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combined severe immunodeficiency

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https://www.readbyqxmd.com/read/28738755/qsar-dft-and-molecular-modeling-studies-of-peptides-from-hiv-1-to-describe-their-recognition-properties-by-mhc-i
#1
S Andrade-Ochoa, J García-Machorro, Martiniano Bello, L M Rodríguez-Valdez, C A Flores-Sandoval, J Correa-Basurto
Human immunodeficiency virus type-1 (HIV-1) has infected more than 40 million people around the world. HIV-1 treatment still has several side effects, and the development of a vaccine, which is another potential option for decreasing human infections, has faced challenges. This work presents a computational study that includes a quantitative structure activity relationship (QSAR) using density functional theory for reported peptides to identify the principal quantum mechanics descriptors related to peptide activity...
July 25, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/28735354/transplantation-sites-for-human-and-murine-islets
#2
Rebecca A Stokes, Kim Cheng, Amit Lalwani, Michael M Swarbrick, Helen E Thomas, Thomas Loudovaris, Tom W Kay, Wayne J Hawthorne, Philip J O'Connell, Jenny E Gunton
AIMS/HYPOTHESIS: Beta cell replacement is a potential cure for type 1 diabetes. In humans, islet transplants are currently infused into the liver via the portal vein, although this site has disadvantages. Here, we investigated alternative transplantation sites for human and murine islets in recipient mice, comparing the portal vein with quadriceps muscle and kidney, liver and spleen capsules. METHODS: Murine islets were isolated from C57BL6/J mice and transplanted into syngeneic recipients...
July 22, 2017: Diabetologia
https://www.readbyqxmd.com/read/28733129/endocannabinoids-exert-cb1-receptor-mediated-neuroprotective-effects-in-models-of-neuronal-damage-induced-by-hiv-1-tat-protein
#3
Changqing Xu, Douglas J Hermes, Blessing Nwanguma, Ian R Jacobs, Kenneth Mackie, Somnath Mukhopadhyay, Aron H Lichtman, Bogna Ignatowska-Jankowska, Sylvia Fitting
In the era of combined antiretroviral therapy (cART), human immunodeficiency virus type 1 (HIV-1) is considered a chronic disease that specifically targets the brain and causes HIV-1-associated neurocognitive disorders (HAND). Endocannabinoids (eCBs) elicit neuroprotective and anti-inflammatory actions in several central nervous system (CNS) disease models, but their effects in HAND remain unknown. HIV-1 does not infect neurons, but produces viral toxins, such as transactivator of transcription (Tat), that disrupt neuronal calcium equilibrium and give rise to synaptodendritic injuries and cell death, the former being highly correlated with HAND...
July 18, 2017: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/28729231/cernunnos-deficiency-associated-with-bcg-adenitis-and-autoimmunity-first-case-from-the-national-iranian-registry-and-review-of-the-literature
#4
Reza Yazdani, Hassan Abolhassani, Javad Tafaroji, Gholamreza Azizi, Raif S Geha, Asghar Aghamohammadi
Non-homologous end-joining (NHEJ) is a pathway that repairs double-strand breaks (DSB) in DNA and plays a vital role in V(D)J recombination of immunoglobulin genes. Cernunnos is a DNA repair factor that is involved in nonhomologous end-joining (NHEJ) process. Impairment in Cernunnos leads to a genetic disease characterized by neural disorders, immunodeficiency and increased radiosensitivity. We herein describe a severe combined immunodeficiency (SCID) patient with T- B+ phenotype who had a mutation in Cernunnos gene and manifested recurrent infections, microcephaly and growth retardation with hypogammaglobulinemia...
July 17, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28727579/enhanced-anticancer-efficacy-of-histone-deacetyl-inhibitor-suberoylanilide-hydroxamic-acid-in-combination-with-a-phosphodiesterase-inhibitor-pentoxifylline-in-human-cancer-cell-lines-and-in-vivo-tumor-xenografts
#5
Saranya Nidhyanandan, Boreddy S Thippeswamy, Kottapalli B Chandrasekhar, Neetinkumar D Reddy, Nagaraj M Kulkarni, Kandasamy Karthikeyan, Farhin R Khan, Jayaprakash Raghul, Govindharajan Vijaykanth, Shridhar Narayanan
Vorinostat [suberoylanilide hydroxamic acid (SAHA)], a histone deacetylase inhibitor, shows limited clinical activity against solid tumors when used alone. The methyl xanthine drug, pentoxifylline (PENT), has been described to have antitumor properties. The aim of this study was to look for the enhanced anticancer activities of both agents when used in combination at doses lower than their respective efficacy dose when used alone. We investigated the antitumor potential of this novel combination in vitro and in vivo...
