Treatments for Adrenoleukodystrophy
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Book Excerpts: Treatment of Adrenoleukodystrophy
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Paraplegia:
Treatment
(In a Page: Signs and Symptoms)
- Paralysis or paraplegia is best managed by identifying and treating the underlying cause
–In cases of compressive lesions of the spinal cord, cauda equina, or nerve roots, surgical therapy is usually required
–Traumatic spinal cord injury often requires surgical stabilization; also, acute high-dose steroid treatment is effective in improving outcomes of traumatic myelopathy
-
Spinal dysraphism is often treated surgically
-
Infectious myelopathies: Antimicrobial agents
-
Multiple sclerosis: Acute exacerbations may be treated with steroids; prevent exacerbations with interferons, glatiramer acetate, and mitoxantrone
-
Guillain-Barré syndrome: Plasmapheresis or IVIG within 2 weeks of onset of symptoms
-
Physical therapy, assistive devices, orthotics, and wheelchairs may all be beneficial in improving the functional abilities of patients with paraplegia/paresis
» READ BOOK EXCERPT ONLINE »
Source: In a Page: Signs and Symptoms, 2004
Adrenal crisis:
Treatment (Tx)
(Professional Guide to Diseases (Eighth Edition))
Replacement of corticosteroids, I.V. fluids, potassium, insulin
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Adrenal hypofunction:
Treatment
(Professional Guide to Diseases (Eighth Edition))
For all patients with primary or secondary adrenal hypofunction, corticosteroid replacement, usually with cortisone or hydrocortisone (both of which also have a mineralocorticoid effect), is the primary treatment and must continue throughout life. Adrenal hypofunction may also necessitate treatment with I.V. desoxycorticosterone, a pure mineralocorticoid, or oral fludrocortisone, a synthetic mineralocorticoid; both prevent dangerous dehydration and hypotension.
Adrenal crisis requires prompt I.V. bolus administration of hydrocortisone. Later, doses are given I.M. or are diluted with dextrose in saline solution and given I.V. until the patient’s condition stabilizes.
With proper treatment, adrenal crisis usually subsides quickly; the patient’s blood pressure should stabilize, and water and sodium levels should return to normal. After the crisis, maintenance doses of hydrocortisone preserve physiologic stability.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Creutzfeldt-Jakob disease:
Treatment
(Professional Guide to Diseases (Eighth Edition))
There’s no cure for CJD, and its progress can’t be slowed. Palliative care is provided to make the patient comfortable and to ease symptoms. Medications may be needed to control aggressive behaviors. These include sedatives and antipsychotics.
The need to provide a safe environment, control aggressive or agitated behavior, and meet physiologic needs may require monitoring and assistance in the home or in an institutionalized setting. Family counseling may help in coping with the changes required for home care.
Behavior modification may be helpful, in some cases, for controlling unacceptable or dangerous behaviors. Reality orientation, with repeated reinforcement of environmental and other cues, may help reduce disorientation.
Legal advice may be appropriate early in the course of the disorder, to form advance directives, power of attorney, and other legal actions that may make it easier to make ethical decisions regarding the care of an individual with CJD.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Encephalitis:
Treatment (Tx)
(Professional Guide to Diseases (Eighth Edition))
Anticonvulsants, acyclovir (if viral), glucocorticoids, mannitol, furosemide, supportive care (mild analgesics, bed rest, seizure precautions)
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
West Nile encephalitis:
Treatment
(Professional Guide to Diseases (Eighth Edition))
There is no specific therapy utilized to treat West Nile encephalitis and no known cure. Treatment is generally aimed at controlling the specific symptoms. Supportive care, such as I.V. fluids, fever control, and respiratory support, is rendered when necessary.
There is no vaccine present to prevent the transmission of West Nile encephalitis. Research trials are underway to determine if ribavirin, an antiviral drug, may be helpful.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Adrenal hypofunction:
Treatment
(Handbook of Diseases)
For all patients with primary or secondary adrenal hypofunction, lifelong corticosteroid replacement, usually with cortisone or hydrocortisone (both of which also have a mineralocorticoid effect) is the primary treatment.
