Great Ormond Street Hospital Pathology Directorate liver and muscle diseases, dermatopathology and gastrointestinal disorders suchas Investigations of primary immune Deficiencies Assays for specific T cell http://www.ich.ucl.ac.uk/path.htm
Extractions: Researchers Discover New Cause of Severe Combined Immune Deficiency Researchers have discovered a new cause of severe combined immune deficiency (SCID) in infants. Children with SCID are born with extensive immune system defects that make them highly susceptible to infections. In the September 7 issue of the journal Nature , investigators from Italy and their colleagues at the National Institutes of Health (NIH) in Bethesda, Md., describe two patients with autosomal recessive SCID who have mutations in the gene encoding a protein called Jak-3. These findings raise the possibility of designing gene therapy for this form of SCID. In the long term these findings might lead to development of a new class of drugs to treat autoimmune diseases such as lupus and rheumatoid arthritis by selectively turning off the immune response. SCID refers to a group of inherited disorders that includes the disease from which the "bubble boy" suffered (X-linked SCID). These disorders are the most serious of the primary immune deficiency disorders, in which inborn defects of the immune system lead to often chronic, frequent, and sometimes severe bacterial, viral and fungal infections. The current treatment of choice for infants with SCID is bone marrow transplantation, which is successful in more than half of all cases. Without treatment, infants with SCID rarely survive beyond their first year.
Extractions: Page 2 of 2 Partial uses of these immunological discoveries is done by studying cells involved in the immune system including lymphocyte enumeration, lymphocyte function and lymphokines and cytokines message and protein levels produced by these lymphocytes. Abnormalities of lymphocyte subsets and production of different lymphokines are involved in many immunological disorders. Disorders and diseases of the immune system affect the health and quality of life of every American. It has been estimated that approximately one of four Americans has a dysfunction of the immune system. The following figures exemplify the magnitude of this problem: 43 million Americans have asthma or allergic disease; 73 million Americans have chronic respiratory problems; 38 million Americans have arthritis of which 250,000 are children; 500,000 Americans have systemic lupus erythematosus (8:1 are women) 11 million American have diabetes; 250,000 Americans have multiple sclerosis;
Extractions: July / August 1999 Newsletter Bone Marrow Transplants in non-SCIDS Primary Immunodeficiency Disorders this type of chemotherapy and indeed survive the procedure. There may also be additional advantages to non-ablative BMT. One way of avoiding organ dysfunction for CID patients is to transplant early following diagnosis before such dysfunction occurs. This is an extremely difficult decision for both the children and their families alike, particularly if the prognosis is uncertain, as any transplant procedure has an associated risk of mortality. Indeed, to elect to undergo BMT while the child is well, with a risk of death in the immediate future, in order to prevent disease and death at an undefined future date, maybe ten years hence, is unimaginably difficult. The recent new transplant procedures were all undertaken in children with advanced organ dysfunction and the success so far is somewhat surprising. So an alternative approach would be to closely monitor children with combined immunodeficiency whose prognosis remained uncertain and in the event of deterioration, despite prophylaxis, proceed to a non-ablative transplant which could be performed in the presence of mild organ dysfunction with a likely successful outcome. Another potential advantage, but one which will only become apparent after many years, is the potential for reduced late effects of transplantation. Following the use of conventional preparation with busulphan and cyclophosphamide, both growth retardation and infertility are very likely. It is not known whether fludarabine and melphelan will affect growth, or indeed be sterilising, but certainly from very early data in the adult world there have been some pregnancies following similar transplant procedures.
