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Sample records for syndrome immunodeficiency resulting

  1. Radiological pulmonary manifestations of acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Marchiori, Edson; Melo, Alessandro Severo Alves de; Ossa, Alfonso Jaramillo

    1999-01-01

    In this article are reviewed the principal radiologic manifestations of inflammatory and tumoral diseases the compromise the lungs of patients with acquired immunodeficiency syndrome. In the group of inflammatory diseases the radiologic aspects of pneumocystosis, cytomegalovirus disease, cryptococcosis, tuberculosis and bacterial pneumonias are emphasized. In the neoplasic diseases' group the aspects of lymphoma and Kaposi's sarcoma are specially presented. (author)

  2. Goldenhar syndrome: a cause of secondary immunodeficiency?

    Directory of Open Access Journals (Sweden)

    De Golovine Serge

    2012-07-01

    Full Text Available Abstract Goldenhar syndrome (GS results from an aberrant development of the 1st and 2nd branchial arches. There is a wide range of clinical manifestations, the most common being microtia, hemifacial microsomia, epibulbar dermoids and vertebral malformations. We present two cases of GS and secondary immunodeficiency due to anatomical defects characteristic of this disorder. Case 1 (3-year-old female averaged 6 episodes of sinusitis and otitis media per year. Case 2 (7-year-old female also had recurrent otitis media, an episode of bacterial pneumonia, and 2 episodes of bacterial meningitis. Their immune evaluation included a complete blood count with differential, serum immunoglobulin levels and specific antibody concentrations, lymphocyte phenotyping, and mitogen and antigen responses, the results of which were all within normal ranges. Both children demonstrated major structural abnormalities of the inner and middle ear structures, retention of fluid in mastoid air cells, and chronic sinusitis by computed tomography. These two cases illustrate how a genetically-associated deviation of the middle ear cleft can cause recurrent infections and chronic inflammation of the middle ear and adjacent sinuses, even meninges, leading to a greatly reduced quality of life for the child and parents.

  3. Acquired immunodeficiency syndrome associated with blood-product transfusions

    International Nuclear Information System (INIS)

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

    1983-01-01

    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

  4. Relationship between current level of immunodeficiency and non-acquired immunodeficiency syndrome-defining malignancies

    DEFF Research Database (Denmark)

    Reekie, Joanne; Kosa, Csaba; Engsig, Frederik

    2010-01-01

    In the combined antiretroviral therapy (cART) era, non-acquired immunodeficiency syndrome (AIDS)-defining malignancies account for more morbidity and mortality in human immunodeficiency virus-infected patients than AIDS-defining malignancies. However, conflicting data have been reported...

  5. Bronchogenic carcinoma in acquired immunodeficiency syndrome - report of two cases

    International Nuclear Information System (INIS)

    Siciliano, Antonio Alexandre de Oliveira; Melo, Alessandro Severo Alves de; Marchiori, Edson

    1999-01-01

    The authors report two cases of bronchogenic carcinoma in patients with acquired immunodeficiency syndrome. The first patient, a ee-year-old male, developed a left hilar adenocarcinoma, with spleen and bilateral adrenal metastases. The disease progressed unfavourably, resulting in the patient's death in less than a month. The second patient, a 47-year-old male, developed a large mass in the left upper lobe, with invasion of the thoracic wall and destruction of adjacent ribs. The histopathologic study revealed a non-oat-cell carcinoma. Both patients received palliative treatment since diagnosis was established late in the course of the disease. Recent studies suggest an association between bronchogenic carcinoma and human immunodeficiency virus infection. However, an actual increase in the prevalence of bronchogenic carcinoma in HIV-positive patients remains controversial. (author)

  6. Ocular Manifestations of Acquired Immunodeficiency Syndrome.

    Science.gov (United States)

    Kim, Young Shin; Sun, Hae Jung; Kim, Tae Hyong; Kang, Kui Dong; Lee, Sung Jin

    2015-08-01

    To investigate the patterns and risk factors of the ocular manifestations of acquired immunodeficiency syndrome (AIDS) and their correlation with CD4+ count in the era of highly active antiretroviral therapy (HAART). This retrospective study examined 127 AIDS patients who presented to Soonchunhyang University Hospital. Data were collected from patient interviews, clinical examinations, and laboratory investigations. Ophthalmologic examinations included the best-corrected visual acuity, intraocular pressure, anterior segment and adnexal examination, and dilated fundus examination. Of the 127 patients with AIDS, 118 were on HAART and 9 were not. The mean CD4+ count was 266.7 ± 209.1 cells/µL. There were ocular manifestations in 61 patients (48.0%). The incidence of anterior segment manifestations was higher than posterior segment manifestations at 28.3% and 19.7%, respectively. The mean CD4+ count was significantly (p AIDS. In this study, anterior segment and external ocular manifestations occurred more frequently than posterior segment manifestations. Also, the mean CD4+ count was significantly lower in patients with posterior segment ocular manifestations versus anterior segment ocular manifestations. We found that CD4+ count and age >35 years were independent risk factors for developing ocular manifestations.

  7. Immunodeficiency syndrome in a 3-year-old llama.

    OpenAIRE

    Sivasankar, M

    1999-01-01

    An adult, castrated male llama was presented for evaluation of a chronic respiratory problem. Complete blood analyses indicated a leukopenia and hypoproteinemia. Radial immunodiffusion, bone marrow core, and lymph node biopsies supported a tentative diagnosis of juvenile llama immunodeficiency syndrome. This diagnosis was confirmed by postmortem findings.

  8. Toxicity of oral radiotherapy in patients with acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Cooper, J.S.; Fried, P.R.

    1987-01-01

    Although radiotherapy is a standard form of management of head and neck tumors, treatment of the oral cavity in patients who have the acquired immunodeficiency syndrome has produced unacceptable toxicity. Five such patients are described as a warning of enhanced toxicity of oral radiotherapy in this patient population

  9. Hemorrhagic abscess in a patient with the acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Casado-Naranjo, I.; Lopez-Trigo, J.; Ferrandiz, A.; Cervello, A.; Navarro, V.

    1989-01-01

    Cerebral toxoplasmosis is frequent among patients with the acquired immunodeficiency syndrome. The common computed tomography appearance of this complication is multiple low density area with ring enhancement. We describe a very rare picture of cerebral toxoplasmosis, this is multiple hemorrhagic toxoplasmic abscess. (orig.)

  10. Cellular radiosensitivity in human severe-combined-immunodeficiency (SCID) syndromes

    International Nuclear Information System (INIS)

    Sproston, Anthony R.M.; West, Catharine M.L.; Hendry, Jolyon H.

    1997-01-01

    Purpose: The aim of the work was to establish to what extent a variety of human severe-combined-immunodeficiency (SCID) disorders are associated with in vitro cellular hypersensitivity to ionizing radiation. Materials and methods: A study was made of fibroblast strains established from individuals with adenosine deaminase deficiency, T(-)B(-) SCID, Omenn's syndrome and a SCID heterozygote. For comparison, an assessment was also made of the radiosensitivity of a series of fibroblast strains derived from: normal donors, a patient with ataxia-telangiectasia (A-T) and an A-T heterozygote. Radiosensitivity was determined using a clonogenic assay following both high (HDR) and low (LDR) dose-rate irradiation. Results: Following HDR irradiation, the fibroblast strains derived from the different human SCID disorders displayed a wide range of radiosensitivity: the adenosine deaminase deficiency cells were similar in radiosensitivity to normal fibroblasts, T(-)B(-) cells were as hypersensitive to radiation as A-T cells and the Omenn's syndrome cells showed intermediate radiosensitivity. However, whereas all four normal cell strains studied showed significant LDR sparing, none of the SCID fibroblasts did. Conclusions: These data indicate that human SCID is variable in terms of radiosensitivity depending on the particular defect. In addition, the lack of LDR sparing of radiation-induced damage suggests the involvement of some form(s) of DNA repair defect in all the human SCID syndromes

  11. Autoimmunity and dysmetabolism of human acquired immunodeficiency syndrome.

    Science.gov (United States)

    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.

  12. Cerebral computed tomography in men with acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Brun, B.; Boesen, F.; Gerstoft, J.; Nielsen, J.O.; Praestholm, J.; Rigshospitalet, Copenhagen; Hvidovre Hospital; Hvidovre Hospital; Hvidovre Hospital

    1986-01-01

    Cerebral CT scannings were performed in 19 homosexual men with the acquired immunodeficiency syndrome (AIDS). Nearly half of them (9 patients) had cortical atrophy. Three patients with toxoplasmosis had cerebral pathology, in two of them with ring enhancement while the third had an ill-defined nonspecific lesion with slight heterogeneous enhancement without ring formation. Two patients with multifocal leucoencephalopathy and non-Hodgkin's lymphoma, respectively, presented non-enhancing, low attenuating lesions at CT. (orig.)

  13. Spinal cord toxoplasmosis in human immunodeficiency virus infection/acquired immunodeficiency syndrome.

    Science.gov (United States)

    García-García, Concepción; Castillo-Álvarez, Federico; Azcona-Gutiérrez, José M; Herraiz, María J; Ibarra, Valvanera; Oteo, José A

    2015-05-01

    Neurological complications in patients with human immunodeficiency virus infection/acquired immunodeficiency syndrome (HIV/AIDS) are still common, even in the era of highly active antiretroviral therapy. Opportunistic infections, immune reconstitution, the virus itself, antiretroviral drugs and neurocognitive disorders have to be considered when establishing the differential diagnosis. Toxoplasmic encephalitis remains the major cause of space-occupying lesions in the brain of patients with HIV/AIDS; however, spinal cord involvement has been reported infrequently. Here, we review spinal cord toxoplasmosis in HIV infection and illustrate the condition with a recent case from our hospital. We suggest that most patients with HIV/AIDS and myelitis with enhanced spine lesions, multiple brain lesions and positive serology for Toxoplasma gondii should receive immediate empirical treatment for toxoplasmosis, and a biopsy should be performed in those cases without clinical improvement or with deterioration.

  14. Acquired immunodeficiency syndrome and its ocular complications

    Directory of Open Access Journals (Sweden)

    Rao Narsing

    1994-01-01

    Full Text Available Human immunodeficiency virus infection is the first major pandemic of the 20th century. At present, almost 10 million people are known to be infected with this virus, and it is estimated that by the year 2000, approximately 40 million people will be infected. Transmission of this deadly infection is predominantly by sexual contact. Individuals infected with this virus pass through several predictable stages with progressive decrease in circulating CD4+ T cells. During the advanced stage, these patients develop various opportunistic infections or malignancies, or both. It is this advanced stage that was first recognized as AIDS, which has a 100% mortality rate. The opportunistic organisms that can involve the eye in patients with AIDS include cytomegalovirus, herpes zoster, Toxoplasma gondii, Mycobacterium tuberculosis, Cryptococcus neoformans, Mycobacterium avium-intracellulare, Pneumocystis carinii, Histoplasma capsulatum, Candida, and others. Intraocular lesions from these agents often represent disseminated infections. Visual morbidity occurs secondary to retinitis due to cytomegalovirus, herpes zoster, or Toxoplasma gondii. Anti-viral agents such as ganciclovir or foscarnet are effective against cytomegalovirus infection. The role of the ophthalmologist in the diagnosis and management of AIDS is becoming increasingly important. Not only does the eye reflect systemic disease, but ocular involvement may often precede systemic manifestations. In the AIDS patient, the ophthalmologist thus has an opportunity to make not only a slight-saving, but also life-saving diagnosis of disseminated opportunistic infections.

  15. Combined Immunodeficiency in Patients With Trichohepatoenteric Syndrome

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    Frédéric Vély

    2018-05-01

    Full Text Available The syndromic diarrhea/trichohepatoenteric syndrome (SD/THE is a rare and multi-system genetic disorder caused by mutation in SKIV2L or in TTC37, two genes encoding subunits of the putative human SKI complex involved in RNA degradation. The main features are intractable diarrhea of infancy, hair abnormalities, facial dysmorphism, and intrauterine growth restriction. Immunologically this syndrome is associated with a hypogammaglobulinemia leading to an immunoglobulin supplementation. Our immune evaluation of a large French cohort of SD/THE patient revealed several immunological defects. First, switched memory B lymphocytes count is very low. Second, IFN-γ production by T and NK cells is impaired and associated with a reduced degranulation of NK cells. Third, T cell proliferation was abnormal in 3/6 TTC37-mutated patients. These three patients present with severe EBV infection and a transient hemophagocytosis which may be related to these immunological defects. Moreover, an immunological screening of patients with clinical features of SD/THE could facilitate both diagnosis and therapeutic management of these patients.

  16. Cerebral involvement in acquired immunodeficiency syndrome (AIDS)

    International Nuclear Information System (INIS)

    Krestin, G.P.; Juergens, R.; Steinbrich, W.; Diederich, N.; Koeln Univ.

    1986-01-01

    Involvement of the central nervous system in acquired immune deficiency syndrome (AIDS) is usually due to opportunistic infections; these frequently offer a difficult differential diagnostic problem. Imaging methods play an important part in the elucidation of symptoms. CT and MR findings were analysed in 13 patients with AIDS and neurological symptoms. Some infections of the central nervous system (encephalitis of unknown aetiology, cytomegalic encephalitis, meningitis) may show cerebral atrophy or even no morphological changes. Toxoplasmosis and PML are the most common opportunistic infections typical changes on CT and MR may lead to diagnosis. MR offers advantages compared with CT in its higher sensitivity for the demonstration even of small lesions. (orig.) [de

  17. An Increased Risk of Osteoporosis during Acquired Immunodeficiency Syndrome.

    Science.gov (United States)

    Annapoorna, N; Rao, G Venkateswara; Reddy, N S; Rambabu, P; Rao, K R S Samabasiva

    2004-01-01

    Osteoporosis is characterized by decreased bone mineral density and mechanistic imbalances of bone tissue that may result in reduced skeletal strength and an enhanced susceptibility to fractures. Osteoporosis in its most common form affects the elderly (both sexes) and all racial groups of human beings. Multiple environmental risk factors like acquired immune deficiency syndrome (AIDS) are believed to be one of the causes of osteoporosis. Recently a high incidence of osteoporosis has been observed in human immunodeficiency virus (HIV) infected individuals. The etiology of this occurrence in HIV infections is controversial. This problem seems to be more frequent in patients receiving potent antiretroviral therapy. In AIDS, the main suggested risk factors for the development of osteoporosis are use of protease inhibitors, longer duration of HIV infection, lower body weight before antiretroviral therapy, high viral load. Variations in serum parameters like osteocalcin, c-telopeptide, levels of elements like Calcium, Magnesium, Phosphorus, concentration of vitamin-D metabolites, lactate levels, bicarbonate concentrations, amount of alkaline phosphatase are demonstrated in the course of development of osteoporosis. OPG/RANKL/RANK system is final mediator of bone remodeling. Bone mineral density (BMD) test is of added value to assess the risk of osteoporosis in patients infected with AIDS. The biochemical markers also aid in this assessment. Clinical management mostly follows the lines of treatment of osteoporosis and osteopenia.

  18. Sweet's syndrome in association with common variable immunodeficiency.

    LENUS (Irish Health Repository)

    O'Regan, G M

    2009-03-01

    Sweet\\'s syndrome (SS), a rare reactive neutrophilic dermatosis, has been reported to occur in association with a variety of systemic disorders, categorized by von den Diesch into idiopathic, paraneoplastic, pregnancy and parainflammatory subgroups. The parainflammatory group has been well defined, and includes a wide spectrum of infectious triggers and disorders of immune dysregulation. To date, however, no cases of SS have been described in the context of common variable immunodeficiency (CVID). We report a case of paediatric-onset SS, previously reported as idiopathic, with a subsequent diagnosis of CVID.

  19. Use of etanercept in human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) patients.

    Science.gov (United States)

    Ting, Patricia T; Koo, John Y

    2006-06-01

    Etanercept (Enbrel, Amgen, Thousand Oaks, CA), a soluble p75 tumor necrosis factor receptor:FC (TNFR:FC) fusion protein for plasma cytokines, specifically tumor necrosis factor-alpha (TNF-alpha), is used in the treatment of immune-mediated rheumatic diseases. To our knowledge, the use of etanercept in patients with human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) is relatively uncommon. The main purpose of this short review is to examine the safety of etanercept in patients with HIV/AIDS. A Medline search was conducted using the keywords etanercept and HIV and/or AIDS for any published articles between 1966 to the present (September 2004). A case report, one case series, and one clinical trial pertained to the use of etanercept in HIV patients. No reports were found on the use of etanercept in AIDS. In addition, two case reports were found documenting the use of infliximab in HIV patients. Preliminary reports indicate that the administration of etanercept does not appear to increase the morbidity or mortality rates in HIV. The inhibition of TNF-alpha may actually improve the symptoms of HIV/AIDS-associated aphthous ulcers, cachexia, dementia, fatigue, and fever, as well as help manage concomitant rheumatic diseases and psoriasis. The use of etanercept shows promise for applications in disease management in patients with HIV/AIDS. Continued research efforts are necessary to establish the long-term safety and efficacy of etanercept and other biologic agents in this patient population.

  20. Management of Diabetes Mellitus in Patients with Acquired Immunodeficiency Syndrome

    Directory of Open Access Journals (Sweden)

    Miulescu Rucsandra Dănciulescu

    2014-06-01

    Full Text Available Acquired immunodeficiency syndrome (AIDS is a human immune system disease characterized by increased susceptibility to opportunistic infections, certain cancers and neurological disorders. The syndrome is caused by the human immunodeficiency virus (HIV that is transmitted through blood or blood products, sexual contact or contaminated hypodermic needles. Antiretroviral treatment reduces the mortality and the morbidity of HIV infection but is increasingly reported to be associated with increasing reports of metabolic abnormalities. The prevalence and incidence of diabetes mellitus in patients on antiretroviral therapy is high. Recently, a joint panel of American Diabetes Association (ADA and European Association for the Study of Diabetes (EASD experts updated the treatment recommendations for type 2 diabetes (T2DM in a consensus statement which provides guidance to health care providers. The ADA and EASD consensus statement concur that intervention in T2DM should be early, intensive, and uncompromisingly focused on maintaining glycemic levels as close as possible to the nondiabetic range. Intensive glucose management has been shown to reduce microvascular complications of diabetes but no significant benefits on cardiovascular diseases. Patients with diabetes have a high risk for cardiovascular disease and the treatment of diabetes should emphasize reduction of the cardiovascular factors risk. The treatment of diabetes mellitus in AIDS patients often involves polypharmacy, which increases the risk of suboptimal adherence

  1. Cystic fibrosis: a mucosal immunodeficiency syndrome

    Science.gov (United States)

    Cohen, Taylor Sitarik; Prince, Alice

    2013-01-01

    Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that regulates the transport of ions and the movement of water across the epithelial barrier. Mutations in CFTR, which form the basis for the clinical manifestations of cystic fibrosis, affect the epithelial innate immune function in the lung, resulting in exaggerated and ineffective airway inflammation that fails to eradicate pulmonary pathogens. Compounding the effects of excessive neutrophil recruitment, the mutant CFTR channel does not transport antioxidants to counteract neutrophil-associated oxidative stress. Whereas mutant CFTR expression in leukocytes outside of the lung does not markedly impair their function, the expected regulation of inflammation in the airways is clearly deficient in cystic fibrosis. The resulting bacterial infections, which are caused by organisms that have substantial genetic and metabolic flexibility, can resist multiple classes of antibiotics and evade phagocytic clearance. The development of animal models that approximate the human pulmonary phenotypes—airway inflammation and spontaneous infection—may provide the much-needed tools to establish how CFTR regulates mucosal immunity and to test directly the effect of pharmacologic potentiation and correction of mutant CFTR function on bacterial clearance. PMID:22481418

  2. Treatment of infectious complications of acquired immunodeficiency syndrome.

    Science.gov (United States)

    Furio, M M; Wordell, C J

    1985-01-01

    The infectious complications of the acquired immunodeficiency syndrome (AIDS) are discussed, and the conventional and nonconventional therapies used for these infections are reviewed. The infections most commonly encountered in patients with AIDS are Pneumocystis carinii pneumonia (58%), Candida esophagitis (31%), toxoplasmosis (21%), cytomegalovirus infections (15%), and herpes-simplex virus infections (12%). Pneumocystis carinii pneumonia is the most common life-threatening process in these patients. Trimethoprim-sulfamethoxazole (TMP-SMZ) is considered the drug of choice for its treatment. Oral candidiasis often indicates the progression to AIDS in the high-risk populations of homosexual or bisexual men, intravenous drug abusers, and individuals with hemophilia. Nystatin suspension is commonly used to treat oral candidiasis, while Candida esophagitis demands systemic therapy with ketoconazole. Toxoplasmosis most commonly manifests itself in patients with AIDS as a cerebral mass lesion. The recommended therapy includes sulfadiazine and pyrimethamine. AIDS patients frequently experience protozoal invasion of the intestinal tract with Giardia lamblia, Isospora belli, and Cryptosporidium muris. Various drugs have been tried for these infections, including quinacrine hydrochloride, metronidazole, TMP-SMZ, and spiramycin. Cytomegalovirus (CMV) infections commonly involve the lungs, gastrointestinal tract, eyes, brain, and nervous system. Attempts to treat these disseminated CMV infections with antiviral agents, including acyclovir, have not been successful. However, acyclovir has been found beneficial in the treatment of herpes-simplex virus infections. Multiple infectious complications may occur in patients with AIDS as a result of the cellular-immune deficiency associated with this disease. Until more research is done with AIDS patients, therapy must be based on the data available from the treatment of these infections in immunosuppressed patients without AIDS.

  3. Metabolic syndrome in human immunodeficiency virus positive patients

    Directory of Open Access Journals (Sweden)

    Sarita Bajaj

    2013-01-01

    Full Text Available Aims and Objectives : To assess the prevalence of metabolic syndrome (MetS in human immunodeficiency virus (HIV positive patients. Prevalence of MetS was compared in patients who were not on highly active antiretroviral therapy (HAART to patients who were on HAART. Materials and Methods: Seventy HIV positive cases were studied. Pregnant and lactating women, patients on drugs other than HAART known to cause metabolic abnormalities and those having diabetes or hypertension were excluded. Cases were evaluated for MetS by using National Cholesterol Education Program Adult Treatment Panel-III. Results: 47 cases were on HAART and 23 cases were not on HAART. Fasting Blood Glucose ≥100 mg/dl was present in 28.6% cases, out of whom 27.7% were on HAART and 30.4% were not on HAART (P = 0.8089. 12.9% cases had BP ≥130/≥85 mm Hg, out of whom 14.9% were on HAART and 8.7% were not on HAART (P = 0.4666. 42.9% cases had TG ≥150 mg/dl, out of whom 44.7% were on HAART and 39.1% were not on HAART (P = 0.6894. HDL cholesterol was low (males <40 mg/dl, females <50 mg/dl in 50% cases, out of whom 55.3% were on HAART and 39.1% were not on HAART (P = 0.2035. Conclusions: Prevalence of MetS was 20%. Majority of patients had only one component of MetS (32.9%. Low HDL was present in 50%, followed by raised triglycerides in 42.9%. Waist circumference was not increased in any of the patients. There was no statistically significant difference between those on HAART and those not on HAART in distribution of risk factors and individual components of MetS.

  4. A mathematical model on Acquired Immunodeficiency Syndrome

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    Buddhadeo Mahato

    2014-10-01

    Full Text Available A mathematical model SEIA (susceptible-exposed-infectious-AIDS infected with vertical transmission of AIDS epidemic is formulated. AIDS is one of the largest health problems, the world is currently facing. Even with anti-retroviral therapies (ART, many resource-constrained countries are unable to meet the treatment needs of their infected populations. We consider a function of number of AIDS cases in a community with an inverse relation. A stated theorem with proof and an example to illustrate it, is given to find the equilibrium points of the model. The disease-free equilibrium of the model is investigated by finding next generation matrix and basic reproduction number R0 of the model. The disease-free equilibrium of the AIDS model system is locally asymptotically stable if R0⩽1 and unstable if R0>1. Finally, numerical simulations are presented to illustrate the results.

  5. Behavioral surveillance survey regarding human immunodeficiency virus/acquired immunodeficiency syndrome among high school and junior college students

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    Bhosale S

    2010-01-01

    Full Text Available Background and Aims: It is necessary to know the baseline knowledge, attitude, and practices about human immunodeficiency virus/acquired immunodeficiency syndrome among young people and the changes in these with intervention to guide prevention efforts. Methods: A cross-sectional pre- and post-survey with health education as a method of intervention was carried out in four different randomly selected schools and junior colleges among the Class IX-XII students of both sex. Instrument developed by the World Health Organization (WHO/UNAIDS in their best practice recommendations was used for data collection. Results: Knowledge about all correct methods was present in 61.23% of the respondents. Knowledge of at least two methods of prevention was present in 70.31% of the respondents. Misconceptions about prevention were that good diet (33.42%, avoiding mosquito bite (49.71% and avoiding public toilets (65.14% could help in the prevention. With intervention, there was an improvement in the knowledge. However, the proportion of students with misconceptions did not come down. Correct knowledge about two methods of prevention also did not reach the WHO recommendation of 90%. Conclusion: It is very difficult to change the attitude and practices by a single health educational intervention and an ongoing behavior change communication is recommended.

  6. Cernunnos/XLF Deficiency: A Syndromic Primary Immunodeficiency

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    Funda Erol Çipe

    2014-01-01

    Full Text Available 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.

  7. Radionuclide brain imaging in acquired immunodeficiency syndrome (AIDS)

    International Nuclear Information System (INIS)

    Costa, D.C.; Gacinovic, S.; Miller, R.F.

    1995-01-01

    Infection with the Human Immunodeficiency Virus type 1 (HIV-1) may produce a variety of central nervous system (CNS) symptoms and signs. CNS involvement in patients with the Acquired Immunodeficiency Syndrome (AIDS) includes AIDS dementia complex or HIV-1 associated cognitive/motor complex (widely known as HIV encephalopathy), progressive multifocal leucoencephalopathy (PML), opportunistic infections such as Toxoplasma gondii, TB, Cryptococcus and infiltration by non-Hodgkin's B cell lymphoma. High resolution structural imaging investigations, either X-ray Computed Tomography (CT scan) or Magnetic Resonance Imaging (MRI) have contributed to the understanding and definition of cerebral damage caused by HIV encephalopathy. Atrophy and mainly high signal scattered white matter abnormalities are commonly seen with MRI. PML produces focal white matter high signal abnormalities due to multiple foci of demyelination. However, using structural imaging techniques there are no reliable parameters to distinguish focal lesions due to opportunistic infection (Toxoplasma gondii abscess) from neoplasm (lymphoma infiltration). It is studied the use of radionuclide brain imaging techniques in the investigation of HIV infected patients. Brain perfusion Single Photon Emission Tomography (SPET), neuroreceptor and Positron Emission Tomography (PET) studies are reviewed. Greater emphasis is put on the potential of some radiopharmaceuticals, considered to be brain tumour markers, to distinguish intracerebral lymphoma infiltration from Toxoplasma infection. SPET with 201 Tl using quantification (tumour to non-tumour radioactivity ratios) appears a very promising technique to identify intracerebral lymphoma

  8. Science and ethics of human immunodeficiency virus/acquired immunodeficiency syndrome controversies in Africa.

    Science.gov (United States)

    Brewster, David

    2011-09-01

    The human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) epidemic in Africa has raised important ethical issues for both researchers and clinicians. The most notorious controversy has been related to the zidovudine (AZT) trials in Africa in the late 1990s, in which the control groups were given a placebo rather than an effective drug to prevent vertical transmission. This raised concerns in the sponsoring country about exploitation of subjects, injustice and an ethical double standard between donor countries and resource-poor settings. However, the real double standard is between clinical practice standards in Western versus African countries, which must be addressed as part of the increasing global inequity of wealth both between countries and also within countries. There are important limitations to ethical declarations, principles and guidelines on their own without contextual ethical reasoning. The focus on research ethics with the HIV epidemic has led to a relative neglect of ethical issues in clinical practice. Although the scientific advances in HIV/AIDS have changed the ethical issues since the 1990s, there has also been progress in the bioethics of HIV/AIDS in terms of ethical review capability by local committees as well as in exposure to ethical issues by clinicians and researchers in Africa. However, serious concerns remain about the overregulation of research by bureaucratic agencies which could discourage African research on specifically African health issues. There is also a need for African academic institutions and researchers to progressively improve their research capacity with the assistance of research funders and donor agencies. © 2011 The Author. Journal of Paediatrics and Child Health © 2011 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  9. Opportunistic Neurologic Infections in Patients with Acquired Immunodeficiency Syndrome (AIDS).

    Science.gov (United States)

    Albarillo, Fritzie; O'Keefe, Paul

    2016-01-01

    Infections of the central nervous system (CNS) in individuals with human immunodeficiency virus (HIV) remain a substantial cause of morbidity and mortality despite the introduction of highly active antiretroviral therapy (HAART) especially in the resource-limited regions of the world. Diagnosis of these infections may be challenging because findings on cerebrospinal fluid (CSF) analysis and brain imaging are nonspecific. While brain biopsy provides a definitive diagnosis, it is an invasive procedure associated with a relatively low mortality rate, thus less invasive modalities have been studied in recent years. Diagnosis, therefore, can be established based on a combination of a compatible clinical syndrome, radiologic and CSF findings, and understanding of the role of HIV in these infections. The most common CNS opportunistic infections are AIDS-defining conditions; thus, treatment of these infections in combination with HAART has greatly improved survival.

  10. Cerebral toxoplasmosis and lymphoma in patients with Acquired Immunodeficiency Syndrome

    International Nuclear Information System (INIS)

    Laviopierre, A.M.; Lawler, G.A.

    1989-01-01

    Toxoplasmosis now constitutes a relatively frequent central nervous system (CNS) complication of AIDS, primary CNS lymphoma being far less common. CT scanning using the double-dose delayed (D-D-D) scan technique has proved an effective way of helping in the diagnosis of these complications. 16 patients with CNS complications of the acquired immunodeficiency syndrome (AIDS) are described. All patients were male homosexuals. The most common demonstrable lesion in the parenchyma was toxoplasmosis, which produced an area of focal oedema, usually containing a central zone of nodular or ring-shaped enhancement. Cerebral atrophy was also a common finding. One patient had diffuse peri-ventricular lymphomatous infiltration, and a further two patients had both cerebral toxoplasmosis and lymphoma. A delayed double dose contrast examination appears to be the most accurate method of outlining the total extent of CNS disease in these patients. 11 refs., 7 figs., 2 tabs

  11. [Primary Intracranial Malignant Lymphoma Associated with Acquired Immunodeficiency Syndrome(AIDS):A Case Report].

    Science.gov (United States)

    Inaka, Yasufumi; Otani, Naoki; Nishida, Sho; Fujii, Kazuya; Ueno, Hideaki; Tomura, Satoshi; Tomiyama, Arata; Osada, Hideo; Wada, Kojiro; Maeda, Takuya; Mori, Kentaro

    2017-11-01

    The spread of human immunodeficiency virus(HIV)infection may result in an increased likelihood of surgery in patients with HIV infection. We treated a patient with intracranial malignant lymphoma associated with acquired immunodeficiency syndrome(AIDS)caused by HIV infection. The recommendations of the countermeasure manual for AIDS were followed. Only surgical staff without finger injury or inflammation were permitted to be involved in the operation. All staff were dressed in a waterproof, full-body surgical gown, and wore double gloves, double foot covers, and an N95 mask. The surgery could be performed safely with such infection control measures. Histological examination revealed a diffuse large B-cell lymphoma. The patient was referred to the Division of Infectious Diseases and Respiratory Medicine for chemotherapy.

  12. Clinical spectrum of immunodeficiency, centromeric instability and facial dysmorphism (ICF syndrome).

    NARCIS (Netherlands)

    Hagleitner, M.M.; Lankester, A.; Maraschio, P.; Hulten, M.; Fryns, J.P.; Schuetz, C.; Gimelli, G.; Davies, E.G.; Gennery, A.R.; Belohradsky, B.H.; Groot, R. de; Gerritsen, E.J.; Mattina, T.; Howard, P.J.; Fasth, A.; Reisli, I.; Furthner, D.; Slatter, M.A.; Cant, A.J.; Cazzola, G.; Dijken, P.J. van; Deuren, M. van; Greef, J.C. de; Maarel, S.M. van der; Weemaes, C.M.R.

    2008-01-01

    BACKGROUND: Immunodeficiency, centromeric instability and facial dysmorphism (ICF syndrome) is a rare autosomal recessive disease characterised by facial dysmorphism, immunoglobulin deficiency and branching of chromosomes 1, 9 and 16 after PHA stimulation of lymphocytes. Hypomethylation of DNA of a

  13. Implantation of cultured thymic fragments in patients with acquired immunodeficiency syndrome

    NARCIS (Netherlands)

    Danner, S. A.; Schuurman, H. J.; Lange, J. M.; Gmelig Meyling, F. H.; Schellekens, P. T.; Huber, J.; Kater, L.

    1986-01-01

    Cultured thymic fragments were implanted in one patient with acquired immunodeficiency syndrome (AIDS)-related complex (ARC) and in eight AIDS patients with opportunistic infections (OIs, four patients), Kaposi's sarcoma (KS, two patients), or both (two patients). Thereafter, objective clinical

  14. Prevalence of atopic disorders and immunodeficiency in patients with ectodermal dysplasia syndromes

    Science.gov (United States)

    Mark, Barry J.; Becker, Bradley A.; Halloran, Donna R.; Bree, Alanna F.; Sindwani, Raj; Fete, Mary D.; Motil, Kathleen J.; Srun, Sopheak W.; Fete, Timothy J.

    2013-01-01

    Background Ectodermal dysplasia (ED) syndromes are a diverse group of disorders that affect multiple ectodermally derived tissues. Small studies and case reports suggest an increase in atopy and primary immunodeficiencies (PIDs) among patients with ED syndromes. Objective To determine the prevalence of clinical symptoms suggestive of atopy or immunodeficiency among a large cohort of children with ED syndromes. Methods A 9-page questionnaire was mailed to families who were members of the National Foundation for Ectodermal Dysplasias. The surveys were completed by parents of children younger than 18 years with a diagnosis of an ED syndrome or carrier state. Portions of the questionnaire were adapted from previously validated questionnaires developed by the International Study of Asthma and Allergies in Childhood (ISAAC). Results We received 347 completed questionnaires (41%). When compared with the 13- to 14-year-old children surveyed by ISAAC, we found both all-aged and age-matched children with ED syndromes, respectively, had significantly higher rates of asthma (32.2% and 37.2% vs 16.4%), rhinitis symptoms (76.1% and 78.3% vs 38.9%), and eczema (58.9% and 48.9% vs 8.2%). The prevalence of physician-diagnosed food allergies (20.7%) and PIDs (6.1%) in these ED patients also exceeded known rates in the general pediatric population. Conclusion This large-scale, retrospective study demonstrates a greater reported prevalence of symptoms suggestive of atopic disorders and PIDs among children with ED syndromes than the general pediatric population. A combination of genetic and environmental factors in ED syndromes may contribute to breaches of skin and mucosal barriers, permitting enhanced transmission and sensitization to irritants, allergens, and pathogens. PMID:22626597

  15. Kaposi sarcoma related to acquired immunodeficiency syndrome: hepatic findings on computed tomography and magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Costa, Daniel Nobrega da; Viana, Publio Cesar Cavalcante; Maciel, Rosangela Pereira; Rocha, Manoel de Souza; Gebrim, Eloisa Maria Mello Santiago [Universidade de Sao Paulo (USP), SP (Brazil). Hospital das Clinicas. Inst. de Radiologia]. E-mail: dnobrega@gmail.com

    2008-03-15

    Kaposi sarcoma is a neoplasm associated with immunosuppressive conditions, and involving blood and lymphatic vessels. It is the most frequent intrahepatic neoplasm in patients with acquired immunodeficiency syndrome. Computed tomography and magnetic resonance imaging demonstrate multiple small nodules, prominence and contrast-enhancement of periportal branches due to the presence of the neoplastic tissue. The authors report a case of a 47-year-old male patient with acquired immunodeficiency syndrome presenting disseminated Kaposi sarcoma. (author)

  16. Cystic fibrosis, common variable immunodeficiency and Aspergers syndrome: an immunological and behavioural challenge.

    LENUS (Irish Health Repository)

    Chotirmall, S H

    2012-02-01

    INTRODUCTION: Cystic fibrosis (CF) is of particular importance in Ireland as the Irish population has both the highest incidence (2.98\\/10,000) and the highest carrier rate (1 in 19) in the world. Primary immunodeficiency has not been previously reported as co-existing with CF. CASE REPORT: We report a unique case of CF associated with a primary immunodeficiency syndrome--common variable immunodeficiency (CVID). DISCUSSION: Our patient has CF, CVID and the additional comorbidity of Aspergers syndrome. The challenges inherent in diagnosing and treating such a case are outlined herein and the successful management of this case is evidenced by the well-preserved lung function of our patient.

  17. Cystic fibrosis, common variable immunodeficiency and Aspergers syndrome: an immunological and behavioural challenge.

    LENUS (Irish Health Repository)

    Chotirmall, S H

    2009-08-07

    INTRODUCTION: Cystic fibrosis (CF) is of particular importance in Ireland as the Irish population has both the highest incidence (2.98\\/10,000) and the highest carrier rate (1 in 19) in the world. Primary immunodeficiency has not been previously reported as co-existing with CF. CASE REPORT: We report a unique case of CF associated with a primary immunodeficiency syndrome-common variable immunodeficiency (CVID). DISCUSSION: Our patient has CF, CVID and the additional comorbidity of Aspergers syndrome. The challenges inherent in diagnosing and treating such a case are outlined herein and the successful management of this case is evidenced by the well-preserved lung function of our patient.

  18. Radiological findings in three acquired immunodeficiency syndrome patients with Rhodococcus equi pneumonia

    International Nuclear Information System (INIS)

    Liu Jinxin; Tang Xiaoping; Zhang Lieguang

    2011-01-01

    Objective: To study the imaging appearances of Rhodococcus equi pneumonia in three patients with acquired immunodeficiency syndrome ( AIDS). Methods: Thoracic imaging appearances of' Rhodococcus equi pneumonia in three patients with AIDS were retrospectively analyzed. Results: The chest radiograph showed patchy consolidations and small nodules (n=3), large consolidations with multiple cavitations (n=2). CT showed large lobar or segmental consolidations with multiple cavitations (n=2), patchy consolidations (n=2), bronchiectasis (n=1), multiple small centrilobular nodules (n=2) and tree-in-bud patterns (n=2). Conclusion: The most common radiological findings in AIDS patients with Rhodococcus equi pulmonary infection are large consolidations with multiple cavitations and multiple centrilobular nodules. (authors)

  19. Computed tomography of the lungs in acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Hartelius, H.; Gaub, J.; Jensen, L.I.; Jensen, J.; Faber, V.; Rigshospitalet, Copenhagen

    1988-01-01

    Computed tomography of the chest was performed on 42 occasions as part of the diagnostic work-up in 26 homosexual men with, or suspected of the acquired immunodeficiency syndrome (AIDS). In 17 cases both the chest radiographs and the lung scans were abnormal, and bronchoscopy and/or lung biopsy established an etiologic diagnosis in the majority of these cases. In 9 cases CT of the lungs revealed unequivocal interstitial infiltration in the presence of a normal chest radiography, and subsequently and etiologic agent was demonstrated in all these cases. In 9 cases, patients with symptoms indicative of pulmonary infection had both a normal chest radiograph and a normal lung scan, and in none of these cases did the clinical course or additional diagnostic procedures indicate the presence of current opportunistic lung infection. CT of the lungs seems to identify accurately those patients with severe HIV-related diseases in whom invasive diagnostic procedures such as bronchoalveolar lavage and/or lung biopsy should be done. (orig.)

  20. Avian Influenza: Potential Impact on Sub-Saharan Military Populations with High Rates of Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome

    National Research Council Canada - National Science Library

    Feldman, Robert L; Nickell, Kent

    2007-01-01

    ...)/acquired immunodeficiency syndrome. With the arrival of avian influenza in Africa, the potential exists that some of those soldiers might also become infected with H5N1, the virus responsible for the disease...

  1. Orbital manifestations in patients with acquired immunodeficiency syndrome.

    Science.gov (United States)

    Sodhi, Punita Kumari

    2014-01-01

    The orbital manifestations of acquired immunodeficiency syndrome(AIDS) are uncommon. To provide a review of orbital manifestations of AIDS, the predisposing factors, investigations, treatment and outcome. Meticulous and systematic literature search of Pubmed to identify manuscripts describing orbital manifestations of AIDS was done and the articles were reviewed.The keywords used in the search were “orbit and AIDS”, “HIV positive and orbit”,“orbit manifestations in AIDS”, “orbital disease and AIDS” and “orbital infections and AIDS”. The orbital involvement in AIDS may present with opportunistic infections from organisms like fungi, viruses, bacteria and protozoa or with malignancies like Kaposi’s sarcoma, squamous cell carcinoma, smooth muscle cell tumors and lymphoma.The predisposing factors for orbital involvement in AIDS are low CD4+ cell count and the immunosuppressive states like diabetes, diabetic ketoacidosis, intravenous drug abuse and neutropenia. A patient may present with fever, headache, nausea, vomiting,decreased vision, ocular pain, and, in cases of mass formation, there is periorbital swelling, axial proptosis, globe displacement and swollen optic disc. Radiologically,mass formation, orbital bony destruction, and spread of disease to contiguous structures including the central nervous system may be seen. The medical management includes therapy for infection and HIV-1 protease inhibitors (highly active antiretroviral therapy)to suppress HIV-1 replication. For tumors, radical surgery including debulking followed by postoperative radiotherapy is generally needed. Orbital involvements with AIDS in any form, infective or malignancy, causes significant morbidity and mortality and should be diagnosed and managed as early as possible.

  2. Progressive multifocal leukoence-phalopathy presenting as homonymous hemianopia in a patient with acquired immunodeficiency syndrome

    Directory of Open Access Journals (Sweden)

    Amit Pandey

    2012-01-01

    Full Text Available We present a case of a Human Immunodeficiency Virus (HIV positive patient who was referred for retinal evaluation to rule out ophthalmic manifestations of Acquired Immunodeficiency Syndrome (AIDS. She complained of some disturbance in vision in both eyes. Fundus examination showed no abnormality. Perimetry, done to rule out optic nerve pathology, showed a left homonymous hemianopia. Magnetic Resonance Imaging (MRI scan showed features of Progressive Multifocal Leukoencephalopathy (PML. She had no other neurological symptoms or signs.

  3. Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome

    DEFF Research Database (Denmark)

    Krejsgaard, Thorbjørn Frej; Ødum, Niels; Geisler, Carsten

    2012-01-01

    Cutaneous T-cell lymphoma (CTCL) is the term for diseases characterized by primary accumulation of malignant T cells in the skin. Patients with the two predominant clinical forms of CTCL called mycosis fungoides (MF) and Sézary syndrome (SS) characteristically develop severe immunodeficiency duri...... in MF and SS and discuss the potential mechanisms driving the immunodeficiency.Leukemia advance online publication, 9 September 2011; doi:10.1038/leu.2011.237....

  4. Progressive multifocal leukoence--phalopathy presenting as homonymous hemianopia in a patient with acquired immunodeficiency syndrome.

    Science.gov (United States)

    Pandey, Amit; Bandivdekar, Karishma; Ramchandani, Suresh; Ramchandani, Sushama

    2012-01-01

    We present a case of a Human Immunodeficiency Virus (HIV) positive patient who was referred for retinal evaluation to rule out ophthalmic manifestations of Acquired Immunodeficiency Syndrome (AIDS). She complained of some disturbance in vision in both eyes. Fundus examination showed no abnormality. Perimetry, done to rule out optic nerve pathology, showed a left homonymous hemianopia. Magnetic Resonance Imaging (MRI) scan showed features of Progressive Multifocal Leukoencephalopathy (PML). She had no other neurological symptoms or signs.

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

    International Nuclear Information System (INIS)

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

    1984-01-01

    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

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

    OpenAIRE

    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 an...

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

    OpenAIRE

    Wang, Ying; Cochran, Christopher; Xu, Peng; Shen, Jay J; Zeng, Gang; Xu, Yanjun; Sun, Mei; Li, Chengyue; Li, Xiaohong; Chang, Fengshui; Lu, Jun; Hao, Mo; Lu, Fan

    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...

  8. Epithelioid angiomatosis in the acquired immunodeficiency syndrome: morphology and differential diagnosis

    NARCIS (Netherlands)

    Walford, N.; van der Wouw, P. A.; Das, P. K.; ten Velden, J. J.; Hulsebosch, H. J.

    1990-01-01

    A rare vascular proliferation found as a skin lesion in patients suffering from the acquired immunodeficiency syndrome and sometimes referred to as epithelioid angiomatosis is believed to be a manifestation of infection by the cat scratch bacillus or a related organism. We describe the histological

  9. Low-dose growth hormone and human immunodeficiency virus-associated lipodystrophy syndrome: a pilot study

    DEFF Research Database (Denmark)

    Andersen, Ove; Haugaard, Steen B; Flyvbjerg, A

    2004-01-01

    BACKGROUND: Treatment with high doses (2-6 mg day(-1)) of human growth hormone (hGH) in patients with human immunodeficiency virus (HIV)-associated lipodystrophy syndrome (HALS) has been shown to increase concentrations of total insulin-like growth-factor-I (IGF-I) more than twofold greater than......-I and fat distribution. Glucose metabolism was examined by oral glucose tolerance tests and hyperinsulinaemic euglycaemic clamps. RESULTS: Total IGF-I increased twofold (P ....01). Patients reported improvements of lipodystrophy, which was supported by a decreased waist-to-thigh ratio (P = 0.01), and waist-to-hip ratio (P = 0.06). Ratio of peripheral to trunk soft tissue mass increased (P = 0.01, measured by dual-energy X-ray absorptiometry scans) and a trend towards reduction...

  10. Toxoplasmosis of spinal cord in acquired immunodeficiency syndrome patient presenting as paraparesis: A rare entity

    Directory of Open Access Journals (Sweden)

    Sachin R Agrawal

    2014-01-01

    Full Text Available Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm 3 based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.

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

    Directory of Open Access Journals (Sweden)

    Elizabeth Fischer

    2005-02-01

    Full Text Available 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.

  12. New clinical and histological patterns of acute disseminated histoplasmosis in human immunodeficiency virus-positive patients with acquired immunodeficiency syndrome.

    Science.gov (United States)

    Ollague Sierra, Jose E; Ollague Torres, Jose M

    2013-04-01

    Histoplasmosis has attained increasing relevance in the past 3 decades because of the appearance of the human immunodeficiency virus (HIV). In most immunocompetent persons, the infection is asymptomatic or can produce a respiratory condition with symptoms and radiological images similar to those observed in pulmonary tuberculosis; in non-HIV+ immunocompromised patients, it can cause respiratory symptoms or evolve into a disseminated infection. The same can occur in acquired immunodeficiency syndrome (AIDS) patients. We have observed a series of HIV+ patients with AIDS who presented with cutaneous histoplasmosis and in whom the clinical and histopathological features were highly unusual, including variable mucocutaneous lesions that were difficult to diagnose clinically. These patients displayed unusual, previously undescribed, histological patterns, including lichenoid pattern, nodular pseudomyxoid pattern, pyogenic granuloma-like pattern, perifollicular pattern, and superficial (S), mid (M), and deep perivascular dermatitis; and more commonly encountered patterns, such as histiocytic lobular panniculitis and focal nodular dermatitis. The novel histopathological patterns of cutaneous involvement by histoplasmosis seen in these patients resembled other common inflammatory and infectious conditions and required a high level of suspicion and the application of special stains for organisms for confirmation. These new, clinical, and histological findings do not seem to be commonly encountered in HIV- patients infected with the fungus but seem to be displayed most prominently in HIV+ patients with AIDS.

  13. Depression among people living with human immunodeficiency virus infection/acquired immunodeficiency syndrome in Benin City, Nigeria: a comparative study.

    Science.gov (United States)

    Chikezie, U E; Otakpor, A N; Kuteyi, O B; James, B O

    2013-01-01

    Depression is a common co-morbidity among persons living with human immunodeficiency virus infection/acquired immunodeficiency syndrome (HIV/AIDS) (PLWHAs). It is associated with poor treatment adherence and higher mortality rates. Few reports have, however, emanated from developing countries where socioeconomic factors may confound this association. We conducted a cross-sectional comparative study of PLWHAs and apparently healthy staff of three LGA's. The depression module of the Schedule for the Clinical Assessment in Neuropsychiatry (SCAN) and the Beck Depression Inventory (BDI) was used to diagnose depression and depression symptom severity, respectively. Depression was commonly co-morbid among individuals with HIV/AIDS. It was five times more common in PLWHAs than in apparently healthy populations (29.3% vs. 7.3%, OR: 5.25, 95% CI: 2.50-11.76). A similar trend was observed for depression symptom severity. Among PLWHAs, depression was significantly more likely among females (OR: 7.91, 95% CI: 1.83-71.00, P 3 years (OR: 7.90, P risk. Depression was commonly co-morbid among PLWHAs studied. Clinicians should be aware of risk factors for depression among PLWHAs in order to improve treatment outcomes.

  14. Human immunodeficiency virus (HIV) is highly associated with giant idiopathic esophageal ulcers in acquired immunodeficiency syndrome (AIDS) patients.

    Science.gov (United States)

    Lv, Bei; Cheng, Xin; Gao, Jackson; Zhao, Hong; Chen, Liping; Wang, Liwei; Huang, Shaoping; Fan, Zhenyu; Zhang, Renfang; Shen, Yinzhong; Li, Lei; Liu, Baochi; Qi, Tangkai; Wang, Jing; Cheng, Jilin

    2016-01-01

    This study aimed to determine whether the human immunodeficiency virus (HIV) exists in giant idiopathic esophageal ulcers in the patients with acquired immune deficiency syndrome (AIDS). 16 AIDS patients with a primary complaint of epigastric discomfort were examined by gastroscopy. Multiple and giant esophageal ulcers were biopsied and analyzed with pathology staining and reverse transcription-polymerase chain reaction (RT-PCR) to determine the potential pathogenic microorganisms, including HIV, cytomegalovirus (CMV) and herpes simplex viruses (HSV). HIV was detected in ulcer samples from 12 out of these 16 patients. Ulcers in 2 patients were infected with CMV and ulcers in another 2 patients were found HSV positive. No obvious cancerous pathological changes were found in these multiple giant esophageal ulcer specimens. HIV may be one of the major causative agents of multiple benign giant esophageal ulcers in AIDS patients.

  15. Reiter's Syndrome associated with the Acquired Immunodeficiency Syndrome: a case report

    Directory of Open Access Journals (Sweden)

    J.B.N.S. Malta

    Full Text Available The association of Reiter's Syndrome (RS with the Acquired Immunodeficiency Syndrome (AIDS is seldom mentioned in the medical literature. This report illustrates this relationship in a 46 years old male patient suffering from AIDS (CD4+ = 240 cells/mm³, CD8+ = 1,301 cells/mm³ and viral load = 330,000 copies/ml, pulmonary tuberculosis (positive catarrhal bacilluscopy, and RS. The diagnosis of RS was based on the combination of dermatological and articular alterations. The patient's cutaneous lesions were characterized by exfoliation and the formation of crusts located on the face, scalp, genitals, hands, and feet; onychodystrophy with opacity; yellowish coloring; and hyperkeratosis of the nails. Articular lesions led to progressive deformity of phalangeal joints of the hands, and intensive arthralgia, mainly of the larger joints (shoulders, elbows, hips and knees. AIDS treatment was administered with anti-retroviral drugs (zidovudine and didanosine; for tuberculosis (isoniazid, rifampicine, and pyrazinamide; and (prednisone and inometacine for the RS. The patient recovered with the improvement of articular symptoms; however, on the eighth day of treatment, the patient showed significant hemoptysis and hypovolemic shock, and died. The association of RS and HIV infection is reviewed.

  16. Otologic manifestations in child with acquired immunodeficiency syndrome

    OpenAIRE

    Rezende, Carlos E. B.; Rodrigues, Rubens E. C.; Haddad, Leonardo; Yoshimura, Ricardo; Rapoport, Priscila B.

    2004-01-01

    As manifestações otológicas são particularmente comuns em crianças portadoras do Vírus da Imunodeficiência Humana (HIV). A disacusia nestes pacientes pode ser condutiva, principalmente decorrente de otite média ou sensorioneural, pela ação direta do vírus ou pela ação de drogas anti-retrovirais. Neste trabalho, relatamos o caso de uma criança de 10 anos de idade, portadora de HIV, com disacusia e revisamos a literatura.Patients infected with Human immunodeficiency virus (HIV) often present wi...

  17. PARTIAL OCULOCUTANEOUS ALBINISM AND IMMUNODEFICIENCY SYNDROMES: TEN YEARS EXPERIENCE FROM A SINGLE CENTER IN TURKEY.

    Science.gov (United States)

    Patiroglu, T; Akar, H H; Unal, E; Chiang, S C; Schlums, H; Tesi, B; Ozkars, M Y; Karakukcu, M

    2016-01-01

    Partial oculocutaneous albinism and immunodeficiency (OCA-ID) diseases are autosomal recessive syndromes characterized by partial hypopigmentation and recurrent infections. Moreover, some OCA-ID syndromes confer susceptibility to develop a life-threatening hyperinflammatory condition called hemophagocytic lymphohistiocytosis (HLH). We investigated the genetic, clinical and immunological characteristics of 20 OCA patients. Herein, we present the clinical and immunological characteristics of 20 OCA patients who referred to the Department of Pediatric Immunology, Erciyes University Medical Faculty in Kayseri, Turkey between 2004 and 2014. Of the 20 OCA patients, 7 fulfilled diagnostic criteria for HLH, 9 showed defective functions of CD8 T cells and natural killer cells, and 8 received a definitive molecular diagnosis. Among the patients, we also report a patient diagnosed with two different genetic defects, in TYR and JAK3 genes, causing, respectively, OCA and ID. Our results illustrate the variability of clinical presentations and disease severity in OCA-ID patients, with consequent challenges in diagnosing and treating these patients.

  18. 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

    Directory of Open Access Journals (Sweden)

    Seyed Ramin Radfar

    2014-01-01

    Full Text Available 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.

  19. Hepatitis C, human immunodeficiency virus and metabolic syndrome: interactions.

    Science.gov (United States)

    Kotler, Donald P

    2009-03-01

    Significant concerns have been raised about the metabolic effects of antiretroviral medication, including the classic triad of dyslipidaemia, insulin resistance (IR) and characteristic alterations in fat distribution (lipoatrophy and lipohypertrophy). Co-infection with hepatitis C appears to exacerbate IR, reduce serum lipids and induce prothrombotic changes in the treated human immunodeficiency virus patient. The effects of co-infection are complex. While combination antiretroviral therapy has been shown to be associated with an increased risk of cardiovascular events through promotion of dyslipidaemia, IR and fat redistribution, co-infection exacerbates IR while reducing serum lipids. Co-infection also promotes a prothrombotic state characterized by endothelial dysfunction and platelet activation, which may enhance risk for cardiovascular disease. Consideration must be given to selection of appropriate treatment regimens and timing of therapy in co-infected patients to minimize metabolic derangements and, ultimately, reduce cardiovascular risk.

  20. Hemophagocytic lymphohistiocytosis (HLH) secondary to disseminated histoplasmosis in the setting of Acquired Immunodeficiency Syndrome (AIDS).

    Science.gov (United States)

    Asanad, Samuel; Cerk, Brendan; Ramirez, Veronica

    2018-06-01

    Hemophagocytic lymphohistiocytosis (HLH) is a rare and aggressive disease involving immune system over-activation leading to hemophagocytosis. HLH requires early diagnosis and prompt treatment initiation, especially in patients with Acquired Immunodeficiency Syndrome (AIDS). We present a case of a middle-aged male with AIDS and renal failure, who developed HLH secondary to disseminated histoplasmosis. Etoposide chemotherapy as recommended by the HLH 2004 Guidelines was deferred and treatment focused instead on anti-fungal therapy. Anti-retroviral therapy followed thereafter.

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

    Directory of Open Access Journals (Sweden)

    Sneha Nandy

    2015-01-01

    Full Text Available 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.

  2. Acquired immunodeficiency syndrome in a patient with no known risk factors: a pathological study.

    OpenAIRE

    Burt, A D; Scott, G; Shiach, C R; Isles, C G

    1984-01-01

    We present the pathological findings in a case of acquired immunodeficiency syndrome (AIDS) in a patient with no known risk factor. Postmortem examination showed klebsiella lung abscess, generalised cytomegalovirus infection, cerebral toxoplasmosis, and a primary cerebral lymphoma. An additional feature was the presence of dilatation of the intrahepatic large bile ducts in association with an atypical distribution of cytomegalovirus. The relation between this case and previously reported case...

  3. Acquired immunodeficiency syndrome in a patient with no known risk factors: a pathological study.

    Science.gov (United States)

    Burt, A D; Scott, G; Shiach, C R; Isles, C G

    1984-04-01

    We present the pathological findings in a case of acquired immunodeficiency syndrome (AIDS) in a patient with no known risk factor. Postmortem examination showed klebsiella lung abscess, generalised cytomegalovirus infection, cerebral toxoplasmosis, and a primary cerebral lymphoma. An additional feature was the presence of dilatation of the intrahepatic large bile ducts in association with an atypical distribution of cytomegalovirus. The relation between this case and previously reported cases of AIDS is discussed.

  4. Radiological pulmonary manifestations of acquired immunodeficiency syndrome; Manifestacoes radiologicas pulmonares da sindrome da imunodeficiencia adquirida

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    Marchiori, Edson; Melo, Alessandro Severo Alves de; Ossa, Alfonso Jaramillo [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia

    1999-06-01

    In this article are reviewed the principal radiologic manifestations of inflammatory and tumoral diseases the compromise the lungs of patients with acquired immunodeficiency syndrome. In the group of inflammatory diseases the radiologic aspects of pneumocystosis, cytomegalovirus disease, cryptococcosis, tuberculosis and bacterial pneumonias are emphasized. In the neoplasic diseases' group the aspects of lymphoma and Kaposi's sarcoma are specially presented. (author)

  5. Thyroid hormone levels in the acquired immunodeficiency syndrome (AIDS) or AIDS-related complex.

    OpenAIRE

    Tang, W W; Kaptein, E M

    1989-01-01

    Hypothalamic-pituitary dysfunction and thyroid gland cytomegalovirus inclusions have been described in patients with the acquired immunodeficiency syndrome (AIDS) and AIDS-related complex (ARC). We evaluated 80 patients with AIDS or ARC for the frequency of hypothalamic-pituitary or thyroid gland failure and altered serum thyroid hormone levels due to nonthyroidal disorders. One patient had subclinical hypothyroidism. Of these patients, 60% had low free triiodothyronine (T3) index values and ...

  6. Fatal respiratory distress syndrome due to coronavirus infection in a child with severe combined immunodeficiency

    OpenAIRE

    Szczawinska‐Poplonyk, Aleksandra; Jonczyk‐Potoczna, Katarzyna; Breborowicz, Anna; Bartkowska‐Sniatkowska, Alicja; Figlerowicz, Magdalena

    2012-01-01

    Please cite this paper as: Szczawinska‐Poplonyk et al. (2012) Fatal respiratory distress syndrome due to coronavirus infection in a child with severe combined immunodeficiency. Influenza and Other Respiratory Viruses DOI: 10.1111/irv.12059. Coronaviruses have been demonstrated to contribute substantially to respiratory tract infections among the child population. Though infected children commonly present mild upper airway symptoms, in high‐risk patients with underlying conditions, particularl...

  7. Defect in IgV gene somatic hypermutation in common variable immuno-deficiency syndrome.

    Science.gov (United States)

    Levy, Y; Gupta, N; Le Deist, F; Garcia, C; Fischer, A; Weill, J C; Reynaud, C A

    1998-10-27

    Common Variable Immuno-Deficiency (CVID) is the most common symptomatic primary antibody-deficiency syndrome, but the basic immunologic defects underlying this syndrome are not well defined. We report here that among eight patients studied (six CVID and two hypogammaglobulinemic patients with recurrent infections), there is in two CVID patients a dramatic reduction in Ig V gene somatic hypermutation with 40-75% of IgG transcripts totally devoid of mutations in the circulating memory B cell compartment. Functional assays of the T cell compartment point to an intrinsic B cell defect in the process of antibody affinity maturation in these two cases.

  8. Role of flexible bronchoscopy and bronchoalveolar lavage in the diagnosis of pediatric acquired immunodeficiency syndrome-related pulmonary disease.

    Science.gov (United States)

    Birriel, J A; Adams, J A; Saldana, M A; Mavunda, K; Goldfinger, S; Vernon, D; Holzman, B; McKey, R M

    1991-06-01

    Flexible fiberoptic bronchoscopy with bronchoalveolar lavage was performed in 16 pediatric patients with the acquired immunodeficiency syndrome (AIDS) and deterioration in pulmonary function suggestive of opportunistic infection. In 62% of the patients Pneumocystis carinii was identified. Culture results showed a pure growth of Pseudomonas aeruginosa for one patient in addition to the Pneumocystis carinii. Bronchoscopy with lavage was well tolerated, with few complications even among patients with significant tachypnea and hypoxia. Because of its relative safety and effectiveness, this procedure should be considered the first invasive measurement used for evaluation of parenchymal lung disease in this population of patients.

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

    Science.gov (United States)

    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 Nocardia.

  10. CT features of pulmonary mycobacterial disease in patients with acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Zhu Ying; Zhang Zhiyong; Shi Yuxin; Feng Feng

    2013-01-01

    Objective: To study the CT features of pulmonary non-tuberculous mycobacteria (NTM) disease in patients with acquired immunodeficiency syndrome (AIDS) and explore the different CT appearances between AIDS-NTM and AIDS-TB. Methods: CT findings of pulmonary NTM disease in 27 AIDS patients (NTM group) were retrospectively analyzed and compared with that of tuberculosis in 30 AIDS patients (TB group). The results were statistically analyzed using Fisher's exact test. Results: CT findings of NTM appeared significantly more than that of TB as follows: high-density nodules (n = 18 vs 1, P < 0.01), ground-glass opacities (n = 10 vs 0, P < 0.01), fibrotic band (n = 17 vs 3, P < 0.01), bronchiectasis (9 vs 2, P = 0.012). CT findings of NTM appeared significantly less than that of TB as follows: miliary nodules (0 vs 6, P = 0.016), air space consolidations (n = 2 vs 11, P < 0.01), pleural effusion (n = 1 vs 9, P < 0.01). Conclusion: Nodule and fibrotic band accompanied with bronchiectasis were the main CT manifestations of pulmonary NTM disease in AIDS patients, while air space consolidation accompanied with pleural effusion and miliary nodules were the predominate CT findings of pulmonary tuberculosis in AIDS patients. (authors)

  11. Erythrovirus B19 infection in acquired immunodeficiency syndrome: screening by histopathology, immunohistochemistry, and in situ hybridization

    Directory of Open Access Journals (Sweden)

    Sérgio Setúbal

    2006-06-01

    Full Text Available Erythrovirus B19 infects erythrocytic progenitors, transiently interrupting erythropoiesis. In AIDS patients it causes chronic anemia amenable to treatment. We looked for evidences of B19 infection in stored bone marrow material from patients with acquired immunodeficiency syndrome. Histological sections were made from stored paraffin blocks from 33 autopsies (39 blocks and 35 biopsies (45 blocks, 30 patients performed from 1988 to 2002. They were examined after hematoxylin-eosin (HE staining, immunohistochemical (IHC, and in situ hybridization. HE revealed intra-nuclear inclusion bodies ("lantern cells" suggesting B19 infection in 19 sections corresponding to 19 of 63 patients examined with this test. Seven of 78 sections subjected to immunohistochemistry were positive, corresponding to 7 of 58 patients examined with this test. Fourteen sections corresponding to 13 of the 20 HE and/or IHC positive patients were subjected to in situ hybridization, with six positives results. Among the 13 patients subjected to the three techniques, only one gave unequivocal positive results in all and was considered a true positive. The frequency of B19 infection (1/63 patients in the material examined can be deemed low.

  12. [The incidence of oral candidiasis in patients with human immunodeficiency virus infection/acquired immunodeficiency syndrome from Yunnan, China].

    Science.gov (United States)

    Wen, Yan; Li, Chengwen; Pei, Junhaoxiang; Bai, Jinsong; Yang, Xianghong; Duan, Kaiwen

    2014-08-01

    To assess the incidence of oral candidiasis and its influencing factors in patients with human immunodeficiency virus infection/acquired immunodeficiency syndrome (HIV/AIDS). An oral examination was conducted in the 1 566 HIV/AIDS patients in the Third Hospital of Kunming from March 2008 to September 2012 (M/F: 1 062/504, age range: 0.2 to 84.0 years old). The HIV viral load (HIV- RNA) and peripheral blood CD4 count were respectively analyzed by Bayer Q340 fluorescence signal surveying instrument (bDNA method) and flow cytometry analysis. The information on usage of highly active anti-retroviral (HAART) drugs and transmission of HIV were obtained through questionnaires. The incidence of oral candidiasis in patients with different HIV-RNA levels and CD4 count and the use of HAART was analyzed and compared. The total incidence of oral candidosis was 31.0% (486/1 566) and there was no difference in sex. The oral lesions were presented by three types, psudomembranous candidosis (PC), erythematous candidosis (EC) and angular cheilitis (AC), and the morbidity was 13.9% (217/1 566), 17.0% (267/1 566) and 4.9% (77/1 566), respectively. The average level of CD4 count in psudomembranous candidosis, erythematous candidosis and angular cheilitis [81.0 (146.0), 74.0 (152.0) and 69.0 (121.5) cell/µl] showed no significant difference (P > 0.05). The incidence of oral candidiasis in non-HAART and HAART subjects were 36.3% (402/1 107) and 18.3% (84/459), respectively (P = 0.000). The CD4 count and absolute counts of HIV viral load in oral candidiasis patients and non-oral candidiasis patients had significant difference (Z = -10.261, P = 0.000 and Z = -4.762, P = 0.000). The morbidity of oral candidiasis in HIV/AIDS patients in Yunnan Province was high, including PC, EC and AC and hyperplastic candidosis was not detected. The incidence was related to the degree of immune suppression and HIV viral load.

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

    Science.gov (United States)

    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

  14. Imaging findings of disseminated pulmonary tuberculosis in patients with acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Song Wenyan; Zhao Zuqi; Zhao Dawei; Jia Cuiyu; Zhang Ruichi; Liu JinXin; Guan Wanhua; Liang Yi

    2013-01-01

    Objective: To study the imaging findings of disseminated pulmonary tuberculosis in patients with acquired immunodeficiency syndrome (AIDS). Methods: X-ray and multi-slice CT (MSCT) data from 33 AIDS patients with disseminated pulmonary tuberculosis confirmed by clinical manifestations and laboratory tests were analyzed retrospectively. Results: Thirty patients underwent initial chest radiography examination, 29 patients showed abnormal appearances, including bilateral disseminations in 21 patients and unilateral multiple disseminations in 8 patients. All patients underwent MSCT examination, 26 patients showed bilateral disseminations and 7 patients showed unilateral multiple disseminations. The abnormal pulmonary appearances included nodule (n = 25), miliary nodule (n = 22), air-space consolidation (n = 22), cavity (n = 11), fibrosis (n = 7), ground-glass opacity (n = 7), pneumatocele (n = 4), calcification (n = 2). There were 20 patients with more than 3 abnormal appearances and 13 patients with one or two abnormal appearances. The extra-pulmonary tuberculosis included pleural effusion (n = 33), lymphadenopathy (n = 30), intestinal tuberculosis (n = 3), splenic tuberculosis (n = 1) and cerebral tuberculosis (n = 1). Conclusion: Disseminated pulmonary tuberculosis should be highly suspected in AIDS patients with diffused nodules, miliary nodules, air-space consolidations or multiple cavities, accompanied with pleural effusion and lymphadenopathy. (authors)

  15. The imaging appearances of the pulmonary mucormycosis in patients with acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Liu Jinxin; Tang Xiaoping; Zhang Lieguang; Jiang Songfeng; Chen Bihua; Gan Xinqing; Huang Ruilian; Shi Hongling; Huang Wuzhi; Huang Deyang; Tang Yong

    2009-01-01

    Objective: To manifest the imaging appearances of the pulmonary mucormycosis in patients with acquired immunodeficiency syndrome (AIDS). Methods: The radiographic and high resolution computed tomography (HRCT) features of the pulmonary, mucormycosis in 13 patients with AIDS were retrospectively analyzed. Results: On radiography, the infiltrative lesions were found in 5 patients, 7 cases had reticular pattern, 4 cases had pleural effusion, 4 cases had enlarged hilar and mediastinal lymph nodes, 3 cases had diffuse milliary lesions, 3 cases had masses, 2 cases had ground-glass shadows, 2 cases had cystic lesions, cavity, pleural thickening, pericardial effusion and focal pneumothorax was presented in 1 case respectively. On HRCT, 7 cases had enlarged mediastinal lymph nodes, 7 cases had interlobular septal thickening, the infiltrative lesion were found in 6 patients, 5 cases had diffuse milliary lesions, 4 cases had pleural effusion, 3 cases had masses, 2 eases had ground-glass shadows, 2 cases had cystic lesions, cavity, pleural thickening, focal bronchiectasis, pericardial effusion and focal pneumothorax was presented in 1 case respectively. Conclusion: The main imaging appearances of the pulmonary mucormycosis in patients with AIDS include diffuse milliary lesion, enlarged hilar and mediastinal lymph node, interlobular septal thickening, infiltrative lesion, pleural effusion and mass. (authors)

  16. Self-care of elderly people after the diagnosis of acquired immunodeficiency syndrome.

    Science.gov (United States)

    Araujo, Graciela Machado de; Leite, Marinês Tambara; Hildebrandt, Leila Mariza; Oliveski, Cinthia Cristina; Beuter, Margrid

    2018-01-01

    to characterize the seropositive elderly for the Human Immunodeficiency Virus and Acquired Immunodeficiency Syndrome (HIV/AIDS) in their socio-demographic aspects; to understand how the elderly take care of themselves from the diagnosis of HIV/AIDS. Qualitative, descriptive, exploratory research conducted at a Voluntary Counseling and Testing Center with 10 elderly people receiving treatment for HIV/AIDS. The data were analyzed according to the content analysis. Data show the elderly people's lack of knowledge about HIV/AIDS transmission, the experience of being elderly and having HIV/AIDS, caring for oneself and life after diagnosis of HIV/AIDS in their daily lives. Final considerations: The diagnosis of HIV/AIDS seropositivity in the elderly generates a blend of feelings and fears that lead to food changes, adherence to treatment and the renunciation of daily and social habits, manifested as ways of self-care.

  17. Treatment of acquired immunodeficiency syndrome with Chinese medicine in China: opportunity, advancement and challenges.

    Science.gov (United States)

    Liu, Zhi-Bin; Wang, Xin; Liu, Hui-Juan; Jin, Yan-Tao; Guo, Hui-Jun; Jiang, Zi-Qiang; Li, Zhen; Xu, Li-Ran

    2013-08-01

    Chinese medicine (CM) has been used in the treatment of human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome (AIDS) for 30 years and the demonstrated therapeutic effects of CM, such as reducing plasma HIV viral load, increasing CD4(+)T cell counts, promoting immunity reconstitution, ameliorating symptoms and signs, improving the health related quality of life (HRQOL) and counteracting against the effects of anti-retroviral drugs, were summarized and reviewed in this article. The authors point out that it had been a good opportunity to use CM for the treatment of HIV infection and AIDS in the past and also there are huge challenges ahead for CM research and clinicians to discover more effective CM and its underlying mechanisms for treatment of AIDS.

  18. Gastric and Peritoneal Involvement of Human Herpes Virus 8 Related Kaposi Sarcoma in a Patient with Acquired Immunodeficiency Syndrome

    Directory of Open Access Journals (Sweden)

    Nuno Ribeiro Ferreira

    2015-09-01

    Full Text Available Kaposi's sarcoma (KS is one of the most frequent neoplastic diseases in patients infected with human immunodeficiency virus (HIV. The authors report the case of a 40-year-old male with ascites, peripheral edema and peritoneal carcinomatosis secondary to a gastric KS related to human herpes virus type 8 (HHV-8. The patient had severe immunodeficiency, with a TCD4+ count of 86 cells/µl and newly diagnosed acquired immunodeficiency syndrome. His clinical condition rapidly deteriorated, with multiorgan failure, and he died without the possibility of initiating antiretroviral therapy or chemotherapy. To the authors’ knowledge, carcinomatosis is a rare feature in KS.

  19. Sexually transmitted infections in India: Current status (except human immunodeficiency virus/acquired immunodeficiency syndrome

    Directory of Open Access Journals (Sweden)

    Thappa Devinder

    2007-01-01

    Full Text Available Sexually transmitted infections (STIs are more dynamic than other infections prevailing in the community. It is important that such dynamic epidemiological changes in STIs are acknowledged and kept track of in a vast and populous developing country like India, particularly in this HIV era. It is with this aim that the authors have reviewed the relevant literature in STI epidemiology in India during the past 25 years. Admittedly, there has been heterogeneity of data to account for the subcontinental dimension of this country. But a basic pattern in the changing epidemiology is discernible. Like the developed countries, in India too the bacterial STIs like chancroid and gonorrhea are declining, while viral STIs like HPV and herpes genitalis are on an upswing. The overall decline in the prevalence of STIs has to be interpreted with caution, however. This may partially reflect the improved facilities of treatment in the peripheral centres that obviates the need of many patients in attending the STD clinics in the tertiary centres. Also, the improved pharmacotherapy of many of the bacterial STIs may result in partial clearance and non-reporting of many of these infections.

  20. Knowledge and attitude about human immunodeficiency virus/acquired immunodeficiency syndrome among higher secondary school students of Jaipur city: A cross-sectional study

    Directory of Open Access Journals (Sweden)

    Pankaj Chaudhary

    2016-01-01

    Full Text Available Introduction: India is estimated to have the third highest number of human immunodeficiency virus (HIV infections in the world with about 20.89 lakh people currently living with HIV/acquired immunodeficiency syndrome (AIDS. Inadequate knowledge, negative attitudes, and ignorance among the school students are major hindrances to prevent the spread of HIV. Aim: To assess the knowledge and attitude toward HIV/AIDS among the higher secondary school students of Jaipur city. Materials and Methods: A questionnaire-based cross-sectional study comprising 613 higher secondary school students (male = 390, female = 223 from Jaipur city were included in the study. The city was divided into 4 zones and one school from each zone was selected randomly. A questionnaire assessing the knowledge and attitude toward HIV/AIDS was distributed among the senior school students. Pilot study was done among 50 students to test the validity of the questionnaire. Results: All the students (100% in our sample knew what is AIDS. About 96.2% of the students knew that AIDS is not a simple disease, the correct knowledge about the modes of transmission of HIV/AIDS was nearly 85.6% and about 94% of students would not leave the school if there was an AIDS student in their class. Eighty-four percent of students believed that students with AIDS should not go to special schools and about 95.8% students believed that HIV individuals must be supported, treated, and helped. Conclusion: The students had satisfactory knowledge about HIV/AIDS and their attitude toward this group of people was good. There is need and opportunity to provide factual and precise knowledge on HIV/AIDS for school students. There should also be a drive to increase education and awareness about HIV/AIDS in educational institutes.

  1. 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

    Directory of Open Access Journals (Sweden)

    Pallabi Dasgupta

    2016-07-01

    Full Text Available 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.