July 19, 2017: Anti-cancer Drugs
https://www.readbyqxmd.com/read/28723333/enhanced-prophylaxis-plus-antiretroviral-therapy-for-advanced-hiv-infection-in-africa
#6
James Hakim, Victor Musiime, Alex J Szubert, Jane Mallewa, Abraham Siika, Clara Agutu, Simon Walker, Sarah L Pett, Mutsa Bwakura-Dangarembizi, Abbas Lugemwa, Symon Kaunda, Mercy Karoney, Godfrey Musoro, Sheila Kabahenda, Kusum Nathoo, Kathryn Maitland, Anna Griffiths, Margaret J Thomason, Cissy Kityo, Peter Mugyenyi, Andrew J Prendergast, A Sarah Walker, Diana M Gibb
BACKGROUND: In sub-Saharan Africa, among patients with advanced human immunodeficiency virus (HIV) infection, the rate of death from infection (including tuberculosis and cryptococcus) shortly after the initiation of antiretroviral therapy (ART) is approximately 10%. METHODS: In this factorial open-label trial conducted in Uganda, Zimbabwe, Malawi, and Kenya, we enrolled HIV-infected adults and children 5 years of age or older who had not received previous ART and were starting ART with a CD4+ count of fewer than 100 cells per cubic millimeter...
July 20, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28710530/proteomic-characterisation-reveals-active-wnt-signalling-by-human-multipotent-stromal-cells-as-a-key-regulator-of-beta-cell-survival-and-proliferation
#7
Miljan Kuljanin, Gillian I Bell, Stephen E Sherman, Gilles A Lajoie, David A Hess
AIMS/HYPOTHESIS: Novel strategies to stimulate the expansion of beta cell mass in situ are warranted for diabetes therapy. The aim of this study was to elucidate the secretome of human bone marrow (BM)-derived multipotent stromal cells (MSCs) with documented islet regenerative paracrine function. We hypothesised that regenerative MSCs will secrete a unique combination of protein factors that augment islet regeneration. METHODS: Human BM-derived MSCs were examined for glucose-lowering capacity after transplantation into streptozotocin-treated NOD/severe combined immunodeficiency (SCID) mice and segregated into samples with regenerative (MSC(R)) vs nonregenerative (MSC(NR)) capacity...
July 14, 2017: Diabetologia
https://www.readbyqxmd.com/read/28708971/short-term-alcohol-abstinence-improves-antibacterial-defenses-of-chronic-alcohol-consuming-mice-against-gut-bacteria-associated-sepsis-caused-by-enterococcus-faecalis-oral-infection
#8
Makiko Kobayashi, Akira Asai, Ichiaki Ito, Sumihiro Suzuki, Kazuhide Higuchi, Fujio Suzuki
The effects of short-term alcohol abstinence on host antibacterial resistance against Enterococcus faecalis oral infection was investigated in chronic alcohol-consuming mice [mice with 0.1 g/day of 20% ethanol consumption for 12 or 16 weeks (CAC-mice)]. These mice were highly susceptible to the infection; however, after 7 days of alcohol abstinence (aaCAC-mice), their antibacterial resistances were completely restored to the normal mouse level. Normal mice inoculated with CAC-mouse hepatic macrophages were shown to be susceptible to the infection, whereas the same macrophage preparation from aaCAC-mice did not impair the antibacterial resistance of normal mice...
July 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28702826/head-and-neck-diffuse-large-b-cell-lymphomas-hn-dlbcl-in-human-immunodeficiency-virus-hiv-positive-patients-long-term-results-in-the-highly-active-antiretroviral-therapy-haart-era
#9
Francesca De Felice, Andrea Di Mino, Lavinia Grapulin, Maria Lusia Mistrulli, Daniela Musio, Vincenzo Tombolini
To report long-term outcomes and toxicity rates after chemotherapy (CHT) followed by radiotherapy (RT) in the highly active antiretroviral therapy (HAART) era in human immunodeficiency virus (HIV) positive patients with head and neck diffuse large B cell lymphomas (HN-DLBCL). Clinical data concerning consecutive HIV patients treated for DLBCL located in head and neck region with CHT and RT were retrospectively reviewed. Systemic treatment consisted of combination CHT agents given with concomitant HAART and regimen was left to oncologists' discretion...
