Carmela L. Domocmat, RN,

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Electromyography Ultrasonography Muscle biopsy

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

http://www.mdausa.org/ http://www.mayoclinic.com/health/muscular dystrophy/DS00200/DSECTION=treatmentsand-drugs

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

There are a three of types of muscle in the body, including:

Rhabdomyolysis occurs when there is damage to the skeletal muscle.

Maria Carmela L. Domocmat, RN, MSN

http://www.emedicinehealth.com/rhabdomyolysis/page2_ Maria Carmela L. Domocmat, RN, em.htm MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

Maria Carmela L. Domocmat, RN, MSN

When muscle is damaged

Maria Carmela L. Domocmat, RN, MSN