61. CBER Letter - Important Drug Warning: Acute Renal Failure Associated With Immune com/products/ins_veno.htm www.alphather.com/products/ins_veno2.htm, VenoglobulinSVenoglobulin-I, Primary immune deficiencies (PID) Immune Thrombocytopenic http://www.fda.gov/cber/ltr/igivrenal.htm

Blood Therapeutics Vaccines Allergenics ... About Us IMPORTANT DRUG WARNING September 24, 1999 Dear Doctor: This letter is intended to alert physicians to safety precautions that should be taken to reduce the potential risk of ACUTE RENAL FAILURE (ARF) reported to be associated with the administration of Immune Globulin Intravenous (Human) (IGIV) products. Since IGIVs were first introduced in 1981, the U.S. Food and Drug Administration (FDA) has received over 114 worldwide (approximately 83 U.S.) adverse event reports a of renal dysfunction and / or acute renal failure associated with the administration of these products Although acute renal failure was successfully managed in the majority of cases, deaths were reported in 17 patients worldwide. Many of the patients who died had serious underlying conditions. Preliminary evidence suggests that IGIV products containing sucrose may present a greater risk for this complication. Hyperosmolality of certain reconstituted products, as well as differences in stabilizer sugar choice and content between IGIVs, may be among the factors that have contributed to different reported rates of renal dysfunction for the various IGIV products. A disproportionate share of the cases (approximately 88% of U.S. reports) have been associated with the sucrose-containing products. The sucrose containing products are: (A) the product manufactured by the Central Laboratory Blood Transfusion Service, Swiss Red Cross (SRC), (Sandoglobulin, distributed by Novartis, and Panglobulin, distributed by the American Red Cross), and (B) the IGIV products manufactured by Centeon L.L.C. (Gammar-P I.V./Gammar-I.V.

62. Primary Immune Deficiency Webring The PID Webring is designed to help those looking for information onany of the Primary immune deficiencies find relevant web sites. http://pidring.bravepages.com/

The PID Webring is designed to help those looking for information on any of the Primary Immune Deficiencies find relevant web sites. Primary Immune Deficiencies include, but are not limited to: Agammaglobulinemia, Selective IgA Deficiency, Common Variable Immunodeficiency, Severe Combined Immunodeficiency, Chronic Granulomatous Disease, Ataxia-Telangiectasia, IgG Subclass Deficiency, Wiskott-Aldrich Syndrome, DiGeorge Anomaly, Hyper-IgM Immune Deficiency, Cd5 - Cd19 PID, and Omenn Syndrome. A Webring is a way for web pages with similar interests to link themselves to one another. By following the links of a Webring you can surf to all of the sites included in the ring. The scripting to control all of this is run from Yahoo. This is a free service. If you have a website which is dedicated to information about any of the Primary Immune Deficiencies you can submit your site for consideration in the PID Webring. You must be able to add HTML coding to your site. You will need to place the PID Webring logo, and the links which connect your site to the rest of the ring, in a prominent location on your website. Would you like to join the Primary Immune Deficiency Webring? This is how it works.

63. Nemours.org - Immunology - Frequently Asked Questions Not all immune deficiencies are acquired, however, and a child maybe born with other conditions causing immune deficiency. These http://www.nemours.org/no/faq/faq1798.html

Find a Doctor About Us Employment Alfred I. duPont Hospital ... Contact Us Frequently Asked Questions Immunology If my child has an immune deficiency, does that mean she has AIDS? AIDS is one very serious type of immune deficiency. It is an acquired immune deficiency, meaning that it develops after birth. Not all immune deficiencies are acquired, however, and a child may be born with other conditions causing immune deficiency. These other conditions are generally far less serious than AIDS. How serious is an immune deficiency? Immune deficiencies vary widely in their seriousness. Some will resolve in time, with or without intervention, leaving the child with a normal immune system. Others are more serious and may require ongoing treatment with intravenous immunoglobulin. A small group of extremely serious primary immune deficiencies require bone marrow transplantation from a parent or sibling. Why is my child sick all the time? Does she have an immune deficiency?

