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Sample records for acquired immunodeficiency syndrome

  1. AIDS: acquired immunodeficiency syndrome *

    PubMed Central

    Gilmore, N.J.; Beaulieu, R.; Steben, M.; Laverdière, M.

    1992-01-01

    Acquired immunodeficiency syndrome, or AIDS, is a new illness that occurs in previously healthy individuals. It is characterized by immunodeficiency, opportunistic infections and unusual malignant diseases. Life-threatening single or multiple infections with viruses, mycobacteria, fungi or protozoa are common. A rare neoplasm, Kaposi's sarcoma, has developed in approximately one third of patients with AIDS. More than 800 cases of AIDS have been reported in North America, over 24 of them in Canada. The majority of patients are male homosexuals, although AIDS has also developed in abusers of intravenously administered drugs, Haitian immigrants, individuals with hemophilia, recipients of blood transfusions, prostitutes, and infants, spouses and partners of patients with AIDS. The cause of AIDS is unknown, but the features are consistent with an infectious process. Early diagnosis can be difficult owing to the nonspecific symptoms and signs of the infections and malignant diseases. Therefore, vigilance by physicians is of the utmost importance. PMID:1544049

  2. Acquired immunodeficiency syndrome: neuroradiologic findings.

    PubMed

    Kelly, W M; Brant-Zawadzki, M

    1983-11-01

    Central nervous system complications depicted by CT in ten patients with acquired immunodeficiency syndrome are described. Three patients had multifocal intra-axial enhancing lesions representing atypical brain abscesses (two with toxoplasmosis, one with candidiasis). A fourth patient with multifocal "ring" lesions whose biopsy was interpreted as suggestive of toxoplasmosis responded poorly to treatment. Following his death three months later of Pneumocystis carinii pneumonia, autopsy revealed primary intracerebral immunoblastic lymphoma. One patient had Kaposi sarcoma involving the right frontal lobe (seen as an enhancing mass on the CT scan). CT findings in the remaining five patients revealed mild to moderate enlargement of cerebrospinal fluid spaces (including ventricles and basal cisternae) as a result of cryptococcal meningitis in three patients and "aseptic" meningitis in two. The two patients in whom early biopsy confirmed toxoplasmosis responded well to anti-infective therapy, resulting in dramatic clinical recoveries. PMID:6622693

  3. [Acquired immunodeficiency syndrome in pediatric patients].

    PubMed

    Molina Moguel, J L; Ruiz Illezcas, R; Forsbach Sánchez, S; Carreño Alvarez, S; Picco Díaz, I

    1990-12-01

    The object of this study was to determine how many of the patients treated at the Pediatric Odontology Clinic, a branch of the Maxillo-Facial Surgery Service at the Veinte de Noviembre Regional Hospital, ISSSTE, are VIH-positive of show serious manifestations of Acquired Immuno-Deficiency Syndrome (AIDS). For such purpose, 100 pediatric patients suffering from different systemic or local diseases were evaluated, the most common being hematological alterations. Results evidenced the presence of VIH in the blood of five of the pediatric subjects, all suffering from Hemophilia. PMID:2132469

  4. The acquired immunodeficiency syndrome in gay men.

    PubMed

    Jaffe, H W; Hardy, A M; Morgan, W M; Darrow, W W

    1985-11-01

    The acquired immunodeficiency syndrome (AIDS) is a major health problem for gay men in the United States. About three fourths of all reported cases have occurred in this population, and the number is projected to double in the next year. In Manhattan and San Francisco, AIDS is now the leading cause of premature mortality in men aged 25 to 44 years who have never married. In a sample of a cohort of gay men enrolled in a San Francisco clinic, 2.7% of the men had the syndrome and 26% had related conditions in 1984. Antibody to human T-lymphotropic virus, type III/lymphadenopathy-associated virus was found in sera from 67% of the men, including 58% of asymptomatic men. Behavioral factors associated with an increased risk of AIDS include large numbers of sexual partners, receptive anal intercourse, and "fisting." The adoption of safer lifestyles is currently the basis of attempts to control the syndrome in gay men. PMID:2996396

  5. Acquired immunodeficiency syndrome: Ga-67 citrate imaging

    SciTech Connect

    Woolfenden, J.M.; Carrasquillo, J.A.; Larson, S.M.; Simmons, J.T.; Masur, H.; Smith, P.D.; Shelhamer, J.H.; Ognibene, F.P.

    1987-02-01

    All gallium-67 citrate scans obtained in patients with acquired immunodeficiency syndrome (AIDS) at the Clinical Center, National Institutes of Health (Bethesda, Md.) were retrospectively analyzed and correlated with the results of bronchoscopy, chest radiography, and endoscopy. There were 164 scans of 95 patients. Twenty scans were from patients with Pneumocystis carinii pneumonia; 19 were abnormal, for a sensitivity of 95%. Ga-67 uptake tended to be less in patients receiving therapy for P. carinii pneumonia. Chest radiographs were normal at least initially in three patients with abnormal scans and P. carinii pneumonia. Unusually prominent colonic activity was associated with infection in some patients. No lesions of Kaposi sarcoma showed tracer uptake. Gallium scanning is useful for detecting P. carinii pneumonia and other opportunistic infections in patients with AIDS, but it is not useful for localizing Kaposi sarcoma.

  6. Gastrointestinal Manifestations of the Acquired Immunodeficiency Syndrome

    PubMed Central

    Rodgers, Vance D.; Kagnoff, Martin F.

    1987-01-01

    In addition to abnormalities in systemic immune function, patients with the acquired immunodeficiency syndrome (AIDS) and the pre-AIDS syndromes have significant abnormalities in the distribution of T-cell subsets in the intestinal tract. Such immune deficits predispose such patients to opportunistic infections and tumors, many of which involve the gastrointestinal tract. For example, Candida albicans often causes stomatitis and esophagitis. Intestinal infections with parasites (Cryptosporidium, Isospora belli, Microsporidia) or bacteria (Mycobacterium avium-intracellulare) are associated with severe diarrhea and malabsorption, whereas viruses like cytomegalovirus and herpes simplex virus cause mucosal ulcerations. Clinically debilitating chronic diarrhea develops in many AIDS patients for which no clear cause can be identified. Enteric pathogens like Salmonella and Campylobacter can be associated with bacteremias. Kaposi's sarcoma and lymphoma involving the intestinal tract are now well-recognized complications of AIDS. Although AIDS is not associated with a pathognomonic liver lesion, opportunistic infections and Kaposi's sarcoma or lymphoma may involve the liver. ImagesFigure 3.Figure 4.Figure 5.Figure 6.Figure 7. PMID:3825111

  7. The acquired immunodeficiency syndrome: an ultrastructural study.

    PubMed

    Sidhu, G S; Stahl, R E; el-Sadr, W; Cassai, N D; Forrester, E M; Zolla-Pazner, S

    1985-04-01

    Blood and a variety of tissues from 97 patients with the acquired immunodeficiency syndrome (AIDS) and 25 with the AIDS prodrome were studied ultrastructurally. Tubuloreticular structures (TRS) were found in 85 per cent of the patients with AIDS and in 92 per cent of those with the prodrome. Test tube and ring-shaped forms (TRF), found in 41 per cent of the patients with AIDS and in 8 per cent of those with the prodrome, increased with disease progression. Among the patients with AIDS, as the number of sites examined per case increased, the incidence of TRS and TRF tended to approach 100 per cent, suggesting that they are present in all patients with AIDS. Other changes seen frequently were immunologic capping of blood lymphocytes, intramitochondrial iron in blood reticulocytes and marrow normoblasts, megakaryocytic immaturity and platelet phagocytosis, collections of membranous rings in hepatocytic cytoplasm, suggestive of non-A, non-B hepatitis, and proliferations and engorgement of hepatic Ito cells with lipid. The data suggest that TRS and TRF can be used as diagnostic and prognostic markers. PMID:3872253

  8. Subject Control of the Literature of Acquired Immunodeficiency Syndrome (AIDS).

    ERIC Educational Resources Information Center

    Bierbaum, Esther Green; And Others

    1992-01-01

    Describes a study that analyzed the Medical Subject Headings (MeSH) terms used to index the literature of Acquired Immunodeficiency Syndrome (AIDS). Subject access to the AIDSLINE database developed by the National Library of Medicine (NLM) is examined, and changes in subject headings that reflect the growth of the field are analyzed. (12…

  9. Acquired immunodeficiency syndrome associated with blood-product transfusions

    SciTech Connect

    Jett, J.R.; Kuritsky, J.N.; Katzmann, J.A.; Homburger, H.A.

    1983-11-01

    A 53-year-old white man had fever, malaise, and dyspnea on exertion. His chest roentgenogram was normal, but pulmonary function tests showed impaired diffusion capacity and a gallium scan showed marked uptake in the lungs. Results of an open-lung biopsy documented Pneumocystis carinii pneumonia. Immunologic test results were consistent with the acquired immunodeficiency syndrome. The patient denied having homosexual contact or using intravenous drugs. Twenty-nine months before the diagnosis of pneumocystis pneumonia was made, the patient had had 16 transfusions of whole blood, platelets, and fresh-frozen plasma during coronary artery bypass surgery at another medical center. This patient is not a member of any currently recognized high-risk group and is believed to have contracted the acquired immunodeficiency syndrome from blood and blood-product transfusions.

  10. Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome in Older Adults.

    PubMed

    Scott, Jake; Goetz, Matthew Bidwell

    2016-08-01

    Improved survival with combination antiretroviral therapy has led to a dramatic increase in the number of human immunodeficiency virus (HIV)-infected individuals 50 years of age or older such that by 2020 more than 50% of HIV-infected persons in the United States will be above this age. Recent studies confirm that antiretroviral therapy should be offered to all HIV-infected patients regardless of age, symptoms, CD4+ cell count, or HIV viral load. However, when compared with HIV-uninfected populations, even with suppression of measurable HIV replication, older individuals are at greater risk for cardiovascular disease, malignancies, liver disease, and other comorbidities. PMID:27394024

  11. Autoimmunity and dysmetabolism of human acquired immunodeficiency syndrome.

    PubMed

    Huang, Yan-Mei; Hong, Xue-Zhi; Xu, Jia-Hua; Luo, Jiang-Xi; Mo, Han-You; Zhao, Hai-Lu

    2016-06-01

    Acquired immunodeficiency syndrome (AIDS) remains ill-defined by lists of symptoms, infections, tumors, and disorders in metabolism and immunity. Low CD4 cell count, severe loss of body weight, pneumocystis pneumonia, and Kaposi's sarcoma are the major disease indicators. Lines of evidence indicate that patients living with AIDS have both immunodeficiency and autoimmunity. Immunodeficiency is attributed to deficits in the skin- and mucosa-defined innate immunity, CD4 T cells and regulatory T cells, presumably relating human immunodeficiency virus (HIV) infection. The autoimmunity in AIDS is evident by: (1) overproduction of autoantibodies, (2) impaired response of CD4 cells and CD8 cells, (3) failure of clinical trials of HIV vaccines, and (4) therapeutic benefits of immunosuppression following solid organ transplantation and bone marrow transplantation in patients at risk of AIDS. Autoantibodies are generated in response to antigens such as debris and molecules de novo released from dead cells, infectious agents, and catabolic events. Disturbances in metabolic homeostasis occur at the interface of immunodeficiency and autoimmunity in the development of AIDS. Optimal treatments favor therapeutics targeting on the regulation of metabolism to restore immune homeostasis. PMID:26676359

  12. Experience with rehabilitation in the acquired immunodeficiency syndrome.

    PubMed

    O'Connell, P G; Levinson, S F

    1991-08-01

    Patients with the acquired immunodeficiency syndrome (AIDS) represent a novel referral population for rehabilitation services. Limited information about the rehabilitation needs of individuals with human immunodeficiency virus infection is available. We reviewed 51 consecutive patients with AIDS referred to a rehabilitation consult service. Common problems encountered included generalized deconditioning (27%) and neurologic dysfunction (45%). Neurologic presentations were diverse and included hemiparesis, diffuse cognitive dysfunction and dementia, myelopathy, myopathy and peripheral neuropathy. Other patients were referred for wound care as well as the management of the local effects of Kaposi's sarcoma, various musculoskeletal syndromes and new onset blindness. Problems identified included impaired mobility (76%), difficulty with self-care (57%), impaired cognition (29%) and uncontrolled pain (37%). Among the rehabilitation interventions utilized were therapeutic exercise (73%), gait aids (45%), bathroom and safety equipment (45%), orthotics (29%), vocational counseling (4%), pain management (29%) and whirlpool treatments (10%). Five patients were too ill or refused treatment. We conclude that AIDS patients referred for rehabilitation have a wide variety of physical deficits, demonstrate a considerable degree of functional impairment and may require multiple rehabilitation interventions. PMID:1878178

  13. [Hemiballismus disclosing cerebral toxoplasmosis and acquired immunodeficiency syndrome].

    PubMed

    Awada, A

    1993-01-01

    A 33-year-old Saudi woman presented with right hemiballismus of recent onset. Brain CT showed a left thalamo-subthalamic lesion which was thought initially to be a metastasis or a tuberculoma. The presence of severe subacute diarrhea, multiple lymphadenopathies and lymphopenia suggested an acquired immunodeficiency syndrome (AIDS). Tests for HIV-1 infection were positive and, despite the absence of antitoxoplasma antibodies in the serum, antitoxoplasmic treatment by pyrimethamine and sulfadiazine was given. One and a half month later, both abnormal movements and CT images had disappeared. The probable source of HIV infection was imported packed red blood cells received by the patient 5 years earlier. Toxoplasmic brain abscess associated with AIDS should be considered as a possible cause of hemiballismus in young adult even in the regions where AIDS is still infrequent. PMID:8303164

  14. Head and neck presentations of acquired immunodeficiency syndrome.

    PubMed

    Rosenberg, R A; Schneider, K L; Cohen, N L

    1984-05-01

    Since December 1980, over 2,000 cases of acquired immunodeficiency syndrome (AIDS) have been reported. The charts of 72 patients admitted to the New York University Medical Center with a diagnosis of AIDS were reviewed with particular emphasis on presenting signs, symptoms and laboratory values. Symptoms tended to be non-specific and most often resembled an upper respiratory infection. Over 95% of the patients presented with either diffuse adenopathy, oral or facial lesions consistent with Kaposi's sarcoma, white oral lesions, or anergy. Laboratory findings included leukopenia, increased erythrocyte sedimentation rate, thrombocytopenia and anemia. The in-hospital mortality rate was 26%. The current status of our knowledge concerning AIDS is reviewed and discussed. The frequency and types of presenting signs and symptoms in the head and neck are reported in order to alert the otolaryngologic community to this entity. PMID:6717222

  15. Head and neck presentations of acquired immunodeficiency syndrome.

    PubMed

    Rosenberg, R A; Schneider, K L; Cohen, N L

    1985-12-01

    Since December 1980, over 3000 cases of acquired immunodeficiency syndrome (AIDS) have been reported. The charts of 102 patients admitted to the New York University Medical Center with a diagnosis of AIDS were reviewed with particular emphasis on presenting signs, symptoms, and laboratory values. Symptoms tended to be nonspecific and most often resembled those of an upper respiratory infection. Over 71% of the patients presented with at least two of the following four signs: diffuse adenopathy, oral and facial lesions consistent with Kaposi's sarcoma, white oral lesions, and anergy. Laboratory findings included leukopenia, increased erythrocyte sedimentation rate, thrombocytopenia, and anemia. The in-hospital mortality rate was 26%. The current status of our knowledge concerning AIDS is reviewed and discussed. The frequency and types of presenting signs and symptoms in the head and neck are reported to alert the otolaryngologic community to this entity. PMID:3937090

  16. Clinical predictors of functioning in persons with acquired immunodeficiency syndrome.

    PubMed

    Wilson, I B; Cleary, P D

    1996-06-01

    To help clinicians better assess and treat functional disabilities in persons with acquired immunodeficiency syndrome (AIDS), the authors estimate empirical relations among biologic and physiologic variables, symptoms, and physical functioning in persons with AIDS. The sample of 305 persons with AIDS for this cross-sectional analysis came from three sites in Boston, Massachusetts: a hospital-based group practice, a human immunodeficiency virus clinic at a city hospital, and a staff-model health maintenance organization. Physical functioning, 10 AIDS-specific symptoms, and mental health were assessed by interview. Clinical diagnoses, comorbidities, health habits such as smoking, laboratory results, and selected medication use were assessed by chart review. Significant predictors of physical functioning P < 0.01, R2 = .58) in a multivariable regression model included energy/fatigue, neurologic symptoms, fever symptoms, a lower hemoglobin level, and current non-pneumonia bacterial infection. Ninety-six percent of the explained variance in physical functioning was accounted for by three symptom complexes: energy/fatigue, neurologic symptoms, and fever symptoms. Significant predictors of energy/fatigue in multivariable models included poorer mental health, lower white blood cell count, longer time since diagnosis, and weight loss (P < 0.01, R2 =.36). Significant predictors of neurologic symptoms included poorer mental health, weight loss, and no zidovudine use (P < 0.001, R2 = .30). Predictors of fever symptoms included poorer mental health, no zidovudine use, weight loss, and history of asthma or chronic obstructive pulmonary disease (P < 0.05, R2 = .25). In conclusion, symptom reports were strong predictors of physical functioning. Poorer mental health and weight loss were correlated consistently with worse symptoms, and not using zidovudine was correlated with worse neurologic and fever symptoms. These variables, and the others the authors identified, may represent

  17. Neurosurgical management of the acquired immunodeficiency syndrome. An update.

    PubMed Central

    Andrews, B T; Kenefick, T P

    1993-01-01

    A retrospective review of a 24-month experience on the neurosurgical service at a large metropolitan hospital identified 33 patients with the acquired immunodeficiency syndrome (AIDS) who underwent diagnostic or therapeutic procedures. Intracranial mass lesions unresponsive to empiric medical therapy for presumed Toxoplasma gondii encephalitis underwent diagnostic biopsy in 22 patients: primary lymphoma was identified in 10 (45%) of these patients, and biopsy led to a treatable diagnosis in 16 of the 22. Patients with lymphoma were significantly more likely to have a single mass lesion than those with other diagnoses. The remaining 11 patients had a wide variety of neurologic disorders, including multiple strokes and transverse myelitis, aspergillous fungal infection of the base of the skull, primary lymphoma of the spinal cord, cat-scratch fever of the spine causing painful radiculopathy, hydrocephalus associated with cryptococcal meningitis, and progressive inflammatory peripheral neuropathy. Two patients had lymphoma within the subarachnoid space. Three patients with well-controlled AIDS underwent elective neurosurgical therapy for intractable radiculopathies due to herniated lumbar discs in 2 and cervical spondylosis in 1. Current treatment strategies in AIDS appear to have limited the need for brain biopsy, but the spectrum of neurologic disorders has broadened, requiring continued participation by neurologists and neurosurgeons. With improved long-term survival, the elective treatment of non-AIDS-related neurologic disorders in selected patients may be appropriate. Images PMID:8460506

  18. Health Administrator Perspectives on Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome Prevention and Services at Historically Black Colleges and Universities

    ERIC Educational Resources Information Center

    Warren-Jeanpiere, Lari; Jones, Sandra; Sutton, Madeline Y.

    2011-01-01

    Objective: Due to the disproportionate impact of human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) among African American young adults, the authors explored (1) number of historically black college and university (HBCU) campuses with existing HIV prevention policies and services and (2) perceived barriers for implementing…

  19. The First Case of Vestibulocochlear Neuritis in a Patient with Acquired Immunodeficiency Syndrome in Korea

    PubMed Central

    Park, Hyun Joo; Cho, Chin Saeng; Kim, Nak Min; Yun, Su A

    2016-01-01

    The incidence of human immunodeficiency virus (HIV) infections continue to increase throughout the world. Although neurologic complications are frequent in individuals with HIV infection or acquired immunodeficiency syndrome (AIDS), vestibulocochlear neuritis is still a relatively rare manifestation. We report the first case of vestibulocochlear neuritis occurring in an AIDS patient in Korea. PMID:27433384

  20. Prevention and treatment of human immunodeficiency virus/acquired immunodeficiency syndrome in resource-limited settings.

    PubMed Central

    Hogan, Daniel R.; Salomon, Joshua A.

    2005-01-01

    Strategies for confronting the epidemic of human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) have included a range of different approaches that focus on prevention and treatment. However, debate persists over what levels of emphasis are appropriate for the different components of the global response. This paper presents an overview of this debate and briefly summarizes the evidence on a range of interventions designed to prevent the spread of HIV infection, paying particular attention to voluntary counselling and testing, treatment for sexually transmitted infections and prevention of mother-to-child transmission. We also review the experience with antiretroviral therapy to date in terms of response rates and survival rates, adherence, drug resistance, behavioural change and epidemiological impact. Although various studies have identified strategies with proven effectiveness in reducing the risks of HIV infection and AIDS mortality, considerable uncertainties remain. Successful integration of treatment and prevention of HIV/AIDS will require a balanced approach and rigorous monitoring of the impact of programmes in terms of both individual and population outcomes. PMID:15744406

  1. The Effects of a Kansas Education Class on Students' Knowledge and Attitudes of Human Immunodeficiency Virus and Acquired Immunodeficiency Syndrome.

    ERIC Educational Resources Information Center

    Sager, R. Warren, Jr.

    This study was undertaken to investigate the knowledge and attitudes of 8th, 9th, and 10th grade Kansas students pertaining to human immunodeficiency virus (HIV) and Acquired Immune Deficiency Syndrome (AIDS). Attitudes and knowledge of 9th and 10th grade students who had participated in a Sex Respect Class offered in the 9th grade were compared…

  2. [Pathology of urologic importance associated with acquired immunodeficiency syndrome].

    PubMed

    Musci, R; Meroni, T; Andres, M; De Cobelli, O; Larcher, P; Franchini, V; Bovo, G

    1993-04-01

    Infection by the HIV virus affects the urogenital system in a minor percentage of cases in comparison to other organs such as the lungs, the central nervous system and the haemolymphopoietic system. In recent years however, with the continued spread of the disease also urologists find themselves dealing with the various urogenital pathologies that are presented in seropositive or fully-blown Aids patients. The Authors present their experience and describe the problems correlated to the dealing with acquired immune deficiency syndrome patients that are affected with urogenital pathologies. PMID:8330057

  3. Ocular examination and diagnosis in patients with the acquired immunodeficiency syndrome.

    PubMed Central

    Gariano, R F; Rickman, L S; Freeman, W R

    1993-01-01

    Ocular involvement is seen frequently and is an important source of morbidity in patients with the acquired immunodeficiency syndrome. We outline here the general skills of physical diagnosis valuable to primary care physicians or infectious diseases specialists in recognizing and evaluating ocular complaints in patients infected with the human immunodeficiency virus. We provide an overview of common ocular diseases in these patients, with an emphasis on signs and symptoms that aid in the differential diagnosis. Images PMID:8384763

  4. Implications of Acquired Immunodeficiency Syndrome for Professionals in the Field of Visual Impairments and Blindness.

    ERIC Educational Resources Information Center

    Daugherty, William E.

    1988-01-01

    Acquired Immunodeficiency Syndrome (AIDS) has significant ocular implications. This article examines: the effect of AIDS on vision, historical and philosophical perspectives on public health education, AIDS education, legal and policy issues of concern to schools and service agencies, and sex education and AIDS-related education of blind and…

  5. [Hemophagocytic syndrome associated with tuberculosis in a patient with acquired immunodeficiency].

    PubMed

    González, Norma E; Álvarez Ponte, Silvia; López, Mariela; Fronti, Pablo; Smith, Silvina; Pawluk, Victor

    2016-10-01

    The secondary hemophagocytic syndrome is rare in children and even rarer associated with tuberculosis. e report the case of a patient with acquired immunodeficiency syndrome, disseminated tuberculosis and hemophagocytic syndrome. An 8-year-old girl, diagnosed with acquired immunodeficiency syndrome, was admitted due to fever, vomiting and abdominal pain. She presented abdominal distension, dehydration, tachypnea, crackles and wheezing in both lungs, anemia, thrombocytopenia and coagulopathy. She received broad-spectrum antibiotics and exploratory laparotomy was performed with appendectomy and lymph node biopsy. After 72 hours the patient presented tonic clonic seizure, impaired sensory, fever, hypoxemia, hepatosplenomegaly, ascites and peripheral edema. She developed bicytopenia, hyperferritinemia and bone marrow microscopic examination with hemophagocytosis. She received intravenous gammaglobulin, steroids and blood transfusions. Mycobacterium tuberculosis was cultured in gastric aspirate, bone marrow and abdominal lymph node biopsy. She was treated with isoniazid, rifampicin, streptomycin and ethambutol, showing marked improvement. PMID:27606663

  6. Kaposi sarcoma and lymphadenopathy syndrome: limitations of abdominal CT in acquired immunodeficiency syndrome

    SciTech Connect

    Moon, K.L. Jr.; Federle, M.P.; Abrams, D.I.; Volberding, P.; Lewis, B.J.

    1984-02-01

    Abdominal computed tomography (CT) was performed in 31 patients with Kaposi sarcoma (KS) related to acquired immunodeficiency syndrome (AIDS), three patients with classic KS, and 12 patients with the newly described lymphadenopathy syndrome (LNS). The frequency, distribution, and appearance of lymphadenopathy and splenomegaly were similar in the AIDS-related KS and LNS groups. Rectal and perirectal disease was identified in 86% of homosexual men studied; rectal KS could not be distinguished from proctitis on CT criteria alone. No CT abnormalities were seen in patients with classic KS. The CT demonstration of retroperitoneal, mesenteric, or pelvic adenopathy or of rectal or perirectal disease in patients with AIDS-related KS is not necessarily indicative of widespread involvement with the disease.

  7. Frequency of hyponatremia and nonosmolar vasopressin release in the acquired immunodeficiency syndrome

    SciTech Connect

    Vitting, K.E.; Gardenswartz, M.H.; Zabetakis, P.M.; Tapper, M.L.; Gleim, G.W.; Agrawal, M.; Michelis, M.F. )

    1990-02-16

    The frequency and pathophysiology of hyponatremia were studied in the acquired immunodeficiency syndrome. Of 71 hospitalized patients surveyed retrospectively, hyponatremia was observed in 37 (52%). Of 48 patients studied prospectively, 27 (56%) were hyponatremic. In 16 hyponatremic patients, volume status; serum and urine osmolalities; renal, adrenal, and thyroid function; and plasma vasopressin levels were assessed. Urine osmolalities were inappropriately elevated relative to serum osmolalities. Four patients had moderate renal insufficiency. Plasma vasopressin levels, measured by radioimmunoassay, were elevated in 15 patients, with the highest levels seen in patients who died. Hyponatremia of multiple etiologies occurred in a majority of inpatients with the acquired immunodeficiency syndrome, often following the administration of hypotonic fluids, and was associated with a 30% (8/27) short-term mortality.

  8. Nontropical pyomyositis as a cause of subacute, multifocal myalgia in the acquired immunodeficiency syndrome

    SciTech Connect

    Wolf, R.F.; Sprenger, H.G.; Mooyaart, E.L.; Tamsma, J.T.; Kengen, R.A.; Weits, J. )

    1990-11-01

    We report a case of nontropical pyomyositis in a patient with acquired immunodeficiency syndrome and disseminated Mycobacterium avium infection, in which severe myalgia was the presenting symptom over several weeks. Multifocal muscle lesions were identified by gallium scanning and magnetic resonance imaging techniques. The epidemiology, possible pathogenesis, clinical features, diagnostic imaging, and therapy are reviewed. Early suspicion of nontropical pyomyositis in severely immunocompromised patients with cryptic myalgia is recommended.

  9. Rapidly progressed acquired immunodeficiency syndrome dementia complex as an initial manifestation.

    PubMed

    Takeuchi, Makoto; Nobukuni, Keigo; Takata, Hiroshi; Kawata, Noriko; Hayashibara, Noriko; Ishizu, Hideki; Takahashi, Kiyoshi

    2005-07-01

    We report a patient with acquired immunodeficiency syndrome dementia complex (ADC) that presented human immunodeficiency virus infection as an initial manifestation. A 34-year-old man developed disturbance of consciousness and severe abulia over 3 months. The CD4 lymphocyte count was 7.9/microl, while human immunodeficiency virus RNA in blood amounted to 4.2 x 10(4) copies/ml. T2-weighted magnetic resonance imaging showed diffusely high signal intensity in the deep white matter of both cerebral hemispheres. On the 20th hospital day, the patient died of sepsis caused by methicillin-resistant Staphylococcus aureus. Autopsy findings in the brain included increased glial cells and multinucleated giant cells in cerebral white matter and subcortical gray matter. These features were compatible with ADC. PMID:16093602

  10. Epstein-Barr and human immunodeficiency viruses in acquired immunodeficiency syndrome-related primary central nervous system lymphoma.

    PubMed Central

    Morgello, S.

    1992-01-01

    The prevalence of Epstein-Barr virus (EBV) and human immunodeficiency virus (HIV) in acquired immunodeficiency syndrome (AIDS)-related primary central nervous system (CNS) lymphoma was examined. Deoxyribonucleic acid (DNA) extracted from 12 formalin-fixed, paraffin-embedded tumors was used as substrate for the polymerase chain reaction (PCR). Targets for amplification were the EBNA-1 region of EBV, the gag region of HIV, and a single copy cellular sequence as a control. The cases studied were autopsy and surgical specimens collected between the years 1985 and 1989. By the working formulation for non-Hodgkin's lymphomas, five had large cell, four had mixed large and small cleaved cell, two had small cleaved cell, and one had an unclassified histology. Epstein-Barr virus was detected in 6 of 12 tumors studied. Human immunodeficiency virus was not detected in any of the tumors. The presence of EBV was not correlated with any particular histologic tumor type. It is concluded that EBV, not HIV, can be detected in a large percentage (50%) of AIDS-related primary central nervous system (CNS) lymphomas. This viral association may be significant in light of the demonstrated ability of EBV to induce lymphoid tumors in experimental mammalian systems. Images Figure 1 Figure 2 PMID:1323221

  11. The diagnostic utility of bone marrow aspiration and biopsy in patients with acquired immunodeficiency syndrome.

    PubMed Central

    Gluckman, R. J.; Rosner, F.; Guarneri, J. J.

    1989-01-01

    Diagnostic bone marrow aspiration, biopsy, and culture are useful procedures in the evaluation of patients with suspected or proven acquired immunodeficiency syndrome (AIDS) who are febrile. In as many as one fourth of these patients, the information provided by the bone marrow examination may establish a diagnosis of a disseminated opportunistic infection when other studies are not informative. We have also discovered a previously unreported association between thrombocytopenia and the presence of bone marrow granulomas in our patients with AIDS and suggest that thrombocytopenia may be a clue to enable the clinician to predict a positive bone marrow result more accurately. The explanation for this apparent association remains to be elucidated. PMID:2733050

  12. Peripheral Blood and Bone Marrow Abnormalities in the Acquired Immunodeficiency Syndrome

    PubMed Central

    Frontiera, Michael; Myers, Adam M.

    1987-01-01

    In reviewing the peripheral hematologic manifestations, bone marrow changes and clinical course in 41 consecutive patients with acquired immunodeficiency syndrome (AIDS), frequent findings included anemia (95%), leukopenia (76%), bone marrow hypercellularity (73%) and pancytopenia (41%). These hematologic abnormalities were not clearly associated with specific clinical manifestations of AIDS, but support the conclusion that the hematopoietic system is a target organ in AIDS. The mechanisms of these abnormalities still need to be evaluated. Clinicians should be aware of these commonly encountered changes. Images PMID:3660772

  13. Specific translocations characterize Burkitt's-like lymphoma of homosexual men with the acquired immunodeficiency syndrome.

    PubMed

    Chaganti, R S; Jhanwar, S C; Koziner, B; Arlin, Z; Mertelsmann, R; Clarkson, B D

    1983-06-01

    A Burkitt's-like B-cell lymphoma (BLL) has recently been shown to be associated with the acquired immunodeficiency syndrome (AIDS), which affects homosexual men. We report cytogenetic studies of two BLL tumors in homosexual men. Both tumors had chromosome translocations characteristic of Burkitt's lymphoma (BL), one the t(8;14) and the other the t(8;22). The pathway of lymphomagenesis in this disorder is discussed in the light of recent data on chromosome change and localization of immunoglobulin genes and oncogenes. PMID:6839024

  14. Detection of thoracic infections by nuclear medicine techniques in the acquired immunodeficiency syndrome

    SciTech Connect

    Kramer, E.L.; Sanger, J.J. )

    1989-11-01

    The challenge of the acquired immunodeficiency syndrome (AIDS) for nuclear medicine has been the early detection of related intrathoracic opportunistic infections, inflammatory conditions, and neoplasms. Gallium-67 citrate scanning has proved a sensitive test not only for Pneumocystis carinii pneumonia but for many of the other opportunistic infections and malignancies, including mycobacterial infections and lymphoma. Patterns and intensity of gallium uptake may suggest more specific diagnoses. Indium-111-labeled white blood cells may also be a valuable diagnostic tool in the AIDS patient.41 references.

  15. Patterns of gallium-67 scintigraphy in patients with acquired immunodeficiency syndrome and the AIDS related complex

    SciTech Connect

    Bitran, J.; Bekerman, C.; Weinstein, R.; Bennett, C.; Ryo, U.; Pinsky, S.

    1987-07-01

    Thirty-two patients with AIDS related complex (ARC) or acquired immunodeficiency syndrome (AIDS) underwent /sup 67/Ga scans as part of their evaluation. Three patterns of /sup 67/Ga biodistribution were found: lymph node uptake alone; diffuse pulmonary uptake; normal scan. Gallium-67 scans were useful in identifying clinically occult Pneumocystis carinii pneumonia in seven of 15 patients with ARC who were asymptomatic and had normal chest radiographs. Gallium scans are a useful ancillary procedure in the evaluation of patients with ARC or AIDS.

  16. Investigation of Potent Lead for Acquired Immunodeficiency Syndrome from Traditional Chinese Medicine

    PubMed Central

    Hung, Tzu-Chieh; Lee, Wen-Yuan; Chen, Kuen-Bao; Chan, Yueh-Chiu; Chen, Calvin Yu-Chian

    2014-01-01

    Acquired immunodeficiency syndrome (AIDS), caused by human immunodeficiency virus (HIV), has become, because of the rapid spread of the disease, a serious global problem and cannot be treated. Recent studies indicate that VIF is a protein of HIV to prevent all of human immunity to attack HIV. Molecular compounds of traditional Chinese medicine (TCM) database filtered through molecular docking and molecular dynamics simulations to inhibit VIF can protect against HIV. Glutamic acid, plantagoguanidinic acid, and Aurantiamide acetate based docking score higher with other TCM compounds selected. Molecular dynamics are useful for analysis and detection ligand interactions. According to the docking position, hydrophobic interactions, hydrogen bonding changes, and structure variation, the study try to select the efficacy of traditional Chinese medicine compound Aurantiamide acetate is better than the other for protein-ligand interactions to maintain the protein composition, based on changes in the structure. PMID:25013765

  17. Altered Virome and Bacterial Microbiome in Human Immunodeficiency Virus-Associated Acquired Immunodeficiency Syndrome.

    PubMed

    Monaco, Cynthia L; Gootenberg, David B; Zhao, Guoyan; Handley, Scott A; Ghebremichael, Musie S; Lim, Efrem S; Lankowski, Alex; Baldridge, Megan T; Wilen, Craig B; Flagg, Meaghan; Norman, Jason M; Keller, Brian C; Luévano, Jesús Mario; Wang, David; Boum, Yap; Martin, Jeffrey N; Hunt, Peter W; Bangsberg, David R; Siedner, Mark J; Kwon, Douglas S; Virgin, Herbert W

    2016-03-01

    Human immunodeficiency virus (HIV) infection is associated with increased intestinal translocation of microbial products and enteropathy as well as alterations in gut bacterial communities. However, whether the enteric virome contributes to this infection and resulting immunodeficiency remains unknown. We characterized the enteric virome and bacterial microbiome in a cohort of Ugandan patients, including HIV-uninfected or HIV-infected subjects and those either treated with anti-retroviral therapy (ART) or untreated. Low peripheral CD4 T cell counts were associated with an expansion of enteric adenovirus sequences and this increase was independent of ART treatment. Additionally, the enteric bacterial microbiome of patients with lower CD4 T counts exhibited reduced phylogenetic diversity and richness with specific bacteria showing differential abundance, including increases in Enterobacteriaceae, which have been associated with inflammation. Thus, immunodeficiency in progressive HIV infection is associated with alterations in the enteric virome and bacterial microbiome, which may contribute to AIDS-associated enteropathy and disease progression. PMID:26962942

  18. Nonspecific interstitial pneumonitis: a common cause of pulmonary disease in the acquired immunodeficiency syndrome

    SciTech Connect

    Suffredini, A.F.; Ognibene, F.P.; Lack, E.E.; Simmons, J.T.; Brenner, M.; Gill, V.J.; Lane, H.C.; Fauci, A.S.; Parrillo, J.E.; Masur, H.

    1987-07-01

    During a 4.4-year period, nonspecific interstitial pneumonitis was seen in 41 of 110 (38%) patients with the acquired immunodeficiency syndrome and accounted for 32% (48/152) of all episodes of clinical pneumonitis. Diffuse alveolar damage was typically a feature of nonspecific interstitial pneumonitis, but neither lung biopsy nor bronchoalveolar lavage detected a pathogen. Of these 41 patients, 13 had no associated pulmonary tumor and had not been exposed to pulmonary toxins, whereas 28 patients had either concurrent pulmonary Kaposi sarcoma, previous experimental therapies, or a history of pneumocystis pneumonia or drug abuse. Of these 41, 23 had normal chest radiographs. The clinical features of patients with nonspecific interstitial pneumonitis were similar to those of patients with pneumocystis pneumonia, although histologic findings showed less severe alveolar damage in patients with nonspecific interstitial pneumonitis (p less than 0.001). Pathologic evaluation and clinical follow-up suggest that many clinical episodes of pneumonitis in patients with the acquired immunodeficiency syndrome are due to nonspecific interstitial pneumonitis of unknown cause.

  19. The human immunodeficiency virus preventive vaccine research at the French National Agency for acquired immunodeficiency syndrome research.

