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  • RESEARCH 4 BUSINESS 2016, Ljubljana, 5 and 6 of May 2016

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    Chapter 15 / Immunodeficiencies Table 4 Congenital Defects of Humoral Immunity Disease X-linked agammaglobulinemia Common variable immunodeficiencies Transient immunoglobulin deficiency of newborns IgA deficiency Defect No mature B-cells present, T-cell defect Heterogeneous, usually in B-cells Symptoms Relapsing infections of sinuses and bronchi, diarrhea Pyogenic infections, autoimmune disorders, malignancies Treatment Immunoglobulins Igs 379 Delayed synthesis IgA synthesis defective Infections Gastrointestinal disturbances, anaphylactic reactions to transfusions Symptomatic Symptomatic 339 350 Table 5 Congenital Defects of Cellular Immunity Disease Di George syndrome (thymic hypoplasia, cardiac malformations, impaired development Purine nucleoside phosphorylase deficiency Wiskott–Aldrich syndrome Defect Cellular how long will viagra make you last immunity Symptoms Abscess forming infections, hypocalcemia, malignancies Treatment Transplantation of fetal thymus, bone marrow transplant 370 Cellular immunity Cellular immunity Variable, infections Infections, thrombocytopenia, BMT eczema, low immunoglobulin M Sakhalkar and Munker Chapter 16 / Immunodeficiencies Table 5 Congenital Defects of Complement Components Disease C1 deficiency Inheritance/Defect Autosomal recessive, nonfunctional, or absent protein/1p6.2, 8p14 Autosomal dominant/10q7 Autosomal recessive / factor I-6q24, H-1q32, D-?. , Properdin-Xp8.3 2p22.6 Presentation Pyogenic infections, SLE-like syndromes Laboratory findings Abnormal total hemolytic complement C1 inhibitor deficiency Factor I, H, D, properdin deficiency 341 C1 deficiency C5 deficiency Autosomal recessive/ 16p9.3 C6 deficiency Autosomal recessive/ 7p20.5 Physical trauma-induced hereditary angioedema Pyogenic (neisserial infections, urticaria, immune complex disease. Isolated congenital asplenia, hyper-IgE syndrome, isolated chronic • • • • • Chapter 13 / Immunodeficiencies Table 1 Congenital Defects of the Phagocytic System Disease Chronic granulomatous disease Defect of myeloperoxidase Defect of adhesion molecules Chediak-Higashi syndrome Hyper-IgE syndrome (multisystem disorder) Reticular dysgenesis Defect Defect of bactericidal capacity (oxidative metabolism) Enzyme defect Chemotaxis, phagocytosis Giant granules in neutrophils, functional defect Chemotaxis Defect of hematopoietic stem cell Symptoms Abscess forming infections Treatment Symptomatic, interferon γ Asymptomatic or frequent No treatment or antimycotic infections with Candida Frequent bacterial infections, Symptomatic sepsis Severe bacterial infections Symptomatic or bone marrow transplant (BMT) Cutaneous infections, Symptomatic candidiasis Lethal within first 4 mo BMT (?. See also Chapter 2) Symptomatic Hyper-IgM syndrome 288 CD40-ligand defective Defect of major histocompatibility complex expression Ataxia telangiectasia Transcription Cellular immunity (IgA, IgE deficiency) (defective ATM gene, cell cycle regulation, and DNA repair defective) BMT Symptomatic Sakhalkar and Munker BMT, bone marrow transplantation. ADA, adenosine deaminase.

    Ig, immunoglobulin. Only four congenital immunodeficiencies appear to have dominant inheritance. ) 367 317 298 Table 2 Congenital Defects of the B- and T-Cell Systems (Combined Immunodeficiency) Disease Severe combined immunodeficiency Defect B- and T-cells Symptoms Severe intestinal and pulmonary infections Pyogenic infections, lymphoproliferative syndrome Severe infections Neurological disturbances, lymphatic malignancies Treatment BMT, infusions of ADA (gene therapy.

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    The persistent signs of abstinence in detoxified subjects, including hyperthermia, mydriasis, increased blood pressure, and increased respiratory rate, have been observed to continue for months after opioid withdrawal and have been termed ‘protracted abstinence’. Protracted abstinence ABUSE AND ADDICTION POTENTIAL 221 TABLE 6.5 Abstinence Signs in Sequential Appearance After Last Dose of Narcotic Hours after last dose Grade of Abstinence Grade 0 Grade 1 Withdrawal signs Craving for drug Anxiety Yawning Perspiration Runny nose Lacrimation Increase in above signs Mydriasis Gooseflesh Piloerection Tremors Muscle twitches Hot and cold flashes Aching bones and muscles Anorexia Increased intensity of above Insomnia Increased blood pressure Increased temperature Increased respiratory rate and depth Increased pulse rate Restlessness Nausea Increased intensity of above Febrile facies Curled up position on hard surface Vomiting Diarrhea Weight loss Spontaneous ejaculation or orgasm Hemoconcentration leucocytosis Eosinopenia Increased blood sugar Methadone 10 34–38 Morphine 4 15 Heroin 7 8 Grade 3 18–42 17 9 Grade 3 na 20–36 19–25 Grade 3 na 36–38 24–16 na, not applicable. There may be diarrhea and dehydration, at 23–26 h.

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    Ozonoff & Jensen, 1995.