1. Metyrapone
    • 11 hydroxylase inhibitor ->
    • increase in 11-B-deoxycortisol (no ACTH inhibiton)
    • -> increased ACTH ->
    • high levels of 11 deoxycortisol in serum
    • high levels of 17 hydroxy-corticosteroid in urine
  2. Neuroblastoma
    • Adrenal medulla tumor in children
    • HVA elevated in urine
    • No episodic hypertension like pheochromocytoma
    • Over expression of N-myc oncogene -> rapid tumor progression
    • Neurofilament stain of Homer Wright Pseudorosettes
  3. Glucocorticoids
    Decrease production of leukotrienes and prostaglandins by inhibiting phospholipase A2 and expression of COX-2

    CLINICAL: Addisons, inflammation, immune suppression, asthma, keloids

    TOXICITY: Cushings, buffalo hump, truncal obesity, muscle wasting, thin skin, easy bruisability, osteoporosis, adrenocortical atrophy, peptic ulcers, diabetes
  4. Metformin toxicity
    • Increases lactic acid production
    • Decreases gluconeogenesis

    • Contraindiacted in Renal failure
    • Also not for patients in heptic failure
  5. Fibronectin
    Connects integrin to the basement membrane and extracellular matrix with collagen and laminin
  6. Lead Poisoning
    • Lead lines on gingivae(burtons lines) and on epiphyses of long bones on x-ray
    • Encephalopathy and erythrocyte basophilic stippling
    • Abdominal colic and sideroblastic anemia
    • Wrist and foot drop

    • TX: Dimercaprol and EDTA
    • Succimer for kids
  7. Distinguishing factor among leukemias
    ALL: Children, TdT +

    CLL: >60yoa, smudge cells, warm antibody hemolytic anemia

    Hairy cell leukemia: TRAP +

    AML: Auer rods(peroxidase +, -> DIC), M3(all-trans retinoic acid)

    CML: Philadelphia chromosome (t[9:22], bcr-abl), low LAP
  8. Patients with lupus have a problem with mRNA what is the problem?
    • Ab to spliceosomal snRNPs are made
    • Therefore there is no splicing of hnRNA
    • therefore RNA cannot be processed and thus can not be transported out of the nucleus
  9. Rifampin
    Rifampin inhibits DNA dependent RNA polmerase

    Tetracycline binds 30S subunit preventing attachment of aminoacyl-tRNA
  10. Quad screen for Down Syndrome
    • dec a-fetoprotein
    • inc B-hCG
    • dec estriol
    • inc inhibin A
  11. Friedreich ataxia
    • Progressive ataxia of all 4 limbs (cerebellar dysfunction/ dorsal column)
    • Hypertrophic cardiomyopathy (arrhythmia/ CHF)
    • Skeletal abnormality - kyphoscoliosis
    • DM (10%)
  12. Cobalamin
    • Cofactor for homocysteine methyltransferase (transfers CH3 groups as methylcobalamin) Homocysteine + N-methyl THF -> Methionine and THF
    • Methylmalonyl-CoA mutase (methylmalonyl-CoA -> succinyl-CoA)

    • Deficiency:
    • macrocytic megaloblastic anemia, hypersegmented PMNS
    • neuro (paresthesias, subacute combined degeneration) because of abnormal myelin
  13. Vitamin E deficiency
    hemolytic anemia

    • Degeneration of spinocerebellar tract, dorsal column of spinal cord, and peripheral nerve
    • - ataxia, dysarthria, and loss of both position and vibration sense
  14. Arsenic inhibits:
    lipoic acid

    • Findings:
    • vomiting, rice water stools, garlic breath
  15. Pyruvate to lactate is seen where
    RBC, leukocytes, kidney, medulla, lens, testes, and cornea
  16. Hyperchylomicronemia
    • Increased chylomicrons
    • Elevated TG, cholesterol

    LPL deficiency or altered apolipoprotein C-II. Causes pancreatitis, hepatosplenomegaly, and eruptive/pruritic xanthomas (no increased risk for atherosclerosis
  17. What are the functions of the receptor that act on the Gq receptor
    a1 - inc vascular smooth muscle contraction, inc pupillary dilator muscle contraction, inc intestinal and bladder sphincter contraction

    M1- CNS, enteric nervous system

    M3- Inc exocrine gland secretion, inc gut peristalsis, inc bladder contraction, bronchonstriction, inc pupillary sphincter muscle contraction, ciliary muscle contraction

    H1- inc nasal and bronchial mucus production, contraction of bronchioles, pruritis and pain

    V1- inc vascular smooth muscle contraction
  18. Disulfiram like drugs
    • Metronidazole
    • procarbazime
    • 1st generation sulfonylureas
    • cephalosporin
  19. Legionella pneumophila
    • Stain with silver stain
    • Grows on charcoal yeast extract culture with iron and cysteine
    • Detected clinically by presence of antigen in urine

    Transmitted via aerosol, no person to person

    Causes Legionnaires disease, Pontiac fever, and interstitial pneumonia

    TX: erythromycin (macrolide)
  20. Hemolytic-uremic syndrome
    • Microangiopathic hemolytic anemia
    • acute renal failure
    • thrombocytopenia

    EHEC - shiga-like toxin -> endothelium swelling and narrowing -> symptoms
  21. Palm and sole rashes
    • Coxsackie A
    • Rocky mountain spotted fever (R. ricketsii)
    • Syphillis

    you drive CARS using your palms and soles
  22. In the tissue = spherule filled with endospores
    • Coccidioidomycosis
    • SW states -> pneumonia and meningitis
    • can disseminate to bone and skin
  23. Cryptococcus neoformans
    • -> Cryptococcal meningitis
    • Heavily encapsulated yeast transmitted by respiration  

