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Primary Immune Disorders: more detail

EATING DISORDERS IN PRIMARY CARE A guide to identification and treatment Screening for eating disorders should be a routine part of medical care for children, ... and young adults. (Postgraduate Medicine) by MD Douglas G. Kondo, MD Mae S. Sokol, 2010-05-27

Combat Syndrome X, Y and Z (The Metabolic Syndrome) by M.D. Stephen Holt, 2002

What You Need to Know About Polycythemia Vera - It's Your Life, Live It! by Michael Braham, 2010-10-26

lists with details

NORD - National Organization For Rare Disorders, Inc.
All of these disorders are characterized by a weakened immune system that Organizationsrelated to Agammaglobulinemias, primary American Academy of Allergy
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Agammaglobuli
HON - News : Children With Immune Problems At Greater Bioterror Risk
of bioterrorism, according to experts who gathered in Washington, DC, on March 3for a conference titled primary immune Deficiency disorders New Research and
http://www.hon.ch/News/HSN/512040.html

Extractions: HealthScoutNews Reporter TUESDAY, March 4 (HealthScoutNews) Smallpox, anthrax, plague, botulism. The words spark considerable anxiety among Americans amid all the warnings about possible bioterrorism attacks. For those with primary immunodeficiency (PI), a genetic immune system disorder that afflicts more children than leukemia and lymphoma combined, the potential danger looms much larger. "Regardless of the agent we're talking about, these kids are more vulnerable," says Dr. Allan Lock, director of the developmental immunology program at the National Institute of Child Health and Human Development. "We're talking about an at-risk population." PI afflicts at least 500,000 Americans, many of them children, but often goes undiagnosed or is diagnosed only after patients become extremely ill or chronically debilitated, sometimes with life-threatening conditions, experts say. Proper diagnosis is all the more critical in the face of the threat of bioterrorism, according to experts who gathered in Washington, D.C., on March 3 for a conference titled "Primary Immune Deficiency Disorders: New Research and the Threat of Bioterrorism."
Bigchalk: HomeworkCentral: Diseases & Disorders (Immune/Lymphatic System)
of Types; primary Secondary immune System disorders; Introduction Overview; Introduction Overview;Introduction Types; Overview
http://www.bigchalk.com/cgi-bin/WebObjects/WOPortal.woa/Homework/Middle_School/S
The Adaptive Immune System: Primary Immunodeficiency
primary Immunodeficiency (def). A primary immunodeficiency is usually a geneticdefect in the immune responses which 1. Blymphocyte (def) disorders.
http://www.cat.cc.md.us/courses/bio141/lecguide/unit3/immunodeficiency/primary/p

Extractions: III. THE ADAPTIVE IMMUNE SYSTEM V. IMMUNODEFICIENCY B. Primary Immunodeficiency LEARNING OBJECTIVES FOR THIS SECTION Adaptive (acquired) immunity, refers to antigen-specific defense mechanisms that take several days to become protective and are designed to remove a specific antigen (def) . This is the immunity one develops throughout life. There are two major branches of the adaptive immune responses: humoral immunity and cell-mediated immunity. humoral immunity (def) : humoral immunity involves the production of antibody molecules in response to an antigen (def) and is mediated by B-lymphocytes. cell-mediated immunity (def) : Cell-mediated immunity involves the production of cytotoxic T-lymphocytes, activated macrophages, activated NK cells, and cytokines in response to an antigen (def) and is mediated by T-lymphocytes. Immunodeficiency results in an inability to combat certain diseases and may be of two types: primary or secondary. Primary immunodeficiency (def) is usually a genetic defect in the immune responses that one is born with. In the case of
Page 35 Home
primary agammaglobulinemia is an immune disorder related to antibody deficiency ismanifested in a variety of immune deficiency disorders in which
http://www.aarda.org/page35.html

Extractions: Primary Agammaglobulinemia Primary agammaglobulinemia is an immune disorder related to antibody deficiency (hypogammaglobulinemia) and is manifested in a variety of immune deficiency disorders in which the immune system is compromised. This group of immune deficiencies may be the consequence of an inherited condition, an impaired immune system from known or unknown causes, a relation to autoimmune diseases, or a malignancy. Immunoglobulin deficiencies may be referred to by many different names, as there are several variables within the separate but related immune disorders; and there are also many subgroups. Antibody deficiency, immunoglobulin deficiency, and gamma globulin deficiency are all synonyms for hypogammaglobulinemia. For more information on agammaglobulinemia or other autoimmune disorders, please click on the request information icon below. Information provided at this web site is of a general nature and is not intended to take the place of a physician's adivice It is vital that persons diagnosed with, or suspected of having, an autoimmune disease consult with their physician or with the appropriate division at a major teaching hospital, to assure proper evaluation, treatment and interpretation of information contained on this site.
MUMS List Of Disorders - C
Cholangitis, primary Sclerosing (1); Cholestasis (2); Cholesteoma (1 Brain Tumor (3);Chromosome disorders; Chronic Fatigue Granulomatous XLinked immune Disease (2
http://www.netnet.net/mums/mum_c.htm

