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What are the possible components of VHL?
- Von Hippel Lindau disease
- hemangioblastomas of the retina, cerebellum, and medulla
adenoma and cysts of liver, kidney, and pancreas
increased risk of renal cell carcinoma
What is the triad of normal pressure hydrocephalus?
incontinence, gait difficulty, and mental decline.
Wet wobbly and wacky
What is the main antibody within the mucosal lining of the gut?
What electrolyte produces peaked T waves and prolonged PR intervals?
What are the features of Waldenstrom's macroglobulinemia?
Weakness, weight loss, monoclonal M spike and hyperviscosity syndrome(-> nosebleeds, headaches, and vision disturbances)
What can be given to prevent the neurotoxicity of a drug that decreases the synthesis of mycolic acids after being activated by bacterial catalase-peroxidase.
Isoniazid- give pyridoxine to to prevent the neurotoxicity.
Also causes hepatoxicity and drug induced lupus.
What is the difference between the drugs that bind ergosterol and forms membrane pores that allow leakage of electrolytes?
- Amphotericin B:
- oral & IV
- FOR serious systemic mycoses(cryptococcus, blastomyces, coccidioides, aspergillus, histoplasma, Candida, and Mucor)
- Topical only
- FOR candida
What subtype drug of the group that inhibits P450 enzyme leading to no lanosterol to ergosterol conversion, is used for cryptococcal meningitis in AIDS and all candidal infections.
- These are azoles
- Subtype = Fluconazole
What is the MOA of the drugs that are used for dermatophytes?
- inhibits fungal enzyme squalene epoxidase -> no cell wall synthesis
- interferes with microtubule function-> disruption of mitosis.
What is the mechanism of action of the 2 drugs used to treat the following condition:
enlarged lymph nodes, recurring fever, somnolence, coma, and recently bitten by the Tsetse fly?
- inhibits enzymes involved in energy metabolism.
- Inhibits sulfhydryl groups in the enzymes. For CNS involvement.
What is the treamtent and its MOA.
Treats disease transmitted by black fly and cases hyperpigmented skin and river blindness.
- Ivermectin: Onchocerca volvulus
- Intensifies GABA-mediated neurotransmission and causes immobilization. Does not cross blood-brain barrier -> no effect on human.
What is the MOA and clinical use of Ribavirin.
MOA: inhibits synthesis of guanine nucleotides by competively inhibiting IMP dehydrogenase.
CLINICAL: RSV and chronic hep C
What separates Foscarnet from acyclovir and ganciclovir?
Acyclovir and ganciclovir rely on the viral thymidine kinase to be activated thus leading to resistance.
Foscarnet does not need activation via thymidine kinase therefore -> can treat CMV retinitis (ganciclovir fails) and acyclovir-resistant HSV. But resistance can be due to mutated DNA polymerase.
What else could you use to treat a AIDS patient with a CD4 <350 or a high viral load?
- These are NRTI's
- you would need 2 of them along with either a protease inhibitor or a NNRTI to complete the HAART
What are the antibiotics you want to avoid in pregnancy and why?
- Sulfonamides: kernicterus
- aminoglycosides: ototoxicity
- Fluoroquinolones: cartilage damage
- Erythromycin: acute cholestatic hepatitis in mom
- Metronidazole: mutagenesis
- tetracyclines: discolored teeth and inhibition of bone growth
- Ribavirin: teratogenic
- Griseofulvin: teratogenic
- Chloramphenicol: gray baby syndrome
Identify the parts of the lymph node from external to internal.
- Follicle of cortex (B cells and germinal center)
- paracortex (T cells and endothelial venules)
- Medullary cords (plasma cells)
- Mdeullary sinus (macrophages & communicate with efferent lymphatics)
Where does lymph from the sigmoid colon drain?
Colic -> inferior mesenteric
What is the pathogenesis of splenic dysfunction?
- decrease IgM -> decrease complement activation -> decrease C3b opsonization -> increase susceptibility to encapsulated organisms.
- S. pneumoniae
- H. influenzae
- N. meningitidis
- (klebsiella pneumoniae) S SHiN
What diseases and cells are associated with DR2?
This is expressed on the MHC II receptors which are CD4 T cells or helper T cells
Diseases: Multiple Sclerosis, hay fever, SLE, Goodpastures.
What are the three main cytokines produced by Th1 cells and what are their functions?
- IL-2 = T cell activation
- IFNy = stimulate macrophages
What 2 signals are need to activate Helper T cell activation?
- APC with antigen on MHC II to the TCR on the Th cell
- costimulatory B7 to CD28 on Th cell
What 2 signals are needs to activate B-cell class switching?
- IL-4, IL-5, IL-6 from Th2 cells
- CD40 receptor binds to CD40 ligand from Th cell
Where does complement binding occur on the antibody?
On the CH2 of IgM and IgG antibodies
What are adverse effects of Polymyxins?
