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NSG213a

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Immune disorder where there is a low level of antibodies, manifested by recurrent infections between infancy and adulthood. CVID Common variable immunodeficiency
Cause of CVID? Unknown- may affect one or more than one family member. Sign is decrease in immunoglobulins
Areas of infection with CVID? *Lungs damage can be permanant *Ears *Sinuses *Join inflammation *stomach/bowel disorder *increase r/f cancer
TX for a patient with CVID? *Immunoglobulin therapy *med for illness *postural drainage of the lungs
Group of rare, life threatening diseases present at birth. Little or no immune system. Absence of __ cell (humoral) and __ cell (cell mediated) immunity. SCID Severe combined immunodeficiency T and B
Known as boy in the bubble syndrome SCID
Cause of SCID? Inherited, many genetic defects an cause; reduced ability of white cells to fight infection. X-linked or autosomal recessive disorder. Affects boys/girls
Disease? X linked or autosomal recessive disorder. Inherited. SCID
S/S of SCID? When do symptoms arise? *Severe pneumonias *meningitis *sepsis *chronic skin infections *yeast of mouth/diaper area *diarrhea *liver infections **S/S arise within first year**
What tx would you expect for a patient with SCID? *hematopoietic stem cell transplan *IV IG infusion, til bone marrow or stem cell transplant *pneumocystitis carinii pneumonia ther. *bone marrow trans *immune boost meds *gene therapy
Best therapy for SCID? Compatible Bone marrow transplant
Wiskot aldrich syndrome (WAS) what is it? Syndrome where there is a primary immune deficiency involving both T and B lymphocytes. Plateles affected as well. X linked
Infant girl is admitted with Wiskott Aldrich syndrome. In what way is this treated differently in a female than a male? A female won't have this. It is X-linked, a male only disease.
S/S of WAS? *increased bleeding (thrombocytopenia) Hallmark: petechia, bloody stool, bleeding gums, prolonged nose bleed, brain hemorrhage *Recurrent bacterial, viral, fungal infection esp respiratory tract *exzema
Recurrent infections commonly seen in WAS? *Respiratory tract *sepsis *meningitis *recurrent Herpes Simplex *Pneumocystitis carinii
An older male with WAS may exhibit these symptoms... *automimmune-like* *an anemia caused by antibodies which destroy RBC's *FUO with no cause *swollen joints *kidney inflamm. *Gi s/s Diarrhea *arterial infl. vasculitis
Malignancies involved in WAS *involves lymphocytes; lymphoma and leukemia
Located on the short arm of the X chromosome. X linked recessive disorder, mutations between families are unique, severity varies WAS
How is WAS diagnosed? platelet count and cell size. Serum antibodies, skin tests to view T-Lymphocyte function.
Tx for WAS? *prophylactic use of IVIG *Bone marrow transp *Iron replacement *Tx infections *vaccines *bath oils *steroid creams *assess food allergy *platelet transfusions
What is the only cure for WAS? Bone marrow or cord blood stem cell transplantation. Possible splenectomy to correct thrombocytopenia
First immunodeficiency disease ever identified as X-linked Agammaglobulinemia (BRutons or congenital)
Disease in which there is an inability to produce antibodies that make up gamma globulins in the plasma. failure of pre-bplymphocytes to mature into b-lymphocytes that produce antibodies. X-linked agammaglobulinemia
child cannot fight off bacterial infections and some viral infections X linked agammaglobulinemia
S/S of x linked agammaglobulinemia in first 6-8 mos-3-5 yrs. First 6-8 mos; sinusitis, rhinitis, pyoderma, conjunctivitis, osteomyelitis, meningitis, sepsis, bronchitis, pneumonia, GI infection, Viral infection: hepatitis, polio, enterovirus
S/S of what disease? Growth failure, absence of tonsils/adenoids,joint disease,autoimmune hemolytic anemia, glomerulonephritis, neutropenia, dermatomyositis, cancers x-link agammaglob
Tx for x-linked agammaglob antibody replacement, infection mgmt, avoidance of live viral vaccines
prognosis of x-linked agammaglob without tx? Death at early age without gamma glob RX, With good med mgmt they may live into adult years.