Extractions: indicates there is a support group which covers that diagnosis. C-PAP (14) CATCH 22 Syndrome(DiGeorge+Velo-Cardio-Facial+Conotruncal) (5) * CHARGE Syndrome (49) * CHARGE-VATER Syndrome (2) Calcium Deficiency (26) Campomelic Dysplasia Syndrome (11) * Camptodactyly (5) Canavan's Leukodystrophy (3) * www.canavan.org/ Cancer (135) * www.cancer.org and www.candlelighters.org Cancer, Acute Lymphatic Leukemia (ALL) (6)* Cancer, Anoplastic Oligodendroglioma Tumor (1) Cancer, Chordoma (Spinal Tumor) (1) * Cancer, Ewings Sarcoma (6) * Cancer, Ovaries (1) * Cancer, Primary Neuroectodermal Tumor of the Spine (PNET) (2) * Cancer, Rhabdomyosarcoma (4)* Candida Yeast Infection (7) Carbamyl Phosphate Synthetase Deficiency (CPS) (2) * Carbohydrate-Deficient Glycoprotein Syndrome (CDGS) (8) * Carbon Monoxide Poisoning (2) Cardiac Pacemaker (23) Cardio-Facio-Cutaneous Syndrome (19) ** Cardiomyopathy (36) * www.childrenscardiomyopathy.org
News Release: Intravenous Immunoglobulin: Prevention And Treatment Of Disease
intramuscularly to bolster the immune system to prevent or treat the variety of infectionsthat can occur in patients with primary immune deficiency disorders.
http://consensus.nih.gov/news/releases/080_release.htm

Extractions: NIH CONSENSUS PANEL MAKES RECOMMENDATIONS ON USE OF INTRAVENOUS IMMUNOGLOBULIN Patients with certain immune system deficiencies that make them susceptible to a variety of infections may benefit from intravenous injections of disease-fighting proteins produced by the immune system, according to a panel of experts convened by the National Institutes of Health. In addition, the panel said that people whose immune systems have been compromised by other disorders or by bone marrow transplants also may benefit from intravenous administration of these proteins, known as immunoglobulins. Since 1952 immunoglobulin preparations made from human blood have been given intramuscularly to bolster the immune system to prevent or treat the variety of infections that can occur in patients with primary immune deficiency disorders. However, in the last decade, 7 preparations of intravenous immunoglobulin have become available in the U.S. Although licensed only for certain disorders, these preparations have been used for a variety of other conditions without complete tests on their effectiveness. After reviewing the latest research data on the efficacy of intravenous immunoglobulin (IVIG) therapy, the 13-member panel, chaired by Dr. Peter E. Lipsky of the University of Texas Southwestern Medical Center in Dallas, made recommendations regarding its use. Among their findings, the panel concluded that:
Immunological Disorders And Tests
(1) Chapter title Immunological disorders and Tests. inadequacy of specific immunityas immune responses normally as being either inborn (primary) or acquired
http://www.mansfield.ohio-state.edu/~sabedon/black18.htm

Extractions: Important words and concepts from Chapter 18, Black, 1999 by Stephen T. Abedon abedon.1@osu.edu ) for Micro 509 at the Ohio State University Course-external links are in brackets Click [ index ] to access site index Click here to access texts website Vocabulary words are found below Chapter title : Immunological Disorders and Tests (a) immunological disorders and tests Google Search index Hypersensitivity (a) Hypersensitivities are inappropriate immune responses to foreign material that is either within or in contact with the body (b) Essentially, the body mounts a sometimes dramatic immune response against an otherwise harmless, or at least less-harmful substance, thereby doing more harm to the body in the course of the immune response than might have the original allergen (c) Hypersensitivities may be divided into four types: (i) Type I Immediate hypersensitivity (ii) Type II Cytotoxic hypersensitivity (iii) Type III Immune complex hypersensitivity (iv) Type IV Cell-mediated Hypersensitivity Delayed Hypersensitivity (d) hypersensitivity reactions Google Search Anaphylaxis anaphylactic shock (a) Anaphylaxis is a general term used to describe the detrimental effect(s) associated with hypersensitivities (b) Anaphylaxis may be localized (annoying but not life threatening) or generalized (systemic and life threatening) (c) Anaphylactic shock is a generalized anaphylaxis characterized by a significant, life-threatening drop in blood pressure
THE MERCK MANUAL, Sec. 11, Ch. 136, Eosinophilic Disorders
Chapter 136. Eosinophilic disorders. Topics. General. The development of eosinophiliahas features of an immune response a primary challenge with an
http://www.merck.com/pubs/mmanual/section11/chapter136/136b.htm