  2. Estimation of CD4+ and CD8+ T-lymphocytes in human immunodeficiency virus infection and acquired immunodeficiency syndrome patients in Manipur

    Directory of Open Access Journals (Sweden)

    Singh H

    2007-01-01

    Full Text Available Purpose : To estimate and stratify CD4 + and CD8 + T-lymphocyte levels in human immunodeficiency virus (HIV infected (asymptomatic and acquired immunodeficiency syndrome (AIDS patients (symptomatic and correlate the clinical features of the patients with CD4+ and CD8+ lymphocyte level. Methods : Between April 2002 and September 2003, a total of 415 HIV seropositive adult patients (297 males and 118 females attending Regional Institute of Medical Sciences (RIMS hospitals were tested for CD4+ and CD8+ T-lymphocytes by fluorescent activated cell sorter (FACS counter (Becton Dickinson. Symptomatic patients were diagnosed as per NACO clinical case definition. Results : Ranges of 0-50, 51-100, 101-200, 201-300, 301-400, 401-500 and above 500 CD4+ T-lymphocyte per microlitre were seen in 68, 52, 101, 73, 47, 31 and 43 patients respectively whereas CD8+ T-lymphocyte ranges of 0-300, 301-600, 601-900, 901-1500, 1501-2000, 2001-3500 per microlitre were seen in 29, 84, 92, 145, 40 and 25 patients respectively. One hundred and fifty patients were asymptomatic and 265 were symptomatic. CD4/CD8 ratio in asymptomatics and symptomatics were 0.13-1.69 and 0.01-0.93 respectively. Tuberculosis and candidiasis occurred in CD4+ T-lymphocyte categories between 0-400 cells per mL in symptomatics. However, cryptosporidiosis, toxoplasmosis, herpes zoster, cryptococcal meningitis, Pneumocystis carinii pneumonia, penicilliosis and cytomegalovirus retinitis were seen in patients having CD4+ T-lymphocyte less than 200 per mL. Conclusions : CD4+ T-lymphocyte was decreased in both asymptomatic and symptomatic HIV patients, The decrease was greater in symptomatics while CD8+ T-lymphocyte was increased in both except advanced stage symptomatics. CD4:CD8 ratio was reversed in both groups. Opportunistic infections correlated with different CD4+ T-lymphocyte categories.

  3. Serodiagnosis of Helicobacter pylori infection in patients with human immunodeficiency virus infection

    DEFF Research Database (Denmark)

    Nielsen, H; Andersen, L P

    1995-01-01

    In contrast to the established role of Helicobacter pylori gastritis in gastritis and duodenal ulcer in general, conflicting results have been reported in patients with human immunodeficiency virus (HIV) infection and the acquired immunodeficiency syndrome. The seroprevalence during early HIV...

  4. Congenital Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a Woman with Human Immunodeficiency Virus: A Case Report.

    Science.gov (United States)

    Fletcher, Amanda; Stowell, Justin; Jamoulis, Socrates

    2017-04-04

    Congenital tracheobronchomegaly (Mounier-Kuhn Syndrome, MKS) is a rare idiopathic disorder characterized by dilation of the central airways, including the trachea and first through fourth order bronchi. MKS disproportionately affects men and results in chronic respiratory tract infections. The diagnosis is made through the synthesis of clinical and radiological data. Here we report a unique case of MKS in a patient with human immunodeficiency virus (HIV) infection. A 45-year-old African American woman with a past medical history of HIV, tobacco and recreational drug abuse, chronic obstructive pulmonary disease, sleep apnea, and a 15-year history of recurrent respiratory infections presented with dyspnea, wheezing, a productive cough, increased yellow-green sputum production, and subjective fevers. Computerized tomography (CT) of the chest revealed striking dilation of the trachea and central bronchi. Fiberoptic bronchoscopy demonstrated a dilated trachea and bronchial tree with complete collapse of the trachea and bilateral mainstem bronchi during expiration. Serial imaging over 14 years allowed the radiologist to confidently diagnose her underlying disorder and recommend appropriate clinical management, which included mucolytics, chest physiotherapy, prophylactic vaccinations, and antibiotics during infectious exacerbations. To the best of our knowledge, there is only one reported case of MKS in the setting of HIV in the English literature. We report the second such case and outline the clinical presentation, diagnostic criteria, and management of MKS with the hope that increased awareness will prevent delayed or misdiagnosis for patients with MKS. This case highlights the common diagnostic delay for MKS and the need to include MKS in the differential diagnosis of recurrent respiratory tract infections.

  5. Imaging features of mycobacterium in patients with acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Yang Jun; Sun Yue; Wei Liangui; Xu Yunliang; Li Xingwang

    2013-01-01

    Objective: To analyze the imaging features of mycobacterium in AIDS patients. Methods: Twenty-three cases of mycobacterium tuberculosis and 13 patients of non-tuberculous mycobacteria were proved etiologically and included in this study. All patients underwent X-ray and CT examinations, imaging data were analyzed and compared. Results: The imaging findings of mycobacterium tuberculosis in AIDS patients included consolidation (n = 11), pleural effusion (n = 11), mediastinal lymphadenopathy (n = 11). Pulmonary lesions were always diffuse distribution, and 14 patients of extrapulmonary tuberculosis were found. Pulmonary lesions in non-tuberculous mycobacteria tend to be circumscribed. Conclusions: Non-tuberculous mycobacterial infection in AIDS patients is more common and usually combined with other infections. Imaging features are atypical. (authors)

  6. Management of a rare presentation of Vogt-Koyanagi-Harada disease in human immunodeficiency virus/acquired immunodeficiency disease syndrome patient.

    Science.gov (United States)

    Priya, D; Sudharshan, S; Biswas, Jyotirmay

    2017-05-01

    Vogt-Koyanagi-Harada (VKH), a multisystem autoimmune bilateral panuveitis with systemic manifestations, is uncommon in immunocompromised patients such as human immunodeficiency virus (HIV)/acquired immunodeficiency disease syndrome (AIDS). We report a rare presentation of VKH in a 45-year-old HIV-positive female on highly active antiretroviral therapy (HAART) who presented with a history of recurrent panuveitis. A diagnosis of probable VKH was made based on ocular and systemic signs and symptoms. She was treated with topical and systemic steroids with close monitoring of CD4 counts and viral loads. After inflammation control, complicated cataract was managed surgically under perioperative steroid cover. VKH in HIV/AIDS has not been reported earlier. This case shows that significant inflammation can be seen even in HIV/AIDS patients on HAART with VKH in spite of moderate CD4 counts. Management is a challenge considering the systemic risks with long-term use of steroids.

  7. Do Students Eventually Get to Publish their Research Findings? The Case of Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome Research in Cameroon.

    Science.gov (United States)

    Munung, Ns; Vidal, L; Ouwe-Missi-Oukem-Boyer, O

    2014-05-01

    Scientific publication is commonly used to communicate research findings and in most academic/research settings, to evaluate the potential of a researcher and for recruitment and promotion. It has also been said that researchers have the duty to make public, the findings of their research. As a result, researchers are encouraged to share their research findings with the scientific world through peer review publications. In this study, we looked at the characteristics and publication rate of theses that documented studies on human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome in Cameroon. TO CHECK IF A THESIS RESULTED IN A PUBLICATION, WE SEARCHED: A database of publications on HIV in Cameroon, African Journals Online, PubMed and Google scholar. For each publication we recorded if the student was an author, the position of the student in the author listing, the journal and where the journal was indexed. We also looked at the impact factor of the journals. One hundred and thirty theses/dissertations were included in the study, 74.6% (97/130) were written as part of a medical degree (MD), 23.8% (31/130) a postgraduate (PG) degree and 1.5% (2/130) for a Doctorate/PhD. On a whole, 13.9% (18/130) of the theses resulted in at least one publication in a scientific journal with a total of 22 journal articles, giving a mean publication rate of 0.17 article/thesis, 86.4% (11/22) were indexed on PubMed, 9.1% (2/22) on African Journals Online and 4.6% (1/22) on Google scholar. One PG thesis led to two book chapters. The student was the first author in 22.7% (5/22) of the articles and not an author in 9.1% (2/22) of the articles. Student supervisor was an author in all the articles. This study reveals that most students in Cameroon failed to transform their theses/dissertations to scientific publications. This indicates an urgent need to sensitize students on the importance of presenting their research findings in scientific meetings and peer reviewed journals

  8. The Basic Nature of Ethical Problems Experienced by Persons with Acquired Immunodeficiency Syndrome: Implications for Nursing Ethics Education and Practice.

    Science.gov (United States)

    Cameron, Miriam E.; And Others

    1993-01-01

    Twenty-five persons with acquired immunodeficiency syndrome (AIDS) described and validated 100 ethical problems that are experienced by people with AIDS from 3 levels of ethical inquiry: descriptive ethics, normative ethics, and metaethics. Findings suggest strategies for improving nursing ethics education. (JOW)

  9. Utility of gallium imaging in the diagnosis of Mycobacterium avium-intracellulare infection in patients with the acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Bach, M.C.; Bagwell, S.P.; Masur, H.

    1986-01-01

    Whole body Ga-67 scans revealed increased uptake in lymph nodes accessible for biopsy in three patients with the acquired immunodeficiency syndrome (AIDS) infected by Mycobacterium avium-intracellulare (MAI). In diagnostically difficult cases where the usual methods for diagnosing MAI are not helpful, Ga-67 studies may be of value

  10. A preliminary report of 99Tcm-ECD brain SPECT imaging in patients with acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Xu Hao; Tong Yuwei; Luo Jinxiang; Chen Jian; Wu Qiulian

    2001-01-01

    Objective: To investigate the changes of regional cerebral blood flow (rCBF) in patients with acquired immunodeficiency syndrome (AIDS). Methods: 99 Tc m -ECD brain SPECT imaging was performed on 5 patients with AIDS and 16 sex- and age-matched normal controls. The rCBF percentages compared to the cerebellum were calculated using a semi-quantitative processing software. Results: Hypo-perfusions in the right and left frontal, temporal, parietal lobe, basal ganglia and left thalamus were seen in 1 patient with dementia. Hypo-perfusions in the right and left frontal and temporal lobe were seen in 4 patients without dementia. The rCBF in the right and left frontal, temporal, parietal lobe, basal ganglia and thalamus, straight gyri and pons decreased significantly in patients with AIDS than those of the control subjects (P < 0.01). Conclusion: There is reduced cortico-subcortical rCBF in patients with AIDS

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

    Science.gov (United States)

    Wang, Ying; Cochran, Christopher; Xu, Peng; Shen, Jay J; Zeng, Gang; Xu, Yanjun; Sun, Mei; Li, Chengyue; Li, Xiaohong; Chang, Fengshui; Lu, Jun; Hao, Mo; Lu, Fan

    2014-02-13

    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. 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. 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. 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 housekeepers and migrant day laborers result in

  12. Pathology of Penicillium marneffei. An emerging acquired immunodeficiency syndrome-related pathogen.

    Science.gov (United States)

    Cooper, C R; McGinnis, M R

    1997-08-01

    To summarize current knowledge regarding the opportunistic dimorphic fungal pathogen Penicillium marneffei. Clinical presentation, differential diagnosis, mycology, histopathology, diagnostic serology, in vitro antifungal agent susceptibility testing, and therapy are discussed for human immunodeficiency virus-infected individuals primarily living in Southeast Asia. Critical evaluation of peer-reviewed publications located through an electronic literature database search, supplemented by unpublished observations, were used to prepare this report. Studies were selected based on either the fungal name Penicillium marneffei, penicilliosis, penicilliosis marneffei, or a combination of these. Articles were reviewed with appropriate data being abstracted and then synthesized into the review. Differential diagnostic criteria for tissue diagnosis and laboratory identification of the fungus are detailed. The usefulness of mycoserology and antifungal therapy are evaluated. Penicillium marneffei is an emerging pathogen, primarily among patients with acquired immunodeficiency syndrome residing in Southeast Asia. Although infection caused by P marneffei is endemic to this portion of the world, cases are being diagnosed and treated involving individuals who have traveled to this region. Penicilliosis marneffei can clinically resemble tuberculosis, molluscum contagiosum, cryptococcosis, and histoplasmosis. The successful treatment of P marneffei infection is dependent on its rapid and accurate diagnosis.

  13. DESTRUCTIVE LESIONS OF BONES AS A RESULT OF MYCOBACTERIAL PROCESS IN CHILDREN WITH INITIAL IMMUNODEFICIENCIES (CLINICAL, DIAGNOSTICAL AND TACTIC PECULIARITIES

    Directory of Open Access Journals (Sweden)

    A.Yu. Mushkin

    2011-01-01

    Full Text Available Initial immunodeficiencies are genetically conditioned defects of immune system; they are the basis for generalized infections including those induced by mycobacteria of tuberculosis complex. The lesions of skeleton in those patients are of different types depending on the kind of immunodeficiency. The article presents the results of clinical observation, conservative and surgical treatment of 12 children with mycobacterial lesions of skeleton on the ground different initial immunodeficiencies — severe combined immune deficiency, chronic granulematosis and insufficiency of interferon and interleukin 12.Key words: children, initial immunodeficiency, mycobacterial infection, bone lesions, surgical treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3: 60–64

  14. Bone paracoccidioidomycosis associated with acquired immunodeficiency syndrome: evaluation by radiological methods - a case report

    International Nuclear Information System (INIS)

    Maeda, Lucimara; Hara, Monica Hiromi; Sabedotti, Ismail Fernando; Montandon, Cristiano; Torriani, Martin; Nanni, Livio

    1999-01-01

    Paracoccidioidomycosis is a chronic granulomatous disease, caused by the Paracoccidioides brasiliensis fungus. This disease involves primarily the lungs, although disseminated forms may occur. Bone involvement is rare, and is associated to systemic disease or may present as a focal lesion. An association between paracoccidioidomycosis and the acquired immunodeficiency syndrome (AIDS) was first established in 1989. There are only a few reports on this association, and none of them was the fungus isolated from bone lesions. This is the first case report on the paracoccidioidomycosis-AIDS association in which bone lesions demonstrated infection by P. brasiliensis. The authors propose the inclusion of paracoccidioidomycosis in the differential diagnosis of lytic bone lesions in patients with AIDS and discuss the role of radiological diagnostic in this case. (author)

  15. [Systemic lupus erythematosus masking the acquired immunodeficiency syndrome. A report on four cases].

    Science.gov (United States)

    Kotyla, Przemysław; Kucharz, Eugeniusz J

    2012-01-01

    Systemic lupus erythematosus (SLE) is a systemic inflammatory disease of connective tissue with an unknown etiology and a rich clinical picture with involvement of multiple organs. Given the rich symptomatology, application of the current classification criteria is associated with a significant risk of attributing symptoms of other pathologies to lupus and/or other connective tissue disease. Inherited and acquired immune deficiencies may sometimes demonstrate a lupus-like clinical symptomatology. In this work we reviewed 4 of cases referred to the Department of Internal Diseases and Rheumatology of the Silesian Medical University in Katowice with suspected or confirmed systemic lupus erythematosus. A positive anti-HIV antibody test led to the diagnosis of the acquired immunodeficiency syndrome (AIDS). Due to the close similarity of the clinical picture and the presence of antinuclear antibodies in both diseases, the authors postulate that the anti-HIV antibody test should be done routinely in patients with connective tissue diseases.

  16. Human papillomavirus-associated cancers as acquired immunodeficiency syndrome defining illnesses

    Directory of Open Access Journals (Sweden)

    Shohreh Shahabi

    2013-04-01

    Full Text Available The Centers for Disease Control currently report cervical, vulvar, vaginal, anal and some head and neck cancers as human papillomavirus (HPV-associated cancers. Only cervical cancer is listed amongst acquired immunodeficiency syndrome (AIDS defining illnesses. All of these cancers may represent progression of the immunocompromised state with the inability to eradicate viral infection. This study reports the case of a 27-year old HIV positive female presenting with a persistent right vulvar exophytic lesion. High-risk HPV analysis and immunostaining for P16 were both positive. A biopsy of the lesion revealed invasive squamous cell carcinoma. The patient underwent neoadjuvant radiation and chemotherapy followed by a radical vulvectomy. During treatment, her CD4 T-lymphocyte count decreased to 120 advancing her condition from HIV to AIDS. This case suggests that all HPV-associated cancers should be included as AIDS defining illnesses.

  17. Immunological studies in acquired immunodeficiency syndrome. Functional studies of lymphocyte subpopulations

    DEFF Research Database (Denmark)

    Hofmann, B; Ødum, Niels; Platz, P

    1985-01-01

    The lymphocyte transformation response in vitro to mitogens (phytohaemagglutinin, concanavalin A, and pokeweed mitogen) and antigens (purified protein derivative and tetanus) was studied in three patients with acquired immunodeficiency syndrome (AIDS), three patients with pre-AIDS, and six healthy...... controls before and after depletion of T4- or T8-positive cells. In controls, T8-depleted lymphocytes responded as well as peripheral blood mononuclear cells (PBMC) when monocytes were added, whereas T4-depleted cells gave about 50% of this response to mitogens and no response at all to antigens....... No evidence of suppression was seen when various mixtures of T4- and T8-depleted cells were made. In particular, there was a virtually linear relationship between the percentage of T8-depleted cells and the response to antigens. The PBMC of all AIDS and pre-AIDS patients had very low or absent responses...

  18. Pneumocystis carinii pneumonia in acquired immunodeficiency syndrome - correlation of high-resolution computed tomography and anatomopathology

    International Nuclear Information System (INIS)

    Marchiori, Edson; Moreira, Luiza Beatriz; Capone, Domenico; Moraes, Heleno Pinto de; Pereira, Cyntia Inez Guedes Soares

    2001-01-01

    We present the main findings observed on the high-resolution computed tomography examinations of 15 patients with acquired immunodeficiency syndrome and Pneumocystis carinii pneumonia. The high-resolution computed tomography and autopsy findings of 5 patients were also compared. The most frequently observed high-resolution computed tomography patterns were ground-glass attenuation, consolidation areas, crazy-paving pattern and cysts. Nodules and intralobular reticulation were less frequently observed. Ground-glass attenuation and consolidation areas corresponded to alveolar filling with inflammatory exudate. Thickening of the interlobular septa was due to cell infiltration and edema. One patient presented interlobular reticulation, and the pathology study revealed alveolar septa thickening due to cell infiltration and fibrosis. Nodules observed in one of the patients corresponded to a patchy intra alveolar accumulation of microorganisms and inflammatory cells forming a 'granulomatous' pattern. (author)

  19. Fear of acquired immunodeficiency syndrome and fear of other illness in suicide

    DEFF Research Database (Denmark)

    Aro, A R; Jallinoja, P T; Henriksson, M M

    1994-01-01

    Suicide victims with fear of acquired immunodeficiency syndrome (AIDS) or other somatic illness were compared for psychosocial and health-related characteristics, triggers and content of fear. Fear of AIDS cases (n = 28), 2% of the 1-year Finnish suicide population (n = 1397), were younger...... and fewer had serious somatic disease (32% vs 64%) compared with cases of fear of other somatic illness. Both groups had more depression, especially major depression (54% and 61% vs 26%), more psychotic disorders (50% and 32% vs 24%) and health care contacts during their final week (61% and 64% vs 36%) than...... other suicides. Suicidal fear of AIDS calls for evaluation of sexual and other risk behaviour, but fear of AIDS was largely generated by the extensive media coverage. Fear of other somatic illness was more diverse in origin and related to illness experiences. Suicidal fear of illness calls...

  20. Pediatric patient with systemic lupus erythematosus & congenital acquired immunodeficiency syndrome: An unusual case and a review of the literature

    Directory of Open Access Journals (Sweden)

    Rezaee Fariba

    2008-05-01

    Full Text Available Abstract The coexistence of systemic lupus erythematosus (SLE in patients with congenital human immunodeficiency virus (HIV infection is rare. This is a case report of a child diagnosed with SLE at nine years of age. She initially did well on non-steroidal anti-inflammatory agents, hydroxychloroquine, and steroids. She then discontinued her anti-lupus medications and was lost to follow-up. At 13 years of age, her lupus symptoms had resolved and she presented with intermittent fevers, cachexia, myalgias, arthralgias, and respiratory symptoms. Through subsequent investigations, the patient was ultimately diagnosed with congenitally acquired immunodeficiency syndrome (AIDS.

  1. Indeterminate human immunodeficiency virus western blot results in Iranian patients with discordant screening assay results

    International Nuclear Information System (INIS)

    Ravanshad, M.; Sabahi, F.; Mahboudi, F.; Sabahi, F.

    2006-01-01

    The Western blot (WB) assay is the most widely accepted confirmatory assay for the detection and confirmation of antibodies to human immunodeficiency virus type 1 (HIV-1) and 2 (HIV-2). However, indeterminate WB reactivity to HIV-1 and HIV-2 proteins may occur in individuals who do not appear to be infected with HIV. In this study, we describe the results of indeterminate WB reactivity in Iranian patients with discordant screening assays. The samples were obtained from Iranian Blood Transfusion Center, Tehran, Iran and evaluated in the Biotechnology Process Development Center, Pasteur Institute of Iran, Tehran, Iran between 2003 and 2004. A total of 4707 were tested for the presence of HIV-1 antibodies. Six hundred and four (12.8%) patients tested for HIV were positive for HIV-1 antibody. Nine (1.49%) have discordant results among screening assays and indeterminate WB results as interpreted by Centers for Disease Control and Prevention (CDC) criteria. Most (66.7%) of these indeterminate WB results were due to p24 reactivity. However, 2(22.2%) display reactivity to both gp41 and gp120 proteins [Positive by World Health Organization (WHO) criteria]. Of 9 WB assays initially indeterminate by the CDC criteria and with follow-up samples 8(88.8%) became negative when retested subsequently while one (11.1%) remained indeterminate for more than a year and were thus considered negative. In addition all the indeterminate samples were negative when assessed by polymerase chain reaction assay. In general, there were was an 88.8% concordance between the CDC and WHO criteria for an indeterminate WB result. The CDC II criteria for an indeterminate WB result. The CDC II criteria best met the specified objectives for diagnosis in our setting. (author)

  2. Borderline tuberculoid leprosy: A manifestation of immune reconstitution inflammatory syndrome in a human immunodeficiency virus infected person

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    Mukhopadhyay Partha

    2006-01-01

    Full Text Available Immune reconstitution inflammatory syndrome describes a collection of inflammatory disorders associated with paradoxical deterioration of various pre-existing processes following start of highly active antiretroviral therapy (HAART in human immunodeficiency virus (HIV-infected patients. Leprosy as an opportunistic infection in immune reconstitution syndrome has been rarely reported in literature. A case of a 30-year-old HIV positive man with extrapulmonary tuberculosis of left foot on HAART having developed borderline tuberculoid leprosy as opportunistic infection in immune reconstitution syndrome has been reported.

  3. Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX syndrome: a paradigm of immunodeficiency with autoimmunity

    Directory of Open Access Journals (Sweden)

    Federica eBarzaghi

    2012-07-01

    Full Text Available Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX syndrome is a rare monogenic primary immunodeficiency (PID due to mutations of FOXP3, a key transcription factor for naturally occurring (n regulatory T (Treg cells. The dysfunction of Treg cells is the main pathogenic event leading to the multi-organ autoimmunity that characterizes IPEX syndrome, a paradigm of genetically determined PID with autoimmunity. IPEX has a severe early onset and can become rapidly fatal within the first year of life regardless of the type and site of the mutation. The initial presenting symptoms are severe enteritis and/or type 1 diabetes mellitus, alone or in combination with eczema and elevated serum IgE. Other autoimmune symptoms, such as hypothyroidism, cytopenia, hepatitis, nephropathy, arthritis, and alopecia, can develop in patients who survive the initial acute phase.The current therapeutic options for IPEX patients are limited. Supportive and replacement therapies combined with pharmacological immunosuppression are required to control symptoms at onset. However, these procedures can allow only a reduction of the clinical manifestations without a permanent control of the disease. The only known effective cure for IPEX syndrome is haematopoietic stem cell transplantation, but it is always limited by the availability of a suitable donor and the lack of specific guidelines for bone marrow transplant in the context of this disease.This review aims to summarize the clinical histories and genomic mutations of the IPEX patients described in the literature to date. We will focus on the clinical and immunological features that allow differential diagnosis of IPEX syndrome and distinguish it from other PID with autoimmunity. The efficacy of the current therapies will be reviewed, and possible innovative approaches, based on the latest highlights of the pathogenesis to treat this severe primary autoimmune disease of childhood, will be discussed.

  4. Bronchogenic carcinoma in acquired immunodeficiency syndrome - report of two cases; Carcinoma broncogenico na sindrome da imunodeficiencia adquirida - relato de dois casos

    Energy Technology Data Exchange (ETDEWEB)

    Siciliano, Antonio Alexandre de Oliveira [Hospital Universitario Clementino Fraga Filho, Rio de Janeiro, RJ (Brazil). Servico de Radiodiagnostico; Melo, Alessandro Severo Alves de; Marchiori, Edson [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia

    1999-12-01

    The authors report two cases of bronchogenic carcinoma in patients with acquired immunodeficiency syndrome. The first patient, a ee-year-old male, developed a left hilar adenocarcinoma, with spleen and bilateral adrenal metastases. The disease progressed unfavourably, resulting in the patient's death in less than a month. The second patient, a 47-year-old male, developed a large mass in the left upper lobe, with invasion of the thoracic wall and destruction of adjacent ribs. The histopathologic study revealed a non-oat-cell carcinoma. Both patients received palliative treatment since diagnosis was established late in the course of the disease. Recent studies suggest an association between bronchogenic carcinoma and human immunodeficiency virus infection. However, an actual increase in the prevalence of bronchogenic carcinoma in HIV-positive patients remains controversial. (author)

  5. [The use of growth hormone to treat endocrine-metabolic disturbances in acquired immunodeficiency syndrome (AIDS) patients].

    Science.gov (United States)

    Spinola-Castro, Angela Maria; Siviero-Miachon, Adriana A; da Silva, Marcos Tadeu Nolasco; Guerra-Junior, Gil

    2008-07-01

    Acquired Immunodeficiency Syndrome (Aids) was initially related to HIV-associated wasting syndrome, and its metabolic disturbances to altered body composition. After Highly Active Antiretroviral Therapy (HAART) was started, malnutrition has declined and HIV-associated lipodystrophy syndrome has emerged as an important metabolic disorder. Aids is also characterized by hormonal disturbances, principally in growth hormone/insulin-like growth factor 1 (GH/IGF-1) axis. The use of recombinant human GH (hrGH) was formerly indicated to treat wasting syndrome, in order to increase lean body mass. Even though the use of hrGH in lipodystrophy syndrome has been considered, the decrease in insulin sensitivity is a limitation for its use, which has not been officially approved yet. Diversity in therapeutic regimen is another limitation to its use in Aids patients. The present study has reviewed the main HIV-related endocrine-metabolic disorders as well as the use of hrGH in such conditions.

  6. Cholecystitis in a child with acquired immunodeficiency syndrome: ultrasonographic findings in a case of uncommon presentation during childhood

    International Nuclear Information System (INIS)

    Rubi-Palomares, I.; Martinez-Leon, M. I.; Alonso-Usabiaga, I.; Ceres-Ruiz, L.

    2001-01-01

    We present the case of a boy with acquired immunodeficiency syndrome (AIDS) in whom ultrasound revealed the presence of a calculous cholecystitis. Abdominal ultrasound was essential in the initial diagnosis and the assessment of the response to treatment. This entity has rarely been reported in the pediatric radiology literature. In our patient, the cholecystitis may have been caused by infection, and the involvement of regional lymphatic obstruction in the pathogenesis is considered. (Author) 14 refs

  7. Presentation and outcome amongst older Singaporeans living with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS): does age alone drive excess mortality?

    Science.gov (United States)

    Huggan, Paul J; Foo, Rui Min; Olszyna, Dariusz; Chew, Nicholas S; Smitasen, Nares; Mukhopadhyay, Amartya; Archuleta, Sophia

    2012-12-01

    There is little detailed information on human immunodeficiency virus (HIV) amongst older adults in Singapore. A retrospective study of 121 consecutive referrals of patients presenting for HIV care was conducted. Demographic, clinical and laboratory variables were collected. A prognostic model derived from the North American Veterans' Affairs Cohort Study (VACS) was used to estimate prognosis. The median age at presentation was 43 (range, 18 to 76). Thirty-eight patients (31%) were aged 50 or older and 106 patients (88%) were male. Older patients were more likely to be of Chinese ethnicity (P = 0.035), married (P = 0.0001), unemployed or retired (P = 0.0001), and to have acquired their infection heterosexually (P = 0.0002). The majority of patients in both groups were symptomatic at presentation. Eighty-one (67%) had CD4 counts less than 200 at baseline with no observable differences in HIV ribonucleic acid (RNA) or clinical stage based on age. Non-Acquired Immunodeficiency Syndrome (AIDS) morbidity was observed more frequently amongst older patients. The estimated prognosis of patients differed significantly based on age. Using the VACS Index and comparing younger patients with those aged 50 and above, mean 5 year mortality estimates were 25% and 50% respectively (P HIV/AIDS cases and present with more non-AIDS morbidity. This confers a poor prognosis despite comparable findings with younger patients in terms of clinical stage, AIDS-defining illness, CD4 count and HIV viral load.

  8. Is human immunodeficiency virus/acquired immunodeficiency syndrome decreasing among Brazilian injection drug users? Recent findings and how to interpret them

    Directory of Open Access Journals (Sweden)

    Francisco I Bastos

    2005-02-01

    Full Text Available We briefly review findings from Brazilian settings where the human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS epidemic among injection drug users (IDUs seems to be decreasing, highlighting recent findings from Rio de Janeiro and discussing methodological alternatives. Former analyses using serologic testing algorithm for recent HIV seroconversion have shown that HIV incidence has been low in IDUs recruited by two different surveys carried out in Rio, where low injection frequencies and infection rates have been found among new injectors. The proportion of AIDS cases among IDUs in Rio has been fairly modest, compared to São Paulo and especially to the southernmost states. Notwithstanding, the interpretation of findings from serial surveys constitutes a challenge, magnified in the assessment of HIV spread among IDUs due to the dynamic nature of the drug scenes and limitations of sampling strategies targeting hard-to-reach populations. Assessment of epidemic trends may profit from the triangulation of data, but cannot avert biases associated with sampling errors. Efforts should be made to triangulate data from different sources, besides exploring specific studies from different perspectives. In an attempt to further assess the observed trends, we carried out original analyses using data from Brazilian AIDS databank.

  9. Angiographic Features and Cardiovascular Risk Factors in Human Immunodeficiency Virus-Infected Patients With First-Time Acute Coronary Syndrome

    DEFF Research Database (Denmark)

    Knudsen, Andreas; Mathiasen, Anders B; Worck, R.H.

    2013-01-01

    A matched cohort study was conducted comparing patients with first-time acute coronary syndromes infected with human immunodeficiency virus (HIV) to non-HIV-infected patients with and without diabetes matched for smoking, gender, and type of acute coronary syndrome who underwent first-time coronary...... angiography. A total of 48 HIV-infected patients were identified from a national database. Coronary angiography showed that the HIV-infected patients had significantly fewer lesions with classification B2/C than the 2 control groups (p...

  10. Síndrome de Edwards asociado a inmunodeficiencia combinada Edwards' syndrome associated to combined immunodeficiency

    Directory of Open Access Journals (Sweden)

    Vianed Marsán Suárez

    2011-09-01

    Full Text Available El síndrome de Edwards es originado por un desbalance cromosómico representado por una trisomía 18. Alrededor de 95 % de los pacientes corresponden a trisomía completa, donde están presentes múltiples malformaciones en órganos y sistemas. El 5 % restante pertenece a trisomía parcial o mosaicismo, con un fenotipo incompleto por la ausencia de algunas anomalías típicas del síndrome. La inmunodeficiencia es una manifestación poco frecuente del síndrome Edwards. Se presenta el caso de una paciente de 9 meses de edad con trisomía 18 parcial e infecciones severas recurrentes desde la etapa neonatal, asociadas a anemia, linfopenia, trombocitopenia y neutrofilia. La ecografía mostró una hipoplasia del timo. Se encontraron cifras disminuidas de linfocitos TCD4+, CD8+ y de células asesinas naturales. La cuantificación de linfocitos B fue normal. Se hallaron concentraciones normales de inmunoglobulinas séricas IgM e IgG y disminuidas de IgA. Se encontró una disminución de la actividad hemolítica total de la vía clásica del complemento. No se encontraron alteraciones en la función opsonofagocítica. Se diagnosticó una inmunodeficiencia combinada asociada, hecho que demostró la heterogeneidad de la expresión clínica del síndrome Edwards y la relación entre el defecto cromosómico y la formación del sistema inmune en el período intrauterino.Edwards' syndrome is caused by a chromosomal imbalance represented by trisomy 18. Complete trisomy accounts for 95% of patients who present multiple malformations in organs and systems. The remaining 5% presents partial trisomy or mosaicism, with incomplete phenotype due to lack of some typical anomalies of this syndrome. Immunodeficiency is a rare manifestation of Edwards' syndrome. The case of a 9-months old female patient with partial trisomy 18 and recurrent severe infections since the neonatal phase, all associated to anemia, lymphopenia, thrombocytopenia and neutrophilia, was

  11. Kaposi sarcoma of the conjunctiva and eyelids associated with the acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Shuler, J.D.; Holland, G.N.; Miles, S.A.; Miller, B.J.; Grossman, I.

    1989-01-01

    Three studies were performed to assess more accurately the prevalence, natural history, and appropriate treatment of acquired immunodeficiency syndrome (AIDS)-related Kaposi sarcoma involving ocular structures. The first study was a prospective examination of 100 male homosexuals with AIDS-related Kaposi sarcoma for signs of ophthalmic involvement. Of the 20 patients who had ophthalmic lesions, 16 had eyelid lesions and seven had conjunctival lesions. In four patients, the ophthalmic lesion was the first, and initially the only, clinically identified manifestation of Kaposi sarcoma. The second study was a retrospective review of all patients with ophthalmic Kaposi sarcoma examined at one institution over a six-year period to determine its natural history and response to therapy. Most lesions were slowly progressive and responded to systemic drug therapy. Six patients were successfully treated with radiation therapy to prevent complications. The third study was a retrospective review of all patients with AIDS-related ophthalmic Kaposi sarcoma treated with local irradiation by one radiation oncologist. Each of 12 patients showed a response to treatment, and ten had a complete resolution of lesions, but recurrences were common. Side effects included skin erythema in six patients and hair loss in one patient. For local treatment of ophthalmic Kaposi sarcoma, irradiation appears to be safe and effective for palliative therapy

  12. Epstein–Barr Virus Susceptibility in Activated PI3Kδ Syndrome (APDS Immunodeficiency

    Directory of Open Access Journals (Sweden)

    Jean-Marie Carpier

    2018-01-01

    Full Text Available Activated PI3Kδ Syndrome (APDS is an inherited immune disorder caused by heterozygous, gain-of-function mutations in the genes encoding the phosphoinositide 3-kinase delta (PI3Kδ subunits p110δ or p85δ. This recently described primary immunodeficiency disease (PID is characterized by recurrent sinopulmonary infections, lymphoproliferation, and susceptibility to herpesviruses, with Epstein–Barr virus (EBV infection being most notable. A broad range of PIDs having disparate, molecularly defined genetic etiology can cause susceptibility to EBV, lymphoproliferative disease, and lymphoma. Historically, PID patients with loss-of-function mutations causing defective cell-mediated cytotoxicity or antigen receptor signaling were found to be highly susceptible to pathological EBV infection. By contrast, the gain of function in PI3K signaling observed in APDS patients paradoxically renders these patients susceptible to EBV, though the underlying mechanisms are incompletely understood. At a cellular level, APDS patients exhibit deranged B lymphocyte development and defects in class switch recombination, which generally lead to defective immunoglobulin production. Moreover, APDS patients also demonstrate an abnormal skewing of T cells toward terminal effectors with short telomeres and senescence markers. Here, we review APDS with a particular focus on how the altered lymphocyte biology in these patients may confer EBV susceptibility.

  13. Progressive outer retinal necrosis associated with occlusive vasculitis in acquired immunodeficiency syndrome

    Directory of Open Access Journals (Sweden)

    Chien-Chi Tseng

    2015-05-01

    Full Text Available A 45-year-old man, a case of acquired immunodeficiency syndrome, received a highly active antiretroviral therapy at the outpatient service for 4 years without regular follow-up. He experienced progressively blurred vision for 6 months and a cutaneous zoster on his back 3 months ago. He was diagnosed with progressive outer retinal necrosis by polymerase chain reaction-restriction fragment length polymorphism using an aqueous humor sample, which revealed an existence of varicella zoster virus. He was given a combination of systemic, intravitreal antiviral and a highly active antiretroviral therapy. Occlusive vasculitis, an unusual finding for progressive outer retinal necrosis, developed in both eyes 1 week after the secondary intravitreal injection. Unfortunately, his vision deteriorated to no light perception in both eyes within 2 weeks. Progressive outer retinal necrosis is characterized clinically as showing minimal or no inflammation in the aqueous and vitreous humors, absence of retinal vasculitis, and patches of yellowish spots located deep in the retina. Physicians should pay attention to this rare case of progressive outer retinal necrosis associated occlusive vasculitis with very poor prognosis in spite of aggressive treatment.

  14. Radiation therapy for Kaposi's sarcoma associated with acquired immunodeficiency syndrome. Tokyo Metropolitan Komagome Hospital experience

    International Nuclear Information System (INIS)

    Ebara, Takeshi; Karasawa, Katsuyuki; Maebayashi, Katsuya; Kurosaki, Hiromasa; Ishikawa, Hitoshi; Kaizu, Toshihide; Tanaka, Yoshiaki; Akagi, Kumiko; Masuda, Gota

    2000-01-01

    Kaposi's sarcoma is frequently found in association with acquired immunodeficiency syndrome (AIDS). We report on radiotherapy for patients with AIDS-related Kaposi's sarcoma at Tokyo Metropolitan Komagome Hospital. Between April 1991 and May 1997, radiotherapy was given to 11 lesions in eight men with AIDS-related Kaposi's sarcoma to relieve their symptoms. The lesions involved the head and neck region, the legs, and the gastrointestinal tract. Radiotherapy was carried out with 4-MV photon through parallel opposed field or high energy electrons. Total doses ranged from 20 to 38 Gy, with a median of 30 Gy, delivered in 2- to 3-Gy fractions. Four patients were given other treatments prior to the radiotherapy. Acute reaction was evaluated according to the modified acute radiation morbidity scoring criteria of the Radiation Therapy Oncology Group (RTOG). Radiotherapy had relieved the symptoms in all patients at completion of this therapy. Lesions that involved the hard palate and vocal cords had completely disappeared. The lesions that received radiotherapy were controlled without symptoms until the patients died. Patients who had the head and neck region treated exhibited severe acute mucosal reaction (at a dose of 30 Gy, there was grade 2 morbidity by modified RTOG criteria, in two patients, and grade 3 in three patients) although the radiation therapy was completed for these patients. Radiotherapy promises a favorable outcome for symptom relief in AIDS-related Kaposi's sarcoma. (author)

  15. Primary Immunodeficiency Diseases in Aguascalientes, Mexico: Results from an Educational Program.

    Science.gov (United States)

    Alvarez-Cardona, Aristoteles; Espinosa-Padilla, Sara Elva; Reyes, Saul Oswaldo Lugo; Ventura-Juarez, Javier; Lopez-Valdez, Jaime Asael; Martínez-Medina, Lucila; Santillan-Artolozaga, Alberto; Cajero-Avelar, Adriana; De Luna-Sosa, Alma R; Torres-Bernal, Luis F; Espinosa-Rosales, Francisco J

    2016-04-01

    Primary immunodeficiencies (PIDs) are a heterogeneous group of disorders characterized mainly by recurrent infections. Late diagnosis remains as one of the main issues to solve. We aimed to increase PID diagnosis in Aguascalientes, a 1.3 million inhabitants state in the center of Mexico, and to describe the clinical features of such patients. We developed an educational program for health personnel and general public; patients with possible PID were referred to a State University clinical center from December 2011 to December 2012. The patients were evaluated at the clinic and their definitive diagnosis pursued through laboratory, molecular and genetic assays. We describe the findings of those patients and analyze the impact of the program in terms of number of referrals. After 41 talks and 12 media appearances 151 patients were referred for evaluation. Fifteen (9.9%) were diagnosed with PID: five (33%) had antibody deficiencies, seven (47%) Well-defined syndromes, two (13%) Severe combined Immunodeficiency (SCID) and one case (7%) of an innate immune deficiency. All of the 15 PID patients had been referred by physicians, as opposed to the public. We estimated a "number needed to teach" of 75 physicians to get one PID patient referral. Educational programs are a fundamental part of the global efforts to increase PID diagnosis and care. To be successful, such programs should include public relations, reach for first-contact physicians, and aim to develop an efficient referral network with molecular diagnostic capability. Enhancing medical knowledge on PID is a successful strategy to improve early diagnosis and treatment.

  16. Identification of a mutation associated with fatal Foal Immunodeficiency Syndrome in the Fell and Dales pony.

    Directory of Open Access Journals (Sweden)

    Laura Y Fox-Clipsham

    2011-07-01

    Full Text Available The Fell and Dales are rare native UK pony breeds at risk due to falling numbers, in-breeding, and inherited disease. Specifically, the lethal Mendelian recessive disease Foal Immunodeficiency Syndrome (FIS, which manifests as B-lymphocyte immunodeficiency and progressive anemia, is a substantial threat. A significant percentage (∼10% of the Fell ponies born each year dies from FIS, compromising the long-term survival of this breed. Moreover, the likely spread of FIS into other breeds is of major concern. Indeed, FIS was identified in the Dales pony, a related breed, during the course of this work. Using a stepwise approach comprising linkage and homozygosity mapping followed by haplotype analysis, we mapped the mutation using 14 FIS-affected, 17 obligate carriers, and 10 adults of unknown carrier status to a ∼1 Mb region (29.8 - 30.8 Mb on chromosome (ECA 26. A subsequent genome-wide association study identified two SNPs on ECA26 that showed genome-wide significance after Bonferroni correction for multiple testing: BIEC2-692674 at 29.804 Mb and BIEC2-693138 at 32.19 Mb. The associated region spanned 2.6 Mb from ∼29.6 Mb to 32.2 Mb on ECA26. Re-sequencing of this region identified a mutation in the sodium/myo-inositol cotransporter gene (SLC5A3; this causes a P446L substitution in the protein. This gene plays a crucial role in the regulatory response to osmotic stress that is essential in many tissues including lymphoid tissues and during early embryonic development. We propose that the amino acid substitution we identify here alters the function of SLC5A3, leading to erythropoiesis failure and compromise of the immune system. FIS is of significant biological interest as it is unique and is caused by a gene not previously associated with a mammalian disease. Having identified the associated gene, we are now able to eradicate FIS from equine populations by informed selective breeding.

  17. Pulmonary cryptococcosis in rheumatoid arthritis (RA) patients: Comparison of imaging characteristics among RA, acquired immunodeficiency syndrome, and immunocompetent patients

    International Nuclear Information System (INIS)

    Yanagawa, Noriyo; Sakai, Fumikazu; Takemura, Tamiko; Ishikawa, Satoru; Takaki, Yasunobu; Hishima, Tsunekazu; Kamata, Noriko

    2013-01-01

    Purpose: The imaging characteristics of cryptococcosis in rheumatoid arthritis (RA) patients were analyzed by comparing them with those of acquired immunodeficiency syndrome (AIDS) and immunocompetent patients, and the imaging findings were correlated with pathological findings. Methods: Two radiologists retrospectively compared the computed tomographic (CT) findings of 35 episodes of pulmonary cryptococcosis in 31 patients with 3 kinds of underlying states (10 RA, 12 AIDS, 13 immunocompetent), focusing on the nature, number, and distribution of lesions. The pathological findings of 18 patients (8 RA, 2 AIDS, 8 immunocompetent) were analyzed by two pathologists, and then correlated with imaging findings. Results: The frequencies of consolidation and ground glass attenuation (GGA) were significantly higher, and the frequency of peripheral distribution was significantly lower in the RA group than in the immunocompetent group. Peripheral distribution was less common and generalized distribution was more frequent in the RA group than in the AIDS group. The pathological findings of the AIDS and immunocompetent groups reflected their immune status: There was lack of a granuloma reaction in the AIDS group, and a complete granuloma reaction in the immunocompetent group, while the findings of the RA group varied, including a complete granuloma reaction, a loose granuloma reaction and a hyper-immune reaction. Cases with the last two pathologic findings were symptomatic and showed generalized or central distribution on CT. Conclusion: Cryptococcosis in the RA group showed characteristic radiological and pathological findings compared with the other 2 groups

  18. Pulmonary cryptococcosis in rheumatoid arthritis (RA) patients: Comparison of imaging characteristics among RA, acquired immunodeficiency syndrome, and immunocompetent patients

    Energy Technology Data Exchange (ETDEWEB)

    Yanagawa, Noriyo, E-mail: noriyo_yana@ybb.ne.jp [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Sakai, Fumikazu [Department of Diagnostic Radiology, Saitama Medical University International Medical Center, 1397-1 Yamane, Hidaka-shi, Saitama 350-1298 (Japan); Takemura, Tamiko [Department of Pathology, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935 (Japan); Ishikawa, Satoru [Department of Respiratory Medicine, National Hospital Organization Chiba-East-Hospital, 673 Nitona-cho, Chuo-ku, Chiba-shi, Chiba 260-8712 (Japan); Takaki, Yasunobu [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Hishima, Tsunekazu [Department of Pathology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Kamata, Noriko [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan)

    2013-11-01

    Purpose: The imaging characteristics of cryptococcosis in rheumatoid arthritis (RA) patients were analyzed by comparing them with those of acquired immunodeficiency syndrome (AIDS) and immunocompetent patients, and the imaging findings were correlated with pathological findings. Methods: Two radiologists retrospectively compared the computed tomographic (CT) findings of 35 episodes of pulmonary cryptococcosis in 31 patients with 3 kinds of underlying states (10 RA, 12 AIDS, 13 immunocompetent), focusing on the nature, number, and distribution of lesions. The pathological findings of 18 patients (8 RA, 2 AIDS, 8 immunocompetent) were analyzed by two pathologists, and then correlated with imaging findings. Results: The frequencies of consolidation and ground glass attenuation (GGA) were significantly higher, and the frequency of peripheral distribution was significantly lower in the RA group than in the immunocompetent group. Peripheral distribution was less common and generalized distribution was more frequent in the RA group than in the AIDS group. The pathological findings of the AIDS and immunocompetent groups reflected their immune status: There was lack of a granuloma reaction in the AIDS group, and a complete granuloma reaction in the immunocompetent group, while the findings of the RA group varied, including a complete granuloma reaction, a loose granuloma reaction and a hyper-immune reaction. Cases with the last two pathologic findings were symptomatic and showed generalized or central distribution on CT. Conclusion: Cryptococcosis in the RA group showed characteristic radiological and pathological findings compared with the other 2 groups.

  19. High rate of mutation K103N causing resistance to nevirapine in Indian children with acquired immunodeficiency syndrome

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    Sehgal S

    2008-01-01

    Full Text Available In north India the number of paediatric cases with acquired immunodeficiency syndrome (AIDS is on the rise. Most drug combinations used for treatment of AIDS incorporate nevirapine, resistance to which develops very fast if given singly or because of unplanned interruptions. This paper investigates presence of mutations at codon 103 and codon 215 of the HIV pol gene causing resistance to nevirapine and zidovudine (AZT respectively in 25 children with AIDS. Mutations T215Y and K103N were detected by a nested cum amplification refractory mutation system polymerase chain reaction (ARMS PCR and the results were confirmed by direct sequencing in five randomly selected cases. Nineteen patients had received nevirapine containing regimen and six were drug naive. Mutation K103N was observed in 56% (14/25 of the children while mutation T215Y was found in none. Two of the six drug naοve children also showed K103N mutation. Thus, Indian children drug naοve or treated with nevirapine containing regimens show a high rate of mutation conferring resistance to nevirapine which calls for a judicious use of nevirapine both in antenatal and postnatal setting.

  20. Oral manifestations of human immunodeficiency virus/acquired immunodeficiency syndrome and their correlation to cluster of differentiation lymphocyte count in population of North-East India in highly active antiretroviral therapy era

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    Sarat Kumar Nayak

    2016-01-01

    Full Text Available Background: The human immunodeficiency virus (HIV infection which manifests as acquired immunodeficiency syndrome (AIDS is a disease involving the defects of the T-lymphocyte arm of the immune system. Certain laboratory parameters such as the cluster of differentiation (CD4 count and clinical parameters have long been used as markers of disease progression. In industrialized countries, many studies show a highly correlation between the incidence of oral lesions and immunosuppression and hence, can be used as a marker of immunosuppression. This might not be applicable to a developing country like India. In this study, efforts have been made to supplement the present knowledge on various aspects of oral manifestations in HIV patients in the Indian subcontinent. Aims: To correlate the oral manifestations in HIV/AIDS patients to the level of circulating CD4+ T-lymphocyte count and their effect in anti-retroviral therapy (ART. Subjects and Methods: A total of 104 HIV positive patients were examined for oral lesions. The CD4 count estimated on the same day by fluorescent activated cell sort count machine was then correlated with various oral lesions. Results: Oral manifestations appeared when CD4 count decreased below 500 cells/mm3. Moreover, oral lesions found at different stages showed very strong correlation to their respective CD4 count. Furthermore, there was considerable decline in the incidence of oral manifestations in patients undergoing highly active ART. Conclusions: Oral manifestations are highly predictive markers of severe immune deterioration and disease progression in HIV patients.

  1. Acquired immunodeficiency syndrome-related primary cerebral lymphoma: response to irradiation

    International Nuclear Information System (INIS)

    Khoo, V.S.; Wilson, P.C.; Sexton, M.J.; Liew, K.H.

    2000-01-01

    Acquired immunodeficiency syndrome-related primary cerebral lymphoma (AIDS-PCL) is uncommon. Fourteen cases of presumed AIDS-PCL between 1986 and 1995 were reviewed retrospectively in order to characterize the natural history, and the response to radiotherapy. The median age was 38 years (range 24-65). The median interval between seropositive diagnosis of HIV and AIDS-PCL was 28 months (range 5-113). The median duration of symptoms was 2 weeks (range 0.2-12). At presentation, the Eastern Cooperative Oncology Group performance status (PS) was PS1 (2/14 patients), PS2 (6/14) and PS3 (6/14). The symptoms and signs were non-specific and depended on the site and extent of cerebral involvement. There was no characteristic pattern of brain imaging in terms of size, number, location or pattern of contrast enhancement of the cerebral lesions. Nine patients received various fractionation-dose schedules (range 8-50 Gy). Complete and partial responses were seen in 2/9 and 3/9 cases, respectively. Clinical stabilization of neurological symptoms was noted in 3/9 cases and disease progression in 1/9. The median survival times (MST) from presentation for irradiated and non-irradiated patients were 9.3 and 2.1 weeks, respectively (range 0.9-43.1). Although patient selection introduced bias, there appears to be a modest improvement in MST for treated patients. The MST with radiotherapy alone remains poor, but radiotherapy may provide palliation. For some selected patients, a prolonged response is possible. Copyright (1999) Blackwell Science Pty Ltd

  2. Changing patterns of Kaposi's sarcoma in Danish acquired immunodeficiency syndrome patients with complete follow-up. The Danish Study Group for HIV Infection (DASHI)

    DEFF Research Database (Denmark)

    Lundgren, Jens Dilling; Melbye, M; Pedersen, C

    1995-01-01

    The objective was to study changes in the occurrence of human immunodeficiency virus type 1-related Kaposi's sarcoma and the association with degree of immunodeficiency over time. Danish patients with acquired immunodeficiency syndrome (AIDS) diagnosed between 1979 and 1990 (n = 687) were followed...... the proportion of patients who died with Kaposi's sarcoma remained constant over time. Furthermore, the CD4 cell count at time of AIDS for patients diagnosed with Kaposi's sarcoma has declined in recent years. A CD4 cell count liter at the time of AIDS diagnosis predicted an increased risk...

  3. Cardiac abnormalities in the acquired immunodeficiency syndrome. A prospective study with a clinical-pathological correlation in twenty-one adult patients

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    Herdy Gesmar Volga Haddad

    1999-01-01

    Full Text Available OBJECTIVE - To evaluate the cardiac abnormalities and their evolution during the course of the acquired immunodeficiency syndrome, as well as to correlate clinical and pathological data. METHODS - Twenty-one patients, admitted to the hospital with the diagnosis of acquired immunodeficiency syndrome, were prospectively studied and followed until their death. Age ranged from 19 to 42 years (17 males. ECG and echocardiogram were also obtained every six months. After death, macro- and microscopic examinations were also performed. RESULTS - The most frequent causes of referral to the hospital were: diarrhea or repeated pneumonias, tuberculosis, toxoplasmosis or Kaposi sarcoma. The most frequent findings were acute or chronic pericarditis (42% and dilated cardiomyopathy (19%. Four patients died of cardiac problems: infective endocarditis, pericarditis with pericardial effusion, bacterial myocarditis and infection by Toxoplasma gondii. CONCLUSION - Severe cardiac abnormalities were the cause of death in some patients. In the majority of the patients, a good correlation existed between clinical and anatomical-pathological data. Cardiac evaluation was important to detect early manifestations and treat them accordingly, even in asymptomatic patients.

  4. Molecular analysis of T-B-NK+ severe combined immunodeficiency and Omenn syndrome cases in Saudi Arabia

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    Al-Kayal Fadi

    2009-11-01

    Full Text Available Abstract Background Children with Severe Combined Immunodeficiency (SCID lack autologous T lymphocytes and present with multiple infections early in infancy. Omenn syndrome is characterized by the sole emergence of oligoclonal auto-reactive T lymphocytes, resulting in erythroderma and enteropathy. Omenn syndrome (OS shares the genetic aetiology of T-B-NK+ SCID, with mutations in RAG1, RAG2, or DCLRE1C. Methods Patients diagnosed with T-B-NK+ SCID or phenotypes suggestive of Omenn syndrome were investigated by molecular genetic studies using gene tightly linked microsatellite markers followed by direct sequencing of the coding regions and splice sites of the respective candidate genes. Results We report the molecular genetic basis of T-B-NK+ SCID in 22 patients and of OS in seven patients all of Arab descent from Saudi Arabia. Among the SCID patients, six (from four families displayed four homozygous missense mutations in RAG1 including V433M, R624H, R394W, and R559S. Another four patients (from three familes showed 3 novel homozygous RAG2 mutations including K127X, S18X, and Q4X; all of which predict unique premature truncations of RAG2 protein. Among Omenn patients, four (from two families have S401P and R396H mutations in RAG1, and a fifth patient has a novel I444M mutation in RAG2. Seven other patients (six SCID and one OS showed a gross deletion in exons 1-3 in DCLRE1C. Altogether, mutations in RAG1/2 and DCLRE1C account for around 50% and 25%, respectively, in our study cohort, a proportion much higher than in previous reported series. Seven (24% patients lack a known genetic aetiology, strongly suggesting that they carry mutations in novel genes associated with SCID and Omenn disorders that are yet to be discovered in the Saudi population. Conclusion Mutation-free patients who lack a known genetic aetiology are likely to carry mutations in the regulatory elements in the SCID-causing genes or in novel genes that are yet to be discovered

  5. Production of acquired immunodeficiency syndrome-associated retrovirus in human and nonhuman cells transfected with an infectious molecular clone

    International Nuclear Information System (INIS)

    Adachi, A.; Gendelman, H.E.; Koenig, S.; Folks, T.; Willey, R.; Rabson, A.; Martin, M.A.

    1986-01-01

    The authors considered an infectious molecular clone of acquired immunodeficiency syndrome-associated retrovirus. Upon transfection, this clone directed the production of infectious virus particles in a wide variety of cells in addition to human T4 cells. The progeny, infectious virions, were synthesized in mouse, mink, monkey, and several human non-T cell lines, indicating the absence of any intracellular obstacle to viral RNA or protein production or assembly. During the course of these studies, a human colon carcinoma cell line, exquisitely sensitive to DNA transfection, was identified

  6. A controlled study of funding for human immunodeficiency virus/acquired immunodeficiency syndrome as resource capacity building in the health system in Rwanda.

    Science.gov (United States)

    Shepard, Donald S; Zeng, Wu; Amico, Peter; Rwiyereka, Angelique K; Avila-Figueroa, Carlos

    2012-05-01

    Because human inmmunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) receives more donor funding globally than that for all other diseases combined, some critics allege this support undermines general health care. This empirical study evaluates the impact of HIV/AIDS funding on the primary health care system in Rwanda. Using a quasi-experimental design, we randomly selected 25 rural health centers (HCs) that started comprehensive HIV/AIDS services from 2002 through 2006 as the intervention group. Matched HCs with no HIV/AIDS services formed the control group. The analysis compared growth in inputs and services between intervention and control HCs with a difference-in-difference analysis in a random-effects model. Intervention HCs performed better than control HCs in most services (seven of nine), although only one of these improvements (Bacille Calmette-Guérin vaccination) reached or approached statistical significance. In conclusion, this six-year controlled study found no adverse effects of the expansion of HIV/AIDS services on non-HIV services among rural health centers in Rwanda.

  7. Epidemiology and clinical parameters of adult human immunodeficiency virus/acquired immunodeficiency syndrome at the initiation of antiretroviral therapy in South eastern Nigeria.

    Science.gov (United States)

    Eleje, Gu; Ele, Pu; Okocha, Ec; Iloduba, Uc

    2014-03-01

    Human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) has continued to ravage the teeming populations in Nigeria, with disastrous consequences. Despite many studies and progress on HIV/AIDS in Africa, the data on the status of the patients at the commencement of therapy is lacking. The aim of this study is to determine the demographic, clinical and some laboratory features of adult HIV/AIDS patients, seen at the commencement of antiretroviral therapy (ART) in Nnamdi Azikiwe University Teaching Hospital, Nnewi, south-east Nigeria between July 2002 and October 2004. The study was a cross-sectional, descriptive study. Adult patients living with HIV/AIDS were studied using an interview administered questionnaire. Data was analyzed using Epi Info 2008 version 3.5.1. A total of 400 respondents participated in this study. The mean age was 36.8 (8.8) years. Almost 60% patients were married and the HIV concordance rate was 53.3% (136/255). Nearly 30% of the families had at least one child positive for HIV. The most common associated risky behavior was injection administered in patent medicine stores 74.5%(302/400) and the most common clinical symptom was respiratory. Of the 400 patients recruited in this study, 19 (4.8%) were lost to follow-up on the 6 months' visit, giving a follow-up rate of 95.2% (381/400). There was statistically significant difference in the mean body weight (P = 0.02), mean total white blood cell count (P < 0.001) and mean CD4(+) count (P < 0.001) at presentation and after 6 months of ART therapy. HIV/AIDS patients present late and body weight, CD4(+) count and total white blood cell count seemed to recover quickly on commencement of ART. The prevalence of concordance among couples and mother to child transmission rates tended to be high. Administration of injectable at patent medicine stores and multiple sexual partners are the most significant risk factors.

  8. Contribution of the human immunodeficiency virus/acquired immunodeficiency syndrome epidemic to de novo presentations of heart disease in the Heart of Soweto Study cohort.

    Science.gov (United States)

    Sliwa, Karen; Carrington, Melinda J; Becker, Anthony; Thienemann, Friedrich; Ntsekhe, Mpiko; Stewart, Simon

    2012-04-01

    The contemporary impact of the human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) epidemic on heart disease in South Africa (>5 million people affected) is unknown. The Heart of Soweto Study provides a unique opportunity to identify the contribution of cardiac manifestations of this epidemic to de novo presentations of heart disease in an urban African community in epidemiological transition. Chris Hani Baragwanath Hospital services the >1 million people living in Soweto, South Africa. A prospective, clinical registry captured data from all de novo cases of heart disease presenting to the Cardiology Unit during 2006-08. We describe all cases where HIV/AIDS was concurrently diagnosed. Overall, 518 of 5328 de novo cases of heart disease were identified as HIV-positive (9.7%) with 54% of these prescribed highly active anti-retroviral therapies on presentation. Women (62%) and Africans (97%) predominated with women being significantly younger than men 38 ± 13 vs. 42 ± 13 years (P = 0.002). The most common primary diagnosis attributable to HIV/AIDS was HIV-related cardiomyopathy (196 cases, 38%); being prescribed more anti-retroviral therapy (127/196 vs. 147/322; odds ratio 2.85, 95% confidence interval 1.81-3.88) with higher viral loads [median 110 000 (inter-quartile range 26 000-510 000) vs. 19 000 (3200-87 000); P = 0.018] and a lower CD4 count [median 180 (71-315) vs. 211 (96-391); P = 0.019] than the rest. An additional 128 cases (25%) were diagnosed with pericarditis/pericardial effusion with a range of other concurrent diagnoses evident, including 42 cases (8.1%) of HIV-related pulmonary arterial hypertension. Only 14 of all 581 cases of coronary artery disease (CAD) (2.4%, mean age 41 ± 13 years) were confirmed HIV-positive. Cardiac manifestations of HIV/AIDS identified within this cohort were relatively infrequent. While HIV-related cardiomyopathy and pericardial disease remain important targets for early detection and treatment in

  9. Magnetic resonance imaging: early detection of central nervous system involvement in acquired immunodeficiency syndrome (AIDS)

    International Nuclear Information System (INIS)

    Trotot, P.M.; Sansonetti, P.J.; Levillain, R.; Cabanis, E.A.; Lavayssiere, R.; Sandoz-Tronca, C.

    1988-01-01

    Central Nervous System (CNS) involvement, whether primary by the Human Immunodeficiency Virus - HIV - itself, or secondary (toxoplasmosis or lymphoma) is remarkably frequent in AIDS, in 40 to 70% of cases, depending upon the author. In order to study the natural history of this illness, a cohort of 25 asymptomatic seropositive patients have been established. Every 6 months these patients undergo biological and clinical examinations, as well as Magnetic Resonance brain scans. After two examinations at a 6 month's interval, the first results are reported. Out of these 25 cases, 9 present anomalies: One patient with diffuse cerebral atrophy and 8 others with high signal intensity areas on T2 weighted sequences, like those of the Multiple Sclerosis. No relationship could be demonstrated between the existence of these lesions and various criteria such as age, sex, risk factors and T4 cells count. The nature of these lesions is not lear. They certainly indicate early involvement of the CNS after primary infection by the HIV virus. They may either represent scars of the primary infection or early alterations announcing developing encephalopathy [fr

  10. X-ray and CT findings of soft tissue and bone infections secondary to acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Jiang Songfeng; Liu Jinxin; Chen Bihua; Zhang Lieguang; Gan Qingxin; Huang Deyang

    2011-01-01

    Objective: To summarize X-ray and CT findings of soft tissue and bone infections secondary to acquired immunodeficiency syndrome (AIDS). Methods: The data of X-ray and CT findings of soft tissue and bone infections in 18 patients with AIDS were retrospectively collected and analyzed. Results: Of 18 patients with AIDS, the CT features of soft tissue demonstrated that subcutaneous patchy high density in 1 case which considered as cellulitis, round low density lesions with ring enhancement in 6 cases which considered as soft tissue abscesses, heterogeneous density lesions with peripheral enhancement in 1 case which considered as pyomyositis. Of 18 patients with AIDS, septic arthritis was found in 4 cases involving knee lesion in 3 cases and hip lesion. In the 4 case, the X-ray films showed bony destruction in 2 cases and the CT showed bone destruction in 3 cases and arthroedema in 4 cases. Of 18 patients with AIDS, osteomyelitis was found in 9 cases of which tuberculosis was considered in. 8 cases and vertebral involvement in 6 cases. In the 9 cases, the X-ray films and CT displayed bony destruction, hyperostosis, small sequestra, and intervertebral space narrowing. Of 18 patients with AIDS, costal lesions were found in 3 cases in which the CT showed expandable bony destruction. Of 18 patients with AIDS, ilium and sacroiliac joint lesions were found in 1 case in which the X-ray films and CT showed bony destruction, sequestra, and joint widening. Of 18 patients with AIDS, chronic pyogenic osteomyelitis of femur was found in 1 case in which the X-ray films showed bony destruction, hyperostosis osteosclerosis, and periosteal reaction. Conclusion: The X-ray and CT features of soft tissue and bone infections secondary to AIDS are characterized. The X-ray and CT are useful tools to early diagnose soft tissue and bone infections secondary to AIDS. (authors)

  11. Risky sexual behaviour and human immunodeficiency virus (HIV and acquired immune deficiency syndrome (AIDS among healthcare workers

    Directory of Open Access Journals (Sweden)

    Natasha Khamisa

    2018-01-01

    Full Text Available Background: South Africa is known to have one of the highest prevalence rates of human immunodeficiency virus (HIV and acquired immune deficiency syndrome (AIDS globally, with one in seven healthcare workers being HIV-positive. An HIV-positive healthcare workforce is less equipped to respond to the increasing spread of the epidemic. Objectives: Assessment of the factors contributing to high HIV prevalence rates among healthcare workers is important in planning the development of human resources. This review sought to identify and understand predominant risky sexual behaviours among healthcare workers in HIV and AIDS-affected countries. Methods: This study reviewed articles focusing on sexual behaviour among healthcare workers. Major health science databases (e.g. ProQuest, Cochrane, PubMed and CINAHL were searched for combinations of keywords including ‘healthcare workers’, ‘risky sexual behaviour’ and ‘HIV and AIDS’. Articles from a range of countries met inclusion and exclusion criteria. Results: Findings of the study revealed three main contributing factors: unprotected sex, multiple sex partners and sexual violence. Sexual violence emerged as the dominant risk factor in the majority of the studies. Most research was conducted in developed countries where the HIV infection rate is much lower than it is in developing countries. Conclusion: More research needs to be conducted in developing countries and appropriate strategies should be implemented to reduce sexual violence among healthcare workers. Appropriate procedures on reporting sexual violence coupled with education on HIV and AIDS as well as influencing attitudes and belief systems could assist in reducing the spread of HIV and AIDS within the healthcare workforce while minimising the effect on patient care.

  12. Novel ZBTB24 Mutation Associated with Immunodeficiency, Centromere Instability, and Facial Anomalies Type-2 Syndrome Identified in a Patient with Very Early Onset Inflammatory Bowel Disease.

    Science.gov (United States)

    Conrad, Máire A; Dawany, Noor; Sullivan, Kathleen E; Devoto, Marcella; Kelsen, Judith R

    2017-12-01

    Very early onset inflammatory bowel disease, diagnosed in children ≤5 years old, can be the initial presentation of some primary immunodeficiencies. In this study, we describe a 17-month-old boy with recurrent infections, growth failure, facial anomalies, and inflammatory bowel disease. Immune evaluation, whole-exome sequencing, karyotyping, and methylation array were performed to evaluate the child's constellation of symptoms and examination findings. Whole-exome sequencing revealed that the child was homozygous for a novel variant in ZBTB24, the gene associated with immunodeficiency, centromere instability, and facial anomalies type-2 syndrome. This describes the first case of inflammatory bowel disease associated with immunodeficiency, centromere instability, and facial anomalies type-2 syndrome in a child with a novel disease-causing mutation in ZBTB24 found on whole-exome sequencing.

  13. Clinical characteristics of abnormal savda syndrome type in human immunodeficiency virus infection and acquired immune deficiency syndrome patients: A cross-sectional investigation in Xinjiang, China.

    Science.gov (United States)

    Peierdun, Mi-ji-ti; Liu, Wen-xian; Renaguli, Ai-ze-zi; Nurmuhammat, Amat; Li, Xiao-chun; Gulibaier, Ka-ha-er; Ainivaer, Wu-la-mu; Halmurat, Upur

    2015-12-01

    To investigate the distribution of abnormal hilit syndromes in traditional Uighur medicine (TUM) among human immunodeficiency virus infection and acquired immune deficiency syndrome (HIV/AIDS) patients, and to find out the clinical characteristics of abnormal savda syndrome type HIV/AIDS patients. Between June and July in 2012, 307 eligible HIV/AIDS patients from in-patient department and out-patient clinics of Xinjiang Uighur Autonomous Region the Sixth People's Hospital in Urumqi were investigated. TUM syndrome differentiation was performed by a senior TUM physician. Each participant completed a Sign and Symptom Check-List for Persons Living with HIV/AIDS (SSC-HIV) questionnaire. Depression was evaluated by using Hamilton Rating Scale for Depression Questionnaire. Blood specimen was collected from each participant to test the levels of blood chemicals. Of 307 HIV/AIDS patients, 189 (61.6%) were abnormal savda syndrome type, 118 (38.4%) were non-abnormal-savda syndrome type. Mean CD4 counts of abnormal savda syndrome type patients was (227.61±192.93) cells/µL, and the prevalence of anemia, thrombocytopenia, and elevated cystatin C were 49.7%, 28.6%, and 44.7%, which were significantly higher than those in the non-abnormal-savda syndrome type patients (26.3%, 16.0% and 25.0%,PHIV/AIDS-related symptoms such as fatigue (42.3%), back aches (40.7%), lack of appetite (33.9%), night sweats (31.7%) were more common among abnormal savda syndrome patients (PHIV/AIDS patients, and they present a more sever clinical manifestation.

  14. CXCL12/CXCR4-Axis Dysfunctions: Markers of the Rare Immunodeficiency Disorder WHIM Syndrome

    Directory of Open Access Journals (Sweden)

    Françoise Bachelerie

    2010-01-01

    Full Text Available The WHIM syndrome features susceptibility to human Papillomavirus infection-induced warts and carcinomas, hypogammaglobulinemia, recurrent bacterial infections, B and T-cell lymphopenia, and neutropenia associated with retention of senescent neutrophils in the bone marrow (i.e. myelokathexis. This rare disorder is mostly linked to inherited heterozygous autosomal dominant mutations in the gene encoding CXCR4, a G protein coupled receptor with a unique ligand, the chemokine CXCL12/SDF-1. Some individuals who have full clinical forms of the syndrome carry a wild type CXCR4 gene. In spite of this genetic heterogeneity, leukocytes from WHIM patients share in common dysfunctions of the CXCR4-mediated signaling pathway upon exposure to CXCL12. Dysfunctions are characterized by impaired desensitization and receptor internalization, which are associated with enhanced responses to the chemokine. Our increasing understanding of the mechanisms that account for the aberrant CXCL12/CXCR4-mediated responses is beginning to provide insight into the pathogenesis of the disorder. As a result we can expect to identify markers of the WHIM syndrome, as well as other disorders with WHIM-like features that are associated with dysfunctions of the CXCL12/CXCR4 axis.

  15. Hashimoto's Thyroiditis Presenting as Acute Painful Thyroiditis and as a Manifestation of an Immune Reconstitution Inflammatory Syndrome in a Human Immunodeficiency Virus-Seropositive Patient.

    NARCIS (Netherlands)

    Visser, R.; Mast, Q. de; Netea-Maier, R.T.; Ven, A.J.A.M. van der

    2012-01-01

    Background: An immune reconstitution inflammatory syndrome (IRIS) may complicate immune restoration following start of antiretroviral therapy (ART) in human immunodeficiency virus (HIV)-infected patients. The occurrence of Graves' disease in the setting of an IRIS is well recognized. We hereby

  16. Reproductive aspects and knowledge of family planning among women with Acquired Immunodeficiency Syndrome.

    Science.gov (United States)

    Lima, Ivana Cristina Vieira de; Cunha, Maria da Conceição Dos Santos Oliveira; Cunha, Gilmara Holanda da; Galvão, Marli Teresinha Gimeniz

    2017-05-25

    To analyze the reproductive aspects and knowledge of family planning among women with Acquired Immunodeficiency Syndrome (AIDS). Cross-sectional and descriptive study carried out from January to December, 2015, in the outpatient care of infectious disease unit in a hospital located in Fortaleza, Ceará. Data were collected through a form applied by interview in a private setting. 102 women participated in the study. Most were aware that they were serologically positive with human immunodeficiency virus (HIV) during prenatal care (96.1%) and did not intend to have more children (63.7%). Women who were less than 39 years of age, had a higher educational level, and a shorter time of antiretroviral therapy had better chances of having children (p≤0.05). Having a steady partner increased the chance of desiring to have children, while tubal ligation was higher among women that did not receive counseling on family planning. Knowledge of family planning was limited because of lack of assistance provided by health professionals. Analisar aspectos reprodutivos e conhecimento sobre planejamento familiar de mulheres com síndrome da imunodeficiência adquirida (Aids). Estudo transversal, descritivo, realizado de janeiro a dezembro de 2015, no ambulatório de infectologia de um hospital em Fortaleza, Ceará. Os dados foram coletados por meio de formulário, aplicado por entrevista em ambiente privativo. Participaram do estudo 102 mulheres. A maioria delas teve conhecimento da sorologia positiva para vírus da imunodeficiência humana (HIV) durante o pré-natal (96,1%), e estas não pretendiam mais ter filhos (63,7%). Mulheres com idade menor que 39 anos, maior escolaridade e menor tempo de terapia antirretroviral tiveram maiores chances de ter filhos (p≤0,05). Mulheres com idade menor que 39 anos e maior escolaridade tiveram maiores chances de ter informações corretas sobre ter filhos na vigência do HIV (p≤0,05). Ter parceiro fixo aumentou a chance de desejar ter

  17. The Role of Faith-Based Organizations in the Education, Support, and Services for Persons Living with Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome.

    Science.gov (United States)

    Stephens, Teresa M

    2018-03-01

    Faith-based organizations are in a unique position to provide resilience-enhancing efforts for persons living with human immunodeficiency virus/AIDS. Many persons living with human immunodeficiency virus/AIDS report having a strong faith or religious affiliation, with a large percentage attending church services on a regular basis. Faith-based organizations can use these factors to reach out to these individuals and effectively promote health, well-being, education, and support. Faith-based organizations can contribute to the reduction of stigma and isolation for persons living with human immunodeficiency virus/AIDS. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. MR findings of central nervous system involvement in acquired immunodeficiency syndrome patient : a report of two cases

    International Nuclear Information System (INIS)

    Hong, Hye Suk; Kim, Dong Ik; Lee, Byeong Hee; Jeong, Sun Yang

    1996-01-01

    Central nervous system (CNS) manifestations in acquired immunodeficiency syndrome (AIDS) patients are an early and common feature. The spectrum of AIDS-related CNS diseases are encephalitis caused by the human immunodeficiency virus(HIV) itself, opportunistic infection, infarct and malignancy. We experienced two cases of CNS involvement in AIDS and they were serologically diagnosed as HIV encephalitis and CNS toxoplasmosis, respectively. In the case of the HIV encephalitis patient, brain MRI showed a non-enhancing lesion with high signal intensity on T2WI and low signal on T1WI and there was no mass effect on the right frontal lobe, periventricular white matter, splenium of the corpus callosum or bilateral basal ganglia. In the other case of CNS toxoplasmosis, MR showed multiple nodular and rim enhanced mass lesions in the right basal ganglia, thalamus and periventricular white matter, which were of low signal intensity on T1WI and of high intensity on T2WI. We thus report the related MRI findings

  19. Diffuse large B-cell lymphoma chemotherapy reveals a combined immunodeficiency syndrome in cartilage hair hypoplasia.

    Science.gov (United States)

    Nguyen, Alexandre; Martin Silva, Nicolas; de Boysson, Hubert; Damaj, Gandhi; Aouba, Achille

    2018-04-24

    Cartilage hair hypoplasia (CHH) is a rare autosomal recessive ribosomopathy characterised by skeletal and integumentary system manifestations. It may also present with varied forms and intensities of haematopoietic and/or immune disorders. We report a 27-year-old female who presented a picture of combined immunodeficiency after receiving an adriamycin-based chemotherapy regimen followed by autologous stem cell transplantation. Her medical history indicated neonatal dwarfism, recurrent ear, nose and throat and respiratory infections, and hypogammaglobulinaemia, which were suggestive of a primary minor B-cell immune deficiency. Taken together, the diagnosis of cartilage hair hypoplasia was suspected and confirmed by means of molecular biological analysis. Here, we discuss the causal relationship and molecular mechanisms existing between both primary immunodeficiency and lymphoma conditions and between chemotherapy cytotoxicity and aggravation of the immune system and associated hematopoietic dysfunction, considering the role of all these components in light of the initially undiagnosed cartilage hair hypoplasia. Finally, this case highlights the importance of screening for primary immunodeficiencies in the setting of a diagnosis of lymphoma and/or dwarfism; moreover, CHH must be distinguished from other causes of small size; its diagnosis and complete check-up must include the molecular characterisation, and its management must be global in collaboration with haematologists, immunologists and internists.

