Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two pediatric patients with life-threatening Epstein-Barr virus infections were studied immunologically and treated with acyclovir [9-(2-hydroxyethoxymethyl) guanine]. The patient with chronic active Epstein-Barr virus infection who experienced massive hepatosplenomegaly, pancytopenia, and failure to thrive demonstrated abnormalities of T and B lymphocytes. A second patient, with the X-linked lymphoproliferative syndrome, experienced a rapidly fatal course of acute Epstein-Barr virus infection which typifies this yet undefined immunodeficiency to Epstein-Barr virus. In each case, objective evidence for clinical improvement or antiviral effect of acyclovir treatment was not apparent. Abnormally productive Epstein-Barr virus infections did not appear to play a major role in the clinical syndromes observed. Current studies are focused on treatment of immunologically normal patients with early complicated Epstein-Barr virus infection.
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PMID:Treatment of life-threatening Epstein-Barr virus infection with acyclovir. 628 18

In this report, we present a 5 months old male baby, who suffered from watery diarrhea since 4 days old. From then on, he had been admitted 3 times in 3 different hospitals but the symptoms still bothered him off and on. During the days of hospitalization, sepsis with positive blood culture of Klebsiella was noted. The patient expired at 5 months of age. The T cell count was 20% active T was 0. Delayed hypersensitivity skin tests including Candida (10 X), PHA (10 micrograms), PHA (1 microgram), SK/SD (50 units) were negative. The granulocyte function study showed normal. Immunoglobulin analysis revealed IgG: 1320 mg%, IgA: 120 mg%, IgM: 100 mg%. Agenesis of thymus, failure of lymphoid differentiation and abnormal lymphoid architecture with absence of germinal centers were noted at autopsy. Combined immunodeficiency with normal immunoglobulins (Nezelof syndrome) is a disease of primary immunodeficiency characterized by recurrent infections, failure to thrive, lymphopenia, diminished lymphoid tissue, abnormal structure or agenesis of the thymus, and presence of normal or increased levels of one or more of the major immunoglobulin classes, but with impaired antibody synthesis. Since its original description by Nezelof and associates in 1964, it has been reported on the subsequent occasion. In this report, we present our one experience and review the clinical and laboratory data in 33 reported cases.
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PMID:Report of a case of Nezelof syndrome. 744 23

A rapid method for determination of zidovudine resistance was developed and results were correlated with clinical outcome in human immunodeficiency virus (HIV)-infected children. The zidovudine susceptibilities of HIV-1 isolates from 34 children were determined through a direct quantitative peripheral blood lymphocyte assay and compared with results of the AIDS Clinical Trials Group resistance assay. Patients' peripheral blood lymphocytes were 5-fold diluted and cocultured with donor lymphocytes and varying concentrations of zidovudine. Isolates were defined as sensitive if inhibited by < or = 1.0 microM zidovudine and resistant at > 1.0 microM. Children (n = 21) with zidovudine-resistant virus had greater evidence of disease progression than did those with zidovudine-sensitive virus (n = 11) as demonstrated by failure to thrive (57% vs. 9%, P = .01) and opportunistic infections (48% vs. 0, P = .006). This assay may be useful as a screening tool for development of clinically relevant zidovudine resistance.
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PMID:Correlation of clinical progression in human immunodeficiency virus-infected children with in vitro zidovudine resistance measured by a direct quantitative peripheral blood lymphocyte assay. 762 78

The goal of this study was to describe seroreversion (SR) in a cohort of human immunodeficiency virus-exposed but uninfected infants. Groups of patients who seroreverted very early or late were examined for salient clinical and immunologic characteristics of the mother or infant. The mean time (+/- s.d.) to seroreversion by enzyme-linked immunoabsorbent assay (ELISA) was 50.1 +/- 14.8 weeks, or 11.6 months (n = 84); the range of times to antibody loss by ELISA was 17.9 to 82.0 weeks. The mean time to seroreversion by Western blot was 68.3 +/- 12.6 weeks, or 15.8 months (n = 51), with a range of 44.9 to 94.1 weeks. Initial anti-human immunodeficiency virus titer as measured by cord blood ELISA optical density (OD) was found to relate significantly to mean time to seroreversion. No relationship to time to seroreversion was demonstrated for gestational age, maternal or neonatal serum immunoglobulin concentrations, maternal CD4 cell counts, maternal alcohol consumption, infantile diarrhea or failure to thrive. The lengthy time to seroreversion seen here demonstrates the 1994 revised Centers for Disease Control and Prevention definition of human immunodeficiency virus infection (based on seropositivity by both ELISA and confirmatory tests persisting beyond 18 months of age) to be accurate in our population. We recommend Western blot testing be used as confirmation for positive ELISAs only after 18 months of age.
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PMID:Seroreversion in human immunodeficiency virus-exposed but uninfected infants. 763 14