Extractions: This Publication Is Searchable The Merck Manual of Diagnosis and Therapy Section 11. Hematology And Oncology Chapter 136. Eosinophilic Disorders Topics [General] Eosinophilia Idiopathic Hypereosinophilic Syndrome Eosinophilia The development of eosinophilia has features of an immune response: a primary challenge with an agent such as Trichinella spiralis invokes a primary response with relatively low levels of eosinophils, whereas repeated challenges result in an augmented or secondary eosinophilic response. Factors that decrease the eosinophil count include -blockers, corticosteroids, stress, and (sometimes) bacterial and viral infections. Several compounds released by mast cells and basophils as a consequence of antigenic coupling of IgE molecules on their surface are chemotactic for eosinophils: eg, eosinophil chemotactic factor of anaphylaxis, leukotriene B4, complement complex (C567), and histamine (over a narrow range of concentration). Etiology Eosinophilia may be primary or secondary to an underlying disorder (see Table 136-1 ). In the USA
THE MERCK MANUAL, Sec. 4, Ch. 48, Extrahepatic Biliary Disorders
Extrahepatic Biliary disorders. The cause of primary sclerosing cholangitis (PSC)is unknown. toxins, infectious agents, and abnormalities in immune regulation.
http://www.merck.com/pubs/mmanual/section4/chapter48/48e.htm

Extractions: This Publication Is Searchable The Merck Manual of Diagnosis and Therapy Section 4. Hepatic And Biliary Disorders Chapter 48. Extrahepatic Biliary Disorders Topics [General] Cholelithiasis Cholecystitis Choledocholithiasis ... Diverticulosis Of The Gallbladder Primary Sclerosing Cholangitis A chronic cholestatic syndrome characterized by fibrosing inflammation in the intrahepatic and extrahepatic bile ducts leading to narrowing and, eventually, obliteration of the bile ducts and development of cirrhosis. Etiology The cause of primary sclerosing cholangitis (PSC) is unknown. Theoretic pathogens include toxins, infectious agents, and abnormalities in immune regulation. Although excess copper has been implicated, patients have not responded to chelation with penicillamine, suggesting that elevated hepatic copper levels are a secondary phenomenon (as in primary biliary cirrhosis). Although both cytomegalovirus and reovirus type 3 may affect the intrahepatic bile ducts, there is little evidence that these viruses are present in all patients with PSC. Altered immune mechanisms appear to be the most likely cause; HLA-B8 and HLA-DR3, often found in autoimmune diseases, have also been associated with PSC. Destruction of the bile ducts in PSC involves T lymphocytes, and alterations in many arms of the immune system have been noted. Symptoms and Signs PSC occurs most often in young men and is commonly associated with inflammatory bowel disease, especially ulcerative colitis. The onset is usually insidious, with gradual, progressive fatigue, pruritus, and jaundice. Episodes of ascending cholangitis with right upper quadrant pain and fever are uncommon. Some patients present with hepatosplenomegaly or features of cirrhosis. The terminal phase is characterized by decompensated cirrhosis, portal hypertension, ascites, and liver failure.
Health Library - Granulomatous Disease, Chronic
National Organization for Rare disorders. Chronic Granulomatous Disease is a veryrare inherited primary immune deficiency disorder that affects certain white
http://health_info.nmh.org/Library/HealthGuide/IllnessConditions/topic.asp?hwid=
Immunology And Allergy Services In Scotland: Page 2
perform blood and tissue tests for the diagnosis and monitoring of hypersensitivity(allergy) disorders, primary immunodeficiency and autoimmune disease.
http://www.scotland.gov.uk/library3/health/iass-02.asp

