The Wiskott Sydrome-Aldrich is a X-linked recessive disorder characterized by the triad of anomalies: thrombocytopenia, eczema and selective functions of B and t lymphocytes immunodeficiency
Pathophysiology
The exact defect is unknown, but recent evidence suggests an anomaly of cellular energy metabolism specifically euphoric basic hematopoietic cells. A variety of pathological findings are evident. Platelets are abnormally small in size and have a shortened, possibly due to a metabolic defect in its short life expectancy.Primary immune defect consists of phagocytes (macrophages) inability to process foreign antigens, particularly of poly-saccharides such as pneumococcus, of as a result, competent immunologically cells fail to produce immunoglobulin normal.Temprano patterns in life, immunoglobulin levels may be normal, but later observed low levels of IgM. General of iso-hemagglutinins (anti-A and agglutinins fast-B blood) are decreased or absent.
The thymus gland and lymph nodes are normal at birth but become increasingly dysfunctional with age until the results of a deep cell immunodeficiency. As a result, these children are highly susceptible to infection and malignancy, particularly of reticular lympho system.
Clinical manifestations
At birth, the principal effect of the disease is due to the trombocitopenia.Como bleeding the child grows, eczema and recurrent infection become more severe and bleeding becomes less frecuente. Eccema is typical of the allergic type and easily becomes super-infected.Chronic infection with herpes simplex is a common problem and may lead to chronic keratitis eye with vision loss. Infection, as a result half of otitis, sinusitis and lung disease chronic. In those children who survive episodes of bleeding and an overwhelming infections, malignancy presents an additional risk for survival.
Evaluation of diagnostic
Diagnosis can usually be made during the neonatal period due to the trombocitopenia.Pruebas function-specific immunological confirms the diagnosis.It is also possible to defection of carrier.
Therapeutic management
Consists mainly of medical treatment
1 Counter trends bled with platelet transfusions
2. Using the immunoglobulin to provide passive immunity, and
3. Administration of prophylactic antibiotics to prevent and control infection.
Bone marrow transplantation have been attempted, but even if successful, do not reverse all the defects of this trastorno.General median survival is only 6.5 years old with more often resulting death of bleeding or infection (pneumonia or sepsis).
Nursing considerations
Due to the severe prognosis for these children, home nursing review is supporting family in the care of a child fatally ill. physical care is intended to handle the challenges imposed by the control of bleeding disorder, prevent or control the infection, treat eczema.
The genetic implications of the X-linked recessive disorder differ little from those of hemophilia, however, due to the multiple defects, emotional adjustment and physical care for these children are higher that those of many other condiciones.La nurse can be especially supported by providing short-term objectives during hospitalization periods and focusing on the needs long-term efforts coordinated with a public health nurse.
Article Directory networks are an excellent, but hidden in lots of money on internet. No strategy is a system of making easy money and does not require that your dollars so that you can get the information of an e-book. Es strategy free that it is happily shared so many people posible. Es 100%, if you follow strategies necesarias.cada website, technicalities and 'know-setlist' are exposed and you need not worry, you'll get everything you need saber.A sí that what networking article directory? click on the link below to obtain adequate information on this stunning revelation