Johns Hopkins Children's Center likely Wink, were born with primary immune Deficiency, a affect the functions of theimmune system. to launch its first clinic for immunodeficiency disorders. http://www.hopkinschildrens.org/pages/feature/archivedetails.cfm?spotlightid=26
Extractions: An update on their multiple causes, manifestations, and evaluation Daniel Sijipunda Dube, MD; David S. Chi, PhD; Judy Y. Hu, MD; Guha Krishnaswamy, MD WEB EXCLUSIVE / JULY 2002 / POSTGRADUATE MEDICINE CME learning objectives This work was supported by grants AI-43310 and HL-63070 from the National Institutes of Health and by the department of internal medicine, East Tennessee State University, Johnson City. Preview : Immunodeficiency disease often becomes apparent early in life. It disables the body's intricate system of defense against pathogens, resulting in myriad disorders and potential complications. In this article, the authors explain why immune deficiency occurs, how it is expressed, when to suspect an immunodeficiency disease, and how to evaluate and manage it. Seventh in a series of articles on allergy and immunology coordinated by Guha Krishnaswamy, MD, professor and chief, division of allergy and immunology, department of internal medicine, East Tennessee State University, Johnson City.
Member Sign In There are no known hereditary risk factors for primary amyloidosis. A and may occurin a wide variety of chronic infectious, inflammatory, or immune disorders. http://www.medscape.com/viewarticle/414212
Extractions: What is the difference between arthritis and other rheumatic diseases? Arthritis, itself a group of more than 100 different diseases, is one category of rheumatic diseases. Rheumatic diseases may cause pain, stiffness, and swelling in the joints and other supporting body structures, such as muscles, tendons, ligaments, and bones. However, rheumatic diseases can affect other areas of the body, including internal organs. Some rheumatic diseases involve connective tissues (called connective tissue diseases), while others may be caused by autoimmune disorders, which are diseases involving the body's immune system attacking its own healthy cells and tissues. What is the immune system? The purpose of the immune system is to keep infectious microorganisms, such as certain bacteria, viruses, and fungi, out of the body, and to destroy any infectious microorganisms that do invade the body. The immune system is made up of a complex and vital network of cells and organs that protect the body from infection. When the immune system does not function properly, it leaves the body susceptible to an array of diseases. Allergies and hypersensitivity to certain substances are considered immune system disorders. In addition, the immune system plays a role in the rejection process of transplanted organs or tissue. Other examples of immune disorders include:
¼²²¡Ïà¹Ø primary immune deficiencies; Heparininduced thrombocytopenia; HIV-related thrombocytopenia;Humanized mouse models of FcR clearance in immune platelet disorders; http://cmbi.bjmu.edu.cn/news/insight/insight-dis.htm
Extractions: Malaria Mitochondrial disease potential roles in embryogenesis and nucleocytoplasmic transfer A nuclear modifier for a mitochondrial DNA disorder Homoplasmic Mitochondrial DNA Diseases as the Paradigm to Understand the Tissue Specificity Beyond the Magic Circle ... Mitochondria, oxygen free radicals, and apoptosis
Immunodeficiency With Hyper-IgM Recent literature LocusLink collection of generelated information OMIM catalogof human genes and disorders Information primary immune deficiency National http://www.ncbi.nlm.nih.gov/disease/higm.html
Extractions: IMMUNODEFICIENCY WITH HYPER-IgM (HIM) is a rare primary immunodeficiency characterized by the production of normal to increased amounts of IgM antibody of questionable quality and an inability to produce sufficient quantities of IgG and IgA. Individuals with HIM are susceptible to recurrent bacterial infections and are at an increased risk of autoimmune disorders and cancer at an early age. In a normal immune response to a new antigen, B cells first produce IgM antibody. Later, the B cells switch to produce IgG, IgA and IgE, antibodies that protect tissues and mucosal surfaces more effectively. In the most common form of HIM there is a defect in the gene , found on chromosome X at q26. This gene normally produces a CD40 antigen ligand (CD154), a protein on T cells which binds to the CD40 receptor on B and other immune cells. Without CD154, B cells are unable to receive signals from T cells, and thus fail to switch antibody production to IgA and IgG. The absence of CD 40 signals between other immune cells makes individuals with HIM susceptible to infections by opportunistic organisms such as Pneumocystis and Cryptosporidium species.