The transmission, diagnosis, and clinical manifestations of human immunodeficiency virus (HIV) infection in children up to 13 years of age are reviewed, and maintenance and prophylactic drug therapies for these patients are discussed. HIV can be transmitted from mother to infant in utero, during delivery, or through breast milk. Perinatal transmission accounts for almost 90% of all pediatric HIV infections. HIV infection can be diagnosed with HIV culturing, polymerase chain reaction testing, the enzyme-linked immunosorbent assay, the Western blot antibody assay, or the p24 core-antigen assay. Testing should begin as soon as possible after the at-risk child reaches one month of age. CD4+ lymphocyte counts are also used in diagnosis and monitoring. The median age at diagnosis of AIDS in children with perinatally acquired HIV infection is 12-24 months. Among the many possible clinical features are Pneumocystis carinii pneumonia (PCP), cytomegalovirus infection, failure to thrive, encephalopathy, recurrent bacterial infection, thrush, lymphoid interstitial pneumonitis, lymphadenopathy, pancreatis, hepatitis, anemia, and thrombocytopenia. Zidovudine is considered the drug of choice for initial therapy in HIV-infected children and is indicated for asymptomatic infection, early symptomatic disease, and advanced disease. However, new research is questioning the role of zidovudine monotherapy. Didanosine is the only agent with FDA-approved labeling for use as second-line therapy in children who do not respond to or become resistant to zidovudine. Agents under investigation for pediatric use are zalcitabine, stavudine, lamivudine, and nevirapine. Drug combinations, such as zidovudine plus didanosine, are also being examined. Zidovudine appears to reduce the rate of maternal transmission of HIV. Agents used prophylactically against PCP in children are trimethoprim-sulfamethoxazole, dapsone, and inhaled or i.v. pentamidine. HIV-infected children should also received prophylaxis against recurrent bacterial infections. The standard pediatric immunization schedule is used, but inactivated injectable poliovirus vaccine must be given instead of the live oral vaccine. Zidovudine remains the first-line agent for treating HIV infection in children. Alternatives are available for those who do not respond to zidovudine.
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PMID:Human immunodeficiency virus infection in children. 764 Oct 35

Infants vertically infected with human immunodeficiency virus type 1 (HIV-1) often manifest profoundly deficient growth with failure to thrive. The pathologic mechanisms that produce growth failure associated with pediatric HIV infection are not clear. Transgenic mice homozygous for a gag/pol deletion mutant of the infectious provirus pNL4-3 have been found to manifest a similar growth failure pattern. To explore the influence of HIV-1 on fetal growth and maternal-fetal interactions, we examined intrauterine growth of transgenic and nontransgenic mice and evaluated the consequence of embryo transfer into normal and heterozygous transgenic mothers. Mice homozygous for the HIV transgene had normal intrauterine and birth weights but uniformly displayed severe growth retardation postnatally. Transgene expression was prominent in transgenic fetuses and their placentas and in uteri of transgenic mothers, as determined by Northern analysis. Although embryo transfer did not affect intrauterine growth, the pregnancy rate in transgenic mothers was markedly lower than in nontransgenic controls. In both fetal and neonatal tissues, transgene expression was significantly greater in homozygous animals when compared with heterozygotes, but the difference was magnified postnatally. These results suggest that HIV gene expression affected both mother and neonate. In the mother, expression of the HIV-1 transgene reduced postfertilization pregnancy rate. Once the animal was pregnant, however, the effects of transgene expression on the homozygous fetus were overcome in utero, possibly by the contribution of maternal factors or by inhibition of HIV-1 gene expression by a fetal or maternal factor(s). In the neonate, HIV-1 transgene expression increased dramatically in homozygotes and was associated with profound growth failure.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Maternal-fetal interactions affect growth of human immunodeficiency virus type 1 transgenic mice. 770 Jul 34

We present the pulmonary findings in 36 autopsies of children affected by the acquired immunodeficiency syndrome (AIDS). Twenty-three patients were male and 13 were female, ranging in age between 3 days and 13 years. Twenty children had human immunodeficiency virus (HIV)-positive parents or parents who were at high risk of exposure (intravenous drug abusers and prostitutes), five had a history of transfusion, and one had a history of renal transplantation and blood transfusion. Clinically, the patients presented with recurrent infections, failure to thrive, hepatosplenomegaly, fever, cough, and/or hemoptysis. Histologically, specific infectious processes were the most common finding (75% of cases), with Pneumocystis carinii pneumonia being the most prevalent type of infection, followed by bacterial pneumonia. Neoplastic conditions and lymphoid interstitial pneumonia were less frequent (approximately 10% of cases). In addition, in approximately 10% of the cases the pulmonary findings were non-specific (ie, pulmonary edema and atelectasis) and probably unrelated to HIV infection. Our findings suggest that specific infectious conditions constitute the most common type of pulmonary pathology in children with AIDS. However, because there is a small percentage of children with nonspecific findings, a transbronchial biopsy is important for proper evaluation before institution of therapy.
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PMID:The spectrum of pathological changes in the lung in children with the acquired immunodeficiency syndrome: an autopsy study of 36 cases. 808 62