Extractions: Home About News Publications ... Contents Immunology and Allergy Services in Scotland As a consequence, the specialty is already beginning to sub-divide into immunology and allergy. The term immunology will be used throughout this report even though it is acknowledged that clinicians who work specifically with patients who suffer from allergic reactions, (i.e. allergists), have recently been recognised by the Specialist Training Authority and the Department of Health (through an Amendment to Schedule 2 of the Medical Order) as constituting a related but distinct specialty in their own right. In large centres, around 30% of requests for tests originate from GPs. These tests can often exclude diagnoses which might warrant referral to hospital consultants in other specialties, e.g. dermatology, ENT, gastroenterology, respiratory medicine and rheumatology. glossary of terms has been added for ease of reference. Contents Home About News ... General enquiries
Indications For Referral
VI. Recurrent Respiratory Infections and primary immune Deficiency disorders.primary immunodeficiency disorders are genetic disorders
http://www.dsallergy.com/new_page_3.htm

Extractions: Phone: (972) 257-1255, Fax: (972) 401-0614, Email: Daniel Suez Anaphylactic reactions Hay fever, Sinus Allergy (Allergic Rhinitis/Sinusitis) Bronchial Asthma Hives (Urticaria/Angioedema) ... Stinging Insect Allergy Anaphylactic reactions are usually characterized by their sudden onset and dramatic presentation and can be fatal. Symptoms may associate a sudden drop in blood pressure with potential loss of consciousness or mental confusion, rapid heart rate and usually general flushing. Other systemic symptoms may also be present such as generalized hives and swelling (urticaria and angioedema), acute shortness of breath with obvious and wheezing. Systemic reactions are more common than true anaphylactic reactions with loss of consciousness. Anaphylactic reactions are mostly due to immediate reactions which can be triggered by foods, insect stings (bee, wasp, etc), drugs (penicillin and others), exercise or may occur without a known trigger. Patients must be evaluated for identification of the potential trigger and treated accordingly. They must be correctly educated and supplied with epinephrine and antihistamines to be used during potential future reactions. II. Hay fever, Sinus Allergy (Allergic Rhinitis/Sinusitis)
MedBioWorld: Online Bioscience Texts & Courses
immune System disorders Immunologic Diseases Karolinska Institute primary immuneDeficiency Diseases National Institute of Allergy Infectious Diseases.
http://www.sciencekomm.at/home/lists/diseases.html
Articles And News
primary immune Deficiency, NIAID Fact Sheet. of lung disease in primary immunodeficiencies.EurJ tomography in children with antibody deficiency disorders.
http://www.pedpid.com/articlesnews.htm

Extractions: Articles and News Effectiveness of Oseltamivir (Tamiflu) in Preventing Influenza in Household Contacts New Treatment for Eczema Hoping to catch a cure ABCNEWS.com Promising French Gene Therapy Results ... Computed tomography scan assessment of lung disease in primary immunodeficiencies Eur J Pediatr 1999 Jan;158(1):29-31 Pulmonary abnormalities in patients with primary hypogammaglobulinemia The Journal of Allergy and Clinical Immunology November 1999 Volume 104 Number 5 Clinical utility of high-resolution pulmonary computed tomography in children with antibody deficiency disorders. Pediatr Radiol 1997 Oct;27(10):794-8 Extended follow-up of antibody levels and antigen responsiveness after 2 Haemophilus influenzae type b conjugate vaccines Kinetics of T-cell development of umbilical cord blood transplantation in severe T-cell immunodeficiency disorders JACI May 1999, part 1 Volume 103 Number 5
PedPID Table Of Contents
Family Issues Gastrointestinal disorders Hyper IgE Hyper IgM IgA Deficiency IgG SubClassDeficiency Intravenous immune Globulin Omenn primary immune Deficiency
http://www.pedpid.com/TOC.htm
NIEHS Center - COEP
Dr. Brooks clinical interests involved primary immune deficiency disease representa common pathway for these disorders. on the developing immune systems appear
http://www.niehs.utmb.edu/people/brooks/research.html