Immunodeficiency Disorders Adults and primary Immunodeficiencies. primary Immunodeficiencies may present inadults. Some disorders like CVID present later in life ie. immune Components. http://www.allergy-asthma.org/Immudef.htm
Vibrio Vulnificus Infections Assoc W/ Raw Oyster Consumption -- FL or undercooked shellfish contaminated with V. vulnificus can lead to primary septicemiaor of the liver, stomach, or blood or have immune disorders, you are at http://www.cdc.gov/epo/mmwr/preview/mmwrhtml/00020736.htm
Extractions: June 04, 1993 / 42(21);405-407 Vibrio vulnificus is a gram-negative bacterium that can cause serious illness and death in persons with preexisting liver disease or compromised immune systems. From 1981 through 1992, 125 persons with V. vulnificus infections, of whom 44 (35%) died, were reported to the Florida Department of Health and Rehabilitative Services (HRS). This report summarizes data on these cases and presents estimates of the at-risk population in Florida. The infections generally occurred each year from March through December and peaked from May through October. Seventy-two persons (58%) had primary septicemia, 35 (28%) had wound infections, and 18 (14%) had gastroenteritis. In patients with primary septicemia, 58 infections (81%) occurred among persons with a history of raw oyster consumption during the week before onset of illness. The mean age of these persons was 60 years (range: 33-90 years; standard deviation: 12.9 years); 51 (88%) were male. Fourteen (78%) of the patients with gastroenteritis also had raw oyster- associated illness. Their mean age was 49 years (range: 19-89 years; standard deviation: 25.7 years); seven (50%) were male. Of the 40 deaths caused by septicemia, 35 (88%) were associated with raw oyster consumption. Nine of these deaths occurred in 1992. The case-fatality rate from raw oyster-associated V. vulnificus septicemia among patients with pre-existing liver disease was 67% (30 of 45) compared with 38% (5 of 13) among those who were not known to have liver disease.
FAQ's Other disorders which may be confused with CFS are primary fibromyalgia (PF),multiple sclerosis, atypical autoimmune disorders and certain atypical http://www.fnmedcenter.com/ccis/faq's.htm
Extractions: The Categories are: Definitions Related Disorders Causes Treatment ... Infectiousness You can also browse our Glossary of terms related to CFS. Question: What is chronic fatigue syndrome? Answer: Chronic fatigue syndrome is a chronic, often debilitating disorder of unknown cause characterized by fatigue, pain and cognitive disorders. The often severe cognitive complaints distinguish the syndrome from other, similar disorders and usually occur later in the evolution of the disease. Return to FAQ Main Menu. Question: Are there other related disorders that can be confused with chronic fatigue syndrome? Answer: Yes, often impossible to distinguish from CFS are the post-viral, post-mono, and post-infectious syndromes especially in the first year or two after onset. It is likely that the pathophysiologies of CFS and most post-infectious syndrome are related. Indeed, perhaps any infectious disorder and especially a viral infection, if prolonged, can evolve into CFS or a CFS-like disorder. Chronic mononucleosis and apparent chronic lyme disease are very hard to separate from CFS. Other disorders which may be confused with CFS are primary fibromyalgia (PF), multiple sclerosis, atypical auto-immune disorders and certain atypical depressive disorders. These disorders are, however, easier on the whole to separate from CFS than the post-infectious disorders. It is possible that, on the basis of the genetic or environmental individuality of patients, CFS and related or unrelated disorders can synergistically co-exist. Common examples might include CFS associated with autoimmune disorders, depressive illness and primary fibromyalgia. It is also possible to have two disorders present in the same individual which exist more or less independently of each other.
