Prognosis of chronic granulomatous disease

Article Abstract:

Chronic granulomatous disease is an inherited disorder (with autosomal recessive inheritance) caused by the inability of phagocytes, an immune cell, to kill ingested microorganisms. It is characterized by inflammation of the lymph nodes, development of abscesses, inflammation of the skin, enlarged organs, and inflammation of the lungs, which leads to chronic pulmonary damage. The syndrome is not directly fatal and usually affects males. It can be diagnosed by a specific chemical test that indicates the number of neutrophils, a type of white blood cell. A defective gene renders phagocytes incapable of killing invading microorganisms because of abnormalities in the immune cell's metabolism. Although no cure for this syndrome exists, patients with chronic granulomatous disease are given antibiotics to prevent and treat infections. This disease can be detected before birth by analyzing fetal blood at 18 to 20 weeks' gestation. Current studies are focused on producing a cure, improving agents that may reduce symptoms of the disease, and developing more efficient screening techniques. The outcomes of 28 cases of chronic granulomatous disease were reviewed. Nine patients died due to infections. Fifty percent of the patients survived to 30 years of age and older. Patients who developed symptoms in infancy had a worse prognosis than patients who developed symptoms at the end of their first year of life. The severity of the disease varied and long-term survival of patients with this syndrome was better than suggested by earlier studies. The early onset of the disease may indicate a poor outcome, and invasive aspergillosis (mold-related infection) is a severe complication. The effectiveness of interferon gamma, an antiviral and antitumor protein, is being assessed as a treatment for chronic granulomatous disease. In addition, the authors recommend that prenatal screening for this disease should be offered to affected families. (Consumer Summary produced by Reliance Medical Information, Inc.)

Testing, Complications and side effects, Physiological aspects, Prognosis, Genetic aspects, Genetic disorders, Phagocytes, Chronic granulomatous disease

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Effective bronchodilator treatment by a simple spacer device for wheezy premature infants

Article Abstract:

Premature infants often have respiratory problems, which may persist for up to 10 years. Following the neonatal period, persistent wheezing or coughing may be twice as frequent among preterm infants as among full term infants. However, the effectiveness of bronchodilator medications (which improve lung function) has been questioned in preterm infants. Other researchers have found that the method of administration affects the effectiveness of the drug. Metered dose inhalers can not be used in infants; vaporizers are expensive and difficult to use appropriately. Ten young children under the age of two, who suffered from chronic respiratory symptoms, were treated with a bronchodilator drug applied through a homemade inhaling device. Compared to placebo treatments, the children had a 65 percent decrease in respiratory symptoms, and lung capacity increased by 32 percent. This study suggests that bronchodilators, administered with simple inhalation devices, may provide effective treatment for respiratory problems in young children. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Drug therapy, Dosage and administration, Bronchodilator agents, Pediatric respiratory diseases

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