Term
| DOC for partial (Simple and complex) and tonic clonic seizures |
|
Definition
carbamazepine
phenytoin
valproid acid |
|
|
Term
| 4 other drugs useful in partial and tonic clonic seizures |
|
Definition
lamotrigine
topirimate
valproic acid
gabapentin (used as adjuvant and for diabetic peripheral neurology) |
|
|
Term
|
Definition
Ethosuximide
Valproic acid |
|
|
Term
| Used to treat status epilepticus and eclampsia |
|
Definition
|
|
Term
| Common side effects of epilepsy drugs |
|
Definition
Diplopia
Sedation
Ataxia
Nystagmus
Dizziness |
|
|
Term
| Additional SE of phenytoin |
|
Definition
gingival hyperplasia
hirsuitism
fetal hydantoin syndrome (cleft lip cleft palate, congenital heart disease,mental retardation)
induces CYP450
Steven johnson syndrome
Drug induced lupus
Lymphadenopath |
|
|
Term
| Which anti- eplipetics are teratogens |
|
Definition
phenytoin
carbamazepine
valproic acid (neural tube defects) |
|
|
Term
| What drugs cause stevens johnson syndrome |
|
Definition
seizure drugs
sulfonamides
cillins
allopurinol |
|
|
Term
| what drugs are known to cause agranulocytosis |
|
Definition
carbamazepine
clozapine
colchicine
PTU/ methemazole |
|
|
Term
| Hepatotoxic anti epileptics |
|
Definition
|
|
Term
| what drugs induce the p450 system |
|
Definition
BCG PQRS
Barbituates, carbamazepine, griseofulvin,phenytoin, quinidine, rifampin, st johns wart |
|
|
Term
| What anti epileptics block Na channels |
|
Definition
carbamazepine
phenytoin
lamotrigine
topirimate
valproic acid |
|
|
Term
| Which antileptics work by potentiating the inhibitory effects of gaba? |
|
Definition
| valproic acid, phenobarbital, benzodiazepines, gabapentin, topirimate, tiagamin, vigabatrin, levetiracetam |
|
|
Term
| Areas of the brain most susceptible to ischemic damage |
|
Definition
| pyramidal cells of hippocampus (Sommers sector), purkinje cells of the cerebellum-- found in the watershed zones |
|
|
Term
| What vitamin inccreases peripheral metabolizm of levodopa metabolism and decrease its effectivness |
|
Definition
|
|
Term
Cyclosporine
MOA
Clinical Use
Tox |
|
Definition
MOA: Binds to cycophilin. Complex blocks the differentiation and activation of T cells by inhibiting calcineurin, thus preventing production of IL-2 and its receptor
Clinical use: supresses organ rejection after transplantation; selected autoimmune disorders
Tox: Predisposes patients to viral infection and lymphoma; nephrotoxic (preventable with mannitol diuresis), gout |
|
|
Term
Tacrolumus:
MOA:
Clinical Use:
Toxicity |
|
Definition
MOA: Similar to cyslosporin- binds to FK binding protein, inhibiting secretion of IL2 and other cytokines
Clinical use: Potent immunosupressant used on organ tansplant recipient
TOx: nephrotox, peripheral neuropathy, htn pelural effusion hyperglycemia |
|
|
Term
Sirolimus (rapamycin)
MOA:
Clinical Use:
TOx: |
|
Definition
MOA: Inhibits mTOR. Inhibits T cell proliferation in response to IL2
Clinical use: immunosupression after kindey transplantation in combination with cyclosporine and corticosteroids. Also use with drug eluting stents
Tox: Hyperlipidemia, thrombocytopenia, leukopenia |
|
|
Term
Daclizumab
MOA:
Clinical Use:
Tox: |
|
Definition
| MOA: monoclonal antibody with high affinity for the IL-2 receptors on activated T cells |
|
|
Term
Azathioprine
MOA:
Clinical Use:
Tox: |
|
Definition
MOA: antimetabolite precursor of 6-mercaptourine that interferes with the metabolism and synthesis of nucleic acids. Toxic to proliferating lymphoctes
Clinical use: kindey transplant, autoimmune disorders (including flomerunonephritis and hemolytic anemia
Tox : bone marrow supression: active metabolite mercaptopurine is metabolized by canthine oxidase, thus toxic effects may be increased by allopurinol |
|
|
Term
Muromanab- CD3
MOA:
Clinical Use:
Tox: |
|
Definition
MOA: Monoclonal antibody that binds to CD3 (episol chain) on the surface of T cells. Blocks cellular interaction wit hCD3 protein responsible for T cell signal transduction
Clinical Use: immunosupression after kindey transplantation
Tox: cytokine release syndrome, hypersensitivity reaction |
|
|
Term
|
Definition
| anti- angiogenic: inhibits TNF alpha, limb deformities/ phocomelia |
|
|
Term
Mycophenolate:
MOA:
Clinical Use:
Tox: |
|
Definition
MOA: inhibits inosine monophosphate dehydrogenase. Prevents production of nucleoside guanine
Use: transplant and lupus nephritis |
|
|
Term
Brutons agammaglobulinemia
Defect:
Presentation:
Labs: |
|
Definition
Defect:X linked recessive, defect in BTK/ tyrosine kinase --> blocks pro B to pre B
Presentation: Recurrent bacterial infection after 6 mo: lower resp infections, g. lamblia indections, serum Ig low, positive reaction to candida albicans, absent thymic shadow
Labs: Decrease proB, decreased number of B cells, decreased immunoglobulins of all classes |
|
|
Term
Hyper IgM syndrome
Defect:
Presentation:
Labs: |
|
Definition
