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How does the mode of inheritance of a genetic condition influence families? A study of guilt, blame, stigma, and understanding of inheritance and reproductive risks in families with X-linked and autosomal recessive diseases.

PURPOSE: While the mode of inheritance of a genetic condition has long been considered to have not only medical, but also psychosocial consequences for families, this supposition has never been tested. METHODS: We surveyed 112 members of 51 families (59% response) with chronic granulomatous disease to determine the influence of mode of inheritance on parents', siblings', and patients' (1) knowledge of inheritance and reproductive risk; (2) concern about risk to future family-members; (3) feelings of guilt and blame; and (4) feelings of stigmatization. Ninety-six members of 51 families (49% response) with Duchenne/Becker muscular dystrophy and spinal muscular atrophy types II/III were also studied. RESULTS: X-linked families had better understanding of inheritance (P < 0.001) and reproductive risks (P < 0.01). X-linked mothers worried more about risks to future generations; other autosomal-recessive family members were as worried. X-linked mothers were more likely to feel guilty (P < 0.01) and blame themselves (P < 0.001). X-linked fathers blamed their child's mother (P < 0.05) and X-linked mothers felt more blamed by the father (P < 0.01). X-linked family-members were more likely to consider being a carrier stigmatizing (P < 0.05). CONCLUSION: When providing genetic counseling, attention should be given to guilt and blame in X-linked families and understanding reproductive risks in autosomal recessive families.

Attitude↗

Staff affirmations and client criticisms: staff and client perceptions of quality of care at sexually transmitted disease clinics.

OBJECTIVES: Public health sexually transmitted disease (STD) clinics play a central role in STD control efforts. This study addressed the perceived quality of client services at two STD clinics in Alabama. GOAL: The goal of this study was to evaluate and develop tools for assessment of client satisfaction at STD clinics. STUDY: Four focus groups of staff (N=16) and clients (N=15) at the clinics were conducted between June and November 2003. RESULTS: Staff and client assessments of respect, privacy protections, and friendliness at the clinics differed in substantial ways. Clients were concerned with visibility, being stigmatized or disrespected, and about a breach of confidentiality. Concerns about being stigmatized focused mainly on nonclinical staff. The staff did not perceive these concerns to be warranted. Quality of medical care was rated high by both groups. CONCLUSION: Staff-client interactions are an important element of STD care, and "front desk" actions set the tone at public STD clinics. Nonclinicians should receive training to improve body language and other behaviors to alleviate the stigma concerns of clients who are anxious, fearful, and sometimes distrustful of the health system.

Adult↗

STIG1 controls exudate secretion in the pistil of petunia and tobacco.

The lipid-rich, sticky exudate covering the stigma of solanaceous species such as tobacco (Nicotiana tabacum) and petunia (Petunia hybrida) contains several proteins, of which only some have been characterized to date. Proteome analysis of the stigmatic exudate in both species revealed the presence of a cysteine-rich, slightly acidic 12-kD protein called stigma-specific protein 1 (STIG1). In both tobacco and petunia, Stig1 is highly expressed at the mRNA level in very young and developing flowers, whereas hardly any Stig1 transcript is detected in mature flowers. This expression pattern coincides with the differentiation of the secretory zone, forming the intercellular spaces into which the exudate is secreted. Using reverse genetics, we show that STIG1 is involved in the secretion and merging of exudate lipids in the intercellular spaces of the secretory zone and that plants lacking STIG1 show an accelerated deposition of exudate onto the stigmatic surface. This phenotype was observed both in a petunia knockout mutant and in tobacco transgenic plants. We therefore propose that STIG1 plays a role in the temporal regulation of the essential exudate secretion onto the stigma.

Amino Acid Sequence↗

Temporal and Spatial Expression of 1-Aminocyclopropane-1-Carboxylate Oxidase mRNA following Pollination of Immature and Mature Petunia Flowers.

