56 y/o F comes to physician 3 wk hx increasing weakness of arms and legs.
- Smoking
- Weakness of hip girdle muscles, lesser weakness of shoulder girdle
... [Show More] muscles.
- Weakness improves with repetitive testing.
- Hilar lung mass
What's impaired:
- Acetylocholinesterase
- Binding of ACh on postsynaptic membrane
- Direct depolarization of muscle by Ca
- Postsynaptic membrane potential
- Presynaptic release of ACh
Presynaptic release of ACh
- Lambert Eaton myasthenic syndrome (ab to voltage-gated calcium channels on presynaptic LMNs, which decreased calcium influx and ACh release to postsynaptic SkM cells
23 y/o F 1 wk history of intermittent fever, chills, rash
- Hx 4 operations (1) scoliosis, (2) tonsillectomy, (3) appendectomy, (4) lipoma.
- Hx admission for N/V
- Dermabrasion to correct acne scarring
- PE: 12 evenly spaced punctate marks in linear patten on abdomen and UE, LE
Diagnosis?
- AML
- Factitious d/o
- ITP
- Lyme disease
- Syphilis
Factitious disorder
- patients produced symptoms for primary gain. Primary gain is motivation to be cared for, which constitutes unconscious motivator in patients production of symptoms. "Care seeking"
tx - regular f/u with one physician who oversees management & psychotherapy
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An investigator is studying the adverse effects of a proteasome inhibitor, bortezomib, on immune responses. Administered to group of pts with relapsed MM and immune response observed. Which immunologic processes affected by drug?
- Activation of complement cascade
- Activation of perforin
- Presentation of antigens to CD8+ T lymphocytes
- Secretion of histamine by mast cells
- Secretion of IL-1 by macrophages
Presentation of antigens to CD8+ T lymphocytes
- Proteasomes ar elarge proteases with numerous domains that ar epresent in cytoplasm adn nuceli. Made of alpha nd beta subunits and have extensive role in cell cycle regulations and immune function. Caspase, chymotrypsin, and trypsin-like protease capabilities of this enzyme complex are critical to their role as APC; intracellular peptides are processed in teh proteasomme for presentation on Class I MHC. Which are regogized by CD8 T lympocytes.
12 y/o girl sliced her left palm with a knife. Would is cleaned and suture. One week later, sutures are removed. At this time, which of the following factors is most instrumental in migration fo fibroblasts into area of would healing?
- collagen
- fibronectin
- heparin
- IG
- plasminogen
Fibronectin.
Collagen is then synthesized by the fibroblasts.
What is the most common brain tumor in adults?
Meningiomas - most common primary brain tumor. Patients may be asymptomatic or experience seisures or focal neurologic deficits related to tumor locaiton.
Malignant ones may grow rapidly - leading to symptoms of ICP
Glioblastoma
highly malignant brain tumor in intraaxial mass in cerebral hemispheres, which typically demonstrate areas of necrosis and can spread to opposite hemisphere across corpus callosum.
infant understanding of death
zero
4 year old understanding of death
temporary and reversible
20 y/o man with 4hr history abd pain, N/V. Binge drinking all weekend. Three doses of tylenol for HA. Increased risk for liver injury why?
Induction of CYP450 that activate acetaminophen to a hepatotoxic metabolite.
Ethanol lead sto induction of P450 enzymes that result in increased production of NAPQI when high doses of tylenol taken.
depletin of glutathione. give NAC.
translocations
balanced - no significant consequence unless the break point affects a regulatory sequence or section of an exam that results in reduced or absent translation of encoded protein.
regulatory sequences: promoters, enhancers, silencers
Frank Starling mechanism
relationship between cardiac output and LVEDV - preload.
CHF - line falls down
AVM - increased CO per this relationship
Tamponade - decreased ventricular filling but no change on this curve
Cor pulmonale - RVF from chronic pulm HTN no change on this curve
Restrictive cardiomyopathy - decreased compliance of ventricular wall disatolic dysfunction and preserved LV systolic function (CO would be preserved)
renal autoregulation
relatively constantly GFR despite change sin glomerular perfusion and is mediated by delivery of filtered sodium to macula densea in ealry DCT. in Renal a. stenosis, there is a decreased glomerular perfusion --> decreased filtration and decreased sodium delivery to macula densa. COmpensates by promoting activatoin of RAASD system --> constriction of efferent arteriolar. Induces increased intraglomerular pressure to maintain GFR.