    PubMed

    Fischer, Elizabeth; Rieux, Véronique; Guillet, Jean-Gérard; Kazatchkine, Michel

    2005-02-01

    The human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) epidemic is of unprecedented gravity and is spreading rapidly, notably in the most disadvantaged regions of the world. The search for a preventive vaccine is thus an absolute priority. For over 10 years the French National Agency for AIDS research (ANRS) has been committed to an original program combining basic science and clinical research. The HIV preventive vaccine research program run by the ANRS covers upstream research for the definition of immunogens, animal models, and clinical research to evaluate candidate vaccines. Most researchers in 2004 believe that it should be possible to obtain partial vaccine protection through the induction of a strong and multiepitopic cellular response. Since 1992, the ANRS has set up 15 phases I and II clinical trials in order to evaluate the safety and the capacity of the candidate vaccines for inducing cellular immune responses. The tested candidate vaccines were increasingly complex recombinant canarypox viruses (Alvac) containing sequences coding for certain viral proteins, utilized alone or combined with other immunogens (whole or truncated envelope proteins). ANRS has also been developing an original strategy based on the utilization of lipopeptides. These comprise synthetic fragments of viral proteins associated with lipids that facilitate the induction of a cellular immune response. These approaches promptly allowed the assessment of a prime-boost strategy combining a viral vector and lipopeptides. PMID:15867969

  20. South Asian Consensus Guidelines for the rational management of diabetes in human immunodeficiency virus/acquired immunodeficiency syndrome

    PubMed Central

    Kalra, Sanjay; Unnikrishnan, Ambika Gopalakrishnan; Raza, Syed Abbas; Bantwal, Ganpathy; Baruah, Manash P.; Latt, Tint Swe; Shrestha, Dina; John, Mathew; Katulanda, Prasad; Somasundaram, Noel; Sahay, Rakesh; Pathan, Faruque

    2011-01-01

    As newer methods of management are made available, and accessible, survival rates with human immunodeficiency virus (HIV) are increasing. This means that chronic, metabolic complications of HIV are becoming more frequent in clinical practice, as acute morbidity is controlled. Management of HIV/acquired immunodeficiency syndrome (AIDS) is gradually expanding to include these chronic and metabolic complications of the disease, and the adverse effects associated with its treatments, including diabetes. Unfortunately, no guidelines are available to help the medical practitioners choose appropriate therapy for patients with these conditions. The aim of the South Asian Consensus Guidelines is to provide evidence-based recommendations to assist healthcare providers in the rational management of type 2 diabetes mellitus in patients with HIV. The development of these guidelines used systematic reviews of available evidence to form its key recommendations. These guidelines and associated review of literature represent a compilation of available knowledge regarding rational management of diabetes in HIV. Patients of diabetes with concomitant HIV infection are managed optimally with insulin therapy and judicious use of highly active antiretroviral therapy with suitable alternatives is also recommended. These guidelines should prove helpful to physicians, not only in South Asia, but also across the globe, while managing patients with coexistent HIV and diabetes. PMID:22028994

  1. Sequence heterogeneity of murine acquired immunodeficiency syndrome virus: the role of endogenous virus.

    PubMed

    Gayama, S; Vaupel, B A; Kanagawa, O

    1995-05-01

    A defective murine leukemia virus is the causative agent of murine acquired immunodeficiency syndrome (MAIDS). We have cloned cDNAs from both virus infected and non-infected cells using the PCR methods with primers corresponding to the franking sequence of the unique p12 gag gene. Sequence analysis of these cDNA clones revealed: (i) the presence of endogenous virus related to MAIDS virus in C57BL/6 mice, (ii) B cell lineage specific expression of endogenous virus and (iii) extensive heterogeneity of MAIDS virus recovered from virus infected cells due to the recombination of the related viruses (defective pathogenic virus, ecotropic virus and endogenous virus). These findings suggest that the creation of virus variants in infected cells may play an important role in virus pathogenesis and escape from immune attack during the development of MAIDS. PMID:7547712

  2. Gallium-67 scans of the chest in patients with acquired immunodeficiency syndrome

    SciTech Connect

    Kramer, E.L.; Sanger, J.J.; Garay, S.M.; Greene, J.B.; Tiu, S.; Banner, H.; McCauley, D.I.

    1987-07-01

    Eighty-six (/sup 67/Ga)citrate chest scans were performed in 71 adult patients with the acquired immunodeficiency syndrome. Forty-five of these patients also had Kaposi's sarcoma. Only 29 of 57 abnormal scans were correlated with abnormal chest radiographs. Chest radiographs were negative for 27 scans and unavailable for one. Several scan patterns were seen. Diffusely increased lung uptake was seen most commonly with Pneumocystis carinii pneumonia, but also other infections and noninfectious inflammatory conditions. Focal uptake corresponding to regional lymph node groups occurred most often with Mycobacterium avium-intracellulare but aslo with lymphoma. Localized intrapulmonary uptake was seen in bacterial pneumonias. Perihilar activity occurred in two cases. When chest radiographs were abnormal and /sup 67/Ga scans negative, the most common diagnosis was pulmonary Kaposi's sarcoma.

  3. First report of Cystoisospora belli parasitemia in a patient with acquired immunodeficiency syndrome.

    PubMed

    Velásquez, Jorge Néstor; di Risio, Cecilia Alicia; Etchart, Cristina Beatriz; Chertcoff, Agustín Víctor; Nigro, Mónica Gabriela; Pantano, María Laura; Ledesma, Bibiana Alba; Vittar, Natalia; Carnevale, Silvana

    2016-01-01

    Cystoisospora belli in patients with the acquired immunodeficiency syndrome (AIDS) has been described as cause of chronic diarrhea and disseminated cystoisosporosis. Diagnosis of intestinal cystoisosporosis can be achieved at the tissue level in the villus epithelium of the small bowel. Disseminated cystoisosporosis is diagnosed by microscopy identification of unizoite tissue cysts in the lamina propria of the intestine. We report a case of disseminated cystoisosporosis in a human immunodeficiency virus (HIV)-infected patient with detection of parasitemia. We studied a 39-year old patient with AIDS and chronic diarrhea by analysis of stool and duodenal biopsy samples. Blood samples were also collected and examined by light microscopy and molecular techniques for C. belli DNA detection. The unizoite tissue cyst stages were present in the lamina propria, with unsporulated oocysts in feces. Zoites were present in blood smears and DNA of C. belli was detected in blood samples. Our study identified a new stage in the life cycle of C. belli. Detection of parasitemia is a novel and noninvasive tool for diagnosis of disseminated cystoisosporosis. PMID:26751889

  4. Selected Laws, Rules and State-Level Activities in Wisconsin Related to Acquired Immunodeficiency Syndrome. Information Memorandum 87-4.

    ERIC Educational Resources Information Center

    Sweet, Richard

    This information memorandum describes the selected laws, rules, and state-level activities in Wisconsin related to acquired immunodeficiency syndrome (AIDS) and tests for antibodies to the virus (HIV) that causes AIDS. A section on current state laws on AIDS and HIV antibody testing describes laws related to informed consent for testing,…

  5. Attitudes toward the care of patients with acquired immunodeficiency syndrome. A survey of community internists.

    PubMed

    Somogyi, A A; Watson-Abady, J A; Mandel, F S

    1990-01-01

    Community physicians may play an increasing role in treating patients with acquired immunodeficiency syndrome (AIDS) because of the shift away from inpatient care. At a community hospital in New York, NY, we surveyed 230 attending physicians in a department of medicine to determine their attitudes toward the care of patients with AIDS. Factor analysis produced three clusters of attitudes termed antipathy, liability, and isolation. These factors, together with physicians' "knowledge" and background, were analyzed as predictors of treating patients with AIDS. While antipathy, isolation, and fear of acquiring AIDS were not predictors, liability scores were inversely correlated with the likelihood of treating the disease (r = -.18). Liability was influenced by patients' attitudes toward AIDS. Primary care physicians had higher liability and isolation scores than subspecialists and were more likely to see support groups, guaranteed funding, and education as incentives to treat patients with AIDS. Further study is needed to target measures that support primary care physicians in their care of patients with this disease. PMID:2297299

  6. Human immunodeficiency virus/acquired immune deficiency syndrome: Using drug from mathematical perceptive

    PubMed Central

    Chatterjee, Amar Nath; Saha, Shubhankar; Roy, Priti Kumar

    2015-01-01

    Entry of acquired immune deficiency syndrome virus into the host immune cell involves the participation of various components of host and viral cell unit. These components may be categorized as attachment of the viral surface envelope protein subunit, gp120, to the CD4+ receptor and chemokine coreceptors, CCR5 and CXCR4, present on T cell surface. The viral fusion protein, gp41, the second cleaved subunit of Env undergoes reconfiguration and the membrane fusion reaction itself. Since the CD4+ T cell population is actively involved; the ultimate outcome of human immunodeficiency virus infection is total collapse of the host immune system. Mathematical modeling of the stages in viral membrane protein-host cell receptor-coreceptor interaction and the effect of antibody vaccine on the viral entry into the susceptible host cell has been carried out using as impulsive differential equations. We have studied the effect of antibody vaccination and determined analytically the threshold value of drug dosage and dosing interval for optimum levels of infection. We have also investigated the effect of perfect adherence of drug dose on the immune cell count in extreme cases and observed that systematic drug dosage of the immune cells leads to longer and improved lives. PMID:26568917

  7. Disseminated histoplasmosis in acquired immunodeficiency syndrome patients in Uberaba, MG, Brazil.

    PubMed

    Mora, Delio José; dos Santos, Celso Tadeu Barbosa; Silva-Vergara, Mario León

    2008-03-01

    Histoplasmosis occurs in approximately 5% of acquired immunodeficiency syndrome (AIDS) patients in endemic areas and often evolves to a disseminated picture if diagnosis is delayed and/or CD4 count falls below 150 cells x mm(3) without high active antiretroviral therapy (HAART). This report presents clinical features of patients with histoplasmosis admitted from 1992 to 2005. Of the 57 individuals, 45 (79%) were male, aged 20-40 years; 30 (52.6%) presented histoplasmosis together with HIV diagnosis and 35 (61.4%) referred illness course up to 4 weeks. Fever, hepatomegaly and/or splenomegaly, dyspnea and skin lesions were noticed in 50 (87.7%), 38 (66.7%), 30 (52.6%) and 25 (43.9%) patients respectively. High levels of lactic acid dehydrogenase, X-ray lung interstitial pattern, pancytopenia and CD4 count <100 cells x mm(3) were observed in 48 (84.2%), 35 (66%), 34 (59.6%) and 33 (94%) patients respectively. Mycological diagnosis was performed by one or more methods in all patients. Thirty nine (68.4%) received amphotericin B and/or itraconazole. A cure rate was observed in 76.9% and nine (23.1%) died early during therapy. Otherwise death occurred in 18 (31.6%) before diagnosis was completed. Despite free HAART disposal in public Brazilian health services, histoplasmosis still occurs as the first AIDS baseline condition in patients without antiretroviral therapy, many of whom are not receiving any medical care for HIV infection. PMID:18254750

  8. Anti-F(ab')2 antibodies in thrombocytopenic patients at risk for acquired immunodeficiency syndrome.

    PubMed Central

    Yu, J R; Lennette, E T; Karpatkin, S

    1986-01-01

    22 homosexual or narcotic addict patients at risk for acquired immunodeficiency syndrome (AIDS) or with AIDS, were studied for the presence of antiimmunoglobulin antibodies and circulating immune complexes (20 were thrombocytopenic, 6 had AIDS). Circulating immune complex levels were 10-fold higher than levels in normal subjects. IgG anti-F(ab')2 antibodies were noted in homosexual as well as narcotic addict patients. Of 16 homosexual patients, 7 had IgG anti-F(ab')2 antibody of moderate to marked titer with broad reactivity against autologous, homologous, and control F(ab')2 fragments. Three others demonstrated limited reactivity against one or two F(ab')2 fragments. The remaining six patients were negative. Six of six narcotic addict patients had IgG anti-F(ab')2 antibody, five with limited reactivity, one with broad reactivity. In contrast, neither elevated circulating immune complexes nor anti-F(ab')2 antibodies were detectable in six autoimmune thrombocytopenic patients. Anti-F(ab')2 antibody could be affinity purified from serum or circulating immune complexes. Anti-F(ab')2 reactivity correlated with circulating immune complex levels, r = 0.83, P less than 0.01. PMID:3011860

  9. The spatial distribution pattern of human immunodeficiency virus/acquired immune deficiency syndrome in China.

    PubMed

    Wang, Ying; Yang, Yongli; Shi, Xuezhong; Mao, Saicai; Shi, Nian; Hui, Xiaoqing

    2016-01-01

    Human immunodeficiency virus (HIV) infection and the acquired immune deficiency syndrome (AIDS) exhibit variable patterns among the provinces of China. Knowledge of the geographical distribution of the HIV/AIDS epidemic is needed for the prevention and control of AIDS. Thus, the cumulative number of reported cases of HIV/AIDS from the period 1985-2013, and the incidence rate of AIDS in 2013 were determined. Spatial autocorrelation analysis and hotspot analysis were conducted using ArcGIS10.2 to explore the spatial distribution of the HIV/AIDS epidemic. Both the thematic map and the global spatial autocorrelation Moran's I statistics revealed a clustered distribution of the spatial pattern. A local spatial autocorrelation analysis indicated hotspots of AIDS incidence rate that were confined to the provinces of Guangxi, Yunnan and Sichuan. The hotspots encompassed Guangxi and Yunnan, while Henan Province displayed a negative autocorrelation with more variable numbers that included neighbouring regions. The Getis-Ord Gi* statistics identified 6 hotspots and 8 coldspots for the incidence of AIDS, and 7 hotspots and 1 coldspot for the cumulative number of reported cases of HIV/AIDS. The spatial distribution pattern of the HIV/AIDS epidemic in China is clustered, demonstrating hotspots located in the Southwest. Specific interventions targeting provinces with severe HIV/AIDS epidemic are urgently needed. PMID:27245799

  10. Progressive Outer Retinal Necrosis Combined with Vitreous Hemorrhage in a Patient with Acquired Immunodeficiency Syndrome

    PubMed Central

    You, Yong Sung; Lee, Sung Jin; Lee, Sung Ho; Park, Chang Hyun

    2007-01-01

    Purpose To describe an unusual case of rapidly progressive outer retinal necrosis (PORN) with vitreous hemorrhage in a 41-year-old woman with acquired immunodeficiency syndrome (AIDS), who had retinitis developed from what was probably varicellar-zoster virus combined with cytomegalovirus (CMV) and herpes simplex type 1,2, as proven by the polymerase chain reaction restriction fragment length polymorphism method (PCR-RFLP). Methods This study is a case report detailing clinical follow-up and an aqueous humor test by PCR-RFLP. Results The deep, white retinal lesions coalesced and progressively expanded in a circumferential manner, with sparing of the perivascular retina. However, retinal and vitreous hemorrhages, unusual findings for PORN, could be noted around the optic nerve. Varicellar-zoster virus (VZV), cytomegalovirus (CMV), and herpes simplex types 1,2 (HSV-1,2) were detected in the aqueous humor by PCR. Conclusions PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS. Although the etiologic agent has been reported to be VZV, concurrent or combined etiologic agents can include HSV-1, HSV-2, and CMV in AIDS patients. Therefore, combined antiviral therapy with acyclovir and ganciclovir could be more reasonable as an initial therapy. PMID:17460434

  11. Infection and T lymphocyte subpopulations: changes associated with bacteremia and the acquired immunodeficiency syndrome.

    PubMed

    Fishman, J A; Martell, K M; Rubin, R H

    1983-01-01

    Patients with bacteremia, bacterial endocarditis, or acquired immunodeficiency syndrome (AIDS) were prospectively studied using monoclonal antibody reagents to assess alterations in T-lymphocyte subpopulations. Patients with endocarditis had significantly higher ratios of T-helper (OKT4+) to T-suppressor-cytotoxic (OKT8+) cells than did patients with bacteremia alone. Staphylococcus aureus endocarditis patients had a mean ratio of 8.49 (range 4.73-22.36) while S aureus bacteremia had a mean ratio of 2.75 (range 2.15 to 3.21). Similar results were found with Staphylococcus epidermidis endocarditis (mean 1.62) and bacteremia (mean 1.23). Klebsiella pneumoniae endocarditis (5.10) and sepsis (4.32), and E coli bacteremia (2.15). Nine male patients with AIDS had markedly depressed ratios (mean 0.25, range 0.04 to 0.67) while eight male homosexuals with unexplained lymphadenopathy ("pre-AIDS") had normal or increased ratios. Bacteremic infections are associated with an increased OKT4+/OKT8+ ratio with the degree of increase dependent upon virulence, location, and duration of infection. The immunomodulating effects of infection are manifested in changes in T-cell subsets, and these measurements can be useful in clinical management. PMID:6094086

  12. Percutaneous needle lung aspiration for diagnosing pneumonitis in the patient with acquired immunodeficiency syndrome (AIDS).

    PubMed

    Wallace, J M; Batra, P; Gong, H; Ovenfors, C O

    1985-03-01

    Fourteen patients with acquired immunodeficiency syndrome (AIDS) or suspected AIDS underwent percutaneous needle lung aspiration (PNLA) for evaluation of 16 occurrences of acute pneumonitis. A 22-gauge spinal needle was passed 2 to 3 times in the area of greatest radiographic involvement under fluoroscopic guidance. The specimen was immediately placed on microscope slides for Gomori's methenamine silver and Papanicolaou staining. The needle was then flushed with sterile water for bacterial, Legionella, viral, mycobacterial, and fungal cultures, and for Legionella immunofluorescent staining. Diagnostic information was provided by 14 of the 16 procedures. Of 11 patients ultimately found to have P. carinii pneumonitis, PNLA specimens were diagnostic in 10 (91%). Infectious agents other than P. carinii also were identified by PNLA, including cytomegalovirus (4 cases), M. avium-intracellulare (1 case), and pyogenic bacteria (3 cases). Complications of PNLA were: pneumothorax in 7 cases (44%), 3 (19%) of which required chest tube evacuation; and minor hemoptysis (less than 50 ml) in 2. The PNLA can be a useful diagnostic procedure in the patient with AIDS and pneumonitis. It has the advantages of being less costly and time-consuming than fiberoptic bronchoscopy. It is, however, frequently complicated by pneumothorax, making it an inappropriate approach for patients with significant respiratory compromise. PMID:3872089

  13. Respiratory failure in patients with acquired immunodeficiency syndrome and Pneumocystis carinii pneumonia.

    PubMed

    Maxfield, R A; Sorkin, I B; Fazzini, E P; Rapoport, D M; Stenson, W M; Goldring, R M

    1986-05-01

    Seven patients with acquired immunodeficiency syndrome (AIDS) and Pneumocystis carinii pneumonia were studied to define the pathophysiology of their respiratory failure. The patients had fever, cough, dyspnea, hypoxemia, and diffuse infiltrates on chest x-ray. Biopsies revealed a spectrum of alveolar filling, interstitial edema and infiltration, and fibrosis. The patients were studied on mechanical ventilation to assess the effect of positive end-expiratory pressure (PEEP) and supplemental oxygen on shunt fraction. Mean anatomic shunt (measured on 100% oxygen) was 34 +/- 8%, which increased significantly (p less than .001) to 43 +/- 9% when the FIO2 was decreased to 40% to 60% (physiologic shunt), indicating ventilation/perfusion (V/Q) imbalance or impaired diffusion. Increasing PEEP by 9 +/- 2 cm H2O reduced the anatomic shunt to 30 +/- 7% (p less than .01) and the physiologic shunt to 37 +/- 7% (p less than .02). There was a similar decrease in anatomic and physiologic shunts in five studies, a greater decrease in physiologic shunt in four, and a greater decrease in anatomic shunt in two. Evidence of alveolar recruitment with PEEP, measured by an increase in static thoracic compliance, was found in only one study. There was no correlation between the effect of PEEP on compliance and its effect on shunt. The data suggest that in patients with AIDS and P. carinii pneumonia, PEEP can decrease shunt by reducing the anatomic shunt, improving V/Q imbalance, and converting areas of anatomic shunt to areas of low V/Q. P. carinii pneumonia in patients with AIDS can produce a clinical and pathophysiologic pattern similar to that described in the adult respiratory distress syndrome. PMID:3516574

  14. Lymphocytic interstitial pneumonitis: a cause of pulmonary gallium-67 uptake in a child with acquired immunodeficiency syndrome

    SciTech Connect

    Zuckier, L.S.; Ongseng, F.; Goldfarb, C.R.

    1988-05-01

    Lymphocytic interstitial pneumonitis (LIP) is currently recognized as a frequent pediatric manifestation of the acquired immunodeficiency syndrome (AIDS). We report the gallium scan findings in a 3-yr-old girl with this disorder and review its clinical, radiologic, and pathologic features. LIP must be a prime consideration in the differential diagnosis of diffuse pulmonary gallium uptake in pediatric AIDS patients. Further experience will afford greater perspective on the diagnostic role that nuclear medicine will ultimately play in this disease. 49 references.

  15. Lymphoma Secondary to Congenital and Acquired Immunodeficiency Syndromes at a Turkish Pediatric Oncology Center.

    PubMed

    Tanyildiz, Hikmet G; Dincaslan, Handan; Yavuz, Gulsan; Unal, Emel; Ikinciogulları, Aydan; Dogu, Figen; Tacyildiz, Nurdan

    2016-10-01

    The prevalence of lymphoma in primary immunodeficiency cases and autoimmune diseases, as well as on a background of immunodeficiency following organ transplants, is increasing. The lymphoma treatment success rate is known to be a low prognosis. Our study aimed to emphasize the low survival rates in immunodeficient vs. immunocompetent lymphoma patients and also to investigate the effect of rituximab in patients with ataxia telangiectasia and other immunodeficiencies. We summarized the clinical characteristics and treatment results of 17 cases with primary immunodeficiency that developed non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL) retrospectively. Seven patients were diagnosed with ataxia-telangiectasia, two with common variable immunodeficiency, two with selective IgA deficiency, one with X-related lymphoproliferative syndrome, one with Wiskott-Aldrich syndrome, one with Epstein-Barr virus-related lymphoproliferative syndrome, one with interleukin-2-inducible T-cell kinase (ITK) deficiency, and one with lymphoma developing after autoimmune lymphoproliferative syndrome (ALPS). One patient underwent a renal transplant. Of the nine males and eight females (aged 3-12 years, median = 7) that developed lymphoma, seven were diagnosed with HL and ten with NHL (seven B-cell, three T-cell). The NHL patients were started on the Berlin-Frankfurt-Münster, POG9317, LMB-96, or R-CHOP treatment protocols with reduced chemotherapy dosages. HL cases were started on the doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) and/or cyclophosphamide, vincristine, procarbazine, and prednisone (COPP) protocol, also with modified dosages. Importantly, all seven cases of HL are alive and in remission, while six of the ten NHL patients have died. Primary immunodeficiency is a strong predisposing factor for developing lymphoma. Low treatment success rates relative to other lymphomas and difficulties encountered during treatment indicate that new treatment agents are needed

  16. Acquired immunodeficiency syndrome-associated cancers in Sub-Saharan Africa.

    PubMed

    Thomas, J O

    2001-04-01

    Sub-Saharan Africa is considered home to more than 60% of all human immunodeficiency virus (HIV) infected cases, with an estimated adult prevalence of 8.0%. It is stated that this region has contributed more than 90% of childhood deaths related to HIV infection and about 93% of childhood acquired immunodeficiency syndrome (AIDS)-related deaths. Although no country in Africa is spared of the infection, the bulk is seen in East and South Africa, with the highest recorded rates of 20% to 50% in Zimbabwe. On the other hand, West Africa is less affected, while countries in Central Africa have relatively stable infection rates. Although infections, especially tuberculosis, have emerged as the most important HIV/AIDS-associated killers in recent times, AIDS-associated malignancies are increasingly identified in the late stages. As a result of incomplete data from African countries, it is unclear whether the epidemiology and risks of these cancers are the same as observed in the developed countries. Since the advent of AIDS, epidemic Kaposi's sarcoma (KS) has become more common in both sexes in Africa, with a dramatic lowering of the male to female ratio from 19:1 to 1.7:1, especially in East Africa. Although there has been a rising trend of AIDS-associated non-Hodgkin's lymphoma (NHL) worldwide, there is an apparently lower risk in Africa compared with that in the developing world. At present, there is no strong evidence linking increased incidence of invasive cervical cancer to the HIV epidemic; however, some studies have demonstrated an association between HIV and the increased prevalence of human papilloma virus (HPV) and cervical intraepithelial neoplasia (CIN). On the other hand, HIV infection is now established as a risk factor for the development of squamous cell neoplasia of the conjunctiva based on studies from Rwanda, Malawi, and Uganda. Despite the problems and limitations of information from sub-Saharan Africa, interesting trends of HIV/AIDS-related cancers

  17. Investigation of chronic diarrhoea in acquired immunodeficiency syndrome. A prospective study of 155 patients.

    PubMed Central

    Blanshard, C; Francis, N; Gazzard, B G

    1996-01-01

    BACKGROUND AND AIMS: The optimum diagnostic investigation for patients with acquired immunodeficiency syndrome (AIDS) and diarrhoea is not known. Often no pathogen is detected and it is unclear whether this is because pathogens are absent in some patients or the investigations used fail to detect them. The hypothesis that AIDS related diarrhoea is usually due to an infection, which can be identified by a simple diagnostic strategy based on the results of intensive investigation of a cohort of such patients, was investigated. METHODS: 155 patients with AIDS and chronic diarrhoea underwent contemporaneous examination of stools, duodenal, jejunal, and rectal biopsy specimens and duodenal aspirate for bacterial, protozoal, and viral pathogens. A decision tree analysis was used to determine the best sequential diagnostic strategy for clinicians. RESULTS: 128 of 155 patients investigated (83%) had at least one potential pathogen. The presenting clinical features could not predict the presence or site of the pathogens. Stool analysis identified the most pathogens (93 of 199, 47%). Rectal biopsy was essential for the diagnosis of cytomegalovirus and adenovirus. Duodenal biopsy was as helpful as jejunal biopsy and detected some treatable pathogens missed by other methods. Electron microscopy, impression smears, and duodenal aspirate yielded little extra information. If gut biopsy was reserved for patients without a stool pathogen, some treatable pathogens would have been missed. CONCLUSION: Most patients with AIDS and chronic diarrhoea have at least one gut pathogen, which can be identified by stool analysis and light microscopic examination of duodenal and rectal biopsies. Some pathogens will be missed unless all these investigations are done on all such patients. PMID:9038664

  18. What High School Students Who Are Mildly Mentally Retarded Know about the Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome.

    ERIC Educational Resources Information Center

    Cobb, Hazel B.; Horn, Charles J., Jr.

    Alabama high school students (N=309) with mild mental retardation completed a questionnaire concerning their knowledge, attitudes, and sources of information about human immune deficiency virus/acquired immune deficiency syndrome (HIV/AIDS). Students demonstrated some basic knowledge of HIV/AIDS, and expressed some concern about getting AIDS. They…

  19. Risk behavior-based model of the cubic growth of acquired immunodeficiency syndrome in the United States.

    PubMed Central

    Colgate, S A; Stanley, E A; Hyman, J M; Layne, S P; Qualls, C

    1989-01-01

    The cumulative number of cases of acquired immunodeficiency syndrome (AIDS) in the United States has grown as the cube of time rather than exponentially. We explain this by interactions involving partner choice and sexual frequency in a risk-behavior model with biased mixing. This leads to a saturation wave of infection moving from high- to low-risk groups. If this description is correct, then the decreasing growth rate of AIDS cases is not due to behavior changes; rather it is due to the intrinsic epidemiology of the disease. PMID:2543987

  20. Transdermal testosterone administration in women with acquired immunodeficiency syndrome wasting: a pilot study.

    PubMed

    Miller, K; Corcoran, C; Armstrong, C; Caramelli, K; Anderson, E; Cotton, D; Basgoz, N; Hirschhorn, L; Tuomala, R; Schoenfeld, D; Daugherty, C; Mazer, N; Grinspoon, S

    1998-08-01

    Although human immunodeficiency virus (HIV) disease is increasing rapidly among women, no prior studies have investigated gender-based therapeutic strategies for the treatment of acquired immunodeficiency syndrome (AIDS) and its complications in this population. Markedly decreased serum androgen levels have been demonstrated in women with AIDS and may be a contributing factor to the wasting syndrome in this population. To assess the effects of androgen replacement therapy in women with AIDS wasting, we conducted a randomized, placebo-controlled, pilot study of transdermal testosterone administration. The primary aim of the study was to determine efficacy in terms of the change in serum testosterone levels, safety parameters and tolerability. A secondary aim of the study was to investigate testosterone effects on weight, body composition, quality of life, and functional indexes. Fifty-three ambulatory women with the AIDS wasting syndrome defined as weight less than 90% of ideal body weight or weight loss of more than 10% of the preillness maximum, free of new opportunistic infection within 6 weeks of study initiation, and with screening serum levels of free testosterone less than the mean of the normal reference range (< 3 pg/mL) were enrolled in the study. Subjects were age 37 +/- 1 yr old (mean +/- SEM), weighed 92 +/- 2% of ideal body weight, and had lost 17 +/- 1% of their maximum weight. CD4 count was 324 +/- 36 cells/mm3, and viral burden was 102,382 +/- 28,580 copies. Subjects were randomized into three treatment groups, in which two placebo patches (PP), one active/one placebo patch (AP group), or two active patches (AA group) were applied twice weekly to the abdomen for 12 weeks. The expected nominal delivery rates of testosterone were 150 and 300 microg/day, respectively, for the AP and AA groups. Forty-five subjects completed the study (PP group, n = 13; AP group, n = 14; AA group, n = 18). Two additional subjects from the PP group and two from the AP

  1. Recursion-based depletion of human immunodeficiency virus-specific naive CD4(+) T cells may facilitate persistent viral replication and chronic viraemia leading to acquired immunodeficiency syndrome.

    PubMed

    Tsukamoto, Tetsuo; Yamamoto, Hiroyuki; Okada, Seiji; Matano, Tetsuro

    2016-09-01

    Although antiretroviral therapy has made human immunodeficiency virus (HIV) infection a controllable disease, it is still unclear how viral replication persists in untreated patients and causes CD4(+) T-cell depletion leading to acquired immunodeficiency syndrome (AIDS) in several years. Theorists tried to explain it with the diversity threshold theory in which accumulated mutations in the HIV genome make the virus so diverse that the immune system will no longer be able to recognize all the variants and fail to control the viraemia. Although the theory could apply to a number of cases, macaque AIDS models using simian immunodeficiency virus (SIV) have shown that failed viral control at the set point is not always associated with T-cell escape mutations. Moreover, even monkeys without a protective major histocompatibility complex (MHC) allele can contain replication of a super infected SIV following immunization with a live-attenuated SIV vaccine, while those animals are not capable of fighting primary SIV infection. Here we propose a recursion-based virus-specific naive CD4(+) T-cell depletion hypothesis through thinking on what may happen in individuals experiencing primary immunodeficiency virus infection. This could explain the mechanism for impairment of virus-specific immune response in the course of HIV infection. PMID:27515208

  2. Toxoplasma encephalitis in Haitian adults with acquired immunodeficiency syndrome: a clinical-pathologic-CT correlation

    SciTech Connect

    Post, M.J.D.; Chan, J.C.; Hensley, G.T.; Hoffman, T.A.; Moskowitz, L.B.; Lippmann, S.

    1983-05-01

    The clinical data, histologic findings, and computed tomographic (CT) abnormalities in eight adult Haitians with toxoplasma encephalitis were analyzed retrospectively. Diagnosis was established by identification of Toxoplasma gondii on autopsy in five and brain biopsy in three specimens and subsequently confirmed by the immunoperoxidase method. All these patiens, six of whom had been in the United States for 24 months or less, had severe idiopathic immunodeficiency syndrome. All were lymphopenic and six were on treatment for tuberculosis when the toxoplasma encephalitis developed. All patients were studied with CT when they developed an altered mental status and fever associated with seizures and/or focal neurologic deficits. Scans before treatment showed multiple intraparenchymal lesions in seven and a single lesion in the thalamus in one. Ring and/or nodular enhancement of the lesions was found in six and hypodense areas in two. Progressions of abnormalities occurred on serial studies. These CT findings that were best shown on axial and coronal thin-section double-dose contrast studies were useful but not diagnostically pathognomonic. In patients with similar clinical presentation CT is recommended to identify focal areas of involvement and to guide brain biopsy or excision so that prompt medical thereapy of this often lethal infection can be instituted.

  3. Lung and chest wall mechanics in patients with acquired immunodeficiency syndrome and severe Pneumocystis carinii pneumonia.

    PubMed

    D'Angelo, E; Calderini, E; Robatto, F M; Puccio, P; Milic-Emili, J

    1997-10-01

    The aim of this study was to assess the mechanical characteristics of the respiratory system in patients with acquired immune deficiency syndrome (AIDS) and acute respiratory distress syndrome (ARDS) caused by Pneumocystis carinii pneumonia (PCP). In 12 mechanically ventilated patients, total respiratory system mechanics was assessed using the technique of rapid airway occlusion during constant flow inflation, and was partitioned into lung and chest wall components using the oesophageal balloon technique. We measured interrupter resistance (Rint), which mainly reflects airway resistance, additional resistance (deltaR) due to viscoelastic behaviour and time constant inequalities, and static elastance (Est). In addition, the static inflation volume-pressure (V-P) curve was assessed. In eight patients, computed tomography scans were performed within 2 days of the assessment of respiratory mechanics. Compared to values reported in the literature for normal subjects, Est and deltaR were markedly increased in AIDS patients with PCP, whilst Rint exhibited a relatively smaller increase. These changes, which involved only the lung and airways, were mainly due to the reduction of ventilated lung units, but additional factors were involved to cause independent modifications of lung stiffness, airway calibre, and viscoelastic properties. The changes in Rint, deltaR, and Est were similar to those observed in other studies on patients with ARDS of different aetiologies. At variance with common observations in the latter patients, none of the AIDS patients with PCP exhibited an inflection point on the static inflation V-P curve, suggesting little or no alveolar recruitment during lung inflation. This finding could be related to the distinctive histopathology of Pneumocystis carinii pneumonia. Indeed, computed tomography revealed homogeneous diffuse interstitial and alveolar infiltration rather than the dense, dependent opacities observed in other studies on acute respiratory

  4. Intersection of Smoking, Human immunodeficiency virus/acquired immunodeficiency syndrome and Cancer: Proceedings of the 8th Annual Texas Conference on Health Disparities

    PubMed Central

    Rajendiran, Smrithi; Kashyap, Meghana V.; Vishwanatha, Jamboor K.

    2013-01-01

    The Texas Center for Health Disparities, a National Institute on Minority Health and Health Disparities Center of Excellence, presents an annual conference to discuss prevention, awareness education and ongoing research about health disparities both in Texas and among the national population. The 2013 Texas Conference on Health Disparities brought together experts, in research, patient care and community outreach, on the “Intersection of Smoking, Human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) and Cancer”. Smoking, HIV/AIDS and cancer are three individual areas of public health concern, each with its own set of disparities and risk factors based on race, ethnicity, gender, geography and socio-economic status. Disparities among patient populations, in which these issues are found to be comorbid, provide valuable information on goals for patient care. The conference consisted of three sessions addressing “Comorbidities and Treatment”, “Public Health Perspectives”, and “Best Practices”. This article summarizes the basic science, clinical correlates and public health data presented by the speakers. PMID:24227993

  5. Consensus on context-specific strategies for reducing the stigma of human immunodeficiency virus/acquired immunodeficiency syndrome in Zambézia Province, Mozambique

    PubMed Central

    Mukolo, Abraham; Torres, Isabel; Bechtel, Ruth M.; Sidat, Mohsin; Vergara, Alfredo E.

    2014-01-01

    Stigma has been implicated in poor outcomes of human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) care. Reducing stigma is important for HIV prevention and long-term treatment success. Although stigma reduction interventions are conducted in Mozambique, little is known about the current nature of stigma and the efficacy and effectiveness of stigma reduction initiatives. We describe action research to generate consensus on critical characteristics of HIV stigma and anti-stigma interventions in Zambézia Province, Mozambique. Qualitative data gathering methods, including indepth key-informant interviews, community interviews and consensus group sessions, were utilized. Delphi methods and the strategic options development analysis technique were used to synthesize qualitative data. Key findings are that stigma enacted by the general public might be declining in tandem with the HIV/AIDS epidemic in Mozambique, but there is likely excessive residual fear of HIV disease and community attitudes that sustain high levels of perceived stigma. HIV-positive women accessing maternal and child health services appear to shoulder a disproportionate burden of stigma. Unintentional biases among healthcare providers are currently the critical frontier of stigmatization, but there are few interventions designed to address them. Culturally sensitive psychotherapies are needed to address psychological distress associated with internalized stigma and these interventions should complement current supports for voluntary counseling and testing. While advantageous for defining stakeholder priorities for stigma reduction efforts, confirmatory quantitative studies of these consensus positions are needed before the launch of specific interventions. PMID:24527744

  6. Behaviors Influencing Human Immunodeficiency Virus Transmission in the Context of Positive Prevention among People Living with HIV/Acquired Immunodeficiency Syndrome in Iran: A Qualitative Study

    PubMed Central

    Radfar, Seyed Ramin; Sedaghat, Abbas; Banihashemi, Arash Tehrani; Gouya, Mohammadmehdi; Rawson, Richard A.

    2014-01-01

    Background: Identifying factors, which influence health behaviors is critical to designing appropriate and effective preventive programs. Human immunodeficiency virus (HIV) transmission is highly related to people behaviors and understanding factors influencing healthy behaviors among Iranian people living with HIVs (PLHIVs)/acquired immunodeficiency syndrome (AIDS) is very important to tailor an effective response to HIV/AIDS epidemic. Methods: This study was conducted as a qualitative study by methods of focus group discussion and in-depth interview in six provinces of Iran with 64 PLHIVs to determine factors influence engagement in positive prevention. Results: Knowledge and education, feelings of responsibility and positive prevention practices were identified as the primary domains of engagement. These domains were found to be influenced by feelings of ostracism and frustration, poverty, barriers to disclosure of HIV status, access to and utilization of drug abuse treatment services and antiretroviral therapy, adherence to treatment, age, religiousness, sex work, singleness, and incarceration. Conclusions: Designing new interventions and updating current interventions directed toward the aforementioned factors should be addressed by responsible Iranian authorities in order to have a national effective response on the HIV/AIDS epidemic. PMID:25489445

  7. Purinergic signaling and human immunodeficiency virus/acquired immune deficiency syndrome: From viral entry to therapy

    PubMed Central

    Passos, Daniela F; Schetinger, Maria Rosa C; Leal, Daniela BR

    2015-01-01

    Human immunodeficiency virus (HIV) infection is a serious condition associated to severe immune dysfunction and immunodeficiency. Mechanisms involved in HIV-associated immune activation, inflammation and loss of CD4+ T cells have been extensively studied, including those concerning purinergic signaling pathways. Purinergic signaling components are involved in viral entry and replication and disease progression. Research involving the participation of purinergic signaling in HIV infection has been not only important to elucidate disease mechanisms but also to introduce new approaches to therapy. The involvement of purinergic signaling in the pathogenesis of HIV infection and its implications in the control of the HIV infection are reviewed in this paper. PMID:26279989

  8. Attitudes of Baccalaureate Nursing Students toward Persons with Acquired Immunodeficiency Syndrome According to Mode of Human Immunodeficiency Virus Transmission.