64. Primary Immune Deficiency Disease Publications At The National Institute Of Alle Primary Immune Deficiency; Understanding the Immune System. News Releases. NewProject Aims to Identify immune deficiencies Early Among Minority Individuals; http://www.niaid.nih.gov/publications/pid.htm

Publications Home Primary Immune Deficiency Diseases Fact Sheets and Brochures Primary Immune Deficiency Understanding the Immune System News Releases New Project Aims to Identify Immune Deficiencies Early Among Minority Individuals October 30, 1997 A New Registry for Primary Immune Deficiency Diseases October 28, 1997 Study Advances Development of Gene Therapy for Chronic Granulomatous Disease Other Links When the Body's Defenses are Missing: Primary Immunodefiency NIAID Home About NIAID Activities ... Opportunities Comments regarding this website are welcome. Please send them to the Webmaster Last Updated June 17, 2001 (rjt)

65. Child Health Library - Allergy, Asthma & Immunology - Immune Deficiencies Main page for information regarding childhood immune deficiencies from the ChildHealth Library of Children's Hospital of Pittsburgh. immune deficiencies. http://www.chp.edu/greystone/allergy/oidhub.php

About Parents Providers Library ... Research Select Another Subject Adolescent Medicine Arthritis Burns Cardiovascular Disorders Craniofacial Anomalies Dermatology Eye Care Growth and Development High-Risk Newborn High-Risk Pregnancy Infectious Diseases Medical Genetics Mental Health Neurological Disorders Normal Newborn Oncology Orthopaedics Respiratory Disorders Surgery Terminally Ill, Care of Transplantation Urology About Pittsburgh Directions and Parking Departments and Services Neighborhood Locations ... Online Resources Immune Deficiencies There are many different immune system deficiencies that require clinical care by a physician or other healthcare professional. Listed in the directory below are some, for which we have provided a brief overview. If you cannot find the condition in which you are interested, please visit the Allergy, Asthma, and Immunology Online Resources page in this Web site for an Internet/World Wide Web address that may contain additional information on that topic. Severe Combined Immunodeficiency (SCID) Common Variable Immunodeficiency (CVID) DiGeorge Syndrome X-linked Agammaglobulinemia ... CHP Store

66. AquaMD, Inc. - Contaminants What it is known to cause Skin changes; thymus gland problems; immune deficiencies;reproductive or nervous system difficulties; increased risk of cancer. http://www.aquamd.com/resources/dispcontam.cfm?contaminant=44

67. Immune Etiology Altered Protein SynthesisOther immune deficiencies associated with altered synthesisof proteins include complement defects in which there is absence of a http://www.jcaai.org/Param/Immune/Etiology.HTM

Etiology of Immunodeficiencies The etiologies of the primary and secondary immunodeficiency disorders are heterogeneous. Many immunodeficiencies have no elucidated etiology. With regard to the primary immunodeficiency disorders, exciting progress has been made in the recent past involving isolation of genes and gene products known to be deficient in certain immunodeficiency disorders. ) to superoxide anions (O ) during phagocytosis. The X-linked form is known to be associated with an abnormal gene that codes for the 91-kD beta-chain of NADPH-oxidase (8). Two of the autosomal recessive forms of CGD are due to abnormalities in genes coding for two of the cytosolic components (47 kD and 67 kD) of this membrane bound electron transport chain which are essential for O generation (9,10). Altered Protein SynthesisOther immune deficiencies associated with altered synthesis of proteins include complement defects in which there is absence of a specific complement component such as with C2, C3, or C4 deficiency, or selective IgA deficiency in which immunity at mucosal surfaces is defective due to lack of secretory IgA (with or without absence of the IgG2 subclass). In complement protein C2 deficiency type I, no translation of complement protein is observed. A 28-base pair deletion has been found in patients with the C2 null allele which results in a premature termination codon and absent C2 protein (11). In some cases of hereditary angioedema, the C1 esterase inhibitor is lacking functional activity (12).