Defect: CD40 ligand on helper T cells- inability to class switch
Presentation: Severe pyogenic infection earliy i nlife ( x linked = no cd40, AR = no CD 40)
Labs: Increased IgM, decreased everything else |
|
|
Term
Selective Ig deficiency
Defect:
Presentation:
Labs: |
|
Definition
Defect: defect in isotype switching, deficiency i nspecific class of immunoglobulins
Presentation: sinus and lung infections, milke allergies and diarrhea. Anaphylaxis on exposure to blood products with IgA
Labs: IgA deficiency most common- failure to mature into plasma cells, decrease secretory IgA- recurrent sinus/ uri- make IgG against IgA |
|
|
Term
Common variable immunodeficiency
Defect:
Presentation:
Labs: |
|
Definition
Defect: Defect in B cell maturation, many causes
Presentation: Can be acqured in 20s- 30s, increased rick of autoimmune dx, lumphoma, sinupulmonary infections
Labs: normal number of B cells: decreased plasma cells, immunoglobulins |
|
|
Term
Thymic Aplasia (DiGeorges syndrome)
Defect:
Presentation:
Labs: |
|
Definition
Defect: 22q11 deletion: failure to develop 3rd and 4th pharyngeal pouches- detect with FISH
Presentation: Tetany (hypocalcemia) recurrent viral fungal infection- t cell deficiency, congenital heart defect and great vessel defects
Labs: Thymus and parathyroids fail to develop: dectreas T cells, PTH, Ca2+, trousseaus sign, chovstecks sign, absent thymic shadow |
|
|
Term
IL12 receptor deficiency
Defect:
Presentation
Labs: |
|
Definition
Defects: decreased TH1 response
Presentation: disseminated mycobacterial infections
Labs: decresed IFN gamma (disseminated mycobacterium infections |
|
|
Term
Hyper IGE syndrome/ Jobs syndrome
Defect:
Presentation:
Labs: |
|
Definition
Defect: TH cells fail to produce INF gama- inability of neurotrphils to respond to chemotactic stimuli (Retained teeth)
Presentation: FATED: coarse Facies, cold (noniflamed) staphylococcal Abscesses, retained primary Teeth, icnreased IgE, Dermatologic problems (eczema)
Labs: increased IgE, increased eosinophil
(eczema, recurrent cold staph aureas abscess, coarce facial features) |
|
|
Term
Chronic mucocutaneous candidiasis
Defect:
Presentation:
Labs: |
|
Definition
Defect: T cell dysfunction (prevents spread of superficial candida infections)
Presentation: Candida albicans infection of skin and mucous membrane
Tx: ketoconazole |
|
|
Term
| 3 x linked immunodeficiencies |
|
Definition
Wiscott aldrich
Brutens Agammmaglobinemia
Chronic granulomatous disease |
|
|
Term
Severe combined immunodeficiency
Defect:
Presentation:
Labs: |
|
Definition
Defect: Several types: defective IL2 receptor (most common X linked) adenosine deaminase deficiency, failure to syntehsize MHC II antigens
Presentation: recurrent viral, bacterial, fungal, and protozoal ifnections due to both B and T cell deficiecny. Basnce of thymic shadow, germinal cells (lymph node biopsy) and B cells ( peripehral blood smear) Tx = bone marrow transplant
Presentation:
Labs:Decreased IL2 = T Cell activation, increased adenine (toxic to B and T cells) decreased dNTPs and decreased DNA synthesis
(severe recurrent infections, chronci diarrhea, failure to thrive, no thymus in x ray- decreased serum gamma globin levels |
|
|
Term
ATM
Defect:
Presentation:
Labs: |
|
Definition
Defect: Defect in ATM gene which codes for DNA repair enzymes
Presentation: cerebellar defects, spider angiomas, IgA deficiency (poor smooth pursuit)
Labs: IgA deficiency- increased risk of lymphoma, leukemia, radiation sensitive |
|
|
Term
Wiskott Aldrich syndrome:
Defect:
Presentation:
Labs: |
|
Definition
Defect: x linked recessive defect, progressive deletion of B and T cells
Presetation: TIE: trhombocytopenia purpura, infections, eczema
Labs: increased IgE, IgA, Decreased IgM |
|
|
Term
Leukocyte adhesion deficiency (type 1)
Defect:
Presentation:
Labs: |
|
Definition
Defect: Defect in LFA-1 integrin(CD18) preotein on phagocytes
Presentation: recurrent bacterial infections, absent pus foormation, delayed speration of umbilicus
Labs: neutophilia |
|
|
Term
Chediak Higashi Syndrome
Defect:
Presentation:
Labs: |
|
Definition
Defected: AR; defect in lysosomal regulation trafficking gene/ microtubule dysfunction in phagosome- lysosome fusion
Presentation: recurrent pyogenic infections by staphylococci and streptococci; partial albinism, peripheral neuropathy/ seizures, neurologic disorders
Labs: giant cytoplasmic granules |
|
|
Term
Chronic granulomatous disease
Defect:
Presentation:
Labs: |
|
Definition
Defect: Lack of NADPH oxidase- decrease ROS and absent respiratory burst in neurophils- cannot make phagolysosomes
Presentation: increased susectiiblty to catalase positive organisms (S. aureus, E. coli, Aspergillus, psudimonas)
Labs: Negative nitroblue tetrazolium dye reduction test (no color change- phagocytes cant oxidize dye)
Tx: prophylactive TMP SMS INFgamma |
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|
