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Biomedical subjects

John B Ziegler

Publications and source records attributed to John B Ziegler.

11 recordsLinked to original sources
PubMed2008

Rituximab for lymphoproliferative disease prior to haematopoietic stem cell transplantation for X-linked severe combined immunodeficiency.

Lymphoproliferative disease (LPD) is a complication of congenital and acquired immunodeficiency states. There are a number of treatment options for LPD arising after haematopoietic stem cell or solid organ transplantation including reduction of immunosuppression, targeted therapies, such as the anti-CD20 monoclonal antibody, rituximab, and EBV specific cytotoxic lymphocytes. Treatment of LPD in children with congenital immunodeficiency syndromes remains unsatisfactory and is associated with a high mortality rate. We recently managed an infant found to have polymorphic LPD concurrent with X-linked severe combined immunodeficiency (SCID). Haematopoietic stem cell transplantation (HSCT) had to be deferred because of progressive LPD. Treatment with rituximab resulted in regression of the LPD following which the patient received a 5/6 HLA matched umbilical cord blood (UCB) transplant. The patient remains well 20 months following transplantation. Rituximab treatment may have a useful role in the control of LPD associated with congenital immunodeficiency prior to HSCT.

Antibodies, Monoclonal↗
PubMed2006

Mutations in the gene encoding the PML nuclear body protein Sp110 are associated with immunodeficiency and hepatic veno-occlusive disease.

We describe mutations in the PML nuclear body protein Sp110 in the syndrome veno-occlusive disease with immunodeficiency, an autosomal recessive disorder of severe hypogammaglobulinemia, combined T and B cell immunodeficiency, absent lymph node germinal centers, absent tissue plasma cells and hepatic veno-occlusive disease. This is the first report of the involvement of a nuclear body protein in a human primary immunodeficiency and of high-penetrance genetic mutations in hepatic veno-occlusive disease.

Female↗
PubMed2006

Protective immunity against hepatitis C virus infection.

There is increasing evidence that a small percentage of individuals exposed to the hepatitis C virus have the capacity to generate a strong cellular immune response against the virus and avoid persistent infection, and perhaps do so repeatedly after re-exposure. This article reviews the evidence that the responses identified in this unique group of individuals represent the protective immunity that will need to be elicited by hepatitis C virus vaccines.

Antibody Formation↗
PubMed2004

Would universal antenatal screening for HIV infection be cost-effective in a setting of very low prevalence? Modelling the data for Australia.

BACKGROUND: The economics of universal antenatal human immunodeficiency virus (HIV) screening should be explored if mother-to-child transmission of HIV occurs, the health-service infrastructure for universal screening exists, and optimal risk-reducing treatments can be supplied. METHODS: We evaluated a hypothetical cohort of the antenatal population of Australia during 2001-2002, to examine whether universal antenatal HIV screening is cost-effective in this setting. A quasi-societal perspective was adopted, secondary data sources were used, and sensitivity analyses were undertaken. Costs and benefits incurred in the future were discounted to their present value. RESULTS: The intervention would be cost-effective if the prevalence of undiagnosed HIV in the currently unscreened Australian antenatal population was >or=0.004372%. We predict 6.95 new diagnoses of HIV, 1.73 infections avoided, and 46.97 discounted-life-years gained. Applying favorable and unfavorable values for key variables suggests that the prevalence at which the intervention would be cost-effective is 0.0016%-0.0106%. CONCLUSIONS: Universal antenatal HIV screening would be cost-effective at a very low prevalence and would generate net benefits under many scenarios; accurate statistics on the true prevalence of HIV in the currently unscreened antenatal population are required.

Australia↗
PubMed2004

Phase 2 evaluation of parainfluenza type 3 cold passage mutant 45 live attenuated vaccine in healthy children 6-18 months old.