68. Immune Humoral total serum IgG level. III. Management of Humoral immune deficiencies.It should be recognized that Intravenous immunoglobulin (IVIG http://www.jcaai.org/Param/Immune/Humoral.HTM

Humoral Immunodeficiency Practice Parameters CLASSIFICATION: ICD-9 CODE NO. Bruton X-linked agammaglobulinemia Dysgammaglobulinemia Gamma globulin deficiency in blood Humoral deficiencies IgA IgM IgG congenital hypogammaglobulinemia hyper-IgM (also increased IgM) X-linked autosomal recessive non-sex linked congenital hypogammaglobulinemia specified NEC Immunodeficiency with: defect predominant B cell defect hyperimmunoglobulinemia common variable immunodeficiency X-linked, with increased IgM Diagnosis and Evaluation I. Clinical Evaluation of Immunodeficiency It should be recognized that: Congenital immunodeficiency usually (but not always) has a characteristic appearance in infants, children, and adults according to the sex of the child, age of the patient, immunization history with live vaccines, and exposure to infections. Recurrent, serious infection, especially with encapsulated organisms is the hallmark of humoral immunodeficiency. Family history, particularly of affected males, is extremely important in the diagnosis of immunodeficiency. Physical examination of the patient is essential with attention to failure-to-thrive, weight loss, enlargement or absence of lymph nodes, organomegaly, dermatitis, oral candidiasis, short stature, clubbing, and listlessness.

69. Common Variable Immune Deficiences: Infectious Causes COMMON VARIABLE immune deficiencies Infectious causes; glutathione by TeresaBinstock Researcher in Developmental and Behavioral Neuroanatomy http://www.jorsm.com/~binstock/cvid.htm

COMMON VARIABLE IMMUNE DEFICIENCIES Infectious causes; glutathione by Teresa Binstock Researcher in Developmental and Behavioral Neuroanatomy My writings do not constitute medical advice. Instead, they represent a seeking to understand autism-spectrum disorders and their causes and associated traits. Aukrust P et al. Decreased levels of total and reduced glutathione in CD4+ lymphocytes in common variable immunodeficiency are associated with activation of the tumor necrosis factor system: possible immunopathogenic role of oxidative stress. Blood 86.4.1383-91 1995. Aukrust P et al. Persistent activation of the tumor necrosis factor system in a subgroup of patients with common variable immunodeficiency possible immunological and clinical consequences. Blood 87.2.674-81 1996. Jaffe JS et al. Functional abnormalities of CD8+ t cells define a unique subset of patients with common variable immunodeficiency. Blood 82.1.192-2001 1993. Suthanthiran M et al. Glutathione regulates activation-dependent DNA synthesis in highly purified normal human T lymphocytes stimulated via the CD2 and CD3 antigens. Proc Natl Acad Sci USA 87.3343-7 1990. Robinson MK et al. Glutathione depletion in rats impairs T-cell and macrophage immune function. Archives of Surgery 128.29-35 1993.

70. KinderStart - Health/Medical/Dental : Major Diseases/Conditions : Primary Immune this Site; KIDS Foundation of New Zealan We offer support to children and adultswith primary immune deficiencies, immune defects and related blood disorders. http://www.kinderstart.com/healthmedicaldental/majordiseasesconditions/primaryim

KinderStart Alta Vista Ask Jeeves Excite Google HotBot GO LookSmart Lycos Webcrawler Adoption Animal Friends Bringing Home Baby Child Development ... Major Diseases/Conditions : Primary Immune Deficiency (PID) Web Pages The following links are in English 10 Warning Signs of PID The 10 Warning Signs of Primary Immune Deficiency Add/View Comments Rate this Site Immune Deficiency Foundation A National non-profit health organization dedicated to improving the diagnosis and treatment of primary immune deficiency diseases through research and education. Add/View Comments Rate this Site International Patient Organisation for Primary Immunodeficiencies IPOPI is an international organization whose members are national patient organizations for the primary immunodeficiencies (PID's). It was formed to benefit and serve its members and patients with primary immunodeficiencies. Its pupose is to unite the experience, expertise, resources and influence of its members in order to achieve worldwide improvement in the care and treatment of patients with PID's. Add/View Comments Rate this Site KIDS Foundation of New Zealan We offer support to children and adults with primary immune deficiencies, immune defects and related blood disorders.