  20. Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome

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    Kwadwo Kyeremanteng

    2016-01-01

    Full Text Available Objective. To describe a single case of Systemic Capillary Leak Syndrome (SCLS with a rare complication of compartment syndrome. Patient. Our patient is a 57-year-old male, referred to our hospital due to polycythemia (hemoglobin (Hgb of 220 g/L, hypotension, acute renal failure, and bilateral calf pain. Measurements and Main Results. The patient required bilateral forearm, thigh, and calf fasciotomies during his ICU stay and continuous renal replacement therapy was instituted following onset of acute renal failure and oliguria. Ongoing hemodynamic (Norepinephrine and Milrinone infusion and respiratory (ventilator support in the ICU was provided until resolution of intravascular fluid extravasation. Conclusions. SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space. Patients present with significant hypotension, hemoconcentration, hypovolemia, and oliguria. Severe edema results from leakage of fluid and proteins into tissue. The most important part of treatment is maintaining stable hemodynamics, ruling out other causes of shock and diligent monitoring for complications. Awareness of the clinical syndrome with the rare complication of compartment syndrome may help guide investigations and diagnoses of these critically ill patients.

  1. Clinical study of acquired immunodeficiency syndrome in domestic cats in São Paulo

    Directory of Open Access Journals (Sweden)

    Archivaldo Reche Jr.

    1997-06-01

    Full Text Available In order to study the magnitude of distribution of feline leukemia virus (FLV and feline immunodeficiency virus (FIV among domestic cats in São Paulo, 401 animals from both sexes, different ages and breeds, were tested for antibodies (FIV and viral soluble antigens (FLV by means of ELISA (feline leukemia virus antigen / feline immunodeficiency virus antibody - CITE ® - Agrytech Sistems Inc.. Among these animals, 123 were healthy cats and 278 were patients at the Department of Medical Clinics / Veterinary Hospital of Faculdade de Medicina Veterinária e Zootecnia, Universidade de São Paulo due to various diseases eight (6,5% FIV positive cats and two (1,6% FLV positive cats were found among healthy animals in opposition to 39 (14% and 30 (10,8% sick cats regents to FIV and FLV antigens and antibodies, respectively. All animals but one presented single infection. FIV infection was four times more frequent among males when compared to females; nevertheless, no difference was found related to FVL infection. Opportunistic infections as those caused by Hemobartonella felis were the most common baseline disease found among FIV or FLV infected cats. When tumors, were considered the mediastinal lymphoma was the most frequent type found among FVL infected cats. A variety of other diseases was observed, associated to both retroviruses infection. The mean age of FIV infect animals was 4,4 + 3,0 years old and 2,4 ± 1,7 years old FLV infected cats. All infected, symptomatic animals died during the two years of  observation, while all healthy, infected cats survived, allowing the conclusion that period of latency post-infection may be long.

  2. An unusual case of primary human immunodeficiency virus infection presenting as mononucleosis-like syndrome and acute aseptic meningoencephalitis. Report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    Marcelo Corti

    2014-01-01

    Full Text Available Clinical presentation of primary human immunodeficiency virus (HIV infection includes a wide spectrum of manifestations from asymptomatic infection to a symptomatic and severe illness. Central nervous system involvement should be always considered as a severe clinical form of primary HIV infection. Physicians should be aware to the broad clinical spectrum of primary HIV infection. We report a case of a female with diagnosis of mononucleosis-like syndrome and acute aseptic meningoencephalitis during primary HIV infection.

  3. Natural killer cell biology illuminated by primary immunodeficiency syndromes in humans.

    Science.gov (United States)

    Voss, Matthias; Bryceson, Yenan T

    2017-04-01

    Natural killer (NK) cells are innate immune cytotoxic effector cells well known for their role in antiviral immunity and tumor immunosurveillance. In parts, this knowledge stems from rare inherited immunodeficiency disorders in humans that abrogate NK cell function leading to immune impairments, most notably associated with a high susceptibility to viral infections. Phenotypically, these disorders range from deficiencies selectively affecting NK cells to complex general immune defects that affect NK cells but also other immune cell subsets. Moreover, deficiencies may be associated with reduced NK cell numbers or rather impair specific NK cell effector functions. In recent years, genetic defects underlying the various NK cell deficiencies have been uncovered and have triggered investigative efforts to decipher the molecular mechanisms underlying these disorders. Here we review the associations between inherited human diseases and NK cell development as well as function, with a particular focus on defects in NK cell exocytosis and cytotoxicity. Furthermore we outline how reports of diverse genetic defects have shaped our understanding of NK cell biology. Copyright © 2015. Published by Elsevier Inc.

  4. Clinical, endoscopic and prognostic aspects of primary gastric non-hodgkin's lymphoma associated with acquired immunodeficiency syndrome

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    Rosamar Eulira Fontes Rezende

    Full Text Available Primary gastric non-Hodgkin's lymphoma (NHL is a co-morbidity that can be observed during the clinical course of acquired immunodeficiency syndrome (AIDS. We evaluated the prevalence, clinical-evolutive aspects and form of endoscopic presentation of primary gastric NHL associated with AIDS. Two hundred and forty-three HIV patients were submitted to upper digestive endoscopy, with evaluation of clinical, endoscopic and histological data. A CD4 count was made by flow cytometry and viral load was determined in a branched-DNA assay. Six cases (five men; mean age: 37 years; range: 29-46 years of primary gastric NHL were detected. The median CD4 count was 140 cells/mm³ and the median viral load was 40,313 copies/mL. Upper digestive endoscopy revealed polypoid (in four patients ulcero-infiltrative (two patients and ulcerated (two patients lesions and combined polypoid and ulcerated lesions (two patients. Histology of the gastric lesions demonstrated B cell NHL (four patients and T cell NHL (two patients. Five of the six patients died of complications related to gastric NHL. We concluded that primary gastric NHL is an important cause of mortality associated with AIDS.

  5. Patients with an inherited syndrome characterized by immunodeficiency, microcephaly, and chromosomal instability: genetic relationship to ataxia telangiectasia

    International Nuclear Information System (INIS)

    Jaspers, N.G.; Taalman, R.D.; Baan, C.

    1988-01-01

    Fibroblast cultures from six unrelated patients having a familial type of immunodeficiency combined with microcephaly, developmental delay, and chromosomal instability were studied with respect to their response to ionizing radiation. The cells from five of them resembled those from individuals with ataxia telangiectasia (AT) in that they were two to three times more radiosensitive on the basis of clonogenic cell survival. In addition, after exposure to either X-rays or bleomycin, they showed an inhibition of DNA replication that was less pronounced than that in normal cells and characteristic of AT fibroblasts. However, the patients are clinically very different from AT patients, not showing any signs of neurocutaneous symptoms. Genetic complementation studies in fused cells, with the radioresistant DNA synthesis used as a marker, showed that the patients' cells could complement representatives of all presently known AT complementation groups. Furthermore, they were shown to constitute a genetically heterogeneous group as well. It is concluded that these patients are similar to AT patients with respect to cytological parameters. The clinical differences between these patients and AT patients are a reflection of genetic heterogeneity. The data indicate that the patients suffer from a chromosome-instability syndrome that is distinct from AT

  6. Radiation therapy for Kaposi's sarcoma associated with acquired immunodeficiency syndrome. Tokyo Metropolitan Komagome Hospital experience

    Energy Technology Data Exchange (ETDEWEB)

    Ebara, Takeshi [Municipal Kanbara General Hospital, Fujikawa, Shizuoka (Japan); Karasawa, Katsuyuki; Maebayashi, Katsuya; Kurosaki, Hiromasa; Ishikawa, Hitoshi; Kaizu, Toshihide; Tanaka, Yoshiaki; Akagi, Kumiko; Masuda, Gota

    2000-12-01

    Kaposi's sarcoma is frequently found in association with acquired immunodeficiency syndrome (AIDS). We report on radiotherapy for patients with AIDS-related Kaposi's sarcoma at Tokyo Metropolitan Komagome Hospital. Between April 1991 and May 1997, radiotherapy was given to 11 lesions in eight men with AIDS-related Kaposi's sarcoma to relieve their symptoms. The lesions involved the head and neck region, the legs, and the gastrointestinal tract. Radiotherapy was carried out with 4-MV photon through parallel opposed field or high energy electrons. Total doses ranged from 20 to 38 Gy, with a median of 30 Gy, delivered in 2- to 3-Gy fractions. Four patients were given other treatments prior to the radiotherapy. Acute reaction was evaluated according to the modified acute radiation morbidity scoring criteria of the Radiation Therapy Oncology Group (RTOG). Radiotherapy had relieved the symptoms in all patients at completion of this therapy. Lesions that involved the hard palate and vocal cords had completely disappeared. The lesions that received radiotherapy were controlled without symptoms until the patients died. Patients who had the head and neck region treated exhibited severe acute mucosal reaction (at a dose of 30 Gy, there was grade 2 morbidity by modified RTOG criteria, in two patients, and grade 3 in three patients) although the radiation therapy was completed for these patients. Radiotherapy promises a favorable outcome for symptom relief in AIDS-related Kaposi's sarcoma. (author)

  7. Disease Evolution and Response to Rapamycin in Activated Phosphoinositide 3-Kinase δ Syndrome: The European Society for Immunodeficiencies-Activated Phosphoinositide 3-Kinase δ Syndrome Registry

    Directory of Open Access Journals (Sweden)

    Maria Elena Maccari

    2018-03-01

    Full Text Available Activated phosphoinositide 3-kinase (PI3K δ Syndrome (APDS, caused by autosomal dominant mutations in PIK3CD (APDS1 or PIK3R1 (APDS2, is a heterogeneous primary immunodeficiency. While initial cohort-descriptions summarized the spectrum of clinical and immunological manifestations, questions about long-term disease evolution and response to therapy remain. The prospective European Society for Immunodeficiencies (ESID-APDS registry aims to characterize the disease course, identify outcome predictors, and evaluate treatment responses. So far, 77 patients have been recruited (51 APDS1, 26 APDS2. Analysis of disease evolution in the first 68 patients pinpoints the early occurrence of recurrent respiratory infections followed by chronic lymphoproliferation, gastrointestinal manifestations, and cytopenias. Although most manifestations occur by age 15, adult-onset and asymptomatic courses were documented. Bronchiectasis was observed in 24/40 APDS1 patients who received a CT-scan compared with 4/15 APDS2 patients. By age 20, half of the patients had received at least one immunosuppressant, but 2–3 lines of immunosuppressive therapy were not unusual before age 10. Response to rapamycin was rated by physician visual analog scale as good in 10, moderate in 9, and poor in 7. Lymphoproliferation showed the best response (8 complete, 11 partial, 6 no remission, while bowel inflammation (3 complete, 3 partial, 9 no remission and cytopenia (3 complete, 2 partial, 9 no remission responded less well. Hence, non-lymphoproliferative manifestations should be a key target for novel therapies. This report from the ESID-APDS registry provides comprehensive baseline documentation for a growing cohort that will be followed prospectively to establish prognostic factors and identify patients for treatment studies.

  8. A new frailty syndrome: central obesity and frailty in older adults with the human immunodeficiency virus.

    Science.gov (United States)

    Shah, Krupa; Hilton, Tiffany N; Myers, Lauren; Pinto, Jonathan F; Luque, Amneris E; Hall, William J

    2012-03-01

    To evaluate the relationships between body composition and physical frailty in community-dwelling older adults with the human immunodeficiency virus (HIV) (HOA). Cross-sectional. Academic hospital-based infectious disease clinic in Rochester, New York. Forty community-dwelling HOA aged 50 and older undergoing antiretroviral therapy who were able to ambulate without assistive devices with a mean age of 58, a mean BMI of 29.0 kg/m(2), mean CD4 count of 569 cells/mL, and a mean duration since HIV diagnosis of 17 years; 28% were female and 57% Caucasian. Subjective and objective measures of functional status were evaluated using the Physical Performance Test (PPT), the graded treadmill test, knee strength, gait speed, balance, and the Functional Status Questionnaire (FSQ). Body composition was evaluated using dual-energy X-ray absorptiometry (DXA) and magnetic resonance imaging (MRI). Sixty percent (25/40) of the participants met standard criteria for physical frailty. Frail (FR) and nonfrail (NF) participants were comparable in age, sex, CD4 count, and viral load. FR HOA had greater impairments in PPT, peak oxygen uptake, FSQ, walking speed, balance, and muscle quality than NF HOA. FR HOA had a greater body mass index (BMI), fat mass, and truncal fat with lipodystrophy. Moreover, PPT score was inversely related to trunk fat (correlation coefficient (r) = -0.34; P = .04) and ratio of intermuscular fat to total fat (r = -0.60; P = .02) after adjusting for covariates. HOA represent an emerging cohort of older adults who frequently experience frailty at a much younger age than the general older population. Central obesity and fat redistribution are important predictors of frailty in community-dwelling HOA. These findings suggest that physical frailty in HOA may be amenable to lifestyle interventions, especially exercise and diet therapy. © 2012, Copyright the Authors Journal compilation © 2012, The American Geriatrics Society.

  9. The correlation between perceived social support and illness uncertainty in people with human immunodeficiency virus/acquired immune deficiency syndrome in Iran

    Directory of Open Access Journals (Sweden)

    Moosa Sajjadi

    2015-01-01

    Full Text Available Background: Illness uncertainty is a source of a chronic and pervasive psychological stress for people living with human immunodeficiency virus (HIV/acquired immune deficiency syndrome (AIDS (PLWH, and largely affects their quality of life and the ability to cope with the disease. Based on the uncertainty in illness theory, the social support is one of the illness uncertainty antecedents, and influences the level of uncertainty perceived by patients. Aim: To examine uncertainty in PLWH and its correlation with social support in Iran. Materials and Methods: This cross-sectional correlational study was conducted with 80 PLWH presenting to AIDS Research Center, Tehran, Iran in 2013. The data collected using illness uncertainty and social support inventories were analyzed through Pearson′s correlation coefficient, Spearman′s correlation coefficient, and regression analysis. Results: The results showed a high level of illness uncertainty in PLWH and a negative significant correlation between perceived social support and illness uncertainty ( P = 0.01, r = -0.29. Conclusion: Uncertainty is a serious aspect of illness experience in Iranian PLWH. Providing adequate, structured information to patients as well as opportunities to discuss their concerns with other PLWH and receive emotional support from their health care providers may be worthwhile.

  10. A novel immunodeficiency syndrome as a rare cause of secondary pulmonary alveolar proteinosis: A diagnosis after 5 decades

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    Pedro G. Ferreira

    2014-09-01

    Full Text Available Case report of a male patient with a five-decade follow-up history in a tertiary care hospital distinguished for malabsorption syndrome, failure-to-thrive, meningitis and recurrent bacterial, fungal and mycobacterial pulmonary infections. Additionally, he developed epidermodysplasia verruciformis, several in situ spinocellular carcinomas and an uncharacteristic parenchymal lung disease. Surgical lung biopsy suggested pulmonary alveolar proteinosis with fibrotic change. Retrospectively, severe monocytopenia had been overlooked in the past, as well as low B and NK cell blood counts. Flow cytometry confirmed the absence of the previous cell subsets along with an undetectable population of dendritic blood cells.Dendritic cell, monocyte, B and NK lymphoid Human Deficiency Syndrome (DCMLS is a novel rare immunodeficiency described in 2010, linked to GATA-2 mutation. This syndrome should be highlighted as a rare cause of acquired PAP, with a radiological pattern encompassing potential fibrotic change. Failure to recognize monocytopenia may impede the chance to diagnose. Resumo: Relato de um caso clínico de um doente do sexo masculino com um historial de acompanhamento de cinco décadas, num hospital de cuidados terciários, caracterizado por um síndrome de malabsorção, insuficiência de crescimento, meningite e infecções pulmonares bacterianas, fúngicas e micobacterianas recorrentes. Além disso, desenvolveu epidermodisplasia verruciforme, diversos carcinomas espinocelulares in situ e uma doença pulmonar parenquimatosa não definida. Uma biópsia pulmonar cirúrgica sugeriu uma Proteinose Alveolar Pulmonar com alterações fibróticas. Em retrospectiva, uma monocitopenia grave negligenciada no passado, bem como uma baixa contagem de células B (linfócitos B e NK (células ‘natural killer’. Uma citometria de fluxo confirmou a ausência dos subconjuntos de células anteriores, juntamente

  11. Knowledge, attitude, and behavioral practices pertaining to human immunodeficiency virus/acquired immune deficiency syndrome among secondary school adolescents in makurdi, Nigeria

    Directory of Open Access Journals (Sweden)

    Ayu Agbecha

    2017-01-01

    Full Text Available Background: Adolescents knowledge with their safe practices pertaining to human immunodeficiency virus (HIV has a critical impact on the prevention of contracting and spreading HIV. Reports have shown that adolescents in the general setting engage in activities that enhance the spread of the virus. Aim: The study assessed school adolescent's HIV/acquired immune deficiency syndrome (AIDS knowledge, with its impact on their behaviors and attitudes regarding the infection. Materials and Methods: Two hundred randomly selected adolescent students from 10 different schools in the city metropolis were involved in the cross-sectional study. Primary data were collected using a validated self-administered questionnaire on students HIV/AIDS knowledge, attitude toward people living with HIV/AIDS (PLWHA, and safe practices preventing the spread of HIV/AIDS. Results: The study observed that majority of the students had good knowledge about HIV/AIDS, had good attitude toward PLWHA, and engaged in safe practices that prevent the spread of HIV. The sources of HIV/AIDS information were hospital, school, home, electronic, and print media. The study also found that HIV/AIDS knowledge instilled good attitudes and behavioral practices in the students. Conclusion: The study shows that school sex education, as well as health promotion campaigns through media platforms, could impact positively on the knowledge, attitude, and behavioral practices of adolescents in curbing the spread of HIV/AIDS.

  12. Human immunodeficiency virus endocrinopathy

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    Uma Sinha

    2011-01-01

    Full Text Available Human immunodeficiency virus (HIV endocrinopathy encompasses a broad spectrum of disorders. Almost all the endocrine organs are virtually affected by HIV infection. HIV can directly alter glandular function. More commonly secondary endocrine dysfunction occurs due to opportunistic infections and neoplasms in immunocompromised state. The complex interaction between HIV infection and endocrine system may be manifested as subtle biochemical and hormonal perturbation to overt glandular failure. Antiretroviral therapy as well as other essential medications often result in adverse endocrinal consequences. Apart from adrenal insufficiency, hypogonadism, diabetes and bone loss, AIDS wasting syndrome and HIV lipodystrophy need special reference. Endocrinal evaluation should proceed as in other patients with suspected endocrine dysfunction. Available treatment options have been shown to improve quality of life and long-term mortality in AIDS patients.

  13. A Clinical Study of Acquired Immunodeficiency Syndrome Associated Penicillium Marneffei Infection from a Non-Endemic Area in China.

    Science.gov (United States)

    Zheng, Jinding; Gui, Xien; Cao, Qian; Yang, Rongrong; Yan, Yajun; Deng, Liping; Lio, Jonathan

    2015-01-01

    To investigate the clinical characteristics, diagnosis, treatment and prognosis of penicilliosis among the patients with acquired immunodeficiency syndrome (AIDS) in non-endemic areas of China, and then to discuss its incubation period and the diagnostic performance of serum galactomannan test for penicilliosis. Medical records and travel histories of penicilliosis patients in Zhongnan hospital from January 2006 to December 2013, and the interval from when the patients left the endemic area to the onset of the disease was analyzed. Serum galactomannan levels of penicilliosis patients and AIDS patients with fever were measured by the Platelia Aspergillus Enzyme Immunoassay Kit. A total of 47 AIDS-associated penicilliosis were confirmed by fungal culture, which accounted for 4.8% of 981 AIDS-related admissions. The sensitivity and specificity of serum galactomannan test for penicilliosis were 95.8% (23/24) and 90.9% (30/33), respectively, (cutoff index = 1.0). Two independent predictors for early mortality (death within 12 weeks) of the patients (21.3%, 10/47) were a delayed diagnosis and no treatment with antifungal therapy. Among 14 patients who became ill after leaving endemic areas, ten patients presented with the onset symptoms within 12 months (from 11 days to 360 days). We found a patient living with asymptomatic P. marneffei fungemia who had not received any antifungal therapy until 18 months' follow up. The co-infection of P. marneffei and HIV was not uncommon in the non-endemic areas of penicilliosis in China. There exists a latent form of infection for P. marneffei. The incubation period of penicilliosis may be quite different from one patient to another. In AIDS patients, the serum galactomannan test has utility for the diagnosis of penicilliosis. When patients with penicilliosis/AIDS were diagnosed early and treated with standardized antifungal therapy and combined antiretroviral therapy, their prognosis improved.

  14. Risky sexual behaviour and human immunodeficiency virus (HIV) and acquired immune deficiency syndrome (AIDS) among healthcare workers.

    Science.gov (United States)

    Khamisa, Natasha; Mokgobi, Maboe

    2018-01-01

    South Africa is known to have one of the highest prevalence rates of human immunodeficiency virus (HIV) and acquired immune deficiency syndrome (AIDS) globally, with one in seven healthcare workers being HIV-positive. An HIV-positive healthcare workforce is less equipped to respond to the increasing spread of the epidemic. Assessment of the factors contributing to high HIV prevalence rates among healthcare workers is important in planning the development of human resources. This review sought to identify and understand predominant risky sexual behaviours among healthcare workers in HIV and AIDS-affected countries. This study reviewed articles focusing on sexual behaviour among healthcare workers. Major health science databases (e.g. ProQuest, Cochrane, PubMed and CINAHL) were searched for combinations of keywords including 'healthcare workers', 'risky sexual behaviour' and 'HIV and AIDS'. Articles from a range of countries met inclusion and exclusion criteria. Findings of the study revealed three main contributing factors: unprotected sex, multiple sex partners and sexual violence. Sexual violence emerged as the dominant risk factor in the majority of the studies. Most research was conducted in developed countries where the HIV infection rate is much lower than it is in developing countries. More research needs to be conducted in developing countries and appropriate strategies should be implemented to reduce sexual violence among healthcare workers. Appropriate procedures on reporting sexual violence coupled with education on HIV and AIDS as well as influencing attitudes and belief systems could assist in reducing the spread of HIV and AIDS within the healthcare workforce while minimising the effect on patient care.

  15. Matrix Degradation in Human Immunodeficiency Virus Type 1-Associated Tuberculosis and Tuberculosis Immune Reconstitution Inflammatory Syndrome: A Prospective Observational Study.

    Science.gov (United States)

    Walker, Naomi F; Wilkinson, Katalin A; Meintjes, Graeme; Tezera, Liku B; Goliath, Rene; Peyper, Janique M; Tadokera, Rebecca; Opondo, Charles; Coussens, Anna K; Wilkinson, Robert J; Friedland, Jon S; Elkington, Paul T

    2017-07-01

    Extensive immunopathology occurs in human immunodeficiency virus (HIV)/tuberculosis (TB) coinfection, but the underlying molecular mechanisms are not well-defined. Excessive matrix metalloproteinase (MMP) activity is emerging as a key process but has not been systematically studied in HIV-associated TB. We performed a cross-sectional study of matrix turnover in HIV type 1 (HIV-1)-infected and -uninfected TB patients and controls, and a prospective cohort study of HIV-1-infected TB patients at risk of TB immune reconstitution inflammatory syndrome (TB-IRIS), in Cape Town, South Africa. Sputum and plasma MMP concentrations were quantified by Luminex, plasma procollagen III N-terminal propeptide (PIIINP) by enzyme-linked immunosorbent assay, and urinary lipoarabinomannan (LAM) by Alere Determine TB LAM assay. Peripheral blood mononuclear cells from healthy donors were cultured with Mycobacterium tuberculosis and extracellular matrix in a 3D model of TB granuloma formation. MMP activity differed between HIV-1-infected and -uninfected TB patients and corresponded with specific TB clinical phenotypes. HIV-1-infected TB patients had reduced pulmonary MMP concentrations, associated with reduced cavitation, but increased plasma PIIINP, compared to HIV-1-uninfected TB patients. Elevated extrapulmonary extracellular matrix turnover was associated with TB-IRIS, both before and during TB-IRIS onset. The predominant collagenase was MMP-8, which was likely neutrophil derived and M. tuberculosis-antigen driven. Mycobacterium tuberculosis-induced matrix degradation was suppressed by the MMP inhibitor doxycycline in vitro. MMP activity in TB differs by HIV-1 status and compartment, and releases matrix degradation products. Matrix turnover in HIV-1-infected patients is increased before and during TB-IRIS, informing novel diagnostic strategies. MMP inhibition is a potential host-directed therapy strategy for prevention and treatment of TB-IRIS. © The Author 2017. Published by Oxford

  16. Immunological studies in the acquired immunodeficiency syndrome. II. Active suppression or intrinsic defect--investigated by mixing AIDS cells with HLA-DR identical normal cells

    DEFF Research Database (Denmark)

    Hofmann, B; Ødum, Niels; Jakobsen, B K

    1986-01-01

    The lymphocyte transformation responses to mitogens (phytohaemagglutinin (PHA), concanavalin A (Con A), and pokeweed mitogen (PWM)), allogeneic cells, and the antigen-purified protein derivative (PPD) were studied in six acquired immunodeficiency syndrome (AIDS) patients and in six healthy controls...... with the strong mitogens PHA and Con A or with allogeneic cells, but suppression may be involved in the decreased responses in cultures stimulated with PWM or PPD. Addition of supernatants from macrocultures of AIDS cells did not suppress responses of control PBMC. Thus, suppression by any lymphocyte subset...

  17. Radioimmunoassay and enzyme-linked immunoassay of antibodies to the core protein (P24) of human T-lymphotropic virus (HTLV III). [Acquired immunodeficiency syndrome (AIDS)

    Energy Technology Data Exchange (ETDEWEB)

    Neurath, A R; Strick, N; Sproul, P

    1985-05-01

    Human T-cell lymphotropic viruses designated HTLV III or LAV are considered to represent the causative agents of the acquired immunodeficiency syndrome (AIDS). Therefore a simple direct RIA or ELISA method for antibodies to distinct epitopes of HTLV III/LAV structural components would be of great value. The authors describe RIA and ELISA assays which obviate the need for purified virus or virus proteins, do not utilize infected cells and thus do not diminish the source for continuous production of viral antigens and are specific for a major core protein of HTLV III/LAV.

  18. The socioeconomic impact of human immunodeficiency virus / acquired immune deficiency syndrome in India and its relevance to eye care

    OpenAIRE

    Murthy GVS

    2008-01-01

    Human immunodeficiency virus (HIV) infection is aptly called the modern day ′plague′ and has the potential to decimate people in the productive age group. On the other hand, the increasing life expectancy in developing countries spirals age-related blindness. One therefore reduces economic productivity while the other increases economic dependency. Both lead to increased expenditure of households though in different proportions. Human immunodeficiency virus and blindness are bot...

  19. Correlation between Lymphocyte CD4 Count, Treatment Duration, Opportunistic Infection and Cognitive Function in Human Immunodeficiency Virus-Acquired Immunodeficiency Syndrome (HIV-AIDS) Patients.

    Science.gov (United States)

    Fitri, Fasihah Irfani; Rambe, Aldy Safruddin; Fitri, Aida

    2018-04-15

    Human immunodeficiency virus (HIV) infection is an epidemic worldwide, despite the marked benefits of antiretroviral therapy (ARV) in reducing severe HIV-associated dementia. A milder form of neurocognitive disorders are still prevalent and remain a challenge. This study aimed to determine the correlation between plasma cluster of differentiation 4 (CD4) lymphocyte, duration of ARV treatment, opportunistic infections, and cognitive function in HIV-AIDS patients. A cross-sectional study involving 85 HIV-AIDS patients was conducted at Adam Malik General Hospital Medan, Indonesia. All subjects were subjected to physical, neurologic examination and Montreal Cognitive Assessment-Indonesian Version (MoCA-INA) to assess cognitive function and measurement of lymphocyte CD4 counts. Out of the 85 subjects evaluated, the proportion concerning sexes include 52 males (61.2 %) and 33 females (38.8%). The mean age was 38.53 ± 9.77 years old. There was a significant correlation between CD4 lymphocyte counts and MoCA-INA score (r = 0.271, p = 0.012), but there was no significant correlation between duration of ARV treatment and MoCA-INA score. There was also no difference in MoCA-INA score based on the presence of opportunistic infection. Lymphocyte CD4 count was independently correlated with cognitive function in HIV-AIDS patients.

  20. Hepatitis B and C Sero-prevalence in Patients with Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome at a Tertiary Care Hospital in Izmir

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    Sabri ATALAY

    2018-04-01

    Full Text Available Objectives: Infections caused by human immunodeficiency virus (HIV, hepatitis B virus (HBV and hepatitis C virus (HCV represent a significant health problem. Co-infection with these viral agents is not uncommon as a result of the similar transmission routes. Our study was planned to investigate the prevalence of HBV and HCV infections in HIV/Acquired Immune Deficiency syndrome (AIDS patients followed up at our institution. Materials and Methods: In this study conducted in the Department of Infectious Diseases and Clinical Microbiology at Izmir Tepecik Training and Research Hospital, medical records of patients followed at the HIV/AIDS outpatient clinic between August 2002 and December 2014 were evaluated. Demographic data, main route of HIV transmission, hepatitis B surface antigen (HBsAg, anti-hepatitis B core (HBc immunoglobulin G (IgG, anti-HBs and anti-HCV results were evaluated. Results: A total of 157 treatment-naïve patients who were followed up at our HIV/AIDS outpatient clinic were included in this study. Four patients (2.6%, had HBsAg positivity. Anti-HBc IgG and anti-HBs positivity were detected in 34% and 28.4% of the patients, respectively. No patients had anti-HCV positivity. Conclusion: The prevalence of HBsAg in HIV-positive individuals was found to be similar to that in other population-based studies in our country. Absence of anti-HCV positivity suggests that hepatitis C infection is not a major health problem in this population.

  1. Atypical Manifestation of LPS-Responsive beige- like anchor (LRBA Deficiency Syndrome as an Autoimmune Endocrine Disorder without Enteropathy and Immunodeficiency.

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    Shahrzad Bakhtiar

    2016-09-01

    Full Text Available Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type1 diabetes mellitus, hypothyroidism, adrenal insufficiency and vitiligo. LPS-responsive beige-like anchor protein (LRBA deficiency was described in 2012 as a novel primary immunodeficiency, predominantly causing immune dysregulation and early onset enteropathy. We describe the heterogeneous clinical course of LRBA deficiency in two siblings, mimicking an autoimmune polyendocrine disorder in one of them in presence of the same underlying genetic mutation. The third child of consanguineous Egyptian parents (Patient 1 presented at six months of age with intractable enteropathy and failure to thrive. Later on he developed symptoms of adrenal insufficiency, autoimmune haemolytic anaemia, thrombocytopenia, and infectious complications due to immunosuppressive treatment. The severe enteropathy was non-responsive to the standard treatment and led to death at the age of 22 years. His younger sister (Patient 2 presented at the age of 12 to the endocrinology department with decompensated hypothyroidism, perioral vitiligo, delayed pubertal development, and growth failure without enteropathy and immunodeficiency.Using whole-exome sequencing (WES we identified a homozygous frameshift mutation (c.6862delT, p.Y2288MfsX29 in the LRBA gene in both siblings. To our knowledge our patient (patient 2 is the first case of LRBA deficiency described with predominant endocrine phenotype without immunodeficiency and enteropathy. LRBA deficiency should be considered as underlying disease in pediatric patients presenting with autoimmune endocrine symptoms. The same genetic mutation can manifest with a broad phenotypic spectrum without genotype-phenotype correlation. The awareness for disease symptoms among non-immunologists might be a key to early diagnosis. Further functional studies in LRBA deficiency are necessary to

  2. Efficacy and safety of traditional Chinese medicine for the treatment of acquired immunodeficiency syndrome: a systematic review.

    Science.gov (United States)

    Deng, Xin; Jiang, Manjun; Zhao, Xiaofang; Liang, Jian

    2014-02-01

    To systematically review the efficacy and safety of Traditional Chinese Medicine (TCM) interventions, compared with control interventions (placebo or conventional Western medical therapy), in the treatment of acquired immunodeficiency syndrome (AIDS). Electronic databases including PubMed, the Cochrane Library, China National Knowledge Infrastructure (CNKI), and Wanfang Data were searched to identify relevant randomized controlled trials (RCTs) published as of May 2012. Studies were selected according to the specified inclusion and exclusion criteria and then subjected to methodological quality assessment, data extraction, and meta-analysis according to the Cochrane Handbook for Systematic Reviews of Interventions. Twelve RCTs involving 881 patients with AIDS were included. Methodological quality assessment showed that two were high-quality, two were moderate-quality, and eight were low-quality. Meta-analysis showed that TCM interventions were associated with significantly reduced plasma viral load compared with placebo [odds ratio OR = 2.46, 95% confidence interval CI (1.02, 5.94); P = 0.04]. However, the reductions in plasma viral load significantly favored conventional Western medical therapy alone over integrated traditional Chinese and Western medical therapy [OR = 0.16, 95% CI (0.05, 0.55); P = 0.004]. Patients receiving TCM interventions had significantly higher CD4+ T lymphocyte counts compared with those on placebo [OR = 2.54, 95% CI (1.40, 4.60); P = 0.002]. In addition, TCM interventions were significantly more likely to have improved clinical symptoms [OR = 2.82, 95% CI (1.85, 4.31); P risk of adverse events (AEs) compared with control interventions [OR = 1.87, 95% CI (0.58, 6.01); P = 0.29]. Current evidence suggests that TCM interventions are significantly more effective than placebo in reducing plasma viral load and increasing CD4+ T lymphocyte count in patients with AIDS. When compared with conventional Western medical therapy, TCM interventions

  3. Epstein-Barr virus-associated primary central nervous system lymphoma in a child with the acquired immunodeficiency syndrome. A case report and review of the literature.

    Science.gov (United States)

    Rodriguez, M M; Delgado, P I; Petito, C K

    1997-12-01

    A 34-month-old black boy who had contracted acquired immunodeficiency syndrome from his mother presented with fever, vomiting, and cough. He was cachectic, hypertonic, and developmentally delayed. A brain computed tomography scan revealed masses in the left frontal horn, subependymal, and periventricular regions; secondary edema; and hydrocephalus. The differential diagnosis was cerebral lymphoma versus toxoplasmosis. The patient had disseminated Mycobacterium avium-intracellulare infection, lymphoid interstitial pneumonitis, as well as Pseudomonas and Klebsiella pneumonia. He died of respiratory insufficiency 53 days after admission. The autopsy confirmed a primary cerebral B-cell lymphoma, large cell type, which was positive for Epstein-Barr virus, latent phase, by in situ hybridization. Primary central nervous system lymphomas are rare in children, in contrast to adults. To our knowledge, only five well-documented cases of primary cerebral lymphomas in infants and children with acquired immunodeficiency syndrome have been reported previously. The current study shows that these childhood lymphomas are associated with and presumably caused by Epstein-Barr virus and thus have a pathogenesis similar to that of primary central nervous system lymphomas in adults.

  4. A recessive founder mutation in regulator of telomere elongation helicase 1, RTEL1, underlies severe immunodeficiency and features of Hoyeraal Hreidarsson syndrome.

    Science.gov (United States)

    Ballew, Bari J; Joseph, Vijai; De, Saurav; Sarek, Grzegorz; Vannier, Jean-Baptiste; Stracker, Travis; Schrader, Kasmintan A; Small, Trudy N; O'Reilly, Richard; Manschreck, Chris; Harlan Fleischut, Megan M; Zhang, Liying; Sullivan, John; Stratton, Kelly; Yeager, Meredith; Jacobs, Kevin; Giri, Neelam; Alter, Blanche P; Boland, Joseph; Burdett, Laurie; Offit, Kenneth; Boulton, Simon J; Savage, Sharon A; Petrini, John H J

    2013-08-01

    Dyskeratosis congenita (DC) is a heterogeneous inherited bone marrow failure and cancer predisposition syndrome in which germline mutations in telomere biology genes account for approximately one-half of known families. Hoyeraal Hreidarsson syndrome (HH) is a clinically severe variant of DC in which patients also have cerebellar hypoplasia and may present with severe immunodeficiency and enteropathy. We discovered a germline autosomal recessive mutation in RTEL1, a helicase with critical telomeric functions, in two unrelated families of Ashkenazi Jewish (AJ) ancestry. The affected individuals in these families are homozygous for the same mutation, R1264H, which affects three isoforms of RTEL1. Each parent was a heterozygous carrier of one mutant allele. Patient-derived cell lines revealed evidence of telomere dysfunction, including significantly decreased telomere length, telomere length heterogeneity, and the presence of extra-chromosomal circular telomeric DNA. In addition, RTEL1 mutant cells exhibited enhanced sensitivity to the interstrand cross-linking agent mitomycin C. The molecular data and the patterns of inheritance are consistent with a hypomorphic mutation in RTEL1 as the underlying basis of the clinical and cellular phenotypes. This study further implicates RTEL1 in the etiology of DC/HH and immunodeficiency, and identifies the first known homozygous autosomal recessive disease-associated mutation in RTEL1.

  5. A recessive founder mutation in regulator of telomere elongation helicase 1, RTEL1, underlies severe immunodeficiency and features of Hoyeraal Hreidarsson syndrome.

    Directory of Open Access Journals (Sweden)

    Bari J Ballew

    2013-08-01

    Full Text Available Dyskeratosis congenita (DC is a heterogeneous inherited bone marrow failure and cancer predisposition syndrome in which germline mutations in telomere biology genes account for approximately one-half of known families. Hoyeraal Hreidarsson syndrome (HH is a clinically severe variant of DC in which patients also have cerebellar hypoplasia and may present with severe immunodeficiency and enteropathy. We discovered a germline autosomal recessive mutation in RTEL1, a helicase with critical telomeric functions, in two unrelated families of Ashkenazi Jewish (AJ ancestry. The affected individuals in these families are homozygous for the same mutation, R1264H, which affects three isoforms of RTEL1. Each parent was a heterozygous carrier of one mutant allele. Patient-derived cell lines revealed evidence of telomere dysfunction, including significantly decreased telomere length, telomere length heterogeneity, and the presence of extra-chromosomal circular telomeric DNA. In addition, RTEL1 mutant cells exhibited enhanced sensitivity to the interstrand cross-linking agent mitomycin C. The molecular data and the patterns of inheritance are consistent with a hypomorphic mutation in RTEL1 as the underlying basis of the clinical and cellular phenotypes. This study further implicates RTEL1 in the etiology of DC/HH and immunodeficiency, and identifies the first known homozygous autosomal recessive disease-associated mutation in RTEL1.

  6. EXAMINATION RESULTS OF CHILDREN WITH CERVICAL SYNDROME

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    N. H. Bakhteeva

    2010-01-01

    Full Text Available By the example of examination of 80 children aged from 4 to 18 with cervical syndrome it is indicated, that the diagnosed abnormalities of hemodynamics in vertebrobasilar basin in patients of all age groups are connected both with bone and vascular pathology of the cervical part of the spine. The pathology has functional or congenital character. Early detection of discicirculatory vascular injuries in the cervical part of the spine in children with cervical syndrome will allow to define the therapeutic management of patients and to prolong juvenile osteochondrosis clinical behaviour.

  7. Low-dose growth hormone and human immunodeficiency virus-associated lipodystrophy syndrome: a pilot study

    DEFF Research Database (Denmark)

    Andersen, O; Haugaard, S B; Flyvbjerg, A

    2004-01-01

    the normal upper range and is accompanied by adverse effects such as joint pain and glucose intolerance. MATERIALS AND METHODS: We performed a 16-week open-labelled prospective pilot study in six male HALS patients using a s.c. low-dose hGH, 0.7 mg day(-1), aiming to examine the impact on total and free IGF......-I and fat distribution. Glucose metabolism was examined by oral glucose tolerance tests and hyperinsulinaemic euglycaemic clamps. RESULTS: Total IGF-I increased twofold (P

  8. Human immunodeficiency virus infection and acquired immune deficiency syndrome vulnerability of men who have sex with men in a border area of West Bengal, India

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    Dibakar Haldar

    2015-01-01

    Full Text Available Background: Studying level of living, awareness about sexually transmitted infections (STIs including human immunodeficiency virus infection and acquired immune deficiency syndrome (HIV/AIDS and sex behavior of men who have sex with men (MSMs is prerequisite for control of increasing AIDS among them in India. Objective: To assess sociodemographics, awareness about STIs including AIDS, and find out the pattern of high risk sex behavior of MSM. Methodology: Cross-sectional survey was undertaken in May, 2012 among MSMs catered by T I program via Nongovernmental Organization "Madhya Banglar Sangram" in Murshidabad District. 62 MSMs were included from five cruising spots sampled randomly out of fourteen such. Information was collected via interview and focused group discussions (FGD using questionnaire and FGD guide. Blood samples were examined for VDRL reactivity. Results: Median age was 25 years and sexual debut at 13.67 ± 4.29 years. 87% respondents were residing in parental house, 20% was married, 40% had low education, 80.33% had additional jobs but 54% reported poor income. About 56% respondents knew "what is AIDS" and its spread via anal sex, mother to child transmission, needle sharing, sex worker, and blood transfusion reported by 52.46, 50.82, 47.54, 45.90, and 34.43%, respectively. More than 2/3rd, about 40 and 34.43% MSMs played "anal and oral receptive," "anal insertive" and "oral insertive" role. About 33% used condom regularly. Majority knew main symptoms of STIs. About 2/3rd reported discrimination by neighbors. Blood examination showed 6.45% VDRL reactivity. Conclusion: Reducing vulnerability of MSMs to HIV/AIDS requires holistic programs.

  9. Knowledge, attitudes and practices regarding human immunodeficiency virus/acquired immune deficiency syndrome and sexually transmitted infections among health care providers in Lahore, Pakistan

    International Nuclear Information System (INIS)

    Khan, M.S.

    2010-01-01

    Human Immunodeficiency Virus (HIV) infection is a global problem of extraordinary dimensions and has so far resulted in nearly 25 million deaths worldwide. Health care providers (HCPs) are considered to play a pivotal role in the provision of preventive and curative services to individuals suffering from HIV/AIDS and sexually transmitted infections. Pakistan, which was previously categorised as having a low-prevalence, high-risk HIV epidemic, is now facing a concentrated HIV epidemic among its most at-risk populations such as injecting drug users. The objective of this study was to assess the knowledge, attitudes and reported practices relating to HIV/AIDS and STIs among private and public sector health care providers providing clinical services in areas where women sell sex. This was an exploratory quantitative study, where a structured questionnaire was administered in face-to-face interviews with 200 HCPs from the public and private sectors. Knowledge about AIDS and correct diagnosis of STIs were defined as according to the national guidelines of NACP. Pearson's chi-square analysis was performed to test associations between predictors and level of knowledge of STIs in each group separately. Multivariate logistic regression analysis was employed to indicate predicting factors for correct management of STIs. Forty-five percent of the HCPs had correct knowledge about the transmission and prevention of HIV, whereas 21% had seen a patient with advanced HIV infection, only two HCPs had been trained to manage such cases and 82% were not aware of syndromic management of STIs. Only 10% could cite the 'correct treatment' of gonorrhoea, syphilis and vaginal discharge. The odds of having the 'correct knowledge' of diagnosing gonorrhoea and syphilis were 2.1 (CI 95%, 1.2-3.8) if the HCP was a female medical doctor working in public sector. Further intensive training is needed to improve the ability of relevant HCPs to correctly diagnose and effectively treat patients

  10. Nocardiose pulmonar em paciente com síndrome da imunodeficiência adquirida: relato de caso Pulmonary nocardiosis in a patient with acquired immunodeficiency syndrome: case report

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    Ana Carina Gamboa da Silva

    2002-07-01

    Full Text Available Os autores descrevem um caso de nocardiose pulmonar em um homem de 37 anos de idade com a síndrome da imunodeficiência adquirida, em tratamento com anti-retrovirais, cujos sintomas de apresentação foram tosse com expectoração, hemoptóicos e emagrecimento progressivo. Foi realizada radiografia do tórax, que demonstrou consolidação no lobo superior do pulmão direito, e tomografia computadorizada do tórax, que evidenciou consolidação pulmonar com áreas escavadas. Diante dos achados radiológicos inespecíficos, foi realizada broncoscopia com lavado broncoalveolar, evidenciando estruturas filamentosas Gram-positivas compatíveis com Nocardia sp. O tratamento utilizado foi sulfametoxazol-trimetoprim, com remissão completa do quadro respiratório. Após revisão da literatura, foram discutidos os principais aspectos radiológicos desta doença.The authors describe a case of pulmonary nocardiosis in a 37-year-old man with acquired immunodeficiency syndrome in treatment with antiretroviral drugs. Clinical symptoms were productive cough, hemoptysis and progressive weight loss. A chest x-ray film showed a right upper lobe consolidation while the computed tomography demonstrated consolidation and areas of cavitation. The radiologic findings were unspecific. Consequently, a bronchoscopy with bronchoalveolar lavage was performed, revealing filamentous Gram-positive bacteria (Nocardia sp. Treatment with trimethoprim-sulfamethoxazole resulted in complete remission of the respiratory symptoms. The authors review the literature and discuss the main radiological features of this disease.

  11. Magnetic resonance evaluation of cerebral toxoplasmosis in patients with the acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Batra, A; Tripathi, R.P.; Gorthi, S.P.

    2004-01-01

    PURPOSE: To evaluate and delineate the characteristics of cerebral toxoplasmosis lesions using a combination of magnetic resonance (MR) spectroscopy, diffusion, and perfusion studies. MATERIAL AND METHODS: A total of 8 patients with 23 lesions were evaluated on a 1.5-T MR system. Diffusion-weighted imaging (DWI) was performed with three 'b' values of 50, 500, and 1000 s/mm2, and the apparent diffusion coefficient maps were calculated. The diffusion-weighted appearances and the T2-weighted MR appearances of the lesions were compared. MR spectroscopy was performed using the point-resolved single-voxel technique with two TE values of 135 ms and 270 ms. Perfusion studies were carried out using the dynamic contrast-enhanced technique, and the relative cerebral blood volume maps were qualitatively and quantitatively analyzed. RESULTS: DWI revealed the majority of the lesions as having increased diffusion within their necrotic centers, with the ADC ranging from 0.5 to 3.01 (mean ± SD: 1.49 ± 0.7). All the lesions revealed a predominant lipid peak on MR spectroscopy and were extremely hypovascular on perfusion MR studies. CONCLUSION: MR diffusion, spectroscopy, and perfusion studies help in characterizing toxoplasmosis lesions and, in most cases, can be used in combination to help establish the diagnosis of toxoplasmosis

  12. Magnetic resonance evaluation of cerebral toxoplasmosis in patients with the acquired immunodeficiency syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Batra, A; Tripathi, R.P.; Gorthi, S.P. [Inst. of Nuclear Medicine and Allied Sciences, Delhi (India). NMR Research Center

    2004-04-01

    PURPOSE: To evaluate and delineate the characteristics of cerebral toxoplasmosis lesions using a combination of magnetic resonance (MR) spectroscopy, diffusion, and perfusion studies. MATERIAL AND METHODS: A total of 8 patients with 23 lesions were evaluated on a 1.5-T MR system. Diffusion-weighted imaging (DWI) was performed with three 'b' values of 50, 500, and 1000 s/mm2, and the apparent diffusion coefficient maps were calculated. The diffusion-weighted appearances and the T2-weighted MR appearances of the lesions were compared. MR spectroscopy was performed using the point-resolved single-voxel technique with two TE values of 135 ms and 270 ms. Perfusion studies were carried out using the dynamic contrast-enhanced technique, and the relative cerebral blood volume maps were qualitatively and quantitatively analyzed. RESULTS: DWI revealed the majority of the lesions as having increased diffusion within their necrotic centers, with the ADC ranging from 0.5 to 3.01 (mean {+-} SD: 1.49 {+-} 0.7). All the lesions revealed a predominant lipid peak on MR spectroscopy and were extremely hypovascular on perfusion MR studies. CONCLUSION: MR diffusion, spectroscopy, and perfusion studies help in characterizing toxoplasmosis lesions and, in most cases, can be used in combination to help establish the diagnosis of toxoplasmosis.

  13. Epidemiological aspects of acquired immunodeficiency syndrome in older Brazilians: a comparative approach

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    Luiz Max Fagundes de Carvalho

    Full Text Available OBJECTIVES: To compare epidemiological aspects of young (15 to 49 years old and older (more than 50 years old AIDS patients. METHODS: We analyzed 511,633 AIDS cases notified to the Brazilian Ministry of Health in the period of 1980-2008 looking at sex, age ranges, educational level and exposure category. Patients were divided into three age groups: under 15, from 15 to 49 and over 50 years old. Using a comparative approach, we analyzed data with regard to category of exposure, education (expressed in years of schooling, and sex ratio among younger (15-49 and older adults (over 50 years old. Time series data were log-transformed and normalized, and the temporal trend was evaluated. RESULTS: AIDS incidence is increasing among people over 50 years old in Brazil, with those older than 50 being responsible for 9.64 % of AIDS cases. There was no significant difference between educational level and gender (p = 0.468, but there was a significant difference in exposure category with a lower proportion of injecting drug users amongst the older group. CONCLUSION: Based on this analysis over the last 10 years, the percentage of AIDS cases has increased almost three times among people over 50 years old when compared with the 15-49 year-old group. Our findings suggest that public campaigns have to be specially targeted to the older segment of the population, aiming at heterosexual transmission.

  14. First Rib Fracture Resulting in Horner's Syndrome.

    Science.gov (United States)

    Lin, You-Cheng; Chuang, Ming-Tsung; Hsu, Chin-Hao; Tailor, Al-Rahim Abbasali; Lee, Jung-Shun

    2015-12-01

    First rib fractures and traumatic Horner's syndrome are both quite rare, which can make it difficult to properly diagnose the combination of these 2 conditions in the emergency department. These conditions may be associated with severe medical emergencies, such as ongoing carotid dissection. We present the case of a 33-year-old man who sustained fractures to his right second, third, and fourth ribs and a delay in the diagnosis of left Horner's syndrome after he was involved in a traffic accident. Left Horner's syndrome was caused by a left transverse fracture of the first rib. This fracture was not detected on chest radiographs and required a 3-dimensional reconstructed neck computed tomography scan for detection. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: In the diagnosis of carotid artery dissection, conventional angiography is the criterion standard but is considered invasive. CTA is less invasive, time-saving, and can show more anatomic structures in the neck in addition to the carotid arteries. It is a good screening diagnostic modality in the traumatology department. Although the treatments for Horner's syndrome and first rib fracture are conservative, the early diagnosis of both conditions can resolve the anxiety and uncertainty experienced by both doctors and patients. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Tuberculose como doença definidora de síndrome da imunodeficiência adquirida: dez anos de evolução na Cidade do Rio de Janeiro Tuberculosis as a disease defining acquired immunodeficiency syndrome: ten years of surveillance in Rio de Janeiro, Brazil

    Directory of Open Access Journals (Sweden)

    Elizabeth Cristina Coelho Soares

    2006-10-01

    Program for the Surveillance of Sexually Transmitted Diseases and Acquired Immunodeficiency Syndrome database from 1993 to 2002. RESULTS: The expanded definition of a case of acquired immunodeficiency syndrome, implemented in 1998, resulted in a substantial increase in the number of reported cases of acquired immunodeficiency syndrome, especially of those defined by immunologic criteria. Among the cases of acquired immunodeficiency syndrome defined only by disease, esophageal candidiasis, in its various forms, remained the most common opportunistic infection present at the time the cases of acquired immunodeficiency syndrome were reported. Although Pneumocystis carinii pneumonia was the second leading opportunistic infection in most of the years evaluated, it was surpassed by tuberculosis in 2001. CONCLUSION: Despite the decreased numbers of cases of acquired immunodeficiency syndrome defined by disease, tuberculosis remains a significant acquired immunodeficiency syndrome-defining event, currently more common than P. carinii pneumonia and toxoplasmosis. This is probably due to the high rate of tuberculosis prevalence in the city.

  16. [Indicator condition guided human immunodeficiency virus requesting in primary health care: results of a collaboration].

    Science.gov (United States)

    Cayuelas-Redondo, Laia; Menacho-Pascual, Ignacio; Noguera-Sánchez, Pablo; Goicoa-Gago, Carmen; Pollio-Peña, Gernónimo; Blanco-Delgado, Rebeca; Barba-Ávila, Olga; Sequeira-Aymar, Ethel; Muns, Mercè; Clusa, Thais; García, Felipe; León, Agathe

    2015-12-01

    The search of HIV infected patients guided by indicator conditions (IC) is a strategy used to increase the early detection of HIV. The objective is to analyze whether a collaboration to raise awareness of the importance of early detection of HIV in 3 primary care centers influenced the proportion of HIV serology requested. Multicenter retrospective study was conducted comparing the baseline and a post-collaboration period. The collaboration consisted of training sessions and participation in the HIDES study (years 2009-2010). Patients between 18 and 64 years old with newly diagnosed herpes zoster, seborrheic eczema, mononucleosis syndrome, and leucopenia/thrombocytopenia in 3 primary care centers in 2008 (baseline period) and 2012 (post-collaboration period). The sociodemographic variables, HIV risk conditions, requests for HIV serology, and outcomes were evaluated. A total of 1,219 ICs were included (558 in 2008 and 661 in 2012). In 2008 the number of HIV tests in patients with an IC was 3.9%, and rose to 11.8% in 2012 (Pde Enfermedades Infecciosas y Microbiología Clínica. All rights reserved.

  17. Direct Epstein-Barr virus (EBV) typing on peripheral blood mononuclear cells: no association between EBV type 2 infection or superinfection and the development of acquired immunodeficiency syndrome-related non-Hodgkin's lymphoma

    NARCIS (Netherlands)

    van Baarle, D.; Hovenkamp, E.; Kersten, M. J.; Klein, M. R.; Miedema, F.; van Oers, M. H.

    1999-01-01

    In the literature, a correlation has been suggested between the occurrence of acquired immunodeficiency syndrome (AIDS)-related non-Hodgkin's lymphomas (NHL) and Epstein-Barr virus (EBV) type 2 infection. To further investigate a possible role for EBV type 2 infection in the development of AIDS-NHL,

  18. Treatment of facial lipoatrophy with polymethylmethacrylate among patients with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS): impact on the quality of life.

    Science.gov (United States)

    Quintas, Rodrigo C S; de França, Emmanuel R; de Petribú, Kátia C L; Ximenes, Ricardo A A; Quintas, Lóren F F M; Cavalcanti, Ernando L F; Kitamura, Marco A P; Magalhães, Kássia A A; Paiva, Késsia C F; Filho, Demócrito B Miranda

    2014-04-01

    The lipodystrophy syndrome is characterized by selective loss of subcutaneous fat on the face and extremities (lipoatrophy) and/or accumulation of fat around the neck, abdomen, and thorax (lipohypertrophy). The aim of this study has been to assess the impact of polymethylmethacrylate facial treatment on quality of life, self-perceived facial image, and the severity of depressive symptoms in patients living with HIV/AIDS. A non-randomized before and after interventional study was developed. Fifty-one patients underwent facial filling. The self-perceived quality of life, facial image, and degree of depressive symptoms were measured by the Short-Form 36 and HIV/AIDS--Targeted quality of life questionnaires, by a visual analogue scale and by the Beck depression inventory, respectively, before and three months after treatment. Six of the eight domains of Short-Form 36 and eight of the nine dimensions of the HIV/AIDS--Targeted quality of life questionnaires, together with the visual analogue scale and by the Beck depression inventory scores, revealed a statistically significant improvement. The only adverse effects registered were edema and ecchymosis. The treatment of facial lipoatrophy improved the self-perceived quality of life and facial image as well as any depressive symptoms among patients with HIV/AIDS. © 2014 The International Society of Dermatology.

  19. X-Linked Lymphoproliferative Syndrome and Common Variable Immunodeficiency May Not Be Differentiated by SH2D1A and XIAP/BIRC4 Genes Sequence Analysis

    Directory of Open Access Journals (Sweden)

    Nesrin Gulez

    2011-01-01

    Full Text Available The X-linked lymphoproliferative syndrome (XLP is a rare, inherited immunodeficiency characterized by recurrent episodes of hemophagocytic lymphohistiocytosis, hypogammaglobulinemia, and/or lymphomas. Recently, X-linked inhibitor of apoptosis (XIAP/BIRC4 gene defects, in families with XLP but without SH2D1A gene defects, has been defined. The distinction from primary immunodeficiencies with a defined genetic cause is mandatory. A six-year-old male patient was admitted with the complaints of persistent general lymphadenopathy, for two years had fever, bilateral cervical multiple microlymphadenopathy, hepatic/splenic enlargement with laboratory findings as decreased serum immunoglobulins, negative EBV VCA IgM (viral capsid antigen and anti-EBV EA (antibody to early D antigen, positive EBV VCA IgG (viral capsid antigen and EBV EBNA (antibody to nuclear antigen. SH2D1A gene analysis was negative. XIAP/BIRC4 sequencing revealed two novel single nucleotide variants (exon 7, 1978G > A, and 1996T > A in the 3′UTR of the gene in both patient and mother which were not disease causing. XIAP protein expression was found to be normal. The clinical and laboratory resemblance, no gene mutations, and normal XIAP protein expression led us to think that there may be another responsible gene for XLP. The patient will to be followed up as CVID until he presents new diagnostic signs or until the identification of a new gene.

  20. Long-term results after cardiac surgery in patients infected with the human immunodeficiency virus type-1 (HIV-1).

    Science.gov (United States)

    Mestres, Carlos A; Chuquiure, Javier E; Claramonte, Xavier; Muñoz, Josefa; Benito, Natividad; Castro, Miguel A; Pomar, José L; Miró, José M

    2003-06-01

    Assessment of long-term results of immunodeficiency virus type-1 (HIV-1)-infected patients undergoing cardiac surgery. Retrospective analysis of profile and outcomes of 31 HIV-1-infected patients (35 operations, 1985-2002). Twenty-seven males and four females (mean age 34.67) in three groups: acute infective endocarditis (AIE) 21 (67.74%), coronary (CAD) 5 (16.13%) and non-infective valvular disease (NIVD) 5 (16.13%). HIV factors: drug addiction (23-74.19%), homosexuality (5-16.12%), heterosexuality (3-9.67%), hemodialysis (1-3.22%). HIV stage: A (17), B (2), C (2) in AIE; A (2), B (3) in CAD and A (3), C (2) in NIVD. Mean preoperative CD4 count was 278 cells/microL (12infected patients requiring cardiac surgery, a decrease in AIE, however NIVD and CAD increasingly seen. Cardiac surgery did not blunt CD4 response induced by antiretrovirals. The late cause of death were not AIDS-related events.

  1. The socioeconomic impact of human immunodeficiency virus / acquired immune deficiency syndrome in India and its relevance to eye care.

    Science.gov (United States)

    Murthy, Gvs

    2008-01-01

    Human immunodeficiency virus (HIV) infection is aptly called the modern day 'plague' and has the potential to decimate people in the productive age group. On the other hand, the increasing life expectancy in developing countries spirals age-related blindness. One therefore reduces economic productivity while the other increases economic dependency. Both lead to increased expenditure of households though in different proportions. Human immunodeficiency virus and blindness are both associated with discrimination, stigma and long-term consequences. They impact the socioeconomic fabric of the affected individuals, communities and countries. The loss in productivity and the cost of support to the affected individuals are seen in both. Each is a potent problem on its own but together they spell disaster in geometric proportions rather than a simple additive effect. Strategies need to be evolved to provide solace and improve the quality of life of an HIV-positive blind individual.

  2. The socioeconomic impact of human immunodeficiency virus / acquired immune deficiency syndrome in India and its relevance to eye care

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    Murthy GVS

    2008-01-01

    Full Text Available Human immunodeficiency virus (HIV infection is aptly called the modern day ′plague′ and has the potential to decimate people in the productive age group. On the other hand, the increasing life expectancy in developing countries spirals age-related blindness. One therefore reduces economic productivity while the other increases economic dependency. Both lead to increased expenditure of households though in different proportions. Human immunodeficiency virus and blindness are both associated with discrimination, stigma and long-term consequences. They impact the socioeconomic fabric of the affected individuals, communities and countries. The loss in productivity and the cost of support to the affected individuals are seen in both. Each is a potent problem on its own but together they spell disaster in geometric proportions rather than a simple additive effect. Strategies need to be evolved to provide solace and improve the quality of life of an HIV-positive blind individual.

  3. Endogenous sodium potassium ATPase inhibition related biochemical cascade and the acquired immunodeficiency syndrome -Neural regulation of viral replication and immune response to the virus

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    Ravikumar A

    2001-11-01

    Full Text Available The isoprenoid pathway and its metabolites - digoxin, dolichol and ubiquinone were assessed in acquired immunodeficiency syndrome. Digoxin is an endogenous regulator of membrane Na+-K+ ATPase secreted by the human hypothalamus. The HMG CoA reductase activity was increased with increased digoxin and dolichol levels and reduced ubiquinone levels in AIDS. Membrane Na+-K+ ATPase activity and serum magnesium levels were reduced. The tryptophan catabolites were increased and the tyrosine catabolites were reduced. The glycoconjugate metabolites were increased and lysosomal stability was reduced. There was reduced incorporation of glycoconjugates into membranes and increased membrane cholesterol: phospholipid ratio. Lipid peroxidation products and NO were increased while free radical scavenging enzymes and reduced glutathione were reduced. The role of the isoprenoid pathway related cascade in the pathogenesis of AIDS is discussed.

  4. Infection of immunodeficient horses with Sarcocystis neurona does not result in neurologic disease.

    Science.gov (United States)

    Sellon, Debra C; Knowles, Donald P; Greiner, Ellis C; Long, Maureen T; Hines, Melissa T; Hochstatter, Tressa; Tibary, Ahmed; Dame, John B

    2004-11-01

    Equine protozoal myeloencephalitis is a progressive neurologic disease of horses most commonly caused by infection with the apicomplexan parasite Sarcocystis neurona. Factors affecting neuroinvasion and neurovirulence have not been determined. We investigated the pathogenesis of infection with S. neurona in horses with severe combined immune deficiency (SCID). Two immunocompetent (IC) Arabian horses and two Arabian horses with SCID were infected orally with 5 x 10(5) sporocysts of S. neurona. Four IC horses and one SCID horse were infected intravenously (i.v.) with 5 x 10(8) merozoites of the WSU-1 isolate of S. neurona. Despite prolonged parasitemia and persistent infection of visceral tissues (skeletal muscle, cardiac muscle, lung, liver, and spleen) as demonstrated by PCR and culture, SCID horses did not develop neurologic signs after oral or i.v. infection. S. neurona was undetectable in the neuronal tissues of SCID horses by either PCR, immunohistochemistry, or culture. In contrast, although parasitemia was undetectable in orally infected IC horses and of only short duration in i.v. infected IC horses, four of six IC horses developed neurologic signs. S. neurona was detectable by PCR and/or culture of neural tissue but not visceral tissue of IC horses with neurologic disease. Infected SCID horses are unable to clear S. neurona from visceral tissues, but the infection does not result in neurologic signs; in contrast, IC horses rapidly control parasitemia and infection of visceral tissues but frequently experience neuroinvasion and exhibit clinical signs of neurologic disease.

  5. Saúde auditiva, o vírus da imunodeficiência humana e a síndrome da imunodeficiência adquirida: uma revisão Hearing health, the human immunodeficiency virus and the acquired immunodeficiency syndrome: a review

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    Thais Catalani Morata

    2010-08-01

    Full Text Available TEMA: a Síndrome da Imunodeficiência Adquirida (SIDA/AIDS é causada pelo Vírus da Imunodeficiência Humana (VIH/HIV, e resulta numa imunidade reduzida, o que torna o indivíduo mais susceptível a doenças e infecções oportunistas. Com o avanço da doença as estruturas do sistema auditivo central podem ser comprometidas pela ação direta do vírus ou decorrente de infecções secundárias e neoplasias. O portador do HIV/AIDS pode também se tornar mais vulnerável a outras patologias do ouvido. OBJETIVO: o objetivo desta revisão foi exploratório, visando identificar os possíveis pontos de interseção entre distúrbios auditivos e a Síndrome da Imunodeficiência Adquirida. Realizou-se uma revisão da literatura sobre a saúde auditiva dos portadores de HIV/AIDS e foi discutido o impacto potencial de patologias auditivas na qualidade de vida. CONCLUSÃO: a literatura sugere que várias possiveis associações existam entre os distúrbios auditivos e a Síndrome da Imunodeficiência Adquirida e o Vírus da Imunodeficiência Humana. Profissionais de saúde, inclusive aqueles dos serviços públicos no Brasil, deveriam examinar a necessidade de iniciativas de saúde auditiva dirigida aos portadores de HIV/AIDS para prevenir patologias auditivas ou reduzir seu impacto na qualidade de vida.BACKGROUND: Acquired Immunodeficiency Syndrome (or Acquired immune deficiency syndrome or AIDS is caused by the Human Immunodeficiency Virus (HIV and results in reduced immunity, leaving affected individuals more susceptible to illness and opportunistic infections. As the disease progresses, structures within the central auditory system can be affected either by the direct action of the virus or as a consequence of secondary infections. Other areas of the auditory system may also be more vulnerable to pathology in people living with HIV or AIDS. PURPOSE: the goal of this review was exploratory, to identify possible points of intersection between auditory

  6. Screening for Human Immunodeficiency Virus (HIV)

    Science.gov (United States)

    ... with antiretroviral therapy (ART). Starting ART early—before symptoms appear—greatly reduces the risk of developing acquired immunodeficiency syndrome (AIDS, the final stage of HIV infection), having AIDS-related complications, or dying of ...

  7. Manifestações reumáticas da síndrome de imunodeficiência adquirida (AIDS Rheumatic manifestations of the acquired immunodeficiency syndrome (AIDS

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    Rodrigo Siqueira-Batista

    2004-10-01

    Full Text Available Diferentes síndromes reumáticas e fenômenos auto-imunes já foram associados à infecção pelo vírus da imunodeficiência humana (HIV, quer pela maior freqüência de adoecimento, quer pela maior gravidade. Ademais, em muitos casos, sintomas e sinais da doença reumática estão presentes na abertura do quadro de infecção pelo HIV (como na síndrome de soroconversão ou da síndrome de imunodeficiência adquirida (AIDS. O reconhecimento deste horizonte é da maior importância para a prática do reumatologista, tornando-se altamente necessário ampliar a familiaridade com estes conceitos. Revisitar as interseções reumatológicas da infecção pelo HIV - enfatizando-se situações como as espondiloartropatias soronegativas, as artralgias, as artrites infecciosas, fibromialgia, miopatias, vasculites, síndrome linfocítica infiltrante difusa, lúpus eritematoso sistêmico e fenômenos auto-imunes - é, pois, o objetivo do presente artigo.Different rheumatic syndromes and auto-immune phenomena have already been associated with the infection by the human immunodeficiency virus (HIV, either by the higher frequency of the disease or by the greater severity. Furthermore, in many cases, symptoms and signs of rheumatic disease are present at the beginning of the infection condition by HIV (as in the serum conversion or the Acquired Immunodeficiency Syndrome (AIDS. Recognition of these possibilities is of fundamental importance to the rheumatologist practice and is necessary to have familiarity with these concepts. Revisiting the rheumatologic intersections of the infection by HIV - emphasizing situations such as seronegative spondyloarthropathies, arthralgia, infectitious arthritis, fibromyalgia, myopathy, vasculitis, difuse infiltranting lymphocytic syndrome systemic lupus erythematous and auto-immune phenomena - is then, the aim of the present article.

  8. [Cerebral infarction and intracranial aneurysm related to the reactivation of varicella zoster virus in a Japanese acquired immunodeficiency syndrome (AIDS) patient].

    Science.gov (United States)

    Yasuda, Chiharu; Okada, Kazumasa; Ohnari, Norihiro; Akamatsu, Naoki; Tsuji, Sadatoshi

    2013-01-01

    A 35-years-old right-handed man admitted to our hospital with a worsening of dysarthria, left facial palsy and left hemiparesis for 2 days. Acquired immunodeficiency syndrome (AIDS) was diagnosed when he was 28 years old. At that time, he also was treated for syphilis. After highly active antiretroviral treatment (HAART) was introduced at the age of 35 years old, serum level of human immunodeficiency virus (HIV) was not detected, but the number of CD4+ T cells was still less than 200/μl. He had no risk factors of atherosclerosis including hypertension, diabetes and hyperlipidemia. He had neither coagulation abnormality nor autoimmune disease. Magnetic resonance imaging (MRI) showed acute ischemic infarction spreading from the right corona radiate to the right internal capsule without contrast enhancement. Stenosis and occlusion of intracranial arteries were not detected by MR angiography. Although argatroban and edaravone were administered, his neurological deficits were worsened to be difficult to walk independently. Cerebrospinal fluid (CSF) examination showed a mild mononuclear pleocytosis (16/μl). Oligoclonal band was positive. The titer of anti-varicella zoster virus (VZV) IgG antibodies was increased, that indicated VZV reactivation in the central nervous system (CNS), although VZV DNA PCR was not detected. Therefore, acyclovir (750 mg/day for 2 weeks) and valaciclovir (3,000 mg/day for 1 month) were administered in addition to stroke therapy. He recovered to be able to walk independently 2 month after the admission.Angiography uncovered a saccular aneurysm of 3 mm at the end of branch artery of right anterior cerebral artery, Heubner artery, 28 days after the admission. We speculated that VZV vasculopathy caused by VZV reactivation in CNS was involved in the pathomechanism of cerebral infarction rather than HIV vasculopathy in the case.

  9. Hyper IgM Syndrome with low IgM and thrombocytosis: an unusual case of immunodeficiency.

    Science.gov (United States)

    Yousef, Ejaz; Arshad Alvi, M

    2016-09-01

    We report a 5 years old male child with low serum IgG, IgA and IgM levels, who presented with recurrent perianal and oral ulcers, intermittent fever, and protracted diarrhea. Despite the lack of typical respiratory symptoms, low serum IgM level and persistent thrombocytosis, an X-linked hyper-IgM syndrome (X-HIGM) was considered. Laboratory investigations revealed a diagnosis of hyper-IgM syndrome caused by CD40L deficiency.

  10. Results of radiotherapy of Duplay's syndrome

    International Nuclear Information System (INIS)

    Kreisfeldt, E.

    1979-01-01

    The observed patient group comprised 185 persons who underwent treatment during 1970 and 1976. The sexual distribution within this group is normal. The age peak ranges between 50 and 60 years. In a late inquiry 31.9% of the patients can indicate the freedom from pain. 29.2% tell about a good, 18.4% about moderate success. In about one half of the treated persons roentgenologically visible calcareous deposits were detected. In both groups their treatment led to an equally good result. However, an accumulation of diseases with calcifications is found in patients who were younger than 50 years. In acute cases without previous treatment the achieved result was better than in those who had been treated already elsewhere. Acute cases respond considerably better to the treatment than the chronicaal ones. For the acute cases also a notably inferior recidivity rate was found. In 38% of all cases recidivation was observed. For the acute cases this rate is only 16%. Although investigations exist which do not hint at an autonomous course of this disease, a therapeutic treatment is not considered as superfluous. Considering the known risks due to ionizing irradiation, radiotherapy of periarticular fibrositis is recommended particularly for patients older than 40 years. (orig./MG) [de

  11. [Acquired immunodeficiency syndrome-related lymphoma: 1. Course during the 20 years of the epidemic. 2. The experience at the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán: 1986-2003].

    Science.gov (United States)

    Hernández-Rivera, E Gabriela; Gómez-Roel, Xóchitl; Villasís-Keever, Angelina

    2004-01-01

    The goal of this presentation is the description of the epidemiologic evolution and changes in natural history of the human immunodeficiency virus infection (HIV) epidemic itself and its relation with the acquired immunodeficiency syndrome-related lymphoma (ARL). We have started with the description of the world's state of the HIV epidemic, its features since the first case report in the United States of America in 1981, through the peak of new diagnoses in 1993 until the event that changed the natural history of the disease: the era of the widespread use of the highly active antiretroviral therapy (HAART), introduced in 1995 in the world and in 1997 in our country. The widespread introduction of HAART led to dramatic reductions in AIDS related mortality and morbidity throughout the developed world with a marked fall in the incidence of the major opportunistic infections in AIDS. We describe the main risk factors for the development of ARL, and the prognostic factors for survival and response to treatment. There is no clear definition in the literature of the roll that has played the use of HAART in relation to survival and response to treatment in ARL, but there is evidence that the basal count of CD4 cells has increased with HAART, leading to a better survival and response in ARL. The debate regarding this issue is surely affected by factors such as degree of antiretroviral treatment compliance, antiretroviral therapy resistance and chemotherapy heterogeneity. Finally we present the preliminary results of the analysis of our experience in ARL from 1986 to 2003.

  12. Cholecystitis in a child with acquired immunodeficiency syndrome: ultrasonographic findings in a case of uncommon presentation during childhood; Colecistitis en un nino con sida: hallazgos ecograficos en una forma infrecuente de presentacion en la infancia

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    Rubi-Palomares, I.; Martinez-Leon, M. I.; Alonso-Usabiaga, I.; Ceres-Ruiz, L. [Hospital Materno Infantil del C.H.U. Carlos Haya. Malaga (Spain)

    2001-07-01

    We present the case of a boy with acquired immunodeficiency syndrome (AIDS) in whom ultrasound revealed the presence of acalculous cholecystitis. Abdominal ultrasound was essential in the initial diagnosis and the assessment of the response to treatment. This entity has rarely been reported in the pediatric radiology literature. In our patient, the cholecystitis may have been caused by infection, and the involvement of regional lymphatic obstruction in the pathogenesis is considered. (Author) 14 refs.

  13. Associação de carcinoma broncogênico com síndrome de Pancoast e síndrome da imunodeficiência adquirida Association of bronchogenic carcinoma with Pancoast's syndrome and acquired immunodeficiency syndrome

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    A.C. FRÖHLICH

    2000-09-01

    Full Text Available É relatado o caso de um paciente portador da síndrome de Pancoast associada à síndrome da imunodeficiência adquirida. Esta apresentação é rara, visto que os tumores mais freqüentemente associados à SIDA são o sarcoma de Kaposi e o linfoma não-Hodgkin. O paciente, com passado de uso de drogas injetáveis, internou-se para investigação de massa em ápice pulmonar, com presença de síndrome de Pancoast, sendo solicitado anti-HIV, com resultado positivo, e tendo sido feito diagnóstico de Ca brônquico não-pequenas células.A case of a patient with Pancoast's syndrome associated with acquired immunodeficiency syndrome is reported. This association is rare and Kaposi's sarcoma and non-Hodgkin lymphoma are the most recurrent tumors in AIDS. The patient was an IV drug user and was admitted to the hospital for diagnosis of apex lung mass with signs of Pancoast's syndrome. HIV serology was positive and pathology of lung mass showed non-small cell lung cancer.

  14. Treatment-related neuroendocrine prostate cancer resulting in Cushing's syndrome.

    Science.gov (United States)

    Ramalingam, Sundhar; Eisenberg, Adva; Foo, Wen Chi; Freedman, Jennifer; Armstrong, Andrew J; Moss, Larry G; Harrison, Michael R

    2016-12-01

    Here we present, to the best of our knowledge, the first case of a paraneoplastic Cushing's syndrome (hypercortisolism) resulting from treatment-related neuroendocrine prostate cancer - a highly aggressive and difficult disease to treat. A 51-year-old man was started on androgen deprivation therapy after presenting with metastatic prostate cancer, characterized by diffuse osseous metastasis. Shortly thereafter, he developed progressive disease with biopsy proven neuroendocrine prostate cancer as well as symptoms of increased skin pigmentation, hypokalemia, hypertension, hyperglycemia and profound weakness, consistent with ectopic Cushing's syndrome. Molecular analysis of the patient's tumor through RNA sequencing showed high expression of several genes including CHGA, ASCL1, CALCA, HES6, PCSK1, CALCB and INSM1 confirming his neuroendocrine phenotype; elevated POMC expression was found, supporting the diagnosis of ectopic Cushing's syndrome. © 2016 The Japanese Urological Association.

