Immunodeficiency

‫بسم ال الرحمن الرحيم‬ Immunodeficiency .The main activity of immune system is protection against infection The leukocytes ( WBC's ) are main cells in immune system : Immunocytes are . ( lymphocytes ( B+T cells .( Macrophages ( responsible for phagocytosis and presenting the antigen to the lymphocytes : Types of Immunity (Humoral immunity ( antibody-mainly B cells ( cell mediated ( mainly T-cells More than 70 types of leukocytes are recognized defined by their cluster of differentiation ( CD ) . antigens Immunocytes are regulated by proteins called Cytokines The activity of leukocytes in the tissues is largely modulated by Interleukins. And has major role in . chemotaxis. And produced from many types of cells like the mast cells and neutrophils Immunodeficiency: it's a state result from a defect in the immune response usually acquired ( secondary .( immunodeficiency ) and rarely genetics ( primary Any components of the system could be affected. But the most important effect of immunodeficiency is .( abnormal susceptibility to infections ( especially opportunistic infections : Types of Leukocytes into . Granulocytes: Neutrophils , Basophils , Eosinophils .Agranulocytes : lymphocytes and monocytes : Immunodeficiency classified into : Primary . Selective IgA deficiency .Common variable immunodeficiency .Mucocilliary syndromes : Secondary .HIV .Idiopathic CD4 T lymphocytopenia .Immunosuppressive therapy .Leucopenia, neutropenia, agranulocytosis .Asplenic patients .Chronic diseases associated with immune defects Complications of immunodeficiency . Infections affecting many part of the body and many systems : it varies in character ( severity ) : depending on .Nature of immune defect so not all immune deficient pt are the same .Type of microorganism

.( Treatment ( like broad spectrum antibiotics increases the chance of fungal infection .( Neoplasms: kaposi's sarcoma ( caused by herpes virus 8 ), lymphomas, SSC ( cervix and anus .Other : thrombocytopenia, lupus erythromatosis, drug allergy, encephalopathy : General management .Taking adequate history and physical examination .Full blood count including differential leukocyte count .Total serum protein levels .Evaluation of B cell ,T cell , and phagocytes function .Evaluation of complement system HIV infection and AIDS AIDS first recognized in 1981 in young homosexual males in USA . HIV is retrovirus that can infect many cells especially lymphocyte and cause AIDS HIV is mainly transmitted by intimate sexual contact blood and blood products, transfusion, tissue .transplantation , contaminated needles and syringes or IV drug abuse .Saliva and breast milk may contain the virus but infection through these routes is uncommon : Prevention of AIDS safe protected sex Cessation of needle sharing Screening blood and tissue before transfer to recipient .The use of antiretroviral drugs for children born to HIV-infected mothers Clinical features : range from asymptomatic infection due to immunodeficiency and severe clinical .illness Infection with HIV may be accompanied by an acute HIV syndrome 3-6 weeks later , with feature .similar o glandular fever, sometimes with neurological involvement or with rash or mouth ulcers .May remain asymptomatic for 10 years but the patients are seropositive and can transmit the disease .Persistant generalized lymphoadenopathy it's a sign of immunodeficiency :Main manifestation of HIV/AIDS .Opportunistic infection : mainly fungal and viral .Neoplasm : mainly Kaposi's sarcoma and lymphoma Neurological disorder : mainly dementia . Autoimmune disorders : mainly idiopathic thrombocytopenic purpura General management Diagnosis through history,clinical examination and lab investigation (CD4/CD8) ratio and the normal .value of this ratio is 2:1 but in immunodeficiency it may become 1:2 Confidential patient counseling 1st step is to test HIV to detect the antibodies to HIV ( detectable from 6-8 weeks ) antibody to HIV means the patient that the patient has been infected with the virus, it implies a carrier state but doesn't .indicate immunity to HIV .The absence of the antibody to HIV does not always mean that infection is absent .In case of doubt, HIV antigens or nucleic acid can be detected in the blood earlier than antibodies .No effective treatment