    Founc in soil and pigeon droppings

    • Sabourauds agar, India ink stain, latex agglutination
    • Soap bubble lesion in brain
  24. RNA viral genomes (positive stranded RNA virus)
    • retrovirus
    • togavirus
    • flavivirus
    • coronavirus
    • hepevirus
    • calicivirus
    • picornavirus

    I went to a RETRO TOGA party, where I drank FLAVored CORONA and ate HIPPY CALIfornia PICKLES

    All RNA are ss except reoviridae and rotavirus
  25. RNA enveloped viruses
    • Influenza virus
    • parainfluenza virus
    • RSV
    • measles virus
    • mumps virus
    • rubella virus
    • rabies virus
    • HTLV
    • HIV
  26. Wnt-7 gene
    • Produced at apical ectodermal ridge(thickened ectoderm at distal end of each developing limb)
    • Necessary for proper organization along dorsal-ventral axis
    • mediates ectodermal function
  27. Mesoderm derivatives
    • Muscle
    • bone
    • connective tissue
    • serous lining of body cavities
    • spleen
    • cardiovascular structures
    • lymphatics
    • blood
    • bladder
    • urethra
    • vagina
    • eustachiantube
    • kidneys
    • adrenal cortex
    • skin dermis
    • testes / ovaries
  28. Posterior fossa malformations
    Chiari II - cerebellar tonsillar herniation through foramen magnum with aqueductal stenosis and hydrocephaly. Presents with syringomyelia, thoracolumbar myelomeningocele.

    Dandy-Walker- large posterior fossa; absent cerebellar vermis with cystic enlargement of 4th ventricle. Can lead to hydrocephalus and spina bifida
  29. Granulomatous diseases
    • Tuberculosis
    • Fungal (histoplasmosis, blastomycosis)
    • Syphillis gummas
    • Leprosy
    • Cat scratch fever
    • Sarcoidosis (noncaseating)
    • Crohns disease
    • Berylliosis
    • Listeria
    • Foreign bodies
    • Wegners granulomatous
    • Chronic granulomatous disease
  30. Oncogenes
    • abl - tyrosine kinase- CML
    • c-myc- transcription factor- Burkitt's lymphoma
    • bcl-2- antiapoptotic- Follicular/ undiff lymphoma
    • erb-B2 tyrosine kinase- breast ovarian and gastric carcinomas
    • ras - GTPase - Colon carcinoma
    • L-myc- Transcription factor - lung tumor (SCC)
    • N-myc - transcription factor- neuroblastoma
    • ret- tyrsine kinase - MEN
    • c-kit - cytokine receptor- gastrointestinal stromal tumor
  31. HLA DR2
    • Multiple sclerosis
    • hay fever
    • SLE
    • Goodpasture's
  32. Cell surface proteins of B cells for EBV
    • CD40
    • MHC II
    • B7
  33. Drugs the prolong AT interval
    • Macrolides (erythromycin)
    • Antimalarials (chloroquine/ mephaquine)
    • Haloperidol
    • Risperidone
    • Methadone
    • Protease inhibitors
    • Antiarrhythmics (Class IA)
    • K channel blockers
  34. What cardiac valve defect is associted with DiGeorge's syndrome
    • Tetralogy of Fallot
    • Transposition of the great vessels
  35. What stimulates release of prolactin
    • Dopamine antagonists (antipsychotics)
    • estrogens (OCPs, pregnancy)
    • TRH
  36. Why is Hydralazine good for patients with Mitral Regurgitation?
    They decrease afterload -> increase in foward stroke volume and a decrease in regurgitant flow.
  37. Side effects of niacin
    • Flushing
    • vasodilation
    • insulin resistance
    • hyperuricemia
  38. Which murmurs increase with inspiration
    Right sided murmurs
  39. What substances have nuclear receptors
    • Thyroid hormones
    • retinoids
    • PPAR
    • Fatty acid
  40. How do ACE inhibitors contribute to cough
    • Block degradation of bradykinin and substance P
    • -> lung irritation
  41. What seurm labs would you see with primary adrenal insufficiency
    • decreased Na and Cl-
    • increased K+
    • metabolic acidosis (dec HCO3-)
  42. B type natriuretic peptide
    From the ventricles in response to hypertrophy via volume overload
  43. What is the cause of tetralogy of fallot
    Abnormal neural crest cell migration
  44. Atenolol actions
    • B1 only:
    • decreased cAMP in heart (dec contractility and HR)
    • decreased cAMP in JG cells
  45. Imprinting enzyme
    • DNA methylation via methyltransferase
    • same enzyme used in the SAM reaction
  46. NEP effect on receptors
    • a1- via IP3 increase in the arteries
    • B1 increase cAMP in the heart
    • a 2- dec cAMP -> dec NEP and insulin
  47. What cholesterol lowring drugs are responsible for myositis when combined
    Statins and fibrates(gemfibrozil)
  48. What is the recommended treatment for Staph epidermidis valve endocarditis
    Vancomycin with Rifampin and/or gentamicin
  49. What is the effect of B-blockers and verapamil combined?
    Very low heart rate (negative chronotropy)
  50. Lipofuscin
    insoluble pigment consisting of lipid polymers and protein complexed phospholipid) that come from free radical injury and lipid peroxidation
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