July 12, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28695547/human-immunodeficiency-virus-promotes-mitochondrial-toxicity
#10
REVIEW
Summer J Rozzi, Valeria Avdoshina, Jerel A Fields, Margarita Trejo, Hoai T Ton, Gerard P Ahern, Italo Mocchetti
Combined antiretroviral therapies (cART) have had remarkable success in reducing morbidity and mortality among patients infected with human immunodeficiency virus (HIV). However, mild forms of HIV-associated neurocognitive disorders (HAND), characterized by loss of synapses, remain. cART may maintain an undetectable HIV RNA load but does not eliminate the expression of viral proteins such as trans-activator of transcription (Tat) and the envelope glycoprotein gp120 in the brain. These two viral proteins are known to promote synaptic simplifications by several mechanisms, including alteration of mitochondrial function and dynamics...
July 10, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28694805/primary-immunodeficiency-diseases-in-highly-consanguineous-populations-from-middle-east-and-north-africa-epidemiology-diagnosis-and-care
#11
REVIEW
Hamoud Al-Mousa, Bandar Al-Saud
Middle East and North Africa region (MENA) populations are of different ethnic origins. Consanguineous marriages are common practice with an overall incidence ranging between 20 and 50%. Primary immunodeficiency diseases (PIDs) are a group of heterogeneous genetic disorders caused by defects in the immune system that predisposes patients to recurrent infections, autoimmune diseases, and malignancies. PIDs are more common in areas with high rates of consanguineous marriage since most have an autosomal recessive mode of inheritance...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28692305/fight-fire-with-fire-gene-therapy-strategies-to-cure-hiv
#12
Jon Huyghe, Sips Magdalena, Linos Vandekerckhove
Human Immunodeficiency Virus (HIV) to date remains one of the most notorious viruses mankind has ever faced. Despite enormous investments in HIV research for more than 30 years an effective cure for HIV has been elusive. Areas covered: Combination antiretroviral therapy (cART) suppresses active viral replication, but is not able to eliminate the virus completely due to stable integration of HIV inside the host genome of infected cells and the establishment of a latent reservoir, that is insensitive to cART...
July 14, 2017: Expert Review of Anti-infective Therapy
https://www.readbyqxmd.com/read/28687959/stem-cell-therapy-for-fanconi-anemia
#13
Qing-Shuo Zhang
Stem cell therapy is the administration of stem cells to a patient to treat or prevent a disease. Since stem cells possess the long-term self-renewal capacity and provide daughter cells that differentiate into the specialized cells of each tissue, stem cell therapy will theoretically improve the disease condition for the lifetime of the patient. As the most widely used stem cell therapy, bone marrow transplantation is the treatment of choice for many kinds of blood disorders, including anemias, leukemias, lymphomas, and rare immunodeficiency diseases...
July 8, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28684198/flow-cytometry-based-diagnosis-of-primary-immunodeficiency-diseases
#14
REVIEW
Hirokazu Kanegane, Akihiro Hoshino, Tsubasa Okano, Takahiro Yasumi, Taizo Wada, Hidetoshi Takada, Satoshi Okada, Motoi Yamashita, Tzu-Wen Yeh, Ryuta Nishikomori, Masatoshi Takagi, Kohsuke Imai, Hans D Ochs, Tomohiro Morio
Primary immunodeficiencies (PIDs) are a heterogeneous group of inherited diseases of the immune system. The definite diagnosis of PID is ascertained by genetic analysis; however, this takes time and is costly. Flow cytometry provides a rapid and highly sensitive tool for diagnosis of PIDs. Flow cytometry can evaluate specific cell populations and subpopulations, cell surface, intracellular and intranuclear proteins, biologic effects associated with specific immune defects, and certain functional immune characteristics, each being useful for the diagnosis and evaluation of PIDs...
July 3, 2017: Allergology International: Official Journal of the Japanese Society of Allergology
https://www.readbyqxmd.com/read/28679969/incidence-of-spontaneous-lymphomas-in-non-experimental-nod-shi-scid-il-2r%C3%AE-null-nog-mice
#15
Masahiko Yasuda, Tomoyuki Ogura, Takayuki Goto, Mika Yagoto, Yoko Kamai, Chie Shimomura, Nobuhito Hayashimoto, Yukito Kiyokawa, Hideki Shinohara, Riichi Takahashi, Kenji Kawai
Severely immunodeficient NOD/Shi-scid, IL-2Rγ(null) (NOG) mice provide an in vivo model for human cell/tissue transplantation studies. NOG mice were established by combining interleukin-2 receptor-γ chain knockout mice and NOD/Shi-scid mice. They exhibit a high incidence of thymic lymphomas and immunoglobulin (Ig) leakiness. In this study, we assessed the incidence of malignant lymphomas and the occurrence of leakiness in 2,184 non-experimental NOG retired breeder mice aged 16-40 weeks. We established that the total incidence of lymphomas was only 0...