    ERIC Educational Resources Information Center

    West, Andrea M.; And Others

    1996-01-01

    Nursing students (n=236) completed the AIDS Knowledge Scale and AIDS Attitude Scale. Results showed most stigma attached to AIDS acquired through drug use or sexual contact, the least through maternal transmission or blood transfusion. Demographic characteristics did not influence attitudes. (SK)

  9. Food Security in Households of People Living With Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome: A Cross-sectional Study in a Subdivision of Darjeeling District, West Bengal

    PubMed Central

    2016-01-01

    Objectives: Human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) adversely impacts food security in households of people living with HIV/AIDS (PLWHA). Little research has focused on food insecurity among PLWHA in India. The purpose of this study was to identify the prevalence of and factors relating to food security in households of PLWHA in the Siliguri subdivision of Darjeeling, West Bengal, India. Methods: A cross-sectional community-based study was carried out among 173 PLWHA residing in Siliguri and registered at the Anti-retroviral Therapy Centre of North Bengal Medical College & Hospital. Data was collected at the household level with interviews of PLWHA using a food security survey instrument. We analyzed the associations using logistic regression. Results: The prevalence of household food security among the participants was 50.9% (88/173). Five years or more of schooling, higher socioeconomic class and males were found to be significantly associated with a higher likelihood of food security. A later stage of the disease and the presence of other family members with HIV/AIDS were significantly associated with a lower likelihood of food security. The major coping strategies to deal with food insecurity in the acute phase HIV infection included borrowing money (56.1%), followed by spousal support, loans from microfinance institutions, banks, or money lenders, borrowing food, or selling agricultural products. Conclusions: The present study revealed that only about half of households with PLWHA were food secure. Prior interventions relating to periods of food and economic crisis as well as strategies for sustaining food security and economic status are needed in this area. PMID:27499166

  10. Thyrotoxicosis followed by Hypothyroidism due to Suppurative Thyroiditis Caused by Nocardia brasiliensis in a Patient with Advanced Acquired Immunodeficiency Syndrome.

    PubMed

    Teckie, G2; Bhana, S A; Tsitsi, J M L; Shires, R

    2014-03-01

    Acute thyroiditis is an extremely rare complication of nocardiosis. We report a patient with hyperthyroidism due to suppurative thyroiditis caused by Nocardia brasiliensis. A 38-year-old Black male presented with features of thyrotoxicosis, sepsis and airway obstruction. He had no evidence of underlying thyroid disease, but was severely immunocompromised as a result of acquired immunodeficiency syndrome. He had previously been diagnosed with pulmonary nocardiosis and also had nocardial abscesses on his anterior chest wall. Investigations revealed thyrotoxicosis, with a FT4 of 43.2 pmol/l and a suppressed TSH <0.01 mIU/l. Serum anti-thyroperoxidase and anti-thyroglobulin antibodies were absent. Computed tomography scan showed a large abscess in the anterior neck involving the left lobe and isthmus, as well as inhomogeneous changes in the right lobe of the thyroid. The radioisotopic scan showed absent uptake of tracer in keeping with thyroiditis. Although the initial presentation was that of hyperthyroidism, destruction of the gland later resulted in sustained hypothyroidism, necessitating thyroid hormone supplementation. The hyperthyroidism can be explained by the release of presynthesized and stored thyroid hormone into the circulation as a result of inflammation and disruption of the thyroid follicles, and the subsequent hypothyroidism by the fact that much of the gland was destroyed by the abscess and the extensive inflammatory process. This is the first documented case of hyperthyroidism in a patient with acute suppurative thyroiditis caused by Nocardia. PMID:24847469

  11. Influence of the greenhouse effect on human health through stratospheric cooling: Possible increase in acquired immunodeficient syndrome

    SciTech Connect

    Okamoto, Kazuto; Tsushima, Hiroshi; Tanimoto, Shin

    1996-09-01

    The greenhouse effect cools the stratosphere and increases formation of PSC (polar stratospheric cloud) in polar regions and enhances ozone depletion. If the enhanced ozone depletion diffused to lower latitudes, it could increase ultraviolet radiation (UV), which might increase acquired immunodeficiency syndrome (AIDS). Epidemiological studies are made to test this hypothesis. The relation between AIDS prevalence R and latitude {theta}. Comparison of analyses shows that R of Caucasians would be higher than Non-Caucasians at the same {theta}. These trends are similar to those of skin cancers known to be caused by UV. In developing countries poverty, malnutrition, etc., could cause high R, and since most developing countries are located at low {theta}, the low {theta} increase may be due to these factors. However if so in Africa they are about the same and the low {theta} increase would disappear, but data on African countries also show the low {theta} increase and the significant correlation. Some countries at low {theta} have low R, probably because HIV is not prevalent for them. Then the upper envelope of the distribution of R would be cases when HIV is prevalent and UV is most effective. Therefore analyses are repeated using maxima of R within intervals of {theta} of 1, 3 and 5{degree}. In all cases the low {theta} increase and the correlation becomes more significant. These results support the hypothesis that AIDS is promoted by UV.

  12. The hypertriglyceridemia of acquired immunodeficiency syndrome is associated with an increased prevalence of low density lipoprotein subclass pattern B

    SciTech Connect

    Feingold, K.R.; Krauss, R.M.; Pang, M.; Doerrler, W.; Jensen, P.; Grunfeld, C. Lawrence Berkeley Lab., CA )

    1993-06-01

    To better define the role of environmental factors on LDL phenotypic expression, the authors determined LDL patterns in patients with acquired immunodeficiency syndrome (AIDS), and infection characterized by hypertriglyceridemia and weight loss. Similar to previous studies, plasma triglyceride levels were increased, whereas plasma cholesterol, LDL cholesterol, and HDL cholesterol levels were decreased in the AIDS subjects compared to those in age-matched controls. The percentage of AIDS subjects with the LDL B phenotype was increased 2.5-fold, demonstrating an increased prevalence of the LDL B phenotype in an acquired form of hypertriglyceridemia. For each LDL phenotype in AIDS, serum triglyceride levels were higher than the same phenotypic pattern in controls, with the most marked elevations in triglycerides found in AIDS subjects with the LDL B phenotype. In contrast to what was observed in controls, HDL cholesterol levels were decreased in all AIDS subjects and were unrelated to LDL pattern. Total and LDL cholesterol levels were higher in controls with the LDL B phenotype than in those with the LDL A phenotype, but there was no difference in total and LDL cholesterol in AIDS subjects with LDL B compared to A. On multiple regression analysis in subjects with AIDS, plasma triglyceride levels, age, and HDL cholesterol all contribute to the occurrence of the LDL B phenotype, but elevations in plasma triglyceride levels are the strongest independent predictor. Body mass index was not a predictor of LDL B phenotype in AIDS. These results suggest that disturbances in triglyceride metabolism that are caused by AIDS lead to the appearance of the LDL subclass B phenotype and provide further evidence that environmental or disease states that perturb lipid metabolism can produce an increased prevalence of the LDL B phenotype. 35 refs., 1 fig., 5 tabs.

  13. The epidemiology of disseminated nontuberculous mycobacterial infection in the acquired immunodeficiency syndrome (AIDS).

    PubMed

    Horsburgh, C R; Selik, R M

    1989-01-01

    We analyzed cases of disseminated nontuberculous mycobacterial infection (DNTM) in patients with AIDS reported to the Centers for Disease Control. Between 1981 and 1987, 2,269 cases were reported. In 96% of cases, infection was caused by M. avium complex (MAC). The number of cases has risen steadily since 1981, but the rate as a percentage of AIDS cases has remained stable at 5.5%. DNTM was seen less frequently in AIDS cases with Kaposi's sarcoma than in other AIDS cases (p less than 0.01). Rates of DNTM were lower in Hispanics and declined with age but were not significantly different by patient sex or means of acquiring HIV infection. Rates of disseminated MAC varied by geographic region from 3.9% to 7.8% (p less than 0.0001). As assessed by helper/suppressor T-cell ratios, AIDS patients with DNTM were not more immunologically impaired than those with other opportunistic infections. Life table analysis revealed that AIDS patients with DNTM survived a shorter time (median, 7.4 months) than did other AIDS patients (median, 13.3 months; p less than 0.0001). We conclude that DNTM is acquired by unpreventable environmental exposures. Because DNTM adversely affects survival of AIDS patients, effective therapeutic agents must be vigorously sought. PMID:2912355

  14. Acquired immunodeficiency syndrome/human immunodeficiency virus knowledge, attitudes, and practices, and use of healthcare services among rural migrants: a cross-sectional study in China

    PubMed Central

    2014-01-01

    Background Today’s rapid growth of migrant populations has been a major contributor to the human immunodeficiency virus (HIV) epidemic. However, relatively few studies have focused on HIV/acquired immunodeficiency syndrome (AIDS)-related knowledge, attitudes, and practice among rural-to-urban migrants in China. This cross-sectional study was to assess HIV/AIDS-related knowledge and perceptions, including knowledge about reducing high-risk sex. Methods Two-phase stratified cluster sampling was applied and 2,753 rural migrants participated in this study. An anonymous self-administered questionnaire was conducted in Guangdong and Sichuan provinces in 2007. Descriptive analysis was used to present the essential characteristics of the respondents. Chi-square test and multiple logistic regression models were performed to examine the associations between identified demographic factors and high-risk sex, sexually transmitted disease (STD) symptoms, and access to HIV screening services among the seven types of workers. Results 58.6% of participants were knowledgeable about HIV/AIDS transmission, but approximately 90% had a negative attitude towards the AIDS patients, and that 6.2% had engaged in high-risk sex in the past 12 months. Logistic regression analysis revealed sex, marital status, income, migration and work experience to be associated with high-risk sex. Among the 13.9% of workers who reported having STD symptoms, risk factors that were identified included female gender, high monthly income, being married, daily laborer or entertainment worker, frequent migration, and length of work experience. Only 3% of migrant workers received voluntary free HIV screening, which was positively associated with monthly income and workplace. Conclusions HIV/AIDS knowledge, attitudes, and practices among rural migrants in China remain a thorny health issue, and use of healthcare services needs to be improved. Low levels of education and knowledge regarding HIV/AIDS among

  15. Acquired immunodeficiency syndrome among patients attending hemophilia treatment centers and mortality experience of hemophiliacs in the United States.

    PubMed

    Johnson, R E; Lawrence, D N; Evatt, B L; Bregman, D J; Zyla, L D; Curran, J W; Aledort, L M; Eyster, M E; Brownstein, A P; Carman, C J

    1985-06-01

    The acquired immunodeficiency syndrome (AIDS) was first recognized among hemophiliacs in 1982. The authors have conducted investigations to determine the onset and incidence of AIDS among hemophiliacs and to determine trends in hemophilia mortality since the introduction of clotting-factor concentrates in the late 1960s. A survey of United States hemophilia treatment centers, supported by the Centers for Disease Control and the National Hemophilia Foundation, defined a population of hemophiliacs which was monitored for AIDS cases through June 1984. Death reports from the United States Vital Statistics System and from the hemophilia treatment center survey provided mortality trends for 1968-1979 and for 1978-1982, respectively. The results of these investigations demonstrate the following points. 1) The AIDS epidemic is a new and important cause of illness and mortality among hemophiliacs, although a very low incidence of AIDS among hemophiliacs prior to 1982 cannot be ruled out. 2) The AIDS cases who attended the surveyed hemophilia treatment centers were distributed throughout the United States and were older than hemophilia treatment center patients without AIDS. AIDS cases also used more lyophilized clotting-factor concentrate, but only a small number of cases were reported with this information. 3) Improved care for hemophilia, including the use of clotting-factor concentrates, dramatically reduced hemophilia mortality rates during the 1970s. 4) In 1982, hemorrhage was the major cause of death among hemophiliacs. Deaths from non-alcoholic liver disease were also increased. AIDS incidence among hemophilia treatment center attendees was stable at 0.6 cases per 1,000 hemophilia treatment center attendees per year during 1982 and 1983 but increased sharply to 5.4 cases per 1,000 during the first quarter of 1984. PMID:4014173

  16. Measures to decrease the risk of acquired immunodeficiency syndrome transmission by blood transfusion. Evidence of volunteer blood donor cooperation.

    PubMed

    Pindyck, J; Waldman, A; Zang, E; Oleszko, W; Lowy, M; Bianco, C

    1985-01-01

    We studied whether volunteers giving blood to the Greater New York Blood Program (GNYBP) cooperated with procedures implementing public health recommendations intended to decrease the risk of acquired immunodeficiency syndrome (AIDS) transmission by blood transfusion. Predonation medical screening was expanded to exclude donors who might be ill with AIDS. To exclude possible asymptomatic carriers of the disease, members of groups at increased risk of AIDS were asked either not to give blood or to give it for laboratory studies. A confidential questionnaire, administered to all donors after medical screening, provided the vehicle for donors to advise the GNYBP whether their donation was for laboratory studies or for patient transfusion. We found that the number of male donors decreased; AIDS-related questions in medical history led to a 2 percent increase in donor rejections; 97 percent of donors said their blood could be used for transfusions; 1.4 percent said their blood could be used for laboratory studies only; and 1.6 percent did not respond. Only units designated for transfusion were released to hospitals. People who indicated that their donation was for laboratory studies had a higher prevalence of markers for hepatitis B virus and of antibodies to cytomegalovirus. White cell counts and helper/suppressor T lymphocyte ratios were not significantly different in the two groups. We conclude that volunteer donors have cooperated with the established procedures. None of the laboratory assays identified blood units donated by individuals who, based on information about AIDS high-risk groups, designated their donation for laboratory studies. PMID:3969698

  17. Acquired immunodeficiency syndrome — an assessment of the present situation in the world: Memorandum from a WHO Meeting*

    PubMed Central

    1984-01-01

    A consultative meeting was convened by the World Health Organization in Geneva on 22-25 November 1983 to assess the present situation of AIDS (the acquired immunodeficiency syndrome) in the world and to encourage collaboration between the different nations affected by this disease. AIDS was first reported in the USA in 1981, but probably existed there as early as 1978. Soon after its recognition in the USA, similar cases were identified in other areas of the world. In most western European countries and Canada, the epidemiological pattern is very similar to that in the United States, the majority of cases being in homosexual men. In other areas such as equatorial Africa and the Caribbean, the pattern seems to be different with no identifiable risk factors for the majority of cases. The disease is manifested by opportunistic infections and/or selected malignancies, with apparent differences in the clinical presentation between the cases in North America and Europe, on the one hand, and those in the tropics. To date there is no treatment that has significantly improved the underlying cellular immune deficiency, and the mortality is very high. The etiology of AIDS is unknown, but the epidemiological pattern is most consistent with its being caused by a transmissible agent; retroviruses come on top of the list of candidate agents. Despite the unknown etiology and the lack of laboratory diagnostic tests, sufficient information is available to permit health authorities to make recommendations that may reduce appreciably the incidence of the disease. AIDS is an important health problem in a number of countries and has international implications. Collaborative laboratory, epidemiological and clinical research between countries is needed to accelerate control efforts. In the meantime, WHO will coordinate exchange of information among countries. ImagesFig. 1 PMID:6331905

  18. Incidence and Long-term Outcomes of the HIV-Neuroretinal Disorder in Patients with the Acquired Immunodeficiency Syndrome

    PubMed Central

    Jabs, Douglas A.; Drye, Lea; Van Natta, Mark L.; Thorne, Jennifer E.; Holland, Gary N.

    2014-01-01

    Objectives Patients with the acquired immunodeficiency syndrome (AIDS) have an abnormality of retina/optic nerve function, manifested as decreased contrast sensitivity (in the absence of ocular opportunistic infections or media opacity), abnormalities on automated perimetry, and loss of retinal nerve fiber layer, even among those with good visual acuity, termed the HIV-neuroretinal disorder. The objectives of this study were to determine the prevalence, incidence, risk factors for, and outcomes of HIV-neuroretinal disorder. Design Prospective cohort study Participants 1822 patients with AIDS without ocular infections or media opacities. Methods Patients with HIV-neuroretinal disorder were identified by a contrast sensitivity < 1.50 log units in either eye in the absence of ocular opportunistic infections or media opacity. Main outcome measures Incidence of HIV-neuroretinal disorder, mortality, visual impairment (visual acuity 20/50 or worse), and blindness (20/200 or worse) on logarithmic visual acuity charts. Results Sixteen percent of participants had HIV-neuroretinal disorder at enrollment. The estimated cumulative incidence by 20 years after AIDS diagnosis was 51% (95% confidence interval [CI] 46%–55%). HIV-neuroretinal disorder was more common in women and African American persons. Risk factors for it included hepatitis C infection, low CD4+ T cells, and detectable HIV RNA in the blood. Patients with HIV neuroretinal disorder had a 70% excess mortality vs. those without it, even after adjusting for CD4+ T cells and HIV load (hazard ratio=1.7, 95% CI= 1.3–2.1, P<0.0001). Patients with HIV-neuroretinal disorder had increased risks of bilateral visual impairment (hazard ratio=6.5, 95% CI=2.6–10.6, P<0.0001) and blindness (hazard ratio=5.9, 95% CI=2.8–13.7, P=0.01) vs. those without HIV neuroretinal disorder. Conclusions HIV-neuroretinal disorder is a common finding among patients with AIDS, and it is associated with an increased mortality and an increased

  19. Immunophenotypic characterization of the cutaneous exanthem of SIV-infected rhesus monkeys. Apposition of degenerative Langerhans cells and cytotoxic lymphocytes during the development of acquired immunodeficiency syndrome.

    PubMed Central

    Ringler, D. J.; Hancock, W. W.; King, N. W.; Letvin, N. L.; Daniel, M. D.; Desrosiers, R. C.; Murphy, G. F.

    1987-01-01

    A T-cell tropic retrovirus, simian immunodeficiency virus (SIV), has recently been isolated from immunodeficient rhesus monkeys. This virus has remarkable similarities to human immunodeficiency virus (HIV), the etiologic agent of acquired immunodeficiency syndrome. Subsequent studies of simian infection with SIV have shown it to be a relevant animal model for studying the pathogenesis of AIDS in man. In both HIV-infected humans and SIV-infected monkeys, a cutaneous maculopapular eruption has been described. To date, the pathogenesis and possible relationship of these exanthema to the evolution of systemic immunosuppression have remained obscure. In this study, the mononuclear cell infiltrates that characterize skin rashes of SIV-infected rhesus monkeys were found to be composed predominantly of cells with phenotypic characteristics of cytotoxic/suppressor (T8+) lymphocytes and natural killer cells. Many of these cells expressed membrane-bound interleukin-2 receptor molecules. Double labeling and immunoelectron microscopy revealed these cells in direct contact with degenerative Langerhans cells within the epidermis and dermis. These observations suggest that the cutaneous rash associated with SIV infection may be the consequence of target cell injury of Langerhans cells by effector cells with cytotoxic potential. Images Figure 1 Figure 2 Figure 3 PMID:3030113

  20. Loss of lean body and muscle mass correlates with androgen levels in hypogonadal men with acquired immunodeficiency syndrome and wasting.

    PubMed

    Grinspoon, S; Corcoran, C; Lee, K; Burrows, B; Hubbard, J; Katznelson, L; Walsh, M; Guccione, A; Cannan, J; Heller, H; Basgoz, N; Klibanski, A

    1996-11-01

    The acquired immunodeficiency syndrome (AIDS) wasting syndrome (AWS) is a devastating complication of human immunodeficiency virus infection characterized by a disproportionate decrease in lean body mass. The pathogenesis of the AWS is unknown, but recent data suggest that endogenous secretion of the potent anabolic hormone, testosterone; is decreased in 30-50% of men with AIDS. However, it is unknown whether decreased androgen levels are associated with decreased lean body mass and/or functional decreases in muscle strength and aerobic capacity in hypogonadal men with the AWS. In addition, testosterone is known to have stimulatory effects on GH secretion, and the loss of these effects on the GH-insulin-like growth factor I (IGF-I) axis may be an additional mechanism of decreased lean body mass in this population. Twenty hypogonadal subjects (free-testosterone < 12 pg/mL) with weight loss > 10% of preillness weight or absolute weight < 90% ideal body weight (IBW) were enrolled in the study. None of the subjects were receiving Megace. Lean body mass and fat-free mass were determined by potassium-40 isotope analysis (40K) and dual-energy x-ray absorptiometry, respectively, and analyzed with respect to gonadal function by linear regression analysis. Muscle mass was determined by urinary creatinine excretion, and exercise functional capacity was assessed by a 6-min walk test, a sit-to-stand test, and a timed get-up-and-go test. Results also were compared with gonadal function by regression analysis. IGF-I and mean overnight GH levels, determined from frequent sampling (q20 min from 2000-0800 h), were compared with results obtained from age- and sex-matched normal controls. Subjects were 26-58 yr of age (39 +/- 7 yr, mean +/- SD) with a CD4 cell count of 150 +/- 186 cells/mm3. Serum levels of FSH were elevated in 30% of the subjects. Muscle mass was significantly reduced, compared with expected mass for height (23.3 +/- 5.5 vs. 29.3 +/- 1.7 kg, P = 0.0001) and was

  1. Prevalence and Predictors of Thyroid Dysfunction in Patients with HIV Infection and Acquired Immunodeficiency Syndrome: An Indian Perspective

    PubMed Central

    Sharma, Neera; Sharma, Lokesh Kumar; Dutta, Deep; Gadpayle, Adesh Kisanji; Anand, Atul; Gaurav, Kumar; Mukherjee, Sabyasachi; Bansal, Rahul

    2015-01-01

    Background. Predictors of thyroid dysfunction in HIV are not well determined. This study aimed to determine the prevalence and predictors of thyroid dysfunction in HIV infected Indians. Methods. Consecutive HIV patients, 18–70 years of age, without any severe comorbid state, having at least 1-year follow-up at the antiretroviral therapy clinic, underwent clinical assessment and hormone assays. Results. From initially screened 527 patients, 359 patients (61.44 ± 39.42 months' disease duration), having good immune function [CD4 count >200 cell/mm3: 90.25%; highly active antiretroviral therapy (HAART): 88.58%], were analyzed. Subclinical hypothyroidism (ScH) was the commonest thyroid dysfunction (14.76%) followed by sick euthyroid syndrome (SES) (5.29%) and isolated low TSH (3.1%). Anti-TPO antibody (TPOAb) was positive in 3.90%. Baseline CD4 count had inverse correlation with TPOAb after adjusting for age and body mass index. Stepwise linear regression revealed baseline CD4 count, TPOAb, and tuberculosis to be best predictors of ScH after adjusting for age, weight, duration of HIV, and history of opportunistic fungal and viral infections. Conclusion. Burden of thyroid dysfunction in chronic HIV infection with stable immune function is lower compared to pre-HAART era. Thyroid dysfunction is primarily of nonautoimmune origin, predominantly ScH. Severe immunodeficiency at disease onset, TPOAb positivity, and tuberculosis were best predictors of ScH. PMID:26798547

  2. Spectrum of Adrenal Dysfunction in Patients with Acquired Immunodeficiency Syndrome Evaluation of Adrenal and Pituitary Reserve with ACTH and Corticotropin-Releasing Hormone Testing.

    PubMed

    Freda, P U; Papadopoulos, A D; Wardlaw, S L; Goland, R S

    1997-07-01

    Patients with acquired immunodeficiency syndrome (AIDS) have been reported to develop abnormalities of the endocrine system and in particular of the hypothalamic-pituitary-adrenal (HPA) axis. To define the abnormalities of HPA function in AIDS patients better, we performed ACTH and ovine corticotropin-releasing hormone (oCRH) testing in a group of AIDS patients and oCRH testing in a group of healthy subjects. Our study found that in AIDS patients with normal ACTH testing, oCRH testing revealed a variety of subclinical abnormalities of ACTH and cortisol responses. Although we did not find frank adrenal insufficiency in any of these AIDS patients, it remains to be determined if any of the subclinical abnormalities we identified are predictive of clinically significant adrenal insufficiency; it may be that as AIDS patients live longer, the subclinical abnormalities will progress to adrenal insufficiency. (Trends Endocrinol Metab 1997;8:173-180). (c) 1997, Elsevier Science Inc. PMID:18406803

  3. Effect of traditional Chinese medicine for treating human immunodeficiency virus infections and acquired immune deficiency syndrome: Boosting immune and alleviating symptoms.

    PubMed

    Zou, Wen; Wang, Jian; Liu, Ying

    2016-01-01

    To respond to the human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) epidemic in China, the integration of antiretroviral therapy (ART) and traditional Chinese medicine (TCM) has important implications in health outcomes, especially in China where the use of TCM is widespread. The National Free TCM Pilot Program for HIV Infected People began in 5 provinces (Henan, Hebei, Anhui, Hubei, and Guangdong) in 2004, and quickly scaled up to 19 provinces, autonomous regions, and municipalities in China including some places with high prevalence, 26,276 adults have been treated thus far. Usually, people with HIV infection seek TCM for four main reasons: to enhance immune function, to treat symptoms, to improve quality of life, and to reduce side effects related to medications. Evidences from randomized controlled clinical trials suggested some beneficial effects of use of traditional Chinese herbal medicine for HIV infections and AIDS. More proofs from large, well-designed, rigorous trials is needed to give firm support. Challenges include interaction between herbs and antiretroviral drugs, stigma and discrimination. The Free TCM Program has made considerable progress in providing the necessary alternative care and treatment for HIV-infected people in China, and has strong government support for continued improvement and expansion, establishing and improving a work mechanism integrating Chinese and Western medicines. PMID:26577109

  4. Clonal analysis of T lymphocytes in the acquired immunodeficiency syndrome. Evidence for an abnormality affecting individual helper and suppressor T cells.

    PubMed Central

    Margolick, J B; Volkman, D J; Lane, H C; Fauci, A S

    1985-01-01

    Purified helper-inducer (T4+) and suppressor-cytotoxic (T8+) lymphocytes from eight patients with acquired immunodeficiency syndrome (AIDS) and eight healthy heterosexual donors were examined by limiting dilution analysis for their ability to be clonally expanded. It was demonstrated that viable T4+ and T8+ lymphocytes from patients with AIDS had markedly reduced proportions of clonable cells compared to the healthy donors (T4 = 1:255 vs. 1:34, P = 0.06; T8 = 1:355 vs. 1:55, P = 0.01). However, the cloned T cells that were obtained from the patients with AIDS demonstrated normal proliferation in response to phytohemagglutinin and alloantigen, and normal ability to help or suppress pokeweed mitogen-driven IgG synthesis. These results strongly suggest that, in addition to a quantitative diminution of T4+ lymphocytes in AIDS, there is an intrinsic functional defect in the surviving T4+ and T8+ lymphocytes, which is reflected by a severe decrease in their potential for clonal expansion. PMID:3161909

  5. Psychopathology in 90 consecutive human immunodeficiency virus-seropositive and acquired immune deficiency syndrome patients with mostly intravenous drug use history.

    PubMed

    Perretta, P; Nisita, C; Zaccagnini, E; Lorenzetti, C; Nuccorini, A; Cassano, G B; Akiskal, H S

    1996-01-01

    This report presents systematic clinical data regarding psychiatric diagnoses, personal and family psychiatric histories, and symptomatologic aspects of 90 consecutive human immunodeficiency virus (HIV)-seropositive and acquired immune deficiency syndrome (AIDS) patients, of whom slightly less than two thirds were at risk due to intravenous drug abuse. In addition, a comparison was made between the distribution patterns of these variables at various stages of HIV illness and related at-risk behaviors. Eighty-four percent of the patients met criteria for a spectrum of DSM-III-R diagnoses (mostly affective) that were associated with high rates of affective and alcohol abuse disorders among first-degree relatives. Mood disorders did not differ significantly between the two main groups at risk (intravenous drug users [IVDUs] v others) by gender, age, or stage of illness. The overall data from the rating scales show high levels of psychic and somatic anxiety in the early stages of illness, whereas cognitive symptoms, retardation, and disorientation are dominant in later stages. A noteworthy finding in this study is that many depressed patients demonstrated current and/or past hypomanic, hyperthymic, or cyclothymic features with no evidence of brain damage detectable by computed axial tomography (CAT). These temperamental attributes, which preceded HIV infection, may have served as risk factors for both drug abuse and impulsive sexual behavior in all types of at-risk groups. PMID:8826691

  6. Factors Associated with Student Nurses' Intent to Provide Physical and Psychosocial Care to Persons with Acquired Immunodeficiency Syndrome.

    ERIC Educational Resources Information Center

    Cole, Frank L.

    1996-01-01

    Responses from 125 of 290 nursing undergraduates indicated their attitudes ranged from most to least positive regarding people with AIDS acquired through blood transfusion, heterosexual activity, homosexual activity, and needle sharing. Homophobia, fear of AIDS, and perceived susceptibility were inversely related with intention to care for AIDS…

  7. Successful treatment with autologous peripheral blood stem cell transplantation for acquired immunodeficiency syndrome (AIDS)-related malignant lymphoma.

    PubMed

    NAGAI, Yuya; MORI, Minako; INOUE, Daichi; KIMURA, Takaharu; SHIMOJI, Sonoko; TOGAMI, Katsuhiro; TABATA, Sumie; MATSUSHITA, Akiko; NAGAI, Kenichi; Imai, Yukihiro; Takafuta, Toshiro; Takahashi, Takayuki

    2009-11-01

    A 62-year-old man was diagnosed with human immunodeficiency virus (HIV) infection while suffering from recurrent herpes zoster infection. Laboratory examination revealed CD4(+) lymphocyte count 16 cells/mul and HIV loading 150,000 copies/ml at presentation. In addition, he had multiple lymph node swelling. Histologic diagnosis of a biopsied lymph node was diffuse, large, B cell-type malignant lymphoma. The karyotype of the lymphoma cells was t(8;14)(q24;q32), which was confirmed by G-banding and fluorescent in situ hybridization. Positron emission tomography (PET)-combined CT scanning revealed systemic extranodal tumors involving the gastrointestinal tract, pancreas, and bone marrow. The clinical stage of the lymphoma was IVB and the international prognosis index was categorized as high. Complete remission (CR) of the lymphoma was obtained after 2 courses of CHOP (cyclophosphamide, adriamycin, vincristine, prednisolone) chemotherapy and 4 subsequent courses of rituximab-combined CHOP (R-CHOP). Highly active antiretroviral therapy (HAART) was started at the initiation of CHOP. Because of the poor prognosis of AIDS-related lymphoma, he received autologous peripheral blood stem cell transplantation with the MEAM protocol (ranimustine, etoposide, cytarabine, melphalan) as a conditioning procedure without a severe infectious episode. He remains in CR 24 months after the transplantation. PMID:20009441

  8. Acquired immunodeficiency syndrome (AIDS) with lymphocytic interstitial pneumonitis (LIP) in a multi transfused child with thalassemia major.

    PubMed

    Sen, S; Goyal, R S; Kumar, A; Pande, I; Khare, S D; Chattopadhya, D; Malaviya, A N

    1992-11-01

    The case of a 10.5-year-old girl, who was diagnosed with a case of thalassemia major at the age of 8 months and had been on regular blood transfusions since then, is related. Donor screening for HIV was started in mid-1988, thus she had received unscreened blood for a number of years. In February 1991, she presented with a dry persistent cough, moderate grade continuous fever, and breathlessness on exertion for over 2 weeks. Chest X-ray showed bilateral infiltrations. She was put on penicillin and chloramphenicol with a provisional diagnosis of bronchopneumonia. In March 1991, she had to be hospitalized for impending respiratory failure. After treatment with intravenous fluids and parenteral antimicrobials, her condition stabilized and she was discharged. In April 1991, she was readmitted because of complaints of difficulty in swallowing and weight loss. Her chest signs had persisted and she had developed oropharyngeal candidiasis with ulcerations. She also had alopecia, a generalized lymphadenopathy, digital clubbing, and bilateral parotid enlargement. Candidiasis responded to vigorous therapy with clotrimazole. Fine needle aspiration of lymph node showed a reactive hyperplasia. HIV antibodies were detected in the serum with ELISA and confirmed by Western blot. Immunologic tests showed evidence of severe immunodeficiency. The Multitest CMI, which simultaneously tests delayed skin hypersensitivity to seven common recall antigens, was totally nonreactive. She was classified as having AIDS according to World Health Organization criteria for children under 13 years of age. The diagnosis of lymphocytic interstitial pneumonitis (LIP) was also made based on the symptoms. Oral prednisolone was given 2 mg/kg/day in 3 divided doses for a month. The cough and dyspnea showed great improvement and the parotid swellings disappeared; lymphadenopathy, clubbing, and alopecia, however, persisted. The child was kept on maintenance therapy of prednisolone and on alternate day co

  9. [Acquired von Willebrand syndrome].

    PubMed

    Franchini, Massimo

    2006-01-01

    Acquired von Willebrand syndrome (aVWS) is a rare, but probably underestimated, bleeding disorder that mimics the congenital form of von Willebrand disease (VWD) in terms of laboratory findings and clinical presentation. However, unlike congenital VWD, it arises in individuals with no personal or family history of bleeding. AVWS occurs in association with a variety of underlying disorders, including lymphoproliferative disorders, myeloproliferative disorders and cardiovascular diseases. The main pathogenic, clinical, laboratory and therapeutic aspects of this syndrome are concisely reported in this review. PMID:16913181

  10. Identical rearranged forms of JC polyomavirus transcriptional control region in plasma and cerebrospinal fluid of acquired immunodeficiency syndrome patients with progressive multifocal leukoencephalopathy.

    PubMed

    Fedele, Cesare Giovanni; Ciardi, Maria Rosa; Delia, Salvatore; Contreras, Gerardo; Perez, José Luis; De Oña, Maria; Vidal, Elisa; Tenorio, Antonio

    2003-10-01

    Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the central nervous system (CNS) caused by the human polyomavirus JC (JCV). JCV has a hypervariable noncoding transcriptional control region (TCR) that spans the origin of replication of the JCV genome through to the first ATG start codon for late gene transcription. The archetype form of TCR is frequently found in the urine and kidneys of healthy and immunocompromised subjects. However the rearranged forms, whose prototype is Mad-1, possibly generated by deletion and duplication of segments of the archetype sequence, are found in the brain and cerebrospinal fluid (CSF) of PML patients. In this study the authors compared JCV TCR detected in paired CSF, plasma, and urine samples of 11 acquired immunodeficiency syndrome (AIDS) patients affected by PML to try to determine where the rearranged JCV TCRs are selected. In one patient, it was also possible to amplify and sequence the TCR in the brain and lymphocytes. Moreover, in 5/11 patients, the CSF, plasma, and urine samples corresponding to 2 months after PML development were available; and in another patient, it was possible to sequence the TCR in plasma and lymphocytes sampled 8 months before the onset of PML. The presence of the same TCR sequences in all the CSF and plasma samples taken from individual patients could strengthen the hypothesis that the blood is a compartment where JCV may replicate and undergo rearrangement of the TCR. This further supports the hypothesis that JCV reaches the brain by a hematogenous route and indicates that the JCV TCR sequences detected in plasma could be used as an early marker of JCV pathogenicity before the clinical appearance of PML in immunocompromised patients. PMID:13129769

  11. Recommendations for assisting in the prevention of perinatal transmission of human T-lymphotropic virus type III/lymphadenopathy-associated virus and acquired immunodeficiency syndrome.

    PubMed

    1985-12-01

    The majority of cases of pediatric acquired immunodeficiency syndrome (AIDS) are transmitted perinatally. 165 (76%) of the cases of AIDS in children under 13 years of age reported as of December 1, 1985, in the US had as the only known risk factor a mother from a high-risk group. However, perinatal transmission from an infected mother to her infant is not automatic; studies have placed the rate of transmission from 0%-65%. A concern in addition to the risk posed to infants born to infected mothers is evidence of an increased likelihood of developing full-blown AIDS when infection with the AIDS virus occurs in association in pregnancy. Target groups for counseling and testing for antibodies to the AIDS virus should include pregnant women or those who may become pregnant who already have evidence of AIDS infection, are intravenous drug abusers, were born in countries where there is a high rate of heterosexual transmission of AIDS, are prostitutes, or are the sexual partners of men in high-risk groups. Such counseling and testing should be made available through the settings that women at increased risk frequent, including drug abuse treatment programs and sexually transmitted diseases clinics. Infected women should be advised to delay pregnancy until more is known about the perinatal transmission of AIDS. Pregnancy infected women should be closely monitored for the development of opportunistic infections as well as psychosocial difficulties. Although these recommendations pertain to women, men who are infected with the AIDS virus also should be counseled about risks of perinatal transmission. PMID:2999576

  12. Influence of the home environment on the prevention of mother to child transmission of human immunodeficiency virus/acquired immune-deficiency syndrome in South Africa.

    PubMed

    Sewnunan, A; Modiba, L M

    2015-01-01

    The human immunodeficiency virus and acquired immune-deficiency syndrome (HIV/AIDS) is still a 'family crises' which marks the beginning of the deterioration of the family unit and the trauma in the emotional, psychological and material lives of both the mother and child. In South African context where the majority of HIV-positive mothers are young single women who live in extended families, disclosure to the sexual partner alone is not an adequate condition for the success of prevention of mother to child transmission (PMTCT). In South Africa, close to one in three women who attend antenatal clinics are HIV positive. KwaZulu-Natal is one of the worst affected provinces, where as many as 40-60% of pregnant women attending antenatal services are living with HIV infection. The study sought to investigate the link between the home environment and its contribution to the success of the programme on PMTCT of HIV/AIDS. A qualitative, explorative, descriptive and contextual study was used in this study to explore whether the home environment for the support system is available for the HIV-positive women on the PMTCT programme. The population of this study included all women who have undergone counselling and tested HIV positive and who have joined the programme on PMTCT of HIV/AIDS in a specific hospital in KwaZulu-Natal Province. Although 14 women agreed to participate in the study, only 10 women were interviewed as saturation was attained. Data were collected using semi-structured interview schedule. Interviews were audio-taped and field notes were taken. Content analysis was used and it was done manually. This study revealed that one of the major issues still surrounding HIV/AIDS and PMTCT is that of non-disclosure, selective disclosure and the stigma and discrimination that surrounds this disease. PMID:26694631

  13. Human B-cell interleukin-10: B-cell lines derived from patients with acquired immunodeficiency syndrome and Burkitt's lymphoma constitutively secrete large quantities of interleukin-10.

    PubMed

    Benjamin, D; Knobloch, T J; Dayton, M A

    1992-09-01

    A recent addition to the lymphokine network is human IL-10 (hIL-10). This novel lymphokine has striking homology to BCRF1 protein, the product of a previously uncharacterized open-reading frame in the Epstein-Barr virus (EBV) genome. To date, IL-10 expression has been described in several T clones induced with anti-CD3 and phorbol myristate acetate (PMA), in monocytes stimulated with lipopolysaccharide (LPS), and in murine B-cell lymphomas. We sought to determine whether human B cells express hIL-10 and, if so, its relationship to EBV and to other B-cell lymphokines. We studied 21 EBV-positive B-cell lines derived from patients with acquired immunodeficiency syndrome (AIDS) and Burkitt's lymphoma (n = 6), American Burkitt's (n = 3), African Burkitt's (n = 5), and normal lymphoblastoid cell lines (n = 7), in comparison with seven EBV-negative cell lines. All cell lines were activated with the tumor promoters PMA and teleocidin and were studied by Northern blot analysis, reverse transcription-polymerase chain reaction (RT-PCR), and enzyme-linked immunoadsorbent assay (ELISA). We demonstrated that EBV-positive cell lines derived from patients with American Burkitt's lymphoma, and especially those from patients with AIDS, constitutively express large quantities of hIL-10 by Northern blot analysis and ELISA (range, 3,101 to 25,915 pg/mL), and that both teleocidin and PMA induce hIL-10 in these cell lines. In contrast, six of seven EBV-negative cell lines did not express hIL-10 even by RT-PCR, and hIL-10 was not triggered by PMA or teleocidin. To assure that the 350 bp amplified by PCR was hIL-10 and not BCRF1, we used PCR primers, which do not amplify a fragment from plasmid templates containing BCRF1. Cloning and sequencing of the 350 bp product also demonstrated that B-cell IL-10 is identical to hIL-10 from the T-cell clone B21. Correlation of hIL-10 with other B-cell lymphokines secreted by these B-cell lines demonstrated that hIL-10 secretor cell lines also

  14. Acquired immune deficiency syndrome (AIDS).

    PubMed

    1987-02-01

    The International Planned Parenthood Medical Advisory Panel has developed recommendations to assist family planning associations in playing a more active role in the prevention and control of acquired immunodeficiency syndrome (AIDS). Of primary importance is an effective program of information and education aimed at communicating the following facts: AIDS is a fatal disease for which there is no cure; AIDS is spread by sexual intercourse, contaminated blood, and contaminated needles; an infected woman can transmit AIDS to her fetus during pregnancy; a monogamous sexual relationship is the surest way to avoid AIDS infection; condom use is good protection; an infected person can look and feel well, yet still be able to transmit the AIDS virus; and AIDS is not spread by ordinary contact with an infected person. Family planning associations should include information on AIDS in all existing IEC projects, as well as develop new materials. Among the target audiences for IEC activities are family planning workers, family planning clients, and the general public including youth, teachers, parents, employers, and national leaders. Special attention should be given to high-risk groups such as homosexual and bisexual men, hemophiliacs, male and female prostitutes, clients of sexually transmitted disease clinics, people with many sexual partners, illegal users of intravenous drugs, and the sexual partners of those in any of these groups. Wide promotion of condom use is a priority activity for family planning organizations. PMID:12340977

  15. Prospective study of cytotoxic T lymphocyte responses to influenza and antibodies to human T lymphotropic virus-III in homosexual men. Selective loss of an influenza-specific, human leukocyte antigen-restricted cytotoxic T lymphocyte response in human T lymphotropic virus-III positive individuals with symptoms of acquired immunodeficiency syndrome and in a patient with acquired immunodeficiency syndrome.