71. Other Programs Field Trips. We coordinate and sponsor trips for children suffering withprimary immune deficiencies, their siblings and their families. http://www.raynidaymiracles.org/currentservices.htm

R a y n i D a y M i r a c l e s I n c Gift Distribution Education Other Programs Christmas Presents We deliver age appropriate presents to children in the stem cell transplant units of Cardinal Glennon Children’s Hospital and St. Louis Children’s Hospital. Field Trips We coordinate and sponsor trips for children suffering with primary immune deficiencies, their siblings and their families. We attempt to provide a safe environment with limited exposure along with supplies to protect immune deficient children. Family Sponsorship We provide gifts to a family with a child suffering from an immune deficiency or a child pre or post lung transplant each year. The gifts can be monetary, but are usually large care packages sent to families and children who are in the hospital. Donations to Larger Organizations We make honorary and memorial contributions to the Immune Deficiency Foundation, The American Lung Association and other large non-profit organizations who benefit children with immune deficiencies. Pre and Post Lung Transplants Because many pre and post lung transplant children also experience decreased immune function, we include them in our trips, gift-giving and sponsorship programs.

72. Lancaster General - Immune Deficiencies immune deficiencies. Health Information / Pediatric Health Library /Allergy immune deficiencies. There are many different immune system http://www.lha.org/content/greystone_6289.asp

73. Member Sign In But while these are general guidelines, it is important to look at specificimmune deficiencies to recognize where the recommendations may vary. http://www.medscape.com/viewarticle/413918

If you are having trouble logging in: In order to use Medscape, your browser must be set to accept "cookies." To find out how to adjust your browser settings, please click here Log In Username Password Forgot your password? Not a Member? Register Now for free access to: MEDLINE (Optimized for Physicians) 200+ Free CME Courses 25 Medical Specialty Sites 100+ Medical Journals Conference Coverage Daily Medical News About Medscape Help WebMD Health

74. Viacord | Questions And Answers They have been used as part of the treatment therapy in nearly 40different cancers, immune deficiencies and genetic disorders. http://www.viacord.com/QA/QA.asp?section=7&s=CordBlood

75. ThinkQuest Library Of Entries Inherited immune deficiencies usually reflect the failure of a gene importantto the generation or function of immune system components. http://library.thinkquest.org/C0115080/?c=aids

76. HealingKids.Net Foundation is a membershipbased international outreach community dedicated to providingenrichment to the lives of children with immune deficiencies, and to http://www.healingkids.net/

www.healingkids.net Last update: NEW PICTURES IN THE GALLERY! To best view this web site, we recommend Microsoft Internet Explorer. Download the latest version HERE IT'S FREE! NEW! DONATE ONLINE: CLICK HERE! HEALINGKIDS.NET is a membership-based international outreach community dedicated to providing enrichment to the lives of children with immune deficiencies, and to offering family members a safe place to learn and interact. HEALINGKIDS.NET HEALINGKIDS.NET exists for these purposes. In order to join, it is necessary to provide some information. We will get back to you with your acceptance as quickly as possible. We will give you a password in your acceptance email - protect it! It is part of what keeps this site safe and private for you. Remember, membership is free. READY TO JOIN? GO! NEW! DONATE ONLINE: CLICK HERE! Kryss.com

77. Immune Globulin There are a wide variety of primary immune deficiencies. The WorldHealth Organization recognizes approximately 70 primary immune http://alphatherserv.com/en/immu1.htm

Established in 1986, Alpha Therapeutic Services, Inc. (ATS) is uniquely focused on specialized services for patients with immunologic disorders. ATS provides IVIG products and supplies for home infusion or use in outpatient facilities. Intravenous Immune Globulin is a plasma-derived product used in the treatment of: Primary Immunodeficiency Diseases Chronic Lymphocytic Leukemia Renal Transplantation Idiopathic Thrombocytopenic Purpura Bone Marrow Transplantation Kawasaki's Disease Pediatric HIV Infection Alpha Therapeutic Services, Inc. offers the following services: Accurate expert Delivery of Medications and Supplies Expert Case Management and Coordination Ancillary Supplies Availability of on-call clinical staff and Pharmacy services 24 hours a day, 7 days a week Coordination of Nursing Services , as necessary Clinical Monitoring Reimbursement Management Patient Education Materials Medical Waste Management MORE INFORMATION... The following information has been provided by: The Immune Deficiency Foundation www.primaryimmune.org

78. Dr. Amos Cohen Genetic immune deficiencies in Signal Transduction Proteins. Role of the p9Oncogene in Leukemia. Research in our laboratory includes three areas http://www.immune.med.utoronto.ca/FACULTY/INDIV_FACULTY_PAGE/cohen.html