  15. High Mortality and Coinfection in a Prospective Cohort of Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome Patients with Histoplasmosis in Guatemala.

    Science.gov (United States)

    Samayoa, Blanca; Roy, Monika; Cleveland, Angela Ahlquist; Medina, Narda; Lau-Bonilla, Dalia; Scheel, Christina M; Gomez, Beatriz L; Chiller, Tom; Arathoon, Eduardo

    2017-07-01

    Histoplasmosis is one of the most common and deadly opportunistic infections among persons living with human immunodeficiency virus (HIV)/acquired immune deficiency syndrome in Latin America, but due to limited diagnostic capacity in this region, few data on the burden and clinical characteristics of this disease exist. Between 2005 and 2009, we enrolled patients ≥ 18 years of age with suspected histoplasmosis at a hospital-based HIV clinic in Guatemala City. A case of suspected histoplasmosis was defined as a person presenting with at least three of five clinical or radiologic criteria. A confirmed case of histoplasmosis was defined as a person with a positive culture or urine antigen test for Histoplasma capsulatum . Demographic and clinical data were also collected and analyzed. Of 263 enrolled as suspected cases of histoplasmosis, 101 (38.4%) were confirmed cases. Median time to diagnosis was 15 days after presentation (interquartile range [IQR] = 5-23). Crude overall mortality was 43.6%; median survival time was 19 days (IQR = 4-69). Mycobacterial infection was diagnosed in 70 (26.6%) cases; 26 (25.7%) histoplasmosis cases were coinfected with mycobacteria. High mortality and short survival time after initial symptoms were observed in patients with histoplasmosis. Mycobacterial coinfection diagnoses were frequent, highlighting the importance of pursuing diagnoses for both diseases.

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

    Science.gov (United States)

    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.

  17. Eradication of hepatitis C virus and non-liver-related non-acquired immune deficiency syndrome-related events in human immunodeficiency virus/hepatitis C virus coinfection.

    Science.gov (United States)

    Berenguer, Juan; Rodríguez-Castellano, Elena; Carrero, Ana; Von Wichmann, Miguel A; Montero, Marta; Galindo, María J; Mallolas, Josep; Crespo, Manuel; Téllez, María J; Quereda, Carmen; Sanz, José; Barros, Carlos; Tural, Cristina; Santos, Ignacio; Pulido, Federico; Guardiola, Josep M; Rubio, Rafael; Ortega, Enrique; Montes, María L; Jusdado, Juan J; Gaspar, Gabriel; Esteban, Herminia; Bellón, José M; González-García, Juan

    2017-08-01

    We assessed non-liver-related non-acquired immunodeficiency syndrome (AIDS)-related (NLR-NAR) events and mortality in a cohort of human immunodeficiency virus (HIV)/hepatitis C virus (HCV)-coinfected patients treated with interferon (IFN) and ribavirin (RBV), between 2000 and 2008. The censoring date was May 31, 2014. Cox regression analysis was performed to assess the adjusted hazard rate (HR) of overall death in responders and nonresponders. Fine and Gray regression analysis was conducted to determine the adjusted subhazard rate (sHR) of NLR deaths and NLR-NAR events considering death as the competing risk. The NLR-NAR events analyzed included diabetes mellitus, chronic renal failure, cardiovascular events, NLR-NAR cancer, bone events, and non-AIDS-related infections. The variables for adjustment were age, sex, past AIDS, HIV transmission category, nadir CD4 + T-cell count, antiretroviral therapy, HIV RNA, liver fibrosis, HCV genotype, and exposure to specific anti-HIV drugs. Of the 1,625 patients included, 592 (36%) had a sustained viral response (SVR). After a median 5-year follow-up, SVR was found to be associated with a significant decrease in the hazard of diabetes mellitus (sHR, 0.57; 95% confidence interval [CI], 0.35-0.93; P = 0.024) and decline in the hazard of chronic renal failure close to the threshold of significance (sHR, 0.43; 95% CI, 0.17-1.09; P = 0.075). Our data suggest that eradication of HCV in coinfected patients is associated not only with a reduction in the frequency of death, HIV progression, and liver-related events, but also with a reduced hazard of diabetes mellitus and possibly of chronic renal failure. These findings argue for the prescription of HCV therapy in coinfected patients regardless of fibrosis stage. (Hepatology 2017;66:344-356). © 2017 The Authors. Hepatology published by Wiley Periodicals, Inc., on behalf of the American Association for the Study of Liver Diseases.

  18. Approach to a child with primary immunodeficiency

    Directory of Open Access Journals (Sweden)

    Özge Yılmaz

    2010-09-01

    Full Text Available Primary immunodeficiencies are clinically and epidemiologically important, despite their low prevalence, due to the associated risk of high morbidity and mortality. Most commonly encountered primary immunodeficiencies include humoral immune system deficiencies, cellular immune system defects, combined immunodeficiencies, phagocyte system defects, complement system defects. Classical clinical findings of immunodeficiencies include recurrent, severe infections which do not respond to treatment or which progress with complications as well as tendency to develop infections with low virulence microorganisms. Moreover, they may present with autoimmunity, autoinflammatory or hemaphagocytic syndromes. Congenital diseases usually start in early childhood and lead to morbidity and mortality. Therefore, early diagnosis may be life saving and allow increasing quality of life, genetic counseling or prenatal diagnosis. Considering primary immunodeficiencies more frequently in differential diagnosis and early immunological evaluation would lead to early diagnosis of these patients and allow them to reach early treatment or preventive measures.

  19. Hemichorea-hemiballismus as an initial manifestation in a Moroccan patient with acquired immunodeficiency syndrome and toxoplasma infection: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Samira Rabhi

    2011-09-01

    Full Text Available Neurologic signs and symptoms may represent the initial presentation of AIDS in 10-30% of patients. Movement disorders may be the result of direct central nervous system infection by human immunodeficiency virus (HIV or the result of opportunistic infections. We report the case of a 59 years old woman who had hemichorea-hemiballismus subsequently found to be secondary to a cerebral toxoplasmosis infection revealing HIV infection. Movement disorders, headache and nausea were resolved after two weeks of antitoxoplasmic treatment. Brain MRI control showed a marked resolution of cerebral lesion. Occurrence of hemichorea-ballismus in patient without familial history of movement disorders suggests a diagnosis of AIDS and in particular the diagnosis of secondary cerebral toxoplasmosis. Early recognition is important since it is a treatable entity.

  20. Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Patients in the Black Sea Region of Turkey

    Directory of Open Access Journals (Sweden)

    Alişan Yıldıran

    2017-12-01

    Full Text Available Hematopoietic stem cell transplantation is a promising curative therapy for many combined primary immunodeficiencies and phagocytic disorders. We retrospectively reviewed pediatric cases of patients diagnosed with primary immunodeficiencies and scheduled for hematopoietic stem cell transplantation. We identified 22 patients (median age, 6 months; age range, 1 month to 10 years with various diagnoses who received hematopoietic stem cell transplantation. The patient diagnoses included severe combined immunodeficiency (n=11, Chediak-Higashi syndrome (n=2, leukocyte adhesion deficiency (n=2, MHC class 2 deficiency (n=2, chronic granulomatous syndrome (n=2, hemophagocytic lymphohistiocytosis (n=1, Wiskott-Aldrich syndrome (n=1, and Omenn syndrome (n=1. Of the 22 patients, 7 received human leukocyte antigen-matched related hematopoietic stem cell transplantation, 12 received haploidentical hematopoietic stem cell transplantation, and 2 received matched unrelated hematopoietic stem cell transplantation. The results showed that 5 patients had graft failure. Fourteen patients survived, yielding an overall survival rate of 67%. Screening newborn infants for primary immunodeficiency diseases may result in timely administration of hematopoietic stem cell transplantation.

  1. Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Patients in the Black Sea Region of Turkey.

    Science.gov (United States)

    Yıldıran, Alişan; Çeliksoy, Mehmet Halil; Borte, Stephan; Güner, Şükrü Nail; Elli, Murat; Fışgın, Tunç; Özyürek, Emel; Sancak, Recep; Oğur, Gönül

    2017-12-01

    Hematopoietic stem cell transplantation is a promising curative therapy for many combined primary immunodeficiencies and phagocytic disorders. We retrospectively reviewed pediatric cases of patients diagnosed with primary immunodeficiencies and scheduled for hematopoietic stem cell transplantation. We identified 22 patients (median age, 6 months; age range, 1 month to 10 years) with various diagnoses who received hematopoietic stem cell transplantation. The patient diagnoses included severe combined immunodeficiency (n=11), Chediak-Higashi syndrome (n=2), leukocyte adhesion deficiency (n=2), MHC class 2 deficiency (n=2), chronic granulomatous syndrome (n=2), hemophagocytic lymphohistiocytosis (n=1), Wiskott-Aldrich syndrome (n=1), and Omenn syndrome (n=1). Of the 22 patients, 7 received human leukocyte antigen-matched related hematopoietic stem cell transplantation, 12 received haploidentical hematopoietic stem cell transplantation, and 2 received matched unrelated hematopoietic stem cell transplantation. The results showed that 5 patients had graft failure. Fourteen patients survived, yielding an overall survival rate of 67%. Screening newborn infants for primary immunodeficiency diseases may result in timely administration of hematopoietic stem cell transplantation.

  2. Knowledge and attitude of Indian clinical dental students towards the dental treatment of patients with human immunodeficiency virus (HIV)/acquired immune-deficiency syndrome (AIDS).

    Science.gov (United States)

    Oberoi, Sukhvinder Singh; Marya, Charu Mohan; Sharma, Nilima; Mohanty, Vikrant; Marwah, Mohita; Oberoi, Avneet

    2014-12-01

    Oral health care of patients with human immunodeficiency virus (HIV)/acquired immune-deficiency syndrome (AIDS) is a growing area of concern. Information on HIV- and AIDS-related knowledge among dental students provides a crucial foundation for efforts aimed at developing an appropriate dental curriculum on HIV and AIDS. The purpose of this study was to assess the knowledge and attitude of Indian clinical dental students towards the treatment of patients with HIV/AIDS and perceived sources of information regarding HIV-related issues. Data were collected from clinical dental students (third year, fourth year and internship) from three dental institutions in Delhi National Capital Region (NCR). The questions assessed the knowledge and attitude towards treatment of patients with HIV and the perceived source of information related to HIV. The willingness to treat HIV-positive patients among dental students was 67.0%, and 74.20% were confident of treating a patient with HIV/AIDS. The potential problems in rendering treatment to these patients were effect on the attitude of other patients (49.90%) and staff fears (52.50%). The correct knowledge regarding the infection-control practice (barrier technique) was found among only 15.50% of respondents. The respondents had sufficient knowledge regarding the oral manifestations of HIV/AIDS. There was no correlation between the knowledge and attitude score, demonstrating a gap between knowledge and attitude among dental students regarding treatment of HIV-infected patients. Appropriate knowledge has to be delivered through the dental education curriculum, which can instil confidence in students about their ability to manage HIV-positive patients. © 2014 FDI World Dental Federation.

  3. Epidemiological situation of acquired immunodeficiency syndrome (AIDS)-related mortality in a municipality in northeastern Brazil. A retrospective cross-sectional study.

    Science.gov (United States)

    Silva, Luana Rodrigues da; Araújo, Ellen Thallita Hill; Carvalho, Moisés Lopes; Almeida, Camila Aparecida Pinheiro Landim; Oliveira, Adélia Dalva da Silva; Carvalho, Patrícia Maria Gomes de; Rodrigues, Tatyanne Silva; Campelo, Viriato

    2018-01-01

    The number of acquired immunodeficiency syndrome (AIDS)-related deaths covers different segments of the population differently, making monitoring of this mortality essential. The aim of this study was to describe the epidemiological situation of AIDS-related mortality in a municipality in the northeastern region of Brazil. Retrospective cross-sectional study based on data from death certificates in the mortality information system of the Health Information Center, Municipal Health Foundation, Brazil. Between 2003 and 2013, we investigated death certificates on which AIDS-related mortality was reported. Sociodemographic data, year, place, type of establishment where death occurred and underlying and associated causes that led to AIDS-related death were described. The Mann-Kendall test was used to verify the growth trend of the standardized mortality rate over the period studied. Among the 1,066 AIDS-related deaths, 69.7% were among men; 47.2% of the individuals were 28-41 years of age, 32.7% had had 4-7 years of schooling, 66.9% were pardos (mixed race), 55.7% were unmarried and 15.3% were housekeepers. Hospitals were the site of 97% of the deaths, and 91% occurred at public hospitals. Respiratory failure was the main cause of death. The prevalence of infectious and parasitic diseases was 99.0%. AIDS-related mortality increased by 160% over the period studied, from 5.5/100,000 inhabitants in 2003 to 14.3/100,000 in 2013. In the Brazilian municipality studied here, AIDS-related mortality was most prevalent among men and young adults of lower socioeconomic level. Over the period studied, the mortality rate increased.

  4. Knowledge, attitude, and perception of disease among persons living with human immunodeficiency virus/acquired immuno deficiency syndrome: A study from a tertiary care center in North India.

    Science.gov (United States)

    Gupta, Mrinal; Mahajan, Vikram K; Chauahn, Pushpinder S; Mehta, Karainder S; Rawat, Ritu; Shiny, T N

    2016-01-01

    Although modification of behavioral practices among human immunodeficiency virus (HIV)-affected patients is important in decreasing HIV disease transmission, the knowledge, attitude, and perception studies about HIV infection rarely include persons living with HIV/acquired immuno deficiency syndrome (AIDS). To assess knowledge, attitude, and perceptions of persons living with HIV/AIDS for the disease and other epidemiological aspects. One-hundred and fifty consecutive persons living with HIV/AIDS were enrolled for this questionnaire-based cross-sectional, descriptive study. These 150 patients comprised 93 men and 57 women, aged between 14 and 78 (mean 37.13) years. The majority, 112 (74.67%) patients were between 20 and 50 years of age and 116 (77.3%) patients were either illiterate or high-school dropouts. Drivers, laborers, and self-employed comprised 69 (74.2%) patients among affected males. Only 129 (86%) respondents had heard about HIV/AIDS and knew about its heterosexual transmission. Ninety-eight (65.3%) respondents were aware of disease transmission from infected blood or needle pricks. Interestingly, 106 (70.7%) respondents were aware of the importance of using condom in preventing disease transmission. Television/radio was the most common sources of information for 135 (90%) patients. Nearly, 69% respondents disfavored disclosing their disease to friends/colleagues fearing stigmatization. Information, education, and communication activities are imperative to educate persons living with HIV/AIDS about life-long nature of the disease, modes of its transmission, and significance of preventive measures to bridge the gaps in their knowledge. While improvement in individual economic status, education, and health services remains highly desirable, mass media can play a pivotal role in creating awareness among masses.

  5. Orbital Apex Syndrome Resulting from Mixed Bacterial Sphenoid Sinusitis

    Directory of Open Access Journals (Sweden)

    Mengfei Xiong

    2018-05-01

    Full Text Available Orbital apex syndrome (OAS is an uncommon disorder characterized by visual loss, ophthalmoplegia, ptosis and hypoaesthesia of the forehead[1]. OAS may result from a variety of inflammatory, infectious, neoplastic and vascular conditions that cause damage to the superior orbital fissure (with resultant oculomotor (III, trochlear (IV, abducens (VI and ophthalmic branch of the trigeminal nerve (V1 palsies and to the optic canal leading to optic nerve (II dysfunction. This case report describes the clinical development of OAS in a patient with bacterial sphenoid sinusitis.

  6. Long-term outcome following hematopoietic stem-cell transplantation in Wiskott-Aldrich syndrome: collaborative study of the European Society for Immunodeficiencies and European Group for Blood and Marrow Transplantation

    DEFF Research Database (Denmark)

    Ozsahin, H.; Cavazzana-Calvo, M.; Notarangelo, L.D.

    2008-01-01

    Wiskott-Aldrich syndrome (WAS) is a rare X-linked immunodeficiency with microthrombocytopenia, eczema, recurrent infections, autoimmune disorders, and malignancies that are life-threatening in the majority of patients. In this long-term, retrospective, multicenter study, we analyzed events...... approach to performing splenectomy in WAS patients. Overall, this study provides the basis for a prospective, standardized, and more in-depth detailed analysis of chimerism and events in long-term follow-up of WAS patients who receive transplants to design better-adapted therapeutic strategies...

  7. Bacteremia por Rhodococcus equi em paciente com síndrome da imunodeficiência adquirida: relato de caso Bacteremia due to Rhodococcus equi in a patient with acquired immunodeficiency syndrome: case report

    Directory of Open Access Journals (Sweden)

    Carina Secchi

    2006-12-01

    Full Text Available Rhodococcus equi é um importante agente de infecções zoonóticas, podendo causar sérias infecções em humanos, principalmente em pacientes imunocomprometidos. Neste estudo, nós relatamos o caso de uma bacteremia fatal devido a Rhodococcus equi em paciente com síndrome da imunodeficiência adquirida (HIV positivo.Rhodococcus equi is an important agent for zoonotic infections, and may cause serious infections in humans, especially immunocompromised patients. In this study, a case of fatal bacteremia due to Rhodococcus equi in a patient with acquired immunodeficiency syndrome (HIV positive is reported.

  8. [Clinical Results of Endoscopic Treatment of Greater Trochanteric Pain Syndrome].

    Science.gov (United States)

    Zeman, P; Rafi, M; Skala, P; Zeman, J; Matějka, J; Pavelka, T

    2017-01-01

    PURPOSE OF THE STUDY This retrospective study aims to present short-term clinical outcomes of endoscopic treatment of patients with greater trochanteric pain syndrome (GTPS). MATERIAL AND METHODS The evaluated study population was composed of a total of 19 patients (16 women, 3 men) with the mean age of 47 years (19-63 years). In twelve cases the right hip joint was affected, in the remaining seven cases it was the left side. The retrospective evaluation was carried out only in patients with greater trochanteric pain syndrome caused by independent chronic trochanteric bursitis without the presence of m. gluteus medius tear not responding to at least 3 months of conservative treatment. In patients from the followed-up study population, endoscopic trochanteric bursectomy was performed alone or in combination with iliotibial band release. The clinical results were evaluated preoperatively and with a minimum follow-up period of 1 year after the surgery (mean 16 months). The Visual Analogue Scale (VAS) for assessment of pain and WOMAC (Western Ontario MacMaster) score were used. In both the evaluated criteria (VAS and WOMAC score) preoperative and postoperative results were compared. Moreover, duration of surgery and presence of postoperative complications were assessed. Statistical evaluation of clinical results was carried out by an independent statistician. In order to compare the parameter of WOMAC score and VAS pre- and post-operatively the Mann-Whitney Exact Test was used. The statistical significance was set at 0.05. RESULTS The preoperative VAS score ranged 5-9 (mean 7.6) and the postoperative VAS ranged 0-5 (mean 2.3). The WOMAC score ranged 56.3-69.7 (mean 64.2) preoperatively and 79.8-98.3 (mean 89.7) postoperatively. When both the evaluated parameters of VAS and WOMAC score were compared in time, a statistically significant improvement (ppain syndrome yields statistically significant improvement of clinical results with the concurrent minimum incidence of

  9. Autoimmunity and primary immunodeficiency: two sides of the same coin?

    Science.gov (United States)

    Schmidt, Reinhold E; Grimbacher, Bodo; Witte, Torsten

    2017-12-19

    Autoimmunity and immunodeficiency were previously considered to be mutually exclusive conditions; however, increased understanding of the complex immune regulatory and signalling mechanisms involved, coupled with the application of genetic analysis, is revealing the complex relationships between primary immunodeficiency syndromes and autoimmune diseases. Single-gene defects can cause rare diseases that predominantly present with autoimmune symptoms. Such genetic defects also predispose individuals to recurrent infections (a hallmark of immunodeficiency) and can cause primary immunodeficiencies, which can also lead to immune dysregulation and autoimmunity. Moreover, risk factors for polygenic rheumatic diseases often exist in the same genes as the mutations that give rise to primary immunodeficiency syndromes. In this Review, various primary immunodeficiency syndromes are presented, along with their pathogenetic mechanisms and relationship to autoimmune diseases, in an effort to increase awareness of immunodeficiencies that occur concurrently with autoimmune diseases and to highlight the need to initiate appropriate genetic tests. The growing knowledge of various genetically determined pathologic mechanisms in patients with immunodeficiencies who have autoimmune symptoms opens up new avenues for personalized molecular therapies that could potentially treat immunodeficiency and autoimmunity at the same time, and that could be further explored in the context of autoimmune rheumatic diseases.

  10. Neurologic manifestations of human immunodeficiency virus infection in children

    NARCIS (Netherlands)

    Epstein, L. G.; Sharer, L. R.; Oleske, J. M.; Connor, E. M.; Goudsmit, J.; Bagdon, L.; Robert-Guroff, M.; Koenigsberger, M. R.

    1986-01-01

    This report describes the neurologic manifestations of 36 children with human immunodeficiency virus (HIV) infection. In this cohort, in 16 of 21 children with acquired immunodeficiency syndrome (AIDS), three of 12 children with AIDS-related complex, and one of three asymptomatic seropositive

  11. Cryptosporidiosis among patients with acquired immunodeficiency syndrome (AIDS) in the county of São Paulo, Brazil

    OpenAIRE

    Dias, Rosa Maria Donini Souza; Mangini, Ana Célia Steffen; Torres, Domingas Maria A. Grispino Vieira; Corrêa, Marcelo Oswaldo A.; Lupetti, Norival; Corrêa, Fernando M. A.; ChieffI, Pedro Paulo

    1988-01-01

    Stool samples of 157 patients with AIDS, living in the county of São Paulo, were submitted to several techniques in the search for Cryptosporidium sp.. Among the various techniques tested for slide preparation (direct smear, spontaneous sedimentation method, and formol-ether concentration), the latter, formol-ether concentration, offered the best results, clearly outdoing all the others. Nineteen samples out of 157 prepared by this technique, after dyeing by the Kinyoun method or by carbol fu...

  12. Esofagites em pacientes com síndrome de imunodeficiência adquirida: estudo histológico e imunoistoquímico Esophagitis in patients with acquired human immunodeficiency syndrome: an histological and immunohistochemistry study

    Directory of Open Access Journals (Sweden)

    Ana Paula Aguiar Vidal

    2007-12-01

    citomegalovírus, respectivamente. O emprego da técnica de imunoistoquímica auxilia no diagnóstico das esofagites virais e torna possível detectar o citomegalovírus em esôfagos normais à endoscopia e/ou ao exame histopatológico.BACKGROUND: Almost all patients with acquired immunodeficiency virus syndrome will have gastrointestinal symptoms during the course of their illness. The high prevalence and complications of esophagitis are well documented. AIM: Graduate esophagitis; identify microorganisms like Candida sp, cytomegalovirus, herpesvirus and mycobacteria; identify by immunohistochemical staining viral agents cytomegalovirus, herpesvirus I, herpesvirus II, Epstein-Barr Virus, human papilloma virus and human immunodeficiency virus; verify how immunohistochemistry changes the profile of esophagitis; verify the association between the histological and endoscopical findings; verify the relevance of the number of fragments studied in the characterization of the histological agents. METHODS: We studied retrospectively esophageal biopsies in 227 patients with acquired immunodeficiency virus syndrome using hematoxylin and eosin, PAS (periodic acid of Schiff, Groccott and Ziehl-Nielsen stains and immunoperoxidase stains to detect opportunistic agents. Endoscopic aspects were studied. RESULTS: The non-specific esophagitis grade III, in the inferior third of the esophagus, was the most frequent type. Candida sp was the most frequent agent, followed by viruses cytomegalovirus, herpesvirus and mycobacteria. The presence of plaque and ulceration suggested the diagnosis of esophageal candidiasis and cytomegalovirus esophagitis. Immunohistochemical allowed the characterization of cytomegalovirus and of herpesvirus in those cases where other techniques could not achieve it, furthermore the cytomegalovirus was also found in histological normal cases, making the use of this technique advisable in routine diagnosis. The herpesvirus I was not found isolated but associated to

  13. Hypothenar hammer syndrome: long-term results of vascular reconstruction.

    Science.gov (United States)

    Endress, Ryan D; Johnson, Craig H; Bishop, Allen T; Shin, Alexander Y

    2015-04-01

    To evaluate long-term patency rates and related outcomes after vascular reconstruction of hypothenar hammer syndrome and identify patient- or treatment-related factors that may contribute to differences in outcome. We used color flow ultrasound to determine the patency of 18 vein graft reconstructions of the ulnar artery at the wrist in 16 patients. Validated questionnaires evaluated patients' functional disability with the Disabilities of the Arm, Shoulder, and Hand score, pain with the visual analog scale, and cold intolerance with the Cold Intolerance Symptom Severity survey. Patient demographics, clinical data, and surgical factors were analyzed for association with graft failure. Patients were asked to grade the result of treatment on a scale of 0 to 10. Of 18 grafts, 14 (78%) were occluded at a mean of 118 months postoperatively. Patients with patent grafts had significantly less disability related to cold intolerance according to the Cold Intolerance Symptom Severity survey in addition to significantly less pain on the visual analog scale. There was no statistical difference in Disabilities of the Arm, Shoulder, and Hand scores between patients with patent or occluded grafts. Patients graded the result significantly higher in patent reconstructions. We noted a higher incidence of graft occlusion than previously reported at a mean follow-up of 9.8 years, which represents a long-duration follow-up study of surgical treatment of hypothenar hammer syndrome. Despite a high percentage of occlusion, overall, patients remained satisfied with low functional disability and all would recommend surgical reconstruction. This study suggests that improved outcomes may result from patent grafts in the long term. Prognostic IV. Copyright © 2015 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

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

    Science.gov (United States)

    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 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.

  15. Recurrent hydramnios as a result of fetal Bartter′s syndrome (a case report.

    Directory of Open Access Journals (Sweden)

    Shah M

    1991-04-01

    Full Text Available Bartter′s syndrome has been reported as a rare case of hydramnios. A unique case of recurrent hydramnios in pregnancy as a result of fetal Bartter′s syndrome on both occasions is presented.

  16. Neuroradiology of acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    De La Paz, R.; Enzmann, D.

    1988-01-01

    This paper discusses MRI and CT scanning in the evaluation of AIDS patients with neurological symptoms. MRI is the more sensitive of the two techniques but has poor tissue specificity. Contrast enhancement and identification of tissue calcification on CT scans may improve tissue characterization, but a specific etiological diagnosis may not be possible with either MRI or CT scanning; confirmation by biopsy or the response to treatment is often needed. The finding of multiple focal cerebral lesions on MRI and CT scans usually indicates T. gondii infection. Toxoplasmosis may, however, coexist with primary CNS lymphoma or with fungal, mycobacterial, or viral infection. The presence of a solitary focal cerebral lesion, especially on MRI scans, suggests lymphoma or infection with an agent other than T. gondii. Focal cerebral lesions show a variety of complex signal patterns on MRI and enhancement patterns on CT, none of which are specific for a single etiology. Focal lesions on MRI and CT scans are usually associated with focal neurological deficits, but a significant minority of these lesions, especially those seen on MRI scans, may cause only generalized complaints, such as headache and encephalopathy. MRI and CT are both useful for monitoring therapy and show a reduction in the size, number, and enhancement of lesions with successful treatment. The authors conclude that, owing to its greater sensitivity, MRI appears to be more useful for detecting recurrent disease

  17. Nigerian dental technology students and human immunodeficiency ...

    African Journals Online (AJOL)

    Background: The rehabilitative dental care is important for maintaining adequate nutrition, guarding against wasting syndrome and malnutrition among human immunodeficiency virus (HIV)‑infected individuals. Aim: The aim of this study is to determine the Nigerian dental technology students' knowledge and ...

  18. The biology of human immunodeficiency virus infection.

    Science.gov (United States)

    Kotler, Donald P

    2004-08-01

    The aim of this article is to review the basic biology of infection with HIV-1 and the development of the acquired immunodeficiency syndrome. The discussion will include epidemiology, general description of the retroviruses, pathogenesis of the immune deficiency, clinical consequences, treatment, and treatment outcomes. Aspects of the infection that affect protein and energy balance will be identified.

  19. Cell-to-Cell Contact Results in a Selective Translocation of Maternal Human Immunodeficiency Virus Type 1 Quasispecies across a Trophoblastic Barrier by both Transcytosis and Infection

    Science.gov (United States)

    Lagaye, S.; Derrien, M.; Menu, E.; Coïto, C.; Tresoldi, E.; Mauclère, P.; Scarlatti, G.; Chaouat, G.; Barré-Sinoussi, F.; Bomsel, M.

    2001-01-01

    Mother-to-child transmission can occur in utero, mainly intrapartum and postpartum in case of breastfeeding. In utero transmission is highly restricted and results in selection of viral variant from the mother to the child. We have developed an in vitro system that mimics the interaction between viruses, infected cells present in maternal blood, and the trophoblast, the first barrier protecting the fetus. Trophoblastic BeWo cells were grown as a tight polarized monolayer in a two-chamber system. Cell-free virions applied to the apical pole neither crossed the barrier nor productively infected BeWo cells. In contrast, apical contact with human immunodeficiency virus (HIV)-infected peripheral blood mononuclear cells (PBMCs) resulted in transcytosis of infectious virus across the trophoblastic monolayer and in productive infection correlating with the fusion of HIV-infected PBMCs with trophoblasts. We showed that viral variants are selected during these two steps and that in one case of in utero transmission, the predominant maternal viral variant characterized after transcytosis was phylogenetically indistinguishable from the predominant child's virus. Hence, the first steps of transmission of HIV-1 in utero appear to involve the interaction between HIV type 1-infected cells and the trophoblastic layer, resulting in the passage of infectious HIV by transcytosis and by fusion/infection, both leading to a selection of virus quasispecies. PMID:11312350

  20. Acute Korsakoff-like amnestic syndrome resulting from left thalamic infarction following a right hippocampal hemorrhage.

    Science.gov (United States)

    Rahme, R; Moussa, R; Awada, A; Ibrahim, I; Ali, Y; Maarrawi, J; Rizk, T; Nohra, G; Okais, N; Samaha, E

    2007-04-01

    Korsakoff-like amnestic syndromes have been rarely described following structural lesions of the central nervous system. In this report, we describe a case of acute Korsakoff-like syndrome resulting from the combination of a left anteromedian thalamic infarct and a right hippocampal hemorrhage. We also review the literature relevant to the neuropathology and pathophysiology of Korsakoff syndrome and anterograde amnesia.

  1. Gastric cryptosporidiosis as a clue for the diagnosis of the acquired immunodeficiency syndrome Criptosporidiose gástrica como pista para o diagnóstico da síndrome da imunodeficiência adquirida

    Directory of Open Access Journals (Sweden)

    Cyntia Mendes CLEMENTE

    2000-07-01

    Full Text Available Cryptosporidium parvum has been detected with increasing frequency in the gastrointestinal tract, but involvement of the stomach is rarely reported. Whenever found in the histologic examination of the gastrointestinal mucosa, it should raise the suspicion of an immunocompromised host. We report a case of Cryptosporidium-associated erosive gastritis in a 64-year-old woman, who was found later to have the acquired immunodeficiency syndrome. Gastroduodenoendoscopy and biopsy of the gastric mucosa played an invaluable role in the diagnosis of cryptosporidiosis and to disclose the underlying immunodeficiency stateInfecções oportunistas do trato gastrointestinal constituem ameaça à população crescente de portadores de imunossupressão. O comprometimento do estômago por Cryptosporidium é incomum. Quando identificado no exame histopatológico da mucosa gástrica, é mandatória a investigação do estado imunológico do hospedeiro. São apresentados os dados clinicopatológicos e endoscópicos de uma paciente de 64 anos com gastrite erosiva associada à infecção por Cryptosporidium. O encontro deste agente oportunista no exame histopatológico da mucosa gástrica foi fundamental para esclarecer a doença de base da paciente, que era a síndrome da imunodeficiência adquirida.

  2. Hyperparathyroidism-Jaw Tumor Syndrome: Results of surgical management

    Science.gov (United States)

    Mehta, Amit; Patel, Dhaval; Rosenberg, Avi; Boufraqech, Myriem; Ellis, Ryan J.; Nilubol, Naris; Quezado, Martha M.; Marx, Stephen J.; Simonds, William F.; Kebebew, Electron

    2014-01-01

    Background Hyperparathyroidism-jaw tumor syndrome (HPT-JT) is a rare autosomal dominant disease secondary to germline inactivating mutations of the tumor suppressor gene HRPT2/CDC73. The aim of the present study is to determine the optimal surgical approach to parathyroid disease in patients with HPT-JT. Method A retrospective analysis of clinical and genetic features, parathyroid operative outcomes, and disease outcomes in seven unrelated HPT-JT families. Results Seven families had five distinct germline HRPT2/CDC73 mutations. Sixteen affected family members (median age of 30.7 years) were diagnosed with primary hyperparathyroidism. Fifteen of the 16 patients underwent preoperative tumor localization studies and uncomplicated bilateral neck exploration at initial operation - all were in biochemical remission at most recent follow up. 31% of patients had multiglandular involvement. 37.5% of patients developed parathyroid carcinoma (median overall survival 8.9 years; median follow-up 7.4 years). Long-term follow-up showed 20% of patients had recurrent primary hyperparathyroidism. Conclusions Given the high risk of malignancy and multiglandular involvement in our cohort, we recommend bilateral neck exploration and en-bloc resection of parathyroid tumors suspicious for cancer and life-long postoperative follow-up. PMID:25444225

  3. [Surgical treatment of Marfan syndrome; late results and new strategy].

    Science.gov (United States)

    Aomi, S; Nonoyama, M; Tomioka, H; Endo, M; Nagashima, H; Sakomura, Y; Aoka, Y; Kasanuki, H; Kurosawa, H

    2002-07-01

    Rapid progress has been made in the treatment of Marfan syndrome. Today, the treatment is relatively established and the results have also improved. Even if surgery is performed, however, vascular lesions may recur late after operation and late prognosis is poor considering the age of patients. Issues such as whether a reoperation should be conducted or how the late results might be improved are subjects of debate. Based on an analysis of recent late data, we have performed operations according to the new treatment policy, and here report the results. A total of 203 consecutive operations were conducted in 141 patients with Marfan syndrome who underwent surgery for aortic aneurysm at our department between February 1973 and August 2001. The mean age of patients was 39 (11 years with a male/female ratio of 95:46. At the first operation, 72 patients were diagnosed with annuloaortic ectasia (AAE), 17 patients with AAE + chronic dissection (DeBakey I), 14 patients with AAE + chronic dissection (DeBakey II), 6 patients with AAE + acute dissection (Stanford A), 11 patients with AAE + dissection (DeBakey III), 9 patients with dissection (DeBakey III) only, 3 patients with AAE + abdominal aortic aneurysm only, and 2 patients with abdominal aortic aneurysm only. The cause of reoperation were a new lesion in 17 patients, dissection in 13 patients and a true aneurysm in 4 patients. In 36 patients, an increase in the remaining lesion occurred or a scheduled stage 2 operation was performed. Reoperation was performed following the Bentall operation in 7 patients, dehiscence of the anastomotic region of the coronary artery in 5 patients, aneurysm of the anastomotic region of the coronary artery in 1 patients, and infection of the artificial valve with aneurysm of the anastomotic region of the coronary artery in 1 patient. Hospital deaths were reported in 8 (6%) patients who underwent composite valve graft replacement (including simultaneous arch replacement) for AAE. Hospital

  4. Epidemiology of the human immunodeficiency virus in Saudi Arabia; 18-year surveillance results and prevention from an Islamic perspective

    Directory of Open Access Journals (Sweden)

    Al-Mazrou Yagob Y

    2004-08-01

    Full Text Available Abstract Background data on HIV epidemiology and preventive measures in Islamic countries is limited. This study describes the results of 18-year of HIV surveillance in Saudi Arabia (SA and the preventive measures implemented from an Islamic perspective. Methods surveillance for HIV has been underway in SA since 1984. Indications for HIV testing include clinical suspicion, screening of contacts of HIV-infected patients, and routine screening of blood and organ donors, prisoners, intravenous drug users, patients with other sexually transmitted infections, and expatriates pre-employment. This is a case series descriptive study of all confirmed HIV infections diagnosed in SA from 1984 through 2001. Results a total of 6046 HIV infections were diagnosed, of which 1285 (21.3% cases were Saudi citizens. Over the 18-year surveillance period the number of HIV infections diagnosed annually among Saudi citizens gradually increased and, over the period 1997–2001, it reached to 84 to 142 cases per year. The number of cases per 100,000 population varied widely between regions with a maximum of 74 cases and a minimum of 2 cases. The infection was most common in the age group 20–40 years (74.6% and predominantly affected men (71.6%. The modes of transmission among Saudi citizens and expatriates, respectively, were as follows: heterosexual contact, 487 (37.9% and 1352 (28.4% cases; blood transfusion, 322 (25.0% and 186 (3.9% cases; perinatal transmission, 83 (6.5% and 19 (0.4% cases; homosexual contact, 32 (2.5% and 38 (0.8% cases; intravenous drug use, 17 (1.3% and 33 (0.7% cases; bisexual contact, 10 (0.8% and 14 (0.3% cases; unknown, 334 (26.0% and 3119 (65.5% cases. The number of HIV infections transmitted by blood or blood products transfusion declined to zero by year 2001 and all such infections occurred due to transfusions administered before 1986. At HIV diagnosis, 4502/6046 (74.5% patients had no symptoms, 787 (13.0% patients had non-AIDS defining

  5. Pneumocystis carinii pneumonia in acquired immunodeficiency syndrome - correlation of high-resolution computed tomography and anatomopathology; Pneumocistose na sindrome da imunodeficiencia adquirida: correlacao da tomografia computadorizada de alta resolucao com a anatomopatologia

    Energy Technology Data Exchange (ETDEWEB)

    Marchiori, Edson; Moreira, Luiza Beatriz [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia]. E-mail: edmarchiori@zipmail.com.br; Capone, Domenico [Universidade do Estado, Rio de Janeiro, RJ (Brazil). Faculdade de Medicina; Moraes, Heleno Pinto de [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Patologia; Pereira, Cyntia Inez Guedes Soares

    2001-12-01

    We present the main findings observed on the high-resolution computed tomography examinations of 15 patients with acquired immunodeficiency syndrome and Pneumocystis carinii pneumonia. The high-resolution computed tomography and autopsy findings of 5 patients were also compared. The most frequently observed high-resolution computed tomography patterns were ground-glass attenuation, consolidation areas, crazy-paving pattern and cysts. Nodules and intralobular reticulation were less frequently observed. Ground-glass attenuation and consolidation areas corresponded to alveolar filling with inflammatory exudate. Thickening of the interlobular septa was due to cell infiltration and edema. One patient presented interlobular reticulation, and the pathology study revealed alveolar septa thickening due to cell infiltration and fibrosis. Nodules observed in one of the patients corresponded to a patchy intra alveolar accumulation of microorganisms and inflammatory cells forming a 'granulomatous' pattern. (author)

  6. Varfarin in the complex treatment of antiphospholipid syndrome: preliminary results

    Directory of Open Access Journals (Sweden)

    T M Reshetnyak

    2003-01-01

    Full Text Available Objective. To assess efficacy and tolerance of varfarin in prophylaxis and therapy of thrombotic complications in patients with antiphospholipid syndrome (APS. Methods. 20 pts with APS (5 male and 15 female received varfarin during a year. 8 of them had primary APS (PAPS and 12 -systemic lupus erythematosus with APS (SLE+APS. 2 other pts (I with SLE+APS and I with PAPS received varfarin during the last 4 years. Nobody from 9 pts with PAPS received corticosteroids (CS. In SLE+APS pts CS dose varied from 4 to 20 mg/day and was not increased during follow up. During the study prothrombine time (PT was examined with thromboplastin ( manufactured by Renam having international sensitivity index 1,2 and international normalization relation (INR. Depending on treatment scheme APS pts were divided into 3 groups. Group 1 included 8 pts with INR<2,0, Group 2-7 with INR >3,0, group 3 - 7 pts with INR<2,0 receiving as additional treatment thrombo ASS 100 mg/day and vasonit from 600 to 1200 mg/day. Results. Two pts with INR = 1,8 had thrombosis recurrence (due to leg thrombophlebitis. There were no recurrences in other groups. 2 from 22 pts had "large" bleedings. "Small" bleedings episodes were noted in 7 from 22 pts. Largely that were subcutaneous bleedings (in 4 pts no more than 5 cm of size. Two pts receiving varfarin with INR 1,8 and 2,4 had renal colic. Conclusion. Our preliminary results prove the necessity of inclusion of varfarin in the treatment of pts with APS and thrombosis but intensive anticoagulant effect is not always desired.

  7. Quality control assessment of human immunodeficiency virus type 2 (HIV-2) viral load quantification assays: results from an international collaboration on HIV-2 infection in 2006

    NARCIS (Netherlands)

    Damond, Florence; Benard, Antoine; Ruelle, Jean; Alabi, Abraham; Kupfer, Bernd; Gomes, Perpetua; Rodes, Berta; Albert, Jan; Böni, Jürg; Garson, Jeremy; Ferns, Bridget; Matheron, Sophie; Chene, Geneviève; Brun-Vezinet, Françoise; Goubau, Patrick; Campa, Pauline; Descamps, Diane; Simon, François; Taieb, Audrey; Autran, Brigitte; Cotten, Matt; Jaye, Assan; Peterson, Kevin; Rowland-Jones, Sarah; Rockstroh, Jürgen; Schwarze-Zander, Carolynne; de Wolf, Frank; van Sighem, Ard; Reiss, Peter; van der Loeff, Maarten Schim; Schutten, Martin; Camacho, Ricardo; Mansinho, Kamal; Antunes, Francisco; Luis, Franca; Valadas, Emilia; Toro, Carlos; Soriano, Vicente; Gyllensten, Katarina; Sonnerborg, Anders; Yilmaz, Aylin; Gisslén, Magnus; Calmy, Alexandra; Rickenbach, Martin; Pillay, Deenan; Tosswill, Jennifer; Anderson, Jane; Chadwick, David

    2008-01-01

    Human immunodeficiency virus type 2 (HIV-2) RNA quantification assays used in nine laboratories of the ACHI(E)V(2E) (A Collaboration on HIV-2 Infection) study group were evaluated. In a blinded experimental design, laboratories quantified three series of aliquots of an HIV-2 subtype A strain, each

  8. Lymphocytes B and primary immunodeficiencies

    Directory of Open Access Journals (Sweden)

    Gabriela López-Herrera

    2016-03-01

    Full Text Available Primary antibody deficiencies represent the most frequent genetic diseases of the immune system and the first to be recognized along immunology history. The antibodies were recognized as part of the humoral immune system long ago, and after immunoglobulin discovery, the first antibody immunodeficiency were recognized and named as “agammaglobulinemia”, followed by the common variable immunodeficiency and the hyper-IgM syndrome. The following discoveries in immunology history made possible the understanding of these pathologies, for example: the discoveries of B cells, pre-B cells, the signaling pathway directed by the antigen receptor and many other cellular and molecular mechanisms. Primary antibody deficiencies have been studied for a long time and the discoveries of new syndromes have been helpful in the understanding of immunological mechanisms that take place in our organism. Then, this manuscript pretends to review the relevant findings in the history of immunology, focused on the B cells and the connection with the description of representative clinical entities of primary antibody deficiencies. The aim of this manuscript is to show to the reader that the generation of scientific knowledge has a direct application in the understanding of the molecular mechanisms that are affected in these diseases.

  9. Efficacy of Antiviral Drugs against Feline Immunodeficiency Virus

    Directory of Open Access Journals (Sweden)

    Katrin Hartmann

    2015-12-01

    Full Text Available Feline immunodeficiency virus (FIV is one of the most common infectious agents affecting cats worldwide .FIV and human immunodeficiency virus (HIV share many properties: both are lifelong persistent lentiviruses that are similar genetically and morphologically and both viruses propagate in T-lymphocytes, macrophages, and neural cells. Experimentally infected cats have measurable immune suppression, which sometimes progresses to an acquired immunodeficiency syndrome. A transient initial state of infection is followed by a long latent stage with low virus replication and absence of clinical signs. In the terminal stage, both viruses can cause severe immunosuppression. Thus, FIV infection in cats has become an important natural model for studying HIV infection in humans, especially for evaluation of antiviral compounds. Of particular importance for chemotherapeutic studies is the close similarity between the reverse transcriptase (RT of FIV and HIV, which results in high in vitro susceptibility of FIV to many RT-targeted antiviral compounds used in the treatment of HIV-infected patients. Thus, the aim of this article is to provide an up-to-date review of studies on antiviral treatment of FIV, focusing on commercially available compounds for human or animal use.

  10. Metabolic syndrome in rheumatoid arthritis: role of adiponectin (preliminary results

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    Yulia Nikolaevna Gorbunova

    2013-01-01

    Full Text Available The clinical value of the disorders and diseases integrated within the metabolic syndrome (MS is in the combination of traditional risk factors for cardiovascular diseases (CVD, which significantly accelerates the development of cardiovascular events (CVEs. The detection rate for MS in patients with rheumatoid arthritis (RA is shown to be higher than in the controls regardless of the diagnostic criteria for MS. At present, there are confusing data on the role of adipokins in RA. Objective: to determine the rate of MS and its components in RA patients and the association of the level of adipokin (adiponectin with the components of MS in relation to the duration of RA. Subjects and methods: The investigation enrolled 69 RA patients divided into two groups: 1 34 patients with early-stage (<2-year RA and 2 35 patients with end-stage (>2-year RA. Results. MS occurred in 12 (17.4% of the 69 patients with RA. There was central (abdominal obesity in 37 (53.6% patients with RA, hypertension in 29 (42%, low high-density cholesterol levels in 20 (29%, hyperglycemia in 11 (15.9%, and hypertriglyceridemia in 10 (14.5%. According to the presence or absence of MS, the patients were divided into 2 groups: 1 12 patients with MS; 2 57 without MS. In the patients with RA and MS, the duration of the disease was shorter; DAS28 and CDAI were higher than in those without MS: 15.4 [7; 24] months versus 51.8 [6; 72] months; DAS28 was 5.8 [4.9; 6.7] scores versus 5.1 [4.5; 5.8] scores; CDAI: 34.8 [21.8; 41.4] scores versus 24.2 [18; 31] scores, respectively (p < 0.05 in all cases. The serum level of adiponectin was lower: 13.1 [5.7; 10.7] ng/ml versus 20.6 [6.9; 30.9] ng/ml in the patients with RA and MS as compared to those without MS; but there were no significant differences. In the patients with early-end RA, the rate of MS was twice higher than that in those with end-stage RA; however, the differences were statistically insignificant (p = 0.1. The components of MS

  11. Allogeneic hematopoietic stem cell transplantation in children with primary immunodeficiencies: Hospital Israelita Albert Einstein experience

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    Juliana Folloni Fernandes

    2011-06-01

    Full Text Available Objective: To report the experience of a tertiary care hospital withallogeneic hematopoietic stem cell transplantation in children withprimary immunodeficiencies. Methods: Seven patients with primaryimmunodeficiencies (severe combined immunodeficiency: n = 2;combined immunodeficiency: n = 1; chronic granulomatous disease:n = 1; hyper-IgM syndrome: n = 2; and IPEX syndrome: n = 1who underwent eight hematopoietic stem cell transplants (HSCTin a single center, from 2007 to 2010, were studied. Results: Twopatients received transplants from HLA-identical siblings; the othersix transplants were done with unrelated donors (bone marrow: n= 1; cord blood: n = 5. All patients had pre-existing infectionsbefore hematopoietic stem cell transplants. One patient receivedonly anti-thymocyte globulin prior to transplant, three transplantswere done with reduced intensity conditioning regimens and fourtransplants were done after myeloablative therapy. Two patientswere not evaluable for engraftment due to early death. Three patientsengrafted, two had primary graft failure and one received a secondtransplant with posterior engraftment. Two patients died of regimenrelated toxicity (hepatic sinusoidal obstruction syndrome; one patient died of progressive respiratory failure due to Parainfluenza infection diagnosed prior to transplant. Four patients are alive and well from 60 days to 14 months after transplant. Conclusion: Patients’ status prior to transplant is the most important risk factor on the outcome of hematopoietic stem cell transplants in the treatment of these diseases. Early diagnosis and the possibility of a faster referral of these patients for treatment in reference centers may substantially improve their survival and quality of life.

  12. Marfan syndrome and cardiovascular complications: results of a family investigation.

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    Sarr, Simon Antoine; Djibrilla, Siddikatou; Aw, Fatou; Bodian, Malick; Babaka, Kana; Ngaidé, Aliou Alassane; Dioum, Momar; Ba, Serigne Abdou

    2017-07-19

    Cardiovascular complications in Marfan syndrome (MFS) make all its seriousness. Taking as a basis the Ghent criteria, we conducted a family screening from an index case. The objective was to describe the clinical characteristics of MFS anomalies and to detect cardiovascular complications in our patients. Six subjects were evaluated. Patients had to be in the same uterine siblings of the index case or be a descendant. The objective was to search for MFS based on the diagnostic criteria of Ghent and, subsequently, detecting cardiovascular damage. The average age was 24 years. The examination revealed three cases of sudden death in a context of chest pain. Five subjects had systemic involvement with a score ≥ 7 that allowed to the diagnosis of MFS. Two patients had simultaneously ectopia lentis and myopia. In terms of cardiovascular damage, there were three cases of dilatation of the aortic root, two cases of aortic dissection of Stanford's type A with severe aortic regurgitation in one case and moderate in the other. There were three patients with moderate mitral regurgitation with a case by valve prolapse. The family screening is crucial in Marfan syndrome. It revealed serious cardiovascular complications including sudden death and aortic dissection.

  13. Strategies for B-cell receptor repertoire analysis in Primary Immunodeficiencies:From severe combined immunodeficiency to common variable immunodeficiency

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    Hanna eIJspeert

    2015-04-01

    Full Text Available The antigen receptor repertoires of B and T cells form the basis of the adaptive immune response. The repertoires should be sufficiently diverse to recognize all possible pathogens. However, careful selection is needed to prevent responses to self or harmless antigens. Limited antigen receptor repertoire diversity leads to immunodeficiency, whereas unselected or misdirected repertoires can result in autoimmunity. The antigen receptor repertoire harbors information about abnormalities in many immunological disorders. Recent developments in next generation sequencing allow the analysis of the antigen receptor repertoire in much greater detail than ever before. Analyzing the antigen receptor repertoire in patients with mutations in genes responsible for the generation of the antigen receptor repertoire will give new insights into repertoire formation and selection. In this perspective we describe strategies and considerations for analysis of the naive and antigen selected B-cell repertoires in primary immunodeficiency (PID patients with a focus on severe combined immunodeficiency (SCID and common variable immunodeficiency (CVID.

  14. Coinfection of Leishmania guyanensis and Human Immunodeficiency Virus–Acquired Immune Deficiency Syndrome: Report of a Case of Disseminated Cutaneous Leishmaniasis in Ecuador

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    Calvopina, Manuel; Aguirre, Cristina; Cevallos, William; Castillo, Alberto; Abbasi, Ibrahim; Warburg, Alon

    2017-01-01

    Reported herein is the first case of Leishmania–human immunodeficiency virus (HIV) coinfection in Ecuador. In Ecuador, HIV infections overlap endemic areas of leishmaniasis. Immunosuppression is a well-established risk factor for developing severe disease. This is a severe case of a 32-year-old man presenting with disseminated pleomorphic ulcers, papules, and cutaneous plaque-like lesions over his whole body. Numerous amastigotes were observed in both skin scrapings and biopsies. The sequence of the cytochrome b gene confirmed the presence of Leishmania guyanensis. The patient was treated but failed to respond to meglumine antimoniate and amphotericin B. Six months later, the patient died due to bacterial septic shock. PMID:28193741

  15. Griscelli syndrome

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    Kumar T

    2006-01-01

    Full Text Available Partial albinism with immunodeficiency is a rare and fatal immunologic disorder characterized by pigmentary dilution and variable cellular immunodeficiency. It was initially described in 1978. Primary abnormalities included silvery grayish sheen to the hair, large pigment agglomerations in hair shafts and an abundance of mature melanosomes in melanocytes, with reduced pigmentation of adjacent keratinocytes. We describe a child with Griscelli syndrome who presented with hepatitis, pancytopenia and silvery hair. The diagnosis was confirmed by microscopic skin and hair examination.

  16. Revisiting the refeeding syndrome: Results of a systematic review.

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    Friedli, Natalie; Stanga, Zeno; Sobotka, Lubos; Culkin, Alison; Kondrup, Jens; Laviano, Alessandro; Mueller, Beat; Schuetz, Philipp

    2017-03-01

    Although described >70 y ago, the refeeding syndrome (RFS) remains understudied with lack of standardized definition and treatment recommendations. The aim of this systematic review was to gather evidence regarding standardized definition, incidence rate and time course of occurrence, association with adverse clinical outcomes, risk factors, and therapeutic strategies to prevent or treat this condition. We searched MEDLINE and EMBASE for interventional and observational clinical trials focusing on RFS, excluding case reports and reviews. We extracted data based on a predefined case report form and assessed bias. Of 2207 potential abstracts, 45 records with a total of 6608 patients were included (3 interventional trials, 16 studies focusing on anorexic patients). Definitions for RFS were highly heterogenous with most studies relying on blood electrolyte disturbances only and others also including clinical symptoms. Incidence rates varied between 0% and 80%, depending on the definition and patient population studied. Occurrence was mostly within the first 72 h of start of nutritional therapy. Most of the risk factors were in accordance with National Institute for Health and Care Excellence guidelines, with older age and enteral feeding being additional factors. There was no strong evidence regarding association of RFS and adverse outcomes, as well as regarding preventive measures and treatment algorithms. This systematic review focusing on RFS found consensus regarding risk factors and timing of occurrence, but wide variations regarding definition, reported incidence rates, preventive measures and treatment recommendations. Further research to fill this gap is urgently needed. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Hyperparathyroidism-jaw tumor syndrome: Results of operative management.

    Science.gov (United States)

    Mehta, Amit; Patel, Dhaval; Rosenberg, Avi; Boufraqech, Myriem; Ellis, Ryan J; Nilubol, Naris; Quezado, Martha M; Marx, Stephen J; Simonds, William F; Kebebew, Electron

    2014-12-01

    Hyperparathyroidism-jaw tumor syndrome (HPT-JT) is a rare, autosomal-dominant disease secondary to germline-inactivating mutations of the tumor suppressor gene HRPT2/CDC73. The aim of the present study was to determine the optimal operative approach to parathyroid disease in patients with HPT-JT. A retrospective analysis of clinical and genetic features, parathyroid operative outcomes, and disease outcomes in 7 unrelated HPT-JT families. Seven families had 5 distinct germline HRPT2/CDC73 mutations. Sixteen affected family members (median age, 30.7 years) were diagnosed with primary hyperparathyroidism (PHPT). Fifteen of the 16 patients underwent preoperative tumor localization studies and uncomplicated bilateral neck exploration at initial operation; all were in biochemical remission at most recent follow-up. Of these patients, 31% had multiglandular involvement; 37.5% of the patients developed parathyroid carcinoma (median overall survival, 8.9 years; median follow-up, 7.4 years). Long-term follow-up showed that 20% of patients had recurrent PHPT. Given the high risk of malignancy and multiglandular involvement in our cohort, we recommend bilateral neck exploration and en bloc resection of parathyroid tumors suspicious for cancer and life-long postoperative follow-up. Published by Elsevier Inc.

  18. [A compound heterozygosity mutation in the interleukin-7 receptor-alpha gene resulted in severe combined immunodeficiency in a Chinese patient].

    Science.gov (United States)

    Zhang, Zhi-yong; Zhao, Xiao-dong; Wang, Mo; Yu, Jie; An, Yun-fei; Yang, Xi-qiang

    2009-09-01

    Mutation in the interleukin-7 receptor-alpha (IL-7R alpha) chain causes a rare type of severe combined immunodeficiency (SCID) with presence of NK cells in the peripheral blood. Here we report the molecular and clinical characterization of a compound heterozygosity mutation in the interleukin-7 receptor-alpha gene that resulted in SCID in a patient firstly from China. A 5 month-old male patient and his parents were enrolled in this study. Since 15 days of age, the patient had had recurrent fever, persistent cough and diarrhea. He was in poor general condition with pyorrhea and ulceration of the BCG scar. His brother died of severe infection at 4 months of age. He was initially diagnosed as SCID according to clinical manifestation and immunological analysis. A panel of SCID candidate genes including IL-2RG, RAG1/RAG2 and IL-7R alpha of patient and his parents were amplified by polymerase chain reaction (PCR) from genomic DNA. Reverse transcription polymerase chain reaction (RT-PCR) was used to amplify the IL-7R alpha transcripts. Sequencing was performed directly on the PCR products forward and reversely. The serum immunoglobulin (Ig) profile was IgG 6867 mg/L (normal range, 3050 - 8870 mg/L); IgM 206 mg/L and IgA 249 mg/L, IgE 2.3 IU/ml (normal range microscope and by culture. The patient had a compound heterozygosity mutation in the IL-7R alpha gene:on one allele, there was a splice-junction mutation in intron 4 (intron 4(+1)G > A), for which his father was a carrier; whereas on the other allele, a nonsense mutation at position 638 in exon 5 with a premature stop codon (638 C > T, R206X) was identified, for which his mother was a carrier. The splice-junction mutation in intron 4 of IL-7R alpha was firstly reported. The IL-7R alpha mRNA expression of the patient was remarkably reduced whereas the parents had relatively normal IL-7R alpha mRNA expression. IL-7R alpha cDNA of the patient was amplified by nested PCR. The PCR products were purified, cloned with a TA

  19. [Behaviour problems of children with Down syndrome in preschool-age - Results from the Heidelberg Down syndrome study].

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    Sarimski, Klaus

    2018-05-01

    We report on the frequency and the correlations of behaviour problems among children with Down syndrome in preschool-age. As part of a longitudinal study 48 mothers of children with Down syndrome completed the German version of the “Strengths and Difficulties Questionnaire” (SDQ-D) and the Parenting Stress Inventory (PSI). The mothers were asked to fill out the questionnaires when the children had a mean age of five years. The results were compared to norms from children with typical development. Thirty per cent of the children with Down syndrome were rated as abnormal. Specifically, mean scores indicating problems with children of the same age and hyperactivity were elevated. A regression analysis predicting the total problem score of the SDQ-D revealed maternal educational level, optimistic attitude, and subjective parental stress at the age of one year and the degree of behavioural abnormalities at the age of three years as significant influential factors. Early intervention for Down syndrome children should include supporting parenting competence and coping skills in order to prevent behaviour problems.

  20. Dynamics of adaptive and innate immunity in patients treated during primary human immunodeficiency virus infection: results from Maraviroc in HIV Acute Infection (MAIN) randomized clinical trial.

    Science.gov (United States)

    Ripa, M; Pogliaghi, M; Chiappetta, S; Galli, L; Pensieroso, S; Cavarelli, M; Scarlatti, G; De Biasi, S; Cossarizza, A; De Battista, D; Malnati, M; Lazzarin, A; Nozza, S; Tambussi, G

    2015-09-01

    We evaluated the dynamics of innate and adaptive immunity in patients treated with combined antiretroviral therapy (cART) during primary human immunodeficiency virus infection (PHI), enrolled in a prospective randomized trial (MAIN, EUDRACT 2008-007004-29). After 48 weeks of cART, we documented a reduction in activated B cells and CD8(+) T cells. Natural killer cell and dendritic cell frequencies were measured and a decrease in CD16(+) CD56(dim) with a reciprocal rise in CD56(high) natural killer cells and an increase in myeloid and plasmacytoid dendritic cells were recorded. In conclusion, 48 weeks of cART during PHI showed significant benefits for both innate and adaptive immunity. Copyright © 2015 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

  1. Cancers related to Immunodeficiencies:Update and perspectives

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    Esmaeil Mortaz

    2016-09-01

    Full Text Available The life span of patients with primary and secondary immunodeficiency is increasing due to recent improvements in therapeutic strategies. Whilst, the incidence of primary immunodeficiencies (PIDs is 1:10.000 births, that of secondary immunodeficiencies is more common and are associated with post transplantation immune dysfunction or with immunosuppressive medication for human immunodeficiency virus (HIV or with human T-cell lymphotropic virus (HTLV infection.After infection, malignancy is the most prevalent cause of death in both children and adults with primary immunodeficiency disorders (PIDs. PIDs more often associated with cancer include common variable immunodeficiency (CVID, Wiskott Aldrich syndrome (WAS, ataxia-telangiectasia (AT and severe combined immunodeficiency (SCID. This suggests that a protective immune response against both infectious non-self (pathogens and malignant self-challenges (cancer exist. The increased incidence of cancer has been attributed to defective elimination of altered or transformed cells and/or defective immunity towards cancer cells. The concept of abberant immune surveillance occurring in PIDs is supported by evidence in mice and from patients undergoing immunosuppression after transplantation. Here, we discuss the importance of PID defects in the development of malignancies, the current limitations associated with molecular pathogenesis of these diseases and emphasize the need for further knowledge of how specific mutations can modulate the immune system to alter immunosurveillance and thereby play a key role in the etiology of malignancies in PID patients.

  2. Prevalence and determinants of metabolic syndrome in Qatar: results from a National Health Survey

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    Al-Thani, Mohamed Hamad; Al-Thani, Al Anoud Mohammed; Cheema, Sohaila; Sheikh, Javaid; Mamtani, Ravinder; Lowenfels, Albert B; Al-Chetachi, Walaa Fattah; Almalki, Badria Ali; Hassan Khalifa, Shamseldin Ali; Haj Bakri, Ahmad Omar; Maisonneuve, Patrick

    2016-01-01

    Objectives To determine optimum measurements for abdominal obesity and to assess the prevalence and determinants of metabolic syndrome in Qatar. Design National health survey. Setting Qatar National STEPwise Survey conducted by the Supreme Council of Health during 2012. Participants 2496 Qatari citizens aged 18–64 representative of the general population. Primary and secondary outcome measures Measure of obesity (body mass index, waist circumference or waist-to-height ratio) that best identified the presence of at least 2 other factors of metabolic syndrome; cut-off values of waist circumference; frequency of metabolic syndrome. Results Waist circumference ≥102 for men and ≥94 cm for women was the best predictor of the presence of other determinants of metabolic syndrome (raised blood pressure, fasting blood glucose, triglycerides and reduced high-density lipoprotein cholesterol). Using these values, we identified 28% of Qataris with metabolic syndrome, which is considerably lower than the estimate of 37% calculated using the International Diabetes Federation (IDF) criteria. Restricting the analysis to participants without known elevated blood pressure, elevated blood sugar or diabetes 16.5% would be classified as having metabolic syndrome. In a multivariable logistic regression analysis, the prevalence of metabolic syndrome increased steadily with age (OR=3.40 (95% CI 2.02 to 5.74), OR=5.66 (3.65 to 8.78), OR=10.2 (5.98 to 17.6) and OR=18.2 (7.01 to 47.5) for those in the age group ‘30–39’, ‘40–49’, ‘50–59’, ‘60–64’ vs ‘18–29’; pQatar. Approximately 28% of adult Qatari citizens satisfy the criteria for metabolic syndrome, which increased significantly with age. Education and physical activity were inversely associated with this syndrome. PMID:27601485

  3. Two siblings with immunodeficiency, facial abnormalities and chromosomal instability without mutation in DNMT3B gene but liability towards malignancy; a new chromatin disorder delineation?

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    Neitzel Heidemarie

    2010-03-01

    Full Text Available Abstract Background ICF syndrome (standing for Immunodeficiency, Centromere instability and Facial anomalies syndrome is a very rare autosomal recessive immune disorder caused by mutations of the gene de novo DNA-methyltransferase 3B (DNMT3B. However, in the literature similar clinical cases without such mutations are reported, as well. Results We report on a family in which the unrelated spouses had two female siblings sharing similar phenotypic features resembling ICF-syndrome, i.e. congenital abnormalities, immunodeficiency, developmental delay and high level of chromosomal instability, including high frequency of centromeric/pericentromeric rearrangements and breaks, chromosomal fragments despiralization or pulverization. However, mutations in DNMT3B could not be detected. Conclusion The discovery of a new so-called 'chromatin disorder' is suggested. Clinical, molecular genetic and cytogenetic characteristics are reported and compared to other 'chromatin disorders'.

  4. Retrospective TREC testing of newborns with Severe Combined Immunodeficiency and other primary immunodeficiency diseases

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    O. Jilkina

    2014-01-01

    Full Text Available In Manitoba, Canada, the overall incidence of Severe Combined Immunodeficiency (SCID is three-fold higher than the national average, with SCID overrepresented in two population groups: Mennonites and First Nations of Northern Cree ancestries. T-cell receptor excision circle (TREC assay is being used increasingly for neonatal screening for SCID in North America. However, the majority of SCID patients in Manitoba are T-cell-positive. Therefore it is likely that the TREC assay will not identify these infants. The goal of this study was to blindly and retrospectively perform TREC analysis in confirmed SCID patients using archived Guthrie cards. Thirteen SCID patients were tested: 5 T-negative SCID (3 with adenosine deaminase deficiency, 1 with CD3δ deficiency, and 1 unclassified and 8 T-positive SCID (5 with zeta chain-associated protein kinase (ZAP70 deficiency and 3 with inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase beta (IKKβ deficiency. As a non-SCID patient group, 5 Primary Immunodeficiency Disease (PID patients were studied: 1 T-negative PID (cartilage-hair hypoplasia and 4 T-positive PID (2 common immune deficiency (CID, 1 Wiskott–Aldrich syndrome, and 1 X-linked lymphoproliferative disease. Both patient groups required hematopoietic stem cell transplantation. In addition, randomly-selected de-identified controls (n = 982 were tested. Results: all T-negative SCID and PID had zero TRECs. Low-TRECs were identified in 2 ZAP70 siblings, 1 CID patient as well as 5 preterm, 1 twin, and 4 de-identified controls. Conclusions: TREC method will identify T-negative SCID and T-negative PID. To identify other SCID babies, newborn screening in Manitoba must include supplemental targeted screening for ethnic-specific mutations.

  5. Use of etanercept to treat toxic epidermal necrolysis in a human immunodeficiency virus-positive patient

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    Yung-Yi Lee

    2013-06-01

    Full Text Available Toxic epidermal necrolysis (TEN is an uncommon and severe cutaneous adverse drug reaction that causes disseminated necrosis of epidermal cells and mucocutaneous detachment. Here, we report the case of a 32-year-old man with human immunodeficiency virus infection who presented with generalized violaceous macules and blister formation 4 days after the administration of mefenamic acid and amoxicillin for a dental procedure. Additional symptoms included oral ulcers and conjunctivitis. Results of skin biopsy were compatible with Stevens–Johnson syndrome (SJS. SJS progressed to TEN within 2 days. Etanercept treatment showed a dramatic improvement in the symptoms of mucocutaneous lesions. To our knowledge, this is the first report on the treatment of TEN using etanercept in a human immunodeficiency virus-positive patient.

  6. Compreendendo o ser familiar de criança com Vírus da Imunodeficiência Humana/ Síndrome da Imunodeficiência Adquirida pelo olhar da complexidade Comprendiendo al familiar de un niño afectado por el Virus de la Inmunodeficiencia Humana/Síndrome de la Inmunodeficiencia Adquirida, bajo la perspectiva de la complejidad Understanding the family member of a child affected by Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome, from the perspective of complexity

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    Hilda Maria Barbosa de Freitas

    2010-10-01

    comprensión que abarca varios factores (biológicos, psicológicos, sociales, espirituales, relacionados con la enfermedad y con el enfermo de una actitud sobre el cuidado que debe ser enfocada en el ser humano - un ser único, complejo y multidimensional.OBJECTIVE: To understand the significance of a family member of a child with the Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome from the perspective of complexity. METHODS: This is a qualitative and exploratory study; data were collected between July and November 2008, through interviews with five family members of children with AIDS, who were sheltered in an institution of support. RESULTS: The meaning of statements were coded and analyzed, resulting in three categories: experiencing the order, disorder and the organization of a family member of a child with AIDS; dialoguing with the assurance and doubt of disclosing the diagnosis to a child with AIDS; and, facing the limits of unknown, decadence, live and death. CONCLUSION: It was revealed that being a family member and caring for a child with AIDS, is a complex process that requires an understanding of multiple factors (such as: biological, psychological, social, spiritual, and the ones related to the disease and the patient and the attitude of caring focused on the human being - a unique being, complex and multidimensional.

  7. Manifestações otológicas em criança com síndrome da imunodeficiência adquirida Otologic manifestations in child with acquired immunodeficiency syndrome

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    Carlos E. B. Rezende

    2004-01-01

    Full Text Available As manifestações otológicas são particularmente comuns em crianças portadoras do Vírus da Imunodeficiência Humana (HIV. A disacusia nestes pacientes pode ser condutiva, principalmente decorrente de otite média ou sensorioneural, pela ação direta do vírus ou pela ação de drogas anti-retrovirais. Neste trabalho, relatamos o caso de uma criança de 10 anos de idade, portadora de HIV, com disacusia e revisamos a literatura.Patients infected with Human immunodeficiency virus (HIV often present with otologic manifestations. The hearing loss may be condutive type, resulting from middle ear disease or sensorineural type due virus action or drug therapy. In this paper we describe and discuss a case of a ten years old child with HIV and otologic manifestations including hearing loss.

  8. Results of Percutaneous Balloon Compression in Trigeminal Pain Syndromes.

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    Grewal, Sanjeet S; Kerezoudis, Panagiotis; Garcia, Oscar; Quinones-Hinojosa, Alfredo; Reimer, Ronald; Wharen, Robert E

    2018-06-01

    To investigate initial pain relief and subsequent recurrence after percutaneous balloon compression (PBC) and describe its association with the nature of trigeminal pain, previous procedures, or other clinical factors. A total of 222 patients with medically refractory trigeminal pain treated with PBC at Mayo Clinic Florida between 1998 and 2017 were enrolled into this study. Patients were divided into those with typical trigeminal neuralgia (TN) and those with atypical trigeminal pain. The postprocedural rate of pain recurrence and associations between patient characteristics and recurrence were studied. One hundred fifty-two patients had TN and 70 patients had atypical pain. At the last follow-up, 158 patients had excellent pain relief, 37 had good pain relief, 11 had fair pain relief, and 16 had poor pain relief. The median duration of follow-up was 31.1 months. Patients with atypical pain were less likely to have an excellent result compared with patients with typical pain (61.4% vs. 82.9%; P < 0.001). Recurrence was observed in 103 patients (46.4%) and was associated with previous procedures (hazard ratio, 1.658; 95% confidence interval, 1.09-2.49; P = 0.017). Other clinical factors were not significant. Our study demonstrates the safety and efficacy of PBC, with 88% of patients pain-free at last follow-up. Patients with atypical pain have worse outcomes, and patients with previous procedures have a higher risk of recurrence. Repeat surgery does not decrease efficacy. We recommend conservative parameter selection at the initial procedure. Copyright © 2018 Elsevier Inc. All rights reserved.

  9. Utilidade da teoria de autocuidado na assistência ao portador do Vírus da Imunodeficiência Humana/ Síndrome da Imunodeficiência Adquirida Utilidad de la teoría del autocuidado en la asistencia al portador del Virus de la Inmunodeficiencia Humana/Síndrome de la Inmunodeficiencia Adquirida Utility of the self-care theory to assist the bearer of Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome

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    Léa Maria Moura Barroso

    2010-01-01

    utilidad de la Teoría de Orem para la práctica, investigación, educación y administración.OBJECTIVE: To analyze, within Orem's general theory, the utility of the Self-care Theory to assist patients who carries the Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome (HIV/AIDS, according to the standard model to analyze theories. METHODS: Literature review conducted in the databases LILACS, BDENF and MEDLINE with the keywords: self-care, Orem and HIV/AIDS. It was utilized the Meleis's analysis model of theories, focusing on the component of usefulness. It was identified a study that used Orem's Theory on patients who carries the HIV/AIDS. RESULTS: The theory proved useful to: guide the systematization of nursing care on patients with HIV/AIDS; build assessment and guidance tools; measure the quality of care; verify models; classify patients; and, as an aid to support education. CONCLUSION: The Meleis model allowed to understand the usefulness of Orem's theory into practice, research, education and administration.

  10. Diagnósticos de enfermagem em pacientes com o Vírus da Imunodeficiência Humana/ Síndrome da Imunodeficiência Adquirida em assistência ambulatorial Diagnósticos de enfermería en pacientes con el Virus de la Inmunodeficiencia Humana/Síndrome de la Inmunodeficiencia Adquirida en asistencia de ambulatorio Nursing diagnoses in patients with Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome in outpatient care

    Directory of Open Access Journals (Sweden)

    Gilmara Holanda da Cunha

    2010-01-01

    identify nursing diagnoses in carriers of the Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome (HIV/AIDS in outpatient care. METHODS: This is a descriptive study performed through the application of a questionnaire based on the theory of Orem Self-care. The sample consisted of 51 patients treated at the clinic of a public hospital in the city of Fortaleza-CE. RESULTS: 17 nursing diagnoses were identified, highlighting: risk of infection, sexual dysfunction, self-care deficit for food and ineffective management of therapeutic regimen. CONCLUSION: The nursing diagnoses obtained identified the main problems presented by the carriers of HIV/AIDS, verifying deficits in self-care and health education. This study contributes to guide the nursing interventions to be appropriated and targeted to existing needs.

  11. Oral Manifestations of Human Immunodeficiency Virus-Infected Patients

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    Atessa Pakfetrat

    2015-01-01

    Full Text Available Background: Oral lesions are among the earliest clinical manifestations of human immunodeficiency (HIV infection and are important in early diagnosis and for monitoring the progression to acquired immunodeficiency syndrome (AIDS. The purpose of this study was to determine the prevalence of oral lesions and their relationship with a number of factors in HIV/AIDS patients attending an HIV center.     Methods: A total of 110 HIV-positive patients were examined to investigate the prevalence of oral lesions according to the criteria established by the European Community Clearing House on Oral Problems Related to HIV Infection. An independent T-test was used for correlation of oral lesions with CD4+ count and a χ2 test was used for analysis of the relationship of co-infection with hepatitis B virus (HBV, sexual contact, route of transmission, history of drug abuse, and history of incarceration.   Results: Most of the cases were male patients (82.7%. The mean age across all participants was 36.2±8.1 years. Rampant carries, severe periodontitis and oral candidiasis were the most notable oral lesions. Oral lesions were more prevalent in patients between 26–35 years of age. There was a significant difference between patients with and without pseudomembranous candidiasis and angular cheilitis according to mean level of CD4+.   Conclusion: The most common oral presentations were severe periodontitis, pseudomembranous candidiasis and xerostomia.

  12. Oral Manifestations of Human Immunodeficiency Virus-Infected Patients

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    Atessa Pakfetrat

    2015-01-01

    Full Text Available Background: Oral lesions are among the earliest clinical manifestations of human immunodeficiency (HIV infection and are important in early diagnosis and for monitoring the progression to acquired immunodeficiency syndrome (AIDS. The purpose of this study was to determine the prevalence of oral lesions and their relationship with a number of factors in HIV/AIDS patients attending an HIV center.     Methods: A total of 110 HIV-positive patients were examined to investigate the prevalence of oral lesions according to the criteria established by the European Community Clearing House on Oral Problems Related to HIV Infection. An independent T-test was used for correlation of oral lesions with CD4+ count and a χ2 test was used for analysis of the relationship of co-infection with hepatitis B virus (HBV, sexual contact, route of transmission, history of drug abuse, and history of incarceration.   Results: Most of the cases were male patients (82.7%. The mean age across all participants was 36.2±8.1 years. Rampant carries, severe periodontitis and oral candidiasis were the most notable oral lesions. Oral lesions were more prevalent in patients between 26–35 years of age. There was a significant difference between patients with and without pseudomembranous candidiasis and angular cheilitis according to mean level of CD4+.   Conclusion: The most common oral presentations were severe periodontitis, pseudomembranous candidiasis and xerostomia. 