    PubMed Central

    Shearer, G M; Salahuddin, S Z; Markham, P D; Joseph, L J; Payne, S M; Kriebel, P; Bernstein, D C; Biddison, W E; Sarngadharan, M G; Gallo, R C

    1985-01-01

    Peripheral blood leukocytes (PBL) from 18 homosexual men who did not have acquired immunodeficiency syndrome (AIDS) and from 9 heterosexual men were repetitively tested for their ability to generate HLA self-restricted cytotoxic T lymphocyte responses to influenza virus (flu-self) over a 2-yr period. The sera of the same donors were tested for antibodies to human T lymphotropic virus-III (HTLV-III). Six of the homosexual and none of the heterosexual donors consistently generated weak cytotoxic T lymphocyte responses to flu-self. Seven of the homosexual and none of the heterosexual donors were seropositive for antibodies to HTLV-III. No obvious correlation was detected between weak flu-self cytotoxic T lymphocyte responses and antibodies to HTLV-III. However, one homosexual donor generated no detectable cytotoxic T lymphocyte activity to flu-self, although he was a strong responder to HLA-alloantigens. This donor had an OKT4:OKT8 ratio of 0.4 and was seropositive for HTLV-III antigens; HTLV-III virus was identified in his PBL; and he developed AIDS during the course of this study. A second donor with lymphadenopathy and who was seropositive for HTLV-III antigens exhibited marginal cytotoxic T lymphocyte activity to flu-self which he subsequently lost. PBL from two patients, one with Kaposi's sarcoma and one with generalized lymphadenopathy, were also tested for cytotoxic T lymphocyte responses to flu-self and to alloantigens. Both donors failed to generate cytotoxic T lymphocyte to flu-self, but generated strong cytotoxic T lymphocyte responses to alloantigens. The selective loss of an HLA-restricted cytotoxic T lymphocyte response without loss of HLA alloantigenic cytotoxic T lymphocyte activity may be an important functional immunologic characteristic in the development of AIDS. PMID:2997287

  16. Primary extranodal non-hodgkin lymphoma of the head and neck in patients with acquired immunodeficiency syndrome: a clinicopathologic study of 24 patients in a single hospital of infectious diseases in Argentina.

    PubMed

    Corti, Marcelo; Villafañe, María; Bistmans, Alicia; Narbaitz, Marina; Gilardi, Leonardo

    2014-07-01

    Introduction Extranodal non-Hodgkin lymphomas (NHLs) are commonly described in patients with acquired immunodeficiency syndrome (AIDS) and are related with an atypical morphology and aggressive clinical course. AIDS-associated lymphomas are characterized by their rapid progression, frequent extranodal manifestations, and poor outcome. Objective The aim of this article is to remake the clinical features of head and neck (HN) NHL in patients with AIDS to facilitate early diagnosis and treatment. Methods We evaluated the epidemiologic, clinical, immunologic, virologic, and histopathologic characteristics of 24 patients with human immunodeficiency virus (HIV)/AIDS with primary HN NHL treated at a single institution between 2002 and 2012. Histopathologic diagnosis was made according to the criteria of the World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues. Additional immunohistochemical stains were applied in all cases. Results Eighteen patients (75%) were men and the median of age was 39 years. The gingiva and the hard palate were the most common sites of the lesions (15 patients, 62.5%). Lactate dehydrogenase levels were elevated in 16 cases (84%). Bone marrow infiltration was detected only in 4 cases (16.6%). The median CD4 T-cell count was 100 cells/µL. According to the histopathologic evaluation, the most common subtype was diffuse large B-cell lymphoma (12 cases, 50%), followed by plasmablastic lymphoma (9 cases, 37.5%) and Burkitt lymphoma (3 cases, 12.5%). Conclusion HN NHL is a severe complication of advanced HIV/AIDS disease. Early diagnosis followed by chemotherapy plus highly active antiretroviral treatment is necessary to improve the prognosis and the survival of these patients. PMID:25992103

  17. Primary Extranodal Non-Hodgkin Lymphoma of the Head and Neck in Patients with Acquired Immunodeficiency Syndrome: A Clinicopathologic Study of 24 Patients in a Single Hospital of Infectious Diseases in Argentina

    PubMed Central

    Corti, Marcelo; Villafañe, María; Bistmans, Alicia; Narbaitz, Marina; Gilardi, Leonardo

    2014-01-01

    Introduction Extranodal non-Hodgkin lymphomas (NHLs) are commonly described in patients with acquired immunodeficiency syndrome (AIDS) and are related with an atypical morphology and aggressive clinical course. AIDS-associated lymphomas are characterized by their rapid progression, frequent extranodal manifestations, and poor outcome. Objective The aim of this article is to remake the clinical features of head and neck (HN) NHL in patients with AIDS to facilitate early diagnosis and treatment. Methods We evaluated the epidemiologic, clinical, immunologic, virologic, and histopathologic characteristics of 24 patients with human immunodeficiency virus (HIV)/AIDS with primary HN NHL treated at a single institution between 2002 and 2012. Histopathologic diagnosis was made according to the criteria of the World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues. Additional immunohistochemical stains were applied in all cases. Results Eighteen patients (75%) were men and the median of age was 39 years. The gingiva and the hard palate were the most common sites of the lesions (15 patients, 62.5%). Lactate dehydrogenase levels were elevated in 16 cases (84%). Bone marrow infiltration was detected only in 4 cases (16.6%). The median CD4 T-cell count was 100 cells/µL. According to the histopathologic evaluation, the most common subtype was diffuse large B-cell lymphoma (12 cases, 50%), followed by plasmablastic lymphoma (9 cases, 37.5%) and Burkitt lymphoma (3 cases, 12.5%). Conclusion HN NHL is a severe complication of advanced HIV/AIDS disease. Early diagnosis followed by chemotherapy plus highly active antiretroviral treatment is necessary to improve the prognosis and the survival of these patients. PMID:25992103

  18. Disseminated vaccine-strain varicella as initial presentation of the acquired immunodeficiency syndrome: a case report and review of the literature.

    PubMed

    Maves, Ryan C; Tripp, Michael S; Dell, Trevor G; Bennett, Jason W; Ahluwalia, Jaspal S; Tamminga, Cindy; Baldwin, James C; Starr, Clarise Rivera; Grinkemeyer, Michael D; Dempsey, Michael P

    2014-01-01

    Varicella-zoster virus (VZV) infections have declined in many industrialized countries due to vaccination with the attenuated Oka strain virus. Rare cases of severe, disseminated vaccine-strain VZV infection have occurred in the immunocompromised, although rarely in HIV-infected persons. We describe a man with previously-undiagnosed human immunodeficiency virus (HIV) infection who received VZV vaccination and subsequently presented to a combat hospital in Afghanistan with disseminated varicella, respiratory failure, and sepsis. The patient recovered with ventilator and hemodynamic support, intravenous acyclovir, and empiric antibiotic therapy. DNA sequencing detected Oka strain virus from patient blood specimens. Although safe in most populations, the VZV vaccine may cause life-threatening disease in immunocompromised patients. Improved detection of HIV infection may be useful in preventing such cases. PMID:24257110

  19. Impact of highly active antiretroviral therapy on oral manifestations of patients with human immunodeficiency virus/acquired immuno deficiency syndrome in South India

    PubMed Central

    Rao, K. V. S. Eswara; Chitturi, Ravi Teja; Kattappagari, Kiran Kumar; Kantheti, Lalith Prakash Chandra; Poosarla, Chandrasekhar; Baddam, Venkat Ramana Reddy

    2015-01-01

    Background: Human immunodeficiency virus (HIV) infection remains a global health problem, although the development of highly active antiretroviral therapy (HAART) has significantly modified the course of HIV disease into a manageable disease with improved quality-of-life mainly in the developed countries. Very few studies are available regarding effect of HAART on oral lesions in developing countries like India. Aims and Objectives: The aim was to document and compare oral lesions in HIV-seropositive patients before and after HAART. Materials and Methods: Oral manifestations were recorded in 320 HIV seropositive patients attending to the Voluntary Counseling and Confidential Testing Centre at the Government General Hospital, Guntur, before and after treating with HAART and the results were statistically analyzed using Student's t-test and Chi-square test. Results: Oral Candidiasis was significantly reduced in patients under HAART after 3 months. Furthermore, there was decreased incidence of periodontal diseases, but increased hyperpigmentation in patients undergoing HAART. Conclusion: The oral manifestations of HIV infection have changed due to the advent of HAART. Many opportunistic infections have resolved as a result of an improved immune system. Though the risk of hyperpigmentation in those with HAART has increased the prevalence of oral candidiasis and periodontal diseases were less in patients who had access to HAART. PMID:26392652

  20. Disseminated cutaneous leishmaniasis resembling post-kala-azar dermal leishmaniasis caused by Leishmania donovani in three patients co-infected with visceral leishmaniasis and human immunodeficiency virus/acquired immunodeficiency syndrome in Ethiopia.

    PubMed

    Gelanew, Tesfaye; Hurissa, Zewdu; Diro, Ermias; Kassahun, Aysheshm; Kuhls, Katrin; Schönian, Gabriele; Hailu, Asrat

    2011-06-01

    We report paired strains of Leishmania parasites, one from the viscera and the other from skin lesions that were isolated from three patients with visceral leishmaniasis and disseminated cutaneous leishmaniasis that were co-infected with human immunodeficiency virus. The causative parasites were characterized by polymerase chain reaction-restriction length polymorphism of the ribosomal DNA internal transcribed spacer 1 and by a panel of multilocus microsatellite markers. We demonstrated that the causative agent was Leishmania donovani in all cases, irrespective of the phenotype of the disease. The paired strains from viscera and skin lesions of the same patients showed genetic identity across the 14 microsatellite markers investigated. These findings demonstrate that the skin lesions in these human immunodeficiency virus-positive patients with visceral leishmaniasis were caused by dissemination of viscerotropic L. donovani parasites as a consequence of severe immunosuppression. However, in all three patients, rapid clearance of the skin lesions was observed after antimonial therapy. PMID:21633027

  1. Immunophenotypic and genotypic analysis of acquired immunodeficiency syndrome-related non-Hodgkin's lymphomas. Correlation with histologic features in 36 cases. French Study Group of Pathology for HIV-Associated Tumors.

    PubMed

    Raphael, M M; Audouin, J; Lamine, M; Delecluse, H J; Vuillaume, M; Lenoir, G M; Gisselbrecht, C; Lennert, K; Diebold, J

    1994-06-01

    High-grade B-cell-type non-Hodgkin's lymphomas are observed in 5% to 8% of patients positive for the human immunodeficiency virus. Nearly all cases belong to one of the three major histologic types: centroblastic or large noncleaved cell, immunoblastic and Burkitt's lymphoma, or small noncleaved cell. Some cases that are polymorphic are termed high-grade B-cell, not otherwise specified (NOS). The authors determined the immunophenotype of each histologic category of acquired immunodeficiency syndrome (AIDS)-related non-Hodgkins' lymphoma and sought a relationship with the presence of the Epstein-Barr virus (EBV). B-cell differentiation antigens, activation marker expression (human leukocyte antigen-DR, CD10, CD19, CD20, CD21, CD22, CD23, CD25, CD30, CD38), and epithelial membrane antigen were analyzed. The clonality was determined by the detection of cytoplasmic immunoglobulin, surface immunoglobulin, and the analysis of joining region (JH) immunoglobulin gene configuration by Southern blot. Epstein-Barr virus was detected either by Southern blot analysis using BamHI W probe fragment or by in situ hybridization with EBV-encoded RNA transcripts-1 specific probe. The immunophenotypic and genotypic results were compared with the morphology results and with the presence or absence of EBV. Burkitt's lymphomas were associated with EBV in 50% of cases, were monoclonal, and expressed mostly immunoglobulin (Ig) MK, CD10, CD19, CD20, CD22, and CD38. This immunophenotypic profile closely resembled those of the centroblastic cases (large noncleaved cell), in which EBV was absent. Epstein-Barr virus was associated with 90% of immunoblastic cases, and only CD10, CD20, and CD38 were expressed. CD71 was expressed in all categories of non-Hodgkin's lymphoma, and CD21 and CD23 were rarely expressed. Two cases of immunoblastic lymphoma and one case of high-grade B-NOS were polyclonal regarding JH rearrangement, but EBV tested with 1.9-Kb Xhol fragment was clonal. No significant

  2. Inherited and acquired immunodeficiencies underlying tuberculosis in childhood

    PubMed Central

    Boisson-Dupuis, Stéphanie; Bustamante, Jacinta; El-Baghdadi, Jamila; Camcioglu, Yildiz; Parvaneh, Nima; Azbaoui, Safaa El; Agader, Aomar; Hassani, Amal; Hafidi, Naima El; Mrani, Nidal Alaoui; Jouhadi, Zineb; Ailal, Fatima; Najib, Jilali; Reisli, Ismail; Zamani, Adil; Yosunkaya, Sebnem; Gulle-Girit, Saniye; Yildiran, Alisan; Cipe, Funda Erol; Torun, Selda Hancerli; Metin, Ayse; Atikan, Basak Yildiz; Hatipoglu, Nevin; Aydogmus, Cigdem; Kilic, Sara Sebnem; Dogu, Figen; Karaca, Neslihan; Aksu, Guzide; Kutukculer, Necil; Keser-Emiroglu, Melike; Somer, Ayper; Tanir, Gonul; Aytekin, Caner; Adimi, Parisa; Mahdaviani, Seyed Alireza; Mamishi, Setareh; Bousfiha, Aziz; Sanal, Ozden; Mansouri, Davood; Casanova, Jean-Laurent; Abel, Laurent

    2015-01-01

    Summary Tuberculosis (TB), caused by Mycobacterium tuberculosis (M.tb) and a few related mycobacteria, is a devastating disease, killing more than a million individuals per year worldwide. However, its pathogenesis remains largely elusive, as only a small proportion of infected individuals develop clinical disease either during primary infection or during reactivation from latency or secondary infection. Subacute, hematogenous, and extrapulmonary disease tends to be more frequent in infants, children, and teenagers than in adults. Life-threatening primary TB of childhood can result from known acquired or inherited immunodeficiencies, although the vast majority of cases remain unexplained. We review here the conditions conferring a predisposition to childhood clinical diseases caused by mycobacteria, including not only M.tb but also weakly virulent mycobacteria, such as BCG vaccines and environmental mycobacteria. Infections with weakly virulent mycobacteria are much rarer than TB, but the inherited and acquired immunodeficiencies underlying these infections are much better known. Their study has also provided genetic and immunological insights into childhood TB, as illustrated by the discovery of single-gene inborn errors of IFN-γ immunity underlying severe cases of TB. Novel findings are expected from ongoing and future human genetic studies of childhood TB in countries that combine a high proportion of consanguineous marriages, a high incidence of TB, and an excellent clinical care, such as Iran, Morocco, and Turkey. PMID:25703555

  3. Negative labeling and social exclusion of people living with human immunodeficiency virus/acquired immune deficiency syndrome in the antiretroviral therapy era: insight from attitudes and behavioral intentions of female heads of households in Zambézia Province, Mozambique.

    PubMed

    Mukolo, Abraham; Blevins, Meridith; Hinton, Nicole; Victor, Bart; Vaz, Lara M E; Sidat, Mohsin; Vergara, Alfredo E

    2014-01-01

    In the age of antiretroviral therapy (ART), unraveling specific aspects of stigma that impede uptake and adherence to human immunodeficiency virus (HIV) services and the complex intersections among them might enhance the efficacy of stigma-reduction interventions targeted at the general public. Few studies have described community stigma in high HIV prevalence regions of Mozambique where program scale-up has been concentrated, but fear of stigma persists as a barrier to HIV service uptake. Principal components analysis of attitudinal data from 3749 female heads of households surveyed in Zambézia Province was used to examine patterns of agreement with stigmatizing attitudes and behavior toward people living with HIV. Inferences were based on comparison of factor loadings and commonality estimates. Construct validity was established through correlations with levels of knowledge about HIV transmission and consistency with the labeling theory of stigma. Two unique domains of community stigma were observed: negative labeling and devaluation (NLD, α = 0.74) and social exclusion (SoE, α = 0.73). NLD is primarily an attitudinal construct, while SoE captures behavioral intent. About one-third of the respondents scored in the upper tertile of the NLD stigma scale (scale: 0-100 stigma points) and the equivalent was 41.3% in the SoE stigma scale. Consistent with literature, NLD and SoE stigma scores were inversely correlated with HIV transmission route knowledge. In item level analysis, fear of being labeled a prostitute/immoral and of negative family affect defined the nature of stigma in this sample. Thus, despite ART scale-up and community education about HIV/acquired immune deficiency syndrome (AIDS), NLD and SoE characterized the community stigma of HIV in this setting. Follow-up studies could compare the impact of these stigma domains on HIV services uptake, in order to inform domain-focused stigma-reduction interventions. PMID:24274172

  4. Recent Advances in DOCK8 Immunodeficiency Syndrome.

    PubMed

    Zhang, Qian; Jing, Huie; Su, Helen C

    2016-07-01

    Since the discovery of the genetic basis of DOCK8 immunodeficiency syndrome (DIDS) in 2009, several hundred patients worldwide have been reported, validating and extending the initial clinical descriptions. Importantly, the beneficial role of hematopoietic stem cell transplantation for this disease has emerged, providing impetus for improved diagnosis. Additionally, several groups have further elucidated the biological functions of DOCK8 in the immune system that help explain disease pathogenesis. Here, we summarize these recent developments. PMID:27207373

  5. Familial hepatopulmonary syndrome in common variable immunodeficiency.

    PubMed

    Holmes, S N; Condliffe, A; Griffiths, W; Baxendale, H; Kumararatne, D S

    2015-04-01

    Common Variable Immunodeficiency (CVID) comprises a heterogeneous group of primary antibody deficiencies which lead to a range of complications, including infectious, neoplastic and inflammatory disorders. This report describes monozygotic twin brothers with CVID who developed cryptogenic liver disease and subsequently hepatopulmonary syndrome (HPS). This is the second report of the association of HPS and CVID. Its occurrence in two identical twins implicates a genetic basis. PMID:25708586

  6. Prevalence of human papillomavirus infection, distribution of viral types and risk factors in cervical samples from human immunodeficiency virus-positive women attending three human immunodeficiency virus-acquired immune deficiency syndrome reference centres in northeastern Brazil

    PubMed Central

    Martins, Albert Eduardo Silva; Lucena-Silva, Norma; Garcia, Renan Gomes; Welkovic, Stefan; Barboza, Aureliana; Menezes, Maria Luiza Bezerra; Maruza, Magda; Tenório, Terezinha; Ximenes, Ricardo AA

    2014-01-01

    Human immunodeficiency virus (HIV)-positive patients have a greater prevalence of coinfection with human papillomavirus (HPV) is of high oncogenic risk. Indeed, the presence of the virus favours intraepithelial squamous cell lesion progression and may induce cancer. The aim of this study was to evaluate the prevalence of HPV infection, distribution of HPV types and risk factors among HIV-positive patients. Cervical samples from 450 HIV-positive patients were analysed with regard to oncotic cytology, colposcopy and HPV presence and type by means of polymerase chain reaction and sequencing. The results were analysed by comparing demographic data and data relating to HPV and HIV infection. The prevalence of HPV was 47.5%. Among the HPV-positive samples, 59% included viral types of high oncogenic risk. Multivariate analysis showed an association between HPV infection and the presence of cytological alterations (p = 0.003), age greater than or equal to 35 years (p = 0.002), number of partners greater than three (p = 0.002), CD4+ lymphocyte count < 200/mm3 (p = 0.041) and alcohol abuse (p = 0.004). Although high-risk HPV was present in the majority of the lesions studied, the low frequency of HPV 16 (3.3%), low occurrence of cervical lesions and preserved immunological state in most of the HIV-positive patients were factors that may explain the low occurrence of precancerous cervical lesions in this population. PMID:25317701

  7. Update: acquired immunodeficiency syndrome (AIDS)--worldwide.

    PubMed

    1988-05-13

    As of March 21, 1988, 136 countries or territories throughout the world had reported a total of 84,256 cases of AIDS to the Global Programme on AIDS (GPA) (formerly the Special Programme on AIDS) of the World Health Organization (WHO). 37 countries or territories had reported no AIDS cases. Reports are based on either the Centers for Disease Control (CDC)/WHO surveillance definition, the WHO clinical definition, or a physician's diagnosis. From 1979 through March 21, 1988, the number of AIDS cases increased markedly in all geographic regions. The cumulative world total increased from 11,965 in 1984 to 25,150 in 1985 (a 11% increase) and to 48,413 in 1986 (a 92% increase). Because of reporting lags, the global total of AIDS cases reported for 1987 is not yet complete; however, as of March 21, 1988, 34,913 cases had been reported for 1987 (a 72% increase). Data on the distribution of AIDS cases by region are presented, followed by a discussion of the findings. Regions include: Americas, Europe, Africa, Oceania, Asia, and eastern Mediterranean countries. Epidemiologic studies indicate 3 broad yet distinct geographic patterns of transmission. Pattern I is typical of industrialized countries with large numbers of reported AIDS cases, such as North America, Western Europe, Australia, New Zealand, and parts of Latin America. In these areas, most cases occur among homosexual or bisexual males and urban intravenous (IV) drug users. Pattern II is observed in areas of central, eastern, and southern Africa and in some Carribean countries. In these areas, most cases occur among heterosexuals. Pattern III is found in areas of Eastern Europe, the Middle East, Asia, and most of the Pacific. Only small numbers of cases have been reported in these areas. PMID:3129644

  8. The pathology of an epizootic of acquired immunodeficiency in rhesus macaques.

    PubMed Central

    Osborn, K. G.; Prahalada, S.; Lowenstine, L. J.; Gardner, M. B.; Maul, D. H.; Henrickson, R. V.

    1984-01-01

    A syndrome of acquired immunodeficiency within a group of outdoor-housed rhesus macaques (Macaca mulatta) with unusually high mortality has been identified at the California Primate Research Center. The cause of death for most of the affected animals included septicemia and/or chronic diarrhea with wasting, often complicated by other problems. In many cases, multiple or unusual infectious agents were isolated or recognized, including cytomegalovirus, Cryptosporidium spp., and Candida albicans. Septicemias due to usually innocuous agents such as Staphylococcus epidermidis and Alcaligenes faecalis were seen. Two animals developed cutaneous fibrosarcomas. Affected animals had generalized lymphadenopathy and splenomegaly, with depletion of T-cell populations, initially follicular hyperplasia followed by depletion, and absence of plasma cells. This spontaneous disease syndrome in nonhuman primates has similarities to acquired immune deficiency syndrome (AIDS) in humans, providing an animal model for the study of the complex factors modulating the immune system. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 Figure 11 Figure 12 Figure 13 PMID:6691418

  9. Testing for Human Immunodeficiency Virus

    MedlinePlus

    ... incisions made in the mother’s abdomen and uterus. Human Immunodeficiency Virus (HIV): A virus that attacks certain cells of the body’s immune system and causes acquired immunodeficiency syndrome (AIDS). Immune System: ...

  10. Early diagnosis of severe combined immunodeficiency syndrome.

    PubMed Central

    Hague, R A; Rassam, S; Morgan, G; Cant, A J

    1994-01-01

    Infants with severe combined immunodeficiency syndrome (SCIDS) have a greatly improved prognosis if diagnosed and treated before they develop overwhelming infection. Clinical and laboratory data on 45 patients with SCIDS were retrospectively reviewed to assess the value of absolute lymphocyte counts in making an early diagnosis. Ninety infants matched for age, sex, and presenting symptoms were used as controls. Thirteen (29%) infants with SCIDS were diagnosed at birth as previous siblings had been affected; 32 (71%) were diagnosed after the development of symptoms. Eighteen (56%) of these remained undiagnosed until after 6 months of age. The first symptoms occurred at a median of 5 weeks (range 1 day to 8 months) and the first admission to hospital was at 4 months (range 1 week to 16 months). Symptoms included respiratory infection (91%), vomiting and diarrhoea (81%), failure to thrive (88%), candidiasis (50%), and skin lesions (28%). The mean lymphocyte count was 1.71 x 10(9)/l compared with 7.2 x 10(9)/l in controls. Excluding one child with Omenn's syndrome (lymphocyte count 23.3 x 10(9)/l, all symptomatic infants with SCIDS had lymphocyte counts less than 2.8 x 10(9)/l at presentation. The median delay between the first abnormal lymphocyte count and diagnosis was seven weeks (range one day to 13 months). Twenty eight (88%) of 32 infants would have been diagnosed before 6 months of age if investigated after the first low lymphocyte count. These data indicate that low lymphocyte counts are predictive of SCIDS. Paediatricians are urged to pay attention to the absolute lymphocyte counts in all infants in whom a full blood count is performed. Those with lymphocyte counts persistently less than 2.8 x 10(9)l should be investigated for SCIDS. PMID:8185357

  11. Acquired von Willebrand syndrome: an update.

    PubMed

    Franchini, Massimo; Lippi, Giuseppe

    2007-05-01

    Acquired von Willebrand syndrome (aVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease (VWD). However, unlike congenital VWD, it arises in individuals with no personal or family history of bleeding. aVWS occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative disorders, myeloproliferative disorders, and cardiovascular diseases. Through an analysis of the more recent literature data, the pathophysiology and the clinical, laboratory, and therapeutic aspects of this syndrome are concisely reported in this review. PMID:17133419

  12. (Immunodeficiency syndromes: disorders of lymphocytopoiesis caused by environmental disorders. ) Annual progress report, January-August 1986

    SciTech Connect

    Not Available

    1986-01-01

    Chronic exposure of mice to certain neoplasms and minor histocompatibility antigens interferes with the production of lymphocytes in the lymphomyeloid complex; the resultant compromised lymphocytopoiesis is a major contributing factor in the development of an acquired immunodeficiency syndrome (IDS). Studies which aim at characterizing the response of leukocytes and thymocytes to transplanted neoplasma, at the in vivo recovery of the immune system on excision of the neoplasm, and at the search for humor factors mediating the immunosuppression are described.

  13. Human immunodeficiency virus infection and pneumothorax

    PubMed Central

    Terzi, Eirini; Zarogoulidis, Konstantinos; Kougioumtzi, Ioanna; Dryllis, Georgios; Kioumis, Ioannis; Pitsiou, Georgia; Machairiotis, Nikolaos; Katsikogiannis, Nikolaos; Tsiouda, Theodora; Madesis, Athanasios; Karaiskos, Theodoros

    2014-01-01

    Pneumothorax is a serious and relatively frequent complication of human immunodeficiency virus (HIV) infection that may associate with increased morbidity and mortality and may prove difficult to manage, especially in patients with acquired immunodeficiency syndrome (AIDS). PMID:25337392

  14. Gut epithelial barrier dysfunction in human immunodeficiency virus-hepatitis C virus coinfected patients: Influence on innate and acquired immunity

    PubMed Central

    Márquez, Mercedes; Fernández Gutiérrez del Álamo, Clotilde; Girón-González, José Antonio

    2016-01-01

    Even in cases where viral replication has been controlled by antiretroviral therapy for long periods of time, human immunodeficiency virus (HIV)-infected patients have several non-acquired immunodeficiency syndrome (AIDS) related co-morbidities, including liver disease, cardiovascular disease and neurocognitive decline, which have a clear impact on survival. It has been considered that persistent innate and acquired immune activation contributes to the pathogenesis of these non-AIDS related diseases. Immune activation has been related with several conditions, remarkably with the bacterial translocation related with the intestinal barrier damage by the HIV or by hepatitis C virus (HCV)-related liver cirrhosis. Consequently, increased morbidity and mortality must be expected in HIV-HCV coinfected patients. Disrupted gut barrier lead to an increased passage of microbial products and to an activation of the mucosal immune system and secretion of inflammatory mediators, which in turn might increase barrier dysfunction. In the present review, the intestinal barrier structure, measures of intestinal barrier dysfunction and the modifications of them in HIV monoinfection and in HIV-HCV coinfection will be considered. Both pathogenesis and the consequences for the progression of liver disease secondary to gut microbial fragment leakage and immune activation will be assessed. PMID:26819512

  15. Treatment of the acquired von Willebrand syndrome.

    PubMed

    Budde, Ulrich; Scheppenheim, Sonja; Dittmer, Rita

    2015-12-01

    Acquired von Willebrand syndrome (aVWS) accounts for 22% of patients with abnormal von Willebrand factor. Most patients with known pathophysiological mechanisms suffer from cardiovascular, myeloproliferative and lymphoproliferative disorders. Less frequent associations are of autoimmune origin, due to hyperfibrinolysis, adsorption to tumor cells, reduced synthesis and prolonged circulation. The mechanisms leading to aVWS is hitherto not known in patients with liver and kidney diseases, drug use, glycogen storage disease, virus infections and at least 18 other disease entities. Diagnosis is complicated by the battery of tests needed, and their inherent rather low sensitivity and specificity for aVWS. Thus, even in acute bleeding situations it may take days until a firm diagnosis is settled and specific therapies can be initiated. The main aim is to shed more light onto this, compared with inherited von Willebrand disease, rare disease which affects at least 2-3% of the older population. PMID:26577336

  16. AIDS: Acquired Immune Deficiency Syndrome; Information and Procedural Guidelines for Providing Services to Persons with AIDS/HIV. Revised.

    ERIC Educational Resources Information Center

    Montana State Dept. of Health and Environmental Sciences, Helena. Health Education Bureau.

    This volume consists of updated information to be inserted into a Montana AIDS Project manual on providing services to persons with acquired immune deficiency syndrome/human immunodeficiency virus (AIDS/HIV), originally published in December 1985. The updates are mainly statistics and terminology, along with the addition of several new sections.…

  17. Therapeutic approaches to acquired von Willebrand syndrome.

    PubMed

    Federici, A B

    2000-02-01

    Acquired von Willebrand syndrome (AVWS) is a rare acquired bleeding disorder similar to the congenital von Willebrand disease (VWD) in terms of laboratory findings. Diagnosis of AVWS can be very difficult, with treatment normally taking an empirical form. Although more than 200 cases have been reported since 1968, no retrospective or prospective studies are available on AVWS. Recently, an International Registry on AVWS, gathering data directly from worldwide Departments of Haematology-Oncology and Haemophilia Centres, has been organised by a group working on behalf of the Subcommittee on VWF in the Scientific Standardisation Committee (SSC) of International Society on Thrombosis and Haemostasis (ISTH). Information about an additional 211 AVWS patients is now available, with more detailed data on demography, type of haemorrhage, diagnostic tests for AVWS and management of bleeding episodes. The additional 211 AVWS cases are associated with lymphoproliferative (47%) or myeloproliferative (19%) disorders, cardiovascular diseases, neoplasia (7%) and other miscellaneous diseases (14%). Bleeding episodes of AVWS patients were managed by different compounds including desmopressin (22%), FVIII/VWF concentrates (26%) and high-dose immunoglobulin (10%), plasmapheresis (2%), steroids (5%) and immunosuppressive drugs (20%). Based on complied data, we can conclude that none of the therapeutic approaches proposed are 100% effective in all AVWS cases. Therefore, treatment must be customized for each patient according to the underlying disorder, as well as to the type and the severity of bleeding episode and must be targeted to each specific case. PMID:11060681

  18. Kaposi Sarcoma of Childhood: Inborn or Acquired Immunodeficiency to Oncogenic HHV-8.

    PubMed

    Jackson, Carolyn C; Dickson, Mark A; Sadjadi, Mahan; Gessain, Antoine; Abel, Laurent; Jouanguy, Emmanuelle; Casanova, Jean-Laurent

    2016-03-01

    Kaposi sarcoma (KS) is an endothelial malignancy caused by human herpes virus-8 (HHV-8) infection. The epidemic and iatrogenic forms of childhood KS result from a profound and acquired T cell deficiency. Recent studies have shown that classic KS of childhood can result from rare single-gene inborn errors of immunity, with mutations in WAS, IFNGR1, STIM1, and TNFRSF4. The pathogenesis of the endemic form of childhood KS has remained elusive. We review childhood KS pathogenesis and its relationship to inherited and acquired immunodeficiency to oncogenic HHV-8. PMID:26469702

  19. Pediatric sensorineural hearing loss, part 2: syndromic and acquired causes.

    PubMed

    Huang, B Y; Zdanski, C; Castillo, M

    2012-03-01

    This article is the second in a 2-part series reviewing neuroimaging in childhood SNHL. Previously, we discussed the clinical work-up of children with hearing impairment, the classification of inner ear malformations, and congenital nonsyndromic causes of hearing loss. Here, we review and illustrate the most common syndromic hereditary and acquired causes of childhood SNHL, with an emphasis on entities that demonstrate inner ear abnormalities on cross-sectional imaging. Syndromes discussed include BOR syndrome, CHARGE syndrome, Pendred syndrome, Waardenburg syndrome, and X-linked hearing loss with stapes gusher. We conclude the article with a review of acquired causes of childhood SNHL, including infections, trauma, and neoplasms. PMID:21596810

  20. A case of acquired Gitelman syndrome presenting as hypokalemic paralysis

    PubMed Central

    Kulkarni, M.; Kadri, P.; Pinto, R.

    2015-01-01

    We report a case of a young female patient who presented with weakness of upper and lower limbs. On evaluation, she had hypokalemia, hypomagnesemia, metabolic alkalosis and hypocalciuria. Anti-Ro (SSA) antibody was positive. She had an acquired Gitelman syndrome due to primary Sjögren's syndrome (SS). SS presenting with features of Gitelman syndrome is very rare. PMID:26199478

  1. A case of acquired Gitelman syndrome presenting as hypokalemic paralysis.

    PubMed

    Kulkarni, M; Kadri, P; Pinto, R

    2015-01-01

    We report a case of a young female patient who presented with weakness of upper and lower limbs. On evaluation, she had hypokalemia, hypomagnesemia, metabolic alkalosis and hypocalciuria. Anti-Ro (SSA) antibody was positive. She had an acquired Gitelman syndrome due to primary Sjögren's syndrome (SS). SS presenting with features of Gitelman syndrome is very rare. PMID:26199478

  2. Neurofibromatosis, Down's syndrome, and acquired abnormalities

    PubMed Central

    Ali, Syed Yousuf; Manne, Vimala; Manne, Ranjit; Himani, Chennamaneni

    2016-01-01

    We report a patient with Down's syndrome and neurofibromatosis who presented with a keloid, sebaceous cyst and acanthosis nigricans, along with dental and ophthalmological defects. The coexistence of neurofibromatosis type 1 and Down's syndrome which are two unrelated genetic conditions is itself a rarity. PMID:27294059

  3. Neurofibromatosis, Down's syndrome, and acquired abnormalities.

    PubMed

    Ali, Syed Yousuf; Manne, Vimala; Manne, Ranjit; Himani, Chennamaneni

    2016-01-01

    We report a patient with Down's syndrome and neurofibromatosis who presented with a keloid, sebaceous cyst and acanthosis nigricans, along with dental and ophthalmological defects. The coexistence of neurofibromatosis type 1 and Down's syndrome which are two unrelated genetic conditions is itself a rarity. PMID:27294059

  4. Introduction and immunopathogenesis of acquired immune deficiency syndrome

    PubMed Central

    Sudharshan, S

    2008-01-01

    India has a large number of patients with acquired immune deficiency syndrome (AIDS), the third largest population of this group in the world. This disease was first described in patients with Pneumocystis pneumonia in 1981. Ocular lesions can occur at any stage of the disease but are more commonly seen at the late stages. Human immunodeficiency virus (HIV), the causative agent of AIDS is a retrovirus with RNA genome and a unique ′Reverse transcriptase enzyme′ and is of two types, HIV-1 and 2. Most human diseases are caused by HIV-1. The HIV-1 subtypes prevalent in India are A, B and C. They act predominantly by reducing the CD4+ cells and thus the patient becomes susceptible to opportunistic infections. High viral titers in the peripheral blood during primary infection lead to decrease in the number of CD4+ T lymphocytes. Onset of HIV-1-specific cellular immune response with synthesis of HIV-1 specific antibodies leads to the decline of plasma viral load and chronification of HIV-1 infection. However, the asymptomatic stage of infection may lead to persistent viral replication and a rapid turnover of plasma virions which is the clinical latency. During this period, there is further decrease in the CD4+ counts which makes the patient′s immune system incapable of controlling opportunistic pathogens and thus life-threatening AIDS-defining diseases emerge. Advent of highly active antiretroviral treatment (HAART) has revolutionized the management of AIDS though there is associated increased development of immune recovery uveitis in a few of these patients. PMID:18711263

  5. Erythema elevatum diutinum in acquired immune deficiency syndrome: Can it be an immune reconstitution inflammatory syndrome?

    PubMed Central

    Jose, Sheethal K; Marfatia, Yogesh S.

    2016-01-01

    A 47-year-old male with acquired immune deficiency syndrome (AIDS) presented with multiple hyperpigmented papules and nodules on both ankles, dorsum of bilateral feet and soles. It was associated with mild itching and pain. The patient was diagnosed with human immunodeficiency virus (HIV) in 2007. First-line antiretroviral therapy (ART) was started in 2009 to which he responded initially. He was shifted to second-line ART 11 months ago in March 2015 due to treatment failure as suggested by CD4 count of 50 cells/mm3. The present skin lesions started 2 months after the initiation of second-line ART. Differential diagnoses considered were Kaposi's sarcoma and immune reconstitution inflammatory syndrome (IRIS) related infections, but biopsy was suggestive of erythema elevatum diutinum (EED). Patient was started on oral dapsone 100 mg/day and increased to 200 mg/day to which he is responding gradually. In the present case, appearance of the lesions after initiation of second-line ART coupled with increase in CD4 count and decrease of viral load below undetectable level suggest that EED could be an IRIS. PMID:27190420

  6. Erythema elevatum diutinum in acquired immune deficiency syndrome: Can it be an immune reconstitution inflammatory syndrome?