Pollination of petunia (Petunia hybrida) flowers induces a rapid increase in ethylene production by styles, which subsequently leads to increased ethylene production by the corolla, inducing senescence. We have investigated the temporal and spatial expression of 1-aminocyclopropane-1-carboxylate (ACC) oxidase transcripts in petunia styles in an attempt to elucidate its role in increased ethylene biosynthesis following pollination. Previously, we reported that the development of petunia flowers was associated with increased ACC oxidase mRNA localized specifically in the stigmatic regions of the style (X. Tang, A.M.T. Gomes, A. Bhatia, W.R. Woodson [1994] Plant Cell 6: 1227-1239). The rapid increase in ethylene production by styles within the 1st h following pollination was correlated with the expression of ACC oxidase mRNAs during development. Pollination of petunia flowers prior to anthesis and the expression of ACC oxidase mRNA led to a substantial increase in ethylene production, but this was delayed by several hours in comparison with flowers at anthesis. This delayed increase in ethylene production by pollinated styles from immature flowers was associated with an increased ACC oxidase transcript abundance. Treatment with the ethylene action inhibitor 2,5-norbornadiene did not affect the early increase in ethylene production or the expression of ACC oxidase mRNAs. No differences in the rate of pollen germination or tube growth were detected when applied to stigmas from immature or mature flowers, indicating that the delay in ethylene production was likely the result of limited ACC oxidase activity. Localization of ACC oxidase mRNAs following pollination by in situ hybridization revealed an abundance of transcripts in transmitting tract tissue within 4 h of pollination of both immature and mature styles, in contrast to their localization in stigmatic cells during development.

Journal Article↗

PCP-A1, a defensin-like Brassica pollen coat protein that binds the S locus glycoprotein, is the product of gametophytic gene expression.

Self-incompatibility (SI) in Brassica species is controlled by a single polymorphic locus (S) with multiple specificities. Two stigmatically expressed genes that have been cloned from this region encode the S locus glycoprotein (SLG) and S receptor kinase (SRK). Both appear to be essential for the operation of SI. It is believed that rejection of incompatible pollen grains is effected by recognition events between an as yet unidentified S locus-encoded pollen coating-borne protein and the SLG/SRK. We previously identified a small pollen coat protein PCP7 (renamed here PCP-A1, for pollen coat protein, class A, 1) that binds with high affinity to SLGs irrespective of S genotype. Here, we report the cloning of PCP-A1 from Brassica oleracea and demonstrate that it is unlinked to the S locus. In situ localization of PCP-A1 transcripts revealed that they accumulate specifically in pollen at the late binucleate/trinucleate stage of development rather than in the tapetum, which previously was taken to be the principal source of the pollen coat. PCP-A1 is characterized by the presence of a structurally important motif consisting of eight cysteine residues shared by the plant defensins. Based on the presence of this motif and other data, homology modeling has been used to produce a putative structure for PCP-A1. Protein-protein interaction analyses demonstrate that SLG exists in monomeric and dimeric forms, both of which bind PCP-A1. Evidence is also presented for the existence of putative membrane-associated PCP-A1 binding proteins in stigmatic tissue.

Amino Acid Sequence↗

Activity of an S Locus Gene Promoter in Pistils and Anthers of Transgenic Brassica.

The pollen-stigma interaction of self-incompatibility in crucifers is correlated with glycoproteins localized in the cell wall of the stigmatic papillae that are encoded by the S locus glycoprotein (SLG) gene. When fused to the [beta]-glucuronidase (GUS) reporter gene, the 5[prime] upstream regulatory region of SLG directed high level expression in the papillae of transgenic Brassica plants. Histochemical and fluorometric assays revealed that, in addition to its primary site of expression in the stigmatic papillae, the SLG-GUS fusion was also expressed in the transmitting tissue of stigma, style, and ovary, and in anthers. This conclusion was verified by the detection of transgene-encoded GUS transcripts and endogenous SLG-homologous transcripts by RNA gel blot analysis. Significantly, in anthers, the SLG promoter was active not only sporophytically in the nurse cells of the tapetum, but also in the haploid microspores. Because self-incompatibility systems exhibiting sporophytic control of pollen phenotype are thought to have evolved from systems with gametophytic control, we suggest that sporophytic control was acquired without loss of gametophytic function.

Journal Article↗

Astigmatic electron diffraction imaging: a novel mode for structure determination.