  13. Clinical and Immunological Features of Common Variable Immunodeficiency in China

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    Lian-Jun Lin

    2015-01-01

    Full Text Available Background: Common variable immunodeficiency (CVID is one of the most common symptomatic primary immunodeficiency syndromes. The purpose of this article was to broaden our knowledge about CVID for better diagnosis and treatment. Methods: Clinical and immunological features of 40 Chinese patients with CVID were analyzed retrospectively. Results: The median age at onset was 11-year-old (range 4-51 years. The median age at diagnosis was 14.5-year-old (range 5-66 years. The average time of delay in diagnosis was 5.3 years (range 1-41 years. The most common main complaint was fever due to infections (35 cases, 87.5%. Pneumonia (28 cases, 70% was the most common type of infections. Bronchiectasis was present in 6 patients (15%. Autoimmune disease was detected in 6 cases of CVID, and malignancy in 2 cases. The median total serum levels of IgG, IgA, and IgM at diagnosis were 1.07 g/L, 0.07 g/L, and 0.28 g/L, respectively. The percentages of CD3− /CD19 + B-cells were 1%-3.14%. Conclusions: Infection is the most frequent presentation of CVID. Patients with unexplainable infections should receive further examination including serum immunoglobulin (Ig and lymphocyte subset analysis. Regular and sufficient substitution with Ig is recommended.

  14. 2',3'-Dideoxycytidine and human immunodeficiency virus reverse transcriptase

    International Nuclear Information System (INIS)

    Starnes, M.C.

    1988-01-01

    2',3'-Dideoxycytidine (ddCyd) is a candidate for clinical trial in the treatment of Acquired Immunodeficiency Syndrome, as a result of its potent inhibition of Human Immunodeficiency Virus (HIV) replication. The cellular metabolism and cytotoxicity of ddCyd are, as well as the interaction of ddCTP and other nucleotide and pyrophosphate analogs with mammalian DNA polymerases and HIV reverse transcriptase (RT). In addition, some structural and functional characteristics of HIV RT are described. 5 μM ddCyd reduced Molt 4 cell division by 50% during a 48 h continuous exposure; however, a 24 h exposure to 0.5 μM ddCyd reduced clonogenic survival by 50%. [ 14 C]-dThd incorporation into DNA was reduced during exposure to ddCyd. Acid-soluble ddCyd metabolites were ddCMP, ddCDP, and ddCTP. Initial ddCyd phosphorylation was catalyzed primarily by cytoplasmic dCyd kinase, and ddCyd was not a substrate for human Cyd-dCyd deaminase. Metabolism of ddCyd was identical in mock and HIV infected H9 cells

  15. Replacement of the V3 domain in the surface subunit of the feline immunodeficiency virus envelope glycoprotein with the equivalent region of a T cell-tropic human immunodeficiency virus type 1 results in a chimeric surface protein that efficiently binds to CXCR4.

    Science.gov (United States)

    González, Silvia A; Falcón, Juan I; Affranchino, José L

    2014-03-01

    Feline immunodeficiency virus (FIV) and the T cell-tropic strains of human immunodeficiency virus type 1 (HIV-1) share the use of the chemokine receptor CXCR4 for cell entry. To study this process further we developed a cell surface binding assay based on the expression of a soluble version of the FIV SU C-terminally tagged with the influenza virus hemagglutinin epitope (HA). The specificity of the assay was demonstrated by the following evidence: (1) the SU-HA protein bound to HeLa cells that express CXCR4 but not to MDCK cells that lack this chemokine receptor; and (2) binding of the SU-HA to HeLa cells was blocked by incubation with the CXCR4 antagonist AMD3100 as well as with the anti-CXCR4 monoclonal antibody (MAb) 12G5. Deletion of the V3 region from the FIV SU glycoprotein abolished its ability to bind CXCR4-expressing cells. Remarkably, substitution of the V3 domain of the FIV SU by the equivalent region of the HIV-1 NL4-3 isolate resulted in efficient cell surface binding of the chimeric SU protein to CXCR4. Moreover, transfection of MDCK cells with a plasmid encoding human CXCR4 allowed the association of the chimeric SU-HA glycoprotein to the transfected cells. Interestingly, while cell binding of the chimeric FIV-HIV SU was inhibited by an anti-HIV-1 V3 MAb, its association with CXCR4 was found to be resistant to AMD3100. Of note, the chimeric FIV-HIV Env glycoprotein was capable of promoting CXCR4-dependent cell-to-cell fusion.

  16. Cardiovascular complications in the acquired immunodeficiency syndrome Complicações cardiovasculares na síndrome de imunodeficiência adquirida

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    Giuseppe Barbaro

    2009-01-01

    Full Text Available The introduction of highly active antiretroviral therapy (HAART has significantly improved the clinical outcome of HIV disease, with increased survival rates. However, the introduction of HAART has generated a contrast in the cardiac manifestations of AIDS. In developed countries, we observed an approximate 30% reduction in the prevalence of HIV-associated cardiomyopathy, possibly related to a reduction of opportunistic infections and myocarditis. In developing countries, however, where the availability of HAART is limited and the pathogenic impact of nutritional factors is significant, we observed an increase of approximately 32% in the prevalence of HIV-associated cardiomyopathy and a related high mortality rate from congestive heart failure. Also, some HAART regimens in developed countries, especially those including protease inhibitors, have been shown to cause, in a high proportion of HIV-infected patients, a iatrogenic metabolic syndrome (HIV-lipodystrophy syndrome.This is associated with an increased risk of atherosclerosis-related cardiovascular events even in young HIV-infected people. A better understanding of the molecular mechanisms responsible for this syndrome will lead to the discovery of new drugs that will reduce cardiovascular risk in HIV-infected patients receiving HAART.A introdução da terapia antitroviral altamente potente (HAART melhorou significativamente a evolução clínica da infecção pelo HIV com um aumento nas taxas de sobrevida. Apesar deste benefício, o uso da HAART gerou contrastes nas manifestações cardíacas da Aids. Nos países desenvolvidos, observou-se uma redução de aproximadamente 30% na prevalência de cardiomiopatia associada ao HIV, possivelmente relacionada à redução das infecções oportunistas e da miocardite. Nos países em desenvolvimento, entretanto, aonde a disponibilidade da HAART é limitada e o impacto patogênico dos fatores nutricionais é significante, observou-se um aumento de

  17. High-dose chemotherapy and immunotherapy in adult Burkitt lymphoma: comparison of results in human immunodeficiency virus-infected and noninfected patients.

    Science.gov (United States)

    Oriol, Albert; Ribera, Josep-Maria; Bergua, Juan; Giménez Mesa, Eduardo; Grande, Carlos; Esteve, Jordi; Brunet, Salut; Moreno, Maria-Jose; Escoda, Lourdes; Hernandez-Rivas, Jesus-Maria; Hoelzer, Dieter

    2008-07-01

    It has been recognized that cure is possible for human immunodeficiency virus (HIV)-infected patients with Burkitt lymphoma/leukemia (BL) if appropriate chemotherapy is used. The introduction of rituximab in BL therapeutic schemes has been scarcely explored. The outcome and toxicity of HIV-positive patients with BL treated in a rituximab and intensive chemotherapy-based trial was evaluated. Thirty-six consecutive patients, 15 to 55 years of age, diagnosed with advanced stage BL were recruited from July 2003 to August 2006, stratified according to HIV infection status and treated with 6 cycles of intensive chemotherapy including 8 doses of rituximab. Nineteen of the patients (53%) were HIV-infected. Their clinical characteristics were comparable to those of the HIV-negative patients. Complete remission (CR) rates were 88% and 84%, respectively, for HIV-negative and -positive patients. Twenty-seven patients (82% and 68%, respectively, for HIV-negative and -positive patients) completed the 6 protocol scheduled cycles. HIV-infected patients presented higher incidences of grade 3-4 mucositis (27% vs 7% of cycles, P = .0005) and severe infectious episodes (26% vs 8%, P = .0025). However, there were no statistically significant differences in 2-year overall survival (82%, 95% confidence interval [CI], 65%-99% and 73%, 95% CI, 54%-92%, respectively) or 2-year disease-free survival (93%, 95% CI, 82%-99% and 87%, 95% CI 72%-99%, respectively). Intensive immunochemotherapy can be administered safely to patients with HIV infection. Despite a higher incidence of severe mucositis and infections the remission and survival rates are comparable to those observed in HIV-negative patients. (Copyright) 2008 American Cancer Society.

  18. Metabolic syndrome after bariatric surgery. Results depending on the technique performed.

    Science.gov (United States)

    Gracia-Solanas, Jose Antonio; Elia, M; Aguilella, V; Ramirez, J M; Martínez, J; Bielsa, M A; Martínez, M

    2011-02-01

    There is a lack of long-term studies for metabolic syndrome after bariatric surgery. Our aim is to show the evolution of the parameters that define the metabolic syndrome after bariatric surgery, up to 10 years of follow-up, in order to clarify what technique gets better results with fewer complications. The IDF definition of the metabolic syndrome was used for this study. One hundred twenty-five morbid obese and superobese patients underwent vertical banded gastroplasty. Two hundred sixty-five morbid obese and superobese patients had biliopancreatic diversion (Scopinaro and modified biliopancreatic diversions), and 152 morbid obese patients underwent laparoscopic gastric bypass. A mean follow-up of up to 7 years was done in all groups. Prior to surgery, metabolic syndrome was diagnosed in 114 patients of Scopinaro group (76%), in 85 patients of modified biliopancreatic diversion group (73.9%), in 81 patients of laparoscopic gastric bypass (53.4%), and in 98 patients of vertical banded gastroplasty (78.4%). When metabolic syndrome parameters were evaluated at 7 years of follow-up, owing to weight gain, these results changed nearby to preoperative values in both laparoscopic gastric bypass and vertical banded gastroplasty groups. According to our results, the best technique to resolve metabolic syndrome is the modified biliopancreatic diversion. Due to its high morbidity, it only must be considered in superobese patients. In obese patients, the laparoscopic gastric bypass may be a less agressive choice, but it should be coupled with lifestyle changes to keep away from the weight gain in the long run. Restrictive procedures may be indicated only in a few well-selected cases.

  19. Basics of primary immunodeficiencies

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    Claudia Hernández-Martínez

    2016-05-01

    Full Text Available Primary immunodeficiencies (PID are a heterogeneous group of inherited disorders, the etiology are the defects in the development or function of the immune system. The principal PID manifestations are the infections in early age, malignancy and diseases of immune dysregulation as autoimmunity and allergy. PIDs are genetics disorders and most of them are inherited as autosomal recessive, also this group of diseases is more prevalent in males and in childhood. The antibody immunodeficiency is the PID more common in adults. The more frequent disorders are the infections in the respiratory tract, abscesses, candidiasis, diarrhea, BCGosis etc. Initial approach included a complete blood count and quantification of immunoglobulins. The delay in diagnosis could be explained due to a perception that the recurrent infections are normal process or think that they are exclusively of childhood. The early diagnosis of PID by primary care physicians is important to opportune treatment and better prognosis.

  20. Pharmacological inhibition of feline immunodeficiency virus (FIV).

    Science.gov (United States)

    Mohammadi, Hakimeh; Bienzle, Dorothee

    2012-05-01

    Feline immunodeficiency virus (FIV) is a member of the retroviridae family of viruses and causes an acquired immunodeficiency syndrome (AIDS) in domestic and non-domestic cats worldwide. Genome organization of FIV and clinical characteristics of the disease caused by the virus are similar to those of human immunodeficiency virus (HIV). Both viruses infect T lymphocytes, monocytes and macrophages, and their replication cycle in infected cells is analogous. Due to marked similarity in genomic organization, virus structure, virus replication and disease pathogenesis of FIV and HIV, infection of cats with FIV is a useful tool to study and develop novel drugs and vaccines for HIV. Anti-retroviral drugs studied extensively in HIV infection have targeted different steps of the virus replication cycle: (1) inhibition of virus entry into susceptible cells at the level of attachment to host cell surface receptors and co-receptors; (2) inhibition of fusion of the virus membrane with the cell membrane; (3) blockade of reverse transcription of viral genomic RNA; (4) interruption of nuclear translocation and viral DNA integration into host genomes; (5) prevention of viral transcript processing and nuclear export; and (6) inhibition of virion assembly and maturation. Despite much success of anti-retroviral therapy slowing disease progression in people, similar therapy has not been thoroughly investigated in cats. In this article we review current pharmacological approaches and novel targets for anti-lentiviral therapy, and critically assess potentially suitable applications against FIV infection in cats.

  1. Lynch syndrome patients' views of and preferences for return of results following whole exome sequencing.

    Science.gov (United States)

    Hitch, Kelly; Joseph, Galen; Guiltinan, Jenna; Kianmahd, Jessica; Youngblom, Janey; Blanco, Amie

    2014-08-01

    Whole exome sequencing (WES) uses next generation sequencing technology to provide information on nearly all functional, protein-coding regions in an individual's genome. Due to the vast amount of information and incidental findings that can be generated from this technology, patient preferences must be investigated to help clinicians consent and return results to patients. Patients (n = 19) who were previously clinically diagnosed with Lynch syndrome, but received uninformative negative Lynch syndrome genetic results through traditional molecular testing methods participated in semi-structured interviews after WES testing but before return of results to explore their views of WES and preferences for return of results. Analyses of interview results found that nearly all participants believed that the benefits of receiving all possible results generated from WES outweighed the undesirable effects. The majority of participants conveyed that relative to coping with a cancer diagnosis, information generated from WES would be manageable. Importantly, participants' experience with Lynch syndrome influenced their notions of genetic determinism, tolerance for uncertain results, and family communication plans. Participants would prefer to receive WES results in person from a genetic counselor or medical geneticist so that an expert could help explain the meaning and implications of the potentially large quantity and range of complicated results. These results underscore the need to study various populations with regard to the clinical use of WES in order to effectively and empathetically communicate the possible implications of this new technology and return results.

  2. Recurrent Infections May Signal Immunodeficiencies

    Science.gov (United States)

    American Academy of Allergy Asthma & Immunology Menu Search Main navigation Skip to content Conditions & Treatments Allergies Asthma Primary Immunodeficiency Disease Related Conditions Drug Guide Conditions Dictionary Just ...

  3. Approach to a child with primary immunodeficiency made simple

    Directory of Open Access Journals (Sweden)

    Dhrubajyoti Sharma

    2017-01-01

    Full Text Available Primary immunodeficiency disorders (PIDs are a group of disorders affecting the capability to fight against infection. These include defects in T cells and B cells affecting cell-mediated and humoral immunity, respectively, combined humoral and cell-mediated immunodeficiency, defects in phagocytosis, complement defects, and defects in cytokine or cytokine signalling pathways which are detrimental for immune function. Depending upon the type and severity, age at onset of symptoms can vary from neonatal period to late childhood. Clinically, this group of disorders can involve any organ system of an individual such as respiratory system, gastrointestinal system, skin and mucous membrane, bone and joints, endocrine organs, and nervous system. Common dermatological manifestations include eczema, warts, molluscum contagiosum, mucocutaneous candidiasis, recurrent nonhealing ulcers, skin abscesses, erythroderma, petechiae, and nail changes. The common skin manifestations of various PIDs include eczema (seen in Wiskott–Aldrich syndrome and autosomal dominant hyper IgE syndrome; erythroderma (in Omen syndrome; viral warts or molluscum contagiosum (in autosomal recessive hyper IgE syndrome; chronic mucocutaneous candidiasis (in hyper IgE syndrome, autoimmune polyendocrinopathy candidiasis ectodermal dysplasia syndrome, Th17 cell defects; recurrent nonhealing ulcers (in leucocyte adhesion defect; skin abscesses (in antibody defects, hyper IgE syndrome, and chronic granulomatous disease; petechial or purpuric spots (in Wiskott–Aldrich syndrome.

  4. Capabilities of gravitational surgery for improvement of treatment results in patients with diabetic foot syndrome

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    M B Akhmedov

    2018-06-01

    Full Text Available Aim. Improvement of complex treatment results in patients with diabetic foot syndrome by introducing methods of gravitational surgery and α-lipoic acid. Methods. The results of treatment were analyzed for 558 patients with diabetic foot syndrome treated in Scientific Centre of Surgery named after M.A. Topchubashov (Baku, Azerbaijan from 1988 to 2015. The age varied from 28 to 83 years. The patients included 416 men and 142 women. The control group included 90 patients who at the perioperative period underwent basic therapy including antibiotics, anticoagulants, antiaggregants, dextrans, angioprotectors, spasmolytics, corticosteroids, narcotic and non-narcotic analgesics. The study group included 468 patients, along with traditional therapy receiving efferent methods (plasmapheresis, ultraviolet blood irradiation, ozone therapy and α-lipoic acid. 282 patients of the study group received outpatient treatment and 186 - complex inpatient surgical treatment. A comparative evaluation of the results was performed separately in three groups: angiopathy, neuropathy, angioneuropathy. The results were evaluated by clinical and instrumental examinations before and after treatment (6, 12, 60 months and more. Results. In the study group a satisfactory result of treatment was registered in 85.5% of patients, in the control group - in 62.2%, unsatisfactory in 14.5 and 37.8% of patients, respectively (p=0.046. Conclusion. The use of efferent methods and α-lipoic acid provided prompt elimination of numerous pathogenetic disorders observed in diabetes mellitus, decrease of amputation frequency and improvement of complex surgical treatment results in patients with diabetic foot syndrome.

  5. Learning about Severe Combined Immunodeficiency (SCID)

    Science.gov (United States)

    ... immunodeficiency From The Journal of Allergy and Clinical Immunology Learning About Severe Combined Immunodeficiency (SCID) What is ... immunodeficiency From The Journal of Allergy and Clinical Immunology Get Email Updates Privacy Copyright Contact Accessibility Plug- ...

  6. Defective IL-10 signaling in hyper-IgE syndrome results in impaired generation of tolerogenic dendritic cells and induced regulatory T cells

    Science.gov (United States)

    Saito, Masako; Nagasawa, Masayuki; Takada, Hidetoshi; Hara, Toshiro; Tsuchiya, Shigeru; Agematsu, Kazunaga; Yamada, Masafumi; Kawamura, Nobuaki; Ariga, Tadashi; Tsuge, Ikuya; Nonoyama, Shigeaki; Karasuyama, Hajime

    2011-01-01

    Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by recurrent staphylococcal infections and atopic dermatitis associated with elevated serum IgE levels. Although defective differentiation of IL-17–producing CD4+ T cells (Th17) partly accounts for the susceptibility to staphylococcal skin abscesses and pneumonia, the pathogenesis of atopic manifestations in HIES still remains an enigma. In this study, we examined the differentiation and function of Th1, Th2, regulatory T cells (Treg cells), and dendritic cells (DCs) in HIES patients carrying either STAT3 or TYK2 mutations. Although the in vitro differentiation of Th1 and Th2 cells and the number and function of Treg cells in the peripheral blood were normal in HIES patients with STAT3 mutations, primary and monocyte-derived DCs showed defective responses to IL-10 and thus failed to become tolerogenic. When treated with IL-10, patient DCs showed impaired up-regulation of inhibitory molecules on their surface, including PD-L1 and ILT-4, compared with control DCs. Moreover, IL-10–treated DCs from patients displayed impaired ability to induce the differentiation of naive CD4+ T cells to FOXP3+ induced Treg cells (iTreg cells). These results suggest that the defective generation of IL-10–induced tolerogenic DCs and iTreg cells may contribute to inflammatory changes in HIES. PMID:21300911

  7. Family and other social factors contributing to differences in human immunodeficiency virus infection between South Africa and Bangladesh

    NARCIS (Netherlands)

    van Ginneken, J.K.S.

    2008-01-01

    The objective of this study is to draw attention to the importance of social, cultural, economic and political factors as causes of the human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) epidemic in South Africa by comparing the current situation in this country with

  8. Immune reconstitution inflammatory syndrome due to Mycobacterium avium complex successfully followed up using 18 F-fluorodeoxyglucose positron emission tomography-computed tomography in a patient with human immunodeficiency virus infection: A case report

    International Nuclear Information System (INIS)

    Namkoong, Ho; Fujiwara, Hiroshi; Ishii, Makoto; Yagi, Kazuma; Haraguchi, Mizuha; Matsusaka, Masako; Suzuki, Shoji; Asakura, Takanori; Asami, Takahiro; Saito, Fumitake; Fukunaga, Koichi; Tasaka, Sadatomo; Betsuyaku, Tomoko; Hasegawa, Naoki

    2015-01-01

    In human immunodeficiency virus (HIV)-infected patients, immune reconstitution inflammatory syndrome (IRIS) due to nontuberculous mycobacteria (NTM) infection is one of the most difficult types of IRIS to manage. 18 F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F-FDG PET/CT) has been suggested as a useful tool for evaluating the inflammatory status of HIV-infected patients. We present the first case of Mycobacterium avium complex (MAC)-associated IRIS (MAC-IRIS) that was successfully followed up using 18 F-FDG PET/CT. A 44-year-old homosexual Japanese man was referred to our hospital with fever and dyspnea. He was diagnosed with Pneumocystis jiroveci pneumonia and found to be HIV positive. After the initiation of combined antiretroviral therapy (cART), the patient’s mediastinal and bilateral hilar lymphadenopathy gradually enlarged, and bilateral infiltrates appeared in the upper lung fields. 18 F-FDG PET/CT was performed five months after the initiation of cART and showed intense accumulation of fluorodeoxyglucose (FDG) corresponding to the lesions of infiltration as well as the mediastinal and bilateral hilar lymphadenopathy. A bronchial wash culture and pathology findings led to a diagnosis of MAC-IRIS. Anti-mycobacterial chemotherapy with rifampicin, ethambutol, clarithromycin, and levofloxacin was started. One year after the chemotherapy was initiated, there was a significant reduction in FDG uptake in the area of the lesions except in the mediastinal lymph node. This implied incomplete resolution of the MAC-IRIS-related inflammation. Anti-mycobacterial chemotherapy was continued because of the residual lesion. To date, the patient has not experienced a recurrence of MAC-IRIS, a period of nine months. We present a case of MAC-IRIS in an HIV-infected patient whose disease activity was successfully followed up using 18 F-FDG PET/CT. Our data suggest that 18 F-FDG PET/CT is useful for evaluating the disease activity of NTM-IRIS and

  9. Virus-host interaction in feline immunodeficiency virus (FIV) infection.

    Science.gov (United States)

    Taniwaki, Sueli Akemi; Figueiredo, Andreza Soriano; Araujo, João Pessoa

    2013-12-01

    Feline immunodeficiency virus (FIV) infection has been the focus of several studies because this virus exhibits genetic and pathogenic characteristics that are similar to those of the human immunodeficiency virus (HIV). FIV causes acquired immunodeficiency syndrome (AIDS) in cats, nevertheless, a large fraction of infected cats remain asymptomatic throughout life despite of persistent chronic infection. This slow disease progression may be due to the presence of factors that are involved in the natural resistance to infection and the immune response that is mounted by the animals, as well as due to the adaptation of the virus to the host. Therefore, the study of virus-host interaction is essential to the understanding of the different patterns of disease course and the virus persistence in the host, and to help with the development of effective vaccines and perhaps the cure of FIV and HIV infections. Copyright © 2013 Elsevier Ltd. All rights reserved.

  10. Treating metabolic syndrome's metaflammation with low level light therapy: preliminary results

    Science.gov (United States)

    Yoshimura, Tania M.; Kato, Ilka T.; Deana, Alessandro M.; Ribeiro, Martha S.

    2014-02-01

    Metabolic syndrome comprises a constellation of morbidities such as insulin resistance, hyperinsulinemia, atherogenic dyslipidemia, dysglycemia and obesity (especially abdominal). Metabolic alterations are observed in major insulin target organs, increasing the risk of cardiovascular diseases, type-2 diabetes and therefore mortality. Tissue alterations are characterized by immune cells infiltrates (especially activated macrophages). Released inflammatory mediators such as TNF-α induce chronic inflammation in subjects with metabolic syndrome, since inflammatory pathways are activated in the neighboring cells. The intra-abdominal adipose tissue appears to be of particular importance in the onset of the inflammatory state, and strategies contributing to modulate the inflammatory process within this adipose tissue can mitigate the metabolic syndrome consequences. Considering the low level light therapy (LLLT) recognized benefits in inflammatory conditions, we hypothesized this therapeutic approach could promote positive effects in modulating the inflammatory state of metabolic syndrome. That being the scope of this study, male C57BL/6 mice were submitted to a high-fat/high-fructose diet among 8 weeks to induce metabolic syndrome. Animals were then irradiated on the abdominal region during 21 days using an 850 nm LED (6 sessions, 300 seconds per session, 60 mW output power, ~6 J/cm2 fluence, ~19 mW/cm2 fluence rate). Before and during treatment, blood was sampled either from the retroorbital plexus or from tail puncture for glucose, total cholesterol and triglycerides analysis. So far our results indicate no alterations on these metabolic parameters after LLLT. For further investigations, blood was collected for plasma inflammatory cytokine quantification and fresh ex vivo samples of liver and intra-abdominal adipose tissue were harvested for immunohistochemistry purposes.

  11. Sarcoma de Kaposi relacionado à síndrome da imunodeficiência adquirida: características do comprometimento hepático na tomografia computadorizada e na ressonância magnética Kaposi sarcoma related to acquired immunodeficiency syndrome: hepatic findings on computed tomography and magnetic resonance imaging

    Directory of Open Access Journals (Sweden)

    Daniel Nobrega da Costa

    2008-04-01

    Full Text Available Sarcoma de Kaposi é uma neoplasia associada a condições de imunossupressão que acomete os vasos linfáticos e sanguíneos. É a neoplasia intra-hepática mais comum na síndrome da imunodeficiência adquirida. A tomografia computadorizada e a ressonância magnética revelam múltiplos pequenos nódulos, proeminência e realce dos planos periportais, devido à presença de tecido neoplásico. Os autores descrevem um caso de paciente masculino, de 47 anos de idade, com síndrome da imunodeficiência adquirida e sarcoma de Kaposi disseminado.Kaposi sarcoma is a neoplasm associated with immunosuppressive conditions, and involving blood and lymphatic vessels. It is the most frequent intrahepatic neoplasm in patients with acquired immunodeficiency syndrome. Computed tomography and magnetic resonance imaging demonstrate multiple small nodules, prominence and contrast-enhancement of periportal branches due to the presence of the neoplastic tissue. The authors report a case of a 47-year-old male patient with acquired immunodeficiency syndrome presenting disseminated Kaposi sarcoma.

  12. Mapping the x-linked lymphoproliferative syndrome

    International Nuclear Information System (INIS)

    Skare, J.C.; Milunsky, A.; Byron, K.S.; Sullivan, J.L.

    1987-01-01

    The X-linked lymphoproliferative syndrome is triggered by Epstein-Barr virus infection and results in fatal mononucleosis, immunodeficiency, and lymphoproliferative disorders. This study shows that the mutation responsible for X-linked lymphoproliferative syndrome is genetically linked to a restriction fragment length polymorphism detected with the DXS42 probe (from Xq24-q27). The most likely recombination frequency between the loci is 4%, and the associated logarithm of the odds is 5.26. Haplotype analysis using flanking restriction fragment length polymorphism markers indicates that the locus for X-linked lymphoproliferative syndrome is distal to probe DXS42 but proximal to probe DXS99 (from Xq26-q27). It is now possible to predict which members of a family with X-linked lymphoproliferative syndrome are carrier females and to diagnose the syndrome prenatally

  13. Mapping the x-linked lymphoproliferative syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Skare, J.C.; Milunsky, A.; Byron, K.S.; Sullivan, J.L.

    1987-04-01

    The X-linked lymphoproliferative syndrome is triggered by Epstein-Barr virus infection and results in fatal mononucleosis, immunodeficiency, and lymphoproliferative disorders. This study shows that the mutation responsible for X-linked lymphoproliferative syndrome is genetically linked to a restriction fragment length polymorphism detected with the DXS42 probe (from Xq24-q27). The most likely recombination frequency between the loci is 4%, and the associated logarithm of the odds is 5.26. Haplotype analysis using flanking restriction fragment length polymorphism markers indicates that the locus for X-linked lymphoproliferative syndrome is distal to probe DXS42 but proximal to probe DXS99 (from Xq26-q27). It is now possible to predict which members of a family with X-linked lymphoproliferative syndrome are carrier females and to diagnose the syndrome prenatally.

  14. Early and long-term results of a valve-sparing operation for Marfan syndrome.

    Science.gov (United States)

    Birks, E J; Webb, C; Child, A; Radley-Smith, R; Yacoub, M H

    1999-11-09

    We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome. Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63. 6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0. Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases.

  15. Prenatal diagnosis of Wolf-Hirschhorn syndrome: Ultrasonography and molecular karyotyping results.

    Science.gov (United States)

    Zhen, Li; Fan, Shu-Shu; Huang, Lv-Yin; Pan, Min; Han, Jin; Yang, Xin; Li, Dong-Zhi

    2018-03-31

    To present the experience on prenatal diagnosis of Wolf-Hirschhorn syndrome (WHS) to further delineate the fetal presentation of this syndrome. This was a retrospective analysis of ten pregnancies with fetal WHS identified by chromosomal microarray (CMA). Clinical data were reviewed for these cases, including maternal demographics, indications for invasive testing, sonographic findings, CMA results and pregnancy outcomes. Three cases were diagnosed at the first trimester because of an increased NT or cystic hygroma. The remaining seven cases were identified at late gestation for abnormal ultrasound findings. CMA revealed 4p deletions to be terminal in all of the ten cases. Deletion sizes ranged from 2.05 to 19.02 Mb. Prenatal findings such as increased NT, severe and early onset intrauterine growth retardation, and renal dysplasia or oligohydramnios should warrant the diagnosis of WHS and invasive testing using CMA. Copyright © 2018 Elsevier B.V. All rights reserved.

  16. Fanconi syndrome

    Science.gov (United States)

    De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

  17. Results of steroid-based therapy for the hepatitis C-autoimmune hepatitis overlap syndrome.

    Science.gov (United States)

    Schiano, T D; Te, H S; Thomas, R M; Hussain, H; Bond, K; Black, M

    2001-10-01

    Overlap syndromes in which persons manifest clinical, histological, or immunological features of both hepatitis C infection and autoimmune hepatitis are well described. The discordant forms of treatment for hepatitis C and autoimmune hepatitis have made medical management of these patients difficult. We report our experience in using corticosteroids as first line therapy for the hepatitis C-autoimmune hepatitis overlap syndrome. Seven patients with this overlap syndrome (diagnosis based on the presence of serum hepatitis C antibody by RIBA and serum hepatitis C RNA by polymerase chain reaction, and serum hypergammaglobulinemia, elevated ANA or ASMA titers, or histological findings consistent with autoimmune hepatitis) were treated with prednisone with or without azathioprine or cyclosporine, and followed for a median duration of 44.5 months. Five patients (71%) showed improvement of median serum ALT level from 162 U/L to 38 U/L (p = 0.04) and median serum gamma-globulin from 2.1 g/dl to 1.4 g/dl (p = 0.04) by 6 months of therapy. The mean modified histological activity index score also decreased from 11.4 +/- 2.5 to 6.6 +/- 2.6 (p = 0.04) by at least 1 yr of therapy. One patient discontinued prednisone while taking azathioprine and experienced a rebound elevation of serum ALT that did not respond to retreatment with prednisone. Antiviral therapy was subsequently administered and resulted in biochemical and virologic response. Hepatitis C virus RNA remained detectable in all other patients. Corticosteroids are beneficial as a first line therapy for some patients with the hepatitis C-autoimmune overlap syndrome, resulting in appreciable biochemical and histological response but without viral eradication.

  18. Surgical treatment results of hand deformities in patients with Apert syndrome

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    Ufuk Nalbantoglu

    2015-12-01

    Results: The mean age at the first operation was 2.7 years and the mean number of operations was 3 per patient. No patient developed graft-flap necrosis and no patients required amputations. All patients were able to perform grasping and pinching functions and families were satisfied with the cosmetic results. Conclusion: Using a two-stage surgical protocol, achieving satisfactory results with a minimal number of operations is possible in patients with Apert Syndrome. [Hand Microsurg 2015; 4(3.000: 53-57

  19. The acquired immunodeficiency syndrome (AIDS). 4

    International Nuclear Information System (INIS)

    Witt, C.

    1987-01-01

    The diagnostic relevance of bronchological examination methods in addition to lung function and X-ray thorax diagnostics as well as 67 Ga thorax scintigraphy in AIDS patients suffering from pneumocystis carinii pneumonia are discussed. Data on the diagnostic strategy regarding pneumological conditions as well as in the out-patient care of HIV-infected and of AIDS patients are presented. (author)

  20. Wle behoort Acquired immunodeficiency syndrome (AIDS)

    African Journals Online (AJOL)

    1983-01-22

    Jan 22, 1983 ... most at risk, the male homosexuals and the drug abusers. Two things should be ... has been clouded by the acceptance of the concept of being explored ... appetite, weight loss, extreme fatigue and enlargement of lymph nodes. ... at risk, the most important task of general practitioners is to allay the fears.

  1. Effects of acupuncture for chronic pelvic pain syndrome with intrapelvic venous congestion. Preliminary results

    International Nuclear Information System (INIS)

    Honjo, Hisashi; Kamoi, Kazumi; Naya, Yoshio; Ukimura, Osamu; Kojima, Munekado; Miki, Tsuneharu; Kitakoji, Hiroshi

    2004-01-01

    The present study was designed to reveal the usefulness of acupuncture for chronic pelvic pain syndrome with intrapelvic venous congestion as evaluated by symptom scores, transrectal ultrasonography (TRUS) and magnetic resonance (MR) venography. Ten male patients suffering from non-inflammatory chronic pelvic pain syndrome (National Institutes of Health (NIH) category IIIB) with intrapelvic venous congestion were treated using acupuncture. Eight patients had previously received pharmacotherapy, which was unsuccessful. Acupuncture was performed using disposable stainless steel needles, which were inserted into the bilateral BL-33 points and rotated manually for 10 min. The treatment was repeated every week for 5 weeks without other therapeutic maneuvers. Results from TRUS and MR venography, as well as clinical symptoms based on the NIH chronic prostatitis symptom index (NIH-CPSI) and the international prostate symptom score (IPSS), were compared before and after the treatment. No side-effects were recognized throughout the treatment period. The average pain and quality of life (QOL) scores of the NIH-CPSI 1 week after the 5th acupuncture treatment decreased significantly (P<0.05 and P<0.01, respectively) compared with the baseline. The maximum width of the sonolucent zone 1 week after the 5th treatment also decreased significantly (P<0.01, compared with the baseline). Intrapelvic venous congestion demonstrated by MR venography was significantly improved in four patients. This study provided novel information concerning the therapeutic effects of acupuncture on non-inflammatory chronic pelvic pain syndrome. (author)

  2. Is Neurofibromatosis Type 1-Noonan Syndrome a Phenotypic Result of Combined Genetic and Epigenetic Factors?

    Science.gov (United States)

    Yapijakis, Christos; Pachis, Nikos; Natsis, Stavros; Voumvourakis, Costas

    2016-01-01

    Neurofibromatosis 1-Noonan syndrome (NFNS) presents combined characteristics of both autosomal dominant disorders: NF1 and Noonan syndrome (NS). The genes causing NF1 and NS are located on different chromosomes, making it uncertain whether NFNS is a separate entity as previously suggested, or rather a clinical variation. We present a four-membered Greek family. The father was diagnosed with familial NF1 and the mother with generalized epilepsy, being under hydantoin treatment since the age of 18 years. Their two male children exhibited NFNS characteristics. The father and his sons shared R1947X mutation in the NF1 gene. The two children with NFNS phenotype presented with NF1 signs inherited from their father and fetal hydantoin syndrome-like phenotype due to exposure to that anticonvulsant during fetal development. The NFNS phenotype may be the result of both a genetic factor (mutation in the NF1 gene) and an epigenetic/environmental factor (e.g. hydantoin). Copyright © 2016 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  3. The impact of Down syndrome screening on Taiwanese Down syndrome births: a nationwide retrospective study and a screening result from a single medical centre.

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    Shin-Yu Lin

    Full Text Available A retrospective analysis of the Taiwanese National Birth Defect Registration and Notification System was conducted in order to determine the live birth- and stillbirth rates in infants with Down syndrome, trisomy 18, trisomy 13 and Turner syndrome between 2001 and 2010. The objective was to investigate the impact of Down syndrome screening on the Taiwanese Down syndrome live birth rate. In addition, the results of first-trimester Down syndrome screening between 2006 and 2011, and of second-trimester quadruple testing between 2008 and 2011, were obtained from the National Taiwan University Hospital. All Taiwanese infants born between 2001 and 2010 were included in the first part of the analysis, and women receiving first-trimester Down syndrome screening or second-trimester quadruple testing from the National Taiwan University Hospital were included in the second part. The live birth rate of infants with Down syndrome, per 100 000 live births, decreased from 22.28 in 2001 to 7.79 in 2010. The ratio of liveborn DS to total DS was 48.74% in 2001, and then decreased to 25.88% in 2006, when first-trimester screening was widely introduced in Taiwan. This ratio dropped to 20.64% in 2008, when the second-trimester quadruple test was implemented. The overall positive rate in first-trimester screening in the National Taiwan University Hospital was 3.1%, with a Down syndrome detection rate of 100%; the quadruple test had values of 9.0% and 75%, respectively. The use of first-trimester screening and the second-trimester quadruple test may be responsible for the marked decrease in the Taiwanese Down syndrome live birth rate observed between 2001 and 2010.

  4. Long-term results of aortic valve-sparing operations in patients with Marfan syndrome.

    Science.gov (United States)

    David, Tirone E; Armstrong, Sue; Maganti, Manjula; Colman, Jack; Bradley, Timothy J

    2009-10-01

    The appropriateness of aortic valve-sparing operations in patients with Marfan syndrome has been questioned. This study examines the long-term results of these operations in patients with Marfan syndrome. From 1988 to 2006, 103 consecutive patients with Marfan syndrome (mean age, 37 +/- 12 years) and aortic root aneurysm had aortic valve-sparing operations. Emergency surgery was performed in 11 patients: 8 for acute type A aortic dissection and 3 for unexplained persistent chest pain. Fourteen patients also had mitral valve surgery. The technique of aortic valve reimplantation was used in 77 patients, and aortic root remodeling was used in 26 patients. Patients were followed prospectively and underwent annual echocardiographic studies. The mean follow-up was 7.3 +/- 4.2 years and 100% complete. There was 1 operative death and 5 late deaths. Four of the 6 deaths were due to complications of aortic dissections. The patients' survival at 15 years was 87.2% compared with 95.6% for the general population of Ontario matched for age and sex. Seven patients had important aortic insufficiency: 4 mild to moderate, 2 moderate, and 1 moderate to severe. Freedom from greater than mild aortic insufficiency at 15 years was 79.2%. Three patients, all after aortic root remodeling, had aortic valve replacement, 2 for aortic insufficiency and 1 for endocarditis. At the most recent follow-up, 97 patients were alive: 86 were in functional class I, and 11 were in functional class II. Aortic valve-sparing operations provided excellent clinical outcomes in this series of patients with Marfan syndrome. Postoperatively, complications of aortic dissections were the leading cause of death.

  5. 5 YEAR PHYSIOTHERAPY AND REHABILITATION RESULTS OF THE PATIENT WITH MILLER FISHER SYNDROME

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    Bihter AKINOĞLU

    2016-08-01

    Full Text Available Background: Miller-Fisher syndrome (MFS is characterized by gait ataxia, external ophtalmoplegia and areflexia and thought as an uncommon variant of Guillain Barre syndrome. Miller-Fisher syndrome is observed in about 5-8% of all Guillain Barre syndrome (GBS cases. In MFS patients, spontaneous improvement was observed in the first 3 months and these improvements were started by the 2nd week. Methods: This case was referred to physiotherapy and rehabilitation program at the 4th week since the appropriate medical treatments were unsuccessful after the attack. The patient was evaluated generally before physiotherapy program, and muscle length, strength loss, deep tendon reflexes, postural impairments and daily difficult activities and positions were assessed. Besides, the desired daily activities were identified by The Canadian Occupational Performance Measure (COPM. Treatment program was adjusted according to the patient and changes during treatment period were observed. Physiotherapy program included classical physiotherapy methods: posture correction, correction of short muscles, muscle strengthening, flexibility increase, balance/coordination, sitting and standing functions and walking improvement and climbing up stairs. Results: After the treatment, lower extremity muscle shortness decreased and muscle strength, standing on one foot duration, independent walk speed increased in time. Before treatment, he could not climbing upstairs, but it was achieved 1 year after the treatment. Berg balance score increased in time and his most desired activities by COPM (10/10 were could be performed after the treatment. Conclusion: The case improved with physiotherapy and rehabilitation program gradually with years.In the treatment of MFS patients, physiotherapy and rehabilitation being part of the treatment will be useful.

  6. The Puzzling Role of CXCR4 in Human Immunodeficiency Virus Infection

    OpenAIRE

    Elisa Vicenzi, Pietro Liò, Guido Poli

    2013-01-01

    The human immunodeficiency virus type-1 (HIV-1) is the etiological agent of the acquired immunodeficiency syndrome (AIDS), a disease highly lethal in the absence of combination antiretroviral therapy. HIV infects CD4+ cells of the immune system (T cells, monocyte-macrophages and dendritic cells) via interaction with a universal primary receptor, the CD4 molecule, followed by a mandatory interaction with a second receptor (co-receptor) belonging to the chemokine receptor family. Apart from som...

  7. Lesões do trato gastrointestinal na síndrome da imunodeficiência adquirida: [title]estudo de 45 necrópsias consecutivas Gastrointestinal tract lesions in the acquired immunodeficiency syndrome: study of forty five consecutive necropsies

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    Maria Goretti Freire de Carvalho

    1994-09-01

    Full Text Available Com objetivo de estudar afrequência e etiologia das lesões do tubo digestivo na Síndrome da Imunodeficiência Adquirida (AIDS, foram analisadas retrospectivamente 45 necrópsias consecutivas de pacientes adultos portadores do vírus da AIDS. Lesões macroscópicas e cortes histológicos de amostras da boca à região anal foram estudados, sendo as lâminas coradas por HE, métodos histoquímicos ou imunohistoquímicos. Trinta e sete (82,3 %pacientes apresentaram lesões no tubo digestivo. O local mais freqüente de lesões foi a boca (73,3%, seguido do cólon (55,5%. Lesões múltiplas foram identificadas em 17 (37,7% casos. O diagnóstico mais prevalente foi infecção pelo citomegalovírus (35,7% identificado predominantemente no cólon. Candidíasefoi mais freqüente na boca (26,6% e infecção herpética no esôfago (8,8%. Verificou-se leucoplasiapilosa oral em 16 (35,5% eneoplasias emsete (15,5%. Asneoplasias incluíram quatro sarcomas de Kaposi, dois Carcinomas intramucosos anais e um linfoma gástrico. Os dados do presente estudo confirmam a importância do trato gastrointestinal como sede de alterações patológicas relacionadas à AIDS.This study was designed to evaluate restropectivefy the frequency and etiology of the gastrointestinal (GI lesions in 45 consecutive necropsies of adult patients with Acquired Immunodeficiency Syndrome (AIDS. Gross descriptions and histological sections of the GI tract, from mouth to anus, were reviewed. The slides were H&E stained, and when necessary special stains and immunohistochemicalmethods were also employed. There were lesions in GI tract in 37(82.3% patients; the mouth was the segment mostfrequently involved (73.3% of the cases, followed by the colon (55.5%. Multiple lesions occurred in 17 (37.7% cases. Cytomegalovirus caused colonic lesions in 35.7% of the cases. Candidiasis was observed in 26.6% mainly in the mouth and herpes simplex (8.8% was the important agent of esophageal lesions. Oral

  8. Severe neonatal marfan syndrome resulting from a De Novo 3-bp insertion into the fibrillin gene on chromosome 15

    Energy Technology Data Exchange (ETDEWEB)

    Milewicz, D.M.; Duvic, M. (Univ. of Texas Medical School, Houston, TX (United States))

    1994-03-01

    Severe neonatal Marfan syndrome has features of the Marfan syndrome and congenital contractural arachnodactyly present at birth, along with unique features such as loose, redundant skin and pulmonary emphysema. Since the Marfan syndrome and congenital contractural arachnodactyly are due to mutations in different genes, it has been uncertain whether neonatal Marfan syndrome is due to mutations in the fibrillin gene on chromosome 15 or in another gene. The authors studied an infant with severe neonatal Marfan syndrome. Dermal fibroblasts were metabolically labeled and found to secrete fibrillin inefficiently when compared with control cells. Reverse transcription and amplification of the proband's fibroblast RNA was used to identify a 3-bp insertion between nucleotides 480-481 or 481-482 of the fibrillin cDNA. The insertion maintains the reading frame of the protein and inserts a cysteine between amino acids 160 and 161 in an epidermal growth-factor-like motif of fibrillin. This 3-bp insertion was not found in the fibrillin gene in 70 unrelated, unaffected individuals and 11 unrelated individuals with the Maran syndrome. The authors conclude that neonatal Marfan syndrome is the result of mutations in the fibrillin gene on chromosome 15 and is part of the Marfan syndrome spectrum. 32 refs., 3 figs.

  9. Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

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    Antoccia Antonio

    2010-04-01

    Full Text Available Abstract Ataxia-telangiectasia (A-T is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively with extensive cutaneous granulomatosis and severe combined immunodeficiency, without neurological abnormalities, at the time of diagnosis. This case clearly emphasizes the variable presentation of A-T syndrome and highlights the difficulties in the early diagnosis of A-T. A-T should be considered in children with evidence of combined humoral and cellular immunodeficiency associated with unexplained skin granulomatous lesions, even in the absence of the classic features of this syndrome.

  10. RESULTS OF THE IMMUNIZATION ACCORDING TO THE NATIONAL CALENDAR ASSOCIATED WITH VACCINATION AGAINST INFLUENZA IN CHILDREN WITH SOMATIC DISORDERS AND IMMUNODEFICIENCY

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    S. M. Kharit

    2014-01-01

    Full Text Available Aim: to study safety and immunological efficacy of vaccination against influenza in separate and associated immunization according to the National Calendar. Patients and methods: 100 children with various disorders as well as children with HIV subjected to revaccination against diphtheria, tetanus, measles, parotiditis and rubella, were included into the study. Children were divided into 5 groups of 20 persons each: groups of separate or associated vaccination. In order to assess safety of vaccination children were observed for 30 days after vaccination. In order to analyze immunologic efficacy participants were taken serum tests before and on the 30th day after vaccination. Results: the study demonstrated high safety of the vaccine against influenza. Mild and moderate topical and general reactions were observed in isolated instances and did not differ in comparison groups. Simultaneous vaccination against influenza with immunization against diphtheria, parotiditis and rubella did not influence synthesis of antibodies, while synthesis of antibodies against measles was decreased; immunization against diphtheria and measles when associated with vaccination against influenza depressed synthesis of antibodies against A/H3N2 influenza. Conclusions: vaccine against influenza has low reactivity, high safety and can be used in association with immunization according to the National calendar in independence to somatic disorders of patients. 

  11. The Clinical Characteristics of Acute Cerebrovascular Accidents Resulting from Ovarian Hyperstimulation Syndrome.

    Science.gov (United States)

    Yang, Shuna; Yuan, Junliang; Qin, Wei; Li, Yue; Yang, Lei; Hu, Wenli

    2017-01-01

    Ovarian hyperstimulation syndrome (OHSS) is a serious complication that occurs after the ovarian-induction treatment. Acute cerebrovascular accident is one of the most dangerous manifestations of the syndrome. However, the characteristics of stroke resulting from OHSS have so far not been well summarised in any study. We reported 2 cases of acute cerebrovascular accidents secondary to OHSS. And then we performed a literature search for reports on this type of stroke, and summarised their characteristics. Thirty-six published cases of this type of stroke were reviewed. Thirty two out of 36 (88.9%) of the women were 35 years old or younger. Stroke in 28 out of 36 (77.8%) of these cases was caused by arterial thrombosis. In 17 out of 28 cases, the involved cerebral vascular branches were mainly middle cerebral artery (MCA) and internal carotid artery (ICA). The acute cerebrovascular accidents happened 7 and 9.25 days after embryo transplantation or 8 and 8.33 days after last human chorionic gonadotropin treatment respectively. The prognosis of patients was relatively good after anticoagulation and some supportive treatments. The MCA and ICA are easily involved in stroke resulting from OHSS. The young age may be a risk factor for developing stroke secondary to OHSS. Once thromboembolism develops, administering appropriate therapy is crucial. © 2017 S. Karger AG, Basel.

  12. Consanguinity and primary immunodeficiencies.

    Science.gov (United States)

    Al-Herz, Waleed; Aldhekri, Hasan; Barbouche, Mohamed-Ridha; Rezaei, Nima

    2014-01-01

    Primary immunodeficiencies (PIDs) are a heterogeneous group of genetic disorders caused by defects in the immune system that predispose patients to infections, autoimmune diseases, lymphoproliferation and malignancies. Most PIDs are inherited in an autosomal recessive pattern; therefore, they are more common in areas with high rates of consanguineous marriage. Reports about PIDs from these areas have demonstrated a peculiar prevalence of more severe forms of diseases compared to other regions, and patients born to consanguineous parents have increased rates of morbidity and mortality compared to other patients. Individuals at high risk of having a child with a PID who wish to have a healthy child have limited options, these include prenatal diagnosis and pre-implantation genetic diagnosis. However, these options require a collaborative team of specialists and may not always be implemented due to geographic, religious, financial or social factors. The recent introduction of newborn-screening programs for a number of T and B lymphocyte deficiencies will facilitate early diagnosis and therapeutic interventions, which may include hematopoietic stem cell transplantation and intravenous immunoglobulin treatment. There is a need for the implementation of strategies to increase public awareness of the health risks associated with consanguineous marriage. It should be stressed that genetic counseling should be an important component of the care of patients with PIDs as well as their families. © 2014 S. Karger AG, Basel.

  13. Radiographic findings in immunodeficiency

    International Nuclear Information System (INIS)

    Obregon, R.; Lynch, D.A.; Cink, T.M.; Newell, J.D.; Kirkpatrick, C.

    1991-01-01

    This paper reviews the chest radiographs and high-resolution CT (HRCT) scans in patients with immunodeficiency disorders and define the role of HRCT. Thirty-three cases were retrospectively graded according to the consensus of two radiologists. Patients with HIV seropositivity and asthma were excluded. HRCT was performed in 12 cases with standard techniques. Diagnoses included common variable hypogammaglobulinemia (n = 19), X-linked agammaglobulinemia (n = 4), chronic mucocutaneous candidiasis (n = 4), and selective immunoglobulin g deficiencies (n = 2). Chest radiographs showed bronchiectasis in 11 of 33 cases with a predominant lower lobe distribution (82%). Nodules were present in six cases and mucus plugs in four cases. HRCT showed bronchiectasis in nine of 12 cases; in five of these nine cases, bronchiectasis was not apparent on chest radiographs. Other HRCT findings included segmental air trapping (four of 12), mucus plugs (three of 12), hazy consolidation (four of 12), nodules (five of 12), and bronchiolectasis (two of 12). Therapy was altered in seven of 12 cases in which HRCT was performed. Most pertinent to clinical management were the presence of a thymoma (n = 1) and severe focal of diffuse bronchiectasis

  14. How effective are the 6 European Society of Immunodeficiency warning signs for primary immunodeficiency disease?

    Science.gov (United States)

    Arslan, Sevket; Ucar, Ramazan; Caliskaner, Ahmet Zafer; Reisli, Ismail; Guner, Sukru Nail; Sayar, Esra Hazar; Baloglu, Ismail

    2016-02-01

    The European Society of Immunodeficiency (ESID) developed 6 warning signs to promote the awareness of adult primary immunodeficiency disease (PID). To screen adult patients for the presence of PID using these 6 warning signs to determine the effectiveness of this protocol. Questions related to the ESID warning signs for adult PID were added to the standard outpatient clinic file system and asked of 3,510 patients who were admitted to our clinic for any reason. Patients with signs and/or suspicion of PID based on their medical history underwent immunologic investigation. In total, 24 patients were diagnosed as having a PID. The most common reason that patients with PID were admitted was frequent infection (n=18 [75%]), and the most common PID subgroup was common variable immunodeficiency (n=12 [50%]). Twenty patients with PID had at least one positive finding according to the ESID warning signs. Two patients with gastrointestinal concerns and 2 with dermatologic symptoms were also diagnosed as having a PID, although they did not have any of the ESID warning signs. The ESID warning signs do not specify the need for symptoms to diagnose a PIDs and do not include a comprehensive list of all signs and symptoms of PIDs. As a result, more than infection-centric questions are needed to identify adult patients with immunodeficiencies. Copyright © 2016 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  15. Metabolic Syndrome and Hypertension Resulting from Fructose Enriched Diet in Wistar Rats

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    Julie Dupas

    2017-01-01

    Full Text Available Increased sugar consumption, especially fructose, is strongly related to the development of type 2 diabetes (T2D and metabolic syndrome. The aim of this study was to evaluate long term effects of fructose supplementation on Wistar rats. Three-week-old male rats were randomly divided into 2 groups: control (C; n=14 and fructose fed (FF; n=18, with a fructose enriched drink (20–25% w/v fructose in water for 21 weeks. Systolic blood pressure, fasting glycemia, and bodyweight were regularly measured. Glucose tolerance was evaluated three times using an oral glucose tolerance test. Insulin levels were measured concomitantly and insulin resistance markers were evaluated (HOMA 2-IR, Insulin Sensitivity Index for glycemia (ISI-gly. Lipids profile was evaluated on plasma. This fructose supplementation resulted in the early induction of hypertension without renal failure (stable theoretical creatinine clearance and in the progressive development of fasting hyperglycemia and insulin resistance (higher HOMA 2-IR, lower ISI-gly without modification of glucose tolerance. FF rats presented dyslipidemia (higher plasma triglycerides and early sign of liver malfunction (higher liver weight. Although abdominal fat weight was increased in FF rats, no significant overweight was found. In Wistar rats, 21 weeks of fructose supplementation induced a metabolic syndrome (hypertension, insulin resistance, and dyslipidemia but not T2D.

  16. Scrambling of the amino acids within the transmembrane domain of Vpu results in a simian-human immunodeficiency virus (SHIVTM) that is less pathogenic for pig-tailed macaques

    International Nuclear Information System (INIS)

    Hout, David R.; Gomez, Melissa L.; Pacyniak, Erik; Gomez, Lisa M.; Inbody, Sarah H.; Mulcahy, Ellyn R.; Culley, Nathan; Pinson, David M.; Powers, Michael F.; Wong, Scott W.; Stephens, Edward B.

    2005-01-01

    Previous studies have shown that the transmembrane (TM) domain of the subtype B Vpu enhances virion release from cells and some studies have shown that this domain may form an oligomeric structure with properties of an ion channel. To date, no studies have been performed to assess the role of this domain in virus pathogenesis in a macaque model of disease. Using a pathogenic molecular clone of simian human immunodeficiency virus (SHIV KU-1bMC33 ), we have generated a novel virus in which the transmembrane domain of the Vpu protein was scrambled but maintained hydrophobic in nature (SHIV TM ), which presumably would disrupt any ion channel TM properties of this protein. Vectors expressing the Vpu as a fusion protein with the enhanced green fluorescent protein (Vpu TM EGFP) indicate that it was transported to the same intracellular compartment as the unmodified Vpu protein but did not down-regulate cell surface expression of CD4. To assess the pathogenicity of SHIV TM , three pig-tailed macaques were inoculated with the SHIV TM and monitored for 6-8 months for CD4 + T cell levels, viral loads and the stability of the sequence of the vpu gene. Our results indicated that unlike the parental SHIV KU-1bMC33 , inoculation of macaques with SHIV TM did not cause a severe CD4 + T cell loss over the course of their infections. Sequence analysis of the vpu gene analyzed from sequential PBMC samples derived from macaques revealed that the scrambled TM was stable during the course of infection. At necropsy, examination of tissues revealed low viral loads and none of the pathology commonly observed in lymphoid and non-lymphoid tissues following inoculation with the pathogenic parental SHIV KU-1bMC33 virus. Thus, these results show for the first time that the TM domain of Vpu contributes to the pathogenicity of SHIV KU-1bMC33 in pig-tailed macaques

  17. Low prevalence of hepatitis B virus, hepatitis D virus and hepatitis C virus among patients with human immunodeficiency virus or acquired immunodeficiency syndrome in the Brazilian Amazon basin Baixa prevalência do vírus da hepatite B, vírus da hepatite D e vírus da hepatite C entre pacientes com o vírus da imunodeficiência humana ou síndrome da imunodeficiência adquirida na Amazônia Brasileira

    Directory of Open Access Journals (Sweden)

    Wornei Silva Miranda Braga

    2006-12-01

    Full Text Available Comorbidities in human immunodeficiency virus infection are of great interest due to their association with unfavorable outcomes and failure of antiretroviral therapy. This study evaluated the prevalence of coinfection by human immunodeficiency virus and viral hepatitis in an endemic area for hepatitis B in the Western Amazon basin. Serological markers for hepatitis B virus, hepatitis C virus and hepatitis D virus were tested in a consecutive sample of all patients referred for treatment of human immunodeficiency virus or acquired immunodeficiency syndrome. The variables sex, age, origin and exposure category were obtained from medical records and from the sexually transmitted diseases and acquired immunodeficiency syndrome surveillance database. Among 704 subjects, the prevalence of chronic hepatitis B carriage was 6.4% and past infection 40.2%. The presence of hepatitis B was associated with birth in hyperendemic areas of the Amazon basin, male sex and illegal drug use. The overall prevalence of hepatitis C was 5% and was associated with illegal drug use. The prevalence of hepatitis B and C among human immunodeficiency virus or acquired immunodeficiency syndrome patients in the Western Amazon basin was lower than seen elsewhere and is probably associated with the local epidemiology of these viruses and the degree of overlap of their shared risk factors. An opportunity presents itself to evaluate the prevention of hepatitis C through harm reduction policies and hepatitis B through vaccination programs among human immunodeficiency virus or acquired immunodeficiency syndrome patients.Co-morbidades na infecção pelo vírus da imunodeficiência humana são de grande interesse devido à associação com desfechos desfavoráveis e falhas na terapia anti-retroviral. Este estudo avalia a prevalência de co-infecção entre o vírus da imunodeficiência humana e hepatites virais, em uma área endêmica de hepatite B, na Amazônia Ocidental. Marcadores

  18. The interrelation between premenstrual syndrome and major depression: Results from a population-based sample

    Directory of Open Access Journals (Sweden)

    Weiss Carine

    2011-10-01

    Full Text Available Abstract Background Research about the relationship between premenstrual syndrome (PMS and major depression is limited. This study examined the relationship between moderate to severe PMS and major depression in a population-based sample of women of reproductive age. The objectives of the study were to assess the association between premenstrual syndrome and major depression, to analyse how PMS and major depression differ and to characterise the group of women who report both PMS and major depression. Methods Data were obtained from the Swiss Health Survey 2007. Included in the analysis was data from women under the age of 55 without hysterectomy and who answered the questions on PMS symptoms. The population-based sample consisted of 3518 women. Weighted prevalence rates were calculated and relative risk ratios for PMS, major depression and women who reported both PMS and major depression, were calculated with logistic multinominal logit regression. Results The prevalence of major depression was 11.3% in women screening positive for moderate PMS and 24.6% in women screening positive for severe PMS. Compared to women without any of these conditions, women who reported moderate to severe alcohol consumption had a lower risk for PMS. Women reporting use of antidepressants, and use of oral contraceptives had a higher risk for major depression compared to women without any of these conditions. Women reporting work dissatisfaction had a higher risk for PMS. A higher relative risk to report both PMS and major depression compared to women without PMS or major depression was related to factors such as high psychological distress, low mastery, psychotropic drug consumption, and low self-rated health. Conclusions The results suggested that women who suffer from both PMS and major depression are more impaired compared to women with only one disorder. The results further indicated that PMS and major depression are different disorders that can, however, co-occur.

  19. Spine deformities in patients with Ehlers-Danlos syndrome, type IV - late results of surgical treatment

    Directory of Open Access Journals (Sweden)

    Tesiorowski Maciej

    2010-11-01

    Full Text Available Abstract Background Spinal deformities in Ehlers-Danlos syndrome are usually progressive and may require operative treatment. There is limited number of studies describing late results of surgery in this disease. Methods This is a retrospective study of the records of 11 patients with Ehlers-Danlos syndrome type IV, treated surgically between 1990 and 2007. All patients underwent surgical treatment for spinal deformity. Duration of operation, type of instrumentation, intraoperative blood loss, complications and number of additional surgeries were noted. Radiographic measurement was performed on standing AP and lateral radiographs acquired before surgery, just after and at final follow up. Results The mean follow up period was 5.5 ± 2.9 years (range 1-10 years. The mean preoperative thoracic and lumbar curve were 109.5 ± 19.9° (range 83° - 142° and 75.6 ± 26.7° (range 40° - 108° respectively. Posterior spine fusion alone was performed on 6 patients and combined anterior and posterior fusion (one- or two stage on 5 cases. Posterior segmental spinal instrumentation was applied with use of hooks, screws and wires. The mean postoperative thoracic and lumbar curve improved to 79.3 ± 16.1° (range 56° - 105° and 58.5 ± 27.7° (range 10° - 95° respectively, with a slight loss of correction during follow up. The average thoracic and lumbar correction was 26.4 ± 14.9% (range 5.3 - 50.4% and 26.3 ± 21.2% (range 7.9 - 75%. Postoperatively, the mean kyphosis was 79.5 ± 40.3° (range 21° -170°, and lordosis was 50.8 ± 18.6° (range 20° -79°. Hyperkyphosis increased during follow up while lordosis remained stable. Mean Th12-L2 angle was -3.5 ±9.9° (range -19° - 15° postoperatively and did not change significantly during follow up. Conclusions Huge spinal deformities in patients with Ehlers-Danlos syndrome require complex and extensive surgery. There is a big risk of sagittal imbalance in this group.

  20. Absolute level of Epstein-Barr Virus (EBV) DNA in human immunodeficiency virus type 1 infection is not predictive of AIDS-related non-Hodgkin lymphoma.

    NARCIS (Netherlands)

    D. van Baarle (Debbie); K.C. Wolthers (Katja); E. Hovenkamp (Egbert); A.D.M.E. Osterhaus (Albert); F. Miedema (Frank); M.H.J. van Oers (Marinus); H.G.M. Niesters (Bert)

    2002-01-01

    textabstractTo study whether Epstein-Barr virus (EBV) load can be used to predict the occurrence of acquired immunodeficiency syndrome-related non-Hodgkin lymphoma (AIDS-NHL), we determined EBV load longitudinally for individuals infected with human immunodeficiency virus type 1. EBV load in

  1. Absolute level of Epstein-Barr virus DNA in human immunodeficiency virus type 1 infection is not predictive of AIDS-related non-Hodgkin lymphoma

    NARCIS (Netherlands)

    van Baarle, Debbie; Wolthers, Katja C.; Hovenkamp, Egbert; Niesters, Hubert G. M.; Osterhaus, Albert D. M. E.; Miedema, Frank; van Oers, Marinus H. J.

    2002-01-01

    To study whether Epstein-Barr virus (EBV) load can be used to predict the occurrence of acquired immunodeficiency syndrome-related non-Hodgkin lymphoma (AIDS-NHL), we determined EBV load longitudinally for individuals infected with human immunodeficiency virus type 1. EBV load in peripheral blood

  2. Aortic valve-sparing in 37 patients with Marfan syndrome: midterm results with David operation.

    Science.gov (United States)

    Forteza, Alberto; De Diego, Javier; Centeno, Jorge; López, Maria Jesus; Pérez, Enrique; Martín, Carlos; Sánchez, Violeta; Rufilanchas, Juan J; Cortina, Jose

    2010-01-01

    We reviewed our experience with aortic valve-sparing operations in Marfan syndrome during last 5 years. Between March 2004 and June 2009, 94 patients with aortic root aneurysms underwent valve-sparing operations. Of these, 37 (68% male) were diagnosed with Marfan syndrome, according to the Ghent diagnostic criteria. Mean age was 30 +/- 10 years (range, 11 to 59 years). Moderate/severe aortic regurgitation was present in 13%, and the mean diameter of the Valsalva sinuses was 50 +/- 4 mm (range, 42 to 62 mm). The David V modification was performed in the last 28 patients. Additional procedures were mitral valve repair in 6, tricuspid valve repair in 3, closure of septal atrial defect in 2, and closure of a patent foramen ovale in 13. Mean follow-up was 27 +/- 16 months (range, 1 to 61 months). There were no in-hospital deaths and no major adverse outcomes. One patient required implantation of a mechanical prosthesis during the same procedure because of moderate aortic regurgitation. One late death occurred. No patients required reoperation. In the last follow-up, 23 patients did not have aortic regurgitation, 12 had grade I, and 1 had grade II. No thromboembolic complications have been documented, and 97% of the patients are free from anticoagulation. Short-term and midterm results with the reimplantation technique for aortic root aneurysms in Marfan patients are excellent. If long-term results are similar, this technique could be the treatment of choice for these patients. 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  3. DiGeorge Syndrome: a not so rare disease

    OpenAIRE

    Fomin,Angela BF; Pastorino,Antonio Carlos; Kim,Chong Ae; Pereira,CA; Carneiro-Sampaio,Magda; Abe-Jacob,Cristina Miuki

    2010-01-01

    INTRODUCTION: The DiGeorge Syndrome was first described in 1968 as a primary immunodeficiency resulting from the abnormal development of the third and fourth pharyngeal pouches during embryonic life. It is characterized by hypocalcemia due to hypoparathyroidism, heart defects, and thymic hypoplasia or aplasia. Its incidence is 1:3000 live births and, despite its high frequency, little is known about its natural history and progression. ?This is probably due to diagnostic difficulties and the ...

  4. The skin in primary immunodeficiency disorders

    NARCIS (Netherlands)

    Sillevis Smitt, J. Henk; Wulffraat, Nico M.; Kuijpers, Taco W.

    2005-01-01

    Primary immunodeficiency disorders comprise serious and rare diseases, predominantly in children. The skin may be involved in a primary immunodeficiency and the cutaneous alterations such as infections, eczematous dermatitis, erythroderma, autoimmune dermatoses and vasculitis may be the basis for

  5. Skeletal and dermatological manifestations of the nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome). Results of 8 patients in 12 years

    International Nuclear Information System (INIS)

    Rupprecht, M.; Barvencik, F.; Amling, M.; Pogoda, P.; Universitaetsklinikum Hamburg-Eppendorf; Mensing, C.H.; Ittrich, H.; Heiland, M.; Rueger, J.M.

    2007-01-01

    Purpose: In 1960 Gorlin and Goltz defined the nevoid basal cell carcinoma syndrome (NBCCS, Gorlin-Goltz Syndrome) as a syndrome comprising multiple basal cell carcinoma, odontogenic keratocysts, and skeletal anomalies. NBCCS is an autosomal dominantly inherited disease with an estimated prevalence of 1:150 000 and diagnosis of this syndrome is often an accidental finding of radiological investigations. The purpose of this study was to report the varied radiological and dermatological manifestations of our patients affected with NBCCS and to present this rare syndrome as a differential diagnosis of skeletal anomalies. Materials and Methods: Between 1994 and 2005 the demographic, clinical, radiological and histological data of 8 patients with NBCCS were retrospectively analyzed. Nevoid basal cell carcinoma syndrome was diagnosed in the event of two major or one major and two minor criteria. The major criteria are more than 2 basal cell carcinoma, odontogenic keratocysts, three or more palmar pits, and calcification of the falx cerebri. Results: Between 1994 and 2005 8 patients (3 females and 5 males) with NBCCS were treated in our departments. The average age at the time of diagnosis of NBCCS was 49.9 years. All patients had a minimum of two major criteria. The major criteria with the most frequency were the basal cell carcinoma (6 patients) and the odontogenic keratocysts (5 patients), followed by the calcification of the falx cerebri and palmoplantar pits (4 patients). There was no gender-related or age-related predilection and only one patient was affected with pain in his fingers which radiologically correlated to small cystic bone lesions (''flame-shaped lucencies''). (orig.)

  6. Knowledge, attitude, and beliefs of young, college student blood donors about Human immunodeficiency virus

    Directory of Open Access Journals (Sweden)

    Anju Dubey

    2014-01-01

    Full Text Available Introduction: Young people, who tend to be healthy, idealistic, and motivated, are an excellent pool of potential voluntary unpaid blood donors. Recruiting and retaining young blood donors improves the long term safety and sufficiency of a country′s blood supply. Knowledge, attitude, and beliefs about Human immunodeficiency virus (HIV should play an important role in prevention of disease transmission. Materials and Methods: This study was a questionnaire based survey, conducted to explore the levels of knowledge, attitude, and beliefs about HIV in young college student blood donors. Results: The results showed that the proportion of participants with comprehensive knowledge of HIV prevention and transmission was lesser than expected. Increase in education level and male gender was found to be significantly associated with high HIV-related knowledge. The responses on the different aspects of HIV-related attitude were also varied and there is still stigma associated with Acquired Immunodeficiency Syndrome (AIDS even in the educated groups. Discussion: There was a spectrum of myths and misperceptions emphasizing the need of education that recognizes the social context of attitude towards HIV. Results from this study may contribute to the development of appropriate educational and training material for this group of donors which in turn, may assist in achieving the elusive goal of safe blood supply in future.

  7. Psychiatric morbidity in asymptomatic human immunodeficiency virus patients

    Directory of Open Access Journals (Sweden)

    V S Chauhan

    2013-01-01

    Full Text Available Background: Psychiatric morbidity in human immunodeficiency virus (HIV patients is being studied all over the world. There is paucity of Indian literature particularly in asymptomatic HIV individuals. Aim: The aim of the following study is to establish the prevalence and the determinants of psychiatric morbidity in asymptomatic HIV patients. Materials and Methods: A cross-sectional study was undertaken to assess psychiatric morbidity as per ICD-10 dacryocystorhinostomy criteria in 100 consecutive asymptomatic seropositive HIV patients and an equal number of age, sex, education, economic and marital status matched HIV seronegative control. All subjects were assessed with the general health questionnaire (GHQ, mini mental status examination, hospital anxiety and depression scale (HADS and sensation seeking scale (SSS and the scores were analyzed statistically. Results: Asymptomatic HIV positive patients had significantly higher GHQ caseness and depression but not anxiety on HADS as compared to HIV seronegative controls. On SSS asymptomatic HIV seropositive subjects showed significant higher scores in thrill and adventure seeking, experience seeking and boredom susceptibility as compared to controls. HIV seropositive patients had significantly higher incidence of total psychiatric morbidity. Among the individual disorders, alcohol dependence syndrome, sexual dysfunction and adjustment disorder were significantly increased compared with HIV seronegative controls. Conclusion: Psychiatric morbidity is higher in asymptomatic HIV patients when compared to HIV seronegative controls. Among the individual disorders, alcohol dependence syndrome, sexual dysfunction and adjustment disorder were significantly increased compared with HIV seronegative controls. High sensation seeking and substance abuse found in HIV seropositive patients may play a vital role in engaging in high-risk behavior resulting in this dreaded illness.

  8. Employment in Adults with Down Syndrome in the United States: Results from a National Survey

    Science.gov (United States)

    Kumin, Libby; Schoenbrodt, Lisa

    2016-01-01

    Background: There is no current data about employment/unemployment of adults with Down syndrome in the United States. The data that exists includes adults with Down syndrome as part of the larger group of people with disabilities or people with intellectual disability. Method: This study used a survey to investigate paid and volunteer employment,…

  9. Outcome in Adult Life for People with Williams Syndrome Results from a Survey of 239 Families

    Science.gov (United States)

    Howlin, P.; Udwin, O.

    2006-01-01

    BACKGROUND: Although there has been considerable research into the genotype and phenotype of Williams syndrome, there have been relatively few studies of long-term prognosis. As a preliminary to a more detailed investigation of adults with Williams syndrome, a parental questionnaire was distributed to members of the UK Williams Syndrome…

  10. Stagnant loop syndrome resulting from small-bowel irradiation injury and intestinal by-pass

    International Nuclear Information System (INIS)

    Swan, R.W.

    1974-01-01

    Stagnant or blind-loop syndrome includes vitamin B12 malabsorption, steatorrhea, and bacterial overgrowth of the small intestine. A case is presented to demonstrate this syndrome occurring after small-bowel irradiation injury with exaggeration postenterocolic by-pass. Alteration of normal small-bowel flora is basic to development of the stagnant-loop syndrome. Certain strains of bacteria as Bacteriodes and E. coli are capable of producing a malabsorption state. Definitive therapy for this syndrome developing after severe irradiation injury and intestinal by-pass includes antibiotics. Rapid symptomatic relief from diarrhea and improved malabsorption studies usually follow appropriate antibiotic therapy. Recolonization of the loop(s) with the offending bacterial species may produce exacerbation of symptoms. Since antibiotics are effective, recognition of this syndrome is important. Foul diarrheal stools should not be considered a necessary consequence of irradiation injury and intestinal by-pass

  11. Current discharge management of acute coronary syndromes: baseline results from a national quality improvement initiative.

    Science.gov (United States)

    Wai, A; Pulver, L K; Oliver, K; Thompson, A

    2012-05-01

    Evidence-practice gaps exist in the continuum of care for patients with acute coronary syndromes (ACS), particularly at hospital discharge. We aimed to describe the methodology and baseline results of the Discharge Management of Acute Coronary Syndromes (DMACS) project, focusing on the prescription of guideline-recommended medications, referral to cardiac rehabilitation and communication between the hospital, patient and their primary healthcare professionals. DMACS employed Drug Use Evaluation methodology involving data collection, evaluation and feedback, and targeted educational interventions. Adult patients with ACS discharged during a 4-month period were eligible to participate. Data were collected (maximum 50 patients) at each site through an inpatient medical record review, a general practitioner (GP) postal/fax survey conducted 14 days post discharge and a patient telephone survey 3 months post discharge. Forty-nine hospitals participated in the audit recruiting 1545 patients. At discharge, 57% of patients were prescribed a combination of antiplatelet agent(s), beta-blocker, statin and angiotensin-converting enzyme inhibitor and/or angiotensin II-antagonist. At 3 months post discharge, 48% of patients reported using the same combination. Some 67% of patients recalled being referred to cardiac rehabilitation; of these, 33% had completed the programme. In total, 83% of patients had a documented ACS management plan at discharge. Of these, 90% included a medication list, 56% a chest pain action plan and 54% risk factor modification advice. Overall, 65% of GPs rated the quality of information received in the discharge summary as 'very good' to 'excellent'. The findings of our baseline audit showed that despite the robust evidence base and availability of national guidelines, the management of patients with ACS can be improved. These findings will inform a multifaceted intervention strategy to improve adherence to guidelines for the discharge management of

  12. Novel features of 3q29 deletion syndrome: Results from the 3q29 registry

    Science.gov (United States)

    Glassford, Megan R.; Rosenfeld, Jill A.; Freedman, Alexa A.; Zwick, Michael E.

    2016-01-01

    3q29 deletion syndrome is caused by a recurrent, typically de novo heterozygous 1.6 Mb deletion, but because incidence of the deletion is rare (1 in 30,000 births) the phenotype is not well described. To characterize the range of phenotypic manifestations associated with 3q29 deletion syndrome, we have developed an online registry (3q29deletion.org) for ascertainment of study subjects and phenotypic data collection via Internet‐based survey instruments. We report here on data collected during the first 18 months of registry operation, from 44 patients. This is the largest cohort of 3q29 deletion carriers ever assembled and surveyed in a systematic way. Our data reveal that 28% of registry participants report neuropsychiatric phenotypes, including anxiety disorder, panic attacks, depression, bipolar disorder, and schizophrenia. Other novel findings include a high prevalence (64%) of feeding problems in infancy and reduced weight at birth for 3q29 deletion carriers (average reduction 13.9 oz (394 g), adjusted for gestational age and sex, P = 6.5e‐07). We further report on the frequency of heart defects, autism, recurrent ear infections, gastrointestinal phenotypes, and dental phenotypes, among others. We also report on the expected timing of delayed developmental milestones. This is the most comprehensive description of the 3q29 deletion phenotype to date. These results are clinically actionable toward improving patient care for 3q29 deletion carriers, and can guide the expectations of physicians and parents. These data also demonstrate the value of patient‐reported outcomes to reveal the full phenotypic spectrum of rare genomic disorders. © 2016 The Authors. American Journal of Medical Genetics Part A Published by Wiley Periodicals, Inc. PMID:26738761

  13. Cancers related to immunodeficiencies : Update and perspectives

    NARCIS (Netherlands)

    Mortaz, Esmaeil; Tabarsi, Payam; Mansouri, Davod; Khosravi, Adnan; Garssen, Johan; Velayati, Aliakbar; Adcock, Ian M.