Dental aspect .It's unethical to withhold treatment from any patient . Dental treatment should be carried out with precaution Pt with profound Immunodeficiency may require antibiotic cover before surgery or after maxillofacial .injury . Aspirin should be avoided because it may aggravate bleeding tendency . Needle stick injury should be avoided .Oral manifestations include ; candidosis , hairy leukoplakia , Kaposi sarcoma , viral infection .Oral features of childhood HIV disease Chronic parotitis is common and pathognomic ( it means that if you see patient with chronic parotitis . and patient is child then you suspect AIDS .(Chronic oral candidosis ( 75% .Difficulties in swallowing .( Herpetic stomitis ( recurrent oral and labial .Herpes zoster is uncommon in children but if they are immunodeficient it may happen :Idiopathic CD4 T lymphocytopenia .Represent a variety of disorders and it's like AIDS but not related to HIV Rare syndrome CD4 decreased around 300/µl . . Does not progress and may even improve spontaneously Immunosuppressive therapy : Immunosuppressive drugs are widely used in .Organ transplant .Autoimmune disease .Control some tumors .Connective tissue diseases Immunosuppressive drugs like : corticosteroids and calcineurin-inhibitors such as ciclosporin and . tacrolimus. And other cytotoxic drugs like azathioprine , cyclophosphamide and clorambucil .This Immunosuppressive drugs mostly effective against cell-mediated responses and autoantibodies .Pt on these drugs are strongly prone to infection and liable to neoplasms : Dental aspects Antimicrobial prophylaxis may be needed .Oral fungal infection are commonly seen in these patients Consult the physician before the surgery may need coverage ( usually double the dose ) to avoid adrenal .insufficiency : Leucopenia, Neutropena, and Agranulocytosis Leucopenia : low levels of circulating functional ( only functional ) leukocytes , either in absolute numbers or as functionally effective cells. It can be a feature of cytotoxic drugs and many other .diseases Neutropenia: low levels of circulating functional ( only functional ) PMN leukocytes either in absolute .numbers or as functionally effective cells Agranulocytosis : Clinical syndrome resulting from leucopenia or neutropenia or total absence of neutrophils . Clinical features : Agranulocytosis is sudden in onset , with fever , weakness and sore throat .Gingival, oral and pharyngeal ulcerations with pseudomembrane formation . Lymphoadenopathy and rashes .Later, haemorrhagic necrosis of MM, respiratory infections

.Purpura and bleeding : General management .Diagnosis is confirmed by blood examination and marrow biopsy .Treatment depend on the underlying cause .The main measure is to control infection and to avoid septicemia .Granulocyte colony stimulating factors (GCSF) are available any may be benefit : Dental aspect .Main oral manifestations are infections and ulcers .Periodontal diseases may be accelerated .Oral infections may be the source of septicemia and sometimes may be fatal .Oral infections can be controlled by topical or systemic antibiotics or sometimes by antiseptics .If broad spectrum antibiotics are used antifungal agents should be used .Dental surgical procedures should be covered with antibiotics .Hemorrhagic tendency with thrombocytopenia .Take into consideration the risks associated with corticosteroid therapy Asplenic patients :Functions of spleen .It's important for controlling the quality of erythrocytes in the circulation .It's the site of sequestration of effete erythrocytes It's essential for the production of Opsonins and thus important in phagocytosis and antibody .production Etiology : Splenectomy ( remove the spleen ) is done in .Hemolytic anemia .Splenic injury in abdominal trauma .Idiopathic thrombocytopenic purpura :Clinical feature Asplenia predispose to infections Children are 10 times more likely than adults to develop sepsis .Infections after splenectomy only rarely emanates from the oral flora .May develop malignant tumors : Dental aspects .No indication for antimicrobial prophylaxis before dental procedures .Exclude thrombocytopenia and corticosteroid therapy Chronic diseases associated with immune defects Diabetes mellitus: most common chronic disease associated with immune defects, and delayed healing, .defective phagocytosis . Cancers of lymphoid or hematopoietic tissues: specialy as a consequence of their treatment Infections : eg HIV (Primary ( congenital ) Immunodeficiency diseases (PIDD .Abnormalities in one or more genes important in immunity Congenital Immunodeficiency disease can be categorized according to the main type of immune cell .that is defective

: PIDD should be suspected if there are recurrent or persistent infections or other features such as or more ear infections within 1 year , 2 or more months on antibiotics with little effect, 2 or more 8 serious sinus infection within 1 year , failure of an infant to gain weight of grow normally , recurrent .deep skin or organ abscesses persistent thrush after age 1 year , a family history The more severe congenital immune defects often cause early death so they are not relevant to dental .practice except if the pt is treated with bone marrow transplantation Selective IgA deficiency .The most common congenital Immunodeficiency disease Prevalence 1 in 600 Only IgA is deficient in serum and other secretions : Clinical features Recurrent bacterial or viral respiratory infections GIT infections Autoimmune disease are abnormally frequent in these patients : Dental aspects ( No evidence that caries or periodontal disease are increased in these pt ( IgA is secreted in the saliva Occasionally associated with oral ulcers and herpes labials ( Common variable Immunodeficiency( CVID . Relatively common also called Hypogammaglobulinaemia . Symptoms do not appear until the teens or early adulthood .Frequent bacterial infections of ears, sinuses, lungs, and bronchi, GIT diseases in 30% of cases .Autoimmune hemolytic anemia : Mucocilliary syndrome Defect in the cilia of the respiratory tract that may lead to chronic sinusitis or other respiratory .infections

The End ‫كل عام وأنتم بخير ورمضان كريـــــم‬ ‫أنس محمد نايف صوالــــحة‬