July 6, 2017: Experimental Animals
https://www.readbyqxmd.com/read/28678090/acute-myeloid-leukemia-in-a-patient-with-x-linked-severe-combined-immunodeficiency
#16
Tomonari Shigemura, Mitsuo Motobayashi, Kazuyuki Matsuda, Takahiro Shimodaira, Takashi Kurata, Norimoto Kobayashi, Kazunaga Agematsu, Yozo Nakazawa
Severe combined immunodeficiency (SCID) is a defect in the differentiation and function of T cells. An increased malignancy risk, mainly lymphatic malignancy, has been described in patients with SCID. We report a patient with X-linked SCID who developed acute myeloid leukemia, derived from the recipient with somatic NRAS mutation 4 months after cord blood transplantation (CBT). Loss of heterozygosity phenomenon of the recipient at 6q14 locus was observed at 2 months post-CBT and progressed to 6q deletion (6q-) chromosome abnormality...
July 3, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28674683/infusion-of-sibling-marrow-in-a-patient-with-purine-nucleoside-phosphorylase-deficiency-leads-to-split-mixed-donor-chimerism-and-normal-immunity
#17
Laura Yeates, Mary A Slatter, Andrew R Gennery
Purine nucleoside phosphorylase (PNP) deficiency, a rare autosomal recessive metabolic disease causes combined immunodeficiency and developmental delay, hypotonia, and spasticity. Patients present with recurrent infections associated with T-lymphocytopenia, characteristically presenting later than patients with classical severe combined immunodeficiency (SCID). PNP, with adenosine deaminase (ADA), is part of the purine salvage pathway. The only curative therapy is hematopoietic stem cell transplantation. Myeloablative conditioning is recommended to prevent rejection caused by residual immune function...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28670638/a-comparison-of-rapid-diagnostic-testing-by-plasmodium-lactate-dehydrogenase-and-quantitative-buffy-coat-technique-in-malaria-diagnosis-in-children
#18
Ifeanyi Kanayo Ifeorah, Biobele J Brown, Olugbemiro O Sodeinde
BACKGROUND: The World Health Organization (WHO) considers early and rapid diagnosis as one of the strategies to control malaria. This study compared the performance of Quantitative Buffy Coat (QBC) test and the Plasmodium lactate dehydrogenase (pLDH) rapid diagnostic test (RDT) with microscopy as the gold standard. MATERIALS AND METHODS: The study involved children ages 0-5 years who presented with a history of fever at the University College Hospital, Ibadan, Nigeria...
2017: African Journal of Infectious Diseases
https://www.readbyqxmd.com/read/28668826/inhibition-of-erg-activity-in-patient-derived-prostate-cancer-xenografts-by-yk-4-279
#19
Brian Winters, Lisha Brown, Ilsa Coleman, Holly Nguyen, Tsion Zewdu Minas, Lori Kollath, Valeri Vasioukhin, Peter Nelson, Eva Corey, Aykut Üren, Colm Morrissey
BACKGROUND/AIM: The aim of the current study was to determine the effects of the ERG small-molecule inhibitor YK-4-279 on ERG(+) prostate cancer patient-derived xenografts (PDX). MATERIALS AND METHODS: ERG activity was blocked using YK-4-279 in three subcutaneously-implanted ERG(+) (LuCaP 23.1, 86.2 and 35) and one ERG(-) (LuCaP 96) PDX. Treated animals tumor volume (TV), body weight (BW) and serum prostate-specific antigen (PSA) were compared to vehicle-treated control animals...
July 2017: Anticancer Research
https://www.readbyqxmd.com/read/28658648/prevention-of-human-lymphoproliferative-tumor-formation-in-ovarian-cancer-patient-derived-xenografts
#20
Kristina A Butler, Xiaonan Hou, Marc A Becker, Valentina Zanfagnin, Sergio Enderica-Gonzalez, Daniel Visscher, Kimberly R Kalli, Piyawan Tienchaianada, Paul Haluska, S John Weroha
Interest in preclinical drug development for ovarian cancer has stimulated development of patient-derived xenograft (PDX) or tumorgraft models. However, the unintended formation of human lymphoma in severe combined immunodeficiency (SCID) mice from Epstein-Barr virus (EBV)-infected human lymphocytes can be problematic. In this study, we have characterized ovarian cancer PDXs which developed human lymphomas and explore methods to suppress lymphoproliferative growth. Fresh human ovarian tumors from 568 patients were transplanted intraperitoneally in SCID mice...
June 25, 2017: Neoplasia: An International Journal for Oncology Research
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