    2016-01-01

    The life span of patients with primary and secondary immunodeficiency is increasing due to recent improvements in therapeutic strategies. While the incidence of primary immunodeficiencies (PIDs) is 1:10,000 births, that of secondary immunodeficiencies are more common and are associated with

  14. National Surveillance of Occupational Exposure to the Human Immunodeficiency Virus

    Directory of Open Access Journals (Sweden)

    Maura Ricketts

    1992-01-01

    Full Text Available In September 1985, a prospective study was initiated to monitor the occurrence of occupational exposures to human immunodeficiency virus (HIV-infected blood and body fluids in Canada. This program was coordinated by the Federal Centre for acquired immune deficiency syndrome (AIDS (now the Division of HIV/AIDS Epidemiology at the Laboratory Centre for Disease Control. The objective was to determine the risk to workers of acquiring HIV infection as a result of exposure to HIV-infected blood and other body fluids. To be eligible, a worker must have sustained a documented parenteral, mucous membrane or skin contact exposure to blood or body fluids from an HIV-infected person. A baseline specimen was collected within a week of the exposure and then at six weeks, 12 weeks, six months and 12 months. Information concerning the type of exposure, precautions used and post exposure treatment was submitted to the Federal Centre for AIDS on standard data collection forms. All information was anonymous, identified only by a code number. Guidelines for counselling an exposed employee were provided with enrollment material. As of July 29, 1991, 414 employees have been included in the study. Two hundred and thirty-seven of the 414 exposures (57% were needlestick injuries of which 167 (70% were sustained by nurses. Other exposures consisted of open wound contamination, eye splashes, scalpel wounds and skin contact with blood and body fluids. To date, there have been no seroconversions among workers enrolled in the surveillance program.

  15. Rapidly progressive periodontal disease associated with human immunodeficiency virus

    International Nuclear Information System (INIS)

    Hezaim, K.A.; Javed, F.; Askar, A.; Rasheed, A.A.

    2012-01-01

    Severe periodontal inflammation with generalized dental plaque accumulation, spontaneous and severe gingival bleeding, fungal infection, and inter dental papillae necrosis are presented in a patient infected with human immunodeficiency virus (HIV). Bite-wing radiographs revealed a generalized horizontal alveolar bone loss of 7-8 millimetres in both arches. Erythematous patches were noted on the gingival mucosa in both jaws. DNA testing was performed to identify the periodontopathogens. The patient had no signs or symptoms of acquired immunodeficiency syndrome. This case-report presents the massive periodontal destruction that occurred in a patient infected with HIV. Therefore, it is highly recommended that patients infected with HIV should be regularly monitored to aid in early detection and to provide proper management of periodontal inflammatory conditions to minimize its destruction. (author)

  16. Knowledge, attitude, and beliefs of young, college student blood donors about Human immunodeficiency virus.

    Science.gov (United States)

    Dubey, Anju; Sonker, Atul; Chaudhary, Rajendra K

    2014-01-01

    Young people, who tend to be healthy, idealistic, and motivated, are an excellent pool of potential voluntary unpaid blood donors. Recruiting and retaining young blood donors improves the long term safety and sufficiency of a country's blood supply. Knowledge, attitude, and beliefs about Human immunodeficiency virus (HIV) should play an important role in prevention of disease transmission. This study was a questionnaire based survey, conducted to explore the levels of knowledge, attitude, and beliefs about HIV in young college student blood donors. The results showed that the proportion of participants with comprehensive knowledge of HIV prevention and transmission was lesser than expected. Increase in education level and male gender was found to be significantly associated with high HIV-related knowledge. The responses on the different aspects of HIV-related attitude were also varied and there is still stigma associated with Acquired Immunodeficiency Syndrome (AIDS) even in the educated groups. There was a spectrum of myths and misperceptions emphasizing the need of education that recognizes the social context of attitude towards HIV. Results from this study may contribute to the development of appropriate educational and training material for this group of donors which in turn, may assist in achieving the elusive goal of safe blood supply in future.

  17. Targeting cysteine residues of human immunodeficiency virus type 1 protease by reactive free radical species.

    Science.gov (United States)

    Basu, A; Sehajpal, P K; Ogiste, J S; Lander, H M

    1999-01-01

    Nitric oxide (NO) is a naturally occurring free radical with many functions. The oxidized form of NO, the nitrosonium ion, reacts with the thiol group of cysteine residues resulting in their modification to S-nitrosothiols. The human immunodeficiency virus type 1 (HIV-1) protease (HIV-PR) has two cysteine residues that are conserved amongst different viral isolates found in patients with acquired immunodeficiency syndrome (AIDS). In an active dimer, these residues are located near the surface of the protease. We have found that treatment of HIV-PR with different NO congeners results in loss of its proteolytic activity and simultaneous formation of S-nitrosothiols. Sodium nitroprusside inhibited HIV-PR up to 70% and S-nitroso-N-acetylpenicillamine completely inhibited the protease within 5 min of treatment. The pattern of inhibition by NO donors is comparable to its inhibition by N-acetyl pepstatin. Using electrospray ionization-mass spectrometry, we identified the modification of HIV-PR by NO as that of S-nitrosation. Our findings point towards a possible role of NO in mediating resistance to HIV-1 infection.

  18. Hyperimmunoglobulin E syndrome with Sjogren's syndrome in a child

    African Journals Online (AJOL)

    We describe a case of hyperimmunoglobulin E syndrome (HIES) associated with Sjogren's syndrome (SS) in a 10-year-old boy and provide a brief review of the literature. Although seen in adults, SS occurring after a primary immunodeficiency disease such as HIES has not previously been reported in a child.

  19. Endoscopic treatment of sciatic nerve entrapment in deep gluteal syndrome: Clinical results.

    Science.gov (United States)

    Aguilera-Bohorquez, B; Cardozo, O; Brugiatti, M; Cantor, E; Valdivia, N

    2018-05-25

    Deep gluteal syndrome (DGS) is characterized by compression, at extra-pelvic level, of the sciatic nerve within any structure of the deep gluteal space. The objective was to evaluate the clinical results in patients with DGS treated with endoscopic technique. Retrospective study of patients with DGS treated with an endoscopic technique between 2012 and 2016 with a minimum follow-up of 12 months. The patients were evaluated before the procedure and during the first year of follow-up with the WOMAC and VAIL scale. Forty-four operations on 41 patients (36 women and 5 men) were included with an average age of 48.4±14.5. The most common cause of nerve compression was fibrovascular bands. There were two cases of anatomic variant at the exit of the nerve; compression of the sciatic nerve was associated with the use of biopolymers in the gluteal region in an isolated case. The results showed an improvement of functionality and pain measured with the WOMAC scale with a mean of 63 to 26 points after the procedure (Pnerve. Four cases required revision at 6 months following the procedure due to compression of the scarred tissue surrounding the sciatic nerve. Endoscopic release of the sciatic nerve offers an alternative in the management of DGS by improving functionality and reducing pain levels in appropriately selected patients. Copyright © 2018 SECOT. Publicado por Elsevier España, S.L.U. All rights reserved.

  20. Peripheral nervous system involvement in primary burning mouth syndrome--results of a pilot study.

    Science.gov (United States)

    Puhakka, A; Forssell, H; Soinila, S; Virtanen, A; Röyttä, M; Laine, M; Tenovuo, O; Teerijoki-Oksa, T; Jääskeläinen, S K

    2016-05-01

    The pathophysiology of primary burning mouth syndrome (BMS) has remained enigmatic, but recent studies suggest pathology within the nervous system at multiple levels. This study aimed to investigate in detail the contribution of either focal or generalized alterations within the peripheral nervous system (PNS) in the etiopathogenesis of BMS. Intraepithelial nerve fiber density (IENFD) of tongue mucosa was assessed in 10 carefully characterized BMS, and the results were compared to 19 age- and gender-matched cadaver controls, 6 with lifetime diabetes. Extensive neurophysiologic and psychophysical examinations of the trigeminal system and distal extremities were performed to profile PNS function in BMS. Patients with BMS had significantly fewer intraepithelial nerve fibers (0,27, s.e. 0,18 mm(-1); P = 0.0253) than non-diabetic controls (0,92, s.e. 0,15 mm(-1)). In the subepithelial space, the amount of nerve fibers did not differ between the groups. The majority (9/10) of patients with BMS showed neurophysiologic or psychophysical signs of a more generalized PNS dysfunction. Our results in neurophysiologically optimally characterized BMS patients confirm that pure focal small fiber neuropathy of the oral mucosa has a role in the pathophysiology of primary BMS. Furthermore, BMS may be related to a more generalized, yet subclinical peripheral neuropathy. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Endovascular Treatment of Malignant Superior Vena Cava Syndrome: Results and Predictive Factors of Clinical Efficacy

    Energy Technology Data Exchange (ETDEWEB)

    Fagedet, Dorothee, E-mail: DFagedet@chu-grenoble.fr [CHU de Grenoble, Clinique universitaire de medecine interne, Pole Pluridisciplinaire de Medecine (France); Thony, Frederic, E-mail: FThony@chu-grenoble.fr [CHU de Grenoble, Clinique universitaire de radiologie et imagerie medicale, Pole d' Imagerie (France); Timsit, Jean-Francois, E-mail: JFTimsit@chu-grenoble.fr [CHU de Grenoble, Clinique universitaire de reanimation, Pole Medecine Aiguee Communautaire (France); Rodiere, Mathieu, E-mail: MRodiere@chu-grenoble.fr [CHU de Grenoble, Clinique universitaire de radiologie et imagerie medicale, Pole d' Imagerie (France); Monnin-Bares, Valerie, E-mail: v-monnin@chu-montpellier.fr [CHRU Arnaud de Villeneuve, Imagerie Medicale Thoracique Cardiovasculaire (France); Ferretti, Gilbert R., E-mail: GFerretti@chu-grenoble.fr [CHU de Grenoble, Clinique universitaire de radiologie et imagerie medicale, Pole d' Imagerie (France); Vesin, Aurelien; Moro-Sibilot, Denis, E-mail: DMoro.pneumo@chu-grenoble.fr [University Grenoble 1 e Albert Bonniot Institute, Inserm U823 (France)

    2013-02-15

    To demonstrate the effectiveness of endovascular treatment (EVT) with self-expandable bare stents for malignant superior vena cava syndrome (SVCS) and to analyze predictive factors of EVT efficacy. Retrospective review of the 164 patients with malignant SVCS treated with EVT in our hospital from August 1992 to December 2007 and followed until February 2009. Endovascular treatment includes angioplasty before and after stent placement. We used self-expandable bare stents. We studied results of this treatment and looked for predictive factors of clinical efficacy, recurrence, and complications by statistical analysis. Endovascular treatment was clinically successful in 95% of cases, with an acceptable rate of early mortality (2.4%). Thrombosis of the superior vena cava was the only independent factor for EVT failure. The use of stents over 16 mm in diameter was a predictive factor for complications (P = 0.008). Twenty-one complications (12.8%) occurred during the follow-up period. Relapse occurred in 36 patients (21.9%), with effective restenting in 75% of cases. Recurrence of SVCS was significantly increased in cases of occlusion (P = 0.01), initial associated thrombosis (P = 0.006), or use of steel stents (P = 0.004). Long-term anticoagulant therapy did not influence the risk of recurrence or complications. In malignancy, EVT with self-expandable bare stents is an effective SVCS therapy. These results prompt us to propose treatment with stents earlier in the clinical course of patients with SVCS and to avoid dilatation greater than 16 mm.

  2. Clinical Characteristics and Results of Laser Peripheral Iridotomy of Pigment Dispersion Syndrome

    Directory of Open Access Journals (Sweden)

    Şerife Bayraktar

    2017-12-01

    Full Text Available Aims: To report the clinical findings, characteristics and the results of peripheral laser iridotomy in pigment dispersion syndrome (PDS patients.Methods: Medical records of 30 patients who had been diagnosed with PDS were evaluated retrospectively at Glaucoma Department of Istanbul Faculty of Medicine at Istanbul University.Results: Sixty eyes of these 30 patients enrolled in the study. 18 of them (60% were male and 12 (40% were female with mean age of 42.4 ±12.3 years (range: 22 to 73 years. Forty-six eyes of the 23 patients had myopia, 12 eyes of 6 patients had hyperopia, 2 eyes of 1 patient had emmetropia. All patients except 2 eyes of the 1 patient had (96.6% Krukenberg spindles. Homogeneous trabecular meshwork(TM pigmentation was seen in all patients. Thirty-two eyes of 17 patients (53.3 % had iris transillumination defects. Neodymium:yttrium–aluminum–garnet (Nd:YAG peripheral laser iridotomy was performed in 44 eyes of 60 eyes (73.3%.Conclusion: The most common clinical findings in Turkish PDS patients were Krukenberg spindles and homogeneous TM pigmentation. If PDS hasn’t advanced in pigmentary ocular hypertension or pigmentary glaucoma, progression can be stabilized by laser iridotomy and medical treatment.

  3. Endovascular Treatment of Malignant Superior Vena Cava Syndrome: Results and Predictive Factors of Clinical Efficacy

    International Nuclear Information System (INIS)

    Fagedet, Dorothée; Thony, Frederic; Timsit, Jean-François; Rodiere, Mathieu; Monnin-Bares, Valérie; Ferretti, Gilbert R.; Vesin, Aurélien; Moro-Sibilot, Denis

    2013-01-01

    To demonstrate the effectiveness of endovascular treatment (EVT) with self-expandable bare stents for malignant superior vena cava syndrome (SVCS) and to analyze predictive factors of EVT efficacy. Retrospective review of the 164 patients with malignant SVCS treated with EVT in our hospital from August 1992 to December 2007 and followed until February 2009. Endovascular treatment includes angioplasty before and after stent placement. We used self-expandable bare stents. We studied results of this treatment and looked for predictive factors of clinical efficacy, recurrence, and complications by statistical analysis. Endovascular treatment was clinically successful in 95% of cases, with an acceptable rate of early mortality (2.4%). Thrombosis of the superior vena cava was the only independent factor for EVT failure. The use of stents over 16 mm in diameter was a predictive factor for complications (P = 0.008). Twenty-one complications (12.8%) occurred during the follow-up period. Relapse occurred in 36 patients (21.9%), with effective restenting in 75% of cases. Recurrence of SVCS was significantly increased in cases of occlusion (P = 0.01), initial associated thrombosis (P = 0.006), or use of steel stents (P = 0.004). Long-term anticoagulant therapy did not influence the risk of recurrence or complications. In malignancy, EVT with self-expandable bare stents is an effective SVCS therapy. These results prompt us to propose treatment with stents earlier in the clinical course of patients with SVCS and to avoid dilatation greater than 16 mm.

  4. The prevalence of metabolic syndrome among older adults in Ecuador: Results of the SABE survey.

    Science.gov (United States)

    Orces, Carlos H; Gavilanez, Enrique Lopez

    2017-12-01

    To describe the prevalence of metabolic syndrome among older adults in Ecuador. A secondary objective was to examine the relationship between metabolic syndrome and its components and insulin resistance among non-diabetic participants. The National Survey of Health, Wellbeing, and Aging survey was used to examine the prevalence of metabolic syndrome according to demographic, behavioral, and health characteristics of the participants. Logistic regression models adjusted for covariates were used to examine the independent association of metabolic syndrome and its components and insulin resistance in non-diabetic older adults. Of 2298 participants with a mean age of 71.6 (SD 8.1) years, the prevalence of metabolic syndrome was 66.0% (95% CI, 62.6%, 69.3%) in women and 47.1% (95% CI, 43.2%, 50.9) in men. However, even higher prevalence rates were seen among literate individuals, residents from urban areas of the coastal and Andes Mountains region, obese subjects, those diagnosed with diabetes, and participants with≥2 comorbidities. Overall, abdominal obesity followed by elevated blood pressure were the metabolic syndrome components more prevalent and associated with insulin resistance among older Ecuadorians. Moreover, after adjustment for covariates, older adults defined as having metabolic syndrome had a 3-fold higher odds of having insulin resistance as compared with those without. The prevalence of metabolic syndrome is high among older adults in Ecuador. The present findings may assist public health authorities to implement programs of lifestyle and behavioral modification targeting older adults at increased risk for this cardio metabolic disorder. Copyright © 2017 Diabetes India. Published by Elsevier Ltd. All rights reserved.

  5. New insights on diabetes in Turner syndrome: results from an observational study in adulthood.

    Science.gov (United States)

    Ibarra-Gasparini, Daniela; Altieri, Paola; Scarano, Emanuela; Perri, Annamaria; Morselli-Labate, Antonio M; Pagotto, Uberto; Mazzanti, Laura; Pasquali, Renato; Gambineri, Alessandra

    2018-03-01

    To explore the characteristics of diabetes mellitus in adults with Turner syndrome. Observational study consisting of a prospective phase after the access of adults with Turner syndrome to the Endocrinology Unit (median period of follow-up 15.6, interquartile range: 12.0-24.5 months) and a retrospective collection of data from the diagnosis of Turner syndrome until the time of access to the Endocrinology Unit. A total of 113 Italian Turner syndrome patients were included in the study. During the prospective phase of the study, each patient underwent physical examination, fasting blood sampling, and an oral glucose tolerance test on a yearly basis. Oral glucose tolerance test was used to perform the diagnosis of diabetes mellitus. Before access to the Endocrinology Unit, diabetes mellitus was diagnosed in two Turner syndrome patients. Another five cases of diabetes mellitus were diagnosed at the first access to the Endocrinology Unit, whereas seven new cases of diabetes mellitus were diagnosed during the prospective phase of the study. At the diagnosis of diabetes mellitus, only one patient had fasting glucose above 126 mg/dL, and only two had an HbA1c value >6.5% (48 mmol/mol). When compared to normo-glucose tolerant patients, the diabetic patients had a significantly lower insulin-to-glucose ratio at 30 and 60 min of the oral glucose tolerance test. In the regression analyses, only age was associated with the development of diabetes mellitus. This study confirms that diabetes mellitus is frequent in Turner syndrome and suggests that it is specific to the syndrome. In addition, this study demonstrates that oral glucose tolerance test is a more sensitive test than HbA1c for the diagnosis of diabetes mellitus in Turner syndrome.

  6. Relationship of cytokines and cytokine signaling to immunodeficiency disorders in the mouse

    Directory of Open Access Journals (Sweden)

    Morawetz R.A.

    1998-01-01

    Full Text Available The contributions of cytokines to the development and progression of disease in a mouse model of retrovirus-induced immunodeficiency (MAIDS are controversial. Some studies have indicated an etiologic role for type 2 cytokines, while others have emphasized the importance of type 1 cytokines. We have used mice deficient in expression of IL-4, IL-10, IL-4 and IL-10, IFN-g, or ICSBP - a transcriptional protein involved in IFN signaling - to examine their contributions to this disorder. Our results demonstrate that expression of type 2 cytokines is an epiphenomenon of infection and that IFN-g is a driving force in disease progression. In addition, exogenously administered IL-12 prevents many manifestations of disease while blocking retrovirus expression. Interruption of the IFN signaling pathways in ICSBP-/- mice blocks induction of MAIDS. Predictably, ICSBP-deficient mice exhibit impaired responses to challenge with several other viruses. This immunodeficiency is associated with impaired production of IFN-g and IL-12. Unexpectedly, however, the ICSBP-/- mice also develop a syndrome with many similarities to chronic myelogenous leukemia in humans. The chronic phase of this disease is followed by a fatal blast crisis characterized by clonal expansions of undifferentiated cells. ICSBP is thus an important determinant of hematopoietic growth and differentiation as well as a prominent signaling molecule for IFNs

  7. [Vaccine for human immunodeficiency virus (HIV)--relevance of these days].

    Science.gov (United States)

    Laiskonis, Alvydas; Pukenyte, Evelina

    2005-01-01

    Since 1980 more than 25 million people have died from acquired immunodeficiency syndrome (AIDS), which results from infection with human immunodeficiency virus (HIV). Number of new cases increases very threateningly. One and the most effective method to stop the progress of epidemic is the development of the vaccine for HIV. There is the presentation of the first stage of the vaccine for HIV testing (structure, methodology), which is now on trial in St. Pierre hospital, Brussels University. HIV characteristics which inflame the process of the vaccine development, historical facts and facts about vaccines on trial in these days are reviewed in this article. More than 10,000 volunteers have been participating in various clinical trials since 1987. The development of the vaccine is a very difficult, long-terming (about 8-10 years) and costly process. The process of the vaccine testing is very difficult in developing countries where the infection spreads the most rapidly. Available data confirm that the vaccine must be multi-componential, inducing cellular, humoral immunity against various subtypes of HIV. The vaccine cannot protect fully but the changes of the natural infection course could decrease virulence, distance the stage of AIDS, and retard the spread of the epidemic.

  8. Projection of human immunodeficiency virus among high-risk groups in Malaysia.

    Science.gov (United States)

    Mondal, Md Nazrul Islam; Shitan, Mahendran

    2013-01-01

    Human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) presents a serious healthcare threat to young individuals in Malaysia and worldwide. This study aimed to identify trends in HIV-related risk behaviors among recognized high-risk groups and to estimate HIV transmission up to the year 2015. Data and necessary information were obtained from the Ministry of Health Malaysia, published reports from the World Health Organization and United Nations Program on HIV/AIDS, and other articles. The Estimation and Projection Package was used to estimate HIV transmission. The results of the present study revealed that within the high-risk groups, intravenous drug users (IDUs) had the highest prevalence rate of HIV transmission, followed by patients with sexually transmitted infections (STIs), female sex workers (SWs), and men who have sex with men (MSM). Within these at-risk populations, patients with STIs have the highest prevalence of HIV, followed by IDUs, MSM, and SWs. If the transmission rate continues to increase, the situation will worsen; therefore, there is an urgent need for a comprehensive prevention program to control HIV transmission in Malaysia.

  9. Risk of the Metabolic Syndrome in Sexual Minority Women: Results from the ESTHER Study.

    Science.gov (United States)

    Kinsky, Suzanne; Stall, Ron; Hawk, Mary; Markovic, Nina

    2016-08-01

    Compared to heterosexuals, sexual minority women (SMW) have higher rates of the metabolic syndrome risk factors (e.g., obesity, smoking, heavy drinking, and depression). Yet, no published research has examined whether SMW have higher rates of the metabolic syndrome. The aim of this study is to describe the prevalence of the metabolic syndrome and its individual factors in a sample of heterosexuals and SMW, and identify whether SMW are at greater risk of having the metabolic syndrome. Data are from the Epidemiologic STudy of HEalth Risk in Women (ESTHER), a cross-sectional convenience sample of 479 SMW and 400 heterosexual women from Pittsburgh, Pennsylvania. Participants provided self-report questionnaire data, clinical data, and blood work. Compared to heterosexuals, SMW had higher mean waist circumference, fasting glucose, and systolic and diastolic blood pressure. Nearly one-quarter (24.3%) of SMW had the metabolic syndrome compared to 15.6% of heterosexual women (p = 0.002). After controlling for demographic and risk factors, SMW had a 44% higher risk of having the metabolic syndrome than heterosexuals (p = 0.031). To our knowledge, this is the first study to identify this health disparity in SMW. Future studies should explore differential risk of mortality and metabolic health between SMW and heterosexuals.

  10. Expression of human immunodeficiency virus in cerebrospinal fluid of children with progressive encephalopathy

    NARCIS (Netherlands)

    Epstein, L. G.; Goudsmit, J.; Paul, D. A.; Morrison, S. H.; Connor, E. M.; Oleske, J. M.; Holland, B.

    1987-01-01

    The retrovirus that causes acquired immune deficiency syndrome (AIDS) is now designated the human immunodeficiency virus (HIV). The cerebrospinal fluid (CSF) of 27 children with HIV infection was assayed for intra-blood-brain barrier (IBBB) synthesis of HIV-specific antibodies and for the presence

  11. Multidisciplinary baseline assessment of homosexual men with and without human immunodeficiency virus infection. III. Neurologic and neuropsychological findings.

    Science.gov (United States)

    Stern, Y; Marder, K; Bell, K; Chen, J; Dooneief, G; Goldstein, S; Mindry, D; Richards, M; Sano, M; Williams, J

    1991-02-01

    We explored the possibility that neurologic and neuropsychological changes constitute the earliest detectable manifestations of human immunodeficiency virus (HIV) infection. Without knowledge of HIV status, we assessed neurologic signs and symptoms and administered a battery of neuropsychological tests to 208 homosexual men, of whom 84 were HIV negative, 49 were HIV positive and asymptomatic, 29 were mildly symptomatic, and 46 had significant medical symptoms but not the acquired immunodeficiency syndrome. There was no difference between the HIV-negative and HIV-positive men in the frequency of neurologic signs or of defective or borderline performance on any neuropsychological test. However, HIV-positive men performed slightly but significantly worse than HIV-negative men on tests of verbal memory, executive function, and language. Similar results were obtained when comparisons were limited to HIV-positive medically asymptomatic and HIV-negative men. There was no degradation of neurologic status or neuropsychological performance across stages of HIV severity, but neurologic and neuropsychological summary scores correlated with CD4/CD8 ratios in the HIV-positive group. Ratings of neurologic signs and symptoms correlated with neuropsychological summary scores in the HIV-positive group only. Cognitive complaints were more frequent in the HIV-positive men; they correlated with actual test performance in the HIV-positive but not HIV-negative men. The constellation of subjective and objective neuropsychological and neurologic findings suggests the possibility of a definable syndrome associated with HIV infection in asymptomatic individuals.

  12. Prevalence of metabolic syndrome and its risk factors in adult Malaysians: results of a nationwide survey.

    Science.gov (United States)

    Mohamud, Wan Nazaimoon Wan; Ismail, Aziz al-Safi; Khir, Amir Sharifuddin Md; Ismail, Ikram Shah; Musa, Kamarul Imran; Kadir, Khalid Abdul; Kamaruddin, Nor Azmi; Yaacob, Nor Azwany; Mustafa, Norlaila; Ali, Osman; Isa, Siti Harnida Md; Bebakar, Wan Mohamad Wan

    2012-04-01

    Aim: To report the national prevalence of metabolic syndrome (MetS) and its risk factors among adult Malaysians (>18 years old) based on World Health Organization (WHO), the National Cholesterol Education Program Expert Panel III (ATP III), International Diabetes Federation (IDF) and the 'Harmonized' criteria.Methods: A multi-stage stratified sampling method was used to select 4341 subjects from Peninsular and East Malaysia. Subjects underwent physical and clinical examinations.Results: Based on the WHO, ATP III, IDF and Harmonized definitions, the overall crude prevalences of MetS were 32.1, 34.3, 37.1 and 42.5%, respectively. Regardless of the criteria used, MetS was higher in urban areas, in females, in the Indian population and increased significantly with age. Risk factors also increased with age; abdominal obesity was most prevalent (57.4%), was higher in females (64.2%) and was highest in Indians (68.8%).Hypertension was higher in males (56.5%) and highest among Malays (52.2%). In contrast,the Chinese had the highest prevalence of hypertriglyceridemia (47.4%).Conclusions: Malaysia has a much higher prevalence of MetS compared with other Asian countries and, unless there is immediate intervention to reduce risk factors, this may pose serious implications on the country's healthcare costs and services.

  13. Capturing public interest toward new tools for controlling human immunodeficiency virus (HIV) infection exploiting data from Google Trends.

    Science.gov (United States)

    Mahroum, Naim; Bragazzi, Nicola Luigi; Brigo, Francesco; Waknin, Roy; Sharif, Kassem; Mahagna, Hussein; Amital, Howard; Watad, Abdulla

    2018-04-01

    Human immunodeficiency virus vaccination and pre-exposure prophylaxis represent two different emerging preventive tools. Google Trends was used to assess the public interest toward these tools in terms of digital activities. Worldwide web searches concerning the human immunodeficiency virus vaccine represented 0.34 percent, 0.03 percent, and 46.97 percent of human immunodeficiency virus, acquired immune deficiency syndrome, and human immunodeficiency virus/acquired immune deficiency syndrome treatment-related Google Trends queries, respectively. Concerning temporal trends, digital activities were shown to increase from 0 percent as of 1 January 2004 percent to 46 percent as of 8 October 2017 with two spikes observed in May and July 2012, coinciding with the US Food and Drug Administration approval. Bursts in search number and volume were recorded as human immunodeficiency virus vaccine trials emerged. This search topic has decreased in the past decade in parallel to the increase in Truvada-related topics. Concentrated searches were noticed among African countries with high human immunodeficiency virus/acquired immune deficiency syndrome prevalence. Stakeholders should take advantage of public interest especially in preventive medicine in high disease burden countries.

  14. Clinical aspects of feline immunodeficiency and feline leukemia virus infection.

    Science.gov (United States)

    Hartmann, Katrin

    2011-10-15

    Feline leukemia virus (FeLV) and feline immunodeficiency virus (FIV) are retroviruses with a global impact on the health of domestic cats. The two viruses differ in their potential to cause disease. FIV can cause an acquired immunodeficiency syndrome that increases the risk of developing opportunistic infections, neurological diseases, and tumors. In most naturally infected cats, however, FIV itself does not cause severe clinical signs, and FIV-infected cats may live many years without any health problems. FeLV is more pathogenic, and was long considered to be responsible for more clinical syndromes than any other agent in cats. FeLV can cause tumors (mainly lymphoma), bone marrow suppression syndromes (mainly anemia) and lead to secondary infectious diseases caused by suppressive effects of the virus on bone marrow and the immune system. Today, FeLV is less important as a deadly infectious agent as in the last 20 years prevalence has been decreasing in most countries. Copyright © 2011 Elsevier B.V. All rights reserved.

  15. Ethnic differences in the prevalence of metabolic syndrome: results from a multi-ethnic population-based survey in Malaysia.

    Directory of Open Access Journals (Sweden)

    Sanjay Rampal

    Full Text Available INTRODUCTION: The prevalence of metabolic syndrome is increasing disproportionately among the different ethnicities in Asia compared to the rest of the world. This study aims to determine the differences in the prevalence of metabolic syndrome across ethnicities in Malaysia, a multi-ethnic country. METHODS: In 2004, we conducted a national cross-sectional population-based study using a stratified two-stage cluster sampling design (N = 17,211. Metabolic syndrome was defined according to the International Diabetes Federation/National Heart, Lung and Blood Institute/American Heart Association (IDF/NHLBI/AHA-2009 criteria. Multivariate models were used to study the independent association between ethnicity and the prevalence of the metabolic syndrome. RESULTS: The overall mean age was 36.9 years, and 50.0% participants were female. The ethnic distribution was 57.0% Malay, 28.5% Chinese, 8.9% Indian and 5.0% Indigenous Sarawakians. The overall prevalence of the metabolic syndrome was 27.5%, with a prevalence of central obesity, raised triglycerides, low high density lipoprotein cholesterol, raised blood pressure and raised fasting glucose of 36.9%, 29.3%, 37.2%, 38.0% and 29.1%, respectively. Among those <40 years, the adjusted prevalence ratios for metabolic syndrome for ethnic Chinese, Indians, and Indigenous Sarawakians compared to ethnic Malay were 0.81 (95% CI 0.67 to 0.96, 1.42 (95% CI 1.19 to 1.69 and 1.37 (95% CI 1.08 to 1.73, respectively. Among those aged ≥40 years, the corresponding prevalence ratios were 0.86 (95% CI 0.79 to 0.92, 1.25 (95% CI 1.15 to 1.36, and 0.94 (95% CI 0.80, 1.11. The P-value for the interaction of ethnicity by age was 0.001. CONCLUSIONS: The overall prevalence of metabolic syndrome in Malaysia was high, with marked differences across ethnicities. Ethnic Chinese had the lowest prevalence of metabolic syndrome, while ethnic Indians had the highest. Indigenous Sarawakians showed a marked increase in metabolic

  16. [Molecular pathogenesis of Waardenburg syndrome type II resulting from SOX10 gene mutation].

    Science.gov (United States)

    Zhang, Hua; Chen, Hongsheng; Feng, Yong; Qian, Minfei; Li, Jiping; Liu, Jun; Zhang, Chun

    2016-08-01

    To explore the molecular mechanism of Waardenburg syndrome type II (WS2) resulting from SOX10 gene mutation E248fs through in vitro experiment. 293T cells were transiently transfected with wild type (WT) SOX10 and mutant type (MT) E248fs plasmids. The regulatory effect of WT/MT SOX10 on the transcriptional activity of MITF gene and influence of E248fs on WT SOX10 function were determined with a luciferase activity assay. The DNA binding capacity of the WT/MT SOX10 with the promoter of the MITF gene was determined with a biotinylated double-stranded oligonucleotide probe containing the SOX10 binding sequence cattgtc to precipitate MITF and E248fs, respectively. The stability of SOX10 and E248fs were also analyzed. As a loss-of-function mutation, the E248fs mutant failed to transactivate the MITF promoter as compared with the WT SOX10 (P<0.01), which also showed a dominant-negative effect on WT SOX10. The WT SOX10 and E248fs mutant were also able to bind specifically to the cattgtc motif in the MITF promoter, whereas E248fs had degraded faster than WT SOX10. Despite the fact that the E248fs has a dominant-negative effect on SOX10, its reduced stability may down-regulate the transcription of MITF and decrease the synthesis of melanin, which may result in haploinsufficiency of SOX10 protein and cause the milder WS2 phenotype.

  17. Combined colonoscopy and endometrial biopsy cancer screening results in women with Lynch syndrome.

    Science.gov (United States)

    Nebgen, Denise R; Lu, Karen H; Rimes, Sue; Keeler, Elizabeth; Broaddus, Russell; Munsell, Mark F; Lynch, Patrick M

    2014-10-01

    Endometrial biopsy (EMBx) and colonoscopy performed under the same sedation is termed combined screening and has been shown to be feasible and to provide a less painful and more satisfactory experience for women with Lynch syndrome (LS). However, clinical results of these screening efforts have not been reported. The purpose of this study was to evaluate the long-term clinical outcomes and patient compliance with serial screenings over the last 10.5 years. We retrospectively analyzed the data for 55 women with LS who underwent combined screening every 1-2 years between 2002 and 2013. Colonoscopy and endometrial biopsy were performed by a gastroenterologist and a gynecologist, with the patient under conscious sedation. Out of 111 screening visits in these 55 patients, endometrial biopsies detected one simple hyperplasia, three complex hyperplasia, and one endometrioid adenocarcinoma (FIGO Stage 1A). Seventy-one colorectal polyps were removed in 29 patients, of which 29 were tubular adenomas. EMBx in our study detected endometrial cancer in 0.9% (1/111) of surveillance visits, and premalignant hyperplasia in 3.6% (4/111) of screening visits. No interval endometrial or colorectal cancers were detected. Combined screening under sedation is feasible and less painful than EMBx alone. Our endometrial pathology detection rates were comparable to yearly screening studies. Our results indicate that screening of asymptomatic LS women with EMBx every 1-2 years, rather than annually, is effective in the early detection of (pre)cancerous lesions, leading to their prompt definitive management, and potential reduction in endometrial cancer. Copyright © 2014 Elsevier Inc. All rights reserved.

  18. Implications of post-gadolinium MRI results in 13 cases with posterior reversible encephalopathy syndrome

    International Nuclear Information System (INIS)

    Ugurel, Mehmet Sahin; Hayakawa, Minako

    2005-01-01

    Background: There is a relative lack of definitive information about the contrast-enhancement characteristics of lesions in posterior reversible encephalopathy syndrome (PRES). Objective: Evaluation of contrast-enhanced MRI findings in PRES with a special emphasis on pathophysiology of post-gadolinium behavior of these lesions. Materials and methods: Contrast-enhanced 1.5 T MRI findings and relevant clinical data of the patients were retrospectively reviewed on 13 cases (six males, seven females; age range: 22-78; mean age 47). Although fluid attenuated inversion recovery (FLAIR) and diffusion-weighted MR images were considered for identification of the entity, primarily post-contrast T1-weighted MR images were searched for traces of enhancement in the lesions. Results: No definitely enhancing lesion was identified in the MR images obtained in 6-48 h after onset of symptoms (mostly headaches, seizures and cortical visual field deficits) in this series. Severity of disease indicated by small hemorrhages, confluence of lesions or progression to cytotoxic edema did not seem to alter this result. Typical lesion characteristics were consistent with vasogenic edema on FLAIR and diffusion MR images. Acute elevation of blood pressure on chronic hypertensive background was responsible in four, eclampsia in three, uremia with blood pressure fluctuations in three, and cyclosporine-toxicity in three cases. Conclusion: Although occasional enhancing brain lesions have been reported in the literature on PRES, contrast-enhancement of lesions may be a factor of scan timing and underlying etiology. Prospective studies with larger series on PRES are required for better evaluation of contrast-enhancement in MRI with respect to scan timing, which in turn may help understand its pathophysiology better

  19. Rethinking platelet function: thrombocytopenia induced immunodeficiency in critical illness

    DEFF Research Database (Denmark)

    Ostrowski, Sisse R; Johansson, Per Ingemar

    2011-01-01

    Thrombocytopenia in critical illness predicts a poor clinical outcome. Apart from its role in microvascular thrombus formation, it is widely anticipated that this association is indirect rather than causal. Emerging evidence however indicates that platelets are also immune competent cells. Like...... per se results in immunodeficiency through loss of platelet-mediated immune functions, and propose that thrombocytopenia induced immunodeficiency in critical illness in part explain the negative predictive value of low or declining platelet count. We propose that rethinking the risks...... of thrombocytopenia to include not only bleeding but also immunodeficiency and immune dysregulation along with the conduct of studies investigating mechanisms contributing to thrombocytopenia induced poor non-hemorrhagic outcome in critical illness, may be means to improve outcome in these patients through...

  20. Alteration in pancreatic islet function in human immunodeficiency virus

    DEFF Research Database (Denmark)

    Haugaard, Steen B

    2014-01-01

    Molecular mechanisms behind the defects in insulin production and secretion associated with antihuman immunodeficiency virus (anti-HIV) therapy and the development of HIV-associated lipodystrophy syndrome (HALS) are discussed in this article. Data suggesting insulin resistance on the beta cell...... and defects in first-phase insulin release of HALS patients are presented. Hepatic extraction of insulin, nonglucose insulin secretagogues and insulin-like growth factor release may exert influence on the demand of circulating insulin and on insulin secretion in HIV-infected patients. Finally, the paucity...

  1. Rhabdomyolysis resulting in concurrent Horner's syndrome and brachial plexopathy: a case report.

    Science.gov (United States)

    Lee, Susan C; Geannette, Christian; Wolfe, Scott W; Feinberg, Joseph H; Sneag, Darryl B

    2017-08-01

    This case report describes a 29-year-old male who presented with immediate onset of Horner's syndrome and ipsilateral brachial plexopathy after sleeping with his arm dangling outside a car window for 8 h. Outside workup and imaging revealed rhabdomyolysis of the left neck musculature. Subsequent electrodiagnostic testing and high-resolution brachial plexus magnetic resonance imaging at the authors' institution attributed the Horner's syndrome and concurrent brachial plexopathy to rhabdomyolysis of the longus colli and scalene musculature, which had compressed-and consequently scar tethered-the cervical sympathetic trunk and brachial plexus. This case of co-existent Horner's syndrome and brachial plexopathy demonstrates the role of high-resolution brachial plexus MRI in diagnosing plexopathy and the importance of being familiar with plexus and paravertebral muscle anatomy.

  2. Rhabdomyolysis resulting in concurrent Horner's syndrome and brachial plexopathy: a case report

    International Nuclear Information System (INIS)

    Lee, Susan C.; Geannette, Christian; Sneag, Darryl B.; Wolfe, Scott W.; Feinberg, Joseph H.

    2017-01-01

    This case report describes a 29-year-old male who presented with immediate onset of Horner's syndrome and ipsilateral brachial plexopathy after sleeping with his arm dangling outside a car window for 8 h. Outside workup and imaging revealed rhabdomyolysis of the left neck musculature. Subsequent electrodiagnostic testing and high-resolution brachial plexus magnetic resonance imaging at the authors' institution attributed the Horner's syndrome and concurrent brachial plexopathy to rhabdomyolysis of the longus colli and scalene musculature, which had compressed - and consequently scar tethered - the cervical sympathetic trunk and brachial plexus. This case of co-existent Horner's syndrome and brachial plexopathy demonstrates the role of high-resolution brachial plexus MRI in diagnosing plexopathy and the importance of being familiar with plexus and paravertebral muscle anatomy. (orig.)

  3. Rhabdomyolysis resulting in concurrent Horner's syndrome and brachial plexopathy: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Susan C.; Geannette, Christian; Sneag, Darryl B. [Hospital for Special Surgery, Department of Radiology and Imaging, New York, NY (United States); Wolfe, Scott W. [Hospital for Special Surgery, Hand and Upper Extremity, Department of Orthopedics, New York, NY (United States); Feinberg, Joseph H. [Hospital for Special Surgery, Physical Medicine and Rehabilitation, New York, NY (United States)

    2017-08-15

    This case report describes a 29-year-old male who presented with immediate onset of Horner's syndrome and ipsilateral brachial plexopathy after sleeping with his arm dangling outside a car window for 8 h. Outside workup and imaging revealed rhabdomyolysis of the left neck musculature. Subsequent electrodiagnostic testing and high-resolution brachial plexus magnetic resonance imaging at the authors' institution attributed the Horner's syndrome and concurrent brachial plexopathy to rhabdomyolysis of the longus colli and scalene musculature, which had compressed - and consequently scar tethered - the cervical sympathetic trunk and brachial plexus. This case of co-existent Horner's syndrome and brachial plexopathy demonstrates the role of high-resolution brachial plexus MRI in diagnosing plexopathy and the importance of being familiar with plexus and paravertebral muscle anatomy. (orig.)

  4. Evaluation of Clinical and Laboratory Data in Patients with Recurrent Infections and Suspected Immunodeficiency

    Directory of Open Access Journals (Sweden)

    Hamid Ahanchian

    2014-09-01

    Full Text Available Background: Frequent infections is among the most frequent clinical dilemmas for primary care physicians. Immunodeficiency disorders are a heterogeneous group of illnesses that predispose patients to the recurrent infections, autoimmunity and malignancies. The aim of this study was to evaluate the clinical and laboratory data collected for the final diagnosis of patients referred with recurrent infections and suspected immunodeficiency to a local immunodeficiency clinic.   Methods: This epidemiological study was carried out between April 2010 and September 2012 at the Immunodeficiency Clinic of Mashhad. All patients with clinical manifestations of immunodeficiency who were referred to our clinic during this period of time were included in this study. 41 patients aged from 10 months to 51 years, were evaluated. Results: Forty one patients, aged between 10 months and 51 years were evaluated. Eleven patients had a primary immunodeficiency, four cases had a secondary immunodeficiency, in three patients an underlying structural disease were found, eight patients were predisposed to recurrent infections as a result of allergies and finally, fifteen cases were found to be normal individuals.   Discussion: Most patients with recurrent infection have a normal immune system. Allergic disorders are the most common predisposing factor to recurrent infection. However, as immunodeficiency disorders are potentially serious, early diagnosis can improve the quality of life and outcome and prevent severe sequels in future.

  5. Pharmacological Inhibition of Feline Immunodeficiency Virus (FIV

    Directory of Open Access Journals (Sweden)

    Dorothee Bienzle

    2012-04-01

    Full Text Available Feline immunodeficiency virus (FIV is a member of the retroviridae family of viruses and causes an acquired immunodeficiency syndrome (AIDS in domestic and non-domestic cats worldwide. Genome organization of FIV and clinical characteristics of the disease caused by the virus are similar to those of human immunodeficiency virus (HIV. Both viruses infect T lymphocytes, monocytes and macrophages, and their replication cycle in infected cells is analogous. Due to marked similarity in genomic organization, virus structure, virus replication and disease pathogenesis of FIV and HIV, infection of cats with FIV is a useful tool to study and develop novel drugs and vaccines for HIV. Anti-retroviral drugs studied extensively in HIV infection have targeted different steps of the virus replication cycle: (1 inhibition of virus entry into susceptible cells at the level of attachment to host cell surface receptors and co-receptors; (2 inhibition of fusion of the virus membrane with the cell membrane; (3 blockade of reverse transcription of viral genomic RNA; (4 interruption of nuclear translocation and viral DNA integration into host genomes; (5 prevention of viral transcript processing and nuclear export; and (6 inhibition of virion assembly and maturation. Despite much success of anti-retroviral therapy slowing disease progression in people, similar therapy has not been thoroughly investigated in cats. In this article we review current pharmacological approaches and novel targets for anti-lentiviral therapy, and critically assess potentially suitable applications against FIV infection in cats.

  6. [Methods in neonatal abstinence syndrome (NAS): results of a nationwide survey in Austria].

    Science.gov (United States)

    Bauchinger, S; Sapetschnig, I; Danda, M; Sommer, C; Resch, B; Urlesberger, B; Raith, W

    2015-08-01

    Neonatal abstinence syndrome (NAS) occurs in neonates whose mothers have taken addictive drugs or were under substitution therapy during pregnancy. Incidence numbers of NAS are on the rise globally, even in Austria NAS is not rare anymore. The aim of our survey was to reveal the status quo of dealing with NAS in Austria. A questionnaire was sent to 20 neonatology departments all over Austria, items included questions on scoring, therapy, breast-feeding and follow-up procedures. The response rate was 95%, of which 94.7% had written guidelines concerning NAS. The median number of children being treated per year for NAS was 4. Finnegan scoring system is used in 100% of the responding departments. Morphine is being used most often, in opiate abuse (100%) as well as in multiple substance abuse (44.4%). The most frequent forms of morphine preparation are morphine and diluted tincture of opium. Frequency as well as dosage of medication vary broadly. 61.1% of the departments supported breast-feeding, regulations concerned participation in a substitution programme and general contraindications (HIV, HCV, HBV). Our results revealed that there is a big west-east gradient in patients being treated per year. NAS is not a rare entity anymore in Austria (up to 50 cases per year in Vienna). Our survey showed that most neonatology departments in Austria treat their patients following written guidelines. Although all of them base these guidelines on international recommendations there is no national consensus. © Georg Thieme Verlag KG Stuttgart · New York.

  7. [Clinical characteristics of human recombination activating gene 1 mutations in 8 immunodeficiency patients with diverse phenotypes].

    Science.gov (United States)

    Yu, G; Wang, W J; Liu, D R; Tao, Z F; Hui, X Y; Hou, J; Sun, J Q; Wang, X C

    2018-03-02

    Objective: To investigate the clinical characteristics of 8 immunodeficiency cases caused by human recombination activating gene 1 (RAG1) mutations, and to explore the relationship among genotypes, clinical manifestations and immunophenotypes. Methods: Clinical data were collected and analyzed from patients with RAG1 mutations who visited the Department of Clinical Immunology, Children's Hospital of Fudan University between October 2013 and June 2017. The data included clinical manifestations, immunophenotypes and genotypes. Results: A total of 8 patients were diagnosed with RAG1 deficiency (6 boys and 2 girls). The minimum age of onset was 2 months, and the maximum age was 4 months. The minimum age of diagnosis was 2 months, and the maximum age was 13 years. Four patients had a family history of infant death due to severe infections. Two cases were born to the same consanguineous parents. All cases had recurrent infections, including involvement of respiratory tract (8 cases), digestive tract (6 cases), urinary tract (1 case), and central nervous system (1 case). The pathogens of infection included bacteria, viruses and fungi. Rotavirus was found in 3 cases, cytomegalovirus (CMV) in 5 cases, bacillus Calmette-Guérin adverse reaction in 2 cases (1 of whom had a positive acid-fast smear from lymph node puncture fluid), fungal infection in 3 cases. One case had multiple nodular space-occupying lesions in lungs and abdominal cavity complicated with multiple bone destruction. The peripheral blood lymphocyte counts of all patients ranged between 0.1 ×10(9)/L and 3.3×10(9)/L (median, 0.65×10(9)/L). Eosinophilia was found in 3 cases (range, (0.48-1.69) ×10(9)/L). The patients were classified according to immunophenotype as severe combined immunodeficiency phenotype (4 cases), leaky severe combined immunodeficiency (2 cases), Omenn syndrome (1 case) and combined immunodeficiency (1 case) . Decreased serum IgG levels were found in 3 cases, increased serum IgM levels in

  8. Pseudotumor Cerebri Resulting in Empty Sella Syndrome and Multiple Pituitary Hormone Deficiencies

    Science.gov (United States)

    2017-09-16

    REPORT TYPE 09/16/2017 Poster 4. TITLE AND SUBTITLE Pseudotu1nor Cercbri Rc~ulling in E1npty ~ella Syndrome and J\\ilultiple Pituitary Honnone...Sc. PROGRAM ELEMENT NUMBER Sd. PROJECT NUMBER Se. TASK NUMBER Sf. WORK UNIT NUMBER 8. PERFORMING ORGANIZATION REPORT NUMBER 17352 10. SPONSOR

  9. How to treat Guyon's canal syndrome? Results from the European HANDGUIDE study: a multidisciplinary treatment guideline

    NARCIS (Netherlands)

    Hoogvliet, P.; Coert, J. H.; Fridén, J.; Huisstede, B. M. A.; Bahm, J.; Dahlin, L.; Jørgsholm, P.; Kvernmo, H.; Lluch, A.; Luchetti, R.; Meuli, C.; Munk, B.; Rosales, R.; Schädel-Höpfner, M.; Stiasny, J.; Taskinen, H.; Thomsen, N.; van Uchelen, J.; Wiberg, M.; Ahlström, M.; Alexander, A.; Enhos, A.; Fairplay, T.; Ferrario, V.; Hermsen, P.; Knijnenburg, S.; Marincek, M.; Pipe, D.; Akre-Roos, K.; Sørensen, A.; Ylvisaker, R.; Zeipel, A.; Emmelot, C.; Gonçalves, L.; de Haart, M.; Paternostro-Sluga, T.; Sousa, A.

    2013-01-01

    Although Guyon's canal syndrome is not highly prevalent, a considerable knowledge of anatomy is needed to localise and treat the pathology. Data on the effectiveness of interventions for this disorder are lacking. To achieve consensus on a multidisciplinary treatment guideline for this disorder

  10. Contrast-Marking Prosodic Emphasis in Williams Syndrome: Results of Detailed Phonetic Analysis

    Science.gov (United States)

    Ito, Kiwako; Martens, Marilee A.

    2017-01-01

    Background: Past reports on the speech production of individuals with Williams syndrome (WS) suggest that their prosody is anomalous and may lead to challenges in spoken communication. While existing prosodic assessments confirm that individuals with WS fail to use prosodic emphasis to express contrast, those reports typically lack detailed…

  11. The Etiology of Vaginal Discharge Syndrome in Zimbabwe Results from the Zimbabwe STI Etiology Study.

    Science.gov (United States)

    Chirenje, Z Mike; Dhibi, Nicholas; Handsfield, H Hunter; Gonese, Elizabeth; Barr, Beth Tippett; Gwanzura, Lovemore; Latif, Ahmed S; Maseko, Dumisili Venessa; Kularatne, Ranmini S; Tshimanga, Mufuta; Kilmarx, Peter H; Machiha, Anna; Mugurungi, Owen; Rietmeijer, Cornelis A

    2017-11-29

    Symptomatic vaginal discharge is a common gynecological condition managed syndromically in most developing countries. In Zimbabwe, women presenting with symptomatic vaginal discharge are treated with empirical regimens that commonly cover both sexually transmitted infections (STI) and reproductive tract infections, typically including a combination of an intramuscular injection of kanamycin, and oral doxycycline and metronidazole regimens. This study was conducted to determine the current etiology of symptomatic vaginal discharge and assess adequacy of current syndromic management guidelines. We enrolled 200 women with symptomatic vaginal discharge presenting at 6 STI clinics in Zimbabwe. Microscopy was used to detect bacterial vaginosis and yeast infection. Nucleic acid amplifications tests were used to detect Neisseria gonorrhoeae, Chlamydia trachomatis, Trichomonas vaginalis and Mycoplasma genitalium. In addition, serologic testing was performed to detect HIV infection. Of the 200 women, 146 (73%) had an etiology detected, including bacterial vaginosis (24.7%); N. gonorrhoeae (24.0%); yeast infection (20.7%); T. vaginalis (19.0%); C. trachomatis (14.0%) and M. genitalium (7.0%). Among women with STIs (N=90), 62 (68.9%) had a single infection, 18 (20.0%) had a dual infection and 10 (11.1%) had three infections.Of 158 women who consented to HIV testing, 64 (40.5%) were HIV infected.The syndromic management regimen covered 115 (57.5%) of the women in the sample who had gonorrhea, chlamydia, M. genitalium, or bacterial vaginosis, while 85 (42.5%) of women were treated without such diagnosis. Among women presenting with symptomatic vaginal discharge, bacterial vaginosis was the most common etiology and gonorrhea was the most frequently detected STI. The current syndromic management algorithm is suboptimal for coverage of women presenting with symptomatic vaginal discharge; addition of point of care testing could compliment the effectiveness of the syndromic approach.

  12. Human immunodeficiency virus seroconversion presenting with acute inflammatory demyelinating polyneuropathy: a case report

    Directory of Open Access Journals (Sweden)

    Sloan Derek J

    2008-12-01

    Full Text Available Abstract Introduction Acute Human Immunodeficiency Virus infection is associated with a range of neurological conditions. Guillain-Barré syndrome is a rare presentation; acute inflammatory demyelinating polyneuropathy is the commonest form of Guillain-Barré syndrome. Acute inflammatory demyelinating polyneuropathy has occasionally been reported in acute Immunodeficiency Virus infection but little data exists on frequency, management and outcome. Case presentation We describe an episode of Guillain-Barré syndrome presenting as acute inflammatory demyelinating polyneuropathy in a 30-year-old man testing positive for Immunodeficiency Virus, probably during acute seroconversion. Clinical suspicion was confirmed by cerebrospinal fluid analysis and nerve conduction studies. Rapid clinical deterioration prompted intravenous immunoglobulin therapy and early commencement of highly active anti-retroviral therapy. All symptoms resolved within nine weeks. Conclusion Unusual neurological presentations in previously fit patients are an appropriate indication for Immunodeficiency-Virus testing. Highly active anti-retroviral therapy with adequate penetration of the central nervous system should be considered as an early intervention, alongside conventional therapies such as intravenous immunoglobulin.

  13. Allogeneic hematopoietic stem cell transplantation in children with primary immunodeficiencies: Hospital Israelita Albert Einstein experience.

    Science.gov (United States)

    Fernandes, Juliana Folloni; Kerbauy, Fabio Rodrigues; Ribeiro, Andreza Alice Feitosa; Kutner, Jose Mauro; Camargo, Luis Fernando Aranha; Stape, Adalberto; Troster, Eduardo Juan; Zamperlini-Netto, Gabriele; Azambuja, Alessandra Milani Prandini de; Carvalho, Bruna; Dorna, Mayra de Barros; Vilela, Marluce Dos Santos; Jacob, Cristina Miuki Abe; Costa-Carvalho, Beatriz Tavares; Cunha, Jose Marcos; Carneiro-Sampaio, Magda Maria; Hamerschlak, Nelson

    2011-06-01

    To report the experience of a tertiary care hospital with allogeneic hematopoietic stem cell transplantation in children with primary immunodeficiencies. Seven pediatric patients with primary immunodeficiencies (severe combined immunodeficiency: n = 2; combined immunodeficiency: n = 1; chronic granulomatous disease: n = 1; hyper-IgM syndrome: n = 2; and IPEX syndrome: n = 1) who underwent eight hematopoietic stem cell transplants in a single center, from 2007 to 2010, were studied. Two patients received transplants from HLA-identical siblings; the other six transplants were done with unrelated donors (bone marrow: n = 1; cord blood: n = 5). All patients had pre-existing infections before hematopoietic stem cell transplants. One patient received only anti-thymocyte globulin prior to transplant, three transplants were done with reduced intensity conditioning regimens and four transplants were done after myeloablative therapy. Two patients were not evaluated for engraftment due to early death. Three patients engrafted, two had primary graft failure and one received a second transplant with posterior engraftment. Two patients died of regimen related toxicity (hepatic sinusoidal obstruction syndrome); one patient died of progressive respiratory failure due to Parainfluenza infection present prior to transplant. Four patients are alive and well from 60 days to 14 months after transplant. Patients' status prior to transplant is the most important risk factor on the outcome of hematopoietic stem cell transplants in the treatment of these diseases. Early diagnosis and the possibility of a faster referral of these patients for treatment in reference centers may substantially improve their survival and quality of life.

  14. Feline Immunodeficiency Virus (FIV Neutralization: A Review

    Directory of Open Access Journals (Sweden)

    Margaret J. Hosie

    2011-10-01

    Full Text Available One of the major obstacles that must be overcome in the design of effective lentiviral vaccines is the ability of lentiviruses to evolve in order to escape from neutralizing antibodies. The primary target for neutralizing antibodies is the highly variable viral envelope glycoprotein (Env, a glycoprotein that is essential for viral entry and comprises both variable and conserved regions. As a result of the complex trimeric nature of Env, there is steric hindrance of conserved epitopes required for receptor binding so that these are not accessible to antibodies. Instead, the humoral response is targeted towards decoy immunodominant epitopes on variable domains such as the third hypervariable loop (V3 of Env. For feline immunodeficiency virus (FIV, as well as the related human immunodeficiency virus-1 (HIV-1, little is known about the factors that lead to the development of broadly neutralizing antibodies. In cats infected with FIV and patients infected with HIV-1, only rarely are plasma samples found that contain antibodies capable of neutralizing isolates from other clades. In this review we examine the neutralizing response to FIV, comparing and contrasting with the response to HIV. We ask whether broadly neutralizing antibodies are induced by FIV infection and discuss the comparative value of studies of neutralizing antibodies in FIV infection for the development of more effective vaccine strategies against lentiviral infections in general, including HIV-1.

  15. Defective immuno- and thymoproteasome assembly causes severe immunodeficiency

    DEFF Research Database (Denmark)

    Treise, Irina; Huber, Eva M.; Klein-Rodewald, Tanja

    2018-01-01

    By N-ethyl-N-nitrosourea (ENU) mutagenesis, we generated the mutant mouse line TUB6 that is characterised by severe combined immunodeficiency (SCID) and systemic sterile autoinflammation in homozygotes, and a selective T cell defect in heterozygotes. The causative missense point mutation results...

  16. Reproduction and fertility in human immunodeficiency virus type-1 infection

    NARCIS (Netherlands)

    van Leeuwen, E.; Prins, J. M.; Jurriaans, S.; Boer, K.; Reiss, P.; Repping, S.; van der Veen, F.

    2007-01-01

    Human immunodeficiency virus type-1 (HIV-1) affects mostly men and women in their reproductive years. For those who have access to highly active antiretroviral therapy (HAART), the course of HIV-1 infection has shifted from a lethal to a chronic disease. As a result of this, many patients with HIV-1

  17. 78 FR 29755 - Human Immunodeficiency Virus Patient-Focused Drug Development and Human Immunodeficiency Virus...

    Science.gov (United States)

    2013-05-21

    ... DEPARTMENT OF HEALTH AND HUMAN SERVICES Food and Drug Administration [Docket No. FDA-2013-N-0473] Human Immunodeficiency Virus Patient-Focused Drug Development and Human Immunodeficiency Virus Cure... an opportunity for public comment on human immunodeficiency virus (HIV) Patient-Focused Drug...

  18. 78 FR 46969 - Human Immunodeficiency Virus Patient-Focused Drug Development and Human Immunodeficiency Virus...

    Science.gov (United States)

    2013-08-02

    ... DEPARTMENT OF HEALTH AND HUMAN SERVICES Food and Drug Administration [Docket No. FDA-2013-N-0473] Human Immunodeficiency Virus Patient-Focused Drug Development and Human Immunodeficiency Virus Cure... for the notice of public meeting entitled ``Human Immunodeficiency Virus (HIV) Patient-Focused Drug...

  19. Mid-term results of different aortic valve-sparing procedures in Marfan syndrome.

    Science.gov (United States)

    Schmidtke, Claudia; Karluss, Antje; Sier, Holger; Hüppe, Michael; Brauer, Kirk; Sievers, Hans-H

    2012-03-01

    Marfan patients with aortic root aneurysm are typically treated with the Bentall procedure, though aortic valve-sparing procedures (AVSPs) are also possible. The study aim was to compare the authors' experience with two such techniques performed at their institution, namely a reimplantation according to David (David I) and remodeling according to Yacoub. Between 1996 and 2009, a total of 37 Marfan patients underwent an AVSP at the authors' institution. Of these patients, 25 (mean age 32 +/- 14.9 years) underwent surgery according to David (group D), and 12 (mean age 35 +/- 10.9 years) according to Yacoub (group Y). The patients underwent both clinical and echocardiographic follow up examinations at a mean of 42.0 +/- 36.4 months after surgery. One patient from each group had moved abroad and was lost to follow up. The remaining 35 patients were alive at follow up, and none presented with any major neurological or bleeding complications. In addition, no significant differences were noted between the groups in terms of NYHA classification, left ventricular function, or left ventricular diameter. At follow up, aortic valve function was also comparable between groups, with a peak/mean gradient of 9.4 +/- 6.4/5.3 +/- 3.5 mmHg and 5.1 +/- 3.3/2.8 +/- 1.5 mmHg for groups D and Y, respectively (p = 0.081/0.058). The measured mean grades of aortic valve regurgitation were comparable in groups D and Y (0.6 +/- 0.7 and 1.1 +/- 0.6, respectively; p = 0.055). However, aortic root dimensions obtained via M-mode were smaller in group D patients (29.6 +/- 2.3 mm) than in group Y patients (36.1 +/- 6.6 mm) (p = 0.027). Only three patients from group Y required reoperation on the aortic valve due to valvular regurgitation (p = 0.028); two of these had presented with aortic dissection at the first operation. Both types of AVSP can be performed with comparably good interim clinical results, and also low mortality and morbidity, in patients with Marfan syndrome.

  20. Metabolic syndrome in Mexican adults: results from the National Health and Nutrition Survey 2006.

    Science.gov (United States)

    Rojas, Rosalba; Aguilar-Salinas, Carlos A; Jiménez-Corona, Aída; Shamah-Levy, Teresa; Rauda, Juan; Avila-Burgos, Leticia; Villalpando, Salvador; Ponce, Eduardo Lazcano

    2010-01-01

    To examine the prevalence of metabolic syndrome (MS) and its associated risk factors in Mexican adults aged 20 years or older, using data derived from the National Health and Nutrition Survey 2006 (ENSANUT 2006). The ENSANUT 2006 was conducted between October 2005 and May 2006. Questionnaires were administered to 45 446 adult subjects aged 20 years or older who were residents from urban and rural areas. Anthropometric and blood pressure measurements were obtained from all subjects and fasting blood specimens were provided by 30% of participants. We randomly selected a sub-sample of 6 613 from which laboratory measurements were carried out for glucose, insulin, triglycerides, total cholesterol and HDL-cholesterol. For this analysis, we included only results from eight or more hours of fasting samples (n=6 021). We used individual weighted factors in the statistical analysis and considered the survey's complex sampling design to obtain variances and confidence intervals. All analyses were done using SPSS 15.0. In accordance with definitions by the National Cholesterol Education Program Adult Treatment Panel III (ATP III), the American Heart Association/National Heart, Lung and Blood Institute (AHA/NHLBI), and the International Diabetes Federation (IDF), the prevalence of MS in Mexican adults aged 20 years or older was 36.8, 41.6 and 49.8%, respectively. Women were more affected than men due to the higher prevalence of central obesity among females. Prevalence of MS increased with age and was higher among populations living in metropolitan areas, in the west-central region, and those with lower education. Regardless of the MS definition, a large proportion of Mexican adults has the condition, so preventive measures are needed to decrease the prevalence of the MS components in this population. MS can predict type 2 diabetes and cardiovascular disease, two of the main causes of death in the adult population in Mexico. The intentional search of MS components allows

  1. Differentiated results of defecography with a special view on the SRU syndrome

    International Nuclear Information System (INIS)

    Hauck, R.; Rath, M.; Geile, D.

    1988-01-01

    Eight hundred defecograms using a specially designed seat attached to the fluoroscopic table showed rectal intussusception in 78.3%, rectocele in 53.0%, enterocele in 19.7%, spastic pelvic floor in 15.1%, and normal findings in 2.1%. Pneumocolon in conjunction with defecograms showed whether enteroceles contained colon or small intestine. Because of the high incidence of enteroceles in descending pelvic floor syndrome, defecography is essential prior to pelvic surgery. In 20 patients, solitary rectal ulcer syndrome was diagnosed by means of same-day defecography, pneumocolon, and biopsy. Eighty percent showed intussusception. In two cases, the defeco gram traced the gastrointestinal (GI) bleeding to a rectal ulcer, indicating its usefulness in diagnostic workup for GI bleeding

  2. The Results of Corneal Hydrops Treatment in Patients with Down Syndrome

    Directory of Open Access Journals (Sweden)

    V. K. Surkova

    2018-01-01

    Full Text Available The article describes the clinical cases of acute keratoconus in three patients with Down syndrome who underwent penetrating  keratoplasty. Acute keratoconus were diagnosed in patients by examination of medical history, biomicroscopy, corneal topography,optical coherence tomography. Acute keratoconus occurs suddenly due to the rupture of Descemet’s membrane in the zone of itsstretching, when chamber moisture seeps into the thickness of the stroma, causing its swelling and perforation. If untreated, theprocess continues for 3–5 months. Most researchers recommend keratoplasty during the cold period of the disease. However, withthe threat of perforation require urgent surgical intervention. There are two effective methods of surgical treatment: epikeratophakiaand penetrating keratoplasty. Patients underwent penetrating keratoplasty. The preference for this method was given in connectionwith the following factors: young age patients (under 40 years, relatively healthy transparent peripheral zone of the cornea, whichwas observed in our patients, genetically determined diseases — Down syndrome, the threat of corneal perforation in the centre, apenchant for rubbing his eye, low vision other eye and the desire to obtain speedy optical effect along with the treatment. All patientsafter penetrating keratoplasty had improvement of visual acuity with observation periods up to 1 year. Due to the relatively highincidence of keratoconus in patients with Down syndrome should focus the attention of ophthalmologists. Thus, difficulties in thediagnosis of ophthalmic pathology in patients with concomitant Down syndrome can cause errors in verification of diagnosis and hencewrong treatment selection. In case of hydrops of the cornea penetrating keratoplasty is the choice treatment and contributes to the preservation of the eye and visual functions.

  3. Neonatal screening for severe combined immunodeficiency in Brazil

    Directory of Open Access Journals (Sweden)

    Marilia Pyles Patto Kanegae

    2016-07-01

    Full Text Available Objective: To apply, in Brazil, the T-cell receptor excision circles (TRECs quantification technique using real-time polymerase chain reaction in newborn screening for severe combined immunodeficiency and assess the feasibility of implementing it on a large scale in Brazil. Methods: 8715 newborn blood samples were collected on filter paper and, after DNA elution, TRECs were quantified by real-time polymerase chain reaction. The cutoff value to determine whether a sample was abnormal was determined by ROC curve analysis, using SSPS. Results: The concentration of TRECs in 8,682 samples ranged from 2 to 2,181 TRECs/μL of blood, with mean and median of 324 and 259 TRECs/μL, respectively. Forty-nine (0.56% samples were below the cutoff (30 TRECs/μL and were reanalyzed. Four (0.05% samples had abnormal results (between 16 and 29 TRECs/μL. Samples from patients previously identified as having severe combined immunodeficiency or DiGeorge syndrome were used to validate the assay and all of them showed TRECs below the cutoff. Preterm infants had lower levels of TRECs than full-term neonates. The ROC curve showed a cutoff of 26 TRECs/μL, with 100% sensitivity for detecting severe combined immunodeficiency. Using this value, retest and referral rates were 0.43% (37 samples and 0.03% (3 samples, respectively. Conclusion: The technique is reliable and can be applied on a large scale after the training of technical teams throughout Brazil. Resumo: Objetivo: aplicar no Brasil a técnica de quantificação de T-cell Receptor Excision Circles (TRECs por polymerase chain reaction em tempo real para triagem neonatal de imunodeficiência combinada grave (SCID e avaliar se é possível realizá-la em larga escala em nosso país. Métodos: foram coletadas 8.715 amostras de sangue de recém-nascidos em papel filtro e, após eluição do DNA, os TRECs foram quantificados por polymerase chain reaction em tempo real. O valor de corte para determinar se uma

  4. Spatial analysis of feline immunodeficiency virus infection in cougars.

    Science.gov (United States)

    Wheeler, David C; Waller, Lance A; Biek, Roman

    2010-07-01

    The cougar (Puma concolor) is a large predatory feline found widely in the Americas that is susceptible to feline immunodeficiency virus (FIV), a fast-evolving lentivirus found in wild feline species that is analogous to simian immunodeficiency viruses in wild primates and belongs to the same family of viruses as human immunodeficiency virus. FIV infection in cougars can lead to a weakened immune system that creates opportunities for other infecting agents. FIV prevalence and lineages have been studied previously in several areas in the western United States, but typically without spatially explicit statistical techniques. To describe the distribution of FIV in a sample of cougars located in the northern Rocky Mountain region of North America, we first used kernel density ratio estimation to map the log relative risk of FIV. The risk surface showed a significant cluster of FIV in northwestern Montana. We also used Bayesian cluster models for genetic data to investigate the spatial structure of the feline immunodeficiency virus with virus genetic sequence data. A result of the models was two spatially distinct FIV lineages that aligned considerably with an interstate highway in Montana. Our results suggest that the use of spatial information and models adds novel insight when investigating an infectious animal disease. The results also suggest that the influence of landscape features likely plays an important role in the spatiotemporal spread of an infectious disease within wildlife populations.

  5. Mood and Anxiety Disorders in Chronic Fatigue Syndrome, Fibromyalgia, and Irritable Bowel Syndrome : Results From the LifeLines Cohort Study

    NARCIS (Netherlands)

    Janssens, Karin A. M.; Zijlema, Wilma L.; Joustra, Monica L.; Rosmalen, Judith G. M.

    Objective: Functional somatic syndromes (FSSs) have often been linked to psychopathology. The aim of the current study was to compare prevalence rates of psychiatric disorders among individuals with chronic fatigue syndrome (CFS), fibromyalgia (FM), and irritable bowel syndrome (IBS). Methods: This

  6. [Traditional Chinese medicine syndrome factors of patients with HIV infection or AIDS in China].

    Science.gov (United States)

    Jiang, Feng; Fu, Lin-chun; Ma, Jian-ping; Zhou, Qing; Peng, Bo; Xie, Shi-ping; Guo, Hui-jun; Dong, Yong-xin; Ma, Xiu-lan; Guo, Xuan-xian; Xie, Zhong-li; Hu, Yan-ping; Li, Qing-ya; Li, Hua-wei; Jin, Yan-tao

    2011-09-01

    To study the characteristics of traditional Chinese medicine (TCM) syndrome factors of patients from different areas of China with human immunodeficiency virus (HIV) infection or acquired immunodeficiency syndrome (AIDS). A cross-sectional investigation study was conducted in Henan, Guangdong and Yunnan Provinces and Xinjiang Uygur Autonomous Region of China from October 2008 to August 2010. Based on literature review and expert opinion, a clinical questionnaire of TCM syndromes was drawn up. This survey was carried out after the investigators were professionally trained. Wenfeng III Auxiliary Diagnosis and Treat System of TCM was used to analyze the frequencies of AIDS patients' signs and symptoms with scores above 70 of syndrome factors respectively. Based on this work, syndrome factors of AIDS were analyzed in different areas. There were 608 HIV/AIDS cases investigated from October 2008 to August 2010 in total; among them, 276 cases were from Henan, 126 cases from Guangdong, 120 cases from Xinjiang and 86 cases from Yunnan. The results of syndrome factor analysis indicated that the syndromes of four provinces were similar. HIV/AIDS patients in the four areas exhibited qi deficiency, blood deficiency, yin deficiency, yang deficiency, dampness, phlegm, qi stagnation and essence deficiency syndromes. Patients in each area also had their own characteristics, such as that the scores of dampness of Guangdong and yin deficiency of Xinjiang were higher than the other syndromes, whereas the scores of Henan Province were higher than the other areas. AIDS patients had higher scores of syndromes than HIV-infected patients. HIV/AIDS patients from different areas had similar syndrome elements. The theory of "AIDS toxin injuring primordial qi" can sum up the TCM etiology and pathogenesis of HIV/AIDS.

  7. Secondary Hemophagocytic Syndrome Associated with Richter’s Transformation in Chronic Lymphocytic Leukemia

    Directory of Open Access Journals (Sweden)

    Nura El-Haj

    2014-01-01

    Full Text Available Hemophagocytic syndrome (HPS is an extremely rare condition arising from the overactivation of one’s own immune system. It results in excessive inflammation and tissue destruction. Prompt initiation of treatment is warranted in either scenario in order to decrease mortality. Most cases are triggered by infectious agents, malignancy, or drugs. We describe the first case of a CLL patient presenting with HPS due to acquisition of EBV-related large cell lymphoma in the setting of profound immunodeficiency.

  8. Advances of gene therapy for primary immunodeficiencies [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Fabio Candotti

    2016-03-01

    Full Text Available In the recent past, the gene therapy field has witnessed a remarkable series of successes, many of which have involved primary immunodeficiency diseases, such as X-linked severe combined immunodeficiency, adenosine deaminase deficiency, chronic granulomatous disease, and Wiskott-Aldrich syndrome. While such progress has widened the choice of therapeutic options in some specific cases of primary immunodeficiency, much remains to be done to extend the geographical availability of such an advanced approach and to increase the number of diseases that can be targeted. At the same time, emerging technologies are stimulating intensive investigations that may lead to the application of precise genetic editing as the next form of gene therapy for these and other human genetic diseases.

  9. Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct – clinical challenges, surgical results, and complications

    DEFF Research Database (Denmark)

    Mey, Kristianna; Bille, Michael; Cayé-Thomasen, Per

    2016-01-01

    , and post-operative complications were retrieved. RESULTS: Complications occurred in 36% of implantations; 5% hereof major complications. Gushing/oozing from the cochleostoma occurred in 10% of implantations and was related to transient, but not prolonged post-operative vertigo. CONCLUSION: Intra......-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients....

  10. Common variable immunodeficiency in a horse.

    Science.gov (United States)

    Flaminio, M Julia B F; LaCombe, Veronique; Kohn, Catherine W; Antczak, Douglas F

    2002-11-01

    A 12-year-old Quarter Horse mare that was nonresponsive to medical treatment was evaluated for chronic respiratory disease and hepatobiliary disease. Serum immunoglobulin concentrations were measured by use of radial immunodiffusion that revealed trace to nondetectable concentrations of IgG, IgG(T), IgM, and IgA. Use of serum protein electrophoresis confirmed agammaglobulinemia by the absence of the expected peak in the gamma region. In addition, vaccination with tetanus toxoid did not result in specific immunoglobulin production. Flow cytometric analysis of blood lymphocyte subpopulations revealed the absence of B cells in blood. Immunohistochemical analysis of tissue sections revealed the absence of B lymphocytes in bone marrow and spleen, with occasional B cells in the peripheral lymph nodes. Blood lymphocyte proliferation assays revealed weak responses to pokeweed mitogen and no response to stimulation with lipopolysaccharide. Considering the age and sex of the horse, results of the immunologic tests suggested a diagnosis of common variable immunodeficiency.