In a conventional transmission electron microscope, stigmators are used to correct for the effects of axial astigmatism in the diffraction lens. It seems feasible that these same stigmators could also be used to form a series of 'astigmatic' diffraction patterns. It is shown how this series of diffraction patterns could then be used to perform exit-surface wavefunction reconstruction. This has the advantage that the diffraction patterns are not resolution limited by the objective aperture as are images when performing exit-surface wavefunction reconstruction from a focal series. A scheme for carrying out phase reconstruction from a series of astigmatic diffraction patterns in an electron microscope is presented.

Journal Article↗

The development and consequences of stereotype consciousness in middle childhood.

Two studies including an ethnically diverse sample of 202 children ages 6 to 10 examined the development and consequences of children's awareness of others' stereotypes ("stereotype consciousness"). Study 1 revealed that between ages 6 and 10, children's ability to infer an individual's stereotype increases dramatically. Children's awareness of broadly held stereotypes also increases with age, and children from academically stigmatized ethnic groups (African Americans and Latinos) are at all ages more likely be aware of broadly held stereotypes than children from academically nonstigmatized ethnic groups (Whites and Asians). Study 2 revealed that among children from stigmatized ethnic groups aware of broadly held stereotypes, on 1 of 2 challenging cognitive tasks and self-reported effort, diagnostic testing conditions led to stereotype threat effects.

Child↗

An emotion-based model of risk perception and stigma susceptibility: cognitive appraisals of emotion, affective reactivity, worldviews, and risk perceptions in the generation of technological stigma.

A study (N=198) was conducted to examine hypotheses derived from an emotion-based model of stigma responses to radiation sources. A model of stigma susceptibility is proposed in which affective reactions and cognitive worldviews activate predispositions to appraise and experience events in systematic ways that result in the generation of negative emotion, risk perceptions, and stigma responses. Results of structural equation modeling supported the hypotheses. Radiation sources that scored higher on a measure of stigma were included in the analyses (i.e., nuclear power plants, radioactive waste from nuclear power plants, radiation from nuclear weapons testing). Individual differences in negative reactivity and worldviews were associated with the strength of emotional appraisals that were associated, in turn, with negative emotion toward stigmatized radiation sources. As hypothesized, the model fit better with perceived risk as a function of negative emotion rather than vice versa. Finally, a measure of stigma was associated with negative emotion and, to a lesser extent, with risk perceptions. Risk communication about stigmatized objects may benefit from a more complete understanding of how affective and emotional reactions are constructed and the routes through which they affect responses and behaviors.

Affect↗

Audio-computerized self-interviewing versus face-to-face interviewing for research data collection at drug abuse treatment programs.

AIMS: To assess audio computer-assisted self-interviewing (A-CASI) as a mode of data collection with injecting drug users (IDUs) entering two drug treatment programs in New York City. A-CASI has been found to increase reporting of sensitive items among a variety of population subgroups. DESIGN: A field test of A-CASI data collection conducted within an ongoing cross-sectional study of drug use and HIV risk behaviors among IDUs entering drug treatment. Participants were assigned without bias to either a computer-assisted interviewer-administered personal interview (CAPI) or to a mixed CAPI/A-CASI interview. In the latter, 'sensitive' portions (dealing with stigmatized behavior) of the questionnaire were self-administered through A-CASI, while the remaining portions were interviewer-administered. SETTING: The Detoxification Program and the Methadone Maintenance Treatment Program (MMTP) at Beth Israel Medical Center in New York City. PARTICIPANTS: Seven hundred and eighty-three IDUs entering drug treatment. MEASUREMENTS: Odds ratios and adjusted odds ratios (controlling for demographic differences) for comparison of A-CASI versus CAPI responses on 111 sensitive questions. FINDINGS: Twenty-three statistically significant differences (each at P < 0.05), all in the direction of more reporting of the behaviors by the A-CASI group. Forty-one per cent of A-CASI participants said they would prefer any subsequent interviews to be fully A-CASI and 46% said they would prefer the mixed CAPI/A-CASI mode. CONCLUSIONS: A-CASI was associated with greater reporting of potentially stigmatized drug, sex and HIV risk behaviors on a moderate number of questions. Moreover, a large majority of participants who used A-CASI would like to be assigned to this method of data collection in future interviews.

Adult↗

AIDS-related Kaposi's sarcoma: epidemiological, diagnostic, treatment and control aspects in sub-Saharan Africa.