    PubMed

    Jose, Sheethal K; Marfatia, Yogesh S

    2016-01-01

    A 47-year-old male with acquired immune deficiency syndrome (AIDS) presented with multiple hyperpigmented papules and nodules on both ankles, dorsum of bilateral feet and soles. It was associated with mild itching and pain. The patient was diagnosed with human immunodeficiency virus (HIV) in 2007. First-line antiretroviral therapy (ART) was started in 2009 to which he responded initially. He was shifted to second-line ART 11 months ago in March 2015 due to treatment failure as suggested by CD4 count of 50 cells/mm(3). The present skin lesions started 2 months after the initiation of second-line ART. Differential diagnoses considered were Kaposi's sarcoma and immune reconstitution inflammatory syndrome (IRIS) related infections, but biopsy was suggestive of erythema elevatum diutinum (EED). Patient was started on oral dapsone 100 mg/day and increased to 200 mg/day to which he is responding gradually. In the present case, appearance of the lesions after initiation of second-line ART coupled with increase in CD4 count and decrease of viral load below undetectable level suggest that EED could be an IRIS. PMID:27190420

  7. Herpes simplex virus type 1 encephalitis in acquired immunodeficiency syndrome.

    PubMed

    Chrétien, F; Bélec, L; Hilton, D A; Flament-Saillour, M; Guillon, F; Wingertsmann, L; Baudrimont, M; de Truchis, P; Keohane, C; Vital, C; Love, S; Gray, F

    1996-10-01

    Herpes simplex (HSV) infection of the central nervous system is uncommon in AIDS and usually has an atypical topography. This review is centred around the case of a 49-year-old homosexual patient with AIDS who died from diffuse encephalopathy. Neuropathological examination revealed necrotic and haemorrhagic changes involving both temporal lobes, insulae and cingulate gyri. Cowdry type A intranuclear inclusion bodies were abundant but inflammation was minimal. Electron microscopy confirmed characteristic herpes virus particles. Immunocyto-chemistry was positive for HSV type 1 and 2. In situ hybridization and PCR, however, were positive for HSV type 1 but excluded HSV type 2. There was associated cytomegalovirus ventriculitis but clearly separated from HSV encephalitis. There were no histological features of HIV encephalitis and HIV could not be demonstrated by immunocytochemistry or by PCR to demonstrate proviral DNA. Apoptotic neurons were numerous in areas with a severe macrophage reaction. Only two pathological cases with characteristic limbic distribution and necrotic haemorrhagic histologic have been reported previously. The rarity of these reports suggests that in advanced AIDS, the immune reaction causing a typical necrotizing encephalitis cannot be mounted. Distinction between HSV type 1 and 2 infection may be difficult by immunocytochemistry and usually requires in situ hybridization, tissue culture or PCR. In AIDS patients, HSV-1 has been identified as responsible for encephalitis whereas HSV-2 has been more responsible for myelitis. Associated productive HIV infection of the CNS was found in none of the cases. In contrast, cytomegalovirus encephalitis was found in nine of 11 cases of AIDS-associated HSV encephalitis. PMID:8930949

  8. Acquired immunodeficiency syndrome: more than a health-related dilemma.

    PubMed Central

    Severin, M J

    1989-01-01

    Many legal issues will affect the health care worker during the AIDS pandemic. These issues are now beginning to be contested in our courts. It is certain that their numbers will continue to grow in the foreseeable future. As local, state, and federal governments design and implement new laws concerning PWA, mechanisms for surveillance, and control of AIDS, new issues are sure to arise. These will undoubtedly involve persons concerned with providing service to those afflicted with this illness. The direction of health care research has already been altered by AIDS. Societal relationships have been affected, as evidenced by the increasing number of legal charges filed when the question of HIV infection involves a patient, student, employee, or other citizen. Inevitably, the health care worker who has contact with PWA will be asked to participate in the mechanisms of the resulting legal contests. If the case reports cited above are an indication of the legal struggles ahead, appearance as a witness to provide scientific information as well as information about the care and treatment afforded PWA will be required of health care workers with increasing frequency. PMID:2680059

  9. Adolescents and human immunodeficiency virus infection.

    PubMed

    Anderson, J R

    1992-12-01

    As of March 31, 1992, individuals 13 to 19 years of age had been diagnosed with acquired immunodeficiency syndrome; over one third were diagnosed in the past 2 years alone. Because of the long incubation period from initial infection to acquired immunodeficiency syndrome diagnosis, the majority of young adults with acquired immunodeficiency syndrome were probably initially infected as adolescents. In 1991, 34% of adolescents with acquired immunodeficiency syndrome were female, and their predominant mode of transmission was heterosexual contact. Human immunodeficiency virus seroprevalence studies of adolescents show a male-to-female ratio approaching 1:1, with many human immunodeficiency virus-infected adolescent women identifying none of the standard risk. Factors such as sexual and drug experimentation, risk taking, and sense of invulnerability so characteristic of adolescence put adolescents at special risk for human immunodeficiency virus. There is no published information on if or how clinical manifestations of human immunodeficiency virus disease in adolescents might differ from those seen in adults. Medical care should be broad-based and should include access to clinical trials for new drug treatments. General knowledge levels about acquired immunodeficiency syndrome are high among US adolescents, but behavioral changes have lagged behind. All adolescents should be targeted for intensive education about human immunodeficiency virus along with interventions designed to enhance their general coping, communication, and decision-making skills. PMID:1450349

  10. Acquired immunodeficiency similar to Gulf War illness in a dead former serviceman.

    PubMed

    Roncati, Luca; Gatti, A M; Pusiol, T; Barbolini, G; Maiorana, A

    2015-06-01

    A 38-year-old non-commissioned officer was certified unfit for military duty several months before his death. The forensic autopsy revealed a severe bone marrow aplasia and a pulmonary angioinvasive aspergillosis. Moreover, the presence of inorganic foreign particles in the pulmonary macrophages and intestinal endothelia was observed. The microanalysis implemented on these last selected specimens revealed the presence of silica particles microimpregnated by lanthanides and steel. The patient's acquired immunodeficiency appears comparable with that of Iraqi civilians suffering from Gulf War illness. This is the first report in the literature of the presence of intestinal endothelia engulfed by foreign war particulates; the silica particles may have entered the intestinal endothelia via the blood stream or by ingestion of impregnated fruit and vegetable foodstuffs. This finding provides new perspectives in the assessment of war-associated diseases and includes electron probe microanalysis among the new techniques of military and forensic medicine. PMID:25428137

  11. Cernunnos/XLF Deficiency: A Syndromic Primary Immunodeficiency.

    PubMed

    Cipe, Funda Erol; Aydogmus, Cigdem; Babayigit Hocaoglu, Arzu; Kilic, Merve; Kaya, Gul Demet; Yilmaz Gulec, Elif

    2014-01-01

    Artemis, DNA ligase IV, DNA protein kinase catalytic subunit, and Cernunnos/XLF genes in nonhomologous end joining pathways of DNA repair mechanisms have been identified as responsible for radiosensitive SCID. Here, we present a 3-year-old girl patient with severe growth retardation, bird-like face, recurrent perianal abscess, pancytopenia, and polydactyly. Firstly, she was thought as Fanconi anemia and spontaneous DNA breaks were seen on chromosomal analysis. After that DEB test was found to be normal and Fanconi anemia was excluded. Because of that she had low IgG and IgA levels, normal IgM level, and absence of B cells in peripheral blood; she was considered as primary immunodeficiency, Nijmegen breakage syndrome. A mutation in NBS1 gene was not found; then Cernunnos/XLF deficiency was investigated due to clinical similarities with previously reported cases. Homozygous mutation in Cernunnos/XLF gene (NHEJ1) was identified. She is now on regular IVIG prophylaxis and has no new infection. Fully matched donor screening is in progress for bone marrow transplantation which is curative treatment of the disease. In conclusion, the patients with microcephaly, bird-like face, and severe growth retardation should be evaluated for hypogammaglobulinemia and primary immunodeficiency diseases. PMID:24511403

  12. Cernunnos/XLF Deficiency: A Syndromic Primary Immunodeficiency

    PubMed Central

    Çipe, Funda Erol; Aydogmus, Cigdem; Babayigit Hocaoglu, Arzu; Kilic, Merve; Kaya, Gul Demet; Yilmaz Gulec, Elif

    2014-01-01

    Artemis, DNA ligase IV, DNA protein kinase catalytic subunit, and Cernunnos/XLF genes in nonhomologous end joining pathways of DNA repair mechanisms have been identified as responsible for radiosensitive SCID. Here, we present a 3-year-old girl patient with severe growth retardation, bird-like face, recurrent perianal abscess, pancytopenia, and polydactyly. Firstly, she was thought as Fanconi anemia and spontaneous DNA breaks were seen on chromosomal analysis. After that DEB test was found to be normal and Fanconi anemia was excluded. Because of that she had low IgG and IgA levels, normal IgM level, and absence of B cells in peripheral blood; she was considered as primary immunodeficiency, Nijmegen breakage syndrome. A mutation in NBS1 gene was not found; then Cernunnos/XLF deficiency was investigated due to clinical similarities with previously reported cases. Homozygous mutation in Cernunnos/XLF gene (NHEJ1) was identified. She is now on regular IVIG prophylaxis and has no new infection. Fully matched donor screening is in progress for bone marrow transplantation which is curative treatment of the disease. In conclusion, the patients with microcephaly, bird-like face, and severe growth retardation should be evaluated for hypogammaglobulinemia and primary immunodeficiency diseases. PMID:24511403

  13. Human Immunodeficiency Syndromes Affecting Human Natural Killer Cell Cytolytic Activity

    PubMed Central

    Ham, Hyoungjun; Billadeau, Daniel D.

    2013-01-01

    Natural killer (NK) cells are lymphocytes of the innate immune system that secrete cytokines upon activation and mediate the killing of tumor cells and virus-infected cells, especially those that escape the adaptive T cell response caused by the down regulation of MHC-I. The induction of cytotoxicity requires that NK cells contact target cells through adhesion receptors, and initiate activation signaling leading to increased adhesion and accumulation of F-actin at the NK cell cytotoxic synapse. Concurrently, lytic granules undergo minus-end directed movement and accumulate at the microtubule-organizing center through the interaction with microtubule motor proteins, followed by polarization of the lethal cargo toward the target cell. Ultimately, myosin-dependent movement of the lytic granules toward the NK cell plasma membrane through F-actin channels, along with soluble N-ethylmaleimide-sensitive factor attachment protein receptor-dependent fusion, promotes the release of the lytic granule contents into the cleft between the NK cell and target cell resulting in target cell killing. Herein, we will discuss several disease-causing mutations in primary immunodeficiency syndromes and how they impact NK cell-mediated killing by disrupting distinct steps of this tightly regulated process. PMID:24478771

  14. Nutrition, the gastrointestinal tract and the acquired immune deficiency syndrome. Facts and perspectives.

    PubMed

    Singer, P; Rothkopf, M M; Kvetan, V; Gaare, J; Mello, L; Askanazi, J

    1989-12-01

    Diarrhoea and malnutrition are common findings in patients with the Acquired Immune Deficiency Syndrome (AIDS). In this disease, enteropathy leads to fat and D-xylose malabsorption and chronic non-specific inflammation of the small bowel. Moreover, gastrointestinal infection can induce severe diarrhoea. Depletion in real body cell mass, body fat content, and weight loss have been observed. Nutritional therapy is mandatory when weight loss is 10% or greater. Enteral feeding is not easily achieved. Parenteral feeding including fat as a nonprotein calorie source improves general condition. The use of intravenous fat emulsions has been hypothesized to have several beneficial effects. Fluidisation of human immunodeficiency virus membranes by lipid emulsions through cholesterol extraction could decrease the infectivity of the virus. Long term intravenous nutrition may be more than a treatment for malabsorption and depletion; it may possibly have direct pharmacological effects. PMID:16837303

  15. Passive hyperimmune therapy: a viable treatment option for the patient with acquired immune deficiency syndrome.

    PubMed

    Raven, N C

    1994-01-01

    New drugs and therapies are continually emerging in an effort to delay the progression of human immunodeficiency virus (HIV)-positive status to acquired immune deficiency syndrome (AIDS). One such treatment is passive hyperimmune therapy (PHT), which was first researched and subsequently published in 1988. Passive hyperimmune therapy involves plasmapheresis of an asymptomatic HIV-positive donor with high p24 antibodies, no detectable p24 antigen, and a helper-inducer T-cell count greater than 400. The plasma is then pooled, sterilized, and administered to symptomatic HIV-positive patients as a monthly intravenous infusion in an effort to provide passive immunotherapy. In this article, an overview of PHT is provided, including benefits, adverse reactions, and other similar therapies available, so that the nurse who cares for HIV-positive patients can continue to be a significant source of information to them. PMID:7965365

  16. Progressive multifocal leukoencephalopathy occurring with the acquired immune deficiency syndrome.

    PubMed

    England, J D; Hsu, C Y; Garen, P D; Goust, J M; Biggs, P J

    1984-08-01

    A 33-year-old homosexual man with symptoms and signs of a focal brain process was subsequently found to have an acquired immune deficiency syndrome (AIDS) with biopsy-proven progressive multifocal leukoencephalopathy. This report reemphasizes the association of progressive multifocal leukoencephalopathy with AIDS and probably is best viewed as another example of an opportunistic CNS infection complicating deficient cell-mediated immunity. PMID:6540476

  17. Addressing Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome in Advanced Practice Nursing Education.

    ERIC Educational Resources Information Center

    Nokes, Kathleen M.; Stein, Gary L.

    1997-01-01

    A survey of 23 advanced practice nursing programs showed only 3 had HIV-specific graduate-level nursing courses. Recommendations were made for HIV-specific courses, integration of HIV content into other courses, use of Centers for Disease Control and Occupational Safety and Health Administration guidelines, and subspecialties in HIV nursing. (SK)

  18. Combined immunodeficiency develops with age in Immunodeficiency-centromeric instability-facial anomalies syndrome 2 (ICF2).

    PubMed

    von Bernuth, Horst; Ravindran, Ethiraj; Du, Hang; Fröhler, Sebastian; Strehl, Karoline; Krämer, Nadine; Issa-Jahns, Lina; Amulic, Borko; Ninnemann, Olaf; Xiao, Mei-Sheng; Eirich, Katharina; Kölsch, Uwe; Hauptmann, Kathrin; John, Rainer; Schindler, Detlev; Wahn, Volker; Chen, Wei; Kaindl, Angela M

    2014-01-01

    The autosomal recessive immunodeficiency-centromeric instability-facial anomalies syndrome (ICF) is characterized by immunodeficiency, developmental delay, and facial anomalies. ICF2, caused by biallelic ZBTB24 gene mutations, is acknowledged primarily as an isolated B-cell defect. Here, we extend the phenotype spectrum by describing, in particular, for the first time the development of a combined immune defect throughout the disease course as well as putative autoimmune phenomena such as granulomatous hepatitis and nephritis. We also demonstrate impaired cell-proliferation and increased cell death of immune and non-immune cells as well as data suggesting a chromosome separation defect in addition to the known chromosome condensation defect. PMID:25330735

  19. Neurologic Complications of the Acquired Immune Deficiency Syndrome

    PubMed Central

    Slade, Walter R.

    1987-01-01

    The acquired immune deficiency syndrome (AIDS) is a syndrome requiring unique knowledge of its versatile manifestations for accurate diagnosis and skillfull management of its numerous complications for successful treatment. The human T-cell lymphotropic virus type III (HTLV-III), a replication-complete virus, is now reported as the etiologic agent. The neurologic complications of AIDS cover the spectrum of neurologic diseases and usually have multiple causative factors, all of which should be appropriately managed. These complications can be successfully treated, although constant monitoring is required because recurrence is frequent. The neurologic complications are the second most frequent cause of death in AIDS patients. Tests that are usually reliable in diagnosing neurologic diseases may not be reliable in patients with AIDS. The management of AIDS is a multidisciplinary effort, and the neurologist should fulfill a role in the management team. ImagesFigure 1Figure 2Figure 3Figure 4 PMID:3334059

  20. Immunodeficiency in DiGeorge Syndrome and Options for Treating Cases with Complete Athymia

    PubMed Central

    Davies, E. Graham

    2013-01-01

    The commonest association of thymic stromal deficiency resulting in T-cell immunodeficiency is the DiGeorge syndrome (DGS). This results from abnormal development of the third and fourth pharyngeal arches and is most commonly associated with a microdeletion at chromosome 22q11 though other genetic and non-genetic causes have been described. The immunological competence of affected individuals is highly variable, ranging from normal to a severe combined immunodeficiency when there is complete athymia. In the most severe group, correction of the immunodeficiency can be achieved using thymus allografts which can support thymopoiesis even in the absence of donor-recipient matching at the major histocompatibility loci. This review focuses on the causes of DGS, the immunological features of the disorder, and the approaches to correction of the immunodeficiency including the use of thymus transplantation. PMID:24198816

  1. Acquired hemophilia complicated by cardiorenal syndrome type 3

    PubMed Central

    Sharma, Rakesh; Dash, Sananta Kumar; Chawla, Rajesh; Kansal, Sudha; Agrawal, Devender Kumar; Dua, Harsh

    2013-01-01

    Development of autoantibodies against coagulation factor VIII (FVIII) leads to a rare condition defined as acquired hemophilia (AH). If not diagnosed and treated early, AH may be associated with high mortality and morbidity. A 65-year-old woman presented with history of macrohematuria, acute renal failure, cardiogenic shock, and acute respiratory failure. Blood investigation revealed azotemia, prolonged activated partial thromboplastin time (aPTT), coagulation FVIII level of <1%, and presence of FVIII inhibitor. Echocardiography showed global hypokinesia and ultrasonography and computed tomography (CT) revealed bilateral hydroureteronephrosis. The final diagnosis was acquired hemophilia A, complicated by acute obstructive renal failure and cardiorenal syndrome (CRS) type 3. Patient was managed with mechanical ventilation, heparin-free hemodialysis, negative fluid balance, recombinant activated factor VII, and prednisolone. Hematuria was relieved, renal function improved, and cardiac function showed improvement on repeat echocardiography. Patient was discharged on prednisolone with subsequent follow ups. PMID:24501492

  2. Acquired Platelet Dysfunction with Eosinophilia (APDE) Syndrome: A Case Report.

    PubMed

    Yadav, Diksha D; Nayar, Priyanka S; Manchanda, Rumma V

    2016-06-01

    Acquired platelet dysfunction with eosinophilia (APDE) is a syndrome which has transient state of platelet dysfunction in the presence of marked eosinophilia. This bleeding disorder, otherwise known as "non-thrombocytopenic purpura with eosinophilia", occurs commonly in children from South-East Asia. We report an 11 years old male child, who presented with ecchymotic patches over lower limbs, of recent onset. His hemogram revealed increased eosinophils with a normal platelet count. Coagulation screen revealed normal parameters except increase in bleeding time. Platelet aggregation studies showed normal platelet aggregation with ristocetin, reduced aggregation with ADP and no aggregation was seen with collagen. PMID:27408400

  3. Angiosarcoma in a patient with immunodeficiency, centromeric region instability, facial anomalies (ICF) syndrome.

    PubMed

    van den Brand, Michiel; Flucke, Uta E; Bult, Peter; Weemaes, Corry M R; van Deuren, Marcel

    2011-03-01

    The Immunodeficiency, Centromeric region instability, and Facial anomalies (ICF) syndrome (OMIM #242860) is a rare autosomal recessive disorder caused by defective DNA methylation. Hematological disease and malignancy (macrophage activation syndrome, myelodysplastic syndrome, and Hodgkin lymphoma) have been reported in three patients. To date, there have been no reports of either epithelial or mesenchymal malignancies. We present a patient with all clinical and laboratory findings of the ICF syndrome who died of a metastatic angiosarcoma of the liver. This is the first report of a non-hematological malignancy in the ICF syndrome. The young age at which our patient developed an angiosarcoma suggests an effect of the defective DNA methylation observed in the ICF syndrome. Therefore, with improvement of recognition and treatment of the ICF syndrome, malignancy could become more common in this condition. PMID:21337690

  4. The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis

    PubMed Central

    Bode, Sebastian FN; Ammann, Sandra; Al-Herz, Waleed; Bataneant, Mihaela; Dvorak, Christopher C; Gehring, Stephan; Gennery, Andrew; Gilmour, Kimberly C; Gonzalez-Granado, Luis I; Groß-Wieltsch, Ute; Ifversen, Marianne; Lingman-Framme, Jenny; Matthes-Martin, Susanne; Mesters, Rolf; Meyts, Isabelle; van Montfrans, Joris M; Schmid, Jana Pachlopnik; Pai, Sung-Yun; Soler-Palacin, Pere; Schuermann, Uta; Schuster, Volker; Seidel, Markus G.; Speckmann, Carsten; Stepensky, Polina; Sykora, Karl-Walter; Tesi, Bianca; Vraetz, Thomas; Waruiru, Catherine; Bryceson, Yenan T.; Moshous, Despina; Lehmberg, Kai; Jordan, Michael B; Ehl, Stephan

    2015-01-01

    Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome defined by clinical and laboratory criteria. Current criteria were created to identify patients with familial hemophagocytic lmyphohistiocytosis in immediate need of immunosuppressive therapy. However, these criteria also identify patients with infection-associated hemophagocytic inflammatory states lacking genetic defects typically predisposing to hemophagocytic lymphohistiocytosis. These patients include those with primary immunodeficiencies, in whom the pathogenesis of the inflammatory syndrome may be distinctive and aggressive immunosuppression is contraindicated. To better characterize hemophagocytic inflammation associated with immunodeficiencies, we combined an international survey with a literature search and identified 63 patients with primary immunodeficiencies other than cytotoxicity defects or X-linked lymphoproliferative disorders, presenting with conditions fulfilling current criteria for hemophagocytic lymphohistiocytosis. Twelve patients had severe combined immunodeficiency with <100/μL T cells, 18 had partial T-cell deficiencies; episodes of hemophagocytic lymphohistiocytosis were mostly associated with viral infections. Twenty-two patients had chronic granulomatous disease with hemophagocytic episodes mainly associated with bacterial infections. Compared to patients with cytotoxicity defects, patients with T-cell deficiencies had lower levels of soluble CD25 and higher ferritin concentrations. Other criteria for hemophagocytoc lymphohistiocytosis were not discriminative. Thus: (i) a hemophagocytic inflammatory syndrome fulfilling criteria for hemophagocytic lymphohistiocytosis can be the initial manifestation of primary immunodeficiencies; (ii) this syndrome can develop despite severe deficiency of T and NK cells, implying that the pathophysiology is distinct and not appropriately described as “lympho”-histiocytosis in these patients; and (iii) current criteria for

  5. Acquired long QT syndrome: a focus for the general pediatrician.

    PubMed

    Marzuillo, Pierluigi; Benettoni, Alessandra; Germani, Claudio; Ferrara, Giovanna; D'Agata, Biancamaria; Barbi, Egidio

    2014-04-01

    Acquired long QT syndrome (LQTS) is a disorder of cardiac repolarization most often due to specific drugs, hypokalemia, or hypomagnesemia that may precipitate torsade de pointes and cause sudden cardiac death. Common presentations of the LQTS are palpitations, presyncope, syncope, cardiac arrest, and seizures. An abnormal 12-lead electrocardiogram obtained while the patient is at rest is the key to diagnosis. The occurrence of drug-induced LQTS is unpredictable in any given individual, but a common observation is that most patients have at least 1 identifiable risk factor in addition to drug exposure. The cornerstone of the management of acquired LQTS includes the identification and discontinuation of any precipitating drug and the correction of metabolic abnormalities, such as hypokalemia or hypomagnesemia. Most of the episodes of torsade de pointes are short-lived and terminate spontaneously. We propose a management protocol that could be useful for the daily practice in the emergency pediatric department to reduce the risk of acquired QT prolongation. PMID:24694881

  6. Three Cases of Acquired Simulated Brown Syndrome after Blowout Fracture Operations

    PubMed Central

    Ji, So Young; Yoo, Jae Hong; Ha, Won; Lee, Ji Won

    2015-01-01

    Brown syndrome is known as limited elevation of the affected eye during adduction. It is caused by a disorder of the superior oblique tendon, which makes it difficult for the eyeball to look upward, especially during adduction. It is classified into congenital true sheath Brown syndrome and acquired simulated Brown syndrome. Acquired simulated Brown syndrome can be caused by trauma, infection, or inflammatory conditions. The surgical restoration of blowout fractures can also lead to limitations of ocular motility, including Brown syndrome. We report on three patients with acquired simulated Brown syndrome, who complained of diplopia and limitation of ocular motility after operations to treat blowout fractures. PMID:26015892

  7. Three cases of acquired simulated brown syndrome after blowout fracture operations.

    PubMed

    Ji, So Young; Yoo, Jae Hong; Ha, Won; Lee, Ji Won; Yang, Wan Suk

    2015-05-01

    Brown syndrome is known as limited elevation of the affected eye during adduction. It is caused by a disorder of the superior oblique tendon, which makes it difficult for the eyeball to look upward, especially during adduction. It is classified into congenital true sheath Brown syndrome and acquired simulated Brown syndrome. Acquired simulated Brown syndrome can be caused by trauma, infection, or inflammatory conditions. The surgical restoration of blowout fractures can also lead to limitations of ocular motility, including Brown syndrome. We report on three patients with acquired simulated Brown syndrome, who complained of diplopia and limitation of ocular motility after operations to treat blowout fractures. PMID:26015892

  8. Herpes simplex virus type 1 colitis in a patient with common variable immunodeficiency syndrome.

    PubMed

    Dray, Xavier; Treton, Xavier; Mazeron, Marie-Christine; Lavergne-Slove, Anne; Joly, Francisca; Mimram, Dora; Attar, Alain; Tobelem, Gérard; Bouhnik, Yoram

    2006-05-01

    We report on a case of herpes simplex virus (HSV) type 1 colitis in a 69-year-old patient with common variable immunodeficiency syndrome. A treatment with polyvalent immunoglobulins was discontinued in April 2001. In March 2004 she developed chronic diarrhoea related to rectosigmoidal and caecal ulcerations. In November 2004, HSV was recovered in tissue culture from colonic biopsies. Valaciclovir was then started, leading the patient to clinical remission at day 4, and continued for a 6-week course (without any secondary antiviral prophylaxis). Colonic biopsies were negative for HSV by tissue culture and PCR within 3 weeks of antiviral treatment. Intravenous polyvalent immunoglobulin infusions were readministered within the third week of antiviral treatment. She has declared no clinical event since this period. Three months after the antiviral treatment was achieved, a rectosigmoidoscopy showed an ad-integrum macroscopic and histological mucosal healing whereas PCR was negative for HSV in the colonic tissue. As a large proportion of patients with common variable immunodeficiency syndrome present not only as a humoral immunodeficiency but also as a defect in the cellular immunity compartment (with T-cell deficits), HSV, as well as cytomegalovirus, should be investigated in patients with common variable immunodeficiency syndrome presenting colitis. PMID:16607152

  9. Acquired von Willebrand syndrome: diagnostic problems and therapeutic options.

    PubMed

    Eikenboom, Jeroen C J; Tjernberg, Pernilla; Van Marion, Vincent; Heering, Karel J

    2007-01-01

    We present a case of acquired von Willebrand syndrome (AVWS) due to a monoclonal gammopathy of undetermined significance. Initially this case was diagnosed as congenital von Willebrand disease (VWD); however, re-examination of the medical history rendered a congenital bleeding disorder unlikely. A normal plasma von Willebrand factor (VWF) propeptide level and a very short half-life of VWF after a test infusion with factor VIII/VWF concentrate confirmed the diagnosis AVWS. Two major surgical procedures were successfully managed using high-dose intravenous immunoglobulin. The differential diagnosis with congenital VWD and the diagnostic and therapeutic approaches of AVWS are discussed. We conclude that the diagnosis of AVWS relies primarily on clinical suspicion and a careful bleeding history. A correct diagnosis is essential for optimal perioperative management and treatment of bleeding episodes. PMID:16986130

  10. Acquired resistance to Giardia muris in X-linked immunodeficient mice.

    PubMed Central

    Skea, D L; Underdown, B J

    1991-01-01

    A previous study from this laboratory (D. P. Snider, D. Skea, and B. J. Underdown, Infect. Immun. 56:2838-2842, 1988) indicated that immunodeficient mice expressing the xid gene develop prolonged infections with Giardia muris, unlike immunocompetent mice, which eliminate the intestinal protozoan parasite in 8 to 10 weeks. In this study, CBA/N (xid) and CBA/Ca mice were infected with G. muris cysts and at various times following this primary infection were cured by treatment with metronidazole. In contrast to the marked differences in the ability of xid and normal mice to eliminate a primary infection, mice of both strains were resistant to a secondary challenge of G. muris cysts. These data imply that the mechanism(s) responsible for elimination of a primary infection is not identical to those required to resist a secondary challenge infection. Splenocytes from immunocompetent CBA/Ca mice (but not immunodeficient CBA/N mice) could transfer the ability to eliminate a primary G. muris infection to irradiated mice of either strain. In contrast, splenocytes from previously infected CBA/Ca mice could not transfer resistance to a challenge infection, further supporting the hypothesis that there are differences between mechanisms required to eliminate a primary infection and those necessary to resist a second challenge infection. PMID:2019439

  11. Autopsy pathology in the acquired immune deficiency syndrome.

    PubMed Central

    Reichert, C. M.; O'Leary, T. J.; Levens, D. L.; Simrell, C. R.; Macher, A. M.

    1983-01-01

    The acquired immune deficiency syndrome (AIDS) is a devastating new illness which appears to be sexually and parenterally transmissible. AIDS was first described in the male homosexual community; however, the disease has more recently been described among intravenous drug abusers, Haitians, hemophiliacs, and others. The etiologic agent is unknown. AIDS may represent an infection by a previously undescribed organism, a mutant of a known microorganism, or a multifactorial combination of environmental, immunologic, and genetic factors. As a consequence of the disease's seemingly irreversible ablation of the cell-mediated immune system, AIDS victims succumb to a variety of infections and/or unusual neoplasms. In its fully developed form, mortality approaches 100%. At autopsy the gross and microscopic pathology of the syndrome can be divided into three general categories: 1) morphologic manifestations of profound lymphoid depletion; 2) infections, usually with mixed opportunistic pathogens; and 3) unusual neoplasms, most frequently Kaposi's sarcoma or high-grade lymphomas. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 Figure 11 Figure 12 Figure 13 Figure 14 Figure 15 Figure 16 Figure 17 Figure 18 Figure 19 Figure 20 Figure 21 PMID:6311021

  12. Pediatric acquired CNS demyelinating syndromes: Features associated with multiple sclerosis.

    PubMed

    Hintzen, Rogier Q; Dale, Russell C; Neuteboom, Rinze F; Mar, Soe; Banwell, Brenda

    2016-08-30

    Approximately one-third of children with an acquired demyelinating syndrome (ADS) will be diagnosed with multiple sclerosis (MS), either at onset according to the 2010 McDonald criteria, or on the basis of clinical or MRI evidence of relapsing disease, in the majority of patients within 2-4 years. ADS in adolescents, female patients, and patients with polyfocal deficits is associated with the highest likelihood of MS, while children with acute disseminated encephalomyelitis, those with documented preceding infection, and ADS presentation in young children more commonly portends a monophasic outcome. While pediatric MS associates with similar genetic risk alleles as have been documented in adult-onset MS, such associations are not diagnostically valuable at the individual level. The presence of antibodies directed against aquaporin-4 strongly supports a diagnosis of neuromyelitis optica, and should be assayed in children manifesting with severe optic neuritis, longitudinally extensive myelitis, or brainstem/hypothalamic syndromes. Further research will determine whether other antibody signatures are indicative of relapsing demyelination distinct from MS. PMID:27572864

  13. Acquired Von Willebrand's Syndrome in Systemic Lupus Erythematosus

    PubMed Central

    Strakhan, Marianna; Gralla, Richard J.; Reed, Louis J.

    2014-01-01

    Acquired von Willebrand syndrome (AVWS) is an uncommon, underdiagnosed, and heterogeneous disease which is increasingly recognized as a cause of bleeding diatheses. Systemic lupus erythematosus (SLE) is an infrequent cause of AVWS. Herein, we report a case of AVWS diagnosed during the initial presentation of SLE in a previously healthy young man with no family history of bleeding diathesis who presented with worsening epistaxis, gastrointestinal bleeding, and anasarca. He was found to have severe anemia and prolonged activated partial thromboplastin time (aPTT) with severely decreased levels of von Willebrand factor (VWF) measurements in addition to markedly decreased factor VIII levels. Further evaluation revealed nephrotic syndrome and interstitial lung disease due to SLE. He initially received combination therapy with intravenous immunoglobulin (IVIG) and von Willebrand factor/factor VIII concentrates without significant improvement. Treatment with steroids, cyclophosphamide, and rituximab was followed by clinical improvement evidenced by cessation of bleeding. The short follow-up did not allow us to definitely prove the therapeutic effect of immunosuppressive treatment on AVWS in SLE patients. This case adds to the literature supporting the relationship between AVWS and SLE and highlights the importance of combination therapy in the treatment of severe AVWS as well as the role of IVIG, cyclophosphamide, and rituximab in AVWS associated with SLE. PMID:25544909

  14. [ANEMIC SYNDROME IN PATIENTS WITH COMMUNITY-ACQUIRED PNEUMONIA].