  11. Treatment with sirolimus results in complete responses in patients with autoimmune lymphoproliferative syndrome

    Science.gov (United States)

    Teachey, David T.; Greiner, Robert; Seif, Alix; Attiyeh, Edward; Bleesing, Jack; Choi, John; Manno, Catherine; Rappaport, Eric; Schwabe, Dirk; Sheen, Cecilia; Sullivan, Kathleen E.; Zhuang, Hongming; Wechsler, Daniel S.; Grupp, Stephan A.

    2010-01-01

    Summary We hypothesized that sirolimus, an mTOR inhibitor, may be effective in patients with autoimmune lymphoproliferative syndrome (ALPS) and treated patients who were intolerant to or failed other therapies. Four patients were treated for autoimmune cytopenias; all had a rapid complete or near complete response. Two patients were treated for autoimmune arthritis and colitis, demonstrating marked improvement. Three patients had complete resolution of lymphadenopathy and splenomegaly and all patients had a reduction in double negative T cells, a population hallmark of the disease. Based on these significant responses, we recommend that sirolimus be considered as second-line therapy for patients with steroid-refractory disease. PMID:19208097

  12. Excellent long-term results with iliac stenting in local anesthesia for post-thrombotic syndrome

    DEFF Research Database (Denmark)

    Klitfod, Lotte; Just, Sven; Foegh, Pia

    2015-01-01

    BACKGROUND: Only 20% of iliac veins will recanalize on anticoagulation (AC) treatment alone and may, therefore, develop venous obstruction after iliofemoral deep venous thrombosis (DVT). A considerable number of these patients will suffer from post-thrombotic syndrome (PTS) leading to impaired qu...... stent was 89% (17/19) and 16 patients (84 %) had almost or total symptom relief at follow-up. CONCLUSION: Endovascular stenting of iliac obstruction in local anesthesia is minimally invasive and shows excellent long-term outcomes for patients suffering from PTS....

  13. [Stickler's syndrome (dystrophia vitreoretinalis hereditaria). Results of surgery for retinal detachment].

    Science.gov (United States)

    Karel, I; Dolezalová, J; Oudová, P

    2001-05-01

    Stickler's syndrome (SS) is an autosomal dominant hereditary disease of the collagenous connective tissue where impaired development of the vitreous body gel and peripheral retina and detachment of the retina are associated with general manifestations. The objective of the retrospective study was to evaluate the long-term results of surgery of retinal detachment in SS. The group of patients comprised 7 patients, 6 men and 1 woman aged 4 to 45 years, average age 16.8 years. Autosomal dominant heredity was obvious in 6 members (85.7%) of two families. General manifestations of SS included abnormalities of the facial skeleton (6 patients), cleft palate (4 patients), impaired hearing (2 patients), marfanoid habitus (2 patients) and hyperextensibility of the joints (4 patients). In the eyes with SS was manifested by myopia from -1 to -9 D and a liquid vitreous body. Multiple foci of lattice degeneration supplemented the finding in 6 patients (85.7%). Detachment of the retina was a manifestation of SS in 12 of 14 eyes (85.7%). It was manifested in 5 of 7 patients concurrently or within 12 years in both eyes. The causes of retinal detachment were multiple equatorial and postequatorial tears due to lattice degeneration in 8 eyes (66.7%) or a giant tear in 4 eyes (33.3%). Advanced proliferative vitreoretinopathy (PVR) was associated with retinal detachment in 8 eyes (66.7%) and in 6 eyes (50%) it was not possible to assess the beginning of retinal detachment. In 3 of 5 patients with bilateral retinal detachment the adverse course of retinal detachment on the first eye was followed 8 to 12 years previously in another department: two retinal detachments with giant tears were evaluated as inoperable and one inveterated detachment with advanced PVR was operated unsuccessfully. Retinal detachment was operated in 9 eyes of 7 patients, in two patients both eyes were operated simultaneously. The patients were followed up after surgery for 11 months to 15 years, on average for 65

  14. Poliovirus Excretion in Children with Primary Immunodeficiency Disorders, India.

    Science.gov (United States)

    Mohanty, Madhu Chhanda; Madkaikar, Manisha Rajan; Desai, Mukesh; Taur, Prasad; Nalavade, Uma Prajwal; Sharma, Deepa Kailash; Gupta, Maya; Dalvi, Aparna; Shabrish, Snehal; Kulkarni, Manasi; Aluri, Jahnavi; Deshpande, Jagadish Mohanrao

    2017-10-01

    Prolonged excretion of poliovirus can occur in immunodeficient patients who receive oral polio vaccine, which may lead to propagation of highly divergent vaccine-derived polioviruses (VDPVs), posing a concern for global polio eradication. This study aimed to estimate the proportion of primary immunodeficient children with enterovirus infection and to identify the long-term polio/nonpolio enterovirus excreters in a tertiary care unit in Mumbai, India. During September 2014-April 2017, 151 patients received diagnoses of primary immunodeficiency (PID). We isolated 8 enteroviruses (3 polioviruses and 5 nonpolio enteroviruses) in cell culture of 105 fecal samples collected from 42 patients. Only 1 patient with severe combined immunodeficiency was identified as a long-term VDPV3 excreter (for 2 years after identification of infection). Our results show that the risk of enterovirus excretion among children in India with PID is low; however, systematic screening is necessary to identify long-term poliovirus excreters until the use of oral polio vaccine is stopped.

  15. Improving self-esteem in women diagnosed with Turner syndrome: results of a pilot intervention.

    Science.gov (United States)

    Chadwick, Paul M; Smyth, Arlene; Liao, Lih-Mei

    2014-06-01

    To evaluate a brief intervention to improve the self esteem of women diagnosed with Turner syndrome (TS). Prospective observational study. Turner Syndrome Support Society, UK. 30 women aged 18-60 years. A 1-day psychology workshop targeting problems of self-esteem in women diagnosed with TS. The workshop drew on cognitive-behavioral therapy and narrative therapy skills and emphasized increased self-awareness of interpersonal difficulties and improved capacity for self-management. Rosenberg Self-esteem Scale (RSS); Hospital Anxiety and Depression Scale (HADS); bespoke user experiences questionnaire. All 30 women provided baseline data, 27/30 provided immediate post-intervention data and 22/30 provided follow-up data at 3 months. The intervention improved RSS and HADS scores at 3 months. Generic skills-based psychological interventions have the potential to be adapted to provide brief and low-cost interventions to improve self-esteem and reduce psychological distress in women diagnosed with TS. Copyright © 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  16. Feline immunodeficiency. ABCD guidelines on prevention and management.

    Science.gov (United States)

    Hosie, Margaret J; Addie, Diane; Belák, Sándor; Boucraut-Baralon, Corine; Egberink, Herman; Frymus, Tadeusz; Gruffydd-Jones, Tim; Hartmann, Katrin; Lloret, Albert; Lutz, Hans; Marsilio, Fulvio; Pennisi, Maria Grazia; Radford, Alan D; Thiry, Etienne; Truyen, Uwe; Horzinek, Marian C

    2009-07-01

    Feline immunodeficiency virus (FIV) is a retrovirus closely related to human immunodeficiency virus. Most felids are susceptible to FIV, but humans are not. Feline immunodeficiency virus is endemic in domestic cat populations worldwide. The virus loses infectivity quickly outside the host and is susceptible to all disinfectants. Feline immunodeficiency virus is transmitted via bites. The risk of transmission is low in households with socially well-adapted cats. Transmission from mother to kittens may occur, especially if the queen is undergoing an acute infection. Cats with FIV are persistently infected in spite of their ability to mount antibody and cell-mediated immune responses. Infected cats generally remain free of clinical signs for several years, and some cats never develop disease, depending on the infecting isolate. Most clinical signs are the consequence of immunodeficiency and secondary infection. Typical manifestations are chronic gingivostomatitis, chronic rhinitis, lymphadenopathy, weight loss and immune-mediated glomerulonephritis. Positive in-practice ELISA results obtained in a low-prevalence or low-risk population should always be confirmed by a laboratory. Western blot is the 'gold standard' laboratory test for FIV serology. PCR-based assays vary in performance. Cats should never be euthanased solely on the basis of an FIV-positive test result. Cats infected with FIV may live as long as uninfected cats, with appropriate management. Asymptomatic FIV-infected cats should be neutered to avoid fighting and virus transmission. Infected cats should receive regular veterinary health checks. They can be housed in the same ward as other patients, but should be kept in individual cages. At present, there is no FIV vaccine commercially available in Europe. Potential benefits and risks of vaccinating FIV-infected cats should be assessed on an individual cat basis. Needles and surgical instruments used on FIV-positive cats may transmit the virus to other cats

  17. Cranial MRI in the Nijmegen breakage syndrome

    International Nuclear Information System (INIS)

    Bekiesinska-Figatowska, M.; Chrzanowska, K.H.; Krajewska-Walasek, M.; Sikorska, J.; Walecki, J.; Jozwiak, S.; Kleijer, W.J.

    2000-01-01

    We present the results of MRI examinations in ten patients with documented Nijmegen breakage syndrome (NBS), aged 1.75-19 years. T1-, Proton-Density- and T2-weighted spin-echo sequences were performed in three planes. All patients showed microcephaly with decreased size of the frontal lobes and narrow frontal horns. In four patients agenesis of the posterior part of the corpus callosum was found, with colpocephaly and temporal horns dilatation. In one patient callosal hypoplasia was accompanied by abnormal cerebrospinal fluid spaces and wide cerebral cortex, suspicious of pachygyria. Sinusitis was present in all ten patients, as a result of primary immunodeficiency. As in ataxia teleangiectasia and other breakage syndromes, patients with NBS show an inherited susceptibility to malignancy and hypersensitivity to X- and γ-radiation. CT is therefore contraindicated in these patients and MRI should be the method of choice for diagnostic imaging. (orig.)

  18. Changes in B cell immunophenotype in common variable immunodeficiency: cause or effect – is bronchiectasis indicative of undiagnosed immunodeficiency?

    Science.gov (United States)

    Bright, P; Grigoriadou, S; Kamperidis, P; Buckland, M; Hickey, A; Longhurst, H J

    2013-01-01

    Common variable immunodeficiency (CVID) is the most common severe primary immunodeficiency, but the pathology of this condition is poorly understood. CVID involves a defect in the production of immunoglobulin from B cells, with a subsequent predisposition to infections. Approximately 10–20% of cases are inherited, but even in families with a genetic defect the penetrance is far from complete. A classification system for CVID has been suggested (EUROclass) based on B cell immunophenotyping, but it has not been shown that altered B cell immunophenotype is not a consequence of the complications and treatment of CVID. This study compares the EUROclass B cell immunophenotype of CVID patients (n = 30) with suitable disease controls with bronchiectasis (n = 11), granulomatous disease (Crohn's disease) (n = 9) and neurological patients on immunoglobulin treatment (n = 6). The results of this study correlate with previous literature, that alterations in B cell immunophenotype are associated strongly with CVID. Interestingly, three of the 11 bronchiectasis patients without known immunodeficiency had an altered B cell immunophenotype, suggesting the possibility of undiagnosed immunodeficiency, or that bronchiectasis may cause a secondary alteration in B cell immunophenotype. This study showed a significant difference in B cell immunophenotype between CVID patients compared to disease control groups of granulomatous disease and immunoglobulin treatment. This suggests that granulomatous disease (in Crohn's disease) and immunoglobulin treatment (for chronic neurological conditions) are not causal of an altered B cell immunophenotype in these control populations. PMID:23286946

  19. Impaired replication stress response in cells from immunodeficiency patients carrying Cernunnos/XLF mutations.

    Directory of Open Access Journals (Sweden)

    Michal Schwartz

    Full Text Available Non-Homologous End Joining (NHEJ is one of the two major pathways of DNA Double Strand Breaks (DSBs repair. Mutations in human NHEJ genes can lead to immunodeficiency due to its role in V(DJ recombination in the immune system. In addition, most patients carrying mutations in NHEJ genes display developmental anomalies which are likely the result of a general defect in repair of endogenously induced DSBs such as those arising during normal DNA replication. Cernunnos/XLF is a recently identified NHEJ gene which is mutated in immunodeficiency with microcephaly patients. Here we aimed to investigate whether Cernunnos/XLF mutations disrupt the ability of patient cells to respond to replication stress conditions. Our results demonstrate that Cernunnos/XLF mutated cells and cells downregulated for Cernunnos/XLF have increased sensitivity to conditions which perturb DNA replication. In addition, under replication stress, these cells exhibit impaired DSB repair and increased accumulation of cells in G2/M. Moreover Cernunnos/XLF mutated and down regulated cells display greater chromosomal instability, particularly at fragile sites, under replication stress conditions. These results provide evidence for the role of Cernunnos/XLF in repair of DSBs and maintenance of genomic stability under replication stress conditions. This is the first study of a NHEJ syndrome showing association with impaired cellular response to replication stress conditions. These findings may be related to the clinical features in these patients which are not due to the V(DJ recombination defect. Additionally, in light of the emerging important role of replication stress in the early stages of cancer development, our findings may provide a mechanism for the role of NHEJ in preventing tumorigenesis.

  20. Early and late results of graft replacement for dissecting aneurysm of thoracoabdominal aorta in patients with Marfan syndrome.

    Science.gov (United States)

    Omura, Atsushi; Tanaka, Akiko; Miyahara, Shunsuke; Sakamoto, Toshihito; Nomura, Yoshikatsu; Inoue, Takeshi; Oka, Takanori; Minami, Hitoshi; Okada, Kenji; Okita, Yutaka

    2012-09-01

    When treating dissecting aneurysm of the thoracoabdominal aorta surgically in patients with Marfan syndrome, we have usually performed graft replacement- including the entire thoracoabdominal aorta and reconstruction of all visceral branches, even if dilatation is mild in some segments-to avoid further aortic operations in the follow-up period. From October 1999 through July 2011, 20 consecutive patients with Marfan syndrome underwent repair of dissecting aneurysm of the thoracoabdominal aorta (median age, 45 years; range, 19-65 years). All patients underwent surgical intervention with cerebrospinal fluid (CSF) drainage and distal aortic and selective organ perfusion. Deep hypothermia was used in 13 patients for spinal cord protection. No in-hospital mortality was observed. One patient had temporary spinal cord ischemia but was fully recovered by discharge. Other complications included exploration for bleeding (n=1), prolonged ventilation (n=1), and graft infection (n=1). At a mean follow-up of 54 months (range, 9-129 months), 1 patient had died of interstitial pneumonia at 38 months postoperatively. Survival at 8% years was 91.2±9.0%. Two patients required additional aortic procedures (total arch replacement and aortic valve-sparing surgery). Actuarial rate of freedom from aortic operations at 8 years was 83.9%±10.5%, but no patient needed required repeated thoracotomy for an aortic procedure. Neither false nor patch aneurysms were observed using computed tomography (CT) during follow-up surveillance. Graft replacement for dissecting aneurysm of the thoracoabdominal aorta in Marfan syndrome offers good early and long-term results. We believe total aortic replacement including the entire thoracoabdominal aorta and reconstruction of all visceral arteries should be recommended for selected patients with Marfan syndrome. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  1. Sick building syndrome - results of the 'Proklima' research project; Befindlichkeitsstoerungen in Buerogebaeuden - Ergebnisse aus dem Proklima Forschungsprojekt

    Energy Technology Data Exchange (ETDEWEB)

    Kruppa, B. [Bundesindustrieverband Heizungs-, Klima-, Sanitaertechnik und Technische Gebaeudesysteme e.V., Bonn (Germany); Bischof, W.; Brasche, S. [Arbeitsgruppe Raumklimatologie der FSU Jena (ARK), Erfurt (Germany); Bullinger-Naber, M. [Hamburg Univ. (Germany). Abt. fuer Medizinische Psychologie; Mayer, E. [Fraunhofer-Institut fuer Bauphysik, Holzkirchen (Germany); Gebhardt, H. [Wuppertal Univ. (Gesamthochschule) (Germany). Inst. fuer Arbeitsmedizin, Sicherheitstechnik und Ergonomie

    2000-07-01

    In the world's biggest research project, the sick building syndrome was investigated in Germany between 1995 and 1999. In all, 8 air-conditioned buildings and 6 non-air-conditioned buildings, 40 space HVAC systems and 1497 places of work were analyzed, and 4,500 persons were asked to provide data. For the first time ever, measured physical, biological and chemical data were compared with the subjective data provided by the inquiry forms. It was found that the concentrations and hygienic status of air conditioning systems do have an effect on subjective comfort. Buildings with well-designed and well-serviced space HVAC systems had better results than buildings without air conditioning systems. However, it is the psychosocial environment and the job description of building inhabitants that decide their response in terms of sick building syndrome. Factors like gender, job satisfaction, job specifications and educational level are more important than space HVAC systems. [German] Im Rahmen eines der weltweit groessten Forschungsprojekte zum Thema 'Sick Building Syndrome' wurden in Deutschland zwischen 1995 und 1999 umfangreiche Untersuchungen durchgefuehrt mit dem Ziel, die Ursachen und Folgen von Befindlichkeitsstoerungen in Buerogebaeuden zu ermitteln. Insgesamt wurden 8 klimatisierte und 6 nicht klimatisierte Gebaeude, 40 raumlufttechnische Anlagen und 1497 Arbeitsplaetze untersucht sowie ueber 4.500 Personen befragt. Die Ergebnisse erlauben zum ersten Mal einen direkten Vergleich der gemessenen physikalischen, biologischen und chemischen Werte mit den subjektiven Angaben (Fragebogendaten) an klimatisierten und nicht klimatisierten Arbeitsplaetzen. Die Auswertung zeigt, dass die Konzentration der Anlagen und deren hygienischer Wartungszustand einen Einfluss auf die Befindlichkeit der Mitarbeiter haben. Gebaeude mit gut geplanten und gewarteten RLT-Anlagen schneiden in der Bewertung sogar besser ab als nicht klimatisierte Gebaeude. Die groessten

  2. Electrostatic potential of human immunodeficiency virus type 2 and rhesus macaque simian immunodeficiency virus capsid proteins

    Directory of Open Access Journals (Sweden)

    Katarzyna eBozek

    2012-06-01

    Full Text Available Human immunodeficiency virus type 2 (HIV-2 and simian immunodeficiency virus isolated from a macaque monkey (SIVmac are assumed to have originated from simian immunodeficiency virus isolated from sooty mangabey (SIVsm. Despite their close similarity in genome structure, HIV-2 and SIVmac show different sensitivities to TRIM5α, a host restriction factor against retroviruses. The replication of HIV-2 strains is potently restricted by rhesus (Rh monkey TRIM5α, while that of SIVmac strain 239 (SIVmac239 is not. Viral capsid protein is the determinant of this differential sensitivity to TRIM5α, as the HIV-2 mutant carrying SIVmac239 capsid protein evaded Rh TRIM5α-mediated restriction. However, the molecular determinants of this restriction mechanism are unknown. Electrostatic potential on the protein-binding site is one of the properties regulating protein-protein interactions. In this study, we investigated the electrostatic potential on the interaction surface of capsid protein of HIV-2 strain GH123 and SIVmac239. Although HIV-2 GH123 and SIVmac239 capsid proteins share more than 87% amino acid identity, we observed a large difference between the two molecules with the HIV-2 GH123 molecule having predominantly positive and SIVmac239 predominantly negative electrostatic potential on the surface of the loop between α-helices 4 and 5 (L4/5. As L4/5 is one of the major determinants of Rh TRIM5α sensitivity of these viruses, the present results suggest that the binding site of the Rh TRIM5α may show complementarity to the HIV-2 GH123 capsid surface charge distribution.

  3. CXCL12 Mediates Aberrant Costimulation of B Lymphocytes in Warts, Hypogammaglobulinemia, Infections, Myelokathexis Immunodeficiency

    Directory of Open Access Journals (Sweden)

    Giuliana Roselli

    2017-09-01

    Full Text Available The Warts, Hypogammaglobulinemia, Infections, Myelokathexis (WHIM syndrome is an immunodeficiency caused by mutations in chemokine receptor CXCR4. WHIM patient adaptive immunity defects remain largely unexplained. We have previously shown that WHIM-mutant T cells form unstable immunological synapses, affecting T cell activation. Here, we show that, in WHIM patients and WHIM CXCR4 knock-in mice, B cells are more apoptosis prone. Intriguingly, WHIM-mutant B cells were also characterized by spontaneous activation. Searching for a mechanistic explanation for these observations, we uncovered a novel costimulatory effect of CXCL12, the CXCR4 ligand, on WHIM-mutant but not wild-type B cells. The WHIM CXCR4-mediated costimulation led to increased B-cell activation, possibly involving mTOR, albeit without concurrently promoting survival. A reduction in antigenic load during immunization in the mouse was able to circumvent the adaptive immunity defects. These results suggest that WHIM-mutant CXCR4 may lead to spontaneous aberrant B-cell activation, via CXCL12-mediated costimulation, impairing B-cell survival and thus possibly contributing to the WHIM syndrome defects in adaptive immunity.

  4. CXCL12 Mediates Aberrant Costimulation of B Lymphocytes in Warts, Hypogammaglobulinemia, Infections, Myelokathexis Immunodeficiency

    Science.gov (United States)

    Roselli, Giuliana; Martini, Elisa; Lougaris, Vassilios; Badolato, Raffaele; Viola, Antonella; Kallikourdis, Marinos

    2017-01-01

    The Warts, Hypogammaglobulinemia, Infections, Myelokathexis (WHIM) syndrome is an immunodeficiency caused by mutations in chemokine receptor CXCR4. WHIM patient adaptive immunity defects remain largely unexplained. We have previously shown that WHIM-mutant T cells form unstable immunological synapses, affecting T cell activation. Here, we show that, in WHIM patients and WHIM CXCR4 knock-in mice, B cells are more apoptosis prone. Intriguingly, WHIM-mutant B cells were also characterized by spontaneous activation. Searching for a mechanistic explanation for these observations, we uncovered a novel costimulatory effect of CXCL12, the CXCR4 ligand, on WHIM-mutant but not wild-type B cells. The WHIM CXCR4-mediated costimulation led to increased B-cell activation, possibly involving mTOR, albeit without concurrently promoting survival. A reduction in antigenic load during immunization in the mouse was able to circumvent the adaptive immunity defects. These results suggest that WHIM-mutant CXCR4 may lead to spontaneous aberrant B-cell activation, via CXCL12-mediated costimulation, impairing B-cell survival and thus possibly contributing to the WHIM syndrome defects in adaptive immunity. PMID:28928741

  5. Universality of physicians' burnout syndrome as a result of experiencing difficulty in relationship with patients.

    Science.gov (United States)

    Sablik, Zbigniew; Samborska-Sablik, Anna; Drożdż, Jarosław

    2013-06-20

    The aim of our work is to present the universality of burnout syndrome among physicians worldwide and to demonstrate selected aspects of the relationship between patients and doctors as a common factor predisposing to burnout. We looked up 20 original pieces of research from the Medline database published in the last 10 years to determine the prevalence of burnout among doctors in different countries. In all quoted works a remarkable percentage of doctors of interventional and non-interventional specialties suffered burnout. Because it is the relationship with patients that constitutes a key denominator for their work, in the discussion we have exposed an important aspect of it, destructive patient games, described on the basis of transactional analysis. Since universal burnout causes a deterioration of doctors' service, for the optimal good of the patient to survive preservation of the doctor's well-being in the patient-doctor relationship is needed everywhere.

  6. Cutaneous manifestations of primary immunodeficiency

    Directory of Open Access Journals (Sweden)

    Safa Abdelhakim

    2017-01-01

    Full Text Available Primary immunodeficiency diseases (PIDs are a group of rare, chronic disorders with deficient or malfunctioning immune system. It commonly affects the hematopoietic system, with skin the second most affected organ. Skin involvement is observed in half of pediatric PID cases and often precedes the final diagnosis. Skin infections and eczemas are the two most common manifestations in PID.[1] Skin manifestations associated with PIDs can be of infectious and noninfectious causes. Common noninfectious causes are eczema, erythroderma, cutaneous granulomas, dysplasia, vasculitis, and telangiectasia. It is important to be aware of skin manifestations in pediatric patients as early detection of PID may aid in the management of serious immunologic conditions and prevent associated morbidity and mortality.

  7. Cancer as secondary immunodeficiency. Review

    Directory of Open Access Journals (Sweden)

    María Eugenia Vargas-Camaño

    2016-05-01

    Full Text Available The secondary immunodeficiency’s, previously presented in immunocompetent individuals. The lack of primary or secondary response to the presence of a foreign antigen, in the case of infections is a sentinel data in the diagnosis of immunodeficiency (can be primary or secondary, in the case of a self antigen may generate the presence of Cancer. Cancer has shown an increase in the prevalence and incidence globally. Most current medical treatments in cancer are focused primarily on immunomodulatory actions (immunosuppression / immune stimulation or both. Knowledge of key concepts from the perspective of innate and acquired immunity lead to cancer development, engaging immune surveillance and escape mechanisms of this that contribute to better understand the origin, behavior and treatment of neoplasm’s. These treatments can cause immunological disorders such as allergy, anaphylaxis, lack of response immunogenicity care fields specialist in Allergy and Clinical Immunology.

  8. Intensive glucose regulation in hyperglycemic acute coronary syndrome: Results of the randomized BIOMarker study to identify the acute risk of a coronary syndrome-2 (BIOMArCS-2) glucose trial

    NARCIS (Netherlands)

    M. de Mulder (Maarten); V.A.W.M. Umans (Victor); J.H. Cornel (Jan); F.M. van der Zant (F.); F. Stam (Frank); R.M. Oemrawsingh (Rohit); K.M. Akkerhuis (Martijn); H. Boersma (Eric)

    2013-01-01

    textabstractIMPORTANCE: Elevated plasma glucose levels in patients with acute coronary syndrome (ACS) on hospital admission are associated with increased mortality. Clinical trials of glucose regulation have provided inconsistent results with respect to cardiovascular outcomes, perhaps because

  9. Predictive Utility of Brief Alcohol Use Disorders Identification Test (AUDIT) for human immunodeficiency virus antiretroviral medication nonadherence.

    Science.gov (United States)

    Broyles, Lauren Matukaitis; Gordon, Adam J; Sereika, Susan M; Ryan, Christopher M; Erlen, Judith A

    2011-10-01

    Alcohol use negatively affects adherence to antiretroviral therapy (ART), thus human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) care providers need accurate, efficient assessments of alcohol use. Using existing data from an efficacy trial of 2 cognitive-behavioral ART adherence interventions, the authors sought to determine if results on 2 common alcohol screening tests (Alcohol Use Disorders Identification Test--Consumption [AUDIT-C] and its binge-related question [AUDIT-3]) predict ART nonadherence. Twenty-seven percent of the sample (n = 308) were positive on the AUDIT-C and 34% were positive on the AUDIT-3. In multivariate analyses, AUDIT-C-positive status predicted ART nonadherence after controlling for race, age, conscientiousness, and self-efficacy (P = .036). Although AUDIT-3-positive status was associated with ART nonadherence in unadjusted analyses, this relationship was not maintained in the final multivariate model. The AUDIT-C shows potential as an indirect screening tool for both at-risk drinking and ART nonadherence, underscoring the relationship between alcohol and chronic disease management.

  10. Malignant B-cell lymphoma in an infant with severe combined immunodeficiency with short-limbed skeletal dysplasia

    NARCIS (Netherlands)

    van den Berg, H.; Wage, K.; Burggraaf, J. D.; Peters, M.

    1997-01-01

    In an infant with skeletal anomalies and haemolytic disease, intestinal perforation was caused by necrosis of an as yet undetected B-cell lymphoma. Severe combined immunodeficiency with short-limbed skeletal dysplasia was diagnosed. This is the first published report of a patient with this syndrome

  11. Aneurisma de artéria cerebral em criança com síndrome da imunodeficiência adquirida: relato de caso Cerebral arterial aneurysm in a child with acquired immunodeficiency syndrome: case report

    Directory of Open Access Journals (Sweden)

    Arnolfo de Carvalho Neto

    2001-06-01

    Full Text Available A dilatação aneurismática dos vasos do polígono de Willis é manifestação incomum da infecção pelo vírus da imunodeficiência adquirida (HIV, tendo sido relatados, até o momento, 15 casos na literatura. O presente estudo tem por objetivo relatar um novo caso dessa apresentação rara, além de revisar aspectos importantes relacionados à mesma. Um paciente, do sexo masculino, 6 anos de idade, com síndrome da imunodeficiência adquirida (AIDS de transmissão perinatal e tetraparesia, desenvolveu sintomas caracterizados por episódios de crises distônicas. A tomografia computadorizada de crânio, que inicialmente era normal, mostrou dilatação aneurismática dos vasos do polígono de Willis. A revisão de literatura demonstra que os principais achados patológicos em casos similares, são, principalmente, fibrose de camada média com destruição da lâmina interna e hiperplasia da íntima. A etiologia da vasculite não é conhecida, acreditando-se que o vírus da varicela-zoster e o próprio HIV possam estar relacionados à mesma. Conclui-se que, apesar de incomum, tal complicação apresenta grande importância pelo fato dos aneurismas colocarem esses pacientes em grupo de alto risco para acidentes vasculares. A sobrevida relatada na literatura é de menos de 6 meses após o diagnóstico da arteriopatia aneurismática cerebral.Cerebral aneurysmal arteriopathy of the circle of Willis is an uncommon manifestation of acquired human immunodeficiency virus (HIV infection and up to now only 15 cases have been published in the literature. For this reason we add our experience of this rare case, and review the most important aspects related to this entity. The patient is a 6 year old male with perinatal transmitted AIDS, tetraparethic, developed symptoms characterized by episodes of dystonic postures. The computed tomography of the brain showed aneurismal arteriopathy of the circle of Willis. He had a previous normal examination. The review

  12. The Erlangen Dose Optimization Trial for radiotherapy of benign painful shoulder syndrome. Long-term results

    International Nuclear Information System (INIS)

    Ott, O.J.; Hertel, S.; Gaipl, U.S.; Frey, B.; Schmidt, M.; Fietkau, R.

    2014-01-01

    To evaluate the long-term efficacy of pain reduction by two dose-fractionation schedules for radiotherapy of painful shoulder syndrome. Between February 2006 and February 2010, 312 evaluable patients were recruited for this prospective trial. All patients received low-dose orthovoltage radiotherapy. One course consisted of 6 fractions in 3 weeks. In the case of insufficient pain remission after 6 weeks, a second course was administered. Patients were randomly assigned to one of two groups to receive single doses of either 0.5 or 1.0 Gy. Endpoint was pain reduction. Pain was measured before radiotherapy, as well as immediately after (early response), 6 weeks after (delayed response) and approximately 3 years after (long-term response) completion of radiotherapy using a questionnaire-based visual analogue scale (VAS) and a comprehensive pain score (CPS). Median follow-up was 35 months (range 11-57). The overall early, delayed and long-term response rates for all patients were 83, 85 and 82%, respectively. The mean VAS scores before treatment and those for early, delayed and long-term response in the 0.5- and 1.0-Gy groups were 56.8±23.7 and 53.2±21.8 (p=0.16); 38.2±36.1 and 34.0±24.5 (p=0.19); 33.0±27.2 and 23.7±22.7 (p=0.04) and 27.9±25.8 and 32.1±26.9 (p=0.25), respectively. The mean CPS values before treatment and those for early, delayed and long-term response were 9.7±3.0 and 9.5±2.7 (p=0.31); 6.1±3.6 and 5.4±3.6 (p=0.10); 5.3±3.7 and 4.1±3.7 (p=0.05) and 4.0±3.9 and 5.3±4.4 (p=0.05), respectively. No significant differences in the quality of the long-term response were found between the 0.5- and 1.0-Gy arms (p=0.28). Radiotherapy is an effective treatment for the management of benign painful shoulder syndrome. For radiation protection reasons, the dose for a radiotherapy series should not exceed 3.0 Gy. (orig.)

  13. Experience of German Red Cross blood donor services with nucleic acid testing: results of screening more than 30 million blood donations for human immunodeficiency virus-1, hepatitis C virus, and hepatitis B virus.

    Science.gov (United States)

    Hourfar, Michael K; Jork, Christine; Schottstedt, Volkmar; Weber-Schehl, Marijke; Brixner, Veronika; Busch, Michael P; Geusendam, Geert; Gubbe, Knut; Mahnhardt, Christina; Mayr-Wohlfart, Uschi; Pichl, Lutz; Roth, W Kurt; Schmidt, Michael; Seifried, Erhard; Wright, David J

    2008-08-01

    The risk of transfusion-transmitted human immunodeficiency virus-1 (HIV-1), hepatitis C virus (HCV), and hepatitis B virus (HBV) infections is predominantly attributable to donations given during the early stage of infection when diagnostic tests may fail. In 1997, nucleic acid amplification technique (NAT)-testing was introduced at the German Red Cross (GRC) blood donor services to reduce this diagnostic window period (WP). A total of 31,524,571 blood donations collected from 1997 through 2005 were screened by minipool NAT, predominantly with pool sizes of 96 donations. These donations cover approximately 80 percent of all the blood collected in Germany during that period. Based on these data, the WP risk in the GRC blood donor population was estimated by using a state-of-the-art mathematic model. During the observation period, 23 HCV, 7 HIV-1, and 43 HBV NAT-only-positive donations were detected. On the basis of these data and estimated pre-NAT infectious WPs, the residual risk per unit transfused was estimated at 1 in 10.88 million for HCV (95% confidence interval [CI], 7.51-19.72 million), 1 in 4.30 million for HIV-1 (95% CI, 2.39-21.37 million), and 1 in 360,000 for HBV (95% CI, 0.19-3.36 million). Based on observed cases of breakthrough infections, the risk of transfusion-related infections may be even lower. The risk of a blood recipient becoming infected with HCV, HIV-1, or HBV has reached an extremely low level. Introduction of individual donation testing for HCV and HIV-1 would have a marginal effect on interception of WP donations.

  14. Urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome: long-term results.

    Science.gov (United States)

    Kajbafzadeh, Abdol-Mohammad; Rasouli, Mohammad Reza; Dianat, SeyedSaeid; Nezami, Behtash G; Mahboubi, Amir Hassan; Sina, Alireza

    2010-11-01

    The aim of the study was to evaluate the efficacy and safety of urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome (PBS). During a 10-year period, 7 infants with PBS and urethral hypoplasia presented either with open urachus or surgically created urinary diversion referred to our hospital. Five milliliters of normal saline was pushed via a 22-gauge plastic angiocatheter into the urethra with simultaneous finger pressure on the perineum to occlude the proximal urethra that was repeated with higher volumes of the solution (up to 20 mL). The procedure was continued until a 6F or 8F feeding tube catheter confirmed the urethral patency. Hydrodistension was repeated in 3-month intervals till complete patency was confirmed by imaging. Median age of the infants was 6 (1-8) months. All urethral hydrodistension were successful after 1 to 3 sessions. Follow-up imaging studies showed significant improvement in all patients except one. Natural and surgically created urinary diversions were closed in 6 infants. The hydrodistension create an equal and constant pressure into the urethral wall without any urethral damage. This technique can be considered along with the other available methods for management of urethral hypoplasia in selected cases of PBS. Copyright © 2010 Elsevier Inc. All rights reserved.

  15. [Preliminary result on the nosocomial infection of severe acute respiratory syndrome in one hospital of Beijing].

    Science.gov (United States)

    He, Yao; Jiang, Yong; Xing, Yu-bin; Zhong, Guang-lin; Wang, Lei; Sun, Zheng-ji; Jia, Hong; Chang, Qing; Wang, Yong; Ni, Bin; Chen, Shi-ping

    2003-07-01

    To study the transmission route of severe acute respiratory syndrome (SARS) nosocomial infection. Ten identified SARS patients were selected from a general hospital in March. Survey was carried out through a standardized questionnaire provided by Chinese Center for Disease Control and Prevention. Contents of the questionnaire would include: history of contact with SARS patient, route of infection, methods used for protection and so on. (1) Distribution os SARS patients were confined to 3 wards: 4, 5, and 6 on the 7, 8, 12, 13 and 14 floors in the west unit of the inpatient building. Most of the inpatients were elderly and having severe original diseases. (2) Index patients were the first generation source of transmission and they infected inpatients and medical staff, making them the second generation. People with latent infection who had close contact with SARS patients might also serve as the possible source of transmission. (3) The major transmission routes were: near distant droplet infection and close contact infection. There was also a clue to the probability of aerosol or droplet nuclei infection through air-conditioning and ventilation system. Nosocomial infection appeared to be the main characteristic of the SARS epidemic in the early stage of this hospital. Other than close contact and near space airborne transmission of SARS virus, the possibility of long-distance aerosol transmission called for further epidemiological and experimental studies in the future.

  16. Genitourinary syndrome of menopause in five Asian countries: results from the Pan-Asian REVIVE survey.

    Science.gov (United States)

    Chua, Y; Limpaphayom, K K; Cheng, B; Ho, C M; Sumapradja, K; Altomare, C; Huang, K

    2017-08-01

    The Pan-Asian REVIVE survey aimed to examine women's experiences with genitourinary syndrome of menopause (GSM) and their interactions with health-care professionals (HCPs). Self-completed surveys were administered face-to-face to 5992 women (aged 45-75 years) in Indonesia, Malaysia, Singapore, Taiwan, and Thailand. Of 638 postmenopausal women with GSM symptoms, only 35% were aware of the GSM condition, most of whom first heard of GSM through their physician (32%). The most common symptoms were vaginal dryness (57%) and irritation (43%). GSM had the greatest impact on sexual enjoyment (65%) and intimacy (61%). Only 25% had discussed their GSM symptoms with a HCP, and such discussions were mostly patient-initiated (64%) rather than HCP-initiated (24%). Only 21% had been clinically diagnosed with GSM and only 24% had ever used treatment for their symptoms. Three-quarters of those who had used treatment for GSM had discussed their symptoms with a HCP compared to only 9% of those who were treatment-naïve. GSM is underdiagnosed and undertreated in Asia. As discussion of GSM with HCPs appears to be a factor influencing women's awareness and treatment status, a more active role by HCPs to facilitate early discussions on GSM and its treatment options is needed.

  17. Loss of PKCδ results in characteristics of Sjögren's syndrome including salivary gland dysfunction.

    Science.gov (United States)

    Banninger, G P; Cha, S; Said, M S; Pauley, K M; Carter, C J; Onate, M; Pauley, B A; Anderson, S M; Reyland, M E

    2011-09-01

    Chronic infiltration of lymphocytes into the salivary and lacrimal glands of patients with Sjögren's syndrome (SS) leads to destruction of acinar cells and loss of exocrine function. Protein kinase C-delta (PKCδ) is known to play a critical role in B-cell maintenance. Mice in which the PKCδ gene has been disrupted have a loss of B-cell tolerance, multiple organ lymphocytic infiltration, and altered apoptosis. To determine whether PKCδ contributes to the pathogenesis of SS, we quantified changes in indicators of SS in PKCδ-/- mice as a function of age. Salivary gland histology, function, the presence of autoantibodies, and cytokine expression were examined. Submandibular glands were examined for the presence of lymphocytic infiltrates, and the type of infiltrating lymphocyte and cytokine deposition was evaluated by immunohistochemistry. Serum samples were tested by autoantibody screening, which was graded by its staining pattern and intensity. Salivary gland function was determined by saliva collection at various ages. PKCδ-/- mice have reduced salivary gland function, B220+ B-cell infiltration, anti-nuclear antibody production, and elevated IFN-γ in the salivary glands as compared to PKCδ+/+ littermates. PKCδ-/- mice have exocrine gland tissue damage indicative of a SS-like phenotype. © 2011 John Wiley & Sons A/S.

  18. Viral gene products and replication of the human immunodeficiency type 1 virus.

    Science.gov (United States)

    Morrow, C D; Park, J; Wakefield, J K

    1994-05-01

    The acquired immunodeficiency syndrome (AIDS) epidemic represents a modern-day plague that has not only resulted in a tragic loss of people from a wide spectrum of society but has reshaped our viewpoints regarding health care, the treatment of infectious diseases, and social issues regarding sexual behavior. There is little doubt now that the cause of the disease AIDS is a virus known as the human immunodeficiency virus (HIV). The HIV virus is a member of a large family of viruses termed retroviruses, which have as a hallmark the capacity to convert their RNA genome into a DNA form that then undergoes a process of integration into the host cell chromosome, followed by the expression of the viral genome and translation of viral proteins in the infected cell. This review describes the organization of the HIV-1 viral genome, the expression of viral proteins, as well as the functions of the accessory viral proteins in HIV replication. The replication of the viral genome is divided into two phases, the early phase and the late phase. The early phase consists of the interaction of the virus with the cell surface receptor (CD4 molecule in most cases), the uncoating and conversion of the viral RNA genome into a DNA form, and the integration into the host cell chromosome. The late phase consists of the expression of the viral proteins from the integrated viral genome, the translation of viral proteins, and the assembly and release of the virus. Points in the HIV-1 life cycle that are targets for therapeutic intervention are also discussed.

  19. Evidence for a Euro-American origin of human immunodeficiency virus (HIV).

    Science.gov (United States)

    Katner, H P; Pankey, G A

    1987-10-01

    Recent reports of the nonspecificity of the enzyme-linked immunosorbent assay (ELISA) test in African populations, significant genomic differences between simian T-cell lymphotropic virus and human immunodeficiency virus (HIV), and the early appearance of clinical acquired immunodeficiency syndroME (AIDS) in the US and Europe are powerful arguments against the assumption that AIDS originated in Africa. The authors postulate that HIV infection has been endemic in the Euro-American population at least since the beginning of the 20th century and that sociocultural changes led to the introduction of the virus into Africa. A search of the literature reveals 28 cases of disseminated Kaposi's sarcoma in the pre-epidemic 1902-66 period. In none of these cases are notations made on intravenous drug abuse, homosexuality, or other risk factors for AIDS. The majority of cases involved men, however. It is pointed out that, in a population where the incidence of a virus such as HIV is low, the number of sexual partners is limited, and intravenous drug abuse is nonexistent, an infection with as long a latency period as HIV may not only be expressed sporadically, but would probably not be recognized as a transmissible infection. On the other hand, the significant changes in these social factors that occurred as a result of the sexual revolution of the late 1960s and early 1970s would be expected to increase the spread of infection and clinical disease so that recognition would be achieved. During the past decade, there have been marked increases in the number of sexually transmitted infections in the homosexual male population. The efficiency of anal intercourse as a mode of transmission probably accounts for the fact that HIV infection first expressed itself in this population.

  20. A Cerebellar Tremor in a Patient with Human Immunodeficiency Virus-1 Associated with Progressive Multifocal Leukoencephalopathy

    Directory of Open Access Journals (Sweden)

    Hee-Jin Kim

    2009-10-01

    Full Text Available Progressive multifocal leukoencephalopathy (PML is a demyelinating disease of the central nervous system (CNS caused by JC virus infection in oligodendrocytes, especially in patients with acquired immunodeficiency syndrome (AIDS. Movement disorders associated with PML are very rare. Here, we report a case of PML in an AIDS patient who presented with a cerebellar tremor, caused by lesions in the cerebellar outflow tract. A cerebellar tremor can be a rare clinical manifestation in patients with PML.

  1. A Cerebellar Tremor in a Patient with Human Immunodeficiency Virus-1 Associated with Progressive Multifocal Leukoencephalopathy

    Science.gov (United States)

    Kim, Hee-Jin; Lee, Jae-Jung; Lee, Phil Hyu

    2009-01-01

    Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system (CNS) caused by JC virus infection in oligodendrocytes, especially in patients with acquired immunodeficiency syndrome (AIDS). Movement disorders associated with PML are very rare. Here, we report a case of PML in an AIDS patient who presented with a cerebellar tremor, caused by lesions in the cerebellar outflow tract. A cerebellar tremor can be a rare clinical manifestation in patients with PML. PMID:24868366

  2. Immune defects in active mycobacterial diseases in patients with primary immunodeficiency diseases (PIDs

    Directory of Open Access Journals (Sweden)

    Wen-I Lee

    2011-12-01

    Full Text Available Natural human immunity to the mycobacteria group, including Mycobacterium tuberculosis, Bacille Calmette-Guérin (BCG or nontuberculous mycobacteria (NTM, and/or Salmonella species, relies on the functional IL-12/23-IFN-γ integrity of macrophages (monocyte/dendritic cell connecting to T lymphocyte/NK cells. Patients with severe forms of primary immunodeficiency diseases (PIDs have more profound immune defects involving this impaired circuit in patients with severe combined immunodeficiencies (SCID including complete DiGeorge syndrome, X-linked hyper IgM syndrome (HIGM (CD40L mutation, CD40 deficiency, immunodeficiency with or without anhidrotic ectodermal dysplasia (NEMO and IKBA mutations, chronic granulomatous disease (CGD and hyper IgE recurrent infection syndromes (HIES. The patients with severe PIDs have broader diverse infections rather than mycobacterial infections. In contrast, patients with an isolated inborn error of the IL-12/23-IFN-γ pathway are exclusively prone to low-virulence mycobacterial infections and nontyphoid salmonella infections, known as Mendelian susceptibility to the mycobacterial disease (MSMD phenotype. Restricted defective molecules in the circuit, including IFN-γR1, IFN-γR2, IL-12p40, IL-12R-β1, STAT-1, NEMO, IKBA and the recently discovered CYBB responsible for autophagocytic vacuole and proteolysis, and interferon regulatory factor 8 (IRF8 for dendritic cell immunodeficiency, have been identified in around 60% of patients with the MSMD phenotype. Among all of the patients with PIDs referred for investigation since 1985, we have identified four cases with the specific defect (IFNRG1 for three and IL12RB for one, presenting as both BCG-induced diseases and NTM infections, in addition to some patients with SCID, HIGM, CGD and HIES. Furthermore, manifestations in patients with autoantibodies to IFN-γ (autoAbs-IFN-γ, which is categorized as an anticytokine autoantibody syndrome, can resemble the relatively

  3. Remote postradiation immunodeficiency a disorder of endocrine regulation and thymic function. Role of intersystems interactions

    International Nuclear Information System (INIS)

    Savina, N.P.

    1999-01-01

    A total of 265 reports on radiation effects on the immune, nervous, and endocrine systems are reviewed. The role of the thymus, hypothalamus, and pituitary in remote manifestations of immunodeficiency in patients with cancer of the head and neck, treated by radiotherapy, is discussed. The pathogenesis of stubborn postradiation disturbances in immunoendocrine homeostasis after local exposure of the thymus and hypothalamus-pituitary at doses of 1-10 Gy, characterized as a syndrome, is analyzed [ru

  4. AGE AND GENDER MAY INFLUENCE THE RESULTS OF ROUX-EN-Y GASTRIC BYPASS? Metabolic syndrome parameters

    Directory of Open Access Journals (Sweden)

    Stephan Garcia ANDRADE-SILVA

    2014-09-01

    Full Text Available Context Severe obesity affects the body favoring the development of serious diseases, including hypertension, diabetes mellitus, atherosclerosis and non alcoholic fatty liver disease. Bariatric procedures increased in Brazil in the last decade. Objectives The purpose of this study was to verify if gender and age in date of procedure resulted significant differences in metabolic syndrome parameters after surgery. Methods The study involved 205 medical records of adult patients undergoing Roux-en-Y gastric bypass, stratified by gender and age groups and followed one year by a multidisciplinary team. Results It was observed significant decrease in body mass index, fasting glucose and insulin at all ages and both genders. Lipid profile showed significant improvements except high density lipoprotein cholesterol. Ectopic fat in the liver has decreased after 6 months in patients classified with steatosis at baseline. Patients classified as hypertensive blood pressure levels decreased 6 months after surgical intervention. Conclusions Roux-en-Y gastric bypass proved to be an important tool in remission of metabolic syndrome parameters. The reduction of body mass accompanied to decrease in insulin resistance resulted in lower prevalence of comorbidities associated with obesity. The benefits were similar and extended both genders and all age groups between 18 and 65 years old.

  5. Assessing the impact of caring for a child with Dravet syndrome: Results of a caregiver survey.

    Science.gov (United States)

    Campbell, Jonathan D; Whittington, Melanie D; Kim, Chong H; VanderVeen, Gina R; Knupp, Kelly G; Gammaitoni, Arnold

    2018-03-01

    The objective of this study was to describe and quantify the impact of caring for a child with Dravet syndrome (DS) on caregivers. We surveyed DS caregivers at a single institution with a large population of patient with DS. Survey domains included time spent/difficulty performing caregiving tasks (Oberst Caregiving Burden Scale, OCBS); caregiver health-related quality of life (EuroQoL 5D-5L, EQ-5D); and work/activity impairment (Work Productivity and Activity Impairment questionnaire, WPAI). Modified National Health Interview Survey (NHIS) questions were included to assess logistical challenges associated with coordinating medical care. Thirty-four primary caregivers responded, and 30/34 respondents completed the survey. From OCBS, providing transportation, personal care, and additional household tasks required the greatest caregiver time commitment; arranging for child care, communication, and managing behavioral problems presented the greatest difficulty. EuroQoL 5D-5L domains with the greatest impact on caregivers (0=none, 5=unable/extreme) were anxiety/depression (70% of respondents≥slight problems, 34%≥moderate) and discomfort/pain (57% of respondents≥slight problems, 23%≥moderate). The mean EQ-5D general health visual analogue scale (VAS) score (0=death; 100=perfect health) was 67 (range, 11-94). Respondents who scored caregiver health. On the WPAI, 26% of caregivers missed >1day of work in the previous week, with 43% reporting substantial impact (≥6, scale=1-10) on work productivity; 65% reported switching jobs, quitting jobs, or losing a job due to caregiving responsibilities. National Health Interview Survey responses indicated logistical burdens beyond the home; 50% of caregivers made ≥10 outpatient visits in the past year with their child with DS. Caring for patients with DS exerts physical, emotional, and time burdens on caregivers. Supportive services for DS families are identified to highlight an unmet need for DS treatments. Copyright

  6. Churg-Strauss syndrome: a new endotype of severe asthma? Results of 14 Turkish patients.

    Science.gov (United States)

    Yılmaz, İnsu; Çelik, Gülfem; Aydın, Ömür; Özdemir, Seçil Kepil; Soyyiğit, Şadan; Sözener, Zeynep; Özgüçlü, Selcan; Atasoy, Çetin; Düzgün, Nurşen; Mungan, Dilşad; Sin, Betül; Demirel, Yavuz Selim; Mısırlıgil, Zeynep

    2015-07-01

    Churg-Strauss syndrome (CSS) is a rare multisystem vasculitis. Considering the variation of autoimmune diseases in different races, it is of interest to determine whether any outstanding features exist for Turkish patients with CSS. The aim of this study was to evaluate the clinical and serological features of the disease, the treatment, and long-term follow-up details, and to investigate possible etiological factors of Turkish CSS patients. The study included 14 patients who were diagnosed with CSS, and followed by our department between 2004 and 2012. Possible etiological factors, initial symptoms, clinical presentations, treatment, as well as outcomes were documented. The study was approved by the local ethics. All patients fulfilled the American College of Rheumatology criteria. Initial symptoms were worsening asthma (n = 14; 100%) and skin lesions (n = 6; 43%). All patients had a diagnosis of asthma and nasal polyps, whereas 57.1% had aspirin hypersensitivity at the time of diagnosis. The lungs (100%) and skin (43%) were most commonly involved. Peripheral eosinophilia dominated on initial presentations of all patients. Initial treatments included oral methyl prednisolone in all cases, whereas cyclophosphamide and azathioprine were used in three cases. Relapses were detected in five cases. None of the cases were able to stop the oral corticosteroid treatment. No fatalities were observed. We herein describe a new severe asthma endotype in connection with CSS. We suggest that physicians who deal with uncontrolled severe asthma cases should consider CSS in the presence of nasal polyps, aspirin hypersensitivity, and especially peripheral blood eosinophilia over 10%. © 2014 John Wiley & Sons Ltd.

  7. Association between Milk Consumption and Metabolic Syndrome among Korean Adults: Results from the Health Examinees Study

    Directory of Open Access Journals (Sweden)

    Sangah Shin

    2017-10-01

    Full Text Available It has been suggested that a greater dairy consumption, particularly of milk, may have contributed in lowering the prevalence of metabolic syndrome (MetS. A cross-sectional analysis was conducted to examine the association between milk consumption and MetS, and its components among Korean adults aged 40–69. A total of 130,420 subjects (43,682 men and 86,738 women from the Health Examinees Study were selected for the final analysis. Milk consumption was estimated using a validated 106-item food frequency questionnaire. MetS was defined using the National Cholesterol Education Program, Adult Treatment Panel III (NCEP III. Logistic regression analyses were performed to calculate the odds ratios (ORs and 95% confidence intervals (CIs between milk consumption and MetS after adjusting for potential confounders. In this study, the average milk consumption was 77.9 g/day, with the overall prevalence of MetS being 26.1% (29.1% in men and 24.6% in women. We found that the prevalence of the MetS was significantly lower in subjects with higher milk consumption (p < 0.0001. Adjusted OR for MetS was significantly lower in the highest milk consumption category (≥1 serving/day among men; ≥2 serving/day among women than those in the lowest milk consumption category (OR: 0.92 95%CI: 0.86–0.99, p trend = 0.0160 in men; OR: 0.68, 95%CI: 0.60–0.76, p trend < 0.0001 in women. Overall, higher milk consumption was inversely associated with the MetS components: elevated waist circumference, elevated triglyceride, and reduced high-density lipoprotein cholesterol (HDL-C (all p trend < 0.05. This study concludes that higher milk consumption is associated with the lower odds of MetS in Korean adults.

  8. Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results.

    Science.gov (United States)

    Patel, Nishant D; Crawford, Todd; Magruder, J Trent; Alejo, Diane E; Hibino, Narutoshi; Black, James; Dietz, Harry C; Vricella, Luca A; Cameron, Duke E

    2017-02-01

    Early experience with Loeys-Dietz syndrome (LDS) suggested an aggressive aortopathy with high risk of aneurysm dissection and rupture at young ages and at smaller aortic diameters than in other connective tissue disorders. We reviewed our experience with LDS to re-examine our indications and outcomes of surgical management. We reviewed all patients with a diagnosis of LDS who underwent cardiovascular surgery at our institution. The primary endpoint was mortality, and secondary endpoints included postoperative complications and need for reintervention. Seventy-nine operated patients with LDS were identified. Mean age at first operation was 25 years, 39 (49%) were female, and 38 (48%) were children (age <18 years). Six (8%) patients presented with acute dissection. Five (6%) patients had a bicuspid aortic valve, and all presented with an ascending aortic aneurysm with a mean root diameter of 3.5cm. Twenty (25%) patients had a previous sternotomy. Sixty-five (82%) patients underwent aortic root replacement, of whom 52 underwent a valve-sparing operation and 4 had concomitant arch replacement. Mean aortic root diameter in this group was 4.2 cm. Nine (11%) patients underwent aortic arch replacement, 2 (3%) had isolated ascending aorta replacement, and 3 (4%) underwent open thoracoabdominal repair. There were 2 (3%) operative and 8 late deaths. Nineteen patients underwent subsequent operations for late aneurysm and/or dissection. Mean follow-up was 6 years (range 0-24 years). Kaplan-Meier survival was 88% at 10 years. Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed. Copyright © 2016 The American Association for Thoracic Surgery

  9. Ethnopharmacology of human immunodeficiency virus in South ...

    African Journals Online (AJOL)

    Administrator

    2006-10-02

    Oct 2, 2006 ... This mini-review takes a look at the evaluations of South African medicinal plants to determine ... Key words: Human immunodeficiency virus, Medicinal plants, South Africa. ... The greatest degree of antiviral activity against.

  10. Human immunodeficiency virus (HIV) infection in tuberculosis ...

    African Journals Online (AJOL)

    Human immunodeficiency virus (HIV) infection in tuberculosis patients in Addis ... METHODS: A cross-sectional survey whereby blood sample was collected ... of co-infection appeared to have increased compared to previous studies, 6.6%, ...

  11. Cauli: a mouse strain with an Ift140 mutation that results in a skeletal ciliopathy modelling Jeune syndrome.

    Directory of Open Access Journals (Sweden)

    Kerry A Miller

    2013-08-01

    Full Text Available Cilia are architecturally complex organelles that protrude from the cell membrane and have signalling, sensory and motility functions that are central to normal tissue development and homeostasis. There are two broad categories of cilia; motile and non-motile, or primary, cilia. The central role of primary cilia in health and disease has become prominent in the past decade with the recognition of a number of human syndromes that result from defects in the formation or function of primary cilia. This rapidly growing class of conditions, now known as ciliopathies, impact the development of a diverse range of tissues including the neural axis, craniofacial structures, skeleton, kidneys, eyes and lungs. The broad impact of cilia dysfunction on development reflects the pivotal position of the primary cilia within a signalling nexus involving a growing number of growth factor systems including Hedgehog, Pdgf, Fgf, Hippo, Notch and both canonical Wnt and planar cell polarity. We have identified a novel ENU mutant allele of Ift140, which causes a mid-gestation embryonic lethal phenotype in homozygous mutant mice. Mutant embryos exhibit a range of phenotypes including exencephaly and spina bifida, craniofacial dysmorphism, digit anomalies, cardiac anomalies and somite patterning defects. A number of these phenotypes can be attributed to alterations in Hedgehog signalling, although additional signalling systems are also likely to be involved. We also report the identification of a homozygous recessive mutation in IFT140 in a Jeune syndrome patient. This ENU-induced Jeune syndrome model will be useful in delineating the origins of dysmorphology in human ciliopathies.

  12. [Computational prediction of human immunodeficiency resistance to reverse transcriptase inhibitors].

    Science.gov (United States)

    Tarasova, O A; Filimonov, D A; Poroikov, V V

    2017-10-01

    Human immunodeficiency virus (HIV) causes acquired immunodeficiency syndrome (AIDS) and leads to over one million of deaths annually. Highly active antiretroviral treatment (HAART) is a gold standard in the HIV/AIDS therapy. Nucleoside and non-nucleoside inhibitors of HIV reverse transcriptase (RT) are important component of HAART, but their effect depends on the HIV susceptibility/resistance. HIV resistance mainly occurs due to mutations leading to conformational changes in the three-dimensional structure of HIV RT. The aim of our work was to develop and test a computational method for prediction of HIV resistance associated with the mutations in HIV RT. Earlier we have developed a method for prediction of HIV type 1 (HIV-1) resistance; it is based on the usage of position-specific descriptors. These descriptors are generated using the particular amino acid residue and its position; the position of certain residue is determined in a multiple alignment. The training set consisted of more than 1900 sequences of HIV RT from the Stanford HIV Drug Resistance database; for these HIV RT variants experimental data on their resistance to ten inhibitors are presented. Balanced accuracy of prediction varies from 80% to 99% depending on the method of classification (support vector machine, Naive Bayes, random forest, convolutional neural networks) and the drug, resistance to which is obtained. Maximal balanced accuracy was obtained for prediction of resistance to zidovudine, stavudine, didanosine and efavirenz by the random forest classifier. Average accuracy of prediction is 89%.

  13. Amyloidosis in association with spontaneous feline immunodeficiency virus infection.

    Science.gov (United States)

    Asproni, Pietro; Abramo, Francesca; Millanta, Francesca; Lorenzi, Davide; Poli, Alessandro

    2013-04-01

    Tissues from 34 naturally feline immunodeficiency virus (FIV)-infected cats, 13 asymptomatic cats and 21 cats with signs of feline acquired immunodeficiency syndrome (F-AIDS), and 35 FIV-seronegative subjects were examined to determine the presence of amyloid deposits. Twenty experimentally FIV-infected cats and five specific pathogen-free (SPF) control cats were also included in the study. Paraffin-embedded sections from kidney and other organs were submitted to histological and histochemical analysis. Amyloid deposits were identified by a modified Congo red stain and confirmed by electron microscopy to demonstrate the presence of amyloid fibrils in amyloid positive glomeruli. In all positive cases, secondary amyloidosis was identified with potassium permanganate pretreatment and amyloid type was further characterised by immunohistochemistry using primary antibodies against human AA and feline AL amyloids. Amyloid deposits were present in different tissues of 12/34 (35%) naturally FIV-infected cats (seven presenting F-AIDS and five in asymptomatic phase) and in 1/30 FIV-seronegative cats. All the experimentally FIV-infected and SPF subjects showed no amyloid deposits. Amyloidosis has been reported in human lentiviral infections, and the data reported here demonstrate the need, in naturally FIV-infected cats, to consider the presence of amyloidosis in differential diagnosis of hepatic and renal disorders to better assess the prognosis of the disease.

  14. Identification of genetic defects in primary immunodeficiencies by whole exome sequencing

    DEFF Research Database (Denmark)

    Christiansen, Mette; Jensen, Jens Magnus Bernth; Veirum, Jens Erik

    2014-01-01

    to hypogammaglobulinaemia, and increased risk of both infections as well as cancer. We employed whole exome sequencing (WES) to identify mutations associated with primary immunodeficiency in severely affected children. We present WES data on 2 patients with severe immunodeficiency. WES was performed using TruSeq exome kit...... and severe infections including sepsis. Second, we identified compound heterozygote stopgain mutations in RAD52 and a heterozygote mutation in LRRC8A in a 7 year-old girl with T-cell deficiency, reduced T-cell mediated B-cell activity, hypogammaglobulinaemia, prolonged splenomegali and benign adenopathy. RAD......52 has not previously been linked to immunodeficiency and we are currently investigating the functional consequences. Knowledge of the mechanisms underlying immunodeficiencies is a prerequisite for understanding disease pathogenesis. WES allows the demonstration of immune defects that may result from...

  15. What are the factors associated with human immunodeficiency virus/sexually transmitted infection screening behaviour among heterosexual men patronising entertainment establishments who engaged in casual or paid sex? - Results from a cross-sectional survey in an Asian urban setting.

    Science.gov (United States)

    Lim, Raymond Boon Tar; Tham, Dede Kam Tyng; Cheung, Olive N Y; Tai, Bee Choo; Chan, Roy; Wong, Mee Lian

    2016-12-19

    Late presentation of human immunodeficiency virus (HIV) is associated with heterosexual transmission, particularly among heterosexual men in Asia. Although data on HIV/sexually transmitted infection (STI) testing behaviour is increasing, information is still lacking among heterosexual men who receive far lesser attention and are generally invisible in HIV/ STI prevention, particularly in the Asian urban setting. The aim of this study was to assess the prevalence of HIV/STI testing among heterosexual men patronising entertainment establishments (EEs) who engaged in casual or paid sex in Singapore, and the factors associated with this behaviour. This was a cross-sectional survey involving 604 participants using time location sampling between March and May 2015. For multivariable analysis, we used a mixed effects Poisson regression model with backward stepwise approach to account for clustering by venue and to obtain the adjusted prevalence ratio (aPR) for the association of various factors with HIV/STI testing. Among 604 at-risk participants, only 163 (27.0%) had gone for HIV or STI testing in the past 6 months. Of this, 83.4% of them specifically underwent HIV testing. In multivariable analysis, HIV/STI testing increased with being non-Chinese (aPR 1.50; 95% CI: 1.08-2.06), having engaged in anal sex with casual or paid partner in the past 6 months (aPR 1.80; 95% CI: 1.27-2.57), number of partners in the past 6 months (aPR 1.03; 95% CI: 1.01-1.05) and HIV knowledge score (aPR 1.11; 95% CI: 1.05-1.16). Among those who reported non-consistent condom use with casual or paid partner, almost half of them (47.9%) perceived that they were at low risk for HIV/STI. Sigmatisation and discrimination was another common barrier for non-testing. Despite being at risk of HIV/STI, the low prevalence of testing coupled with a high prevalence of risky sexual behaviour among this group of heterosexual men in Singapore calls for a need for HIV/STI prevention interventions in the EE

  16. Nijmegen breakage syndrome (NBS

    Directory of Open Access Journals (Sweden)

    Chrzanowska Krystyna H

    2012-02-01

    Full Text Available Abstract Nijmegen breakage syndrome (NBS is a rare autosomal recessive syndrome of chromosomal instability mainly characterized by microcephaly at birth, combined immunodeficiency and predisposition to malignancies. Due to a founder mutation in the underlying NBN gene (c.657_661del5 the disease is encountered most frequently among Slavic populations. The principal clinical manifestations of the syndrome are: microcephaly, present at birth and progressive with age, dysmorphic facial features, mild growth retardation, mild-to-moderate intellectual disability, and, in females, hypergonadotropic hypogonadism. Combined cellular and humoral immunodeficiency with recurrent sinopulmonary infections, a strong predisposition to develop malignancies (predominantly of lymphoid origin and radiosensitivity are other integral manifestations of the syndrome. The NBN gene codes for nibrin which, as part of a DNA repair complex, plays a critical nuclear role wherever double-stranded DNA ends occur, either physiologically or as a result of mutagenic exposure. Laboratory findings include: (1 spontaneous chromosomal breakage in peripheral T lymphocytes with rearrangements preferentially involving chromosomes 7 and 14, (2 sensitivity to ionizing radiation or radiomimetics as demonstrated in vitro by cytogenetic methods or by colony survival assay, (3 radioresistant DNA synthesis, (4 biallelic hypomorphic mutations in the NBN gene, and (5 absence of full-length nibrin protein. Microcephaly and immunodeficiency are common to DNA ligase IV deficiency (LIG4 syndrome and severe combined immunodeficiency with microcephaly, growth retardation, and sensitivity to ionizing radiation due to NHEJ1 deficiency (NHEJ1 syndrome. In fact, NBS was most commonly confused with Fanconi anaemia and LIG4 syndrome. Genetic counselling should inform parents of an affected child of the 25% risk for further children to be affected. Prenatal molecular genetic diagnosis is possible if disease

  17. Immunodeficiency among children with recurrent invasive pneumococcal disease

    DEFF Research Database (Denmark)

    Ingels, Helene; Schejbel, Lone; Lundstedt, A C

    2015-01-01

    examined. RESULTS: In total, rIPD were observed in 54 children (68 cases of rIPD of 2192 IPD cases). Children with classical risk factors for IPD were excluded, and among the remaining 22 children, 15 were eligible for analysis. Of these 6 (40%) were complement C2-deficient. Impaired vaccination response......BACKGROUND: Recurrent invasive pneumococcal disease (rIPD) occurs mostly in children with an underlying disease, but some cases remain unexplained. Immunodeficiency has been described in children with rIPD, but the prevalence is unknown. We used a nationwide registry of all laboratory......-confirmed cases of rIPD to identify cases of unexplained rIPD and examine them for immunodeficiency. METHODS: Cases of rIPD in children 0-15 years of age from 1980 to 2008 were identified. Children without an obvious underlying disease were screened for complement function, T-cell, B-cell, natural killer...

  18. Cardiovascular system and physical working capacity in patients who had acute radiation syndrome as the result of Chernobyl accident

    International Nuclear Information System (INIS)

    Belyi, D.; Gergel, O.; Kovalenko, A.

    1996-01-01

    The functional state of cardiovascular system has been studied since 1986 in 168 patients who had acute radiation syndrome as the result of Chernobyl accident. There was revealed a progressive increase of cardiovascular system pathology. The number of patients with pathological signs at ECG increased from 4.8 % in 1987 to 11.3 % in 1994 and with myocardial hypertrophy from 1.2 % to 22.6 %. The number of patients with coronary heart disease increased on 17.2% and with essential hypertension on 15.5%. The physical working capacity reduced to 50-60 % of a due level for healthy persons. Two patients suffered from acute myocardial infarction during this period of observation. Thirteenth patients died from 1987 to 1995. Among them 4 patient died in a result of acute cardiac failure. The development of cardiovascular pathology has no any correlation with a dose of exposure. Three factors of cardiovascular pathology growth are supposed

  19. Human immunodeficiency virus and menopause.

    Science.gov (United States)

    Kanapathipillai, Rupa; Hickey, Martha; Giles, Michelle

    2013-09-01

    This article aims to review currently available evidence for women infected with human immunodeficiency virus (HIV) and menopause and to propose clinical management algorithms. Key studies addressing HIV and menopause have been reviewed, specifically age of menopause onset in HIV-infected women, frequency of menopausal symptoms, comorbidities associated with HIV and aging (including cardiovascular disease and bone disease), treatment of menopausal symptoms, and prevention of comorbidities in HIV-infected women. Studies suggest an earlier onset of menopause in HIV-infected women, with increased frequency of symptoms. Cardiovascular disease risk may be increased in this population, with combination antiretroviral therapy (cART) and chronic inflammation associated with HIV, contributing to increased risk. Chronic inflammation and cART have been independently implicated in bone disease. No published data have assessed the safety and efficacy of hormone therapy in relation to symptoms of menopause, cardiovascular risk, and bone disease among HIV-infected women. Few studies on menopause have been conducted in HIV-infected women compared with HIV-uninfected women. Many questions regarding age of menopause onset, frequency of menopausal symptoms and associated complications such as bone disease and cardiovascular disease, and efficacy of treatment among HIV-infected women remain. The incidence and severity of some of these factors may be increased in the setting of HIV and cART.

  20. Do Pregnancy and Parenthood Affect the Course of PCO Syndrome? Initial Results from the LIPCOS Study (Lifestyle Intervention for Patients with Polycystic Ovary Syndrome [PCOS]).

    Science.gov (United States)

    Stassek, J; Ohnolz, F; Hanusch, Y; Schmidmayr, M; Berg, D; Kiechle, M; Seifert-Klauss, V R

    2015-11-01

    Introduction: The impact of pregnancy and parenthood on the long-term course of PCOS (polycystic ovary syndrome is still not known. The LIPCOS study (Lifestyle Intervention for Patients with Polycystic Ovary Syndrome [PCOS] - using the example of pregnancy and parenthood) systematically investigates long-term changes in PCOS symptoms. Method and Patients: The LIPCOS pilot study sent out a questionnaire to 403 patients who had presented with oligomenorrhea between 1991 and 2002. The prospective LIPCOS main study systematically investigated 64 women using structured interviews about lifestyle changes in the last 10 years, created a detailed hormone profile of these women and carried out vaginal ultrasound to calculate ovarian score. Results: Ovarian volume and ovarian score were not significantly lower for women with children (n = 25) compared to women with PCOS who had not had children (n = 39; p = 0.226). More women with children than women who did not have children currently reported a regular daily lifestyle, and the difference was statistically significant (92 % [n = 23/25] vs. 61.5 % [n = 24/39]; p = 0.009). Ten years ago or before the birth of their first child, respectively, no such difference was found between both groups (52 vs. 51.3 %). Over the last 10 years, women with children were more likely to have shorter cycles compared to women without children (p = 0.441). 88 % of women with children compared to 69.2 % of women without children reported that currently they had a "healthy diet" (p = 0.130). Serum testosterone levels were slightly lower for women with children (67.6 % of the upper limits of normal ranges) compared to women without children (80 % of the upper limits of normal ranges), but because of the small subgroup sizes the difference was not statistically significant (p = 0.106). Conclusion: The LIPCOS study shows for the first time that pregnancy and parenthood may have an impact on the long

  1. A case of the interval form of carbon monoxide poisoning with apallic syndrome resulted in complete recovery after treatment with oxygen at high pressure

    International Nuclear Information System (INIS)

    Matsuishi, Takeshi; Motomura, Hiroshi; Boku, Shoji; Mori, Koichi.

    1984-01-01

    A 44-year-old male patient had apallic syndrome 32 days after 12-hour coma resulting from acute carbon monoxide poisoning and could return to the social life by undergoing fifty oxygen treatments at high pressure. EEG revealed no low voltage, which is usually seen in cases of apallic syndrome. Cranial CT scan revealed that low density areas in the white matter of the brain had improved with improvement of clinical symptoms. (Namekawa, K.)

  2. Association between opium use and metabolic syndrome among an urban population in Southern Iran: Results of the Kerman Coronary Artery Disease Risk Factor Study (KERCADRS

    Directory of Open Access Journals (Sweden)

    Gholamreza Yousefzadeh

    2015-01-01

    Full Text Available BACKGROUND: Along with the established effects of opium on metabolic parameters, stimulatory or inhibitory effects of opium on metabolic syndrome are also predictable. This study aimed to examine the association of opium use with metabolic syndrome and its components. METHODS: This study was conducted on 5332 out of 5900 original sample participants enrolled in a population-based cohort entitled the Kerman Coronary Artery Disease Risk Study in Iran from 2009 to 2011. The subjects were divided into three groups of “non-opium users” (NOUs = 4340 subjects, “former opium users” (FOUs = 176 subjects, and dependent and occasional people named “current opium users” (COUs = 811 subjects. Metabolic syndrome was defined according to two International Diabetes Federation (IDF and National Cholesterol Education Program Adult Treatment Panel III (NCEP ATP III definition criteria. RESULTS: The overall prevalence of IDF defined-metabolic syndrome among NOUs, FOUs, and COUs was 36.4%, 27.3%, and 39.0%, respectively; which was significantly higher in the COUs group (P = 0.012. However, no significant difference was revealed across the three groups in prevalence of NCEP defined-metabolic syndrome (NOUs = 37.2%, FOUs = 30.1%, and COUs = 39.6%, P = 0.058. The odds for IDF defined-metabolic syndrome was higher in both COUs [odd ratio (OR = 1.28, P = 0.028] and FOUs (OR = 1.57, P = 0.045 compared with NOUs as the reference adjusting gender, age, body mass index, and cigarette smoking. However, the appearance of NCEP defined-metabolic syndrome could not be predicted by opium use. CONCLUSION: Opium use can be associated with an increased risk for metabolic syndrome based on IDF criteria and thus preventing the appearance of metabolic syndrome by avoiding opium use can be a certain approach to preventing cardiovascular disease.   

  3. PRIMARY IMMUNODEFICIENCY: STATUS OF A PROBLEM TODAY. RUSSIAN NETWORK OF JMF-CENTERS

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    E. A. Latysheva

    2013-01-01

    Full Text Available The problems of primary immunodeficiency in Russia and the ways of solving of them are discussed in the article. Primary immunodeficiency is a group of rare diseases, so awareness of this pathology in the medical community and among patients is very low. This leads to late diagnosis and inadequate treatment of patients with such conditions. The result of the late beginning of treatment is early development of disability, and the high mortality rate of patients, as well as the high costs of the treatment of complications of primary immunodeficiency and sick-leave certificates for the government. Today in time and adequate therapy allows patients not only to reach adulthood without signs of disability, and to lead an active way of life, but to have healthy children. Given the high cost of therapy in many countries, the issue of providing patients with life-saving drugs remains unresolved. The global practice is to involve social organizations and funds. One of the foundations supporting educational programs, development of laboratories and research in the field of primary immunodeficiency is the Foundation of the Jeffrey Modell. A network of centres for primary immunodeficiency supported by the Jeffrey Modell Foundation (JMF-centers has started its functioning over the territory of the Russian Federation since 2011 in order to improve diagnostics and treatment of patients with primary immunodeficiency. A brief description of activity of these centers is presented in the article.

