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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
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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
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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
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Metformin toxicity
- Increases lactic acid production
- Decreases gluconeogenesis
- Contraindiacted in Renal failure
- Also not for patients in heptic failure
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Fibronectin
Connects integrin to the basement membrane and extracellular matrix with collagen and laminin
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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
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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
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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
-
Rifampin
Tetracycline
Rifampin inhibits DNA dependent RNA polmerase
Tetracycline binds 30S subunit preventing attachment of aminoacyl-tRNA
-
Quad screen for Down Syndrome
- dec a-fetoprotein
- inc B-hCG
- dec estriol
- inc inhibin A
-
Friedreich ataxia
- Progressive ataxia of all 4 limbs (cerebellar dysfunction/ dorsal column)
- Hypertrophic cardiomyopathy (arrhythmia/ CHF)
- Skeletal abnormality - kyphoscoliosis
- DM (10%)
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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
-
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
-
Arsenic inhibits:
lipoic acid
- Findings:
- vomiting, rice water stools, garlic breath
-
Pyruvate to lactate is seen where
RBC, leukocytes, kidney, medulla, lens, testes, and cornea
-
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
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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
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Disulfiram like drugs
- Metronidazole
- procarbazime
- 1st generation sulfonylureas
- cephalosporin
-
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)
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Hemolytic-uremic syndrome
- Microangiopathic hemolytic anemia
- acute renal failure
- thrombocytopenia
EHEC - shiga-like toxin -> endothelium swelling and narrowing -> symptoms
-
Palm and sole rashes
- Coxsackie A
- Rocky mountain spotted fever (R. ricketsii)
- Syphillis
you drive CARS using your palms and soles
-
In the tissue = spherule filled with endospores
- Coccidioidomycosis
- SW states -> pneumonia and meningitis
- can disseminate to bone and skin
-
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
-
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
-
RNA enveloped viruses
- Influenza virus
- parainfluenza virus
- RSV
- measles virus
- mumps virus
- rubella virus
- rabies virus
- HTLV
- HIV
-
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
-
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
-
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
-
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
-
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
-
HLA DR2
- Multiple sclerosis
- hay fever
- SLE
- Goodpasture's
-
Cell surface proteins of B cells for EBV
-
Drugs the prolong AT interval
- Macrolides (erythromycin)
- Antimalarials (chloroquine/ mephaquine)
- Haloperidol
- Risperidone
- Methadone
- Protease inhibitors
- Antiarrhythmics (Class IA)
- K channel blockers
-
What cardiac valve defect is associted with DiGeorge's syndrome
- Tetralogy of Fallot
- Transposition of the great vessels
-
What stimulates release of prolactin
- Dopamine antagonists (antipsychotics)
- estrogens (OCPs, pregnancy)
- TRH
-
Why is Hydralazine good for patients with Mitral Regurgitation?
They decrease afterload -> increase in foward stroke volume and a decrease in regurgitant flow.
-
Side effects of niacin
- Flushing
- vasodilation
- insulin resistance
- hyperuricemia
-
Which murmurs increase with inspiration
Right sided murmurs
-
What substances have nuclear receptors
- Thyroid hormones
- retinoids
- PPAR
- Fatty acid
-
How do ACE inhibitors contribute to cough
- Block degradation of bradykinin and substance P
- -> lung irritation
-
What seurm labs would you see with primary adrenal insufficiency
- decreased Na and Cl-
- increased K+
- metabolic acidosis (dec HCO3-)
-
B type natriuretic peptide
From the ventricles in response to hypertrophy via volume overload
-
What is the cause of tetralogy of fallot
Abnormal neural crest cell migration
-
Atenolol actions
- B1 only:
- decreased cAMP in heart (dec contractility and HR)
- decreased cAMP in JG cells
-
Imprinting enzyme
- DNA methylation via methyltransferase
- same enzyme used in the SAM reaction
-
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
-
What cholesterol lowring drugs are responsible for myositis when combined
Statins and fibrates(gemfibrozil)
-
What is the recommended treatment for Staph epidermidis valve endocarditis
Vancomycin with Rifampin and/or gentamicin
-
What is the effect of B-blockers and verapamil combined?
Very low heart rate (negative chronotropy)
-
Lipofuscin
insoluble pigment consisting of lipid polymers and protein complexed phospholipid) that come from free radical injury and lipid peroxidation
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