Until the 1980s, little attention had been accorded to endemic Kaposi's sarcoma (KS), a neoplasm noted in several parts of Southern Europe and the African continent but with relatively slow progression, except in children and young adults. Furthermore, therapeutic approaches based on surgery, radiation and topical treatment were of limited efficacy, mostly used to overcome the disabling and stigmatizing effects of the disease. With the emergence of the HIV/AIDS epidemic, and the profound impact of KS on AIDS-related mortality, the pathogenesis of KS has been better studied, and the realisation that a virus (KS-associated Herpesvirus or Human Herpesvirus 8, or KSHV/HHV-8), combined with immunosuppression and cytokine-induced growth, was responsible for the development of this disease has led to novel therapeutic approaches. These are unfortunately still highly toxic, require careful monitoring, and are expensive, thus limiting their use in most parts of Africa. However, the use of highly active antiretroviral therapy (HAART), which has led to a considerable decline in KS incidence in populations of industrialized countries, constitutes the best hope for the control of this stigmatizing and lethal disease in Africa. Trials comparing different regimens of antiretroviral drugs in combination with systemic chemotherapeutic agents are urgently needed.

Acquired Immunodeficiency Syndrome↗

Harm-doing and victim's racial or orthopedic stigma as determinants of helping behavior.

Three experiments tested a prediction derived from an ambivalence theory of behavior toward the stigmatized. The prediction was that unintentional harm-doers would be more likely to make restitution by doing a favor for a stigmatized victim than for a nonstigmatized one. In Experiment 1 it was upheld for black versus white victims. In Experiment 2, where the victim of harm-doing was either physically handicapped or normal, the prediction was upheld only among older subjects, following the post hoc introduction of an age variable. Experiment 3 also used a physical disability variable. The data were consistent with the prediction, although the adequacy of the harm-doing manipulation was not checked empirically. On the whole, these results are seen as complementing previous findings of amplified post-harm-doing denigration of black and disabled victims. An assumption that the relationship between harm-doing and altruism was mediated by guilt arousal received only fragmentary support. That is, self-reports of guilt were highest in the harm-doing black confederate condition in Experiment 1, and self-reports of negative affect were highest in the harm-doing/wheelchair confederate condition in Experiment 3, but there were no significant relationships between individual differences in guilt or negative affect on the one hand, and amount of helping, on the other.

Adolescent↗

Perceived risk, stigma, and potential economic impacts of a high-level nuclear waste repository in Nevada.

This study investigates the potential impacts of the proposed nuclear waste repository at Yucca Mountain, Nevada, upon tourism, retirement and job-related migration, and business development in Las Vegas and the state. Adverse impacts may be expected to result from perceptions of risk, stigmatization, and socially amplified reactions to "unfortunate events" associated with the repository (major and minor accidents, discoveries of radiation releases, evidence of mismanagement, attempts to sabotage or disrupt the facility, etc.). The conceptual underpinnings of risk perception, stigmatization, and social amplification are discussed and empirical data are presented to demonstrate how nuclear images associated with Las Vegas and the State of Nevada might trigger adverse economic effects. The possibility that intense negative imagery associated with the repository may cause significant harm to Nevada's economy can no longer be ignored by serious attempts to assess the risks and impacts of this unique facility. The behavioral processes described here appear relevant as well to the social impact assessment of any proposed facility that produces, uses, transports, or disposes of hazardous materials.

Humans↗

Stigma in HIV-positive women.

PURPOSE: To present a metasynthesis of qualitative findings on stigma in HIV-positive women. METHOD: Metasummary and metasynthesis techniques were used to integrate findings on stigma in 93 reports of qualitative studies conducted between 1991 and 2002 with a total of 1,780 women, mostly from minority groups. FINDINGS: Both perceived and enacted stigma were pervasive in the lives of HIV-positive women. HIV-related stigma was intensified in women because they were women. Stigma management largely involved efforts to control information in the service of preserving social relations and maintaining moral identity. DISCUSSION: This metasynthesis reprises and clarifies the connections between recurring themes in primary quantitative studies and metastudies of HIV-positive people and of stigmatizing diseases and conditions. This work also shows how affected people's location on key axes of difference can both facilitate and complicate efforts to manage stigma. CONCLUSIONS: HIV-positive women experience stigmatization in relationships with others. HIV-related stigma is as much a reflection of these others as it is central to the experience of HIV-positive people themselves. Even those not infected with HIV are still affected by it and are thus appropriate targets for interventions to reduce its negative effects.