    PubMed

    Budnevsky, A V; Esaulenko, I E; Ovsyannikov, E S; Labzhaniya, N B; Voronina, E V; Chernov, A V

    2016-01-01

    Community-acquired pneumonia remains a most widespread acute infectious disease of socio-economic significance all over the world. Up to 30% of the patients present with anemia responsible for the unfavourable prognosis and elevated mortality. Not infrequently, anemia is not diagnosed during the hospital stay und therefore remains uncorrected. Severe anemia results in enhanced hypercapnia and slowed maturation of red blood cells in the bone marrow which facilitates the development of ischemic syndrome. Hepcidin, a mediator of inflammation and iron-regulatory hormone, plays an important role in the clinical course of community-acquired pneumonia. Hepsidin production increases during inflammation; it suppresses erythtropoiesis and depletes the iron depot leading to so-called anemia of inflammation. Hypoxia and anemia activate erythtropoiesis, and the released erythropoietin inhibits hepsidin production. During pneumonia resolution, hepsidin promotes recovery from anemia by activating iron absorption. The curreni literature contains few data on the use of hepcidin as a diagnostic marker of anemia. The necessity oftreating anemia in patients with pneumonia under hospital conditions is a matter of discussion. Direct involvement of hepcidin in iron metabolism creates a prerequisite for the treatment of anemia. Medicamental suppression of its activity by stimulating erythtropoiesis can facilitate normalization of iron metabolism and restoration of hemoglobin level. PMID:27172725

  15. Association of acquired von Willebrand syndrome with AL amyloidosis.

    PubMed

    Kos, Cynthia A; Ward, Jennifer E; Malek, Karim; Sanchorawala, Vaishali; Wright, Daniel G; O'Hara, Carl; Connors, Lawreen; Skinner, Martha; Seldin, David C

    2007-05-01

    Acquired loss of functional von Willebrand factor (VWF) has been termed the acquired von Willebrand syndrome (AVWS). AVWS is a rare adult-onset bleeding diathesis that is clinically similar to congenital von Willebrand disease (VWD), and occurs with a variety of autoimmune, lymphoproliferative, or myeloproliferative disorders. We have identified four patients with AVWS in association with immunoglobulin light chain (AL) amyloidosis. These patients, lacking any pre-existing or family history of abnormal bleeding, developed cutaneous, mucosal, or gastrointestinal bleeding in the course of their disease without deficiency of clotting factor X or other factors; the activated partial thromboplastin time (aPTT) was prolonged in three out of the four cases. Despite normal VWF antigen levels, VWF ristocetin cofactor activity (VWF:RCo) was low. Electrophoresis patterns of high molecular weight (HMW) VWF multimers were abnormal in two of the four cases. Two of the patients were treated with high-dose intravenous melphalan followed by autologous stem cell transplantation (HDM/SCT) and achieved hematologic remission. In these two patients, the bleeding diathesis improved and the coagulation parameters normalized, confirming a causal relationship between the plasma cell dyscrasia and the AVWS. AVWS should be considered in AL amyloidosis patients with hemorrhagic diatheses and normal clotting factor levels. PMID:17205535

  16. Immune reconstitution syndrome in a human immunodeficiency virus infected child due to giardiasis leading to shock

    PubMed Central

    Nandy, Sneha; Shah, Ira

    2015-01-01

    Human immunodeficiency virus (HIV)-associated immune reconstitution inflammatory syndrome has been reported in association with tuberculosis, herpes zoster (shingles), Cryptococcus neoformans, Kaposi's sarcoma, Pneumocystis pneumonia, hepatitis B virus, hepatitis C virus, herpes simplex virus, Histoplasma capsulatum, human papillomavirus, and Cytomegalovirus. However, it has never been documented with giardiasis. We present a 7-year-old HIV infected girl who developed diarrhea and shock following the initiation of antiretroviral therapy, and her stool showed the presence of giardiasis. PMID:26985424

  17. Immune reconstitution syndrome in a human immunodeficiency virus infected child due to giardiasis leading to shock.

    PubMed

    Nandy, Sneha; Shah, Ira

    2015-01-01

    Human immunodeficiency virus (HIV)-associated immune reconstitution inflammatory syndrome has been reported in association with tuberculosis, herpes zoster (shingles), Cryptococcus neoformans, Kaposi's sarcoma, Pneumocystis pneumonia, hepatitis B virus, hepatitis C virus, herpes simplex virus, Histoplasma capsulatum, human papillomavirus, and Cytomegalovirus. However, it has never been documented with giardiasis. We present a 7-year-old HIV infected girl who developed diarrhea and shock following the initiation of antiretroviral therapy, and her stool showed the presence of giardiasis. PMID:26985424

  18. Primary central nervous system lymphoma in acquired immune deficiency syndrome mimicking toxoplasmosis.

    PubMed

    Utsuki, Satoshi; Oka, Hidehiro; Abe, Katsutoshi; Osawa, Shigeyuki; Yamazaki, Tomoya; Yasui, Yoshie; Fujii, Kiyotaka

    2011-02-01

    A 37-year-old man, a hepatitis B virus carrier due to mother-to-child transmission, had a medical examination in September 2008 in nearby hospitals due to anorexia and weight loss. He was transported to our hospital because computed tomography (CT) detected intracranial lesions, and he had a positive human immunodeficiency virus (HIV) antibody test. Head computed tomography (CT) revealed multiple hemorrhagic lesions and enhancement effect, suggesting a thin wall. Also, an enhancement effect was present in the ventricle walls and the subarachnoid space. No accumulation was found in the thallium-201 scintigraphy. The enhancement effect of the ventricle walls and the subarachnoid space disappeared after oral administration of pyrimethamine, sulfadiazine, and calcium folinate, contributing to the diagnosis of an abscess and meningitis due to toxoplasma. However, mass lesions did not reduce. A biopsy was performed on 30 October, and the pathological diagnosis was malignant lymphoma. He died from respiratory function deterioration on 8 November. Lymphoma cells were found in ventricle wall tissue and the subarachnoid space at the autopsy. Toxoplasmosis will typically occur as a brain lesion most commonly in acquired immune deficiency syndrome (AIDS), whereas malignant lymphoma commonly manifests as a brain neoplastic lesion. However, differentiating between images of these lesions is difficult, so diagnosis by early biopsy is recommended. PMID:21210240

  19. Effectiveness and safety of traditional Chinese medicine in treating acquired immune deficiency syndrome: 2004-2014.

    PubMed

    Liu, Zhi-Bin; Yang, Ji-Ping; Xu, Li-Ran

    2015-01-01

    Substantial progress has been made in China in using traditional Chinese medicine (TCM) to treat acquired immune deficiency syndrome (AIDS). Our objective was to review the latest developments in TCM treatment of AIDS in China between 2004 and 2014. We reviewed the content of original articles investigating the efficacy and safety of TCM for treating AIDS published in Chinese and English language journals. Relevant references from 2004 to 2014 were found using PubMed and the China National Knowledge Infrastructure Database. We found that TCM has been widely used for treating AIDS and its complications in China. The number of TCM studies has increased, which indicates efficacy and safety. Measures of efficacy in the reviewed articles included the alleviation of human immunodeficiency virus (HIV)-related signs and symptoms, improvements in quality of life, improvements in long-term survival, counteraction of the adverse side effects of antiviral drugs, promotion of immune reconstitution, and improvement of laboratory results. In sum, the literature indicates that TCM is safe. TCM plays an important role in the treatment of AIDS. Some studies have attempted to measure the efficacy and safety of TCM for treating AIDS, but more evidence is needed. Therefore, more research on this topic is required in the future. PMID:26699285

  20. Mutations in CDCA7 and HELLS cause immunodeficiency-centromeric instability-facial anomalies syndrome.

    PubMed

    Thijssen, Peter E; Ito, Yuya; Grillo, Giacomo; Wang, Jun; Velasco, Guillaume; Nitta, Hirohisa; Unoki, Motoko; Yoshihara, Minako; Suyama, Mikita; Sun, Yu; Lemmers, Richard J L F; de Greef, Jessica C; Gennery, Andrew; Picco, Paolo; Kloeckener-Gruissem, Barbara; Güngör, Tayfun; Reisli, Ismail; Picard, Capucine; Kebaili, Kamila; Roquelaure, Bertrand; Iwai, Tsuyako; Kondo, Ikuko; Kubota, Takeo; van Ostaijen-Ten Dam, Monique M; van Tol, Maarten J D; Weemaes, Corry; Francastel, Claire; van der Maarel, Silvère M; Sasaki, Hiroyuki

    2015-01-01

    The life-threatening Immunodeficiency, Centromeric Instability and Facial Anomalies (ICF) syndrome is a genetically heterogeneous autosomal recessive disorder. Twenty percent of patients cannot be explained by mutations in the known ICF genes DNA methyltransferase 3B or zinc-finger and BTB domain containing 24. Here we report mutations in the cell division cycle associated 7 and the helicase, lymphoid-specific genes in 10 unexplained ICF cases. Our data highlight the genetic heterogeneity of ICF syndrome; however, they provide evidence that all genes act in common or converging pathways leading to the ICF phenotype. PMID:26216346

  1. Acquired von Willebrand syndrome associated with left ventricular assist device.

    PubMed

    Nascimbene, Angelo; Neelamegham, Sriram; Frazier, O H; Moake, Joel L; Dong, Jing-Fei

    2016-06-23

    Left ventricular assist devices (LVAD) provide cardiac support for patients with end-stage heart disease as either bridge or destination therapy, and have significantly improved the survival of these patients. Whereas earlier models were designed to mimic the human heart by producing a pulsatile flow in parallel with the patient's heart, newer devices, which are smaller and more durable, provide continuous blood flow along an axial path using an internal rotor in the blood. However, device-related hemostatic complications remain common and have negatively affected patients' recovery and quality of life. In most patients, the von Willebrand factor (VWF) rapidly loses large multimers and binds poorly to platelets and subendothelial collagen upon LVAD implantation, leading to the term acquired von Willebrand syndrome (AVWS). These changes in VWF structure and adhesive activity recover quickly upon LVAD explantation and are not observed in patients with heart transplant. The VWF defects are believed to be caused by excessive cleavage of large VWF multimers by the metalloprotease ADAMTS-13 in an LVAD-driven circulation. However, evidence that this mechanism could be the primary cause for the loss of large VWF multimers and LVAD-associated bleeding remains circumstantial. This review discusses changes in VWF reactivity found in patients on LVAD support. It specifically focuses on impacts of LVAD-related mechanical stress on VWF structural stability and adhesive reactivity in exploring multiple causes of AVWS and LVAD-associated hemostatic complications. PMID:27143258

  2. Acquired Myelodysplasia or Myelodysplastic Syndrome: Clearing the Fog

    PubMed Central

    Natelson, Ethan A.; Pyatt, David

    2013-01-01

    Myelodysplastic syndromes (MDS) are clonal myeloid disorders characterized by progressive peripheral blood cytopenias associated with ineffective myelopoiesis. They are typically considered neoplasms because of frequent genetic aberrations and patient-limited survival with progression to acute myeloid leukemia (AML) or death related to the consequences of bone marrow failure including infection, hemorrhage, and iron overload. A progression to AML has always been recognized among the myeloproliferative disorders (MPD) but occurs only rarely among those with essential thrombocythemia (ET). Yet, the World Health Organization (WHO) has chosen to apply the designation myeloproliferative neoplasms (MPN), for all MPD but has not similarly recommended that all MDS become the myelodysplastic neoplasms (MDN). This apparent dichotomy may reflect the extremely diverse nature of MDS. Moreover, the term MDS is occasionally inappropriately applied to hematologic disorders associated with acquired morphologic myelodysplastic features which may rather represent potentially reversible hematological responses to immune-mediated factors, nutritional deficiency states, and disordered myelopoietic responses to various pharmaceutical, herbal, or other potentially myelotoxic compounds. We emphasize the clinical settings, and the histopathologic features, of such AMD that should trigger a search for a reversible underlying condition that may be nonneoplastic and not MDS. PMID:24194760

  3. Gastrointestinal surgery and the acquired immune deficiency syndrome

    PubMed Central

    Weledji, Elroy P.; Nsagha, Dickson; Chichom, Alain; Enoworock, George

    2015-01-01

    Acquired immune-deficiency syndrome (AIDS) is becoming an increasing problem to the surgeon. The impact of HIV/AIDS on surgical practice include the undoubted risk to which the surgeon will expose him or herself, the atypical conditions that may be encountered and the outcome and long term benefit of the surgical treatment in view of disease progression. The two factors most associated with surgical outcome and poor wound healing were AIDS and poor performance status (ASA score). This article questions whether gastrointestinal surgical procedures can be safe and effective therapeutic measures in HIV/AIDS patients and if surgical outcome is worthy of the surgeon's ethical responsibility to treat. As HIV/AIDS patients are not a homogeneous group, with careful patient selection, emergency laparotomy for peritonitis confers worthwhile palliation. However, aggressive surgical intervention must be undertaken with caution and adequate peri-operative care is required. Symptomatic improvement of anorectal pathology may make delayed wound healing an acceptable complication. Alternatives to surgery can be contemplated for diagnosis, prophylaxis or palliation. PMID:25685343

  4. How I treat the acquired von Willebrand syndrome.

    PubMed

    Tiede, Andreas; Rand, Jacob H; Budde, Ulrich; Ganser, Arnold; Federici, Augusto B

    2011-06-23

    The acquired von Willebrand syndrome (AVWS) is a bleeding disorder that is frequently unrecognized or is misdiagnosed as von Willebrand disease. AVWS is characterized by structural or functional defects of von Willebrand factor (VWF) that are secondary to autoimmune, lymphoproliferative or myeloproliferative, malignant, cardiovascular, or other disorders. VWF abnormalities in these disorders can result from (1) antibody-mediated clearance or functional interference, (2) adsorption to surfaces of transformed cells or platelets, or (3) increased shear stress and subsequent proteolysis. Diagnosis can be challenging as no single test is usually sufficient to prove or exclude AVWS. Furthermore, there are no evidence-based guidelines for management. Treatments of the underlying medical condition, including chemo/radiotherapy, surgery, or immunosuppressants can result in remission of AVWS, but is not always feasible and successful. Because of the heterogeneous mechanisms of AVWS, more than one therapeutic approach is often required to treat acute bleeds and for prophylaxis during invasive procedures; the treatment options include, but are not limited to, desmopressin, VWF-containing concentrates, intravenous immunoglobulin, plasmapheresis or recombinant factor VIIa. Here, we review the management of AVWS with an overview on the currently available evidence and additional considerations for typical treatment situations. PMID:21540459

  5. Systemic lupus erythematosus complicated by acquired von Willebrand's syndrome.

    PubMed

    Hong, Sc; Lee, Jh; Chi, Hs; Lee, Ck; Nah, Ss; Kim, Yg; Oh, Js; Moon, Hb; Yoo, B

    2008-09-01

    Haematological abnormalities are common in systemic lupus erythematosus (SLE). In some cases of acquired von Willebrand syndrome (AvWS), von Willebrand disease (vWD) is associated with autoimmune or lymphoproliferative disorders. In this study, we describe a 36-year-old woman with SLE and AvWS. The patient was referred to our hospital because of easy bruisability and recurrent vaginal bleeding. She had no history of bleeding tendency and no family history of bleeding diathesis, but she had a history of recurrent arthralgia, photosensitivity and sicca symptoms. Tests for antinuclear, anti-double stranded DNA, anticardiolipin and anti-beta2-glycoprotein I antibodies were all positive. Analysis of haemostatic parameters showed complete absence of von Willebrand factor ristocetin cofactor (vWF:Rco), von Willebrand antigen (vWF:Ag) and ristocetin-induced platelet aggregation (RIPA). Electrophoretic analysis of plasma showed a complete absence of high-molecular weight vWF multimer. The presence of antibody to vWF was detected by enzyme linked immunosorbent assay (ELISA). Treatment with corticosteroids improved SLE symptoms and corrected bleeding diasthesis. Also, the multimeric patterns of vWF became normalised and anti-vWF antibody disappeared. These findings indicated that this patient had SLE associated with AvWS, which was ameliorated by corticosteroid treatment. PMID:18755868

  6. Shigellosis in Subjects with Traveler's Diarrhea Versus Domestically Acquired Diarrhea: Implications for Antimicrobial Therapy and Human Immunodeficiency Virus Surveillance.

    PubMed

    Toro, Carlos; Arroyo, Ana; Sarria, Ana; Iglesias, Nuria; Enríquez, Ana; Baquero, Margarita; de Guevara, Concepción Ladrón

    2015-09-01

    An increase of sexually transmitted shigellosis is currently being reported in developed countries. In addition, travel-related shigellosis can introduce resistant strains that could be disseminated within this new scenario. Epidemiological features and antimicrobial susceptibility of shigellosis depending on where infection was acquired were investigated. From 2008 to 2013, subjects with shigellosis were studied. Patients were classified according to acquisition of Shigella as traveler's diarrhea (TD) or domestically acquired diarrhea (DAD). Ninety cases of shigellosis were identified: 76 corresponding to the TD group and 14 to the DAD group. In the DAD group, most of patients were human immunodeficiency virus (HIV)-positive men who have sex with men (MSM), being shigellosis associated to male sex (P = 0.007) and HIV infection (P < 0.0001). S. sonnei (47.8%) and S. flexneri (42.2%) were the predominant species. The highest resistance was detected for trimethoprim/sulfamethoxazole (SXT) (81.8%), followed by ampicillin (AMP) (37.8%) and ciprofloxacin (CIP) (23.3%). Resistant Shigella strains were more frequent in subjects with TD than those with DAD, although only for CIP the difference was significant (P = 0.034). Continuous monitoring of patients with shigellosis is necessary to control the spread of resistant Shigella strains and for effective therapy. Men with shigellosis who have not traveled to an endemic area should be screened for HIV infection. PMID:26195465

  7. Shigellosis in Subjects with Traveler's Diarrhea versus Domestically Acquired Diarrhea: Implications for Antimicrobial Therapy and Human Immunodeficiency Virus Surveillance

    PubMed Central

    Toro, Carlos; Sarria, Ana Arroyo, Ana; Iglesias, Nuria; Enríquez, Ana; Baquero, Margarita; de Guevara, Concepción Ladrón

    2015-01-01

    An increase of sexually transmitted shigellosis is currently being reported in developed countries. In addition, travel-related shigellosis can introduce resistant strains that could be disseminated within this new scenario. Epidemiological features and antimicrobial susceptibility of shigellosis depending on where infection was acquired were investigated. From 2008 to 2013, subjects with shigellosis were studied. Patients were classified according to acquisition of Shigella as traveler's diarrhea (TD) or domestically acquired diarrhea (DAD). Ninety cases of shigellosis were identified: 76 corresponding to the TD group and 14 to the DAD group. In the DAD group, most of patients were human immunodeficiency virus (HIV)-positive men who have sex with men (MSM), being shigellosis associated to male sex (P = 0.007) and HIV infection (P < 0.0001). S. sonnei (47.8%) and S. flexneri (42.2%) were the predominant species. The highest resistance was detected for trimethoprim/sulfamethoxazole (SXT) (81.8%), followed by ampicillin (AMP) (37.8%) and ciprofloxacin (CIP) (23.3%). Resistant Shigella strains were more frequent in subjects with TD than those with DAD, although only for CIP the difference was significant (P = 0.034). Continuous monitoring of patients with shigellosis is necessary to control the spread of resistant Shigella strains and for effective therapy. Men with shigellosis who have not traveled to an endemic area should be screened for HIV infection. PMID:26195465

  8. Diagnosis and treatment of acquired von Willebrand syndrome.

    PubMed

    Tiede, Andreas

    2012-12-01

    Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that is characterized by structural or functional alterations in von Willebrand factor (VWF) caused by a range of lymphoproliferative, myeloproliferative, cardiovascular, autoimmune, and other disorders. The pathogenic mechanisms responsible for the VWF abnormalities depend on the underlying condition, but include clearance due to binding of paraproteins, inhibition of VWF, adsorption to the surface of platelets, increased fluid shear stress, and resultant proteolysis or, more rarely, decreased synthesis. The diagnosis and treatment of AVWS are complicated by the need for multiple laboratory tests and the management of bleeding risk in a typically elderly population with serious underlying conditions that predispose towards thrombosis. Recently developed diagnostic algorithms, based on standard laboratory assays, may assist clinicians with the diagnostic workup and help differentiate between AVWS and von Willebrand disease (VWD) types 1 and 2. AVWS should be considered in all patients with new-onset bleeding whenever laboratory findings suggest VWD, particularly in the presence of an AVWS-associated disorder. AVWS testing is also recommended prior to surgery or an intervention with a high risk of bleeding in any individual with an AVWS-associated disorder. Treatment of the underlying condition using immunosuppressants, surgery, or chemotherapy, can lead to remission of AVWS in some individuals and should always be considered. Strategies to prevent and/or treat bleeding episodes should also be in place, including the use of VWF-containing factor VIII concentrates, desmopressin and tranexamic acid. Treatment success will depend largely on the underlying pathogenesis of the disorder. PMID:23439003

  9. Restricted heterogeneity of T lymphocytes in combined immunodeficiency with hypereosinophilia (Omenn's syndrome).

    PubMed Central

    de Saint-Basile, G; Le Deist, F; de Villartay, J P; Cerf-Bensussan, N; Journet, O; Brousse, N; Griscelli, C; Fischer, A

    1991-01-01

    We report the immunological characteristics of five patients with Omenn's syndrome, a rare inherited immunodeficiency also known as combined immunodeficiency with hypereosinophilia. The syndrome is characterized by T cell infiltration of skin, gut, liver, and spleen leading to diffuse erythroderma, protracted diarrhea, failure to thrive, and hepatosplenomegaly. Blood T cells as well as those infiltrating the skin and gut were found to express activation markers and were partially activated by mitogens but not by antigens. Although the lesions resembled those in graft-versus-host disease, the blood T cells were shown by DNA haplotype analysis using probes revealing variable number of tandem repeats to belong to the patients as well as the T cells infiltrating the gut and skin in one patient. A given T cell subset (TCR alpha beta+, CD4+/CD8+, or TCR gamma delta+) was predominant in each patient, with a specific distribution in the skin lesions. Moreover, the study of T cell receptor beta, gamma, and delta gene rearrangements in four patients revealed oligoclonality involving C beta 1, C beta 2, or different V gamma J gamma or V delta J delta genes. This indicates that restricted heterogeneity of the T cell repertoire, previously reported in one case, is a major feature of this syndrome. The occurrence of alymphocytosis-type severe combined immunodeficiency in the brother of one of the patients suggests that the restricted heterogeneity of T cell receptor gene usage in Omenn's syndrome may arise from leakiness, within the context of a genetically determined faulty T cell differentiation. Images PMID:2010548

  10. Acquired von Willebrand syndrome: data from an international registry.

    PubMed

    Federici, A B; Rand, J H; Bucciarelli, P; Budde, U; van Genderen, P J; Mohri, H; Meyer, D; Rodeghiero, F; Sadler, J E

    2000-08-01

    The acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings similar to those of congenital von Willebrand disease (vWD). Despite the numerous cases reported in the literature until 1999 (n = 266), large studies on AvWS are not available. Moreover, diagnosis of AvWS has been difficult and treatment empirical. These considerations prompted us to organize an international registry. A questionnaire, devised to collect specific information on AvWS, was sent to all the members of the International Society on Thrombosis and Haemostasis (ISTH), who were invited to respond if they had diagnosed cases with the AvWS cases. 156 members answered the questionnaire and 54 of them sent information on 211 AvWS cases from 50 centers. Data were compared with those already published in the literature and 25 cases already described or not correctly diagnosed were excluded. The 186 AvWS cases that qualified for the registry were associated with lymphoproliferative (48%) and myeloproliferative disorders (15%), neoplasia (5%), immunological (2%), cardiovascular (21%) and miscellaneous disorders (9%). Ristocetin cofactor activity (vWF:RCo) or collagen binding activity (vWF:CBA) were usually low in AvWS (median values 20 U/dL, range 3-150), while factor VIII coagulant activity was sometimes normal (median 25 U/dL, range 3-191). FVIII/vWF inhibiting activities were present in only a minority of cases (16%). Bleeding episodes in AvWS were mostly of mucocutaneous type (68%) and were managed by DDAVP (32%), FVIII/vWF concentrates (37%), intravenous immunoglobulins (33%), plasmapheresis (19%), corticosteroids (19%) and immunosuppressive or chemotherapic agents (35%). Based upon the data of this international registry, it appears that AvWS is especially frequent in lympho- or myeloproliferative and cardiovascular diseases. Therefore, AvWS should be suspected and searched with the appropriate laboratory tests especially when excessive bleeding occurs in

  11. Cytomegalovirus Retinitis and the Acquired Immune Deficiency Syndrome: Bench to Bedside: LXVII Edward Jackson Memorial Lecture

    PubMed Central

    Jabs, Douglas A.

    2010-01-01

    Purpose To update information on cytomegalovirus (CMV) retinitis in patients with the acquired immune deficiency syndrome (AIDS) and to integrate information on its pathogenesis and clinical outcomes. Design Literature review. Methods Selected articles from the medical literature, particularly large epidemiologic studies, including the Johns Hopkins Cytomegalovirus Retinitis Cohort Study, the Longitudinal Study of the Ocular Complications of AIDS, and the Cytomegalovirus Retinitis and Viral Resistance Study, were reviewed. Clinical information is discussed in light of knowledge on CMV, its pathogenesis, and its interactions with human immunodeficiency virus (HIV). Results Cytomegalovirus uses several mechanisms to evade the immune system and establish latent infection in immunologically normal hosts. With immune deficiency, such as late-stage AIDS, CMV reactivates, is disseminated to the eye, and establishes a productive infection, resulting in retinal necrosis. HIV and CMV potentiate each other: CMV accelerates HIV disease, and CMV retinitis is associated with increased mortality. Randomized clinical trials have demonstrated the efficacy of treatments for CMV retinitis. Systemically-administered treatment for CMV retinitis decreases AIDS mortality. Highly active antiretroviral therapy (HAART), effectively suppresses HIV replication, resulting in immune recovery, which, if sufficient, controls retinitis without anti-CMV therapy. Resistant CMV, detected in the blood, correlates with resistant virus in the eye and is associated with worse clinical outcomes, including mortality. Host factors, including host genetics and access to care, play a role in the development of CMV retinitis. Conclusions Clinical outcomes of CMV retinitis in patients with AIDS are dependent on characteristics of the virus and host and on HIV–CMV interactions. PMID:21168815

  12. Neuromuscular complications of human immunodeficiency virus infection and antiretroviral therapy.

    PubMed Central

    Miller, R G

    1994-01-01

    At least 4 distinct peripheral neuropathy syndromes occur in patients infected with the human immunodeficiency virus. The most common, painful sensory neuropathy, may be related to the viral infection or may be medication induced and is treated symptomatically. The other 3, chronic inflammatory demyelinating polyradiculoneuropathy, mononeuropathy multiplex (some patients), and the progressive polyradiculopathies related to the acquired immunodeficiency syndrome, may all respond to appropriate therapy. Both inflammatory myopathy and zidovudine myopathy also abate with early diagnosis and treatment. PMID:8048229

  13. A Case Series of Acquired Drug Resistance-Associated Mutations in Human Immunodeficiency Virus-Infected Children: An Emerging Public Health Concern in Rural Africa

    PubMed Central

    Gamell, Anna; Muri, Lukas; Ntamatungiro, Alex; Nyogea, Daniel; Luwanda, Lameck B.; Hatz, Christoph; Battegay, Manuel; Felger, Ingrid; Tanner, Marcel; Klimkait, Thomas; Letang, Emilio

    2016-01-01

    The acquisition of drug-resistance mutations among African children living with in human immunodeficiency virus on antiretroviral treatment has been scarcely reported. This threatens the overall success of antiretroviral programs and the clinical outcomes of children in care. We present a well characterized series of children from rural Tanzania with acquired drug-resistance mutations to contribute to the better understanding of this emerging public health concern. PMID:26807427

  14. Vitamin D status in a Brazilian cohort of adolescents and young adults with perinatally acquired human immunodeficiency virus infection

    PubMed Central

    Schtscherbyna, Annie; Gouveia, Carla; Pinheiro, Maria Fernanda Miguens Castelar; Luiz, Ronir Raggio; Farias, Maria Lucia Fleiuss; Machado, Elizabeth Stankiewicz

    2016-01-01

    The purpose was to determine the prevalence and related factors of vitamin D (VitD) insufficiency in adolescents and young adults with perinatally acquired human immunodeficiency virus. A cohort of 65 patients (17.6 ± 2 years) at the Federal University of Rio de Janeiro, Brazil, were examined for pubertal development, nutrition, serum parathormone and serum 25-hydroxyvitamin D [s25(OH)D]. s25(OH)D levels < 30 ng/mL (< 75 nmol/L) were defined as VitD insufficiency. CD4+ T-cell counts and viral load, history of worst clinical status, immunologic status as nadir, current immunologic status, and antiretroviral (ART) regimen were also evaluated as risk factors for VitD insufficiency. Mean s25(OH)D was 37.7 ± 13.9 ng/mL and 29.2% had VitD insufficiency. There was no difference between VitD status and gender, age, nutritional status, clinical and immunological classification, and type of ART. Only VitD consumption showed tendency of association with s25(OH)D (p = 0.064). Individuals analysed in summer/autumn season had a higher s25(OH)D compared to the ones analysed in winter/spring (42.6 ± 14.9 vs. 34.0 ± 11.9, p = 0.011). Although, the frequency of VitD insufficiency did not differ statistically between the groups (summer/autumn 17.9% vs. winter/spring 37.8%, p = 0.102), we suggest to monitor s25(OH)D in seropositive adolescents and young adults, especially during winter/spring months, even in sunny regions. PMID:26872341

  15. Vitamin D status in a Brazilian cohort of adolescents and young adults with perinatally acquired human immunodeficiency virus infection.

    PubMed

    Schtscherbyna, Annie; Gouveia, Carla; Pinheiro, Maria Fernanda Miguens Castelar; Luiz, Ronir Raggio; Farias, Maria Lucia Fleiuss; Machado, Elizabeth Stankiewicz

    2016-02-01

    The purpose was to determine the prevalence and related factors of vitamin D (VitD) insufficiency in adolescents and young adults with perinatally acquired human immunodeficiency virus. A cohort of 65 patients (17.6 ± 2 years) at the Federal University of Rio de Janeiro, Brazil, were examined for pubertal development, nutrition, serum parathormone and serum 25-hydroxyvitamin D [s25(OH)D]. s25(OH)D levels < 30 ng/mL (< 75 nmol/L) were defined as VitD insufficiency. CD4+ T-cell counts and viral load, history of worst clinical status, immunologic status as nadir, current immunologic status, and antiretroviral (ART) regimen were also evaluated as risk factors for VitD insufficiency. Mean s25(OH)D was 37.7 ± 13.9 ng/mL and 29.2% had VitD insufficiency. There was no difference between VitD status and gender, age, nutritional status, clinical and immunological classification, and type of ART. Only VitD consumption showed tendency of association with s25(OH)D (p = 0.064). Individuals analysed in summer/autumn season had a higher s25(OH)D compared to the ones analysed in winter/spring (42.6 ± 14.9 vs. 34.0 ± 11.9, p = 0.011). Although, the frequency of VitD insufficiency did not differ statistically between the groups (summer/autumn 17.9% vs. winter/spring 37.8%, p = 0.102), we suggest to monitor s25(OH)D in seropositive adolescents and young adults, especially during winter/spring months, even in sunny regions. PMID:26872341

  16. Precipitable immune complexes in healthy homosexual men, acquired immune deficiency syndrome and the related lymphadenopathy syndrome.

    PubMed Central

    Euler, H H; Kern, P; Löffler, H; Dietrich, M

    1985-01-01

    Increased levels of 3% PEG precipitable circulating immune complexes (CIC) were found in healthy homosexual men, in homosexual patients with the acquired immune deficiency syndrome (AIDS), and in the AIDS related lymphadenopathy syndrome (LAS). The degree of CIC elevation increases from healthy homosexual men to LAS and AIDS. Patients suffering from AIDS associated with opportunistic infections had a more pronounced increase in CIC than patients with AIDS associated Kaposi's sarcoma. In LAS and AIDS the amount of CIC correlated with the degree of inversion of the T4/T8 lymphocyte ratio, whereas in healthy homosexual men with increased levels of CIC the T4/T8 ratio was not significantly altered. Laser nephelometric partial components analysis revealed that these complexes were of a complement poor subtype with low component levels of C4, C1q and C3c. IgM and IgG were found to be the major components. It is suggested that these CIC might represent a marker of the total antigenic burden of the immune system. Possibly, they are of prognostic and monitoring value for clinical handling of patients at risk for AIDS. PMID:3156700

  17. Experimental oral polio vaccines and acquired immune deficiency syndrome.

    PubMed

    Hooper, E

    2001-06-29

    The simian immunodeficiency virus (SIV) of the common chimpanzee is widely acknowledged as the direct ancestor of HIV-1. There is increasing historical evidence that during the late 1950s, kidneys were routinely excised from central African chimpanzees by scientists who were collaborating with the polio vaccine research of Dr Hilary Koprowski, and sent - inter alia - to vaccine-making laboratories in the USA and Africa, and to unspecified destinations in Belgium. While there is no direct evidence that cells from these kidneys were used as a substrate for growing Dr Koprowski's oral polio vaccines, there is a startling coincidence between places in Africa where his CHAT vaccine was fed, and the first appearances in the world of HIV-1 group M and group-M-related AIDS. Because of the enormous implications of the hypothesis that AIDS may be an unintended iatrogenic (physician-caused) disease, it is almost inevitable that this theory will engender heated opposition from many of those in the scientific establishment, and those with vested interests. PMID:11405924

  18. Immunodeficiency, Centromeric instability, Facial anomalies (ICF) syndrome, due to ZBTB24 mutations, presenting with large cerebral cyst

    PubMed Central

    Cerbone, Manuela; Wang, Jun; Van der Maarel, Silvère M.; d’Amico, Alessandra; d’Agostino, Antonio; Romano, Alfonso; Brunetti-Pierri, Nicola

    2012-01-01

    The Immunodeficiency, Centromeric instability, Facial anomalies (ICF) syndrome is an autosomal recessive disease presenting with immunodeficiency secondary to hypo- or agammaglobulinemia, developmental delay, and facial anomalies. Centromeric instability is the cytogenetic hallmark of the disorder which results from targeted chromosomal rearrangements related to a genomic methylation defect. We describe a patient carrying a homozygous mutation of the ZBTB24 gene, which has been recently shown to be responsible for ICF syndrome type 2. Our patient presented with intellectual disability, multiple café-au-lait spots, and a large cerebral arachnoidal cyst. Although laboratory signs of impaired immune function, such as reduced serum IgM were detected, our patient did not present clinical manifestations of immunodeficiency. Brain malformations have not been reported so far in ICF syndrome and it can be speculated that ZBTB24 mutations may alter cerebral development. Nevertheless, we cannot rule out that the presence of the cerebral cyst in the patient is coincidental. In summary, our patient illustrates that clinical evidence of immunodeficiency is not a universal feature of ICF2 syndrome type 2 and suggests that brain malformations may be present in other ICF cases. PMID:22786748

  19. Immunodeficiency, centromeric instability, facial anomalies (ICF) syndrome, due to ZBTB24 mutations, presenting with large cerebral cyst.

    PubMed

    Cerbone, Manuela; Wang, Jun; Van der Maarel, Silvère M; D'Amico, Alessandra; D'Agostino, Antonio; Romano, Alfonso; Brunetti-Pierri, Nicola

    2012-08-01

    The immunodeficiency, centromeric instability, facial anomalies (ICF) syndrome is an autosomal recessive disease presenting with immunodeficiency secondary to hypo- or agamma-globulinemia, developmental delay, and facial anomalies. Centromeric instability is the cytogenetic hallmark of the disorder which results from targeted chromosomal rearrangements related to a genomic methylation defect. We describe a patient carrying a homozygous mutation of the ZBTB24 gene, which has been recently shown to be responsible for ICF syndrome type 2. Our patient presented with intellectual disability, multiple café-au-lait spots, and a large cerebral arachnoidal cyst. Although laboratory signs of impaired immune function, such as reduced serum IgM were detected, our patient did not present clinical manifestations of immunodeficiency. Brain malformations abnormalities have not been reported so far in ICF syndrome and it can be speculated that ZBTB24 mutations may alter cerebral development. Nevertheless, we cannot rule out that the presence of the cerebral cyst in the patient is coincidental. In summary, our patient illustrates that clinical evidence of immunodeficiency is not a universal feature of ICF2 syndrome type 2 and suggests that brain malformations may be present in other ICF cases. PMID:22786748

  20. Genome-Wide Expression Analysis in Down Syndrome: Insight into Immunodeficiency

    PubMed Central

    Li, Chong; Jin, Lei; Bai, Yun; Chen, Qimin; Fu, Lijun; Yang, Minjun; Xiao, Huasheng; Zhao, Guoping; Wang, Shengyue

    2012-01-01

    Down syndrome (DS) is caused by triplication of Human chromosome 21 (Hsa21) and associated with an array of deleterious phenotypes, including mental retardation, heart defects and immunodeficiency. Genome-wide expression patterns of uncultured peripheral blood cells are useful to understanding of DS-associated immune dysfunction. We used a Human Exon microarray to characterize gene expression in uncultured peripheral blood cells derived from DS individuals and age-matched controls from two age groups: neonate (N) and child (C). A total of 174 transcript clusters (gene-level) with eight located on Hsa21 in N group and 383 transcript clusters including 56 on Hsa21 in C group were significantly dysregulated in DS individuals. Microarray data were validated by quantitative polymerase chain reaction. Functional analysis revealed that the dysregulated genes in DS were significantly enriched in two and six KEGG pathways in N and C group, respectively. These pathways included leukocyte trans-endothelial migration, B cell receptor signaling pathway and primary immunodeficiency, etc., which causally implicated dysfunctional immunity in DS. Our results provided a comprehensive picture of gene expression patterns in DS at the two developmental stages and pointed towards candidate genes and molecular pathways potentially associated with the immune dysfunction in DS. PMID:23155455

  1. Early-onset Evans syndrome, immunodeficiency, and premature immunosenescence associated with tripeptidyl-peptidase II deficiency

    PubMed Central

    Stepensky, Polina; Rensing-Ehl, Anne; Gather, Ruth; Revel-Vilk, Shoshana; Fischer, Ute; Nabhani, Schafiq; Beier, Fabian; Brümmendorf, Tim H.; Fuchs, Sebastian; Zenke, Simon; Firat, Elke; Pessach, Vered Molho; Borkhardt, Arndt; Rakhmanov, Mirzokhid; Keller, Bärbel; Warnatz, Klaus; Eibel, Hermann; Niedermann, Gabriele; Elpeleg, Orly

    2015-01-01

    Autoimmune cytopenia is a frequent manifestation of primary immunodeficiencies. Two siblings presented with Evans syndrome, viral infections, and progressive leukopenia. DNA available from one patient showed a homozygous frameshift mutation in tripeptidyl peptidase II (TPP2) abolishing protein expression. TPP2 is a serine exopeptidase involved in extralysosomal peptide degradation. Its deficiency in mice activates cell death programs and premature senescence. Similar to cells from naïve, uninfected TPP2-deficient mice, patient cells showed increased major histocompatibility complex I expression and most CD8+ T-cells had a senescent CCR7-CD127−CD28−CD57+ phenotype with poor proliferative responses and enhanced staurosporine-induced apoptosis. T-cells showed increased expression of the effector molecules perforin and interferon-γ with high expression of the transcription factor T-bet. Age-associated B-cells with a CD21− CD11c+ phenotype expressing T-bet were increased in humans and mice, combined with antinuclear antibodies. Moreover, markers of senescence were also present in human and murine TPP2-deficient fibroblasts. Telomere lengths were normal in patient fibroblasts and granulocytes, and low normal in lymphocytes, which were compatible with activation of stress-induced rather than replicative senescence programs. TPP2 deficiency is the first primary immunodeficiency linking premature immunosenescence to severe autoimmunity. Determination of senescent lymphocytes should be part of the diagnostic evaluation of children with refractory multilineage cytopenias. PMID:25414442

  2. Semantic Differential Responses to Educational Posters on Acquired Immune Deficiency Syndrome (AIDS).

    ERIC Educational Resources Information Center

    Wilson, Christopher; Stewin, Leonard L.

    1992-01-01

    Undergraduate students (n=131) responded to eight educational posters dealing with the Acquired Immune Deficiency Syndrome (AIDS) using a nine-item semantic differential scale. Two posters were consistently rated as more informative, reassuring, effective, decent, and better than the others. The first utilized an objective and informative…

  3. Acquired Immune Deficiency Syndrome, AIDS: A Selected Bibliography of Federal Government Publications. Research Guide 90 104.

    ERIC Educational Resources Information Center

    Alexander, Margaret

    This research guide presents a selected bibliography of federal government publications about the Acquired Immune Deficiency Syndrome (AIDS). These documents are listed in five categories: (1) Bibliographies (7); (2) Congressional Publications (69 hearings and reports); (3) Executive Branch Publications (43 reports); (4) Federal Government…

  4. Teaching AIDS. A Resource Guide on Acquired Immune Deficiency Syndrome. Third Edition.

    ERIC Educational Resources Information Center

    Quackenbush, Marcia; Sargent, Pamela

    The first edition of this resource guide for educators on how to teach students about Acquired Immune Deficiency Syndrome (AIDS) was published in 1986. Since then, basic facts about the transmission and prevention of the AIDS virus have not changed substantially. The terminologies about the disease, however, have changed and the changing…

  5. Acquired Immune Deficiency Syndrome: A Preliminary Examination of the Effects on Gay Couples and Coupling.

    ERIC Educational Resources Information Center

    Carl, Douglas

    1986-01-01

    The Acquired Immune Deficiency Syndrome (AIDS) epidemic significantly influences attitudes about life and lifestyles. Homosexuals have to give increased consideration to coupling, the nature of coupled relationships, sex and intimacy, and death long before the normal time. Discusses impact of AIDS on the early stages of gay coupling and on the…

  6. Mycobacterium simiae and Mycobacterium avium-M. intracellulare mixed infection in acquired immune deficiency syndrome.

    PubMed Central

    Lévy-Frébault, V; Pangon, B; Buré, A; Katlama, C; Marche, C; David, H L

    1987-01-01

    Acquired immune deficiency syndrome was diagnosed in a 43-year-old man, born and living in Congo. The patient presented a disseminated infection caused by mycobacteria which were recovered from blood, jejunal fluid, and duodenal and rectal biopsies. Identification, according to conventional tests and mycolate profile determination, showed that Mycobacterium avium-M. intracellulare and M. simiae were both involved. Images PMID:3793869

  7. Acquired aphasia without deafness in childhood--the Landau-Kleffner syndrome.

    PubMed

    Hughes, A P; Appleton, R E; Hodgson, J

    1993-07-01

    A young boy presented with loss of speech and behaviour disturbance and was thought to be deaf. He was subsequently found to have the Landau-Kleffner syndrome (LKS), or acquired aphasia with epilepsy. Children with this disorder commonly present to an audiology or ENT clinic. Early recognition is important to initiate supportive, speech and educational care. PMID:15125283

  8. Use of V(D)J recombination excision circles to identify T- and B-cell defects and to monitor the treatment in primary and acquired immunodeficiencies

    PubMed Central

    2013-01-01

    T-cell receptor excision circles (TRECs) and kappa-deleting recombination excision circles (KRECs) are circular DNA segments generated in T and B cells during their maturation in the thymus and bone marrow. These circularized DNA elements persist in the cells, are unable to replicate, and are diluted as a result of cell division, thus are considered markers of new lymphocyte output. The quantification of TRECs and KRECs, which can be reliably performed using singleplex or duplex real-time quantitative PCR, provides novel information in the management of T- and B-cell immunity-related diseases. In primary immunodeficiencies, when combined with flow cytometric analysis of T- and B-cell subpopulations, the measure of TRECs and KRECs has contributed to an improved characterization of the diseases, to the identification of patients’ subgroups, and to the monitoring of stem cell transplantation and enzyme replacement therapy. For the same diseases, the TREC and KREC assays, introduced in the newborn screening program, allow early disease identification and may lead to discovery of new genetic defects. TREC and KREC levels can also been used as a surrogate marker of lymphocyte output in acquired immunodeficiencies. The low number of TRECs, which has in fact been extensively documented in untreated HIV-infected subjects, has been shown to increase following antiretroviral therapy. Differently, KREC number, which is in the normal range in these patients, has been shown to decrease following long-lasting therapy. Whether changes of KREC levels have relevance in the biology and in the clinical aspects of primary and acquired immunodeficiencies remains to be firmly established. PMID:23656963

  9. Continuing improvement in survival for children with acquired immunodeficiency syndrome in Brazil.

    PubMed

    Matida, Luiza H; Ramos, Alberto N; Heukelbach, Jorg; Hearst, Norman

    2009-10-01

    This retrospective cohort study examined a nationally representative sample of 945 Brazilian children (ages 0-12 years) diagnosed with AIDS in 1999 to 2002 and followed through 2007. The probability of survival to 60 months was 86.3% (95% CI, 84.1%-88.5%). This compares with 52.8% (95% CI, 41.9%-60.8%) in a study of cases diagnosed in 1983 to 1998. These results demonstrate substantial improvement in survival for children with AIDS in Brazil. PMID:19672214

  10. Sexually transmitted disease (STD) and acquired immunodeficiency syndrome (AIDS) in South East Asia.

    PubMed

    Ismail, R

    1999-01-01

    This article reports on the prevalence of AIDS and sexually transmitted diseases (STDs) in Southeast Asia. The spread of HIV infection in this region has been predicted to be worse than that of Africa. The high-prevalence countries are Thailand, Cambodia, and Myanmar, where prevalence rates in the population at risk (15-49 year olds) are up to 2%; while low prevalence countries with rates of 0.1% include the Philippines, Indonesia, Laos, Brunei, and Singapore. Heterosexual transmission in Southeast Asia is the main mode of spread of HIV. Another route is through migration, rural-to-urban or international migration of people seeking jobs; with concurrent loneliness and anonymity, they become vulnerable to STDs and HIV infection. Intravenous drug use poses an increasing risk of transmission. The unavailability of data in some countries makes it difficult to evaluate the extent of the epidemic or if there's an impending epidemic. There are a number of caveats to the data compilation from various countries. These include the following: under-reporting of cases; underdiagnosis; missed diagnosis; and differences in the time of data collection. It is clear that poverty, illiteracy, and poor access to educational information in most countries in this region facilitate the rapid spread of HIV. These coupled with lack of primary health care services, and in most instances, enormously high cost of drugs make the pain and suffering due to the HIV/AIDS epidemic a human disaster far worse than the ravages of war. PMID:10330595

  11. [Immunologic findings in homosexual males with generalized lymphadenopathy. Prodromal state of acquired immunodeficiency syndrome?].