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Round ligament
fibromusclar band of tissue that extends from superolateral part of uterus where fallopian tubes insert, passes through the inguinal canal, ends in labia majora. remnant of gubernaculum. this is stretched in preganancy --> pain
an investigator is conducting a study of the effetcs of a newly discovered spider venom on NM transmission. Isolated nerve muscle prepartion is bathed in a solution containing the venom; there is a decreased in teh end-plate potential amplitude following stimulation of the nerve. Presence of venom does not change the amplitude of the nerve AP or the repsosne to exogenous acetylcholine. BLockage of which fo the following is the MOA?
- Chloride conductance
- Function of acetylocholinesterase
- Inactiavte of voltage-gated Na channels in muscle
- Insertion of AChR into muscle membrane
- Presynaptic, voltage-gated calcium channels
Presynaptic, voltage-gated calcium channels
decreased end-plate potential amplitude.
normallY: AP --> depolarization of terminal bouton of axon --> presynaptic voltage-gated Ca channels consequently open and allow calcium influx which tiggers exocytosis of ACh filled vesicle sinto synaptic cleft.
brief psychotic disorder
characterized by acute onset of one or more psychotic symptoms (delusions, hallucinations disorganized speech, d/o behavior) lasting less that one month.
schizoaffective = 6 m/o
vasopressin is made in
the paraventricular and supraoptic nuclei
transformation required ____ DNA
naked (not a/w proteins/lipids/molecules)
Waardenburg syndrome
syndrome of pathy depigmentation of skin, ahir, irises, cochlear dyscuntion
AD
neural crest cells do not properly differentiare into melanoblasts
- white forelock, eyelashes, depigmented skin patches, iridic heterochromia, sensorineural deafness, eyes may be laterally displaced
Glucose-dependent insulinotropic peptide (GIP)
secreted by duodenum & jejunum (K cells), paracrine and classical signaling to pancreas. Stimulates insulin secretion, triggered by glucose in the duodenum.
to decreased gastric acid production and stimulate insulin release from pancrease
RR = x per 10,000 / y per 10,000
RR = x/y
obese vs. non obese same drug administered
greater volume of distribution os takes longer ot get to therapeutic dose
pores of Kohn
connections between alveoli and are present in normla lung tissue. type II alveolar cell sand llow for the passage of air, gluid, phagocytes, and in the setting of pneumonia, bacteria between adjacent alveoli
PKU patient supplement with what for catecholamine production
tyrosine
(can't convert phenylalanine to tyrosine)
PO2 in the renal vein is high compared with venous PO2 from other organs. What explains this finding?
blood flow per gram of tissue is greater in kidneys than in other organs
it is in excess of what is needed to meet its metabolic demands
Gerstmann syndrome
Angular gyrus lesion
finger tap agnosia, alexia (inability to read), acalculia (inability to use arithmetic operations), agraphia (inability to copy), and left-right confusion.
Dejerine-Roussy syndrome
dorsolateral thalamus stroke
connects wiht the limbic system adn may mediate emotional aspects of pain. post-stroke intractable pain known as thalamic pain syndrome
Wallenberg syndrome
lower medulla (lesions may affects nuceli of multiple CNs, spinal trigeminal nucleus, solitary nucleus, vestibular nuclei, nucleus ambiguus, the sympathetic, spinothalamic, and spinocerebellar tracts. ipsilateral facial numbness, impaired taste sensation, vertigo with nystagmus, dysphonia, dysgia, Horner syndrome, pain and temperatiev sensation deficits in the contralateral arms and legs and/or ipslateral ataxia. constellation of symptoms is wallenberg syndrome
Weber syndrome
lower midbrain
paramedian branchs of PCA affect the cerebral peduncle contianing the corticospinal tract and neighboring oculomotor nerve. Diplopia, ptosis, primary gaze palsy, popully constriction deficits) and contralalteral hemiparesis with UMN patter of dysfuntion.