Most childhood cancers arise from what layer? embryonic mesodermal layer; lesser amount from the ectodermal layer
Childhood cancer presents in the deeper organs, making it different from adult cancers. Examples of childhood cancer presentations? *Compression (tumor) *Obstruction *altered CBC *abnormal secretion of hormones
S/S of childhood cancers *wt loss *anorexia *fever without cause *pallor *NV *fatigue *masses/swollen body parts *visual disturbance *bruising/petechiae *HA without cause *unsteady gait/limp *cough/dyspnea
Types of childhood leukemia? *Acute lymphocytic leukemia (accounts for 3 out of 4 cases. *Chronic lympho leukemia (rare) *acute myelogenous accounts for bulk of the remaining cases. *chronic myelogenous- rare
This type of leukemia starts from the lymphoid cells in the bone marrow. ALL (acute lymphocytic leukemia)aka acute lymphoblastic
This leukemia starts from the cells that form white blood cells (other than lymphocytes) AML- acute myelogenous/aka acute myeloid, acute myelocytic, acute non-lymphocytic leukemia
Rare leukemia in children r/t CML philadelphia chromosome Chronic myelogenous
If a child with Chronic myelogenous leukemia goes into remission after treatment, what additional treatment would be suggested? Stem cell transplant
Cause of fatigue and paleness of skin in presence of leukemia? anemia
Cause of infections and fever in presence of leukemia? Fever of unknown origins, often r/t infection, which may not improve with antibio. R/T a lack of normal WBC's particularly mature granulocytes.
Cause of bleeding/bruising of skin in presence of leukemia? r/t bleed from tiny blood vessels
Bone or joint pain causes in leukemia? R/T buldup of leukemia cells near surface of bone or inside joint
What causes swelling of abdomen in leukemia? cells may collect in liver and spleen causing enlargement.
What causes the loss of appetite/weight loss in leukemia? Enlargement of liver and spleen causing them to place pressure on the stomach.
Enlarged lymph nodes in children, are most often a sign of leukemia. T/F? False, they are often a sign of infection only.
Enlargement of __________ can be the cause of coughing. Thymus or lymph nodes in chest press on trachea.
Leukemia Cause of swelling in face and neck? superia vena cava is next to the thymus, when thymus enlarges, it can press on SVC causing blood to back up. EMERGENCY
In the presence of superior vena cava compression in a child with leukemia, what signs beyond swelling of the face and arms may be seen? bluish-red coloration of head,arms and upper chest. can also cause trouble breathing and change in consciousness- affecting brain
headache, concentration problems, weakness, seizure, vomiting, balance problems, blurred vision can be systems of what complication in leukemia? Central system involved leukemia
Collection of AML cells under skin or other parts of the body are called.... chloroma or granulocytic sarcoma
When symptoms of extreme tiredness, weakness, slurred speech occurs, what is the cause? high numbers of leukemia cells, making the blood too thick interfering with small vessel brain circulation.
Diagnostics in leukemia- CBC is done to reveal? Determines how many of each type of blood cell is present in the blood. Blood smear also done for microscopic analysis
Many of the WBC found in the blood in leukemia will be ______ which is found only in the bone marrow. Bone marrow
Bone marrow biopsies are taken from the back of the pelvic bone, but may also be taken from this area. Sternum or other bones.
Why would a lumbar puncture be done in leukemia? Determines cells present in the CSF, and also can be a route to admin chemo drugs to prevent or tx spread of leukemia to the spinal cord and brain.
Lymph node biopsy is important in dx lymphomas but-- is rarely needed for children with leukemias
What 3 chemo phases are used in tx of children with acute lymphocytic leukemia? 1. induction 2. consolidation/intensification 3. maintenance
Created by: purpleapple87