Postdelivery head bleeding in hemophilic neonates: causes and management

Article Abstract:

Hemophilia is a genetic disorder affecting only men born to mothers carrying the defective gene. The disease is a bleeding disorder caused by a deficiency of clotting factors. Although prenatal diagnosis can identify affected male fetuses, many women do not elect pregnancy termination in such cases. Bleeding in the brain is a leading cause of death among hemophiliac infants. To examine whether the delivery process itself influences head bleeding, five hemophiliac infants were studied. The delivery records revealed that three infants had a traumatic delivery which required the use of forceps (in two patients) or vacuum extraction (one patient) to remove the fetus. Although the fetus diagnosed with hemophilia in advance was delivered vaginally, bleeding still occurred. There was a delay in diagnosis in three patients. One infant with a family history of hemophilia was not tested prior to circumcision. In two cases an infection was suspected, while bleeding from circumcision was overlooked. In most cases, if hemophilia had been detected a day or so earlier, more attention could have been directed to potential bleeding complications in the brain. One mother who was adopted did not have her family health history. One infant died at seven days of life, after a decrease was observed in factor VIII, one of the necessary clotting factors, when bleeding from a circumcision was followed by bleeding in the brain. Clotting studies should be performed on all pregnant women with a positive family history of bleeding disorders. Pregnant carriers of the gene responsible for hemophilia should undergo prenatal diagnosis. The least traumatic delivery possible should be utilized. Although bleeding in premature infants is not unusual, in a full-term infant it is suspect. Treatment of hemophilia includes fresh-frozen plasma (the portion of whole blood that remains after the blood cells are removed), which contains factors necessary for clotting. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Usage, Diagnosis, Blood clotting disorders, Blood coagulation disorders, Pregnancy, Prenatal diagnosis, Hematologic diseases, Blood diseases in pregnancy

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Pregnancies in human immunodeficiency virus-infected sex partners of hemophilic men

Article Abstract:

Hemophiliacs are at considerable risk for harboring and transmitting the human immunodeficiency virus (HIV). The National Hemophilia Foundation and the Public Health Service have recommended that hemophiliac men, who have tested positive for HIV or who have not been tested, refrain from unprotected sexual intercourse. It is also recommended that their partners not become pregnant until more is known about the transmission of the virus. Despite these warnings, there are many pregnancies among sex partners of hemophiliac men who are HIV-positive. In a study of 24 completed pregnancies, it was determined that 37.5 percent of the pregnant women learned of their partners' HIV-positivity and received counseling prior to the fifth month of pregnancy, when abortion was an option; at least nine women knew they were HIV-positive prior to their fifth month of pregnancy. Despite recommendations to the contrary, the pregnancies were carried to term; 37 percent of these pregnancies had been planned. Thirty percent of the children born were HIV-positive. Breast milk was considered an important source of HIV transmission. Three infants contracted pneumonia and two died. Two children had HIV-related symptoms of lymphadenopathy (lymph node disease) and idiopathic thrombocytopenic purpura (abnormal decrease in blood platelets). These findings are thought to represent the minimal level of HIV transmission, since they were based solely on clinical symptoms; there is increasing evidence that infected children, unlike infected adults, may test negative. This study demonstrates that infants of HIV-infected women are at extremely high risk for HIV infection, even if the infected mother is healthy, and does not engage in high risk activities such as sexual promiscuity or intravenous drug use. (Consumer Summary produced by Reliance Medical Information, Inc.)

Sexual behavior, HIV (Viruses), HIV, Disease transmission, AIDS (Disease), AIDS (Disease) in children, Pediatric AIDS (Disease)

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Trimethoprim-sulfamethoxazole oral desensitization in hemophiliacs infected with human immunodeficiency virus with a history of hypersensitivity reactions

Article Abstract:

Patients who have human immunodeficiency virus (HIV) infection are at risk for developing serious opportunistic infections, especially Pneumocystis carinii pneumonia (PCP). To prevent this complication, many HIV patients are treated prophylactically with antibiotics such as pentamidine or a combination of trimethoprim and sulfamethoxazole. However, patients with HIV may have an increased incidence of hypersensitivity to such drugs. To allow use of the trimethoprim-sulfamethoxazole protocol, oral desensitization was attempted in seven patients with hemophilia who had previously exhibited hypersensitivity to these two drugs. In this procedure, increasing dosages of the drugs were given every eight hours, starting with a dilution that caused no hypersensitivity reaction. The procedure continued as long as only mild skin rashes, but not systemic symptoms, resulted. All patients were successfully desensitized, with only two developing rashes that were treated with antihistamines. Subsequently, all but one patient successfully received prophylactic treatment with trimethoprim-sulfamethoxazole for five to seven months. One patient who missed several doses developed hives, and had to discontinue treatment. Repeat desensitization failed in this patient, and he had to use pentamidine. The results suggest that oral desensitization can be used with trimethoprim and sulfamethoxazole for HIV-infected patients with drug hypersensitivity, although further study is needed to substantiate this. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Prevention, Complications and side effects, HIV infection, HIV infections, Pneumocystis carinii pneumonia, Co-trimoxazole, Trimethoprim-sulfamethoxazole (Drug combination), Allergy desensitization

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