    PubMed

    Kalden, J R; Burmester, G R; Manger, B; Coester, C H; Bienzle, U

    1983-11-01

    Peripheral blood of 6 homosexual men with lymphadenopathy associated with fever, weight loss and night sweat, 13 homosexuals with lymphadenopathy without further clinical symptoms, 13 healthy homosexuals and 20 healthy hetero-sexual blood donors was evaluated for different lymphocyte subpopulation, mitogen induced T-cell proliferation and Gamma-interferon production. In the six patients with lymphadenopathy lymphocyte subpopulations were also analysed in lymph node-biopsies. delayed type hypersensitivity was established by skin test's using different recall antigens. Sera of all homosexual men were screened for immunoglobulin levels, complement activity, presence of immunocomplexes and autoantibodies. Most of the homosexuals with lymphadenopathy but without clinical symptoms and all patients with lymphadenopathy associated with clinical symptoms had a significantly decreased helper-(OKT 4) suppressor- (OKT 8) cell ratio. In contrast, the OKT 4/OKT 8 ratio was found to be normal in lymphocyte suspension from lymph node-biopsy from the 6 patients with lymphadenopathy and clinical symptoms. Within the group of healthy homosexuals this ratio exhibited a rather large variation, in three of the tested persons the ratio was below 1. No functional abnormalities of T-cell immunity were observed in the homosexual men as compared to the control group, with the exception of a significantly increased LAG- and PWM-induced lymphocyte proliferation in the group of healthy homosexual men. Likewise no significant differences were obtained with regard to the further analysed immunological parameters. It is concluded that the obtained immunological results do not provide the possibility to define persons at immediate risk for the development of AIDS. PMID:6227767

  12. Multiple coronary aneurysms in a young adult with acquired immunodeficiency syndrome

    PubMed Central

    Heizer, Justin; Petersen, Timothy C.; Flemmer, Mark C.

    2016-01-01

    HIV infection can cause multiple deleterious effects on the cardiovascular system. Emerging evidence has supported a direct association between HIV infection and accelerated atherosclerosis. The mechanism for atherosclerosis in HIV-positive patients is multifactorial, an interplay between conventional risk factors, HIV itself and highly active antiretroviral therapy. The case described is a 29-year-old man with HIV, non-adherent to antiretroviral therapy and with few cardiovascular risk factors, who presented with chest pain and non-ST elevation myocardial infarction. Cardiac catheterization revealed multiple coronary artery aneurysms in the left main coronary artery and the right coronary artery. Aneurysmal formation may develop from vasculitis, HIV itself, accelerated atherosclerosis, congenital formation or medications (e.g. protease inhibitors). The researchers provide a review of coronary artery disease, aneurysmal formation and vasculitic processes in the context of HIV. As this clinical entity becomes more apparent, alternative therapeutic options may need to be explored. PMID:27168940

  13. Intracranial lesions in the acquired immunodeficiency syndrome: radiological (computed tomographic) features

    SciTech Connect

    Elkin, C.M.; Leon, E.; Grenell, S.L.; Leeds, N.E.

    1985-01-18

    Computed tomography (CT) delineates the presence or absence of intracerebral focal lesions in most instances. The presence of contrast enhancement, cerebral atrophy, and an intracranial mass are important in consideration of the differential diagnosis and in establishing the diagnosis. Initially the authors utilized a double dose of contrast medium in all patients after single-dose study, but little additional information was obtained. A second dose of contrast medium is now administered only to evaluate further a suspected lesion. Angiography can confirm the location of the lesion(s) and the cortical veins before biopsy. Of one hundred patients with AIDS examined, 33% had neurological symptoms excluding headache and herpes zoster. All patients with neurological symptoms were studied with noncontrast and contrast CT scanning. Twenty-seven patients in the group had abnormal scans. In 13, the abnormality was limited to a diffuse atrophic appearance, while in 14, focal lesions were identified. Representative cases are discussed and illustrated.

  14. Intestinal microsporidiosis in patients with acquired immunodeficiency syndrome--report of three more German cases.

    PubMed

    Franzen, C; Fätkenheuer, G; Salzberger, B; Müller, A; Mahrle, G; Diehl, V; Schrappe, M

    1994-01-01

    Intestinal microsporidiosis with Enterocytozoon bieneusi was diagnosed in three of 18 HIV-infected patients with chronic diarrhoea. In two cases all known stages of the life cycle of E. bieneusi (merogonial plasmodia, sporogonial plasmodia, sporoblasts, spores) were found in duodenal biopsies by electron microscopical examination, whereas in the third case only merogonial and sporogonial stages were seen. Spores were also visible by light microscopy in semithin sections. Two patients were treated with albendazole (2 x 400 mg/day for 4 weeks) but showed no response. These findings underline the concept of the worldwide distribution of this parasite and verify that it is also frequent in Germany. PMID:7698840

  15. Therapeutic Trial of Rifabutin After Rifampicin-Associated DRESS Syndrome in Tuberculosis-Human Immunodeficiency Virus Coinfected Patients.

    PubMed

    Lehloenya, Rannakoe J; Dlamini, Sipho; Muloiwa, Rudzani; Kakande, Betty; Ngwanya, Mzudumile R; Todd, Gail; Dheda, Keertan

    2016-09-01

    Elimination of a rifamycin from the treatment regimen for tuberculosis negatively impacts outcomes. Cross-reactivity between the rifamycins after drug eruptions is unclear. We report 6 consecutive human immunodeficiency virus-infected patients with rifampicin-associated drug rash with eosinophilia and systemic symptoms (DRESS) syndrome confirmed on diagnostic rechallenge. The patients subsequently tolerated rifabutin. These data inform clinical management of tuberculosis-associated drug reactions. PMID:27419190

  16. Therapeutic Trial of Rifabutin After Rifampicin-Associated DRESS Syndrome in Tuberculosis-Human Immunodeficiency Virus Coinfected Patients

    PubMed Central

    Lehloenya, Rannakoe J.; Dlamini, Sipho; Muloiwa, Rudzani; Kakande, Betty; Ngwanya, Mzudumile R.; Todd, Gail; Dheda, Keertan

    2016-01-01

    Elimination of a rifamycin from the treatment regimen for tuberculosis negatively impacts outcomes. Cross-reactivity between the rifamycins after drug eruptions is unclear. We report 6 consecutive human immunodeficiency virus-infected patients with rifampicin-associated drug rash with eosinophilia and systemic symptoms (DRESS) syndrome confirmed on diagnostic rechallenge. The patients subsequently tolerated rifabutin. These data inform clinical management of tuberculosis-associated drug reactions. PMID:27419190

  17. Mutations in ZBTB24 are associated with immunodeficiency, centromeric instability, and facial anomalies syndrome type 2.

    PubMed

    de Greef, Jessica C; Wang, Jun; Balog, Judit; den Dunnen, Johan T; Frants, Rune R; Straasheijm, Kirsten R; Aytekin, Caner; van der Burg, Mirjam; Duprez, Laurence; Ferster, Alina; Gennery, Andrew R; Gimelli, Giorgio; Reisli, Ismail; Schuetz, Catharina; Schulz, Ansgar; Smeets, Dominique F C M; Sznajer, Yves; Wijmenga, Cisca; van Eggermond, Marja C; van Ostaijen-Ten Dam, Monique M; Lankester, Arjan C; van Tol, Maarten J D; van den Elsen, Peter J; Weemaes, Corry M; van der Maarel, Silvère M

    2011-06-10

    Autosomal-recessive immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is mainly characterized by recurrent, often fatal, respiratory and gastrointestinal infections. About 50% of patients carry mutations in the DNA methyltransferase 3B gene (DNMT3B) (ICF1). The remaining patients carry unknown genetic defects (ICF2) but share with ICF1 patients the same immunological and epigenetic features, including hypomethylation of juxtacentromeric repeat sequences. We performed homozygosity mapping in five unrelated ICF2 patients with consanguineous parents and then performed whole-exome sequencing in one of these patients and Sanger sequencing in all to identify mutations in the zinc-finger- and BTB (bric-a-bric, tramtrack, broad complex)-domain-containing 24 (ZBTB24) gene in four consanguineously descended ICF2 patients. Additionally, we found ZBTB24 mutations in an affected sibling pair and in one patient for whom it was not known whether his parents were consanguineous. ZBTB24 belongs to a large family of transcriptional repressors that include members, such as BCL6 and PATZ1, with prominent regulatory roles in hematopoietic development and malignancy. These data thus indicate that ZBTB24 is involved in DNA methylation of juxtacentromeric DNA and in B cell development and/or B and T cell interactions. Because ZBTB24 is a putative DNA-binding protein highly expressed in the lymphoid lineage, we predict that by studying the molecular function of ZBTB24, we will improve our understanding of the molecular pathophysiology of ICF syndrome and of lymphocyte biology in general. PMID:21596365

  18. Mutations in ZBTB24 Are Associated with Immunodeficiency, Centromeric Instability, and Facial Anomalies Syndrome Type 2

    PubMed Central

    de Greef, Jessica C.; Wang, Jun; Balog, Judit; den Dunnen, Johan T.; Frants, Rune R.; Straasheijm, Kirsten R.; Aytekin, Caner; van der Burg, Mirjam; Duprez, Laurence; Ferster, Alina; Gennery, Andrew R.; Gimelli, Giorgio; Reisli, Ismail; Schuetz, Catharina; Schulz, Ansgar; Smeets, Dominique F.C.M.; Sznajer, Yves; Wijmenga, Cisca; van Eggermond, Marja C.; van Ostaijen-ten Dam, Monique M.; Lankester, Arjan C.; van Tol, Maarten J.D.; van den Elsen, Peter J.; Weemaes, Corry M.; van der Maarel, Silvère M.

    2011-01-01

    Autosomal-recessive immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is mainly characterized by recurrent, often fatal, respiratory and gastrointestinal infections. About 50% of patients carry mutations in the DNA methyltransferase 3B gene (DNMT3B) (ICF1). The remaining patients carry unknown genetic defects (ICF2) but share with ICF1 patients the same immunological and epigenetic features, including hypomethylation of juxtacentromeric repeat sequences. We performed homozygosity mapping in five unrelated ICF2 patients with consanguineous parents and then performed whole-exome sequencing in one of these patients and Sanger sequencing in all to identify mutations in the zinc-finger- and BTB (bric-a-bric, tramtrack, broad complex)-domain-containing 24 (ZBTB24) gene in four consanguineously descended ICF2 patients. Additionally, we found ZBTB24 mutations in an affected sibling pair and in one patient for whom it was not known whether his parents were consanguineous. ZBTB24 belongs to a large family of transcriptional repressors that include members, such as BCL6 and PATZ1, with prominent regulatory roles in hematopoietic development and malignancy. These data thus indicate that ZBTB24 is involved in DNA methylation of juxtacentromeric DNA and in B cell development and/or B and T cell interactions. Because ZBTB24 is a putative DNA-binding protein highly expressed in the lymphoid lineage, we predict that by studying the molecular function of ZBTB24, we will improve our understanding of the molecular pathophysiology of ICF syndrome and of lymphocyte biology in general. PMID:21596365

  19. Modeling the human 8p11-myeloproliferative syndrome in immunodeficient mice.

    PubMed

    Agerstam, Helena; Järås, Marcus; Andersson, Anna; Johnels, Petra; Hansen, Nils; Lassen, Carin; Rissler, Marianne; Gisselsson, David; Olofsson, Tor; Richter, Johan; Fan, Xiaolong; Ehinger, Mats; Fioretos, Thoas

    2010-09-23

    The 8p11 myeloproliferative syndrome (EMS), also referred to as stem cell leukemia/lymphoma, is a chronic myeloproliferative disorder that rapidly progresses into acute leukemia. Molecularly, EMS is characterized by fusion of various partner genes to the FGFR1 gene, resulting in constitutive activation of the tyrosine kinases in FGFR1. To date, no previous study has addressed the functional consequences of ectopic FGFR1 expression in the potentially most relevant cellular context, that of normal primary human hematopoietic cells. Herein, we report that expression of ZMYM2/FGFR1 (previously known as ZNF198/FGFR1) or BCR/FGFR1 in normal human CD34(+) cells from umbilical-cord blood leads to increased cellular proliferation and differentiation toward the erythroid lineage in vitro. In immunodeficient mice, expression of ZMYM2/FGFR1 or BCR/FGFR1 in human cells induces several features of human EMS, including expansion of several myeloid cell lineages and accumulation of blasts in bone marrow. Moreover, bone marrow fibrosis together with increased extramedullary hematopoiesis is observed. This study suggests that FGFR1 fusion oncogenes, by themselves, are capable of initiating an EMS-like disorder, and provides the first humanized model of a myeloproliferative disorder transforming into acute leukemia in mice. The established in vivo EMS model should provide a valuable tool for future studies of this disorder. PMID:20554971

  20. Refractory and/or Relapsing Cryptococcosis Associated with Acquired Immune Deficiency Syndrome: Clinical Features, Genotype, and Virulence Factors of Cryptococcus spp. Isolates.

    PubMed

    Nascimento, Erika; Vitali, Lucia H; Tonani, Ludmilla; Kress, Marcia R Von Zeska; Takayanagui, Osvaldo M; Martinez, Roberto

    2016-05-01

    Refractory and relapsing crytocococcosis in acquired immune deficiency syndrome (AIDS) patients have a poor prognosis. The risk factors for this complicated infection course were evaluated by comparing refractory and/or relapsing cryptococcosis in human immunodeficiency virus-coinfected patients (cohort 1) with another group of AIDS patients who adequately responded to antifungals (cohort 2). Except for one isolate of Cryptococcus gattii from a cohort 2 case, all other isolates were identified as Cryptococcus neoformans var. grubii, sex type α, genotype VNI, including Cryptococcus reisolated from the relapse or in the refractory state. No differences were observed with respect to Cryptococcus capsule size and in the melanin and phospholipase production. The cohort 1 patients presented higher prevalence of cryptococcemia, cerebral dissemination, chronic liver disease, and leucopenia, and have increased death rate. Apparently, the refractory and/or relapsing cryptococcosis in the AIDS patients were more related to the host and the extent of the infection than to the fungal characteristics. PMID:26928832

  1. Unilateral acquired Brown's syndrome in systemic scleroderma: An unusual cause for diplopia

    PubMed Central

    Pawar, Neelam; Ravindran, Meenakshi; Ramakrishnan, Renagappa; Maheshwari, Devendra; Trivedi, Bhakti

    2015-01-01

    Brown's syndrome can be congenital or acquired with multiple causes. It has been described as a ocular complication in various rheumatic and nonrheumatic diseases. We describe a case of 27-year-old female patient with 5 years old history of systemic scleroderma who developed vertical diplopia, a left head tilt, and restriction of left eye on elevation in adduction. The patient responded to systemic steroids with resolution of diplopia. PMID:26669341

  2. Congenital immunodeficiency in an individual with Wiedemann-Steiner syndrome due to a novel missense mutation in KMT2A.

    PubMed

    Stellacci, Emilia; Onesimo, Roberta; Bruselles, Alessandro; Pizzi, Simone; Battaglia, Domenica; Leoni, Chiara; Zampino, Giuseppe; Tartaglia, Marco

    2016-09-01

    Wiedemann-Steiner Syndrome (WSS) is an autosomal dominant disorder characterized by hypertrichosis, short stature, intellectual disability, developmental delay, and facial dysmorphism. Since the original reports by Wiedemann and co-workers, and Steiner and Marques, only a few cases have been described. Recently, the clinical variability of the disorder has more precisely been characterized by Jones and co-workers, who also identified heterozygous KMT2A mutations as the molecular defect underlying this condition. Here, we report on a boy with a complex phenotype overlapping WSS but exhibiting epilepsy, feeding difficulties, microcephaly, and congenital immunodeficiency with low levels of immunoglobulins as additional features. Whole exome sequencing allowed identifying a previously unreported de novo KMT2A missense mutation affecting the DNA binding domain of the methyltransferase. This finding expands the clinical phenotype associated with KMT2A mutations to include immunodeficiency and epilepsy as clinically relevant features for this disorder. © 2016 Wiley Periodicals, Inc. PMID:27320412

  3. A novel deletion in ZBTB24 in a Lebanese family with immunodeficiency, centromeric instability, and facial anomalies syndrome type 2.

    PubMed

    Chouery, E; Abou-Ghoch, J; Corbani, S; El Ali, N; Korban, R; Salem, N; Castro, C; Klayme, S; Azoury-Abou Rjeily, M; Khoury-Matar, R; Debo, G; Germanos-Haddad, M; Delague, V; Lefranc, G; Mégarbané, A

    2012-11-01

    The immunodeficiency, centromeric instability and facial anomalies (ICF) syndrome is a rare autosomal recessive disease characterized by targeted chromosome breakage, directly related to a genomic methylation defect. It manifests with phenotypic and clinical variability, with the most consistent features being developmental delay, facial anomalies, cytogenetic defects and immunodeficiency with a reduction in serum immunoglobulin levels. From the molecular point of view, ICF syndrome was always divided into ICF type I (ICF1) and ICF type 2 (ICF2). Mutations in DNMT3B gene are responsible for ICF1, while mutations in ZBTB24 have been reported to be responsible for ICF2. In this study, we describe a Lebanese family with three ICF2 affected brothers. Sanger sequencing of the coding sequence of ZBTB24 gene was conducted and revealed a novel deletion: c.396_397delTA (p.His132Glnfs*19), resulting in a loss-of-function of the corresponding protein. ZBTB24 belongs to a large family of transcriptional factors and may be involved in DNA methylation of juxtacentromeric DNA. Detailed molecular and functional studies of the ZBTB24 and DNMT3B genes are needed to understand the pathophysiology of ICF syndrome. PMID:21906047

  4. Mutations in AP3D1 associated with immunodeficiency and seizures define a new type of Hermansky-Pudlak syndrome.

    PubMed

    Ammann, Sandra; Schulz, Ansgar; Krägeloh-Mann, Ingeborg; Dieckmann, Nele M G; Niethammer, Klaus; Fuchs, Sebastian; Eckl, Katja Martina; Plank, Roswitha; Werner, Roland; Altmüller, Janine; Thiele, Holger; Nürnberg, Peter; Bank, Julia; Strauss, Anne; von Bernuth, Horst; Zur Stadt, Udo; Grieve, Samantha; Griffiths, Gillian M; Lehmberg, Kai; Hennies, Hans Christian; Ehl, Stephan

    2016-02-25

    Genetic disorders affecting biogenesis and transport of lysosome-related organelles are heterogeneous diseases frequently associated with albinism. We studied a patient with albinism, neutropenia, immunodeficiency, neurodevelopmental delay, generalized seizures, and impaired hearing but with no mutation in genes so far associated with albinism and immunodeficiency. Whole exome sequencing identified a homozygous mutation in AP3D1 that leads to destabilization of the adaptor protein 3 (AP3) complex. AP3 complex formation and the degranulation defect in patient T cells were restored by retroviral reconstitution. A previously described hypopigmented mouse mutant with an Ap3d1 null mutation (mocha strain) shares the neurologic phenotype with our patient and shows a platelet storage pool deficiency characteristic of Hermansky-Pudlak syndrome (HPS) that was not studied in our patient because of a lack of bleeding. HPS2 caused by mutations in AP3B1A leads to a highly overlapping phenotype without the neurologic symptoms. The AP3 complex exists in a ubiquitous and a neuronal form. AP3D1 codes for the AP3δ subunit of the complex, which is essential for both forms. In contrast, the AP3β3A subunit, affected in HPS2 patients, is substituted by AP3β3B in the neuron-specific heterotetramer. AP3δ deficiency thus causes a severe neurologic disorder with immunodeficiency and albinism that we propose to classify as HPS10. PMID:26744459

  5. Non-pulmonary Rhodococcus equi infections in patients with acquired immune deficiency syndrome (AIDS).

    PubMed Central

    Fierer, J; Wolf, P; Seed, L; Gay, T; Noonan, K; Haghighi, P

    1987-01-01

    Rhodococcus equi, formerly known as Corynebacterium equi, was isolated repeatedly from the blood of two patients with the acquired immune deficiency syndrome (AIDS). Neither of the patients had pneumonia while they were bacteraemic, whereas pneumonia has been present in all previously reported cases of human infection with R equi. One of our patients had diarrhoea and the organism was isolated from a stool culture; the other patient had a large granulomatous soft tissue mass in his pelvis caused by R equi. Both isolates were resistant to penicillin and one produced a beta-lactamase. Both patients were treated with vancomycin but only one recovered. Images Figure PMID:3584508

  6. Gingival mass in acquired immune deficiency syndrome patient: An unusual manifestation

    PubMed Central

    Shah, Jigna S.; Prajapati, Monali N.

    2016-01-01

    Non-Hodgkin's lymphoma (NHL) is designated as an acquired immune deficiency syndrome defining condition. Although uncommon, it is essential to be wary of this neoplasm since intraoral manifestations may be the first clinical manifestation of HIV disease. The gingiva is one of the rarest intraoral sites with a prevalence of 0.6%. Careful evaluation of patients presenting with solitary atypical gingival mass can lead to early detection of HIV disease. Here, we report a case of NHL manifesting as a gingival mass in a 45-year-old HIV-positive female patient. PMID:27190419

  7. Primary cardiac lymphoma in a patient with acquired immune deficiency syndrome

    SciTech Connect

    Constantino, A.; West, T.E.; Gupta, M.; Loghmanee, F.

    1987-12-01

    A 34-year-old male prisoner with a history of intravenous drug abuse presented with fever, lymphadenopathy, weight loss, and recent onset of congestive heart failure. Serologic testing was positive for antibodies to human immune deficiency virus. There was intense myocardial uptake of gallium. Autopsy showed a primary immunoblastic lymphoma involving only the myocardium. While primary cardiac lymphoma is an extremely rare condition, the incidence may be higher in patients with acquired immune deficiency syndrome (AIDS) and should be suspected in cases with atypical cardiomyopathy.

  8. HLA-DRB1 and HLA-DQB1 Are Associated with Adult-Onset Immunodeficiency with Acquired Anti-Interferon-Gamma Autoantibodies

    PubMed Central

    Pithukpakorn, Manop; Roothumnong, Ekkapong; Angkasekwinai, Nasikarn; Suktitipat, Bhoom; Assawamakin, Anunchai; Luangwedchakarn, Voravich; Umrod, Pinklow; Thongnoppakhun, Wanna; Foongladda, Suporn; Suputtamongkol, Yupin

    2015-01-01

    Recently a newly identified clinical syndrome of disseminated non-tuberculous mycobacterial diseases (with or without other opportunistic infections in adult patients who were previously healthy, has been recognized in association with an acquired autoantibody to interferon-gamma. This syndrome is emerging as an important cause of morbidity and mortality, especially among people of Asian descent. Trigger for the production of this autoantibody remains unknown, but genetic factors are strongly suspected to be involved. We compared HLA genotyping between 32 patients with this clinical syndrome, and 38 controls. We found that this clinical syndrome was associated with very limited allele polymorphism, with HLA-DRB1 and DQB1 alleles, especially HLA-DRB1*15:01, DRB1*16:02, DQB1*05:01 and DQB1*05:02. Odds ratio of DRB1*15:01, DRB1*16:02, DQB1*05:01 and DQB1*05:02 were 7.03 (95% CI, 2.18–22.69, P<0.0001, 9.06 (95% CI, 2.79–29.46, P<0.0001), 6.68 (95% CI, 2.29–19.52, P = 0.0004), and 6.64 (95% CI, 2.30–19.20, P = 0.0004), respectively. Further investigation is warranted to provide better understanding on pathogenesis of this association. PMID:26011559

  9. Acquired infantile Horner syndrome and spontaneous internal carotid artery dissection: a case report and review of literature.

    PubMed

    Pirouzian, Amir; Holz, Huck A; Ip, Kenneth C; Sudesh, Rattehalli

    2010-04-01

    Horner syndrome, a triad of ptosis, anisocoria, and anhidrosis, results from interruption in the oculosympathetic pathway. It is classically described as either congenital or acquired to depict its underlying pathophysiology and requisite work-up. We report a case of a 10-month-old infant presenting with an acute onset of left Horner syndrome secondary to a spontaneous extracranial internal carotid artery dissection. To the best of our knowledge, this is the first case report in the literature of acute onset of acquired infantile Horner syndrome in association with spontaneous carotid artery dissection confirmed with magnetic resonance angiogram. PMID:20451860

  10. Acquired constriction ring syndrome as a cause of inconsolable cry in a child: a case report

    PubMed Central

    Singh, Vinay; Singh, Pankaj; Sharma, Amit; Sarkar, Jay

    2008-01-01

    Acute constriction ring syndrome (ACRS) is a rare clinical condition characterized by formation of a circumferential constriction ring around an appendage or genitalia. Cases are mostly reported in infants and young children. Early recognition and a definitive treatment are of paramount importance in order to avoid irreversible ischemia and possible auto-amputation. We describe a case of a 14-month-old child presented to casualty with a history of refusal to feed and inconsolable cry. Parents noticed a recent swelling of left third toe. On careful examination the child was found to have an acquired constriction ring secondary to a tightly wrapped hair around left third toe. An urgent surgical decompression was done by the orthopaedic team with complete resolution of symptoms. We summarized the pathophysiology of ACRS underlining the need of awareness in treating physicians. The possible medico legal implications should be kept in mind bearing a suggested link with non-accidental injury. PMID:18702819

  11. Turner syndrome associated with acquired von Willebrand disease, primary biliary cirrhosis, and inflammatory bowel disease.

    PubMed

    Sokol, Lubomir; Stueben, Eugen T; Jaikishen, Jay P; Lamarche, Maximo B

    2002-07-01

    We report a unique case of Turner syndrome associated with acquired von Willebrand disease (AvWD), primary biliary cirrhosis (PBC), and inflammatory bowel disease (IBD). During 7 years of close follow-up, the patient presented with multiple major episodes of upper and lower gastrointestinal bleeding caused by different pathogenic mechanisms, such as IBD, AvWD, gastric varices, and thrombocytopenia. AvWD mimicking familial vWD type III on laboratory testing was most probably triggered by autoimmune mechanism associated with PBC. Therapy of PBC with ursodeoxycholic acid (UDCA) resulted in significant decrease of liver enzymes followed by normalization of vWF and FVIII levels. Portosystemic shunt placement with ligation of gastric varices improved hypersplenism and severe thrombocytopenia and led to clinical stability for more than 24 months. The clinicopathological features of these disorders and of the recurrent bleeding episodes are discussed in the text along with a review of the literature. PMID:12116986

  12. Immunodeficiency in RFM/(T6xRFM)F1 mouse chimaeras with lethal host-versus-graft syndrome.

    PubMed Central

    Hard, R C; Montour, J L; Fuchs, B A

    1995-01-01

    Rather than central tolerance, the perinatal inoculation of related F1 hybrid spleen cells into inbred mice may result in host-versus-graft (HVG) reactions manifested as transient autoimmunity, or as a lethal immunodeficiency syndrome. RFM/(T6xRFM)F1 chimaeras with lethal disease die in 30 days with lymphosplenomegaly, immune complexes and impaired immune responses. The present studies used in vitro proliferation assays to show that the HVG reaction caused hyperplasia sufficient to account for the lymphosplenomegaly, while also causing severe impairment of splenic and nodal cell responses to concanavalin A (Con A) and to bacterial lipopolysaccharide (LPS). By 25 days, HVG mice could not distinguish between self and non-self as judged by mixed lymphocyte reactions (MLR) to RFM, (T6xRFM)F1 and third party A/J cells. There were no indications that host cells reactive to F1 donor cells had undergone clonal deletion, anergy or expansion. Flow cytometry revealed that donor T lymphocytes achieved stable engraftment, mostly in the nodes, despite the HVG reaction. Taken together with previous observations, these studies showed that HVG reactions in young parent F1/chimaeras can result in an immunodeficiency state which is characterized by an early appearing, profound and persistent impairment of both host and donor T and B cell functions. The results suggest that HVG reactions can contribute directly to immune deficits seen after clinical allogeneic bone marrow transplantation. PMID:7743676

  13. A case of acquired Brown syndrome after surgical repair of a medial orbital wall fracture.

    PubMed

    Seo, Il-Hun; Rhim, Jay-Won; Suh, Young-Woo; Cho, Yoonae A

    2010-02-01

    A case of acquired Brown syndrome caused by surgical repair of medial orbital wall fracture is reported in the present paper. A 23-year-old man presented at the hospital with right periorbital trauma. Although the patient did not complain of any diplopia, the imaging study revealed a blow-out fracture of the medial orbital wall. Surgical repair with a calvarial bone autograft was performed at the department of plastic surgery. The patient was referred to the ophthalmologic department due to diplopia that newly developed after surgery. The prism cover test at distant fixation showed hypotropia of the right eye, which was 4 prism diopters (PD) in primary gaze, 20 PD in left gaze, while orthophoric in right gaze. Eye movement of the right eye was markedly limited on elevation in adduction with normal elevation in abduction with intorsion in the right eye present. Forced duction test of the right eye showed restricted elevation in adduction. Computerized tomography scan of the orbits showed the right superior oblique muscle was entrapped between the autografted bone fragment and posterior margin of the fracture. When repairing medial orbital wall fracture that causes Brown syndrome, surgeons should always be careful of entrapment of the superior oblique muscle if the implant is inserted without identifying the superior and posterior margin of the orbital fracture site. PMID:20157416

  14. Antibody levels for cytomegalovirus, herpes simplex virus, and rubella in patients with acquired immune deficiency syndrome.

    PubMed Central

    Halbert, S P; Kiefer, D J; Friedman-Kien, A E; Poiesz, B

    1986-01-01

    Significantly higher proportions of patients with acquired immune deficiency syndrome (AIDS) or lymphadenopathy syndrome (LAS) were positive for antibodies to cytomegalovirus (CMV) and herpes simplex virus (HSV) compared with control groups of commercial blood donors. In contrast, no differences were found in the incidence of individuals positive for antibodies to rubella in these groups of subjects. Of those positive for antibodies to CMV and HSV in each group, the mean antibody levels were significantly higher in AIDS-LAS patients compared with the controls. The entire distribution of antibody concentrations to CMV and HSV in AIDS patients was shifted upward, so that significantly more patients showed high values and significantly fewer showed low values, indicating hyperactive humoral immune responses to these viruses. In sharp contrast, the AIDS patients with antibody levels for rubella showed the same distribution of antibody levels as did two groups of controls. No correlation was found between concentrations of CMV and HSV antibodies in individual AIDS-LAS patients. PMID:3009534

  15. Acquired von Willebrand syndrome associated with hypothyroidism: a mild bleeding disorder to be further investigated.

    PubMed

    Federici, Augusto B

    2011-02-01

    Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease (VWD). Unlike VWD, AVWS usually occurs in individuals with no personal or family history of bleeding. The prevalence of AVWS in the general population is unknown because data from large prospective studies of this syndrome are not available. Although AVWS is more frequently associated with lympho-myeloproliferative and cardiovascular disorders, it can also occur in solid tumors and in immunological and other miscellaneous conditions. Among these miscellaneous conditions, hypothyroidism has been associated with AVWS type 1 with a frequency of ~2 to 5%. In the 47 cases reported in the literature, the low VWF is apparently due to a reduction in its synthesis and/or secretion. Diagnosis of AVWS is based on assays measuring the level and activity of von Willebrand factor (VWF). These tend to be low, whereas factor VIII (FVIII) coagulant activity can be normal. In patients with AVWS associated with hypothyroidism, mucocutaneous bleeding episodes are the most frequent and can be managed with local therapy and/or systemic administrations of antifibrinolytic agents and desmopressin. VWF/FVIII concentrates have been used in only a few patients. The use of thyroid hormones can reverse this abnormality. PMID:21305800

  16. Acquired von Willebrand syndrome--report of 10 cases and review of the literature.

    PubMed

    Nitu-Whalley, I C; Lee, C A

    1999-09-01

    Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with clinical and laboratory features closely resembling hereditary von Willebrand disease (vWD), arising in previously haemostatically normal individuals. We present a retrospective review of 10 cases with AvWS diagnosed over 17 years. The severity of the bleeding tendency varied from mild to severe forms. Multimers electrophoresis showed that 8/10 patients had a normal pattern similar to type 1 vWD, 1/10 had a type 2A vWD pattern (with absence of high and intermediate molecular weight multimers) and 1/10 had a type 3 vWD pattern. An inhibitor screen was performed in 6/10 patients and autoantibodies against von Willebrand factor were found in only two cases. The underlying cause/associated conditions were identified in 8/10 patients. Treatment of the bleeding diathesis was successfully achieved with desmopressin or clotting factor concentrates. Resolution of underlying hypothyroidism (in two cases) and multiple myeloma (in one case) led to normalization of the coagulation parameters. The report on this cohort of 10 patients with AvWS illustrates the complexity of AvWS and its multifactorial aetiology. A brief review of the recent literature on AvWS is also presented, with emphasis on the current opinions in pathogenesis and treatment. Acquired von Willebrand syndrome (AvWS) is an acquired bleeding disorder, characterized by a phenotype similar to the inherited von Willebrand disease (vWD), with a prolonged bleeding time and low plasma levels of factor VIII - von Willebrand factor (vWF) measurements. It occurs in patients with no family history of vWD, who present with recent onset of bleeding symptoms. AvWS appears to be associated mainly with lymphoproliferative disorders, immunological conditions and neoplasia. AvWS is a rare condition and it is difficult to conduct prospective studies, therefore it is important to document the experience with such cases. The aim of this paper is first, to

  17. Allogeneic Bone Marrow Transplantation in Patients With Primary Immunodeficiencies

    ClinicalTrials.gov

    2009-10-14

    Immunologic Deficiency Syndromes; Chediak-Higashi Syndrome; Common Variable Immunodeficiency; Graft Versus Host Disease; X-Linked Lymphoproliferative Syndrome; Familial Erythrophagocytic Lymphohistiocytosis; Hemophagocytic Lymphohistiocytosis; X-linked Agammaglobulinemia; Wiskott-Aldrich Syndrome; Chronic Granulomatous Disease; X-linked Hyper IgM Syndrome; Severe Combined Immunodeficiency; Leukocyte Adhesion Deficiency Syndrome; Virus-Associated Hemophagocytic Syndrome

  18. AIDS: Acquired Immune Deficiency Syndrome, Information and Procedural Guidelines for Providing Services to Persons with AIDS/HTLV-III.

    ERIC Educational Resources Information Center

    Montana State Dept. of Health and Environmental Sciences, Helena.

    This manual presents information about the disease, Acquired Immune Deficiency Syndrome (AIDS), and guidelines for service delivery to Montana residents who have been diagnosed with AIDS or related disorders. The first section describes the disease's causes, symptoms, and transmission; risk factors; high-risk populations; prevention suggestions;…

  19. Coping Strategies of Patients with Haemophilia as a Risk Group for AIDS (Acquired Immune Deficiency Syndrome). Brief Research Report.

