Porphyria cutanea tarda in human immunodeficiency virus-seropositive men: case report and literature review

Article Abstract:

Heme is the iron-carrying part of the hemoglobin molecule, present in red blood cells, which carries oxygen to the cells of the body. Porphyria cutanea tarda (PCT) is a disorder in which the synthesis of heme is impaired. Individuals with PCT become overly sensitive to light. Manifestations which occur in early stages of the disease are fragility of the skin, bruising, blistering, heavy pigmentation, and excessive body hair. Late manifestations include scarring, the formation of cysts containing keratin, and changes similar to scleroderma, where there is degeneration of the connective tissue of the skin, lungs and internal organs. A case study of a person infected with the human immunodeficiency virus (HIV) with PCT is reported, and a review of the 17 case studies of HIV-infected individuals with PCT is presented. In 71 percent of the cases, PCT was diagnosed before or at the same time as HIV infection. All of the patients had blisters of the back of the hands, and laboratory tests showed increased levels of porphyrins (iron-containing molecules) in their urine. Other symptoms present in some of the cases were abnormal liver function, heavy pigmentation, erosions, excessive hair growth and skin fragility. Symptoms rarely seen were polycythemia (an abnormal increase in red blood cells), increased iron storage, blistering and scleroderma. Treatment for HIV-infected individuals with PCT should include avoidance of exposure to the sun; elimination of agents that increase the severity of disease, such as alcohol, estrogen, iron and chlorinated hydrocarbons; and removal of some blood on a periodic basis. The cause of PCT is not certain, but it is felt that HIV is involved in the development of PCT in infected individuals. (Consumer Summary produced by Reliance Medical Information, Inc.)

Physiological aspects, HIV infection, HIV infections, Porphyria

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Low-dose multidrug chemotherapy plus Pneumocystis carinii pneumonia prophylaxis for HIV-related Kaposi's sarcoma

Article Abstract:

Kaposi's sarcoma was one of the very first indications of the emergence of the disease we now call AIDS. Ironically, most patients with Kaposi's sarcoma do not die of the tumor. The tumor responds well to chemotherapy, but the chemotherapy increases the likelihood of the opportunistic infections, to which AIDS patients are already so susceptible. In fact, the majority of patients with Kaposi's sarcoma die of Pneumocystis carinii (PC) pneumonia. To improve the survival of AIDS patients with Kaposi's sarcoma, a chemotherapeutic protocol was designed to reduce bone marrow toxicity and included a prophylactic treatment for PC pneumonia. Treatment with adriamycin and vinblastine or etoposide was alternated weekly with vincristine with or without bleomycin. All but one patient received inhalant pentamidine for PC pneumonia prophylaxis. (The remaining patient was already receiving Fansidar prophylaxis.) Of the 17 patients available for evaluation, a partial response of the sarcoma to chemotherapy was seen in 16 individuals, but no complete responses were observed. Within an average follow-up of 17 months, two cases of PC pneumonia were observed, as well six other opportunistic infections. The overall average survival was 12 months, and 8 of 14 deaths resulted from recurrent sarcoma. The chemotherapeutic protocol achieved the goal of reducing the likelihood of PC pneumonia, and produced a good response rate, but the recurrence rate of the tumor was high. Although the 12-month average survival rate is good and compares favorably with some other published reports, the results suggest that a greater benefit may be obtained by identifying more aggressive drug regimens, while protecting the bone marrow as much as possible. (Consumer Summary produced by Reliance Medical Information, Inc.)

Chemotherapy, Kaposi's sarcoma, Immunosuppression

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Synergistic anti-pneumocystis carinii effects of erythromycin and sulfisoxazole

Article Abstract:

Pneumocystis carinii pneumonia (PCP) is an opportunistic infection that often occurs in patients with AIDS, and causes high morbidity (illness) and mortality. A number of drug therapies have been developed that are effective in preventing PCP in AIDS patients. Some use a combination of drugs that have little or no effect alone, but are highly effective when administered together, thus providing a synergistic effect. This study examined a combination of erythromycin and sulfisoxazole treatment to prevent PCP in an animal model. Rats were given a drug to suppress their immune systems and increase their vulnerability to the development of PCP. The rats were grouped and either given no drug, azithromycin, clarithromycin, erythromycin, sulfisoxazole, or a combination of erythromycin and sulfisoxazole. None of the drugs alone had much effect in preventing PCP; 80 to 100 percent of the rats in each group developed PCP infection. When erythromycin and sulfisoxazole were given in combination, only 10 percent of the rats developed PCP. This drug combination has been safely used in humans for other diseases for a number of years. Drug models using animals have worked well for PCP studies. The results from this study indicate that the combination therapy of erythromycin and sulfisoxazole should be examined as a means of preventing PCP in patients with AIDS. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Erythromycin, Sulfisoxazole

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