Extractions: RESEARCH INTERESTS: Introduction T-cell Development in Immune Deficiency Disease Toxicological Effects of Retinoic Acid on the Developing Immune System (Brooks, Goldblum, et al manuscript in preparation Thymic development was severely affected, inhibiting TCR expression within the thymus and severly limiting the numbers of total thymocytes. As seen within the human diseases described above, abnormal recombination events were detected within the thymuses of affected animals. These studies demonstrated that defects extrinsic to the recombinational machinery could nonetheless disrupt the normal recombinational mechanisms. Thus, toxicological effects of various environmental agents on the developing immune systems appear to have a profound effect on the ability of the TCR loci to efficiently recombine, leading to abnormal repertoires and immune deficiency. Characterization of Allergens from Mountain Cedar Pollen Mountain cedar pollen is a common cause of allergic pollinosis in central Texas and many other parts of the country and the world. Diseases caused by pollen, including allergic rhinitis and bronchial asthma, are a major health care problem. The cloning of proteins that react with IgE and cells involved in the allergic response is a basis for understanding and treating these diseases. Dr. Midoro-Horiuti joined the laboratory in 1995 and initiated a study of mountain cedar pollinosis. She has isolated, characterized, and cloned mountain cedar allergens Jun a 1 and Jun a 3
Auckland Allergy Clinic - Primary Immunodeficiency
Q Is it possible to outgrow a primary immune Deficiency? A primary Immunodeficienciesare a diverse group of disorders that predisposes people to develop
http://www.allergyclinic.co.nz/guides/35.html

Extractions: A Cause of Recurrent Infections Primary immunodeficiency diseases are caused by intrinsic defects in the cells of the immune system and are often caused by inherited genetic defects. This is in contrast to secondary immunodeficiency diseases such as acquired immunodeficiency syndrome caused by infection with human immunodeficiency virus. The hallmark of primary immunodeficiency diseases is increased susceptibility to infection, but they can cause other problems as well. The diseases are grouped according to what part of the immune system is affected. The World Health Organization recognizes approximately 70 primary immune deficiency diseases including: Common Variable Immune deficiency (Hypogammaglobulinemia) Selective IgA Deficiency Severe Combined Immune Deficiency (boy-in-bubble disease) Antibody Deficiencies Antibodies are proteins made by white blood cells called B Lymphocytes and plasma cells. The function of antibodies is to recognize and mark bacteria, viruses, and other foreign invaders so that other parts of the immune system will respond to these invaders by killing them. One example of antibody deficiency is:
THE NATIONAL LUPRON VICTIMS NETWORK - Autoimmune Disorders & An Effect On The Im
LUPRON AUTOimmune disorders AN EFFECT ON THE immune vivo which results in suppressedimmune responses. Rao A. EFFECTS OF LUPRON ON primary LYMPHOID ORGANS
http://www.lupronvictims.com/immune2.html

Extractions: In 1994, Fritzler reported that "a number of drugs recently have been implicated in a syndrome that resembles systemic lupus erythematous."(8) "New drugs most frequently implicated in a syndrome resembling lupus systemic erythematous (SLE), are recombinant protein molecules now undergoing use in the treatment of malignancies." (8) Lupron is currently approved by the FDA for the palliative treatment of advanced prostate cancer. In the article, "Table I" is entitled "Drugs recently associated with lupus syndromes." (8) Leuprolide Acetate (Lupron) is one of the 15 drugs listed. (8) II. Lupron: An Effect On The Immune System GnRH agonists are known to exert effects on the immune system.(1) In 1994, Grau et al reported a case of a women who developed leukopenia * while on Lupron depot for a fibroid.(2) Grau claimed that this was the first reported case in a woman taking Lupron for fibroids. He stated that previously leukopenia had only been noted occassionally in patients who were given Lupron for ovarian cancer and that the leukopenia was due to the other chemotherapies that they were given. This was initially reported by Miller who was doing a Phase II trial of Leuprolide Acetate in patients with advanced epithelial ovarian carcinoma funded in part by the manufacturer; an indication that has not gained approval by the US Food and Drug Administration to date.(3) However, we found numerous sources including the NDA (New Drug Application) for Lupron which stated the following:
Bayer BP To Appear In Healthy Solutions Cable TV Series Episode Entitled Advance
Physicians and patients have come to rely on IGIV therapy for treatingautoimmune disorders and primary immune deficiencies. There
http://www.bayerbiologicals.com/News_Center/Press/2002/20021220.asp

Extractions: Immunoglobulins (antibodies), derived from human blood plasma, were first used 50 years ago. Today, intravenous immunoglobulin preparations, also called IGIV's, have become a life-saving treatment for patients with antibody deficiencies. Physicians and patients have come to rely on IGIV therapy for treating auto-immune disorders and primary immune deficiencies. There has been a dramatic increase in the utilization of IGIV therapy over the past 10 years. The manufacturing process is a delicate one and with an increase in usage, an immediate and dependable source of IGIV is essential. In response to the critical concern of creating a reliable supply, Bayer Biological Products, a leader in the immunoglobulin arena, has designed a novel IGIV manufacturing process utilizing caprylate, which both inactivates viruses and removes impurities. For more information, log on to www.bayerbiologicals.com

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