    ERIC Educational Resources Information Center

    Naji, Simon; And Others

    1986-01-01

    Plans are described for a 2-year project whose major focus is the identification of ways in which patients with hemophilia and their families assimilate, interpret, and act on information about Acquired Immune Deficiency Syndrome (AIDS). Findings will be related to perceived risk, anxiety levels, and the development of coping strategies.…

  20. X-ring Turner's syndrome with combined immunodeficiency and selective gonadotropin defect.

    PubMed

    Donti, E; Nicoletti, I; Venti, G; Filipponi, P; Gerli, R; Spinozzi, F; Cernetti, C; Rambotti, P

    1989-04-01

    A rare association of chromosomal, immunological and endocrine defects is described in a young woman with short stature, recurrent pulmonary infections and primary amenorrhea. Cytogenetic studies showed a 45, X karyotype in 65% of peripheral blood lymphocytes and 46,Xr(X) (p22q27) karyotype in the remaining 35%. Severe immunodeficiency was revealed by phenotypical and functional studies and a selective gonadotropin defect was disclosed by endocrinological investigations. An attempt is made to explain the coexistence of the three abnormal pictures. PMID:2745937

  1. Economic consequences for Medicaid of human immunodeficiency virus infection

    PubMed Central

    Baily, Mary Ann; Bilheimer, Linda; Wooldridge, Judith; well, Kathryn Lang; Greenberg, Warren

    1990-01-01

    Medicaid is currently a major source of financing for health care for those with acquired immunodeficiency syndrome (AIDS) and to a lesser extent, for those with other manifestations of human immunodeficiency virus (HIV) infection. It is likely to become even more important in the future. This article focuses on the structure of Medicaid in the context of the HIV epidemic, covering epidemiological issues, eligibility, service coverage and use, and reimbursement. A simple methodology for estimating HI\\'-related Medicaid costs under alternative assumptions about the future is also explained. PMID:10113503

  2. Primary immunodeficiency syndromes associated with defective DNA double-strand break repair.

    PubMed

    Gennery, A R

    2006-01-01

    Damaging DNA double-strand breaks (DNA-DSBs) following ionizing radiation (IR) exposure, potentially lead to cell death or carcinogenesis. Non-homologous end-joining (NHEJ) is the main repair pathway employed by vertebrate cells to repair such damage. Many repair pathway proteins have been identified. The creation of many diverse lymphocyte receptors to identify potential pathogens has evolved by breaking and randomly re-sorting the gene segments coding for antigen receptors. Subsequent DNA-DSB repair utilizes the NHEJ proteins. Individuals with defective repair pathways are increasingly recognized with radiosensitivity and immunodeficiency. Patients with defects in ataxia-telangiectasia mutated, nibrin, MRE11, Rad50, Artemis, DNA ligase IV and Cernunnos-XRCC4-like factor have been identified. Most exhibit immunodeficiency, with a spectrum of presentation and overlap between conditions. Conventional treatment with immunoglobulin replacement or haematopoietic stem cell transplantation (HSCT) can be effective. A greater understanding of the molecular defect will enable better, tailored therapies to improve survival. PMID:16971555

  3. Loss-of-function mutations in the IL-21 receptor gene cause a primary immunodeficiency syndrome

    PubMed Central

    Kotlarz, Daniel; Ziętara, Natalia; Uzel, Gulbu; Weidemann, Thomas; Braun, Christian J.; Diestelhorst, Jana; Krawitz, Peter M.; Robinson, Peter N.; Hecht, Jochen; Puchałka, Jacek; Gertz, E. Michael; Schäffer, Alejandro A.; Lawrence, Monica G.; Kardava, Lela; Pfeifer, Dietmar; Baumann, Ulrich; Pfister, Eva-Doreen; Hanson, Eric P.; Schambach, Axel; Jacobs, Roland; Kreipe, Hans; Moir, Susan; Milner, Joshua D.; Schwille, Petra; Mundlos, Stefan

    2013-01-01

    Primary immunodeficiencies (PIDs) represent exquisite models for studying mechanisms of human host defense. In this study, we report on two unrelated kindreds, with two patients each, who had cryptosporidial infections associated with chronic cholangitis and liver disease. Using exome and candidate gene sequencing, we identified two distinct homozygous loss-of-function mutations in the interleukin-21 receptor gene (IL21R; c.G602T, p.Arg201Leu and c.240_245delCTGCCA, p.C81_H82del). The IL-21RArg201Leu mutation causes aberrant trafficking of the IL-21R to the plasma membrane, abrogates IL-21 ligand binding, and leads to defective phosphorylation of signal transducer and activator of transcription 1 (STAT1), STAT3, and STAT5. We observed impaired IL-21–induced proliferation and immunoglobulin class-switching in B cells, cytokine production in T cells, and NK cell cytotoxicity. Our study indicates that human IL-21R deficiency causes an immunodeficiency and highlights the need for early diagnosis and allogeneic hematopoietic stem cell transplantation in affected children. PMID:23440042

  4. Low T3 syndrome is a strong predictor of poor outcomes in patients with community-acquired pneumonia

    PubMed Central

    Liu, Jinliang; Wu, Xuejie; Lu, Fang; Zhao, Lifang; Shi, Lingxian; Xu, Feng

    2016-01-01

    Low T3 syndrome was previously reported to be linked to poor clinical outcomes in critically ill patients. The aim of this study was to evaluate the predictive power of low T3 syndrome for clinical outcomes in patients with community-acquired pneumonia (CAP). Data for 503 patients were analyzed retrospectively, and the primary end point was 30-day mortality. The intensive care unit (ICU) admission rate and 30-day mortality were 8.3% and 6.4% respectively. The prevalence of low T3 syndrome differed significantly between survivors and nonsurvivors (29.1% vs 71.9%, P < 0.001), and low T3 syndrome was associated with a remarkable increased risk of 30-day mortality and ICU admission in patients with severe CAP. Multivariate logistic regression analysis produced an odds ratio of 2.96 (95% CI 1.14–7.76, P = 0.025) for 30-day mortality in CAP patients with low T3 syndrome. Survival analysis revealed that the survival rate among CAP patients with low T3 syndrome was lower than that in the control group (P < 0.01). Adding low T3 syndrome to the PSI and CURB-65 significantly increased the areas under the ROC curves for predicting ICU admission and 30-day mortality. In conclusion, low T3 syndrome is an independent risk factor for 30-day mortality in CAP patients. PMID:26928863

  5. Risk factor analysis among men referred for possible acquired immune deficiency syndrome.

    PubMed

    Newell, G R; Mansell, P W; Wilson, M B; Lynch, H K; Spitz, M R; Hersh, E M

    1985-01-01

    Responses to a lifestyle questionnaire among 13 patients with Kaposi's sarcoma and 18 with an opportunistic infection were compared with those of 29 symptom-free referred individuals. Odds ratios (OR) with 95% confidence limits were calculated as an estimate of risk. Significantly elevated odds ratios (P less than 0.05) were found for cigarette smoking (OR = 3.4), marijuana use (OR = 3.7), nitrite use (OR = 5.5), frequenting bathhouses (OR = 7.6), prior syphilis (OR = 3.4), and fist-rectal sexual practices (OR = 3.5). A response gradient for the risk estimates was found for marijuana use (OR = 2.7 for occasional, OR = 4.3 for frequent use); nitrites (OR = 4.0 for occasional; OR = 6.3 for frequent use); and prior syphilis (OR = 2.9 for one to two previous infections and 9.0 for three or more). We believe the evidence is now sufficient to recommend preventive practices which may reduce the male homosexual's risk for developing acquired immune deficiency syndrome, Kaposi's sarcoma, and/or opportunistic infections. These include cessation of cigarette smoking, marijuana use, and nitrite inhalation; reduction in number of anonymous sexual partners to decrease risk of sexually transmitted diseases; and avoidance of fisting. PMID:3839923

  6. Predictive factors for the Nursing Diagnoses in people living with Acquired Immune Deficiency Syndrome 1

    PubMed Central

    da Silva, Richardson Augusto Rosendo; Costa, Romanniny Hévillyn Silva; Nelson, Ana Raquel Cortês; Duarte, Fernando Hiago da Silva; Prado, Nanete Caroline da Costa; Rodrigues, Eduardo Henrique Fagundes

    2016-01-01

    Abstract Objective: to identify the predictive factors for the nursing diagnoses in people living with Acquired Immune Deficiency Syndrome. Method: a cross-sectional study, undertaken with 113 people living with AIDS. The data were collected using an interview script and physical examination. Logistic regression was used for the data analysis, considering a level of significance of 10%. Results: the predictive factors identified were: for the nursing diagnosis of knowledge deficit-inadequate following of instructions and verbalization of the problem; for the nursing diagnosis of failure to adhere - years of study, behavior indicative of failure to adhere, participation in the treatment and forgetfulness; for the nursing diagnosis of sexual dysfunction - family income, reduced frequency of sexual practice, perceived deficit in sexual desire, perceived limitations imposed by the disease and altered body function. Conclusion: the predictive factors for these nursing diagnoses involved sociodemographic and clinical characteristics, defining characteristics, and related factors, which must be taken into consideration during the assistance provided by the nurse. PMID:27384466

  7. Liver biopsies in the acquired immune deficiency syndrome: influence of endemic disease and drug abuse.

    PubMed

    Comer, G M; Mukherjee, S; Scholes, J V; Holness, L G; Clain, D J

    1989-12-01

    A retrospective review of 48 liver biopsies in 34 patients with acquired immune deficiency syndrome (AIDS) and 10 patients with AIDS-related complex (ARC) was performed at Harlem Hospital Center to assess the diagnostic yield of liver biopsies in this distinct patient population. Among the patients, 93.2% were black and 32 were males, with a mean age of 36.7 yr. Intravenous drug abuse was a risk factor for AIDS in 81.8% of patients. Liver biopsies were particularly useful in patients with fever of unclear origin, which was positively correlated with the presence of granulomas (p = 0.01). Granulomas due to mycobacteria were present in 16 (33.3%) of the biopsies. Liver biopsy proved to be clinically significant in 14 of 17 patients (82.3%) with mycobacterial disease, or 29.3% of the liver biopsies. Chronic active hepatitis was present in 12 (29.2%) of the biopsies, and in all but one was due to non-A non-B hepatitis viruses. All patients with chronic active hepatitis were intravenous drug abusers or the sexual partners of intravenous drug abusers. Liver biopsy can provide important diagnostic information in AIDS patients. The pathological findings in this series reflect the high risk of exposure to tuberculosis and hepatitis in the intravenous drug abusers in Harlem. PMID:2596454

  8. The acquired hyperostosis syndrome: a little known skeletal disorder with distinctive radiological and clinical features.

    PubMed

    Dihlmann, W; Schnabel, A; Gross, W L

    1993-12-01

    The acquired hyperostosis syndrome (AHS) is a chronic inflammatory disorder of bone of unknown etiology. It is accompanied by circumscribed hyperostosis which can be associated with ossifying lesions at sites of tendinous and ligamentary insertions and erosive or non-erosive arthritis. The predominant location of lesions is the sternocostoclavicular region (approximately 80% of patients), less frequent are involvement of the spine, pelvis, and appendicular skeleton. In 20%-60% of cases AHS is associated with palmoplantar pustulosis, psoriasis, or severe acne (acne fulminans or conglobata). The X-ray appearance of AHS is a more or less homogeneous increase in density with blurred margins, which on scintiscan with labeled phosphate compounds is associated with intense accretion of tracer. These features are associated with a variable increase in the acute phase reactants and a conspicuously low increase, if any, in serum alkaline phosphatase. The therapeutic modalities which have been used so far are entirely symptomatic. Long-lasting improvement has been reported following percutaneous anti-inflammatory radiation therapy. PMID:8136615

  9. Partial albinism, immunodeficiency, hypergammaglobulinemia and Dandy-Walker cyst--a Griscelli syndrome variant.

    PubMed

    Dinakar, Chitra; Lewin, S; Kumar, Karuna R; Harshad, Sujatha R

    2003-10-01

    A 6-year-old girl presented with recurrent infections, seizures, regression of milestones, silvery hair and organomegaly. A diagnosis of Griscelli syndrome with unusual features of a Dandy Walker cyst and hypergammaglobulinemia, not previously described in literature, was made. The child was treated with supportive measures. PMID:14581742

  10. Invasive aspergillosis in primary immunodeficiencies.

    PubMed

    Almyroudis, N G; Holland, S M; Segal, B H

    2005-05-01

    Primary immunodeficiencies are rare and usually first manifest during childhood. Invasive aspergillosis is the leading cause of mortality in chronic granulomatous disease (CGD), reflecting the key role of the phagocyte NADPH oxidase in host defense against opportunistic fungi. Despite interferon-gamma prophylaxis, invasive filamentous fungal infections are a persistent problem in CGD. Key principles of management of fungal infections involve early recognition and aggressive treatment and appropriate surgical debridement of localized disease. Because CGD is a disorder of phagocyte stem cells in which the gene defects are well defined, it is a model disease to evaluate immune reconstitution through stem cell transplantation and gene therapy. Patients with the hyper-IgE syndrome with recurrent infections (Job syndrome) are prone to colonization of lung cavities (pneumatoceles) by Aspergillus species leading to local invasion and rarely disseminated infection. Other primary phagocytic disorders, T-cell disorders, and mitochondrial disorders are uncommonly associated with invasive aspergillosis. Taken together, these rare primary immunodeficiencies highlight the complex coordination of both innate and acquired pathways mediating host defense against Aspergillus infection. PMID:16110817

  11. Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias.

    PubMed

    Dicke, Christina; Schneppenheim, Sonja; Holstein, Katharina; Spath, Brigitte; Bokemeyer, Carsten; Dittmer, Rita; Budde, Ulrich; Langer, Florian

    2016-05-01

    Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that may cause life-threatening hemorrhages in patients with plasma cell dyscrasias (PCDs). Early diagnosis and treatment require a thorough understanding of its underlying pathophysiology. Two patients with IgG MGUS presented with dramatically decreased plasma von Willebrand factor (VWF) and a severe type-1 pattern on multimer analysis. A prompt response to intravenous immunoglobulins (IVIG), but not to VWF/FVIII, was consistent with accelerated immunologic clearance of plasma VWF. Another IgG MGUS patient showed a type-2 pattern and a less pronounced response to IVIG, suggesting that additional mechanism(s) contributed to AVWS evolution. In a patient with Waldenström's macroglobulinemia and severe depletion of plasma VWF, multimer analysis indicated association of the IgM paraprotein with VWF before, but not after plasmapheresis, resulting in destruction of the agarose gel and a characteristically distorted band structure of VWF multimers. A type-2 pattern with highly abnormal VWF triplets and laboratory evidence of excessive fibrinolytic activity suggested that plasmin-mediated VWF degradation contributed to AVWS in a patient with multiple myeloma (MM) and AL amyloidosis. Finally, in a patient with IgG MM, maximally prolonged PFA-100® closure times and a specific defect in ristocetin-induced platelet agglutination, both of which resolved after remission induction, indicated interference of the paraprotein with VWF binding to platelet GPIb. Importantly, in none of the six patients, circulating autoantibodies to VWF were detected by a specific in-house ELISA. In summary, when evaluating PCD patients with severe bleeding symptoms, AVWS due to various pathogenic mechanisms should be considered. PMID:27040683

  12. Duodenal mucosal T cell subpopulation and bacterial cultures in acquired immune deficiency syndrome.

    PubMed

    Budhraja, M; Levendoglu, H; Kocka, F; Mangkornkanok, M; Sherer, R

    1987-05-01

    Enteric infections, chronic diarrhea frequently with no obvious etiology, and weight loss cause major morbidity and mortality in acquired immune deficiency syndrome (AIDS). Alterations in mucosal immunity may explain the increased incidence of enteric infections, and contamination of the upper small intestine with bacteria may be the cause of weight loss observed in these patients. To test this hypothesis we studied the mucosal T lymphocyte subset in duodenal mucosal biopsies in 14 AIDS and seven control patients. Duodenal fluid was also cultured for aerobic and anaerobic bacteria. There was a significant decrease among leu-3a T cells (helper/inducer) subset in AIDS. The proportion of mucosal T cells reacting with leu-2a (cytotoxic/suppressor) was significantly increased in AIDS patients. These patients also had a significant reversal of the normal mucosal helper/suppressor T cell ratio. There was no change in the number of leu-7 cells (cells mediate natural killer and antibody-dependent cellular cytotoxicity) as compared to controls. All patients with diarrhea and three of five patients without diarrhea had bacteria in their duodenal fluid. Mean number of organisms was 4.5 X 10(4)/ml. Cultures were negative in all control subjects. The results reveal that the abnormalities of T cell subpopulation in the blood of AIDS patients also occur in their duodenal mucosa. This immunological abnormality is associated with the bacterial colonization of upper gastrointestinal tract which may explain the diarrhea and weight loss observed in majority of our patients. The results also indicate that increased incidence of enteric infections in AIDS may be explained on the basis of altered mucosal immunity. PMID:2953237

  13. Lack of multimer organization of von Willebrand factor in an acquired von Willebrand syndrome.

    PubMed

    Casonato, A; Pontara, E; Doria, A; Bertomoro, A; Cattini, M G; Gambari, P F; Girolami, A

    2002-03-01

    We report a case of acquired von Willebrand syndrome (AVWS) in a 20-year-old-woman with systemic lupus erythematosus, in whom severe bleeding complications followed kidney biopsy. Coagulation studies demonstrated undetectable levels of ristocetin-induced platelet aggregation (RIPA), von Willebrand factor antigen (VWF:Ag) and VWF ristocetin cofactor activity (VWF:RCo), associated with significantly prolonged bleeding time; unlike type 3 von Willebrand disease (VWD), platelet VWF was reduced but not undetectable. The plasma VWF multimer pattern was characterized by the presence of only two bands, one of low molecular weight (MW) running as the protomer of plasma VWF in normals, the other of abnormally high MW without detectable intermediate multimers; this pattern resembles that of VWF present in endothelial cells. A search for an anti-VWF antibody demonstrated the presence of an inhibitor at high titre. This anti-VWF antibody did not interfere in the interaction of VWF with platelet glycoprotein (GP) Ib through the A1 domain, and did not react with the A2 domain of VWF; instead, it seemed to modify the relative representation of high and low MW VWF multimers released by normal human umbilical vein endothelial cells (HUVEC). After Azathioprine and corticosteroid treatment, the anti-VWF antibody disappeared and the patient's haemostatic profile normalized, except for the platelet VWF content which still remained decreased. We suggest that the anti-VWF antibody present in the AVWS described compromised both circulating VWF levels and their multimeric organization, inducing the maintenance of the multimer structure that VWF normally has before or in the early phase after secretion from endothelial cells. PMID:11886398

  14. Acquired von Willebrand syndrome with a type 2B phenotype: diagnostic and therapeutic dilemmas.

    PubMed

    Scepansky, Ellen; Othman, Maha; Smith, Hedy

    2014-01-01

    In this report, we provide evidence of an acquired von Willebrand syndrome (AVWS) with a type 2B phenotype rather than the expected type 1 or 2A. The patient was referred prior to surgical removal of a fibrous mass within the maxillary sinus. His first bleeding 7 years earlier following a retinal tear had been complicated by monocular blindness. Several mucocutanous bleedings followed. Hematological investigations revealed von Willebrand factor (VWF):Ag 91 IU/ml, factor VIII 86 IU/ml, VWF:RCo 34 IU/ml and profound thrombocytopenia with platelet clumping. VWF multimer analysis showed a loss of high-molecular-weight multimers and his plasma aggregated normal platelets under low ristocetin concentration, consistent with type 2B von Willebrand disease (VWD). Sequencing of VWF exon 28 and of the platelet GP1BA gene to investigate the possibility of platelet-type VWD failed to reveal mutations. Serum protein electrophoresis showed a monoclonal IgG protein and led to the diagnosis of monoclonal gammopathy of unknown significance (MGUS), raising suspicion of an AVWS. Over 2 years, he experienced severe gingival bleedings and traumatic intracerebral hemorrhage. Following debridement of the sinus mass, the patient required 20 units of packed red blood cells, despite high-dose Humate-P, continuous Amicar and twice-daily platelet transfusions. Bleeding finally ceased following infusion of activated factor VIIa. A history of prior uncomplicated vasectomy and tendon laceration, no family history of bleeding, the inability to identify a causative mutation in either exon 28 VWF or platelet GP1BA and the MGUS led to diagnosis of AVWS with a type 2B phenotype. This case highlights the difficulties in assigning a diagnosis and the management of bleeding in a patient with an atypical presentation of AVWS. PMID:24296552

  15. A hepatitis C-positive patient with new onset of nephrotic syndrome and systemic amyloidosis secondary to common variable immunodeficiency.

    PubMed

    Turkmen, Kultigin; Anil, Melih; Solak, Yalcin; Atalay, Huseyin; Esen, Hasan; Tonbul, Halil Z

    2010-01-01

    Common variable immunodeficiency (CVID) is a heterogenous group of predominantly antibody-deficiency disorders that make up the greatest proportion of patients with symptomatic primary hypogammaglobulinemia. The rare coincidence of amyloidosis and hypogammaglobulinemia has been reported previously. Contrary to the usual insidious, slowly progressive disease following hepatitis C infection, a rapidly progressive cirrhotic form can develop in hypogammaglobulinemic patients. We report a HCV-positive patient with a new onset of nephrotic syndrome and systemic amyloidosis secondary to CVID. Blood analyses showed serum creatinine of 1.8 mg/dL and serum albumin of 3.1 gm/dL; 24-h urinary protein was 11 800 mg/day. Serum immunoglobulin levels were IgG 340 mg/dL, IgM 18 mg/dL, IgA 11 mg/dL. Duodenal biopsy revealed AA-type amyloidosis with potassium permanganate and Congo red staining. After a month of antiproteinuric therapy, the proteinuria was reduced to 3350 mg/day. PMID:20697163

  16. A Novel SMAD4 Mutation Causing Severe Juvenile Polyposis Syndrome with Protein Losing Enteropathy, Immunodeficiency, and Hereditary Haemorrhagic Telangiectasia

    PubMed Central

    Johansson, Joel; Sahin, Christofer; Pestoff, Rebecka; Ignatova, Simone; Forsberg, Pia; Edsjö, Anders; Ekstedt, Mattias; Stenmark Askmalm, Marie

    2015-01-01

    Juvenile polyposis syndrome (JPS) is a rare genetic disorder characterized by juvenile polyps of the gastrointestinal tract. We present a new pathogenic mutation of the SMAD4 gene and illustrate the need for a multidisciplinary health care approach to facilitate the correct diagnosis. The patient, a 47-year-old Caucasian woman, was diagnosed with anaemia at the age of 12. During the following 30 years, she developed numerous gastrointestinal polyps. The patient underwent several operations, and suffered chronic abdominal pain, malnutrition, and multiple infections. Screening of the SMAD4 gene revealed a novel, disease-causing mutation. In 2012, the patient suffered hypoalbuminemia and a large polyp in the small bowel was found. Gamma globulin was given but the patient responded with fever and influenza-like symptoms and refused more treatment. The patient underwent surgery in 2014 and made an uneventful recovery. At follow-up two months later albumin was 38 g/L and IgG was 6.9 g/L. Accurate diagnosis is essential for medical care. For patients with complex symptomatology, often with rare diseases, this is best provided by multidisciplinary teams including representatives from clinical genetics. Patients with a SMAD4 mutation should be followed up both for JPS and haemorrhagic hereditary telangiectasia and may develop protein loosing enteropathy and immunodeficiency. PMID:25705527

  17. Deficient Peptide Loading and MHC Class II Endosomal Sorting in a Human Genetic Immunodeficiency Disease: the Chediak-Higashi Syndrome

    PubMed Central

    Faigle, Wolfgang; Raposo, Graça; Tenza, Daniele; Pinet, Valérie; Vogt, Anne B.; Kropshofer, Harald; Fischer, Alain; de Saint-Basile, Geneviève; Amigorena, Sebastian

    1998-01-01

    The Chediak-Higashi syndrome (CHS) is a human recessive autosomal disease caused by mutations in a single gene encoding a protein of unknown function, called lysosomal-trafficking regulator. All cells in CHS patients bear enlarged lysosomes. In addition, T- and natural killer cell cytotoxicity is defective in these patients, causing severe immunodeficiencies. We have analyzed major histocompatibility complex class II functions and intracellular transport in Epstein Barr Virus–transformed B cells from CHS patients. Peptide loading onto major histocompatibility complex class II molecules and antigen presentation are strongly delayed these cells. A detailed electron microscopy analysis of endocytic compartments revealed that only lysosomal multilaminar compartments are enlarged (reaching 1–2 μm), whereas late multivesicular endosomes have normal size and morphology. In contrast to giant multilaminar compartments that bear most of the usual lysosomal markers in these cells (HLA-DR, HLA-DM, Lamp-1, CD63, etc.), multivesicular late endosomes displayed reduced levels of all these molecules, suggesting a defect in transport from the trans-Golgi network and/or early endosomes into late multivesicular endosomes. Further insight into a possible mechanism of this transport defect came from immunolocalizing the lysosomal trafficking regulator protein, as antibodies directed to a peptide from its COOH terminal domain decorated punctated structures partially aligned along microtubules. These results suggest that the product of the Lyst gene is required for sorting endosomal resident proteins into late multivesicular endosomes by a mechanism involving microtubules. PMID:9606205

  18. Humoral immunodeficiency with facial dysmorphology and limb anomalies: a new syndrome.

    PubMed

    Hoffman, H M; Bastian, J F; Bird, L M

    2001-01-01

    We report a 6 year old girl with an isolated humoral immune deficiency and a unique combination of dysmorphic features. Physical findings include microcephaly, micrognathia, sickle shaped eyebrows, hypoplastic alae nasi, thenar hypoplasia, partial 4-5 syndactyly of toes, recessed great toes, anterior anus, and hypoplastic labia minora. Radiographic findings include triphalangeal thumbs and hypoplastic first metatarsals. She has postnatal growth retardation and her development is substantially slower than her twin's. Her clinical course has been complicated by recurrent sinopulmonary infections and pneumococcal bacteraemia. Laboratory evaluation revealed hypogammaglobulinaemia, absent B cells, and a 46,XX karyotype. A review of the literature and the London Dysmorphology Database did not produce any recognizable syndromes that match her constellation of findings. She may represent a unique syndrome of unknown etiology. PMID:11152140

  19. Computer simulations of the interaction of human immunodeficiency virus (HIV) aspartic protease with spherical gold nanoparticles: implications in acquired immunodeficiency syndrome (AIDS).

    PubMed

    Whiteley, Chris G; Lee, Duu-Jong

    2016-09-01

    The interaction of gold nanoparticles (AuNP) with human immune-deficiency virus aspartic protease (HIVPR) is modelled using a regime of molecular dynamics simulations. The simulations of the 'docking', first as a rigid-body complex, and eventually through flexible-fit analysis, creates 36 different complexes from four initial orientations of the nanoparticle strategically positioned around the surface of the enzyme. The structural deviations of the enzymes from the initial x-ray crystal structure during each docking simulation are assessed by comparative analysis of secondary structural elements, root mean square deviations, B-factors, interactive bonding energies, dihedral angles, radius of gyration (R g), circular dichroism (CD), volume occupied by C α , electrostatic potentials, solvation energies and hydrophobicities. Normalisation of the data narrows the selection from the initial 36 to one 'final' probable structure. It is concluded that, after computer simulations on each of the 36 initial complexes incorporating the 12 different biophysical techniques, the top five complexes are the same no matter which technique is explored. The significance of the present work is an expansion of an earlier study on the molecular dynamic simulation for the interaction of HIVPR with silver nanoparticles. This work is supported by experimental evidence since the initial 'orientation' of the AgNP with the enzyme is the same as the 'final' AuNP-HIVPR complex generated in the present study. The findings will provide insight into the forces of the binding of the HIVPR to AuNP. It is anticipated that the protocol developed in this study will act as a standard process for the interaction of any nanoparticle with any biomedical target. PMID:27483476

  20. Antifungal activity of Arctotis arctotoides (L.f.) O. Hoffm. and Gasteria bicolor Haw. against opportunistic fungi associated with human immunodeficiency virus/acquired immunodeficiency syndrome

    PubMed Central

    Otang, Wilfred M.; Grierson, Donald S.; Ndip, Roland N.

    2012-01-01

    Background: In South Africa where many patients are immunocompromised as a result of the AIDS pandemic, opportunistic fungal infections such as candidiasis caused mainly by Candida albicans are common. Arctotis arctotoides and Gasteria bicolor are two plants which are frequently and commonly used in traditional medicine in the treatment of HIV patients. Aim: The aim of this study was to investigate the antifungal activity of A. arctotoides and G. bicolor against opportunistic fungi common in HIV/AIDS patients. Materials and Methods: The agar diffusion and micro-dilution methods were used to determine the antifungal activities of the medicinal plant extracts against 10 opportunistic fungi. Results: All the hexane and acetone extracts were active against at least one of the fungi with zones of inhibition varying from 8 to 32 mm, while none of the aqueous extracts was active against any of the fungi. The inhibitory activity of the active extracts, based on the overall mean inhibition diameters, was in the order: A. arctotoides (hexane) > A. arctotoides (acetone) > G. bicolor (hexane) > G. bicolor (acetone). The most susceptible fungi, based on the overall mean diameter of growth inhibition, were Candida glabrata, C. krusei, and Microsporum canis, while Cyptococcus neoformans, Trycophyton tonsurans, and Microsporum gypseum were not susceptible to any of the extracts even at 5 mg/ml which was the highest concentration used. Conclusion: This study validates the use of these plants in traditional medicine in the treatment of secondary fungal infections in HIV/AIDS patients. PMID:22701287

  1. Computer simulations of the interaction of human immunodeficiency virus (HIV) aspartic protease with spherical gold nanoparticles: implications in acquired immunodeficiency syndrome (AIDS)

    NASA Astrophysics Data System (ADS)

    Whiteley, Chris G.; Lee, Duu-Jong

    2016-09-01

    The interaction of gold nanoparticles (AuNP) with human immune-deficiency virus aspartic protease (HIVPR) is modelled using a regime of molecular dynamics simulations. The simulations of the ‘docking’, first as a rigid-body complex, and eventually through flexible-fit analysis, creates 36 different complexes from four initial orientations of the nanoparticle strategically positioned around the surface of the enzyme. The structural deviations of the enzymes from the initial x-ray crystal structure during each docking simulation are assessed by comparative analysis of secondary structural elements, root mean square deviations, B-factors, interactive bonding energies, dihedral angles, radius of gyration (R g), circular dichroism (CD), volume occupied by C α , electrostatic potentials, solvation energies and hydrophobicities. Normalisation of the data narrows the selection from the initial 36 to one ‘final’ probable structure. It is concluded that, after computer simulations on each of the 36 initial complexes incorporating the 12 different biophysical techniques, the top five complexes are the same no matter which technique is explored. The significance of the present work is an expansion of an earlier study on the molecular dynamic simulation for the interaction of HIVPR with silver nanoparticles. This work is supported by experimental evidence since the initial ‘orientation’ of the AgNP with the enzyme is the same as the ‘final’ AuNP-HIVPR complex generated in the present study. The findings will provide insight into the forces of the binding of the HIVPR to AuNP. It is anticipated that the protocol developed in this study will act as a standard process for the interaction of any nanoparticle with any biomedical target.

  2. Practical diagnostic testing for human immunodeficiency virus.

    PubMed Central

    Jackson, J B; Balfour, H H

    1988-01-01

    Since the discovery of human immunodeficiency virus (HIV) as the causative agent of acquired immunodeficiency syndrome in 1983, there has been a proliferation of diagnostic tests. These assays can be used to detect the presence of HIV antibody, HIV antigen, HIV ribonucleic and deoxyribonucleic acids, and HIV reverse transcriptase. Enzyme-linked immunosorbent assays, Western blot, radioimmunoprecipitation assays, indirect immunofluorescence assays, reverse transcriptase assays, and several molecular hybridization techniques are currently available. Enzyme-linked immunosorbent, Western blot, and indirect immunofluorescence assays for HIV antibody are very sensitive, specific, and adaptable to most laboratories. An enzyme-linked immunosorbent assay for HIV antigen is also readily adaptable to most laboratories and will be commercially available soon. While the other assays are more tedious, they are valuable confirmatory tests and are suitable for reference laboratories. The biohazards of performing HIV testing can be minimized with proper biosafety measures. Images PMID:3060241

  3. Public perceptions about HIV/AIDS and discriminatory attitudes toward people living with acquired immunodeficiency syndrome in Iran.

    PubMed

    Masoudnia, Ebrahim

    2015-01-01

    Negative and discriminatory attitudes towards people living with HIV/AIDS (PLWHA) are one of the biggest experienced challenges by people suffering from HIV, and these attitudes have been regarded as a serious threat to the fundamental rights of all infected people who are affected or associated with this disease in Iran. This study aimed to determine the relationship between public perception about HIV/AIDS and discriminatory attitudes toward PLWHA . The present study was conducted using a descriptive and survey design. Data were collected from 450 patients (236 male and 214 female) in Tehran and Yazd cities. The research instruments were modified HIV-related knowledge/attitude and perception questions about PLWHA, and discriminatory attitudes toward PLWHA. The results showed that prevalence of discriminatory attitudes toward PLWHA in the studied population was 60.0%. There was a significant negative correlation between citizens' awareness about HIV/AIDS, HIV-related attitudes, negative perception toward people with HIV/AIDS symptoms and their discriminatory attitudes toward PLWHA (p < .01). The hierarchical multiple regression analysis showed that components of public perception about HIV/AIDS explained for 23.7% of the variance of discriminatory attitudes toward PLWHA. Negative public perceptions about HIV/AIDS in Iran associated with discriminatory attitudes toward PLWHA and cultural beliefs in Iran tend to stigmatize and discriminate against the LWHA. PMID:26726933

  4. Eleven Polish patients with microcephaly, immunodeficiency, and chromosomal instability: The Nijmegan breakage syndrome

    SciTech Connect

    Chrzanowska, K.H.; Krajewska-Walasek, M.; Gutkowska, A.

    1995-07-03

    We report on 11 patients with 8 independent families (3 pairs of sibs) with a complex clinical pattern including microcephaly, peculiar {open_quotes}bird-like{close_quotes} face, growth retardation, and, in some cases, mild-to-moderate mental deficiency. Most of the patients have recurring respiratory tract infections. One girl has developed B-cell lymphoma. A detailed anthropometric study of 15 physical parameters, including 3 cephalic traits, was performed. It was possible to study the chromosomes of PHA-stimulated lymphocytes in all of the patients. We found structural aberrations with multiple rearrangements, preferentially involving chromosomes 7 and 14 in a proportion of metaphases in all individuals. Profound humoral and cellular immune defects were observed. Serum AFP levels were within normal range. Radioresistant DNA synthesis was strongly increased in all 8 patients who were hitherto studied in this respect. Our patients fulfill the criteria of the Nijmegen breakage syndrome, which belongs to the growing category of ataxia telangiectasia-related genetic disorders. In light of the increased predisposition to malignancy in this syndrome, an accurate diagnosis is important for the patient. 27 refs., 5 figs., 4 tabs.

  5. A Glimpse of the Early Years of the Human Immunodeficiency Virus Epidemic: A Fellow's Experience in 2014

    PubMed Central

    Colasanti, Jonathan; Armstrong, Wendy S.

    2014-01-01

    Human immunodeficiency virus (HIV) is a manageable chronic disease in the United States, yet the first author's experience on a general infectious diseases (ID) consult service illustrates that certain areas of the United States still experience high rates of acquired immune deficiency syndrome-related complications. PMID:25734112

  6. Vaccine-associated paralytic poliomyelitis and BCG-osis in an immigrant child with severe combined immunodeficiency syndrome - Texas, 2013.

    PubMed

    Trimble, Robert; Atkins, Jane; Quigg, Troy C; Burns, Cara C; Wallace, Gregory S; Thomas, Mary; Mangla, Anil T; Infante, Anthony J

    2014-08-22

    Poliovirus transmission has been eliminated in most of the world through the use of inactivated poliovirus vaccine (IPV) and live, attenuated oral poliovirus vaccine (OPV). In the United States, use of OPV was discontinued by the year 2000 because of the potential for vaccine-associated paralytic polio (VAPP); an average of eight cases were reported each year in the United States during 1980-2000. Polio eradication efforts in other parts of the world continue to rely on OPV to take advantage of transmission of poliovirus vaccine strains to unvaccinated persons in the population, lower cost, and ease of administration. In 2013, an infant aged 7 months who recently immigrated to the United States from India was referred to a hospital in San Antonio, Texas. The infant had fever, an enlarging skin lesion in the deltoid region with axillary lymphadenopathy, decreased activity, and inability to bear weight on the left leg, progressing to paralysis of the left leg over a 6-week period. Recognition of lymphopenia on complete blood count led to immune evaluation, which revealed the presence of severe combined immunodeficiency syndrome (SCIDS), an inherited disorder. A history of OPV and bacille Calmette-Guérin (BCG) vaccination in India led to the diagnoses of VAPP and BCG-osis, which were confirmed microbiologically. This report demonstrates the importance of obtaining a comprehensive clinical history in a child who has recently immigrated to the United States, with recognition that differing vaccine practices in other countries might require additional consideration of potential etiologies. PMID:25144542

  7. [Case report: Löffler's syndrome due to Ascaris lumbricoides mimicking acute bacterial community--acquired pneumonia].

    PubMed

    Acar, Ali; Oncül, Oral; Cavuşlu, Saban; Okutan, Oğuzhan; Kartaloğlu, Zafer

    2009-01-01

    In this study we present a patient with Loeffler's syndrome caused by Ascaris lumbricoides who presented with the clinical findings of community-acquired pneumonia (CAP). Our patient, who was twenty-five years old, and who had had symptoms such as coughing, expectorating, dyspnea and fever for approximately ten days, was hospitalized. We auscultated polyphonic rhonchuses at the both hemithoraxes. A chest X-ray revealed bilateral lower zone patch consolidation. Acute bacterial community acquired pneumonia (CAP) was diagnosed due to these findings and empirical antibiotic treatment was begun. Repeated sputum Gram stains were negative, and both sputum and blood cultures were sterile. A sputum smear was negative for acid-fast bacilli. The patient's fever and respiratory complaint did not respond to the empirical antibiotics therapy. During the course of advanced investigations, we measured peripheric eosinophilia, and high levels of total Eo and total IgE, and observed Ascaris lumbricoides eggs during stool examination. The patient was given a diagnosis of Loeffler's syndrome. Thereupon the patient was treated successfully with one dose of albendazol 400 mg. In conclusion, we suggest that Loeffler's syndrome must be considered early in the differential diagnosis for CAP when peripheric eosinophilia is seen in patients if they live in an endemic area for parasitic disease. PMID:19851973

  8. [Acquired von Willebrand's disease in the course of severe primary hypothyroidism in a patient with autoimmune polyglandular syndrome type 3].

    PubMed

    Lubińska, Monika; Swiatkowska-Stodulska, Renata; Kazimierska, Ewa; Sworczak, Krzysztof

    2008-01-01

    The case of a 20-year old female, who had been followed because of von Willebrand disease (vWD) was presented in this paper . She had a past history of menorrhagia and bleeding after dental procedures and the activity of von Willebrand factor (vWF) was decreased. Because of suggestive clinical features, the workup for hypothyroidism was performed and the patient was found to have severe hypothyroidism due to Hashimoto thyroiditis. After the institution of replacement therapy with levothyroxine, von Willebrand factor activity returned to normal range and symptoms of von Willebrand disease disappeared. Based on these findings, the diagnosis of acquired von Willebrand syndrome (AvWS) due to hypothyroidism was made. The development of myasthenia led to the final diagnosis of autoimmune polyglandular syndrome type 3 (APS) with myasthenia gravis and vitiligo. PMID:18335399

  9. Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG

    PubMed Central

    Zoghi, Behyar; Shaughnessy, Paul; Lyons, Roger M.; Helmer, Richard; Bachier, Carlos; LeMaistre, C. Frederick

    2015-01-01

    The use of high dose chemotherapy followed by autologous hematopoietic stem cell transplantation for remission consolidation after initial induction represents standard of care for patients with multiple myeloma. Patients with myeloma and Acquired von Willebrand Syndrome (AVWS) undergoing autologous stem cell transplant (ASCT) are at significant risk of bleeding due to the profound thrombocytopenia, low Factor VIII levels, fever, and toxicities associated with the preparative regimen. We report a patient with AVWS associated with multiple myeloma who underwent autologous stem cell transplants as consolidation after initial induction and again at relapse. He was successfully treated with high dose intravenous immunoglobulin (IVIG) prior to each transplant with rapid resolution of AVWS. PMID:25922770

  10. [Bovine immunodeficiency virus: short review].

    PubMed

    Bouillant, A M; Archambault, D

    1990-01-01

    A bovine visna-like virus was isolated by Van Der Maaten et al (1972) but it did not draw attention since, at that time, most efforts were directed towards research on bovine leukemia virus. However, new interest was shown on the bovine visna-like virus after the isolation of the human immunodeficiency virus (HIV), because of the urgent need for developing animal models for the acquired immunodeficiency syndrome (AIDS). The purpose of this paper is to describe the different stages of the identification of the bovine virus and to up-date knowledge about it. The bovine visna-like virus has recently been named the bovine immuno-deficiency-like virus (BIV) and is the sole bovine lentivirus known to-date. BIV shares morphologic, antigenic and genomic characteristics with other lentiviruses. It grows and induces large syncytia in vitro and generates virus-productive and latent infections in cell culture. It causes persistent infection and slow progressive disease in cattle and probably in sheep. As target cells of the virus are leukocytes, the type of which is unknown, perturbations of the immune system are expected. Consequently, BIV may potentiate the occurrence of secondary infections and play a role in retroviral, multiple infections. It is not oncogenic. Transmission appears to occur in cattle by contact, but evidence of transmission in human beings has not been shown. Finally, BIV may be a potential model in vitro and in vivo for HIV and AIDS. PMID:1963056