A phase 2 evaluation of live attenuated parainfluenza type 3 (PIV3)-cold passage mutant 45 (cp45) vaccine was conducted in 380 children 6-18 months old; 226 children (59%) were seronegative for PIV3. Of the 226 seronegative children, 114 received PIV3-cp45 vaccine, and 112 received placebo. No significant difference in the occurrence of adverse events (i.e., runny nose, cough, or temperature > or =38 degrees C) was noted during the 14 days after vaccination. There was no difference between groups in the occurrence of acute otitis media or serous otitis media. Paired serum samples were available for 109 of the seronegative vaccine recipients and for 110 of the seronegative placebo recipients; 84% of seronegative vaccine recipients developed a > or =4-fold increase in antibody titers. The geometric mean antibody titer after vaccination was 1 : 25 in the vaccine group and <1 : 4 in the placebo group. PIV3-cp45 vaccine was safe and immunogenic in seronegative children and should be evaluated for efficacy in a phase 3 field trial.

Acute Disease↗
PubMed2004

Chronic fatigue syndrome in adolescents: a follow-up study.

OBJECTIVES: To compare the frequency of persistent symptoms up to 8 years after illness onset in adolescents diagnosed as having chronic fatigue syndrome, idiopathic chronic fatigue, and unexplained fatigue for less than 6 months, and to determine if hospital admission is associated with outcome. DESIGN: A cohort study using questionnaire follow-up. SETTING: A tertiary referral hospital. PATIENTS: Consecutive adolescents referred for assessment of persistent fatigue were identified and retrospectively divided into 3 groups according to the diagnostic criteria for chronic fatigue syndrome and idiopathic chronic fatigue. INTERVENTION: A questionnaire was designed and administered by telephone at a mean of 4.57 years after the initial examination. MAIN OUTCOME MEASURE: The persistence of self-reported symptoms was compared with respect to patient group and admission. RESULTS: Outcome data were obtained for 34 (69%) of the 49 eligible subjects. Twenty-five percent of the chronic fatigue syndrome group showed near to complete improvement, 31% showed partial improvement, and 44% showed no improvement. The idiopathic chronic fatigue group had near to complete recovery in 50%, partial in 10%, and no improvement in 40%. Those with unexplained fatigue for less than 6 months had all recovered. There was no difference between the outcome of the subjects admitted to the hospital and those managed as outpatients. CONCLUSIONS: Adolescents with less than 6 months of fatigue have a good outcome. Unexplained fatigue lasting more than 6 months has a similar outcome regardless of the presence of minor criteria for chronic fatigue syndrome.

Adolescent↗
PubMed2003

Peanut allergy.

Explore the source record for details and available documents.

Arachis↗
PubMed2003

Effector HIV-specific cytotoxic T-lymphocyte activity in long-term nonprogressors: associations with viral replication and progression.

Ex vivo effector cytotoxic T-lymphocyte (CTL) activity was assessed in 27 members of the Australian Long-Term Nonprogressor cohort and correlated with genetic, virological, and immunological markers. The 27 individuals were antiretroviral naive with CD4(+) T-cell counts of >500 cells/ microl for more than 8 years after human immunodeficiency virus type 1 (HIV-1) infection. Effector CTL activity was determined using a standard ex vivo chromium release assay. Individuals with CTL activity (HIV-1 env(IIIB) or pol or gag) were then compared to those without CTL activity in relation to plasma HIV-1 RNA, ICD p24 antigen, beta(2)-microglobulin, CD4 and CD8 T-cell counts, CCR5 and CCR2b genotypes, and progression to CD4 <500 cells/microl or commencement of antiretroviral treatment. Of the 27 individuals examined, 19 had no detectable effector CTL activity. The eight individuals with detectable CTL activity had significantly higher plasma levels of HIV-1 RNA (P = 0.014), immune complex dissociated p24 antigen (P = 0.006), and beta(2)-microglobulin (P = 0.009). There was increased risk of progression within 4 years of study entry in individuals with detectable effector CTL activity, higher plasma levels of HIV-1 RNA, higher beta(2)-microglobulin levels, and higher immune complex dissociated p24 antigen levels at enrollment (P = 0.017, P = 0.004, P = 0.027, P = 0.008 respectively). Multivariate analysis demonstrated viral load remained the strongest predictor of disease progression within this group (P = 0.017). There were no significant associations between CTL response and chemokine receptor genotype. These findings demonstrate the importance of HIV replication in generating an effector CTL response and show that effector CTL activity may be an early predictor of progression in people with long-term asymptomatic HIV infection.