- Detergent like antibiotic
- SIDE EFFECTS:
What would you see on immunoflurescence of the kidney with Goodpasture's syndrome?
linear pattern of IgG deposition along the basement membrane.
What is derived from the neural crest cells?
Melanocytes, odontoblasts, pia and arachnoid mater, schwann cells, cells of the ganglia, parafollicular C cells of thyroid, chromaffin cells, aorticopulmonary septum, pharyngeal arch skeletal components, and neurocranium
What is the mechanism of action for Mycophenolate mofetil?
Inhibits de novo synthesis of the guanine nucleotide. Used for prophylaxis of organ rejection in patients recieving allogenic renal, cardiac or hepatic transplants.
What are the 4 families of viruses that have segmented genomes?
- Genetic shifts - oh shit!!!!
Which antibody is this?
Involved in delayed response to an antigen. most abundant in blood. Fixes complement, crosses the placenta(passive immunity for infants), opsonizes bacteria, neutralizes bacterial toxins and viruses.
What does a deficiency of C3 cause?
Leads to severe, recurrent pyogenic sinus and respiratory tract infections. Increase susceptibility to type III hypersensitivity reactions.
What is it that helps prevent complement activation on self-cells?
Decay-acclerating factor (CD55 & 59) and C1 esterase inhibitor.
activates macrophages and TH1 cells.
Suppresses Th2 cells
Has antiviral and anitumor properties
is secreted by Th1 cells
What is the function of IL-10
- Modulates inflammatory response.
- Inhibits actions of activated T cells and Th1.
- Activates Th2
Secreted by Th2 cells
What cell has the following receptors?
MHC II, B7, CD40, CD14, recpeptors for Fc and C3b
What are the receptors for NK cells
MHC I, CD16(binds Fc of IgG) , and CD56.
To which patients would you give preformed antibodies?
- Those exposed to:
- Tetanus toxin
- Botulinum toxin
- Rabies virus
Describe Type II hypersensitivity
and give examples
- Antobody mediated- IgM or IgG bind to fixed antigen on host cell, leading to lysis or phagocytosis via:
- 1. opsonization or complement activation
- 2. Antibody recruitment of neutrophils and macrophages -> inciting tissue damage
- 3. Bind to normal cellular receptors and interfere with functioning.
- Goodpasture's syndrome, hemolytic anemia
- Bullous pemphigoid, pernicious anemia
- pemphigus vulgaris , idiopathic thrombocytopenic purpura
- Myasthenia gravis, erythroblastosis fetalis
- Acute hemolytic transfusion reactions
- Rheumatic fever, graves
What type of hypersensitivity is this?
Type 1 DM
- Type IV hypersensitivity
- Delayed (T-cell -> macrophage activation)
What is the assoicated disorder for anti-smooth muscle autoantibody?
What is the auto-antibody that is associated with Type 1 diabetes mellitus?
How do you treat the most common cause of croup?
The most common cause is parainfluenza virus and treatment is supportive.
What is central pontine myelinosis?
Overaggressive treatment of hponatremia. (Faster than 1mEq/L/hr = faster than brain can compensate -> neuronal shrinkage and death -> dysarthria, dysphagia, and flaccid quadriparesis.
What uses the Gq pathway?
Muscarinic acetylcholine receptor, alpha1-adrenergic and histamine receptors.
Characteristics of multiple myeloma
Pathologic fractures, lytic lesions on X-ray, Bence Jones protein in the urine, M spike on serum protein electrophoresis, and systemic amyloidosis(secondary to Ig light chain overproduction)
How does a patient with Henoch-Schonlein purpura present?
- It is a systemic vasculitis caused by deposition of IgA immune complexes. Follows respiratory infection.
- purpura(on buttocks and legs)
- abdominal pain
- and glomerulonephritis
- Some present with arthritis
- THIS IS A DISEASE OF CHILDREN
How does hydrochlorothiazide cause hepatic encephalopathy in a patient with cirrhosis?
Hydrochlorothiazide causes hypokalemia metabolic alkalosis which is known to preicpitate hepatic encephalopahty (along with azotemia and hypovolemia)
What is the bacteria:
non-lactose-fermenting, oxidase positive organism that produces procyanin. Virulence is its exopolysaccharide capsule --> slime layer for adherence. Leading cause of death in patients with cystic fibrosis.
What are the effects of prostaglandins and Angiotensin II on the kidneys?
- Angiotensin II vasoconstricts the efferent arterioles
- Prostaglandins vasodilate the afferent arterioles
- Overall effect: increase in GFR but decrease in RPF
What is labetalol?
A nonselective alpha and beta antagonist.
What would you administer in the following situation:
Patient presents with slurred speech, lack of coordination, unsteady gait, and impaired attention or memory.
- This is benzodiazepine overdose.
- Treatment is Flumazenil, a benzodiazepine receptor antagonist.
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