MIT - Irvine Lab the key importance of the lymph node in supporting primary immune responses makesthe a valuable goal for clinical treatment of immune system disorders. http://web.mit.edu/~biomaterials/research3.html
Extractions: Research Engineering Lymphoid Tissue Microenvironments Primary immune responses are generated in lymph nodes and other secondary lymphoid tissues. This process is initiated when dendritic cells (DCs) carrying foreign peptide antigens migrate to the lymph nodes from the periphery and meet antigen-specific naive T cells in the T zone (paracortex) of the node, triggering T cell activation, expansion, and a resultant cascade of molecular and cellular events within these tissues. It is known that T cells are more sensitive to antigen in vivo than in in vitro 2D cultures, however, it is unknown exactly how the 3D microenvironment of the T zone influences T cell activation. The microenvironment of the T zone is an open network of collagen fibers encased by a layer of fibroblast-like stromal cells, which provides a 'living highway' for T cell and DC migration, scanning, and activation. We hypothesize that both the unique 3D architecture of the T zone and the resident stromal cells, fibroblastic reticular cells (FRCs), play a significant role in generation of immune responses. To test this hypothesis, we are taking a tissue engineering approach to reconstitute aspects of lymph node structure and function in an
PHILADELPHIA FIGHT - The Jonathan Lax Center The Jonathan Lax immune disorders Treatment Center is a primary caresite for people living with HIV/AIDS. It provides upto-date http://www.fight.org/lax.asp
Extractions: More Resources Clinical Trials Critical Path Project Project TEACH Y-HEP ... Online Resources The Jonathan Lax Immune Disorders Treatment Center is a primary care site for people living with HIV/AIDS. It provides up-to-date, state-of-the-art care for people, regardless of ability to pay. Teams of physicians, nurse practitioners and physician assistants aided by social workers, peer educators and volunteers provide comprehensive care in a consumer-friendly environment. We seek to make patients full partners in the development and implementation of their treatment plans. The U.S. Department of Health and Human Services estimates that at least 22,000 people are living with HIV or AIDS in the Philadelphia metropolitan area. Yet, the availability of state-of-the-art care remains extremely limited relative to this need. At a time when treatment advances in HIV have changed the face of AIDS care, everyone living with the virus needs access to experienced, up-to-date clinical practice. Yet, efforts to identify patients in care with HIV-experienced physicians suggest that fewer than half actually have access to this care. Equally important, people living with HIV have told us they want the kind of comprehensive, consumer-friendly, integrated care provided by the Lax Center. This model of care is available nowhere in the Philadelphia metropolitan area today.
Extractions: Arthritis, itself a group of more than 100 different diseases, is one category of rheumatic diseases. Rheumatic diseases may cause pain, stiffness, and swelling in the joints and other supporting body structures, such as muscles, tendons, ligaments, and bones. However, rheumatic diseases can affect other areas of the body, including internal organs. Some rheumatic diseases involve connective tissues (called connective tissue diseases), while others may be caused by autoimmune disorders, which are diseases involving the bodys immune system attacking its own healthy cells and tissues. What is the immune system? The purpose of the immune system is to keep infectious microorganisms, such as certain bacteria, viruses, and fungi, out of the body, and to destroy any infectious microorganisms that do invade the body. The immune system is made up of a complex and vital network of cells and organs that protect the body from infection. When the immune system does not function properly, it leaves the body susceptible to an array of diseases. Allergies and hypersensitivity to certain substances are considered immune system disorders. In addition, the immune system plays a role in the rejection process of transplanted organs or tissue. Other examples of immune disorders include:
THE NATIONAL HOME INFUSION ASSOCIATION: Resources For Patients prevent normal functioning of the GI system; congestive heart failure; immune disorders;growth hormone What are the primary IV therapies administered at home? http://www.nhianet.org/patientresources/
Extractions: Home infusion therapy involves the administration of medications using intravenous, subcutaneous, and epidural routes (into the bloodstream, under the skin, and into the membranes surrounding the spinal cord). Drug therapies commonly administered via infusion include antibiotics, chemotherapy, pain management, parenteral nutrition, and immune globulin. Diagnoses commonly requiring infusion therapy include infections that are unresponsive to oral antibiotics; cancer and cancer-related pain; gastrointestinal diseases or disorders which prevent normal functioning of the GI system; congestive heart failure; immune disorders; growth hormone deficiencies; and more. Back to top Why are infusion therapies performed at home?
KidsNewzealand.com New Zealand Epsom, Auckland The Foundation offers support to children and adultswith primary immune deficiencies, immune defects and related blood disorders. http://www.kidsauckland.com/kidsnz/content.asp?Page=Health Organisation&side=sid