Chronic granulomatous disease presenting in a 69-year-old man

Article Abstract:

One of the front lines of defense against bacterial and fungal infections is the phagocyte. These phagocytes, white blood cells called granulocytes, ingest microorganisms which are then killed by powerful oxidizing radicals generated by enzymes. Some people suffer a rare genetic defect that causes a deficiency in one of these enzymes. As a result, the microorganisms cannot be killed by the phagocyte after they are ingested. These patients develop granulomatous masses of infected cells and suffer recurrent infections. In most cases, the genetic disorder, chronic granulomatous disease, is recognized in childhood. In the few cases of chronic granulomatous disease that have been diagnosed in adulthood, the patients have been found to have a lifelong history of recurrent infection. An unusual case is reported of a man who was 69 years old when he was first diagnosed with chronic granulomatous disease. Prior to the development of fever, anorexia, headache, and malaise, the patient had been healthy with no history of recurrent infection. The majority of patients with chronic granulomatous disease have a genetic mutation in the gene for a protein weighing 91,000 Daltons. This 91 kiloDalton protein is called phagocyte oxidase and is abbreviated gp91-phox. This protein serves as a subunit in the enzyme cytochrome b. Since the gp91-phox gene is located on the X chromosome, the disorder is sex-linked; males are primarily affected and females are carriers. In most cases, the cytochrome b is virtually undetectable. In the present patient, the amounts of cytochrome b appeared to be normal. However, further investigation revealed that although the amount of the enzyme was normal, its function was strikingly reduced. It is not known why this patient was able to live for 69 years without experiencing the serious infections suffered by most patients with chronic granulomatous disease. The explanation probably does not lie within this particular patient's unusual mutation. The patient had a grandson who died at the age of five from infection with Pseudomonas cepacia. The boy's mother was found to be a carrier of the gene, and although it can not be confirmed, it seems very likely that this child suffered fatal consequences of the same X-linked gene carried by his grandfather. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Abnormalities, Familial diseases, Phagocytes, Chronic granulomatous disease

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An acquired chemotactic defect in neutrophils from patients receiving interleukin-2 immunotherapy

Article Abstract:

Interleukin-2 is a naturally occurring protein molecule that is produced and released by a specific class of white blood cells (activated T lymphocytes). This molecule is biologically quite active and has been shown to be important in enhancing cellular immunity, which can produce regression of dispersed (metastatic) cancer. The ability of interleukin-2 to induce tumor regression has been extensively researched both in animal models and in a large multi-institutional trial. A significant complication of interleukin-2 therapy, however, is generalized bacterial infection, or bacteremia. The incidence of bacteremia ranged between 19 to 38.1 percent at the various institutions that participated in the trial. Often a central venous catheter, a tube placed through the skin into the venous system, is left in place throughout therapy for easy access to the vascular system and for blood sampling. This indwelling catheter is considered a likely site of the bacteremia, and 20 out of 107 cases of infection following interleukin-2 treatment have been linked to infections along the vascular catheter. This evidence led researchers to suspect that treatment with interleukin-2 was affecting the ability of neutrophils, a class of white blood cells involved with prevention of infection, from functioning normally. To further explore this possibility, a sample of neutrophils was drawn from 31 patients with metastatic cancer who would undergo treatment with interleukin-2, and again after treatment. These cells were assayed for various physiologic capabilities, including their ability to migrate toward a foreign stimulus, capacity to release granules, and ability to engulf and destroy foreign material. Following treatment with interleukin-2, neutrophils were found to be acutely but reversibly defective in their ability to respond to foreign protein and presumably infection. The authors believe that the increased morbidity that is seen after interleukin-2 treatment is directly related to the observed impairment of neutrophils. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Complications and side effects, Physiological aspects, Drug therapy, Cancer, Neutrophils, Bacteremia, Intravenous catheterization, Interleukin-2

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Crossing the species barrier-one small step to man, one giant leap to mankind

Article Abstract:

Many infectious diseases cross the species barrier for which one reason is that humans come into contact with a microorganism that is already capable of causing human infection. The other reason could be an alteration that occurs in the spectrum of species for which the organism is pathogenic.

Science & research, Research, Influence, Communicable diseases, Microorganisms, Pathogenic microorganisms

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