  4. Establishing the tolerability and performance of tamarind seed polysaccharide (TSP in treating dry eye syndrome: results of a clinical study

    Directory of Open Access Journals (Sweden)

    Valente Cristiana

    2007-03-01

    Full Text Available Abstract Background One of the problems arising from available preparations for dry eye syndrome is the limited residence time of products on the ocular surface. In this paper, we look at an innovative new treatment for dry eye, tamarind seed polysaccharide (TSP. TSP possesses mucomimetic, mucoadhesive and pseudoplastic properties. The 'mucin-like' molecular structure of TSP is similar to corneal and conjunctival mucin 1 (MUC1, a transmembrane glycoprotein thought to play an essential role in protecting and wetting the corneal surface and may explain its increased retention on the eye surface. Methods The activity of TSP and hyaluronic acid (HA in the treatment of dry eye syndrome was compared in an open-label, randomised, single-centre clinical study. Thirty patients were randomised to receive three or more applications per day of either TSP 0.5%, TSP 1% or HA 0.2% (Hyalistil™ over a period of 90 days. The primary objective of tolerability was assessed by visual analogue scale (VAS, scoring of specific symptoms and the incidence of adverse events. Secondary objectives included improvement in stability of the precorneal tear film, subjective symptoms and corneal and conjunctival staining. Results TSP 0.5% and 1% were comparable to HA 0.2% with regard to both primary and secondary objective parameters. TSP 1% showed benefits over HA 0.2% for the subjective symptoms; trouble blinking, ocular burning and foreign body sensation. Conclusion This study suggests that TSP 0.5% and 1% offer at least equivalent relief to HA 0.2% for dry eye syndrome. All treatments demonstrated optimal tolerability and are suitable for frequent use in the therapy of dry eye. TSP 1% produced promising results in terms of improvements in certain patient symptoms and suggests benefits of the TSP formulation. This study paves the way for a larger study to further establish the performance and safety of TSP compared with HA and highlights the need to expand this therapeutic

  5. Epstein-Barr virus-associated adult respiratory distress syndrome in a patient with AIDS: a case report and review

    DEFF Research Database (Denmark)

    Stopyra, G A; Multhaupt, H A; Alexa, L

    1999-01-01

    BACKGROUND: Epstein-Barr virus (EBV) infection has been associated with fatal pneumonitis in immunocompetent patients. We present a case of fatal adult respiratory distress syndrome caused by EBV infection in a patient with acquired immunodeficiency syndrome (AIDS), to our knowledge the first....... RESULTS: Strikingly numerous lymphocytes were positive for EBV early RNA in the case patient's spleen, lymph nodes, and hepatic portal areas. In addition to positive lymphocytes in the lung, EBV-infected pneumocytes were also present. Electron microscopy also demonstrated viral material in lymphocytes...

  6. Bronchial obstruction syndrome as a predictor of mortality in cardiac surgery: interim results of prospective cohort study

    Directory of Open Access Journals (Sweden)

    Д. Н. Пономарев

    2016-01-01

    Full Text Available Objective. To investigate the effect of bronchial obstruction syndrome on perioperative characteristics in patients undergoing coronary artery bypass grafting (CABG.Methods. Body plethysmography and spiroergometry at rest were used to study respiratory function and consumption of O2. To evaluate the effectiveness of pulmonary ventilation, the oxygen utilization coefficient of (O2CU was calculated in 178 patients prior to scheduled surgery. The relationship of external ventilation parameters and perioperative clinical characteristics was analyzed. Results. A bronchial obstruction syndrome was detected in 30 (16.9% patients, with 21 (11.2% of them having no chronic obstructive pulmonary disease diagnosis. 4 patients (2.3% with obstructive pulmonary disease had no bronchial obstruction. Thus, chronic obstructive pulmonary disease was misdiagnosed in 13.5% cases. In patients undergoing isolated CABG, negative relationship was revealed between the Tiffno index and hospital stay (regression coefficient –6.9, 95%, confidence interval –14.4… 0.6; p = 0.07. In patients operated with the myocardium stabilized, bimammary grafting tended to increase hospital stay by an average of 4.3 days (95% CI 1.5–7.1; p=0.003. The majority of patients had a low O2CU. O2CU lower than 16.2 ml/l is associated with an increased risk of postoperative atrial fibrillation (AF in the postoperative period (OR 2.96; 95% CI 1.01–9.83; p = 0.04. No significant associations were observed between the degree of bronchial obstruction and the number of perioperative complications. Conclusion. The degree of bronchial obstruction could be positively associated with the length of hospital stay. A critically low level of O2CU is explained by an increased risk of postoperative AF. Further research might result in the identification of predictors for respiratory complications and long-term mortality in patients after CABG.

  7. Immunodeficiency and laser magnetic therapy in urology

    Science.gov (United States)

    Maati, Moufagued; Rozanov, Vladimir V.; Avdoshin, V. P.

    1996-11-01

    The importance of immunodeficiency problem has increased last time not only due to AIDS appearance, but also to a great extent as a result of the development and active practical use of the methods of immunology parameters investigations. Al great pharmaceutical firms are organizing the process of creating the drugs, influencing on the different phases of immunity, but unfortunately, the problem of their adverse effect and connected complications is till today a milestone. A great number of investigations, proving a good effect of laser-magnetic therapy concerning immune system have been done today. There is, in particular, changing of blood counts and immunologic tests after intravenous laser irradiation of blood. Intravenous laser irradiation of blood results in increasing of lymphocytes, T-immuno stimulation, stabilization of t-lymphocyte subpopulation, increasing of t-lymphocyte helper activity and decreasing of suppressor one.Under this laser action number of circulating immune complexes is decreased, and blood serum bactericide activity and lisozyme number are increased.

  8. Radiotracer transit measurements as an index of regional cerebral blood flow. Pt. 2. Results in acute alcohol withdrawal syndromes

    International Nuclear Information System (INIS)

    Dobrzanski, T.

    1975-01-01

    The data obtained in 72 male chronic alcoholics with acute alcohol withdrawal syndroms give evidence that there is a significant correlation between the numerical value of the cerebral radiorheographic index and the severity of the psychopathological syndrome (especially of the clouding of sensorium) in these patients. (author)

  9. Pseudotumor Cerebri Resulting in Empty Sella Syndrome and Multiple Pituitary Hormone Deficiencies

    Science.gov (United States)

    2017-09-14

    A 17 year old male was referred to pediatric endocrinology with concerns for stalled puberty in the setting of known PTC. He was diagnosed with PTC...size led to a preliminary laboratory evaluation. This resulted In a referral to pediatric endocrinology for significantly low testosterone and an...studies were reviewed and a partially empty sella was appreciated by a pediatric radiologist on retrospective evaluation (Image 1 ). There were no

  10. [Endoscopic calcaneoplasty (ECP) in Haglund's syndrome. Indication, surgical technique, surgical findings and results].

    Science.gov (United States)

    Jerosch, J; Sokkar, S; Dücker, M; Donner, A

    2012-06-01

    Posterior calcaneal exostosis treatment modalities have given rise to many controversial opinions. After failure of the conservative treatment, surgical bursectomy and resection of the calcaneal exostosis are indicated by many authors. But clinical studies also show a high rate of unsatisfactory results with a relative high incidence of complications. The minimally invasive surgical technique by an endoscopic calcaneoplasty (ECP) could be an option to overcome some of these problems. Between 1999 und 2010 we operated 164 patients with an age range between 16 and 67 years, 81 males and 83 females. The radiological examination prior to surgery documented in all cases a posterior superior calcaneal exostosis that showed friction to the Achilles tendon. All patients included in the study had no clinical varus of the hind foot, nor cavus deformities. All patients had undergone a trial of conservative treatment for at least 6 months and did not show a positive response. The average follow-up was 46.3 (range: 8-120) months. According to the Ogilvie-Harris score 71 patients presented good and 84 patients excellent results, while 5 patients showed fair results, and 4 patients only poor results. All the post-operative radiographs showed sufficient resection of the calcaneal spur. In 61 patients the preoperative MRI showed a partial rupture of the Achilles tendon close to the insertion side. In no case could we observe a complete tear at the time of follow-up. Only minor postoperative complications were observed. In many patients we could observe a chondral layer at the posterior aspect of the calcaneus. Close to the intersion the Achilles tendon showed also in many patients a chondroide metaplasia. ECP is an effective and minimally invasive procedure for the treatment of patients with calcaneal exostosis. After a short learning curve the endoscopic exposure is superior to the open technique, has less morbidity, less operating time, and nearly no complications. Moreover, the

  11. Prevalence of metabolic syndrome in Iranian professional drivers: results from a population based study of 12,138 men.

    Directory of Open Access Journals (Sweden)

    Iraj Mohebbi

    Full Text Available BACKGROUND: It is evident that professional driving is associated with substantial changes in lifestyle habits. Professional drivers are prone to metabolic syndrome (MetS and its complications because their working environment is characterized by numerous stress factors such as lack of physical activity due to working in a fixed position, disruption in diet, and irregular sleep habits. The aim of the present study was to estimate the prevalence of MetS among long distance drivers residing in West Azerbaijan province in Iran. MATERIALS: To assess the prevalence of metabolic syndrome among professional long distance drivers, 12138 participants were enrolled in this cross sectional study. The MetS was defined using International Diabetes Federation criteria. RESULTS: Among 12138 participants, 3697 subjects found to be MetS. The crude and age-adjusted rates of MetS were 30.5% and 32.4% respectively. Based on Body mass index (BMI, 5027 subjects (41.4% were overweight (BMI ≥ 25.01-30 kg/m2, and 2592 (21.3% were obese (BMI ≥ 30.01 kg/m2. The presence of central obesity was more common than other components. The associations of MetS with BMI, pack-year smoking, age, weekly driving duration and driving experiences were significant in the logistic regression. By increasing BMI, pack-year smoking, age, weekly driving duration and driving experiences, odds ratio of MetS was increased. CONCLUSION: The study suggests that MetS has become a noteworthy health problem among Iranian long distance drivers. This might be due to the following facts: sitting in a fixed position for long hours while working, cigarette smoking, job stress, unhealthy diet and lack of physical activity. Educational programs should be established for promoting healthy lifestyle and also for early detection and appropriate interventions.

  12. Ethnic differences in the prevalence of metabolic syndrome: results from a multi-ethnic population-based survey in Malaysia.

    Science.gov (United States)

    Rampal, Sanjay; Mahadeva, Sanjiv; Guallar, Eliseo; Bulgiba, Awang; Mohamed, Rosmawati; Rahmat, Ramlee; Arif, Mohamad Taha; Rampal, Lekhraj

    2012-01-01

    The prevalence of metabolic syndrome is increasing disproportionately among the different ethnicities in Asia compared to the rest of the world. This study aims to determine the differences in the prevalence of metabolic syndrome across ethnicities in Malaysia, a multi-ethnic country. In 2004, we conducted a national cross-sectional population-based study using a stratified two-stage cluster sampling design (N = 17,211). Metabolic syndrome was defined according to the International Diabetes Federation/National Heart, Lung and Blood Institute/American Heart Association (IDF/NHLBI/AHA-2009) criteria. Multivariate models were used to study the independent association between ethnicity and the prevalence of the metabolic syndrome. The overall mean age was 36.9 years, and 50.0% participants were female. The ethnic distribution was 57.0% Malay, 28.5% Chinese, 8.9% Indian and 5.0% Indigenous Sarawakians. The overall prevalence of the metabolic syndrome was 27.5%, with a prevalence of central obesity, raised triglycerides, low high density lipoprotein cholesterol, raised blood pressure and raised fasting glucose of 36.9%, 29.3%, 37.2%, 38.0% and 29.1%, respectively. Among those Malaysia was high, with marked differences across ethnicities. Ethnic Chinese had the lowest prevalence of metabolic syndrome, while ethnic Indians had the highest. Indigenous Sarawakians showed a marked increase in metabolic syndrome at young ages.

  13. Common Variable Immunodeficiency: Diagnosis, Management, and Treatment.

    Science.gov (United States)

    Abbott, Jordan K; Gelfand, Erwin W

    2015-11-01

    Common variable immunodeficiency (CVID) refers to a grouping of antibody deficiencies that lack a more specific genetic or phenotypic classification. It is the immunodeficiency classification with the greatest number of constituents, likely because of the numerous ways in which antibody production can be impaired and the frequency in which antibody production becomes impaired in human beings. CVID comprises a heterogeneous group of rare diseases. Consequently, CVID presents a significant challenge for researchers and clinicians. Despite these difficulties, both our understanding of and ability to manage this grouping of complex immune diseases has advanced significantly over the past 60 years. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Malfunction of nuclease ERCC1-XPF results in diverse clinical manifestations and causes Cockayne syndrome, xeroderma pigmentosum, and Fanconi anemia.

    Science.gov (United States)

    Kashiyama, Kazuya; Nakazawa, Yuka; Pilz, Daniela T; Guo, Chaowan; Shimada, Mayuko; Sasaki, Kensaku; Fawcett, Heather; Wing, Jonathan F; Lewin, Susan O; Carr, Lucinda; Li, Tao-Sheng; Yoshiura, Koh-ichiro; Utani, Atsushi; Hirano, Akiyoshi; Yamashita, Shunichi; Greenblatt, Danielle; Nardo, Tiziana; Stefanini, Miria; McGibbon, David; Sarkany, Robert; Fassihi, Hiva; Takahashi, Yoshito; Nagayama, Yuji; Mitsutake, Norisato; Lehmann, Alan R; Ogi, Tomoo

    2013-05-02

    Cockayne syndrome (CS) is a genetic disorder characterized by developmental abnormalities and photodermatosis resulting from the lack of transcription-coupled nucleotide excision repair, which is responsible for the removal of photodamage from actively transcribed genes. To date, all identified causative mutations for CS have been in the two known CS-associated genes, ERCC8 (CSA) and ERCC6 (CSB). For the rare combined xeroderma pigmentosum (XP) and CS phenotype, all identified mutations are in three of the XP-associated genes, ERCC3 (XPB), ERCC2 (XPD), and ERCC5 (XPG). In a previous report, we identified several CS cases who did not have mutations in any of these genes. In this paper, we describe three CS individuals deficient in ERCC1 or ERCC4 (XPF). Remarkably, one of these individuals with XP complementation group F (XP-F) had clinical features of three different DNA-repair disorders--CS, XP, and Fanconi anemia (FA). Our results, together with those from Bogliolo et al., who describe XPF alterations resulting in FA alone, indicate a multifunctional role for XPF. Copyright © 2013 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  15. The breakdown of the cytokine network subsequent to human immunodeficiency virus infection

    Directory of Open Access Journals (Sweden)

    M. Clerici

    1995-01-01

    Full Text Available The acquired immunodeflciency syndrome (AIDS is a clinically multifaceted disease induced by infection with the human immunodeficiency virus (HIV. HIV infection results in a complex pattern of immunologic alterations that leads to the development of AIDS in the majority of HIV seropositive (HIV+ individuals. The reduction in CD4 T lymphocyte counts is the hallmark of HIV infection; nevertheless, long before the reduction in CD4 counts reaches critical levels, a series of profound and complex defects that impair the function of CD4 T lymphocytes can be detected. Thus, HIV infection is characterized by quantitative and qualitative defects affecting CD4 T lymphocytes. It was suggested recently that programmed cell death (PCD is an important mechanism leading to CD4 depletion in HIV infection, and that susceptibility of peripheral lymphocytes to PCD is differentially regulated by diverse cytokines. Thus, type 1 cytokines would protect CD4 lymphocytes against PCD, whereas type 2 cytokines would not protect against, and could augment, PCD. We suggest that the qualitative alterations of the immune response provoke the CD4 depletion characteristic of HIV disease via type 2 cytokinemediated augmentation of PCD, and are therefore ultimately responsible for the progression of HIV infection. Finally, we summarize recent data showing that three correlates of disease progression: emergence of HIV strains with syncitium-inducing ability (SI, type 1-to-type 2 cytokine shift, and CD4 depletion, are significantly associated, suggesting a complex interconnected virologic-immunologic pathogenesis of HIV infection.

  16. Primary immunodeficiency investigation in patients during and after hospitalization in a pediatric Intensive Care Unit

    Directory of Open Access Journals (Sweden)

    Erica Suavinho

    2014-03-01

    Full Text Available Objective: To analyze whether the patients with severe infections, admitted in the Pediatric Intensive Care Unit of the Hospital de Clínicas of the Universidade Federal de Uberlândia, underwent the active screening for primary immunodeficiencies (PID. Methods: Retrospective study that assessed the data records of patients with any severe infections admitted in the Pediatric Intensive Care Unit, covering a period from January 2011 to January 2012, in order to confirm if they performed an initial investigation for PID with blood count and immunoglobulin dosage. Results: In the studied period, 53 children were hospitalized with severe infections in the Pediatric Intensive Care Unit, and only in seven (13.2% the initial investigation of PID was performed. Among these patients, 3/7 (42.8% showed quantitative alterations in immunoglobulin G (IgG levels, 1/7 (14.3% had the diagnosis of cyclic neutropenia, and 1/7 (14.3% presented thrombocytopenia and a final diagnosis of Wiskott-Aldrich syndrome. Therefore, the PID diagnosis was confirmed in 5/7 (71.4% of the patients. Conclusions: The investigation of PID in patients with severe infections has not been routinely performed in the Pediatric Intensive Care Unit. Our findings suggest the necessity of performing PID investigation in this group of patients.

  17. Psychological problems of families and health workers dealing with people infected with human immunodeficiency virus 1.

    Science.gov (United States)

    Maj, M

    1991-03-01

    The psychological problems of the families of human immunodeficiency virus 1 (HIV-1)-infected people, and of the health workers taking care of them, have been addressed in a few empirical studies and in several anecdotal reports and theoretical contributions. Apparently, HIV-1 infection and acquired immunodeficiency syndrome (AIDS) are able to elicit a wide range of emotional reactions, from rejection and refusal to provide care to immersion in the infected person's needs and burnout. Since irrational fears and attitudes play an important role in conditioning these reactions, education may not be sufficient to change behaviour. Counselling sessions and mutual support groups are often the most appropriate contexts where fears and concerns can receive an individually tailored response, and where formal and informal caregivers can be helped to manage stress.

  18. Diagnosis and therapy of polycystic ovarian syndrome: results of a survey among German gynecologists with a review on literature.

    Science.gov (United States)

    Doll, Ines; Doll, Richard; Buhling, Kai J

    2012-03-01

    Polycystic ovarian syndrome is one of the most frequent endocrine dysfunctions in women in their fertile age. To date, a clear definition of the disease remains controversial among experts, partly because study results concerning diagnostics and therapy are incoherent. In this survey, we intend to give an insight into the diagnostics and treatment regimes of PCOS in Germany and subsequently compare these results to the current recommendations found in international literature. In 2005, 7,000 outpatient gynecologists received a questionnaire with questions on PCOS definition, diagnostics and treatment. The survey shows that it remains difficult for gynecologists to restrict themselves to the few definition criteria defined in the 2003 Rotterdam PCOS Consensus Workshop. Nevertheless, therapy and diagnosis of PCOS show a uniform treatment pattern. The current recommendations found in international literature have been widely implemented into clinical practice. Modern therapeutic approaches such as the use of metformin are increasingly acknowledged. Further studies dealing with the diagnosis and therapy of PCOS will be necessary in order to establish guidelines, especially when looking at patients without a desire for reproduction.

  19. Cost-effectiveness of internet-based cognitive behavior therapy for irritable bowel syndrome: results from a randomized controlled trial

    Directory of Open Access Journals (Sweden)

    Paxling Björn

    2011-04-01

    Full Text Available Abstract Background Irritable Bowel Syndrome (IBS is highly prevalent and is associated with a substantial economic burden. Cognitive behavior therapy (CBT has been shown to be effective in treating IBS. The aim of this study was to evaluate the cost-effectiveness of a new treatment alternative, internet-delivered CBT based on exposure and mindfulness exercises. Methods Participants (N = 85 with IBS were recruited through self-referral and were assessed via a telephone interview and self-report measures on the internet. Participants were randomized to internet-delivered CBT or to a discussion forum. Economic data was assessed at pre-, post- and at 3-month and 1 year follow-up. Results Significant cost reductions were found for the treatment group at $16,806 per successfully treated case. The cost reductions were mainly driven by reduced work loss in the treatment group. Results were sustained at 3-month and 1 year follow-up. Conclusions Internet-delivered CBT appears to generate health gains in IBS treatment and is associated with cost-savings from a societal perspective.

  20. Brain anatomy in adults with velocardiofacial syndrome with and without schizophrenia - Preliminary results of a structural magnetic resonance Imaging study

    NARCIS (Netherlands)

    van Amelsvoort, Therese; Daly, Eileen; Henry, Jayne; Robertson, Dene; Ng, Virginia; Owen, Michael; Murphy, Kieran C.; Murphy, Declan G. M.

    2004-01-01

    Context: Velocardiofacial syndrome is associated with interstitial deletions of chromosome 22q11, mild to borderline learning disability, characteristic dysmorphology, and a high prevalence of schizophrenia. The biological basis for this increased risk for schizophrenia is unknown, but people with

  1. Limited impact on self-concept in individuals with Lynch syndrome; results from a national cohort study

    DEFF Research Database (Denmark)

    Petersen, Helle Vendel; Esplen, Mary Jane; Ladelund, Steen

    2011-01-01

    An increasing number of individuals seek genetic counseling and hereby learn about hereditary cancer in the family. Lynch syndrome is associated with an inherited high risk for colorectal and gynecological cancer, but knowledge about how family members at risk perceive their situation is limited....... We used the national Danish HNPCC register to collect data on self-concept from 413 individuals with Lynch syndrome. The recently developed Lynch syndrome self-concept scale contains 20 items within two subscales related to stigma-vulnerability and bowel symptom-related anxiety. Significantly higher...... more often reported by women (odds ratio 1.8) and by individuals with less education (OR 1.8). This study provides the first extended use of the Lynch syndrome self-concept scale and suggests that the majority of the Danish mutation carriers adapt well to the situation, though knowledge about...

  2. Associations Between Geriatric Syndromes and Mortality in Community-Dwelling Elderly: Results of a National Longitudinal Study in Taiwan.

    Science.gov (United States)

    Huang, Chi-Chang; Lee, Jenq-Daw; Yang, Deng-Chi; Shih, Hsin-I; Sun, Chien-Yao; Chang, Chia-Ming

    2017-03-01

    Although geriatric syndromes have been studied extensively, their interactions with one another and their accumulated effects on life expectancy are less frequently discussed. This study examined whether geriatric syndromes and their cumulative effects are associated with risks of mortality in community-dwelling older adults. Data were collected from the Taiwan Longitudinal Study in Aging in 2003, and the participant survival status was followed until December 31, 2007. A total of 2744 participants aged ≥65 years were included in this retrospective cohort study; 634 died during follow-up. Demographic factors, comorbidities, health behaviors, and geriatric syndromes, including underweight, falls, functional impairment, depressive condition, and cognitive impairment, were assessed. Cox proportional hazard regression analysis was used to estimate the hazard ratios (HRs) and 95% confidence intervals (CIs) for the probability of survival according to the cumulative number of geriatric syndromes. The prevalence of geriatric syndromes increased with age. Mortality was significantly associated with age ≥75 years; male sex; ≤6 years of education; history of stroke, malignancy; smoking; not drinking alcohol; and not exercising regularly. Geriatric syndromes, such as underweight, functional disability, and depressive condition, contributed to the risk of mortality. The accumulative model of geriatric syndromes also predicted higher risks of mortality (N = 1, HR 1.50, 95% CI 1.19-1.89; N = 2, HR 1.69, 95% CI 1.25-2.29; N ≥ 3, HR 2.43, 95% CI 1.62-3.66). Community-dwelling older adults who were male, illiterate, receiving institutional care, underweight, experiencing a depressive condition, functionally impaired, and engaging in poor health behavior were more likely to have a higher risk of mortality. The identification of geriatric syndromes might help to improve comprehensive care for community-dwelling older adults. Copyright © 2016 AMDA – The Society for

  3. Leisure time physical activity in middle age predicts the metabolic syndrome in old age: results of a 28-year follow-up of men in the Oslo study

    Science.gov (United States)

    Holme, Ingar; Tonstad, Serena; Sogaard, Anne Johanne; Larsen, Per G Lund; Haheim, Lise Lund

    2007-01-01

    Background Data are scarce on the long term relationship between leisure time physical activity, smoking and development of metabolic syndrome and diabetes. We wanted to investigate the relationship between leisure time physical activity and smoking measured in middle age and the occurrence of the metabolic syndrome and diabetes in men that participated in two cardiovascular screenings of the Oslo Study 28 years apart. Methods Men residing in Oslo and born in 1923–32 (n = 16 209) were screened for cardiovascular diseases and risk factors in 1972/3. Of the original cohort, those who also lived in same area in 2000 were invited to a repeat screening examination, attended by 6 410 men. The metabolic syndrome was defined according to a modification of the National Cholesterol Education Program criteria. Leisure time physical activity, smoking, educational attendance and the presence of diabetes were self-reported. Results Leisure time physical activity decreased between the first and second screening and tracked only moderately between the two time points (Spearman's ρ = 0.25). Leisure time physical activity adjusted for age and educational attendance was a significant predictor of both the metabolic syndrome and diabetes in 2000 (odds ratio for moderately vigorous versus sedentary/light activity was 0.65 [95% CI, 0.54–0.80] for the metabolic syndrome and 0.68 [0.52–0.91] for diabetes) (test for trend P < 0.05). However, when adjusted for more factors measured in 1972/3 including glucose, triglycerides, body mass index, treated hypertension and systolic blood pressure these associations were markedly attenuated. Smoking was associated with the metabolic syndrome but not with diabetes in 2000. Conclusion Physical activity during leisure recorded in middle age prior to the current waves of obesity and diabetes had an independent predictive association with the presence of the metabolic syndrome but not significantly so with diabetes 28 years later in life, when

  4. CXCR4-specific Nanobodies as potential therapeutics for WHIM syndrome

    DEFF Research Database (Denmark)

    de Wit, Raymond H; Heukers, Raimond; Brink, Hendrik

    2017-01-01

    WHIM syndrome is a rare congenital immunodeficiency disease, named after its main clinical manifestations: Warts, Hypogammaglobulinemia, Infections and Myelokathexis. The disease is primarily caused by C-terminal truncation mutations of the chemokine receptor CXCR4. Consequently, these CXCR4-WHIM...... as alternative therapeutics for CXCR4-associated diseases like WHIM syndrome....

  5. Wolf-Hirschhorn Syndrome Candidate 1 Is Necessary for Correct Hematopoietic and B Cell Development.

    Science.gov (United States)

    Campos-Sanchez, Elena; Deleyto-Seldas, Nerea; Dominguez, Veronica; Carrillo-de-Santa-Pau, Enrique; Ura, Kiyoe; Rocha, Pedro P; Kim, JungHyun; Aljoufi, Arafat; Esteve-Codina, Anna; Dabad, Marc; Gut, Marta; Heyn, Holger; Kaneda, Yasufumi; Nimura, Keisuke; Skok, Jane A; Martinez-Frias, Maria Luisa; Cobaleda, Cesar

    2017-05-23

    Immunodeficiency is one of the most important causes of mortality associated with Wolf-Hirschhorn syndrome (WHS), a severe rare disease originated by a deletion in chromosome 4p. The WHS candidate 1 (WHSC1) gene has been proposed as one of the main genes responsible for many of the alterations in WHS, but its mechanism of action is still unknown. Here, we present in vivo genetic evidence showing that Whsc1 plays an important role at several points of hematopoietic development. Particularly, our results demonstrate that both differentiation and function of Whsc1-deficient B cells are impaired at several key developmental stages due to profound molecular defects affecting B cell lineage specification, commitment, fitness, and proliferation, demonstrating a causal role for WHSC1 in the immunodeficiency of WHS patients. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

  6. Wolf-Hirschhorn Syndrome Candidate 1 Is Necessary for Correct Hematopoietic and B Cell Development

    Directory of Open Access Journals (Sweden)

    Elena Campos-Sanchez

    2017-05-01

    Full Text Available Immunodeficiency is one of the most important causes of mortality associated with Wolf-Hirschhorn syndrome (WHS, a severe rare disease originated by a deletion in chromosome 4p. The WHS candidate 1 (WHSC1 gene has been proposed as one of the main genes responsible for many of the alterations in WHS, but its mechanism of action is still unknown. Here, we present in vivo genetic evidence showing that Whsc1 plays an important role at several points of hematopoietic development. Particularly, our results demonstrate that both differentiation and function of Whsc1-deficient B cells are impaired at several key developmental stages due to profound molecular defects affecting B cell lineage specification, commitment, fitness, and proliferation, demonstrating a causal role for WHSC1 in the immunodeficiency of WHS patients.

  7. Renal disease in cats infected with feline immunodeficiency virus.

    Science.gov (United States)

    Baxter, K J; Levy, J K; Edinboro, C H; Vaden, S L; Tompkins, M B

    2012-01-01

    Feline immunodeficiency virus (FIV) and human immunodeficiency virus (HIV) infection cause similar clinical syndromes of immune dysregulation, opportunistic infections, inflammatory diseases, and neoplasia. Renal disease is the 4th most common cause of death associated with HIV infection. To investigate the association between FIV infection and renal disease in cats. Client-owned cats (153 FIV-infected, 306 FIV-noninfected) and specific-pathogen-free (SPF) research colony cats (95 FIV-infected, 98 FIV-noninfected). A mixed retrospective/prospective cross-sectional study. Blood urea nitrogen (BUN), serum creatinine, urine specific gravity (USG), and urine protein:creatinine ratio (UPC) data were compared between FIV-infected and FIV-noninfected cats. In FIV-infected cats, total CD4+ and CD8+ T lymphocytes were measured using flow cytometry, and CD4+:CD8+ T lymphocyte ratio was calculated. Renal azotemia was defined as a serum creatinine ≥ 1.9 mg/dL with USG ≤ 1.035. Proteinuria was defined as a UPC > 0.4 with an inactive urine sediment. Among the client-owned cats, no association was detected between FIV infection and renal azotemia (P = .24); however, a greater proportion of FIV-infected cats were proteinuric (25.0%, 16 of 64 cats) compared to FIV-noninfected cats (10.3%, 20 of 195 cats) (P < .01). Neither neuter status nor health status were risk factors for proteinuria in FIV-infected cats, but UPC was positively correlated with the CD4+:CD8+ T lymphocyte ratio (Spearman's rho = 0.37, P = .01). Among the SPF research colony cats, no association was detected between FIV infection and renal azotemia (P = .21) or proteinuria (P = .25). Proteinuria but not azotemia was associated with natural FIV infection. Copyright © 2012 by the American College of Veterinary Internal Medicine.

  8. Changing patterns of human immunodeficiency virus-associated neuropathology

    Directory of Open Access Journals (Sweden)

    Gray Francoise

    2007-01-01

    Full Text Available This paper describes the evolution of the pathogenic concepts associated with the infection by the human immunodeficiency virus (HIV, with emphasis to the pathology of the nervous system. Although the first description of damage to the nervous system in the acquired immunodeficiency syndrome (AIDS only appeared in 1982, the dramatic diffusion of the epidemic worldwide, as well as the invariably rapidly fatal outcome of the disease before the introduction of efficient treatment, generated from the beginning an enormous amount of research and re-thinking on a number of pathogenetic concepts. Less than 25 years after the first autopsy series on AIDS patients were published and the virus responsible for AIDS was identified, satisfactory definition and classification of a number of neuropathological complications of HIV infection have been established. This has led to the establishment of accurate clinical and biological diagnosis of the main neurological complications of the disease, which remain a major cause of disability and death in patients. Clinical and experimental studies have provided essential insight into the pathogenesis of CNS lesions and the natural history of the disorder. The relatively recent introduction of effective antiretroviral therapy in 1995-6 dramatically improved the course of prognosis of HIV disease. However, there remain a number of unsolved pathogenetic issues, the most puzzling of which remains the precise mechanism of neuronal damage underlying the specific HIV-related cognitive disorder (HIV-dementia. In addition, although antiretroviral therapy has changed the course of neurological complications, new issues have emerged, such as the lack of improvement or even paradoxical deterioration of the neurological status in treated patients. Interpretation of these complications remains largely speculative, partly because of the small number of neuropathological studies related to the beneficial consequence of this

  9. CCR5 Signal Transduction in Macrophages by Human Immunodeficiency Virus and Simian Immunodeficiency Virus Envelopes

    OpenAIRE

    Arthos, James; Rubbert, Andrea; Rabin, Ronald L.; Cicala, Claudia; Machado, Elizabeth; Wildt, Kathryne; Hanbach, Meredith; Steenbeke, Tavis D.; Swofford, Ruth; Farber, Joshua M.; Fauci, Anthony S.

    2000-01-01

    The capacity of human immunodeficiency virus (HIV) and simian immunodeficiency virus (SIV) envelopes to transduce signals through chemokine coreceptors on macrophages was examined by measuring the ability of recombinant envelope proteins to mobilize intracellular calcium stores. Both HIV and SIV envelopes mobilized calcium via interactions with CCR5. The kinetics of these responses were similar to those observed when macrophages were treated with MIP-1β. Distinct differences in the capacity o...

  10. Seroprevalence and Risk Factors for Human Immunodeficiency ...

    African Journals Online (AJOL)

    This study sought to determine the seroprevalence of human immunodeficiency virus (HIV), hepatitis C virus (HCV), and hepatitis B surface antigen (HBsAg) among blood donors at Bolga-tanga Regional Hospital, Ghana by blood group type, sex and age and also determining the asso-ciation, if any, in the occurrence of the ...

  11. Seroprevalence of Helicobacter pylori in human immunodeficiency ...

    African Journals Online (AJOL)

    Background: This study assessed the seroprevalence of Helicobacter pylori antibodies among Iranian patients with human immunodeficiency virus (HIV) infection. It also examines whether anti H. pylori seroprevalence was associated with the severity of the HIV infection or the antiretroviral treatment. Material and Methods: ...

  12. Primary immunodeficiencies and B-cell lymphomas.

    Science.gov (United States)

    Martín-Mateos, María Anunciación; Piquer Gibert, Mónica

    In primary immunodeficiencies there is a failure in the anti-tumor defense. Common variable immunodeficiency (CVID) is one of the most common primary immunodeficiencies characterized by an alteration in the differentiation of B lymphocytes (BL). Epstein-Barr virus (EBV) is an ubiquitous virus that selectively infects the BL. In patients with immunodeficiency, uncontrolled proliferation of infected BL and the action of viral proteins promote the development of lymphomas. At the University Hospital Sant Joan de Deu, Barcelona, 28 patients were diagnosed with CVID from 2000 to 2013. This paper describes four patients who developed non-Hodgkin's lymphoma (NHL). The lymphoma was associated with EBV in two of the cases. Patients were<18 years old, diagnosed with lymphoma between 4 and 13 years old. Two patients were treated with rituximab as monotherapy and achieved complete remission. Two patients were treated with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) and radiotherapy or rituximab and achieved complete remission. Early detection of EBV infections and NHL in all patients diagnosed with CVID is recommended, regardless of age at diagnosis. Copyright © 2016 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  13. Exposure to Human Immunodeficiency Disease. What Precautions ...

    African Journals Online (AJOL)

    Background: The Human Immunodeficiency Virus (HIV) epidemic is more pronounced in sub-Saharan Africa. The ever-increasing prevalence of HIV infection and the continued improvement in clinical management has increased the likelihood of these patients being managed by healthcare workers. The aim of the review ...

  14. Human Immunodeficiency Virus (HIV) Seropositivity In African ...

    African Journals Online (AJOL)

    A seroprevalence study of Human immunodeficiency virus (HIV) infection in new patients attending the eye clinic of LAUTECH Teaching Hospital in Osogbo, Osun State, Nigeria showed that twenty-nine patients 2.7%) were positive to HIV1. No patient was positive to HIV 2. There were 21 males (72.4%) and 8 females ...

  15. Post exposure prophylaxis against human immunodeficiency virus ...

    African Journals Online (AJOL)

    Objective: To determine the level of awareness, knowledge and practice of human immunodeficiency virus post exposure prophylaxis (HIV PEP) among paediatricians in Nigeria. Methodology: The study was a cross sectional questionnairebased survey conducted among paediatrcians that attended the Paediatric ...

  16. Post exposure prophylaxis against human immunodeficiency virus ...

    African Journals Online (AJOL)

    2015-11-23

    Nov 23, 2015 ... Abstract: Objective: To deter- mine the level of awareness, knowledge and practice of human immunodeficiency virus post ex- posure prophylaxis (HIV PEP) among paediatricians in Nigeria. Methodology: The study was a cross sectional questionnaire- based survey conducted among paediatrcians that ...

  17. Mitochondrial dysfunction and human immunodeficiency virus ...

    African Journals Online (AJOL)

    Human immunodeficiency virus (HIV) infection and the pharmacological treatment thereof have both been shown to affect mitochondrial function in a number of tissues, and each may cause specific organ pathology through specific mitochondrial pathways. HIV has been shown to kill various tissue cells by activation of ...

  18. Comparative periodontal status of human immunodeficiency virus ...

    African Journals Online (AJOL)

    Background: There are diverse reports on the prevalence and severity of chronic periodontitis in human immunodeficiency virus (HIV) positive persons. Few studies have been carried out in developing countries in Sub.Saharan Africa. This study was aimed at comparing the prevalence and severity of chronic periodontitis of ...

  19. Transurethral prostatectomy in human immunodeficiency virus ...

    African Journals Online (AJOL)

    A 63-year old man was admitted to our Hospital with urethral catheter in situ and having failed medical therapy, he opted for transurethral prostatectomy (TURP) which was done without any post-operative complication. He was known to be afflicted with human immunodeficiency virus and on treatment for 3 years. He also ...

  20. Transurethral prostatectomy in human immunodeficiency virus ...

    African Journals Online (AJOL)

    Human immunodeficiency virus (HIV) infection is increasing world-wide and highly active antiretroviral treatment ... Hospital with urethral catheter in situ and having failed medical therapy, he opted for transurethral ... endoscopic visualization of operation field, the .... percutaneous exposure: Centers for Disease Control and.

  1. Benign painful shoulder syndrome. Initial results of a single-center prospective randomized radiotherapy dose-optimization trial

    International Nuclear Information System (INIS)

    Ott, O.J.; Hertel, S.; Gaipl, U.S.; Frey, B.; Schmidt, M.; Fietkau, R.

    2012-01-01

    Background and purpose: To compare the efficacy of two different dose-fractionation schedules for radiotherapy of patients with benign painful shoulder syndrome. Patients and methods: Between February 2006 and February 2010, 312 consecutive evaluable patients were recruited for this prospective randomized trial. All patients received radiotherapy with an orthovoltage technique. One radiotherapy course consisted of 6 single fractions in 3 weeks. In case of insufficient remission of pain after 6 weeks, a second radiation series was performed. Patients were randomly assigned to receive either single doses of 0.5 or 1.0 Gy. The endpoint was pain reduction. Pain was measured before, right after, and 6 weeks after radiotherapy using a visual analogue scale (VAS) and a comprehensive pain score (CPS). Results: The overall response rate for all patients was 83% directly after and 85% 6 weeks after radiotherapy. The mean VAS values before, directly after, and 6 weeks after treatment for the 0.5 and 1.0 Gy groups were 56.8 ± 23.7 and 53.2 ± 21.8 (p = 0.158), 38.2 ± 26.1 and 34.0 ± 24.5 (p = 0.189), and 33.0 ± 27.2 and 23.7 ± 22.7 (p = 0.044), respectively. The mean CPS before, directly after, and 6 weeks after treatment was 9.7 ± 3.0 and 9.5 ± 2.7 (p = 0.309), 6.1 ± 3.6 and 5.4 ± 3.6 (p = 0.096), 5.3 ± 3.7 and 4.1 ± 3.7 (p = 0.052), respectively. Despite a slight advantage in the VAS analysis for the 1.0 Gy group for delayed response, the CPS analysis revealed no statistically significant differences between the two single-dose trial arms for early (p = 0.652) and delayed response quality (p = 0.380). Conclusion: Radiotherapy is an effective treatment option for the management of benign painful shoulder syndrome. Concerning radiation protection, the dose for a radiotherapy series is recommended not to exceed 3-6 Gy. (orig.)

  2. The Acquired Immunodeficiency Syndrome. An Air Force Readiness Issue

    Science.gov (United States)

    1989-09-01

    will continue to harbor the infection. The term retroviral refers to the genettic makeup and specific enzyme of the virus that a;lows it to replicate...stages and criteria. Table 1 Walter Reed Classification System HTLV- III Chronic T Helper STAGE Antibody Lymohadenonathy Qells/mm- DHS Thrush 0.1 WR 1...0.80 Reserve 88,411 122 1.38 Guard 126,934 96 0.76 Infection rates are per thousand (Wolfe, 1988; 1989). The figures in table 3 reflect the entire

  3. Association between Depressive Symptoms and Metabolic Syndrome in Police Officers: Results from Two Cross-Sectional Studies

    International Nuclear Information System (INIS)

    Hartley, T.A.; Fekedulegn, D.; Andrew, M.E.; Burchfiel, C.M.; Hartley, T.A.; Knox, S.S.; Barbosa-Leiker, C.; Violanti, J.M.

    2012-01-01

    Policing is one of the most dangerous and stressful occupations and such stress can have deleterious effects on health. The purpose of this study was to examine the association between depressive symptoms and metabolic syndrome (Met Syn) in male and female police officers from two study populations, Buffalo, NY and Spokane, WA. Depressive symptoms were measured using the Center for Epidemiologic Studies-Depression (CES-D) scale. Met Syn was defined using the 2005 AHA/NHBLI guidelines. Analysis of covariance was used to describe differences in number of Met Syn components across depressive symptom categories. The number of Met Syn components increased significantly across categories of CES-D for Spokane men only (p-trend = 0.003). For each 5-unit increase in CES-D score, odds increased by 47.6% for having hypertriglyceridemia, by 51.8% for having hypertension, and by 56.7% for having glucose intolerance. Exploring this association is important since both are predictors of future chronic health problems and the results could be helpful in developing future gender-specific prevention and intervention efforts among police officers

  4. [Prevalence and Comorbidity of Self-Reported Diagnosis of Burnout Syndrome in the General Population - Results of the German Health Interview and Examination Survey for Adults (DEGS1)].

    Science.gov (United States)

    Maske, Ulrike E; Riedel-Heller, Steffi G; Seiffert, Ingeburg; Jacobi, Frank; Hapke, Ulfert

    2016-01-01

    Objective: To determine the prevalence and comorbid mental disorders of self-reported diagnosis of burnout syndrome in the general population of Germany. Methods: In the German Health Interview and Examination Survey (DEGS1) self-reported diagnosis of a burnout syndrome made by a physician or psychotherapist was assessed in a standardized interview (N = 7987). For N = 4483 mental disorders were determined with the Composite International Diagnostic Interview (CIDI). Weighted lifetime and 12-month prevalences were calculated. Results: Lifetime prevalence of diagnosed burnout syndrome was 4.2 % (women 5.2 %, men 3.3 %), 12-month prevalence was 1.5 % (women 1.9 %, men 1.1 %). Highest prevalences were found in 40 - 59 year olds, in people with middle and high socio-economic status and in women with low and men with high social support. Among the 12-month cases, 70.9 % had at least one DSM-IV disorder. Associations were found for the diagnosis of burnout syndrome with somatoform, affective and anxiety disorders. Conclusion: The diagnosis of burnout syndrome is less frequently given and reported than expected. People with a burnout diagnosis often have a manifest mental disorder. © Georg Thieme Verlag KG Stuttgart · New York.

  5. A literature review on cardiovascular risk in human immunodeficiency virus-infected patients: implications for clinical management

    Directory of Open Access Journals (Sweden)

    Mansueto Gomes Neto

    Full Text Available INTRODUCTION: In recent years, there has been growing concern about an increasing rate of cardiovascular diseases in human immunodeficiency virus-infected patients, which could be associated with side effects of highly active antiretroviral therapy. It is likely that the metabolic disorders related to anti-human immunodeficiency virus treatment will eventually translate into a increased cardiovascular risk in patients submitted to such regimens. OBJECTIVE: To evaluate if human immunodeficiency virus-infected patients receiving highly active antiretroviral therapy are at higher risk of cardiovascular diseases than human immunodeficiency virus infected patients not receiving highly active antiretroviral therapy, or the general population. RESEARCH DESIGN AND METHODS: We conducted a computer-based search in representative databases, and also performed manual tracking of citations in selected articles. RESULT: The available evidence suggests an excess risk of cardiovascular events in human immunodeficiency virus-infected persons compared to non-human immunodeficiency virus infected individuals. The use of highly active antiretroviral therapy is associated with increased levels of total cholesterol, triglycerides, low-density lipoprotein and morphological signs of cardiovascular diseases. Some evidence suggested that human immunodeficiency virus-infected individuals on highly active antiretroviral therapy regimens are at increased risk of dyslipidemia, ischemic heart disease, and myocardial infarction, particularly if the highly active antiretroviral therapy regimen contains a protease inhibitor. CONCLUSION: Physicians must weigh the cardiovascular risk against potential benefits when prescribing highly active antiretroviral therapy. Careful cardiac screening is warranted for patients who are being evaluated for, or who are receiving highly active antiretroviral therapy regimens, particularly for those with known underlying cardiovascular risk

  6. Avaliação nutricional em pacientes infectados pelo Vírus da Imunodeficiência Adquirida Nutritional assessment in patients infected with Human Immunodeficiency Virus

    Directory of Open Access Journals (Sweden)

    Rosana Mendes Reis Barbosa

    2003-12-01

    Full Text Available Pacientes infectados com o Vírus da Imunodeficiência Humana ou com Síndrome da Imunodeficiência Adquirida freqüentemente apresentam desnutrição protéico-energética, com manifestações heterogêneas, as quais dependem do estado de saúde prévio do paciente. O estado nutricional deve ser avaliado e monitorado cuidadosamente para que o plano terapêutico possa ser efetivo. Uma avaliação nutricional abrangente é fundamental; além disso, toda a equipe de profissionais da saúde e os próprios pacientes devem estar atentos para uma ação precoce e integrada.Patients infected with the Human Immunodeficiency Virus or with the Acquired Immunodeficiency Syndrome often present protein-energy malnutrition. The manifestations are heterogeneous and depend on the patient's previous health status. The nutritional status must be assessed and carefully controlled, so that satisfactory results can be achieved and the therapeutic plan can be effective. A complete detailed nutritional assessment is fundamental; besides, the health professional team and the patients themselves need to be aware of the symptoms and the disease progress, in order to take an early and integrated action.

  7. Mood and Anxiety Disorders in Chronic Fatigue Syndrome, Fibromyalgia, and Irritable Bowel Syndrome: Results From the LifeLines Cohort Study.

    Science.gov (United States)

    Janssens, Karin A M; Zijlema, Wilma L; Joustra, Monica L; Rosmalen, Judith G M

    2015-05-01

    Functional somatic syndromes (FSSs) have often been linked to psychopathology. The aim of the current study was to compare prevalence rates of psychiatric disorders among individuals with chronic fatigue syndrome (CFS), fibromyalgia (FM), and irritable bowel syndrome (IBS). This study was conducted in 94,516 participants (mean [standard deviation] age = 44.6 [12.5] years, 58.7% women) of the general-population cohort LifeLines. FSSs were assessed by self-reports. Mood disorders (i.e., major depressive disorder and dysthymia) and anxiety disorders (i.e., generalized anxiety disorder, social phobia, panic disorder with/without agoraphobia, and agoraphobia) were assessed by means of the Mini International Neuropsychiatric Interview. Risks on psychiatric disorders were compared for individuals with CFS, FM, and IBS by using logistic regression analyses adjusted for age and sex. Prevalence rates of CFS, FM, and IBS were 1.3%, 3.0%, and 9.7%, respectively. Individuals with CFS, FM, and IBS had significantly more mood (odds ratios [ORs] = 1.72-5.42) and anxiety disorders (ORs = 1.52-3.96) than did individuals without FSSs, but prevalence rates were low (1.6%-28.6%). Individuals with CFS more often had mood (ORs = 2.00-4.08) and anxiety disorders (ORs = 1.63-2.32) than did individuals with FM and IBS. Major depressive disorder was more common in FM than in IBS (OR = 1.58, 95% confidence interval = 1.24-2.01), whereas these groups did not differ on dysthymia or anxiety disorders. Mood and anxiety disorders are more prevalent in individuals with FSSs, and particularly CFS, than in individuals without FSSs. However, most individuals with FSSs do not have mood or anxiety disorders.

  8. A comparison of the impact of screen-positive results obtained from ultrasound and biochemical screening for Down syndrome in the first trimester : a pilot study

    NARCIS (Netherlands)

    Weinans, M.J.; Kooij, L.; Muller, M.A.; Bilardo, K.M.; van Lith, J.M.; Tymstra, T.

    2004-01-01

    OBJECTIVE: To compare the experiences of women who received a screen-positive test result for Down syndrome after nuchal translucency screening or after biochemical screening in the first trimester of pregnancy in the Netherlands. METHOD: Semi-quantitative questionnaires were sent to 40 women with a

  9. Recent advances in treatment of severe primary immunodeficiencies [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Andrew Gennery

    2015-12-01

    Full Text Available Primary immunodeficiencies are rare, inborn errors that result in impaired, disordered or uncontrolled immune responses. Whilst symptomatic and prophylactic treatment is available, hematopoietic stem cell transplantation is an option for many diseases, leading to cure of the immunodeficiency and establishing normal physical and psychological health. Newborn screening for some diseases, whilst improving outcomes, is focusing research on safer and less toxic treatment strategies, which result in durable and sustainable immune function without adverse effects. New conditioning regimens have reduced the risk of hematopoietic stem cell transplantation, and new methods of manipulating stem cell sources should guarantee a donor for almost all patients. Whilst incremental enhancements in transplantation technique have gradually improved survival outcomes over time, some of these new applications are likely to radically alter our approach to treating primary immunodeficiencies.

  10. Long-Term Results of Aortic Root Surgery in Marfan Syndrome Patients: A Single-Center Experience.

    Science.gov (United States)

    Nicolo, Francesco; Romeo, Francesco; Lio, Antonio; Bovio, Emanuele; Scafuri, Antonio; Bassano, Carlo; Polisca, Patrizio; Pellegrino, Antonio; Nardi, Paolo; Chiariello, Luigi; Ruvolo, Giovanni

    2017-07-01

    The study aim was to compare long-term results of Marfan syndrome (MFS) patients affected by aortic root disease undergoing aortic root replacement with the Bentall or David operation. Since 1994, a total of 59 patients has been followed at the authors' Marfan Center, having undergone either a Bentall operation (Bentall group, n = 30) or a David operation (David group, n = 29). No operative mortality was recorded. After 20 years (mean follow up 97 ± 82 months; range 1 to 369 months) no prosthesis-related major bleeding or thromboembolic events had been observed; the 20-year survival was 94 ± 6% in the Bentall group, and 100% in the David group (p = 0.32). Freedom from reintervention for aortic valve dysfunction was 100% in the Bentall group, and 75 ± 13% in the David group (p = 0.04). This inter-group difference became relevant after the first eight-year period of follow-up, and was mainly associated with a particular familiar genetic phenotype involving three out of four reoperated patients. Freedom from all-cause death, myocardial infarction, stroke, prosthetic valve-related complications, and reintervention on any aortic segment was 69 ± 12% in the Bentall group, and 67 ± 14% in the David group (p = 0.33). The Bentall and David operations are both associated with satisfactory long-term results in MFS patients. The low rate of valve prosthesis-related complications suggested that the Bentall operation would continue to be a standard surgical treatment. The reimplantation technique, adopted for less-dilated aortas, provides satisfactory freedom from reoperation. Careful attention should be paid to the reimplantation technique in patients affected by a serious familiar genetic phenotype.

  11. Limited impact on self-concept in individuals with Lynch syndrome; results from a national cohort study

    DEFF Research Database (Denmark)

    Petersen, Helle Vendel; Esplen, Mary Jane; Ladelund, Steen

    2011-01-01

    . We used the national Danish HNPCC register to collect data on self-concept from 413 individuals with Lynch syndrome. The recently developed Lynch syndrome self-concept scale contains 20 items within two subscales related to stigma-vulnerability and bowel symptom-related anxiety. Significantly higher...... total scores, indicating a greater impact on self-concept, were reported by females and by individuals with experience from cancer in close relatives, whereas individuals with less formal education scored significantly higher on the stigma and vulnerability subscale. Scores in the upper quartile were...... more often reported by women (odds ratio 1.8) and by individuals with less education (OR 1.8). This study provides the first extended use of the Lynch syndrome self-concept scale and suggests that the majority of the Danish mutation carriers adapt well to the situation, though knowledge about...

  12. Irregular eating of meals in adolescence and the metabolic syndrome in adulthood: results from a 27-year prospective cohort.

    Science.gov (United States)

    Wennberg, Maria; Gustafsson, Per E; Wennberg, Patrik; Hammarström, Anne

    2016-03-01

    The objective was to investigate whether irregular eating of meals in adolescence predicts the metabolic syndrome and its components in adulthood, and if any specific meal is of particular importance. Prospective cohort study with 27 years of follow-up. Information on meals (breakfast, school lunch and dinner with family), lifestyle (alcohol consumption, smoking habits, physical activity, consumption of sweets and pastries) at age 16 years was assessed from questionnaires, and presence or not of the metabolic syndrome and its components were defined at age 43 years in 889 participants (82·1% of total cohort). Logistic regression was used to calculate odds ratios and confidence intervals. The Northern Swedish Cohort; all school-leavers of the 9th grade in the town Luleå in 1981. Adolescents (age 16 years). Irregular eating of meals at age 16 years was associated with higher prevalence of the metabolic syndrome at age 43 years (OR=1·74; 95% CI 1·12, 2·71), but this was explained by concurrent unhealthy lifestyle at age 16 years. Poor breakfast at age 16 years was the only meal associated with the metabolic syndrome at age 43 years, independent of other meals, BMI (kg/m2) and lifestyle at age 16 years (OR=1·67; 95% CI 1·00, 2·80). Irregular eating of meals in adolescence predicted the metabolic syndrome in adulthood, but not independently of BMI and lifestyle in adolescence. Poor breakfast in adolescence was the only specific meal associated with future metabolic syndrome, even after adjustments. Breakfast eating should be encouraged in adolescence.

  13. Cancer spectrum in DNA mismatch repair gene mutation carriers: results from a hospital based Lynch syndrome registry.

    Science.gov (United States)

    Pande, Mala; Wei, Chongjuan; Chen, Jinyun; Amos, Christopher I; Lynch, Patrick M; Lu, Karen H; Lucio, Laura A; Boyd-Rogers, Stephanie G; Bannon, Sarah A; Mork, Maureen E; Frazier, Marsha L

    2012-09-01

    The spectrum of cancers seen in a hospital based Lynch syndrome registry of mismatch repair gene mutation carriers was examined to determine the distribution of cancers and examine excess cancer risk. Overall there were 504 cancers recorded in 368 mutation carriers from 176 families. These included 236 (46.8 %) colorectal and 268 (53.2 %) extracolonic cancers. MLH1 mutation carriers had a higher frequency of colorectal cancers whereas MSH2, MSH6 and PMS2 mutation carriers had more extracolonic cancers although these differences were not statistically significant. Men had fewer extracolonic cancers than colorectal (45.3 vs. 54.7 %), whereas women had more extracolonic than colorectal cancers (59.0 vs. 41.0 %). The mean age at diagnosis overall for extracolonic cancers was older than for colorectal, 49.1 versus 44.8 years (P ≤ 0.001). As expected, the index cancer was colorectal in 58.1 % of patients and among the extracolonic index cancers, endometrial was the most common (13.8 %). A significant number of non-Lynch syndrome index cancers were recorded including breast (n = 5) prostate (n = 3), thyroid (n = 3), cervix (n = 3), melanoma (n = 3), and 1 case each of thymoma, sinus cavity, and adenocarcinoma of the lung. However, standardized incidence ratios calculated to assess excess cancer risk showed that only those cancers known to be associated with Lynch syndrome were significant in our sample. We found that Lynch syndrome patients can often present with cancers that are not considered part of Lynch syndrome. This has clinical relevance both for diagnosis of Lynch syndrome and surveillance for cancers of different sites during follow-up of these patients.

  14. Evidence for the Spread of the Human Immunodeficiency Virus Epidemic into Low Prevalence Areas of the United States

    Science.gov (United States)

    1989-01-01

    inmune Defllenq Syndrpmes,. Vol. 2. NVo, 6, 1989 524 L. 1. GARDNER, JR. ET AL. prevalence territory. For uniformity, we assigned limits around rate...Conference on AIDS, Washington, D.C., June black males in the intravenous drug abuser popula- 1987. 4. Centers for Disease Control. Coolfont report. A...18. Centers for Disease Control. Acquired immunodeficiency ato and Sally Fuller assured the quality of laboratory test- syndrome in the San -Francisco

  15. Oxygen Exposure Resulting in Arterial Oxygen Tensions Above the Protocol Goal Was Associated With Worse Clinical Outcomes in Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Aggarwal, Neil R; Brower, Roy G; Hager, David N; Thompson, B Taylor; Netzer, Giora; Shanholtz, Carl; Lagakos, Adrian; Checkley, William

    2018-04-01

    High fractions of inspired oxygen may augment lung damage to exacerbate lung injury in patients with acute respiratory distress syndrome. Participants enrolled in Acute Respiratory Distress Syndrome Network trials had a goal partial pressure of oxygen in arterial blood range of 55-80 mm Hg, yet the effect of oxygen exposure above this arterial oxygen tension range on clinical outcomes is unknown. We sought to determine if oxygen exposure that resulted in a partial pressure of oxygen in arterial blood above goal (> 80 mm Hg) was associated with worse outcomes in patients with acute respiratory distress syndrome. Longitudinal analysis of data collected in these trials. Ten clinical trials conducted at Acute Respiratory Distress Syndrome Network hospitals between 1996 and 2013. Critically ill patients with acute respiratory distress syndrome. None. We defined above goal oxygen exposure as the difference between the fraction of inspired oxygen and 0.5 whenever the fraction of inspired oxygen was above 0.5 and when the partial pressure of oxygen in arterial blood was above 80 mm Hg. We then summed above goal oxygen exposures in the first five days to calculate a cumulative above goal oxygen exposure. We determined the effect of a cumulative 5-day above goal oxygen exposure on mortality prior to discharge home at 90 days. Among 2,994 participants (mean age, 51.3 yr; 54% male) with a study-entry partial pressure of oxygen in arterial blood/fraction of inspired oxygen that met acute respiratory distress syndrome criteria, average cumulative above goal oxygen exposure was 0.24 fraction of inspired oxygen-days (interquartile range, 0-0.38). Participants with above goal oxygen exposure were more likely to die (adjusted interquartile range odds ratio, 1.20; 95% CI, 1.11-1.31) and have lower ventilator-free days (adjusted interquartile range mean difference of -0.83; 95% CI, -1.18 to -0.48) and lower hospital-free days (adjusted interquartile range mean difference of -1.38; 95

  16. Abnormal Resting-State Functional Connectivity in Patients with Chronic Fatigue Syndrome: Results of Seed and Data-Driven Analyses.

    Science.gov (United States)

    Gay, Charles W; Robinson, Michael E; Lai, Song; O'Shea, Andrew; Craggs, Jason G; Price, Donald D; Staud, Roland

    2016-02-01

    Although altered resting-state functional connectivity (FC) is a characteristic of many chronic pain conditions, it has not yet been evaluated in patients with chronic fatigue. Our objective was to investigate the association between fatigue and altered resting-state FC in myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). Thirty-six female subjects, 19 ME/CFS and 17 healthy controls, completed a fatigue inventory before undergoing functional magnetic resonance imaging. Two methods, (1) data driven and (2) model based, were used to estimate and compare the intraregional FC between both groups during the resting state (RS). The first approach using independent component analysis was applied to investigate five RS networks: the default mode network, salience network (SN), left frontoparietal networks (LFPN) and right frontoparietal networks, and the sensory motor network (SMN). The second approach used a priori selected seed regions demonstrating abnormal regional cerebral blood flow (rCBF) in ME/CFS patients at rest. In ME/CFS patients, Method-1 identified decreased intrinsic connectivity among regions within the LFPN. Furthermore, the FC of the left anterior midcingulate with the SMN and the connectivity of the left posterior cingulate cortex with the SN were significantly decreased. For Method-2, five distinct clusters within the right parahippocampus and occipital lobes, demonstrating significant rCBF reductions in ME/CFS patients, were used as seeds. The parahippocampal seed and three occipital lobe seeds showed altered FC with other brain regions. The degree of abnormal connectivity correlated with the level of self-reported fatigue. Our results confirm altered RS FC in patients with ME/CFS, which was significantly correlated with the severity of their chronic fatigue.

  17. Longitudinal weight gain in women identified with polycystic ovary syndrome: results of an observational study in young women.

    Science.gov (United States)

    Teede, Helena J; Joham, Anju E; Paul, Eldho; Moran, Lisa J; Loxton, Deborah; Jolley, Damien; Lombard, Catherine

    2013-08-01

    Polycystic ovary syndrome (PCOS) affects 6-18% of women. The natural history of weight gain in women with PCOS has not been well described. Here we aimed to examine longitudinal weight gain in women with and without PCOS and to assess the association between obesity and PCOS prevalence. The observational study was set in the general community. Participants were women randomly selected from the national health insurance scheme (Medicare) database. Mailed survey data were collected by the Australian Longitudinal Study on Women's Health. Data from respondents to survey 4, aged 28-33 years (2006, n = 9,145) were analyzed. The main outcome measures were PCOS prevalence and body mass index (BMI). Self-reported PCOS prevalence was 5.8% (95% CI: 5.3%-6.4%). Women reporting PCOS had higher weight, mean BMI [2.5 kg/m(2) (95% CI: 1.9-3.1)], and greater 10-year weight gain [2.6 kg (95% CI: 1.2-4.0)]. BMI was the strongest correlate of PCOS status with every BMI increment increasing the risk of reporting PCOS by 9.2% (95% CI: 6%-12%). This community based observational study with longitudinal reporting of weight shows that weight, BMI, and 10-year weight gain were higher in PCOS. We report the novel finding that obesity and greater weight gain are significantly associated with PCOS status. Considering the prevalence, major health and economic burden of PCOS, the increasing weight gain in young women, and established benefits of weight loss, these results have major public health implications. Copyright © 2012 The Obesity Society.

  18. Acceptance of referral for partners by clients testing positive for human immunodeficiency virus

    Directory of Open Access Journals (Sweden)

    Netsanet F

    2013-01-01

    Full Text Available Fetene Netsanet,1 Ayalew Dessie21IMA World Health SuddHealth Multi Donor Trust Fund-Basic Package of Health Services Project, Juba, South Sudan; 2United States Agency for International Development, Private Health Sector Program, Abt Associates Inc, Addis Ababa, EthiopiaBackground: Human immunodeficiency virus (HIV-positive individuals who do not disclose their HIV status to their partners are more likely to present late for HIV and acquired immune deficiency syndrome (AIDS care than those who have disclosed their HIV status to their partners. A major area of challenge with regards to HIV counseling for clients is disclosure of their HIV status to their partners. The main methods of partner notification are patient referral, provider referral, contract referral, and outreach assistance. The emphasis on a plausible and comprehensive partner referral strategy for widespread positive case detection in resource-limited countries needs to be thought out and developed.Methods: A qualitative study was conducted among newly HIV-positive clients to identify partners for notification and acceptance of referral by their partners. Health service providers working in HIV testing and counseling clinics were also provided with semistructured questionnaires in order to assess their view towards partner notification strategies for clients testing positive for HIV.Results: Fifteen newly diagnosed HIV-positive clients were counseled to provide referral slips to their partners. All clients agreed and took the referral card. However, only eight were willing and actually provided the card to their partners. Five of the eight partners of clients who tested HIV-positive and who were provided with referral cards responded to the referral and were tested for HIV. Three were positive and two were negative. Nine of 11 counselors did not agree to requesting partner locator information from HIV-positive clients for contractual referral and/or outreach assistance. The findings

  19. A New Face of Cardiac Emergencies: Human Immunodeficiency Virus-Related Cardiac Disease.

    Science.gov (United States)

    Tsabedze, Nqoba; Vachiat, Ahmed; Zachariah, Don; Manga, Pravin

    2018-02-01

    The human immunodeficiency virus epidemic is a major health challenge of the twenty-first century as the transition from infectious complications to noncommunicable disease becomes more evident. These patients may present to the emergency department with a variety of cardiovascular diseases, such as acute coronary syndromes, heart failure, pericardial disease, infective endocarditis, venothromboembolism, and other conditions. Increased awareness is needed among health care professionals to enhance adequate identification and promote prompt management of these patients. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

    OpenAIRE

    Folgori, Laura; Scarselli, Alessia; Angelino, Giulia; Ferrari, Francesca; Antoccia, Antonio; Chessa, Luciana; Finocchi, Andrea

    2010-01-01

    Abstract Ataxia-telangiectasia (A-T) is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM) gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively wi...

  1. Feline immunodeficiency virus: disease association versus causation in domestic and nondomestic felids.

    Science.gov (United States)

    White, Joanna; Stickney, Alison; Norris, Jacqueline M

    2011-11-01

    Feline immunodeficiency virus (FIV) is an important infection in both domestic and nondomestic cats. Although many studies have provided insight into FIV pathophysiology and immunologic responses to infection in cats, questions remain regarding the association of FIV with specific disease syndromes. For many diseases, both association and causation of disease with FIV remain to be confirmed and clarified. The use of experimental infection models is unlikely to yield answers about naturally infected domestic cats and is not feasible in nondomestic felids, many of which are endangered species. Researches might consider further study of naturally occurring disease with an emphasis on confirming which diseases have a likely association with FIV.

  2. Results of a questionnaire among Dutch urologists and general practitioners concerning diagnostics and treatment of patients with prostatitis syndromes

    NARCIS (Netherlands)

    de la Rosette, J. J.; Hubregtse, M. R.; Karthaus, H. F.; Debruyne, F. M.

    1992-01-01

    By means of a questionnaire, all Dutch urologists (n = 250, 136 responded) and regional general practitioners (GPs; n = 400, 176 responded) were contacted concerning current diagnostics and treatment modalities applied in patients with prostatitis syndromes. The patients seen by urologists seem to

  3. Spontaneous Transient Lateral Thoracic Lung Herniation Resulting in Systemic Inflammatory Response Syndrome (SIRS and Subsequent Contralateral Lung Injury

    Directory of Open Access Journals (Sweden)

    Antony Kaliyadan

    2011-01-01

    Full Text Available Lung herniation is a relatively rare clinical entity that is most commonly either congenital or acquired traumatically. We describe a case of spontaneous lung herniation secondary to acute cough in an obese male smoker complicated by contralateral acute lung injury and systemic inflammatory response syndrome (SIRS. Mechanisms of lung herniation, classification, diagnosis, and management will be discussed.

  4. Leigh syndrome associated with a deficiency of the pyruvate dehydrogenase complex: results of treatment with a ketogenic diet

    NARCIS (Netherlands)

    Wijburg, F. A.; Barth, P. G.; Bindoff, L. A.; Birch-Machin, M. A.; van der Blij, J. F.; Ruitenbeek, W.; TURNBULL, D. M.; Schutgens, R. B.

    1992-01-01

    A one-year-old boy suffering from intermittent lactic acidosis, muscular hypotonia, horizontal gaze paralysis and spasticity in both legs had low activity of the pyruvate dehydrogenase complex associated with low amounts of immunoreactive E 1 alpha and E 1 beta. Leigh syndrome was diagnosed on the

  5. Results Of A Lifestyle Intervention Involving Healthy Diet, Exercise and Cognitive Behavioral Therapy In Polycystic Ovary Syndrome (PCOS)

    NARCIS (Netherlands)

    L.G. Jiskoot (Geranne); R. Timman (Reinier); A. Beerthuizen (Annemerle); Dietz de Loos, A (Alexandra); J.J. van Busschbach (Jan); J.S.E. Laven (Joop)

    2018-01-01

    markdownabstract_Context_ Long-term weight loss is important for women with polycystic ovary syndrome. Although no protocol exist for effective and long-term weight loss in this population. Three-component interventions including diet, exercise, and cognitive behavioral therapy (CBT) have shown

  6. A physical activity intervention to treat the frailty syndrome in older persons-results from the LIFE-P study

    Science.gov (United States)

    BACKGROUND: The frailty syndrome is as a well-established condition of risk for disability. Aim of the study is to explore whether a physical activity (PA) intervention can reduce prevalence and severity of frailty in a community-dwelling elders at risk of disability. METHODS: Exploratory analyses ...

  7. Deferasirox in iron-overloaded patients with transfusion-dependent myelodysplastic syndromes: Results from the large 1-year EPIC study

    DEFF Research Database (Denmark)

    Gattermann, Norbert; Finelli, Carlo; Porta, Matteo Della

    2010-01-01

    The prospective 1-year EPIC study enrolled 341 patients with myelodysplastic syndromes (MDS); although baseline iron burden was >2500ng/mL, approximately 50% were chelation-naïve. Overall median serum ferritin decreased significantly at 1 year (p=0.002). Decreases occurred irrespective of whether...

  8. PET Imaging of Tau Pathology and Relationship to Amyloid, Longitudinal MRI, and Cognitive Change in Down Syndrome: Results from the Down Syndrome Biomarker Initiative (DSBI).

    Science.gov (United States)

    Rafii, Michael S; Lukic, Ana S; Andrews, Randolph D; Brewer, James; Rissman, Robert A; Strother, Stephen C; Wernick, Miles N; Pennington, Craig; Mobley, William C; Ness, Seth; Matthews, Dawn C

    2017-01-01

    Adults with Down syndrome (DS) represent an enriched population for the development of Alzheimer's disease (AD), which could aid the study of therapeutic interventions, and in turn, could benefit from discoveries made in other AD populations. 1) Understand the relationship between tau pathology and age, amyloid deposition, neurodegeneration (MRI and FDG PET), and cognitive and functional performance; 2) detect and differentiate AD-specific changes from DS-specific brain changes in longitudinal MRI. Twelve non-demented adults, ages 30 to 60, with DS were enrolled in the Down Syndrome Biomarker Initiative (DSBI), a 3-year, observational, cohort study to demonstrate the feasibility of conducting AD intervention/prevention trials in adults with DS. We collected imaging data with 18F-AV-1451 tau PET, AV-45 amyloid PET, FDG PET, and volumetric MRI, as well as cognitive and functional measures and additional laboratory measures. All amyloid negative subjects imaged were tau-negative. Among the amyloid positive subjects, three had tau in regions associated with Braak stage VI, two at stage V, and one at stage II. Amyloid and tau burden correlated with age. The MRI analysis produced two distinct volumetric patterns. The first differentiated DS from normal (NL) and AD, did not correlate with age or amyloid, and was longitudinally stable. The second pattern reflected AD-like atrophy and differentiated NL from AD. Tau PET and MRI atrophy correlated with several cognitive and functional measures. Tau accumulation is associated with amyloid positivity and age, as well as with progressive neurodegeneration measurable using FDG and MRI. Tau correlates with cognitive decline, as do AD-specific hypometabolism and atrophy.

  9. Defibrotide for Patients with Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome: Interim Results from a Treatment IND Study.

    Science.gov (United States)

    Richardson, Paul G; Smith, Angela R; Triplett, Brandon M; Kernan, Nancy A; Grupp, Stephan A; Antin, Joseph H; Lehmann, Leslie; Shore, Tsiporah; Iacobelli, Massimo; Miloslavsky, Maja; Hume, Robin; Hannah, Alison L; Nejadnik, Bijan; Soiffer, Robert J

    2017-06-01

    Hepatic veno-occlusive disease, or sinusoidal obstruction syndrome (VOD/SOS), is a serious and potentially fatal complication of conditioning for hematopoietic stem cell transplantation (HSCT) or of chemotherapy regimens alone. Defibrotide is a complex mixture of single-stranded polydeoxyribonucleotides that is approved in the United States for treating hepatic VOD/SOS with renal or pulmonary dysfunction post-HSCT and in the European Union, Israel, and South Korea for treating severe hepatic VOD/SOS post-HSCT. Defibrotide was previously available in the United States as an investigational drug through a treatment protocol (treatment IND) study. Interim results of that large, treatment IND study of patients with VOD/SOS and with or without multiorgan dysfunction (MOD; also known as multiorgan failure) are presented here. Defibrotide was administered i.v. at 6.25 mg/kg every 6 hours (25 mg/kg/day), with a recommended treatment duration of at least 21 days. Enrolled patients (n = 681) were diagnosed with VOD/SOS based on Baltimore or modified Seattle criteria or liver biopsy analysis. Among the 573 HSCT recipients, 288 (50.3%; 95% confidence interval [CI], 46.2% to 54.4%) were alive at day +100 post-HSCT. Day +100 survival for the pediatric (≤16 years) and adult (>16 years) subgroups was 54.5% (95% CI, 49.1% to 60.0%; n = 174 of 319) and 44.9% (95% CI, 38.8% to 51.0%; n = 114 of 254), respectively. In the MOD subgroup, 159 of 351 patients (45.3%; 95% CI, 40.1% to 50.5%) of patients were alive at day +100 post-HSCT. Treatment with defibrotide was generally well tolerated, and drug-related toxicities were consistent with previous studies. Adverse events were reported in 69.6% of safety-evaluable patients (399 of 573). Other than VOD/SOS and associated MOD symptoms, the most commonly reported treatment-emergent adverse event was hypotension (13.8%). Day +100 survival results observed in this trial were consistent with results seen in previous trials of

  10. Results of Treatment of Posterior Ankle Impingement Syndrome and Flexor Hallucis Longus Tendinopathy in Dancers: A Systematic Review.

    Science.gov (United States)

    Rietveld, A B M Boni; Hagemans, F M T; Haitjema, S; Vissers, T; Nelissen, R G H H

    2018-03-15

    Dancing on pointe and relevé requires extreme plantar flexion of the talo-crural joint. Hence, these positions may lead to posterior ankle impingement syndrome (PAIS). PAIS often coincides with flexor hallucis longus tendinopathy (FHL tendinopathy, or "dancers' tendinitis"). Both injuries can appear in isolation as well. The goal of this review is to evaluate the results and the available levels of evidence of conservative and operative treatment (both open and endoscopic) of PAIS and FHL tendinopathy in dancers. It also offers an insight into the history of dance medical publications on this subject. In October 2016, a systematic search of PubMed, Embase, Cochrane, CINAHL, Web of Science, and (in French) ScienceDirect databases was undertaken. Five hundred and seventy-six publications were found, of which a total of 27 reported the results of operative treatment in 376 ankles (344 open, 32 endoscopic) in 324 dancers. The outcome was good to excellent in most cases (89%). The mean period of return to dance for all surgeries combined (PAIS and FHL tendinopathy, open and endo) was 11 weeks (range: 4 to 36 weeks), and for isolated FHL tendinopathy 16 weeks (range: 8 to 36 weeks). Only six publications reported the results of conservative treatment in 33 ankles (13 PAIS, 20 FHL tendinopathy) of 28 dancers, which does not allow for any evidence-based recommendations. Most studies failed to include dance-specific baseline characteristics, like dance style and level of participation. We concluded that only retrospective studies with levels of evidence four and five show that operative treatment for PAIS and FHL tendinopathy is successful with few complications. Since isolated PAIS, PAIS combined with FHL tendinopathy, and isolated FHL injuries appear to be different pathological entities, more research taking into account demography, dance type, and level of participation is needed to find out in which cases early operative management should be considered or avoided. The

  11. [The spa and health resort-based treatment of metabolic syndrome with the application of the therapeutic courses of different duration: the evaluation according long-term results].

    Science.gov (United States)

    Chalaya, E N; Botvineva, L A; Tsallagova, L V; Ahkubekova, N K; Efimenko, N V; Kaysinova, A S; Amiyants, V Yu; Vasin, V A

    The objective of the present study was to estimate the effectiveness of the new therapeutic modalities for the spa and health resort-based treatment of metabolic syndrome based on the results of the long-term follow-up observations. A total of 100 patients presenting with metabolic syndrome were recruited for the participation in the study. All of them underwent a shortened course of mineral water intake in the combination with the use of the herbal medicinal products. The results of the study give evidence of the more favorable changes in the clinical and instrumental characteristics of the patients with metabolic syndrome treated with the use of the proposed approach in comparison the patients treated by the conventional methods. Moreover, the beneficial effects of the proposed approach persisted during a longer period. The basal treatment given to our patients was supplemented by dietary therapy, therapeutic physical exercise, narzan baths, intake of mineral water having a temperature of 35-37 °С in conjunction with sulphate mineral water (narzan) cooled to 13-15 °С, and a phytococktail. The results of the long-term observations confirmed the enhanced effectiveness of the spa and health resort-based treatment of metabolic syndrome including the therapeutic courses with the intake of cooled narzan mineral water in the combination with phytococktails. This therapeutic modality increased the duration of the favorable changes of various parameters that characterize metabolic syndrome. Specifically, they persisted during 5-6 months after the application of the standard methods, were still apparent within 6-7 months after the two-week course of the treatment with cold narzan and phytococktails, and during 8-9 months when the duration of the treatment course was extended up to 3 weeks.