Antigen-Antibody Complex↗
PubMed2002

Tolerabilities of antiretrovirals in paediatric HIV infection.

Data on the efficacy and tolerability of antiretrovirals in children are limited as, in contrast to adult studies, large paediatric cohort studies are lacking. Thus, data pertaining to adults are often extrapolated to children despite the acknowledgement that children are not little adults. This review summarises information gathered from existing reports and focuses on the tolerabilities of antiretrovirals in children infected with HIV-1. The efficacy of antiretrovirals is not included in the scope of the discussion. Taste of antiretrovirals should be an important factor when considering the tolerability of antiretrovirals in children. However, antiretroviral options are often limited in young children as only some of the antiretrovirals are available as paediatric formulations. All antiretrovirals have been associated with toxicities in children, but in general, they are relatively well tolerated. The gastrointestinal system including hepatic system is most prone to being affected by these drugs. Skin rashes and hypersensitivity reactions are also associated with antiretroviral use, particularly with the non-nucleoside reverse transcriptase inhibitors. Mitochondrial toxicities that lead to impairment of liver function, pancreatic function and lactic acidosis are associated with most of the nucleoside analogues. Haematological toxicity is often a dose limiting adverse effect especially of the nucleoside analogues, in particular zidovudine. The protease inhibitors are associated with gastrointestinal intolerance (diarrhoea) and metabolic derangements that can lead to hypercholesterolaemia and hypertriglyceridaemia, which in turn and can lead to changes in body habitus. The renal system is also affected by several drugs, the most important of which is indinavir, which has been associated with renal stones and damage to the renal tubules. Fortunately, with lower incidence of major toxicity and with the range of drugs now available for paediatric use, toxicities are usually not a barrier to effect antiretroviral therapy in children.

Anti-HIV Agents↗
PubMed1990

The Faces Pain Scale for the self-assessment of the severity of pain experienced by children: development, initial validation, and preliminary investigation for ratio scale properties.

Altogether 553 children (195 first graders, mean age 6.8 years, and 358 third graders, mean age 8.7 years) participated in the development of a self-report measure to assess the intensity of children's pain. The first step was the derivation, from children's drawings of facial expressions of pain, of 5 sets of 7 schematic faces depicting changes in severity of expressed pain from no pain to the most pain possible. With the set of faces that achieved the highest agreement in pain ordering, additional studies were conducted to determine whether the set had the properties of a scale. In one study, children rank-ordered the faces on 2 occasions, separated by 1 week. All 7 faces were correctly ranked by 64% (retest 1 week later, 61%) of grade 1 children and by 86% (retest 89%) of grade 3 children. In a second study, the faces were presented in all possible paired combinations. All 7 faces were correctly placed by 62% (retest 86%) of the younger and by 75% (retest 71%) of the older subjects. A third study asked children to place faces along a scale: a procedure allowing a check on the equality of intervals. The fourth study checked on whether pain was acting as an underlying construct for ordering the faces in memory. We asked whether children perceived the set as a scale by asking if memory for an ordered set of faces was more accurate than for a random set. The final study checked, with 6-year-old children, the test-retest reliability of ratings for recalled experiences of pain. Overall, the faces pain scale incorporates conventions used by children, has achieved strong agreement in the rank ordering of pain, has indications that the intervals are close to equal, and is treated by children as a scale. The test-retest data suggest that it may prove to be a reliable index over time of self-reported pain.

Child↗