Decreasing susceptibility to zidovudine (ZDV) has been described in persons infected with human immunodeficiency virus (HIV) type 1 who are receiving ZDV therapy. However, the clinical significance of decreased ZDV susceptibility remains unclear. In this study, HIV isolates obtained from children with symptomatic HIV infection treated with ZDV were monitored for their susceptibility to the antiretroviral agent and correlated with disease progression. Using a peripheral blood mononuclear cell-based assay to measure ZDV susceptibility, we evaluated HIV isolates from 19 children (mean age, 6.8 years; range, 5 months to 12 years) during ZDV therapy for susceptibility to ZDV. Of the 19 children studied, 10 continued to have susceptible HIV strains during ZDV treatment, and 9 acquired resistant viruses. All eight isolates from children without previous exposure to ZDV were initially susceptible. After a median of 11 months of ZDV therapy, three (38%) of these eight children had acquired resistant HIV strains (defined as ZDV susceptibility > or = 10 mumol/L). Children with resistant strains had worse clinical outcomes than children whose viruses remained susceptible, as determined by a 50% decline in absolute CD4+ cell counts after 1 year of treatment, failure to thrive, or death. Children with resistant viruses who were given alternative antiretroviral therapy frequently responded to the new treatment with improved growth and stabilization of their HIV-related disease. These data suggest that, in HIV-infected children, ZDV-resistant HIV strains are associated with diminished drug efficacy and more rapid disease progression.
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PMID:Development and significance of zidovudine resistance in children infected with human immunodeficiency virus. 810 May 79

The microsporidian Enterocytozoon bieneusi has been recognized as an important cause of chronic diarrhea in severely immunodeficient adults infected with human immunodeficiency virus (HIV). We report the first case of intestinal E. bieneusi infection in a child. The 9-year-old boy with connatal HIV infection presented with failure to thrive, chronic diarrhea, and intermittent abdominal pain. His CD4 lymphocyte count was 0.05 x 10(9)/L and dropped to 0.01 x 10(9)/L. No HIV-associated opportunistic infection other than oral hairy leukoplakia and oral candidiasis had been found before microsporidia were detected. Treatment of microsporidiosis with albendazole was of no benefit. During follow-up, the boy also developed intestinal cryptosporidiosis. Evaluation of chronic diarrhea in severely immunodeficient HIV-infected children should include examination for intestinal microsporidia. We recommend the use of a new coprodiagnostic technique that allows detection of microsporidial spores in stool specimens. Furthermore, consideration of dual or even multiple parasitic infections in the differential diagnosis of chronic diarrhea may have both important clinical and epidemiological implications.
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PMID:Intestinal coinfection with Enterocytozoon bieneusi and Cryptosporidium in a human immunodeficiency virus-infected child with chronic diarrhea. 821 93

Low birth weight, prematurity, and intra-uterine growth retardation (IUGR) are major determinants of child survival. Therefore, it is important to assess excess mortality due to human immunodeficiency virus (HIV) infection in populations where low birth weight is common. A prospective study was conducted on a total of 1385 children born to 679 HIV-seropositive and 687 seronegative women in urban Malawi. Children were regularly examined and tested for HIV. The mortality rate of children of HIV-seropositive mothers was substantiality higher (223/1000 at 12 months, 317/1000 at 24 months, and 360/1000 at 30 months, p 0.0001) than that of children of seronegative mothers (68/1000 at 12 months, 106/1000 at 24 months, and 118/1000 at 30 months). The incidence of prematurity and IUGR was also higher in infants of HIV-seropositive mothers than in infants of seronegative mothers (12.5% versus 3.8%, p 0.001 for premature and 7.7% versus 4.4%, p = 0.02 for IUGR infants). The mother-to-infant HIV-1 transmission rate was 35.1%. The overall incidence of low birth weight was 14.1%, but the incidence was 20.1% among children of seropositive mothers and 8.3% among those of seronegative mothers (p 0.001). After 12 months of age, HIV-infected children showed the highest mortality; however, uninfected children of HIV-seropositive and children of HIV-seronegative mothers had similar mortality. The mean birth weight of HIV-infected and uninfected children was not significantly different. In HIV-infected children the most frequent causes of death were diarrhea, pneumonia, and failure to thrive. Less common risk factors for child mortality included active maternal syphilis and cervicitis/vaginitis. A possible enrolment bias could have resulted in lower mortality estimates among babies of HIV-seronegative mothers. To decrease childhood mortality, a combination of interventions such as treatment of sexually transmitted infections during pregnancy and measures to reduce mother-to-infant transmission should be adopted.
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PMID:The effect of human immunodeficiency virus infection on birthweight, and infant and child mortality in urban Malawi. 855 35


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