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The aim of treatment is to help the child lead as normal a life as possible. The type of treatment depends on the organ systems involved.
To combat electrolyte losses in sweat, salt foods generously and, in hot weather, administer sodium supplements.
To offset pancreatic enzyme deficiencies, give oral pancreatic enzymes with meals and snacks, as ordered. Maintain a diet that's low in fat, but high in protein and calories, and provide supplements of water-miscible, fat-soluble vitamins (A, D, E, and K).
Management of pulmonary dysfunction includes chest physiotherapy, postural drainage, and breathing exercises several times daily to aid removal of secretions from lungs. Antihistamines are contraindicated because they have a drying effect on mucous membranes, making expectoration of mucus difficult or impossible. Aerosol therapy includes intermittent nebulizer treatments before postural drainage to loosen secretions.
Dornase alfa or DNase (recombinant human deoxyribonuclease), genetically engineered pulmonary enzymes given by aerosol nebulizer, helps thin airway mucus, improving lung function and reducing the risk of pulmonary infection.
Treatment of pulmonary infection requires:
❑ broad-spectrum antimicrobials
❑ oxygen therapy as needed
❑ loosening and removal of mucopurulent secretions, using an intermittent nebulizer and postural drainage to relieve obstruction. Use of a mist tent is controversial because mist particles may become trapped in the esophagus and stomach and never even reach the lungs.
Lung transplantation may be considered in some cases. Genetic research is ongoing, with researchers hoping to cure cystic fibrosis by artificially inserting a “healthy” gene into a person through gene therapy. The gene would be inserted by using an intranasal form.Research on correcting the disorder before birth is promising.
Source: Professional Guide to Diseases (Eighth Edition), 2005
Treatment is designed to remove or alleviate the underlying cause of cirrhosis or fibrosis, prevent further liver damage, and prevent or treat complications. The patient may benefit from a high-calorie and moderate- to high-protein diet, but developing hepatic encephalopathy mandates restricted protein intake. In addition, sodium is usually restricted to 200 to 500 mg/day and fluids to 1 to 1½ qt (1 to 1.5 L)/day.
If the patient’s condition continues to deteriorate, he may need tube feedings or total parenteral nutrition. He may also need supplemental vitamins — A, B complex, D, and K — to compensate for the liver’s inability to store them and vitamin B12, folic acid, and thiamine for deficiency anemia. Rest, moderate exercise, and avoidance of exposure to infections and toxic agents are essential.
Drug therapy requires special caution because the cirrhotic liver can’t detoxify harmful substances efficiently. When absolutely necessary, vasopressin may be prescribed for esophageal varices, and diuretics may be given for edema. However, diuretics require careful monitoring because fluid and electrolyte imbalance may precipitate hepatic encephalopathy. Encephalopathy is treated with lactulose. Antibiotics are used to decrease intestinal bacteria and reduce ammonia production, which causes encephalopathy. Coagulopathy may be treated with blood products or vitamin K.
Low-protein diets are controversial. They aid in managing acute hepatic encephalopathy but are rarely necessary in chronic conditions because of the underlying protein-calorie malnutrition.
Paracentesis and infusions of salt-poor albumin, in addition to fluid and salt restriction, may alleviate ascites. Surgical procedures include treatment of varices by upper endoscopy with banding or sclerosis, splenectomy, esophagogastric resection, and splenorenal or portacaval anastomosis to relieve portal hypertension. (See Portal hypertension and esophageal varices, page 758, and Circulation in portal hypertension, page 759.)
Source: Professional Guide to Diseases (Eighth Edition), 2005
Although it can’t change the pathology of IPF, oxygen therapy can prevent the problems related to dyspnea and tissue hypoxia in the early stages of the disease process. The patient may require little or no supplemental oxygen while at rest initially, but he’ll need more as the disease progresses and during exertion.
No known cure exists. Corticosteroids and cytotoxic drugs may be given to suppress inflammation but are usually unsuccessful. Recently, interferon-gamma-1B has shown some promise in treating the disease.
Lung transplantation may be successful for younger, otherwise healthy individuals.
Source: Professional Guide to Diseases (Eighth Edition), 2005
The aim of treatment is to help the child lead as normal a life as possible. Specific treatment depends on the organ systems involved.
❑ To combat sweat electrolyte losses, treatment includes generous salting of foods and, during hot weather, administration of sodium supplements.
❑ To offset pancreatic enzyme deficiencies, treatment includes oral pancreatic enzymes with meals and snacks. The child’s diet should be low in fat but high in protein and calories, and it should include supplements of water-miscible, fat-soluble vitamins (A, D, E, and K). ❑ Management of pulmonary dysfunction includes chest physiotherapy, postural drainage, and breathing exercises several times daily to aid removal of secretions from the lungs; a flutter valve may be used in some cases. Antihistamines are contraindicated; they have a drying effect on mucous membranes, making expectoration of mucus difficult or impossible.
Aerosol therapy includes intermittent nebulizer treatments before postural drainage to loosen secretions. Dornase alfa, a genetically engineered pulmonary enzyme given by aerosol nebulizer, helps thin airway mucus, improving lung function and reducing the risk of pulmonary infection.
A patient with pulmonary infection will need mucopurulent secretions loosened and removed, using an intermittent nebulizer and postural drainage to relieve obstruction. Use of a mist tent is controversial because mist particles may become trapped in the esophagus and stomach and never even reach the lungs. Broad-spectrum antimicrobials help combat infection. Oxygen therapy is used as needed.
Recently, some patients have undergone lung transplantation to reduce the effects of the disease. Also, clinical trials of aerosol gene therapy show promise in reducing pulmonary symptoms.
Source: Handbook of Diseases, 2003
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