Adaptation, Psychological↗

Ethical aspects of neonatal screening for sickle cell disease in Western European countries.

UNLABELLED: Sickle cell disease raises some important ethical questions regarding neonatal screening in Western European countries such as France, England or Belgium, which have already introduced either universal or selective screening. Such screening is aimed at benefiting children affected with major sickle cell syndrome. It also detects heterozygous babies and, in doing so, heterozygous parents. The latter information, which is ignored most of the time, risks making parents feel guilty and can raise fears of stigmatization. Whether it would change their future reproductive decisions requires further studies, for cultural and religious reasons may have a strong negative influence on the request for prenatal diagnosis. Disclosure of the child's illness may oblige the family to remain in the country they have emigrated to because it offers the best chance of treatment. As a result, links between the family and its original community are modified. Parents must more or less sever links with their family in Africa and try to trust and adapt to the public health services in Europe. CONCLUSION: Neonatal screening of sickle cell disease is highly ethical in facilitating the prevention of the early death of affected children. It also detects heterozygous parents and offers at-risk couples the possibility to perform a prenatal diagnosis during the next pregnancy. Adequate counselling must consider the risk of stigmatization that carrier status represents, especially for women in many cultural beliefs, and the numerous cultural and religious reasons which limit parental uptake for prenatal diagnosis. Disclosure of their child's illness may oblige immigrant families to stay in Europe, where free and adapted healthcare is available.

Anemia, Sickle Cell↗

The male determinant of self-incompatibility in Brassica.

In the S locus-controlled self-incompatibility system of Brassica, recognition of self-related pollen at the surface of stigma epidermal cells leads to inhibition of pollen tube development. The female (stigmatic) determinant of this recognition reaction is a polymorphic transmembrane receptor protein kinase encoded at the S locus. Another highly polymorphic, anther-expressed gene, SCR, also encoded at the S locus, fulfills the requirements for the hypothesized pollen determinant. Loss-of-function and gain-of-function studies prove that the SCR gene product is necessary and sufficient for determining pollen self-incompatibility specificity, possibly by acting as a ligand for the stigmatic receptor.

Alleles↗

Perceived and measured stigma among workers with serious mental illness.

OBJECTIVE: This research analyzed the extent to which self-reports of job-related discrimination by persons with serious mental illness are associated with econometric measures of discrimination. METHODS: Data were from the 1994-1995 National Health Interview Survey-Disability Supplement. Data for workers with mood, psychotic, or anxiety disorders (N=1,139) were compared with data for those without such disorders (N=66,341). The main outcome measures were self-reports of wages and stigmatizing experiences in the workplace. RESULTS: After the analyses controlled for functional limitations and job characteristics, no significant difference in mean wages was found between workers with serious mental illness who did not report experiencing stigma and those with no mental illness. In contrast, for all types of mental disorders examined, mean wages for workers with serious mental illness who reported experiencing stigma were significantly lower than mean wages for those with no mental illness. CONCLUSIONS: Workers' self-reports of stigmatizing experiences in the labor market appear to be consistent with econometric measures of the effect of stigma on wages, suggesting that workers know when they are being discriminated against.

Adolescent↗

Sex differences in opinion towards mental illness of secondary school students in Hong Kong.

Sex differences in social attitudes have been well documented. Women hold more positive attitudes toward mental illness than men do. This paper reports on the effect of sex differences in a study of secondary school students' opinions about mental illness in Hong Kong. A total of 2,223 secondary school students, drawn by random sample, completed a 45-item questionnaire on Opinion about Mental Illness in Chinese Community (OMICC) with a six-point Likert Scale. Individual items with weak correlations were eliminated, leaving 33 items for analysis (Cronbach's Alpha = .866). Using factor analysis six factors were identified. These include: Benevolence, Separatism, Stereotyping, Restrictiveness, Pessimistic Prediction and Stigmatization. Results showed that girls scored higher regarding benevolence. Boys were found to have more stereotyping, restrictive, pessimistic and stigmatizing attitudes towards mental illness.

Adolescent↗