For patients with Addison’s disease, treatment with oral fludrocortisone, a synthetic mineralocorticoid, is necessary to prevent dangerous dehydration, hypotension, and electrolyte disturbances with hyponatremia and hyperkalemia. (See Avoiding adrenal crisis.)
For those with adrenal crisis, prompt I.V. bolus administration of 100 mg of hydrocortisone is key. Later, 50- to 100-mg doses are given I.M. or are diluted with dextrose in saline solution and given I.V. until the patient’s condition stabilizes; up to 300 mg/day of hydrocortisone and 3 to 5 L of I.V. saline solution are required during the acute stage of adrenal crisis.
With proper treatment, adrenal crisis usually subsides quickly; the patient’s blood pressure stabilizes, and water and sodium levels return to normal. After the crisis, maintenance doses of hydrocortisone preserve physiologic stability.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
Creutzfeldt-Jakob disease:
Treatment
(Handbook of Diseases)
There is no cure for CJD, and its progress can’t be slowed. Palliative care is provided to make the patient comfortable and to ease symptoms.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
Encephalitis:
Treatment
(Handbook of Diseases)
The antiviral agent acyclovir is effective only against herpes encephalitis. Treatment of all other forms of encephalitis is entirely supportive.
Drug therapy includes phenytoin or another anticonvulsant, usually given I.V.; glucocorticoids to reduce cerebral inflammation and edema; furosemide or mannitol to reduce cerebral swelling; sedatives for restlessness; and aspirin or acetaminophen to relieve headache and reduce fever.
Other supportive measures include adequate fluid and electrolyte intake to prevent dehydration and antibiotics for an associated infection such as pneumonia. Isolation is unnecessary.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
West Nile encephalitis:
Treatment
(Handbook of Diseases)
No specific therapy is used to treat West Nile encephalitis, and no known cure exists. Treatment is generally aimed at controlling the specific symptoms. Supportive care, such as intravenous fluids, fever control, and respiratory support, is rendered when necessary.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
Skin, bronze:
Patient counseling
(Signs & Symptoms: A 2-in-1 Reference for Nurses)
Encourage the patient to discuss his concerns about changes in body image. Encourage frequent rest periods if fatigue is a problem. A referral for nutritional counseling may be needed if the patient experiences weight loss, nausea, or vomiting.
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Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007
Muscle spasticity:
Emergency Actions
(Signs & Symptoms: A 2-in-1 Reference for Nurses)
Keep in mind that generalized spasticity and trismus in a patient with a recent skin puncture or laceration indicates tetanus. If you suspect this rare disorder, look for signs of respiratory distress. Provide ventilatory support, if necessary, and monitor the patient closely.
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Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007
Muscle spasticity [Muscle hypertonicity]:
Nursing considerations
(Nursing: Interpreting Signs and Symptoms)
▪ Prepare the patient for diagnostic tests, which may include electromyography, muscle biopsy, or intracranial or spinal magnetic resonance imaging or computed tomography.
▪ Administer pain medication and an antispasmodic, as ordered.
▪ Perform passive range-of-motion exercises, splinting, traction, and application of heat to help relieve spasms and prevent contractures.
▪ Maintain a calm, quiet environment to help relieve muscle spasms and prevent recurrence, and encourage bed rest.
▪ In cases of prolonged, uncontrollable muscle spasticity, as with spastic paralysis, prepare the patient for nerve blocks or surgical transection to provide permanent relief, as indicated.
Patient teaching
▪ Teach the patient to use assistive devices as needed.
▪ Help the patient to identify ways to maintain independence.
» READ BOOK EXCERPT ONLINE »
Source: Nursing: Interpreting Signs and Symptoms, 2007
Gait, spastic [Hemiplegic gait]:
Nursing considerations
(Nursing: Interpreting Signs and Symptoms)
▪ Provide the patient with daily exercise and active and passive ROM exercises.
▪ Refer the patient to a physical therapist for gait retraining and possible in-shoe splints or leg braces to maintain proper foot alignment for standing and walking.
▪ Assist the patient with ambulation.
Patient teaching
▪ Reinforce the importance of ambulating with assistance.
▪ Teach the patient to use a cane or a walker, as indicated.
» READ BOOK EXCERPT ONLINE »
Source: Nursing: Interpreting Signs and Symptoms, 2007
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