AMOS COHEN, Ph.D. Infection, Immunity, Allergy The Hospital for Sick Children 555 University Avenue Toronto, Ontario M5G 1X8 Tel: (416) 813-8625 E-mail ac@sickkids.on.ca Genetic Immune Deficiencies in Signal Transduction Proteins. Role of the p9 Oncogene in Leukemia Research in our laboratory includes three areas: i) Primary immune deficiency diseases ii) Transcriptional regulation of the gamma T cell antigen receptor (TCR) iii) Growth regulation of childhood acute lymphoblastic leukemia 1. Primary immune-deficiencies in human The purine degradation enzymes adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP) and 5' nucleotidase are closely linked to lymphoid differentiation as evidenced from the association of immunodeficiency diseases with genetic defects of these enzymes. We are interested in the mechanism by which deficiencies in these enzymes affects T cell differentiation and signal transduction, as well as in gene therapy prospects (1,2). Other inherited immune-deficiencies which interest us include selective CD8 deficiency caused by mutations in the ZAP70 tyrosine kinase (3,4). 2. Transcriptional regulation of the gamma TCR

79. Immune System Immunodeficiency Diseases People with advanced cancer may experience immune deficiencies as a resultof the disease process or from extensive anticancer therapy. http://www.immunecentral.com/immune-system/iss21.cfm

Immune System Series Immunodeficiency Diseases Lack of one or more components of the immune system results in immunodeficiency disorders. These can be inherited, acquired through infection or other illness, or produced as an inadvertent side effect of certain drug treatments. People with advanced cancer may experience immune deficiencies as a result of the disease process or from extensive anticancer therapy. Transient immune deficiencies can develop in the wake of common viral infections, including influenza, infectious mononucleosis, and measles. Immune responsiveness can also be depressed by blood transfusions, surgery malnutrition, and stress. Some children are born with defects in their immune systems. Those with flaws in the B cell components are unable to produce antibodies (immunoglobulins). These conditions, known as agammaglobulinemias or hypogammaglobulinemias, leave the children vulnerable to infectious organisms; such disorders can be combated with injections of immunoglobulins. Other children, whose thymus is either missing or small and abnormal, lack T cells. The resultant disorders have been treated with thymic transplants.

80. Immunopathology 2 immune deficiencies can be primary (genetic) or secondary (caused by an exogenousagent), and secondary immune deficiencies can result from a variety of agents http://www.ucihs.uci.edu/com/pathology/faculty/robinson_corenotes/immunopatholog

Interactive Lecture Notes for Core Immunopathology Lectures IMMUNOPATHOLOGY II Immune Deficiency Syndromes Lecturer: Ed Robinson, Jr., M.D., Ph.D. Lecture II: Wednesday, September 18, 2002 LEARNING OBJECTIVES Understand the differences between primary and secondary imune deficiencies and clinical examples of each. Be able, given a short clinical history, to differentiate between the different clinical syndromes (i.e., Bruton's disease versus severe combined immune deficiency). Understand how the underlying immunologic deficits in each primary and secondary immune deficiency syndrome predict what types of opportunistic infections are seen in affected patients. Know the common causes of acquired immune deficiency syndromes. Be able to describe the lifecycle of HIV and how its lifecycle can be exploited to develop new antiviral agents. Know the newest HIV findings including co-receptor usage, HAART, and the role of viral load in disease progression. Know the key features of amyloidosis including its causes both in the United States and globally as well as the histologic criteria for diagnosis of amyloid. INTRODUCTION The text and lectures will concentrate on acquired immune deficiency syndromes. The student should recognize that immune deficiency syndromes exist which affect many cells of the immnue system. Immune deficiencies can be primary (genetic) or secondary (caused by an exogenous agent), and secondary immune deficiencies can result from a variety of agents. Indeed, with the exception of AIDS, the single most common cause of acquired immune deficiency is iatrogenic. Most importantly the underlying immune deficiency, regardless of its cause, predicts the clinical and laboratory features of the disease. For example, the student should understand that AIDS is primary a malfunction/depletion of CD4+ lymphocytes and as such demonstrates the unique role of CD4+ lymphocytes in immune function.

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