Horner syndrome
upper spinal cord
sympathetic nerve supply to ipsilateral face and eye - ipsilateral ptosis, miosis, anhidrosis.
malignant mesothelioma
chronic cough, SOB, pleural effusion + calcified pleural plague a/w asbestosis (working in shipyard, roofing, plumbing)
exudative pleural effusion caused by the malignant pleural disease
leuprolide for prostate cancer
decreased LH and testosterone
GnRH analon - contuned non-pulsatile fasino GnRH R antagonist
androgen insensitivity syndrome
caused by defect in androgen receptor complex resulting genotypic XY male to develop external demale or ambiguous genitalia and femal esecondary sex characterisitics. testes are present.
increased concentrations of testosterone, estrogen, and LH (no uterus or ovaries)
chronic hypertension on kidneys
hyperplastic arteriolitis
marasmus
acute protein energy malcutrition = decrease din mid-upper arm circumderence, weight for length, BMI. maintain linear growth because weight is more quickly ipacted than height.
- deficient caloric intade but no specfic vitamins or minerals are missing. wasing of mucle mass and consumption of body fat stores
NO EDEMA
what produces progesterone in pregnancy
corpus luteum to maintian the endometrial lining slowly, the placenta that begins to secrete HCG which then maintains the corpus letum and its secretion of progesterone. as the placenta develops it becomes primarily repsonisble for progesterone production around seven to ten weeks of gestation.
HIV encephalopathy
most likely in AIDS patient wiht progressive memory loss and motor deficits who does does not adhere to antiretroviral therpay. diagnosis of exclusion in patients with HIV
movement disorders
psychomotor impairment
memory deficits
poorly demarcated T2 hyperintense lesion scattered in subcortical white amtter.
brain biopsey: microglial nodules iwth multinuclearted giant cells
acromegaly glucose regulation
decreased muscle glucose uptake
increaed adipose lipolysis
increased hepatic gluconeogenesis
** increase dperipherla insulin resistnace impairing muscle and adipose uptake of glucose, increasing adipose lipolysis, increasing hepatic gluconeogenesis
white vs. black skin pigmentation
difference in number of melanosomes not the number of melanocytes themselvs
URI infection clearance is most dependent on what
class I MHC viral peptide complex presentation and subsequent activation of CD8+ T lymphocytes
40 year old man evaluated because skin etremely sensitivy to sunlight. -->> vesciles and blisters on skin which take weeks to heal which biochemical pathwya is most defective in patient
heme synthesis
porphyria cutanea tarda - severre cutaneous photosentivity wiht blistering and hyperpigmentation after exposure to sunlight and is most common of the porphyrias. decreaed acitvity of uroporphyringogen decarboxylase. which is also used in the production of heme.
no OCPs in
people who smoke
PEEP is for
preventing absorption atelectasis - keeps alveoli stented open to prevent impaired gas exchange (further)
Muir-Torre syndrome
hereditary nonpolyposis CC affected individuals are prone to developing colon, genitourinary, skinc ancer, keratoacanthomas and sebaceous glad tumors
Peutz-Jeghers syndrome
AD
hamartomatous polyps in colon/GI tract and pigmented macules i hte mouth/lips/hands/genitalia
cowden disease
AD polyposis a/e hamartomatous polyps in GI tract. a/w mucocutaneous neuromas, oral papillomas, and cutaneous trichilemmomas.
clamp coronary artery first hting that's going to happen
swelling of endoplasmic reticulum (swelling fo the cell and its organelles is the first cellular change that occurs after cessation fo blood flow from shut down fo NA/K ATPase which results intracellular accumulation of sodium which shifts the osmotic graident and leads to cellular swelling
Zellweger syndrome
genetic mutations that leads to absense of peroxisomes - increased VLACFA, seizures, ID< hypotonia, hepatomegaly, jaundice, kidney diease, cataracts, hearing loss,a dn craniofacial abormalities
allosteric activator
if the concentration of substrate at one-half of V-max decreases in presence of a substance than that substance must icnreased the affnity of the enzyme for its substrante - results froma conformational change in the active site of the enzyme where the substrate binds.
catabolite acitvator/respressor
transciption of genes is affected. [Show Less]